PMID- 11573043
OWN - NLM
STAT- MEDLINE
DCOM- 20011204
LR  - 20190514
IS  - 0003-4932 (Print)
IS  - 1528-1140 (Electronic)
IS  - 0003-4932 (Linking)
VI  - 234
IP  - 4
DP  - 2001 Oct
TI  - Comparison of surgical results in patients with advanced and limited disease with 
      multiple endocrine neoplasia type 1 and Zollinger-Ellison syndrome.
PG  - 495-505; discussion 505-6
AB  - OBJECTIVE: To determine the role of surgery in patients with Zollinger-Ellison 
      syndrome (ZES) and multiple endocrine neoplasia type 1 (MEN1) with either limited 
      or advanced pancreatic endocrine tumors (PETs). SUMMARY BACKGROUND DATA: The role 
      of surgery in patients with MEN1 and ZES is controversial. There have been 
      numerous previous studies of surgery in patients with PETs; however, there are no 
      prospective studies on the results of surgery in patients with advanced disease. 
      METHODS: Eighty-one consecutive patients with MEN1 and ZES were assigned to one 
      of four groups depending on the results of imaging studies. Group 1 (n = 17) (all 
      PETs smaller than 2.5 cm) and group 3 (n = 8) (diffuse liver metastases) did not 
      undergo surgery. All patients in group 2A (n = 17; single PET 2.5-6 cm [limited 
      disease]) and group 2B (n = 31; two or more lesions, 2.5 cm in diameter or 
      larger, or one lesion larger than 6 cm) underwent laparotomy. Tumors were 
      preferably removed by simple enucleation, or if not feasible resection. Patients 
      were reevaluated yearly. RESULTS: Pancreatic endocrine tumors were found in all 
      patients at surgery, with groups 2A and 2B having 1.7 +/- 0.4 and 4.8 +/- 1 PETs, 
      respectively. Further, 35% of the patients in group 2A and 88% of the patients in 
      group 2B had multiple PETs, 53% and 84% had a pancreatic PET, 53% and 68% had a 
      duodenal gastrinoma, 65% and 71% had lymph node metastases, and 0% and 12% had 
      liver metastases. Of the patients in groups 2A and 2B, 24% and 58% had a distal 
      pancreatectomy, 0% and 13% had a hepatic resection, 0% and 6% had a Whipple 
      operation, and 53% and 68% had a duodenal resection. No patient was cured at 5 
      years. There were no deaths. The early complication rate, 29%, was similar for 
      groups 2A and 2B. Mean follow-up from surgery was 6.9 +/- 0.8 years, and during 
      follow-up liver metastases developed in 6% of the patients in groups 2A and 2B. 
      Groups 1, 2A, and 2B had similar 15-year survival rates (89-100%); they were 
      significantly better than the survival rate for group 3 (52%). CONCLUSIONS: 
      Almost 40% of patients with MEN1 and ZES have advanced disease without diffuse 
      distant metastases. Despite multiple primaries and a 70% incidence of lymph node 
      metastases, tumor can be removed with no deaths and complication rates similar to 
      those in patients with limited disease. Further, despite previous studies showing 
      that patients with advanced disease have decreased survival rates, in this study 
      the patients with advanced tumor who underwent surgical resection had the same 
      survival as patients with limited disease and patients without identifiable 
      tumor. This suggests that surgical resection should be performed in patients with 
      MEN1 who have ZES and advanced localized PET.
FAU - Norton, J A
AU  - Norton JA
AD  - Department of Surgery, University of California, San Francisco, CA, USA.
FAU - Alexander, H R
AU  - Alexander HR
FAU - Fraker, D L
AU  - Fraker DL
FAU - Venzon, D J
AU  - Venzon DJ
FAU - Gibril, F
AU  - Gibril F
FAU - Jensen, R T
AU  - Jensen RT
LA  - eng
PT  - Comparative Study
PT  - Journal Article
PL  - United States
TA  - Ann Surg
JT  - Annals of surgery
JID - 0372354
SB  - IM
MH  - Adult
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Magnetic Resonance Imaging
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/diagnosis/mortality/*surgery
MH  - Pancreatic Neoplasms/diagnosis/mortality/*surgery
MH  - Probability
MH  - Prospective Studies
MH  - Severity of Illness Index
MH  - Statistics, Nonparametric
MH  - Surgical Procedures, Operative/*methods
MH  - Survival Rate
MH  - Tomography, X-Ray Computed
MH  - Treatment Outcome
MH  - Zollinger-Ellison Syndrome/diagnosis/mortality/*surgery
PMC - PMC1422073
EDAT- 2001/09/27 10:00
MHDA- 2002/01/05 10:01
PMCR- 2002/04/01
CRDT- 2001/09/27 10:00
PHST- 2001/09/27 10:00 [pubmed]
PHST- 2002/01/05 10:01 [medline]
PHST- 2001/09/27 10:00 [entrez]
PHST- 2002/04/01 00:00 [pmc-release]
AID - 0000658-200110000-00009 [pii]
AID - 10.1097/00000658-200110000-00009 [doi]
PST - ppublish
SO  - Ann Surg. 2001 Oct;234(4):495-505; discussion 505-6. doi: 
      10.1097/00000658-200110000-00009.