PMID- 9681848
OWN - NLM
STAT- MEDLINE
DCOM- 19980813
LR  - 20220316
IS  - 0954-6820 (Print)
IS  - 0954-6820 (Linking)
VI  - 243
IP  - 6
DP  - 1998 Jun
TI  - 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.
PG  - 495-500
AB  - 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.
FAU - Thompson, N W
AU  - Thompson NW
AD  - Division of Endocrine Surgery, University of Michigan, Ann Arbor, USA. 
      normant@umich.edu
LA  - eng
PT  - Journal Article
PL  - England
TA  - J Intern Med
JT  - Journal of internal medicine
JID - 8904841
SB  - IM
MH  - Duodenal Neoplasms/genetics/*surgery
MH  - Follow-Up Studies
MH  - Gastrinoma/genetics/*surgery
MH  - Humans
MH  - Hypoglycemia/genetics/*surgery
MH  - Insulinoma/genetics/*surgery
MH  - Multiple Endocrine Neoplasia Type 1/*complications/surgery
MH  - Pancreatic Neoplasms/genetics/*surgery
MH  - Survival Analysis
MH  - Treatment Outcome
MH  - Zollinger-Ellison Syndrome/genetics/*surgery
EDAT- 1998/07/29 00:00
MHDA- 1998/07/29 00:01
CRDT- 1998/07/29 00:00
PHST- 1998/07/29 00:00 [pubmed]
PHST- 1998/07/29 00:01 [medline]
PHST- 1998/07/29 00:00 [entrez]
AID - 10.1046/j.1365-2796.1998.00307.x [doi]
PST - ppublish
SO  - J Intern Med. 1998 Jun;243(6):495-500. doi: 10.1046/j.1365-2796.1998.00307.x.