Testosterone-Secreting Ovarian Tumor: A Rare Cause of Erythrocytosis and Pulmonary Embolus

OBJECTIVE: To raise awareness of the clinical presentation of a testosterone-secreting steroid cell tumor, not otherwise specified, causing pulmonary embolus (PE) and erythrocytosis. METHODS: Report of the first known case of ovarian steroid cell tumor not otherwise specified leading to PE. RESULTS: A 67-year-old Caucasian postmenopausal woman was referred for endocrine evaluation of a 1-year history of hirsutism, weight gain, and elevated total testosterone level of 672 ng/dL (normal, <75 ng/dL). She reported increased hair growth on her chin for the past year, unintentional weight gain, and low energy levels. Laboratory data from the initial visit included a total testosterone level of 672 ng/dL (normal, <75 ng/dL), hemoglobin level of 18.0 g/dL (normal, 11.7-15 g/dL), and hematocrit level of 50.4% (normal, 35%-45%). Four months after initial presentation, the patient developed acute-onset chest pain and shortness of breath and was diagnosed with a right PE on computed tomography chest angiogram. Evaluation with imaging for an ovarian mass revealed a negative workup including computed tomography abdomen pelvis, transvaginal ultrasound, and pelvic magnetic resonance imaging. Despite negative findings during imaging, because of the markedly elevated testosterone levels, this presentation was thought to correspond to a testosterone-secreting ovarian tumor. The patient was referred for bilateral oophorectomy. Pathology of the right ovary revealed a 2-cm steroid cell tumor, not otherwise specified. CONCLUSIONS: PE and erythrocytosis can be presentations of a testosterone-secreting ovarian tumor, not otherwise specified. This case is the first known presentation of an ovarian steroid cell tumor, not otherwise specified, leading to PE and erythrocytosis.