Testosterone-Secreting Endometrioid Ovarian Carcinoma Presenting With Hyperandrogenism

Epithelial cell ovarian carcinomas rarely secrete steroid hormones, while sex cord and stromal cell ovarian carcinomas often do so. The objective of this report is to describe a patient with endometrioid ovarian carcinoma, an epithelial cell tumor, who presented with hyperandrogenism due to testosterone production by the tumor.

A 67-year-old postmenopausal woman with no history of endometriosis presented with new onset of hirsutism. Her testosterone level was 282 ng/dL (8-60 ng/dL), estradiol level was 72 pg/mL (≤32.2 pg/mL), and 17-hydroxyprogesterone level was 592 ng/dL (≤45 ng/dL). Pelvic ultrasound showed a right adnexal mass measuring 14.7 × 9.7 × 12.3 cm and an endometrial thickness of 9 mm with calcifications within the endometrium. Human epididymis protein 4 level was 210 pmol/L (0-140 pmol/L), and cancer antigen 125 level was 144 U/mL (0-34 U/mL). The patient underwent exploratory laparotomy with removal of the pelvic mass. Pathology showed an endometrioid adenocarcinoma with positive immunohistochemistry staining for the following steroidogenic enzymes: side-chain cleavage enzyme, 17α-hydroxylase, and aromatase. There was no evidence of tumor metastases within the pelvic cavity. Ovarian tumor markers normalized and remained stable 1 year after surgery.

Although endometrioid ovarian carcinomas do not typically produce clinically significant levels of sex steroids, in rare cases, these tumors can do so, leading to symptoms and promoting early detection and treatment of the cancer.

Sex hormone secretion by epithelial cell ovarian carcinomas should be considered in cases of new-onset steroid hormone excess in postmenopausal women.