PMID- 21464564
OWN - NLM
STAT- MEDLINE
DCOM- 20120118
LR  - 20140730
IS  - 1423-0194 (Electronic)
IS  - 0028-3835 (Linking)
VI  - 94
IP  - 1
DP  - 2011
TI  - Presentation and outcome of pancreaticoduodenal endocrine tumors in multiple 
      endocrine neoplasia type 1 syndrome.
PG  - 58-65
LID - 10.1159/000326164 [doi]
AB  - AIM: To assess presentation and outcome of pancreaticoduodenal endocrine tumors 
      (PDETs) in a single center series of multiple endocrine neoplasia type 1 (MEN1) 
      patients. METHODS: Retrospective analysis of prospectively collected data of MEN1 
      patients observed at the University of Verona. RESULTS: Thirty-one MEN1 patients 
      had PDETs, including 16 nonfunctioning (NF), 6 insulinomas and 9 
      Zollinger-Ellison syndrome (ZES). In 16 of these patients (52%), PDET was the 
      manifestation which led to the diagnosis of MEN1; among this group, 15 patients 
      (94%) previously had unidentified primary hyperparathyroidism (PHPT), which was 
      asymptomatic in 9 cases (60%). Of the 31 patients, 19 (61%) underwent 
      curativesurgery and 13 (68%, 7 NF-PDETs, 4 insulinomas and 2 ZES) were 
      disease-free after a median follow-up of 3 years (range: 0.5-15). One patient had 
      debulking surgery with stable disease after 2 years of follow-up. Eight patients 
      with NF-PDETs </=20 mm and 2 with ZES, treated with a conservative approach, showed 
      stable disease. One patient with insulinoma was lost to follow-up. CONCLUSIONS: 
      PDET may be the manifestation that leads to MEN1 diagnosis since the almost 
      constant presence of PHPT is very often unrecognized or considered sporadic. 
      Conversely, the presence of PDETs should be looked for in all patients presenting 
      PHPT, even if asymptomatic, particularly before age 50. Surgery may be curative 
      in the majority of insulinomas and can prolong disease-free survival in NF-PDET, 
      but is not proven to be effective in ZES. A conservative approach can be safely 
      reserved for patients with NF-PDETs </=20 mm.
CI  - Copyright (c) 2011 S. Karger AG, Basel.
FAU - Davi, Maria Vittoria
AU  - Davi MV
AD  - Department of Medicine, University of Verona, Italy. mariavittoria.davi @ 
      ospedaleuniverona.it
FAU - Boninsegna, Letizia
AU  - Boninsegna L
FAU - Dalle Carbonare, Luca
AU  - Dalle Carbonare L
FAU - Toaiari, Marco
AU  - Toaiari M
FAU - Capelli, Paola
AU  - Capelli P
FAU - Scarpa, Aldo
AU  - Scarpa A
FAU - Francia, Giuseppe
AU  - Francia G
FAU - Falconi, Massimo
AU  - Falconi M
LA  - eng
PT  - Journal Article
DEP - 20110405
PL  - Switzerland
TA  - Neuroendocrinology
JT  - Neuroendocrinology
JID - 0035665
RN  - Non functioning pancreatic endocrine tumor
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Disease-Free Survival
MH  - Duodenal Neoplasms/*diagnosis/surgery
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Insulinoma/*diagnosis/surgery
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*diagnosis/surgery
MH  - Pancreatectomy
MH  - Pancreatic Neoplasms/*diagnosis/surgery
MH  - Pancreaticoduodenectomy
MH  - Prognosis
MH  - Prospective Studies
MH  - Retrospective Studies
MH  - Treatment Outcome
MH  - Young Adult
MH  - Zollinger-Ellison Syndrome/*diagnosis/surgery
EDAT- 2011/04/06 06:00
MHDA- 2012/01/19 06:00
CRDT- 2011/04/06 06:00
PHST- 2010/09/09 00:00 [received]
PHST- 2011/02/21 00:00 [accepted]
PHST- 2011/04/06 06:00 [entrez]
PHST- 2011/04/06 06:00 [pubmed]
PHST- 2012/01/19 06:00 [medline]
AID - 000326164 [pii]
AID - 10.1159/000326164 [doi]
PST - ppublish
SO  - Neuroendocrinology. 2011;94(1):58-65. doi: 10.1159/000326164. Epub 2011 Apr 5.