title: Extrapancreatic gastrinomas. Surgical experience.
abstract: OBJECTIVE
  Although widely reported on, the clinical diversity and eventual varied outcome of patients with extrapancreatic gastrinomas remain a medical mystery. In an attempt to help clarify conflicting management of extrapancreatic gastrinomas, we reviewed our experience with these unique tumors.
  
  
  METHODS
  Retrospective analysis with long-term follow-up (mean, 8 years).
  
  
  METHODS
  Tertiary care referral center.
  
  
  METHODS
  From January 1958 through January 1993, we identified and operated on 23 patients with extrapancreatic gastrinomas (duodenum, n = 18; stomach, n = 3; nodal, n = 2). The 12 men and 11 women (none with multiple endocrine neoplasia type I syndrome) ranged in age from 12 to 68 years (mean, 47 years). Preoperatively, all patients were symptomatic with peptic ulcer disease (duodenal [n = 18, 78%], jejunal [n = 4, 17%]) and/or diarrhea (n = 17, 74%).
  
  
  METHODS
  Preoperatively, tumor localization was successful in only three patients (13%). Surgical management included tumor excision only in 14 patients (61%), partial gastroduodenectomy in six (27%), total gastrectomy in one (4%), limited enterectomy in one (4%), and tumor biopsy alone in one (4%). Seven patients had evidence of lymphatic metastases at the time of operation, including a single patient with hepatic metastases (malignancy rate, 30%). Postoperatively, complications developed in seven patients (30%): wound infection in two, ileus in two, pulmonary sepsis in one, intra-abdominal abscess in one, and diabetic ketoacidosis in one. The postoperative mortality rate was 4%.
  
  
  METHODS
  Emphasis was placed on rendering patients eugastrinemic.
  
  
  RESULTS
  Long-term follow-up (mean, 8 years) of all patients revealed that 11 patients (48%) were eugastrinemic, asymptomatic, and not receiving gastric acid-reducing medication. Sixteen patients remain alive and well. Of the six now decreased patients who had been participating in long-term follow-up (mean survival, 14 years), death was due to atherosclerotic coronary artery disease in four and tumor progression in two.
  
  
  CONCLUSIONS
  Following surgical excision, patients with extrapancreatic gastrinomas have a favorable outcome, with nearly half being cured.
authors.affiliation: Department of Gastroenterologic and General Surgery, Mayo Clinic, Rochester, Minn.
authors.forename: D R
authors.initials: DR
authors.lastname: Farley
authors.forename: J A
authors.initials: JA
authors.lastname: van Heerden
authors.forename: C S
authors.initials: CS
authors.lastname: Grant
authors.forename: G B
authors.initials: GB
authors.lastname: Thompson
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002648:Child; D004379:Duodenal Neoplasms / Q000097:blood / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D005260:Female; D005500:Follow-Up Studies; D015408:Gastrinoma / Q000097:blood / Q000175:diagnosis / Q000556:secondary / Q000601:surgery*; D005755:Gastrins / Q000097:blood; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications / Q000401:mortality; D012086:Reoperation; D012189:Retrospective Studies; D013274:Stomach Neoplasms / Q000097:blood / Q000175:diagnosis / Q000473:pathology / Q000601:surgery*; D013514:Surgical Procedures, Operative / Q000379:methods; D015996:Survival Rate; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D005755:Gastrins