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Introduction: Cholangiocarcinoma (CCA) are sub-divided into intrahepatic (iCCA) or extrahepatic (eCCA). eCCA are further subdivided into perihilar (pCCA) and distal (dCCA). Current and previous versions of the WHO International Coding of Disease and Oncology classifications (ICD) have separate topography codes for iCCA and eCCA, but none for pCCA. Over recent decades, multiple studies report rising incidence rates of iCCA with declining rates of eCCA, without reference to pCCA. We hypothesised the lack of a specific code for pCCA has led to errors CCA coding, specifically with miscoding of pCCA as iCCA., Methods: Clinical notes of cases coded as hepatobiliary carcinoma using ICD-10 criteria (C22.1/Intrahepatic Bile Duct carcinoma, C24.0/Extrahepatic Bile Duct carcinoma, C23X/Malignant Neoplasm Gall Bladder, C22.0/Malignant Neoplasm Liver Cell Carcinoma) over a 2 year period (2015-2017), were reviewed by two independent clinicians at three independent UK regional HepatoPancreatoBiliary centres. The agreed final diagnosis was compared to the originally allocated ICD-10 code., Results: Of the 625 CCA cases fully reviewed, 226 were coded as C22.1/iCCA. 98 (43%) of these were true iCCA and coded correctly, while 76 cases (34%) were actually pCCA. 92% all pCCA cases were incorrectly coded as iCCA., Conclusion: CCA coding misclassification in UK HPB centres is common, particularly the miscoding of pCCA, which is extrahepatic and the commonest form of CCA, as iCCA. This may be contributing to apparent rising incidence rates of iCCA. Our findings confirm the need to implement distinct topographical codes for iCCA, pCCA and dCCA in future iterations of ICD., (Copyright © 2020 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Objectives: The objective of this study was to investigate the differences between cholangiocarcinoma (CC) subtypes in terms of incidence rate, clinical presentation, management and survival by applying a stable anatomical classification in a population-based study., Patients and Methods: Cancer data for the period 2000-2012 were obtained from a specialized digestive cancer registry in the Department of Calvados, France. Patients' files were checked, and the diagnosis was verified by two hepatobiliary surgeons. This approach prevents classifying perihilar cholangiocarcinoma (PHCC) as intrahepatic cholangiocarcinoma (ICC), thereby allowing an accurate estimation of their respective epidemiological characteristics., Results: A total of 320 patients with CC were included. ICC represented 41% (130), whereas PHCC and distal cholangiocarcinoma represented 36 (116) and 23% (74), respectively. The mean age at the time of diagnosis differed significantly between the three subtypes (P<0.05). ICC was discovered accidently more frequently than PHCC, which was associated significantly with clinical symptoms. No change in the incidence or survival rates of CC subtypes were noticed, except for PHCC, in which female individuals had a significantly shorter median and 5-year survival rate of 0% (P<0.05)., Conclusion: The frequency of PHCC is overestimated in the literature. The anatomical reclassification of CC subtypes shows the stability of their incidence and survival rates. Considering ICC and PHCC as two different entities implies the need to assign a specific topographic code for PHCC.

Background and Aims: It has been reported that the incidence of intrahepatic cholangiocarcinoma (ICC) has increased in the USA, while extrahepatic cholangiocarcinoma (ECC) has decreased or remained stable. However, neither the recent trends nor the effects of the misclassification of Klatskin tumors are known., Methods: Using the Surveillance, Epidemiology, and End Results program databases, we calculated the average annual age-adjusted incidence rates (AA-IRs) of ICC and ECC in 4-year time periods (1992-1995, 1996-1999, 2000-2003, 2004-2007). These AA-IRs were calculated with misclassified as well as correctly classified Klatskin tumors. AA-IRs were also calculated based on age, sex, and race. Multivariable Poisson regression models were used to evaluate the secular trends of ICC and ECC., Results: The AA-IR of ICC was 0.92 in 1992-1995 and 0.93 in 2004-2007, while the AA-IR of ECC increased from 0.70 in 1992-1995 to 0.95 in 2004-2007. There was no significant trend in AA-IR of ICC (p = 0.07), while there was a significant increase in ECC across the 4-year time periods (p < 0.001). Klatskin tumors comprised 6.7% of CCs with approximately 90 and 45% misclassified as ICC during 1992-2000 and 2001-2007, respectively. Adjusted Poisson models showed no significant differences in the temporal trend of ICC or ECC due to misclassification of Klatskin tumors., Conclusions: The incidence of ICC has remained stable between 1992 and 2007 with only slight fluctuations, while the incidence of ECC has been increasing. Misclassification of Klatskin tumors does not appear to play a significant role in the trends of CCs.

Background & Aims: Cholangiocarcinomas (CC) can be sub-divided into intrahepatic (IHCC) or extrahepatic (EHCC). Hilar or 'Klatskin' tumours are anatomically extrahepatic. Most international studies, also from the UK, report increasing IHCC and decreasing EHCC incidence. The second edition of the International Classification of Diseases for Oncology (ICD-O-2) assigned 'Klatskin' tumours a unique histology code (8162/3), but this was cross-referenced to the topography code for intrahepatic (IHBD) rather than extrahepatic bile duct tumours (EHBD). Under the third ICD-O edition, 'Klatskin' tumours are cross-referenced to either IHBD or EHBD. New editions of the ICD-O classification are adopted at different time points by different countries. We investigated the impact of changing ICD-O classifications and the potential misclassification of hilar/'Klatskin' tumours on bile duct tumour and CC incidence rates in England and Wales and the US. We also examined whether coding practices by cancer registries in England and Wales could be influencing these rates., Methods: We analysed age-standardised incidence rates (ASIR) in England and Wales for IHBD and EHBD tumours between 1990 and 2008, then transferred all 'Klatskin' tumours from IHBD to EHBD and reanalysed rates from 1995, when ICD-O-2 was introduced in the UK. We also compared trends in IHBD, EHBD, and 'Klatskin' tumours in England and Wales with those in the USSEER (Surveillance, Epidemiology and End Results) database. Coding practice at Cancer registry level in England and Wales was investigated via a questionnaire completed by all national cancer registries., Results: In England and Wales, 1990-2008, ASIR of IHBD cancers rose (0.43-1.84/100,000 population in males; 0.27-1.51 in females) but fell for EHBD (0.78-0.51/100,000 population in males; 0.62-0.39 in females). After transferring all 'Klatskin' tumours from IHBD to EHBD, there remained a marked increase in ASIR of IHBD cancers and a decrease in ASIR for EHBD, as only 1% of CC were reportedly 'Klatskin'. The US SEER data showed that ASIR for IHBD gradually rose from 0.59/100,000 population in 1990 to 0.91 in 2001, then sharply fell before plateauing at 0.60 by 2007. ASIR for EHBD remained relatively stable at around 0.80/100,000 population until 2001, then began increasing, to 0.97 by 2007. Annually, between 1995 and 2008, the vast majority of 'Klatskin' tumours in England and Wales were coded as IHBD. This was also the case in the SEER data until 2001, when the situation was reversed and subsequently most 'Klatskin' tumours were coded as EHBD. US trends coincide with a switch from ICD-O2 to ICD-O-3 in 2001. In the UK, the switch to ICD-O-3 only occurred in 2008. On questioning, cancer registries in England and Wales stated they would not code a CC described as 'hilar' with the designated 'Klatskin' histology code. If the tumour site is unspecified, most registries classify CC as intrahepatic., Conclusions: Changes in ICD-classification may be influencing observed changes in IHBD and EHBD incidence rates. Coding misclassification is likely to have been skewing CC registration to an intrahepatic site, thereby contributing to the previously reported rise in intrahepatic tumours., (Copyright © 2011 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.)

Background: The term "cholangiocarcinoma" was originally used only for intrahepatic bile duct (adeno)carcinomas, but is now regarded as inclusive of intrahepatic, perihilar, and distal extrahepatic tumors of the bile ducts. A rise in incidence of intrahepatic cholangiocarcinoma has been recently reported in Western countries but comparatively little is known about recent cholangiocarcinoma incidence trends in East and South-Eastern Asia., Methods: We compared age-adjusted incidence rates of both intrahepatic and extrahepatic cholangiocarcinomas, as well as coding practices, using data from 18 cancer registries in Asia and 4 selected registries in Western countries. Intrahepatic cholangiocarcinoma incidence rates were calculated after reallocation of cases with unknown or unspecified histology among liver cancer cases., Results: Age-adjusted incidence rates of intrahepatic cholangiocarcinoma varied by more than 60-fold by region. The highest rates were found in Khon Kaen, Thailand, where 90% of liver tumors were cholangiocarcinomas. The next highest rates were found in the People's Republic of China, followed by the Republic of Korea. The highest age-adjusted incidence rate for extrahepatic cholangiocarcinoma was found in Korea. Coding practices for perihilar (Klatskin tumor) or unspecified sites of cholangiocarcinoma differed from one cancer registry to the other. The proportion of Klatskin tumors among cholangiocarcinomas was less than the one reported in clinical settings., Conclusion: Developing a consistent and uniform topographical classification for acceptable coding practice to all health professionals is necessary. In addition, epidemiological research on risk factors according to anatomical location (intrahepatic versus extrahepatic) and the macroscopic appearance and/or new histological classification of cholangiocarcinoma is also needed.

Cholangiocarcinomas are topographically categorized as intrahepatic or extrahepatic by the International Classification of Diseases for Oncology (ICD-O). Although hilar cholangiocarcinomas (Klatskin tumors) are extrahepatic cholangiocarcinomas, the second edition of the ICD-O (ICD-O-2) assigned them a histology code 8162/3, Klatskin, which was cross-referenced to intrahepatic cholangiocarcinoma. Recent studies in the United States that included this code (8162/3, Klatskin) with intrahepatic cholangiocarcinoma reported an increasing incidence of intrahepatic cholangiocarcinoma and a decreasing incidence of extrahepatic cholangiocarcinoma. To investigate the impact of this misclassification on site-specific cholangiocarcinoma incidence rates, we calculated annual percent changes (APCs) with data from the Surveillance, Epidemiology, and End Results (SEER) program using a Poisson regression model that was age-adjusted to the year 2000 U.S. population. All statistical tests were two-sided. During 1992-2000, when SEER used ICD-O-2, 1710 intrahepatic cholangiocarcinomas, 1371 extrahepatic cholangiocarcinomas, and 269 hilar cholangiocarcinomas identified by code 8162/3, Klatskin were diagnosed. Ninety-one percent (246 of 269) of the hilar cholangiocarcinomas were incorrectly coded as intrahepatic cholangiocarcinomas, resulting in an overestimation of intrahepatic cholangiocarcinoma incidence by 13% and underestimation of extrahepatic cholangiocarcinomas incidence by 15%. However, even after the exclusion of tumors that were coded to the histology code 8162/3, Klatskin, age-adjusted annual intrahepatic cholangiocarcinoma incidence increased during this period (APC = 4%, 95% confidence interval = 2% to 6%, P<.001).