PMID- 15657577
OWN - NLM
STAT- MEDLINE
DCOM- 20050324
LR  - 20050119
IS  - 0039-6060 (Print)
IS  - 0039-6060 (Linking)
VI  - 136
IP  - 6
DP  - 2004 Dec
TI  - The surgical management of MEN-1 pancreatoduodenal neuroendocrine disease.
PG  - 1205-11
AB  - BACKGROUND: The management of multiple endocrine neoplasia, type 1 (MEN-1) 
      pancreatoduodenal neuroendocrine neoplasms (NENs) is controversial. An aggressive 
      surgical approach is intended to control the functional syndromes and malignant 
      potential for nodal or distant metastasis. METHODS: The results of treating 39 
      patients with MEN-1 pancreatoduodenal NENs over a 35-year period are available 
      from chart reviews and patient interviews. This study focuses on pattern of 
      disease, disease recurrence, and long-term functional outcomes. RESULTS: Between 
      1967 and 2003, 39 patients ages 19 to 58 years (mean age, 37) had abdominal 
      operations for their pancreatoduodenal NENs: 26 with Zollinger-Ellison syndrome, 
      4 with hypoglycemia, 3 with both Zollinger-Ellison syndrome and hypoglycemia, and 
      6 with nonfunctional neoplasms. Fifteen of these 39 patients had malignant 
      disease on initial abdominal operation; 24 of 39 patients have not required 
      abdominal reoperation, 17 of whom have available follow-up data. Of these 17 
      patients, 11 have biochemical evidence of disease recurrence (increased serum 
      concentrations of gastrin, insulin, or pancreatic polypeptide), while 6 have no 
      biochemical evidence of recurrence. A total of 30 abdominal reoperations were 
      performed in 15 patients; 14 of 15 patients undergoing 1 or more reoperations 
      developed evident malignant disease by their most recent operation. Nine of 13 
      reoperative patients with follow-up data have evidence of disease recurrence. 
      Functional outcomes available in 20 patients showed that 10 patients require 
      insulin and that 6 require oral hypoglycemic medications. Ninety percent have no 
      abdominal pain or nausea/vomiting, while 4 are unable to return to work secondary 
      to this disease. CONCLUSIONS: Treatment of MEN-1 pancreatoduodenal NENs is met 
      with frequent recurrence and some treatment-related morbidity and mortality. Most 
      patients (22 of 39) eventually demonstrated malignant growth, but, with this 
      strategy, few died of this disease.
FAU - Hausman, Mark S Jr
AU  - Hausman MS Jr
AD  - Division of Endocrine Surgery, Department of Surgery, University of Michigan, 
      Taubman Center, 1500 East Medical Center Drive, Ann Arbor, MI 48109-0331, USA.
FAU - Thompson, Norman W
AU  - Thompson NW
FAU - Gauger, Paul G
AU  - Gauger PG
FAU - Doherty, Gerard M
AU  - Doherty GM
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Surgery
JT  - Surgery
JID - 0417347
SB  - IM
MH  - Adult
MH  - Digestive System Surgical Procedures
MH  - Duodenal Neoplasms/complications/*surgery
MH  - Female
MH  - Humans
MH  - Hyperinsulinism/etiology/surgery
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/etiology/*surgery
MH  - Neuroendocrine Tumors/complications/*surgery
MH  - Pancreatic Neoplasms/complications/*surgery
MH  - Recurrence
MH  - Retrospective Studies
MH  - Survival Analysis
MH  - Treatment Outcome
MH  - Zollinger-Ellison Syndrome/etiology/surgery
EDAT- 2005/01/20 09:00
MHDA- 2005/03/25 09:00
CRDT- 2005/01/20 09:00
PHST- 2005/01/20 09:00 [pubmed]
PHST- 2005/03/25 09:00 [medline]
PHST- 2005/01/20 09:00 [entrez]
AID - S0039606004005410 [pii]
AID - 10.1016/j.surg.2004.06.049 [doi]
PST - ppublish
SO  - Surgery. 2004 Dec;136(6):1205-11. doi: 10.1016/j.surg.2004.06.049.