Results of treatment of fifty‐six patients with localized retroperitoneal and pelvic rhabdomyosarcoma: A report from The Intergroup Rhabdomyosarcoma Study‐IV, 1991–1997

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Abstract
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 ≥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 P = 0.02].Conclusions: These results are superior to those of previous protocols for patients with RMS of the retroperitoneum/pelvis. Initial excision of ≥50% of the tumor may be associated with increased FFS.

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