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Prophylaktische Chirurgie des Pankreas

Prophylactic pancreas surgery

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Zusammenfassung

Prophylaktische Operationen haben zum Ziel, die mit hoher Wahrscheinlichkeit entstehenden malignen Tumoren im Rahmen von vererbten Tumorprädispositionssyndromen zu verhindern. Das Pankreas stellt hier eine Besonderheit dar, da Pankreasoperationen chirurgisch anspruchsvoll und mit vergleichsweise hoher Morbidität und teilweise auch Mortalität belastet sind. Zudem sind die Auswirkungen von Pankreasresektionen, insbesondere der totalen Pankreatektomie mit exokriner Pankreasinsuffizienz und Briddle-Diabetes, für den Patienten erheblich. Vererbte Tumorprädispositionssyndrome, die zu endokrinen pankreatikoduodenalen Tumoren (PET) disponieren, sind das MEN1-Syndrom und das von Hippel-Lindau-Syndrom. Da die Penetranz allerdings maximal 70–80% beträgt und die Langzeitprognose der PETs mit einer 10-Jahres-Überlebensrate von über 80% vergleichsweise gut ist, ist eine prophylaktische Pankreasresektion ohne Tumornachweis nicht indiziert, allerdings ist eine prophylaktische Erweiterung des Resektionsausmaßes bei nachgewiesenen PETs sinnvoll. Zur Entwicklung eines exokrinen duktalen Pankreaskarzinoms (PC) prädisponieren das familiäre Pankreaskarzinomsyndrom (FPC), die hereditäre Pankreatitis sowie einige vererbte Tumorprädispositionssyndrome (z. B. Peutz-Jeghers-Syndrom, familiäres atypisches multiples Mole-Melanom-Syndrom etc.). Da beim familiären Pankreaskarzinomsyndrom der Hauptgendefekt unbekannt und bei allen anderen prädisponierenden Tumorsyndromen die Penetranz des PC niedrig oder noch unbekannt ist, ist eine prophylaktische Pankreatektomie nach heutigem Kenntnisstand nicht indiziert. Eine prophylaktische Ausweitung des Resektionsausmaßes bei nachgewiesenem PC bzw. höhergradiger PanIN-Läsionen erscheint sinnvoll, da bei betroffenen Patienten häufig multifokale Dysplasien bzw. Karzinome vorliegen.

Abstract

The goal of prophylactic surgery is to prevent malignant growth in patients with hereditary tumor predisposition. The pancreas presents as particularly challenging, due to the difficulty of operation and comparatively high risk of morbidity and even mortality. In addition, partial operative procedures and, more significantly, total resection lead to exocrine pancreas insufficiency and secondary diabetes, with grave consequences for the patient. Hereditary tumor predisposition syndromes that can result in pancreaticoduodenal endocrine tumors (PET) include multiple endocrine neoplasia type 1 syndrome and von Hippel-Lindau syndrome. As penetrance is maximally 70–80% and the 10-year survival rate over 80%, prophylactic pancreatic resection without evidence of a tumor is not indicated. However, prophylactic extension of a resection would be advised, should a PET be diagnosed. Patients predisposed to developing ductal pancreatic carcinoma (PC) are at risk of familial pancreatic cancer syndrome (FPC), hereditary pancreatitis, and other hereditary tumor predisposition syndromes such as Peutz-Jeghers syndrome and familial atypical multiple mole-melanoma syndrome. As the gene defect responsible for FPC has yet to be identified and the penetrance of PC in the other tumor predisposition syndromes is low or unknown, a prophylactic pancreatectomy based on today’s knowledge is not indicated. Prophylactic extension of the resection is advisable should PC or high-grade PanIN lesions be diagnosed, as these patients often present with multifocal dysplasia and even carcinoma.

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Authors and Affiliations

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

    P. Langer & M. Rothmund

  2. Klinik für Allgemein- und Visceralchirurgie der Städtischen Kliniken Bielefeld-Mitte

    D. K. Bartsch

  3. Klinik für Allgemein- und Visceralchirurgie, Städtische Kliniken Bielefeld-Mitte, Teutoburger Straße 50, 33604 , Bielefeld

    D. K. Bartsch

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  1. P. Langer
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Correspondence to D. K. Bartsch.

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Langer, P., Rothmund, M. & Bartsch, D.K. Prophylaktische Chirurgie des Pankreas. Chirurg 77, 25–32 (2006). https://doi.org/10.1007/s00104-005-1113-z

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