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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 Heitz; Günter Klöppel
- Abstract
Abstract  Approximately 5â10% 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 HippelâLindau 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.
- Subjects
NEUROENDOCRINE tumors; NEUROFIBROMATOSIS; NEUROFIBROMA; TUBEROUS sclerosis
- Publication
Virchows Archiv: European Journal of Pathology, 2007, Vol 451, Issue 0, p29
- ISSN
0945-6317
- Publication type
Article
- DOI
10.1007/s00428-007-0450-3