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Duodenopancreatic resections in patients with multiple endocrine neoplasia type 1
T C Lairmore1, V Y Chen, M K DeBenedetti
1Section of Endocrine and Oncologic Surgery, Washington University School of Medicine, St. Louis, Missouri 63110, USA. lairmoret@msnotes.wustl.edu
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.
Summary Background Data:
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.
Insights
Surgical management of pancreatic and duodenal neuroendocrine tumors (NETs) in patients with multiple endocrine neoplasia type 1 (MEN 1) is safe and effective. An aggressive surgical approach, including early intervention, is justified due to the high mortality associated with MEN 1-related NETs.
Area of Science:
- Endocrinology
- Surgical Oncology
- Genetics
Background:
- Multiple Endocrine Neoplasia type 1 (MEN 1) is an autosomal dominant syndrome.
- It is characterized by duodenal and pancreatic neuroendocrine tumors (NETs).
- While some NETs are benign, others can be malignant and lethal.
Purpose of the Study:
- To review a 7-year surgical experience with pancreatic and duodenal NETs in MEN 1 patients.
- To assess a surgical approach aimed at complete tumor removal with minimal complications and preserved pancreatic function.
Main Methods:
- Retrospective review of clinical, biochemical, genetic, and pathologic data.
- Analysis of 21 consecutive MEN 1 patients undergoing pancreatic resection for NETs (1993-1999).
- Evaluation of age, symptoms, localization studies, complications, and pathology, including metastases.
Main Results:
- Surgical approach varied based on tumor location and size.
- Procedures included pancreaticoduodenectomy (5 patients), non-Whipple resections (11 patients), and enucleation (5 patients).
- Regional lymph node metastases were observed in 33% of patients.
Conclusions:
- Major pancreatic procedures for MEN 1-related NETs can be performed safely.
- NETs are a leading cause of death in MEN 1 kindreds.
- An aggressive surgical strategy, including early intervention and resection when indicated, is warranted.