Ovarian Sertoli-leydig Cell Tumor, Retiform Variant with Heterologous Elements in a Pediatric Case

Abstract

Ovarian Sertoli-Leydig cell tumors (SLTs) are rare neoplasms, representing less than 0.5% of ovarian tumors and 1 to 2% of pediatric ovarian tumors. We report the case of a 15-year-old adolescent girl with retiform variant SLT with heterologous elements, characterized by a solid, cystic abdominopelvic mass, associated with menometrorrhagia and dysuria, without signs of virilization. Histological examination revealed a mixed proliferation of Sertoli and Leydig cells, with the presence of heterologous elements of hepatoid appearance and a retiform component. Immunohistochemistry confirmed the diagnosis by the expression of calretinin, inhibin, CD99, and WT-1 in Sertoli cells, and Melan-A in Leydig cells. Treatment consisted of a right adnexectomy, without the use of adjuvant chemotherapy. This case illustrates the morphological and immunophenotypic diversity of pediatric SLTs, the importance of differential diagnosis with other ovarian tumors, as well as the need for multidisciplinary management and long-term monitoring, particularly due to the risk of associated DICER1 syndrome.