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Wilms Tumor and Other Childhood Kidney Tumors Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment of Wilms Tumor

Table 3. Overview of Wilms Tumor Standard Treatment by Stage continued...

Focal Anaplastic

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this trial, patients will be treated with the UH-1 regimen and radiation therapy.

Diffuse Anaplastic (No Measurable Disease)

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this trial, patients will be treated with the UH-1 regimen and radiation therapy.

Diffuse Anaplastic (Measurable Disease)

  • COG-AREN0321 (Combination Chemotherapy, Radiation Therapy, and/or Surgery in Treating Patients With High-Risk Kidney Tumors): In this trial, patients will be treated with window therapy consisting of vincristine and irinotecan for 12 weeks. If they respond to the window therapy, they will receive therapy consisting of UH-2 (cyclophosphamide, carboplatin, and etoposide; vincristine, doxorubicin, and cyclophosphamide; vincristine, irinotecan, and radiation therapy) for 30 weeks. Patients not responding to the window therapy would then be treated on UH-1 and radiation therapy.

Stage V and those predisposed to developing Wilms tumor

The following treatment option is currently under investigation in COG clinical trials. Information about ongoing clinical trials is available from the NCI Web site. Patients with multicentric tumors, patients with high-risk bilateral tumors, and patients with diffuse hyperplastic nephrogenic rests are treated on the following protocol:

COG-AREN0534 (Combination Chemotherapy and Surgery in Treating Young Patients With Wilms Tumor): Children with bilateral tumors are eligible for COG-AREN0534, which is the first protocol to prospectively study bilateral tumors. The goals of therapy are to eradicate all tumor and to preserve as much normal renal tissue as possible with the hope of decreasing the risk of chronic renal failure among these children.[25,26] When children are identified with bilateral tumors by CT or magnetic resonance imaging, central radiologic review will be performed to exclude tumor extension, invasion, rupture, metastases, or thrombus. Central review will also assess characteristics of nephrogenic rests versus tumor and differentiate active from sclerotic rests or tumors. Biopsy will not be mandated. Upfront intensification with three drugs (vincristine, doxorubicin and dactinomycin), will be used in large part to move patients earlier to definitive surgery. Repeat imaging will be mandated at 6 weeks. Based on response to treatment surgery, biopsy or continued chemotherapy will be performed. If biopsy or surgery is performed, chemotherapy or radiation therapy will be given based on histology. Repeat imaging will be performed at 12 weeks. If there is a complete response, definitive surgery or continued therapy will be performed. This approach will identify patients with anaplasia, rhabdomyomatous differentiation, complete necrosis, or stromal differentiation, select them for early surgery, and define the intensity of chemotherapy to be administered.[16,18,27] Chemotherapy and/or radiation therapy following the second-look operation is dependent on the response to initial therapy, with more aggressive therapy required for patients with inadequate response to initial therapy observed at the second procedure.[17,18,19,20,21,22]

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