Embryonal rhabdomyosarcoma of the uterine corpus and cervix

Abstract

Embryonal rhabdomyosarcomas of the uterus and cervix are rare tumors with a very poor prognosis historically. Adjuvant multidrug chemotherapy combined with local radiation therapy and/or surgery has resulted in markedly improved survival rates. Six patients with embryonal rhabdomyosarcoma of the uterus/cervix are reported, with a compilation of 28 additional patients from the literature. Nineteen of 24 patients (79%) treated with combination therapy were alive and well 4–147 + months (median 61 months) after diagnosis. The six cases from USC Medical Center and affiliated hospitals are all well 17–144 months (mean 95 months) after diagnosis. Analysis of the collected series indicates that extent of disease is an important prognostic factor. Adjuvant chemotherapy with vincristine, actinomycin D, and cyclophosphamide (VAC) improves survival. Initial therapy for embryonal rhabdomyosarcoma confined to the uterus/cervix should include radical hysterectomy, pelvic lymphadenectomy, and adjuvant chemotherapy with VAC. Pelvic radiotherapy should be employed for involved surgical margins or positive nodes. Advanced disease managed initially with chemotherapy and radiotherapy may allow subsequent curative extirpative surgery.