PMID- 19125015
OWN - NLM
STAT- MEDLINE
DCOM- 20090317
LR  - 20090106
IS  - 1531-703X (Electronic)
IS  - 1040-8746 (Linking)
VI  - 21
IP  - 1
DP  - 2009 Jan
TI  - Molecular genetics of gastroenteropancreatic neuroendocrine tumors.
PG  - 29-33
LID - 10.1097/CCO.0b013e328319ea7b [doi]
AB  - PURPOSE OF REVIEW: Gastroenteropancreatic neuroendocrine tumors (GEP NETs) are 
      relatively rare neoplasias arising from the embryonic neural crest, neuroectoderm 
      and endoderm. GEP NETs occur either sporadically or as part of endocrine tumor 
      susceptibility syndromes such as multiple endocrine neoplasia type 1 (MEN1), von 
      Hippel Lindau (VHL) syndrome, neurofibromatosis (NF-1), and possibly tuberous 
      sclerosis (TSC). The overall incidence of GEP NETs shows a significant increase 
      over the past three decades. Improved understanding of the molecular genetics 
      associated with these lesions will likely enhance the diagnosis and treatment of 
      patients with GEP NET. RECENT FINDINGS: The molecular and clinical genetics of 
      familial GEP NETs have been further elucidated by the characterization of the 
      tumor suppressor genes, MEN1, VHL, NF-1, TSC1, and TSC2. The vastly improved 
      technology in the field of cancer genetics with higher resolution of the study of 
      genetic alterations, and the ability of unbiased mutational analyses of entire 
      tumor genomes is likely to further the understanding of the genetic mechanisms of 
      sporadic GEP NET as well. SUMMARY: Recent advances in the molecular genetics of 
      sporadic and familial GEP NET are reviewed.
FAU - Starker, Lee F
AU  - Starker LF
AD  - Department of Surgery, Yale University School of Medicine, New Haven, Connecticut 
      06520, USA.
FAU - Carling, Tobias
AU  - Carling T
LA  - eng
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Curr Opin Oncol
JT  - Current opinion in oncology
JID - 9007265
SB  - IM
MH  - Gastrointestinal Neoplasms/*genetics
MH  - Genes, Tumor Suppressor
MH  - Humans
MH  - *Molecular Biology
MH  - Neuroendocrine Tumors/*genetics
MH  - Pancreatic Neoplasms/*genetics
RF  - 58
EDAT- 2009/01/07 09:00
MHDA- 2009/03/18 09:00
CRDT- 2009/01/07 09:00
PHST- 2009/01/07 09:00 [entrez]
PHST- 2009/01/07 09:00 [pubmed]
PHST- 2009/03/18 09:00 [medline]
AID - 00001622-200901000-00007 [pii]
AID - 10.1097/CCO.0b013e328319ea7b [doi]
PST - ppublish
SO  - Curr Opin Oncol. 2009 Jan;21(1):29-33. doi: 10.1097/CCO.0b013e328319ea7b.