Cholangiocarcinoma What is it Dr Shahid A Khan

AMMF Information Day at Imperial College London Cholangiocarcinoma (CCA) • What is it? •

Cholangiocarcinoma (CCA) • Cancer = a group of diseases involving abnormal cell growth with

CCA: Intrahepatic/ Perihilar/ Extrahepatic 50 -60% “Perihilar”: arise at bifurcation of main ducts -

Cholangiocarcinoma (CCA) • A cancer in a body organ can be primary or secondary

Epidemiology of CCA: Worldwide Incidence varies, reflecting geographical risk factors & genetic differences 8

ASMR of all parenchymal tumours, HCC, unspecified tumours and intra + extrahepatic CCA in

Studies from around the world show changing trends in Incidence/Mortality of CCA: • Intrahepatic

Intrahepatic CCA mortality increased 9% in M & F, 1990 -2008, reaching rates of

CCA: Causes (Aetiology) & Known Risk Factors • • • Primary sclerosing cholangitis Parasitic

CCA: Causes (Aetiology) & Known Risk Factors 14 Bergquist et al. 2015 Best Pract

CCA: How is diagnosed? • Symptoms not specific and occur late in the disease

Unmet Needs and Future (hope) in CCA • Greater awareness of this disease •

Acknowledgments NIHR Biomedical Research Centre Biomedical Research Council (BMRC) Imperial College Healthcare Trustees Page

Slides: 20

Download presentation

Cholangiocarcinoma – What is it? Dr Shahid A Khan Consultant Liver Specialist St Mary's Hospital Imperial College London AMMF Information Day at Imperial College London 10 th May 2016 St Mary’s Hospital Campus 1

AMMF Information Day at Imperial College London Cholangiocarcinoma (CCA) • What is it? • How many people are affected (epidemiology)? • What causes it? • How is it diagnosed? • What are the unmet needs? 2

Cholangiocarcinoma (CCA) • Cancer = a group of diseases involving abnormal cell growth with the potential to invade or spread to other parts of the body • Cholangiocarcinoma (CCA) is a cancer of the bile ducts 4

CCA: Intrahepatic/ Perihilar/ Extrahepatic 50 -60% “Perihilar”: arise at bifurcation of main ducts - p. CCA 20 -30% distal CBD - e. CCA 10 -20% arise in intrahepatic ducts of liver - i. CCA 5

Cholangiocarcinoma (CCA) • A cancer in a body organ can be primary or secondary • CCA is the second commonest primary liver tumour after Hepatocellular Carcinoma (HCC) • 5 -10% all primary liver cancers • Peak age 7 th decade • Slight male preponderance 6

Epidemiology of CCA: Worldwide Incidence varies, reflecting geographical risk factors & genetic differences 8

ASMR of all parenchymal tumours, HCC, unspecified tumours and intra + extrahepatic CCA in Men, Eng &Wales, 1968 - 1996 Page 9 Taylor-Robinson et al. , Gut 2001

Studies from around the world show changing trends in Incidence/Mortality of CCA: • Intrahepatic CCA↑ • Extrahepatic CCA↓ • CCA Overall↑ • Since mid-1990’s, i. CCA is commonest recorded cause of death from a primary liver tumour in England & Wales, ahead of HCC • Total deaths risen 30 -fold: 36 in 1968 to > 2100 in 2013 • Large rise in i. CCA Age-standardised Mortality Rates (ASMR): males 0. 1 to 1. 5; females 0. 05 to 1. 25 • Largest statistical increase in any tumour over this time period • Total deaths from HCC: 472 in 1968 to approx 1500 in 2014 10

Intrahepatic CCA mortality increased 9% in M & F, 1990 -2008, reaching rates of 1. 1/100, 000 men and 0. 75/100, 000 women Highest rates in UK, Germany, and France (1. 2– 1. 5/100, 000 men, 0. 8– 1. 1/100, 000 women) 11 Bertuccio P et al. Ann Oncol 2013

CCA: Causes (Aetiology) & Known Risk Factors • • • Primary sclerosing cholangitis Parasitic Infection (Opisthorcis viverrini, Clonorchis sinensis) Fibropolycystic Liver Disease Intrahepatic Biliary Stones Chemical Carcinogen Exposure Chronic Liver Disease Viral Hepatitis Obesity Type 2 Diabetes >70% of CCA cases in West have NO known risk factors 13

CCA: Causes (Aetiology) & Known Risk Factors 14 Bergquist et al. 2015 Best Pract Res Clin Gastro

CCA: How is diagnosed? • Symptoms not specific and occur late in the disease process – Discomfort, weight loss, jaundice, itching, sometimes dark urine, pale stool • Imaging – Ultrasound, CT, MRI scans – but the appearances are non-specific • Biopsies (various routes) – Can be difficult due to location • Tumour markers in blood – None are very accurate Hence most CCA cases are diagnosed very late 16

Unmet Needs and Future (hope) in CCA • Greater awareness of this disease • We need more accurate early diagnostic tools to enable more patients having curative surgery • Need for better 2 nd line treatments • Ongoing trials in advanced CCA – chemo; local techniques e. g. ablation • Future hope: CCA treatment will be more individualized, when the genetic profile of a tumor can predict sensitivity or resistance to an agent 18

Acknowledgments NIHR Biomedical Research Centre Biomedical Research Council (BMRC) Imperial College Healthcare Trustees Page 19 (donations from Mr. and Mrs. Barry Winter)