Malignant Ovarian Steroid Cell Tumor Causing Severe Hyperandrogenism: Case Report And Review Of The Literature

ABSTRACT Objective: To report a rare case of malignant ovarian steroid cell tumor with androgen excess. Methods: We describe the clinical presentation and management of a malignant ovarian steroid tumor with severe hyperandrogenism in a postmenopausal woman. Results: An 83-year-old patient had a 6-year history of features of androgen excess. Biochemistry confirmed elevated testosterone and dehydroepiandrosterone sulfate; investigations identified a 4.5-cm adnexal mass that was resected, and the histopathology diagnosis was a malignant steroid cell tumor. Eight months later, disease recurrence prompted a second surgical intervention, and a 3.5-cm mass adherent to the right pelvic sidewall was removed. The patient was not able to tolerate mitotane due to severe side effects. Eighteen months later, the disease relapsed with recurrence of clinical and biochemical features of androgen excess. Conclusion: This case illustrates the challenges of diagnosis and management of malignant ovarian steroid cell tumors. Abbreviations: CT computed tomography; DHEAS dehydroepiandrosterone sulfate; GnRH gonadotropin-releasing hormone; MRI magnetic resonance imaging; SCT steroid cell tumor