Sertoli-Leydig tumor of the ovary with an extensive heterologous intestinal mucinous component. Case report and a brief review of the literature

Sertoli-Leydig cell tumors (SLCTs) represent an uncommon group of ovarian sex cord-stromal neoplasms constituting less than 0.5 percent of all ovarian neoplasms. Heterologous elements, especially of intestinal type, are rare, and usually form small component. We present a diagnostically difficult case of an SLCT that has an exceptionally large intestinal heterologous component that resembles a mucinous epithelial neoplasm. A 67-year-old woman complained of progressive abdominal distension, pelvic pain, and virilization. A solid-cystic adnexal mass was detected by imaging. The laboratory workup revealed a high level of serum testosterone and normal tumor markers. The intraoperative frozen section was not conclusive, and the concern was a possible mucinous epithelial tumor. Conclusive histopathology identified a moderately differentiated SLCT with large (about 30 per cent) intestinal-type mucinous components. Positive reaction with Inhibin, calretinin, SF1, FOXL2, and CDX2 supported the diagnosis. The staging was initially FIGO IA, but after intraoperative communication clarified the possibility of a tumor spillage, the final staging was revised to IC1, which was later confirmed by positive peritoneal cytology. The patient received adjuvant platinum-based chemotherapy and was disease-free at 28 months. This case demonstrates that the presence of a large amount of heterologous mucinous intestinal component may cause diagnostic errors, especially with limited sampling or during frozen section intraoperative consultation. It highlights the importance of being cautious when making intraoperative diagnosis where the findings are uncertain to prevent any unneeded surgical procedures. Above all, it points out the significant role of multidisciplinary communication in the process of correct staging and efficient management of rare ovarian neoplasms.