Postmenopausal hyperandrogenism due to an ovarian sex cord-stromal tumour causing elevated dehydroepiandrosterone sulphate: a case report

Abstract

Background: The source of excess androgen can be obscure in postmenopausal women with new-onset hyperandrogenism. If serum dehydroepiandrosterone sulphate (DHEAS) is raised, it is presumed to be of adrenal origin because DHEAS is exclusively produced from adrenal cortical cells. This reports an elderly female presenting with new-onset hyperandrogenism due to an ovarian sex cord-stromal tumour, associated with increased serum DHEAS levels.

Case description: A 76-year-old female with long-standing diabetes and hypertension presented with hirsutism and male type alopecia for six months. She had menopause at 55 years of age. There was a pelvic mass on examination. Total testosterone was 6.106 ng/ml (0.124-0.357) and DHEAS was > 1000 µg/dL (35-430). Contrast-enhanced computed tomography of the abdomen and pelvis showed a heterogeneously enhancing complex mass measuring 11 × 8 cm in the left adnexal region. Adrenal glands were normal. She underwent total abdominal hysterectomy, bilateral salphingo-oophorectomy, and omentectomy. Both testosterone and DHEAS normalised following surgery. Histology revealed a sex cord-stromal tumour, likely a steroid cell tumour with malignant potential. Fluorodeoxyglucose-Positron emission tomography did not show any additional lesions.

Conclusions: Due to the lack of sulfotransferase in ovarian tissue, markedly elevated DHEAS originating from an ovarian neoplasm is unusual. This phenomenon has not been described except in a patient with a steroid cell tumour causing Cushing syndrome and hyperandrogenism. The mechanism of this rare occurrence remains elusive. Knowledge of this unusual presentation would enable the clinicians to be cautious in localising the androgen source in women with hyperandrogenism.

Keywords: DHEAS; Dehydroepiandrosterone sulphate; Hyperandrogenism; Ovary; Sex cord-stromal tumour.

Fig. 1

Contrast-enhanced CT of the abdomen and pelvis showing a heterogeneously enhancing complex mass arising from the left adnexal region. In the axial image (a), it measures 11 × 8 cm. There is no calcification, fat content, or air-fluid level within. In the coronal (b) and sagittal (c) images, it extends up to the level of the umbilicus abutting the anterior abdominal wall. Mass merges with uterus and bladder with no intervening fat planes. The mass displaces the small and large bowel

Fig. 2

Gross appearance of the surgical specimen. Yellowish left ovarian tumour measuring 16 cm × 8 cm × 9 cm with haemorrhagic and necrotic areas

Fig. 3

Microscopic appearance of the Haematoxylin and Eosin-stained surgical specimen in × 400 magnification. There are nests and sheets of polygonal cells with abundant eosinophilic cytoplasm and brisk mitotic activity. There is moderate nuclear pleomorphism. Occasional spindled cells are present

Fig. 4

Steps of androgen synthesis. Synthesis of dehydroepiandrosterone (DHEA), androstenedione, and testosterone are common to both adrenal and ovary. Conversion of DHEA to dehydroepiandrosterone sulphate (DHEAS) is specific to the adrenal cortex. Conversion of testosterone to dihydrotestosterone is seen in peripheral tissues. CYP11A—P450 side-chain cleavage enzyme, CYP17A1—17α hydroxylase, HSD3B2—3β hydroxysteroid dehydrogenase type 2, SULT2A1—sulfotransferase 2A1