alexa Recent advances in the diagnosis and treatment of pheochromocytoma in children


Medical & Surgical Urology

Author(s): Ludwig AD, Feig DI, Brandt ML, Hicks MJ, Fitch ME

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BACKGROUND: The purpose of this study was to analyze the effects of changes in the diagnosis and treatment of pheochromocytoma in a pediatric population.

METHODS: We reviewed the medical records of all children who had resection of pheochromocytoma or paraganglioma at a major children's hospital since 1968.

RESULTS: Fifteen children underwent surgery at 11.9 +/- 4.2 years of age. Presenting symptoms included headache, hypertension, and sweating. Three children had a mutation of the succinate dehydrogenase enzyme, and 1 child had nonsyndromic, familial pheochromocytoma. The most sensitive diagnostic modalities included 24-hour urinary and plasma norepinephrine and 24-hour urinary total metanephrines, magnetic resonance imaging, and 123I-meta-iodobenzylguanidine scintigraphy. Laparoscopic cortical-sparing adrenalectomy was performed in 3 patients with von Hippel-Lindau disease. Compared with those with open procedures (n = 7), patients who had laparoscopic resection (n = 5) had a statistically shorter hospital length of stay, and time to eating ambulation.

CONCLUSIONS: The addition of 123I-meta-iodobenzylguanidine scanning, genetic testing, and laparoscopic surgery has changed the diagnosis and treatment of pheochromocytoma in children. Laparoscopic cortical-sparing adrenalectomy can be accomplished safely and is the preferred treatment for children at risk for multifocal disease.

This article was published in Am J Surg and referenced in Medical & Surgical Urology

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