PMID- 19725300
OWN - NLM
STAT- MEDLINE
DCOM- 20091008
LR  - 20090903
IS  - 0003-1348 (Print)
IS  - 0003-1348 (Linking)
VI  - 75
IP  - 8
DP  - 2009 Aug
TI  - Long-term results of a selective surgical approach to management of 
      Zollinger-Ellison syndrome in patients with MEN-1.
PG  - 730-3
AB  - The role of operation in patients with Multiple Endocrine Neoplasia Type 1 
      (MEN-1) and Zollinger-Ellison Syndrome (ZES) is controversial. Our institutional 
      bias for this disease has, in general, been towards aggressive imaging and 
      operative removal of localized gastrinomas. Few studies have reported long-term 
      outcomes in patients with MEN-1 and ZES. A single institution retrospective 
      review of all patients with MEN-1 and ZES from 1970 to present was performed. 
      Twelve patients were identified (median age = 37 years at diagnosis). The median 
      follow-up was 18 years from diagnosis of ZES. Common symptoms associated with 
      gastrinoma in these patients were diarrhea (n = 6), abdominal pain (n = 4), and 
      nausea/vomiting (n = 4). Most commonly identified sites of gastrinoma were: 
      pancreas (n = 10), duodenum (n = 4), lymph nodes (n = 3), and liver (n = 1). 
      Fifteen celiotomies were performed in total (median = 1; range 0-3). Operative 
      procedures performed included: distal pancreatectomy (n = 4), acid reducing 
      procedure (n = 4), enucleation of pancreatic gastrinoma (n = 3), duodenal 
      resection (n = 3), pancreaticoduodenectomy (n = 1), and other (n = 7). One 
      patient had a transient biochemical cure after operation lasting 3 years. Only 
      one patient in this series had documented liver metastases of gastrinoma and no 
      patients expired of metastatic gastrinoma. There was one postoperative patient 
      death, secondary to respiratory arrest thought to be a result of aspiration or 
      pulmonary embolus. Three patients died of nondisease related causes, and seven 
      patients were alive at the time of last follow-up. Operations rarely result in 
      biochemical cures in patients with MEN-1 and ZES. In our experience, resection of 
      localized gastrinomas often did not require extended surgical resection and were 
      associated with excellent long-term outcomes.
FAU - Mortellaro, Vincent E
AU  - Mortellaro VE
AD  - Division of Surgical Oncology, Department of Surgery, University of Florida, 1600 
      SW Archer Road, Gainesville, Florida 32609, USA.
FAU - Hochwald, Steven N
AU  - Hochwald SN
FAU - McGuigan, James E
AU  - McGuigan JE
FAU - Copeland, Edward M
AU  - Copeland EM
FAU - Vogel, Stephen B
AU  - Vogel SB
FAU - Grobmyer, Stephen R
AU  - Grobmyer SR
LA  - eng
PT  - Journal Article
PL  - United States
TA  - Am Surg
JT  - The American surgeon
JID - 0370522
SB  - IM
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Cohort Studies
MH  - Disease-Free Survival
MH  - Female
MH  - Gastrinoma/complications/pathology/*surgery
MH  - Humans
MH  - Male
MH  - Middle Aged
MH  - Multiple Endocrine Neoplasia Type 1/*complications
MH  - Pancreatic Neoplasms/complications/pathology/*surgery
MH  - Patient Selection
MH  - Retrospective Studies
MH  - Time Factors
MH  - Treatment Outcome
MH  - Zollinger-Ellison Syndrome/complications/*pathology/*surgery
EDAT- 2009/09/04 06:00
MHDA- 2009/10/09 06:00
CRDT- 2009/09/04 09:00
PHST- 2009/09/04 09:00 [entrez]
PHST- 2009/09/04 06:00 [pubmed]
PHST- 2009/10/09 06:00 [medline]
PST - ppublish
SO  - Am Surg. 2009 Aug;75(8):730-3.