Document Detail


Solid tumors associated with multiple endocrine neoplasias.
MedLine Citation:
PMID:  20951316     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
We present an update on molecular and clinical genetics of solid tumors associated with the various multiple endocrine neoplasias (MEN) syndromes. MEN type 1 (MEN1) describes the association of pituitary, parathyroid, and pancreatic islet cell tumors with a variety of many other lesions. MEN type 2 (MEN2) conditions represent at least four different syndromes that associate pheochromocytoma with medullary thyroid carcinoma, hyperparathyroidism, and a number of other manifestations. Other pheochromocytoma-associated syndromes include von Hippel-Lindau disease; neurofibromatosis 1; the recently defined paraganglioma syndromes type 1, 3, and 4; Carney-Stratakis syndrome; and the Carney triad. Carney-Stratakis syndrome is characterized by the association of paragangliomas and familial gastrointestinal stromal tumors. In the Carney triad, patients can manifest gastrointestinal stromal tumors, lung chondroma, paraganglioma, adrenal adenoma and pheochromocytoma, esophageal leiomyoma, and other conditions. The Carney complex is yet another form of MEN that is characterized by skin tumors and pigmented lesions, myxomas, schwannomas, and various endocrine neoplasias.
Authors:
Madson Q Almeida; Constantine A Stratakis
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Cancer genetics and cytogenetics     Volume:  203     ISSN:  1873-4456     ISO Abbreviation:  Cancer Genet. Cytogenet.     Publication Date:  2010 Nov 
Date Detail:
Created Date:  2010-10-18     Completed Date:  2010-11-02     Revised Date:  2011-11-01    
Medline Journal Info:
Nlm Unique ID:  7909240     Medline TA:  Cancer Genet Cytogenet     Country:  United States    
Other Details:
Languages:  eng     Pagination:  30-6     Citation Subset:  IM    
Copyright Information:
Copyright © 2010 Elsevier Inc. All rights reserved.
Affiliation:
Section on Endocrinology and Genetics, Program on Developmental Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Building 10, CRC, Room I-3330, 10 Center Dr., MSC 1103, Bethesda, MD 20892, USA.
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MeSH Terms
Descriptor/Qualifier:
Adrenal Gland Neoplasms / genetics
Germ-Line Mutation
Humans
Multiple Endocrine Neoplasia / complications*,  genetics
Multiple Endocrine Neoplasia Type 1 / complications
Multiple Endocrine Neoplasia Type 2a / complications
Neoplasms / genetics*
Pheochromocytoma / genetics
Thyroid Neoplasms / etiology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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