Malignant Extrarenal Rhabdoid Tumor of the Bladder: 9-Year Survival After Chemotherapy and Partial Cystectomy

Abstract

A 4-year-old black female presented with persistent painless gross hematuria in December 1994. Excretory urography demonstrated normal upper tracts and a large filling defect in the bladder. Computerized tomography (CT) showed a 3 5 cm mass in the anterosuperior wall of the bladder (fig. 1). Cystoscopy revealed a large lobulated tumor arising from the anterior bladder wall. Bladder biopsies demonstrated a poorly differentiated neoplasm in the submucosa and muscularis propria, composed of polygonal cells with ovoid nuclei and prominent nucleoli. Many of the cells contained eosinophilic perinuclear inclusions typical of rhabdoid tumors. Immunoperoxidase staining was positive for vimentin and negative for cytokeratin, S-100, desmin, muscle specific actin and myoglobin. No cytogenetic studies were done. Malignant rhabdoid tumor was diagnosed. Metastatic evaluation including bone marrow aspirate was negative. Neoadjuvant chemotherapy was planned with the hope of preserving the native bladder. No protocols were followed. The patient underwent 4 courses of neoadjuvant chemotherapy with ifosfamide, vincristine and actinomycin D. The tumor shrank dramatically on CT (fig. 2). Postchemotherapy cystoscopy showed a significantly reduced tumor.