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- Title
Surgery versus surveillance of pancreaticoduodenal endocrine tumours in multiple endocrine neoplasia type 1.
- Authors
Bartsch, D. K.; Langer, P.; Schilling, T.; Wild, A.; Celik, I.; Nies, C.; Rothmund, M.
- Abstract
Background: Management of pancreaticoduodenal endocrine tumours remains controversial in multiple endocrine neoplasia type 1 (MEN1). The authors' experience with MEN1-related pancreaticoduodenal tumours has been reviewed in order to evaluate a rational therapeutic approach. Methods: Twenty-one patients with MEN1 with pancreaticoduodenal endocrine tumours were analysed with special regard to outcome of surgery and surveillance. In addition, a phenotype/genotype analysis was performed based on MEN1 gene mutation analysis. Results: Twelve of 21 patients had multiple tumours. Nine patients had gastrinomas, six non-functioning tumours, three insulinomas, two insulinomas and gastrinomas, and one had a vipoma. Six tumours were malignant with lymph node metastases (five gastrinomas, one vipoma). Seventeen patients were treated initially by either pancreatic left resection with tumour enucleation of the duodenum and/or pancreatic head (n = 9), tumour enucleation alone (n = 7) or pylorus-preserving partial pancreatectomy (PPPD; n = 1). Sixteen of 17 patients were cured biochemically after initial surgery and 11 remained free of disease after a median follow-up of 55 (range 2–197) months. Six patients underwent reoperation for recurrence and/or lymph node metastases, including two PPPDs in patients with gastrinoma. All six patients remained asymptomatic and three remained biochemically free of disease. Three of the 17 operated patients died from either unrelated causes (n = 2) or postoperative complications (n = 1). On the other hand, four asymptomatic patients with either gastrinomas (n = 2) or non-functioning tumours (n = 2) were closely surveyed and showed no progress or metastases on somatostatin (SMS) scans and computed tomography after 9–110 months. The genotype/phenotype analysis revealed that patients with truncating nonsense or frameshift MEN1 gene mutations in the N- or C-terminal regions (exons 2, 9 or 10) had a significantly higher rate of m...
- Subjects
PANCREATIC tumors; SURGERY -- Evaluation
- Publication
British Journal of Surgery, 2000, Vol 87, Issue 9, p1256
- ISSN
0007-1323
- Publication type
Abstract
- DOI
10.1046/j.1365-2168.2000.01601-16.x