PMID- 18798544
OWN - NLM
STAT- MEDLINE
DCOM- 20081104
LR  - 20220409
IS  - 0008-543X (Print)
IS  - 1097-0142 (Electronic)
IS  - 0008-543X (Linking)
VI  - 113
IP  - 7 Suppl
DP  - 2008 Oct 1
TI  - Inherited pancreatic endocrine tumor syndromes: advances in molecular 
      pathogenesis, diagnosis, management, and controversies.
PG  - 1807-43
LID - 10.1002/cncr.23648 [doi]
AB  - Pancreatic endocrine tumors (PETs) can occur as part of 4 inherited disorders, 
      including Multiple Endocrine Neoplasia type 1 (MEN1), von Hippel-Lindau disease 
      (VHL), neurofibromatosis 1 (NF-1) (von Recklinghausen disease), and the tuberous 
      sclerosis complex (TSC). The relative frequency with which patients who have 
      these disorders develop PETs is MEN1>VHL>NF-1>TSC. Over the last few years, there 
      have been major advances in the understanding of the genetics and molecular 
      pathogenesis of these disorders as well in the localization and the medical and 
      surgical treatment of PETs in such patients. The study of PETs in these disorders 
      not only has provided insights into the possible pathogenesis of sporadic PETs 
      but also has presented several unique management and treatment issues, some of 
      which are applicable to patients with sporadic PETs. Therefore, the study of PETs 
      in these uncommon disorders has provided valuable insights that, in many cases, 
      are applicable to the general group of patients with sporadic PETs. In this 
      article, these areas are reviewed briefly along with the current state of 
      knowledge of the PETs in these disorders, and the controversies that exist in 
      their management are summarized briefly and discussed.
FAU - Jensen, Robert T
AU  - Jensen RT
AD  - Digestive Diseases Branch, National Institutes of Diabetes, Digestive and Kidney 
      Diseases, National Institutes of Health, Bethesda, Maryland, USA. 
      roberj@bdg10.niddk.nih.gov
FAU - Berna, Marc J
AU  - Berna MJ
FAU - Bingham, David B
AU  - Bingham DB
FAU - Norton, Jeffrey A
AU  - Norton JA
LA  - eng
GR  - R01 CA096645-01A2/CA/NCI NIH HHS/United States
GR  - Z01 DK053200-16/ImNIH/Intramural NIH HHS/United States
GR  - R01 CA 096645-01/CA/NCI NIH HHS/United States
GR  - Z01 DK053215-01/ImNIH/Intramural NIH HHS/United States
GR  - R01 CA096645/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, N.I.H., Extramural
PT  - Research Support, N.I.H., Intramural
PT  - Review
PL  - United States
TA  - Cancer
JT  - Cancer
JID - 0374236
SB  - IM
MH  - Humans
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/*genetics/therapy
MH  - Neurofibromatosis 1/diagnosis/*genetics/therapy
MH  - Pancreatic Neoplasms/diagnosis/*genetics/therapy
MH  - Prognosis
MH  - Tuberous Sclerosis/diagnosis/*genetics/therapy
MH  - von Hippel-Lindau Disease/diagnosis/*genetics/therapy
PMC - PMC2574000
MID - NIHMS72696
EDAT- 2008/09/19 09:00
MHDA- 2008/11/05 09:00
PMCR- 2009/10/01
CRDT- 2008/09/19 09:00
PHST- 2008/09/19 09:00 [pubmed]
PHST- 2008/11/05 09:00 [medline]
PHST- 2008/09/19 09:00 [entrez]
PHST- 2009/10/01 00:00 [pmc-release]
AID - 10.1002/cncr.23648 [doi]
PST - ppublish
SO  - Cancer. 2008 Oct 1;113(7 Suppl):1807-43. doi: 10.1002/cncr.23648.