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TREATMENT OF SOFT TISSUE SARCOMA
IN TURKEY

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1. SOFT TISSUE SARCOMA IN AN ADULT
1.1. General information about soft tissue sarcoma

1.2. Risks of developing sarcoma in adults 

1.3. Sarcoma Symptoms

1.4. Diagnosis of Sarcoma

1.5. Factors affecting the chance of recovery 

1.6. Stages of soft tissue sarcoma in adults

1.7. Overview of treatment options
 

2. TREATMENT OF STROMAL TUMORS OF THE GASTROINTESTINAL TRACT (adults)
2.1. Risks of developing a stromal tumor of the gastrointestinal tract

2.2. Symptoms of the development of a stromal tumor of the gastrointestinal tract

2.3. Diagnostics of the stromal tumor of the gastrointestinal tract

2.4. Recovery prognosis

2.5. Overview of treatment options
 

3. TREATMENT OF STROMAL TUMORS OF THE GASTROINTESTINAL TRACT IN CHILDREN 
3.1.
  Symptoms

3.2 Diagnostics

3.3. Overview of treatment options
 

4. TREATMENT OF KAPOSHA'S SARCOMA
4.1. Diagnostics

4.2. Factors affecting the prognosis of recovery

4.3. Classic Kaposi's sarcoma

4.4. Epidemic Kaposi's sarcoma (HIV-associated Kaposi's sarcoma)

4.5. Overview of treatment options

 

5. TREATMENT WITH RABDOMOISARCOM IN CHILDHOOD 
5.1. Risks of developing rhabdomyosarcoma in children

5.2. Symptoms

5.3. Diagnostics

5.4. Factors influencing the forecast

5.5. Stages of childhood rhabdomyosarcoma

5.6. Overview of treatment options

5.7. Side Effects of Treatment

5.8. Treatment of childhood rhabdomyosarcoma

5.9. Treatment of progressive or recurrent childhood rhabdomyosarcoma

 

6. TREATMENT OF SOFT TISSUE SARCOMA  CHILDREN 
6.1. Risks of developing sarcoma of myanic tissues in children

6.2. Symptoms 

6.3. Diagnostics 

6.4. Types of soft tissue sarcoma tumors in children

6.5. Factors affecting the prognosis of recovery

6.6. Overview of treatment options  infantile soft tissue sarcoma

6.7. Treatment for soft tissue sarcoma in children can cause side effects.

6.8. Treatment options for soft tissue sarcoma in children

 

7. TREATMENT OF VASCULAR TUMORS IN CHILDREN 
7.1. Tests are used to detect (search) and diagnose vascular tumors in children.

7.2. Groups of vascular tumors in children

7.3. Overview of treatment options

7.4. Benign tumors

7.5. Intermediate tumors that spread locally

7.6. Intermediate tumors that rarely spread

7.7. Malignant tumors

1. SOFT TISSUE SARCOMA IN AN ADULT

Soft tissue sarcoma is a broad term for cancers that begin in soft tissues (muscles, tendons, fat, lymphatic and blood vessels, and nerves). These cancers can develop anywhere in the body, but are more common in the arms, legs, chest, and abdomen. Explore the links on this page to learn more about the different types of soft tissue sarcomas and how to treat them. We also have information on research and clinical trials.

1.1. General information about soft tissue sarcoma

Adult soft tissue sarcoma is a disease in which malignant (cancerous) cells form in the soft tissues of the body.

The soft tissues of the body include muscles, tendons (bands of fibers that connect muscles to bones), fat, blood vessels, lymphatic vessels, nerves, and tissues around joints. Soft tissue sarcomas in adults can form almost anywhere in the body, but are most common in the head, neck, arms, legs, trunk, abdomen, and retroperitoneal space.

1.1.
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Soft tissue sarcoma forms in the soft tissues of the body, including muscles, tendons, fat, blood vessels, lymph vessels, nerves, and tissues around joints.

There are many types of soft tissue sarcomas. In each type of cell, sarcomas look different under a microscope, based on the type of soft tissue in which the cancer started.

1.2. Risks of developing sarcoma in adults  

Having certain inherited conditions can increase the risk of soft tissue sarcoma in adults.

Anything that increases the risk of a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; the absence of risk factors does not mean that you will not get cancer. Talk to your doctor if you think you are at risk. Risk factors for soft tissue sarcoma include the following inherited conditions:

  • Retinoblastoma.

  • Neurofibromatosis type 1 (NF1; von Recklinghausen's disease).

  • Tuberous sclerosis (Bourneville disease).

  • Familial adenomatous polyposis (FAP; Gardner's syndrome).

  • Li-Fraumeni syndrome.

  • Werner's syndrome (progeria in adults).

  • Nevoid basal cell carcinoma syndrome (Gorlin's syndrome).

 

Other risk factors for soft tissue sarcoma include the following:

  • Past radiation therapy for certain cancers.

  • Exposure to certain chemicals such as thorotrast (thorium dioxide), vinyl chloride, or arsenic.

  • Swelling (lymphedema) of the hands or feet for a long time.

1.3. Sarcoma Symptoms

A sign of adult soft tissue sarcoma is a lump or swelling in the soft tissues of the body.

A sarcoma can present as a painless mass under the skin, often on an arm or leg. Sarcomas that start in the abdomen may not cause signs or symptoms until very large. As the sarcoma grows larger and puts pressure on nearby organs, nerves, muscles, or blood vessels, signs and symptoms may include:

  • Pain.

  • Labored breathing.

 

Other conditions can cause the same signs and symptoms. Check with your doctor if you have any of these problems.

1.4. Diagnosis of Sarcoma

Adult soft tissue sarcoma is diagnosed by biopsy.

If your doctor thinks you may have soft tissue sarcoma, a biopsy will be done. The type of biopsy depends on the size of the tumor and its location in the body. Three types of biopsies can be used:

  • Postoperative biopsy: Removal of part of a tumor or tissue sample.

  • Primary biopsy: Removal of tissue with a wide needle.

  • Excisional biopsy: Removal of an entire tumor or area of tissue that does not appear normal.

 

Samples will be taken from the primary tumor, lymph nodes, and other suspicious areas. The pathologist examines the tissue under a microscope for the presence of cancer cells and finds out the grade of the tumor. The extent of the tumor depends on how abnormal the cancer cells look under the microscope and how quickly the cells divide. High-grade tumors usually grow and spread faster than low-grade tumors.

Because soft tissue sarcoma is difficult to diagnose, patients should ask that tissue samples be screened by a pathologist experienced in diagnosing soft tissue sarcoma.

The following tests can be performed on the removed tissue:

  • Immunohistochemistry:  a laboratory test that uses antibodies to check for specific antigens (markers) in a tissue sample from a patient. Antibodies are usually associated with an enzyme or fluorescent dye. After antibodies bind to a specific antigen in a tissue sample, the enzyme or dye is activated and the antigen can be seen under a microscope. This type of test is used to help diagnose cancer and to help distinguish one type of cancer from another.

  • Light and electron microscopy: A laboratory test in which cells in a tissue sample are viewed under a conventional and high-power microscope to detect specific changes in the cells.

  • Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a tissue sample are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in some chromosomes can be a sign of cancer. Cytogenetic analysis is used to diagnose cancer, plan treatment, or determine the effectiveness of a treatment.

  • FISH (Fluorescence In Situ Hybridization): A laboratory test used to study and count genes or chromosomes in cells and tissues. Pieces of DNA containing fluorescent dyes are made in the laboratory and added to a sample of the patient's cells or tissues. When these stained DNA fragments attach to specific genes or regions of chromosomes in a sample, they light up when viewed under a fluorescent microscope. The FISH test is used to diagnose cancer and plan treatment.

  • Flow cytometry: A laboratory test that measures the number of cells in a sample, the percentage of living cells in the sample, and certain characteristics of cells such as size, shape, and the presence of tumor (or other) markers on the cell surface. Cells from a sample of blood, bone marrow, or other tissue from a patient are stained with a fluorescent dye, placed in a liquid, and then passed one at a time through a beam of light. The test results are based on how cells stained with a fluorescent dye respond to a beam of light.

After a soft tissue sarcoma in an adult is diagnosed, tests are done to find out if the cancer cells have spread to the soft tissue or to other parts of the body.

The process used to determine if cancer has spread to soft tissue or to other parts of the body is called staging. The stage of soft tissue sarcoma also depends on the extent and size of the tumor and whether it has spread to the lymph nodes or other parts of the body. The information gathered during the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.

During the preparation process, the following tests and procedures can be used:

  • Physical examination and health history: Examining the body to check for general signs of health, including checking for signs of illness, such as tumors or anything else that seems unusual. A history of the patient's health-related habits as well as past illnesses and treatments will also be recorded.

  • Chest X-ray: An X-ray of organs and bones inside the chest. X-rays are a type of energy beam that can pass through the body onto film, creating an image of areas within the body.

  • Blood chemistry tests: A procedure in which a blood sample is tested to measure the amount of certain substances that enter the bloodstream by organs and tissues of the body. An unusual (more or less than usual) amount of a substance may be a sign of illness.

  • Complete blood count (CBC): A procedure in which a blood sample is taken and checked for the following:

    • The number of erythrocytes, leukocytes and platelets.

    • The amount of hemoglobin (oxygen-carrying protein) in red blood cells.

    • The portion of a blood sample made up of red blood cells.

  • CT (computed tomography): A procedure in which a series of detailed pictures of the internal parts of the body, such as the lungs and the abdomen, are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to help organs or tissues show more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • PET scan (positron emission tomography): a procedure to look for malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into the vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Malignant tumor cells appear brighter in the image because they are more active and consume more glucose than normal cells.

 

The results of these tests are reviewed along with the results of the tumor biopsy to determine the stage of the soft tissue sarcoma prior to treatment. Sometimes chemotherapy or radiation therapy is prescribed as the initial treatment, and then the soft tissue sarcoma is re-inserted.

1.5. Factors affecting the chance of recovery  

Several factors influence treatment options and prognosis (chance of recovery).

Treatment options and prognosis depend on the following:

  • A type of soft tissue sarcoma.

  • The size, grade and stage of the tumor.

  • How quickly cancer cells grow and divide.

  • Where is the tumor in the body.

  • Has the entire tumor been removed?  surgical  by.

  • Patient's age and general health.

  • If the cancer has recurred (come back).

1.6. Stages of soft tissue sarcoma in adults

The grade of the tumor is also used to describe the cancer and the treatment plan.

The grade of the tumor describes how abnormal the cancer cells look under the microscope and how quickly the tumor can grow and spread. Low, medium and high grade are used to describe soft tissue sarcoma:

  • Low grade: In low grade soft tissue sarcoma, cancer cells look more like normal cells under a microscope and grow and spread more slowly than in moderate to high grade soft tissue sarcoma.

  • Moderate: In moderate soft tissue sarcoma, the malignant cells appear more abnormal under the microscope and grow and spread faster than in low-grade soft tissue sarcoma.

  • High Grade: In high-grade soft tissue sarcoma, cancer cells appear more abnormal under the microscope and grow and spread faster than in low to moderate soft tissue sarcoma.

 

For sarcoma of soft tissues of the trunk, arms and legs in adults, the following stages are used:

Stage I

Stage I of adult soft tissue sarcoma of the trunk, arms and legs is divided into stages IA and IB:

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1.3.
1.4.
1.5.
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саркома 1 стадии

Tumor sizes are often measured in centimeters (cm) or inches. Common foods that can be used to display tumor size in cm include: pea (1 cm), peanuts (2 cm), grapes (3 cm), walnut (4 cm), lime (5 cm or 2 cm). inches), egg (6 cm), peach (7 cm) and grapefruit (10 cm or 4 inches).

  • In stage IA, the tumor is 5 centimeters or less and is of low grade or unknown grade.

  • In stage IB, a tumor larger than 5 centimeters is of low grade or unknown grade.

 

Stage II

In stage II adult soft tissue sarcomas of the trunk, arms and legs, the swelling is 5 centimeters or less and middle-class or high-class.

Stage III

Sarcoma of soft tissues of the trunk, arms and legs in stage III adults is divided into stages IIIA and IIIB:

  • In stage IIIA, the tumor is larger than 5 centimeters, but not more than 10 centimeters, and has a moderate to high grade.

  • In stage IIIB, the tumor is larger than 10 centimeters and has a moderate to high grade.

 

Stage IV

With sarcoma of the soft tissues of the trunk, arms and legs in a stage IV adult, one of the following is found:

  • the tumor is of any size, any grade, and has spread to nearby lymph nodes; or

  • a tumor of any size, of any degree, and could have spread to nearby lymph nodes. The cancer has spread to other parts of the body, such as the lungs.

For sarcoma of the soft tissues of the retroperitoneal space in adults, the following stages are used:

 

Stage I

Stage I of adult retroperitoneal soft tissue sarcoma is divided into stages IA and IB:

При саркоме мягких тканей забрюшинного пространства у взрослых используются следующие стадии:

Tumor sizes are often measured in centimeters (cm) or inches. Common foods that can be used to display tumor size in cm include: pea (1 cm), peanuts (2 cm), grapes (3 cm), walnut (4 cm), lime (5 cm or 2 cm). inches), egg (6 cm), peach (7 cm) and grapefruit (10 cm or 4 inches).

  • In stage IA, the tumor is 5 centimeters or less and is of low grade or unknown grade.

  • In stage IB, a tumor larger than 5 centimeters is of low grade or unknown grade.

Stage II

In stage II adult retroperitoneal soft tissue sarcoma, the tumor is 5 centimeters or less and middle class or high class.

Stage III

Stage III retroperitoneal soft tissue sarcoma in adults is divided into stages IIIA and IIIB:

  • In stage IIIA, the tumor is larger than 5 centimeters, but not more than 10 centimeters, and has a moderate to high grade.

  • Stage IIIB shows one of the following:

    • tumors larger than 10 centimeters and middle grade or high grade; or

    • a tumor of any size, of any degree, and has spread to nearby lymph nodes.

 

Stage IV

In stage IV adult retroperitoneal soft tissue sarcomas, the tumor is of any size, any grade, and can spread to nearby lymph nodes. The cancer has spread to other parts of the body, such as the lungs.

There is no standardized staging system for soft tissue sarcomas in the head, neck, chest, or abdomen.

Soft tissue sarcoma may recur (come back) after treatment.

Cancer can return to the same soft tissue or to other parts of the body.

1.7. Overview of treatment options

1.7.
Хирургия Мооса.

Mohs Surgery. A surgical procedure to remove a visible lesion on the skin in several stages. First, a thin layer of cancerous tissue is removed. Then a second thin layer of tissue is removed and viewed under a microscope to check for cancer cells. Several layers are removed one at a time until the remaining cancer is found on the tissue viewed under a microscope. This type of surgery is used to remove as little healthy tissue as possible.

1. Wide local excision: removal of the tumor along with the normal tissue around it. For tumors of the head, neck, abdomen and trunk, as little normal tissue as possible is removed.

  • Limb-sparing surgery: Removal of a tumor on an arm or leg without amputation, while maintaining the use and appearance of the limb. Radiation therapy or chemotherapy may be given first to shrink the tumor. Then the tumor is removed by wide local excision. The removed tissue and bone can be replaced with a graft using tissue and bone taken from another part of the patient's body, or with an implant such as artificial bone.

  • Amputation: Surgery to remove part or all of a limb or appendage, such as an arm or leg. Amputation is rarely used to treat soft tissue sarcoma of the arm or leg.

  • Lymphadenectomy: A surgical procedure in which lymph nodes are removed and a sample of tissue is checked under a microscope for signs of cancer. This procedure is also called lymphadenectomy.

 

Radiation therapy or chemotherapy may be given before or after surgery to remove the tumor. If given before surgery, radiation therapy or chemotherapy will shrink the tumor and reduce the amount of tissue that needs to be removed during surgery. Treatment before surgery is called neoadjuvant therapy. When given after surgery to remove all visible tumor, radiation therapy or chemotherapy kills any remaining cancer cells. Treatment given after surgery to reduce the risk of cancer recurrence is called adjuvant therapy.

2. Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy X-rays or other types of radiation to kill cancer cells or prevent them from growing. There are two types of radiation therapy:

  • External beam therapy  uses a device outside the body to direct radiation to an area of the body affected by cancer.

  • Internal beam therapy  uses a radioactive substance sealed in needles, seeds, wires, or catheters that are inserted directly into or near the tumor.

 

Intensity modulated radiation therapy (IMRT) is a type of three-dimensional (3-D) radiation therapy that uses a computer to produce images of the size and shape of a tumor. Thin beams of radiation of different intensity (strength) are directed at the tumor at different angles. This type of external radiation therapy causes less damage to nearby healthy tissue and is less likely to cause dry mouth, trouble swallowing, and skin damage.

How radiation therapy is given depends on the type and stage of cancer treatment. External radiation therapy and internal radiation therapy can be used to treat soft tissue sarcoma in adults.

3. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by either killing the cells or stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).

4. Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and target certain cancer cells. Targeted therapy can do less harm to normal cells than chemotherapy or radiation therapy.

  • Tyrosine kinase inhibitor therapy: Tyrosine kinase inhibitors are small molecule drugs that travel across the cell membrane and work inside cancer cells, blocking the signals that cancer cells need to grow and divide. Some tyrosine kinase inhibitors also have angiogenesis inhibitory effects. Pazopanib is used to treat advanced soft tissue sarcoma.

2. TREATMENT OF STROMAL TUMORS OF THE GASTROINTESTINAL TRACT (adults)

A stromal tumor of the gastrointestinal tract is a disease in which abnormal cells form in the tissues of the gastrointestinal tract.

The gastrointestinal (GI) tract is part of the body's digestive system. It helps digest food and removes nutrients from food (  vitamins,  minerals,  carbohydrates, fats, proteins and water) so that they can be used by the body. The gastrointestinal tract consists of the following organs:

  • Stomach .

  • Small intestine .

  • Colon (  colon ).

 

Some gastrointestinal stromal tumors (GIST) grow slowly over time and may never cause problems for the patient, while others can grow and spread very quickly. They are most commonly found in the stomach and small intestine, but can be found anywhere in or near the gastrointestinal tract. Some scientists believe that GIST begins in cells called interstitial Cajal cells (ICCs) in the wall of the gastrointestinal tract.

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Сторомальные опухоли желудочно-кишечного тракта

Gastrointestinal stromal tumors (GIST) can be found anywhere or near the gastrointestinal tract.

2.1. Development risks  stromal tumor of the gastrointestinal tract

Genetic factors can increase the risk of developing gastrointestinal stromal tumors.

Anything that increases the risk of a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; the absence of risk factors does not mean that you will not get cancer. Talk to your doctor if you think you are at risk.

These genes in cells carry hereditary information received from a person's parents. The risk of GIST is increased in people who are inherited with a mutation (change) in a particular gene. In rare cases, GIST can be found in several members of the same family.

GIST can be part of a genetic syndrome, but this is rare. A genetic syndrome is a collection of symptoms or conditions that occur together and are usually caused by abnormal genes. The following genetic syndromes are associated with GIST:

  • Neurofibromatosis type 1 (NF1)

A rare genetic disorder that causes brown spots and tumors on the skin, freckles on areas of the skin not exposed to the sun, nerve tumors, and changes in the development of the nervous system, muscles, bones, and skin. Also called NF1.

  • Carney's triad.

A very rare disease characterized by tumors of the gastrointestinal tract (usually the stomach), tumors that form in the nervous tissue of the embryo in the head, neck and trunk, and tumors that form in the cartilage of the lungs. Sometimes tumors also form in the adrenal glands and esophagus. The Carney triad is most common in young women.

2.2. Symptoms of the development of a stromal tumor of the gastrointestinal tract

Signs of gastrointestinal stromal tumors include blood in the stool or vomiting.

These and other signs and symptoms can be caused by GIST or other causes. Check with your doctor if you have any of the following:

  • Blood (bright red or very dark) in stool or vomit.

  • Abdominal pain, which can be very severe.

  • Feeling very tired.

  • Problems or pain when swallowing.

  • Feeling full after eating very little food.

2.3. Diagnostics  stromal tumor of the gastrointestinal tract

Tests that examine the gastrointestinal tract are used to diagnose tumors of the gastrointestinal stroma.

The following tests and procedures can be used:

  • Physical examination and health history: Examining the body to check for general signs of health, including checking for signs of illness such as bumps or anything else that seems out of the ordinary. There will also be a history of the patient's health habits, as well as past illnesses and treatments.

  • CT (computed tomography): a procedure in which a series of detailed pictures of areas inside the body are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to make organs or tissues show more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • Endoscopic ultrasound and biopsy:  Endoscopy and ultrasound are used to take an image of the upper gastrointestinal tract and a biopsy is performed. An endoscope (a thin, tubular instrument with light and viewing lenses) is inserted through the mouth and into the esophagus, stomach, and first part of the small intestine. A probe at the end of an endoscope is used to reflect high-energy sound waves (ultrasound) from internal tissues or organs and create an echo. The echo forms a picture of body tissue called a sonogram. This procedure is also called endosonography. Based on the sonogram, the doctor removes the tissue with a thin hollow needle. A pathologist examines the tissue under a microscope for cancer cells.

When cancer is found, the following tests can be performed to look at cancer cells:

  • Immunohistochemistry:  a laboratory test that uses antibodies to check for specific antigens (markers) in a tissue sample from a patient. Antibodies are usually associated with an enzyme or fluorescent dye. After antibodies bind to a specific antigen in a tissue sample, the enzyme or dye is activated and the antigen can be seen under a microscope. This type of test is used to diagnose cancer and help distinguish one type of cancer from another.

  • Mitosis rate: A measure of how quickly cancer cells divide and grow. The rate of mitosis is determined by counting the number of cells dividing in a given amount of cancer tissue.

Very small GISTs are common.

Sometimes GIST is less than a pencil eraser. Tumors can be found during a procedure that is done for another reason, such as an X-ray or surgery. Some of these small tumors will not grow and cause signs or symptoms or spread to the abdomen or other parts of the body. Doctors disagree on whether these small tumors should be removed or monitored to see if they begin to grow.

After a gastrointestinal stromal tumor has been diagnosed, tests are done to find out if cancer cells have spread through the gastrointestinal tract or other parts of the body.

The process used to determine if a cancer has spread in the gastrointestinal (GI) tract or to other parts of the body is called staging. The information gathered during the staging process determines the stage of the disease. During the preparation process, the following tests and procedures can be used:

  • PET scan (positron emission tomography): a procedure to look for malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Malignant tumor cells appear brighter in the image because they are more active and consume more glucose than normal cells.

  • CT (computed tomography): a procedure in which a series of detailed pictures of areas inside the body are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed so that organs or tissues can be seen more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • Chest X-ray: An X-ray of the internal organs and bones of the chest. An X-ray is a type of energy beam that can pass through a body onto a film, creating an image of areas within the body.

  • Bone scan: A procedure to check for rapidly dividing cells such as cancer cells in the bone. A very small amount of radioactive material is injected into a vein and passed through the bloodstream. The radioactive material builds up in the bones in cancer and is detected by a scanner.

Additional tests may be required.

Some tests that have been done to diagnose cancer or to determine the stage of cancer may be repeated. Some tests will be repeated to see how effective the treatment is. Decisions to continue, change, or stop treatment may be based on the results of these tests.

Some tests will continue from time to time after treatment ends. The results of these tests can show if your condition has changed or if the cancer has recurred (returned). These tests are sometimes called follow-up or follow-up examinations.

Follow-up for GST that have been surgically removed may include CT of the liver and pelvis or wait-and-see. For GISTs that are treated with tyrosine kinase inhibitors, follow-up tests such as CT, MRI, or PET scans may be done to check how well targeted therapy is working.

2.4. Recovery prognosis

Several factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment option depends on the following conditions:

  • How quickly cancer cells grow and divide.

  • The size of the tumor.

  • Where is the tumor in the body.

  • Is it possible to completely remove the tumor with surgery?

  • Whether the tumor has spread to other parts of the body.

2.5. Overview of treatment options

The results of diagnostic and staging tests are used to plan treatment.

For many cancers, it is important to know the stage of the cancer in order to plan treatment. However, GIST treatment is independent of the stage of the cancer. Treatment is based on whether the tumor can be surgically removed and whether the tumor has spread to other parts of the abdomen or distant parts of the body.

Treatment is based on whether the tumor is:

  • Resectable: These tumors can be removed with surgery.

  • Inoperable: These tumors cannot be completely removed with surgery.

  • Metastatic and recurrent: Metastatic tumors have spread to other parts of the body. Recurrent tumors recurred (returned) after treatment. Recurrent GISTs may return to the gastrointestinal tract or to other parts of the body. They are usually found in the abdomen, peritoneum, and / or liver.

  • Refractory: These tumors did not improve after treatment.

There are various treatments for patients with gastrointestinal stromal tumors.

Various treatments are available for patients with gastrointestinal stromal tumors (GIST). Some treatments are standard (currently used) and some are in clinical trials. A clinical trial of treatment is a scientific study designed to help improve current treatments or provide information about new treatments for patients with cancer. When clinical trials show that a new treatment is better than standard treatment, the new treatment may become the standard treatment. Patients may want to take part in a clinical trial. Some clinical trials are only open to patients who have not yet started treatment.

Four types of standard treatments are used:

1. Operation

If the GIST has not spread and is in a safe place for surgery, the tumor and some tissue around it may be removed. Sometimes surgery is done using a laparoscope (a thin, illuminated tube) to look inside the body. Small incisions (incisions) are made in the abdominal wall, into one of which a laparoscope is inserted. Instruments can be inserted through the same incision or through other incisions to remove organs or tissues.

2. Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and target certain cancer cells. Targeted therapy usually does less damage to normal cells than chemotherapy or radiation therapy.

Tyrosine kinase inhibitors (TKIs) are targeted therapies that block signals required for tumor growth. TKIs can be used to treat GISTs that cannot be surgically removed, or to shrink the GISTs so that they are small enough to be surgically removed. Imatinib mesylate and sunitinib are two BCIs used to treat GIST. TKI is sometimes prescribed until the tumor grows and serious side effects occur.

 

3. Watchful waiting

Expectant closely monitor the patient's condition without giving any treatment until signs or symptoms appear or change.

 

4. Supportive therapy

If GIST worsens during treatment or side effects occur, supportive care is usually given. The goal of supportive care is to prevent or treat the symptoms of the disease, the side effects caused by the treatment, and the psychological, social and spiritual problems associated with the disease or its treatment. Supportive care helps improve the quality of life for patients with serious or life-threatening medical conditions. Radiation therapy is sometimes prescribed as supportive therapy to relieve pain in patients with large, spreading tumors.

Treatment of resectable stromal tumors of the gastrointestinal tract

 

Resectable gastrointestinal stromal tumors (GIST) can be completely or almost completely removed by surgery. Treatment may include the following:

  • Surgery to remove tumors measuring 2 centimeters or more. Laparoscopic surgery can be done if the tumor is 5 cm or less. If there are cancer cells remaining at the edges of the area where the tumor was removed, careful waiting or targeted therapy with imatinib mesylate may follow.

  • Clinical study on targeted therapy with imatinib mesylate after surgery to reduce the chance of tumor recurrence (return).

Treatment of inoperable stromal tumors of the gastrointestinal tract

 

Inoperable FSW cannot be completely removed by surgery because they are too large, or in a location where there would not be too much damage to nearby organs if the tumor is removed. Treatment is usually a clinical study of targeted therapy with imatinib mesylate to shrink the tumor, followed by surgery to remove as much of the tumor as possible.

Treatment of metastatic and recurrent stromal tumors of the gastrointestinal tract

 

Treatments for GISTs that are metastatic (spread to other parts of the body) or recur (come back after treatment) may include the following:

  • Targeted therapy with imatinib mesylate.

  • Targeted sunitinib therapy if tumor begins to grow during imatinib mesylate therapy or if side effects are too severe.

  • Surgery to remove tumors that have been treated with targeted therapy and that are shrinking, stable (not changing), or slightly increased in size. Targeted therapy can be continued after surgery.

  • Surgery to remove tumors for serious complications such as bleeding, a hole in the gastrointestinal tract, a blockage in the gastrointestinal tract, or an infection.

  • Clinical study of a new treatment method.

Treatment of refractory stromal tumors of the gastrointestinal tract

 

Many GISTs treated with a tyrosine kinase inhibitor (TKI) become unresponsive (stop responding) to the drug over time. Treatment is usually a clinical trial with a different TKI or a clinical trial of a new drug.

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2.3.
2.4
2.5.

3. TREATMENT OF STOMAL TUMORS OF THE GASTROINTESTINAL TRACT IN CHILDREN 

Stromal tumors of the gastrointestinal tract are diseases in which malignant (cancerous) cells form in the tissues of the wall of the stomach or intestines.

Gastrointestinal stromal tumors (GIST) usually begin in cells in tissues in the wall of the stomach or intestines. These cells, called Cajal interstitial cells, help food move through the digestive tract.

During childhood, GIST usually occurs in the stomach. It is more common in girls and usually appears during adolescence.

GIST in children is different from GIST in adults. Patients should be visited at centers that specialize in GIST treatment.

Якорь 3
Стромальные опухоли ЖКТ у детей

Gastrointestinal stromal tumors (GIST) are most common in the stomach and small intestine, but can be found anywhere in or near the gastrointestinal tract.

GIST can develop as part of the syndrome.

GIST can occur as part of the following syndromes:

  • Carney triad (GIST, lung chondroma and paraganglioma). A very rare disease characterized by tumors of the gastrointestinal tract (usually the stomach), tumors that form in the nervous tissue of the embryo in the head, neck and trunk, and tumors that form in the cartilage of the lungs. Sometimes tumors also form in the adrenal glands and esophagus. The Carney triad is most common in young women.

  • Carney-Stratakis syndrome (GIST and paraganglioma). A rare hereditary disease characterized by tumors of the gastrointestinal tract and tumors that form in the nervous tissue of the embryo in the head, neck and trunk. Also called the Karni dyad and the Karni-Stratakis dyad.

3.1.  Symptoms

Signs and symptoms of GIST include anemia and abdominal induration.

These and other signs and symptoms can be caused by GIST or other conditions.

Check with your child's doctor if your child has any of the following:

  • Anemia (tiredness, dizziness, fast or irregular heartbeat, shortness of breath, or pale skin).

  • Lump in the abdomen.

  • Intestinal blockage (cramping abdominal pain, nausea, vomiting, diarrhea, constipation, and bloating).

3.2 Diagnostics

Tests that examine the digestive tract are used to diagnose GIST.

The following tests and procedures can be used:

  • Physical examination and health history: Examining the body to check for general signs of health, including checking for signs of illness such as bumps or anything else that seems out of the ordinary. There will also be a history of the patient's health habits, as well as past illnesses and treatments.

  • Gene testing: A laboratory test in which cells or tissues are analyzed to look for changes in the KIT, PDGFA, and SDH genes. It is important to know if there are changes in these genes in order to diagnose GIST and plan treatment.

  • MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • CT (computed tomography): A procedure in which a series of detailed images of areas inside the body are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to help organs or tissues show more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • PET scan (positron emission tomography): a procedure to look for malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into the vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Malignant tumor cells appear brighter in the image because they are more active and consume more glucose than normal cells.

3.1.
3.2.
ПЭТ детям

Positron Emission Tomography (PET). The baby lies on a table that goes through a PET scanner. The headrest and white strap help the baby to lie quietly. A small amount of radioactive glucose (sugar) is injected into the baby's vein and the scanner takes a picture of where the glucose is being used in the body. Cancer cells appear brighter in the image because they consume more glucose than normal cells.

  • X-ray: An X-ray is a type of energy beam that can pass through the body and onto the film, creating an image of areas inside the body, such as the abdomen or the area where a tumor has formed.

  • Biopsy: Removal of cells or tissues so that a pathologist can examine them under a microscope to check for signs of cancer.

     

    • Fine needle aspiration: Removal of tissue with a fine needle.

    • Endoscopy: A procedure to examine organs and tissues inside the body for abnormal areas. The endoscope is inserted through an incision (incision) in the skin or holes in the body, such as the mouth or anus. An endoscope is a thin tubular instrument with a light and a viewing lens. He may also have an instrument to remove tissue samples or lymph nodes that are checked under a microscope for signs of disease.

 

The following laboratory test can be done to examine tissue samples:

Immunohistochemistry: A laboratory test that uses antibodies to check for specific antigens (markers) in a tissue sample from a patient. Antibodies are usually associated with an enzyme or fluorescent dye. After antibodies bind to a specific antigen in a tissue sample, the enzyme or dye is activated and the antigen can be seen under a microscope. This type of test looks for the SDH enzyme in a tissue sample from a patient. When SDH is missing, it is called SDH deficient GIST. To plan treatment, it is important to know if the cancer is insufficient for SDH.

 

After GIST has been diagnosed, tests are done to find out if cancer cells have spread to nearby areas or to other parts of the body.

The process used to determine if GIST has spread to nearby areas or other parts of the body is called staging. GIST can spread to the lymph nodes, liver, and peritoneum. There is no standard GIST staging system in children. The results of tests and procedures performed to diagnose GIST are used to make treatment decisions.

Sometimes GIST in childhood relapses (comes back) after treatment.

3.3. Overview of treatment options

There are various types of treatment for children with GIST.

Some treatments are standard (currently used) and some are in clinical trials. A treatment clinical trial is a scientific study designed to help improve existing treatments or provide information about new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment can become the standard treatment.

Because cancer is rare in children, clinical trials should be considered. Some clinical trials are only open to patients who have not yet started treatment.

Treatment for children with GIST should be planned by a team of doctors who are experts in the treatment of childhood cancers.

The treatment will be supervised by a pediatric oncologist, a doctor specializing in the treatment of oncological children. The pediatric oncologist works with other pediatric health professionals who are experts in treating children with cancer and specialize in specific areas of medicine. This may include the following professionals and others:

  • Pediatrician.

  • Children's surgeon.

  • Pathologist.

  • Specialist Pediatric Nurse.

  • Social worker .

  • Rehabilitation specialist.

  • Psychologist .

  • Child life specialist.

The following types of standard treatments are used:

1. Operation

Cancer removal surgery is an SDH deficient GIST treatment.

 

2. Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to target cancer cells. Targeted therapy usually does less damage to normal cells than chemotherapy or radiation therapy.

  • Tyrosine kinase inhibitor therapy: These drugs block signals required for tumor growth. Imatinib and sunitinib are used to treat GIST and GIST with SDH deficiency with KIT or PDGFR gene changes.

3. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by either killing the cells or stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, drugs enter the bloodstream and can reach cancer cells throughout the body

(systemic chemotherapy).

GIST treatment in children can cause side effects.

 

Side effects from cancer treatments that start after treatment and last for months or years are called late effects. Late effects of cancer treatments may include:

  • Physical problems.

  • Changes in mood, feelings, thinking, learning, or memory.

  • Second cancer (new cancers) or other conditions.

 

Some late effects can be treated or controlled. It is important to talk to your child's doctors about the possible late effects of some treatments.  

GIST treatment

 

Treatment for children newly diagnosed with tumors with changes in the KIT or PDGFR gene include the following:

  • Targeted therapy with a tyrosine kinase inhibitor (imatinib or sunitinib).

 

Treatment for children newly diagnosed with an SDH-deficient tumor may include the following:

  • Surgery to remove the tumor. If intestinal obstruction or bleeding occurs, additional surgery may be required.

  • Targeted therapy with a tyrosine kinase inhibitor (sunitinib).

Treatment of recurrent GIST

 

Treatment for recurrent GIST in children may include the following:

  • A clinical trial that tests a patient's sample of a tumor for some gene changes. The type of targeted therapy that will be given to a patient depends on the type of gene change.

  • Clinical trials of a new chemotherapy drug.

3.3.

4. TREATMENT OF KAPOSHA'S SARCOMA

Якорь 4

Kaposi's sarcoma is a disease in which malignant neoplasms (cancer) can form on the skin, mucous membranes, lymph nodes, and other organs.

Kaposi's sarcoma is cancer that causes lesions (abnormal tissue) to grow on the skin; on the mucous membranes lining the mouth, nose and throat; The lymph nodes ; or other bodies. The lesions are usually purple in color and consist of cancer cells, new blood vessels, red blood cells, and white blood cells. Kaposi's sarcoma differs from other cancers in that lesions can start in more than one place in the body at the same time.

Human herpes virus-8 (HHV-8) is found in lesions in all patients with Kaposi's sarcoma. This virus is also called Kaposi's sarcoma herpes simplex virus (KSHV). Most people with HHV-8 do not develop Kaposi's sarcoma. People with HHV-8 are more likely to develop Kaposi's sarcoma if their immune systems are weakened by a disease such as human immunodeficiency virus (HIV) or medications taken after organ transplants.

There are several types of Kaposi's sarcoma. This summary discusses two types:

  • Classic Kaposi's sarcoma.

  • Epidemic Kaposi's sarcoma (HIV-associated Kaposi's sarcoma).

4.1. Diagnostics

Tests that examine the skin, lungs, and gastrointestinal tract are used to diagnose Kaposi's sarcoma.

The following tests and procedures can be used:

  • Physical examination and health history: A body exam to check for general signs of health, including checking the skin and lymph nodes for signs of disease, such as lumps or anything else that seems unusual. There will also be a history of the patient's health habits, as well as past illnesses and treatments.

  • Chest X-ray:  X-ray of internal organs and bones of the chest. An X-ray is a type of energy beam that can pass through a body onto a film, creating an image of areas within the body. It is used to detect Kaposi's sarcoma in the lungs.

  • Biopsy: Removal of cells or tissues so that a pathologist can examine them under a microscope to check for signs of cancer.

 

​​

One of the following types of biopsies can be done to check for Kaposi's sarcoma on the skin:

 

Endoscopy or bronchoscopy can be done to check for Kaposi's sarcoma lesions in the gastrointestinal tract or lungs.

  • Excisional biopsy: A scalpel is used to remove all skin growth.

  • Postoperative biopsy: A scalpel is used to remove part of the skin growth.

  • Primary biopsy: A wide needle is used to remove part of the skin lesion.

  • Fine needle aspiration (FNA) biopsy: A fine needle is used to remove part of the skin growth.

  • Endoscopy for biopsy: A procedure to examine organs and tissues inside the body for abnormal areas. The endoscope is inserted through an incision (incision) in the skin or holes in the body, such as the mouth. An endoscope is a thin tubular instrument with a light and a viewing lens. He may also have an instrument to remove tissue samples or lymph nodes that are checked under a microscope for signs of disease. It is used to detect lesions of Kaposi's sarcoma in the gastrointestinal tract.

  • Bronchoscopy for biopsy: A procedure to examine the trachea and large airways in the lungs for abnormal areas. A bronchoscope is inserted through the nose or mouth into the trachea and lungs. A bronchoscope is a thin, tubular instrument with a light and a viewing lens. He may also have an instrument for taking tissue samples, which are checked under a microscope for signs of disease. It is used to detect lesions of Kaposi's sarcoma in the lungs.

 

After Kaposi's sarcoma has been diagnosed, tests are done to find out if the cancer cells have spread to other parts of the body.

The following tests and procedures can be used to find out if cancer has spread to other parts of the body:

  • Blood chemistry tests: A procedure in which a blood sample is tested to measure the amount of certain substances that enter the bloodstream by organs and tissues of the body. An unusual (more or less than usual) amount of a substance may be a sign of illness.

  • CT (computed tomography): A procedure in which a series of detailed pictures of areas inside the body, such as the lungs, liver, and spleen, are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to make organs and tissues more visible. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • PET scan (positron emission tomography): a procedure to detect malignant growths in the body. A small amount of radioactive glucose (sugar) is injected into the vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Malignant growths appear brighter in the image because they are more active and consume more glucose than normal cells. This imaging test checks for signs of lung, liver, and spleen cancer.

  • CD34 lymphocyte count: A procedure in which a blood sample is tested to measure the number of CD34 cells (a type of white blood cell). A lower than normal CD34 cell count may be a sign that the immune system is not working properly.

4.2. Factors affecting the prognosis of recovery

The prognosis and treatment option depends on the following conditions:

  • Type of Kaposi's sarcoma.

  • The patient's general health, especially his immune system.

  • Whether the cancer has just been diagnosed or has recurred (returned).

4.3. Classic Kaposi's sarcoma

Classic Kaposi's sarcoma is most common in older men of Italian or Eastern European Jewish descent.

Classic Kaposi's sarcoma is a rare disease that progresses slowly over many years.

Signs of classic Kaposi's sarcoma may include slow-growing lesions on the legs and feet.

Patients may have one or more red, purple, or brown skin lesions on the legs and feet, most often on the ankles or soles of the feet. Over time, lesions can form in other parts of the body, such as the stomach, intestines, or lymph nodes. The lesions usually do not cause any symptoms, but may increase in size and number over a period of 10 years or more. The pressure from the lesions can block the flow of lymph and blood in the legs and cause painful swelling. Digestive tract lesions can cause gastrointestinal bleeding.

Another cancer may develop.

Some patients with classic Kaposi's sarcoma may develop a different type of cancer before the appearance of foci of Kaposi's sarcoma or at a later age. Most often, this second cancer is non-Hodgkin's lymphoma. Frequent follow-up is necessary to keep track of these second forms of cancer.

4.4. Epidemic Kaposi's sarcoma (HIV-associated Kaposi's sarcoma)

Patients with human immunodeficiency virus (HIV) are at risk of developing epidemic Kaposi's sarcoma (HIV-associated Kaposi's sarcoma).

Acquired Immunodeficiency Syndrome (AIDS) is caused by HIV, which attacks and weakens the body's immune system. A weakened immune system is unable to fight off infections and diseases. People with HIV have an increased risk of infection and cancer.

A person with HIV and certain types of infection or cancer, such as Kaposi's sarcoma, is diagnosed with AIDS. Sometimes a person is diagnosed with AIDS and Kaposi's sarcoma at the same time.

The use of a drug therapy called highly active antiretroviral therapy (HAART) reduces the risk of epidemic Kaposi's sarcoma in people with HIV.

HAART is a combination of several drugs used to reduce the damage to the immune system caused by HIV infection. Treatment with HAART reduces the risk of epidemic Kaposi's sarcoma, although a person can develop epidemic Kaposi's sarcoma while on HAART.

For information on AIDS and its treatment, visit the AIDSinfo website.

Signs of epidemic Kaposi's sarcoma can include lesions that form in many parts of the body.

The signs of epidemic Kaposi's sarcoma can include lesions in various parts of the body, including one of the following:

  • Leather.

  • Mouth lining.

  • The lymph nodes .

  • Stomach and intestines.

  • Lightweight and chest lining.

  • Liver .

  • Spleen.

 

Kaposi's sarcoma is sometimes found on the lining of the mouth during a regular dental check-up.

In most patients with epidemic Kaposi's sarcoma, the disease spreads over time to other parts of the body.

4.5. Overview of treatment options

There are different treatments for patients with Kaposi's sarcoma.

Various treatments are available for Kaposi's sarcoma patients. Some treatments are standard (currently used) and some are in clinical trials. A treatment clinical trial is a scientific study designed to help improve existing treatments or provide information about new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment can become the standard treatment. Patients may want to take part in a clinical trial. Some clinical trials are only open to patients who have not yet started treatment.

Six standard treatments are used to treat Kaposi's sarcoma:

Treatment for Epidemic Kaposi's sarcoma combines treatment for Kaposi's sarcoma with treatment for acquired immunodeficiency syndrome (AIDS). The six types of standard treatments used to treat Kaposi's sarcoma include:

1. HAART

Highly active antiretroviral therapy (HAART) is a combination of several drugs used to reduce damage to the immune system caused by human immunodeficiency virus (HIV) infection. For many patients, HAART alone may be sufficient to treat epidemic Kaposi's sarcoma. For other patients, HAART may be combined with other standard treatments for epidemic Kaposi's sarcoma.

2. Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy X-rays or other types of radiation to kill cancer cells or prevent them from growing. There are two types of radiation therapy:

  • External beam therapy  uses a device outside the body to direct radiation to an area of the body affected by cancer.

  • Internal beam therapy  uses a radioactive substance sealed in needles, seeds, wires, or catheters that are inserted directly into or near the tumor.

 

How radiation therapy is given depends on the type of cancer being treated. Several types of external beam radiation therapy are used to treat lesions of Kaposi's sarcoma. Photon radiation therapy treats injuries with high energy light. Electron beam therapy uses tiny, negatively charged particles called electrons.

3. Operation

The following surgical procedures can be used for Kaposi's sarcoma to treat small, superficial lesions:

  • Local excision: The tumor is excised from the skin along with some normal tissue around it.

  • Electrodesification and scraping:  the tumor is excised from the skin with a curette (a sharp, spoon-shaped instrument). A needle electrode is then used to shock the area with an electric current, which stops the bleeding and destroys the cancer cells remaining at the edge of the wound. This process can be repeated one to three times during surgery to remove the entire tumor.

 

4. Cryosurgery

Cryosurgery is a treatment that uses an instrument to freeze and destroy abnormal tissue. This type of treatment is also called cryotherapy.

 

5. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by either killing the cells or stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (  systemic chemotherapy  ). When chemotherapy is injected directly into the cerebrospinal fluid, organ, tissue, or body cavity, such​​ like the abdomen, drugs mainly target cancer cells in these areas (regional chemotherapy).

With electrochemotherapy , intravenous chemotherapy is given and a probe is used to send electrical impulses to the tumor. The pulses make a hole in the membrane around the tumor cell and allow the chemotherapist to enter.

 

How chemotherapy is administered depends on where the foci of Kaposi's sarcoma occur in the body. For Kaposi's sarcoma, chemotherapy can be performed in the following ways:

  • For local lesions of Kaposi's sarcoma, such as in the mouth, anticancer drugs can be injected directly into the lesion (intralesional chemotherapy).

  • For localized skin lesions, a topical gel can be applied to the skin. Electrochemotherapy can also be used.

  • For extensive skin lesions, intravenous chemotherapy may be prescribed.

 

Liposomal chemotherapy uses liposomes (very tiny particles of fat) to carry anticancer drugs. Liposomal doxorubicin is used to treat Kaposi's sarcoma. Liposomes accumulate in the tissue of Kaposi's sarcoma more than in healthy tissue, and doxorubicin is released slowly. This enhances the action of doxorubicin and causes less damage to healthy tissue.

5. Immunotherapy

Immunotherapy is a treatment that uses a patient's immune system to fight cancer. Substances produced by the body or in the laboratory are used to enhance, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biological therapy. Interferon alpha and interleukin-12 are biological agents used to treat Kaposi's sarcoma.

6. Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and target certain cancer cells. Targeted therapy usually does less damage to normal cells than chemotherapy or radiation therapy. Monoclonal antibody therapy and tyrosine kinase inhibitors (TKIs) are types of targeted therapies that are being studied in the treatment of Kaposi's sarcoma.

  • Monoclonal antibodies are proteins of the immune system created in the laboratory for the treatment of many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that can promote cancer cell growth. The antibodies are then able to kill cancer cells, block their growth, or prevent them from spreading. Monoclonal antibodies are given by infusion. They can be used alone or to carry drugs, toxins, or radioactive materials directly to cancer cells. Bevacizumab is a monoclonal antibody that can be used to treat Kaposi's sarcoma.

  • TKIs block signals required for tumor growth. Imatinib mesylate is a TKI that can be used to treat Kaposi's sarcoma.

Treatment of classic Kaposi's sarcoma

 

Treatment for isolated skin lesions may include the following:

  • Radiation therapy .

  • Surgery.

 

Treatments for skin lesions throughout the body may include the following:

  • Radiation therapy.

  • Chemotherapy.

  • Electrochemotherapy.

 

Treatment for Kaposi's sarcoma, which affects the lymph nodes or the gastrointestinal tract, usually includes chemotherapy with or without radiation therapy.

Treatment of epidemic Kaposi's sarcoma

 

Treatment for epidemic Kaposi's sarcoma may include the following:

  • Surgery, including local excision or electrodesiccation and curettage.

  • Cryosurgery.

  • Radiation therapy .

  • Chemotherapy using one or more anticancer drugs.

  • Biological therapy using interferon alpha or interleukin-12.

  • Targeted therapy using imatinib or bevacizumab.

4.1.
4.2.
4.3.
4.4.
4.5.

5. TREATMENT WITH RABDOMOISARCOM IN CHILDHOOD 

Якорь 5

Infantile rhabdomyosarcoma is a condition in which malignant (cancerous) cells form in muscle tissue.

Rhabdomyosarcoma is a type of sarcoma. Sarcoma is cancer of soft tissue (such as muscle), connective tissue (such as tendons or cartilage), or bone. Rhabdomyosarcoma usually starts in the muscles that are attached to the bones and help the body move, but it can start in many parts of the body. Rhabdomyosarcoma is the most common type of soft tissue sarcoma in children.

There are four main types of rhabdomyosarcoma:

  • Fetal: This type is most commonly found in the head and neck area, as well as in the genitals or urinary tract, but can occur anywhere on the body. This is the most common type of rhabdomyosarcoma.

  • Alveolar: This type is most commonly found in the arms or legs, chest, abdomen, genitals, or anal area.

  • Spindle cell / sclerosis: The spindle cell type is most commonly found in the paratesticular region (testis or spermatic cord). There are two other subtypes of spindle / sclerosis cells. One is more common in babies and is found in the trunk region. The other can affect children, adolescents, and adults. It is often found in the head and neck area and is more aggressive.

  • Pleomorphic: This is the least common type of rhabdomyosarcoma in children.

5.1. Development risks  rhabdomyosarcoma in children

Certain genetic conditions increase the risk of developing rhabdomyosarcoma in children.

Anything that increases the risk of a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; the absence of risk factors does not mean that you will not get cancer. Talk to your child's doctor if you think your child may be at risk.

Risk factors for rhabdomyosarcoma include the following inherited conditions:

  • Li-Fraumeni syndrome.

  • Dicer syndrome 1.

  • Neurofibromatosis type 1 (NF1).

  • Costello's Syndrome.

  • Beckwith-Wiedemann syndrome.

  • Noonan's syndrome.

 

Babies who were high birth weight or were larger than expected at birth may have an increased risk of fetal rhabdomyosarcoma.

In most cases, the cause of rhabdomyosarcoma is unknown.

5.2. Symptoms

The hallmark of rhabdomyosarcoma in childhood is a swelling or lump that continues to grow.

Signs and symptoms can be caused by rhabdomyosarcoma in children or other medical conditions. The signs and symptoms that occur depend on where the cancer starts. Check with your child's doctor if your child has any of the following:

  • A lump or lump that keeps getting bigger or doesn't go away. It can be painful.

  • Crossed eyes or bulging eye.

  • Headache.

  • Problems with urination or bowel movements.

  • Blood in the urine.

  • Bleeding from the nose, throat, vagina, or rectum.

5.3. Diagnostics

Diagnostic tests and biopsies are used to diagnose rhabdomyosarcoma in children.

The diagnostic tests that are done depends in part on where the cancer is. The following tests and procedures can be used:

  • Physical examination and health history: Examining the body to check for general signs of health, including checking for signs of illness such as bumps or anything else that seems out of the ordinary. There will also be a history of the patient's health habits, as well as past illnesses and treatments.

  • X-rays: X-rays of organs and bones inside the body, such as the chest. An X-ray is a type of energy beam that can pass through a body onto a film, creating an image of areas within the body.

  • CT (computed tomography): A procedure in which a series of detailed pictures of areas inside the body, such as the chest, abdomen, pelvis, or lymph nodes, are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to make organs or tissues show more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas of the body, such as the skull, brain, and lymph nodes. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • PET scan (positron emission tomography): a procedure to look for malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into the vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Malignant tumor cells appear brighter in the image because they are more active and consume more glucose than normal cells.

  • Bone scan: A procedure to check for rapidly dividing cells such as cancer cells in the bone. A very small amount of radioactive material is injected into a vein and passed through the bloodstream. The radioactive material builds up in the bones in cancer and is detected by a scanner.

  • Bone marrow aspiration and biopsy: Removal of bone marrow, blood, and a small piece of bone by inserting a cannula into the hip bone. Samples are removed from both hip bones. A pathologist examines the bone marrow, blood, and bones under a microscope to look for signs of cancer.

  • Lumbar puncture: A procedure used to collect cerebrospinal fluid (CSF) from the spine. This is done by inserting a needle between two bones in the spine and into the cerebrospinal fluid and taking a sample of the fluid. A sample of cerebrospinal fluid is checked under a microscope for signs of cancer cells. This procedure is also called LP or lumbar puncture.

 

If these tests show that there may be rhabdomyosarcoma, a biopsy is done.  A biopsy is the removal of cells or tissues so that a pathologist can examine them under a microscope to check for signs of cancer. Because treatment depends on the type of rhabdomyosarcoma, biopsy specimens should be screened by a pathologist experienced in diagnosing rhabdomyosarcoma.

It may use one of the following types of biopsies:

  • Fine needle aspiration (FNA) biopsy: Removal of tissue or fluid with a fine needle.

  • Shaft biopsy: Removal of tissue with a wide needle. This procedure can be guided by ultrasound, computed tomography, or MRI.

  • Open biopsy: Removal of tissue through an incision (incision) in the skin.

  • Sentinel lymph node biopsy: Removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node in a group of lymph nodes to receive lymphatic drainage from the primary tumor. This is the first lymph node to which cancer can spread from the primary tumor. A radioactive substance and / or blue dye is injected near the tumor. The substance or dye flows through the lymphatic tract. To the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist examines tissue under a microscope for cancer cells. If no cancer cells are found, it may not be necessary to remove additional lymph nodes. Sometimes a sentinel lymph node is found in more than one group of nodes. Sentinel lymph node biopsy may be used in patients with rhabdomyosarcoma of the extremities or trunk when enlarged lymph nodes are not found on imaging or physical examination.

The following tests can be performed on a sample of tissue removed:

  • Light microscopy:  a laboratory test in which cells in a tissue sample are viewed under a normal, high-power microscope to detect certain changes in the cells.

  • Immunohistochemistry: A laboratory test that uses antibodies to check for specific antigens (markers) in a tissue sample from a patient. Antibodies are usually associated with an enzyme or fluorescent dye. After antibodies bind to a specific antigen in a tissue sample, the enzyme or dye is activated and the antigen can be seen under a microscope. This type of test is used to diagnose cancer and help distinguish one type of cancer from another.

  • FISH (Fluorescence In Situ Hybridization): A laboratory test used to study and count genes or chromosomes in cells and tissues. Pieces of DNA containing fluorescent dyes are made in the laboratory and added to a sample of the patient's cells or tissues. When these stained DNA fragments attach to specific genes or regions of chromosomes in a sample, they light up when viewed under a fluorescent microscope. The FISH test is used to diagnose cancer and plan treatment.

  • Reverse transcription polymerase chain reaction (RT-PCR) test: A laboratory test that measures the amount of a genetic substance called mRNA created by a particular gene. An enzyme called reverse transcriptase is used to convert a specific piece of RNA into a corresponding piece of DNA that can be amplified (produced in large quantities) by another enzyme called DNA polymerase. Amplified copies of DNA help determine if a particular mRNA is being made by the genome. RT-PCR can be used to check for the activation of certain genes that may indicate the presence of cancer cells. This test can be used to look for specific changes in a gene or chromosome that can help diagnose cancer.

  • Cytogenetic analysis: A laboratory test in which the chromosomes of cells in a tissue sample are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in some chromosomes can be a sign of cancer. Cytogenetic analysis is used to diagnose cancer, plan treatment, or determine the effectiveness of a treatment.

Once children are diagnosed with rhabdomyosarcoma, treatment depends in part on the stage of the cancer and sometimes on whether the cancer has been completely removed by surgery.

The process used to determine if cancer has spread to tissue or to other parts of the body is called staging. It is important to know the stage in order to plan treatment. The doctor, based on the results of diagnostic tests, will help determine the stage of the disease.

Treatment for rhabdomyosarcoma in children is based in part on the stage and sometimes on the size of the cancer that remains after surgery to remove the tumor. The pathologist will use a microscope to check tissue removed during surgery, including tissue samples from the edges of areas where cancer was removed and lymph nodes. This is done to see if all of the cancer cells have been removed during surgery.

5.4. Factors influencing the forecast

Several factors affect prognosis (chance of recovery) and treatment options.

The prognosis and treatment option depends on the following conditions:

  • The age of the patient.

  • Where the tumor started in the body.

  • The size of the tumor at the time of diagnosis.

  • Whether the tumor has been completely removed by surgery.

  • Type of rhabdomyosarcoma (embryonic, alveolar, spindle cell / sclerosing, or pleomorphic).

  • Are there any changes in the genes.

  • Whether the tumor has spread to other parts of the body at the time of diagnosis.

  • Whether there was a tumor in the lymph nodes at the time of diagnosis.

  • Whether the tumor is responding to chemotherapy and / or radiation therapy.

 

For patients with recurrent cancer, prognosis and treatment also depend on the following:

  • Where in the body the tumor has recurred (returned).

  • How much time elapsed between the end of cancer treatment and its relapse.

  • Has the cancer been previously treated with radiation therapy?

Additional tests may be required.

Some tests that have been done to diagnose cancer or to determine the stage of cancer may be repeated. Some tests will be repeated to see how effective the treatment is. Decisions to continue, change, or stop treatment may be based on the results of these tests.

Some tests will continue from time to time after treatment ends. The results of these tests can show if your child's condition has changed or if the cancer has recurred (returned). These tests are sometimes called control or control tests.

5.5. Stages of childhood rhabdomyosarcoma

The stage of rhabdomyosarcoma in children has three parts.

Childhood rhabdomyosarcoma is assessed using three different ways to describe cancer:

  • System setting.

  • Grouping system.

  • Risk group.

 

The staging system is based on the size of the tumor, its location in the body, and its spread to other parts of the body:

Stage 1

In stage 1, a tumor of any size may have spread to the lymph nodes and is found only in one of the following “favorable” sites:

  • The eye or the area around the eye.

  • Head and neck (but not in tissues around the ear, nose, sinuses, base of the skull, brain, or spinal cord).

  • Gallbladder and bile ducts.

  • Ureters or urethra.

  • Testes, ovary, vagina, or uterus.

 

Rhabdomyosarcoma, which develops in a “favorable” location, has a better prognosis. If the site where the cancer occurs is not among the favorable sites listed above, it is considered an “unfavorable” site.

5.1.
5.2.
5.3.
5.4.
5.5.
рабдомиосаркома

Tumor sizes are often measured in centimeters (cm) or inches. Common foods that can be used to display tumor size in cm include: pea (1 cm), peanuts (2 cm), grapes (3 cm), walnut (4 cm), lime (5 cm or 2 cm). inches), egg (6 cm), peach (7 cm) and grapefruit (10 cm or 4 inches).

Stage 2  

In stage 2, cancer is found in an “unfavorable” location (in any area not described as “favorable” in stage 1). The tumor is no more than 5 centimeters long and has not spread to the lymph nodes.

Stage 3

In stage 3, cancer is found in an “unfavorable” location (in any area not described as “favorable” in stage 1) and one of the following conditions is met:

  • The tumor is no more than 5 centimeters long and the cancer has spread to nearby lymph nodes.

  • The tumor is larger than 5 centimeters, and the cancer could have spread to nearby lymph nodes.

 

Stage 4

In stage 4, the tumor can be of any size and the cancer can spread to nearby lymph nodes. The cancer has spread to distant parts of the body, such as the lungs, bone marrow, or bones.

The grouping system is based on whether the cancer has spread and whether all of the cancer has been surgically removed:

 

Group I

The cancer was only found where it started and was completely removed by surgery. The tissue was taken from the edges where the tumor was removed. This tissue was examined under a microscope by a pathologist and no cancer cells were found.

II group

Group II is divided into groups IIA, IIB and IIC.

  • IIA:  Cancer is removed by surgery, but cancer cells were seen when tissue taken from the edges where the tumor was removed was examined under a microscope by a pathologist.

  • IIB: The cancer has spread to nearby lymph nodes, and the cancer and lymph nodes have been surgically removed.

  • IIC: Cancer has spread to nearby lymph nodes, cancer and lymph nodes have been surgically removed, and at least one of the following is true:

    • Tissue taken from the edges of where the tumor was removed was examined under a microscope by a pathologist and cancer cells were found.

    • The lymph node farthest from the removed tumor was examined under a microscope by a pathologist and cancer cells were found.

 

III group

The cancer was partially removed by biopsy or surgery, but a tumor remains that can be seen with the naked eye.

 

IV group

When the diagnosis was made, the cancer had spread to distant parts of the body.

  • Cancer cells are detected by imaging; or

  • Cancer cells are found in fluid around the brain, spinal cord, or lungs, or in abdominal fluid; or tumors are found in these areas.

The risk group is built on the basis of a stepwise​​ systems and systems of grouping.

The risk group describes the likelihood that rhabdomyosarcoma will recur (come back). Every child treated for rhabdomyosarcoma should receive chemotherapy to reduce the likelihood of cancer recurrence. The type of cancer drug, dose, and number of treatments prescribed will depend on whether the child has low, medium, or high risk rhabdomyosarcoma.

The following risk groups are used:

1. Low-risk childhood rhabdomyosarcoma

Low-risk childhood rhabdomyosarcoma is one of the following:

  • An embryonic tumor of any size, which is located in a “favorable” place. After the operation, a tumor may remain, which can be seen with or without a microscope. Cancer can spread to nearby lymph nodes. The following areas are "favorable" sites:

    • The eye or the area around the eye.

    • Head or neck (but not in tissue around the ear, nose, sinuses, base of the skull, brain, or spinal cord).

    • Gallbladder and bile ducts.

    • Ureter or urethra.

    • Testes, ovary, vagina, or uterus.

 

  • An embryonic tumor of any size that is not in a “favorable” location. After the operation, a tumor may remain that can only be seen under a microscope. The cancer could have spread to nearby lymph nodes.

2. Pediatric rhabdomyosarcoma of medium risk

Mid-risk childhood rhabdomyosarcoma is one of the following:

  • An embryonic tumor of any size that does not occur at one of the “favorable” sites listed above. After the operation, a tumor remains that can be seen with or without a microscope. Cancer can spread to nearby lymph nodes.

  • An alveolar tumor of any size at a “favorable” or “unfavorable” site. After the operation, a tumor may remain, which can be seen with or without a microscope. The cancer could have spread to nearby lymph nodes.

3. High-risk childhood rhabdomyosarcoma

A high risk of childhood rhabdomyosarcoma can be of the embryonic type or the alveolar type. It may have spread to nearby lymph nodes and has spread to one or more of the following:

  • Other parts of the body not located near the site where the tumor first formed.

  • Fluid around the brain or spinal cord.

  • Fluid in the lungs or abdomen.

Sometimes rhabdomyosarcoma in children continues to grow or returns after treatment.

Progressive rhabdomyosarcoma  is cancer that keeps growing, spreading, or getting worse. Progressive illness may be a sign that the cancer has become refractory to treatment.

Recurrent rhabdomyosarcoma in children is cancer that has recurred (returned) after treatment. Cancer may reappear in the same place or in other parts of the body, such as the lungs, bone, or bone marrow. Less commonly, rhabdomyosarcoma can recur in the breasts of adolescent girls or in the liver.

5.6. Overview of treatment options

There are various treatments for rhabdomyosarcoma patients in children.

Some treatments are standard (currently used) and some are in clinical trials. A treatment clinical trial is a scientific study designed to help improve existing treatments or provide information about new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment can become the standard treatment.

Because cancer is rare in children, clinical trials should be considered. Some clinical trials are only open to patients who have not yet started treatment.

Children with rhabdomyosarcoma should plan for treatment by a team of healthcare professionals who are experts in treating childhood cancer.

Because rhabdomyosarcoma can form in many different parts of the body, many different treatments are used. The treatment will be supervised by a pediatric oncologist, a doctor specializing in the treatment of oncological children. The pediatric oncologist works with other health care providers who are experts in treating children with rhabdomyosarcoma and specialize in specific areas of medicine. This may include the following specialists:

  • Pediatrician.

  • Children's surgeon.

  • Radiologist oncologist.

  • Children's hematologist.

  • Children's radiologist.

  • Specialist Pediatric Nurse.

  • Geneticist or cancer genetics consultant.

  • Social worker .

  • Rehabilitation specialist.

Three types of standard treatments are used:

1. Operation

Surgery (removal of cancer during surgery) is used to treat rhabdomyosarcoma in children. An operation called a wide local excision is often performed. A wide local excision is the removal of the tumor and part of the tissue around it, including the lymph nodes. A second surgery may be required to remove all of the cancer. Whether the operation will be performed and its type depends on the following:

  • Where the tumor started in the body.

  • Influence of the operation on the appearance of the child.

  • Influence of the operation on important functions of the child's body.

  • How did the tumor respond to chemotherapy or radiation therapy, which may have been prescribed in the first place.

 

In most children with rhabdomyosarcoma, it is not possible to remove the entire tumor with surgery.

Rhabdomyosarcoma can form in different parts of the body, and the operation will be different for each one. Surgery to treat rhabdomyosarcoma of the eye or genital area is usually a biopsy. Chemotherapy, and sometimes radiation therapy, may be given before surgery to shrink large tumors.

After the doctor removes any tumors that can be seen during surgery, patients will be given chemotherapy after surgery to kill any remaining cancer cells. Radiation therapy may also be prescribed. Treatment given after surgery to reduce the risk of cancer recurrence is called adjuvant therapy.

2. Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy X-rays or other types of radiation to kill or stop cancer cells from growing. There are two types of radiation therapy:

  • External beam therapy  uses a device outside the body to direct radiation to an area of the body affected by cancer. Certain treatments for radiation therapy can help prevent radiation from damaging nearby healthy tissue. These types of external radiation therapy include:

    • Conformal radiation therapy: Conformal radiation therapy is a type of external radiation therapy in which a computer creates a three-dimensional (3-D) image of the tumor and generates beams of radiation that match the tumor. This allows a high dose of radiation to reach the tumor and cause less damage to nearby healthy tissue.

    • Intensity Modulated Radiation Therapy (IMRT): I MRT is a type of three-dimensional (3-D) radiation therapy that uses a computer to produce images of the size and shape of a tumor. Thin beams of radiation of different intensity (strength) are directed at the tumor at different angles.

    • Volumetric Modulated Arc Therapy (VMAT): VMAT is a type of three-dimensional radiation therapy in which a computer takes pictures of the size and shape of the tumor. During treatment, the radiation machine goes around the patient once in a circle and directs thin beams of radiation of different intensities to the tumor. Treatment with VMAT is faster than treatment with IMRT.

    • Stereotactic body radiation therapy: Stereotactic body radiation therapy is a type of external radiation therapy. Special equipment is used to position the patient in the same position for every radiation therapy session. Once a day for several days, a radiation unit directs a higher than usual dose of radiation directly to the tumor. If the patient is in the same position during each treatment session, there is less damage to nearby healthy tissue. This procedure is also called stereotaxic external beam radiation therapy and stereotaxic radiation therapy.

    • Proton beam therapy: Proton beam therapy is a type of high-energy external beam therapy. A radiation therapy machine directs beams of protons (tiny, invisible, positively charged particles) to cancer cells to kill them. This type of treatment can cause less damage to nearby healthy tissue.

 

  • Internal beam therapy  uses a radioactive substance sealed in needles, seeds, wires, or catheters that are inserted directly into or near the tumor. It is used to treat cancer in areas such as the vagina, vulva, uterus, bladder, prostate, head, or neck. Internal radiation therapy is also called brachytherapy, internal radiation, implant radiation, or interstitial radiation therapy. This approach requires specialized technical skills and is offered in only a few health centers.

 

The type and amount of radiation therapy, as well as when it is given, depends on the child's age, the type of rhabdomyosarcoma, the site of the tumor, the amount of tumor left after surgery, and the presence of a tumor in nearby lymph nodes. ...

External radiation therapy is commonly used to treat rhabdomyosarcoma in children, but internal radiation therapy is sometimes used.

3. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by either killing the cells or stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy).

Chemotherapy may also be prescribed to shrink the tumor before surgery to preserve as much healthy tissue as possible. This is called neoadjuvant chemotherapy.

Every child receiving treatment for rhabdomyosarcoma should receive systemic chemotherapy to reduce the likelihood of cancer recurrence. The type of anticancer drug, dose and number of treatments prescribed will depend on the child's age and whether the child has low, medium, or high risk rhabdomyosarcoma.

In addition to the standard treatment, the following treatments are used:

 

1. Immunotherapy

Immunotherapy  is a treatment that uses the patient's immune system to fight cancer. Substances produced by the body or in the laboratory are used to enhance, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biological therapy. There are different types of immunotherapy:

  • Vaccine therapy  is a cancer treatment that uses a substance or group of substances to stimulate the immune system to find a tumor and kill it. Vaccine therapy is being studied for the treatment of metastatic rhabdomyosarcoma.

  • Immune Checkpoint Inhibitor Therapy  uses the body's immune system to destroy cancer cells. Two types of immune checkpoint inhibitors are being studied in the treatment of childhood rhabdomyosarcoma that has returned from treatment:

    • CTLA-4 is a protein on the surface of T cells that helps control the body's immune responses. When CTLA-4 attaches to another protein, called B7, on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow T cells to kill cancer cells. Ipilimumab is being studied for the treatment of childhood rhabdomyosarcoma that has returned or progressed during treatment.

    • Therapy with PD-1 and PD-L1 inhibitors: PD-1 is a protein on the surface of T cells that helps control the body's immune responses. PD-L1 is a protein found on some types of cancer cells. When PD-1 binds to PD-L1, it stops the T cell from killing the cancer cell. PD-1 and PD-L1 inhibitors prevent the PD-1 and PD-L1 proteins from attaching to each other. This allows T cells to kill cancer cells. Nivolumab and pembrolizumab are types of PD-1 inhibitors that are being studied in the treatment of rhabdomyosarcoma in children that recurs or progresses during treatment.

 

2. Targeted therapy

Targeted therapy  is a type of treatment that uses drugs or other substances to identify and destroy certain cancer cells. Targeted therapy usually does less damage to normal cells than chemotherapy or radiation therapy. There are different types of targeted therapy:

  • MTOR inhibitors stop a protein that helps cells divide and survive. Sirolimus is a type of mTOR inhibitor therapy being studied in the treatment of recurrent rhabdomyosarcoma.

  • Tyrosine kinase inhibitors block signals that cancer cells should grow and divide. MK-1775, cabozantinib-s-malate, and palbociclib are tyrosine kinase inhibitors that are being studied in the treatment of newly diagnosed or recurrent rhabdomyosarcoma.

5.7. Side Effects of Treatment

Treatment for rhabdomyosarcoma in children can cause side effects.

 

Side effects from cancer treatments that start after treatment and last for months or years are called late effects. Late effects of rhabdomyosarcoma cancer treatments may include:

  • Physical issues affecting the following:

    • Teeth, eyes, or gastrointestinal tract.

    • Fertility (the ability to have children).

  • Changes in mood, feelings, thinking, learning, or memory.

  • Second cancer (newer cancers).

 

Some late effects can be treated or controlled. It is important to talk with your child's doctors about the impact cancer treatment can have on your child and the types of symptoms to expect after completing cancer treatment.

5.8. Treatment of childhood rhabdomyosarcoma

Treatment for newly diagnosed rhabdomyosarcoma in children often includes surgery, radiation therapy, and chemotherapy. The order in which these procedures are performed depends on where the tumor started in the body, the size of the tumor, the type of tumor, and whether the tumor has spread to lymph nodes or other parts of the body. See the Treatment Options Overview section of this summary for more information on surgery, radiation therapy, and chemotherapy used to treat children with rhabdomyosarcoma.

Rhabdomyosarcoma of the brain, head and neck

  • For tumors in the brain: Treatment may include surgery to remove the tumor, radiation therapy, and chemotherapy.

  • For head and neck tumors in or near the eye: Treatment may include chemotherapy and radiation therapy. If the tumor remains or returns after chemotherapy and radiation therapy, surgery may be required to remove the eye and some tissue around the eye.

  • For head and neck tumors that are near the ear, nose, sinuses, or base of the skull, but not in or near the eye: Treatment may include radiation therapy and chemotherapy.

  • For head and neck tumors that are not in or near the eye, or near the ear, nose, sinuses, or base of the skull: Treatment may include chemotherapy, radiation therapy, and surgery to remove the tumor.

  • For head and neck tumors that cannot be removed with surgery: Treatment may include chemotherapy and radiation therapy, including stereotactic radiation therapy.

  • For tumors of the larynx (voice box): Treatment may include chemotherapy and radiation therapy. Surgery to remove the larynx is usually not done to avoid damaging the voice.

 

Rhabdomyosarcoma of the arms or legs

Chemotherapy followed by surgery to remove the tumor. If the tumor has not been removed completely, a second surgery may be performed to remove the tumor. Radiation therapy may also be prescribed.

  • For tumors of the hand or foot, radiation therapy and chemotherapy may be prescribed. The tumor cannot be removed because it will affect the function of the hand or foot.

  • Dissection of the lymph nodes (one or more lymph nodes are removed and a tissue sample is checked under a microscope for signs of cancer).

    • For arm tumors, the lymph nodes near the tumor and in the armpits are removed.

    • For leg tumors, lymph nodes near the tumor and in the groin area are removed.

 

Rhabdomyosarcoma of the chest, abdomen, or pelvis

  • For tumors of the chest or abdomen (including the chest wall or abdominal wall): Surgery (wide local excision) may be performed. If the tumor is large, chemotherapy and radiation therapy are given before surgery to shrink the tumor.

  • For pelvic tumors: surgical intervention is possible (wide local excision). If the tumor is large, chemotherapy is given before surgery to shrink the tumor. Radiation therapy may be prescribed after surgery.

  • For tumors of the diaphragm: Tumor biopsy is followed by chemotherapy and radiation therapy to shrink the tumor. Later, surgery may be done to remove the remaining cancer cells.

  • For tumors of the gallbladder or bile ducts: Tumor biopsy is followed by chemotherapy and radiation therapy. Later, surgery may be done to remove the remaining cancer cells.

  • For muscle or tissue tumors around the anus, between the vulva and the anus, or between the scrotum and anus: surgery may be performed to remove as much of the tumor and some nearby lymph nodes as possible, followed by chemotherapy and radiation therapy.

 

Rhabdomyosarcoma of the kidney

  • For kidney tumors:  surgery to remove as much of the tumor as possible. Chemotherapy and radiation therapy may also be prescribed.

 

Rhabdomyosarcoma of the bladder or prostate

  • For tumors that are only in the upper part of the bladder: Surgery (wide local excision) is performed.

  • For tumors of the prostate or bladder (other than the upper part of the bladder):

    • Chemotherapy and radiation therapy are given first to shrink the swelling. If cancer cells remain after chemotherapy and radiation therapy, the tumor is surgically removed. Surgery may involve removal of the prostate, part of the bladder, or pelvic exenteration without removing the rectum. (This may include removing the lower colon and bladder. In girls, the cervix, vagina, ovaries, and nearby lymph nodes may be removed.)

    • Chemotherapy is given first to shrink the tumor. Surgery is performed to remove the tumor, but not the bladder or prostate. After surgery, internal or external radiation therapy may be prescribed.

    • Surgery to remove the tumor, but not the bladder or prostate. After the operation, internal radiation therapy is prescribed.

 

Rhabdomyosarcoma of the testicular area

  • Surgery to remove the testicle and spermatic cord. The lymph nodes in the back of the abdomen can be checked for cancer, especially if the lymph nodes are large. Nerve-sparing retroperitoneal lymph node dissection should be performed in patients over 10 years of age without evidence of lymph node enlargement in the posterior abdomen.

  • Radiation therapy may be prescribed if the tumor cannot be completely removed with surgery.

 

Rhabdomyosarcoma of the vulva, vagina, uterus, or ovary

  • For tumors in the vulva and vagina: Treatment may include chemotherapy followed by surgery to remove the tumor. After surgery, internal or external radiation therapy may be prescribed.

  • For tumors of the uterus: Treatment may include chemotherapy with or without radiation therapy. Surgery may sometimes be required to remove any remaining cancer cells.

  • For ovarian tumors: Treatment may include chemotherapy followed by surgery to remove the remaining tumor.

Metastatic rhabdomyosarcoma

Treatment, such as chemotherapy followed by radiation therapy or surgery to remove the tumor, is done at the site where the tumor first formed. If the cancer has spread to the brain, spinal cord, or lungs, radiation therapy may also be given where the cancer has spread.

The following treatments are being investigated for metastatic rhabdomyosarcoma:

  • Clinical study on immunotherapy (vaccine therapy).

5.9. Treatment of progressive or recurrent childhood rhabdomyosarcoma

 

Treatment options for progressive or recurrent rhabdomyosarcoma in children are based on many factors, including where the cancer has returned in the body, what type of treatment the child has received before, and the child's needs.

Treatment for progressive or recurrent rhabdomyosarcoma may include one or more of the following:

  • Surgery.

  • Radiation therapy .

  • Chemotherapy.

  • Clinical trial in combination chemotherapy with or without Temsirolimus.

  • Clinical study for targeted therapy or immunotherapy (sirolimus, Ipilimumab, nivolumab, or pembrolizumab).

  • Clinical trial of targeted therapy with a tyrosine kinase inhibitor (MK-1775, cabozantinib-s-malate or palbociclib) and chemotherapy.

  • A clinical trial that tests a sample of a patient's tumor for certain gene changes. The type of targeted therapy that will be given to a patient depends on the type of gene change.

  • New therapies being investigated in the early stages of clinical trials should be considered for patients with recurrent rhabdomyosarcoma.

5.6.
5.7.
5.8.
5.9.

6. TREATMENT OF SOFT TISSUE SARCOMA
CHILDREN 

Якорь 6

Soft tissue sarcoma in childhoodis a disease in which malignant (cancer) cells form in the soft tissues of the body.

General information about soft tissue sarcoma (clickhere)

6.1. Risks of developing soft tissue sarcoma in children

The presence of certain diseases and hereditary disorders can increase the risk of developing soft tissue sarcoma in children.

Anything that increases the risk of a disease is called a risk factor. Having a risk factor does not mean you will get cancer; the absence of risk factors does not mean that you will not get cancer. Talk to your child's doctor if you think your child may be at risk.

Risk factors for soft tissue sarcoma in children include the presence of the following inherited conditions:

  • Li-Fraumeni syndrome.

  • Familial adenomatous polyposis (FAP).

  • Changes in the RB1 gene.

  • Changes in the SMARCB1 (INI1) gene.

  • Neurofibromatosis type 1 (NF1).

  • Werner syndrome.

  • Tuberous sclerosis.

  • Severe combined immunodeficiency with adenosine deaminase deficiency.

 

Other risk factors include the following:

  • Past treatment with radiation therapy.

  • Sick of AIDS (acquired immunodeficiency syndrome) and Epstein-Barr virus infection at the same time.

6.2. Symptoms 

The most common symptom of childhood soft tissue sarcoma is a painless swelling or swelling in the soft tissues of the body.

Sarcomas may appear as a painless lump under the skin, often on the arm, leg, chest, or abdomen. At first, there may be no other signs or symptoms. As the sarcoma gets larger and presses on nearby organs, nerves, muscles, or blood vessels, it may cause signs or symptoms, such as pain or weakness.

Other conditions may cause the same signs and symptoms. Check with your child's doctor if your child has any of these concerns.

6.3. Diagnosis 

The following tests and procedures may be used:

  • Medical examination and health history:examining the body to check for general signs of health, including checking for signs of disease such as tumors or anything else that seems unusual. An anamnesis of the patient's health habits as well as past illnesses and treatments will also be taken.

  • X-rays :X-rays are a type of energy beam that can pass through the body onto film, taking pictures of areas inside the body.

  • MRI (magnetic resonance imaging) :a procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas of the body, such as the chest, abdomen, arms, or legs. This procedure is also called nuclear magnetic resonance imaging (NMRI).

  • CT (computed tomography) :a procedure in which a series of detailed pictures of areas inside the body, such as the chest or abdomen, are taken from different angles. The pictures were taken by a computer connected to an x-ray machine. The dye may be injected into a vein or swallowed to make organs or tissues show more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • Ultrasound procedure :a procedure in which high-energy sound waves (ultrasound) bounce off internal tissues or organs and create an echo. The echo forms a picture of body tissues called a sonogram. The picture can be printed to view later.

If tests show that there may be a soft tissue sarcoma, a biopsy is performed.

The type of biopsy depends in part on the size of the growth and whether it is close to the surface of the skin or deeper in the tissue. One of the following types of biopsy is usually used:

  • Core needle biopsy:removal of tissue with a wide needle. Several tissue samples are taken. This procedure can be guided by ultrasound, computed tomography, or MRI.

  • Postoperative biopsy:removal of part of the tumor or tissue sample.

  • Excision biopsy :removal of the entire tumor or an area of tissue that does not look normal. A pathologist examines tissue under a microscope for the presence of cancer cells. An excisional biopsy can be used to completely remove smaller tumors near the surface of the skin. This type of biopsy is rarely used because cancer cells may remain after the biopsy. If cancer cells remain, the cancer may return or spread to other parts of the body.

 

An MRI of the tumor is performed before the excisional biopsy. This is done to show where the original tumor formed and can be used to guide future surgery or radiation therapy.

Whenever possible, the surgeon who removes any tumor found should be involved in planning the biopsy. The placement of biopsy needles or incisions may affect the ability to remove the entire tumor during later surgery.

To plan the best treatment, the tissue sample taken during the biopsy must be large enough to determine the type of soft tissue sarcoma and perform other laboratory tests. Tissue samples will be taken from the primary tumor, lymph nodes, and other areas that may have cancer cells. The pathologist examines the tissue under a microscope to look for cancer cells and determine the type and grade of the tumor. The extent of the tumor depends on how abnormal the cancer cells look under the microscope and how quickly the cells divide. High- and intermediate-grade tumors usually grow and spread faster than low-grade tumors.

Because soft tissue sarcoma is difficult to diagnose, the tissue sample should be reviewed by a pathologist experienced in diagnosing soft tissue sarcoma.

One or more of the following laboratory tests may be performed to study tissue samples:

  • Molecular test :a laboratory test to check for certain genes, proteins, or other molecules in a sample of tissue, blood, or other body fluid. A molecular test may be done with other procedures, such as a biopsy, to help diagnose some types of cancer. Molecular tests check for certain gene or chromosomal changes that occur in some soft tissue sarcomas.

  • Reverse transcription polymerase chain reaction (RT-PCR) test :a laboratory test that measures the amount of a genetic substance called mRNA made by a particular gene. An enzyme called reverse transcriptase is used to convert a specific piece of RNA into a matching piece of DNA that can be amplified (done in large quantities) by another enzyme called DNA polymerase. Amplified DNA copies help determine if a particular mRNA is being created by a genome. RT-PCR can be used to check for the activation of certain genes that may indicate the presence of cancer cells. This test can be used to look for specific changes in a gene or chromosome that can help diagnose cancer.

  • Cytogenetic analysis:a laboratory test in which the chromosomes of cells in a sample of tumor tissue are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in some chromosomes can be a sign of cancer. Cytogenetic analysis is used to diagnose cancer, plan treatment, or determine the effectiveness of a treatment. Fluorescence in situ hybridization (FISH) is a type of cytogenetic analysis.

  • Immunocytochemistry:a laboratory test that uses antibodies to test for specific antigens (markers) in a sample of a patient's cells. The antibodies are usually associated with an enzyme or fluorescent dye. After the antibodies bind to the antigen in the patient's cell sample, the enzyme or dye is activated and the antigen can be seen under a microscope. This type of test can be used to tell the difference between different types of soft tissue sarcoma.

  • Light and electron microscopy:a laboratory test in which the cells in a tissue sample are looked at under a conventional and powerful microscope to look for specific changes in the cells.

After being diagnosed with soft tissue sarcoma in childhood, tests are done to see if the cancer cells have spread to other parts of the body.

The process used to determine if cancer has spread to soft tissues or to other parts of the body is called staging. There is no standard staging system for soft tissue sarcoma in children.

To plan treatment, it is important to know the type of soft tissue sarcoma, whether the tumor can be surgically removed, and whether the cancer has spread to other parts of the body.

To find out if the cancer has spread, the following procedures can be used:

  • Sentinel lymph node biopsy:removal of the sentinel lymph node during surgery. The sentinel lymph node is the first lymph node in the lymph node group to receive lymphatic drainage from the primary tumor. This is the first lymph node to which cancer can spread from the primary tumor. A radioactive substance and/or a blue dye is injected near the tumor. The substance or dye flows through the lymphatic pathways to the lymph nodes. The first lymph node to receive the substance or dye is removed. A pathologist examines tissue under a microscope to look for cancer cells. If no cancer cells are found, it may not be necessary to remove additional lymph nodes. Sometimes a sentinel lymph node is found in more than one group of nodes. This procedure is used for epithelioid and clear cell sarcomas.

  • CT (computed tomography) :a procedure that takes a series of detailed pictures of areas inside the body, such as the chest, taken from different angles. The pictures were taken by a computer connected to an x-ray machine. The dye can be injected into a vein or swallowed to make organs or tissues more clearly visible. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • PET scan :A PET scan is a procedure to detect malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into a vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. The cells of malignant tumors appear brighter in the picture because they are more active and consume more glucose than normal cells. This procedure is also called a positron emission tomography (PET) scan.

  • PET-CT scan :a procedure that combines PET scan and computed tomography (CT) images. PET and CT scans are performed simultaneously on the same machine. The images from both scans are combined to give a more detailed picture than either test could do on its own.

6.4. Types of soft tissue sarcoma tumors in children

There are many different types of soft tissue sarcomas.

Cells of each type of sarcoma look different under a microscope. Soft tissue tumors are grouped according to the type of soft tissue cells in which they first formed.

This summary is about the following types of soft tissue sarcomas:

Tumors of adipose tissue

Liposarcoma. This is a cancer of the fat cells. Liposarcoma usually forms in the fatty layer just under the skin. In children and adolescents, liposarcoma is often of low severity (it is likely to grow and spread slowly). There are several different types of liposarcomas, including:

  • Myxoid liposarcoma . It is usually a low-grade cancer that responds well to treatment.

  • Pleomorphic liposarcoma . It is usually a high-grade cancer that is less likely to be cured.

 

Tumors of bones and cartilage

Bone and cartilage tumors are a mixture of bone and cartilage cells. Tumors of bones and cartilage are of the following types:

  • Extraskeletal mesenchymal chondrosarcoma. This type of bone and cartilage tumor often affects young people and occurs in the head and neck. It is usually highly malignant (can grow rapidly) and can spread to other parts of the body. It may also return many years after treatment.

  • Extraskeletal osteosarcoma . This type of bone and cartilage tumor is very rare in children and adolescents. It will likely return after treatment and may spread to the lungs.

 

Tumors of fibrous (connective) tissue

Fibrous (connective tissue) tumors include the following types:

  • Desmoid fibromatosis (also called desmoid tumor or aggressive fibromatosis). This is a low grade fibrous tissue tumor (likely to grow slowly). It may return to nearby tissues but does not usually spread to distant parts of the body. Sometimes fibromatosis of the desmoid type can stop growing for a long time. In rare cases, the tumor may disappear without treatment.

Children sometimes develop desmoid tumors with a change in the APC gene. Changes in this gene can also cause familial adenomatous polyposis (FAP). FAP is a hereditary disease (passed from parents to offspring) in which many polyps (growths on mucous membranes) form on the inner walls of the colon and rectum. Genetic counseling may be required (discussing hereditary diseases and genetic testing options with a trained specialist).

  • Protuberan dermatofibrosarcoma . This is a tumor of the deep layers of the skin, which most often forms in the trunk, arms or legs. The cells of this tumor have a specific genetic change called a translocation (part of the COL1A1 gene switches places with part of the PDGFRB gene). To diagnose protuberans dermatofibrosarcoma, tumor cells are tested for this genetic change. Dermatofibrosarcoma protuberance usually does not spread to the lymph nodes or other parts of the body.

  • Inflammatory myofibroblastic tumor . This cancer is made up of muscle cells, connective tissue cells, and certain immune cells. It occurs in children and adolescents. It forms most commonly in the soft tissues, lungs, spleen, and breasts. It often returns after treatment but rarely spreads to distant parts of the body. Some genetic variation has been found in about half of these tumors.

  • Fibrosarcoma.

    • Pediatric fibrosarcoma (also called congenital fibrosarcoma). This type of fibrosarcoma usually occurs in children 1 year of age and younger and can be detected on prenatal ultrasound. This tumor grows rapidly and is often large at diagnosis. It rarely spreads to distant parts of the body. The cells of this tumor usually have some kind of genetic change called a translocation (part of one chromosome switches places with part of another chromosome). To diagnose childhood fibrosarcoma, tumor cells are tested for this genetic change. A similar tumor has been observed in older children, but it does not have the translocation that is often seen in younger children.

    • Fibrosarcoma in adults. This is the same type of fibrosarcoma that occurs in adults. The cells of this tumor do not have genetic changes characteristic of childhood fibrosarcoma. 

  • There are two types of fibrosarcoma in children and adolescents:

  • Myxofibrosarcoma . It is a rare fibrous tissue tumor that is less common in children than in adults.

  • Low grade fibromyxoid sarcoma . It is a slow-growing tumor that forms deep in the arms or legs and mostly affects young and middle-aged people. The tumor may return many years after treatment and spread to the lungs and chest lining. Lifelong monitoring is required.

  • Sclerosing epithelioid fibrosarcoma. This is a rare tumor of fibrous tissue that grows rapidly. It may return and spread to other parts of the body years after treatment. Long-term follow-up is required.

 

Skeletal muscle tumors

Skeletal muscles attach to bones and help the body move.

  • Rhabdomyosarcoma . Rhabdomyosarcoma is the most common soft tissue sarcoma in children 14 years of age and younger. (For more information, see PDQ summary for treatment of pediatric rhabdomyosarcoma.)

 

Smooth muscle tumors

Smooth muscles line the interior of blood vessels and hollow internal organs such as the stomach, intestines, bladder, and uterus.

  • Leiomyosarcoma . This smooth muscle tumor has been linked to the Epstein-Barr virus in children who also have HIV or AIDS. Leiomyosarcoma may also form as a second cancer in survivors of hereditary retinoblastoma, sometimes many years after initial treatment for retinoblastoma.

 

So-called fibrohistiocytic tumors

  • Plexiform fibrohistiocytic tumor . It is a rare tumor that usually affects children and young adults. The tumor usually starts as a painless growth on or just below the skin of the arm, hand, or wrist. Rarely, it can spread to nearby lymph nodes or the lungs.

 

Tumors of the nerve sheaths

The nerve sheath consists of protective layers of myelin that cover nerve cells that are not part of the brain or spinal cord. Nerve sheath tumors are of the following types:

  • Malignant tumor of the peripheral nerve sheath. Some children with peripheral nerve sheath cancer have a rare genetic disorder called neurofibromatosis type 1 (NF1). This tumor can be low grade or high grade.

  • Malignant newt tumor. These are very fast growing tumors that most often occur in children with NF1.

  • Ectomesenchymoma . It is a fast growing tumor that occurs mainly in children. Ectomesenchymomas can form in the eye socket, abdomen, arms, or legs.

 

Pericytic (perivascular) tumors

Pericytic tumors form in cells that surround blood vessels. Pericytic tumors are of the following types:

  • Myopericytoma . Pediatric hemangiopericytoma is a type of myopericytoma. Children younger than 1 year of age at diagnosis may have a better prognosis. In patients older than 1 year, infantile hemangiopericytoma is more likely to spread to other parts of the body, including the lymph nodes and lungs.

  • Childhood myofibromatosis. Children's myofibromatosis is another type of myopericytoma. This is a fibrous tumor that often forms in the first 2 years of life. There may be a single nodule under the skin, usually in the head and neck (myofibroma), or multiple nodules in the skin, muscles, or bones (myofibromatosis). In patients with infantile myofibromatosis, the cancer can also spread to organs. These tumors may disappear without treatment.

 

Tumors of unknown cellular origin

Tumors of unknown cellular origin (the type of cell in which the tumor first formed is unknown) include the following types:

  • Synovial sarcoma . Synovial sarcoma is a common type of soft tissue sarcoma in children and adolescents. It usually forms in the tissues around the joints of the arms or legs, but it can also form in the trunk, head, or neck. The cells of this tumor usually have some kind of genetic change called a translocation (part of one chromosome switches places with part of another chromosome). Larger tumors have a greater risk of spreading to other parts of the body, including the lungs. Children younger than 10 years of age who have a tumor that is 5 centimeters or smaller in the arms or legs have a better prognosis.

  • Epithelioid sarcoma . This is a rare sarcoma that usually starts deep in the soft tissues as a slowly growing hard lump and may spread to the lymph nodes. If cancer has formed in the arms, legs, or buttocks, a sentinel lymph node biopsy may be done to check for cancer in the lymph nodes.

  • Soft alveolar sarcomaparts . This is a rare tumor of soft, supportive tissue that connects and surrounds organs and other tissues. It most commonly occurs in the arms and legs, but can occur in the tissues of the mouth, jaws, and face. It can grow slowly and often spreads to other parts of the body. Soft alveolar sarcoma may have a better prognosis when the tumor is 5 centimeters or smaller, or when the tumor is completely removed by surgery. The cells of this tumor usually have a certain genetic change called a translocation (part of the ASSPL gene switches places with part of the TFE3 gene). To diagnose soft alveolar sarcoma, tumor cells are tested for this genetic change.

  • Soft tissue clear cell sarcoma . It is a slow-growing soft tissue tumor that starts in a tendon (tough, fibrous, umbilical cord tissue that connects a muscle to a bone or to another part of the body). Clear cell sarcoma most commonly occurs in the deep tissues of the foot, heel, and ankle. It can spread to nearby lymph nodes. The cells of this tumor usually have some kind of genetic change called a translocation (part of the EWSR1 gene switches places with part of the ATF1 or CREB1 gene). To diagnose clear cell soft tissue sarcoma, tumor cells are tested for this genetic change.

  • Extraskeletal myxoid chondrosarcoma. This type of soft tissue sarcoma can occur in children and adolescents. Over time, it tends to spread to other parts of the body, including the lymph nodes and lungs. The tumor may return many years after treatment.

  • Extraskeletal Ewing's sarcoma

  • Desmoplastic small cell tumor . This tumor most commonly forms in the peritoneum of the abdomen, pelvis, and/or peritoneum in the scrotum, but may form in the kidneys or other solid organs. Dozens of small tumors can form in the peritoneum. Desmoplastic small cell tumor can also spread to the lungs and other parts of the body. The cells of this tumor usually have some kind of genetic change called a translocation (part of one chromosome switches places with part of another chromosome). To diagnose a desmoplastic small cell tumor, tumor cells are tested for this genetic change.

  • Extrarenal (extracranial) rhabdoid tumor . This rapidly growing tumor forms in soft tissues such as the liver and bladder. It usually occurs in young children, including newborns, but may occur in older children and adults. Rhabdoid tumors may be associated with a change in a tumor suppressor gene called SMARCB1. This type of gene makes a protein that helps control cell growth. Changes in the SMARCB1 gene can be inherited. Genetic counseling may be required (discussion with a qualified specialist of hereditary diseases and the possible need for genetic testing).

  • Perivascular epithelioid cell tumors (PEComas).  Benign PEComas can occur in children with an inherited condition called tuberous sclerosis. They occur in the stomach, intestines, lungs and urinary organs. PEComas grow slowly and most of them are unlikely.

  • Undifferentiated/unclassified sarcoma. These tumors usually occur in the bones or muscles that are attached to the bones and help the body move.

    • Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma (high grade). This type of soft tissue tumor can form in parts of the body where patients have previously received radiation therapy, or as a second cancer in children with retinoblastoma. The tumor usually forms in the arms or legs and may spread to other parts of the body.

 

Tumors of blood vessels

Blood vessel tumors include the following types:

  • Epithelioid hemangioendothelioma . Epithelioid hemangioendotheliomas can occur in children but are most common in adults between the ages of 30 and 50. They usually originate in the liver, lungs, or bones. They can be either fast growing or slow growing. In about a third of cases, the tumor spreads very quickly to other parts of the body.

  • Angiosarcoma of soft tissues. Soft tissue angiosarcoma is a rapidly growing tumor that forms in the blood or lymph vessels anywhere in the body. Most angiosarcomas are in or under the skin. Those found in deeper soft tissues may form in the liver, spleen, or lungs. They are very rare in children, who sometimes have more than one tumor in the skin or liver. In rare cases, childhood hemangioma can become soft tissue angiosarcoma. 

Sometimes soft tissue sarcoma in children continues to grow or comes back after treatment.

Progressive soft tissue sarcoma in children is a cancer that continues to grow, spread, or get worse. Progressive disease may be a sign that the cancer has become refractory to treatment.

Recurrent soft tissue sarcoma in childrenis cancer that has recurred (come back) after treatment. The cancer could reappear in the same place or in other parts of the body.

6.5. Factors affecting the prognosis of recovery

Prognosis and treatment options depend on the following:

  • The part of the body where the tumor first formed.

  • Size and grade of the tumor.

  • type of soft tissue sarcoma.

  • How deep is the tumor under the skin.

  • Whether the tumor has spread to other parts of the body and where it has spread.

  • The amount of tumor left after surgery to remove it.

  • Whether radiation therapy was used to treat the tumor.

  • Whether the cancer was just diagnosed or recurred (came back).

6.6. Overview of treatment options for  children's soft tissue sarcoma

There are various methods of treatment for patients with soft tissue sarcoma in childhood.

A variety of treatments are available to patients with childhood soft tissue sarcoma. Some treatments are standard (currently used) and some are in clinical trials. A clinical trial of a treatment is a scientific study designed to help improve existing treatments or provide information about new treatments for cancer patients. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Since childhood cancer is rare, participation in clinical trials should be considered. Some clinical trials are only open to patients who have not yet started treatment.

Children with soft tissue sarcoma in childhood should be planned for treatment by a team of healthcare professionals who are experts in treating childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. A pediatric oncologist works with other health care providers who are experts in treating children with soft tissue sarcoma and specialize in specific areas of medicine. This may be a pediatric surgeon who has special training in the removal of soft tissue sarcomas. The following specialists may also be included:

  • Pediatrician.

  • Radiation Oncologist.

  • Pediatric hematologist.

  • Pediatric Nurse Specialist.

  • Rehabilitation Specialist.

  • Psychologist .

  • Social worker .

  • Children's Life Specialist.

 

Seven types of standard treatment are used:

1. Operation

If possible, an operation is performed to completely remove the soft tissue sarcoma. If the tumor is very large, radiation therapy or chemotherapy may be given first in order to shrink the tumor and reduce the amount of tissue that needs to be removed during surgery. This is called neoadjuvant (preoperative) therapy.

The following types of surgical interventions can be used:

  • Wide local excision : removal of the tumor along with the normal tissue around it.

  • Amputation :surgery to remove all or part of an arm or leg with cancer.

  • Lymphadenectomy :removal of lymph nodes for cancer.

  • Mohs surgery:a surgical procedure used to treat skin cancer. Individual layers of cancerous tissue are removed and examined under a microscope one at a time until all of the cancerous tissue has been removed. This type of surgery is used to treat protuberance dermatofibrosarcoma. It is also called Mohs micrographic surgery.

  • Hepatectomy : surgery to remove all or part of the liver.

 

A second operation may be required for:

  • Removal of all remaining cancer cells.

  • Check the area around where the tumor was removed for cancer cells and then remove more tissue if necessary.

 

If the cancer is in the liver, a hepatectomy and liver transplant (the liver is removed and replaced with a healthy one from a donor) may be done.

After the doctor removes any tumors that can be seen during surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any remaining cancer cells. Treatment given after surgery to reduce the risk of cancer coming back is called adjuvant therapy.

 

2. Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or prevent them from growing. There are two types of radiation therapy:

  • External Beam Therapy uses a device outside the body to direct radiation to an area of the body affected by cancer. Certain ways of delivering radiation therapy can help prevent radiation damage to nearby healthy tissue. This type of radiation therapy may include the following:

    • Stereotactic Body Radiation Therapy : Stereotactic Body Radiation Therapy is a type of external beam radiation therapy. Special equipment is used to position the patient in the same position for each radiation therapy session. Once a day for several days, the radiation machine delivers a larger than usual dose of radiation directly to the tumor. If the patient is in the same position for each treatment session, there is less damage to nearby healthy tissue. This procedure is also called stereotactic external beam radiation therapy and stereotactic radiation therapy.

    • Conformal radiotherapy:conformal radiation therapy is a type of external beam radiation therapy in which a computer creates a three-dimensional (3-D) image of the tumor and generates radiation beams that match the tumor. This allows a high dose of radiation to reach the tumor and cause less damage to nearby healthy tissue.

    • Intensity Modulated Radiation Therapy (IMRT): IMRT is a type of three-dimensional (3-D) radiation therapy that uses a computer to produce images of the size and shape of a tumor. Thin beams of radiation of different intensity (strength) are directed to the tumor at different angles. This type of external beam radiation therapy does less damage to nearby healthy tissue.

  • Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires or catheters that are injected directly into or near the tumor.

 

Whether radiation therapy is given before or after cancer surgery depends on the type and stage of cancer being treated, the presence of any cancer cells after surgery, and the expected side effects of the treatment. External and internal radiation therapy is used to treat soft tissue sarcoma in children.

 

3. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by either killing the cells or stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). When chemotherapy is injected directly into the cerebrospinal fluid, organ, or body cavity, such as the abdominal cavity, the drugs mainly target cancer cells in those areas (regional chemotherapy). Combination chemotherapy is the use of more than one anticancer drug.

Hyperthermic Intraperitoneal Chemotherapy (HIPEC)is a type of treatment used during surgery that is being studied for desmoplastic small cell tumor. After the surgeon has removed as much of the tumor tissue as possible, warm chemotherapy is directed directly into the abdomen.

Method of conducting chemotherapydepends on the type of soft tissue sarcoma being treated. Most types of soft tissue sarcomas do not respond to chemotherapy.

 

4. Surveillance

Observation is closely monitoring the patient's condition without giving any treatment until signs or symptoms appear or change. Observation may be carried out when:

  • Complete removal of the tumor is not possible.

  • There are no other treatments available.

  • The tumor is unlikely to damage any vital organs.

 

Observation may be used to treat desmoid-type fibromatosis, childhood fibrosarcoma, PEComa, or epithelioid hemangioendothelioma.

 

5. Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to target and target specific cancer cells. Targeted therapy usually causes less damage to normal cells than chemotherapy or radiation therapy.

  • Kinase inhibitors block an enzyme called a kinase (a type of protein).There are different types of kinases in the body that have different actions.

    • ALK inhibitors can stop the growth and spread of cancer. Crizotinib may be used to treat inflammatory myofibroblastic tumor, childhood fibrosarcoma, and soft tissue clear cell sarcoma.

    • Tyrosine kinase inhibitors (TKIs) block the signals necessary for tumor growth. Imatinib is used to treat protuberance dermatofibrosarcoma. Pazopanib may be used to treat desmoid-type fibromatosis, epithelioid hemangioendothelioma, and some types of recurrent and progressive soft tissue sarcoma. Sorafenib can be used to treat desmoid-type fibromatosis and epithelioid hemangioendothelioma. Sunitinib can be used to treat soft alveolar sarcoma. Larotrectinib is used to treat childhood fibrosarcoma. Ceritinib is used to treat inflammatory myofibroblastic tumor. Axitinib may be used to treat some types of advanced soft tissue sarcoma, including alveolar soft tissue sarcoma.

  • mTOR inhibitors are a type of targeted therapy that stops a protein that helps cells divide and survive. mTOR inhibitors are used to treat recurrent desmoplastic small cell tumors, PEComas, and epithelioid hemangioendothelioma and are also being studied to treat malignant peripheral nerve sheath tumors. Sirolimus and temsirolimus are mTOR inhibitor therapies 

 

New types of tyrosine kinase inhibitors are being studied, such as:

  • Entrectinib and selitrectinib in childhood fibrosarcoma.

  • Trametinib for epithelioid hemangioendothelioma.

6. Immunotherapy

Immunotherapy is a treatment that uses the patient's immune system to fight cancer. Substances produced by the body or produced in a laboratory are used to enhance, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biological therapy.

Interferon and immune checkpoint inhibitor therapy are types of immunotherapy.

  • Interferon prevents tumor cells from dividing and can slow tumor growth. It is used to treat epithelioid hemangioendothelioma.

  • Immune checkpoint inhibitor therapy: Some types of immune cells, such as T cells and some cancer cells, have certain proteins on their surface, called checkpoint proteins, that control immune responses. When cancer cells contain large amounts of these proteins, they will not be attacked and killed by T cells. Immune checkpoint inhibitors block these proteins, and the ability of T cells to kill cancer cells is increased.

    There are two types of immune checkpoint inhibitor therapy:

    • CTLA-4 inhibitor therapy: CTLA-4 is a protein on the surface of T cells that helps control the body's immune responses. When CTLA-4 attaches to another protein called B7 on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow T cells to kill cancer cells. Ipilimumab is a type of CTLA-4 inhibitor that is being studied for the treatment of angiosarcoma.

    • PD-1 and PD-L1 inhibitor therapy: PD-1 is a protein on the surface of T cells that helps control the body's immune responses. PD-L1 is a protein found on some types of cancer cells. When PD-1 attaches to PD-L1, it stops the T cell from killing the cancer cell. PD-1 and PD-L1 inhibitors prevent the PD-1 and PD-L1 proteins from attaching to each other. This allows T cells to kill cancer cells. Pembrolizumab is a type of PD-1 inhibitor that is used to treat progressive and recurrent soft tissue sarcoma. Nivolumab is a type of PD-1 inhibitor that is being studied for the treatment of angiosarcoma. Atezolizumab is a type of PD-L1 inhibitor that is being studied for the treatment of soft alveolar sarcoma.

 

7. Other drug therapy

Steroid therapy has an antitumor effect in inflammatory myofibroblastic tumors.

hormone therapyis a cancer treatment that removes or blocks hormones and stops the growth of cancer cells. Hormones are substances produced by glands in the body and circulate in the bloodstream. Certain hormones can cause certain types of cancer to grow. If tests show that cancer cells have places where hormones can attach (receptors), drugs, surgery, or radiation therapy are used to reduce the production of hormones or block them from working. Antiestrogens (drugs that block estrogen), such as tamoxifen, may be used to treat desmoid-type fibromatosis. Prasterone is being studied for the treatment of synovial sarcoma.

Non-steroidal anti-inflammatory drugs (NSAIDs) are drugs (such as aspirin, ibuprofen, and naproxen) commonly used to reduce fever, swelling, pain, and redness. When treating desmoid-type fibromatosis, an NSAID called sulindac can be used to help block the growth of cancer cells.

 

6.7. Treatment for soft tissue sarcoma in children can cause side effects.

 

Side effects from cancer treatment that begin after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include:

  • physical problems.

  • Changes in mood, feelings, thinking, learning, or memory.

  • Second cancer (new types of cancer).

6.8. Treatment options soft tissue sarcomas in children

Tumors of adipose tissue

 

Liposarcoma

Treatment for newly diagnosed liposarcoma may include the following:

  • Surgery to completely remove the tumor. If the cancer is not completely removed, a second operation may be done.

  • Chemotherapy to shrink the tumor followed by surgery.

  • Radiation therapy before or after surgery.

 

Tumors of bones and cartilage

Extraskeletal mesenchymal chondrosarcoma

Treatment for newly diagnosed extraskeletal mesenchymal chondrosarcoma may include the following:

  • Surgery to completely remove the tumor. Radiation therapy may be given before and/or after surgery.

  • Chemotherapy followed by surgery. After surgery, chemotherapy is prescribed with or without radiation therapy.

 

Extraskeletal osteosarcoma

Treatment for newly diagnosed extraskeletal osteosarcoma may include the following:

  • Surgery to completely remove the tumor followed by chemotherapy.

 

Fibrous (connective) tissue tumors

Fibromatosis desmoid type

Treatment for newly diagnosed desmoid-type fibromatosis may include the following:

  • Surveillance for asymptomatic tumors, tumors that cannot damage any vital organs, and tumors that cannot be completely removed by surgery.

  • Chemotherapy for tumors that have not been completely removed by surgery or that have recurred.

  • Targeted therapy (sorafenib or pazopanib).

  • Non-steroidal anti-inflammatory drugs (NSAIDs) therapy.

  • Antiestrogenic drug therapy.

  • Surgery to completely remove the tumor.

  • Radiation therapy .

  • Clinical study on targeted therapy with an inhibitor of the NOTCH pathway.

 

Protuberan dermatofibrosarcoma

Treatment for newly diagnosed protuberance dermatofibrosarcoma may include the following:

  • Surgery to completely remove the tumor, if possible. This may include the Mohs operation.

  • Radiation therapy before or after surgery.

  • Radiation therapy and targeted therapy (imatinib) if the tumor cannot be removed or has returned.

 

Inflammatory myofibroblastic tumor

 

Treatment for a newly diagnosed inflammatory myofibroblastic tumor may include the following:

  • Surgery to completely remove the tumor, if possible.

  • Chemotherapy.

  • Steroid therapy.

  • Non-steroidal anti-inflammatory drugs (NSAIDs) therapy.

  • Targeted therapy (crizotinib and ceritinib).

 

fibrosarcoma

Pediatric fibrosarcoma

Treatment for newly diagnosed childhood fibrosarcoma may include the following:

  • Surgery to remove the tumor, if possible, followed by follow-up.

  • Surgery followed by chemotherapy.

  • Chemotherapy to shrink the tumor followed by surgery.

  • Targeted therapy (crizotinib and larotrectinib).

  • A clinical trial that tests a sample of a patient's tumor for certain gene changes. The type of targeted therapy that will be administered to a patient depends on the type of gene change.

  • Clinical trials of targeted therapies (larotrectinib, entrectinib, or selitrectinib).

 

Fibrosarcoma in adults

Treatment for newly diagnosed fibrosarcoma in adults may include the following:

  • Surgery to completely remove the tumor, if possible.

  • Myxofibrosarcoma

  • Treatment for newly diagnosed myxofibrosarcoma may include the following:

  • Surgery to completely remove the tumor.

 

Fibromyxoid sarcoma, low grade

 

Treatment for newly diagnosed low-grade fibromyxoid sarcoma may include the following:

  • Surgery to completely remove the tumor.

 

Sclerosing epithelioid fibrosarcoma

Treatment for newly diagnosed sclerosing epithelioid fibrosarcoma may include the following:

  • Surgery to completely remove the tumor.

  • Skeletal muscle tumors

  • Rhabdomyosarcoma

 

Smooth muscle tumors

Leiomyosarcoma

Treatment for newly diagnosed leiomyosarcoma may include the following:

  • Chemotherapy.

 

So-called fibrohistiocytic tumors

Plexiform fibrohistiocytic tumor

Treatment for a newly diagnosed plexiform fibrohistiocytic tumor may include the following:

  • Surgery to completely remove the tumor.

 

Tumors of the nerve sheath

Malignant tumor of the peripheral nerve sheath

Treatment for a newly diagnosed malignant peripheral nerve sheath tumor may include the following:

  • Surgery to completely remove the tumor, if possible.

  • Radiation therapy before or after surgery.

  • Chemotherapy for tumors that cannot be removed surgically.

  • A clinical trial that tests a sample of a patient's tumor for certain gene changes. The type of targeted therapy that will be administered to a patient depends on the type of gene change.

  • Clinical trials of targeted therapy (ganetespib or sirolimus) for tumors that cannot be surgically removed.

  • Clinical trials of targeted therapy (tazemetostat).

 

It is unclear whether radiation therapy or chemotherapy after surgery improves tumor response to treatment.

Malignant triton tumor

Newly diagnosed malignant newt tumors can be treated in the same way as rhabdomyosarcomas and include surgery, chemotherapy, or radiation therapy. It is unclear whether radiation therapy or chemotherapy improves tumor response to treatment.

Ectomesenchymoma

Treatment for a newly diagnosed ectomesenchymoma may include the following:

  • Surgery.

  • Chemotherapy.

  • Radiation therapy .

 

Pericytic (perivascular) tumors

Pediatric hemangiopericytoma

Treatment for newly diagnosed childhood hemangiopericytoma may include the following:

  • Chemotherapy.

 

Childhood myofibromatosis

Treatment for newly diagnosed infantile myofibromatosis may include the following:

  • Combined chemotherapy.

 

Tumors of unknown cell origin (the place where the tumor first formed is unknown)

synovial sarcoma

Treatment for newly diagnosed synovial sarcoma may include the following:

  • Surgery. Radiation therapy and/or chemotherapy may be given before or after surgery.

  • Chemotherapy.

  • Stereotactic radiotherapy for tumors that have spread to the lungs.

  • Clinical Research on Gene Therapy.

  • Clinical Trials of Hormone Therapy.

 

Epithelioid sarcoma

Treatment for newly diagnosed epithelioid sarcoma may include the following:

  • If possible, surgery to remove the tumor.

  • Chemotherapy.

  • Radiation therapy before or after surgery.

  • Clinical study on targeted therapy (tazemetostat).

 

Sarcoma of the alveolar soft part

Treatment for newly diagnosed soft alveolar sarcoma may include the following:

  • Surgery to completely remove the tumor, if possible.

  • Radiation therapy before or after surgery if the tumor cannot be completely removed by surgery.

  • Targeted therapy (sunitinib).

  • Clinical study on targeted therapy (cediranib or sunitinib).

  • Clinical trials of immunotherapy (atezolizumab).

 

Clear cell soft tissue sarcoma

Treatment for newly diagnosed clear cell soft tissue sarcoma may include the following:

  • If possible, surgery to remove the tumor.

  • Radiation therapy before or after surgery.

  • Targeted therapy (crizotinib).

 

Extraskeletal myxoid chondrosarcoma

 

Treatment for newly diagnosed extraskeletal myxoid chondrosarcoma may include the following:

  • If possible, surgery to remove the tumor.

  • Radiation therapy .

  • Clinical study on targeted therapy (tazemetostat).

 

Extraskeletal Ewing's sarcoma

  • Four types of standard treatment are used:

    • Chemotherapy

    • Radiation therapy

    • Operation

    • High-dose chemotherapy with stem cell rescue

  • New treatments are undergoing clinical trials.

    • Targeted Therapy

    • Immunotherapy

 

Desmoplastic small cell tumor

There is no standard treatment for a newly diagnosed desmoplastic small round cell tumor. Treatment may include the following:

  • Surgery to completely remove the tumor, if possible.

  • Surgery and Hyperthermic Intraperitoneal Chemotherapy.

  • Chemotherapy followed by surgery.

  • Radiation therapy .

  • Chemotherapy and targeted therapy (temsirolimus) for recurrent tumors.

 

Extrarenal (extracranial) rhabdoid tumor

Treatment for a newly diagnosed extrarenal (extracranial) rhabdoid tumor may include the following:

  • If possible, surgery to remove the tumor.

  • Chemotherapy.

  • Radiation therapy .

  • A clinical trial that tests a sample of a patient's tumor for certain gene changes. The type of targeted therapy that will be administered to a patient depends on the type of gene change.

  • Clinical trials of targeted therapy (tazemetostat).

 

Perivascular epithelioid cell tumors (PEComas)

 

Treatment for newly diagnosed perivascular epithelioid cell tumors may include the following:

  • Surgery to remove the tumor.

  • Observation followed by surgery.

  • Targeted therapy (sirolimus) for tumors that have certain genetic changes and cannot be removed by surgery.

 

Undifferentiated / unclassified sarcoma

Undifferentiated pleomorphic sarcoma/malignant fibrous histiocytoma (high grade)

There is no standard treatment for these tumors.

 

Tumors of blood vessels

Epithelioid hemangioendothelioma

Treatment for newly diagnosed epithelioid hemangioendothelioma may include the following:

  • Observation.

  • If possible, surgery to remove the tumor.

  • Immunotherapy (interferon) and targeted therapy (thalidomide, sorafenib, pazopanib, sirolimus) for tumors that can spread.

  • Chemotherapy.

  • Total hepatectomy and liver transplantation for liver tumors.

  • Clinical study on targeted therapy ( trametinib ).

 

Soft tissue angiosarcoma

Treatment for newly diagnosed angiosarcoma may include the following:

  • Surgery to completely remove the tumor.

  • Combination of surgery, chemotherapy and radiotherapy for advanced angiosarcoma.

  • Targeted therapy (bevacizumab) and chemotherapy for angiosarcomas that started as infantile hemangiomas.

  • Clinical trial of chemotherapy with or without targeted therapy (Pazopanib).

  • Clinical trials for immunotherapy (nivolumab and ipilimumab).

 

Metastatic soft tissue sarcoma in children

Treatment for childhood soft tissue sarcoma that has spread to other parts of the body at diagnosis may include the following:

  • Chemotherapy and Radiation Therapy. Surgery may be done to remove tumors that have spread to the lung.

  • Stereotactic radiotherapy for tumors that have spread to the lungs.

Treatment of progressive or recurrent soft tissue sarcoma in children

 

Treatment for advanced or recurrent soft tissue sarcoma in children may include the following:

  • Surgery to remove cancer that has returned to where it first formed or has spread to the lungs.

  • Surgery followed by external or internal radiation therapy, if radiation therapy has not yet been performed.

  • Surgery to remove the cancerous arm or leg if radiation therapy has already been given.

  • Surgical treatment of recurrent synovial sarcoma with or without chemotherapy.

  • Chemotherapy.

  • Targeted therapy (Pazopanib or axitinib).

  • Immunotherapy (pembrolizumab).

  • Stereotactic radiation therapy to treat cancer that has spread to other parts of the body, especially the lungs.

  • Clinical trial of a new chemotherapy regimen with or without targeted therapy (Pazopanib).

  • A clinical trial that tests a sample of a patient's tumor for certain gene changes. The type of targeted therapy that will be administered to a patient depends on the type of gene change.

6.1.
6.2.
6.3
6.4.
6.5.
6.6
6.7.
6.8.

Vascular tumors in children are formed from cells that form blood or lymphatic vessels.

Vascular tumors can form from abnormal cells in the blood or lymph vessels anywhere in the body. They can be benign (not cancer) or malignant (cancer). There are many types of vascular tumors. The most common type of vascular tumor in children is childhood hemangioma, a benign tumor that usually resolves on its own.

Since malignant vascular tumors are rare in children, there is not much information about what is the best treatment.

 

7.1. The tests are used to detect (search for) and diagnose vascular tumors in children.

The following tests and procedures may be used:

  • Medical examination and history:examining the body to check for general signs of health, including checking for signs of disease such as lumps, lesions, or anything else that seems unusual. The patient's history, health habits, past illnesses, and treatments will also be recorded.

  • Ultrasound procedure :a procedure in which high-energy sound waves (ultrasound) bounce off internal tissues or organs and create an echo. The echo forms a picture of body tissues called a sonogram. The picture can be printed to view later.

     

  • CT (computed tomography) :a procedure in which a series of detailed photographs of areas within the body taken from different angles are taken. The pictures were taken by a computer connected to an x-ray machine. The dye may be injected into a vein or swallowed to make organs and tissues more visible. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • MRI (magnetic resonance imaging) :a procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (NMRI)

7.2. Groups of vascular tumors in children

 

Vascular tumors in children can be divided into four groups.

 

1. Benign tumors

 

Benign tumors are not cancer. This summary contains information about the following benign vascular tumors :

  • Children's hemangioma.

  • congenital hemangioma.

  • Benign vascular tumors of the liver.

  • Hemangioma spindle cell.

  • Epithelioid hemangioma.

  • Pyogenic granuloma (lobular capillary hemangioma).

  • Angiofibroma.

  • Juvenile angiofibroma of the nasopharynx.

 

2. Intermediate (locally aggressive) tumors

Interstitial tumors that are locally aggressive often spread to the area around the tumor. This review provides information on the following locally aggressive vascular tumors:

  • Kaposiform hemangioendothelioma and bundle angioma.

 

3. Intermediate (rarely metastasizing) tumors

Intermediate (rarely metastatic) tumors sometimes spread to other parts of the body. This summary provides information on the following vascular tumors that rarely metastasize:

  • Pseudomyogenic hemangioendothelioma.

  • Retiform hemangioendothelioma.

  • Papillary intralymphatic angioendothelioma.

  • Composite hemangioendothelioma.

  • Kaposi's sarcoma.

 

4. Malignant tumors

Malignant tumors are cancer. This summary provides information on the following vascular malignancies:

  • Epithelioid hemangioendothelioma.

  • Soft tissue angiosarcoma.

7.3. Overview of treatment options

There are various treatments for vascular tumors in children.

A variety of treatments are available for children with vascular tumors. Some treatments are standard (currently used) and some are in clinical trials. A clinical trial of a treatment is a scientific study designed to help improve existing treatments or provide information about new treatments. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.

Since vascular tumors are rare in children, participation in clinical trials should be considered. Some clinical trials are only open to patients who have not yet started treatment.

Treatment of children with childhood vascular tumors should be planned by a team of healthcare professionals who are experts in the treatment of childhood cancer.

Treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. A pediatric oncologist works with other pediatric providers who are experts in treating children with cancer and specialize in specific areas of medicine. This may include the following professionals:

  • Pediatric specialist in vascular anomalies (specialist in the treatment of vascular tumors in children).

  • Pediatric surgeon.

  • Orthopedic surgeon.

  • Radiation Oncologist.

  • Pediatric Nurse Specialist.

  • Rehabilitation Specialist.

  • Psychologist .

  • Social worker .

 

Treatment of vascular tumors in children may cause side effects.

 

Some treatments, such as chemotherapy and radiation therapy, cause side effects that continue or appear months or years after treatment ends. These are the so-called late effects. Late effects of treatment may include the following:

  • physical problems.

  • Changes in mood, feelings, thinking, learning, or memory.

  • Second cancer (new types of cancer).

 

Some late effects can be treated or controlled. It is important to talk to your child's doctors about possible late effects of certain treatments.

Eleven types of standard treatment are used:

1. Beta-blocker therapy

Beta blockers are drugs that lower blood pressure and heart rate. When used in patients with vascular tumors, beta-blockers may help shrink the tumor. Beta-blocker therapy may be given by vein (IV), by mouth, or placed on the skin (topical). The method of application of beta-blockers depends on the type of vascular tumors and where the tumor first formed.

The beta-blocker propranolol is usually the first drug to treat hemangiomas. Infants receiving intravenous propranolol may need to start treatment in the hospital. Propranolol is also used to treat benign vascular tumors in the liver and kaposiform hemangioendothelioma.

Other beta-blockers used to treat vascular tumors include atenolol, nadolol, and timolol.

Pediatric hemangioma can also be treated with propranolol and steroid therapy, or propranolol and topical beta-blocker therapy.

 

2. Operation

The following types of surgery can be used to remove many types of vascular tumors:

  • Excision :an operation to remove the entire tumor and part of the healthy tissue around it.

  • Laser surgery :a surgical procedure in which a laser beam (a narrow beam of intense light) is used as a knife to make bloodless cuts in tissue or remove skin lesions such as a tumor. For some hemangiomas, a pulsed dye laser can be used. This type of laser uses a beam of light that affects the blood vessels in the skin. The light is converted to heat and the blood vessels are destroyed without damaging the adjacent skin.

  • Curettage :a procedure in which abnormal tissue is removed using a small, spoon-shaped instrument called a curette.

  • Total hepatectomy and liver transplant:a surgical procedure to remove the entire liver, followed by transplantation of a healthy liver from a donor.

 

The type of surgery used depends on the type of vascular tumor and its location in the body.

For malignant tumors, after the doctor has removed all tumors that can be seen during surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any remaining cancer cells. Treatment given after surgery to reduce the risk of cancer coming back is called adjuvant therapy.

3. Photocoagulation

Photocoagulation  is the use of an intense beam of light, such as a laser, to close blood vessels or destroy tissue. Used to treat pyogenic granuloma.

4. Embolization

Embolization is a procedure in which particles, such as tiny gelatin sponges or beads, block blood vessels in the liver. It can be used to treat certain benign vascular tumors of the liver and kaposiform hemangioendothelioma.

5. Chemotherapy

Chemotherapyis a treatment that uses drugs to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. There are different ways of giving chemotherapy:

  • Systemic chemotherapy:when chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach tumor cells throughout the body. Sometimes more than one anticancer drug is prescribed. This is called combination chemotherapy.

  • Local chemotherapy:when chemotherapy is applied to the skin in the form of a cream or lotion, the drugs primarily target the tumor cells in the treated area.

  • Regional chemotherapy:when chemotherapy is injected directly into the cerebrospinal fluid, organ, or body cavity, such as the abdominal cavity, the drugs mainly target tumor cells in those areas.

 

How chemotherapy is given depends on the type of vascular tumor being treated. Systemic and local chemotherapy are used to treat some vascular tumors.

6. Sclerotherapy

Sclerotherapy  is a treatment used to destroy a blood vessel leading to swelling and swelling. Fluid is injected into the blood vessel, causing it to become scarred and destroyed. Over time, the destroyed blood vessel is absorbed into normal tissues. Instead, blood flows through nearby healthy veins. Sclerotherapy is used in the treatment of epithelioid hemangioma.

7. Radiation therapy

Radiation therapyis a treatment that uses high-energy x-rays or other types of radiation to kill tumor cells or prevent their growth. There are two types of radiation therapy:

  • External Beam Therapy  uses a device located outside the body to direct radiation at a tumor.

  • Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires or catheters that are injected directly into or near the tumor.

 

The way radiation therapy is given depends on the type of vascular tumor being treated. External radiation is used to treat some vascular tumors.

8. Targeted therapy

Targeted Therapy  is a type of treatment that uses drugs or other substances to attack specific tumor cells. Targeted therapy usually causes less damage to normal cells than chemotherapy or radiation therapy. Various types of targeted therapies are being used or are being studied to treat vascular tumors in children:

  • Angiogenesis inhibitors : angiogenesis inhibitors are drugs that stop cell division and prevent the growth of new blood vessels needed for tumor growth. The targeted therapy drugs thalidomide, sorafenib, pazopanib, and sirolimus are angiogenesis inhibitors used to treat vascular tumors in children.

  • Mammalian target for rapamycin (mTOR) inhibitors: mTOR inhibitors block a protein called mTOR, which can inhibit the growth of cancer cells and prevent the growth of new blood vessels needed for tumor growth.

  • Kinase inhibitors: kinase inhibitors block the signals necessary for tumor growth. Trametinib is being studied for the treatment of epithelioid hemangioendothelioma.

 

9. Immunotherapy

Immunotherapy  is a treatment that uses the patient's immune system to fight disease. Substances produced by the body or produced in a laboratory are used to enhance, direct, or restore the body's natural defenses against disease.

In the treatment of vascular tumors in children, the following types of immunotherapy are used:

  • Interferon  is a type of immunotherapy used to treat vascular tumors in children. It prevents tumor cells from dividing and can slow tumor growth. It is used in the treatment of juvenile angiofibroma of the nasopharynx, kaposiform hemangioendothelioma, and epithelioid hemangioendothelioma.

  • Therapy with immune checkpoint inhibitors:some types of immune cells, such as T cells and some cancer cells, have certain proteins on their surface called checkpoint proteins that control immune responses. When cancer cells contain large amounts of these proteins, they will not be attacked and killed by T cells. Immune checkpoint inhibitors block these proteins, and the ability of T cells to kill cancer cells is increased.

    There are two types of immune checkpoint inhibitor therapy:

  • CTLA-4 inhibitor: CTLA-4 is a protein on the surface of T cells that helps control the body's immune responses. When CTLA-4 attaches to another protein called B7 on a cancer cell, it stops the T cell from killing the cancer cell. CTLA-4 inhibitors attach to CTLA-4 and allow T cells to kill cancer cells. Ipilimumab is a type of CTLA-4 inhibitor being studied in the treatment angiosarcomas of soft tissue .

 

10. Other drug therapy

Other medicines used to treat or reverse the effects of vascular tumors in children include the following:

  • Steroid Therapy: Steroidsare hormones produced naturally by the body. They can also be made in a laboratory and used as medicines. Steroid drugs help reduce vascular tumors. Corticosteroids such as prednisone and methylprednisolone are used to treat childhood hemangioma.

  • Non-steroidal anti-inflammatory drugs (NSAIDs):NSAIDs are commonly used to reduce fever, swelling, pain, and redness. Examples of NSAIDs are aspirin, ibuprofen and naproxen. In the treatment of vascular tumors, NSAIDs can increase blood flow through tumors and reduce the chance of unwanted thrombus formation.

  • Antifibrinolytic therapy:these drugs aid blood clotting in patients with Kasabach-Merritt syndrome. Fibrin is the main protein in a blood clot that helps stop bleeding and heal wounds. Some vascular tumors cause fibrin to break down and the patient's blood does not clot normally, causing uncontrolled bleeding. Antifibrinolytics help prevent the breakdown of fibrin.

 

11. Surveillance

Observation is closely monitoring the patient's condition without giving any treatment until signs or symptoms appear or change.

 

7.4. benign tumors

In this section

  1. Pediatric hemangioma

  2. congenital hemangioma

  3. Benign vascular tumors of the liver

  4. Hemangioma spindle cell

  5. Epithelioid hemangioma

  6. Pyogenic granuloma

  7. Angiofibroma

  8. Juvenile angiofibroma of the nasopharynx

 

1. Pediatric hemangioma

Infantile hemangiomas- the most common type of benign vascular tumor in children. Baby hemangiomas form when immature cells that should form blood vessels form a tumor instead. An infantile hemangioma can also be called a "strawberry mark".

These tumors do not usually appear at birth, but appear between 3 and 6 weeks of age. Most hemangiomas enlarge for about 5 months and then stop growing. Hemangiomas gradually disappear over the next few years, but a red mark or loose or wrinkled skin may remain. Infantile hemangioma rarely comes back.

Childhood hemangiomas can be on the skin, in tissues under the skin, and/or in an organ. They are usually found on the head and neck, but can be anywhere on the body or anywhere. Hemangiomas may present as a single lesion, one or more lesions spreading over a large area of the body, or multiple lesions in more than one part of the body. Lesions that spread over a large area of the body or multiple lesions are more likely to cause problems.

Infantile hemangioma with minimal or delayed growth (IH-MAG)is a specific type of infantile hemangioma that occurs at birth and does not tend to increase. The lesion appears as light and dark patches of redness on the skin. The lesions are usually found on the lower body, but may be on the head and neck. This type of hemangiomas resolves over time without treatment.

 

Risk factors

Anything that increases the risk of a disease is called a risk factor. Having a risk factor does not mean you will get sick; the absence of risk factors does not mean that you will not get sick. Talk to your child's doctor if you think your child may be at risk.

Infantile hemangiomas are more common in the following cases:

  • Girls.

  • White.

  • Premature babies.

  • Twins, triplets or other multiple births.

  • Babies from mothers who are older at the time of pregnancy or who have problems with the placenta during pregnancy.

 

Other risk factors for infantile hemangiomas include the following:

  • A family history of childhood hemangioma, usually in the mother, father, sister, or brother.

  • Presence of certain syndromes:

  • PHACE Syndrome:a syndrome in which the hemangioma spreads to most of the body (usually the head or face). Other health problems may also occur that affect large blood vessels, the heart, eyes, and/or brain.

  • LUMBAR/Pelvic/CUT Syndrome :a syndrome in which the hemangioma extends over much of the lower back. Other health problems may also occur that affect the urinary system, reproductive organs, rectum, anus, brain and spinal cord, and nerve function.

 

Having more than one hemangioma, respiratory hemangioma, or ophthalmic hemangioma increases the risk of other health problems.

  • Multiple hemangiomas:the presence of more than five hemangiomas on the skin is a sign that there may be hemangiomas in the organ. Liver dependent most often. Heart, muscle, and thyroid problems may also occur.

  • Airway hemangiomas:airway hemangiomas usually occur along with a large beard-shaped hemangioma on the face (from the ears, around the mouth, lower chin, and front of the neck). It is important to treat airway hemangiomas before the child has breathing problems.

  • Ophthalmic hemangiomas:hemangiomas that affect the eye can cause vision problems or blindness. Infantile hemangiomas can occur in the conjunctiva (the membrane that lines the inside of the eyelid and covers the front of the eye). These hemangiomas may be associated with other abnormal eye conditions. It is important that children with ophthalmic hemangioma be evaluated by an ophthalmologist.

 

Signs and symptoms

Childhood hemangiomas can cause any of the following signs and symptoms. Check with your child's doctor if your child has any of the following:

  • Skin lesions:an area of spider veins, as well as clarified or discolored skin, may appear earlier than a hemangioma. Hemangiomas are hard, warm, bright red or crimson skin lesions or may look like a bruise. Lesions that form ulcers are also painful. Later, as the hemangiomas pass, they begin to pale in the center, then become flatter and lose color.

  • Skin lesions:lesions that grow under the skin in fatty tissue may appear blue or purple. If the lesions are deep enough under the surface of the skin, they may not be noticed.

  • Lesions in the body:signs of hemangioma formation in the organ may be absent.

 

While most childhood hemangiomas are nothing to worry about, if your child develops bumps or red or blue spots on their skin, check with your child's doctor. If necessary, he can refer the child to a specialist.

 

Diagnostic tests

Medical examination and historyis usually all that is needed to diagnose infantile hemangiomas. If there is anything unusual in the tumor, a biopsy may be done. If the hemangioma is deeper inside the body with no skin changes, or the lesions spread over a large area of the body, an ultrasound may be performed. 

If hemangiomas are part of the syndrome, additional tests may be performed such as an echocardiogram, MRI, magnetic resonance angiogram, and an eye exam.

Treatment

Most hemangiomas disappear and shrink without treatment. If the hemangioma is large or causing other health problems, treatment may include the following:

  • Propranolol or other beta-blocker therapy.

  • Steroid therapy before starting beta-blocker therapy or when beta-blockers cannot be used.

  • Pulsed dye laser surgery for hemangiomas that have ulcers or have not completely disappeared.

  • Surgical intervention (excision) of hemangiomas that have ulcers, have vision problems, or have not completely disappeared. Surgery may also be used for facial lesions that do not respond to other treatments.

  • Local therapy with beta-blockers for hemangiomas in one area of the skin.

  • Combination therapy such as propranolol and steroid therapy or propranolol and topical beta-blocker therapy.

  • Clinical trial with beta-blockers (Nadolol and propranolol).

  • Clinical trials of local therapy with beta-blockers (timolol).

 

2. Congenital hemangioma

congenital hemangiomais a benign vascular tumor that begins to form even before birth and is fully formed at the birth of a child. They are usually found on the skin, but may be elsewhere. Congenital hemangioma may appear as a rash of purple patches, with the skin around the patch may be lighter in color.

There are three types of congenital hemangiomas:

  • Rapidly invasive congenital hemangioma:these tumors go away on their own 12 to 15 months after birth. They can ulcerate, bleed, and cause temporary problems with blood and heart clotting. The skin may look slightly different even after the hemangiomas are gone.

  • Partial involutional congenital hemangioma:these tumors do not disappear completely.

  • Non-involutional congenital hemangioma:these tumors never go away on their own.

Treatment

Treatment for rapidly involuting congenital hemangioma and partial involutive congenital hemangioma may include the following:

  • Only observation.

 

Treatment for non-involutional congenital hemangioma may include the following:

  • Surgery to remove the tumor, depending on where it is located and whether it is causing symptoms.

 

3. Benign vascular tumors of the liver

Benign vascular tumors of the liver can be focal vascular lesions (single lesion in one area of the liver), multiple hepatic lesions (multiple lesions in one area of the liver), or diffuse hepatic lesions (multiple lesions in more than one area of the liver).

The liver performs many functions, including filtering the blood and making proteins needed for blood clotting. Sometimes the blood that normally flows through the liver is blocked or slowed down by the tumor. This directs blood directly to the heart without passing through the liver and is called a hepatic shunt. This can cause heart failure and blood clotting problems.

Focal vascular lesions

Focal vascular lesions are usually rapidly involutional congenital hemangiomas or non-involutional congenital hemangiomas.

 

Treatment

Treatment of focal vascular lesions of the liver depends on the presence of symptoms and may include the following:

  • Observation.

  • Medicines to treat symptoms including heart failure and bleeding problems.

  • Embolization of the liver to treat symptoms.

  • Surgery for lesions not amenable to other treatment.

 

 Multiple and diffuse liver lesions

Multifocal and diffuse liver lesions are usually infantile hemangiomas. Diffuse liver damage can cause serious consequences, including thyroid and heart problems. The liver may enlarge, press on other organs, and cause more symptoms.

 

Treatment

Treatment for multifocal liver lesions may include the following:

  • Monitor for lesions that do not cause symptoms.

  • Therapy with beta-blockers (propranolol) for lesions that are starting to grow.

  • Treatment for diffuse liver disease may include the following:

  • Beta-blocker therapy (propranolol).

  • Chemotherapy.

  • Steroid therapy.

  • Total hepatectomy and liver transplantation when lesions are not amenable to drug therapy. This is done only when the lesions have spread widely in the liver and more than one organ has failed.

 

If a vascular lesion in the liver does not respond to standard treatment, a biopsy may be done to see if the tumor has become malignant.

4. Hemangioma spindle cell

Spindle cell hemangiomas contain cells called spindle cells. Under a microscope, spindle cells look long and thin.

Risk factors

Anything that increases the risk of a disease is called a risk factor. Having a risk factor does not mean you will get sick; the absence of risk factors does not mean that you will not get sick. Talk to your child's doctor if you think your child may be at risk. Spindle cell hemangiomas are more common in children with the following syndromes:

  • Maffucci syndrome, which affects cartilage and skin.

  • Klippel-Trenaune syndrome, which affects blood vessels, soft tissues, and bones.

  • Signs

  • Spindle cell hemangiomas appear on or under the skin. These are painful red-brown or bluish growths that usually appear on the arms or legs. They can start as one defeat and develop into new ones over time.

 

Treatment

 

There is no standard treatment for spindle cell hemangiomas. Treatment may include the following:

  • Surgery to remove the tumor.

  • Spindle cell hemangiomas may return after surgery.

 

5. Epithelioid hemangioma

Epithelioid hemangiomas usually form on or in the skin, especially on the head, but can also occur in other areas, such as bones.

Signs and symptoms

  • Epithelioid hemangiomas sometimes result from trauma. On the skin, they may look like hard pink or red bumps and may be itchy.

  • An epithelioid hemangioma of bone can cause swelling, pain, and weakening of the bone in the affected area.

 

Treatment

There is no standard treatment for epithelioid hemangiomas. Treatment may include the following:

  • Operation (curettage or resection).

  • Sclerotherapy.

  • Radiation therapy in rare cases.

  • Epithelioid hemangiomas often return after treatment.

 

6. Pyogenic granuloma

Pyogenic granuloma is also called lobular capillary hemangioma. It is most common in older children and young adults, but can occur at any age.

These lesions sometimes result from injury or from the use of certain medications, including birth control pills and retinoids. They can also form, for no known reason, inside capillaries (tiny blood vessels), arteries, veins, or other places on the body. Usually there is only one lesion, but sometimes multiple lesions occur in the same area or lesions may spread to other areas of the body.

 

Symptoms

Pyogenic granulomas are raised, bright red masses that can be small or large, smooth or bumpy. They grow rapidly over weeks or months and may bleed heavily. These lesions are usually found on the surface of the skin, but may form in the tissues under the skin and look like other vascular lesions.

 

Treatment

Some pyogenic granulomas go away without treatment. Other pyogenic granulomas require treatment, which may include the following:

  • Operation (excision or curettage) to remove the lesion.

  • Photocoagulation.

  • Local therapy with beta-blockers.

  • Pyogenic granulomas often return after treatment.

 

7. Angiofibroma

Angiofibromas are rare. These are benign skin lesions that usually occur in a condition called tuberous sclerosis (an inherited disorder that causes skin lesions, seizures, and mental disorders).

Symptoms

Angiofibromas look like red bumps on the face.

 

Treatment

Treatment for angiofibroma may include the following:

  • Operation (excision) to remove the tumor.

  • laser therapy.

  • Targeted therapy (sirolimus).

 

8. Juvenile angiofibroma of the nasopharynx

Juvenile angiofibromas of the nasopharynx are benign tumors, but they can grow into nearby tissues. They begin in the nasal cavity and may spread to the nasopharynx, paranasal sinuses, bones around the eyes, and sometimes to the brain.

Treatment

Treatment for juvenile nasopharyngeal angiofibromas may include the following:

  • Surgery (excision) to remove the tumor.

  • Radiation therapy .

  • Chemotherapy.

  • Immunotherapy (interferon).

  • Targeted therapy (sirolimus).

 

7.5. Intermediate tumors spreading locally

 

Kaposiform hemangioendothelioma and crested angioma

Kaposiform hemangioendotheliomas and tufted angiomas are blood vessel tumors that occur in infants or young children. These tumors can cause the Kasabagh-Merritt phenomenon, a condition in which blood cannot clot and severe bleeding can occur. In the Kasabah-Merritt phenomenon, the tumor traps and destroys platelets (blood clotting cells). Then there are not enough platelets in the blood to stop the bleeding. This type of vascular tumor is unrelated to Kaposi's sarcoma.

Signs and symptoms

  • Kaposiform hemangioendotheliomas and fascicular angiomas usually occur on the skin of the arms and legs, but can also form in deeper tissues such as muscle or bone, or in the chest or abdomen.

  • Signs and symptoms may include the following:

  • Dense painful areas of skin with bruises.

  • Purple or brownish-red patches of skin.

  • Light bruising.

  • More severe than normal bleeding from mucous membranes, wounds, and other tissues.

  • Patients with kaposiform hemangioendothelioma and tuft angioma may have anemia (weakness, feeling tired, or pale).

 

If the physical examination and MRI clearly show that the tumor is a kaposiform hemangioendothelioma or tufted angioma, a biopsy may not be required. A biopsy is not always done because serious bleeding can occur.

 

Treatment

Treatment of kaposiform hemangioendotheliomas and tufted angiomas depends on the child's symptoms. Infection, delay in treatment, and surgery can cause life-threatening bleeding. Kaposiform hemangioendotheliomas and tufted angiomas are best treated by a specialist in vascular anomalies.

Treatment and supportive care to stop bleeding may include the following:

  • Steroid therapy, which may be followed by chemotherapy.

  • Non-steroidal anti-inflammatory drugs (NSAIDs) such as aspirin.

  • Immunotherapy (interferon).

  • Antifibrinolytic therapy to improve blood clotting.

  • Chemotherapy with one or more anticancer drugs.

  • Beta-blocker therapy (propranolol).

  • Surgery ( excision ) to remove the tumor with or without embolization .

  • Targeted therapy (sirolimus) with or without steroid therapy.

  • Clinical trial of chemotherapy or targeted therapy (sirolimus).

 

Even after treatment, these tumors do not completely go away and may return. Pain and inflammation may increase with age, often around puberty.

 

Long-term effects include chronic pain, heart failure, bone problems, and lymphedema (accumulation of lymph fluid in tissues).

 

7.6. Interstitial tumors that rarely spread

 

In this section

1) Pseudomyogenic hemangioendothelioma

2) Retiform hemangioendothelioma

3) Papillary intralymphatic angioendothelioma

4) Composite hemangioendothelioma

5) Kaposi's sarcoma

 

1) Pseudomyogenic hemangioendothelioma

Pseudomyogenic hemangioendothelioma can occur in children but is most common in men between the ages of 20 and 50. These tumors are rare and usually occur on or under the skin or in the bone. They may spread to nearby tissues but do not usually spread to other parts of the body. In most cases, multiple tumors occur.

Signs and symptoms

  • Pseudomyogenic hemangioendotheliomas may present as a soft tissue lump or cause pain in the affected area.

 

Treatment

Treatment for pseudomyogenic hemangioendotheliomas may include the following:

  • If possible, surgery to remove the tumor. If there are multiple tumors in the bone, amputation may be required.

  • Chemotherapy.

  • Targeted therapy (mTOR inhibitors).

  • Because pseudomyogenic hemangioendothelioma is very rare in children, treatment options are based on clinical trials in adults.

 

2) Retiform hemangioendothelioma

Reform hemangioendotheliomas are slow-growing, flat tumors that occur in young people and sometimes in children. These tumors usually occur on or under the skin of the arms, legs, and torso. These tumors usually do not spread to other parts of the body.

 

Treatment

Treatment for retiform hemangioendotheliomas may include the following:

  • Operation (excision) to remove the tumor. Follow-up will include monitoring to see if the tumor comes back.

  • Radiation therapy and chemotherapy when surgery cannot be performed or when the tumor has returned.

 

Retiform hemangioendothelioma may return after treatment.

 

3) Papillary intralymphatic angioendothelioma

 

Papillary intralymphatic angioendotheliomas are also called Dubsky tumors. These tumors form in or under the skin anywhere on the body. Sometimes the lymph nodes are affected.

 

Symptoms

Papillary intralymphatic angioendotheliomas may appear as firm, raised purplish bumps that may be small or large.

 

Treatment

Treatment for papillary intralymphatic angioendotheliomas may include the following:

  • Operation (excision) to remove the tumor.

 

4) Composite hemangioendothelioma

Compound hemangioendotheliomas have features of both benign and malignant vascular tumors. These tumors usually occur on or under the skin of the hands or feet. They can also occur on the head, neck, or chest. Composite hemangioendotheliomas are unlikely to metastasize (spread), but they may reappear in the same location. When a tumor metastasizes, it usually spreads to nearby lymph nodes.

 

Treatment

Treatment for composite hemangioendotheliomas may include the following:

  • Surgery to remove the tumor.

  • Radiation therapy and chemotherapy for advanced tumors.

 

5) Kaposi's sarcoma

Kaposi's sarcoma is a cancer that causes lesions to grow on the skin; on the mucous membranes that line the mouth, nose and throat; The lymph nodes ; or other bodies. It is caused by the Kaposi's sarcoma herpes virus (KSHV). In the United States, it occurs most often in children who have a weak immune system caused by rare immune system diseases, HIV infection, or drugs used in organ transplants.

 

Symptoms

Signs in children may include the following:

  • Lesions of the skin, mouth or throat. Skin lesions are red, purple, or brown in color and vary from flat to raised, scaly areas called plaques, and nodules.

  • Enlarged lymph nodes.

 

Treatment

Treatment for Kaposi's sarcoma may include the following:

  • Chemotherapy.

  • Immunotherapy (interferon).

  • Radiation therapy .

 

Because Kaposi's sarcoma is very rare in children, some treatment options are based on clinical trials in adults. 

 

7.7. Malignant tumors

 

In this section

1) Epithelioid hemangioendothelioma

2) Angiosarcoma of soft tissues

 

1) Epithelioid hemangioendothelioma

Epithelioid hemangioendotheliomas can occur in children but are most common in adults between the ages of 30 and 50. They usually originate in the liver, lungs, or bones. They may grow quickly or slowly. In about a third of cases, the tumor spreads very quickly to other parts of the body.

Signs and symptoms

Signs and symptoms depend on where the tumor is located:

  • On the skin, the tumors may be raised, round, or flat, reddish-brown patches that feel warm.

  • In the lungs, there may be no early symptoms. Signs and symptoms that may occur include:

  • Chest pain.

  • Spitting up blood.

  • Anemia (weakness, feeling tired or pale).

  • Breathing problems (due to scarring of the lung tissue).

  • In bones, tumors can cause fractures.

  • Tumors that originate in the liver or soft tissues can also cause signs and symptoms.

 

Diagnostic tests

Epithelioid hemangioendotheliomas in the liver are detected using computed tomography and magnetic resonance imaging. You can also do x-rays.

Treatment

Treatment for slow-growing epithelioid hemangioendotheliomas includes the following:

  • Observation.

  • Treatment for rapidly growing epithelioid hemangioendotheliomas may include the following:

  • If possible, surgery to remove the tumor.

  • Immunotherapy (interferon) and targeted therapy (thalidomide, sorafenib, pazopanib, sirolimus) for tumors that can spread.

  • Chemotherapy.

  • Total hepatectomy and liver transplantation for liver tumors.

  • Clinical study on targeted therapy ( trametinib ).

  • Clinical trials of chemotherapy and targeted therapy (pazopanib).

 

2) Angiosarcoma of soft tissues

Angiosarcomas are fast-growing tumors that form in the blood or lymph vessels anywhere in the body, usually the soft tissues. Most angiosarcomas are on or near the skin. Those found in deeper soft tissues can form in the liver, spleen, and lungs.

These tumors are very rare in children. Children sometimes have more than one tumor in the skin and/or liver.

 

Risk factors

Anything that increases the risk of a disease is called a risk factor. Having a risk factor does not mean you will get sick; the absence of risk factors does not mean that you will not get sick. Talk to your child's doctor if you think your child may be at risk. Risk factors for angiosarcoma include the following:

  • Exposure to radiation.

  • Chronic (long-term) lymphedema is a condition in which excess lymphatic fluid accumulates in the tissues, causing swelling.

  • Having a benign vascular tumor. A benign tumor such as a hemangioma can become an angiosarcoma, but this is rare.

 

Symptoms

Signs of angiosarcoma depend on where the tumor is located and may include the following:

  • Red spots on the skin that bleed easily.

  • Purple Tumors.

Treatment

 

Treatment for angiosarcoma may include the following:

  • Surgery to completely remove the tumor.

  • Combination of surgery, chemotherapy and radiotherapy for advanced angiosarcoma.

  • Targeted therapy (bevacizumab) and chemotherapy for angiosarcomas that started as infantile hemangiomas.

  • Clinical trial of chemotherapy with or without targeted therapy (Pazopanib).

  • Clinical trials for immunotherapy (nivolumab and ipilimumab).

7. TREATMENT OF VASCULAR TUMORS IN CHILDREN 

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8. TREATMENT OF EUING'S SARCOMA

Якорь 8

Ewing's sarcoma is a type of tumor that forms in bone or soft tissue.

Ewing's sarcoma  is a type of tumor that forms from a specific type of cells in bone or soft tissue. Ewing's sarcoma can be found in the bones of the legs, arms, feet, hands, chest, pelvis, spine, or skull. Ewing's sarcoma can also be found in the soft tissues of the trunk, arms, legs, head, neck, and retroperitoneal space (the area in the back of the abdomen behind the tissue that lines the abdominal wall and covers most of the abdominal organs). or in other areas.

Ewing's sarcoma is most common in adolescents and young adults (from adolescence to 25 years).

Ewing's sarcoma has also been called peripheral primitive neuroectodermal tumor, Askin's tumor (Ewing's sarcoma of the chest wall), extraosseous Ewing's sarcoma (Ewing's sarcoma in tissues other than bone), and the Ewing's sarcoma family of tumors.

Undifferentiated round cell sarcoma can also occur in bones or soft tissues.

 

Undifferentiated round cell sarcoma usually occurs in the bones or muscles that attach to the bones and help the body move. There are three types of undifferentiated round cell sarcoma that are treated as Ewing's sarcoma:

  • Undifferentiated round cell sarcoma with rearrangements BCOR-CCNB3. This type of bone tumor usually forms in the pelvis, arms, or legs. It can spread to other parts of the body. In this type of round cell sarcoma, the BCOR gene is linked to the CCNB3 gene. To diagnose round cell sarcoma, tumor cells are tested for this gene change.

  • Undifferentiated round cell sarcoma with rearrangements CIC-DUX4. This type of soft tissue tumor usually forms in the trunk, arms, or legs. It is most common in men and young adults between the ages of 21 and 40. In this type of round cell sarcoma, the CIC gene is linked to the DUX4 gene. To diagnose round cell sarcoma, tumor cells are tested for this gene change.

  • Undifferentiated round cell sarcoma with CIC-NUTM1 rearrangements. This type of soft tissue tumor usually forms in the central nervous system, but can also form in the trunk. Most often seen in young patients.

8.1. Symptoms

 

Ewing's sarcoma signs and symptoms include swelling and pain near the tumor.

These and other signs and symptoms can be caused by Ewing's sarcoma or other conditions. Check with your child's doctor if your child has any of the following:

  • Pain and / or swelling, usually in the arms, legs, chest, back, or pelvis.

  • A lump (which may feel soft and warm) in the arms, legs, chest, or pelvis.

  • Fever for no known reason.

  • A bone that breaks for no known reason.

8.2. Diagnostics  

Tests that examine bones and soft tissue are used to diagnose and stage Ewing's sarcoma.

Procedures that take pictures of bones, soft tissue, and surrounding areas help diagnose Ewing's sarcoma and show how far the cancer has spread. The process used to determine if cancer cells have spread in and around bones and soft tissues or to other parts of the body is called staging.

To plan treatment, it is important to know if the cancer has spread to other parts of the body. Tests and procedures to detect, diagnose, and stage Ewing's sarcoma are usually done at the same time.

The following tests and procedures can be used to diagnose or stage Ewing's sarcoma:

  • Physical examination and health history: Examining the body to check for general signs of health, including checking for signs of illness, such as tumors or anything else that seems unusual. There will also be a history of the patient's health habits, as well as past illnesses and treatments.

  • MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the body, such as an area where a tumor has formed. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • CT (computed tomography): A procedure in which a series of detailed pictures of areas inside the body, such as the area of a tumor or the chest, are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to make organs and tissues more visible. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • PET scan (positron emission tomography): a procedure to look for malignant tumor cells in the body. A small amount of radioactive glucose (sugar) is injected into the vein. The PET scanner rotates around the body and takes a picture of where glucose is being used in the body. Cancer cells appear brighter because they are more active and consume more glucose than normal cells. PET and computed tomography are often done at the same time. If there is any cancer, it increases the chance of finding it.

  • Bone scan: A procedure to check for rapidly dividing cells such as cancer cells in the bone. A very small amount of radioactive material is injected into a vein and passed through the bloodstream. The radioactive material builds up in the bones in cancer and is detected by a scanner.

  • Bone marrow aspiration and biopsy: Removal of bone marrow and a small piece of bone by inserting a cannula into the hip bone. Samples are removed from both pelvic bones. A pathologist examines the bone marrow and bones under a microscope to see if the cancer has spread.

  • X-ray: An X-ray is a type of energy beam that can pass through the body onto a film, creating an image of areas inside the body, such as the chest or the area where a tumor has formed.

  • Complete blood count (CBC): A procedure in which a blood sample is taken and checked for the following:

​​

  1. The number of erythrocytes, leukocytes and platelets.

  2. The amount of hemoglobin (oxygen-carrying protein) in red blood cells.

  3. The portion of a blood sample made up of red blood cells.

 

  • Blood chemistry tests: A procedure in which a blood sample is tested to measure the amount of certain substances, such as lactate dehydrogenase (LDH), released into the blood by the body's organs and tissues. An unusual (more or less than usual) amount of a substance may be a sign of illness.

A biopsy is done to diagnose Ewing's sarcoma.

Tissue samples are taken during a biopsy so that a pathologist can examine them under a microscope to check for signs of cancer. It is helpful if the biopsy is done at the same center where the treatment will be given.

  • Needle biopsy: For a needle biopsy, tissue is removed with a needle. This type of biopsy can be done if it is possible to take tissue samples large enough to be used for testing.

  • Postoperative biopsy: For a postoperative biopsy, a tissue sample is removed through an incision in the skin.

  • Excisional biopsy: Removal of an entire tumor or area of tissue that does not appear normal.

 

The specialists (pathologist, oncologist and surgeon) who will treat the patient will usually work together to select the best site to place the needle or biopsy incision. The choice of the biopsy site is very important. An incorrectly selected biopsy site can lead to more extensive surgery to remove the tumor or a larger area that is being treated with radiation therapy.

If there is a chance that the cancer has spread to nearby lymph nodes, one or more of the lymph nodes may be removed and checked for signs of cancer.

The following tests can be performed on the removed tissue:

  • Cytogenetic analysis:  a laboratory test in which the chromosomes of cells in a tissue sample are counted and checked for any changes, such as broken, missing, rearranged, or extra chromosomes. Changes in some chromosomes can be a sign of cancer. Cytogenetic analysis is used to diagnose cancer, plan treatment, or determine the effectiveness of a treatment.

  • Immunohistochemistry: A laboratory test that uses antibodies to check for specific antigens (markers) in a tissue sample from a patient. Antibodies are usually associated with an enzyme or fluorescent dye. After antibodies bind to a specific antigen in a tissue sample, the enzyme or dye is activated and the antigen can be seen under a microscope. This type of test is used to diagnose cancer and help distinguish one type of cancer from another.

  • Flow cytometry: A laboratory test that measures the number of cells in a sample, the percentage of living cells in the sample, and certain characteristics of cells such as size, shape, and the presence of tumor (or other) markers on the cell surface. Cells from a sample of blood, bone marrow, or other tissue from a patient are stained with a fluorescent dye, placed in a liquid, and then passed one at a time through a beam of light. The test results are based on how cells stained with a fluorescent dye respond to a beam of light.

Additional tests may be required.

Some of the tests that have been done to diagnose cancer or to determine the stage of cancer may be repeated. Some tests will be repeated to see how effective the treatment is. Decisions to continue, change, or stop treatment may be based on the results of these tests.

Some tests will continue from time to time after treatment ends. The results of these tests can show if your child's condition has changed or if the cancer has recurred (returned). These tests are sometimes called follow-up or follow-up examinations.

8.3. Factors affecting the prognosis of recovery

Factors affecting prognosis are different before and after treatment.

Before any treatment is prescribed, the prognosis depends on:

  • Whether the tumor has spread to lymph nodes or distant parts of the body.

  • Where the tumor started in the body.

  • Whether the tumor has formed in the bone or soft tissue.

  • How large is the tumor when a tumor is diagnosed.

  • Whether the tumor is caused by bone fractures.

  • The LDH level in the blood is higher than normal.

  • Whether there are specific gene changes in the tumor.

  • Has the tumor DNA been found in the blood.

  • Whether the patient is younger than 15 years old.

  • Patient gender.

  • Whether the patient was being treated for another cancer.

  • Whether the tumor has just been diagnosed or whether it has relapsed (come back).

 

After treatment, the prognosis is influenced by:

  • Whether the tumor has been completely removed by surgery.

  • If the tumor has responded to chemotherapy or radiation therapy.

 

If cancer recurs after initial treatment, the prognosis depends on:

  • Whether cancer has returned more than two years after initial treatment.

  • Whether the cancer originated in the place where it first formed or in other parts of the body.

8.4. Stages of Ewing's sarcoma

The results of diagnostic and staging tests are used to find out if cancer cells have spread.

The process used to determine if cancer has spread to other parts of the body is called staging. There is no standard staging system for Ewing's sarcoma. The results of tests and procedures performed to diagnose and stage Ewing's sarcoma are used to describe tumors as localized or metastatic.

Ewing's sarcoma is described as localized, metastatic, or  recurrent  ...

Localized Ewing's sarcoma

Cancer is found in the bones or soft tissues where it started and can spread to nearby tissues, including nearby lymph nodes.

Ewing's metastatic sarcoma

The cancer has spread from bone or soft tissue, where it started to other parts of the body. With Ewing's bone tumor, cancer most often spreads to the lung, other bones, and bone marrow.

Recurrent Ewing's sarcoma

The cancer has recurred (returned) after treatment. Cancer can return to the bones or soft tissue where it originated or to another part of the body.

8.5. Overview of treatment options

There are various treatments for children with Ewing's sarcoma.

Various treatments are available for children with Ewing's sarcoma. Some treatments are standard (currently used) and some are in clinical trials. A treatment clinical trial is a scientific study designed to help improve existing treatments or provide information about new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment can become the standard treatment.

Since cancer is rare in children and adolescents, clinical trials should be considered. Some clinical trials are only open to patients who have not yet started treatment.

Four types of standard treatments are used:

 

1. Chemotherapy

Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells by either killing the cells or stopping them from dividing. When chemotherapy is taken by mouth or injected into a vein or muscle, the drugs enter the bloodstream and can reach cancer cells throughout the body (systemic chemotherapy). Combination chemotherapy is treatment that uses more than one anticancer drug.

Systemic combination chemotherapy is part of the treatment for all patients with Ewing's tumors. This is often the first treatment that lasts 6 to 12 months. Chemotherapy is often used to shrink the tumor before surgery or radiation therapy and to kill any tumor cells that may have spread to other parts of the body.

 

2. Radiation therapy

Radiation therapy is a cancer treatment that uses high-energy X-rays or other types of radiation to kill cancer cells or prevent them from growing. External beam radiation therapy uses a device outside the body to direct radiation to an area of the body affected by cancer.

Radiation therapy is used when the tumor cannot be removed with surgery, or when surgery to remove the tumor affects important body functions or how the baby will look. It can be used to shrink the tumor and reduce the amount of tissue that needs to be removed during surgery. It can also be used to treat any tumors left over from surgery and tumors that have spread to other parts of the body.

3. Operation

Surgery is usually done to remove cancer left over from chemotherapy or radiation therapy. If possible, the entire tumor is surgically removed. The removed tissue and bone can be replaced with a graft that uses tissue and bone taken from another part of the patient's or donor's body. Sometimes an implant is used, such as an artificial bone.

After a doctor has removed any tumors that can be seen during surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any remaining cancer cells. Treatment given after surgery to reduce the risk of cancer recurrence is called adjuvant therapy.

 

4. High-dose stem cell rescue chemotherapy

High doses of chemotherapy are prescribed to kill cancer cells. Healthy cells, including hematopoietic cells, are also destroyed in cancer treatments. Stem cell transplantation is a treatment to replace hematopoietic cells. Stem cells (immature blood cells) are removed from the blood or bone marrow of a patient or donor, frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and returned to the patient as an infusion. These re-injected stem cells germinate (and regenerate) the body's blood cells. Stem cell rescue chemotherapy is used to treat localized and recurrent diseases. Ewing's sarcoma.

Also used to treat Ewing's sarcoma:

1. Targeted therapy

Targeted therapy is a type of treatment that uses drugs or other substances to identify and target certain cancer cells. Targeted therapy usually does less damage to normal cells than chemotherapy or radiation therapy. The types of targeted therapies used to treat Ewing's sarcoma include the following:

  • Monoclonal Antibody Therapy: Monoclonal antibodies of the immune system are proteins made in the laboratory for the treatment of many diseases, including cancer. As a cancer treatment, these antibodies can attach to a specific target on cancer cells or other cells that can promote cancer cell growth. The antibodies are then able to kill cancer cells, block their growth, or prevent them from spreading. Monoclonal antibodies are given by infusion. They can be used alone or to carry drugs, toxins, or radioactive materials directly to cancer cells. Ganitumab is a monoclonal antibody that is being studied for the treatment of metastatic Ewing's sarcoma.

  • Kinase inhibitor therapy: This treatment blocks a protein that is required for cancer cells to divide. Cabosantinib is a kinase inhibitor being studied for the treatment of recurrent Ewing's sarcoma.

  • NEDD8 Activating Enzyme Inhibitor (NAE) Therapy: NAE inhibitors are drugs that attach to the NAE and stop cancer cells from dividing. Pevonedistat is an NAE inhibitor being studied for the treatment of recurrent Ewing's sarcoma.

 

2. Immunotherapy

Immunotherapy is a treatment that uses a patient's immune system to fight cancer. Substances produced by the body or in the laboratory are used to enhance, direct, or restore the body's natural defenses against cancer. This cancer treatment is a type of biological therapy.

  • Immune Checkpoint Inhibitor Therapy: This treatment blocks certain proteins produced by certain cells of the immune system, such as T cells and some cancer cells. These proteins help control immune responses and prevent T cells from killing cancer cells. When these proteins are blocked, the “brakes” of the immune system are released and T cells are better able to kill cancer cells. Nivolumab and ipilimumab are types of immune checkpoint inhibitors that are being studied for the treatment of recurrent Ewing's sarcoma.

  • CAR T Cell Therapy: This treatment alters the patient's T cells (a type of cells in the immune system) so they will attack certain proteins on the surface of the cancer cells. T cells are taken from the patient, and special receptors are added to their surface in the laboratory. The altered cells are called chimeric antigen receptor (CAR) T cells. CAR T cells are grown in the laboratory and infused into the patient. CAR T cells multiply in the patient's blood and attack cancer cells. CAR T-cell therapy is being investigated for the treatment of recurrent Ewing's sarcoma.

 

8.6. Treatment for Ewing's sarcoma can cause side effects.

 

Side effects from cancer treatments that start after treatment and last for months or years are called late effects. Late effects of cancer treatments may include the following:

  • Physical problems.

  • Changes in mood, feelings, thinking, learning, or memory.

  • Second cancer (newer cancers). Patients treated for Ewing's sarcoma are at increased risk of acute myeloid leukemia and myelodysplastic syndrome. There is also an increased risk of sarcoma in the area that has undergone radiation therapy.

 

Some late effects can be treated or controlled. It is important to talk with your child's healthcare providers about the impact cancer treatment can have on your child.

8.7. Treatment options

Treatment of localized Ewing's sarcoma

Standard treatments  first  identified  localized  Ewing's sarcomas  include:

  • Chemotherapy.

  • Surgery and / or radiation therapy.

  • High-dose stem cell rescue chemotherapy.

 

Treatment of metastatic Ewing's sarcoma

 

Standard treatments for newly diagnosed metastatic Ewing's sarcoma include:

  • Chemotherapy.

  • Surgery.

  • Radiation therapy .

 

Treatment of recurrent Ewing's sarcoma

 

There is no standard treatment for recurrent Ewing's sarcoma, but treatment options may include the following:

  • Combined chemotherapy.

  • Radiation therapy for bone tumors as palliative care  to relieve symptoms and improve quality of life.

  • Radiation therapy, which may be followed by surgery to remove tumors that have spread to the lungs.

  • High-dose chemotherapy  with  stem cell rescue  ...

 

Treatment options for recurrent Ewing's sarcoma that are being explored include the following:

  • Checking a sample of a patient's tumor for certain gene changes. The type of targeted therapy that will be given to a patient depends on the type of gene change.

  • Targeted therapy with a tyrosine kinase inhibitor (cabozantinib).

  • Immunotherapy with an immune checkpoint inhibitor (nivolumab or Ipilimumab).

  • CAR T-cell therapy.

  • Targeted therapy with a NEDD8-activating enzyme inhibitor (pevoneedistat) and chemotherapy.

  • Clinical study of a new type of targeted therapy.

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9. TREATMENT OF OSTEOSARCOMA

Якорь 9

Osteosarcoma and undifferentiated pleomorphic sarcoma (UPS) of bone are diseases in which malignant (cancer) cells form in the bone.

Osteosarcoma usually starts with osteoblasts, which are a type of bone cell that becomes new bone. Osteosarcoma is most common in adolescents. It usually forms at the ends of the long bones of the body, including the bones of the arms and legs. In children and adolescents, it often forms in the long bones near the knee. In rare cases, osteosarcoma can be found in the soft tissues or organs of the chest or abdomen.

Osteosarcoma is the most common type of bone cancer. UPS (formerly called malignant fibrous histiocytoma [MFH]) is a rare type of bone cancer that usually begins in soft tissue but can form in bone. In bone, UPS cells look like osteosarcoma under the microscope. UPS is treated like osteosarcoma.

9.1. Development risks

Undergoing treatment with chemotherapy or radiation therapy may increase the risk of osteosarcoma.

Anything that increases the risk of a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; the absence of risk factors does not mean that you will not get cancer. Talk to your child's doctor if you think your child may be at risk. Risk factors for osteosarcoma include the following:

  • Received chemotherapy or radiation therapy.

  • Having a certain change in the RB1 gene.

  • Having certain conditions, for example the following:

    • Bloom's syndrome.

    • Diamond-Blackfan anemia.

    • Li-Fraumeni syndrome.

    • Paget's disease.

    • Hereditary retinoblastoma.

    • Rothmund-Thomson syndrome.

    • Werner's syndrome.

9.2. Symptoms

Signs and symptoms of osteosarcoma and UPS include swelling of the bone or bone of the body and joint pain.

These and other signs and symptoms can be caused by osteosarcoma, UPS, or other conditions. Check with your doctor if your child has any of the following:

  • Swelling over a bone or bony part of the body.

  • Bone or joint pain.

  • A bone that breaks for no known reason.

9.3. Diagnostics

Imaging tests are used to detect (detect) osteosarcoma and UPS.

Imaging tests are done before the biopsy. The following tests and procedures can be used:

  • Physical examination and health history: Examining the body to check for general signs of health, including checking for signs of illness, such as tumors or anything else that seems unusual. There will also be a history of the patient's health habits, as well as past illnesses and treatments.

  • X-rays: X-rays of organs and bones inside the body. X-rays are a type of energy beam that can pass through the body onto film, creating an image of areas within the body.

  • CT (computed tomography): a procedure in which a series of detailed pictures of areas inside the body are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to make organs and tissues more visible. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • MRI (Magnetic Resonance Imaging): A procedure that uses a magnet, radio waves, and a computer to create a series of detailed pictures of areas inside the body. This procedure is also called nuclear magnetic resonance imaging (MRI).

 

A biopsy is done to diagnose osteosarcoma.

Cells and tissues are removed during a biopsy so that a pathologist can examine them under a microscope to check for signs of cancer. It is important that the biopsy is done by a surgeon who is an expert in the treatment of bone cancer. It is best if this surgeon removes the tumor. The biopsy and surgery to remove the tumor are planned together. The way the biopsy is taken will affect which surgery can be done later.

The type of biopsy depends on the size of the tumor and its location in the body. Two types of biopsies can be used:

  • Primary biopsy: Removal of tissue with a wide needle.

  • Postoperative biopsy: Removal of a portion of a tumor or tissue sample that does not appear normal.

 

The following test can be performed on the removed tissue:

  • Electron microscopy:  a laboratory test in which cells in a tissue sample are viewed under a regular and powerful microscope to detect specific changes in the cells.

9.4. Factors affecting the prognosis of recovery

Several factors can affect prognosis (chance of recovery) and treatment options.

On  forecast  certain factors before and after treatment may be affected.

The prognosis of untreated osteosarcoma and IBD may depend on the following:

  • Where is the tumor in the body and whether tumors have formed in more than one bone.

  • The patient has two or more tumors in the same bone.

  • The size of the tumor.

  • Whether the cancer has spread to other parts of the body and where it has spread.

  • The type of tumor (based on how cancer cells look under a microscope).

  • Gender, age and weight of the patient at the time of diagnosis.

  • Whether the patient was being treated for another cancer.

  • Whether the patient has certain genetic diseases.

 

After treatment for osteosarcoma or IBD, the prognosis also depends on the following:

  • How much of the cancer has been killed by chemotherapy.

  • Whether the tumor has been completely removed by surgery.

  • If the cancer has recurred (come back) within 2 years after diagnosis.

 

Treatment options for osteosarcoma and IBD depend on the following:

  • Where is the tumor in the body and whether it has spread.

  • The size of the tumor.

  • Cancer assessment.

  • Are the bones still growing?

  • Patient's age and general health.

  • The desire of the patient and his family for the patient to be able to participate in sports activities or to look a certain way.

  • Whether the cancer was diagnosed for the first time or whether there was a relapse after treatment.

9.5. Stages of osteosarcoma and undifferentiated pleomorphic sarcoma (UPS)

After osteosarcoma or undifferentiated pleomorphic sarcoma (UPS) is diagnosed, tests are done to find out if cancer cells have spread to other parts of the body.

The process used to determine if cancer has spread to other parts of the body is called staging. Most patients with osteosarcoma and IBD are grouped according to whether the cancer is localized or metastatic.

  • Localized osteosarcoma, or UPS, has not spread from the bone where the cancer started. There may be one or more areas of cancer in the bone that can be removed during  operations  ...

  • Metastatic osteosarcoma, or IBD, has spread from the bone in which the cancer started to other parts of the body. Cancer most often spreads to  lungs  ... It can also spread to other bones.

 

The following tests and procedures can be used to find out if the cancer has spread:

  • X-rays: X-rays of organs such as the chest and bones inside the body. X-rays are a type of energy beam that can pass through the body onto film, creating an image of areas within the body. An x-ray of the chest and area of the tumor will be taken.

  • CT (computed tomography): A procedure in which a series of detailed pictures of areas inside the body, such as the chest, are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye can be injected into a vein or swallowed to make organs or tissues show more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography. X-rays of the chest and the area where the tumor is forming will be taken.

  • PET-CT scan: A procedure that combines positron emission tomography (PET) and computed tomography (CT) images. PET and CT scans are performed simultaneously on the same machine. Images from both scans are combined to create a more d