Cholangiocarcinoma

• • Arises from biliary epithelium

  • Risk factors

  • -Primary sclerosing cholangitis

    -Choledochal cysts

    -Clonorchis infection

Epidemiology

• • < 4500 patients per year

  • Average age, 50-70 years

  • Evenly distributed among men and women

Symptoms and Signs

• • Painless jaundice

  • Right upper quadrant pain

  • Pruritus

  • Anorexia

  • Malaise

  • Weight loss

  • Cholangitis

  • Asymptomatic

Laboratory Findings

• • Hyperbilirubinemia

  • Elevated alkaline phosphatase

  • Elevated CA 19-9

Imaging Findings

• • 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

• • History of pancreatitis (possible benign stricture)

  • History of ulcerative colitis (possible primary sclerosing cholangitis)

  • Choledocholithiasis

Rule Out

• • Extrahepatic disease or bilobar involvement

  • Choledocholithiasis

• • 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

Surgery

• • 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

Indications

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

  • Presence of choledochal cyst

Contraindications

• • Bilobar involvement or second order biliary radicals bilaterally

  • Extrahepatic disease

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

Complications

• • Anastomotic leak or stricture

  • Cholangitis

  • Recurrent disease

  • Liver failure

  • Hemorrhage

Prognosis

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

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

References