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Familial Pancreatic Cancer
Cancers 2010, 2(4), 1901-1910; doi:10.3390/cancers2041901
Review

Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors

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Received: 19 October 2010; in revised form: 8 November 2010 / Accepted: 16 November 2010 / Published: 18 November 2010
(This article belongs to the Special Issue Pancreatic Cancer)
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Abstract: 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.
Keywords: pancreatic neuroendocrine tumors; genetics; pathogenesis; menin; MEN-1; VHL; NF-1; mTOR pancreatic neuroendocrine tumors; genetics; pathogenesis; menin; MEN-1; VHL; NF-1; mTOR
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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MDPI and ACS Style

Ehehalt, F.; Franke, E.; Pilarsky, C.; Grützmann, R. Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors. Cancers 2010, 2, 1901-1910.

AMA Style

Ehehalt F, Franke E, Pilarsky C, Grützmann R. Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors. Cancers. 2010; 2(4):1901-1910.

Chicago/Turabian Style

Ehehalt, Florian; Franke, Ellen; Pilarsky, Christian; Grützmann, Robert. 2010. "Molecular Pathogenesis of Pancreatic Neuroendocrine Tumors." Cancers 2, no. 4: 1901-1910.


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