Endocrine Abstracts

Introduction: Adrenocortical carcinomas (ACCs) are rare, frequently aggressive tumors. Pure virilizing carcinomas are infrequent, constituting 5 to 10% in most series.

Clinical case: A 36-year-old female consults for 5 years evolution of amenorrhea associated with hirsutism attributed to polycistic ovary syndrome (PCOS) and treated with oral contraceptives during the previous 2 years. There was no other data on virilization, changes in body weight, hypertension or gastrointestinal symptoms. In the biochemical exam the following were observed: total testosterone 405 ng/dl (15–110), free testosterone 26.9 pmol/l (0.19–8.9), DHEA-sulphate 1000 μg/dl (35–430), FSH 2.9 mU/ml and LH 11.4 mU/ml. Basal and urinary cortisol, prolactin, beta-estradiol, 17-OH-progesterone, thyroid, hepatic and renal function, ions, were normal. The response to LHRH and ACTH and ovarian ultrasound were compatible with PCOS and ruled out late onset congenital adrenal hyperplasia. The imaging tests showed a solid adrenal mass in the left 10×13×14 cm, with prominent vascular structures inside it, which were embolized prior to surgical excision performed by bilateral subcostal laparotomy. The histological diagnosis of the tumor which weighed 1.152 kg and measured 20×12.5×8.5 cm, proved to be an adrenocortical tumor of low degree malignancy, without invasion of the capsule or adjacent tissues. Menstruation, hormonal tests and ovarian ultrasound became normal after surgery. The patient did not receive any therapy and the follow-up has not shown recurrence.

Discussion: Overall, survival is poor for ACC. Five-year survival is approximately 45–60% for early stage disease, and 10–25% for advanced stage disease. For patients with stage I to III disease, complete surgical resection as initial therapy is the only potentially curative treatment and open surgery remains the standard approach. Despite the large tumor size, the patient had a good prognosis based on the absence of invasion of the surrounding tissues, the histological grade of differentiation and the complete tumor resection. The interest of her case is due not only to the prolonged survival with an optimal quality of life, but also is a representative example of PCOS secondary to hyperandrogenism that disappears from the functional and morphological point of view after the suppression of its cause.