MULTIMODAL THERAPY FOR STAGE IV ADULT WILMS TUMOR

Abstract

A 34-year-old Native American, with no significant medical history, presented with a 2 to 3 month history of persistent right low back pain, 10 lb. weight loss, and acute nausea and vomiting during a 2-week period. Abdominal ultrasonography revealed a large, predominantly solid, heterogeneous mass near the lower pole of an enlarged right kidney. Computerized tomography (CT) revealed a 15 cm. mass arising from the superior pole of the right kidney, multiple lesions in the liver and both lungs, with significant pleural effusions (fig. 1, A). Positron emission tomography verified fluorodeoxyglucose uptake by these lesions in the liver and lungs, consistent with metastatic lesions. Additional staging included a bone scan, brain magnetic resonance imaging, testicular ultrasound, tumor markers and urinalysis, which were all normal. CT guided core biopsy of the renal mass was performed. Histopathological analysis revealed malignant small blue cells, consistent with Wilms tumor of a favorable histology (fig. 2). Pathological diagnosis was reviewed and independently confirmed by the director of the National Wilms’ Tumor Study pathology section. The patient was treated preoperatively according to a 48week pediatric high risk Wilms tumor protocol, which consisted of 5 cycles of multiagent chemotherapy (cyclophosphamide, etoposide, doxorubicin, vincristine and dactinomycin). Right radical nephrectomy was performed with the placement of a right hepatic artery perfusion catheter (Arrow pump*). Mediastinum and lungs were subjected to 2,000 cGy. radiation. A month after resection, systemic chemotherapy with vincristine, dactinomycin, cyclophosphamide and etoposide, and intrahepatic therapy (via the Arrow pump) with doxorubicin was resumed. Subsequently, the patient received 2 cycles of continuous intrahepatic infusion of doxorubicin, and a course of systemic 200 mg./m. 2 paclitaxel. Followup CT at 6 months after resection showed resolution of lung metastases, stable liver lesions and no residual disease in the renal bed (fig. 1, B). DISCUSSION