An overview of CCA: Why better coding is needed - PowerPoint Presentation

1. An overview of CCA:Why better coding is needed

2. There is currently a lack of cholangiocarcinoma (CCA)-specific educational resources that are designed to support the adoption of ICD-11 among healthcare professionals and those involved in coding. This ICD-11 resource aims to: Provide an educational overview of the coding changes for each CCA classification Highlight the importance and benefits of widespread adoption of ICD-11 Provide material for discussion and knowledge sharing on the topic Encourage adoption of the new classifications among HCPs and coders1234Objectives

3. What is cholangiocarcinoma?Cholangiocarcinoma (CCA) is a cancer of the bile ducts.There are three distinct subtypes depending on the location of the tumor1:Intrahepatic (iCCA) – above the second order bile ductsPerihilar (pCCA) – in the right and left hepatic ducts to the cystic ductDistal/extrahepatic (dCCA) – in the common bile duct, below the cystic ductThe different subtypes have differences in risk factors, clinical diagnosis/presentation, and management. Image adapted from Kendal et al. 2019.21. Banales JM, et al. Nat Rev Gastroenterol Hepatol. 2020;17(9):557-588. 2. Kendal T, et al. Liver Int. 2019;39,Suppl 1:7-18.BAPerihilar CCADistal CCAIntrahepatic CCASeptal and interlobular ductsSegmental ducts Left hepatic ductCommon hepatic ductRight hepatic ductCystic duct

4. Signs and symptoms of CCA1CCA, cholangiocarcinoma.1. Banales JM, et al. Nat Rev Gastroenterol Hepatol. 2020;17(9):557-588.Abdominal pain(in right-upper quadrant)Weight lossFeverFatigue Yellowing of the skin and whites of the eye Hard to flush pale stools and dark urineItchy skinEarly stagesOften asymptomatic, though some patients experience vague symptoms such as: Late stagesClinical features depend on the location of the tumor:Intrahepatic CCA – generally presents with a constant, dull, abdominal pain.Perihilar and distal CCA – painless jaundice, resulting in:

5. Risk factors1,2Most cases of CCA arise sporadically, but there are some known risk factors, such as:CCA, cholangiocarcinoma.1. Banales JM, et al. Nat Rev Gastroenterol Hepatol. 2020;17(9):557-588. 2. Banales JM, et al. Nat Rev Gastro Hepat. 2016;13:261-280.Age >65 yearsNon-alcoholic fatty liver diseaseDiabetes mellitusGeneral risk factorsPrimary sclerosing cholangitisCaroli’s diseaseLiver cirrhosisMore common risk factorsChronic pancreatitisCholelithiasisInfection with liver flukes (Southeast Asia)Inflammatory bowel diseaseObesityHepatitis B or CCholedochalcystCholedocholithiasis

6. Incidence and mortalityIn Western countries, CCA affects <6/100,000 persons each year.1 In Southeast Asia, specifically Thailand, South Korea, and China, the incidence of CCA is higher, affecting >6/100,000 persons each year.1 Over the last two decades, mortality rates for CCA have been increasing globally.2Variations in incidence and mortality are likely due to differences in local risk factors and potential genetic predispositions.1However, the current data on incidence and mortality may be inaccurate due to the miscoding of CCA.1,3CCA, cholangiocarcinoma; eCCA, extrahepatic cholangiocarcinoma; iCCA, intrahepatic cholangiocarcinoma. 1. Banales JM, et al. Nat Rev Gastro Hepat. 2016;13:261-280. 2. Banales JM, et al. Nat Rev Gastroenterol Hepatol. 2020;17(9):557-588. 3. Selvadurai, et al. Eur J Surg Oncol. 2021;47(3 Pt B):635-639.YearSoutheast Asia†Australasia‡Europe§North America‖South America¶20024.33–6.521.89–2.041.09–3.681.05–1.710.32–1.3120074.31–6.192.47–2.591.12–3.791.00–2.050.32–1.4320124.36–5.852.43–2.881.71–4.041.17–2.710.45–1.47*Adapted from Banales et al. 2020.2 Countries with available data include: †Japan and Hong Kong; ‡Australia and New Zealand; §the Netherlands, Norway, Finland, Denmark, Sweden, Czech Republic, Hungary, Lithuania, Austria, Croatia, Belgium, United Kingdom, Germany, France, Portugal, Italy, Switzerland, Spain; ‖Canada, Untied States of America, Mexico, Puerto Rico; ¶Venezuela, Colombia, Brazil, Chile, Argentina. Age-standardized annual mortality rates for CCA per 100,000 persons*

7. Treatment of CCASurgery is the only potential curative treatment for CCA, however only ~25% of patients are eligible for resection.1,2For patients with iCCA who have undergone surgical resection, 6 months of adjuvant chemotherapy with capecitabine is recommended.First-line treatment for patients with unresectable tumors is cisplatin and gemcitabine.1,2Second-line treatment includes FOLFOX.1,2Some countries use radiation therapy in combination with chemotherapy or as adjuvant therapy after surgery.1,2Immunotherapies and targeted therapies are being investigated for patients with specific mutations – pemigatinib is already approved in the US, UK, and Europe for those with an FGFR2-fusion.3–5CCA, cholangiocarinoma; iCCA, intrahepatic cholangiocarcinoma; FOLFOX, folinic acid, fluorouracil, and oxaliplatin.1. Banales JM, et al. Nat Rev Gastroenterol Hepatol. 2020;17(9):557-588. 2. National Cancer Institute. https://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq. Accessed Oct 14, 2021. 3. U.S. FDA. https://www.fda.gov/drugs/resources-information-approved-drugs/fda-grants-accelerated-approval-pemigatinib-cholangiocarcinoma-fgfr2-rearrangement-or-fusion. Published Apr 20, 2020. Accessed Oct 14, 2021. 4. European Medicines Agency. https://www.ema.europa.eu/en/medicines/human/EPAR/pemazyre. Accessed Oct 14, 2021. 5. National Institute for Health and Care Excellence. https://www.nice.org.uk/guidance/ta722/chapter/1-Recommendations. Published Aug 25, 2021. Accessed Oct 14, 2021.

8. Surgical treatment of CCA differs depending on type*Intrahepatic CCAPerihilar/hilar CCADistal CCAMobilization of affected liverDivision of the bile duct within the pancreasMobilization of the pancreatic head and division of the pancreatic neckResection of affected liver +/− cholecystectomyExtensive neurectomy and lymphadenectomyCholecystectomy and division of bile duct with regional lymphadenectomyClosure of abdomen Mobilization and resection of the affected liver or to gain greater duct clearanceReconstruction with gastrojejunostomy, pancreaticojejunostomy, and hepaticojejunostomyResection of the caudate lobe (segment 1)Closure of the abdomenBilateral resection and reconstruction of the portal vein and hepatic artery when necessaryHepaticojejunostomy to individual segmental or subsegmental ducts within the hepatic remnantClosure of the abdomen*Information provided by Hassan Malik, Aintree University Hospital, Liverpool, UK.CCA, cholangiocarcinoma.

9. International Statistical Classification of Diseases and Related Health Problems (ICD)1,2The ICD is the diagnostic classification standard for all clinical and research purposes.It can be used to calculate health trends and statistics globally.There have been various iterations of the ICD since it’s inception.ICD-10 was introduced in 1983. The International Classification of Diseases for Oncology (ICD-O) was introduced in 1976 as a special adaption of ICD for those requiring greater coding detail of the topography and histology of a neoplasm. The 3rd Edition was introduced in 2000. 1. World Health Organization. International Statistical Classification of Diseases and Related Health Problems (ICD). https://www.who.int/standards/classifications/classification-of-diseases. Accessed Oct 14, 2021. 2. World Health Organization. ICD-O Third Edition. https://apps.who.int/iris/bitstream/handle/10665/96612/9789241548496_eng.pdf. Accessed Oct 12, 2021.

10. Issues with the current coding systems (ICD-10 and ICD-O)ICD-10 has a code for iCCA (C22.1) and eCCA (C24.0), but not for pCCA.1ICD-O has a histology code for pCCA (8162/3) but sometimes cross-references this to iCCA and sometimes eCCA.2A retrospective analysis of 625 CCA cases across three independent UK regional hepatobiliary centers revealed that of 226 iCCA cases reported, only 43% were coded correctly, while 34% were actually pCCA.392% of all pCCA cases were incorrectly coded as iCCA.3Image adapted from Selvadurai et al. 2021.3CCA, cholangiocarcinoma; eCCA, extrahepatic cholangiocarcinoma; GB, gall bladder; iCCA, intrahepatic cholangiocarcinoma; pCCA, perihilar cholangiocarcinoma.1. ICD-10 Version:2010. https://icd.who.int/browse10/2010/en. Accessed Oct 12, 2021. 2. World Health Organization. ICD-O Third Edition. https://apps.who.int/iris/bitstream/handle/10665/96612/9789241548496_eng.pdf. Accessed Oct 12, 2021. 3. Selvadurai, et al. Eur J Surg Oncol. 2021;47(3 Pt B):635-639.

11. ICD-11 differentiates between the three different types of CCA12C12.10, intrahepatic cholangiocarcinoma “A carcinoma that arises from the intrahepatic bile duct epithelium in any site of the intrahepatic biliary tree. Grossly, the malignant lesions are solid, nodular, and grayish. Morphologically, the vast majority of cases are adenocarcinomas.”2C15.0, adenocarcinoma of biliary tract, distal bile duct “An adenocarcinoma that arises from the common bile duct distal to the insertion of the cystic duct.”2C18, malignant neoplasms of perihilar bile duct. “Includes left, right and common hepatic ducts to the origin of the cystic duct.”CCA, cholangiocarcinoma.1. ICD-11 for Mortality and Morbidity Statistics (Version : 05/2021). https://icd.who.int/browse11/l-m/en. Accessed Oct 14, 2021.

12. ICD-11 differentiates between the three different types of CCA1CCA, cholangiocarcinoma.1. ICD-11 for Mortality and Morbidity Statistics (Version : 05/2021). https://icd.who.int/browse11/l-m/en. Accessed Oct 14, 2021.

13. Other important key changes from ICD-101**Information provided by Robert Jakob, World Health Organization, Geneva, CH. †Such as stage/grade or histopathology, medications used. stat, statistical; URI, uniform resource identifier.1. World Health Organization. ICD–11 Fact Sheet. https://icd.who.int/en/docs/icd11factsheet_en.pdf. Accessed Oct 14, 2021.ICD-1014,000 categoriesSeparate text indexSeparate rule baseNeed terminology linkOutdated content ICD-11Two levels of categoriesKnowledge network: Stem codes with additional extension codes for detail†17,000 stat. categories (codes)Codes and URI80,000 entitiesURI code combinations120,000 termsFully electronicTranslation tool>1,600,000 terms with formalized postcoordination

14. Other uses for ICD-1111. World Health Organization. International Statistical Classification of Diseases and Related Health Problems (ICD). https://www.who.int/standards/classifications/classification-of-diseases. Accessed Oct 14. 2021. Casemix, costing, resources, and cancer registration Cause of deathClinical terms, records, and surveillancePatient safety, drug safety, and device safetyPrimary care, prevention, and researchFunctioning assessmentICD-11

15. Importance of getting coding right for CCAICD-11 allows for:Better understanding of epidemiology of disease, so that we do not skew incidence/mortality ratesFacilitating future research – identifying a patient’s suitability for clinical trialsBilling and costing – each subtype requires different operating procedures associated with expertise, theater time, equipment, hospital length stay, etc.ICD-11 will come into effect from January 2022CCA, cholangiocarcinoma.