Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a rare aggressive endocrine neoplasm. It’s a heterogeneous malignant tumor with incompletely understood pathogenesis and poor prognosis. The aim of our study was to assess clinical and paraclinical characteristics of ACC and to determine its outcome.

Methods: In a retrospective and descriptive study, we included patients with adrenocortical carcinoma confirmed with histopathological examination. Clinical, biological, hormonal, radiological and therapeutic data were collected.

Results: We included four women and a man with an adrenocortical carcinoma. Their median age was 45.8 years [extremes: 31–60]. Their past medical history included diabetes mellitus, intellectual disability and ovarian teratoma in one case, respectively. Three patients presented with abdominal pain and were referred to our department for the exploration of an adrenal mass. One patient was admitted for clinical signs of Cushing’s syndrome and the fifth patient presented with weight loss, asthenia and anorexia. On physical examination, two patients had android obesity, hirsutism, high blood pressure, muscle atrophy, skin atrophy, purple striae and facial plethora. Routine blood tests revealed dyslipidemia and diabetes in two cases and severe hypokalemia at 2.4 mmol/l in one case. Three patients had an active hormone secreting tumor with a Cushing syndrome. Cortisol levels after standard Dexamethasone suppression test were 3.5, 12.6 and 33.5 µg/dl, respectively. Among these three patients, one had mixed hormone secretion of cortisol and androgen. Two patients had hormone-inactive tumor. Adrenal computed tomography (CT) scan revealed a right tumor in four patients and a left tumor in one patient. The tumor had heterogeneous enhancement with central intratumoral calcifications in all the cases. Its diameter varied between 5 and 11 cm. A focal extension into the inferior vena cava, liver and kidney was present in one patient. Surgical treatment was performed to four patients. Only one patient had received mitotane. For the other patient, this drug was not available. Two patients died 6 and 48 months after the surgery, respectively. Two patients showed no signs of recurrence in their last CT scan and one patient was lost to follow up.

Conclusion: ACC is a rare and aggressive endocrine neoplasm that may present when hormonally active or after they have become large. The study of the clinical features could conclude to the type of the secretion. Surgical resection represents the treatment of choice in localized ACC. Mitotane, alone or in combination with other agents, remains the most effective chemotherapy.