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

[摘要] We describe a young male patient with longstanding hypertension, who was diagnosed with primary hyperaldosteronismand treated by an attempted retroperitoneoscopic total unilateral adrenalectomy for a left-sided presumed aldosteronesecreting adenoma. Imaging had shown an unremarkable focal adrenal lesion with normal contralateral adrenalmorphology, and histology of the resected specimen showed no adverse features. Post-operatively, his blood pressureand serum aldosterone levels fell to the normal range, but 9 months later, his hypertension recurred, primaryaldosteronism was again confirmed and he was referred to our centre. Repeat imaging demonstrated an irregularleft-sided adrenal lesion with normal contralateral gland appearances. Adrenal venous sampling was performed, whichsupported unilateral (left-sided) aldosterone hypersecretion. Redo surgery via a laparoscopically assisted transperitonealapproach was performed and multiple nodules were noted extending into the retroperitoneum. It was thought unlikelythat complete resection had been achieved. His blood pressure returned to normal post-operatively, althoughhypokalaemia persisted. Histological examination, from this second operation, showed features of an adrenocorticalcarcinoma (ACC; including increased mitoses and invasion of fat) that was assessed as malignant using the scoring systemsof Weiss and Aubert. Biochemical hyperaldosteronism persisted post-operatively, and detailed urine steroid profilingshowed no evidence of adrenal steroid precursors or other mineralocorticoid production. He received flank radiotherapyto the left adrenal bed and continues to receive adjunctive mitotane therapy for a diagnosis of a pure aldosteronesecreting ACC.

[学科分类] 血液学

[关键词] Adult;Male;White;United Kingdom;Adrenal;Adrenal;Aldosterone;Mineralocorticoids;Adrenocortical carcinoma;Hypertension;Aldosteronism;Conn's syndrome;Polydipsia;Polyuria;Palpitations;Paraesthesia;Hypertension;Hypokalaemia;Aldosterone (blood);Renin (blood);CT scan;Dexamethasone suppression (low dose);Adrenal venous sampling;Histopathology;Potassium;Adrenalectomy;Radiotherapy;Mitotane;Amlodipine;Eplerenone;Oncology;Unique/unexpected symptoms or presentations of a disease;August;2015