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).


CONCLUSIONS
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.
Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota 55905, USA.
Y Nancy
YN
You
Geoffrey B
GB
Thompson
William F
WF
Young
Dirk
D
Larson
David R
DR
Farley
Melanie
M
Richards
Clive S
CS
Grant
D000223:Adaptation, Psychological; D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D005500:Follow-Up Studies; D006306:Health Surveys; D006801:Humans; D007431:Intraoperative Complications; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000401:mortality / Q000523:psychology* / Q000601:surgery*; D016577:Pancreaticoduodenectomy; D011183:Postoperative Complications; D011788:Quality of Life; D011795:Surveys and Questionnaires; D015996:Survival Rate; D016896:Treatment Outcome