Anlauf, Martin; Garbrecht, Nele; Bauersfeld, Juliane; Schmitt, Anja; Henopp, Tobias; Komminoth, Paul; Heitz, Philipp U.; Perren, Aurel; Klöppel, Günter (2007). Hereditary neuroendocrine tumors of the gastroenteropancreatic system. Virchows Archiv, 451(S1), pp. 29-38. Springer 10.1007/s00428-007-0450-3
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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.
Item Type: |
Journal Article (Original Article) |
|---|---|
Division/Institute: |
04 Faculty of Medicine > Service Sector > Institute of Pathology |
UniBE Contributor: |
Perren, Aurel |
Subjects: |
500 Science > 570 Life sciences; biology 600 Technology > 610 Medicine & health |
ISSN: |
0945-6317 |
Publisher: |
Springer |
Language: |
English |
Submitter: |
Aurel Perren |
Date Deposited: |
19 Feb 2016 15:16 |
Last Modified: |
05 Dec 2022 14:51 |
Publisher DOI: |
10.1007/s00428-007-0450-3 |
PubMed ID: |
17684762 |
BORIS DOI: |
10.48350/75131 |
URI: |
https://boris.unibe.ch/id/eprint/75131 |
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