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Hereditäre duodenopankreatische neuroendokrine Neoplasien

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Viszeral- und Allgemeinchirurgie

Part of the book series: Springer Reference Medizin ((SRM))

Zusammenfassung

Etwa 5–10 % der duodenopankreatischen neuroendokrinen Neoplasien (dpNEN) treten im Zusammenhang mit einem erblichen Tumorsyndrom auf. Die häufigste zugrundeliegende Erkrankung ist die multiple endokrine Neoplasie Typ 1 (MEN1). Weitere assoziierte Tumorsyndrome sind das Von-Hippel-Lindau-Syndrom (VHL), die Neurofibromatose Typ 1, die tuberöse Sklerose, die MEN4, die Glukagonzellhyperplasie/-neoplasie (GCHN) und die adulte Insulinomatose. Die dpNEN können nichtfunktionell oder durch unkontrollierte Hormonsekretion funktionell aktiv sein. Aufgrund der geringen Inzidenz hereditärer dpNEN beruhen die Empfehlungen zur Operationsindikation und -verfahrenswahl in Leitlinien ausschließlich auf retrospektiven Fallserien und Expertenmeinungen. Eine operative Therapie ist bei nichtfunktionellen dpNEN >2 cm, bei Malignitätsverdacht und bei funktionell aktiven dpNEN indiziert. Bei den meist jungen Patienten sollte neben dem onkologischen Ergebnis auch dem Erhalt der Pankreasfunktion und Lebensqualität hohe Priorität eingeräumt werden.

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Authors and Affiliations

  1. Klinik für Visceral-, Thorax- und Gefäßchirurgie, Philipps-Universität Marburg, Marburg, Deutschland

    Max B. Albers, Jerena Manoharan & Detlef K. Bartsch

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  1. Max B. Albers
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  2. Jerena Manoharan
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  3. Detlef K. Bartsch
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Correspondence to Max B. Albers .

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Editors and Affiliations

  1. Allgemein- Viszeral- u. Gefäßchirurgie, Charité Universitätsmedizin Berlin, Berlin, Berlin, Deutschland

    Martin Kreis

  2. Klinik für Visceral-, Thorax- und G, Universitätsklinikum Marburg, Marburg, Hessen, Deutschland

    Detlef K. Bartsch

  3. Klinik & Poliklinik Allgemein & Abdomin, Universitätsklinikum Mainz, Mainz, Rheinland-Pfalz, Deutschland

    Hauke Lang

Section Editor information

  1. Klinik für Visceral-, Thorax- und Gefäßchirurgie, Philipps-Universität Marburg, Marburg, Deutschland

    Detlef K. Bartsch

  2. Klinik für Visceral-, Thorax- und Gefäßchirurgie, Philipps-Universität Marburg, Marburg, Deutschland

    Katharina Holzer

1 Elektronisches Zusatzmaterial

Duodenumexploration und submuköse Enukleation duodenaler Gastrinome bei MEN1-assoziiertem ZES (MP4 641682 kb)

Roboterassistierte milz- und milzgefäßerhaltende distale Pankreasresektion bei MEN1-assoziierten NF-pNEN (MP4 633491 kb)

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Albers, M.B., Manoharan, J., Bartsch, D.K. (2022). Hereditäre duodenopankreatische neuroendokrine Neoplasien. In: Kreis, M., Bartsch, D.K., Lang, H. (eds) Viszeral- und Allgemeinchirurgie. Springer Reference Medizin. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-61724-3_45-1

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