Sarcoma botryoides of the female genital tract

Results of treating 14 patients with sarcoma botryoides of the female genital tract are reviewed. Nine patients were younger than four years old and five were older than 14. Primary tumors were in the vagina (eight), cervix (three), vulva (one), and cervicovaginal region (two). All but one patient underwent surgery, including wide local excision (one), vaginectomy (one), hysterectomy (one), hysterectomy and vaginectomy (two), anterior exenteration (two), and total pelvic exenteration (six). A combination of vincristine, actinomycin-D, and cyclophosphamide was the chemotherapy regimen most frequently administered. Only one of the nine patients receiving chemotherapy died from recurrence. One patient with disease too extensive for surgery received intraarterial vincristine and radiation therapy; 16 years later she developed an adenosquamous carcinoma of the uterus. Sarcoma recurred in three patients. This review of patients treated between 1956 and 1983 reflects the evolution of therapy over 30 years. Conservative surgery alone was inadequate; therefore, radical (exenterative) surgery was adopted; recently less extensive surgery has been combined with chemotherapy, producing satisfactory results.