EXTRAPANCREATIC GASTRINOMAS - SURGICAL EXPERIENCE

Objective: Although widely reported on, the clinical diversity and eve ntual varied outcome of patients with extrapancreatic gastrinomas rema in a medical mystery. In an attempt to help clarify conflicting manage ment of extrapancreatic gastrinomas, we reviewed our experience with t hese unique tumors. Design: Retrospective analysis with long-term foll ow-up (mean, 8 years). Setting: Tertiary care referral center. Patient s: From January 1958 through January patients with extrapancreatic gas trinomas (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 pati ents were symptomatic with peptic ulcer disease (duodenal [n=18, 78%], jejunal [n=4, 17%]) and/or diarrhea (n=17,74%). Intervention: Preoper atively, 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 enterectomoy 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 (mali gnancy rate, 30%). Postoperatively, complications developed in seven p atients (30%): wound infection in two, ileus in two, pulmonary sepsis in one, intra-abdominal abscess in one, and diabetic ketoacidosis in o ne. The postoperative mortality rate was 4%. Main Outcome Measure: Emp hasis was placed on rendering patients eugastrinemic.Results: Long-ter m 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 s ix now deceased patients who had been participating in long-term follo w-up (mean survival, 14 years), death was due to atherosclerotic coron ary artery disease in four and tumor progression in two. Conclusion: F ollowing surgical excision, patients with extrapancreatic gastrinomas have a favorable outcome, with nearly half being cured.