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J Pediatr Endocrinol Metab. 2013;26(3-4):369-72. doi: 10.1515/jpem-2012-0255.

Bilateral pheochromocytomas in a child who had hemihypertrophy and alteration in the VHL gene.

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  • 1Division of Pediatric Endocrinology and Metabolism, Mayo Clinic College of Medicine, Rochester, MN, USA.


The association of hypertrophy with neoplasm is well-known. Pheochromocytoma is a rare neoplasm in children. Isolated hemihypertrophy and hemihypertrophy linked to other genetic disorders have rarely been associated with the development of pheochromocytoma, with only two cases reported to date. We report a novel case of a 4-year-old male with bilateral adrenal pheochromocytomas and lower extremity hemihypertrophy in the setting of von Hippel-Lindau syndrome.

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