A 12-year-old male was referred to the Children's Hospital of Philadelphia on September 13, 1982, after a large abdominal mass was discovered. He had had several days of difficulty voiding, followed by the appearance of microscopic hematuria. On admission, he appeared well, and was without discomfort. The physical examination was unremarkable except for a blood pressure of 120/90 mmHg and the presence of a large, firm abdominal mass. It filled the pelvis and lower abdomen and reached the level of the umbilicus. The tumor measured 16 cm. in height and 15 cm. in width, and was easily palpable on rectal examination, anterior to the rectum and extending down to the perineum. There was no ascites. Serum creatinine was 1.2 mg/dl, and serum electrolytes and uric acid were unremarkable. Intravenous urography showed a large mass, which appeared to arise in the pelvis, probably in the prostate, and which elevated the bladder. The left ureter was obstructed. Two days after admission, cystoscopy was performed. The bladder and urethra were displaced anteriorly, but there was no mucosal abnormality. A perineal needle biopsy was performed and a diagnosis of undifferentiated small round cell malignant tumor was made. A percutaneous left nephrostomy was performed just after the needle biopsy in order to promote ludney drainage. There was no evidence of metastases in the chest, bones, or bone marrow.
Because of its location, the tumor was thought to be compatible with a sarcoma arising in the prostate or other pelvic soft tissues. Accordingly, the patient was given an intensive chemotherapy regimen of vincristine, adriamycin, cyclophosphamide, and actinomycin D, all administered intravenously in accordance with the Intergroup Rhabdomyosarcoma Study Regimen 26 [ 11. In addition, cisplatinum was added at a dose of 90 mg/m2 at weeks 0, 3 , 6 , and 9, as part of a pilot program (called Regimen 29) for patients with advanced disease. A tube nephrogram 10 days later showed good drainage from the left ureter, and the nephrostomy tube was then removed.