Molecular genetics of neuroendocrine tumors

Neuroendocrine tumors can develop either sporadically or in association with familial syndromes such as multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2 (MEN2) or von Hippel–Lindau (VHL). A variety of genetic approaches has been utilized to dissect the underlying molec...

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Bibliographic Details
Published in:Baillière's best practice & research. Clinical endocrinology & metabolism 2007-03, Vol.21 (1), p.1-14
Main Authors: Duerr, Eva-Maria, MD, Chung, Daniel C., MD
Format: Article
Language:English
Subjects:
Animals
carcinoid
Cell Cycle - genetics
CGH
Chromosome Mapping
Chromosomes, Human
Endocrinology & Metabolism
Gene Expression Profiling - methods
Humans
LOH
Loss of Heterozygosity
MEN1
MEN2
microarray
Models, Biological
Multiple Endocrine Neoplasia Type 1 - genetics
Multiple Endocrine Neoplasia Type 2a - genetics
NET
neuroendocrine tumors
Neuroendocrine Tumors - genetics
Nucleic Acid Hybridization - methods
Syndrome
VHL
von Hippel-Lindau Disease - genetics
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Summary:Neuroendocrine tumors can develop either sporadically or in association with familial syndromes such as multiple endocrine neoplasia type 1 (MEN1), multiple endocrine neoplasia type 2 (MEN2) or von Hippel–Lindau (VHL). A variety of genetic approaches has been utilized to dissect the underlying molecular pathogenesis of these distinctive tumors, including genome-wide screens such as comparative genomic hybridization, loss of heterozygosity and DNA microarray analysis as well as targeted investigations into specific tumor suppressor gene and oncogene candidates. The identification of the MEN1 tumor suppressor gene that underlies the MEN1 syndrome has provided important new insights into tumor pathogenesis. In addition, a number of independent approaches has converged on a pivotal role for regulators of the cell cycle. However, our understanding of the molecular biology of these tumors remains far from complete. In this review we highlight some of the key approaches, findings and implications of these genetic studies.
ISSN:1521-690X
1878-1594
DOI:10.1016/j.beem.2006.12.001