Cholangiocarcinoma is an uncommon cancer that occurs within the intrahepatic and extrahepatic portions of the bile duct system. In North America, the incidence of extrahepatic cholangiocarcinoma is 0.5–2 per 100,000 and 0.95 per 100,000 for intrahepatic cholangiocarcinoma. Up to 50% of patients will be lymph node (LN) positive at presentation, 5% are multifocal tumors, and 10–20% will have peritoneal involvement at presentation. Risk factors for cholangiocarcinoma are primary sclerosing cholangitis (PSC) with a lifetime risk 10–40%, parasitic infection, previous sphincteroplasty, congenital anomalies of the biliary tree (choledochal cyst, Caroli’s disease, anomalous pancreaticobiliary duct junction), and chronic biliary inflammatory disease (hepatitis B/C, liver cirrhosis, recurrent pyogenic cholangitis). The most common presentation is painless jaundice and weight loss in the setting of extrahepatic duct involvement. In Western countries, 80% are extrahepatic (20% distal and 60% hilar) and 20% are intrahepatic.
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• Ca 19–9 can be elevated in up to 85% of patients with cholangiocarcinoma, but is not specific; elevation can also occur in the setting of obstructive jaundice without malignancy. If it remains elevated after biliary decompression, it could indicate the presence of malignancy. Elevated pre- and postoperative Ca 19–9 predict poor survival.
• For perihilar tumors, decisions regarding which side of the liver to resect depend on right- or left-sided dominance, volume of future liver remnant, and the extent of vascular and ductal involvement.
• Some centers report that 30–50% of tumors will be deemed unresectable at the time of surgery, despite accurate preoperative imaging.
• Quality Indicators: Pathologic Analysis—R0 margin, regional lymphadenectomy includes three or more LN.
Intrahepatic cholangiocarcinoma: Surgical resection is the only potential cure with removal of involved liver segments. There is emerging evidence that recommends a routine hilar LN and dissection for its prognostic value, M1 disease includes involvement of celiac,periaortic or caval LN.
Distal bile duct (below the cystic duct): Surgical resection is the only potential cure. Pancreaticoduodenectomy including en bloc resection of extrahepatic bile duct and gallbladder. Regional nodes include: Hilar (CBD, common hepatic, portal, cystic), Posterior and anterior pancreaticoduodenal Nodes along SMV, Nodes along right and lateral wall of SMA.
Hilar (above the cystic duct): En bloc resection of extrahepatic bile duct and gallbladder, including right and left hepatectomy, or extended right/left hepatectomy, Caudate lobe should be removed. Regional nodes include: Hilar (CBD, hepatic,portal, cystic), Pericholedochal nodes in hepatoduodenal ligament.