KINESIOL 1Y03 Chapter Notes - Chapter 13: Polysomy, Viral Hepatitis, Cholangiocyte
22 Jun 2018
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Cholangiocarcinoma
= 90% are adenocarcinomas arising from ductular epithelium of biliary tree
Can be intrahepatic or extrahepatic
Second commonest primary liver cancer
Difficult to diagnose as there is a lack of characteristic imaging phenomena
Usually fatal due to late presentation and lack of non-surgical therapeutic
modalities: can’t ablate, recurs hence transplant outcomes poor, can’t predict risk
factors – 90% occur ne novo in those with no risk factors
Bismuth classification for where in biliary tree cholangiocarcinoma occurs:
1 - tumours below confluence of L and R hepatic ducts
2 - tumours reaching confluence but not affecting either R or L duct
3a – occluding common hepatic duct and R hepatic duct
3b – occluding common hepatic duct and L hepatic duct
4 – are multicentric (several locations) or involve both R and L hepatic duct
60-70% are peri-hilar = at bifurcation of hepatic ducts = “Klatskin tumours” –
generally classified is intrahepatic
20-30% distal CBD
5-10% peripheral = within intra-hepatic ducts of liver parenchyma itself
Clinical features
Presenting features depend on location
At bifurcation of hepatic ducts or in distal CBD = biliary obstruction: painless jaundice,
pale stools, dark urine, pruritis (Painless jaundice in the elderly is poor prognostic sign)
Peripheral tumours arising within intrahepatic ducts of liver present with non-specific
symptoms
can also present RARELY as cholangitis (due to obstruction causing stasis in gallbladder
infection abdo pain, fever, jaundice indication for urgent ERCP, hard to treat as difficult
to penetrate with ABx
Prognosis
= Terrible
- Most Px have unresectable disease = die within 12 months of:
Liver failure
Cancer cachexia
Recurrent sepsis secondary to biliary obstruction
- 5-year survival = <5% even included resected Px, as high rate of recurrence / incomplete
resection
find more resources at oneclass.com
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Epidemiology
3% of all GI cancers
peak age in 70s, Rare <40yo
slight male preponderance
Leading COD from primary liver tumour in UK: has increased drastically over
last 30 years – most striking of all tumours: 1200 died of cholangiocarcinoma in 2007
Varies regionally worldwide; highest rates in NE Thailand and other parts of
SE Asia
Incidence overall rising – Khan et al 2002
ICC is increasing but ECC is decreasing this is true in most regions worldwide
– Patel, 2002
May be that ICC is increasing as it is better diagnosed, whilst cases used to be
classified as HCC
Risk factors
Primary sclerosing cholangitis
-commonest predisposing condition in the west
-8-40% get CC
-presents earlier than normal, in 30-50 age group
-1/3 develop within 2 years of diagnosis hence monitor closely at this time
-risk unrelated to duration
-difficult to identify CC as structuring can foster CCs and make them less obvious
Parasitic infection
-liver fluke (opisthorcis viverrini and Clonorchis sinensins)
-epidemiological data from Thailand and animal model evidence (Syrian hamsters)
-humans are infected by undercooked fish; adult worms lay eggs in the biliary tree
Fibropolycystic liver disease
-congenital abnormalities of the biliary tree associated with Caroli’s syndrome,
congenital hepatic fibrosis and choledochal cysts
-15% of malignant change after 20s – mean age 34yo
-incidence in untreated cysts = 28%
Intrahepatic biliary stones (hepatolithiasis)
-rare in west but common in Asia
-associated with peripheral ICC
-up to 10% get CC
-biliary stones cause bile stasis recurrent infections / inflammation
Chemical carcinogen exposure
-promutagenic DNA adducts demonstrated in CC tissue
-thorotrast exposure caused RR of 300! – was a radiological contrast agent used in
the 1930s-1960s
-also chemicals in rubber and chemical industries: dioxins, nitrosamines
-alcohol, smoking
Cirrhosis and viral hepatitis
-cirrhosis of any cause is a risk factor
-10-fold risk – Sorenson 1998
-Hep C and B demonstrated to correlate with CC risk in case-control studies
-Prospective study also showed CC 1000X more likely in Hep C – Kobayashi et al, 2000
-HCV RNA detected in cholangiocarcinoma tissue
find more resources at oneclass.com
find more resources at oneclass.com
Document Summary
= 90% are adenocarcinomas arising from ductular epithelium of biliary tree. Difficult to diagnose as there is a lack of characteristic imaging phenomena. Usually fatal due to late presentation and lack of non-surgical therapeutic modalities: can"t ablate, recurs hence transplant outcomes poor, can"t predict risk factors 90% occur ne novo in those with no risk factors. Bismuth classification for where in biliary tree cholangiocarcinoma occurs: 1 - tumours below confluence of l and r hepatic ducts. 2 - tumours reaching confluence but not affecting either r or l duct. 3a occluding common hepatic duct and r hepatic duct. 3b occluding common hepatic duct and l hepatic duct. 4 are multicentric (several locations) or involve both r and l hepatic duct. 60-70% are peri-hilar = at bifurcation of hepatic ducts = klatskin tumours generally classified is intrahepatic. 5-10% peripheral = within intra-hepatic ducts of liver parenchyma itself.
