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Zentrales Gallengangskarzinom (Klatskin-Tumor)

Perihilar cholangiocarcinoma (Klatskin tumor)

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Zusammenfassung

Das perihiläre Gallengangskarzinom (Klatskin-Tumor) ist ein seltener Tumor ausgehend von der extrahepatischen Gallengangsgabel. In Anbetracht der anatomisch engen Beziehung der Gallengangsgabel zu Leberparenchym, Pfortadergabel und auch Leberarterien bedeutet die Behandlung dieser Patienten eine große Herausforderung. Mit einer Inzidenz von 2 bis 4 Erkrankungen/100.000 Einwohner/Jahr ist eine Behandlung nur an Zentren sinnvoll, die über eine entsprechende Erfahrung verfügen. Histologisch liegt meist ein mäßig differenziertes Adenokarzinom vor, das diffus infiltrierend entlang der Gallenwege und Perineuralscheide nach proximal, aber auch nach distal wachsen kann. Als einzige kurative Option hat sich die radikale chirurgische Resektion von Gallenwegen, Gallengangsgabel en bloc mit Leberresektion und auch die Gefäßresektion durchgesetzt, dennoch ist das Erreichen eines proximalen und lateralen Sicherheitsabstands vom Tumor technisch problematisch.

Abstract

Perihilar cholangiocarcinoma or Klatskin tumors are a rare entity arising from the extrahepatic bile duct bifurcation. Considering the close anatomical relationship of the bile duct bifurcation with the portal vein bifurcation and hepatic arteries, surgical treatment is demanding. With an incidence of only 2–4 cases/100,000 population/year patients should be referred to a specialized center. The tumors are usually poorly differentiated adenocarcinomas growing diffusely along the duct and also the perineural sheath. Only radical surgery offers a curative option and currently surgical strategy usually consists of en bloc resection of the bile duct, extended liver resection and portal vein resection. Proximal and lateral safety margin R0 resections are technically very demanding procedures because of the local anatomy.

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Abbreviations

A./Aa.:

Arteria/Arteriae

ALPPS:

associated liver partition and portal vein ligation for staged hepatectomy

CA 19-9:

Carboanyhdrat-Antigen 19-9

CCC:

cholangiozelluläre Karzinome

CEA:

karzinoembryonales Antigen

CT:

Computertomographie

ERCP:

endoskopische retrograde Cholangiopankreatikographie

IORT:

intraoperative Radiotherapie

MRCP:

Magnetresonanzcholangiopankreatikographie

MRT:

Magnetresonanztomographie

PET:

Positronenemissionstomographie

PSC:

primär sklerosierende Cholangitis

PTCD:

perkutane transhepatische Cholangiodrainage

PVE:

portalvenöse Embolisation

V. /Vv.:

Vena/Venae

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Danksagung

Die Autoren sind der Künstlerin Frau Prof. Giuliana Brogi (Siena, Italien) für die Erstellung der Abb. 1 außerordentlich dankbar.

Einhaltung ethischer Richtlinien

Interessenkonflikt. G.A. Stavrou, M. Donati, S. Faiss, R.M. Jenner, K.J. Niehaus und K.J. Oldhafer geben an, dass kein Interessenkonflikt besteht. Dieser Beitrag beinhaltet keine Studien an Menschen oder Tieren.

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Authors and Affiliations

  1. Klinik für Allgemein- und Viszeralchirurgie, Asklepios Klinik Barmbek, Medizinische Fakultät der Semmelweis Universität, Asklepios Campus Hamburg, Rübenkamp 220, 22291, Hamburg, Deutschland

    G.A. Stavrou, M. Donati, R.M. Jenner, K.J. Niehaus &  K.J. Oldhafer

  2. Klinik für Gastroenterologie und Interventionelle Endoskopie,Asklepios Klinik Barmbek , Medizinische Fakultät der Semmelweis Universität, Asklepios Campus Hamburg, Hamburg, Deutschland

    S. Faiss

  3. Department Chirurgie, Universität Catania, Catania, Italien

    M. Donati

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  1. G.A. Stavrou
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  2. M. Donati
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  3. S. Faiss
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  4. R.M. Jenner
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  5. K.J. Niehaus
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  6. K.J. Oldhafer
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Correspondence to K.J. Oldhafer.

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Stavrou, G., Donati, M., Faiss, S. et al. Zentrales Gallengangskarzinom (Klatskin-Tumor). Chirurg 85, 155–168 (2014). https://doi.org/10.1007/s00104-012-2390-y

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