Androgen-secreting adenoma – a possibility in hyperandrogenism differential diagnosis

Abstract

Abstract Introduction Hirsutism affects almost 10% of women of reproductive age. The two most common causes of hirsutism are polycystic ovary syndrome (PCOS) and idiopathic hirsutism. Virilizing adrenocortical tumors are rare, nonetheless, it is a differential diagnosis that physicians must consider. Clinical Case We present the case of a 23-year-old woman that was referred from the Dermatology Department due to the abrupt and progressive appearance of hirsutism and facial acne. Her menstrual cycles were regular. She had a BMI of 21.7 kg/m2, facial acne, and a Ferriman-Gallwey scale of 11 points. She did not have hypertension, nor alopecia, clitoris hypertrophy or any other virilizing sign. Table 1 summarizes the analytical determinations requested in the early follicular phase of the menstrual cycle. Pelvic ultrasound scan did not revealed abnormalities. Computed tomography (CT) scan of the abdomen revealed a well-delimited and solid mass of 4.9 × 3.8 cm in the right adrenal gland, with an average density of 48 UH with enhancement on the portal phase without the presence of fat, hemorrhage or calcifications suggesting malignancy more likely (Figure 1). After multidisciplinary assessment, laparoscopic right adrenalectomy was advised. Pathology revealed an adrenal cortex adenoma which showed cytoplasmic immunoreactivity of the cells against inhibin and Melan-A with a KI-67 of 1%, negative for anti-p53 without atypical mitotic figures, necrosis, venous invasion, sinusoidal invasion nor capsular invasion. The post-surgical evolution was satisfactory with an early normalization of androgens and resolution of the virilizing signs. The most common cause of hirsutism in premenopausal women is PCOS; another cause, and far from the first, is the non-classical form of congenital adrenal hyperplasia; and even more uncommon, androgen-secreting ovarian and adrenal tumours (1,2). Adrenal tumours, unlike ovarian, usually secrete DHEAS. Most tumours are carcinomas, which commonly also secrete cortisol (3). A very high DHEAS level (>7 µg/mL) is suggestive of adrenal carcinoma. Pure virilizing adrenal tumours are extremely rare (4). Independently from hormone secretion, the suspicion of malignancy depends on radiological CT characteristics of the tumour: size (>4–6 cm), density (>10 HU with slow contrast washout), homogeneity, and shape (5). Magnetic resonance may assess local invasion (6). Adrenalectomy is the treatment of choice when virilizing adrenal tumours with malignant characteristics are found (7). In the surgical piece, it is useful to determine the Ki 67 (carcinomas: >5%) (8). Somatic mutations in the TP53 gene are associated with aggressive phenotypes(9). Conclusion Malignant ovarian and adrenal pathology should be ruled out in the scenario of new-onset hirsutism. Adrenal malignancy is usually related with tumor size, type of hormones secreted, and the velocity of the tumor progression. Exceptionally, benign adrenal tumours are producers of virilizing hormones.