BSG Chol

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BSG Guidelines for the Diagnosis and Treatment of Cholangiocarcinoma:

BSG Guidelines for the Diagnosis and Treatment of Cholangiocarcinoma By Matt Johnson

Background to Guidelines:

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:

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:

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:

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


Pathology WHO Classification of Intrahepatic Carcinomas Hepatocellular Ca Combined Hepatocellular Cholangiocarcinoma Cholangiocarcinoma, intrahepatic Bile duct cystadenocarcinoma Undifferentiated carcinoma


Pathology WHO Classification of Extrahepatic bile duct carcinomas Carcinoma in situ (* = not graded ) Adenocarcinoma ( 95%, graded 1-4 on glandular tissue ) Papillary adenocarcinoma (*) Adenocarcinoma, intestinal type Mucinous adenocarcinoma Clear Cell adenocarcinoma (*) Signet ring adenocarcinoma ( grade 3 ) Adeno-squamous carcinoma Squamous carcinoma ( graded on degree of undifferentiation ) Small cell carcinoma (“oat cell”) ( grade 4 ) Undifferentiated carcinoma

Molecular Diagnosis:

Molecular Diagnosis Inactivation of tumour suppressor genes P53, APC, Smad-4, bcl-2, p16 Mutations in Oncogenes K-ras, C-myc, C-erbB-2, C-neu Chromasomal aneuploidy 25% of perihilar tumours These mutations can lead to phenotypic changes Diagnostic and prognostic usefullness is unclear

Clinical Features:

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

Blood tests:

Blood tests Obstructive LFTs Transaminases (us. Normal, high with acute obstruction) Deficiency in Vit D,E,A,K (in chronic obstruction) Chronic Systemic Markers (Hb, Alb, LDH) CA 19-9 (85%) Can be elevated by obstruction alone > 100 U/ml = sensitivity of 75% and specificity of 80% CA 125 (40-50%) May signify the presence of peritoneal involvement) CEA (30%)


Imaging U/S CT + MRI + MRCP + MRA Cholangiography (MRCP, ERCP, PTC) EUS PET Intra ductal U/S, endoscopic/percutaneous flexible cholangioscopy, radiolabelled ligand imaging


Imaging U/S 1 st line for obstruction Small lesions missed Colour doppler can reveal compression/ thrombosis of the portal V or hepatic A CT Localises lymphadenopathy (NB. + PSC) Enhanced spiral/ helical CT should be used if involvement of hilum or vascular system suspected MRI Optimal for anatomy + extent MRCP for ductal involvement MRA for hilar/vascular involvement


Imaging Cholangiography Essential for early diagnosis and assessing resectability MRCP is non-invasive ERCP (preferred) or PTC allows cytology and stenting Cytology (+ in 30%) Angiography will predict resectability NB = Biopsies should be avoided if resectability is possible


Staging TNM or Type 1 = limited to mucosa or muscle Type 2 = local invasion Type 3 = invasion of adjacent tissues or regional / hepatoD LNs (50%) Type 4 = extensive invasion +/- distant metastases 20% have perintoneal involvement at diagnosis

Imaging Recommendation:

Imaging Recommendation U/S (for initial screening) CXR MRI / MRCP or contrast enhanced spiral /helical CT Invasive Cholangiography For tissue diagnosis +/- therapeutic decompression Laparoscopy (if considered resectable)

Excluding Metastatic Disease:

Excluding Metastatic Disease Metastatic adenocarcinoma can mimic cholangiocarcinoma Pancreas = MRI, CT, EUS Stomach = AXR, OGD Breast = Ex, Mammography (if mass) Lung = CXR Colon = colonoscopy, spiral CT

Treatment – Curative Surgery:

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

Surgery - OLTx:

Surgery - OLTx Contra-indicated due to rapid recurrence and death within 3y Survival may improve in some with chemoirradiation

Palliative Procedures:

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

Resection Reporting:

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)


Oncology 50% at presentation are LN+ Treat patients early Aim for symptom control via a multidisciplinary group as this translates into survival advantage Aim to stabilize disease progress


Chemotherapy Response correlates to performance status at onset QOL is improved No evidence to support post-surgical adjuvant therapy No strong evidence for a survival benefit, but phase II trials suggest Single agent 5FU partial response = 10-20% Single agent gemcitabine partial response = 20-30% Combination partial response = 20-40% Gemcitabine + cisplatin partial response = 30-50% Downstaging can convert to operability


Radiotherapy External Beam XRT (+ChemoTx) Palliation of painful mets + bleeding No evidence for adjuvant post op XRT No improvement of QOL or Survival in advanced tumours ? Chemoradiation Local radiation intra-operative or intra-luminal brachytherapy No good data to support their use could be promising

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