SAT-415 - Rapid Onset Hyperandrogenism in a Premenopausal Woman: A Case of Co-Secreting Adrenal Adenoma

Hyperandrogenism is characterized by the presence of excessive levels of androgenic steroids and occurs in about 10% of premenopausal women. Polycystic Ovarian Syndrome (PCOS) contributes to 80% of all causes of hyperandrogenism, whereas adrenal and other ovarian causes account for the remaining 20%. While mild hyperandrogenism is a frequent condition mostly caused by PCOS, severe hyperandrogenism accompanied by virilization or clitoromegaly is less frequent and warrants evaluation for androgen secreting tumors.

We report a case of a 27-year-old healthy woman who presented with acute onset of acne, hirsutism and weight gain. Clitoromegaly was noted on physical exam. Laboratory evaluation revealed elevated total testosterone (446 ng/dL) and dehydroepiandrosterone sulfate (DHEA-S) (1120 ug/dL). Abdominal CT (computed tomography) demonstrated a 3.5 x 2.7 cm right adrenal mass with a precontrast Hounsfield Unit (HU) value of 35. Pelvic ultrasound showed no ovarian abnormalities. She was started on spironolactone with minimal improvement in hirsutism. Further evaluation revealed normal fractionated free plasma metanephrine levels and ACTH independent cortisol excess with a morning serum cortisol (29.7 ug/dL) and adrenocorticotropic hormone level ( < 5.0pg/mL). She underwent two 1-mg dexamethasone suppression cortisol tests with cortisol level of 13.2 ug/dL and 15.7 ug/dL, and appropriate dexamethasone level. The patient underwent right laparoscopic adrenalectomy. Pathology revealed a benign adrenocortical adenoma with 2.8cm greatest dimension. Postoperatively, the patient developed adrenal insufficiency, requiring hydrocortisone supplementation. Spironolactone was discontinued. At 3 weeks follow up, there was significant improvement in her symptoms with drastic decrease in levels of testosterone (6.04 ng/dL) and DHEA-S ( < 10 ug/dL).

Androgen and cortisol overproduction are the most common biochemical manifestations found in adrenocortical carcinoma (ACC). Moreover, radiologically, the detection of a precontrast HU value exceeding 20 further increases the suspicion of malignancy. Thus, the combination of androgen and cortisol overproduction along with a high precontrast HU value raises a high level of suspicion for ACC.

As far as we know, this is the second case of the benign adrenal adenoma associated with the co-secretion of androgens and cortisol. This case involves a very pronounced degree of hyperandrogenism and rapid progression of virilization, which could easily suggest a malignant condition. However, all these potential risk factors notwithstanding, the histopathology report showed that this was a benign tumor. In addition, the rapid remission of symptoms after surgical intervention also proves that it is important to consider other diagnostic variants despite the clear presence of ACC symptoms.

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