An ectopic renin‐secreting adrenal corticoadenoma in a child with malignant hypertension. Issue 5 (20th March 2016)

• Record Type: Journal Article
• Authors: Kaslow, Abraham M.; Riquier‐Brison, Anne; Peti‐Peterdi, Janos; Shillingford, Nick; HaDuong, Josephine; Venkatramani, Rajkumar; Gayer, Christopher P.
• Is Part Of: Physiological reports. Volume 4:Issue 5(2016:Mar.)
• Page Start: n/a
• Page End: n/a
• Publication Date: 2016-03-20
• Subjects: Adrenal mass -- aldosterone -- ectopic renin -- malignant hypertension -- pheochromocytoma; Physiology -- Periodicals; 571
• DOI: 10.14814/phy2.12728
• Languages: English
• ISSNs: 2051-817X

Abstract

Abstract: A previously healthy 7‐year‐old male presented with hypertensive emergency, hypokalemia, and elevated plasma renin activity and aldosterone levels. There was no evidence of virilization or cushingoid features. MRI of the abdomen revealed a large (5 × 5 × 3 cm) peripherally enhancing, heterogeneous mass arising from the left adrenal gland. The patient was treated for a suspected pheochromocytoma. However, his blood pressure was not responsive to alpha‐blockade. Blood pressure was controlled with a calcium channel blocker and an angiotensin‐converting enzyme (ACE) inhibitor. A complete surgical resection of the mass was performed. Postoperatively, his blood pressure normalized and he did not require antihypertensives. On pathological examination, the tumor tissue stained negative for chromogranin and positive for renin. The final diagnosis was renin‐secreting adrenal corticoadenoma, an extremely rare adrenal tumor not previously reported in a pediatric patient. Malignant hypertension due to a renin‐secreting tumor may need to be distinguished from a pheochromocytoma if alpha‐adrenergic blockade is ineffective. Abstract : This report summarizes a case of a hypertensive emergency in an otherwise healthy 7‐year‐old patient. A large adrenal mass was detected, and patient was unresponsive to the treatment with alpha‐blockade for pheochromocytoma. Surgical removal normalized blood pressure. Tumor tissue stained negative for chromogranin and positive for renin providing evidence for a rare renin‐secreting adrenal corticoadenoma, not previously reported in a pediatric patient.