Extrapancreatic Gastrinomas

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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.Design:Retrospective analysis with long-term follow-up (mean, 8 years).Setting:Tertiary care referral center.Patients: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%).Intervention: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%.Main Outcome Measure: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 deceased 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.Conclusion:Following surgical excision, patients with extrapancreatic gastrinomas have a favorable outcome, with nearly half being cured.(Arch Surg. 1994;129:506-512)