Pure aldosterone-secreting adrenocortical carcinoma in a patient with refractory primary hyperaldosteronism

Hussain, S
Panteliou, E
Berney, D M
Carpenter, R
Matson, M
Sahdev, A
Bell, M
O'Sullivan, E
Drake, W M
Repository DOI
Publication Date
Hussain, S; Panteliou, E; Berney, D M; Carpenter, R; Matson, M; Sahdev, A; Bell, M; O'Sullivan, E; Drake, W M (2015). Pure aldosterone-secreting adrenocortical carcinoma in a patient with refractory primary hyperaldosteronism. Endocrinology, Diabetes & Metabolism Case Reports ,
We describe a young male patient with longstanding hypertension, who was diagnosed with primary hyperaldosteronism and treated by an attempted retroperitoneoscopic total unilateral adrenalectomy for a left-sided presumed aldosterone-secreting adenoma. Imaging had shown an unremarkable focal adrenal lesion with normal contralateral adrenal morphology, and histology of the resected specimen showed no adverse features. Post-operatively, his blood pressure and serum aldosterone levels fell to the normal range, but 9 months later, his hypertension recurred, primary aldosteronism was again confirmed and he was referred to our centre. Repeat imaging demonstrated an irregular left-sided adrenal lesion with normal contralateral gland appearances. Adrenal venous sampling was performed, which supported unilateral (left-sided) aldosterone hypersecretion. Redo surgery via a laparoscopically assisted transperitoneal approach was performed and multiple nodules were noted extending into the retroperitoneum. It was thought unlikely that complete resection had been achieved. His blood pressure returned to normal post-operatively, although hypokalaemia persisted. Histological examination, from this second operation, showed features of an adrenocortical carcinoma (ACC; including increased mitoses and invasion of fat) that was assessed as malignant using the scoring systems of Weiss and Aubert. Biochemical hyperaldosteronism persisted post-operatively, and detailed urine steroid profiling showed no evidence of adrenal steroid precursors or other mineralocorticoid production. He received flank radiotherapy to the left adrenal bed and continues to receive adjunctive mitotane therapy for a diagnosis of a pure aldosterone-secreting ACC.
Publisher DOI
Attribution-NonCommercial-NoDerivs 3.0 Ireland