Gynecologic Malignancies in Children and Adolescents: How Common is the Uncommon?

Abstract

The aim of this study is to assess the projected incidence and prognostic indicators of gynecologic malignancies in the pediatric population. In this population-based retrospective cohort study, girls ≤18 years with ovarian, uterine, cervical, vaginal and vulvar malignancies diagnosed between 2000 and 2016 were identified from the Surveillance, Epidemiology and End Results (SEER)-18 registry. The Kaplan-Meier method was used to analyze overall survival (OS). The age-adjusted annual incidence of gynecologic malignancies was 6.7 per 1,000,000 females, with neoplasms of the ovary accounting for 87.5%, vagina 4.5%, cervix 3.9%, uterus 2.5% and vulva 1.6% of all gynecologic malignancies. Malignant germ-cell tumors represented the most common ovarian neoplasm, with an increased incidence in children from 5-18 years. Although certain subtypes were associated with advanced disease stages, the 10-year OS rate was 96.0%. Sarcomas accounted for the majority of vaginal, cervical, uterine and vulvar malignancies. The majority of vaginal neoplasms were observed in girls between 0-4 years, and the 10-year OS rate was 86.1%. Overall, gynecologic malignancies accounted for 4.2% of all malignancies in girls aged 0-18 years and the histologic subtypes and prognosis differed significantly from patients in older age groups.

Keywords: adolescent; female; gynecologic cancer; gynecologic malignancies; pediatric; pediatric cancer.

Figure 1

Distribution of gynecologic malignancies according to histology.

Figure 2

(A–C) Annual incidence of gynecologic malignancies by age from 0–18 years and distribution of disease stage at diagnosis. Abbreviations: n.a., not applicable. (A) The graph illustrates the annual incidence of ovarian, vaginal, cervical, uterine and vulvar cancer by age groups from 0–18 years per 1,000,000 women. (B) Distribution of disease stage of ovarian, vaginal, cervical, uterine and vulvar cancer in children and adolescents from 0–18 years of age. (C) Distribution of disease stage in ovarian malignancies by underlying histologic subtype in the same cohort. Abbreviations: G.C., germ-cell.

Figure 3

(A–F) Kaplan–Meier plots for 5- and 10-year overall survival for children and adolescents with gynecologic malignancies. (C) The survival curves of germ-cell tumor and choriocarcinoma are overlapping. (E) The survival curves of adenocarcinomas and germ-cell tumors are overlapping. Abbreviations: n.s., not specified; OS, overall survival.