Cholangiocarcinoma Tamar Taddei, MD

Key Points PSC is a risk factor for developing cholangiocarcinoma (CCA) Screening is controversial Remaining in close contact and having routine visits with your healthcare team is essential to early diagnosis Stress is unproductive A healthy lifestyle is key to most preventive medicine

Definition Cholangiocarcinoma (CCA) and bile duct cancer are the same thing In general the incidence of CCA is on the rise This is a primary liver cancer that arises in the biliary cells (cholangiocytes) of the liver

Causes The pathogenesis of CCA is linked to bile stasis, inflammation, periductal fibrosis, and hyperplasia 1/256 patients with UC Lifetime risk 10-15% in PSC Cumulative risk of CCA is 11.2% at 10 years after diagnosis (0.6-1.5% per year)

Causes Other important risk factors Liver fluke Intrahepatic gallstones Choledochal cysts (Caroli’s disease) Exposures (thorotrast)

https://www. jhmicall. org/GDL_Disease. aspx https://www.jhmicall.org/GDL_Disease.aspx?CurrentUDV=31&GDL_Cat_ID=83F0F583-EF5A-4A24-A2AF-0392A3900F1D&GDL_Disease_ID=A6D10E80-887D-49A7-B3BB-0517D38CE757

Diagnosis Laboratory tests Imaging/endoscopic tests Sampling CEA, CA 19-9, AFP (positive in < 50%, but at late stage) Imaging/endoscopic tests US MRI EUS (also very helpful for staging) ERCP Sampling Brushings may be of low yield

A Helpful Schematic Rizvi S and Gores GJ, Hepatology 2016 63:1356.

Understanding Liver Anatomy

Treatment: Curative Where is the tumor? Is it surgically resectable? Bilateral hepticojejunostomy with Roux-en-Y anastomosis Unilateral hepticojejunostomy with Roux-en-Y anastomosis and left hepatectomy

Treatment: Curative Where is the tumor? Is it surgically resectable? Percent resectable ▪ Intrahepatic (peripheral): 50% ▪ Perihilar: 56% ▪ Distal: 91% 5 year survival ▪ Intrahepatic (peripheral): 44% (26 mos) ▪ Perihilar: 11% (19 mos) ▪ Distal: 28% (22 mos) Whipple with hepatojejunostomy, duodenojejunoscopy, and pancreaticojejunostomy

Treatment: Palliative Treating cholestasis/jaundice (biliary stents) Improves fatigue, diarrhea, anorexia, pruritus, jaundice, sleep pattern Prevents cholangitis Decrease tumor growth rates Chemotherapy Radiotherapy Photodynamic therapy