Ovarian Sertoli-leydig Cell Tumor, Retiform Variant with Heterologous Elements in a Pediatric Case
Mohamed Ali Kamil *
Anatomic Pathology Laboratory, Ibn Sina University Hospital Center of Rabat, Morocco and Mohammed V University, Faculty of Medicine and Pharmacy of Rabat, Morocco.
Abdillahi Moustapha Osman
Anatomic Pathology Laboratory, Ibn Sina University Hospital Center of Rabat, Morocco and Pediatric Hematology and Oncology Department, Children's Hospital of Rabat, Morocco.
Maachi Malak
Anatomic Pathology Laboratory, Ibn Sina University Hospital Center of Rabat, Morocco and Mohammed V University, Faculty of Medicine and Pharmacy of Rabat, Morocco.
Alaoui Driss
Anatomic Pathology Laboratory, Ibn Sina University Hospital Center of Rabat, Morocco and Mohammed V University, Faculty of Medicine and Pharmacy of Rabat, Morocco.
Hssissen Laila
Anatomic Pathology Laboratory, Ibn Sina University Hospital Center of Rabat, Morocco and Pediatric Hematology and Oncology Department, Children's Hospital of Rabat, Morocco.
Rouas Lamia
Anatomic Pathology Laboratory, Ibn Sina University Hospital Center of Rabat, Morocco and Mohammed V University, Faculty of Medicine and Pharmacy of Rabat, Morocco.
*Author to whom correspondence should be addressed.
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.
Keywords: Sertoli-Leydig tumor, ovary, child, retiform variant, heterologous elements, DICER1