Document Detail

Current concepts in the surgical management of multiple endocrine neoplasia type 1 pancreatic-duodenal disease. Results in the treatment of 40 patients with Zollinger-Ellison syndrome, hypoglycaemia or both.
MedLine Citation:
PMID:  9681848     Owner:  NLM     Status:  MEDLINE    
The management of multiple endocrine neoplasia type 1 (MEN-1) pancreatic-duodenal disease, particularly when the Zollinger-Ellison syndrome (ZES) is the presenting manifestation, has remained controversial. The management of hypoglycaemia and other syndromes as well as large tumours detected by imaging is less controversial, although standardized surgical techniques have not been generally adapted. The rationale for an aggressive operative management plan for ZES and other syndromes is based on the facts that neuroendocrine tumours of both the pancreas and the duodenum have malignant potential and that the functional manifestations can be controlled with appropriate surgical procedures based on current concepts of the MEN-1 disease. Of the ten concepts presented, the one critical to the surgical treatment of ZES is that a duodenotomy is essential in detecting the source of hypergastrinaemia in most MEN-1 patients. The complete operation is multifaceted and includes peripancreatic lymph node dissection (ZES), enucleation of any head or uncinate tumours and a distal pancreatectomy. Our results in 40 MEN-1 patients with functional syndromes treated with these procedures are encouraging. Ten patients with hypoglycaemia (four with concomitant ZES) have been 'cured' with follow-up as long as 18 years. Sixty-eight per cent of 34 patients with ZES have remained eugastrinaemic during follow-up as long as 19 years. One patient developed a solitary liver metastasis that was excised a year ago without other evidence of recurrence. There has been no operative mortality and three subsequent deaths were due to unrelated disease.
N W Thompson
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of internal medicine     Volume:  243     ISSN:  0954-6820     ISO Abbreviation:  J. Intern. Med.     Publication Date:  1998 Jun 
Date Detail:
Created Date:  1998-08-13     Completed Date:  1998-08-13     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  8904841     Medline TA:  J Intern Med     Country:  ENGLAND    
Other Details:
Languages:  eng     Pagination:  495-500     Citation Subset:  IM    
Division of Endocrine Surgery, University of Michigan, Ann Arbor, USA.
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MeSH Terms
Duodenal Neoplasms / genetics,  surgery*
Follow-Up Studies
Gastrinoma / genetics,  surgery*
Hypoglycemia / genetics,  surgery*
Insulinoma / genetics,  surgery*
Multiple Endocrine Neoplasia Type 1 / complications*,  surgery
Pancreatic Neoplasms / genetics,  surgery*
Survival Analysis
Treatment Outcome
Zollinger-Ellison Syndrome / genetics,  surgery*

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine

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