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CHOLANGIOCARCINOMA

CHOLANGIOCARCINOMA. (KLATSKIN TUMOUR). TR, 84 YRS FEMALE, BG- OSTEOARTHRITIS . Admitted with painless obstructive jaundice Her symptoms were- jaundice, weight loss, anorexia, RUQ discomfort, heart burn, and changes in stool and urine colour.

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CHOLANGIOCARCINOMA

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  1. CHOLANGIOCARCINOMA (KLATSKIN TUMOUR)

  2. TR, 84 YRS FEMALE, BG-OSTEOARTHRITIS • Admitted with painless obstructive jaundice • Her symptoms were- jaundice, weight loss, anorexia, RUQ discomfort, heart burn, and changes in stool and urine colour. • She was clinically jaundiced with a non tender palpable gallbladder.

  3. BLOODS • Blood- LFT : AST 287, ALT 345, bilirubin 113.5, • ALP737, LDH 855, • FBC& U/E-Normal • INR- 1.0 • Amylase-78

  4. IMAGING • CXR & PFA. • US Gallstone, grossly distended GB, Markedly dilated CBD and IHBD. • CT - abdomen intra & extra-hepatic bile duct dilatation to the level of the hepatic hilium. Suggestion of 2cm mass at hilium-?cholangiocarcinoma-(Klatskin tumour) • MRCP-grossly dilated CBD/IHBR, abrupt narrowing of CBD with no obvious filling defect ?cholangiocarcinoma. Grossly distended GB.

  5. USS- Gallstone, grossly distended GB, Markedly dilated CBD and IHBD.

  6. CT- abdomen- intra & extra-hepatic bile duct dilatation to the level of the hepatic hilium. Suggestion of 2cm mass at hilium-?cholangiocarcinoma-(Klatskin tumour)

  7. MRCP-grossly dilated CBD/IHBD, abrupt narrowing of CBD with no obvious filling defect ?cholangiocarcinoma. Grossly distended GB.

  8. ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY-(ERCP). Performed twice- unsuccessful on both occassions.

  9. PERCUTANEOUS TRANSHEPATIC CHOLANGIOGRAPHY (PTC) • PTC was successfully performed with the placement of a metal stent with good result- biliary decompression and relieve of her obstructive symptoms.

  10. KLATSKIN TUMOUR (KT) • BG- Bile duct tumours recognized for over a century. • Musser first reported 18 cases of primary extrahepatic biliary cancer. • Sako and colleagues found 570 cases while reviewing literature from 1935-1954. • Malignancy of the intrahepatic bile duct was described later by Altmeir (1957). • Gerald Klatskin described cancer of the hepatic duct bifurcation in 1965 following a review of 13 cases. • KT tumours are generally small, sharply localized and seldom metastasizing.

  11. Epidemiology • Tumours of bile duct are rare-2% of all cancers found at autopsy. • Malignant tumours more common than benign adenomas and papillomas. • Cholangiocarcinoma most common malignancy of bile ducts, >50%-Holland et at…2007. • More common in Israel, Japan and American indians • Annual incidence of bile duct Ca in USA is 1/100,000 people. Autopsy studies show and incidence of 0.01-0.46%. 4,000 new cases reported annually in USA. • England and Wales - 2.8/100,000 females & 2/100,000 males.

  12. ETIOLOGY • Risk factors for bile duct cancer include: • Ulcerative colitis • Primary sclerosing cholangitis-10-30% • Parasitic infestations:Liver fluke common in Far East-intrahepatic CC accounts for 20% of primary liver tumour. • Opisthorchis viverrini-found inThailand, and West Malaysia.

  13. ETIOLOGY • Toxic chemicals-thorium dioxide (thorotrast), radionuclides, carcinogens-arsenic, nitrosamines • Congenital fibrosis or cysts-cogenital hepatic fibrosis, cystic dilatation, choledochal cyst, polycystic liver • Drugs: methyldopa, isoniazide, OCP. • Gallstones and hepatolithiasis-decrease incidence >10 years post cholecystectomy. • Biliary cirrhosis and typhoid carriers.

  14. PATHOPHYSIOLOGY • Bile duct tumours cause bile duct obstruction - biliary stasis and alteration of liver function tests • Prolonged obstruction then leads to- • Hepatocellular dysfunction, renal dysfunction • Progressive malnutrition, Pruritus, coagulopathy • Cholangitis- esp if previous endoscopic, percutaneous or surgical biliary interventions have been performed.

  15. Anatomically, biliary tree is divided into 3 parts, upper 3rd-55%, middle 3rd 15% and lower 3rd 10%.Of these tumours, 10% are diffuse.

  16. Bismuth Classification • Type i-involvement of common hepatic duct. • Type ii-bifurcation involved without involvement of secondary intrahepatic duct. • Type iiia-extends into the right secondary intrahepatic duct. • Type iiib-extends into the left secondary intrahepatic duct. • Type iv- secondary intrahepatic ducts involved on both sides.

  17. PRESENTATION • CC seen in advanced unresectable stage • Early diagnosis unusual • Typically elderly- average age 60-65years though Klatskin slightly younger age group • Abnormal LFTs / Jaundice-90% • Abdominal pain / Weight loss- in (30-50%) of cases -Patel et al 2006 • Pruritus seen in 66% of patients

  18. PRESENTATION • Fever- 20% • Diarrhoea, anorexia, changes in urine & stool colour and weight loss. • Liver may be enlarged and smooth-25-40% • Distended and non tender gallbladder 10% • Epigastric tenderness.

  19. DIAGNOSIS • History / physical examination • Labouratory-CEA and CA19.9 –sensitivity of 66% and a specificity of 100% in diagnosing CC in pt with PSC. • Imaging-tumours are generally small-USS/ CT may fail to show the lesion. • Cholangiography via a transhepatic or endoscopic approach reqired to define biliary anatomy and extent of the lesion.

  20. DIAGNOSIS • Cholangiographic appearance of Klatskin tumour is characteristic. • PTC preferred over ERCP for demonstrating ductal anatomy-PTC-almost 100% sensitivity & specificity. • MRCP non-invasive and now more available. • Histology –a well defferentiated adenocarcinoma-short annular constricting lesion 75%, diffusely infiltrating with long strictures 15%& intraluminal polypoid mass-3-5%

  21. TREATMENT • Management challenging with relatively poor prognosis. • Surgery continues to be the mainstay of therapy with 5year survival of 10-40%. • Complete resection with negative histologic margins –long -term survival. • Yang WL et at.. 2007 reported in a study of 185 cases (1972-2006) a median survival of 37 months following radical resection, 17months for palliative resection and death within 1.5years if no resection. • Hepatic resection- a critical component of operative approach.

  22. TREATMENT • Adjuvant chemoradiotherapy-no benefit. • Liver transplant for unresectable tumour remains controversial, tumour recurrence >90%. • Advances in interventional Radiology and endoscopy- facilitate non surgical option. • Benefit of external beam radiotherapy for palliation of proximal CC uncertain. • Photodynamic therapy a new palliative treatment modality for failed stent. Thomas Zoepf et al concluded in a series in 2008 -offers similar survival time as incomplete resection.

  23. CONCLUSION • Klatskin tumour is tumour of bile duct bifurcation. • Diagnosis can be quite challenging as presentation is in an advanced stage with non-specific symptoms. • Surgery offers the only hope of cure.

  24. THANK YOU!

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