Florid hyperandrogenism due to a benign adrenocortical adenoma

Abstract

A 26-year-old woman with a history of polycystic ovarian syndrome presented with secondary amenorrhea, worsening hirsutism, acne, deepening of voice and unexplained 10-20 kg weight gain. Her Ferriman-Gallway hirsutism score was 12 with cystic facial acne and increased masculine phenotype. Urine Beta-Human Chorionic Gonadotropins (bHCG) was negative. She had elevated serum testosterone of 551 ng/dL, androstenedione at 7.46 ng/mL and dehydroepiandrosterone sulfate (DHEAS) at 4243 µg/L. Overnight dexamethasone suppression test showed mildly unsuppressed cortisol (2.89 µg/dL). Urinary free cortisol along with paired serum cortisol and adrenocorticotrophic hormone (ACTH) tests were normal (55.4 µg/24 hours, 13.44 mcg/dL, 30.4 pg/mL respectively). Her leutinizing hormone (LH) was low(<0.1 mIU/mL), follicle stimulating hormone (FSH) low/normal (1.41 mIU/mL) with sex hormone binding globulin (SHBG) level 45nmol/L and the rest of the pituitary and adrenal workup was unremarkable. Thyroid stimulating hormone (TSH) was 2.15mU/mL. MRI revealed a 3.1 cm, indeterminate but well-defined left adrenal lesion and polycystic ovaries without abdominal lymphadenopathy. Given radiological appearances and despite biochemical concerns for adrenocortical malignancy, a multidisciplinary team meeting decision was made to proceed with laparoscopic adrenalectomy. Histology was consistent with a benign adenoma. Postoperatively, there was clinical and biochemical resolution of the disease.

Keywords: adrenal disorders; metabolic disorders.

Figure 1

Acne before adrenalectomy.

Figure 2

MRI scan of the adrenals. Well-defined, ovoid left adrenal lesion identified inferiorly at the lateral limb measuring 3.1×2.6×1.8 cm. This was hypointense on T1 with mild signal loss on out of phase images. On T2 images, the lesion had intermediate to slightly high signal with mild internal heterogeneity. Postcontrast images showed prominent enhancement with no major wash out on delayed images. Overall, the adrenal adenoma was characterised as indeterminate.

Figure 3

Pathology specimen. 13 g adrenal gland with well circumscribed 3 cm tumour.

Figure 4

Pathology report. The tumour is composed of sheets of eosinophilic and clear cells (50:50) without any nuclear atypia or necrosis and mitoses <1/10HPF. Ki-67=6% and Weiss score 0.

Figure 5

Almost complete resolution of acne 1 month after adrenalectomy.