Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1

Pancreatico-duodenal tumors are the second most common endocrinopathy in multiple endocrine neoplasia syndrome type 1, and have a pronounced effect on life expectancy as the principal cause of disease-related death. Previous discussions about surgical management have focused mainly on syndromes of h...

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Main Authors: Göran Åkerström, Peter Stålberg, Per Hellman
Format: Article
Language:English
Published: Elsevier España 2012-01-01
Series:Clinics
Subjects:
Online Access:http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322012001300029
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author Göran Åkerström
Peter Stålberg
Per Hellman
author_facet Göran Åkerström
Peter Stålberg
Per Hellman
author_sort Göran Åkerström
collection DOAJ
description Pancreatico-duodenal tumors are the second most common endocrinopathy in multiple endocrine neoplasia syndrome type 1, and have a pronounced effect on life expectancy as the principal cause of disease-related death. Previous discussions about surgical management have focused mainly on syndromes of hormone excess and, in particular, the management of multiple endocrine neoplasia syndrome type 1-related Zollinger-Ellison syndrome. Since hormonal syndromes tend to occur late and indicate the presence of metastases, screening with biochemical markers and endoscopic ultrasound is recommended for early detection of pancreatico-duodenal tumors, and with early surgery before metastases have developed. Surgery is recommended in patients with or without hormonal syndromes in the absence of disseminated liver metastases. The suggested operation includes distal 80% subtotal pancreatic resection together with enucleation of tumors in the head of the pancreas, and in cases with Zollinger-Ellison syndrome, excision of duodenal gastrinomas together with clearance of regional lymph node metastases. This strategy, with early and aggressive surgery before metastases have developed, is believed to reduce the risks for tumor recurrence and malignant progression.
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spelling doaj.art-1e60572640864fb7be65cc37443531ce2022-12-22T01:19:15ZengElsevier EspañaClinics1807-59321980-53222012-01-016717317810.6061/clinics/2012(Sup01)29Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1Göran ÅkerströmPeter StålbergPer HellmanPancreatico-duodenal tumors are the second most common endocrinopathy in multiple endocrine neoplasia syndrome type 1, and have a pronounced effect on life expectancy as the principal cause of disease-related death. Previous discussions about surgical management have focused mainly on syndromes of hormone excess and, in particular, the management of multiple endocrine neoplasia syndrome type 1-related Zollinger-Ellison syndrome. Since hormonal syndromes tend to occur late and indicate the presence of metastases, screening with biochemical markers and endoscopic ultrasound is recommended for early detection of pancreatico-duodenal tumors, and with early surgery before metastases have developed. Surgery is recommended in patients with or without hormonal syndromes in the absence of disseminated liver metastases. The suggested operation includes distal 80% subtotal pancreatic resection together with enucleation of tumors in the head of the pancreas, and in cases with Zollinger-Ellison syndrome, excision of duodenal gastrinomas together with clearance of regional lymph node metastases. This strategy, with early and aggressive surgery before metastases have developed, is believed to reduce the risks for tumor recurrence and malignant progression.http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322012001300029Multiple Endocrine Neoplasia Type 1Neuroendocrine Pancreatico-Duodenal TumorsSurgical Treatment
spellingShingle Göran Åkerström
Peter Stålberg
Per Hellman
Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1
Clinics
Multiple Endocrine Neoplasia Type 1
Neuroendocrine Pancreatico-Duodenal Tumors
Surgical Treatment
title Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1
title_full Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1
title_fullStr Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1
title_full_unstemmed Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1
title_short Surgical management of pancreatico-duodenal tumors in multiple endocrine neoplasia syndrome type 1
title_sort surgical management of pancreatico duodenal tumors in multiple endocrine neoplasia syndrome type 1
topic Multiple Endocrine Neoplasia Type 1
Neuroendocrine Pancreatico-Duodenal Tumors
Surgical Treatment
url http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1807-59322012001300029
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