Malignant rhabdoid tumor of the extremity

Two cases of malignant rhabdoid tumor (MRT) involving the sciatic nerve are described. Despite the close affiliation of the tumor with neural tissue, staining characteristics in these patients do not suggest a neural origin of MRT. Survival in patients with renal and extrarenal MRT has been poor. Our patients were treated with an aggressive chemotherapy program using cisplatin, Adriamycin (doxorubicin), vincristine, cyclophosphamide, actinomycin D, and DTIC. One child died of progressive disease; the other is well 35 months after diagnosis.