PMID- 24356648
OWN - NLM
STAT- MEDLINE
DCOM- 20141124
LR  - 20140319
IS  - 1423-0194 (Electronic)
IS  - 0028-3835 (Linking)
VI  - 98
IP  - 4
DP  - 2013
TI  - Enucleation and limited pancreatic resection provide long-term cure for 
      insulinoma in multiple endocrine neoplasia type 1.
PG  - 290-8
LID - 10.1159/000357779 [doi]
AB  - AIM: To assess the characteristics and long-term outcome after surgery in 
      patients with multiple endocrine neoplasia type 1 (MEN1)-associated insulinoma. 
      METHODS: Retrospective analysis of prospectively collected data of MEN1 patients 
      with organic hyperinsulinism at a tertiary referral center. RESULTS: Thirteen 
      (17%) of 74 patients with MEN1 had organic hyperinsulinism. The median age at 
      diagnosis was 27 (range 9-48) years. In 7 patients insulinoma was the first 
      manifestation of the syndrome. All patients had at least one pancreatic 
      neuroendocrine neoplasm (pNEN) upon imaging, including CT, MRI or endoscopic 
      ultrasonography. Seven patients had solitary lesions upon imaging, 4 patients had 
      one dominant tumor with coexisting multiple small pNENs, and 2 patients had 
      multiple lesions without dominance. Eight patients had limited resections (1 
      segmental resection, 7 enucleations), 4 subtotal distal pancreatectomies, and 1 
      patient a partial duodenopancreatectomy. There was no postoperative mortality. 
      Six patients experienced complications, including pancreatic fistula in 5 
      patients. Pathological examination revealed median three (range 1-14) macro-pNENs 
      sized between 6 and 40 mm, and a total of 14 potentially benign insulinomas were 
      detected in the 13 patients. After median follow-up of 156 months, only 1 patient 
      developed recurrent hyperinsulinism after initial enucleation. Twelve patients 
      developed new pNENs in the pancreatic remnant and 4 patients underwent 
      reoperations (3 for metastatic ZES, 1 for recurrent hyperinsulinism). One of 5 
      patients with an initial extended pancreatic resection developed 
      insulin-dependent diabetes mellitus. CONCLUSION: Enucleation and limited 
      resection provide long-term cure for MEN1 insulinoma in patients with solitary or 
      dominant tumors. Subtotal distal pancreatectomy should thus be preserved for 
      patients with multiple pNENs without dominance given the risk of exocrine and 
      endocrine pancreas insufficiency in the mostly young patients.
CI  - (c) 2013 S. Karger AG, Basel.
FAU - Bartsch, Detlef K
AU  - Bartsch DK
AD  - Department of Visceral, Thoracic and Vascular Surgery, Philipps University 
      Marburg, Marburg, Germany.
FAU - Albers, Max
AU  - Albers M
FAU - Knoop, Richard
AU  - Knoop R
FAU - Kann, Peter H
AU  - Kann PH
FAU - Fendrich, Volker
AU  - Fendrich V
FAU - Waldmann, Jens
AU  - Waldmann J
LA  - eng
PT  - Journal Article
DEP - 20131217
PL  - Switzerland
TA  - Neuroendocrinology
JT  - Neuroendocrinology
JID - 0035665
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Child
MH  - Diabetes Mellitus, Type 1/etiology
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Insulinoma/pathology/*surgery
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/pathology/*surgery
MH  - Neoplasm Recurrence, Local/surgery
MH  - *Pancreatectomy/adverse effects/methods
MH  - Pancreatic Fistula/etiology
MH  - Pancreatic Neoplasms/pathology/*surgery
MH  - Prospective Studies
MH  - Reoperation
MH  - Retrospective Studies
MH  - Tertiary Care Centers
MH  - Time Factors
MH  - Treatment Outcome
MH  - Young Adult
EDAT- 2013/12/21 06:00
MHDA- 2014/12/15 06:00
CRDT- 2013/12/21 06:00
PHST- 2013/08/23 00:00 [received]
PHST- 2013/12/06 00:00 [accepted]
PHST- 2013/12/21 06:00 [entrez]
PHST- 2013/12/21 06:00 [pubmed]
PHST- 2014/12/15 06:00 [medline]
AID - 000357779 [pii]
AID - 10.1159/000357779 [doi]
PST - ppublish
SO  - Neuroendocrinology. 2013;98(4):290-8. doi: 10.1159/000357779. Epub 2013 Dec 17.