Long-term survival in an adolescent with widely metastatic renal cell carcinoma with rhabdoid features

Renal cell carcinoma is rarely seen in children and adolescents. Patients w ith widespread disease at diagnosis have a particularly poor survival rate. Currently, all known chemotherapy has been ineffective in improving the me dian survival in patients with advanced disease. A 13-year-old black boy wi th stage IV renal cell carcinoma with rhabdoid features is a long-term dise ase-free survivor after aggressive multiagent chemotherapy. After the initi al evaluation and histologic diagnosis of renal cell carcinoma, the patient received three courses of an aggressive chemotherapy regimen consisting of vincristine, doxorubicin, cyclophosphamide with mesna uroprotection, granu locyte colony-stimulating factor and erythropoietin (Epogen). After an almo st complete response, a radical nephrectomy was performed and results demon strated a solitary small nodule with viable tumor. After surgery, he receiv ed floxuridine infusion for 14 days by circadian schedule at 28-day interva ls for a total of 1 year. The patient is well and free of disease 5 years a fter initial presentation. The dramatic response to treatment and long-term disease-free survival of this patient suggest this chemotherapeutic approa ch warrants additional investigation.