New Trends in Treatment of Hilar Cholangiocarcionma.

Abstract

Cholangiocarcinomas arise from the epithelial cells of the intrahepatic and extrahepatic bile ducts. Although these cancers are rare, they are highly lethal because most are locally advanced at presentation. Cholangiocarcinoma has been used to refer to bile duct cancers arising in the intrahepatic, perihilar, or distal (extrahepatic) biliary tree, exclusive of the gallbladder or ampulla of Vater (De Groen et al, 1999). Cholangiocarcinomas have an extremely poor prognosis, with an average five-year survival rate of 5 to 10 percent. Surgery provides the only possibility for a cure. Distal cholangiocarcinomas have the highest resectability rates while proximal (both intrahepatic and perihilar) tumors have the lowest. In one large series, the resectability rates for distal, intrahepatic, and perihilar lesions were 91, 60, and 56 percent, respectively (Nakeeb et al, 1996). Substantial progress has been made in curative resection for perihilar cholangiocarcinomas. In selected series, five-year survival rates are between 20 and 50 percent, with the best results reported from Japan. (Hidalgo et al, 2008). Cholangiocarcinomas usually become symptomatic when the tumor obstructs the biliary drainage system, causing painless jaundice. Common symptoms include pruritus (66 percent), abdominal pain (30 to 50 percent), and weight loss (30 to 50 percent), and fever (up to 20 percent). The pain is generally described as a constant dull ache in the right upper quadrant. Cholangitis is an unusual presentation. (Nakeeb et al, 1996). Patients with primary sclerosing cholangitis and cholangiocarcinoma tend to present with a declining performances status and increasing cholestasis. Other symptoms related to biliary obstruction include clay-colored stools and dark urine. Physical signs include jaundice (90 percent), hepatomegaly (25 to 40 percent), or a right upper quadrant mass (10 percent). A palpable gallbladder, caused by obstruction distal to the takeoff of the cystic duct (Courvoisier's sign), occurs rarely. (Nakeeb et al, 1996). The Bismuth-Corlette classification is a classification system for perihilar cholangiocarcinomas, which is based on the extent of ductal infiltration. Type I, limited to the common hepatic duct, below the level of the confluence of the right and left hepatic ducts Type 2 involves the confluence of the right and left hepatic ducts. Type 3a, type 2 + extends to the bifurcation of the right hepatic duct. Type 3b, type 2+ extends to the bifurcation of the left hepatic duct. Type 4, extending to the bifurcations of both right and left hepatic ducts. Type V, stricture at the junction of common bile duct and cystic duct (Snyder et al, 2009). The accuracy of non-invasive imaging techniques is continuously improving. This improvement has resulted in highly accurate staging for many hepato-pancreato-biliary malignancies, and so, its use is nowadays recommended in selected patients .In spite of all the advances in preoperative imaging, evaluation of HCCA remains a challenge (Hariharan et al, 2012). Most jaundiced patients undergo initial transabdominal ultrasound (US) to confirm biliary ductal dilatation, localize the site of the obstruction, and exclude gallstones. The sequence of subsequent evaluation is slightly different for hilar as compared to distal lesions. For hilar lesions (intrahepatic ductal dilatation with normal caliber extrahepatic ducts), magnetic resonance cholangiopancreatography (MRCP) is emerging as the imaging technique of choice, while the use of invasive cholangiography, particularly ERCP, is diminishing (Khan et al, 2005). CT scan — Because of its widespread availability, CT is commonly obtained in patients with suspected biliary malignancy. It is useful for detecting intrahepatic tumors, the level of biliary obstruction and the presence of liver atrophy (Abu-Hamda et al,