Meier P N, Manns M P
Abteilung Gastroenterologie und Hepatologie, Medizinische Hochschule Hannover.
Leber Magen Darm. 1994 Nov;24(6):234-8, 241.
Cholangiocarcinoma affects both sexes equally, is more prevalent in individuals age 50 to 70. Associations with many other diseases are known, in particular with primary sclerosing cholangitis, ulcerative colitis and parasitic infestation (Clonorchis sinensis, Opisthorchis viverrini). About 95% are histopathologically classified as adenocarcinoma. The localisation of the tumor determines clinical course and prognosis. If the tumor is located above the hepatic duct bifurcation, only one side of the biliary tree may be obstructed, resulting in atrophy of the corresponding liver lobe, without clinical manifestation of jaundice. Obstructive jaundice is the characteristic symptom of hilar tumor localisation. Laboratory examinations show hyperbilirubinemia and liver enzymes indicating cholestatis are elevated. Pathological levels of the tumor markers carcinoembryonic antigen (CEA) and CA 19-9 are frequently found. Ultrasonography in combination with endoscopic retrograde or percutaneous transhepatic cholangiography and cytological examination of aspirates are of main diagnostic importance. Treatment consists of curative resection or palliative decompression to relieve jaundice, the latter can be performed using endoscopic implantable self-expanding metal stents. Cytotoxic chemotherapy or liver transplantation show no satisfactory results. The prognosis is mainly poor, only few patients survive more than 6 months after diagnosis.
胆管癌在男女中的发病率相同,在50至70岁的人群中更为常见。已知它与许多其他疾病有关,特别是原发性硬化性胆管炎、溃疡性结肠炎和寄生虫感染(华支睾吸虫、麝猫后睾吸虫)。约95%的病例在组织病理学上被分类为腺癌。肿瘤的位置决定临床病程和预后。如果肿瘤位于肝管分叉上方,胆管树的一侧可能仅被阻塞,导致相应肝叶萎缩,而无黄疸的临床表现。阻塞性黄疸是肝门部肿瘤定位的特征性症状。实验室检查显示高胆红素血症,提示胆汁淤积的肝酶升高。经常发现肿瘤标志物癌胚抗原(CEA)和CA 19-9水平异常。超声检查结合内镜逆行或经皮经肝胆管造影以及抽吸物的细胞学检查具有主要诊断价值。治疗包括根治性切除或姑息性减压以缓解黄疸,后者可使用内镜植入式自膨式金属支架进行。细胞毒性化疗或肝移植效果均不理想。预后主要较差,诊断后仅有少数患者能存活超过6个月。