Zusammenfassung
Hintergrund
Cholangiozelluläre Karzinome (CCA) sind seltene, aber äußerst aggressive Tumoren des hepatobiliären Systems. Es stehen nur wenige evidenzbasierte therapeutische Optionen für Patienten mit fortgeschrittenen Tumoren zur Verfügung.
Fragestellung
Es werden die Diagnosefindung und aktuelle Therapiekonzepte dargestellt.
Material und Methoden
Die Arbeit basiert auf einer selektiven Literaturrecherche zur aktuellen Diagnostik und Therapie.
Ergebnisse und Schlussfolgerung
Das CCA gehört trotz einer steigenden Inzidenz weiterhin zu den seltenen Tumorentitäten. Bei begründetem Verdacht stehen mit der kontrastmittelverstärkten Computertomographie und der Magnetresonanztomographie zwei hochauflösende bildgebende Verfahren zur Diagnostik und zum Staging zur Verfügung. Aufgrund eines oft lange klinisch inapparenten Verlaufs und fehlender Strategien zur Früherkennung werden die CCA in den meisten Fällen erst im fortgeschrittenen oder metastasierten Stadium diagnostiziert, in dem eine potenziell kurative chirurgische Resektion nicht mehr möglich ist. Die Prognose bleibt allerdings auch bei primär resektablen Patienten äußerst schlecht, sodass derzeit verschiedene Strategien in der adjuvanten und palliativen Therapie evaluiert werden. Basierend auf der aktuellen Datenlage kann eine vorsichtige Empfehlung zur adjuvanten Behandlung mit Capecitabin ausgesprochen werden. In der palliativen Situation stellt die Chemotherapie mit Gemcitabin und Cisplatin weiterhin den Standard dar. Erste Studien mit molekularen Therapien in selektionierten Patientenkollektiven sind sehr vielversprechend und unterstreichen die zunehmende Bedeutung einer frühzeitigen genetischen Diagnostik. Dieser Artikel fasst die aktuellen Therapiemöglichkeiten zusammen und gibt insbesondere einen Überblick über systemische Therapiestrategien.
Abstract
Background
Cholangiocarcinoma (CCA) is a rare, but highly aggressive malignancy of the hepatobiliary system. Only few evidence-based treatment options are available for patients who present with advanced disease.
Objectives
Diagnostic and therapeutic strategies are reviewed and discussed.
Materials and methods
The work is based on a literature search of current diagnostic and therapeutic concepts.
Results and conclusion
Despite its increasing incidence, CCA is still considered a rare malignancy. Contrast-enhanced imaging modalities, such as computed tomography and magnetic resonance imaging, serve as sensitive diagnostic tools for the detection and staging of CCA. However, due to the frequently late clinical manifestation and insufficient surveillance strategies, most CCA patients are diagnosed with locally advanced or metastatic disease, which precludes potentially curative resection. Even in R0 resected patients, overall prognosis is poor due to rapid recurrence of the disease. Therefore, adjuvant strategies are currently under investigation and there is preliminary evidence that adjuvant treatment with capecitabin may be beneficial. Palliative systemic therapy with gemcitabine and cisplatin is still considered standard of care in the unresectable patient. However, promising first results from targeted therapeutic approaches in selected patient cohorts underscore the increasing importance of early molecular diagnostics in order to offer CCA patients tailored treatment strategies. This article summarizes current treatment regimens, with an emphasis on systemic therapeutic approaches.
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A. Vogel gibt an, Honorare für Vorträge und Advisory Boards von Roche, Lilly, Bayer, Incyte, Ipsen, Pierre Fabre und MSD erhalten zu haben. A. Saborowski gibt an, dass kein Interessenkonflikt besteht.
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Dieser Beitrag wurde in der Zeitschrift Der Gastroenterologe (2018) 13(5):366–371 https://doi.org/10.1007/s11377-018-0280-7 erstveröffentlicht. Zweitpublikation mit freundlicher Genehmigung der Autoren.
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Saborowski, A., Vogel, A. Das intrahepatische Gallengangskarzinom – Diagnostik und Therapie. Wien klin Mag 21, 220–225 (2018). https://doi.org/10.1007/s00740-018-0251-0
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DOI: https://doi.org/10.1007/s00740-018-0251-0