CHOLANGIOCARCINOMACHOLANGIOCARCINOMA
(KLATSKIN TUMOUR)(KLATSKIN TUMOUR)
TR, 84 YRS FEMALE, BG-OSTEOARTHRITISTR, 84 YRS FEMALE, BG-OSTEOARTHRITIS
Admitted with painless obstructive jaundiceAdmitted with painless obstructive jaundice
Her symptoms were- jaundice, weight loss,anorexia, RUQ discomfort, heart burn, andchanges in stool and urine colour.Her symptoms were- jaundice, weight loss,anorexia, RUQ discomfort, heart burn, andchanges in stool and urine colour.
She was clinically jaundiced with a non tenderpalpable gallbladder.She was clinically jaundiced with a non tenderpalpable gallbladder.
BLOODSBLOODS
Blood- LFT : AST 287, ALT 345, bilirubin 113.5,Blood- LFT : AST 287, ALT 345, bilirubin 113.5,
ALP737, LDH 855,ALP737, LDH 855,
FBC& U/E-NormalFBC& U/E-Normal
INR- 1.0INR- 1.0
Amylase-78Amylase-78
IMAGINGIMAGING
CXR & PFA.CXR & PFA.
US Gallstone, grossly distended GB, Markedly dilated CBD and IHBD.US Gallstone, grossly distended GB, Markedly dilated CBD and IHBD.
CT - abdomen intra & extra-hepatic bile duct dilatation to the level of thehepatic hilium. Suggestion of 2cm mass at hilium-?cholangiocarcinoma-(Klatskin tumour)CT - abdomen intra & extra-hepatic bile duct dilatation to the level of thehepatic hilium. Suggestion of 2cm mass at hilium-?cholangiocarcinoma-(Klatskin tumour)
MRCP-grossly dilated CBD/IHBR, abrupt narrowing of CBD with noobvious filling defect ?cholangiocarcinoma. Grossly distended GB.MRCP-grossly dilated CBD/IHBR, abrupt narrowing of CBD with noobvious filling defect ?cholangiocarcinoma. Grossly distended GB.
USS- Gallstone, grossly distended GB, Markedly dilated CBDand IHBD.USS- Gallstone, grossly distended GB, Markedly dilated CBDand IHBD.
CT- abdomen- intra & extra-hepatic bile duct dilatation to the level of thehepatic hilium. Suggestion of 2cm mass at hilium-?cholangiocarcinoma-(Klatskin tumour)CT- abdomen- intra & extra-hepatic bile duct dilatation to the level of thehepatic hilium. Suggestion of 2cm mass at hilium-?cholangiocarcinoma-(Klatskin tumour)
MRCP-grossly dilated CBD/IHBD, abrupt narrowing of CBD with noobvious filling defect ?cholangiocarcinoma. Grossly distended GB.MRCP-grossly dilated CBD/IHBD, abrupt narrowing of CBD with noobvious filling defect ?cholangiocarcinoma. Grossly distended GB.
ENDOSCOPIC RETROGRADECHOLANGIOPANCREATOGRAPHY-(ERCP).ENDOSCOPIC RETROGRADECHOLANGIOPANCREATOGRAPHY-(ERCP).
Performed twice- unsuccessful on bothoccassions.Performed twice- unsuccessful on bothoccassions.
PERCUTANEOUS TRANSHEPATICCHOLANGIOGRAPHY (PTC)PERCUTANEOUS TRANSHEPATICCHOLANGIOGRAPHY (PTC)
PTC was successfully performed with theplacement of a metal stent with good result-biliary decompression and relieve of herobstructive symptoms.PTC was successfully performed with theplacement of a metal stent with good result-biliary decompression and relieve of herobstructive symptoms.
KLATSKIN TUMOUR (KT)KLATSKIN TUMOUR (KT)
BG- Bile duct tumours recognized for over a century.BG- Bile duct tumours recognized for over a century.
Musser first reported 18 cases of primary extrahepaticbiliary cancer.Musser first reported 18 cases of primary extrahepaticbiliary cancer.
Sako and colleagues found 570 cases while reviewingliterature from 1935-1954.Sako and colleagues found 570 cases while reviewingliterature from 1935-1954.
Malignancy of the intrahepatic bile duct wasdescribed later by Altmeir (1957).Malignancy of the intrahepatic bile duct wasdescribed later by Altmeir (1957).
Gerald Klatskin described cancer of the hepatic ductbifurcation in 1965 following a review of 13 cases.Gerald Klatskin described cancer of the hepatic ductbifurcation in 1965 following a review of 13 cases.
KT tumours are generally small, sharply localizedand seldom metastasizing.KT tumours are generally small, sharply localizedand seldom metastasizing.
EpidemiologyEpidemiology
Tumours of bile duct are rare-2% of all cancers found at autopsy.Tumours of bile duct are rare-2% of all cancers found at autopsy.
Malignant tumours more common than benign adenomas andMalignant tumours more common than benign adenomas and
papillomas. papillomas.
Cholangiocarcinoma most common malignancy of bile ducts, >50%-Holland et at…2007.Cholangiocarcinoma most common malignancy of bile ducts, >50%-Holland et at…2007.
More common in Israel, Japan and American indiansMore common in Israel, Japan and American indians
Annual incidence of bile duct Ca in USA is 1/100,000 people. Autopsystudies show and incidence of 0.01-0.46%. 4,000 new cases reportedannually in USA.Annual incidence of bile duct Ca in USA is 1/100,000 people. Autopsystudies show and incidence of 0.01-0.46%. 4,000 new cases reportedannually in USA.
England and Wales - 2.8/100,000 females & 2/100,000 males.England and Wales - 2.8/100,000 females & 2/100,000 males.
ETIOLOGYETIOLOGY
Risk factors for bile duct cancer include:Risk factors for bile duct cancer include:
Ulcerative colitisUlcerative colitis
Primary sclerosing cholangitis-10-30%Primary sclerosing cholangitis-10-30%
Parasitic infestations:Liver fluke common in Far East-intrahepatic CC accounts for 20% of primary livertumour.Parasitic infestations:Liver fluke common in Far East-intrahepatic CC accounts for 20% of primary livertumour.
Opisthorchis viverrini-found inThailand, and WestMalaysia.Opisthorchis viverrini-found inThailand, and WestMalaysia.
ETIOLOGYETIOLOGY
Toxic chemicals-thorium dioxide (thorotrast),radionuclides, carcinogens-arsenic, nitrosaminesToxic chemicals-thorium dioxide (thorotrast),radionuclides, carcinogens-arsenic, nitrosamines
Congenital fibrosis or cysts-cogenital hepatic fibrosis,cystic dilatation, choledochal cyst, polycystic liverCongenital fibrosis or cysts-cogenital hepatic fibrosis,cystic dilatation, choledochal cyst, polycystic liver
Drugs: methyldopa, isoniazide, OCP.Drugs: methyldopa, isoniazide, OCP.
Gallstones and hepatolithiasis-decrease incidence >10years post cholecystectomy.Gallstones and hepatolithiasis-decrease incidence >10years post cholecystectomy.
Biliary cirrhosis and typhoid carriers.Biliary cirrhosis and typhoid carriers.
PATHOPHYSIOLOGYPATHOPHYSIOLOGY
Bile duct tumours cause bile duct obstruction - biliarystasis and alteration of liver function testsBile duct tumours cause bile duct obstruction - biliarystasis and alteration of liver function tests
Prolonged obstruction then leads to-Prolonged obstruction then leads to-
Hepatocellular dysfunction, renal dysfunctionHepatocellular dysfunction, renal dysfunction
Progressive malnutrition, Pruritus, coagulopathyProgressive malnutrition, Pruritus, coagulopathy
Cholangitis- esp if previous endoscopic, percutaneous orsurgical biliary interventions have been performed.Cholangitis- esp if previous endoscopic, percutaneous orsurgical biliary interventions have been performed.
Anatomically, biliary tree is divided into 3 parts, upper 3rd-55%, middle3rd 15% and lower 3rd 10%.Of these tumours, 10% are diffuse.Anatomically, biliary tree is divided into 3 parts, upper 3rd-55%, middle3rd 15% and lower 3rd 10%.Of these tumours, 10% are diffuse.
Bismuth ClassificationBismuth Classification
Type i-involvement of common hepatic duct.Type i-involvement of common hepatic duct.
Type ii-bifurcation involved without involvement ofsecondary intrahepatic duct.Type ii-bifurcation involved without involvement ofsecondary intrahepatic duct.
Type iiia-extends into the right secondary intrahepaticduct.Type iiia-extends into the right secondary intrahepaticduct.
Type iiib-extends into the left secondary intrahepatic duct.Type iiib-extends into the left secondary intrahepatic duct.
Type iv- secondary intrahepatic ducts involved on bothsides.Type iv- secondary intrahepatic ducts involved on bothsides.
PRESENTATIONPRESENTATION
CC seen in advanced unresectable stageCC seen in advanced unresectable stage
Early diagnosis unusualEarly diagnosis unusual
Typically elderly- average age 60-65years though Klatskinslightly younger age groupTypically elderly- average age 60-65years though Klatskinslightly younger age group
Abnormal LFTs / Jaundice-90%Abnormal LFTs / Jaundice-90%
Abdominal pain / Weight loss- in (30-50%) of cases -Patelet al 2006Abdominal pain / Weight loss- in (30-50%) of cases -Patelet al 2006
Pruritus seen in 66% of patientsPruritus seen in 66% of patients
PRESENTATIONPRESENTATION
Fever- 20%Fever- 20%
Diarrhoea, anorexia, changes in urine & stoolcolour and weight loss.Diarrhoea, anorexia, changes in urine & stoolcolour and weight loss.
Liver may be enlarged and smooth-25-40%Liver may be enlarged and smooth-25-40%
Distended and non tender gallbladder 10%Distended and non tender gallbladder 10%
Epigastric tenderness.Epigastric tenderness.
DIAGNOSISDIAGNOSIS
History / physical examinationHistory / physical examination
Labouratory-CEA and CA19.9 –sensitivity of 66% and aspecificity of 100% in diagnosing CC in pt with PSC.Labouratory-CEA and CA19.9 –sensitivity of 66% and aspecificity of 100% in diagnosing CC in pt with PSC.
Imaging-tumours are generally small-USS/ CT may fail toshow the lesion.Imaging-tumours are generally small-USS/ CT may fail toshow the lesion.
Cholangiography via a transhepatic or endoscopicapproach reqired to define biliary anatomy and extent ofthe lesion.Cholangiography via a transhepatic or endoscopicapproach reqired to define biliary anatomy and extent ofthe lesion.
DIAGNOSISDIAGNOSIS
Cholangiographic appearance of Klatskin tumour ischaracteristic.Cholangiographic appearance of Klatskin tumour ischaracteristic.
PTC preferred over ERCP for demonstrating ductalanatomy-PTC-almost 100% sensitivity & specificity.PTC preferred over ERCP for demonstrating ductalanatomy-PTC-almost 100% sensitivity & specificity.
MRCP non-invasive and now more available.MRCP non-invasive and now more available.
Histology –a well defferentiated adenocarcinoma-shortannular constricting lesion 75%, diffusely infiltrating withlong strictures 15%& intraluminal polypoid mass-3-5%Histology –a well defferentiated adenocarcinoma-shortannular constricting lesion 75%, diffusely infiltrating withlong strictures 15%& intraluminal polypoid mass-3-5%
TREATMENTTREATMENT
Management challenging with relatively poor prognosis.Management challenging with relatively poor prognosis.
Surgery continues to be the mainstay of therapy with 5yearsurvival of 10-40%.Surgery continues to be the mainstay of therapy with 5yearsurvival of 10-40%.
Complete resection with negative histologic margins –long-term survival.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 followingradical resection, 17months for palliative resection anddeath within 1.5years if no resection.Yang WL et at.. 2007 reported in a study of 185 cases(1972-2006) a median survival of 37 months followingradical resection, 17months for palliative resection anddeath within 1.5years if no resection.
Hepatic resection- a critical component of operativeapproach.Hepatic resection- a critical component of operativeapproach.
TREATMENTTREATMENT
Adjuvant chemoradiotherapy-no benefit.Adjuvant chemoradiotherapy-no benefit.
Liver transplant for unresectable tumour remainscontroversial, tumour recurrence >90%.Liver transplant for unresectable tumour remainscontroversial, tumour recurrence >90%.
Advances in interventional Radiology and endoscopy-facilitate non surgical option.Advances in interventional Radiology and endoscopy-facilitate non surgical option.
Benefit of external beam radiotherapy for palliation ofproximal CC uncertain.Benefit of external beam radiotherapy for palliation ofproximal CC uncertain.
Photodynamic therapy a new palliative treatment modalityfor failed stent. Thomas Zoepf et al concluded in a series in2008 -offers similar survival time as incomplete resection.Photodynamic therapy a new palliative treatment modalityfor failed stent. Thomas Zoepf et al concluded in a series in2008 -offers similar survival time as incomplete resection.
CONCLUSIONCONCLUSION
Klatskin tumour is tumour of bile ductbifurcation.Klatskin tumour is tumour of bile ductbifurcation.
Diagnosis can be quite challenging aspresentation is in an advanced stage with non-specific symptoms.Diagnosis can be quite challenging aspresentation is in an advanced stage with non-specific symptoms.
Surgery offers the only hope of cure.Surgery offers the only hope of cure.
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