原發性硬化性膽管炎 Primary Sclerosing Cholangitis
潰瘍性結腸炎(256人中有一個)和原發性硬化性膽管炎(4-20%)病人有高膽管癌發病率(圖7)。膽管癌的累計危險度在上述疾病診斷后的10年為11.2%。

Figure 7. A, Klatskin’s tumor (tumor located in the hepatic duct bifurcation) in a patient with primary sclerosing cholangitis; B, corresponding cholangiogram (ERCP image).

Liver Flukes

Cholangiocarcinoma is more common in areas endemic to liver fluke infection (Hong Kong, Thailand). Liver flukes, such as Clonorchis sinensis or Opisthorchis viverrini, usually enter human’s gastrointestinal tract after ingestion of raw fish. Parasites travel via the duodenum into the host’s intrahepatic or extrahepatic biliary ducts. Liver flukes cause bile stasis, inflammation, periductal fibrosis and hyperplasia, with the subsequent development of cholangiocarcinoma (Figure 8).
肝吸虫 Liver Flukes
膽管癌更常見于肝吸虫感染的高發地區(香港﹐泰國)。Clonorchis sinensis和Opisthorchis viverrini之類的肝吸虫一般在人攝取生魚后進入胃腸道。寄生虫經十二指腸進入宿主的肝內和肝外膽管。肝吸虫會導致膽汁淤積﹐炎症﹐膽管周圍纖維化﹐和增生﹐最后發展為膽管癌(圖8)。

Figure 8. A, Liver flukes; B, micrograph of liver fluke eggs in the liver (reused with permission: Sun et al., Ann. Clin. Lab. Sci., 1984).

Gallstones

Gallstones vary in size, shape and number, and may be found throughout the biliary tract. The link between cholangiocarcinoma and gallstones is unclear. Intrahepatic gallstones may cause chronic obstruction to bile flow, promote micro injury of the bile ducts, and are associated with a 2–10% risk of the development of cholangiocarcinoma (Figure 9). Congenital cystic dilation of intrahepatic biliary ducts (Caroli’s disease), and choledochus cysts have also been closely associated with development of cholangiocarcinoma.
膽囊結石 Gallstones
膽囊結石的大小﹐形狀﹐和數目差异很大﹐可以發生在整個膽道內。膽管癌与膽囊結石之間的聯系還不清楚。肝內膽結石可能導致膽汁流通的慢性堵塞﹐促使膽管的微型傷害﹐并与2-10%的膽管癌發生有關(圖9)。先天性肝內膽管囊性擴張(Caroli病)和膽總管囊腫与膽管癌的發展密切相關。

Figure 9. Intrahepatic biliary gallstones resulting in ductal dilation.

Thorotrast

The radiocontrast agent, Thorotrast, was in use from the late 1920s through the 1950s. There are many reports of development of cholangiocarcinoma 30–35 years after exposure to this contrast material.

胶质二氧化钍造影剂
曾于上世纪20-50年代应用。有报道应用30-35年后发生胆管癌。

Laboratory tests

Biochemical tests of liver function may reveal a cholestatic picture with elevated total bilirubin and alkaline phosphatase. This pattern is non-specific for cholangiocarcinoma and may be found with any cause of obstruction to bile flow. The levels of blood bilirubin and alkaline phosphatase usually correlate with degree and duration of obstruction of the biliary ducts. Fluctuation in the serum bilirubin level may reflect incomplete obstruction and involvement of one hepatic duct.

CEA and CA19-9
Carcinoembriogenic antigen (CEA) and CA 19-9 are blood tests for non-specific markers of underlying gastrointestinal malignancies. These tests are positive in more than 40% of patients with cholangiocarcinoma, but usually only in late stages of the tumor.

Alpha-Fetoprotein (AFP)
Alpha-fetoprotein is another blood test commonly used to identify markers of possible hepatobiliary malignancy. This test is usually elevated in patients with cholangiocarcinoma, but not to the degree of elevations in patients with hepatocellular carcinoma.

實驗室檢查 Laboratory tests
肝功能的生化檢查可能顯示高總膽紅素和鹼性磷酸脢的膽汁淤積情況。這种表現對膽管癌沒有特异性﹐可能發生在任何膽汁流程受阻的病人中。血膽紅素和鹼性磷酸脢的水平一般与膽管梗阻的程度和時間長度相關。血清膽紅素水平的波動可能反應了不完全梗阻以及其中一支肝管的受累。

CEA和CA19-9
癌胚抗原(CEA)和CA19-9是胃腸道惡性腫瘤非特异性標志的血液檢查。這些試驗通常只是在腫瘤的晚期才在多于40%膽管癌的病人中呈陽性。

甲胎蛋白(AFP)
甲胎蛋白是一般用于檢測肝膽惡性腫瘤標記的另一血液檢查。膽管癌病人的這項檢查結果一般增高﹐但增高的程度不如肝細胞癌病人。

Radiological Diagnosis

Ultrasound
Transabdominal ultrasound is a totally painless, non-invasive procedure. The test does not require special preparation, although it is technically easier in patients with at least six hours of fasting. Transabdominal ultrasound is usually recommended as the first imaging modality for the investigation of patients with suspected cholangiocarcinoma. In hilar cholangiocarcinoma, ultrasound demonstrates bilateral dilation of intrahepatic ducts, and right and left hepatic ducts. In rare cases, the tumor itself can be visualized as either a hypoechoic (decreased echodensity) or hyperechoic (increased echodensity) rounded mass located just distal to dilated biliary ducts. Peripheral cholangiocarcinoma may be suspected if abdominal ultrasound demonstrates local dilation of intrahepatic ducts or isolated dilation of the biliary tree inside one lobe of the liver. In both peripheral and hilar cholangiocarcinoma, biliary ducts distal to the obstruction (common hepatic duct and common bile duct) are not dilated. In patients with hilar cholangiocarcinoma and complete obstruction of both right and left hepatic ducts, extrahepatic bile ducts and the gallbladder appear empty (collapsed) because there is no bile flow out of the liver. In patients with distal cholangiocarcinoma, ultrasound demonstrates dilated intra- and extrahepatic ducts along with significant dilation of the gallbladder. Peripherally located tumors cause segmental or lobular obstruction of the biliary tree. Bile flow from the rest of the liver is preserved. Extrahepatic bile ducts and the gallbladder appear normal (filled with bile) in patients with peripheral cholangiocarcinoma.

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