Isolated primary aldosteronism in adrenocortical carcinoma: A case report and review of literature

Background Adrenocortical carcinoma synthesizing solely mineralocorticoids is an unusual disorder which has only been reported in literature in 22 other patients. Methods This case report describes a patient presenting with hypokalaemia, hypertension, metabolic acidosis and a left adrenal tumor. Extensive laboratory testing allowed the diagnosis of pure primary hyperaldosteronism without secretion of other steroids. Results The treatment of choice after establishing the diagnosis biochemically is to remove the tumor surgically. Histologically the lesion within the left adrenal was classified as a benign adrenocortical adenoma. After recurrence of, disease 6 months postoperatively, a widespread retroperitoneal tumor was en bloc resected and malignancy was confirmed by the histological features. When extended total histological examination of the first tumor was performed, capsular invasion was detected in one slice and the diagnosis of adrenocortical cancer was established retrospectively not only on the basis of clinical behavior. Despite precise surgical treatment of the recurrence and chemotherapy, the patient died 14 months after the diagnosis of primary hyperaldosteronism. Conclusions Close biochemical and clinical follow-up examinations are necessary in each patient with hormonlly active tumors. Sometimes only the postoperative course discriminates benign and malignant lesions.