PMID- 22902777
OWN - NLM
STAT- MEDLINE
DCOM- 20130322
LR  - 20130430
IS  - 1542-7714 (Electronic)
IS  - 1542-3565 (Linking)
VI  - 10
IP  - 11
DP  - 2012 Nov
TI  - Importance of surveillance for multiple endocrine neoplasia-1 and surgery in 
      patients with sporadic Zollinger-Ellison syndrome.
PG  - 1262-9
LID - S1542-3565(12)00934-2 [pii]
LID - 10.1016/j.cgh.2012.08.014 [doi]
AB  - BACKGROUND & AIMS: Zollinger-Ellison syndrome (ZES) is a rare disorder 
      characterized by gastrin-secreting tumors of the gastrointestinal tract and 
      gastric acid hypersecretion. There is controversy over the best way to manage 
      these patients; we investigated outcomes of patients with different forms of the 
      disease, who did and did not undergo surgery. METHODS: We performed a 
      retrospective chart review of patients with ZES associated with multiple 
      endocrine neoplasia type 1 (MEN-1) (n = 16) and those with sporadic ZES (n = 33) 
      seen at a tertiary care center from August 1994 to January 2012. Cox proportional 
      hazards modeling was used to compare survival times among groups, based on 
      treatment with surgery (n = 34) and the presence of MEN-1 (n = 9 with surgery; n 
      = 7 without surgery). Differences were compared using the unpaired Student t test 
      and the Fisher exact test. RESULTS: Patients with MEN-1 syndrome-associated ZES 
      presented at a younger age than patients with sporadic ZES (34.9 vs 45.7 y, 
      respectively; P < .05) and were diagnosed at a younger age (39.3 vs 49.7 y, 
      respectively; P < .01), yet lived a similar number of years (55.9 vs 55.1 y, 
      respectively; P = .91). None of the patients with MEN-1-associated ZES died of 
      progressive disease, compared with 86% of deaths among patients with sporadic ZES 
      (P < .05). Lymph node involvement, detected during surgery, increased the risk of 
      metastasis to liver (P = .13) and lack of cure by surgery (P = .01). Surgery 
      reduced all-cause mortality (hazard ratio, 0.11; 95% confidence interval, 
      0.2-0.6; P = .011) and disease-related mortality (hazard ratio, 0.14; 95% 
      confidence interval, 0.2-0.84; P = .032) of patients with sporadic, but not MEN-1 
      syndrome-associated, ZES. CONCLUSIONS: The presence of MEN-1 is associated with 
      earlier onset and diagnosis of ZES, but a benign clinical course that rarely 
      results in disease-related death; surgery therefore can be deferred for these 
      patients. However, 86% of deaths among patients with sporadic ZES are attributed 
      to disease-related causes, and mortality is reduced by early surgical 
      intervention. Patients with sporadic ZES should undergo surgery soon after 
      diagnosis.
CI  - Copyright (c) 2012 AGA Institute. Published by Elsevier Inc. All rights reserved.
FAU - Singh, Maneesh H
AU  - Singh MH
AD  - Department of Medicine, Hospital of the University of Pennsylvania, Perelman 
      School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 
      19104, USA. maneesh.singh@uphs.upenn.edu
FAU - Fraker, Douglas L
AU  - Fraker DL
FAU - Metz, David C
AU  - Metz DC
LA  - eng
PT  - Journal Article
DEP - 20120817
PL  - United States
TA  - Clin Gastroenterol Hepatol
JT  - Clinical gastroenterology and hepatology : the official clinical practice journal 
      of the American Gastroenterological Association
JID - 101160775
SB  - IM
CIN - Clin Gastroenterol Hepatol. 2013 Apr;11(4):448. PMID: 23270867
CIN - Clin Gastroenterol Hepatol. 2013 Apr;11(4):448-9. PMID: 23313841
MH  - Adult
MH  - Female
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*diagnosis/*epidemiology/mortality/surgery
MH  - Retrospective Studies
MH  - Survival Analysis
MH  - Treatment Outcome
MH  - Zollinger-Ellison Syndrome/*complications/*diagnosis
EDAT- 2012/08/21 06:00
MHDA- 2013/03/23 06:00
CRDT- 2012/08/21 06:00
PHST- 2012/05/26 00:00 [received]
PHST- 2012/07/09 00:00 [revised]
PHST- 2012/08/06 00:00 [accepted]
PHST- 2012/08/21 06:00 [entrez]
PHST- 2012/08/21 06:00 [pubmed]
PHST- 2013/03/23 06:00 [medline]
AID - S1542-3565(12)00934-2 [pii]
AID - 10.1016/j.cgh.2012.08.014 [doi]
PST - ppublish
SO  - Clin Gastroenterol Hepatol. 2012 Nov;10(11):1262-9. doi: 
      10.1016/j.cgh.2012.08.014. Epub 2012 Aug 17.