KLATSKIN TUMOUR - Pilgrims Hospital
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Transcript KLATSKIN TUMOUR - Pilgrims Hospital
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.
BLOODS
Blood- LFT : AST 287, ALT 345, bilirubin 113.5,
ALP737, LDH 855,
FBC& U/E-Normal
INR- 1.0
Amylase-78
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.
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)
PTC was successfully performed with the
placement of a metal stent with good resultbiliary decompression and relieve of her
obstructive symptoms.
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.
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.
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, and West
Malaysia.
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.
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.
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
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.
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
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.
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.
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%
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.
TREATMENT
Adjuvant chemoradiotherapy-no benefit.
Liver transplant for unresectable tumour remains
controversial, tumour recurrence >90%.
Advances in interventional Radiology and endoscopyfacilitate 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.
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.
THANK YOU!