A Virilizing Malignant Steroid Cell Tumor-Leydig Cell Subtype in a 24-Year-Old Palestinian Female with Challenging Histopathological and Biochemical Features: A Case Report

Abstract

Background: Ovarian steroid cell tumors are a rare subtype of sex-cord stromal cell tumors. Overall, these tumors make less than 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 or malignant, with variable presentation. Case presentation: We report a case of 24-year-old virgin Palestinian 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. The patient did not improve. Ultrasonography demonstrated a large abdominal mass measuring 13x14 cm. Computed tomography confirmed the presence of a large pelvic mass with mixed solid and cystic component, displacing bowel loops peripherally. Hormonal studies showed an elevated dehydroepiandrosterone sulfate (DHEA-S) and morning fasting cortisol. 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 4 cycles of BEP 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 6 cycles of carboplatin-paclitaxel, albeit inconsistently, her assessment showed disease progression. The patient was referred back to her local hospital to continue management there. Conclusion: 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.