CHOLANGIOCARCINOMA
(KLATSKIN TUMOUR)
TR, 84 YRS FEMALE, BGOSTEOARTHRITIS
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Admitted with painless obstructive jaundice
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Her symptoms were- jaundice, weight loss,
anorexia, RUQ discomfort, heart burn, and
changes in stool and urine colour.
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She was clinically jaundiced with a non tender
palpable gallbladder.
BLOODS
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Blood- LFT : AST 287, ALT 345, bilirubin 113.5,
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ALP737, LDH 855,
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FBC& U/E-Normal
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INR- 1.0
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Amylase-78
IMAGING
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CXR & PFA.
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US Gallstone, grossly distended GB, Markedly dilated CBD and IHBD.
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CT - abdomen intra & extra-hepatic bile duct dilatation to the level of the
hepatic
hilium. Suggestion of 2cm mass at hilium-?cholangiocarcinoma(Klatskin tumour)
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MRCP-grossly dilated CBD/IHBR, abrupt narrowing of CBD with no
obvious filling defect ?cholangiocarcinoma. Grossly distended GB.
USS- 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/IHBD, abrupt narrowing of CBD with no
obvious filling defect ?cholangiocarcinoma. Grossly distended GB.
ENDOSCOPIC RETROGRADE
CHOLANGIOPANCREATOGRAPHY(ERCP).
Performed twice- unsuccessful on both
occassions.
PERCUTANEOUS TRANSHEPATIC
CHOLANGIOGRAPHY (PTC)
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PTC was successfully performed with the
placement of a metal stent with good resultbiliary decompression and relieve of her
obstructive symptoms.
KLATSKIN TUMOUR (KT)
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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.
Epidemiology
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Tumours of bile duct are rare-2% of all cancers found at autopsy.
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Malignant tumours more common than benign adenomas and
papillomas.
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Cholangiocarcinoma most common malignancy of bile ducts, >50%Holland et at…2007.
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More common in Israel, Japan and American indians
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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.
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England and Wales - 2.8/100,000 females & 2/100,000 males.
ETIOLOGY
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Risk factors for bile duct cancer include:
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Ulcerative colitis
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Primary sclerosing cholangitis-10-30%
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Parasitic infestations:Liver fluke common in Far Eastintrahepatic CC accounts for 20% of primary liver
tumour.
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Opisthorchis viverrini-found inThailand, and West
Malaysia.
ETIOLOGY
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Toxic chemicals-thorium dioxide (thorotrast),
radionuclides, carcinogens-arsenic, nitrosamines
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Congenital fibrosis or cysts-cogenital hepatic fibrosis,
cystic dilatation, choledochal cyst, polycystic liver
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Drugs: methyldopa, isoniazide, OCP.
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Gallstones and hepatolithiasis-decrease incidence >10
years post cholecystectomy.
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Biliary cirrhosis and typhoid carriers.
PATHOPHYSIOLOGY
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Bile duct tumours cause bile duct obstruction - biliary
stasis and alteration of liver function tests
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Prolonged obstruction then leads to-
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Hepatocellular dysfunction, renal dysfunction
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Progressive malnutrition, Pruritus, coagulopathy
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Cholangitis- esp if previous endoscopic, percutaneous or
surgical biliary interventions have been performed.
Anatomically, biliary tree is divided into 3 parts, upper 3rd-55%, middle
3rd 15% and lower 3rd 10%.Of these tumours, 10% are diffuse.
Bismuth Classification
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Type i-involvement of common hepatic duct.
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Type ii-bifurcation involved without involvement of
secondary intrahepatic duct.
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Type iiia-extends into the right secondary intrahepatic
duct.
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Type iiib-extends into the left secondary intrahepatic duct.
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Type iv- secondary intrahepatic ducts involved on both
sides.
PRESENTATION
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CC seen in advanced unresectable stage
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Early diagnosis unusual
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Typically elderly- average age 60-65years though Klatskin
slightly younger age group
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Abnormal LFTs / Jaundice-90%
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Abdominal pain / Weight loss- in (30-50%) of cases -Patel
et al 2006
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Pruritus seen in 66% of patients
PRESENTATION
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Fever- 20%
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Diarrhoea, anorexia, changes in urine & stool
colour and weight loss.
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Liver may be enlarged and smooth-25-40%
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Distended and non tender gallbladder 10%
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Epigastric tenderness.
DIAGNOSIS
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History / physical examination
Labouratory-CEA and CA19.9 –sensitivity of 66% and a
specificity of 100% in diagnosing CC in pt with PSC.
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Imaging-tumours are generally small-USS/ CT may fail to
show the lesion.
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Cholangiography via a transhepatic or endoscopic
approach reqired to define biliary anatomy and extent of
the lesion.
DIAGNOSIS
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Cholangiographic appearance of Klatskin tumour is
characteristic.
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PTC preferred over ERCP for demonstrating ductal
anatomy-PTC-almost 100% sensitivity & specificity.
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MRCP non-invasive and now more available.
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Histology –a well defferentiated adenocarcinoma-short
annular constricting lesion 75%, diffusely infiltrating with
long strictures 15%& intraluminal polypoid mass-3-5%
TREATMENT
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Management challenging with relatively poor prognosis.
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Surgery continues to be the mainstay of therapy with 5year
survival of 10-40%.
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Complete resection with negative histologic margins –long
-term survival.
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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.
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Hepatic resection- a critical component of operative
approach.
TREATMENT
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Adjuvant chemoradiotherapy-no benefit.
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Liver transplant for unresectable tumour remains
controversial, tumour recurrence >90%.
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Advances in interventional Radiology and endoscopyfacilitate non surgical option.
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Benefit of external beam radiotherapy for palliation of
proximal CC uncertain.
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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.
CONCLUSION
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Klatskin tumour is tumour of bile duct
bifurcation.
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Diagnosis can be quite challenging as
presentation is in an advanced stage with nonspecific symptoms.
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Surgery offers the only hope of cure.
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THANK YOU!