ABSTRACT
Ovarian Sertoli-Leydig cell tumors (SLCTs) are extremely rare ovarian sex-cord stromal tumors. Alpha-fetoprotein (AFP) production by SLCTs is a rare event generally linked to the presence of hepatocytes or intestinal mucinous epithelium as heterologous elements. We report here a case of a 15-year-old female complaining about abdominal pain, constipation, and spaniomenorrhea with high level of serum AFP leading to a clinical suspicion of malignant germ cell tumor. Final histopathological diagnosis was a moderately differentiated Sertoli-Leydig cell tumor of the ovary with alpha-fetoprotein-producing cells without hepatocytic or intestinal epithelium differentiation. NGS analysis showed mutation in DICER1 gene. SLCTs occur in patients at any age with a mean age of 25 years. The presence of alpha-fetoprotein-producing cells is an important tool in the differential diagnosis of germ cell tumors and challenging in this case of SLCT because of its rarity in this context. An adequate sampling and exhaustive immunohistochemical analyses are mandatory to make the correct differential diagnosis and confirm the presence of alpha-fetoprotein-producing cells and also define the differentiation because of therapeutic strategies between conservative surgery and/or chemotherapy.