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Reviews in Endocrine and Metabolic Disorders
Published in: Reviews in Endocrine and Metabolic Disorders 3/2016

01-09-2016

Hereditary syndromes predisposing to endocrine tumors and their skin manifestations

Author: Constantine A. Stratakis

Published in: Reviews in Endocrine and Metabolic Disorders | Issue 3/2016

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Abstract

We often think of the lentiginoses, phacomatoses and other neurocutaneous syndromes as conditions that affect the skin and also predispose to a variety of tumors. However, we rarely think of Peutz-Jeghers syndrome (PJS), Carney complex (CNC), Cowden disease (CD), neurofibromatosis type-1 (NF-1) or tuberous sclerosis (TSC) as conditions that are multiple endocrine neoplasias (MEN). Indeed, all of these conditions predispose to a variety of endocrine tumors, in addition to many other neoplasms. On the other hand, the classic MENs, type 1 and 2 (MEN-1 and MEN-2, respectively) are almost never thought in terms of their skin manifestations. In this review, we present extensively the MEN-1, MEN-2 and PJS syndromes, and briefly refer to CD, NF-1, and TSC. CNC is discussed in another article in this journal issue.
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Metadata
Title
Hereditary syndromes predisposing to endocrine tumors and their skin manifestations
Author
Constantine A. Stratakis
Publication date
01-09-2016
Publisher
Springer US
Published in
Reviews in Endocrine and Metabolic Disorders / Issue 3/2016
Print ISSN: 1389-9155
Electronic ISSN: 1573-2606
DOI
https://doi.org/10.1007/s11154-016-9401-0

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