MON-913 Testosterone Secreting Ovarian Tumor: A Rare Cause of Erythrocytosis and Pulmonary Embolus

Abstract

Background: Ovarian testosterone producing tumors are an uncommon cause of hirsutism, pulmonary embolus and polycythemia.Clinical Case: A 67-year-old Caucasian postmenopausal woman was referred for Endocrine evaluation of a one-year history of hirsutism, weight gain and elevated total testosterone level of 672 ng/dL (n <75 ng/dL). She reported increased hair growth on her chin for the past year. She denied any male pattern hair loss or any increased hair growth on her legs or chest. She also reported unintentional weight gain of thirteen pounds and low energy levels. Past medical history is remarkable for gastroesophageal reflux disease. Her last menstrual period was at age forty and periods occurred at regular intervals without heavy bleeding. She never became pregnant by choice. At this time physical exam was notable for shaved terminal hair on her chin, vellus hair on abdomen, there was no hirsutism or voice change noted. There were no abdominal striae, moon facies, buffalo hump.Labs from the initial visit included a total testosterone of 672 ng/dL (n<75 ng/dL), 2pm cortisol 5.6 ug/dL (2.7-10.5 ug/dL in PM), TSH 2.28 uiU/mL (0.27 – 4.20), Androstenedione 133 ng/dL (n<10-93 ng/dL), 11 deoxycortisol 29.3ng/dL (n <32), ACTH 12 pg/mL (n<46), DHEAS 87 ug/dL (13-130 ug/dL) free testosterone index 24.2 (n<2.1), 17-hydroxyprogesterone 207ng/dL (n<272), FSH 26.1 miU/mL (25.8-134.8), LH 17.9 IU/L (7.7-58.5), Hemoglobin 18. g/dL (11.7-15g/dL), hematocrit 50.4% (35-45%), HbA1c 5.4%.Four months after this initial presentation, the patient developed acute onset of chest pain and shortness of breath and was diagnosed with a right pulmonary embolus on CT Chest Angiogram. Evaluation with imaging for an ovarian mass revealed a negative workup. CT Abdomen Pelvis did not reveal any adrenal masses. Transvaginal Ultrasound did not demonstrate any ovarian masses. MRI of pelvis did not show any adnexal masses.Despite the negative imaging, because of the markedly elevated testosterone levels, this presentation was thought to correspond to a testosterone secreting ovarian tumor. Furthermore, the normal adrenal hormone levels and significantly elevated testosterone level is consistent with neoplasm range. High levels of testosterone are known to cause a hypercoagulable state leading to a PE. Furthermore, testosterone causes an increase in RBC mass leading to marked elevations in hemoglobin. The patient was referred for bilateral oophorectomy. Pathology of the right ovary revealed a 2 cm steroid cell tumor, not otherwise specified.Conclusion: This is the first known case of a steroid cell ovarian tumor, not otherwise specified, that secretes testosterone leading to pulmonary embolism and erythrocytosis. Furthermore, testosterone secreting tumors should be considered in the diagnosis of PE as a rare cause of hypercoagulable state.