CHOLANGIOCARCINOMA

(KLATSKIN TUMOUR)

TR, 84 YRS FEMALE, BGOSTEOARTHRITIS

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.

1.BLOODS  Blood. bilirubin 113.   FBC& U/E-Normal INR. ALP737.0 Amylase-78   . LDH 855.LFT : AST 287. ALT 345.5.

grossly distended GB. Grossly distended GB. abrupt narrowing of CBD with no obvious filling defect ?cholangiocarcinoma.IMAGING  CXR & PFA. Markedly dilated CBD and IHBD. . US Gallstone.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.   CT .

Markedly dilated CBD and IHBD. .USS. grossly distended GB.Gallstone.

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

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

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

PERCUTANEOUS TRANSHEPATIC CHOLANGIOGRAPHY (PTC)  PTC was successfully performed with the placement of a metal stent with good resultbiliary decompression and relieve of her obstructive symptoms. .

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

Japan and American indians Annual incidence of bile duct Ca in USA is 1/100. 4. England and Wales . Cholangiocarcinoma most common malignancy of bile ducts.2.Epidemiology  Tumours of bile duct are rare-2% of all cancers found at autopsy.000 new cases reported annually in USA.46%.000 people.000 males. Malignant tumours more common than benign adenomas and papillomas.      .01-0.8/100.000 females & 2/100. Autopsy studies show and incidence of 0. More common in Israel. >50%Holland et at…2007.

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

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

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

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

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

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

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

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

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%    .DIAGNOSIS  Cholangiographic appearance of Klatskin tumour is characteristic.

17months for palliative resection and death within 1. 2007 reported in a study of 185 cases (1972-2006) a median survival of 37 months following radical resection.TREATMENT  Management challenging with relatively poor prognosis. Hepatic resection. Yang WL et at.. Surgery continues to be the mainstay of therapy with 5year survival of 10-40%.a critical component of operative approach.   .   Complete resection with negative histologic margins –long -term survival.5years if no resection.

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

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

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