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Correspondence
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Volume 357:306-308 July 19, 2007 Number 3
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Somatic SDHB Mutation in an Extraadrenal Pheochromocytoma

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To the Editor: As many as 25% of pheochromocytomas — catecholamine-producing tumors located along the sympathetic nervous system, including the adrenals — occur in hereditary tumor syndromes that include von Hippel–Lindau disease (VHL gene),1 multiple endocrine neoplasia type 2 (RET gene), neurofibromatosis type 1 (NF1 gene), and the pheochromocytoma–paraganglioma syndrome (SDHB and SDHD genes). The last two genes are also associated with extraadrenal pheochromocytoma.2,3 To date, except for one sporadic SDHD mutation, only germ-line mutations in SDHB and SDHD have been described, even among reported mutations in these genes in apparently sporadic pheochromocytomas and paragangliomas.4,5

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