Bladder and prostatic tumors in the intergroup rhabdomyosarcoma study (IRS-I). Results of therapy

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Abstract
Sixty-four children with primary rhabdomyosarcoma of the bladder or prostate were enrolled in the IRS during the initial five years (October 1972—November 1977). Observation of these patients for from 2 1/2–8 years forms the basis of this report. The chemotherapy-radiotherapy regimens employed (IRS) for each Clinical Group were: (1) Group I (completely excised tumor), sequential actinomycin-D (ACD), vincristine (VCN), and cyclophosphamide (CYP), i.e., standard (VAC), with or without radiotherapy (RT); (2) Group II (resected local disease with node involvement, “microscopic” residual, or local extension), RT plus sequential ACD and VCN, or RT plus standard VAC; (3) Group III (gross residual disease); and (4) Group IV (dissemination), RT and either pulse VAC or pulse VAC plus Adriamycin (ADR). Pulse VAC consisted of VCN (day 1), plus daily intravenous ACD and CYP (days 1–5). Relapse rates were: Group I, 0/8; Group II, 6/23; and the mortality in Group III, 6/23; and in Group IV, 7/10. Among patients with bladder tumors, the rate of relapse was 2/11 following pelvic exenteration (anterior, 10; total 1); 5/12 following partial cystectomy or gross tumor excision; and 3/5 following a primary chemotherapy-radiotherapy (PCR) regimen. In patients with prostatic tumors (Groups I—III), relapse occurred in 0/14 patients treated initially by pelvic exenteration (anterior, 12; total, 2); and in 2/11 patients treated by a PCR regimen.