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TREATMENT OF KIDNEY CANCER IN TURKEY

1.  Risk factors for kidney cancer

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 renal cell carcinoma include the following:

  • Smoking.

  • Abusing certain pain medications, including over-the-counter pain relievers, for a long time.

  • Excess weight .

  • High blood pressure.

  • Has a family history of renal cell carcinoma.

  • The presence of certain genetic diseases, such as von Hippel-Lindau disease or hereditary papillary renal cell carcinoma

2. Renal cell carcinoma

Kidney cancer can develop in adults and children. The main types of kidney cancer are renal cell carcinoma, transitional cell carcinoma, and Wilms' tumor. Certain inherited conditions increase the risk of kidney cancer. Explore the links on this page to learn more about kidney cancer treatments, statistics, research, and clinical trials.

Renal cell carcinoma is a disease in which malignant (cancerous) cells form in the tubules of the kidneys.

Renal cell carcinoma (also called kidney cancer or renal cell adenocarcinoma) is a disease in which malignant (cancerous) cells are found in the lining of the tubules (very small tubes) in the kidneys. Above the waist are 2 kidneys, one on each side of the spine. Tiny tubules in the kidneys filter and purify the blood. They excrete waste products and produce urine. Urine travels from each kidney through a long tube called the ureter into the bladder. The bladder retains urine until it passes through the urethra and leaves the body.

Cancer that begins in the ureters or the renal pelvis (the part of the kidney that collects urine and diverts it to the ureters) is different from renal cell carcinoma.

 

2.1. Signs of renal cell carcinoma

These and others  signs  and  symptoms  can be caused by renal cell carcinoma or other conditions. Early  stages  there may be no signs or symptoms. Signs and symptoms may appear as you grow  tumors  ...

 

Check with your doctor if you have any of the following:

  • Blood in the urine.

  • Bump in  belly  ...

  • Side pain persists.

  • A loss  appetite  ...

  • Losing weight for an unknown reason.

  • Anemia  ...

 

2.2. Diagnosis of renal cell carcinoma

 

Tests that examine the abdomen and kidneys are used to diagnose renal cell carcinoma.

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 for signs of illness such as bumps 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.

  • Ultrasound examination : A procedure in which high energy sound waves (ultrasound) are reflected off internal tissues or organs and create an echo. The echo forms a picture of body tissue called a sonogram.

  • Studies of the chemical composition of blood  : 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.

  • General urine analysis  : a test to check the color of urine and its contents such as sugar, protein, red blood cells and white blood cells.

  • CT (computed tomography) : A procedure in which a series of detailed pictures of areas inside the body, such as the abdomen and pelvis, 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).

  • Biopsy  : Removing cells or tissues so that a pathologist can examine them under a microscope to check for signs of cancer. To get a biopsy for renal cell carcinoma, a thin needle is inserted into the tumor and a tissue sample is taken.

 

After renal cell cancer is diagnosed, tests are done to find out if the cancer cells have spread to the kidneys or other parts of the body.

The process used to determine if cancer has spread to the kidneys or to other parts of the body is called staging. 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:

  • CT (computed tomography)  : A procedure in which a series of detailed pictures of areas inside the body, such as the chest or brain, 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, such as the brain. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • 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.

  • 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.

2.3. How does cancer spread in renal cell carcinoma patients?

Cancer spreads through the body in three ways.

Cancer can spread through tissues, the lymphatic system, and the blood:

  • Textile. Cancer spreads from where it originated to surrounding areas.

  • The lymphatic system. Cancer spreads from where it originated to the lymphatic system. Cancer spreads through the lymphatic vessels to other parts of the body.

  • Blood. Cancer spreads from where it originated into the bloodstream. Cancer spreads through the blood vessels to other parts of the body.

 

Cancer can spread from where it originated to other parts of the body.

 

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they originated (primary tumor) and travel through the lymphatic system or blood.

  • The lymphatic system.  Cancer enters the lymphatic system, travels through the lymphatic vessels and forms  tumor  (  metastatic  swelling) in another part of the body.

  • Blood.  Cancer enters the bloodstream, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

  • A metastatic tumor is the same type of cancer as the primary tumor. For example, if  renal cell carcinoma  spreads to the bone, the cancer cells in the bone are actually cancerous kidney cells. The disease is metastatic renal cell carcinoma, not bone cancer.

2.4. Treatments for patients with renal cell carcinoma

There are various treatments for renal cell carcinoma patients.

Various treatments are available for renal cell carcinoma 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.

Five types of standard treatments are used:

1.Operation

Surgery to remove part or all of the kidney is often used to treat renal cell carcinoma. The following types of surgical procedures can be used:

  • Partial nephrectomy  : A surgical procedure to remove a tumor of the kidney and some tissue around it. Partial nephrectomy can be performed to prevent loss of kidney function when the other kidney is damaged or has already been removed.

  • Simple nephrectomy  : a surgical procedure to remove only the kidney.

  • Radical nephrectomy  : A surgical procedure to remove the kidney, adrenal gland, surrounding tissue and usually nearby lymph nodes.

A person can live with part 1 of a working kidney, but if both kidneys are removed or not working, they will need dialysis (a procedure to purify blood with an apparatus outside the body) or a kidney transplant (replacement of a healthy kidney). donor kidney). A kidney transplant can be done if only the kidney is affected and a donor kidney can be found. If the patient needs to wait for a kidney donation, another treatment is prescribed if necessary.

When surgery to remove the cancer is not possible, a treatment called arterial embolization can be used to shrink the tumor. A small incision is made and a catheter (thin tube) is inserted into the main blood vessel that flows to the kidney. Small pieces of a special gelatinous sponge are inserted through a catheter into a blood vessel. The sponges block blood flow to the kidneys and prevent cancer cells from receiving oxygen and other substances necessary for growth.

After a 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 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. External beam radiation therapy uses a device outside the body to direct radiation to an area of the body affected by cancer. External beam radiation therapy is used to treat renal cell carcinoma and can also be used as palliative therapy to relieve symptoms and improve quality of life.

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. 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 type of cancer treatment is also called biotherapy or biological therapy.

The following types of immunotherapy are used in the treatment of renal cell carcinoma:

  • Immune checkpoint inhibitor therapy: Certain 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. Inhibitors of immune checkpoints block these proteins and the ability of T cells to kill cancer cells is increased. They are used to treat some patients with advanced renal cell carcinoma that cannot be surgically removed.

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 one of the CTLA-4 inhibitors.

  • Inhibitor  PD-1  : PD-1 is a protein on the surface of T cells that helps control the body's immune responses. When PD-1 attaches to another protein, called PDL-1, on a cancer cell, it stops the T cell from killing it. PD-1 inhibitors attach to PDL-1 and allow T cells to kill cancer cells.  Nivolumab  ,  pembrolizumab  and  avelumab  are types of PD-1 inhibitors.

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  • Interferon: Interferon affects cancer cell division and may slow tumor growth.

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  • Interleukin-2 (IL-2): IL-2 enhances the growth and activity of many immune cells, especially lymphocytes (a type of white blood cell). Lymphocytes can attack and kill cancer cells.

5. Targeted therapy

Targeted therapy uses drugs or other substances to identify and attack certain cancer cells without harming normal cells. Targeted anti-angiogenic therapy is used to treat advanced renal cell carcinoma. Anti-angiogenic agents interfere with the formation of blood vessels in the tumor, causing the tumor to starve and stop growing or shrinking.

Monoclonal antibodies and kinase inhibitors are two types of anti-angiogenic agents used to treat renal cell carcinoma.

  • Monoclonal antibody therapy uses antibodies obtained in the laboratory from a single cell type of the immune system. These antibodies can identify substances on cancer cells or normal substances that can promote the growth of cancer cells. Antibodies attach to substances and 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. Monoclonal antibodies, used to treat renal cell carcinoma, attach to and block substances that cause new blood vessels to form in tumors. Bevacizumab is a monoclonal antibody.

  • Kinase inhibitors stop cell division and can prevent the growth of new blood vessels necessary for tumor growthVascular endothelial growth factor (VEGF) inhibitors and mTOR inhibitors are kinase inhibitors used in the treatment of renal cell carcinoma.

  • VEGF inhibitors: Cancer cells produce a substance called VEGF, which causes new blood vessels to form (angiogenesis) and helps the cancer grow. VEGF inhibitors block VEGF and prevent new blood vessel formation. This can kill cancer cells because they need new blood vessels to grow. Sunitinib, Pazopanib, cabozantinib, axitinib, sorafenib, and lenvatinib are VEGF inhibitors.

  • MTOR inhibitors: mTOR is a protein that helps cells divide and survive. MTOR inhibitors block mTOR and can inhibit the growth of cancer cells and prevent the growth of new blood vessels necessary for tumor growth. Everolimus and temsirolimus are mTOR inhibitors.

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3. Transitional cell carcinoma of the renal pelvis and ureter

 

Transitional cell carcinoma of the renal pelvis and ureter is a disease in which malignant (cancerous) cells form in the renal pelvis and ureter.

The renal pelvis is the upper part of the ureter. The ureter is a long tube that connects the kidney to the bladder. Above the waist are two kidneys, one on each side of the spine. The kidneys of an adult are about 5 inches long and 3 inches wide and are shaped like a bean. Tiny tubules in the kidneys filter and purify the blood. They excrete waste products and produce urine. Urine collects in the middle of each kidney in the renal pelvis. Urine passes from the renal pelvis through the ureter into the bladder. The bladder retains urine until it passes through the urethra and leaves the body.

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Anatomy of the male urinary system (left panel) and the female urinary system (right panel), showing the kidneys, ureters, bladder and urethra. Urine is produced in the renal tubules and collected in the renal pelvis of each kidney. Urine flows from the kidneys through the ureters into the bladder. Urine is stored in the bladder until it leaves the body through the urethra.

The renal pelvis and ureters are lined with transitional cells. These cells can change shape and stretch without collapsing. Transitional cell carcinoma begins in these cells.

Transitional cell carcinoma can form in the renal pelvis, ureter, or both.

Renal cell carcinoma is the more common type of kidney cancer.

3.1. Signs and symptoms of transitional cell carcinoma of the renal pelvis and ureter  

These and other signs and symptoms can be caused by transitional cell carcinoma of the renal pelvis and ureter or other conditions. In the early stages, there may be no signs or symptoms. Signs and symptoms may appear as the tumor grows. Check with your doctor if you have any of the following:

  • Blood in the urine.

  • Back pain that does not go away.

  • Great fatigue.

  • Weight loss for no known reason.

  • Painful or frequent urination.

3.1.
Уретероскопия

Ureteroscopy. A ureteroscope (a thin, tube-like instrument with a light and viewing lens) is inserted through the urethra into the ureter. The doctor looks at an image of the inside of the ureter on a computer monitor.

  • Urine cytology  : A laboratory test in which a urine sample is checked under a microscope for abnormal cells. Cancer of the kidney, bladder, or ureter can shed cancer cells into the urine.

  • 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 help organs or tissues show more clearly. This procedure is also called computed tomography, computed tomography, or computed axial tomography.

  • Ultrasound  : A procedure in which high energy sound waves (ultrasound) are reflected from internal tissues or organs and create an echo. The echo forms a picture of body tissue called a sonogram. An abdominal ultrasound can help diagnose cancers of the renal pelvis and ureter.

  • 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 the pelvis. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • Biopsy  : Removing cells or tissues so that a pathologist can examine them under a microscope to check for signs of cancer. This can be done during ureteroscopy or surgery.

3.2. Factors influence prognosis (chance of recovery) and treatment options.

 

Forecast  depends on the stage and  degree  tumors.

 

Treatment options depend on the following:

  • Stage and grade of the tumor.

  • Where is the tumor.

  • Whether the patient's other kidney is healthy.

  • Does the cancer have a relapse.

 

Most transitional cell cancers of the renal pelvis and ureter can be cured if detected early.

3.3. Tests for detecting metastases

The process used to determine if cancer has spread to the renal pelvis and ureter or to other parts of the body is called staging. 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. The doctor, based on the results of diagnostic tests, will help determine the stage of the disease.

The following tests and procedures can also be used in the preparation process:

  • Chest x-ray  : X-ray of organs and bones inside 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.

  • PET scan (positron emission tomography) : a procedure to search 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.

  • 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.

3.4. How cancer spreads throughout the body

Cancer can spread through tissues, the lymphatic system, and the blood:

  • Textile. Cancer spreads from where it originated to surrounding areas.

  • The lymphatic system . Cancer spreads from where it originated to the lymphatic system. Cancer spreads through the lymphatic vessels to other parts of the body.

  • Blood. Cancer spreads from where it originated into the bloodstream. Cancer spreads through the blood vessels to other parts of the body.

Cancer can spread from where it originated to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they originated (primary tumor) and travel through the lymphatic system or blood.

  • The lymphatic system. Cancer enters the lymphatic system, travels through the lymphatic vessels, and forms a tumor (metastatic tumor) in another part of the body.

  • Blood. Cancer enters the bloodstream, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

A metastatic tumor is the same type of cancer as the primary tumor. For example, if transitional cell carcinoma of the ureter has spread to the lungs, the lung cancer cells are actually ureteral cancer cells. The disease is metastatic cancer of the ureter, not lung cancer.

3.5. Types of transitional cell carcinoma of the renal pelvis and ureter

 

Transitional cell carcinoma of the renal pelvis and ureter is also described as localized, regional, metastatic, or recurrent:

1. Localized

Cancer is found only in the kidneys.

2. Regional

The cancer has spread to tissues around the kidney and nearby lymph nodes and blood vessels in the pelvic region.

3. Metastatic

The cancer has spread to other parts of the body.

 

4. Recurrent

The cancer was recurred (come back) after it was treated. Cancer can return to the renal pelvis, ureter, or other parts of the body such as the lungs, liver, or bone.

3.6. Treatment methods for patients with transitional cell carcinoma of the renal pelvis and ureter.

 

Various treatments are available for patients with transitional cell carcinoma of the renal pelvis and ureter. 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.

One type of standard treatment is used:

 

1. Operation

One of the following surgical procedures can be used to treat transitional cell carcinoma of the renal pelvis and ureter:

  • Nephroureterectomy  : Surgery to remove the entire kidney, ureter, and bladder cuff (the tissue that connects the ureter to the bladder).

  • Segmental ureteral resection: A surgical procedure to remove a portion of the ureter with cancer and a portion of the healthy tissue around it. Then the ends of the ureter are reattached. This procedure is used when the cancer is superficial and only near the bladder in the lower third of the ureter.

2. Fulguration  is a surgical procedure in which tissue is destroyed by an electric current. An instrument with a small wire loop at the end is used to remove a tumor or to burn off a tumor with electricity.

 

3. Segmental resection of the renal pelvis

It is a surgical procedure to remove localized cancer from the renal pelvis without removing the entire kidney. Segmental resection can be done to preserve renal function when the other kidney is damaged or has already been removed.

 

4. Laser surgery

A laser beam (a narrow beam of intense light) is used like a knife to remove cancer. The laser beam can also be used to kill cancer cells. This procedure can also be called laser fulguration.

5. Regional chemotherapy and regional biological therapy

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. Biological therapy is a treatment that uses the patient's immune system to fight cancer; substances produced by the body or made in a laboratory are used to enhance, direct or restore the body's natural defenses against cancer. Local treatment means that anticancer drugs or biological agents are injected directly into an organ or body cavity, such as the abdominal cavity, so the drugs will affect cancer cells in that area. Clinical trials are looking at chemotherapy or biologic therapy using drugs that are injected directly into the renal pelvis or ureter.

4. Wilms' tumors and other kidney tumors in children

 

Kidney tumors in children are diseases in which malignant (cancerous) cells form in the tissues of the kidneys.

Above the waist are two kidneys, one on each side of the spine. Tiny tubules in the kidneys filter and purify the blood. They excrete waste products and produce urine. Urine travels from each kidney through a long tube called the ureter into the bladder. The bladder retains urine until it passes through the urethra and leaves the body.

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3.4.
3.5.
3.6.
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Anatomy of the female urinary system: kidneys, adrenal glands, ureters, bladder and urethra. Urine is produced in the renal tubules and collected in the renal pelvis of each kidney. Urine flows from the kidneys through the ureters into the bladder. Urine is stored in the bladder until it leaves the body through the urethra.

4.1. Types of Wilms tumor in children

 

There are many types of kidney tumors in children.

 

1. Wilms' tumor

 

With Wilms' tumor, one or more tumors may be found in one or both kidneys. Wilms' tumor can spread to the lungs, liver, bones, brain, or nearby lymph nodes. In children and adolescents under the age of 15, the majority of kidney cancers are Wilms' tumors.

 

2. Renal cell carcinoma (RCC)

 

Renal cell carcinoma is rare in children and adolescents under 15 years of age. It is much more common in adolescents from 15 to 19 years old. Children and adolescents are more likely to be diagnosed with a large renal cell tumor or cancer that has spread. Renal cell carcinoma can spread to the lungs, liver, bones, or lymph nodes. Renal cell carcinoma can also be called renal cell carcinoma.

 

3. Rhabdoid tumor of the kidney

 

Rhabdoid kidney tumor is a type of kidney cancer that is more common in infants and young children. At the time of diagnosis, the disease is often progressing. A renal rhabdoid tumor grows rapidly and spreads, often to the lungs or brain.

Children with a specific SMARCB1 gene change can also grow tumors in the kidney, brain, or soft tissue. These children are regularly checked for a rhabdoid tumor in the kidney or brain:

 

4. Clear cell sarcoma of the kidney

 

Clear cell renal sarcoma is an uncommon kidney cancer that can spread to the bones, lungs, brain, liver, or soft tissue. Most often occurs before the age of 3 years. It can recur (come back) up to 14 years after treatment, often in the brain or lungs.

5. Congenital mesoblastic nephroma

 

Congenital mesoblastic nephroma is a tumor of the kidney that is often diagnosed during the first year of life or before birth. It is the most common kidney tumor found in children under 6 months of age and is more common in men than women. It can usually be cured.

 

6. Ewing's sarcoma of the kidneys

 

Ewing's sarcoma (formerly called neuroepithelial tumor) of the kidney is rare and usually occurs in young adults. This cancer grows rapidly and spreads to other parts of the body.

 

7. Primary myoepithelial carcinoma of the kidney

 

Primary myoepithelial carcinoma of the kidney is a rare type of cancer that usually affects soft tissue but sometimes forms in internal organs (such as the kidneys). This cancer grows and spreads rapidly.

 

8. Cystic partially differentiated nephroblastoma

 

Cystic partially differentiated nephroblastoma is a very rare type of Wilms tumor, consisting of cysts.

9. Multilocular cystic nephroma

Multilocular cystic nephroma is a benign tumor of cysts that is most common in infants, young children, and adult women. These tumors can occur in one or both kidneys.

 

Children with this type of tumor may also have pleuropulmonary blastoma, so imaging tests are done to check the lungs for cysts or solid tumors. Since multilocular cystic nephroma can be hereditary, genetic counseling and genetic testing may be considered. For more information, see the PDQ Summary of Pediatric Pleuropulmonary Blastoma Management.

10. Primary renal synovial sarcoma

 

Primary synovial renal sarcoma is a cystic tumor of the kidney that is most common in young adults. These tumors grow and spread rapidly.

11. Anaplastic renal sarcoma

 

Anaplastic renal sarcoma is a rare tumor that most often occurs in children or adolescents under 15 years of age. Anaplastic renal sarcoma often spreads to the lungs, liver, or bones. Imaging tests may be performed that check the lungs for cysts or solid tumors. Since anaplastic sarcoma can be hereditary, genetic counseling and genetic testing may be considered.

Nephroblastomatosis is not cancer, but it can develop into Wilms tumor.

Sometimes, after the kidneys form in the fetus, abnormal groups of kidney cells remain in one or both of the kidneys. In nephroblastomatosis (diffuse hyperplastic perilobar nephroblastomatosis), these abnormal groups of cells can grow in many places within the kidney or form a thick layer around the kidney. When these groups of abnormal cells are found in a kidney after removal for a Wilms tumor, the child is at increased risk of a Wilms tumor in the other kidney. Regular follow-up is important at least every 3 months for at least 7 years after the child is diagnosed or treated.

4.2. Wilms tumor risk factors

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.

Wilms' tumor may be part of a genetic syndrome that affects growth or development. A genetic syndrome is a collection of signs and symptoms or conditions that occur together and are caused by certain changes in genes. Certain conditions or environmental influences can also increase a child's risk of developing Wilms tumor. The following has been associated with Wilms' tumor:

  • WAGR syndrome (Wilms tumor, aniridia, anomaly of the genitourinary system and mental retardation).

  • Denis-Drash syndrome (anomaly of the genitourinary system).

  • Fraser's syndrome (an anomaly of the genitourinary system).

  • Beckwith-Wiedemann syndrome (abnormally large growth of one or more body parts, large tongue, umbilical hernia at birth and abnormalities of the genitourinary system).

  • Family history of Wilms tumor.

  • Aniridia (part or all of the iris, the colored part of the eye is missing).

  • Isolated hemihyperplasia (abnormally large growth of one or more parts of the body).

  • Urinary tract problems such as cryptorchidism or hypospadias.

  • The baby's mother was exposed to pesticides during pregnancy.

Having certain conditions can increase your risk of renal cell cancer.

 

Renal cell carcinoma can be associated with the following conditions:

  • Von Hippel-Lindau disease (an inherited disorder that causes abnormal growth of blood vessels). Children with von Hippel-Lindau disease should be screened annually for kidney cancer by abdominal ultrasound or MRI (magnetic resonance imaging), starting at 8-11 years of age.

  • Tuberous sclerosis (an inherited disorder characterized by benign fatty cysts in the kidneys).

  • Familial renal cell carcinoma (an inherited disorder that occurs when certain changes in the genes that cause kidney cancer are passed from parent to child).

  • Medullary kidney cancer (rare kidney cancer that grows and spreads rapidly).

  • Hereditary leiomyomatosis (an inherited disorder that increases the risk of cancers of the kidney, skin, and uterus).

 

Chemotherapy or radiation therapy for childhood cancers such as neuroblastoma, soft tissue sarcomas, leukemia, or Wilms' tumor may also increase the risk of kidney cancer.

4.3. Williams Tumor Screening Tests

Screening tests are performed on children at increased risk of Wilms' tumor. These tests can help detect cancer early and reduce the chances of dying from cancer.

In general, children with an increased risk of Wilms tumor should be screened for Wilms tumor every 3 months until they are 8 years old. Abdominal ultrasound is usually used for screening. Small Wilms tumors can be detected and removed before symptoms appear.

Children with Beckwith-Wiedemann syndrome or hemihyperplasia are also screened for liver and adrenal tumors that are associated with these genetic syndromes. A test to check the level of alpha-fetoprotein (AFP) in the blood and an ultrasound of the abdominal cavity are performed up to 4 years of age. An ultrasound of the kidneys is done at the age of 4 to 7 years. A medical examination by a specialist (geneticist or pediatric oncologist) is done twice a year. In children with certain gene changes, a different abdominal ultrasound schedule may be used.

Children with aniridia and a certain gene change are tested for Wilms tumor every 3 months until they are 8 years old. An abdominal ultrasound is used for screening.

Some children develop Wilms' tumor in both kidneys. They often appear when a Wilms tumor is first diagnosed, but a Wilms tumor can also occur in a second kidney after successfully treating a child for a Wilms tumor in one kidney. Children with an increased risk of a second Wilms tumor in another kidney should be screened for Wilms tumor every 3 months for up to 8 years. Abdominal ultrasound can be used for screening.

4.4. Signs of Williams' tumor

Sometimes childhood kidney tumors do not cause signs and symptoms, and the parent finds the mass in the abdomen by accident or the mass is in the process of a good child health check. These and other signs and symptoms can be caused by kidney tumors or other conditions. Check with your child's doctor if your child has any of the following:

  • A lump, swelling, or pain in the abdomen.

  • Blood in the urine.

  • High blood pressure (headache, feeling very tired, chest pain, or problems with vision or breathing).

  • Hypercalcemia (loss of appetite, nausea and vomiting, weakness or feeling very tired).

  • Fever for no known reason.

  • Loss of appetite.

  • Losing weight for an unknown reason.

 

A Wilms tumor that has spread to the lungs or liver can cause the following signs and symptoms:

  • Cough.

  • Blood in sputum.

  • Labored breathing.

  • Abdominal pain.

4.5. Tests  for the diagnosis of Wilms tumor and other kidney tumors in children

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 for signs of illness such as bumps 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.

  • 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.

  • Studies of the chemical composition of blood  : 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. This test is done to check how well the liver and kidneys are working.

  • Kidney function test: A procedure in which blood or urine samples are analyzed to measure the amount of certain substances excreted by the kidneys into the blood or urine. An increased or less than normal amount of a substance may be a sign that the kidneys are not working properly.

  • Urinalysis: A test to check the color of urine and its contents, such as sugar, protein, blood, and bacteria.

  • Ultrasound procedure  : A procedure in which high energy sound waves (ultrasound) are reflected from internal tissues or organs and create an echo. The echo forms a picture of body tissue called a sonogram. To diagnose a kidney tumor, an ultrasound of the abdominal cavity is done.

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Abdominal ultrasound. An ultrasound probe connected to the computer is pressed against the abdominal skin. The transducer reflects sound waves from internal organs and tissues, creating an echo that forms a sonogram (computer image).

  • CT (computed tomography)  : A procedure in which a series of detailed images of areas inside the body, such as the chest, abdomen and pelvis, are taken from different angles. The pictures were taken by a computer connected to an X-ray machine. The dye will 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) with gadolinium  : A procedure that uses a magnet, radio waves, and a computer to create a series of detailed images of areas inside the body, such as the abdomen. A substance called gadolinium is injected into a vein. Gadolinium gathers around cancer cells, so they appear brighter in the picture. This procedure is also called nuclear magnetic resonance imaging (MRI).

  • 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 and abdomen.

  • PET-CT  : 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 detailed picture than any test could do on its own. A PET scan is 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.

  • Biopsy  : Removing cells or tissues so that a pathologist can examine them under a microscope to check for signs of cancer. The decision to have a biopsy is based on the following:

     

    • The size of the tumor.

    • Stage of cancer. If the tumor appears to be resectable or Wilms' tumor is stage I or II, biopsy is not performed to avoid proliferation of tumor cells during the procedure.

    • Whether there is cancer in one or both kidneys.

    • Whether cancer is clearly shown on imaging.

    • Whether the patient is in a clinical trial.

 

A biopsy can be done before starting treatment, after chemotherapy to shrink a tumor, or after surgery to remove a tumor.

4.6. Factors  influencing the forecast  convalescence  and treatment options

The prognosis and treatment options for Wilms' tumor depend on the following conditions:

  • How tumor cells differ from normal kidney cells under a microscope.

  • Stage of cancer.

  • The type of tumor.

  • Child's age.

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

  • Are there any changes in chromosomes or genes.

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

 

The prognosis for renal cell carcinoma depends on the following:

  • Stage of cancer.

  • Whether the cancer has spread to the lymph nodes.

 

The prognosis for rhabdoid kidney tumors depends on the following:

  • The age of the child at the time of diagnosis.

  • Stage of cancer.

  • Whether the cancer has spread to the head or  spinal cord  ...

 

The prognosis for clear cell sarcoma of the kidney depends on the following:

  • The age of the child at the time of diagnosis.

  • Stage of cancer.

4.7. How cancer spreads throughout the body

Cancer spreads through the body in three ways.

Cancer can spread through tissues, the lymphatic system, and the blood:

  • Textile.  Cancer spreads from where it originated to surrounding areas.

  • The lymphatic system.  Cancer spreads from where it originated to the lymphatic system. Cancer spreads through the lymphatic vessels to other parts of the body.

  • Blood.  Cancer spreads from where it originated into the bloodstream. Cancer spreads through the blood vessels to other parts of the body.

 

Cancer can spread from where it originated to other parts of the body.

When cancer spreads to another part of the body, it is called metastasis. Cancer cells break away from where they originated (primary tumor) and travel through the lymphatic system or blood.

  • The lymphatic system.  Cancer enters the lymphatic system, travels through the lymphatic vessels, and forms a tumor (metastatic tumor) in another part of the body.

  • Blood.  Cancer enters the bloodstream, travels through the blood vessels, and forms a tumor (metastatic tumor) in another part of the body.

 

A metastatic tumor is the same type of cancer as the primary tumor. For example, if a Wilms tumor spreads to the lungs, the cancer cells in the lungs are actually Wilms tumor cells. The disease is Wilms' metastatic tumor, not lung cancer.

4.8. Treatments for Williams' tumor in children

There are various treatments for patients with Wilms' tumor and other kidney tumors in children.

Various treatments are available for children with Wilms kidney tumors and other childhood kidney tumors. 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 Wilms' tumor or other kidney tumors in children should plan for treatment with a team of healthcare professionals who are experts in treating childhood cancer.

Your child's treatment will be overseen by a pediatric oncologist, a doctor who specializes in treating children with cancer. The pediatric oncologist works with other pediatric providers who are experts in treating children with Wilms' tumor or other kidney tumors in children and who specialize in specific areas of medicine. This may include the following specialists:

  • Pediatrician.

  • Pediatric surgeon or urologist.

  • Radiologist oncologist.

  • Rehabilitation specialist.

  • Specialist Pediatric Nurse.

  • Social worker .

 

Six types of treatments are used:

 

1. Operation

 

Two types of surgeries are used to treat kidney tumors:

  • Nephrectomy  : Wilms tumor and other kidney tumors in children are usually treated with nephrectomy (surgery to remove the entire kidney). You can also remove nearby lymph nodes and check them for signs of cancer. Sometimes a kidney transplant (surgery to remove a kidney and replace it with a kidney from a donor) is done when the cancer affects both kidneys and the kidneys are not working properly.

  • Partial nephrectomy  : If cancer is found in both kidneys or may have spread to both kidneys, surgery may include partial nephrectomy (removal of a tumor in the kidney and a small amount of normal tissue around it). A partial nephrectomy is done to keep the kidneys working. Partial nephrectomy is also called renal sparing surgery.

 

After a 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 recurrence is called adjuvant therapy. Sometimes surgery is done again to see if the cancer remains after chemotherapy or radiation therapy.

Sometimes the tumor cannot be removed with surgery for one of the following reasons:

  • The tumor is too close to important organs or blood vessels.

  • The tumor is too large to be removed.

  • Cancer affects both kidneys if the tumors are not very small.

  • There is a blood clot in the vessels near the liver.

  • The patient has trouble breathing because the cancer has spread to the lungs.

 

In this case, a biopsy is done first. Chemotherapy is then given to reduce the size of the tumor before surgery, to preserve as much healthy tissue as possible and reduce problems after surgery. This is called neoadjuvant chemotherapy. Radiation therapy is given after surgery.

 

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.

External beam radiation therapy is used to treat Wilms' tumor and other kidney tumors 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). Combination chemotherapy is treatment with two or more anticancer drugs.

Systemic chemotherapy is used to treat Wilms' tumor and other kidney tumors in children.

Chemotherapy is sometimes given before surgery to reduce the size of the tumor, to preserve as much healthy tissue as possible and to reduce problems after surgery. This is called neoadjuvant chemotherapy.

4. 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.

Interferon and interleukin-2 (IL-2) are types of immunotherapy used to treat renal cell carcinoma in children. Interferon can slow tumor growth and help kill cancer cells. IL-2 enhances the growth and activity of many immune cells, especially lymphocytes (a type of white blood cell). Lymphocytes can attack and kill cancer cells.

5. 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 rescue is a treatment to replace hematopoietic cells. Stem cells (immature blood cells) are removed from the patient's blood or bone marrow, frozen and stored. After the patient completes chemotherapy, the stored stem cells are thawed and returned to the patient as an infusion. These stem cells turn into (and repair) blood cells.

High-dose stem cell rescue chemotherapy can be used to treat rhabdoid kidney tumor or recurrent Wilms tumor.

 

6. 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. Targeted therapies used to treat kidney tumors in children may include the following:

  • Tyrosine kinase inhibitors: This targeted therapy blocks the signals that cancer cells need to grow and divide.

    • Larotrectinib and entrectinib can be used to treat congenital mesoblastic nephroma with a specific gene change that cannot be removed with surgery, has spread to other parts of the body, or has continued to grow during treatment. This combination is also being studied for the treatment of congenital mesoblastic nephroma that has returned after treatment.

    • Sunitinib or cabozantinib can be used to treat renal cell carcinoma.

    • Axitinib is being studied in combination with a monoclonal antibody (nivolumab) to treat renal cell carcinoma that cannot be surgically removed or has spread to other parts of the body.

  • Histone methyltransferase inhibitors: This targeted therapy can inhibit the growth and division of cancer cells. Tasemetostat is being studied for the treatment of a rhabdoid kidney tumor that does not respond or has returned after treatment.

  • 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. Nivolumab, or a combination of nivolumab and a tyrosine kinase inhibitor (axitinib), is being studied for renal cell carcinoma that cannot be removed with surgery or has spread to other parts of the body.

4.9. Side Effects of Treating Williams' Kidney Tumor in Children

Treating Wilms' tumor and other kidney tumors 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 the following:

  • Physical problems such as heart, kidney, or pregnancy problems.

  • Infertility.

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

  • A second cancer (newer cancers), such as leukemia, gastrointestinal cancer, or breast cancer.

 

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.

 

Clinical trials are being done to find out if lower doses from chemotherapy and radiation can be used to reduce the late effects of treatment without changing how well the treatment works.

Monitoring for late kidney-related effects in patients with Wilms tumor and related conditions includes the following:

  • Children with WAGR syndrome are monitored throughout their lives as they are at increased risk of developing hypertension and kidney disease.

  • Children with Wilms' tumor and genitourinary abnormalities are monitored because they are at increased risk of late renal failure.

  • Patients with Wilms tumor and aniridia without genitourinary pathology are at lower risk but are monitored for kidney disease or renal failure.

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COST OF TREATMENT AND DIAGNOSIS OF KIDNEY CANCER IN TURKEY

  • Kidney cancer biopsy from $ 450

  • CT (computed tomography) for kidney cancer from $ 50

  • PET-CT for kidney cancer from $ 500

  • Chemotherapy for breast cancer from $ 1200

  • Cyberknife for kidney cancer from $ 4400

  • Da Vinci robotic system for kidney cancer from $ 16,000

  • Gamma Knife for Kidney Cancer from $ 6000

  • Nano Knife for Kidney Cancer from $ 12,000

  • Removal of the kidney for kidney cancer from $ 10,000

  • Radiation therapy for rectal cancer on request

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