Background to Guidelines Mortality rates from intrahepatic cholangiocarcinoma have steeply risen over the course of the last 30y and continue to rise 1998+9 = 1000 deaths / year Men = Women Previously no clear national consensus for optimal diagnosis and treatment

Risk Factors Age (65% are >65y) PSC (lifetime risk = 5-15%) Chronic intraductal gall stones Bile duct adenoma + biliary papillomatosis Caroli’s disease (cystic dilatation of ducts) Lifetime risk = 7% Choledochal cysts (5% will transform with time) Smoking Chemical Exposure ( Thorotrast, aircraft, rubber) Tropiocal (liver flukes, chronic typhoid carriers)

Anatomical Classification A) Intrahepatic (20-25%) B) Perihilar (50-60%) “Klatskin” = involve the duct bifurcation Many are coded as intrahepatic C) Distal Extrahepatic (20-25%) D) Multifocal (5%)
Bismuth’s Perihilar Classification Type 1 Below the confluence Type 2 Involving the confluence but not the L or R duct Type 3 Occluding the CHD and involving either the L (IIIa) or the R (IIIb) hepatic duct Type 4 Multicentric or Involve the CHD + both the L and the R hepatic ducts

Clinical Features Obstructive Jaundice RUQ pain, Fever + Rigors Suggesting cholangitis Systemic (malaise, weight loss, fatigue) Deranged LFTs Usually present late (esp. prox tumours)

Treatment – Curative Surgery 5y Survival for intrahepatic Ca = 9-18% 5y Survival for proximal Ca = 9-18% 5y Survival for distal Ca = 20-30% Survival depends on stage with tumour free margins absence of LN involvement Median Survival With hilar involvement = 12-24/12 Without hilar involvement = 18-30/12

Palliative Procedures Stenting Reduces sepsis Improves survival Surgical bypass has not proved superior Irradiation Intraoperative Coeliac plexus block

Resection Reporting 1) Tumour Type Extent Blood / lymphatic involvement Perineural invasion (worse prognosis) 2) Margins 3) Regional LNs (peripancreatic = distant) 4) Additional pathological findings (PSC) 5) Metastases

Decompression Pre-op biliary drainage / stenting is not advised if resection being considered May be necessary in severely malnourished or in acute suppurative cholangitis Preop placement of biliary catheters may be a helpful technical aid when dissecting a proximal Ca

Stenting Complex CholangioCa MRCP will help planning management ? Bilateral > unilateral Plastic Vs Metal Metal stents in those due to survive >6/12 Metal = shorter hospital stay Stenosis of metal stents can be treated with Cotton-Leung plastic stent through lumen Mesh metal stent Semicovered stents (?reduce Ca ingrowth)

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