Abstract
Background. The management of pancreaticoduodenal endocrine tumors (PETs) remains controversial
in multiple endocrine neoplasia type 1 (MEN 1). Methods. Twenty-one patients with MEN 1 and PETs were analyzed for outcome of surgery and
surveillance with special regard to the genotype based on MEN1 gene mutation analysis.
Results. Nine patients had gastrinomas, 5 had nonfunctioning tumors, 4 had insulinomas, 2
had insulinomas and gastrinomas, and 1 had a VIPoma. Seven patients (33%) had malignant
tumors. Sixteen patients (76%) were initially treated by pancreatic resections or
tumor enucleations or both. Six patients underwent reoperations for recurrences or
lymph node metastases or both. Fifteen of the 16 operated patients are alive, and
12 have no evidence of disease after a median follow-up of 78 months (range, 1-198
months). Five patients with gastrinomas or nonfunctioning tumors, but no symptoms,
underwent surveillance; 1 of them developed lymph node metastases. Patients with truncating
mutations in the N- or C-terminal region (exons 2, 9, or 10) of the MEN1 gene had
a significantly higher rate of malignant tumors (55% vs 10%; P <.05) than patients with other mutations. Conclusions. An aggressive surgical approach is justified for PETs in patients with MEN 1. However,
MEN1 gene mutations in exons 3 to 8 seem to be associated with mild behavior of PETs,
possibly allowing surveillance in asymptomatic patients. (Surgery 2000;128:958-66.)
To read this article in full you will need to make a payment
Purchase one-time access:
Academic & Personal: 24 hour online accessCorporate R&D Professionals: 24 hour online accessOne-time access price info
- For academic or personal research use, select 'Academic and Personal'
- For corporate R&D use, select 'Corporate R&D Professionals'
Subscribe:
Subscribe to SurgeryAlready a print subscriber? Claim online access
Already an online subscriber? Sign in
Register: Create an account
Institutional Access: Sign in to ScienceDirect
References
- Positional cloning of the gene for multiple endocrine neoplasia type 1.Science. 1997; 276: 404-406
- Endocrine adenomatosis and peptic ulcer in a large kindred.Am J Med. 1963; 35: 205-208
- Multiple endocrine neoplasia type 1: a 10-year prospective screening in four kindreds.J Clin Endocrinol Metab. 1991; 73: 281-287
- Management of islet cell tumors in patients with multiple endocrine neoplasia: a prospective study.Surgery. 1989; 106: 1108-1117
- Lethality of multiple endocrine neoplasia type 1.World J Surg. 1998; 22: 581-587
- Current approach to the management of gastrinoma and insulinoma in adults with multiple endocrine neoplasia I.World J Surg. 1993; 17: 489-497
- Management of the Zollinger-Ellison syndrome in patients with multiple endocrine neoplasia type 1.Surgery. 1986; 100: 971-976
- Prospective study of the clinical course, prognostic factors, causes of death, and survival in patients with long-standing Zollinger-Ellison syndrome.J Clin Oncol. 1999; 17: 615-630
- Surgery for asymptomatic pancreatic lesion in multiple endocrine neoplasia type I.World J Surg. 1996; 20: 872-877
- Current concepts in the surgical management of multiple endocrine neoplasia type 1 pancreatic-duodenal disease. Results in the treatment of 40 patients with Zollinger-Ellison syndrome, hypoglycaemia or both.J Intern Med. 1998; 243: 495-1450
- Gastrinomas in the duodenums of patients with multiple endocrine neoplasia type 1 and the Zollinger-Ellison syndrome.N Engl J Med. 1990; 322: 723-727
- Total pancreatectomy in the MEN-1 syndrome.Br J Surg. 1988; 75: 154-157
- The surgical treatment of gastrinoma in MEN 1 syndrome patients.Surgery. 1989; 106: 1081-1086
- MEN 1 gene mutations in 12 MEN 1 families and their associated tumors.Eur J Endocrinol. 1998; 139: 416-420
- Surgery to cure the Zollinger-Ellison syndrome.N Engl J Med. 1999; 341: 635-644
- Prognostic factors in patients with Zollinger-Ellison syndrome and multiple endocrine neoplasia type I.Gastroenterology. 1999; 116: 286-293
- Surgery for Zollinger-Ellison syndrome alters the natural history of gastrinoma.Ann Surg. 1994; 220: 320-330
- Treatment of gastrinomas with pancreatoduo-denectomy.in: Endocrine tumors of the pancreas: recent advances in research and management. Switzerland: Karger, Basal1995: 333-341 (Vol 23 of Frontiers of gastrointestinal research)
- Role of pancreaticoduodenectomy in the management of primary duodenal wall gastrinomas in patients with Zollinger-Ellison syndrome.Surgery. 1995; 112: 1016-1023
- The surgical management of patients with multiple endocrine neoplasia type 1.J Clin Endocrinol Metab. 1997; 82: 359-361
- Surgical management of insulinoma associated with multiple endocrine neoplasia type I.World J Surg. 1994; 18: 488-494
- Insulinomas associated with multiple endocrine neoplasia type I: the need for a different surgical approach.Surgery. 1991; 110: 998-1005
- Menin interacts with the AP1 transcription factor JunD and represses JunD-activated transcription.Cell. 1999; 96: 143-152
- Germ-line mutation analysis in patients with multiple endocrine neoplasia type 1 and related disorders.Am J Hum Genet. 1998; 63: 455-467
- Recommendations for a nomenclature system for human gene mutations.Hum Mutat. 1998; 11: 1-3
Article info
Footnotes
*Supported by a grant from the Else-Kröner-Fresenius-Stiftung (D. K. B.).
**Reprint requests: Detlef K. Bartsch, Department of Surgery, Philipps-University Marburg, Baldingerstrasse, 35033 Marburg/Germany.
★Surgery 2000;128:958-66.
Identification
Copyright
© 2000 Mosby, Inc. Published by Elsevier Inc. All rights reserved.
