Renal artery stenosis and pheochromocytoma: Coexistence and treatment

Purpose: The aim of this study was to develop an optimal treatment plan for coexisting pheochromocytoma and renal artery stenosis. Methods: A retrospective analysis of the evaluation and management of a hyp ertensive 14-year-old boy was conducted. Results: Secondary causes of hypertension were excluded initially including intracranial, cardiac, and endocrine abnormalities. A renal scan showed ri ght renal function at 39%. Angiography confirmed a 90% subsegmental stenosi s, as well as noting a blush suggesting a tumor. A computerized tomography (CT) scan of the abdomen showed a right adrenal mass. Serum epinephrine was 3,006 pg/mL (normal, <1,009 pg/mL) and 24-hour urinary norepinephrine was 2,001 mu g (normal, <105 mu g/24 h), suggesting the presence of a pheochrom ocytoma. During the operation for resection, a right subsegmental renal art ery was found to be draped around a renal hilar mass; the adrenal gland was normal. The tumor was resected, and pathology confirmed a pheochromocytoma , Postoperatively, the patient had some mild hypertension that gradually re solved. Conclosions: Extraadrenal pheochromocytomas (paragangliomas) occur more fre quently in children and are most commonly located in the renal hilum, In th is location, they may present initially as renal artery stenosis as a resul t of direct arterial compression, fibrous bands, or catecholamine-induced v asospasm, Our experience supports tumor resection of extraadrenal pheochrom ocytomas as the definitive treatment for both conditions. Copyright (C) 200 0 by W.B. Saunders Company.