Fard-Aghaie, Mohammad H., Makridis, Georgios, Reese, Tim, Feyerabend, Bernd, Wagner, Kim C., Schnitzbauer, Andreas, Bechstein, Wolf O., Oldhafer, Felix, Kleine, Moritz, Klempnauer, Jurgen, Rolinger, Jens, Nadalin, Silvio, Konigsrainer, Alfred, Vassos, Nikolaos, Grutzmann, Robert, Benko, Tamas, Paul, Andreas, Li, Jun, Fischer, Lutz, Beaumont, Katharina, Nussler, Natascha, Fahrner, Rene, Settmacher, Utz, Fichtner-Feigl, Stefan, Schoning, Wenzel, Pratschke, Johann, Eckhoff, Jennifer A., Wahba, Roger, Bruns, Christiane, Bernsmeier, Alexander, Braun, Felix, Becker, Thomas, Lurje, Georg ORCID: 0000-0001-9674-0756, Neumann, Ulf P., Dohmen, Jonas, Manekeller, Steffen, Kalff, Jorg C., Mehrabi, Arianeb, Buchler, Markus W., Lang, Sven A., Schlitt, Hans J., Tripke, Verena, Lang, Hauke and Oldhafer, Karl J. (2022). The rate of cholangiocarcinoma in Caroli Disease A German multicenter study. HPB, 24 (2). S. 267 - 277. OXFORD: ELSEVIER SCI LTD. ISSN 1477-2574

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Abstract

Background: Caroli Disease (CD) and Caroli Syndrome (CS) are rare disorders presenting with dilation of the intrahepatic bile ducts. CD/CS are associated with cholangiocarcinoma (CCA). However, the true incidence of CCA is still unclear, although it may serve as an indication for surgery. In this paper, we analyzed (I) the incidence of CCA in German centers, (II) reviewed our single center population together with its clinical presentation and (III) performed a thorough literature review. Methods: 17 large HPB-centers across Germany were contacted and their patients after surgical treatment due to CD/CS with histopathology were included. Medline search for all studies published in English or German literature was performed. Patients who underwent surgery at our department between 2012 and 2020 due to CD or CS were analyzed. Results: In the multicenter study, 79 patients suffered from CD and 119 patients from CS, with a total number of 198 patients. In 14 patients, CCA was found (Overall: 7,1%; CD: 6,3%, CS 7,6%). Between 2012 and 2020, 1661 liver resections were performed at our department. 14 patients underwent surgery due to CD or CS. Histological examination showed synchronous cholangiocarcinoma in one patient. The literature review revealed a CCA-rate of 7,3% in large series, whereas in case reports a rate of 6,8% was found. Conclusion: There is risk of malignant transformation and patients with CD might also benefit from resection due to improvement of symptoms. Therefore, resection is strongly advised. As certain patients with CS require transplantation, treatment should not be guided by the relatively low rate of CCA but by the concomitant diseases that come along with hepatic failure.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Fard-Aghaie, Mohammad H.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Makridis, GeorgiosUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Reese, TimUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Feyerabend, BerndUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wagner, Kim C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schnitzbauer, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bechstein, Wolf O.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Oldhafer, FelixUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kleine, MoritzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Klempnauer, JurgenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Rolinger, JensUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nadalin, SilvioUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Konigsrainer, AlfredUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Vassos, NikolaosUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Grutzmann, RobertUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Benko, TamasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Paul, AndreasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Li, JunUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fischer, LutzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Beaumont, KatharinaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Nussler, NataschaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fahrner, ReneUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Settmacher, UtzUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Fichtner-Feigl, StefanUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schoning, WenzelUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Pratschke, JohannUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Eckhoff, Jennifer A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Wahba, RogerUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bruns, ChristianeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Bernsmeier, AlexanderUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Braun, FelixUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Becker, ThomasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lurje, GeorgUNSPECIFIEDorcid.org/0000-0001-9674-0756UNSPECIFIED
Neumann, Ulf P.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Dohmen, JonasUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Manekeller, SteffenUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kalff, Jorg C.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Mehrabi, ArianebUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Buchler, Markus W.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lang, Sven A.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Schlitt, Hans J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Tripke, VerenaUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Lang, HaukeUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Oldhafer, Karl J.UNSPECIFIEDUNSPECIFIEDUNSPECIFIED
URN: urn:nbn:de:hbz:38-697045
DOI: 10.1016/j.hpb.2021.06.423
Journal or Publication Title: HPB
Volume: 24
Number: 2
Page Range: S. 267 - 277
Date: 2022
Publisher: ELSEVIER SCI LTD
Place of Publication: OXFORD
ISSN: 1477-2574
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
BILE-DUCT DILATATION; LIVER RESECTION; TRANSPLANTATION; CLASSIFICATION; COMPLICATIONS; SURGERYMultiple languages
Gastroenterology & Hepatology; SurgeryMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/69704

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