Malignant transformation of a testosterone-secreting ovarian steroid cell tumor: a case report

Background: Ovarian Steroid Cell Tumors (SCT) are rare sex cord-stromal tumors accounting for less than 0.1% of ovarian neoplasms. Most are hormonally active and present with symptoms of androgen excess. Steroid cell tumor, not otherwise specified (SCT-NOS), represents the majority of cases and carries the greatest malignant potential, although most tumors are benign and cured with surgical excision. Malignant transformation of an initially benign SCT-NOS is rarely reported, and the molecular mechanisms underlying progression remain poorly characterized. Case: . These alterations involve pathways regulating DNA damage response, RAS/MAPK signaling, and cellular growth. Conclusion: This case presents a rare ovarian steroid cell tumor, not otherwise specified, initially managed with unilateral salpingo-oophorectomy and surveillance given its benign pathologic features. Three years later, the patient developed malignant recurrence. Despite complete surgical cytoreduction and administration of platinum-based adjuvant chemotherapy, the patient experienced disease progression. This case highlights the potential for malignant transformation in benign-appearing SCT-NOS and underscores the importance of counseling patients on the rare risk of recurrence and the need for novel therapeutic strategies in recurrent disease. Integration of clinical, pathologic, and molecular data may improve risk stratification and management of this rare tumor.