| What is cholangiocarcinoma? | Epithelial carcinoma arises from intra and extra hepatic biliary tract.
Most common bile duct tumor, second most common primary hepatic malignancy (after hepatocellular carcinoma) |
| How is the classification of cholangiocarcinoma? | Intrahepatic (20%), perihilar (between live and pancreas), and distal extrahepatic (80% both extrahepatic)
Perihilar are classified as :
Type I (common hepatic duct away from union of right and left) type II (common hepatic w/ union), Type IIIa (common hepatic and right hepatic duct), Type IIIb (common hepatic and left), Type IV (all ducts) |
| How is epidemiology of cholangiocarcinomas? | Most common biliary and second most common primary hepatic malignancy.
less than 2% of all malignancies, 9th most common GI one (hepatobiliary carcinomas are 13-30% of death, 10% of them are cholangiocarcinomas)
52-54% are male, older than 50 years old usually uncommon before 40 unless pt w/PSC |
| What are risk factors for cholangiocarcinoma? | Definite: Caroli's disease , choledochal cyst, hepatolithiasis, PSC, O.viverrini infection, thorotrast
Probable: Biliary-enteric drainage procedures, cirrhosis, C.sinesis infection, heavy alcoholic, HepC, toxins. |
| What is the algorithm of choices in case we see an intrahepatic mass on CT/MRI? | . |
| What is the algorithm of choices in case we suspect cholangiocarcinoma? | . |
| What is the clinical presentation of cholangiocarcinoma? | Intrahepatic: abdominal pain, systemic symptoms (cachexia, malaise, fatigue)
Extrahepatic (painless jaundice (due to biliary obstruction), 10% see bacterial cholangitis |
| What are lab biology findings for cholangiocarcinoma? | Evidence of obstructive cholestasis, CA19-9 (pt w/PSC) specific and sensitive w/cut off 129
w/out PSC sensitivity is 54% only (cutoff 100) |
| How is dx of cholangiocarcinoma? | Cholantiography (for extrahepatic)
ERCP, percutaneous THC (transhep cholangiography) [sees tumor extension and sampling and therapeutic], and MRCP [extrabiliary tumor, vascular encasement, surrounding tumors, intra and extrahepatic metastasis].
Intrahepatic: >2cm: dynamic CT/ MRI
Cross-sectional imaging for guiding biliary drainage, PET very specific and sensitive (if more than 1cm up to 100%)
For perihilar not as much as well as lymph node metastasis.
Tissue diagnosis (biopsy) is desired but difficult to obtain (new techniques like choledochoscopy/ intraductal US... can be used. |
| What is the Memorial Sloan-Kettering Cancer Center Staging System (MSKCC)? | T1 (tumor involve biliary confluence w/ or w/out extension to secondary radicles)
T2 (T1 and ipsilateral portal vein involvement/hepatic lobe atrophy)
T3 (T2 + contralateral involvement/bilateral portal vein/main portal vein involved)
Used for perihilar cholangiocarcinoma |
| How is the tx of cholangiocarcinoma? | Surgery (recurrence high 62%, 5 year survival low (22-42%) R0 resection is for 63% of pt and get higher 5 year survival (40-60%) Surgery morbidity (35%) and mortality (5%)
No result of chemo (neo or adjuvant)
Liver transplant not recommended (0% 5-year survival)
Whipple resection for distal extrahepatic.
Pt w/PSC and perihilar cholangio should consider liver transplant instead of resection (since low resectability)
Can restore biliary drainage by endoscopic/percutaneous stents/ surgical.
Photoynamic therapy (PDT) paliative tx of cholestasis survival similar to R1/R2 resection. |
