CONSERVATIVE MANAGEMENT OF UTERINE PEDIATRIC RHABDOMYOSARCOMA - A REPORT FROM THE INTERGROUP RHABDOMYOSARCOMA STUDY-III AND STUDY-IV PILOT

Previous studies have suggested that women with uterine rhabdomyosarco mas (RMS) represent a distinct group of patients who present at an old er age, are less responsive to treatment, and have a poorer prognosis than patients with vaginal RMS. During the intergroup Rhabdomyosarcoma Study (IRS) III arid the IRS IV pilot study, 14 patients were registe red with uterine primary RMS. Three patients presented with cervical t umors that were completely removed (group I). Eight patients had initi al biopsies with gross residual disease (group 3), and 3 had metastati c disease at presentation (group 4). Of the 5 patients treated with pr imary chemotherapy or chemotherapy and radiation, 2 had delayed hyster ectomy and vaginectomy, I had no further surgery, and 2 had explorator y laparotomy with no evidence of disease. There were no relapses or de aths in this group. One patient underwent initial resection of a broad ligament mass, experienced an early (3-week) recurrence of the mass w hile on chemotherapy, and progressed to developing distant metastases and death. Four patients died of chemotherapy toxicity or sepsis, one after achieving a complete response from chemotherapy and hysterectomy . This primary chemotherapy or chemotherapy and radiotherapy regimen r esulted in 8 of 9 (89%) patients (not including those who died of chem otoxicity) surviving between 1.5 and 6 years without evidence of disea se. Of the surviving patients, 2 had hysterectomy and vaginectomy, but pathological specimens showed only localized microscopic residual tum or. This report suggests that less vigorous operative resection may be possible in combination with primary chemotherapy when treating uteri ne rhabdomyosarcomas. However, evaluation of the excellent response to chemotherapy must include consideration of the 4 patients who died of sepsis or treatment complications. Copyright (C) 1995 by W.S. Saunder s Company.