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:

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

Pathology:

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:

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:

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:

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:

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:

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:

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:

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:

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:

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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