Abstract
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 hypertensive 14- year-old boy was conducted. Results: Secondary causes of hypertension were excluded initially including intracranial, cardiac, and endocrine abnormalities. A renal scan showed right renal function at 39%. Angiography confirmed a 90% subsegmental stenosis, 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 μg (normal, <105 χ/24 h), suggesting the presence of a pheochromocytoma. During the operation for resection, a right subsegmental renal artery 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 resolved. Conclusions: Extraadrenal pheochromocytomas (paragangliomas) occur more frequently in children and are most commonly located in the renal hilum. In this location, they may present initially as renal artery stenosis as a result of direct arterial compression, fibrous bands, or catecholamine-induced vasospasm. Our experience supports tumor resection of extraadrenal pheochromocytomas as the definitive treatment for both conditions. Copyright (C) 2000 by W.B. Saunders Company.