The following guidelines are usually but not always followed in treating patients with Wilms tumor. Selection of treatment is based on the stage of the cancer and whether the histology (appearance of the Wilms tumor under the microscope) is favorable or unfavorable. In the United States, pediatric cancer specialists generally prefer that surgery is done before chemotherapy. In Europe, experts prefer to begin the chemotherapy before surgery. The results seem to be the same.

Stage I favorable or unfavorable appearance: Treatment begins with surgical removal of the tumor by radical nephrectomy. Children younger than 2 with small tumors (less than 550 grams) with favorable histology may have surgery without any radiation or chemotherapy. But they need to be watched closely because the chance the cancer will come back is slightly higher than if they had chemotherapy. But chemotherapy can cure most of the children who have recurrence.

For children older than 2 years and those of any age with larger tumors or unfavorable histology, surgery is followed by chemotherapy with actinomycin D and vincristine. The chemotherapy is given for 18 weeks.

Stage II favorable appearance: Surgery (radical nephrectomy) is followed by chemotherapy with actinomycin D and vincristine. The chemotherapy is given for 18 weeks.

Stage II unfavorable appearance (with anaplasia): Treatment begins with radical nephrectomy. When the child recovers, radiation therapy is given to the abdomen for 6 or 7 days. When this is finished, chemotherapy is given. A third drug, doxorubicin, is added to actinomycin D and vincristine, and the treatment is given for 6 months.

If the anaplasia is widespread throughout the tumor, these children are given more intense chemotherapy. The drugs used are vincristine, doxorubicin, etoposide, and cyclophosphamide along with MESNA (a drug that prevents bladder irritation from the cyclophosphamide). They are given for 6 months.

Stage III favorable appearance or unfavorable appearance with focal (only a little) anaplasia: Radical nephrectomy is followed by radiation therapy to the abdomen. This is followed by chemotherapy with 3 drugs (actinomycin D, vincristine, and doxorubicin) for 6 months.

Stage III unfavorable appearance with diffuse (widespread throughout the tumor) anaplasia: Treatment begins with radical nephrectomy followed by radiation therapy to the abdomen. The drugs usually given are vincristine, doxorubicin, etoposide, and cyclophosphamide along with MESNA (a drug that prevents bladder irritation from the cyclophosphamide). The chemotherapy lasts 6 months.

In rare instances the tumor may be so large that it cannot be removed safely. In these cases a small tissue sample is taken from the tumor for testing to be sure that it is a Wilms tumor and then chemotherapy is started. If there is no response or inadequate response to chemotherapy, then radiation therapy is given to shrink the tumor. Usually the tumor will shrink within 6 weeks so that a radical nephrectomy can be done. Chemotherapy will be resumed after surgery.

Stage IV favorable or unfavorable appearance with focal (only a little) anaplasia: Radical nephrectomy is followed by radiation therapy to the abdomen. This is followed by chemotherapy with 3 drugs (actinomycin D, vincristine, and doxorubicin) for 6 months.

Stage IV unfavorable appearance with diffuse (widespread throughout the tumor) anaplasia: Treatment begins with radical nephrectomy followed by radiation therapy to the abdomen. The drugs usually given are vincristine, doxorubicin, etoposide, and cyclophosphamide along with MESNA (a drug that prevents bladder irritation from the cyclophosphamide). The chemotherapy lasts 6 months.

Stage V: Treatment for children with bilateral tumors is individualized. Your surgeon will remove only as much tumor as is necessary to spare the kidneys. Chemotherapy and radiotherapy are also used. Usually biopsies (tissue samples) of tumors of both kidneys and of lymph nodes are taken, and chemotherapy (vincristine, doxorubicin, and actinomycin D) is given. After chemotherapy, a second operation may be done to see if any tumor remains. If the cancer is still present, treatment may include more chemotherapy, radiation therapy, and/or surgery to remove tumors but not the entire kidney. If one or both tumors have anaplasia, chemotherapy will usually be more aggressive (vincristine, doxorubicin, etoposide, cyclophosphamide, and MESNA).

Recurrent Wilms tumor: The prognosis and treatment for children with Wilms tumor that recurs (comes back after treatment) depends on their prior treatment and the cancer's histology (favorable or unfavorable). The outlook is better for children with favorable histology tumors who were previously treated with vincristine and actinomycin D but not given abdominal radiation therapy. The usual treatment for these children is surgery to remove recurrent cancer, radiation therapy, and more chemotherapy (vincristine, doxorubicin, and actinomycin D).

The outlook is generally poor for recurrent Wilms tumor with any of the following features:

• unfavorable histology
• initial treatment that included radiation therapy
• initial chemotherapy that included doxorubicin
• recurrence within 6 months of nephrectomy
• failure of the tumor to completely disappear with the above treatment

Treatment for these children usually involves aggressive chemotherapy, often including new drugs being studied in clinical trials. Very high-dose chemotherapy followed by bone marrow transplantation is an option in this situation.

Parents and the stress of watching your child�s treatment for cancer. Most parents will find watching their child go through cancer treatment stressful. Sometimes this will lead to a minor form of what is called post traumatic stress disorder or PTSD. This can lead to irritability and outbursts of anger. It may also affect your child�s treatment, because parents with this problem will try to avoid the thing that is causing the problem, namely your child�s treatment. If you think you may be experiencing PTSD, talk to your child�s doctor and ask for help in dealing with the problem.

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EditText of this page (last edited December 29, 2009)