Zusammenfassung
Die Caroli-Erkrankung ist eine Lebererkrankung mit segmentaler zystischer Dilatation der intrahepatischen Gallenwege, die zu den angeborenen Duktalplattenmalformationen gezählt wird. Sie ist mit einer Inzidenz von 0,05% bezogen auf alle Fälle des Kölner Leberregisters extrem selten. Sie geht meist mit Cholangitis und Hepatolithiasis einher. Therapeutische Ziele sind die Infektionskontrolle und der Erhalt der biliären Drainage. Als Komplikationen können eine intraepitheliale Neoplasie des auskleidenden Epithels und auch Karzinome entstehen. Wir stellen hier den Fall einer Caroli-Erkrankung mit Entwicklung eines Cholangiokarzinoms vor und diskutieren die Literatur.
Abstract
Caroli’s disease is a liver disease with segmental cystic dilatation of the intrahepatic bile ducts. It belongs to the group of congenital ductal plate malformations. With an incidence of only 0.05% of all liver cases in the Liver Registry of the University of Cologne, it is a very rare disorder. Caroli’s disease is usually combined with cholangitis and bile duct stones. Control of these infections and maintenance of biliary drainage are the main therapeutic aims. The development of intra epithelial neoplasia and invasive carcinoma are rare complications.
We report a case of Caroli’s disease with the development of cholangiocarcinoma and review the literature.
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Kasper, HU., Stippel, D.L., Töx, U. et al. Primäres Cholangiokarzinom auf dem Boden einer Caroli-Erkrankung. Pathologe 27, 300–304 (2006). https://doi.org/10.1007/s00292-006-0840-3
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DOI: https://doi.org/10.1007/s00292-006-0840-3