Conservative management of primary vaginal endodermal sinus tumor and rhabdomyosarcoma.

Weimin Xie,Mei Yu,Yao Wang,Keng Shen,Jiaxin Yang,Dongyan Cao

doi:10.18632/oncotarget.18829

Abstract

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.

Highlights

Primary vaginal malignancies are rare tumors of the female genital system, accounting for approximately 0.3% of all invasive cancers among women, and 1%–2% of all gynecologic malignant neoplasms [1]
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

Summary

Introduction

Primary vaginal malignancies are rare tumors of the female genital system, accounting for approximately 0.3% of all invasive cancers among women, and 1%–2% of all gynecologic malignant neoplasms [1]. Rhabdomyosarcoma (RMS) is the most common vaginal malignancy in childhood, followed by malignant germ cell tumors (MGCTs) and clear cell adenocarcinoma [3,4]. RMS is the most common soft-tissue sarcoma in childhood, which usually occurs in the head and neck, followed by the genitourinary tract [5]. The embryonal subtype can be subdivided to classic, botryoid, and spindle cell variants [6, 7]. Among these subtypes, botryoid RMS mainly occurs in the vagina during infancy and early childhood (mean age, 3 years) [8,9]. Vaginal MGCTs are relatively rare tumors; endodermal sinus tumor (EST) is the most common histological subtype that occurs primarily in infants [10]

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