Materiały źródłowe

• #1 Cholangiocarcinoma 2020: the next horizon in mechanisms and management | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/s41575-020-0310-z

  Cholangiocarcinoma (CCA) includes a cluster of highly heterogeneous biliary malignant tumours that can arise at any point of the biliary tree. Their incidence is increasing globally, currently accounting for ~15% of all primary liver cancers and ~3% of gastrointestinal malignancies. The silent presentation of these tumours combined with their highly aggressive nature and refractoriness to chemotherapy contribute to their alarming mortality, representing ~2% of all cancer-related deaths worldwide yearly. The current diagnosis of CCA by non-invasive approaches is not accurate enough, and histological confirmation is necessary. Furthermore, the high heterogeneity of CCAs at the genomic, epigenetic and molecular levels severely compromises the efficacy of the available therapies. […] The global mortality for CCA increased worldwide during the periods 2000-2004, 2005-2009 and 2010-2014, according to the WHO and Pan American Health Organization databases for 32 selected locations in Europe, America, Asia and Oceania. Furthermore, CCA mortality was higher in men than in women worldwide, and in countries/regions in Asia versus those in the West.

• #2 Cholangiocarcinoma – Diagnosis & Disease Information

  https://www.cancertherapyadvisor.com/ddi/cholangiocarcinoma/

  Cholangiocarcinoma, commonly known as bile duct cancer, is a rare but aggressive malignancy originating in the bile ducts.1 This cancer can occur at any point along the bile ducts, including within the liver (intrahepatic), at the liver hilum (perihilar), or outside the liver (distal extrahepatic).1,2 Because it is often asymptomatic in early stages and resistant to standard chemotherapies, cholangiocarcinoma is characterized by a late presentation and poor prognosis.1 Early diagnosis is critical but challenging, which makes advanced imaging techniques and biopsies essential for accurate detection and staging.1,2 Treatment typically involves a combination of surgical resection, chemotherapy, and radiation therapy, but the survival rate remains low.1 […] Globally, cholangiocarcinoma accounts for approximately 3% of all gastrointestinal cancers and is the second most common primary liver cancer (after hepatocellular carcinoma), making up 10% to 15% of cases of liver cancer.2,3 In the United States, the incidence of cholangiocarcinoma has been rising over the past few decades, with an estimated annual occurrence of 1.6 cases per 100,000 people.2 Cholangiocarcinoma predominantly affects older adults, typically presenting in the seventh decade of life, with a slight male predominance.4

• #3 Cholangiocarcinoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560708/

  Cholangiocarcinomas comprise about 3% of gastrointestinal malignancies, are the second most common primary liver tumors, and account for approximately 10% to 15% of all hepatobiliary malignancies. The incidence of intrahepatic cholangiocarcinoma has been rising, possibly due to improved diagnostic and classification techniques, while the incidence of extrahepatic lesions has been falling in recent years. The incidence of cholangiocarcinoma varies markedly according to the geographic area; the incidence rates are up to 50-fold higher in parts of Thailand than in the United States. The incidence of cholangiocarcinoma increases with age, is slightly more common in men, and is most commonly diagnosed between the ages of 50 and 70 years. […] Perihilar cholangiocarcinoma is the most commonly encountered subtype, accounting for approximately 50% of cases. Distal (40%) and intrahepatic cholangiocarcinoma (10%) are less common.

• #4 Statistics About Bile Duct Cancer | Cholangiocarcinoma Stats | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/about/key-statistics.html

  Bile duct cancer (cholangiocarcinoma) is rare in the United States, but more common in Southeast Asia. It occurs most often in older people. […] Bile duct cancer (cholangiocarcinoma) is rare. About 8,000 people in the United States are diagnosed with it each year. This includes both intrahepatic (inside the liver) and extrahepatic (outside the liver) bile duct cancers. But the actual number of cases is likely to be higher, because these cancers can be hard to diagnose, and some might be misclassified as other types of cancer. […] Bile duct cancer is more common in Southeast Asia. This is mostly because liver flukes (a parasitic infection that can cause bile duct cancer) are much more common there. […] Bile duct cancer is seen mainly in older people, but it can occur in younger people. The average age of people in the US diagnosed with bile duct cancer is in the 70s. […] The chances of survival for people with bile duct cancer depend largely on its location and how advanced it is when it’s found.

• #5 Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq

  Cancer of the bile duct (also called cholangiocarcinoma) is extremely rare. The true incidence of bile duct cancer is unknown because establishing an accurate diagnosis is difficult. […] Approximately 50% of cholangiocarcinomas arise in the bile ducts of the perihilar region, 40% in the distal region, and 10% in the intrahepatic region. […] Many bile duct cancers are multifocal. In most patients, the tumor cannot be completely removed by surgery and is incurable. Palliative measures such as resection, radiation therapy (e.g., brachytherapy or external-beam radiation therapy), or stenting procedures may maintain adequate biliary drainage and allow for improved quality of life. […] Bile duct cancer may occur more frequently in patients with a history of primary sclerosing cholangitis, chronic ulcerative colitis, choledochal cysts, or infections with the liver fluke Clonorchis sinensis.

• #6 Cholangiocarcinoma: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/277393-overview

  Cholangiocarcinomas (CCCs) are malignancies of the biliary duct system that may originate in the liver and extrahepatic bile ducts, which terminate at the ampulla of Vater. […] Each year, approximately 2500 cases of cholangiocarcinoma occur, compared with 5000 cases of gallbladder cancer and 15,000 cases of hepatocellular cancer. The average incidence is one case per 100,000 population per year. […] A study by Singal et al found that the frequency of intrahepatic cholangiocarcinoma has increased over time and is most commonly noted in women older than 60 years. […] Worldwide, cholangiocarcinoma is the second most common primary hepatic malignancy, after hepatocellular carcinoma, comprising about 15% of all primary liver tumors. The incidence and mortality rates of cholangiocarcinoma have increased steadily over the past decades. Currently, cholangiocarcinoma has an incidence rate of 0.3-6/100,000 inhabitants per year, with a mortality rate of 1-6/100,000 inhabitants per year.

• #7 Cholangiocarcinoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/cholangiocarcinoma?lang=us

  Cholangiocarcinomas (commonest type of bile duct cancers) are malignant epithelial tumors arising from the biliary tree, excluding the gallbladder or ampulla of Vater. Cholangiocarcinoma is the third most common primary hepatobiliary malignancy after hepatocellular carcinoma (HCC) and gallbladder cancer. They tend to have a poor prognosis and high morbidity. […] Although overall cholangiocarcinoma is rare, there are significant regional variations in incidence with much higher rates seen in Southeast Asia and the Middle East. The incidence ranges from 0.3 to 6 per 100,000 inhabitants per year. […] Cholangiocarcinomas correspond to ~15% of all primary liver tumors and to ~3% of all gastrointestinal malignancies. […] A number of risk factors for cholangiocarcinoma have been identified, and bile stasis and chronic inflammation of the biliary epithelium are identified as common features among many of them.

• #8 Cholangiocarcinoma 2020: the next horizon in mechanisms and management | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/s41575-020-0310-z

  The age-standardized incidence of CCA shows considerable geographical variation, with the highest incidence in Eastern countries/regions; incidence varies from 85 per 100,000 in northeastern Thailand (the highest reported value globally) to 0.4 per 100,000 in Canada. […] Over the past few decades, the reported age-standardized incidence for iCCA has been steadily increasing in most locations worldwide, whereas the age-standardized incidence for eCCA has been decreasing. […] The three subtypes of CCA can have different risk factors, pathobiology, clinical presentations, management and prognosis, as well as distinct epidemiological trends. […] Therefore, a detailed study of these types of cancers is urgently needed to improve patient welfare and outcomes. Considering the high heterogeneity of CCAs, individual characterization of these tumours at the genomic, epigenetic and molecular levels is an indispensable approach to ascertain their pathogenesis, paving the path for new therapeutic options and personalized medicine.

• #9 Cholangiocarcinoma: Epidemiology and risk factors – Bragazzi – Translational Gastrointestinal Cancer

  https://tgc.amegroups.com/article/view/74/64

  The incidence of CCA shows large geographic variation. The description of the incidence of the CCA in the areas depicted in Figure 1, takes in consideration cases diagnosed either clinically (ICD-9, -10, others classifications) or cases verified by morphology (ICD-O-1, ICD-O-2, ICD-O-3). Very high incidence rates (85/100,000) has been reported in northeast Thailand, where CCA represents approx. 85% of total primitive liver cancers and other Asian countries such as China and Korea. […] The age-standardized incidence in New-Zeland (0.4/100,000), Costa Rica (0.3/100,00), Puerto Rico (0.35/100,000), Israel (0.35/100,000), Australia (0.3/100,000) and Canada (0.43/100,000) was estimated to be about a third the world-wide incidence. […] In Italy, in the last decade (1995-2005), CCA incidence was 3.36/100,000, where CCAs accounted approximately for 3% of total primitive liver cancers. An important linear increase in CCA incidence has been registered in the last two decades in Italy, crude incidence rates being higher for EH- than for IH-CCA, but the average percentage of increase per year was higher for IH-CCA.

• #10 Characteristics and outcomes of cholangiocarcinoma by region in Thailand: A nationwide study

  https://www.wjgnet.com/1007-9327/full/v23/i39/7160.htm

  Cholangiocarcinoma (CCA), a malignancy of the biliary tract epithelium, is of increasing importance due to its continually increasing incidence worldwide. The incidence of CCA varies substantially from region to region, with a high incidence in Asia and a low incidence in the United States and Europe. Variation in the global incidence of CCA is due to variations in risk factors, particularly host and environmental risk factors. Within Thailand, the incidence of CCA also varies geographically. The Northeast region of Thailand has the most CCA cases, with an incidence rate of 85 cases per 100000 persons per year, whereas the South region has the lowest number of cases at 5.7 cases per 100000 persons per year. The high incidence of CCA in Northeast Thailand is related to the high prevalence of liver fluke or Opisthorchis viverrini (OV) infection.

• #11 Statistics About Bile Duct Cancer | Cholangiocarcinoma Stats | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/about/key-statistics.html

  Bile duct cancer (cholangiocarcinoma) is rare in the United States, but more common in Southeast Asia. It occurs most often in older people. […] Bile duct cancer (cholangiocarcinoma) is rare. About 8,000 people in the United States are diagnosed with it each year. This includes both intrahepatic (inside the liver) and extrahepatic (outside the liver) bile duct cancers. But the actual number of cases is likely to be higher, because these cancers can be hard to diagnose, and some might be misclassified as other types of cancer. […] Bile duct cancer is more common in Southeast Asia. This is mostly because liver flukes (a parasitic infection that can cause bile duct cancer) are much more common there. […] Bile duct cancer is seen mainly in older people, but it can occur in younger people. The average age of people in the US diagnosed with bile duct cancer is in the 70s. […] The chances of survival for people with bile duct cancer depend largely on its location and how advanced it is when it’s found.

• #12 Bile Duct Cancer (Cholangiocarcinoma) and Liver Fluke Infection – Public Health

  https://www.publichealth.va.gov/exposures/infectious-diseases/cholangiocarcinoma.asp

  Bile duct cancer (cholangiocarcinoma) is a cancer of the biliary duct system, which includes the gallbladder, bile ducts, and certain cells inside the liver. It is a rare cancer but becomes more common as people grow older. […] Key risk factors for bile duct cancer are: Primary sclerosing cholangitis, Chronic ulcerative colitis, Cysts in the bile ducts, Cirrhosis of the liver, Hepatitis B or C virus, Diabetes, Obesity, Genetic factors. […] Another risk factor for bile duct cancer is past infection with tiny parasitic worms called liver flukes, which are found in the fresh waters of Southeast Asia. […] Veterans who ate raw or undercooked freshwater fish during their service in Southeast Asia, such as Vietnam War Veterans, might have been infected. However, currently VA is not aware of any studies that show that bile duct cancer occurs more often in U.S. Vietnam War Veterans than in other groups of people.

• #13 Cholangiocarcinoma 2020: the next horizon in mechanisms and management | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/s41575-020-0310-z

  Cholangiocarcinoma (CCA) includes a cluster of highly heterogeneous biliary malignant tumours that can arise at any point of the biliary tree. Their incidence is increasing globally, currently accounting for ~15% of all primary liver cancers and ~3% of gastrointestinal malignancies. The silent presentation of these tumours combined with their highly aggressive nature and refractoriness to chemotherapy contribute to their alarming mortality, representing ~2% of all cancer-related deaths worldwide yearly. The current diagnosis of CCA by non-invasive approaches is not accurate enough, and histological confirmation is necessary. Furthermore, the high heterogeneity of CCAs at the genomic, epigenetic and molecular levels severely compromises the efficacy of the available therapies. […] The global mortality for CCA increased worldwide during the periods 2000-2004, 2005-2009 and 2010-2014, according to the WHO and Pan American Health Organization databases for 32 selected locations in Europe, America, Asia and Oceania. Furthermore, CCA mortality was higher in men than in women worldwide, and in countries/regions in Asia versus those in the West.

• #14 Epidemiology and Risk Factors of Cholangiocarcinoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5290446/

  Cholangiocarcinoma (CCA) is the second most common primary liver cancer, being characterized by its late diagnosis and fatal outcome. Recent epidemiological reports indicate an increasing worldwide incidence of intrahepatic CCA but a decreasing incidence of extrahepatic CCA. […] Efficient strategies for the screening and surveillance of CCA have not been established so far. […] Several risk factors for CCA have been identified. Given the dismal prognosis of advanced CCA, regular surveillance examinations with a combination of ultrasonography and laboratory tests appear to be useful in patients at risk and need to be explored in prospective trials. […] Epidemiologic studies suggest that its incidence has been increasing in Western countries during the last decades. […] There are consistent reports of an increasing incidence of IH-CCA and a decreasing or stable incidence of EH-CCA from the World Health Organization (WHO) database.

• #15 Epidemiology and Risk Factors of Cholangiocarcinoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5290446/

  A very recent analysis from the Surveillance, Epidemiology, and End Results (SEER) database confirmed that the incidence of IH-CCA has risen by 128% in the USA between 1973 and 2012, whereas the incidence of EH-CCA remained stable. […] Cholangiocarcinogenesis has been associated with different forms of cholelithiasis. […] Surveillance including imaging, laboratory tests, and biliary cytology has been suggested for early cancer detection in patients with PSC as the major risk factor for CCA. However, there is a lack of consistent recommendations concerning the methods and frequency of investigations, pointing out an urgent need for the development of surveillance standards for patients with PSC and also for patients with other biliary disorders and cholelithiasis.

• #16 Biliary tract cancers: epidemiology, molecular pathogenesis and genetic risk associations – Marcano-Bonilla – Chinese Clinical Oncology

  https://cco.amegroups.org/article/view/12259/html

  Biliary tract cancers (BTC) are malignancies that arise from the epithelium of the biliary system and comprise the second most common type of hepatobiliary cancer worldwide. […] The geographic diversity of risk factors for the subtypes of biliary cancers results in profound differences in the worldwide incidence of each. […] In this article we provide a review of the current epidemiology of BTC and their associated risk factors. […] The incidence rate of GBC is highest in South America, specifically in the Andean region as discussed in further details in this issue by Arroyo et al. […] In the US the incidence of GBC is relatively low and actually decreased 0.5% per year between 1999-2011 among women (from 1.5 to 1.38 cases per 100,000) while remaining stable among men. […] Regarding the global epidemiology of CCA, the incidence of iCCA has been increasing in Europe, Asia, Japan, Australia, and North America.

• #17 Cholangiocarcinoma: a guide for the nonspecialist | IJGM

  https://www.dovepress.com/cholangiocarcinoma-a-guide-for-the-nonspecialist-peer-reviewed-fulltext-article-IJGM

  Cholangiocarcinoma (CCA) is a tumor with increasing prevalence around the world. The prevalence of CCA is highest in East Asia and most significantly in the countries through which the Mekong River flows, owing to the presence of liver flukes, which are consumed in raw fish dishes. Outside Asia, the causes of bile duct cancers for the most part are unknown. […] Recent studies have shown an unexplained global increase in CCA incidence and mortality. For instance, in England, the number of CCA deaths increased from 1,720 in 2010 to 2,161 in 2013. A similar trend was found in the United States; the incidence rate of iCCA increased by 165% in 30 years, from the 1970s to the 1990s, from 0.32 per 100,000 to 0.85 per 100,000. The geographical distribution of CCA cases is uneven, and it closely mirrors the prevalence of its predisposing risk factors. The vast majority of CCA cases in the Western developed nations have a sporadic nature. Primary sclerosing cholangitis (PSC), a chronic condition causing biliary obliterative fibrosis, is the commonest risk factor in Western countries, but it is associated with only 10% of cases, indicating the diversity of influencing factors on disease risk. The highest reported CCA incidence internationally is in Northeast of Thailand, 118.5 per 100,000, in Khon Kaen, which is some 100 times higher than the global rate. The high CCA burden is associated with liver fluke infestations that is endemic to countries through which the Mekong River traverses on its route from Yunnan Province in China through to its delta in southern Vietnam.

• #18 Intra-hepatic and extra-hepatic cholangiocarcinoma: New insight into epidemiology and risk factors

  https://www.wjgnet.com/1948-5204/full/v2/i11/407.htm

  In summary, with the exception of data from Denmark, studies investigating CCA epidemiology indicate a progressive worldwide increase of incidence and mortality for IH-CCA, whereas EH-CCA seems to be stable or slightly decreasing. Large differences in incidence and mortality between IH-CCA and EH-CCA observed in these studies suggest that these two types of CCA emerge from pathological conditions (risk factors) which are largely different. […] Several putative risk factors have been evaluated including, biliary diseases [choledochal cysts, cholangitis, primitive sclerosing cholangitis (PSC), biliary cirrhosis, primary biliary cirrhosis (PBC), cholelithiasias, choledocholithiasis, hepatolithiasis, cholecystitis], surgery (cholecystectomy), liver flukes, gastrointestinal diseases [inflammatory bowel diseases (IBD), duodenal ulcer, chronic pancreatitis], type II diabetes, cigarette smoking, alcohol intake, obesity and parenchymal liver diseases (chronic liver diseases, alcoholic liver disease, nonspecific cirrhosis, hemochromatosis, chronic non-alcoholic liver disease, HCV infection, HBV infection).

• #19 Epidemiology and risk factors: intrahepatic cholangiocarcinoma – Gupta – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/13610/html

  There are several risk factors that predispose to ICC, many of which have geographical prevalence. It should also be mentioned that almost 40% of patients diagnosed with ICC will have no currently identifiable risk factor, highlighting the need for further research in this area. […] Parasitic infection with the liver flukes Clonorchis sinensis and Opisthorchis viverrini, is a well-established ICC risk factor. These organisms cause bile duct inflammation predisposing to ICC, and have been designated as group 1 carcinogens by the World Health Organization. […] Primary sclerosing cholangitis (PSC) is a well-known risk factor for the development of ICC. The underlying biliary inflammation with resulting progenitor cell proliferation is thought to predispose to ICC formation. […] Viral hepatitis has recently been shown to be associated with ICC. Multiple studies in Europe and Asia have demonstrated a relationship between hepatitis C virus (HCV) and hepatitis B virus (HBV) infection and ICC.

• #20 Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®) – NCI

  https://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq

  Cancer of the bile duct (also called cholangiocarcinoma) is extremely rare. The true incidence of bile duct cancer is unknown because establishing an accurate diagnosis is difficult. […] Approximately 50% of cholangiocarcinomas arise in the bile ducts of the perihilar region, 40% in the distal region, and 10% in the intrahepatic region. […] Many bile duct cancers are multifocal. In most patients, the tumor cannot be completely removed by surgery and is incurable. Palliative measures such as resection, radiation therapy (e.g., brachytherapy or external-beam radiation therapy), or stenting procedures may maintain adequate biliary drainage and allow for improved quality of life. […] Bile duct cancer may occur more frequently in patients with a history of primary sclerosing cholangitis, chronic ulcerative colitis, choledochal cysts, or infections with the liver fluke Clonorchis sinensis.

• #21 Cholangiocarcinoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK560708/

  Cholangiocarcinomas comprise about 3% of gastrointestinal malignancies, are the second most common primary liver tumors, and account for approximately 10% to 15% of all hepatobiliary malignancies. The incidence of intrahepatic cholangiocarcinoma has been rising, possibly due to improved diagnostic and classification techniques, while the incidence of extrahepatic lesions has been falling in recent years. The incidence of cholangiocarcinoma varies markedly according to the geographic area; the incidence rates are up to 50-fold higher in parts of Thailand than in the United States. The incidence of cholangiocarcinoma increases with age, is slightly more common in men, and is most commonly diagnosed between the ages of 50 and 70 years. […] Perihilar cholangiocarcinoma is the most commonly encountered subtype, accounting for approximately 50% of cases. Distal (40%) and intrahepatic cholangiocarcinoma (10%) are less common.

• #22 Cholangiocarcinoma 2020: the next horizon in mechanisms and management | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/s41575-020-0310-z

  The age-standardized incidence of CCA shows considerable geographical variation, with the highest incidence in Eastern countries/regions; incidence varies from 85 per 100,000 in northeastern Thailand (the highest reported value globally) to 0.4 per 100,000 in Canada. […] Over the past few decades, the reported age-standardized incidence for iCCA has been steadily increasing in most locations worldwide, whereas the age-standardized incidence for eCCA has been decreasing. […] The three subtypes of CCA can have different risk factors, pathobiology, clinical presentations, management and prognosis, as well as distinct epidemiological trends. […] Therefore, a detailed study of these types of cancers is urgently needed to improve patient welfare and outcomes. Considering the high heterogeneity of CCAs, individual characterization of these tumours at the genomic, epigenetic and molecular levels is an indispensable approach to ascertain their pathogenesis, paving the path for new therapeutic options and personalized medicine.

• #23 Intra-hepatic and extra-hepatic cholangiocarcinoma: New insight into epidemiology and risk factors

  https://www.wjgnet.com/1948-5204/full/v2/i11/407.htm

  Cholangiocarcinoma (CCA) is a malignant tumour that arises from biliary epithelium at any portion of the bile duct system, from bile ductules to the ampulla of Vater. Recent evidences suggest that intra-hepatic CCA (IH-CCA) and EH-CCA are biologically different cancers, giving further support to a number of recent epidemiological studies showing large differences in terms of incidence, mortality and risk factors. The purpose of this manuscript is to review recent literature dealing with the descriptive epidemiology and risk factors of CCA with a special effort to compare IH- with EH-CCA. […] The incidence of IH-CCA (excluding hilar CCA) in the USA over the period from 1992 to 2000, showed a 4% annual increase. The incidence of EH-CCA (including code 8162/3) remained constant (annual percent changes = 1%), as did the incidence of hilar CCA reported under the code 8162/3.

• #24 Statistics About Bile Duct Cancer | Cholangiocarcinoma Stats | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/about/key-statistics.html

  Bile duct cancer (cholangiocarcinoma) is rare in the United States, but more common in Southeast Asia. It occurs most often in older people. […] Bile duct cancer (cholangiocarcinoma) is rare. About 8,000 people in the United States are diagnosed with it each year. This includes both intrahepatic (inside the liver) and extrahepatic (outside the liver) bile duct cancers. But the actual number of cases is likely to be higher, because these cancers can be hard to diagnose, and some might be misclassified as other types of cancer. […] Bile duct cancer is more common in Southeast Asia. This is mostly because liver flukes (a parasitic infection that can cause bile duct cancer) are much more common there. […] Bile duct cancer is seen mainly in older people, but it can occur in younger people. The average age of people in the US diagnosed with bile duct cancer is in the 70s. […] The chances of survival for people with bile duct cancer depend largely on its location and how advanced it is when it’s found.

• #25 Cholangiocarcinoma: MedlinePlus GeneticsLock

  https://medlineplus.gov/genetics/condition/cholangiocarcinoma/

  Cholangiocarcinoma is a group of cancers that begin in the bile ducts. […] Cholangiocarcinoma affects 8,000 people each year in the United States. […] For unknown reasons, cholangiocarcinoma occurs slightly more often in men than in women. […] Most people who develop cholangiocarcinoma are older than 65. […] Because this cancer is often not discovered until it has already spread, it can be challenging to treat effectively. […] Studies suggest that blood relatives of a person with cholangiocarcinoma may have an increased risk of developing this cancer compared with the general population. However, most people with cholangiocarcinoma do not have a family history of the disease.

• #26 Key Statistics – Cholangiocarcinoma Australia

  https://cholangiocarcinomaaustralia.org/key-statistics/

  Around 1300 people in Australia are diagnosed with Cholangiocarcinoma each year. […] About 23,000 people in the United States develop cholangiocarcinoma each year. […] Cholangiocarcinoma is much more common in Asia, mostly because of a common parasitic infection of the bile duct. […] Almost 2 out of 3 people with cholangiocarcinoma are 65 or older when it is found. […] The average age of people diagnosed with cancer of the intrahepatic bile ducts is 70. […] The average age of people diagnosed with cancer of the extrahepatic bile ducts (perihilar or distal cholangiocarcinoma) is 72. […] The chances of survival for patients with bile duct cancer depends to a large extent on its location and how advanced it is when it is found.

• #27 Cholangiocarcinoma – Wikipedia

  https://en.wikipedia.org/wiki/Cholangiocarcinoma

  Cholangiocarcinoma is a relatively rare form of cancer; each year, approximately 2,000 to 3,000 new cases are diagnosed in the United States, translating into an annual incidence of 12 cases per 100,000 people. […] There is a higher prevalence of cholangiocarcinoma in Asia, which has been attributed to endemic chronic parasitic infestation. The incidence of cholangiocarcinoma increases with age, and the disease is slightly more common in men than in women (possibly due to the higher rate of primary sclerosing cholangitis, a major risk factor, in men). […] Multiple studies have documented a steady increase in the incidence of intrahepatic cholangiocarcinoma; increases have been seen in North America, Europe, Asia, and Australia. […] The reasons for the increasing occurrence of cholangiocarcinoma are unclear; improved diagnostic methods may be partially responsible, but the prevalence of potential risk factors for cholangiocarcinoma, such as HIV infection, has also been increasing during this time frame.

• #28 Exploring Gender-Based Differences in the Incidence, Diagnosis, and Treatment of Cholangiocarcinoma | CCA News Online

  https://ccanewsonline.com/issues/2020/october-2020-vol-1-no-2/2688-exploring-gender-based-differences-in-the-incidence-diagnosis-and-treatment-of-cholangiocarcinoma

  Cholangiocarcinoma (CCA) is a rare type of cancer that arises from the intrahepatic or extrahepatic biliary ductal epithelium, accounting for approximately 10% to 15% of all primary hepatic malignancies. CCA is classified as intrahepatic or extrahepatic CCA. In the United States, the incidence of CCA has been rising consistently since 1973. […] The incidence of CCA is higher among men compared with women, with an increased disparity observed with increasing age. Based on an updated analysis of data from the 2000 to 2011 Surveillance, Epidemiology, and End Results (SEER) registry, the annual incidence of patients with intrahepatic CCA aged 80 years was 9.8 per 100,000 persons among men compared with 6.9 per 100,000 persons among women; during that period, the incidence of extrahepatic CCA was 10.4 per 100,000 persons among men compared with 7.0 per 100,000 persons per year among women.

• #29 Cholangiocarcinoma: Practice Essentials, Pathophysiology, Epidemiology

  https://emedicine.medscape.com/article/277393-overview

  Native Americans have the highest annual incidence in North America, at 6.5 cases per 100,000 people. This rate is about 6 times higher than that in nonNative American populations. […] According to the American Cancer Society, the number of new cases of liver and intrahepatic bile duct cancer in 2024 is estimated to be 28,000 for men and 13,630 for women, with deaths estimated at 19,120 and 10,720, respectively.

• #30 Exploring Gender-Based Differences in the Incidence, Diagnosis, and Treatment of Cholangiocarcinoma | CCA News Online

  https://ccanewsonline.com/issues/2020/october-2020-vol-1-no-2/2688-exploring-gender-based-differences-in-the-incidence-diagnosis-and-treatment-of-cholangiocarcinoma

  The increasing incidence of CCA in the United States was most evident in Asian patients aged 80 years, with an intrahepatic CCA incidence of 13.8 per 100,000 persons per year versus 7.2 per 100,000 persons per year in non-Hispanic white patients. The extrahepatic CCA incidence was 12.9 per 100,000 persons per year in Asian patients compared with 7.6 per 100,000 persons per year in non-Hispanic whites. […] Data from the ICPR revealed that women are more likely to undergo next-generation sequencing testing, and to test positive for alterations in TP53, IDH1, and KRAS, compared with men. […] The ICPR data show that women are more likely than men to receive treatment with platinum-based therapy, such as the XELOX or CAPOX regimen (intravenous oxaliplatin and oral capecitabine) regimen, fluorodeoxyuridine, and HER2-directed therapy.

• #31 The epidemiological trends of biliary tract cancers in the United States of America | BMC Gastroenterology | Full Text

  https://bmcgastroenterol.biomedcentral.com/articles/10.1186/s12876-022-02637-8

  The ethnic groups exhibiting the highest incidence rates of these different BTCs were American Indians and Alaska Natives for GBC, and Asian and Pacific Islanders for ICC, ECC, and AVC. The incidence of all of these forms of BTC rose with age. […] The epidemiological characteristics, staging, locoregional surgical treatments, adjuvant therapies, and prognostic outcomes were distinct for each of these BTCs. […] Several recent studies have suggested that BTC incidence is rising throughout the globe, with some changes in the prevalence of these tumors and their associated mortality rates in recent decades. Strikingly, the incidence of ICC has risen substantially, with an average annual increase of 4.36% over the last 10 years. […] Overall, the BTC incidence continued to rise over the analyzed 19-year period, primarily due to increases in the rates of ICC, in line with the findings from previous epidemiological studies.

• #32 Cholangiocarcinoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/cholangiocarcinoma?lang=us

  Cholangiocarcinomas (commonest type of bile duct cancers) are malignant epithelial tumors arising from the biliary tree, excluding the gallbladder or ampulla of Vater. Cholangiocarcinoma is the third most common primary hepatobiliary malignancy after hepatocellular carcinoma (HCC) and gallbladder cancer. They tend to have a poor prognosis and high morbidity. […] Although overall cholangiocarcinoma is rare, there are significant regional variations in incidence with much higher rates seen in Southeast Asia and the Middle East. The incidence ranges from 0.3 to 6 per 100,000 inhabitants per year. […] Cholangiocarcinomas correspond to ~15% of all primary liver tumors and to ~3% of all gastrointestinal malignancies. […] A number of risk factors for cholangiocarcinoma have been identified, and bile stasis and chronic inflammation of the biliary epithelium are identified as common features among many of them.

• #33 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies

  https://www.mdpi.com/2072-6694/15/20/4947

  Cholangiocarcinoma is a major concern in patients with primary sclerosing cholangitis, and it is associated with a high mortality rate. Recent data have demonstrated several differences between primary sclerosing cholangitis-associated cholangiocarcinoma and de novo cholangiocarcinoma in terms of epidemiology and pathogenesis, raising several questions about the pathological mechanisms and the best clinical approach to this tumor. […] Cholangiocarcinoma (CCA) is the most common malignancy in patients with primary sclerosing cholangitis (PSC), accounting for 2–8% of cases and being the leading cause of death in these patients. The majority of PSC-associated CCAs (PSC-CCA) develop within the first few years after PSC diagnosis. Older age and male sex, as well as concomitant inflammatory bowel disease (IBD) or high-grade biliary stenosis, are some of the most relevant risk factors.

• #34 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies

  https://www.mdpi.com/2072-6694/15/20/4947

  Despite PSC-CCA representing a small part of all biliary neoplasms, reaching an average frequency of almost 10%, CCA is the most frequent tumor in patients with PSC. Indeed, PSC is associated with 400–600-fold higher risk of developing CCA compared to the general population. The assessed annual risk ranges between 0.5% and 1.5%. According to that, PSC patients are affected by 5- and 10-year cumulative risk of developing CCA around 7% and 6–11%, respectively. […] The reported lifetime prevalence of PSC-CCA ranges from 6 and 13%. The risk of developing CCA tends to be higher soon after PSC diagnosis; in fact, approximately 30–50% of CCA are diagnosed within the first year. […] Despite the significative two-fold reduction of mortality in PSC patients who undergo scheduled imaging surveillance, CCA still accounts for almost 30% of all PSC-related deaths, being the main cause of death in these patients.

• #35 Cholangiocarcinoma: Epidemiology and risk factors – Bragazzi – Translational Gastrointestinal Cancer

  https://tgc.amegroups.com/article/view/74/64

  The incidence of both IH-CCA and EH-CCA is decreasing in Denmark, stable in France and increasing in Italy and Germany where the increase mainly affect the IH-CCA. […] The main risk factors are liver flukes in Asian countries, PSC and HCV in western countries. The IH-CCA frequently emerges in the setting of chronic liver diseases requiring differential diagnosis with respect to HCC. […] Hepatic infections by liver flukes (Clonorchis sinensis, Opisthorchis viverrini) are associated with the high incidence of IH- and EH-CCA in Asian countries. […] CCA is a heterogeneous devastating cancer with a worldwide increasing incidence and mortality.

• #36 Characteristics and outcomes of cholangiocarcinoma by region in Thailand: A nationwide study

  https://www.wjgnet.com/1007-9327/full/v23/i39/7160.htm

  Cholangiocarcinoma (CCA), a malignancy of the biliary tract epithelium, is of increasing importance due to its continually increasing incidence worldwide. The incidence of CCA varies substantially from region to region, with a high incidence in Asia and a low incidence in the United States and Europe. Variation in the global incidence of CCA is due to variations in risk factors, particularly host and environmental risk factors. Within Thailand, the incidence of CCA also varies geographically. The Northeast region of Thailand has the most CCA cases, with an incidence rate of 85 cases per 100000 persons per year, whereas the South region has the lowest number of cases at 5.7 cases per 100000 persons per year. The high incidence of CCA in Northeast Thailand is related to the high prevalence of liver fluke or Opisthorchis viverrini (OV) infection.

• #37 Bile Duct Cancer Risk Factors | Cholangiocarcinoma Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/causes-risks-prevention/risk-factors.html

  Scientists have found a few risk factors that make a person more likely to develop bile duct cancer. […] Certain conditions of the liver or bile ducts have been found to either cause bile duct cancer or to increase the risk of developing it. […] Primary sclerosing cholangitis (PSC) is a condition in which inflammation of the bile ducts (cholangitis) leads to the formation of scar tissue (sclerosis). […] Bile duct stones (hepatolithiasis) are a lot like gallstones, but much smaller. They can also cause inflammation that increases the risk of bile duct cancer. […] Choledochal cyst disease is a rare condition which some people are born with. […] Liver fluke infections can happen when you eat raw or undercooked fish that is infected with these tiny parasitic worms. […] Some people have abnormalities where the bile duct and pancreatic duct normally meet.

• #38 Biliary tract cancers: epidemiology, molecular pathogenesis and genetic risk associations – Marcano-Bonilla – Chinese Clinical Oncology

  https://cco.amegroups.org/article/view/12259/html

  Worldwide, the highest incidence rates of CCA are found in Thailand, which has age-standardized rates of 113 per 100,000 person years in males, and 50 per 100,000 person years in females. […] Other key factors influencing the risk of BTC are age, gender, race, and presence of comorbidities. […] The increasing number of BTC worldwide is linked to several important risk factors. […] The association of diabetes with BTC is difficult to assess due to the close associations of diabetes with obesity and gallstones, but many studies support the concept that obesity increases the risk of BTC. […] Patients with choledochal cysts and Carolis disease, a rare congenital fibropolycystic liver disease characterized by cystic dilations of the large intrahepatic bile ducts, appear to be at risk for iCCA.

• #39 Epidemiology and Risk Factors of Cholangiocarcinoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5290446/

  A very recent analysis from the Surveillance, Epidemiology, and End Results (SEER) database confirmed that the incidence of IH-CCA has risen by 128% in the USA between 1973 and 2012, whereas the incidence of EH-CCA remained stable. […] Cholangiocarcinogenesis has been associated with different forms of cholelithiasis. […] Surveillance including imaging, laboratory tests, and biliary cytology has been suggested for early cancer detection in patients with PSC as the major risk factor for CCA. However, there is a lack of consistent recommendations concerning the methods and frequency of investigations, pointing out an urgent need for the development of surveillance standards for patients with PSC and also for patients with other biliary disorders and cholelithiasis.

• #40 Intra-hepatic and extra-hepatic cholangiocarcinoma: New insight into epidemiology and risk factors

  https://www.wjgnet.com/1948-5204/full/v2/i11/407.htm

  Biliary diseases (cholangitis, PSC, choledochal cysts, biliary cirrhosis, choledocholithiasis and cholecystitis) are also indicated to be independent strong risk factors for both IH-CCA and EH-CCA as assessed by multivariate analysis. […] PSC, a disease affecting both intra-hepatic and extra-hepatic bile ducts, represents the strongest independent risk factor both for IH-CCA and for EH-CCA as evaluated by multivariate analysis. […] The association between PBC and EH-CCA, described by Welzel et al deserves some comment. PBC does not affect extra-hepatic bile ducts and, therefore, the reasons for increased susceptibility of cancer transformation in these large ducts are unclear. […] Hepatolithiasis has been indicated as a strong independent predictor of IH-CCA development, whereas none of the case-control studies have investigated the role of hepatolithiasis in EH-CCA.

• #41 Bile Duct Cancer Risk Factors | Cholangiocarcinoma Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/causes-risks-prevention/risk-factors.html

  Cirrhosis can be caused by irritants like alcohol and diseases like hepatitis or non-alcoholic fatty liver disease. Studies have found it increases the risk of bile duct cancer. […] Infection with hepatitis B virus or hepatitis C virus appears to be associated with increased risk for bile duct cancers. […] People with these diseases have an increased risk of bile duct cancer. […] Lynch syndrome, BAP1 tumor predisposition syndrome, cystic fibrosis, and multiple biliary papillomatosis are genetic disorders associated with an increased risk of bile duct cancer. […] Older people are more likely than younger people to get bile duct cancer. […] In the US, the risk of bile duct cancer is highest among Hispanic Americans. […] Having excess body weight or obesity can increase the risk of cancers of the gallbladder and bile ducts.

• #42 Epidemiology and risk factors: intrahepatic cholangiocarcinoma – Gupta – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/13610/html

  The development of nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepatitis (NASH) are clearly associated with development of chronic liver disease and cirrhosis. […] Tobacco smoking has been shown to be associated with increased risk of several different malignancies, and the same is true for ICC. Alcoholic liver disease has also been implicated in ICC development, further demonstrating the association between liver injury and ICC susceptibility. […] Cirrhosis has long been identified as a significant risk factor in HCC development, and a similar pathogenesis has been suggested for ICC in cirrhotics. Non-specific cirrhosis was identified to be a very strong predictor of ICC development.

• #43 Bile Duct Cancer Risk Factors | Cholangiocarcinoma Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/causes-risks-prevention/risk-factors.html

  Cirrhosis can be caused by irritants like alcohol and diseases like hepatitis or non-alcoholic fatty liver disease. Studies have found it increases the risk of bile duct cancer. […] Infection with hepatitis B virus or hepatitis C virus appears to be associated with increased risk for bile duct cancers. […] People with these diseases have an increased risk of bile duct cancer. […] Lynch syndrome, BAP1 tumor predisposition syndrome, cystic fibrosis, and multiple biliary papillomatosis are genetic disorders associated with an increased risk of bile duct cancer. […] Older people are more likely than younger people to get bile duct cancer. […] In the US, the risk of bile duct cancer is highest among Hispanic Americans. […] Having excess body weight or obesity can increase the risk of cancers of the gallbladder and bile ducts.

• #44 Intra-hepatic and extra-hepatic cholangiocarcinoma: New insight into epidemiology and risk factors

  https://www.wjgnet.com/1948-5204/full/v2/i11/407.htm

  Cholelithiasias was indicated as an independent predictor of EH-CCA development, but not of IH-CCA. […] Type II diabetes has been identified as an independent predictor of IH-CCA in 2/4 studies and of EH-CCA in 2/3 studies. […] Heavy alcohol intake (80 g/d) has been identified by multivariate analysis as an independent risk factor associated with both IH-CCA and EH-CCA. […] The evidence for a possible association between liver flukes and CCA has been evaluated by the International Agency for Research on Cancer (IARC) since 1994. Opisthorchis viverrini infestation, endemic in South East Asia, was considered definitively carcinogenic. […] The role of hepatitis viruses as risk factors for IH-CCA, independently of liver cirrhosis, needs to be further addressed together with the possible pathogenic mechanism responsible for this association.

• #45 Bile Duct Cancer Risk Factors | Cholangiocarcinoma Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/causes-risks-prevention/risk-factors.html

  Cirrhosis can be caused by irritants like alcohol and diseases like hepatitis or non-alcoholic fatty liver disease. Studies have found it increases the risk of bile duct cancer. […] Infection with hepatitis B virus or hepatitis C virus appears to be associated with increased risk for bile duct cancers. […] People with these diseases have an increased risk of bile duct cancer. […] Lynch syndrome, BAP1 tumor predisposition syndrome, cystic fibrosis, and multiple biliary papillomatosis are genetic disorders associated with an increased risk of bile duct cancer. […] Older people are more likely than younger people to get bile duct cancer. […] In the US, the risk of bile duct cancer is highest among Hispanic Americans. […] Having excess body weight or obesity can increase the risk of cancers of the gallbladder and bile ducts.

• #46 Intra-hepatic and extra-hepatic cholangiocarcinoma: New insight into epidemiology and risk factors

  https://www.wjgnet.com/1948-5204/full/v2/i11/407.htm

  Cholelithiasias was indicated as an independent predictor of EH-CCA development, but not of IH-CCA. […] Type II diabetes has been identified as an independent predictor of IH-CCA in 2/4 studies and of EH-CCA in 2/3 studies. […] Heavy alcohol intake (80 g/d) has been identified by multivariate analysis as an independent risk factor associated with both IH-CCA and EH-CCA. […] The evidence for a possible association between liver flukes and CCA has been evaluated by the International Agency for Research on Cancer (IARC) since 1994. Opisthorchis viverrini infestation, endemic in South East Asia, was considered definitively carcinogenic. […] The role of hepatitis viruses as risk factors for IH-CCA, independently of liver cirrhosis, needs to be further addressed together with the possible pathogenic mechanism responsible for this association.

• #47 Bile Duct Cancer Risk Factors | Cholangiocarcinoma Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/causes-risks-prevention/risk-factors.html

  Cirrhosis can be caused by irritants like alcohol and diseases like hepatitis or non-alcoholic fatty liver disease. Studies have found it increases the risk of bile duct cancer. […] Infection with hepatitis B virus or hepatitis C virus appears to be associated with increased risk for bile duct cancers. […] People with these diseases have an increased risk of bile duct cancer. […] Lynch syndrome, BAP1 tumor predisposition syndrome, cystic fibrosis, and multiple biliary papillomatosis are genetic disorders associated with an increased risk of bile duct cancer. […] Older people are more likely than younger people to get bile duct cancer. […] In the US, the risk of bile duct cancer is highest among Hispanic Americans. […] Having excess body weight or obesity can increase the risk of cancers of the gallbladder and bile ducts.

• #48

  https://ammf.org.uk/causes-and-risk-factors/

  Newly discovered likely risk factors include obesity, diabetes and fatty liver disease. […] However, the majority of people diagnosed in the West with cholangiocarcinoma have none of the risk factors mentioned above, their cholangiocarcinoma develops sporadically (ie, no known cause). […] To read the paper Cholangiocarcinoma: Epidemiology and risk factors (Shahid A Khan, Simona Tavolari, Giovanni Brandi), published in 2019 in the journal Liver International, which gives current findings on the causes and risk factors of cholangiocarcinoma, click here. […] To read the research article from Imperial College London, Risk factors for intrahepatic and extrahepatic cholangiocarcinoma: A systematic review and meta-analysis published in the Journal of Hepatology in 2019, click here.

• #49 Epidemiology and risk factors: intrahepatic cholangiocarcinoma – Gupta – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/13610/html

  The development of nonalcoholic fatty liver disease (NAFLD) and nonalcoholic steatohepatitis (NASH) are clearly associated with development of chronic liver disease and cirrhosis. […] Tobacco smoking has been shown to be associated with increased risk of several different malignancies, and the same is true for ICC. Alcoholic liver disease has also been implicated in ICC development, further demonstrating the association between liver injury and ICC susceptibility. […] Cirrhosis has long been identified as a significant risk factor in HCC development, and a similar pathogenesis has been suggested for ICC in cirrhotics. Non-specific cirrhosis was identified to be a very strong predictor of ICC development.

• #50 Bile Duct Cancer Risk Factors | Cholangiocarcinoma Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/causes-risks-prevention/risk-factors.html

  A radioactive substance called Thorotrast (thorium dioxide) was found to increase the risk for bile duct cancer, as well as other types of liver cancer. […] People with diabetes (type 1 or type 2) have been found to have a higher risk of bile duct cancer. […] People who drink alcohol are more likely to get intrahepatic bile duct cancer. […] Studies have found other factors may also increase the risk of bile duct cancer.

• #51 Intra-hepatic and extra-hepatic cholangiocarcinoma: New insight into epidemiology and risk factors

  https://www.wjgnet.com/1948-5204/full/v2/i11/407.htm

  Cholelithiasias was indicated as an independent predictor of EH-CCA development, but not of IH-CCA. […] Type II diabetes has been identified as an independent predictor of IH-CCA in 2/4 studies and of EH-CCA in 2/3 studies. […] Heavy alcohol intake (80 g/d) has been identified by multivariate analysis as an independent risk factor associated with both IH-CCA and EH-CCA. […] The evidence for a possible association between liver flukes and CCA has been evaluated by the International Agency for Research on Cancer (IARC) since 1994. Opisthorchis viverrini infestation, endemic in South East Asia, was considered definitively carcinogenic. […] The role of hepatitis viruses as risk factors for IH-CCA, independently of liver cirrhosis, needs to be further addressed together with the possible pathogenic mechanism responsible for this association.

• #52 Early detection of occupational cholangiocarcinoma in a high-risk patient under intensive surveillance: a case study | Surgical Case Reports | Full Text

  https://surgicalcasereports.springeropen.com/articles/10.1186/s40792-024-01871-4

  Occupational cholangiocarcinoma is associated with exposure to organic solvents, such as dichloromethane (DCM) and 1,2-dichloropropane (DCP). […] Given the high risk of cholangiocarcinoma development, the patient underwent regular monitoring with abdominal US and blood tests at a local doctor’s office. […] Continuous monitoring is key to the early detection and effective management of occupational cholangiocarcinoma. […] Occupational cholangiocarcinoma was initially recognized as an occupational disease in 2013 by the Japanese Ministry of Health, Labor, and Welfare owing to an outbreak of cholangiocarcinoma among employees of a printing company in Osaka, Japan. […] Exposure to high concentrations of DCM and/or DCP should raise concerns regarding late-onset carcinogenesis, even after exposure ceases.

• #53 Bile Duct Cancer Risk Factors | Cholangiocarcinoma Risk Factors | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/causes-risks-prevention/risk-factors.html

  A radioactive substance called Thorotrast (thorium dioxide) was found to increase the risk for bile duct cancer, as well as other types of liver cancer. […] People with diabetes (type 1 or type 2) have been found to have a higher risk of bile duct cancer. […] People who drink alcohol are more likely to get intrahepatic bile duct cancer. […] Studies have found other factors may also increase the risk of bile duct cancer.

• #54

  https://ammf.org.uk/causes-and-risk-factors/

  Newly discovered likely risk factors include obesity, diabetes and fatty liver disease. […] However, the majority of people diagnosed in the West with cholangiocarcinoma have none of the risk factors mentioned above, their cholangiocarcinoma develops sporadically (ie, no known cause). […] To read the paper Cholangiocarcinoma: Epidemiology and risk factors (Shahid A Khan, Simona Tavolari, Giovanni Brandi), published in 2019 in the journal Liver International, which gives current findings on the causes and risk factors of cholangiocarcinoma, click here. […] To read the research article from Imperial College London, Risk factors for intrahepatic and extrahepatic cholangiocarcinoma: A systematic review and meta-analysis published in the Journal of Hepatology in 2019, click here.

• #55 Epidemiology and Risk Factors of Cholangiocarcinoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5290446/

  Cholangiocarcinoma (CCA) is the second most common primary liver cancer, being characterized by its late diagnosis and fatal outcome. Recent epidemiological reports indicate an increasing worldwide incidence of intrahepatic CCA but a decreasing incidence of extrahepatic CCA. […] Efficient strategies for the screening and surveillance of CCA have not been established so far. […] Several risk factors for CCA have been identified. Given the dismal prognosis of advanced CCA, regular surveillance examinations with a combination of ultrasonography and laboratory tests appear to be useful in patients at risk and need to be explored in prospective trials. […] Epidemiologic studies suggest that its incidence has been increasing in Western countries during the last decades. […] There are consistent reports of an increasing incidence of IH-CCA and a decreasing or stable incidence of EH-CCA from the World Health Organization (WHO) database.

• #56 Why do people with primary sclerosing cholangitis require so much cancer screening? | AASLD

  https://www.aasld.org/liver-fellow-network/core-series/why-series/why-do-people-primary-sclerosing-cholangitis-require-so

  The American Association for the Study of Liver Disease (AASLD) currently recommends annual screening for CCA in adults with large-duct PSC. […] Given the high percentage of patients with PSC having IBD, a diagnostic colonoscopy is recommended at time of PSC diagnosis to establish whether concomitant IBD is present. […] Once a diagnosis of PSC is made, a diagnostic colonoscopy should be performed to evaluate for concomitant IBD (PSC-IBD).

• #57 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies

  https://www.mdpi.com/2072-6694/15/20/4947

  However, the majority of CCA develops asymptomatically, representing an incidental event in up to 40% of all PSC-CCA, also discovered in liver explant pathology or at autopsy. […] PSC-CCA usually occurs at younger ages, around the fourth decade, while de novo CCA rarely develops before the seventh decade. […] In PSC patients, the radiological surveillance has a crucial role to detect early CCA and to allow curative treatments, such as liver resection or transplantation in patients who are usually young and fit for surgery. An ideal surveillance strategy should be simple, cheap, accepted by the patient, non-invasive, and with high sensitivity, aiming to increase overall survival. […] The positive impact of radiological surveillance for PSC patients has already been proven by different studies, reporting better outcomes with a reduction in hepatobiliary cancers-related deaths in patients exposed to at least a routinary imaging technique.

• #58 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies

  https://www.mdpi.com/2072-6694/15/20/4947

  Also, the large multi-center study from the IPSCSG proved that scheduled imaging was associated with improved overall survival. […] The guidelines suggested yearly MRI/MRCP for CCA surveillance in large-duct PSC patients. […] The majority of PSC international guidelines discouraged the 6-month use of CA19-9 for CCA surveillance because it has a low positive predictive value and a low accuracy.

• #59 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies

  https://www.mdpi.com/2072-6694/15/20/4947

  However, the majority of CCA develops asymptomatically, representing an incidental event in up to 40% of all PSC-CCA, also discovered in liver explant pathology or at autopsy. […] PSC-CCA usually occurs at younger ages, around the fourth decade, while de novo CCA rarely develops before the seventh decade. […] In PSC patients, the radiological surveillance has a crucial role to detect early CCA and to allow curative treatments, such as liver resection or transplantation in patients who are usually young and fit for surgery. An ideal surveillance strategy should be simple, cheap, accepted by the patient, non-invasive, and with high sensitivity, aiming to increase overall survival. […] The positive impact of radiological surveillance for PSC patients has already been proven by different studies, reporting better outcomes with a reduction in hepatobiliary cancers-related deaths in patients exposed to at least a routinary imaging technique.

• #60 Treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis: a comprehensive review | eGastroenterology

  https://egastroenterology.bmj.com/content/2/1/e100045

  The potential survival benefit of early CCA detection has called for surveillance in patients with PSC, and retrospective studies from tertiary centres have reported a survival benefit and/or risk reduction of hepatobiliary cancer-related death, in patients exposed to regular surveillance. […] However, in a recent prospective surveillance study, yearly MRI/MRCP was found ineffective in diagnosing CCA early enough to benefit survival in an unselected cohort of patients with PSC. […] Instead, surveillance with MRI/MRCP may be recommended in patients at time of PSC diagnosis, in the event of new symptoms and more regularly in patients with advanced disease.

• #61 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies

  https://www.mdpi.com/2072-6694/15/20/4947

  Also, the large multi-center study from the IPSCSG proved that scheduled imaging was associated with improved overall survival. […] The guidelines suggested yearly MRI/MRCP for CCA surveillance in large-duct PSC patients. […] The majority of PSC international guidelines discouraged the 6-month use of CA19-9 for CCA surveillance because it has a low positive predictive value and a low accuracy.

• #62 Cholangiocarcinoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/cholangiocarcinoma?lang=us

  Regarding inflammatory bowel disease, cholangiocarcinoma develops in one in every 100 to 200 patients, which means they have a 4 times greater risk than IBD-free patients. Individuals with ulcerative colitis are at higher risk of having cholangiocarcinoma in comparison to those with Crohn disease. […] The most important factor in prognosis is whether or not the tumor can be resected. Unfortunately, when discovered, most cases are too advanced for curative resection. Even with resection, the prognosis is poor, with a 5-year survival of only 10-44%, with the prognosis favoring extrahepatic tumors (around 30% 5-year survival vs 15% for intrahepatic tumors). […] All these modalities not only allow evaluation of the biliary tree but are invaluable in planning treatment and assessing for resectability.

• #63 Cholangiocarcinoma 2020: the next horizon in mechanisms and management | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/s41575-020-0310-z

  Cholangiocarcinoma (CCA) includes a cluster of highly heterogeneous biliary malignant tumours that can arise at any point of the biliary tree. Their incidence is increasing globally, currently accounting for ~15% of all primary liver cancers and ~3% of gastrointestinal malignancies. The silent presentation of these tumours combined with their highly aggressive nature and refractoriness to chemotherapy contribute to their alarming mortality, representing ~2% of all cancer-related deaths worldwide yearly. The current diagnosis of CCA by non-invasive approaches is not accurate enough, and histological confirmation is necessary. Furthermore, the high heterogeneity of CCAs at the genomic, epigenetic and molecular levels severely compromises the efficacy of the available therapies. […] The global mortality for CCA increased worldwide during the periods 2000-2004, 2005-2009 and 2010-2014, according to the WHO and Pan American Health Organization databases for 32 selected locations in Europe, America, Asia and Oceania. Furthermore, CCA mortality was higher in men than in women worldwide, and in countries/regions in Asia versus those in the West.

• #64 Finding a rare bile duct cancer’s weaknesses | Fred Hutchinson Cancer Center

  https://www.fredhutch.org/en/news/center-news/2024/07/boila-kugel-dod-ccf-icc.html

  Cholangiocarcinoma, or CCA, is cancer of the bile ducts. ICC is intra-hepatic or intra-liver CCA. It arises in the bile ducts within the liver and makes up about 10-20% of all cases of CCA. While the overall incidence of CCA is quite rare no more than six people per 100,000 in most countries the disease comes with a heavy cost. Globally, about 2% of cancer-related deaths every year can be attributed to CCA, which predominantly occurs in older people. Vietnam veterans, who have a median age of 71 years and are the U.S. largest veteran cohort, have an abnormally high incidence of CCA. […] If ICC is diagnosed early, the prognosis is much better, Boila said. But that almost never happens. […] Because ICC often produces no symptoms, it is usually diagnosed at a late stage when surgery is no longer an option. According to the American Cancer Society, only 9% as many patients with intra-liver bile duct cancer will survive five years, compared to the percentage of people without the same disease who will survive five years. This relative survival rate is 23% for patients whose ICC is diagnosed early, but can be as low as 3% if the tumor is diagnosed after it has spread through the body.

• #65

  https://winshipcancer.emory.edu/cancer-types-and-treatments/bile-duct-cancer/symptoms.php

  Bile duct cancer is rare and there are no screening tests to detect the disease. […] Cholangiocarcinoma (bile duct cancer) is relatively rare, affecting about 10,000 people in the U.S. each year. Given the location of the bile ducts deep within the abdomen, there are currently no bile duct cancer screening tests available to check for the disease before symptoms appear. […] Patients with a diagnosis of primary sclerosing cholangitis should remain in surveillance with their hepatologist to screen for the development of bile duct cancer.

• #66 Cholangiocarcinoma 2020: the next horizon in mechanisms and management | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/s41575-020-0310-z

  The age-standardized incidence of CCA shows considerable geographical variation, with the highest incidence in Eastern countries/regions; incidence varies from 85 per 100,000 in northeastern Thailand (the highest reported value globally) to 0.4 per 100,000 in Canada. […] Over the past few decades, the reported age-standardized incidence for iCCA has been steadily increasing in most locations worldwide, whereas the age-standardized incidence for eCCA has been decreasing. […] The three subtypes of CCA can have different risk factors, pathobiology, clinical presentations, management and prognosis, as well as distinct epidemiological trends. […] Therefore, a detailed study of these types of cancers is urgently needed to improve patient welfare and outcomes. Considering the high heterogeneity of CCAs, individual characterization of these tumours at the genomic, epigenetic and molecular levels is an indispensable approach to ascertain their pathogenesis, paving the path for new therapeutic options and personalized medicine.

• #67 Cholangiocarcinoma 2020: the next horizon in mechanisms and management | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/s41575-020-0310-z

  The age-standardized incidence of CCA shows considerable geographical variation, with the highest incidence in Eastern countries/regions; incidence varies from 85 per 100,000 in northeastern Thailand (the highest reported value globally) to 0.4 per 100,000 in Canada. […] Over the past few decades, the reported age-standardized incidence for iCCA has been steadily increasing in most locations worldwide, whereas the age-standardized incidence for eCCA has been decreasing. […] The three subtypes of CCA can have different risk factors, pathobiology, clinical presentations, management and prognosis, as well as distinct epidemiological trends. […] Therefore, a detailed study of these types of cancers is urgently needed to improve patient welfare and outcomes. Considering the high heterogeneity of CCAs, individual characterization of these tumours at the genomic, epigenetic and molecular levels is an indispensable approach to ascertain their pathogenesis, paving the path for new therapeutic options and personalized medicine.

• #68 Cholangiocarcinoma: a guide for the nonspecialist | IJGM

  https://www.dovepress.com/cholangiocarcinoma-a-guide-for-the-nonspecialist-peer-reviewed-fulltext-article-IJGM

  Chronic host inflammatory responses may persist even after the eradication of the liver flukes through the eggs they produce, which can remain entrapped in the periductal tissue causing granulomatous inflammation. […] The mortality from CCA remains equal to the disease incidence. New biomarkers for screening, diagnosis, staging, and prognosis would be crucial in the management of CCA, as current methods are ineffective in detecting small tumors. […] CCA incidence in Thailand appears to be increasing, not decreasing, and even if the worm is eradicated, it will take 20-30 years to achieve CCA-free population. Hence, those with history of parasitosis are still at risk of developing malignant biliary tumors.

• #69 Biliary tract cancers: epidemiology, molecular pathogenesis and genetic risk associations – Marcano-Bonilla – Chinese Clinical Oncology

  https://cco.amegroups.org/article/view/12259/html

  Infectious diseases, including parasites, bacteria and viruses, cause inflammation of the biliary tract, and a number of these diseases have been linked to the development of biliary tract malignancies. […] Chronic infection with HBV and HCV also contribute to the unique geographical distribution of BTC. […] The molecular pathogenesis of BTC is poorly understood. […] The strongest risk factors for CCA are related to chronic inflammation, which is characterized by up-regulation of the inflammation-related genes, cyclooxygenase (COX-2), also known as prostaglandin-endoperoxide synthase 2 (PTGS-2), and the cytokine interleukin-6 (IL-6). […] The epidemiology of BTC demonstrates substantial geographical variability due to large differences in regional prevalence of the main environmental risk factors. […] Therefore, there is an unmet need for better identification strategies for those patients at increased risk for BTC, with the aims of early detection or prevention.

• #70 Targeting Bile Duct Cancer | American Association for Cancer Research

  https://www.aacr.org/patients-caregivers/progress-against-cancer/targeting-bile-duct-cancer/

  The U.S. Food and Drug Administration (FDA) has approved a new molecularly targeted therapeutic called pemigatinib (Pemazyre) for treating certain patients with bile duct cancer, also known as cholangiocarcinoma. […] Cholangiocarcinoma is a type of cancer that arises in cells that form the bile ducts, which are small tubes that connect the liver and gallbladder to the small intestine. […] Despite the challenges, researchers have learned that the incidence of cholangiocarcinoma has been rising in the United States in recent years. […] Most patients have advanced cholangiocarcinoma at the time they are first diagnosed, and the prognosis for these patients is poor. […] Numerous genomic studies, including one published in the AACR journal Clinical Cancer Research, has led researchers to estimate that from 10 percent to 20 percent of patients with intrahepatic cholangiocarcinoma have an alteration in the FGFR2 gene. […] Pemigatinib is a new therapeutic that targets FGFR2. Its approval by the FDA was based on results from the phase II FIGHT-202 clinical trial, which were published in the Lancet Oncology. […] The FDA approval was rendered on April 17, 2020.

• #71

  https://cosmic-blog.sanger.ac.uk/bile-duct-cancer-cholangiocarcinoma-focus/

  The genomic landscape of BDC differs depending on disease location. […] In iBDC, genetic alterations in KRAS, TP53 or MAPK/mTOR are significantly associated with a worse prognosis while FGFR alterations correlate with a relatively indolent disease course. […] Genomic studies of BDC have shown that they have many potential actionable targets. […] In cases with FGFR2 fusions, promising therapeutic activity has been demonstrated with tyrosine kinase inhibitors such as BGJ398 that inhibit the FGFR2 pathway but such success is tempered by follow-up reports of resistance.

• #72 Finding a rare bile duct cancer’s weaknesses | Fred Hutchinson Cancer Center

  https://www.fredhutch.org/en/news/center-news/2024/07/boila-kugel-dod-ccf-icc.html

  Only about half of patients diagnosed with ICC will live a year past treatment. More effective and targeted treatments are desperately needed, Boila said. […] Liver cancer incidence, including ICC, is rising, but ICCs rarity makes it hard to study. With so few patients, its difficult to gather enough tissue samples to make confident inferences about whats driving the cancer and where it may be vulnerable. […] Its true of most rare cancers: small cohorts make it difficult to get statistically significant observations from patient data. Its why we depend more on experimental models, Boila said. […] About 20% of human ICC cases have a mutation in the ARID1A gene. ARID1A is part of a protein complex that restructures DNA packaging to influence expression of certain genes. Its loss may shift which genes are turned on or off in a way that promotes tumor formation. Boilas DOD funding will allow him to begin untangling this mechanism.

• #73 Frontiers | Global, regional, and national burden and trends analysis of gallbladder and biliary tract cancer from 1990 to 2019 and predictions to 2030: a systematic analysis for the Global Burden of Disease Study 2019

  https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2024.1384314/full

  The highest age-standardized incidence rate was observed in Southern Latin America, where the most frequently diagnosed anatomic subtype was gallbladder cancer, which is likely due to the high prevalence of cholelithiasis. […] Previous research has revealed that having gallstones is the primary risk factor for gallbladder and biliary tract cancers; the incidence rates of gallbladder and biliary tract cancers was strongly associated with the global prevalence of gallstone. […] The highest age-standardized incidence rate was observed in Southern Latin America (Chile, Bolivia, Uruguay, Argentina, and Peru), where the most frequently diagnosed anatomic subtype was gallbladder cancer, which is likely due to the high prevalence of cholelithiasis. […] The increasing burden of early-onset gallbladder and biliary tract cancer is a cause for concern, especially in lower SDI areas and among males. […] Our study provides comprehensive insights into the distribution and dynamic trends of gallbladder and biliary tract burden over the past three decades, from multiple perspectives.

• #74 Epidemiology of cholangiocarcinoma

  https://www.oaepublish.com/articles/2394-5079.2021.130

  Aim: We aimed to analyze temporal trends in mortality from intrahepatic (ICC) and extrahepatic (ECC) cholangiocarcinoma in selected countries worldwide. […] In both sexes, ICC mortality rates increased in most countries considered, including the USA, the UK, and Australia; in some countries, including Italy and France, the increasing trends leveled off over the most recent years. […] ICC mortality increased in most areas of the world, likely due to increased prevalence of risk factors and improved cancer recognition and classification. […] Cholangiocarcinoma is a heterogeneous group of aggressive neoplasms of the biliary duct system, accounting for approximately 3%-5% of all gastrointestinal cancers. […] The incidence of cholangiocarcinoma is relatively low in most high-income countries (0.3-2 cases per 100,000 people) but much higher (even 40-fold greater) in some regions of Thailand and China, where infection with liver flukes – a key determinant of cholangiocarcinoma – is endemic.

• #75 Epidemiology of cholangiocarcinoma

  https://www.oaepublish.com/articles/2394-5079.2021.130

  Although both ICC and ECC are associated with selected biliary tract conditions, hepatitis B and C, and NAFLD, these associations are quantitatively different, which suggests that the two cancers have distinct epidemiology and biology. […] Various studies indicated that the incidence of ICC has increased over the last decades in most countries. […] As for mortality, a study based on official death certification data showed a global increase in ICC mortality and a decrease or stabilization in ECC mortality over the 1995-2016 period, with wide variations in rates across geographic regions. […] The present global analysis, based on countries with acceptably reliable data, showed rising trends of ICC mortality for both sexes in most of the countries considered, with some decelerations over the most recent years in selected countries; in the USA, the UK, and Australia, ICC mortality trends steadily increased over the whole period.

• #76 Primary Sclerosing Cholangitis-Associated Cholangiocarcinoma: From Pathogenesis to Diagnostic and Surveillance Strategies

  https://www.mdpi.com/2072-6694/15/20/4947

  The frequency of CCA in patients with PSC is highly variable, as past studies reported a wide range between 4% and 36%. The variability is mainly due to the retrospective and monocentric design of the studies, including a limited number of patients, and to different patient populations with heterogeneous tumor characteristics. […] More recent studies (on larger and multicentric populations) reported a higher frequency of CCA in PSC, registering a range from 2% to 8%. […] In transplant centers, the frequency of PSC-CCA seems to be higher (up to 19.9%), probably associated with a higher prevalence of patients with advanced PSC. […] The clinical presentation of PSC-CCA is difficult to recognize also because the clinical and radiological features are overlapping in the two diseases, and CCA could appear as a precipitating event in misdiagnosed PSC.

• #77 Cholangiocarcinoma 2020: the next horizon in mechanisms and management | Nature Reviews Gastroenterology & Hepatology

  https://www.nature.com/articles/s41575-020-0310-z

  The age-standardized incidence of CCA shows considerable geographical variation, with the highest incidence in Eastern countries/regions; incidence varies from 85 per 100,000 in northeastern Thailand (the highest reported value globally) to 0.4 per 100,000 in Canada. […] Over the past few decades, the reported age-standardized incidence for iCCA has been steadily increasing in most locations worldwide, whereas the age-standardized incidence for eCCA has been decreasing. […] The three subtypes of CCA can have different risk factors, pathobiology, clinical presentations, management and prognosis, as well as distinct epidemiological trends. […] Therefore, a detailed study of these types of cancers is urgently needed to improve patient welfare and outcomes. Considering the high heterogeneity of CCAs, individual characterization of these tumours at the genomic, epigenetic and molecular levels is an indispensable approach to ascertain their pathogenesis, paving the path for new therapeutic options and personalized medicine.

• #78 Cholangiocarcinoma: a guide for the nonspecialist | IJGM

  https://www.dovepress.com/cholangiocarcinoma-a-guide-for-the-nonspecialist-peer-reviewed-fulltext-article-IJGM

  Chronic host inflammatory responses may persist even after the eradication of the liver flukes through the eggs they produce, which can remain entrapped in the periductal tissue causing granulomatous inflammation. […] The mortality from CCA remains equal to the disease incidence. New biomarkers for screening, diagnosis, staging, and prognosis would be crucial in the management of CCA, as current methods are ineffective in detecting small tumors. […] CCA incidence in Thailand appears to be increasing, not decreasing, and even if the worm is eradicated, it will take 20-30 years to achieve CCA-free population. Hence, those with history of parasitosis are still at risk of developing malignant biliary tumors.

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