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  • • Arises from biliary epithelium

    • Risk factors

    • -Primary sclerosing cholangitis

      -Choledochal cysts

      -Clonorchis infection

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Epidemiology

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  • • < 4500 patients per year

    • Average age, 50-70 years

    • Evenly distributed among men and women

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Symptoms and Signs

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  • • Painless jaundice

    • Right upper quadrant pain

    • Pruritus

    • Anorexia

    • Malaise

    • Weight loss

    • Cholangitis

    • Asymptomatic

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Laboratory Findings

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  • • Hyperbilirubinemia

    • Elevated alkaline phosphatase

    • Elevated CA 19-9

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Imaging Findings

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  • • US showing dilated extrahepatic and intrahepatic biliary ducts (depending on level of tumor)

    • CT or MRI with biliary dilatation and occasional visible hepatic tumor

    • Percutaneous transhepatic cholangiography (PTC) or magnetic resonance cholangiopancreatography (MRCP) visualizing proximal and distal extent of tumor

    • -PTC provides opportunity for brushings for cytologic studies of tumor

    • Mesenteric angiography for question of portal vasculature involvement

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  • • History of pancreatitis (possible benign stricture)

    • History of ulcerative colitis (possible primary sclerosing cholangitis)

    • Choledocholithiasis

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Rule Out

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  • • Extrahepatic disease or bilobar involvement

    • Choledocholithiasis

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  • • History and physical exam

    • Liver function tests

    • CA 19-9

    • US to screen for anatomic causes of hyperbilirubinemia

    • Abdominal CT

    • PTC or MRCP (PTC if brushings needed)

    • Angiography if portal vessel involvement suspected

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Surgery

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  • • Biliary resection followed by biliary-enteric resection

    • Extended right or left lobectomy if proximal disease noted (isolated to 1 side) above secondary radicals or if unilateral portal vein or hepatic artery involvement

    • Pancreaticoduodenectomy (Whipple) for distal common bile duct (CBD) tumors

    • Biliary-enteric bypass for PTC-placed wall stent for palliation

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Indications

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  • • Resectable cholangiocarcinoma or diagnosis of benign stricture can be difficult to distinguish

    • Presence of choledochal cyst

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Contraindications

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  • • Bilobar involvement or second order biliary radicals bilaterally

    • Extrahepatic disease

    • Main portal vein, bilateral portal vein, or bilateral hepatic artery involvement

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Complications

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  • • Anastomotic leak or stricture

    • Cholangitis

    • Recurrent disease

    • Liver failure

    • Hemorrhage

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Prognosis

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  • • 10-30% 5-year survival with curative resection of proximal biliary tumor

    • 30-50% 5-year survival with distal CBD tumor

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References

Ahrendt SA et al. Cholangiocarcinoma. Clin Liver Dis. 2001;5:191.  [PubMed: 11218916]
Jarnagin WR. Cholangiocarcinoma of the extrahepatic bile ducts. Semin Surg Oncol. 2000;19:156.  [PubMed: 11126380]
Kosuge T et al: Improved surgical results for hilar cholangiocarcinoma with procedures including major hepatic resection. Ann Surg 1999;230:663.  [PubMed: 10561090]
Lillemoe KD, Cameron JL: Surgery for hilar cholangiocarcinoma: the Johns Hopkins approach. J Hepatobiliary Pancreat Surg 2000;7:115.  [PubMed: 10982602]

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Practice Guidelines

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