Zusammenfassung
Karzinome des Gallenwegsystems sind seltene Tumoren des Gastrointestinaltrakts mit in den letzten Jahren zunehmender Inzidenz. Sie werden unterteilt in intra- und extrahepatische Gallengangskarzinome (Klatskin-Tumor, mittleres und distales Gallengangskarzinom), Gallenblasenkarzinom und Papillenkarzinom. Die Umwandlung von normalem in malignes Gallengangsgewebe erfordert eine Reihe von aufeinander folgenden Genmutationen, ähnlich der Adenom-Dysplasie-Karzinom-Sequenz beim Kolonkarzinom. Der abdominelle Ultraschall, die nichtinvasive kombinierte Magnetresonanzcholangiographie/-tomographie (MRC/MRT) und fakultativ bei fraglicher Diagnose die endoskopische retrograde Cholangiographie (ERC) stellen in der Primärdiagnostik den Goldstandard dar. Beim Papillenkarzinom ist die Endosonographie neben der endoskopischen Biopsie diagnostisches Mittel der Wahl. Eine Heilung ist bei allen Tumorarten nur durch eine radikal chirurgische Resektion im Gesunden möglich. Durch eine Zunahme der Radikalität konnte in den letzten Jahren eine deutliche Verbesserung der 5-Jahres-Überlebensraten erreicht werden. Ein klinischer Benefit für adjuvante bzw. neoadjuvante Therapiemaßnahmen konnte dagegen bisher nicht nachgewiesen werden. In der Palliation sind die endoskopische Drainage der Gallenwege und die photodynamische Therapie fest etabliert. Radio- und Chemotherapie sind nur im Rahmen von Studien sinnvoll. Neuere Therapieansätze sind die Brachytherapie, moderne Chemotherapeutika sowie die COX-2- und Tyrosinkinaserezeptorinhibition.
Abstract
Carcinoma of the biliary tree are rare tumours of the gastrointestinal tract with a rising incidence during the last years. Biliary neoplasms are classified into intra- and extrahepatic cholangiocarcinoma (Klatskin tumour, middle and distal extrahepatic tumours), gallbladder cancer, and ampullary carcinoma. Transformation of normal into malignant bile duct tissue requires a chain of consecutive gene mutations, similar to the adenoma-dysplasia-carcinoma-sequence in colon cancer. Abdominal ultrasound, combined non-invasive magnetic resonance cholangiography/tomography (MRC/MRT), and facultatively endoscopic retrograde cholangiography (ERC) for unclear diagnosis, represent the gold standard for primary diagnosis. For ampullary carcinoma, endosonography and endoscopic biopsy are the diagnostic tools of choice. Cure is attainable only by formal curative radical surgical resection. Increasing surgical radicality within the last years enabled clearly improved 5-year survival rates. In contrast, there has been no clinical benefit for adjuvant and neoadjuvant therapies. For palliation, bile duct stenting and photodynamic therapy are established methods. Radio- and chemotherapy should be reserved for clinical studies. New therapeutic approaches include brachytherapy, the use of modern chemotherapeutics, COX-2- and tyrosine kinase-receptor-inhibitors.
Schlüsselwörter
Intrahepatisches Gallengangskarzinom - hiläres Gallengangskarzinom - Gallenblasenkarzinom - Ampulla-Vateri-Karzinom - Diagnostik - Therapie
Key words
Intrahepatic cholangiocarcinoma - hilar cholangiocarcinoma - gallbladder cancer - ampulla-Vateri carcinoma - diagnosis - therapy
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Dr. med. Marcus Wiedmann
Medizinische Klinik II, Universität Leipzig
Philipp-Rosenthal-Str. 27
04103 Leipzig
Phone: 03 41/9 71 22 25
Fax: 03 41/9 71 22 39
Email: wiedm@medizin.uni-leipzig.de