Timing and extent of surgery in symptomatic and asymptomatic neuroendocrine tumors of the pancreas in MEN 1

Pancreaticoduodenal tumors develop in a majority of patients with multiple endocrine neoplasia type 1 (MEN 1) and have a pronounced effect on life expectancy as the principal cause of disease related death. Previous discussion of therapy has focused mainly on syndromes of hormone excess and especial...

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Bibliographic Details
Published in:Langenbeck's archives of surgery 2002-03, Vol.386 (8), p.558-569
Main Authors: Akerström, Göran, Hessman, Ola, Skogseid, Britt
Format: Article
Language:English
Subjects:
Adenoma - etiology
Adenoma - pathology
Adenoma - surgery
Biomarkers, Tumor - metabolism
Duodenal Neoplasms - etiology
Duodenal Neoplasms - pathology
Duodenal Neoplasms - surgery
Glucagonoma - etiology
Glucagonoma - pathology
Glucagonoma - surgery
Humans
Hyperparathyroidism - diagnosis
Hyperparathyroidism - etiology
Insulinoma - etiology
Insulinoma - pathology
Insulinoma - surgery
Multiple Endocrine Neoplasia Type 1 - complications
Multiple Endocrine Neoplasia Type 1 - diagnosis
Multiple Endocrine Neoplasia Type 1 - genetics
Multiple Endocrine Neoplasia Type 1 - surgery
Pancreatic Neoplasms - etiology
Pancreatic Neoplasms - pathology
Pancreatic Neoplasms - surgery
Prognosis
Survival Analysis
Tomography, X-Ray Computed
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Summary:Pancreaticoduodenal tumors develop in a majority of patients with multiple endocrine neoplasia type 1 (MEN 1) and have a pronounced effect on life expectancy as the principal cause of disease related death. Previous discussion of therapy has focused mainly on syndromes of hormone excess and especially the management of MEN 1 associated Zollinger-Ellison syndrome (ZES). The syndromes of hormone excess, however, may be late features of the endocrinopathy and, when developed, indicate presence of metastases in more than one-third of patients. Recent possibilities for genetic diagnosis have emphasized requirements of prophylactic operation for prevention of malignant development. We recommend screening with biochemical markers and endoscopic ultrasound for early detection, and strong efforts of operative tumor removal before metastases have occurred. Surgery is generally recommended in patients with or without hormonal syndromes in the absence of spread hepatic metastases. Operative procedures include enucleation of tumors in the head of the pancreas, excision of duodenal gastrinomas together with clearance of lymph gland metastases, and as prophylaxis against tumor recurrence combination with distal 80% subtotal pancreatic resection. More extensive surgical tumor reduction is believed to reduce the risks for malignant progression of the pancreaticoduodenal tumors, but this requires further evaluation in MEN 1.
ISSN:1435-2443
1435-2451
DOI:10.1007/s00423-001-0274-6