Duodenopancreatic resections in patients with multiple endocrine neoplasia type 1.
OBJECTIVE
To review the authors' 7-year experience with a surgical approach for pancreatic and duodenal neuroendocrine tumors (NETs) in patients with multiple endocrine neoplasia type 1 (MEN 1) designed to remove all gross tumor with limited complications, preserving pancreatic function.


BACKGROUND
MEN 1 is an autosomal dominant familial neoplasia syndrome characterized by the development of NETs of the duodenum and pancreas. Some tumors are clinically insignificant or follow a benign course, although a subset pursues a malignant, lethal natural history; the risk of surgical management must be appropriate to the disease course.


METHODS
The clinical, biochemical, genetic, and pathologic data were retrospectively reviewed for 21 consecutive MEN 1 patients undergoing pancreatic resection for NETs between 1993 and 1999 at one institution. Age at operation, presenting symptoms, results of preoperative and intraoperative localization studies, major and minor complications, and pathology, including metastases, were analyzed.


RESULTS
The surgical approach was selected based on the location and size of the tumors. Five patients required pancreaticoduodenectomy, 11 patients underwent non-Whipple pancreatic resections, and 5 underwent simple enucleation of benign NETs. The incidence of regional lymph node metastases was 33%.


CONCLUSIONS
Major pancreatic procedures can be performed safely in most patients with MEN 1 and NETs. Because NETs are the most common MEN 1-related cause of death in the authors' kindreds, an aggressive surgical approach, including early intervention before malignant spread and major pancreatic resection where indicated, appears justified.
Lairmore
T C
TC
Section of Endocrine and Oncologic Surgery, Washington University School of Medicine, St. Louis, Missouri 63110, USA. lairmoret@msnotes.wustl.edu
Chen
V Y
VY
DeBenedetti
M K
MK
Gillanders
W E
WE
Norton
J A
JA
Doherty
G M
GM
D000328:Adult; D000368:Aged; D004379:Duodenal Neoplasms / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D005260:Female; D016368:Frameshift Mutation; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D008875:Middle Aged; D018761:Multiple Endocrine Neoplasia Type 1 / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D020125:Mutation, Missense; D010190:Pancreatic Neoplasms / Q000235:genetics / Q000473:pathology / Q000601:surgery*; D012189:Retrospective Studies