Abstract
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. 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. 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]." 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.
