1. Cholangiocarcinoma
Tamar Taddei, MD

2. 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

3. 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

4. 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 ( % per year)

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

6. https://www. jhmicall. org/GDL_Disease. aspx

7. 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

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

9. Understanding Liver Anatomy

10. 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

11. 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

12. 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