| Literature DB >> 17684762 |
Martin Anlauf1, Nele Garbrecht, Juliane Bauersfeld, Anja Schmitt, Tobias Henopp, Paul Komminoth, Philipp U Heitz, Aurel Perren, Günter Klöppel.
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.Entities:
Mesh:
Year: 2007 PMID: 17684762 DOI: 10.1007/s00428-007-0450-3
Source DB: PubMed Journal: Virchows Arch ISSN: 0945-6317 Impact factor: 4.064