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Adrenal and Extra-adrenal Pheochromocytomas in a Family With Germline RET V804L Mutation

Ola Nilsson, MD, PhD; Lars E. Tisell, MD, PhD; Svante Jansson, MD, PhD; Håkan Ahlman, MD, PhD; Oliver Gimm, MD; Charis Eng, MD, PhD
JAMA. 1999;281(17):1587-1588. doi:10-1001/pubs.JAMA-ISSN-0098-7484-281-17-jbk0505.
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To the Editor: Testing of the RET gene in patients with multiple endocrine neoplasia type 2 (MEN 2) and apparently isolated medullary thyroid carcinoma (MTC) is standard in molecular medicine. The MEN 2 syndromes and familial MTC are autosomal dominant disorders comprising 3 subtypes. MEN 2A includes MTC, pheochromocytoma (PC), and hyperparathyroidism. MEN 2B comprises MTC, PC, and characteristic developmental abnormalities (ie, marfanoid habitus and gastrointestinal tract ganglioneuromas). The operational classification of familial MTC is 4 or more family members with MTC without objective evidence of PC and hyperparathyroidism on screening of all living affected and at-risk individuals.1

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Figure. A Family With RET V804L Mutation
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The family's genetic makeup was traced back to the mid-1800s. The coding system referred to in the key may apply to either men or women.



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