Der Onkologe

, Volume 12, Issue 12, pp 1220–1225 | Cite as

Primär sklerosierende Cholangitis und zentrales Gallengangskarzinom

Überwachung, Therapieoptionen und Ergebnisse
Leitthema
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Zusammenfassung

Die primär sklerosierende Cholangitis (PSC) ist eine idiopathische, destruierende Erkrankung der Gallengänge, welche zum terminalen Leberversagen führen kann. Das Cholangiokarzinom (CCA) ist eine gefürchtete Komplikation der PSC. Die jährliche Inzidenz des CCA beträgt bei Patienten mit einer PSC 0,6–1,5% pro Jahr und ist besonders hoch in den ersten 2 Jahren nach Diagnose der PSC. Hierbei trägt die infauste Prognose eines fortgeschrittenen CCA entscheidend zur Gesamtmortalität der PSC bei. Damit die Chance auf eine Erfolg versprechende Therapie des CCA gewahrt bleibt, müssen bei PSC-Patienten regelmäßige Tumorvorsorgeuntersuchungen erfolgen. Bei ungenügender diagnostischer Sicherheit der einzelnen Screeningverfahren ergibt sich die beste Spezifität und Sensitivität aus der Kombination von endoskopischer, laborchemischer und zytologischer Diagnostik. Im Falle eines lokalisierten Befunds kann die radikale Resektion des Cholangiokarzinoms erwogen werden. Als experimentelles kuratives Therapieverfahren wird die orthotope Lebertransplantation im Anschluss an eine neoadjuvante Radiochemotherapie diskutiert. In der palliativen Situation beschränken sich die Maßnahmen auf die Kontrolle der cholestatischen Komplikationen des CCA durch lokale Therapien. Die palliative Chemotherapie des CCA ist der palliativ-supportiven Therapie kaum überlegen.

Schlüsselwörter

Cholangiokarzinom Primär sklerosierende Cholangitis Überwachung Therapie Prognose 

Primary sclerosing cholangitis and central cholangiocarcinoma

Surveillance, therapy and results

Abstract

Primary sclerosing cholangitis (PSC) is an idiopathic destructive disease of the bile ducts, which can lead to terminal liver failure. Cholangiocarcinoma (CCA) is a much-feared complication of PSC. The incidence of CCA is about 0.6–1.5% per year for PSC patients and is particularly high in the first 2 years after diagnosis of PSC. The poor prognosis of advanced CCA is a significant factor in the overall mortality of PSC. For there to be any chance that treatment of CCA might be successful, frequent tumor screening is essential for patients with PSC. Since none of the separate screening tools is satisfactory in diagnosis in these circumstances, the highest specificity and sensitivity results from a combined approach with endoscopic examination, laboratory and cytological investigations. If CCA is in an early stage and localized a radical resection may be considered as a method of curative treatment. Orthotopic liver transplantation after neoadjuvant radiochemotherapy is currently in discussion as an alternative experimental and curative approach. In contrast, palliative therapy is restricted to the management of cholestatic complications of CCA by means of local therapy. Palliative chemotherapy of CCA is of little benefit for patients and provides only a marginal survival advantage over best supportive care.

Keywords

Cholangiocarcinoma Primary sclerosing cholangitis Monitoring Treatment Prognosis 
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Notes

Interessenkonflikt

Es besteht kein Interessenkonflikt. Der korrespondierende Autor versichert, dass keine Verbindungen mit einer Firma, deren Produkt in dem Artikel genannt ist, oder einer Firma, die ein Konkurrenzprodukt vertreibt, bestehen. Die Präsentation des Themas ist unabhängig und die Darstellung der Inhalte produktneutral.

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Copyright information

© Springer Medizin Verlag 2006

Authors and Affiliations

  1. 1.I. Medizinische Klinik und Poliklinik, Johannes Gutenberg-UniversitätMainzDeutschland

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