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Hereditary neuroendocrine tumors of the gastroenteropancreatic system

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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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Acknowledgment

This paper was supported by Hensel Stiftung, Kiel, Germany (F370011; MA and GK), Swiss National Foundation (SNF 31-108257; AP and PK). Nele Garbrecht and Tobias Henopp have a fellowship sponsored by Ipsen GmbH, Ettlingen, and Hensel-Stiftung, Kiel, Germany.

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  1. Department of Pathology, University of Kiel, Michaelisstr. 11, 24105, Kiel, Germany

    Martin Anlauf, Nele Garbrecht, Juliane Bauersfeld, Tobias Henopp & Günter Klöppel

  2. Department of Pathology, University Hospital Zürich, Zurich, Switzerland

    Anja Schmitt, Philipp U. Heitz & Aurel Perren

  3. Institute of Pathology, Triemli Spital, Zurich, Switzerland

    Paul Komminoth

  4. Department of Pathology, Munich University of Technology, Munich, Germany

    Aurel Perren

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Anlauf, M., Garbrecht, N., Bauersfeld, J. et al. Hereditary neuroendocrine tumors of the gastroenteropancreatic system. Virchows Arch 451 (Suppl 1), 29–38 (2007). https://doi.org/10.1007/s00428-007-0450-3

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  • DOI: https://doi.org/10.1007/s00428-007-0450-3

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