Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors
Pancreatic neuroendocrine tumors (PNETs) are rare primary neoplasms of the pancreas and arise sporadically or in the context of genetically determined syndromes. Depending on hormone production and sensing, PNETs clinically manifest due to a hormone-related syndrome (functional PNET) or by symptoms...
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2010-11-01
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Online Access: | http://www.mdpi.com/2072-6694/2/4/1901/ |
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doaj-4c85a0bfedb04d8baa5e382b1e782c872020-11-25T00:58:59ZengMDPI AGCancers2072-66942010-11-01241901191010.3390/cancers2041901Molecular Pathogenesis of Pancreatic Neuroendocrine TumorsRobert GrützmannFlorian EhehaltEllen FrankeChristian PilarskyPancreatic neuroendocrine tumors (PNETs) are rare primary neoplasms of the pancreas and arise sporadically or in the context of genetically determined syndromes. Depending on hormone production and sensing, PNETs clinically manifest due to a hormone-related syndrome (functional PNET) or by symptoms related to tumor bulk effects (non-functional PNET). So far, radical surgical excision is the only therapy to cure the disease. Development of tailored non-surgical approaches has been impeded by the lack of experimental laboratory models and there is, therefore, a limited understanding of the complex cellular and molecular biology of this heterogeneous group of neoplasm. This review aims to summarize current knowledge of tumorigenesis of familial and sporadic PNETs on a cellular and molecular level. Open questions in the field of PNET research are discussed with specific emphasis on the relevance of disease management. http://www.mdpi.com/2072-6694/2/4/1901/pancreatic neuroendocrine tumorsgeneticspathogenesismeninMEN-1VHLNF-1mTOR |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Robert Grützmann Florian Ehehalt Ellen Franke Christian Pilarsky |
spellingShingle |
Robert Grützmann Florian Ehehalt Ellen Franke Christian Pilarsky Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors Cancers pancreatic neuroendocrine tumors genetics pathogenesis menin MEN-1 VHL NF-1 mTOR |
author_facet |
Robert Grützmann Florian Ehehalt Ellen Franke Christian Pilarsky |
author_sort |
Robert Grützmann |
title |
Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors |
title_short |
Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors |
title_full |
Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors |
title_fullStr |
Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors |
title_full_unstemmed |
Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors |
title_sort |
molecular pathogenesis of pancreatic neuroendocrine tumors |
publisher |
MDPI AG |
series |
Cancers |
issn |
2072-6694 |
publishDate |
2010-11-01 |
description |
Pancreatic neuroendocrine tumors (PNETs) are rare primary neoplasms of the pancreas and arise sporadically or in the context of genetically determined syndromes. Depending on hormone production and sensing, PNETs clinically manifest due to a hormone-related syndrome (functional PNET) or by symptoms related to tumor bulk effects (non-functional PNET). So far, radical surgical excision is the only therapy to cure the disease. Development of tailored non-surgical approaches has been impeded by the lack of experimental laboratory models and there is, therefore, a limited understanding of the complex cellular and molecular biology of this heterogeneous group of neoplasm. This review aims to summarize current knowledge of tumorigenesis of familial and sporadic PNETs on a cellular and molecular level. Open questions in the field of PNET research are discussed with specific emphasis on the relevance of disease management. |
topic |
pancreatic neuroendocrine tumors genetics pathogenesis menin MEN-1 VHL NF-1 mTOR |
url |
http://www.mdpi.com/2072-6694/2/4/1901/ |
work_keys_str_mv |
AT robertgrutzmann molecularpathogenesisofpancreaticneuroendocrinetumors AT florianehehalt molecularpathogenesisofpancreaticneuroendocrinetumors AT ellenfranke molecularpathogenesisofpancreaticneuroendocrinetumors AT christianpilarsky molecularpathogenesisofpancreaticneuroendocrinetumors |
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