Retiform pattern of Sertoli-Leydig cell tumor of the ovary in a 4-year-old girl

Abstract

Sertoli-Leydig cell tumors are rare sex cord-stromal neoplasms that account for <0.2% of ovarian tumors. These tumors with a retiform pattern pose difficult diagnostic problems, with the majority being misinterpreted as serous papillary cystadenocarcinoma and endodermal sinus tumor. Retiform pattern Sertoli-Leydig cell tumor seen mostly in young patients is clinically malignant and has a worse prognosis, underlining the importance of recognition of this histopathologic entity. In the literature only two such cases have been described in patients under the age of 5 years; reported herein is the third case. Described here is a rare case of ovarian Sertoli-Leydig cell tumor with retiform pattern in a 4-year-old child without any endocrine manifestations.