Results of treatment of fifty-six patients with localized retroperitoneal and pelvic rhabdomyosarcoma: a report from The Intergroup Rhabdomyosarcoma Study-IV, 1991-1997.
BACKGROUND
We reviewed 56 IRS-IV patients with localized rhabdomyosarcoma [RMS] of the retroperitoneum/pelvis to assess outcome and prognostic factors, including the value of initially excising >or=50% of the tumor (debulking) before chemotherapy.


METHODS
Patients had embryonal RMS [N=38], alveolar RMS [N = 7], RMS not otherwise specified [NOS, N = 7], or undifferentiated sarcoma [N = 4]. Fifteen patients were debulked; 41 patients were biopsied. All received VAC; most received radiotherapy.


RESULTS
Estimated 5-year failure-free survival [FFS] and overall survival rates were 70 and 75%, respectively. FFS rates were better for patients <10 years old and those with embryonal RMS compared to alveolar RMS/undifferentiated sarcoma. After adjusting for age and histological differences, FFS was better for patients whose tumor was debulked prior to beginning therapy [P = 0.02].


CONCLUSIONS
These results are superior to those of previous protocols for patients with RMS of the retroperitoneum/pelvis. Initial excision of >or=50% of the tumor may be associated with increased FFS.
Raney
R Beverly
RB
Department of Clinical Pediatrics, University of Texas M. D. Anderson Cancer Center, Houston, Texas 77030, USA. Braney@mdanderson.org
Stoner
Julie A
JA
Walterhouse
David O
DO
Andrassy
Richard J
RJ
Donaldson
Sarah S
SS
Laurie
Fran
F
Meyer
William H
WH
Qualman
Stephen J
SJ
Crist
William M
WM
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D008297:Male; D010386:Pelvic Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D011379:Prognosis; D012186:Retroperitoneal Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D018232:Rhabdomyosarcoma, Alveolar / Q000401:mortality / Q000628:therapy; D018233:Rhabdomyosarcoma, Embryonal / Q000401:mortality / Q000628:therapy; D012509:Sarcoma / Q000401:mortality / Q000628:therapy; D015996:Survival Rate; D016896:Treatment Outcome