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Wilms’ Tumor

Wilms’ Tumor is a disease in which cancer (malignant) cells are found in certain parts of the kidney. The kidneys are a “Matched” pair of organs found on either side of the backbone. They are shaped like a kidney bean. Inside each kidney are tiny tubes that filter and clean the blood, taking out unneeded products, and making urine. The urine produced in the kidneys passes through a tube called a ureter into the bladder where it is held until it is passed out of the body through the urethra.

Wilms’ tumor is curable in the majority of affected children. If your child has symptoms, his/her doctor will usually feel your child’s abdomen for lumps and run blood and urine tests. The doctor may order a special X–ray called an intravenous pyelogram. During this test, a dye containing iodine is injected into your child’s bloodstream. This allows your child’s doctor to see the kidney more clearly on the X–ray. Your child’s doctor may also do an ultrasound, which uses sound waves to make a picture, or a special X–ray called a computed tomographic scan to look for lumps in the kidney. A special scan called magnetic resonance imaging (MRI), which uses magnetic waves to make a picture, may also be done. Chest and bone X–rays may also be taken.

If tissue that is not normal is found, your child’s doctor will need to cut out a small piece and look at it under the microscope to see if there are any cancer cells. This is called a biopsy.

Your child’s chances of recovery (prognosis) and choice of treatment depend on the stage of your child’s cancer (whether it is just in the kidney or has spread to other places in the body), how the cancer cells look under a microscope (histology), tumor size, and your child’s age and general health.

Stage explanation
Stages of Wilms’ tumor
Once Wilms’ tumor has been found, more tests will be done to find out if cancer cells have spread from the kidney to other parts of the body. This is called staging. Your child’s doctor needs to know the stage of the disease to plan treatment. The following stages are used for Wilms’ tumor:
Stage I
Cancer is found only in the kidney and can be completely removed by surgery.
Stage II
Cancer has spread to the areas near the kidney, such as fat or soft tissue, to blood vessels, or to the renal sinus (a large part of the kidney through which blood and fluid enter and exit the kidney). The cancer can be completely removed by surgery.
Stage III
Cancer has spread to areas near the kidney, but cannot be completely removed by surgery. The cancer may have spread to important blood vessels or organs near the kidney or the cancer may have spread throughout the abdomen, so that the doctor cannot remove all the cancer during surgery. The cancer may also have spread to the lymph nodes (small bean–shaped structures found throughout the body that produce and store infection–fighting cells) near the kidney.
Stage IV
Cancer has spread to organs further away from the kidney, such as the lungs, liver, bone, and brain.
Stage V
Cancer cells are found in both kidneys.

Recurrent
Recurrent disease means that the cancer has come back (recurred) after it has been treated. It may come back where it started or in another part of the body.

In Wilms’ tumor, how the cancer cells look under a microscope (histology) is also very important. The cancer cells can be of favorable histology or unfavorable histology (which includes diffuse anaplastic and clear cell sarcoma of the kidney).

Treatment of Wilms’ tumor
There are methods of treatment for all patients with Wilms’ tumor. Three kinds of treatment are used:

Surgery (taking out the cancer in an operation). Chemotherapy (using drugs to kill cancer cells). Radiation therapy (using high–dose X–rays or other high–energy X–rays to kill cancer cells).

Surgery is a common treatment for Wilms’ tumor. Your doctor may take out the cancer using one of the following:
Partial nephrectomy removes the cancer and part of the kidney around the cancer. This operation is usually used only in special cases, such as when the other kidney is damaged or has already been removed.

Simple nephrectomy removes the whole kidney. The kidney on the other side of the body can take over filtering blood.
Radical nephrectomy removes the whole kidney with the tissues around it. Some lymph nodes in the area may also be removed.
Chemotherapy uses drugs to kill cancer cells. Chemotherapy may be taken by pill, or it may be put into the body by a needle in a vein or muscle.
Chemotherapy is called a systemic treatment because the drugs enter the bloodstream, travel through the body, and can kill cancer cells throughout the body. Chemotherapy given after an operation when there are no cancer cells that can be seen is called adjuvant therapy.

When very high doses of chemotherapy are used to kill cancer cells, these high doses can destroy the blood–forming tissue in the bones (the bone marrow). If very high doses of chemotherapy are needed to treat the cancer, bone marrow may be taken from the bones before therapy and frozen until it is needed.
Following chemotherapy, the bone marrow is given back through a needle in a vein. This is called autologous bone marrow reinfusion.
Radiation therapy uses X–rays or other high–energy rays to kill cancer cells and shrink tumors. Radiation for Wilms’ tumor usually comes from a machine outside the body (external radiation therapy). Radiation may be used before or after surgery and/or chemotherapy. After several years, some patients develop another form of cancer as a result of their treatment with chemotherapy and radiation. Clinical trials are on to determine if lower doses of chemotherapy and radiation can be used.

Treatment for Wilms’ tumor depends on the stage of your child’s disease, the cell type (histology), and your child’s age and general health.
Your child may receive treatment that is considered standard based on its effectiveness in a number of patients in past studies, or you may choose to have your child take part in a clinical trial. Not all patients are cured with standard therapy and some standard methods of treatments may have more side effects than are desired.

Stage I Wilms’ tumor
Your child’s treatment depends on the histology of the cancer.

If your child has a favorable histology tumor or if he/she has anaplastic Wilms’ tumor, your child’s treatment will probably be surgery to remove the cancer followed by chemotherapy. If your child has either clear cell sarcoma of the kidney or rhabdoid tumor, his/her treatment will probably be surgery to remove the cancer followed by radiation therapy plus chemotherapy.

Stage II Wilms’ tumor
Your child’s treatment depends on the histology of the cancer.
If your child has a favorable histology tumor, your child’s treatment will probably be surgery to remove the cancer followed by chemotherapy. If your child has an unfavorable histology tumor (anaplasia, clear cell sarcoma, or rhabdoid tumor of the kidney), your child’s treatment will probably be surgery to remove the cancer followed by radiation therapy plus chemotherapy.

Stage III Wilms’ Tumor
If your child has a favorable or an unfavorable histology tumor (anaplasia, clear cell sarcoma, or rhabdoid tumor), your child’s treatment will probably be surgery to remove the cancer followed by radiation therapy plus chemotherapy. Sometimes the cancer cannot be removed during surgery because it is too close to important organs or blood vessels, or because it is too large to remove. In this case, the doctor may only perform a biopsy and then give chemotherapy with or without radiation therapy. Once the cancer has become smaller, surgery can be performed, followed by additional chemotherapy and radiation therapy.

Stage IV Wilms’ tumor
If your child has a favorable or an unfavorable histology tumor (anaplasia, clear cell sarcoma, or rhabdoid tumor), your child’s treatment will probably be surgery, to remove the cancer followed by radiation therapy with chemotherapy. Patients whose cancer has metastasized to the lungs will receive additional chemotherapy.

Stage V Wilms’ tumor
Since both kidneys contain cancer, it is not usually possible to remove both kidneys. Your child’s doctor will probably take out a piece of the cancer in both kidneys and remove some lymph nodes around the kidney to see whether they contain cancer. Following surgery, chemotherapy is given to shrink the cancer. A second operation is then performed to remove as much of the cancer as possible, while leaving as much of the kidneys as possible. Surgery may be followed by more chemotherapy and/or radiation therapy.

Recurrent Wilms’ tumor
If your child’s cancer comes back (recurs), treatment depends on what kind of treatment he/she had received before, how much time has passed since the first cancer was treated, the histology of the cancer, and where it came back.

Depending on the above factors, your child’s treatment may be surgery to remove the cancer, plus radiation therapy and chemotherapy. Clinical trials are evaluating new forms of treatment, such as new chemotherapy drug combinations, and very high doses of chemotherapy followed by bone marrow reinfusion.

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