PMID- 10816635
OWN - NLM
STAT- MEDLINE
DCOM- 20000628
LR  - 20190514
IS  - 0003-4932 (Print)
IS  - 1528-1140 (Electronic)
IS  - 0003-4932 (Linking)
VI  - 231
IP  - 6
DP  - 2000 Jun
TI  - Duodenopancreatic resections in patients with multiple endocrine neoplasia type 
      1.
PG  - 909-18
AB  - OBJECTIVE: To review the authors' 7-year experience with a surgical approach for 
      pancreatic and duodenal neuroendocrine tumors (NETs) in patients with multiple 
      endocrine neoplasia type 1 (MEN 1) designed to remove all gross tumor with 
      limited complications, preserving pancreatic function. SUMMARY BACKGROUND DATA: 
      MEN 1 is an autosomal dominant familial neoplasia syndrome characterized by the 
      development of NETs of the duodenum and pancreas. Some tumors are clinically 
      insignificant or follow a benign course, although a subset pursues a malignant, 
      lethal natural history; the risk of surgical management must be appropriate to 
      the disease course. METHODS: The clinical, biochemical, genetic, and pathologic 
      data were retrospectively reviewed for 21 consecutive MEN 1 patients undergoing 
      pancreatic resection for NETs between 1993 and 1999 at one institution. Age at 
      operation, presenting symptoms, results of preoperative and intraoperative 
      localization studies, major and minor complications, and pathology, including 
      metastases, were analyzed. RESULTS: The surgical approach was selected based on 
      the location and size of the tumors. Five patients required 
      pancreaticoduodenectomy, 11 patients underwent non-Whipple pancreatic resections, 
      and 5 underwent simple enucleation of benign NETs. The incidence of regional 
      lymph node metastases was 33%. CONCLUSIONS: Major pancreatic procedures can be 
      performed safely in most patients with MEN 1 and NETs. Because NETs are the most 
      common MEN 1-related cause of death in the authors' kindreds, an aggressive 
      surgical approach, including early intervention before malignant spread and major 
      pancreatic resection where indicated, appears justified.
FAU - Lairmore, T C
AU  - Lairmore TC
AD  - Section of Endocrine and Oncologic Surgery, Washington University School of 
      Medicine, St. Louis, Missouri 63110, USA. lairmoret@msnotes.wustl.edu
FAU - Chen, V Y
AU  - Chen VY
FAU - DeBenedetti, M K
AU  - DeBenedetti MK
FAU - Gillanders, W E
AU  - Gillanders WE
FAU - Norton, J A
AU  - Norton JA
FAU - Doherty, G M
AU  - Doherty GM
LA  - eng
GR  - M01 RR000036/RR/NCRR NIH HHS/United States
GR  - M01RR00036/RR/NCRR NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Ann Surg
JT  - Annals of surgery
JID - 0372354
SB  - IM
MH  - Adult
MH  - Aged
MH  - Duodenal Neoplasms/genetics/pathology/*surgery
MH  - Female
MH  - Frameshift Mutation
MH  - Humans
MH  - Lymphatic Metastasis
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/genetics/pathology/*surgery
MH  - Mutation, Missense
MH  - Pancreatic Neoplasms/genetics/pathology/*surgery
MH  - Retrospective Studies
PMC - PMC1421081
EDAT- 2000/05/19 09:00
MHDA- 2000/07/06 11:00
PMCR- 2000/12/01
CRDT- 2000/05/19 09:00
PHST- 2000/05/19 09:00 [pubmed]
PHST- 2000/07/06 11:00 [medline]
PHST- 2000/05/19 09:00 [entrez]
PHST- 2000/12/01 00:00 [pmc-release]
AID - 0000658-200006000-00016 [pii]
AID - 10.1097/00000658-200006000-00016 [doi]
PST - ppublish
SO  - Ann Surg. 2000 Jun;231(6):909-18. doi: 10.1097/00000658-200006000-00016.