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. In the past decade, increasing efforts have been made to understand the complexity of these tumours and to develop new diagnostic tools and therapies that might help to improve patient outcomes.

• #1 Cholangiocarcinoma: Practice Essentials, Pathophysiology, Epidemiology

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

  Cholangiocarcinoma (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. CCCs are encountered in three anatomic regions: intrahepatic, extrahepatic (ie, perihilar), and distal extrahepatic. […] 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. […] 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.

• #1 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.

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

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

  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. Accordingly, Asian individuals were reported to have the highest mortality (2.81 per 100,000 men in Japan). However, in the USA, the more noticeable increases in mortality between 2004 and 2014 were found for African American individuals (45%), followed by Asian (22%) and white (20%) individuals. 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. Variations in incidence probably reflect differences in local risk factors and potential genetic predispositions.

• #1 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.

• #1 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, from US cancer registries, and also from Japanese and different European cohorts.

• #1 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. […] The Khon Kaen University in northeast Thailand, a region with very high incidences of liver flukes, has started the Cholangiocarcinoma Screening and Care Program (CASCAP) in collaboration with the National Health Security Office (NHSO) and the Ministry of Public Health. The aim of this study is to systematically improve the diagnosis and treatment of CCA patients throughout the northeast of Thailand.

• #1 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.

• #1 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.

• #1 Epidemiologic patterns of biliary tract cancer in the United States: 2001–2015 | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-022-10286-z

  Biliary tract cancer (BTC) includes intrahepatic cholangiocarcinoma (ICC), extrahepatic cholangiocarcinoma, gallbladder cancer, and ampulla of Vater cancer (AVC). Although BTC is rare in the US, incidence is increasing and elevated in certain populations. This study examined BTC epidemiology in the US by age, sex, race/ethnicity, geographic region, and anatomic site. […] BTC incidence increased during the study period (annual percent change=1.76, 95% confidence interval [1.591.92]), with the highest increase in ICC (6.65 [6.117.19]). […] Hispanic, Asian/Pacific Islander, Black, or American Indian/Alaska Native race/ethnicity was associated with higher BTC mortality rates than White race/ethnicity. […] Five-year survival was 15.2% for all BTC, ranging from 8.5% (ICC) to 34.5% (AVC), and patients with distant disease at diagnosis had lower survival (3%) compared with those with regional (19.1%) or locally advanced disease (31.5%).

• #1 Changing incidence and survival of intrahepatic cholangiocarcinoma based on Surveillance, Epidemiology, and End Results Database (2000-2017)

  https://www.ahbps.org/journal/view.html?volume=26&number=3&spage=235

  Changing incidence and survival of intrahepatic cholangiocarcinoma based on Surveillance, Epidemiology, and End Results Database (2000-2017) […] Historically, the incidence and prognosis of patients diagnosed with intrahepatic cholangiocarcinoma (ICC) have been inadequately understood. […] ICC incidence has been increasing since 2000, especially in White males. The risk of ICC rises with age. […] The overall observed survival at 12, 36, and 60 months was 36.3%, 12.8%, and 8.1%, respectively. […] The incidence of disease was categorized into three groups based on the year of diagnosis: 2000-2006, 2007-2011, and above 2012. […] The ICC age-adjusted incidence rates gradually increased between 2000-2006, 2007-2011, and 2012 onwards (0.6, 0.8 vs. 1.1 per 100,000, respectively). […] The APC for the ICC incidence rate increased from 2000 to 2017 with a cumulative rate of 5.7 (95% CI, 4.3-7.2; p < 0.05).

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

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

  Intrahepatic cholangiocarcinoma (ICC) is a rare entity with a distinct clinical course and epidemiology from hilar and extrahepatic cholangiocarcinoma. ICC makes up 810% of cholangiocarcinomas and 1020% of all primary liver tumors. There remains a considerable amount of geographic variation in the incidence of ICC worldwide; however, the overall incidence of this malignancy appears to be rising. […] Despite the relative rarity of ICC, the incidence of ICC has been reported to be increasing worldwide. This higher incidence is independent of tumor size or tumor stage, and thus it is unlikely secondary to earlier detection, but in fact a true increase in ICC incidence. […] There is a tremendous discrepancy in the worldwide incidence of ICC, with significantly higher rates of ICC seen in Eastern Asia when compared to Western countries. This geographic disparity is explained largely by the prevalence of risk factors for ICC in these Eastern countries.

• #1 Cholangiocarcinoma: Practice Essentials, Pathophysiology, Epidemiology

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

  Despite aggressive anticancer therapy and interventional supportive care (ie, wall stents or percutaneous biliary drainage), the median survival rate is low, since most patients (90%) are not eligible for curative resection. The overall survival is approximately 6 months. […] 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. The high prevalence of cholangiocarcinoma in people of Asian descent is attributable to endemic chronic parasitic infestation. […] In both males and females, cholangiocarcinoma is most common in persons in their 60s and 70s. The male-to-female ratio for cholangiocarcinoma is 1:2.5 in patients in their 60s and 70s and 1:15 in patients younger than 40 years. 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. The estimated number of new cases of gallbladder and other biliary cancers (extrahepatic cholangiocarcinoma) are 5900 for men and 6450 for women, with estimated deaths of 1950 and 2580, respectively.

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

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

  McLean et al. demonstrated a significantly higher incidence of ICC of Hispanic-Americans compared to other ethic groups, with African-Americans having the lowest incidence. The authors concluded that this variation may reflect potential genetic, cultural, or socioeconomic differences in ICC susceptibility. […] 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.

• #1 Epidemiology and genomic features of biliary tract cancer and its unique features in Korea

  https://www.e-jlc.org/journal/view.php?number=589

  Biliary tract cancer (BTC) is a rare but highly aggressive malignancy that includes intrahepatic cholangiocarcinoma (ICC), extrahepatic cholangiocarcinoma, and gallbladder cancer (GBC). While BTC has a low global incidence, its regional variations are notable. Among nations, Korea has the second-highest incidence of BTC globally, with the highest mortality rate worldwide, underscoring the need for a deeper understanding of this cancer. Liver fluke infection and hepatitis B virus infection are key risk factors unique to Korea, contributing to regional differences in BTC incidence. […] Korea has the second-highest incidence of BTC globally and the highest mortality rate worldwide, emphasizing the need for a deeper understanding of this disease. […] Globally, BTC mortality rates remain relatively low, but several European countries, including Italy (4.2 deaths per 100,000 person-years), Germany (3.8), France (3.1), and the United Kingdom (3.2), report particularly low mortality rates. In contrast, in East Asia, Japan has significantly higher rates, with a reported mortality rate of 7.2, while Korea has the highest mortality rate in the world, at 11.0.

• #1 Liver and Intrahepatic Bile Duct Cancer — Cancer Stat Facts

  https://seer.cancer.gov/statfacts/html/livibd.html

  The rate of new cases of liver and intrahepatic bile duct cancer was 9.4 per 100,000 men and women per year based on 20182022 cases, age-adjusted. […] Liver and intrahepatic bile duct cancer is most frequently diagnosed among people aged 6574. […] Liver and intrahepatic bile duct cancer is the sixth leading cause of cancer death in the United States. The death rate was 6.6 per 100,000 men and women per year based on 20192023 deaths, age-adjusted. […] The percent of liver and intrahepatic bile duct cancer deaths is highest among people aged 6574. […] Keeping track of new cases, deaths, and survival over time (trends) can help scientists understand whether progress is being made and where additional research is needed to address challenges, such as improving screening or finding better treatments. […] Using statistical models for analysis, age-adjusted rates for new liver and intrahepatic bile duct cancer cases have been falling on average 0.7% each year over 20132022. Age-adjusted death rates have been stable over 20142023.

• #1 Liver and Intrahepatic Bile Duct Cancer — Cancer Stat Facts

  https://seer.cancer.gov/statfacts/html/livibd.html

  Estimated New Cases in 2025 42,240 […] Estimated Deaths in 2025 30,090 […] % of All New Cancer Cases 2.1% […] % of All Cancer Deaths 4.9% […] The rate of new cases of liver and intrahepatic bile duct cancer was 9.4 per 100,000 men and women per year. The death rate was 6.6 per 100,000 men and women per year. […] Approximately 1.1 percent of men and women will be diagnosed with liver and intrahepatic bile duct cancer at some point during their lifetime, based on 20182021 data, excluding 2020 due to COVID. […] In 2022, there were an estimated 113,557 people living with liver and intrahepatic bile duct cancer in the United States. […] Liver and intrahepatic bile duct cancer represents 2.1% of all new cancer cases in the U.S. […] In 2025, it is estimated that there will be 42,240 new cases of liver and intrahepatic bile duct cancer and an estimated 30,090 people will die of this disease.

• #1 Survival for bile duct cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/bile-duct-cancer/survival

  There are no UK wide statistics available for bile duct cancer survival by stage. […] The statistics below come from America. They come from the National Cancer Institute’s SEER programme. They are for people diagnosed with bile duct cancer between 2012 and 2018. […] The statistics are also split into 2 types of bile duct cancer: intrahepatic bile duct cancer that starts in the liver and extrahepatic bile duct cancer that starts outside the liver (perihilar and distal bile duct cancer). […] Almost 30 out of 100 people (almost 30%) survived their cancer for 1 year or more after they were diagnosed. […] 5 out of 100 people (5%) survived their cancer for 5 years or more after they were diagnosed. […] Your outlook depends on the stage of the cancer when it was diagnosed. This means how big it is and whether it has spread. […] It also depends on which bile ducts the cancer is in and whether you can have surgery to completely remove it. […] Your general health and fitness also affect survival.

• #1 Epidemiologic patterns of biliary tract cancer in the United States: 2001–2015 | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-022-10286-z

  The overall mortality ASR for BTC patients was 3.40 (95% CI, 3.383.42). […] Compared with White individuals, all other race/ethnicity groups had a higher mortality rate for BTC with the highest rates among Hispanic and Asian/Pacific Islander individuals. […] Among the anatomic sites, the mortality rate was highest for ICC, particularly for Asian/Pacific Islander individuals. […] Five-year survival for BTC was 15.2% (95% CI, 14.815.7). […] The lowest survival was reported for ICC (8.5% [95% CI, 7.89.2]) and the highest for AVC (34.5% [95% CI, 33.036.0]). […] Five-year survival was low for patients with distant disease at first diagnosis (3.0% [95% CI, 2.63.4]), compared with regional (19.1% [95% CI, 18.320.0]) or localized (31.5% [95% CI, 30.332.7]) disease. […] From 2001 to 2015 in the US, BTC incidence increased, with the greatest increase occurring in ICC.

• #1 Prognosis for bile duct cancer: What to know

  https://www.medicalnewstoday.com/articles/bile-duct-cancer-prognosis-2

  A relative survival rate compares individuals with the same type and stage of cancer with people in the general population. […] The 5-year relative survival rates for intrahepatic bile duct cancer are as follows: localized: 24%, regional: 9%, distant: 2%, all stages combined: 9%. […] The 5-year relative survival rates for extrahepatic bile duct cancer are as follows: localized: 17%, regional: 16%, distant: 2%, all stages combined: 10%. […] It is important to note that these figures do not take all factors into account. […] Bile duct cancer is rare and aggressive. Although the disease typically has an unfavorable outlook, an individuals outlook depends on various factors, such as age, overall health, and their bodys response to treatment. […] The 5-year relative survival rates range between 224% for bile duct cancers that start within the liver, and 217% for bile duct cancers that start outside the liver.

• #1 Changing incidence and survival of intrahepatic cholangiocarcinoma based on Surveillance, Epidemiology, and End Results Database (2000-2017)

  https://www.ahbps.org/journal/view.html?volume=26&number=3&spage=235

  The five-year survival of ICC was only 8.1%, which may be explained by the increased rate in the older population (75% of cases were above age 60 years). […] Surgery remains the treatment modality with a proven increase in 5-year survival. […] New information regarding ICC incidence and survival based on race, sex, and age will have a positive impact on resource planning and management of this disease.

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

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

  Prognosis depends in part on the tumors anatomical location, which affects resectability. Because of proximity to major blood vessels and diffuse extension within the liver, a bile duct tumor can be difficult to resect. Total resection is possible in 25% to 30% of lesions that originate in the distal bile duct; the resectability rate is lower for lesions that occur in more proximal sites. […] Complete resection with negative surgical margins offers the only chance of cure for bile duct cancer. For localized, resectable extrahepatic and intrahepatic tumors, the presence of involved lymph nodes and perineural invasion are significant adverse prognostic factors. […] Bile duct cancer is classified as resectable (localized) or unresectable, with obvious prognostic importance. The TNM (tumor, node, metastasis) staging system is used for staging bile duct cancer, commonly after surgery and pathological examination of the resected specimen. Evaluation of the extent of disease at laparotomy is an important component of staging.

• #1 Outcomes (prognosis) for bile duct cancer treatment – Liver Cancer UK

  https://livercanceruk.org/liver-cancer-information/types-of-liver-cancer/bile-duct-cancer/treating-bile-duct-cancer/outcomes-prognosis-after-bile-buct-cancer-treatment/

  Bile duct cancer is a rare type of cancer. […] The most important thing is whether you can have an operation to completely remove the cancer and try to cure it. With this type of treatment, around 4 out of 10 people (40%) live for at least 5 years afterwards. […] Because staging is very complicated, and varies between types of bile duct cancer, it’s a good idea to check with your hospital doctor or specialist nurse about which group you’re in. […] Overall statistics for bile duct cancer indicate that in men, more than half (57%) lived for at least one year after their diagnosis, while in women, around half (49%) lived for at least one year after their diagnosis.

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

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

  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. […] 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.

• #1 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

  Primary sclerosing cholangitis (PSC) is a heterogenous and progressive disease of the biliary tree, resulting in fibrosis and the eventual development of cirrhosis. […] The combination of inflammation and cholestasis is also highly carcinogenic, leading to biliary cancer in these patients. […] In particular, patients with PSC are at increased risk for hepatobiliary (cholangiocarcinoma, gallbladder cancer, hepatocellular carcinoma) and colorectal cancers. […] Cholangiocarcinoma (CCA) is an uncommon cancer of the bile ducts with a very poor prognosis. […] While relatively rare in the general population, people with PSC are up to 400 times more likely to develop CCA. […] Due to the differential survival in early-stage disease and high incidence in PSC patients, screening for CCA is routinely performed.

• #1 Cholangiocarcinoma (Bile duct cancer) | Doctor

  https://patient.info/doctor/cholangiocarcinoma

  Chronic inflammation of the biliary epithelium is thought to be the critical shared feature of risk factors. Patients with chronic ulcerative colitis who develop primary sclerosing cholangitis are prone to cholangiocarcinoma. The lifetime risk of developing this cancer is 10-20% with primary sclerosing cholangitis. Some patients with Crohn’s disease may also be at risk. […] Diagnosis should be made on the basis of radiological investigations (including CT or MRI) and pathological assessment from a biopsy, fine-needle aspiration or biliary brush cytology. There are no blood tests that are diagnostic for cholangiocarcinoma, but testing is usually performed as part of the diagnostic process to look for other conditions, assess liver synthetic function, and aid the diagnosis. […] Staging is based on the tumour, node and metastasis (TNM) classification with some modifications for intrahepatic cholangiocarcinoma, perihilar cholangiocarcinoma and distal cholangiocarcinoma. The initial diagnostic tests may provide staging information. Additionally, a contrast CT of the abdomen, chest and pelvis should be performed to look for metastatic disease.

• #1 Risks and causes of bile duct cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/bile-duct-cancer/risks-causes

  Bile duct cancer is also called cholangiocarcinoma. It is a rare type of cancer that develops in the small tubes that connect the liver and gallbladder to the small bowel. […] Survival for bile duct cancer depends on many factors including whether the cancer is in the bile ducts in the liver or outside of the liver. […] People with PSC may have an increased risk of developing cancer of the bile ducts. One study based in the Netherlands found that more than 5 in 100 people with PSC (more than 5%) went on to develop bile duct cancer. […] Liver cirrhosis can increase the risk of bile duct cancer. Especially intrahepatic bile duct cancer. […] Some IBDs like ulcerative colitis, can increase the risk of bile duct cancer. […] People who have had Thorotrast in the past have a higher chance of developing bile duct cancer.

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

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

  Overall, CCA incidence is higher among men than women. […] 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.

• #1 Biliary cancer – Knowledge @ AMBOSS

  https://www.amboss.com/us/knowledge/biliary-cancer/

  Cholangiocarcinoma (CCA) is a malignancy of the bile duct. […] Incidence: 1/100,000 per year in the US. […] Peak incidence: 60-70 years of age. […] Extrahepatic CCA: 90% of CCA cases. […] Intrahepatic CCA: 10% of CCA cases; the second most common intrahepatic malignancy after hepatocellular carcinoma (HCC). […] Suspected CCA lesions are often found during imaging surveillance for PSC or cirrhosis. […] Surgical resection with adjuvant chemotherapy is the only curative treatment for CCA. […] 5-year survival rates following curative resection: 16-44% for intrahepatic bile duct tumors; 20-30% for extrahepatic bile duct tumors. […] Individuals with biliary cysts have an increased risk of CCA; therefore, all patients should undergo surgery, even if asymptomatic.

• #1 Cholangiocarcinoma – Diagnosis & Disease Information

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

  Because it is often asymptomatic in early stages and resistant to standard chemotherapies, cholangiocarcinoma is characterized by a late presentation and poor prognosis. Early diagnosis is critical but challenging, which makes advanced imaging techniques and biopsies essential for accurate detection and staging. Treatment typically involves a combination of surgical resection, chemotherapy, and radiation therapy, but the survival rate remains low.

• #1 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. […] The primary aim of screening is the early identification CCA to increase the probability of curative treatment. […] Incidence of CCA is highest in the first year of PSC diagnosis (2.5%), but it can develop any time throughout a patients life. […] By 30 years, the risk of developing CCA is as high as 20%. […] 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-IBD has been established or ruled out, patients are shunted into their respective CRC screening protocols. […] Dysplasia surveillance is performed by careful examination under high-resolution white light endoscopy and random colonic biopsies (typically every 10cm), with or without an alternative viewing modality such as chromoendoscopy or narrow-band imaging.

• #1 Tumors of the Gallbladder and Bile Ducts – Hepatic and Biliary Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/hepatic-and-biliary-disorders/gallbladder-and-bile-duct-disorders/tumors-of-the-gallbladder-and-bile-ducts

  Cholangiocarcinomas and other bile duct tumors are rare (1 to 2/100,000 people) but are usually malignant (1). Cholangiocarcinomas occur predominantly in the extrahepatic bile ducts: 60 to 70% in the perihilar region (Klatskin tumors), about 25% in the distal extrahepatic ducts, and the rest in the liver. Established risk factors include primary sclerosing cholangitis, older age, infestation with liver flukes, and a choledochal cyst. […] Cholangiocarcinomas and gallbladder carcinomas are suspected when extrahepatic biliary obstruction is unexplained. […] For cholangiocarcinoma, stenting or surgically bypassing the obstruction relieves pruritus, jaundice, and perhaps fatigue. […] Biliary tract cancer (usually cholangiocarcinoma or gallbladder carcinoma) is uncommon. […] Suspect cancer if patients have an unexplained extrahepatic biliary obstruction or abdominal mass.

• #1 Gallbladder & Biliary Tract Cancer – GI Cancer

  https://gicancer.org.au/cancer/gallbladder-and-biliary-cancer/

  Biliary tract cancer (also known as cholangiocarcinoma) is cancer that occurs in the bile ducts (tubes that transport bile from the liver). […] Biliary tract cancer is a rare cancer that affects the bile tract. […] In Australia, it is estimated that 1,427 people were diagnosed with either gallbladder or biliary tract cancer in 2024. […] Gallbladder and biliary tract cancer are extremely rare cancers in Australia. […] There is currently no standard or routine screening test available in Australia.

• #1 Epidemiologic patterns of biliary tract cancer in the United States: 2001–2015 | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-022-10286-z

  BTC incidence increased, survival was low across all subtypes, and mortality was greatest in patients with ICC. […] Epidemiological insights may improve patient care and inform the integration of novel therapies for BTC. […] In the US, liver and intrahepatic bile duct cancer are predicted to account for approximately 41,260 new cases in 2022, and GBC and other biliary cancers for approximately 12,130 new diagnoses in 2022. […] In the US, American Indian and Hispanic individuals are at greater risk for BTC, constituting an increased public health burden in these populations. […] Late diagnosis, poor prognosis, and limited treatment options also increase disease burden related to BTC. […] Epidemiologic research for BTC is often limited to one anatomic site (e.g. ICC) or does not differentiate BTC from liver cancer, making it difficult to extract relevant information about this cancer group.

• #1 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

  Overall, AVC is a relatively rare form of cancer, accounting for just 15.1% of all BTC cases in the USA. […] Effective treatment of BTC remains a significant clinical challenge as patients often do not present for diagnosis until the disease is in an advanced stage. […] The rates of adjuvant therapy for BTC patients have also risen over the past 19 years, with several studies demonstrating a link between adjuvant therapy and improved BTC patient survival outcomes. […] The survival rates were low among ECC patients as well as ICC patients, potentially due to the fact that these two forms of BTC are relatively difficult to resect. […] In summary, the results of this analysis of SEER registry data over a 19-year period revealed that both ICC incidence and mortality rates have risen with time, while the incidence and mortality of ECC, GBC, and AVC have remained relatively stable.

• #1 Encouraging Treatment Advances for Aggressive Cancers of the Biliary Tract  – American Association for Cancer Research (AACR)

  https://www.aacr.org/blog/2023/02/06/encouraging-treatment-advances-for-aggressive-cancers-of-the-biliary-tract/

  According to a recent publication in the AACR journal Cancer Epidemiology, Biomarkers Prevention, bile duct cancers within the liver constituted the majority of biliary tract cancers between 2013 and 2017 (with an incidence rate of 1.49 per 100,000 individuals), followed by gallbladder cancers (1.11 per 100,000), bile duct cancers outside the liver (0.96 per 100,000), ampullary cancers (0.45 per 100,000), and cancers in other regions of the biliary tract (0.24 per 100,000). […] The incidence and mortality rates of biliary tract cancers increased with age, and incidence was highest among men and among individuals who identified as Hispanic or American Indian/Alaska Native. Asian and Hispanic patients had the highest rates of mortality compared with other racial/ethnic groups. […] Understanding the lifestyle factors that contribute to the onset of biliary tract cancers may ultimately help lower risk, identify patients who might benefit from increased monitoring, and/or lessen disparities between different demographic groups.

• #1 Encouraging Treatment Advances for Aggressive Cancers of the Biliary Tract  – American Association for Cancer Research (AACR)

  https://www.aacr.org/blog/2023/02/06/encouraging-treatment-advances-for-aggressive-cancers-of-the-biliary-tract/

  Additional risk factors for biliary tract cancers include gallstones, gallbladder polyps, primary sclerosing cholangitis, chronic ulcerative colitis, cysts in the bile ducts, and certain parasitic or bacterial infections. […] The high mortality rates associated with biliary tract cancers can be daunting for the patients and families faced with a diagnosis, but research continues to move the dial forward, with the ultimate goal of improving patient outcomes.

• #1 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 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.

• #1 Gallbladder and Bile Duct Cancer Awareness Month | The AACR

  https://www.aacr.org/patients-caregivers/awareness-months/gallbladder-cancer-and-bile-duct-cancer-awareness-month/

  Gallbladder cancer and bile duct cancer are relatively rare forms of cancer with an estimated 12,350 cases of both types combined diagnosed in 2024, according to the National Cancer Institute (NCI). Further, the NCI estimated that about 4,530 people would die from these cancers in 2024. […] Bile duct cancer, also called cholangiocarcinoma, can either form in the ducts on the outside or inside of the liver. […] Disparities exist in the rates at which these cancers occur among minority populations. For example, Hispanic men and women are more than twice as likely as white men and women to be diagnosed with gallbladder cancer. […] The five-year relative survival rates for gallbladder cancer range from 4% when the cancer is diagnosed at an advanced stage to 68% when diagnosed while the cancer is still localized. For bile duct cancers, the overall five-year relative survival rate is 11% for intrahepatic bile duct cancer and 21% for extrahepatic bile duct cancer.

• #1 Geographic Variation of Intrahepatic Cholangiocarcinoma, Extrahepatic Cholangiocarcinoma, and Hepatocellular Carcinoma in the United States | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0120574

  Demographic patterns and geographical variation were more closely related between ICC and ECC than HCC, suggesting that the etiology of ICC and ECC may be similar. […] Geographic variation of disease is often explained by differences in demographics and environmental risk factors, which suggest that risk factors for HCC may be more dissimilar than those of ICC and ECC. […] While HCC and ICC may have some common etiologies, such as obesity, chronic non-alcoholic liver disease, and tobacco, geographic areas with high ICC incidence do not completely correspond with areas of high HCC incidence. […] This study suggests a need for cancer control efforts focused in the Pacific region, particularly Hawaii and Alaska, for all three cancer sites. While additional control efforts for ICC and ECC should be focused in the Northeast and upper Midwest, HCC control efforts should be additionally focused in the South.

• #1 Cholangiocarcinoma (Bile duct cancer) | Doctor

  https://patient.info/doctor/cholangiocarcinoma

  Complete surgical resection is the only intervention to offer a chance of cure but fewer than 33% are resectable at diagnosis. Intrahepatic and Klatskin tumours require liver resection. Distal cholangiocarcinomas are managed by pancreatoduodenectomy. […] Progressive deterioration with average survival of 12-18 months from diagnosis. The overall survival rates are low because many patients present with unresectable or metastatic disease. Even in patients undergoing aggressive surgery, five-year survival rates are 10-40% for cholangiocarcinoma. Prognosis is much better for those with extrahepatic tumours who are suitable for early surgical intervention. Intrahepatic lesions carry the worst prognosis.

• #1 Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente

  https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.bile-duct-cancer-cholangiocarcinoma-treatment-pdq%C2%AE-treatment-health-professional-information-nci.ncicdr0000062905

  The treatment of bile duct cancer depends primarily on whether the cancer can be completely removed by surgery. […] Localized intrahepatic and extrahepatic bile duct cancer may be completely removed by surgery. […] Most cases of intrahepatic, distal, and perihilar bile duct cancer are unresectable and cannot be completely removed. […] In locally advanced disease, phase II trials have evaluated chemoradiotherapy with the goal of improved local control and potential downstaging for surgical resection. […] For patients with unresectable bile duct cancer, management is directed at palliation.

• #1 About Bile Duct Cancer | University Hospitals

  https://www.uhhospitals.org/services/cancer-services/gastrointestinal-cancer/bile-duct-cancer/about-bile-duct-cancer

  Complete resection with negative surgical margins offers the only chance of cure for bile duct cancer. For localized, resectable extrahepatic and intrahepatic tumors, the presence of involved lymph nodes and perineural invasion are significant adverse prognostic factors. […] Bile duct cancer is classified as resectable (localized) or unresectable, with obvious prognostic importance. The TNM (tumor, node, metastasis) staging system is used for staging bile duct cancer, commonly after surgery and pathological examination of the resected specimen. Evaluation of the extent of disease at laparotomy is an important component of staging. […] Most cases of intrahepatic, distal, and perihilar bile duct cancer are unresectable and cannot be completely removed. Often the cancer invades directly into the portal vein, the adjacent liver, along the common bile duct, and to adjacent lymph nodes. […] Pending further clinical trials, cisplatin plus gemcitabine is considered the reference standard first-line chemotherapy backbone for patients with unresectable, metastatic, or recurrent bile duct cancer.

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

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

  Most cases of intrahepatic, distal, and perihilar bile duct cancer are unresectable and cannot be completely removed. Often the cancer directly invades the portal vein, the adjacent liver, along the common bile duct, and the adjacent lymph nodes. […] Treatment options for bile duct cancer are described in Table 16. […] Pending further clinical trials, cisplatin plus gemcitabine is considered the reference standard first-line chemotherapy backbone for patients with unresectable, metastatic, or recurrent bile duct cancer.

• #1 Gallbladder and Bile Duct Cancer Awareness Month: Improving Screening and Treatment Options With Waqas Haque, MD, MPH, and Matthew Hadfield, DO – Oncology Data Advisor

  https://oncdata.com/gallbladder-and-bile-duct-cancer-awareness-month-improving-screening-and-treatment-options-with-waqas-haque-md-mph-and-matthew-hadfield-do/

  In honor of Gallbladder and Bile Duct Cancer Awareness Month this February, Fellows Forum members Dr. Waqas Haque and Dr. Matthew Hadfield sat down for a discussion on the challenges in diagnosis of gallbladder and bile duct cancers and the importance of genetic sequencing for making targeted treatments available for patients. […] This is a condition that affects 5,000 to 8,000 people annually, and even though it’s rare, it can be very devastating for patients. […] Cholangiocarcinoma is a very tough cancer to treat, and I’ve had multiple patients throughout my fellowship training who presented to the hospital. […] Thankfully, over the last several years, we’ve had some advancements in treatment of cholangiocarcinoma, particularly the advent of using immune checkpoint inhibitors in combination with chemotherapy, which has improved overall survival.

• #1 Gallbladder and Bile Duct Cancer Awareness Month: Improving Screening and Treatment Options With Waqas Haque, MD, MPH, and Matthew Hadfield, DO – Oncology Data Advisor

  https://oncdata.com/gallbladder-and-bile-duct-cancer-awareness-month-improving-screening-and-treatment-options-with-waqas-haque-md-mph-and-matthew-hadfield-do/

  Typically, there seems to be a predilection for females over males with cholangiocarcinoma. There are about 10,000 to 12,000 cases diagnosed each year, and as I mentioned, these patients typically present at later stages of disease, typically beyond the point at which they can have a resection. […] Unfortunately, no, there aren’t currently any guidelines. We don’t have good screening measures as we do for even things like low-dose computed tomography (CT) scans for lung cancer in at-risk populations who are smokers. […] The big thing that I would reiterate is that I think it’s ingrained at large academic medical centers that whole exome sequencing really informs care, and it does make a huge difference in opening up treatment options—not just standard of care for more nuanced and rare mutations that could open up doors for targeted therapy, but also for clinical trials. […] There are not really any universal screening guidelines for detecting gallbladder or biliary cancer, so I really emphasize the importance of having a healthy lifestyle, avoiding smoking, trying to cut down on drinking, having a healthy diet, and exercising.

• #1 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. […] Boilas suite of preclinical models will also aid his efforts to discover potential new therapies for patients with ARID1A-mutated ICC. Currently the only targeted treatments for patients with ICC are aimed at tumors with mutations in IDH or a growth factor gene, FGFR.

• #1 Cholangiocarcinoma | Nature Reviews Disease Primers

  https://www.nature.com/articles/s41572-021-00300-2

  Cholangiocarcinoma (CCA) is a highly lethal adenocarcinoma of the hepatobiliary system, which can be classified as intrahepatic, perihilar and distal. […] In endemic regions, liver fluke infection is associated with CCA, owing to the oncogenic effect of the associated chronic biliary tract inflammation. […] CCA remains a highly lethal disease and further scientific and clinical insights are needed to improve patient outcomes. […] Khan, S. A., Tavolari, S. Brandi, G. Cholangiocarcinoma: epidemiology and risk factors. […] Saha, S. K., Zhu, A. X., Fuchs, C. S. Brooks, G. A. Forty-year trends in cholangiocarcinoma incidence in the U.S.: intrahepatic disease on the rise. […] Taylor-Robinson, S. D. et al. Increase in mortality rates from intrahepatic cholangiocarcinoma in England and Wales 1968-1998.

• #1 Prognosis for bile duct cancer: What to know

  https://www.medicalnewstoday.com/articles/bile-duct-cancer-prognosis-2

  Most people receive a bile duct diagnosis in the later stages of the disease. In this case, healthcare professionals may offer them palliative care to alleviate their symptoms. […] Experts continue to develop new cancer treatments, so the outlook for people with bile duct cancer may change as newer treatments become available.

• #2 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.

• #2 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.

• #2 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.

• #2 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.

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

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

  CCA is the second most common primary hepatic malignancy after hepatocellular carcinoma (HCC), comprising approximately 15% of all primary liver tumours and 3% of gastrointestinal cancers. CCAs are usually asymptomatic in early stages and, therefore, often diagnosed when the disease is already in advanced stages, which highly compromises therapeutic options, resulting in a dismal prognosis. CCA is a rare cancer, but its incidence (0.36 per 100,000 inhabitants per year) and mortality (16 per 100,000 inhabitants per year, globally, not taking into account specific regions with incidence 6 per 100,000 habitants such as South Korea, China and Thailand) have been increasing in the past few decades worldwide, representing a global health problem. […] The three subtypes of CCA can have different risk factors, pathobiology, clinical presentations, management and prognosis, as well as distinct epidemiological trends. 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. However, these trends need cautious interpretation given that all versions of the main International Classification of Diseases (ICD) have so far failed to include a separate code for the largest group of CCA (pCCA) and previous versions of ICD-Oncology (ICD-O) have cross-referenced pCCA (technically extrahepatic) to iCCA. Importantly, for the first time, subsequent iterations of both ICD and ICD-O (ICD-11 and ICD-O-4, respectively) which are due to come into effect by 2021 will have separate codes for recording iCCA, pCCA and dCCA. Having clearly defined codes for the three subtypes of CCA might facilitate more accurate and meaningful epidemiological data. In the meantime, reported epidemiological trends for CCA and/or biliary tract cancer need to be interpreted carefully.

• #2 Cholangiocarcinoma: Practice Essentials, Pathophysiology, Epidemiology

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

  Despite aggressive anticancer therapy and interventional supportive care (ie, wall stents or percutaneous biliary drainage), the median survival rate is low, since most patients (90%) are not eligible for curative resection. The overall survival is approximately 6 months. […] 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. The high prevalence of cholangiocarcinoma in people of Asian descent is attributable to endemic chronic parasitic infestation. […] In both males and females, cholangiocarcinoma is most common in persons in their 60s and 70s. The male-to-female ratio for cholangiocarcinoma is 1:2.5 in patients in their 60s and 70s and 1:15 in patients younger than 40 years. 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. The estimated number of new cases of gallbladder and other biliary cancers (extrahepatic cholangiocarcinoma) are 5900 for men and 6450 for women, with estimated deaths of 1950 and 2580, respectively.

• #2 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.

• #2 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.

• #2 Epidemiology and genomic features of biliary tract cancer and its unique features in Korea

  https://www.e-jlc.org/journal/view.php?number=589

  Biliary tract cancer (BTC) is a rare but highly aggressive malignancy that includes intrahepatic cholangiocarcinoma (ICC), extrahepatic cholangiocarcinoma, and gallbladder cancer (GBC). While BTC has a low global incidence, its regional variations are notable. Among nations, Korea has the second-highest incidence of BTC globally, with the highest mortality rate worldwide, underscoring the need for a deeper understanding of this cancer. Liver fluke infection and hepatitis B virus infection are key risk factors unique to Korea, contributing to regional differences in BTC incidence. […] Korea has the second-highest incidence of BTC globally and the highest mortality rate worldwide, emphasizing the need for a deeper understanding of this disease. […] Globally, BTC mortality rates remain relatively low, but several European countries, including Italy (4.2 deaths per 100,000 person-years), Germany (3.8), France (3.1), and the United Kingdom (3.2), report particularly low mortality rates. In contrast, in East Asia, Japan has significantly higher rates, with a reported mortality rate of 7.2, while Korea has the highest mortality rate in the world, at 11.0.

• #2 Liver and Intrahepatic Bile Duct Cancer — Cancer Stat Facts

  https://seer.cancer.gov/statfacts/html/livibd.html

  The rate of new cases of liver and intrahepatic bile duct cancer was 9.4 per 100,000 men and women per year based on 20182022 cases, age-adjusted. […] Liver and intrahepatic bile duct cancer is most frequently diagnosed among people aged 6574. […] Liver and intrahepatic bile duct cancer is the sixth leading cause of cancer death in the United States. The death rate was 6.6 per 100,000 men and women per year based on 20192023 deaths, age-adjusted. […] The percent of liver and intrahepatic bile duct cancer deaths is highest among people aged 6574. […] Keeping track of new cases, deaths, and survival over time (trends) can help scientists understand whether progress is being made and where additional research is needed to address challenges, such as improving screening or finding better treatments. […] Using statistical models for analysis, age-adjusted rates for new liver and intrahepatic bile duct cancer cases have been falling on average 0.7% each year over 20132022. Age-adjusted death rates have been stable over 20142023.

• #2 Epidemiologic patterns of biliary tract cancer in the United States: 2001–2015 | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-022-10286-z

  The overall mortality ASR for BTC patients was 3.40 (95% CI, 3.383.42). […] Compared with White individuals, all other race/ethnicity groups had a higher mortality rate for BTC with the highest rates among Hispanic and Asian/Pacific Islander individuals. […] Among the anatomic sites, the mortality rate was highest for ICC, particularly for Asian/Pacific Islander individuals. […] Five-year survival for BTC was 15.2% (95% CI, 14.815.7). […] The lowest survival was reported for ICC (8.5% [95% CI, 7.89.2]) and the highest for AVC (34.5% [95% CI, 33.036.0]). […] Five-year survival was low for patients with distant disease at first diagnosis (3.0% [95% CI, 2.63.4]), compared with regional (19.1% [95% CI, 18.320.0]) or localized (31.5% [95% CI, 30.332.7]) disease. […] From 2001 to 2015 in the US, BTC incidence increased, with the greatest increase occurring in ICC.

• #2 Prognosis for bile duct cancer: What to know

  https://www.medicalnewstoday.com/articles/bile-duct-cancer-prognosis-2

  A relative survival rate compares individuals with the same type and stage of cancer with people in the general population. […] The 5-year relative survival rates for intrahepatic bile duct cancer are as follows: localized: 24%, regional: 9%, distant: 2%, all stages combined: 9%. […] The 5-year relative survival rates for extrahepatic bile duct cancer are as follows: localized: 17%, regional: 16%, distant: 2%, all stages combined: 10%. […] It is important to note that these figures do not take all factors into account. […] Bile duct cancer is rare and aggressive. Although the disease typically has an unfavorable outlook, an individuals outlook depends on various factors, such as age, overall health, and their bodys response to treatment. […] The 5-year relative survival rates range between 224% for bile duct cancers that start within the liver, and 217% for bile duct cancers that start outside the liver.

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

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

  McLean et al. demonstrated a significantly higher incidence of ICC of Hispanic-Americans compared to other ethic groups, with African-Americans having the lowest incidence. The authors concluded that this variation may reflect potential genetic, cultural, or socioeconomic differences in ICC susceptibility. […] 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.

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

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

  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. […] 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.

• #2 Risks and causes of bile duct cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/bile-duct-cancer/risks-causes

  Bile duct cancer is also called cholangiocarcinoma. It is a rare type of cancer that develops in the small tubes that connect the liver and gallbladder to the small bowel. […] Survival for bile duct cancer depends on many factors including whether the cancer is in the bile ducts in the liver or outside of the liver. […] People with PSC may have an increased risk of developing cancer of the bile ducts. One study based in the Netherlands found that more than 5 in 100 people with PSC (more than 5%) went on to develop bile duct cancer. […] Liver cirrhosis can increase the risk of bile duct cancer. Especially intrahepatic bile duct cancer. […] Some IBDs like ulcerative colitis, can increase the risk of bile duct cancer. […] People who have had Thorotrast in the past have a higher chance of developing bile duct cancer.

• #2 Encouraging Treatment Advances for Aggressive Cancers of the Biliary Tract  – American Association for Cancer Research (AACR)

  https://www.aacr.org/blog/2023/02/06/encouraging-treatment-advances-for-aggressive-cancers-of-the-biliary-tract/

  Additional risk factors for biliary tract cancers include gallstones, gallbladder polyps, primary sclerosing cholangitis, chronic ulcerative colitis, cysts in the bile ducts, and certain parasitic or bacterial infections. […] The high mortality rates associated with biliary tract cancers can be daunting for the patients and families faced with a diagnosis, but research continues to move the dial forward, with the ultimate goal of improving patient outcomes.

• #2 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. […] The primary aim of screening is the early identification CCA to increase the probability of curative treatment. […] Incidence of CCA is highest in the first year of PSC diagnosis (2.5%), but it can develop any time throughout a patients life. […] By 30 years, the risk of developing CCA is as high as 20%. […] 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-IBD has been established or ruled out, patients are shunted into their respective CRC screening protocols. […] Dysplasia surveillance is performed by careful examination under high-resolution white light endoscopy and random colonic biopsies (typically every 10cm), with or without an alternative viewing modality such as chromoendoscopy or narrow-band imaging.

• #2 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. […] The Khon Kaen University in northeast Thailand, a region with very high incidences of liver flukes, has started the Cholangiocarcinoma Screening and Care Program (CASCAP) in collaboration with the National Health Security Office (NHSO) and the Ministry of Public Health. The aim of this study is to systematically improve the diagnosis and treatment of CCA patients throughout the northeast of Thailand.

• #2 Cholangiocarcinoma (Bile duct cancer) | Doctor

  https://patient.info/doctor/cholangiocarcinoma

  Chronic inflammation of the biliary epithelium is thought to be the critical shared feature of risk factors. Patients with chronic ulcerative colitis who develop primary sclerosing cholangitis are prone to cholangiocarcinoma. The lifetime risk of developing this cancer is 10-20% with primary sclerosing cholangitis. Some patients with Crohn’s disease may also be at risk. […] Diagnosis should be made on the basis of radiological investigations (including CT or MRI) and pathological assessment from a biopsy, fine-needle aspiration or biliary brush cytology. There are no blood tests that are diagnostic for cholangiocarcinoma, but testing is usually performed as part of the diagnostic process to look for other conditions, assess liver synthetic function, and aid the diagnosis. […] Staging is based on the tumour, node and metastasis (TNM) classification with some modifications for intrahepatic cholangiocarcinoma, perihilar cholangiocarcinoma and distal cholangiocarcinoma. The initial diagnostic tests may provide staging information. Additionally, a contrast CT of the abdomen, chest and pelvis should be performed to look for metastatic disease.

• #2 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

  Overall, AVC is a relatively rare form of cancer, accounting for just 15.1% of all BTC cases in the USA. […] Effective treatment of BTC remains a significant clinical challenge as patients often do not present for diagnosis until the disease is in an advanced stage. […] The rates of adjuvant therapy for BTC patients have also risen over the past 19 years, with several studies demonstrating a link between adjuvant therapy and improved BTC patient survival outcomes. […] The survival rates were low among ECC patients as well as ICC patients, potentially due to the fact that these two forms of BTC are relatively difficult to resect. […] In summary, the results of this analysis of SEER registry data over a 19-year period revealed that both ICC incidence and mortality rates have risen with time, while the incidence and mortality of ECC, GBC, and AVC have remained relatively stable.

• #2 Encouraging Treatment Advances for Aggressive Cancers of the Biliary Tract  – American Association for Cancer Research (AACR)

  https://www.aacr.org/blog/2023/02/06/encouraging-treatment-advances-for-aggressive-cancers-of-the-biliary-tract/

  According to a recent publication in the AACR journal Cancer Epidemiology, Biomarkers Prevention, bile duct cancers within the liver constituted the majority of biliary tract cancers between 2013 and 2017 (with an incidence rate of 1.49 per 100,000 individuals), followed by gallbladder cancers (1.11 per 100,000), bile duct cancers outside the liver (0.96 per 100,000), ampullary cancers (0.45 per 100,000), and cancers in other regions of the biliary tract (0.24 per 100,000). […] The incidence and mortality rates of biliary tract cancers increased with age, and incidence was highest among men and among individuals who identified as Hispanic or American Indian/Alaska Native. Asian and Hispanic patients had the highest rates of mortality compared with other racial/ethnic groups. […] Understanding the lifestyle factors that contribute to the onset of biliary tract cancers may ultimately help lower risk, identify patients who might benefit from increased monitoring, and/or lessen disparities between different demographic groups.

• #2 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 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.

• #2 Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente

  https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.bile-duct-cancer-cholangiocarcinoma-treatment-pdq%C2%AE-treatment-health-professional-information-nci.ncicdr0000062905

  The treatment of bile duct cancer depends primarily on whether the cancer can be completely removed by surgery. […] Localized intrahepatic and extrahepatic bile duct cancer may be completely removed by surgery. […] Most cases of intrahepatic, distal, and perihilar bile duct cancer are unresectable and cannot be completely removed. […] In locally advanced disease, phase II trials have evaluated chemoradiotherapy with the goal of improved local control and potential downstaging for surgical resection. […] For patients with unresectable bile duct cancer, management is directed at palliation.

• #2 About Bile Duct Cancer | University Hospitals

  https://www.uhhospitals.org/services/cancer-services/gastrointestinal-cancer/bile-duct-cancer/about-bile-duct-cancer

  Complete resection with negative surgical margins offers the only chance of cure for bile duct cancer. For localized, resectable extrahepatic and intrahepatic tumors, the presence of involved lymph nodes and perineural invasion are significant adverse prognostic factors. […] Bile duct cancer is classified as resectable (localized) or unresectable, with obvious prognostic importance. The TNM (tumor, node, metastasis) staging system is used for staging bile duct cancer, commonly after surgery and pathological examination of the resected specimen. Evaluation of the extent of disease at laparotomy is an important component of staging. […] Most cases of intrahepatic, distal, and perihilar bile duct cancer are unresectable and cannot be completely removed. Often the cancer invades directly into the portal vein, the adjacent liver, along the common bile duct, and to adjacent lymph nodes. […] Pending further clinical trials, cisplatin plus gemcitabine is considered the reference standard first-line chemotherapy backbone for patients with unresectable, metastatic, or recurrent bile duct cancer.

• #2 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. […] Boilas suite of preclinical models will also aid his efforts to discover potential new therapies for patients with ARID1A-mutated ICC. Currently the only targeted treatments for patients with ICC are aimed at tumors with mutations in IDH or a growth factor gene, FGFR.

• #2 Cholangiocarcinoma | Nature Reviews Disease Primers

  https://www.nature.com/articles/s41572-021-00300-2

  Patel, T. Worldwide trends in mortality from biliary tract malignancies. […] Bertuccio, P. et al. Global trends in mortality from intrahepatic and extrahepatic cholangiocarcinoma. […] Banales, J. M. et al. Cholangiocarcinoma 2020: the next horizon in mechanisms and management. […] Yao, K. J., Jabbour, S., Parekh, N., Lin, Y. Moss, R. A. Increasing mortality in the United States from cholangiocarcinoma: an analysis of the National Center for Health Statistics Database. […] Khan, S. A. et al. Rising trends in cholangiocarcinoma: is the ICD classification system misleading us? […] Rizvi, S., Eaton, J. E. Gores, G. J. Primary sclerosing cholangitis as a premalignant biliary tract disease: surveillance and management. […] Darwish Murad, S. et al. Efficacy of neoadjuvant chemoradiation, followed by liver transplantation, for perihilar cholangiocarcinoma at 12 US centers. […] This multicentre study established neoadjuvant chemoradiation plus liver transplantation as an effective option for perihilar cholangiocarcinoma.

• #3 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. In the past decade, increasing efforts have been made to understand the complexity of these tumours and to develop new diagnostic tools and therapies that might help to improve patient outcomes.

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

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

  Overall, CCA incidence is higher among men than women. […] 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.

• #3 Cholangiocarcinoma: Practice Essentials, Pathophysiology, Epidemiology

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

  Despite aggressive anticancer therapy and interventional supportive care (ie, wall stents or percutaneous biliary drainage), the median survival rate is low, since most patients (90%) are not eligible for curative resection. The overall survival is approximately 6 months. […] 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. The high prevalence of cholangiocarcinoma in people of Asian descent is attributable to endemic chronic parasitic infestation. […] In both males and females, cholangiocarcinoma is most common in persons in their 60s and 70s. The male-to-female ratio for cholangiocarcinoma is 1:2.5 in patients in their 60s and 70s and 1:15 in patients younger than 40 years. 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. The estimated number of new cases of gallbladder and other biliary cancers (extrahepatic cholangiocarcinoma) are 5900 for men and 6450 for women, with estimated deaths of 1950 and 2580, respectively.

• #3 Cholangiocarcinoma and Gallbladder Cancer Survival Rate | Roswell Park Comprehensive Cancer Center – Buffalo, NY

  https://www.roswellpark.org/cancer/biliary-cancers/survival-rate

  Survival rates for bile duct cancers are different depending on where the cancer began: Intrahepatic (the bile ducts within the liver) or Extrahepatic (the bile ducts outside the liver). […] In the United States, overall 5-year relative survival for intrahepatic bile duct cancer is 8%, according to data from the Surveillance, Epidemiology and End Results (SEER) program; survival rates by stage: […] In the United States, overall 5-year relative survival for extrahepatic bile duct cancer is 10%, according to data from the Surveillance, Epidemiology and End Results (SEER) program; survival rates by stage:

• #3 Outcomes (prognosis) for bile duct cancer treatment – Liver Cancer UK

  https://livercanceruk.org/liver-cancer-information/types-of-liver-cancer/bile-duct-cancer/treating-bile-duct-cancer/outcomes-prognosis-after-bile-buct-cancer-treatment/

  Bile duct cancer is a rare type of cancer. […] The most important thing is whether you can have an operation to completely remove the cancer and try to cure it. With this type of treatment, around 4 out of 10 people (40%) live for at least 5 years afterwards. […] Because staging is very complicated, and varies between types of bile duct cancer, it’s a good idea to check with your hospital doctor or specialist nurse about which group you’re in. […] Overall statistics for bile duct cancer indicate that in men, more than half (57%) lived for at least one year after their diagnosis, while in women, around half (49%) lived for at least one year after their diagnosis.

• #3 Risks and causes of bile duct cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/bile-duct-cancer/risks-causes

  Bile duct cancer is also called cholangiocarcinoma. It is a rare type of cancer that develops in the small tubes that connect the liver and gallbladder to the small bowel. […] Survival for bile duct cancer depends on many factors including whether the cancer is in the bile ducts in the liver or outside of the liver. […] People with PSC may have an increased risk of developing cancer of the bile ducts. One study based in the Netherlands found that more than 5 in 100 people with PSC (more than 5%) went on to develop bile duct cancer. […] Liver cirrhosis can increase the risk of bile duct cancer. Especially intrahepatic bile duct cancer. […] Some IBDs like ulcerative colitis, can increase the risk of bile duct cancer. […] People who have had Thorotrast in the past have a higher chance of developing bile duct cancer.

• #3 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. […] The Khon Kaen University in northeast Thailand, a region with very high incidences of liver flukes, has started the Cholangiocarcinoma Screening and Care Program (CASCAP) in collaboration with the National Health Security Office (NHSO) and the Ministry of Public Health. The aim of this study is to systematically improve the diagnosis and treatment of CCA patients throughout the northeast of Thailand.

• #3 Bile Duct Cancer (Cholangiocarcinoma) Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente

  https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.bile-duct-cancer-cholangiocarcinoma-treatment-pdq%C2%AE-treatment-health-professional-information-nci.ncicdr0000062905

  The treatment of bile duct cancer depends primarily on whether the cancer can be completely removed by surgery. […] Localized intrahepatic and extrahepatic bile duct cancer may be completely removed by surgery. […] Most cases of intrahepatic, distal, and perihilar bile duct cancer are unresectable and cannot be completely removed. […] In locally advanced disease, phase II trials have evaluated chemoradiotherapy with the goal of improved local control and potential downstaging for surgical resection. […] For patients with unresectable bile duct cancer, management is directed at palliation.

• #3 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. […] Boilas suite of preclinical models will also aid his efforts to discover potential new therapies for patients with ARID1A-mutated ICC. Currently the only targeted treatments for patients with ICC are aimed at tumors with mutations in IDH or a growth factor gene, FGFR.

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