Endocrine Abstracts

Introduction: Adrenocortical carcinoma (ACC) is a rare neoplasm with a variable but overall poor prognosis. The presentation is heterogeneous usually with mass effect and less often with evidence of hormonal secretion. About half of cases the tumors are hormonally active most commonly with cortisol secretion followed by sexual hormones production, being rare the aldosterone secretion.

Case report: A 41-year-old male presented with hypertensive crisis and marked hypokalemia. A primary hyperaldosteronism was diagnosed and Computed Tomography (CT) showed a 9 cm suspicious adrenal mass. The patient started on spironolactone and underwent open adrenalectomy. A total resection was done (R0) and it was a 10 cm adrenal carcinoma, Weiss score 5, Ki67 30% and stage II ENSAT. There was clinical and biochemical normalization and at 9 months CT didn’t show any recurrence. Due to high risk carcinoma therapy with mitotane was proposed, however the patient refused and left follow-up. 30 months after surgery, he was admitted to the Emergency Department for adrenal carcinoma relapse and disseminated metastatic disease with simultaneous secretion of aldosterone and cortisol. He started on chemotherapy with EDP-M (etoposide, doxorubicin, cisplatin and mitotane). Despite initial favorable course, the patient got worse with disease progression, culminating in death 44 months after the diagnosis.

Discussion: The management of ACC is challenging. Surgery is crucial for treatment and mitotane is important as an adjuvant therapy, as a chemotherapeutic agent and also to control the hormonal secretion. We report a case of an aldosterone-producing ACC, an extremely rare cause of primary hyperaldosteronism. Even rarer was the change on secretion profile throughout disease evolution with multiple steroid production. The hormonal profile should be carefully investigated in an ACC and the possibility of multiple combined hormonal secretion or the shift of the profile secretion should be considered.