Ovarian hilus cell hyperplasia as the cause of virilization in 45,X Turner's syndrome

Abstract

Objective: To describe a rare case of 45,X Turner's syndrome with elevated T and clitoromegaly caused by ovarian hilus cell hyperplasia.

Design: Case report.

Setting: Tertiary care pediatric hospital.

Patient(s): An 11-and-a-half-year-old girl with 45,X karyotype showed signs of accelerated growth and clitoromegaly and was found to have elevated serum T. Fluorescence in situ hybridization was used to confirm her karyotype as monosomy X and absence of the SRY gene. Elevated gonadotropins indicated absence of ovarian function.

Intervention(s): Laparoscopic bilateral gonadectomy.

Main outcome measure(s): Serum T level.

Result(s): Pathology showed ovarian hilus cell hyperplasia within the removed gonads. Serum T levels returned to normal after gonadectomy.

Conclusion(s): Ovarian hilus cell hyperplasia is an extremely rare cause of virilization in Turner's syndrome. Gonadectomy should be considered for patients with confirmed 45,X karyotype who exhibit virilization.