Conservative management of primary vaginal endodermal sinus tumor and rhabdomyosarcoma

摘要

The aim of this study was to evaluate the conservative management and prognosis of primary vaginal endodermal sinus tumor and rhabdomyosarcoma in children. Medical records of children with vaginal endodermal sinus tumor and rhabdomyosarcoma between 1996 and 2015 were reviewed. A total of 24 patients (median age, 12 months; range, 7–44 months) were included in this study, comprising 17 patients with endodermal sinus tumor and 7 patients with rhabdomyosarcoma. Among the 17 patients with endodermal sinus tumor, 15 were initially treated at our hospital with chemotherapy alone, and 2 were initially treated in other hospitals with conservative surgery and chemotherapy. All 7 patients with botryoid rhabdomyosarcoma received chemotherapy without well-defined protocols. At a median follow-up of 51 months (range, 4–237 months), 3 patients (12.5%; 1 with endodermal sinus tumor and 2 with rhabdomyosarcoma) developed recurrence. At the last follow-up, 22 patients (91.7%) were alive without evidence of disease, 1 patient with botryoid rhabdomyosarcoma died of disease progression, and 1 patient with endodermal sinus tumor died of respiratory and circulatory failure. To allow preservation of sexual and reproductive function, conservative therapeutic strategies should be considered for children with vaginal endodermal sinus tumor and botryoid rhabdomyosarcoma.