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Klatskin tumor

Key sources
The following summarized guidelines for the evaluation and management of klatskin tumor are prepared by our editorial team based on guidelines from the American Society for Gastrointestinal Endoscopy (ASGE 2021; 2013), the American Society of Clinical Oncology (ASCO 2019), the American College of Radiology (ACR 2019), the European Society of Medical Oncology (ESMO 2016), and the American Association for the Study of Liver Diseases (AASLD 2014).
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Guidelines

1.Screening and diagnosis

Indications for screening: consider obtaining screening for biliary cancer only in at-risk patients, such as patients with PSC.
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2.Classification and risk stratification

Staging: use the AJCC/UICC (7th edition) staging system for biliary tract cancer with subclassifications for intrahepatic, perihilar and distal cholangiocarcinomas.
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3.Diagnostic investigations

Diagnostic imaging: as per ESMO 2016 guidelines, obtain initial radiological imaging in patients presenting with jaundice before ERCP or percutaneous transhepatic cholangiography, as the inserted drains/stents obscure the diagnosis and assessment of the extent of disease.
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4.Diagnostic procedures

Biopsy: perform ERCP-guided biopsy whenever possible over biliary brush cytology.
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5.Medical management

General principles: provide patients with biliary tract cancer a designated point of contact within the multidisciplinary team for advice and support (such as nurse specialist).
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6.Therapeutic procedures

Biliary stenting: as per ASGE 2021 guidelines, consider performing either endoscopic or percutaneous transhepatic biliary drainage in patients with unresectable malignant hilar obstruction undergoing palliative drainage. Make the final decision based on patient preferences, disease characteristics, and local expertise.
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7.Surgical interventions

Surgical resection: perform radical surgery with lymphadenectomy as the only curative treatment in patients with biliary tract cancer. Decide the exact nature and extent of surgery depending on tumor subtype/location agreed with specialist hepatobiliary multidisciplinary tumor board.
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8.Patient education

Patient education: inform patients about the likely duration of stent patency. Educate patients about symptoms and signs indicative of biliary obstruction or infection and instruct what they need to do in such an event.
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9.Follow-up and surveillance

Reassessment after good treatment response: reassess patients in the multidisciplinary team in the event of a good response to any treatment.
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