Hereditary neuroendocrine tumors of the gastroenteropancreatic system

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:

04 Aug 2022 15:59

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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