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01-08-2007 | Review and Perspective

Hereditary neuroendocrine tumors of the gastroenteropancreatic system

Authors: Martin Anlauf, Nele Garbrecht, Juliane Bauersfeld, Anja Schmitt, Tobias Henopp, Paul Komminoth, Philipp U. Heitz, Aurel Perren, Günter Klöppel

Published in: Virchows Archiv | Special Issue 1/2007

Abstract

Approximately 5–10% of neuroendocrine tumors (NETs) of the gastroenteropancreatic system (GEP) have a hereditary background. The known inherited syndromes include multiple endocrine neoplasia type 1, neurofibromatosis type 1, von Hippel–Lindau disease, and the tuberous sclerosis complex. This review discusses for each of these syndromes the: (1) involved genes and specific types of mutations, (2) disease prevalence and penetrance, (3) affected neuroendocrine tissues and related clinical syndromes, (4) special morphological features of NETs and their putative precursor lesions. In addition, GEP-NETs clustering in individual families or associated with other malignancies without known genetic background are discussed.

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Title: Hereditary neuroendocrine tumors of the gastroenteropancreatic system
Authors: Martin Anlauf
Nele Garbrecht
Juliane Bauersfeld
Anja Schmitt
Tobias Henopp
Paul Komminoth
Philipp U. Heitz
Aurel Perren
Günter Klöppel
Publication date: 01-08-2007
Publisher: Springer-Verlag
Published in: Virchows Archiv / Issue Special Issue 1/2007
Print ISSN: 0945-6317
Electronic ISSN: 1432-2307
DOI: https://doi.org/10.1007/s00428-007-0450-3

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Hereditary neuroendocrine tumors of the gastroenteropancreatic system

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Keynote webinar | Spotlight on medication adherence

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