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When Wilms tumor is suspected based on presenting symptoms of the patient, a surgical biopsy is needed to confirm the diagnosis. This involves a tissue sample of the tumor, which is sent away to pathologist who evaluates the histology of the cells and checks for signs of cancer.
Based of the diagnostic results from these tests, people with Wilms tumor should be given timely treatment to avoid the progression of the disease. The specific treatment plan differs for each individual according to the type of disease and level of progression, but most management plans involve surgery, chemotherapy and radiotherapy.
If the tumor is localized to the kidney, as is the case for the vast majority of patients with Wilms tumor, the initial treatment involves nephrectomy with the removal of the entire kidney and the contained tumor.
If there are multiple tumors affecting both kidneys, also known as bilateral disease, a section of the kidney will be surgically removed. This is to ensure some of the kidney function remains intact and the renal system of the patient will continue to work unassisted following successful treatment of Wilms tumor.
Nearly every patient with Wilm’s tumor will require some form of adjuvant chemotherapy as a part of their treatment plan; the only main exception is patients with a stage 1 tumor with favorable histology.
The most common chemotherapy regimens for Wilms tumor are:
The choice of which chemotherapeutic agents to use depends on the severity of the tumor and the prognosis. More aggressive treatments, such as regimen I, are reserved for patients with more severe disease, as it is associated with greater side effects.
As Wilms tumor is usually localized to the abdominal area, radiotherapy can offer localized treatment that is able to specifically target the tumor cells. Radiotherapy is a common option in the treatment of Wilms disease, usually following surgical nephrectomy and chemotherapy.
The stage of cancer is an important factor in the decision of which type of treatment should be administered. For a tumor with favorable histology (without anaplasia) the standard treatment is:
Children with Wilms tumor with anaplasia generally have a poorer prognosis than those with favorable histology and, as a result, the treatment usually differs considerably.
Those with Stage I anaplastic tumors have the best prognosis and can be managed in the same way as that of favorable histology, with nephrectomy and chemotherapy regimen EE-4A for 18 weeks.