title: Comparison of outcomes based on treatment algorithms for rhabdomyosarcoma of the bladder/prostate: combined results from the Children's Oncology Group, German Cooperative Soft Tissue Sarcoma Study, Italian Cooperative Group, and International Society of Pediatric Oncology Malignant Mesenchymal Tumors Committee.
abstract: The purpose of this study was to determine patient characteristics and outcomes for bladder/prostate (BP) rhabdomyosarcoma (RMS) using an international cohort of prospectively treated patients comparing different treatment algorithms. Data were collected from 379 patients (1979-1998) treated on protocol; Intergroup Rhabdomyosarcoma Study, IRS-IV (n = 239 patients), International Society of Pediatric Oncology Malignant Mesenchymal Tumors (MMT) Committee MMT-84 and -89 (n = 74), Italian Cooperative Group, RMS-79 and RMS-88 Studies (n = 37) or German Cooperative Soft Tissue Sarcoma Study CWS-91 protocols (n = 29). A total of 322 (85%) patients had localized embryonal RMS (ERMS) and 27 had metastatic disease. Thirty patients (21 local disease; 9 metastatic) had nonembryonal BP RMS. Patients with localized ERMS had large tumors (64% >5 cm) that were invasive (54%) with uninvolved regional lymph nodes (N0, 93%). The 5-year failure-free survival (FFS) was 75% and the overall survival (OS) was 84%, with 89% of deaths attributed to disease. Treatment failures were usually local disease recurrence (60%). Predictors of FFS included T-stage (invasiveness), size, and histology. FFS was decreased for patients not receiving initial radiotherapy but this did not translate into a decreased OS. The 21 patients with localized nonembryonal BP RMS had a FFS and OS of 47%. The 36 patients with metastatic disease were more likely to be older and had large tumors that were invasive with alveolar histology and regional lymph node involvement. The 5-year FFS and OS were 41 and 44%, respectively. In conclusion, the majority of BP RMS patients had localized ERMS with a resultant good prognosis using current treatment algorithms. There were differences in FFS between treatment protocols but this did not result in an altered OS.
authors.affiliation: Department of Pediatric Surgery, Children's Hospital of Pittsburgh, University of Pittsburgh, Pittsburgh, PA, USA. rodebergd@ecu.edu
authors.forename: David A
authors.initials: DA
authors.lastname: Rodeberg
authors.forename: James R
authors.initials: JR
authors.lastname: Anderson
authors.forename: Carola A
authors.initials: CA
authors.lastname: Arndt
authors.forename: Fernando A
authors.initials: FA
authors.lastname: Ferrer
authors.forename: Richard Beverly
authors.initials: RB
authors.lastname: Raney
authors.forename: Meriel E
authors.initials: ME
authors.lastname: Jenney
authors.forename: Ines B
authors.initials: IB
authors.lastname: Brecht
authors.forename: Ewa
authors.initials: E
authors.lastname: Koscielniak
authors.forename: Modesto
authors.initials: M
authors.lastname: Carli
authors.forename: Gianni
authors.initials: G
authors.lastname: Bisogno
authors.forename: Odile
authors.initials: O
authors.lastname: Oberlin
authors.forename: Annie
authors.initials: A
authors.lastname: Rey
authors.forename: Fred
authors.initials: F
authors.lastname: Ullrich
authors.forename: Michael C G
authors.initials: MC
authors.lastname: Stevens
authors.forename: William H
authors.initials: WH
authors.lastname: Meyer
mesh_terms: D000465:Algorithms; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D009362:Neoplasm Metastasis; D011471:Prostatic Neoplasms / Q000473:pathology / Q000628:therapy*; D012208:Rhabdomyosarcoma / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome; D001749:Urinary Bladder Neoplasms / Q000473:pathology / Q000628:therapy*