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home - Biliary - Biliary Cancers - Cholangiocarcinoma Risk Factors Written by Dr Sebastian Zeki

Knows the epidemiology pathology and clinical presentation of bile
duct tumours

Can recognise the presentation of biliary tumours arising de novo or
in the context of PSC Can plan programme of investigations
including detailed staging

Understands treatment options including surgery chemotherapy and
endoscopic management

Aware of the treatment options including biliary drainage
chemotherapy radiotherapy photodynamic therapy or surgery

Understands rationale for selection of particular therapy in individual

Awareness of the diagnostic modalities including CT MRI scanning
brush cytology intra ductal cholangioscopy and biopsy

Understands importance of multidisciplinary team of oncologist
surgeon radiologist histopathologist in decision making

Discusses cases with the specialist MDT

Cholangiocarcinoma Risk Factors

Cholangiocarcinoma- Risk Factors and Clinical Presentation HistologyUsually adenocarcinomas- from bile duct epithelium:Subtypes:NodularSclerosingPapillary (best prognosis)Clin Pres:HPB: Pruritis(66%)/obstructive sx/Hepatomegaly/RUQ massCourvoisier’s sign (painless palpable gallbladder) rareIntrahepatic duct cholangio less likely to be jaundicedSystemic: Fever (20%), weight loss (40%), abdo pain (40%)Labs: Cholestatic- may get ALT risa due to chronicity Ages: 60-70Sex: M=FGreatest incidence Israel/ Japan/ Native Americans Primary sclerosing cholangitis 30% also have PSC and present at earlier age (40yrs).30% are present within 2 years of diagnosis of PSC.The lifetime risk of 10 to 15%.The risk unrelated to the duration of the inflammatory disease. Risk factors for getting cholangiocarcinoma with PSCSmoking.Alcohol consumption.Certain genetic polymorphisms. HepatolithiasisThese are usually calcium bilirubinate (brown pigment stones) in intrahepatic ducts.Very common in South East Asia. Nonviral chronic liver diseaseThe risk is increased x10.Obesity is assoc with extrahepatic cholangioca.Diabetes The risk involved is x2. Viral hepatitisHep Chas a risk of 3.5 % at 10 yrs (<for HCC).Hep B has less of an association than Hep C. Fibropolycystic liver disease There is a 15 % risk of malignant change in the adult years (av age 34). Cholelithiasis and hepatolithiasis This has a less risk than for gallbladder Ca. Lynch syndrome and biliary pap-illomatosis Parasitic infection Clonorchis and Opisthorchis. Toxic exposuresRisk of cholangioca occurs around 35 years post thorotrast.There are weaker associations with the auto, rubber, chemical, and wood-finishing industries.EtOH and smoking associations are not consistent. Screening strategies in PSC:Screen with USS and CA 19-9 6 monthly if have cirrhosis, otherwise yearly Clinical manifestations and diag-nosis of cholangiocarcinoma A) Lynch syndrome IIB) Multiple biliary papillo-matosis -83% get malignancy. Written by Dr Sebastian Zeki

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