Endocrine Abstracts

Introduction: ACC is a rare tumour with a incidence of 0.7–2.0/per million, more common in women between 40 and 50 years. Can be diagnosis as a incidentaloma or for clinic of abdominal pain and autonomous o hormone secretion symptoms (Cushing syndrome and hirsutism). We present the management of a particular case of hirsutism for ACC.

Case report: Sixty four year old woman referring increase hair and a deeper voice for 1 year. As personal antecedents include hypertension (treated with four drugs) and kidney stones. On physical examination she presents BMI 31 kg/m², blood pressure 157/87 mmHG, Ferriman Galway WITH 14 points, and no signs of hypercortisolism. In the hormonal profile highlights: DHEA 8.01 μg/ml, androstenedione >10 ng/ml, testosterone 3.83 ng/ml, cortisol after 1 mg of dexamethasone 12.2 μg/dl, urinary cortisol 34.2 μg/24 h, 17-OH progesterone 8.28 ng/ml and 11 dexosicortisol 27 ng/ml. Catecholamines, metanephrines and renin/aldosterone axis were normal. Body CT with contrast shows a heterogeneous mass adrenal of 8 cm maximun diameter with a density of 75 H, without lymphadenopathy or metastasis. The patient is operated by laparotomy performing right adrenalectomy with complete resection. Corticosteroid replacement downward is performed. The pathology reaches four criteria Weiss (nuclear grade III, multifocal necrosis, venous invasion muscular wall and capsular invasion). Ki 67 (+) was 10–15%. It is diagnosed of ACC completely respected stage II. The patient refuses adjuvant treatment with mitotane and decided closely monitored. At one year follow hormonal markers were normal and CT hadn’t show signs of recurrence. Gradually returned hirsutism and hypertension is controlled with dual therapy.

Conclusions: The ACC is a malignant and aggressive tumor with poor prognosis especially in advanced. Complete resection is the best guarantee of cure. Mitotane is the first line drug as adjuvant therapy, but its role is unclear when complete resection can be performed. In our case it was decided to closely monitor whitin signs of recurrence at 1 year. Finally, more studies are needed to improve and clarify the role of adjuvant therapy in ACC.