A rare case of testosterone-secreting ovarian tumor

A 49-year-old parous postmenopausal woman presented with increasing hair growth on her chin and neck two years after menopause. 

She was under treatment for hypertension, diabetes, and hypothyroidism.

The lady’s BMI was 27 kg/m², and Ferriman-Gallwey's score was 20. A vaginal examination revealed a 5 x 4 cm right adnexal mass. 

Ultrasound and MRI showed a uterus with mild myometrial heterogenous echotexture and a solid-cystic right adnexal lesion with thin septations measuring 5.4 x 4.6 x 4.5 cm. Laboratory results included CA125 at 15.8 U/L (0.0-35 U/L), total testosterone at 256 ng/ml (8.4-48.1 ng/ml), free testosterone at 7.36 pg/ml (0.2-4.1 pg/ml), DHEAS at 234 µg/dl (35.4-256 µg/dl), LDH at 172 U/L (135-214 U/L), AFP at 1.67 ng/ml (<7 ng/ml), CEA at 0.96 ng/ml (0.0-4.7 ng/ml), inhibin B at 25.22 pg/ml (5-33 pg/ml), and normal serum cortisol levels. A breast ultrasound did not reveal abnormalities.

Given the suspected hormone-secreting tumor, she was scheduled for exploratory laparotomy and total abdominal hysterectomy with right oophorectomy.

Intraoperatively, the left ovary and bilateral tubes were absent due to prior family planning surgery. In cytology, minimal ascitic fluid was found to be negative for malignant cells. The right ovary was enlarged and cystic, measuring 5 x 6 cm, with an intact capsule. 

Frozen section analysis indicated a benign sex cord-stromal tumor. Histopathological examination revealed a steroid cell tumor in the right ovary, classified as “Not Otherwise Specified” (NOS), with no nuclear atypia or mitotic activity, indicating a benign nature. Examination of the uterus and endometrium revealed adenomyosis. Immunohistochemistry showed immunoreactivity for inhibin, calretinin, and Melan-A, with patchy reactivity for vimentin and no reactivity for Pan CK and PAX8.

The findings supported the diagnosis of an ovarian steroid cell tumor (NOS). Postoperative recovery was unremarkable, and the patient’s serum total testosterone levels decreased to 15.60 ng/dl within three months post-surgery.

In this case, serum testosterone levels normalized six weeks after surgery. Ovarian steroid cell tumors are rare, making early detection challenging. This case underscores the importance of vigilance in assessing unusual hormonal symptoms, such as hirsutism. Clinicians should suspect these tumors in atypical hormonal presentations. Timely diagnosis, surgical intervention, and appropriate postoperative monitoring can lead to symptom resolution and a favorable outcome in these rare cases.

Source: Kaur H, Singh N, Bharti S, et al. Autops Case Rep. 2024;14:e2024478.