Hirsutism as the initial presentation of malignant ovarian Leydig cell tumor: A case report

Ovarian steroid cell tumors are a rare subtype of sex-cord stromal cell tumors. Overall, these tumors make <0.1% of all ovarian tumors. These neoplasms can be divided according to the cell of origin into stromal luteomas, Leydig cell tumors, and steroid cell tumors not otherwise specified. These tumors can be benign, malignant, or borderline, with variable presentation. We report a case of 24-year-old virgin female who was referred to our hospital after being diagnosed with steroid cell tumor-not otherwise specified. Prior to her admission, the patient had been treated unsuccessfully with oral contraceptive pills due to male-pattern facial hair growth, abdominal cramps, and irregular menstrual cycle. Lack of improvement warranted further investigations. Hormonal studies showed an elevated total testosterone, dehydroepiandrosterone sulfate, and morning fasting cortisol. Ultrasonography and computed tomography confirmed the presence of a large pelvic mass with mixed solid and cystic component. Therefore, unilateral salpingo-oophorectomy was performed. Pathological and immunohistochemical examination suggested the presence of a large ovarian steroid cell tumor-not otherwise specified with malignant behavior. The patient did not receive adjuvant therapy and developed metastatic disease. She received four cycles of BEP protocol with no improvement, so she was referred to our center to continue oncological management. Case revision confirmed the presence of steroid cell tumor, but of a different subtype: Leydig cell. She received six cycles of carboplatin-paclitaxel, but her assessment showed disease progression. We report this case with review of literature regarding the appropriate approach to these rare tumors. Although rare, ovarian steroid cell tumors should be included in the differential diagnosis of virilization in young females, especially those refractory to hormonal therapy. In our study, we aimed to present the first reported Palestinian case, which highlights the importance of detailed morphological examination in addition to the difficulties encountered to reach a proper diagnosis. We also provided a review of the existing literature regarding chemotherapeutic lines used in such cases and the response to each.