Journal of pediatric surgery · 2000
Renal artery stenosis and pheochromocytoma: coexistence and treatment.
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Camberos A, Bautista N, Rubenzik M, Applebaum H
Аннотация
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 microg (normal, <105 microg/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.
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 microg (normal, <105 microg/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.