PMID- 21805182
OWN - NLM
STAT- MEDLINE
DCOM- 20120227
LR  - 20211020
IS  - 1435-2451 (Electronic)
IS  - 1435-2443 (Linking)
VI  - 396
IP  - 8
DP  - 2011 Dec
TI  - Long-term results of surgery for pancreatic neuroendocrine neoplasms in patients 
      with MEN1.
PG  - 1187-96
LID - 10.1007/s00423-011-0828-1 [doi]
AB  - PURPOSE: This study was made to evaluate long-term results of an aggressive 
      surgical approach for pancreaticoduodenal neuroendocrine neoplasms (pNENs) in 
      patients with multiple endocrine neoplasia type 1 (MEN1). METHODS: MEN1 patients 
      with either biochemical evidence of functioning or non-functioning pNENs larger 
      than 1 cm in size on imaging underwent duodenopancreatic surgery. Since 1997, 
      patients were followed annually by biochemical testing and imaging studies. 
      RESULTS: Thirty-eight genetically confirmed MEN1 patients underwent 
      duodenopancreatic resection for functioning (n = 22) or non-functioning (n = 16) 
      pNENs, nine patients were yet not operated. Malignant disease occurred in 12 
      (35%) patients defined by either lymph node (12 patients) and/or distant 
      metastases (2 patients). Six patients with Zollinger-Ellison syndrome (ZES) 
      required pylorus-preserving pancreaticoduodenectomy (PPPD) as initial or redo 
      procedure and 32 patients underwent other duodenopancreatic resections. Ten (26%) 
      patients underwent up to four reoperations for either recurrent or metastatic 
      disease that resulted in completion pancreatectomy in four patients. After median 
      109 months, 44 patients were alive and three patients died, one due to thymic 
      carcinoid and two of unrelated causes. All eight patients with organic 
      hyperinsulinism and 7 of 13 patients with ZES were biochemically cured. However, 
      24 of 38 (78%) patients developed new pNENs in the pancreatic remnant, but none 
      developed distant metastases. CONCLUSIONS: Early resection of pNENs in MEN1 may 
      prevent the development of distant metastases. However, the majority of patients 
      develop new pNENs in the duodenopancreatic remnant which may require completion 
      pancreatectomy in the long term.
FAU - Lopez, Caroline L
AU  - Lopez CL
AD  - Department of Surgery, Philipps University Marburg, Baldingerstrasse, 35043, 
      Marburg, Germany. lopez@med.uni-marburg.de
FAU - Waldmann, Jens
AU  - Waldmann J
FAU - Fendrich, Volker
AU  - Fendrich V
FAU - Langer, Peter
AU  - Langer P
FAU - Kann, Peter H
AU  - Kann PH
FAU - Bartsch, Detlef K
AU  - Bartsch DK
LA  - eng
PT  - Comparative Study
PT  - Journal Article
DEP - 20110730
PL  - Germany
TA  - Langenbecks Arch Surg
JT  - Langenbeck's archives of surgery
JID - 9808285
RN  - Non functioning pancreatic endocrine tumor
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Aged
MH  - Analysis of Variance
MH  - Biopsy, Needle
MH  - Child
MH  - Cohort Studies
MH  - Databases, Factual
MH  - Disease-Free Survival
MH  - Endosonography/methods
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Immunohistochemistry
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/mortality/*pathology/*surgery
MH  - Neoplasm Invasiveness/pathology
MH  - Neoplasm Staging
MH  - Pancreatic Neoplasms/mortality/*pathology/*surgery
MH  - Pancreaticoduodenectomy/methods
MH  - Prospective Studies
MH  - Risk Assessment
MH  - Statistics, Nonparametric
MH  - Survival Analysis
MH  - Time Factors
MH  - Treatment Outcome
MH  - Young Adult
EDAT- 2011/08/02 06:00
MHDA- 2012/03/01 06:00
CRDT- 2011/08/02 06:00
PHST- 2011/02/13 00:00 [received]
PHST- 2011/07/11 00:00 [accepted]
PHST- 2011/08/02 06:00 [entrez]
PHST- 2011/08/02 06:00 [pubmed]
PHST- 2012/03/01 06:00 [medline]
AID - 10.1007/s00423-011-0828-1 [doi]
PST - ppublish
SO  - Langenbecks Arch Surg. 2011 Dec;396(8):1187-96. doi: 10.1007/s00423-011-0828-1. 
      Epub 2011 Jul 30.