Pancreatoduodenal surgery in patients with multiple endocrine neoplasia type 1: Operative outcomes, long-term function, and quality of life

Background: Pancreatoduodenal (PD) neoplasms represent the principal disease-specific lethality in multiple endocrine neoplasia type 1 (MEN1). Potential oncologic benefits of PD resection must be weighed against operative morbidities, compromised pancreatic function, and quality of life (QOL). Methods: Fifty MEN1 patients underwent PD resections during 1984-2004. Postoperative pancreatic function and QOL were assessed by EORTC QLQ-C30 and a disease-specific questionnaire (response rate, 78%). Results: Twelve patients (24%) had asymptomatic disease detected by screening; 38 patients (76%) were symptomatic. All gross neoplasm was resected in 80% of patients. No patients died; 21 patients (42%) had complications. At 5 years postoperatively, 60% of patients were alive without disease, 24% of patients were with disease, 10% of patients died of PD neoplasms, 4% of patients died of other malignancies, and 2% of patients died of an unknown cause. Diabetes that requires insulin or oral hypoglycemics developed in 20% of patients. Frequent steatorrhea (>once/week) occurred in 25% of patients, early dumping occurred in 25% of patients, bloating occurred in 25% of patients, late dumping occurred in 7% of patients, hypoglycemia occurred in 7% of patients, and vomiting occurred in 4% of patients. Global QOL did not differ from that of the reference population (72.8 vs 75.3; P = .58). Conclusion: PD resections in MEN1 are associated with perioperative risks and altered pancreatic function. The moderate compromise in patient-perceived QOL suggests that most patients accept and adapt to these trade-offs for the potential of prolonged survival.