General Information About Wilms Tumor and Other Childhood Kidney Tumors
Stages of Wilms Tumor and Other Childhood Kidney Tumors
Recurrent Wilms Tumor and Other Childhood Kidney Tumors
Treatment Option Overview
Treatment Options for Wilms Tumor and Other Childhood Kidney Tumors
To Learn More About Wilms Tumor and Other Childhood Kidney Tumors
Changes to This Summary (03/08/2012)
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General Information About Wilms Tumor and Other Childhood Kidney Tumors
Wilms tumor and other childhood kidney tumors are diseases in which malignant (cancer) cells form in the tissues of the kidney.
Wilms tumor
Wilms tumor and other kidney tumors are diseases in which malignant (cancer) cells are found in the kidney. In Wilms tumor, one or more tumors may be found in one or both kidneys. There are two kidneys, one on each side of the backbone, above the waist. Tiny tubules in the kidneys filter and clean the blood, taking out waste products and making urine. The urine passes from each kidney through a long tube called a ureter into the bladder. The bladder holds the urine until it is passed from the body.
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Wilms tumor may spread to the lungs, liver, or nearby lymph nodes.
Nephroblastomatosis is a condition in which abnormal tissue grows on the outer part of one or both kidneys. Children with this condition are at risk for developing a type of Wilms tumor that grows quickly. Frequent follow-up testing is important for at least 7 years after the child is treated.
Other kidney tumors
Other childhood kidney tumors, which are diagnosed and treated in different ways, include:
- Clear cell sarcoma of the kidney is a type of kidney tumor that may spread to the lung, bone, brain, and soft tissue.
- Rhabdoid tumor of the kidney is a type of kidney cancer that occurs mostly in infants and young children. It is often advanced at the time of diagnosis. Rhabdoid tumor of the kidney grows and spreads quickly, often to the lungs and brain.
- Neuroepithelial tumors of the kidney are rare and usually occur in young adults. They grow and spread quickly.
- Desmoplastic small round cell tumor of the kidney is a rare soft tissue sarcoma. See the PDQ summary on Childhood Soft Tissue Sarcoma Treatment 2 for more information.
- Cystic partially differentiated nephroblastoma is a very rare type of Wilms tumor made up of cysts.
- Renal cell carcinoma is rare in children or in adolescents younger than 15 years of age. However, it is much more common in adolescents between 15 and 19 years of age. Renal cell carcinomas can spread to the lungs, bones, liver, and lymph nodes.
- Congenital mesoblastic nephroma is a tumor of the kidney that is usually diagnosed within the first year of life and can usually be cured. One type of congenital mesoblastic nephroma may appear on an ultrasound exam before birth or may occur within the first 3 months after the child is born. Congenital mesoblastic nephroma occurs more often in males than females.
- Primary renal synovial sarcoma is a rare tumor of the kidney and is most common in young adults.
- Anaplastic sarcoma of the kidney is a rare tumor that is most commonly found in children or adolescents younger than 15 years of age. Anaplastic sarcoma of the kidney often spreads to the lungs, liver, or bones. There is no standard treatment for anaplastic sarcoma.
Having certain genetic syndromes or birth defects can increase the risk of developing Wilms tumor.
Anything that increases the risk of getting a disease is called a risk factor. Having a risk factor does not mean that you will get cancer; not having risk factors doesn't mean that you will not get cancer. Parents who think their child may be at risk should discuss this with the child's doctor.
Wilms tumor may be part of a genetic syndrome that affects growth or development. A genetic syndrome is a set of symptoms or conditions that occur together and is usually caused by abnormal genes. Certain birth defects can also increase a child's risk for developing Wilms tumor. The following genetic syndromes and birth defects have been linked to Wilms tumor:
- WAGR syndrome (Wilms tumor, aniridia, abnormal genitourinary system, and mental retardation).
- Beckwith-Wiedemann syndrome.
- Idiopathic hemihypertrophy (abnormally large growth of one side of the body or a body part).
- Denys-Drash syndrome.
- Cryptorchidism.
- Hypospadias.
Children with these genetic syndromes and birth defects should be screened for Wilms tumor every three months until at least age 8. An ultrasound test of the abdomen may be used for screening. Children with Beckwith-Wiedemann syndrome or hemihypertrophy are also screened for liver and adrenal tumors that are linked to these genetic syndromes. A test to check the alpha-fetoprotein level in the blood and an ultrasound of the abdomen are done until age 4 years. An ultrasound of the kidneys is done after age 4 years.
Some children develop Wilms tumor in both kidneys. These often appear when Wilms tumor is first diagnosed, but a Wilms tumor may also occur in the second kidney after the child is successfully treated for Wilms tumor in one kidney. A second tumor is much more likely to develop in the other kidney when a child's first Wilms tumor is diagnosed before age 12 months or when embryonic cells remain in the kidney.
Children at risk for developing a second Wilms tumor in the other kidney should be screened for Wilms tumor every three months for two to six years. An ultrasound test of the abdomen may be used for screening.
Having certain conditions may be associated with renal cell carcinoma.
Renal cell carcinoma may be related to the following conditions:
- Von Hippel-Lindau disease (an inherited condition that causes abnormal growth of blood vessels). Children with Von Hippel-Lindau disease should be checked yearly for renal cell carcinoma with an ultrasound of the abdomen or MRI (magnetic resonance imaging) beginning at age 8 to 11 years.
- Tuberous sclerosis (an inherited disease marked by noncancerous fatty cysts in the kidney).
- Familial renal cell carcinoma (an inherited condition that occurs when certain changes in the genes that cause kidney cancer are passed down from the parent to the child).
- Renal medullary carcinoma (a rare kidney cancer that grows and spreads quickly).
- Hereditary leiomyomatosis (a disorder that increases the risk of having cancer of the kidney, skin, and uterus).
- Second cancers (renal cell carcinoma may be found in patients several years after treatment for neuroblastoma).
Possible signs of Wilms tumor and other childhood kidney tumors include a lump in the abdomen and blood in the urine.
These and other symptoms may be caused by kidney tumors. Other conditions may cause the same symptoms. A doctor should be consulted if any of the following problems occur in the child:
Tests that examine the kidney and the blood are used to detect (find) Wilms tumor and other childhood kidney tumors.
The following tests and procedures may be used:
- Physical exam and history: An exam of the body to check general signs of health, including checking for signs of disease, such as lumps or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
- Complete blood count (CBC): A procedure in which a sample of blood is drawn and checked for the following:
- The number of red blood cells, white blood cells, and platelets.
- The amount of hemoglobin (the protein that carries oxygen) in the red blood cells.
- The portion of the blood sample made up of red blood cells.
- Blood chemistry studies: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by organs and tissues in the body. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it.
- Liver function test: A procedure in which a blood sample is checked to measure the amounts of certain substances released into the blood by the liver. A higher than normal amount of a substance can be a sign that the liver is not working as it should.
- Renal function test: A procedure in which blood or urine samples are checked to measure the amounts of certain substances released into the blood or urine by the kidneys. A higher or lower than normal amount of a substance can be a sign that the kidneys are not working as they should.
- Urinalysis: A test to check the color of urine and its contents, such as sugar, protein, blood, and bacteria.
- Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. An ultrasound of the abdomen is done to diagnose a kidney tumor.
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- Abdominal x-ray: An x-ray of the organs inside the abdomen. An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- Biopsy: The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer. Whether a biopsy is done depends on one or more of the following:
- Cancer is in one or both kidneys.
- Imaging tests clearly show the cancer.
- The patient is on a clinical trial.
Wilms tumor and other childhood kidney tumors are usually diagnosed and removed in surgery.
Once a kidney tumor is found, surgery is done to find out whether or not the tumor is cancer. If the tumor is only in the kidney, the surgeon will remove the whole kidney (nephrectomy). If there are tumors in both kidneys or if the tumor has spread outside the kidney, a piece of the tumor will be removed. In any case, a sample of tissue from the tumor is sent to a pathologist, who looks at it under a microscope to check for signs of cancer.
Certain factors affect prognosis (chance of recovery) and treatment options.
The prognosis (chance of recovery) and treatment options depend on the following:
- How different the tumor cells are from normal kidney cells.
- The stage of the cancer.
- The type and size of the tumor.
- The age of the child.
- Whether the tumor can be completely removed in surgery.
- Whether the cancer has just been diagnosed or has recurred (come back).
- Whether there are any abnormal chromosomes or genes.
- Whether the patient is treated by pediatric experts with experience in treating patients with Wilms tumor.
Stages of Wilms Tumor and Other Childhood Kidney Tumors
Wilms tumors and other childhood kidney tumors are staged during surgery and with imaging tests.
The process used to find out if cancer has spread outside of the kidney to other parts of the body is called staging. The information gathered from the staging process determines the stage of the disease. It is important to know the stage in order to plan treatment.
For Wilms tumor, the stage is determined during the initial surgery and with the results from imaging tests. The following imaging tests may be done to see if cancer has spread to other places in the body:
- CT scan (CAT scan): A procedure that makes a series of detailed pictures of areas inside the body, such as the chest or brain, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
- X-ray of the chest and bones: An x-ray is a type of energy beam that can go through the body and onto film, making a picture of areas inside the body.
- MRI (magnetic resonance imaging): A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body, such as the brain. This procedure is also called nuclear magnetic resonance imaging (NMRI).
- Bone scan: A procedure to check if there are rapidly dividing cells, such as cancer cells, in the bone. A very small amount of radioactive material is injected into a vein and travels through the bloodstream. The radioactive material collects in the bones and is detected by a scanner.
- Ultrasound exam: A procedure in which high-energy sound waves (ultrasound) are bounced off internal tissues or organs and make echoes. The echoes form a picture of body tissues called a sonogram. An ultrasound of the major heart vessels is done to stage Wilms tumor.
- Cystoscopy: A procedure to look inside the bladder and urethra to check for abnormal areas. A cystoscope is inserted through the urethra into the bladder. A cystoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue samples, which are checked under a microscope for signs of cancer.
There are three ways that cancer spreads in the body.
The three ways that cancer spreads in the body are:
- Through tissue. Cancer invades the surrounding normal tissue.
- Through the lymph system. Cancer invades the lymph system and travels through the lymph vessels to other places in the body.
- Through the blood. Cancer invades the veins and capillaries and travels through the blood to other places in the body.
When cancer cells break away from the primary (original) tumor and travel through the lymph or blood to other places in the body, another (secondary) tumor may form. This process is called metastasis. The secondary (metastatic) tumor is the same type of cancer as the primary tumor. For example, if breast cancer spreads to the bones, the cancer cells in the bones are actually breast cancer cells. The disease is metastatic breast cancer, not bone cancer.
In addition to the stages, Wilms tumors are described by their histology.
The histology (how the cells look under a microscope) of the tumor affects the prognosis and the treatment of Wilms tumor. The histology may be favorable or anaplastic (unfavorable). Tumors with a favorable histology have a better prognosis and respond better to chemotherapy than those with anaplastic histology. Tumor cells that are anaplastic divide rapidly and do not look like the type of cells they came from. Anaplastic tumors are harder to treat with chemotherapy than other Wilms tumors at the same stage.
The following stages are used for both favorable histology and anaplastic Wilms tumors:
In stage I, the tumor was completely removed by surgery and all of the following are true:
- Cancer was found only in the kidney and did not spread to blood vessels of the kidney.
- The outer layer of the kidney did not break open.
- The tumor did not break open.
- A biopsy of the tumor was not done.
- No cancer cells were found at the edges of the area where the tumor was removed.
In stage II, the tumor was completely removed by surgery and no cancer cells were found at the edges of the area where the cancer was removed. Before the tumor was removed, one of the following was true:
- Cancer had spread out of the kidney to nearby soft tissue.
- Cancer had spread to blood vessels of the kidney.
In stage III, cancer remains in the abdomen after surgery and at least one of the following is true:
- Cancer spread to lymph nodes in the abdomen or pelvis (the part of the body between the hips).
- Cancer spread to or through the surface of the peritoneum (the layer of tissue that lines the abdominal cavity and covers most organs in the abdomen).
- Chemotherapy was given before surgery and a biopsy of the tumor was done during surgery to remove it.
- The tumor broke open before or during surgery to remove it.
- The tumor was removed in more than one piece.
In stage IV, cancer has spread through the blood to organs such as the lungs, liver, bone, or brain, or to lymph nodes outside of the abdomen and pelvis.
Stage V and those at high risk of developing Wilms tumor
In stage V, cancer cells are found in both kidneys when the disease is first diagnosed.
Recurrent Wilms Tumor and Other Childhood Kidney Tumors
Recurrent cancer is cancer that has recurred (come back) after it has been treated.
Treatment Option Overview
There are different types of treatment for patients with Wilms tumor and other childhood kidney tumors.
Different types of treatment are available for children with Wilms and other childhood kidney tumors. Some treatments are standard (the currently used treatment), and some are being tested in clinical trials. A treatment clinical trial is a research study meant to help improve current treatments or obtain information on new treatments for patients with cancer. When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment.
Because cancer in children is rare, taking part in a clinical trial should be considered. Some clinical trials are open only to patients who have not started treatment.
Children with Wilms tumor or other childhood kidney tumors should have their treatment planned by a team of health care providers who are experts in treating cancer in children.
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 health care providers who are experts in treating children with Wilms tumor or other childhood kidney tumors and who specialize in certain areas of medicine. These may include the following specialists:
- Pediatric surgeon or urologist.
- Radiation oncologist.
- Rehabilitation specialist.
- Pediatric nurse specialist.
- Social worker.
Some cancer treatments cause side effects months or years after treatment has ended.
Side effects from cancer treatment that begin during or after treatment and continue for months or years are called late effects. Late effects of cancer treatment may include the following:
- Physical problems.
- Changes in mood, feelings, thinking, learning, or memory.
- Second cancers (new types of cancer).
Some late effects may be treated or controlled. It is important to talk with your child's doctors about the effects cancer treatment can have on your child. (See the PDQ summary on Late Effects of Treatment for Childhood Cancer 3 for more information).
Clinical trials are ongoing to find out if lower doses of chemotherapy and radiation can be used.
Four types of standard treatment are used:
Wilms tumor and other childhood kidney tumors are usually treated with nephrectomy (surgery to remove the whole kidney). Nearby lymph nodes may also be removed.
If cancer is found in both kidneys, surgery may include a partial nephrectomy (removal of the cancer in the kidney and a small amount of normal tissue around it). Partial nephrectomy is done to keep the kidney working.
Chemotherapy may be given before surgery to make the tumor smaller so less kidney tissue needs to be removed and there are fewer problems after surgery. This is called neoadjuvant chemotherapy.
Even if the doctor removes all the cancer that can be seen at the time of the surgery, some patients may be given chemotherapy or radiation therapy after surgery to kill any cancer cells that are left. Treatment given after the surgery, to lower the risk that the cancer will come back, is called adjuvant therapy. Sometimes, a second-look surgery is done to see if cancer remains after chemotherapy or radiation therapy.
Radiation therapy is a cancer treatment that uses high-energy x-rays or other types of radiation to kill cancer cells or keep them from growing. There are two types of radiation therapy. External radiation therapy uses a machine outside the body to send radiation toward the cancer. Internal radiation therapy uses a radioactive substance sealed in needles, seeds, wires, or catheters that are placed directly into or near the cancer. The way the radiation therapy is given depends on the type and stage of the cancer being treated and whether a biopsy was done before surgery to remove the tumor.
Chemotherapy is a cancer treatment that uses drugs to stop the growth of cancer cells, either by killing the cells or by 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 placed directly into the cerebrospinal fluid, an organ, or a body cavity such as the abdomen, the drugs mainly affect cancer cells in those areas (regional chemotherapy). The way the chemotherapy is given depends on the type and stage of the cancer being treated.
Combination chemotherapy is treatment using two or more anticancer drugs.
See Drugs Approved for Wilms Tumor and Other Childhood Kidney Cancers 4 for more information.
Biologic therapy is a treatment that uses the patient's immune system to fight cancer. Substances made by the body or made in a laboratory are used to boost, direct, or restore the body's natural defenses against cancer. This type of cancer treatment is also called biotherapy or immunotherapy.
New types of treatment are being tested in clinical trials.
This summary section describes treatments that are being studied in clinical trials. It may not mention every new treatment being studied. Information about clinical trials is available from the NCI Web site 5.
Watchful waiting is closely monitoring a patient’s condition without giving any treatment until symptoms appear or change.
High-dose chemotherapy with stem cell transplant
High-dose chemotherapy with stem cell transplant is a method of giving high doses of chemotherapy and replacing blood -forming cells destroyed by the cancer treatment. Stem cells (immature blood cells) are removed from the blood or bone marrow of the patient or a donor and are frozen and stored. After the chemotherapy is completed, the stored stem cells are thawed and given back to the patient through an infusion. These re-infused stem cells grow into (and restore) the body's blood cells.
Targeted therapy is a treatment that uses drugs or other substances to identify and attack specific cancer cells without harming normal cells. Wilms tumor may be treated with a targeted therapy drug that stops cells from dividing and prevents the growth of new blood vessels that tumors need to grow.
Patients may want to think about taking part in a clinical trial.
For some patients, taking part in a clinical trial may be the best treatment choice. Clinical trials are part of the cancer research process. Clinical trials are done to find out if new cancer treatments are safe and effective or better than the standard treatment.
Many of today's standard treatments for cancer are based on earlier clinical trials. Patients who take part in a clinical trial may receive the standard treatment or be among the first to receive a new treatment.
Patients who take part in clinical trials also help improve the way cancer will be treated in the future. Even when clinical trials do not lead to effective new treatments, they often answer important questions and help move research forward.
Patients can enter clinical trials before, during, or after starting their cancer treatment.
Some clinical trials only include patients who have not yet received treatment. Other trials test treatments for patients whose cancer has not gotten better. There are also clinical trials that test new ways to stop cancer from recurring (coming back) or reduce the side effects of cancer treatment.
Clinical trials are taking place in many parts of the country. See the Treatment Options section that follows for links to current treatment clinical trials. These have been retrieved from NCI's listing of clinical trials.
Follow-up tests may be needed.
Some of the tests that were done to diagnose the cancer or to find out the stage of the cancer may be repeated. Some tests will be repeated in order to see how well the treatment is working. Decisions about whether to continue, change, or stop treatment may be based on the results of these tests. This is sometimes called re-staging.
Some of the tests will continue to be done from time to time after treatment has ended. The results of these tests can show if your condition has changed or if the cancer has recurred (come back). These tests are sometimes called follow-up tests or check-ups.
Treatment Options for Wilms Tumor and Other Childhood Kidney Tumors
A link to a list of current clinical trials is included for each treatment section. For some types or stages of cancer, there may not be any trials listed. Check with your doctor for clinical trials that are not listed here but may be right for you.
Stage I Wilms TumorTreatment of stage I Wilms tumor with favorable histology may include the following:
- Nephrectomy with lymph node removal followed by combination chemotherapy.
- A clinical trial of nephrectomy with lymph node removal, followed by watchful waiting or new combinations of chemotherapy.
Treatment of stage I anaplastic Wilms tumor may include the following:
- Nephrectomy with lymph node removal followed by combination chemotherapy and radiation therapy to the flank (either side of the body between the ribs and hipbone) of the body.
- A clinical trial of nephrectomy with lymph node removal, followed by new combinations of chemotherapy with radiation therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage I Wilms tumor 6. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Stage II Wilms TumorTreatment of stage II Wilms tumor with favorable histology may include the following:
- Nephrectomy with removal of lymph nodes, followed by combination chemotherapy.
- A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy.
Treatment of stage II anaplastic Wilms tumor may include the following:
- Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
- A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy with radiation therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage II Wilms tumor 8. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Stage III Wilms TumorTreatment of stage III Wilms tumor with favorable histology may include the following:
- Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
- A clinical trial of nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and new combinations of chemotherapy.
Treatment of stage III anaplastic Wilms tumor may include the following:
- Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
- Combination chemotherapy given before nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen.
- Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy.
- A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy with radiation therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage III Wilms tumor 9. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Stage IV Wilms TumorTreatment of stage IV Wilms tumor with favorable histology may include the following:
- Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to the lungs, patients will also receive radiation therapy to the lungs.
- A clinical trial of nephrectomy with removal of lymph nodes, followed by chemotherapy for cancer that has spread only to the lungs, and by radiation therapy to the abdomen. If cancer in the lungs remains after chemotherapy, radiation therapy may be given.
- A clinical trial of nephrectomy with removal of lymph nodes, followed by chemotherapy, and radiation therapy to all places where cancer has spread.
Treatment of stage IV anaplastic Wilms tumor may include the following:
- Combination chemotherapy given before nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen. If cancer has spread to the lungs, patients will also receive radiation therapy to the lungs.
- Nephrectomy with removal of lymph nodes, followed by radiation therapy to the abdomen and combination chemotherapy. If cancer has spread to the lungs, patients will also receive radiation therapy to the lungs.
- A clinical trial of nephrectomy with removal of lymph nodes, followed by new combinations of chemotherapy with radiation therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage IV Wilms tumor 10. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Stage V Wilms Tumor and those at high risk of developing Wilms tumorTreatment of stage V Wilms tumor may be different for each patient and may include:
- Combination chemotherapy to shrink the tumor, followed by repeat imaging at 4 to 8 weeks to guide the choice of further therapy (partial nephrectomy, biopsy, continued chemotherapy, and/or radiation therapy).
- A biopsy of the kidneys is followed by chemotherapy to shrink the tumor. Second-look surgery is done to remove as much of the cancer as possible. This may be followed by more chemotherapy and/or radiation therapy if cancer remains after surgery.
- A clinical trial of chemotherapy to shrink the tumor, followed by repeat imaging at 6 and 12 weeks to guide the choice of further therapy (surgery, biopsy, continued chemotherapy and/or radiation therapy).
If a kidney transplant is needed because of kidney problems, it is delayed until 1 to 2 years after treatment is completed and there are no signs of cancer.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage V Wilms tumor 11. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Inoperable TumorsSometimes the tumor is inoperable (cannot be removed by surgery) because it is too close to important organs or blood vessels, too large to remove, or there is cancer in both kidneys. In this case, chemotherapy may be given to reduce the size of the tumor so as much tumor as possible can be removed in surgery. Radiation therapy is given after surgery.
Clear Cell Sarcoma of the KidneyTreatment of clear cell sarcoma of the kidney may include the following:
- Nephrectomy with removal of lymph nodes followed by combination chemotherapy and radiation therapy to the abdomen.
- A clinical trial of nephrectomy with lymph node removal, with or without radiation therapy to the abdomen, and new combinations of chemotherapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with clear cell sarcoma of the kidney 12. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Rhabdoid Tumor of the KidneyThere is no standard treatment for rhabdoid tumor of the kidney. Treatment is usually within a clinical trial and may include new combinations of chemotherapy and radiation therapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with rhabdoid tumor of the kidney 13. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Neuroepithelial Tumor of the KidneyThere is no standard treatment for neuroepithelial tumor of the kidney. Treatment is usually within a clinical trial. It may be treated in the same way that Ewing family of tumors or primitive neuroectodermal tumors are treated. See the PDQ summary on Ewing Sarcoma Family of Tumors Treatment 14 for more information.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with peripheral primitive neuroectodermal tumor of the kidney 15. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Congenital Mesoblastic NephromaTreatment for congenital mesoblastic nephroma is usually surgery with or without chemotherapy.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with congenital mesoblastic nephroma 16. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Renal Cell CancerTreatment of renal cell cancer is usually nephrectomy with removal of lymph nodes. In some cases, treatment may be a partial nephrectomy with removal of lymph nodes. If cancer has spread, treatment may include biologic therapy. See the PDQ summary on Renal Cell Cancer Treatment 17 for more information.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with childhood renal cell carcinoma 18. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
NephroblastomatosisTreatment of nephroblastomatosis may include the following:
- Combination chemotherapy.
- Partial nephrectomy may be done to keep as much kidney function as possible.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with diffuse hyperplastic perilobar nephroblastomatosis 19. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
Recurrent Wilms Tumor and Other Childhood Kidney TumorsTreatment of recurrent Wilms tumor may include the following:
- Combination chemotherapy, surgery, and radiation therapy, with or without stem cell transplant, using the child's own blood stem cells.
- A clinical trial of targeted therapy.
Treatment of recurrent clear cell sarcoma of the kidney may include chemotherapy. Treatment of clear cell sarcoma of the kidney that has recurred in the brain may include chemotherapy, surgery, and radiation therapy.
Treatment of recurrent rhabdoid tumor of the kidney, neuroepithelial tumor of the kidney, and renal cell cancer is usually within a clinical trial.
Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with recurrent Wilms tumor and other childhood kidney tumors 20. For more specific results, refine the search by using other search features, such as the location of the trial, the type of treatment, or the name of the drug. General information about clinical trials is available from the NCI Web site 7.
To Learn More About Wilms Tumor and Other Childhood Kidney Tumors
For more information from the National Cancer Institute about Wilms tumor and other childhood kidney tumors, see the following:
- Wilms Tumor and other Childhood Kidney Tumors Home Page 21
- What You Need to Know About™ Kidney Cancer 22
- Drugs Approved for Wilms Tumor and Other Childhood Kidney Cancers 4
- Biological Therapies for Cancer: Questions and Answers 23
- Understanding Cancer Series: Targeted Therapies 24 (Advances in Targeted Therapies)
- Targeted Cancer Therapies 25
For more childhood cancer information and other general cancer resources from the National Cancer Institute, see the following:
- What You Need to Know About™ Cancer 26
- Childhood Cancers 27
- CureSearch for Children's Cancer 28
- Late Effects of Treatment for Childhood Cancer 3
- Adolescents and Young Adults with Cancer 29
- Young People with Cancer: A Handbook for Parents 30
- Care for Children and Adolescents with Cancer 31
- Understanding Cancer Series: Cancer 32
- Cancer Staging 33
- Coping with Cancer: Supportive and Palliative Care 34
- Questions to Ask Your Doctor About Cancer 35
- Cancer Library 36
- Information for Survivors/Caregivers/Advocates 37
Changes to This Summary (03/08/2012)
The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.
Changes were made to this summary to match those made to the health professional version.
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About PDQ
PDQ is a comprehensive cancer database available on NCI's Web site.
PDQ is the National Cancer Institute's (NCI's) comprehensive cancer information database. Most of the information contained in PDQ is available online at NCI's Web site 39. PDQ is provided as a service of the NCI. The NCI is part of the National Institutes of Health, the federal government's focal point for biomedical research.
PDQ contains cancer information summaries.
The PDQ database contains summaries of the latest published information on cancer prevention, detection, genetics, treatment, supportive care, and complementary and alternative medicine. Most summaries are available in two versions. The health professional versions provide detailed information written in technical language. The patient versions are written in easy-to-understand, nontechnical language. Both versions provide current and accurate cancer information.
The PDQ cancer information summaries are developed by cancer experts and reviewed regularly.
Editorial Boards made up of experts in oncology and related specialties are responsible for writing and maintaining the cancer information summaries. The summaries are reviewed regularly and changes are made as new information becomes available. The date on each summary ("Date Last Modified") indicates the time of the most recent change.
PDQ also contains information on clinical trials.
A clinical trial is a study to answer a scientific question, such as whether one treatment is better than another. Trials are based on past studies and what has been learned in the laboratory. Each trial answers certain scientific questions in order to find new and better ways to help cancer patients. During treatment clinical trials, information is collected about the effects of a new treatment and how well it works. If a clinical trial shows that a new treatment is better than one currently being used, the new treatment may become "standard." In the United States, about two-thirds of children with cancer are treated in a clinical trial at some point in their illness.
Listings of clinical trials are included in PDQ and are available online at NCI's Web site 7. Descriptions of the trials are available in health professional and patient versions. For additional help in locating a childhood cancer clinical trial, call the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
The PDQ database contains listings of groups specializing in clinical trials.
The Children's Oncology Group (COG) is the major group that organizes clinical trials for childhood cancers in the United States. Information about contacting COG is available on the NCI Web site 39 or from the Cancer Information Service at 1-800-4-CANCER (1-800-422-6237).
Glossary Termsabdomen (AB-doh-men)The area of the body that contains the pancreas, stomach, intestines, liver, gallbladder, and other organs.abdominal ultrasound (ab-DAH-mih-nul UL-truh-SOWND) A procedure used to examine the organs in the abdomen. An ultrasound transducer (probe) is pressed firmly against the skin of the abdomen. High-energy sound waves from the transducer bounce off tissues and create echoes. The echoes are sent to a computer, which makes a picture called a sonogram. Also called transabdominal ultrasound.abdominal x-ray (ab-DAH-mih-nul EX-ray) An x-ray of the organs inside the abdomen. An x-ray is a type of radiation that can pass through the body and onto film, making pictures of areas inside the body. X-rays may be used to help diagnose disease.abnormal (ab-NOR-mul) Not normal. An abnormal lesion or growth may be cancer, premalignant (likely to become cancer), or benign (not cancer).adrenal gland (uh-DREE-nul...) A small gland that makes steroid hormones, adrenaline, and noradrenaline. These hormones help control heart rate, blood pressure, and other important body functions. There are two adrenal glands, one on top of each kidney. Also called suprarenal gland.advanced cancer (ad-VANST KAN-ser) Cancer that has spread to other places in the body and usually cannot be cured or controlled with treatment.alpha-fetoprotein (AL-fuh-FEE-toh-PROH-teen) A protein normally produced by a fetus. AFP levels are usually undetectable in the blood of healthy adult men or women (who are not pregnant). An elevated level of AFP suggests the presence of either a primary liver cancer or germ cell tumor. Also called AFP.anaplastic (A-nuh-PLAS-tik) A term used to describe cancer cells that divide rapidly and have little or no resemblance to normal cells.aniridia (A-nih-RIH-dee-uh) A disorder in which a person is born without part or all of the iris (colored tissue at the front of the eyeball). Aniridia usually affects both eyes and causes other eye problems, including being sensitive to light and loss of vision.backbone (BAK-bone) The bones, muscles, tendons, and other tissues that reach from the base of the skull to the tailbone. The backbone encloses the spinal cord and the fluid surrounding the spinal cord. Also called spinal column, spine, and vertebral column.bacteria (bak-TEER-ee-uh) A large group of single-cell microorganisms. Some cause infections and disease in animals and humans. The singular of bacteria is bacterium.Beckwith-Wiedemann syndrome (BEK-with-VEE-deh-mahn SIN-drome) A rare, overgrowth disorder in which babies are large at birth and may develop low blood sugar. Other common symptoms are a large tongue, large internal organs, and defects of the abdominal wall near the navel. Beckwith-Wiedemann syndrome increases the risk of developing certain cancers, especially Wilms tumor.biopsy (BY-op-see) The removal of cells or tissues for examination by a pathologist. The pathologist may study the tissue under a microscope or perform other tests on the cells or tissue. There are many different types of biopsy procedures. The most common types include: (1) incisional biopsy, in which only a sample of tissue is removed; (2) excisional biopsy, in which an entire lump or suspicious area is removed; and (3) needle biopsy, in which a sample of tissue or fluid is removed with a needle. When a wide needle is used, the procedure is called a core biopsy. When a thin needle is used, the procedure is called a fine-needle aspiration biopsy.bladder (BLA-der) The organ that stores urine.blood (blud) A tissue with red blood cells, white blood cells, platelets, and other substances suspended in fluid called plasma. Blood takes oxygen and nutrients to the tissues, and carries away wastes.blood chemistry study (blud KEH-mih-stree STUH-dee) A procedure in which a sample of blood is examined to measure the amounts of certain substances made in the body. An abnormal amount of a substance can be a sign of disease in the organ or tissue that produces it.blood vessel (blud VEH-sel) A tube through which the blood circulates in the body. Blood vessels include a network of arteries, arterioles, capillaries, venules, and veins.cancer (KAN-ser) A term for diseases in which abnormal cells divide without control and can invade nearby tissues. Cancer cells can also spread to other parts of the body through the blood and lymph systems. There are several main types of cancer. Carcinoma is a cancer that begins in the skin or in tissues that line or cover internal organs. Sarcoma is a cancer that begins in bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. Leukemia is a cancer that starts in blood-forming tissue such as the bone marrow, and causes large numbers of abnormal blood cells to be produced and enter the blood. Lymphoma and multiple myeloma are cancers that begin in the cells of the immune system. Central nervous system cancers are cancers that begin in the tissues of the brain and spinal cord. Also called malignancy.cell (sel) The individual unit that makes up the tissues of the body. All living things are made up of one or more cells.chemotherapy (KEE-moh-THAYR-uh-pee) Treatment with drugs that kill cancer cells.chromosome (KROH-muh-some) Part of a cell that contains genetic information. Except for sperm and eggs, all human cells contain 46 chromosomes.clear cell sarcoma of the kidney (kleer sel sar-KOH-muh ...KID-nee) A rare type of kidney cancer, in which the inside of the cells look clear when viewed under a microscope. Clear cell sarcoma can spread from the kidney to other organs, most commonly the bone, but also including the lungs, brain, and soft tissues of the body.clinical trial (KLIH-nih-kul TRY-ul) A type of research study that tests how well new medical approaches work in people. These studies test new methods of screening, prevention, diagnosis, or treatment of a disease. Also called clinical study.complete blood count (kum-PLEET blud kownt) A test to check the number of red blood cells, white blood cells, and platelets in a sample of blood. Also called blood cell count and CBC.condition (kun-DIH-shun) In medicine, a health problem with certain characteristics or symptoms.congenital mesoblastic nephroma (kun-JEH-nih-tul MEH-zoh-BLAS-tik neh-FROH-muh) A type of kidney tumor that is usually found before birth by ultrasound or within the first 3 months of life. It contains fibroblastic cells (connective tissue cells), and may spread to the other kidney or to nearby tissue. Congenital mesoblastic nephroma is more common in males.contrast material (KON-trast muh-TEER-ee-ul) A dye or other substance that helps show abnormal areas inside the body. It is given by injection into a vein, by enema, or by mouth. Contrast material may be used with x-rays, CT scans, MRI, or other imaging tests.cryptorchidism (krip-TOR-kih-dih-zum) A condition in which one or both testicles fail to move from the abdomen, where they develop before birth, into the scrotum. Cryptorchidism may increase the risk for development of testicular cancer. Also called undescended testicles.CT scan (… skan) A series of detailed pictures of areas inside the body taken from different angles. The pictures are created by a computer linked to an x-ray machine. Also called CAT scan, computed tomography scan, computerized axial tomography scan, and computerized tomography.cure (kyoor) To heal or restore health; a treatment to restore health.cyst (sist) A sac or capsule in the body. It may be filled with fluid or other material.Denys-Drash syndrome (deh-NEES-drash SIN-drome) A rare disorder that causes kidney failure before age 3, abnormal development of the sexual organs, and, in most cases, Wilms tumor (a type of kidney cancer). Children with Denys-Drash syndrome are also at high risk of some other types of cancer. Also called DDS.desmoplastic small round cell tumor (DES-moh-PLAS-tik ... TOO-mer) A rare, aggressive cancer that usually affects young males and usually is located in the abdomen.diagnosis (DY-ug-NOH-sis) The process of identifying a disease, such as cancer, from its signs and symptoms.differentiation (DIH-feh-REN-shee-AY-shun) In cancer, refers to how mature (developed) the cancer cells are in a tumor. Differentiated tumor cells resemble normal cells and tend to grow and spread at a slower rate than undifferentiated or poorly differentiated tumor cells, which lack the structure and function of normal cells and grow uncontrollably.disorder (dis-OR-der) In medicine, a disturbance of normal functioning of the mind or body. Disorders may be caused by genetic factors, disease, or trauma.embryonic (EM-bree-AH-nik) Having to do with an embryo, which is an early stage in the development of a plant or animal.fever (FEE-ver) An increase in body temperature above normal (98.6 degrees F), usually caused by disease.follow-up (FAH-loh-up) Monitoring a person's health over time after treatment. This includes keeping track of the health of people who participate in a clinical study or clinical trial for a period of time, both during the study and after the study ends.gene (jeen) The functional and physical unit of heredity passed from parent to offspring. Genes are pieces of DNA, and most genes contain the information for making a specific protein.genetic (jeh-NEH-tik) Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.genitourinary system (jeh-nih-toh-YOOR-ih-nayr-ee SIS-tem) The parts of the body that play a role in reproduction, getting rid of waste products in the form of urine, or both.hemihypertrophy (HEH-mee-hy-PER-troh-fee) A condition in which one side of the body or a part of one side is larger than the other. Children with hemihypertrophy have an increased risk of developing certain types of cancer, including Wilms tumor (a childhood kidney cancer) and liver cancer.hemoglobin (HEE-moh-GLOH-bin) The substance inside red blood cells that binds to oxygen in the lungs and carries it to the tissues.hereditary leiomyomatosis and renal cell cancer syndrome (heh-REH-dih-TAYR-ee LY-oh-MY-oh-muh-TOH-sis ... REE-nul sel KAN-ser SIN-drome) A rare inherited disorder that increases the risk of developing benign (not cancer) tumors of the skin and the uterus (leiomyomas) and malignant (cancer) tumors of the uterus (leiomyosarcoma) and the kidney. Also called HLRCC.hypospadias (HY-poh-SPAY-dee-us) A birth defect in which the opening of the urethra (the tube through which urine leaves the body) is not in its normal place. In males with hypospadias, the urethra opens on the underside of the penis or between the anus and the scrotum. In females with hypospadias, it opens into the vagina. Hypospadias is much more common in males than in females, and can be corrected by surgery. Children with hypospadias have an increased risk of developing Wilms tumor (a type of kidney cancer).idiopathic (IH-dee-oh-PA-thik) Describes a disease of unknown cause.imaging test (IH-muh-jing …) A type of test that makes pictures of areas inside the body. Some examples of imaging tests are CT scans and MRIs. Also called imaging procedure.inherited (in-HAYR-ih-ted) Transmitted through genes that have been passed from parents to their offspring (children).injection (in-JEK-shun) Use of a syringe and needle to push fluids or drugs into the body; often called a "shot."kidney (KID-nee) One of a pair of organs in the abdomen. Kidneys remove waste from the blood (as urine), produce erythropoietin (a substance that stimulates red blood cell production), and play a role in blood pressure regulation.kidney cancer (KID-nee KAN-ser) Cancer that forms in tissues of the kidneys. Kidney cancer includes renal cell carcinoma (cancer that forms in the lining of very small tubes in the kidney that filter the blood and remove waste products) and renal pelvis carcinoma (cancer that forms in the center of the kidney where urine collects). It also includes Wilms tumor, which is a type of kidney cancer that usually develops in children under the age of 5.liver (LIH-ver) A large organ located in the upper abdomen. The liver cleanses the blood and aids in digestion by secreting bile.liver function test (LIH-ver FUNK-shun ...) A blood test to measure the blood levels of certain substances released by the liver. A high or low level of certain substances can be a sign of liver disease.lung (lung) One of a pair of organs in the chest that supplies the body with oxygen, and removes carbon dioxide from the body.lymph node (limf node) A rounded mass of lymphatic tissue that is surrounded by a capsule of connective tissue. Lymph nodes filter lymph (lymphatic fluid), and they store lymphocytes (white blood cells). They are located along lymphatic vessels. Also called lymph gland.malignant (muh-LIG-nunt) Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.medical history (MEH-dih-kul HIH-stuh-ree) A record of information about a person’s health. A personal medical history may include information about allergies, illnesses, surgeries, immunizations, and results of physical exams and tests. It may also include information about medicines taken and health habits, such as diet and exercise. A family medical history includes health information about a person’s close family members (parents, grandparents, children, brothers, and sisters). This includes their current and past illnesses. A family medical history may show a pattern of certain diseases in a family.microscope (MY-kroh-SKOPE) An instrument that is used to look at cells and other small objects that cannot be seen with the eye alone.MRI A procedure in which radio waves and a powerful magnet linked to a computer are used to create detailed pictures of areas inside the body. These pictures can show the difference between normal and diseased tissue. MRI makes better images of organs and soft tissue than other scanning techniques, such as computed tomography (CT) or x-ray. MRI is especially useful for imaging the brain, the spine, the soft tissue of joints, and the inside of bones. Also called magnetic resonance imaging, NMRI, and nuclear magnetic resonance imaging.National Cancer Institute (NA-shuh-nul KAN-ser IN-stih-TOOT) The National Cancer Institute, part of the National Institutes of Health of the United States Department of Health and Human Services, is the Federal Government's principal agency for cancer research. The National Cancer Institute conducts, coordinates, and funds cancer research, training, health information dissemination, and other programs with respect to the cause, diagnosis, prevention, and treatment of cancer. Access the National Cancer Institute Web site at http://www.cancer.gov. Also called NCI.nephrectomy (neh-FREK-toh-mee) Surgery to remove a kidney or part of a kidney. In a partial nephrectomy, part of one kidney or a tumor is removed, but not an entire kidney. In a simple nephrectomy, one kidney is removed. In a radical nephrectomy, an entire kidney, nearby adrenal gland and lymph nodes, and other surrounding tissue are removed. In a bilateral nephrectomy, both kidneys are removed.neuroblastoma (NOOR-oh-blas-TOH-muh) Cancer that arises in immature nerve cells and affects mostly infants and children.neuroepithelial (NOOR-oh-eh-pih-THEE-lee-ul) Having to do with tissue made up of sensory cells, such as tissue found in the ear, nose, and tongue.organ (OR-gun) A part of the body that performs a specific function. For example, the heart is an organ.oxygen (OK-sih-jen) A colorless, odorless gas. It is needed for animal and plant life. Oxygen that is breathed in enters the blood from the lungs and travels to the tissues.pathologist (puh-THAH-loh-jist) A doctor who identifies diseases by studying cells and tissues under a microscope.PDQ PDQ is an online database developed and maintained by the National Cancer Institute. Designed to make the most current, credible, and accurate cancer information available to health professionals and the public, PDQ contains peer-reviewed summaries on cancer treatment, screening, prevention, genetics, complementary and alternative medicine, and supportive care; a registry of cancer clinical trials from around the world; and directories of physicians, professionals who provide genetics services, and organizations that provide cancer care. Most of this information, and more specific information about PDQ, can be found on the NCI's Web site at http://www.cancer.gov/cancertopics/pdq. Also called Physician Data Query.pediatric (pee-dee-A-trik) Having to do with children.physical examination (FIH-zih-kul eg-ZA-mih-NAY-shun) An exam of the body to check for general signs of disease.platelet (PLAYT-let) A tiny piece of a cell found in the blood that breaks off from a large cell found in the bone marrow. Platelets help wounds heal and prevent bleeding by forming blood clots. Also called thrombocyte.prognosis (prog-NO-sis) The likely outcome or course of a disease; the chance of recovery or recurrence.protein (PROH-teen) A molecule made up of amino acids that are needed for the body to function properly. Proteins are the basis of body structures such as skin and hair and of substances such as enzymes, cytokines, and antibodies.recover (ree-KUH-ver) To become well and healthy again.recur (ree-KER) To come back or to return.recurrent cancer (ree-KER-ent KAN-ser) Cancer that has recurred (come back), usually after a period of time during which the cancer could not be detected. The cancer may come back to the same place as the original (primary) tumor or to another place in the body. Also called recurrence.red blood cell (red blud sel) A cell that carries oxygen to all parts of the body. Also called erythrocyte and RBC.renal cell cancer (REE-nul sel KAN-ser) The most common type of kidney cancer. It begins in the lining of the renal tubules in the kidney. The renal tubules filter the blood and produce urine. Also called hypernephroma, renal cell adenocarcinoma, and renal cell carcinoma.renal function test (REE-nul FUNK-shun...) A test in which blood or urine samples are checked for the amounts of certain substances released by the kidneys. A higher- or lower-than-normal amount of a substance can be a sign that the kidneys are not working the way they should. Also called kidney function test.rhabdoid tumor (RAB-doyd TOO-mer) A malignant tumor of either the central nervous system (CNS) or the kidney. Malignant rhabdoid tumors of the CNS often have an abnormality of chromosome 22. These tumors usually occur in children younger than 2 years.risk factor (... FAK-ter) Something that increases the chance of developing a disease. Some examples of risk factors for cancer are age, a family history of certain cancers, use of tobacco products, being exposed to radiation or certain chemicals, infection with certain viruses or bacteria, and certain genetic changes.sarcoma (sar-KOH-muh) A cancer of the bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue.screening (SKREE-ning) Checking for disease when there are no symptoms. Since screening may find diseases at an early stage, there may be a better chance of curing the disease. Examples of cancer screening tests are the mammogram (breast), colonoscopy (colon), and the Pap test and HPV test (cervix). Screening can also include checking for a person’s risk of developing an inherited disease by doing a genetic test.second primary cancer (SEH-kund PRY-mayr-ee KAN-ser) Refers to a new primary cancer in a person with a history of cancer.soft tissue (... TIH-shoo) Refers to muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.soft tissue sarcoma (…TIH-shoo sar-KOH-muh) A cancer that begins in the muscle, fat, fibrous tissue, blood vessels, or other supporting tissue of the body.sonogram (SAH-noh-gram) A computer picture of areas inside the body created by bouncing high-energy sound waves (ultrasound) off internal tissues or organs. Also called ultrasonogram.stage (stayj) The extent of a cancer in the body. Staging is usually based on the size of the tumor, whether lymph nodes contain cancer, and whether the cancer has spread from the original site to other parts of the body.standard therapy (... THAYR-uh-pee) Treatment that experts agree is appropriate, accepted, and widely used. Also called best practice, standard medical care, and standard of care.surgeon (SER-jun) A doctor who removes or repairs a part of the body by operating on the patient.surgery (SER-juh-ree) A procedure to remove or repair a part of the body or to find out whether disease is present. An operation.symptom (SIMP-tum) An indication that a person has a condition or disease. Some examples of symptoms are headache, fever, fatigue, nausea, vomiting, and pain.syndrome (SIN-drome) A set of symptoms or conditions that occur together and suggest the presence of a certain disease or an increased chance of developing the disease.synovial sarcoma (sih-NOH-vee-ul sar-KOH-muh) A malignant tumor that develops in the synovial membrane of the joints.tissue (TIH-shoo) A group or layer of cells that work together to perform a specific function.tuberous sclerosis (TOO-ber-us skleh-ROH-sis) A genetic disorder in which benign (not cancer) tumors form in the kidneys, brain, eyes, heart, lungs, and skin. This disease can cause seizures, mental disabilities, and different types of skin lesions.tumor (TOO-mer) An abnormal mass of tissue that results when cells divide more than they should or do not die when they should. Tumors may be benign (not cancer), or malignant (cancer). Also called neoplasm.ultrasound (UL-truh-SOWND) A procedure in which high-energy sound waves are bounced off internal tissues or organs and make echoes. The echo patterns are shown on the screen of an ultrasound machine, forming a picture of body tissues called a sonogram. Also called ultrasonography.ureter (YER-eh-ter) The tube that carries urine from the kidney to the bladder.urinalysis (YOOR-ih-NA-lih-sis) A test that determines the content of the urine.urine (YOOR-in) Fluid containing water and waste products. Urine is made by the kidneys, stored in the bladder, and leaves the body through the urethra.uterus (YOO-teh-rus) The small, hollow, pear-shaped organ in a woman's pelvis. This is the organ in which a fetus develops. Also called womb.vein (vayn) A blood vessel that carries blood to the heart from tissues and organs in the body.von Hippel-Lindau syndrome (von HIH-pul-LIN-dow SIN-drome) A rare inherited disorder in which blood vessels grow abnormally in the eyes, brain, spinal cord, adrenal glands, or other parts of the body. People with von Hippel-Lindau syndrome have a higher risk of developing some types of cancer. Also called VHL syndrome.WAGR syndrome (... SIN-drome) A rare, genetic disorder that is present at birth and has two or more of the following symptoms: Wilms tumor (a type of kidney cancer); little or no iris (the colored part of the eye); defects in the sexual organs and urinary tract (the organs that make urine and pass it from the body); and below average mental ability. This syndrome occurs when part of chromosome 11 is missing. Also called Wilms tumor-aniridia-genitourinary anomalies-mental retardation syndrome.white blood cell (hwite blud sel) A type of immune cell. Most white blood cells are made in the bone marrow and are found in the blood and lymph tissue. White blood cells help the body fight infections and other diseases. Granulocytes, monocytes, and lymphocytes are white blood cells. Also called leukocyte and WBC.Wilms tumor (wilmz TOO-mer) A disease in which malignant (cancer) cells are found in the kidney, and may spread to the lungs, liver, or nearby lymph nodes. Wilms tumor usually occurs in children younger than 5 years old.x-ray (EX-ray) A type of radiation used in the diagnosis and treatment of cancer and other diseases. In low doses, x-rays are used to diagnose diseases by making pictures of the inside of the body. In high doses, x-rays are used to treat cancer. |
