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.

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 result-
biliary 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 at2007.

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 East-

intrahepatic 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 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.
 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.

 THANK YOU!