Paediatric Virilizing Ovarian Tumors: A Systematic Review

Virilizing ovarian tumors (VOT) are rare in the paediatric population. The literature regarding their clinical spectrum, hormonal profile, imaging characteristics, histology, and outcomes is limited. Here, we perform a systematic review on the characteristics of paediatric VOT.

A systematic review was conducted in accordance with PRISMA guidelines. Databases including PubMed and Google Scholar were searched to identify English-language reports of paediatric (< 20 years) VOT with biochemical evidence of hyperandrogenemia and histological confirmation of an ovarian neoplasm. Data on demographics, clinical features, hormone profiles, imaging, tumor histology, management and outcomes were extracted and analysed.

A total of 117 paediatric patients (median age: 15 years) with VOT were included. The most common symptoms were hirsutism (73.5%), clitoromegaly (68.4%), menstrual irregularity (n = 79), and voice change (60.7%). Children < 8 years had shorter diagnostic latency with less frequent hyperandrogenic manifestations. Baseline 17-hydroxyprogesterone (17-OHP) and dehydroepiandrosteroe sulfate (DHEAS) were elevated in 70% and 18.3% of patients, respectively. Median tumor size was 6.6 cm, whereas tumors were bilateral in eight patients. Juvenile granulosa cell tumors and Sertoli-Leydig cell tumors were most common in the < 8 years and > 13 years groups, respectively. Recurrence was rare over a median follow-up of 1 year.

Hyperandrogenic manifestations are less frequent in children < 8 years with VOT. Elevated 17-OHP and/or DHEAS should not exclude the possibility of VOT. Etiological spectrum of paediatric VOT is relatively age-specific. Larger multicenter studies with longer follow-up are warranted to better delineate prognosis and management strategies.