Materiały źródłowe

• #1 Multi-Disciplinary Care of Hilar Cholangiocarcinoma: Review of Guidelines and Recent Advancements

  https://www.mdpi.com/2072-6694/16/1/30

  Cholangiocarcinoma (CCA) is a rare malignancy of the intrahepatic and extrahepatic biliary ducts. […] The incidence of CCAs (0.3–6 per 100,000) and mortality have been rising worldwide, particularly in South Asian countries including South Korea, China, and Thailand. […] HC represents 50–60% of all cholangiocarcinomas. […] The majority of HC tumors are sporadic, with an unknown etiology. […] Risk factors include choledochal cysts, choledocholithiasis or Caroli’s disease, cigarette smoking, alcohol, and systemic diseases like primary sclerosing cholangitis (PSC), cirrhosis, or chronic hepatitis B and C. […] The preponderance of HC in South Asia may be attributable to liver fluke infection. […] For patients with resectable disease, the standard of care is upfront surgery with adjuvant capecitabine.

• #1 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. […] In the meantime, reported epidemiological trends for CCA and/or biliary tract cancer need to be interpreted carefully. […] Furthermore, in the USA, the incidence of iCCA is higher in older people (45 years old) than in younger people and in Hispanic individuals than in non-Hispanic individuals, and is associated with a worse 5-year survival in both these populations.

• #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.

• #1 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. […] 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. […] ICC represents 10%-15% of all primary liver tumors and is the second primary hepatic malignancy after hepatocellular carcinoma. […] ICC appears to be the most common biliary tract cancer and accounts for over two-thirds of all cholangiocarcinomas; however, the literature on the topic is ambiguous, with some studies indicating that ECC, including hilar cholangiocarcinoma, accounts for the large majority of all cholangiocarcinoma cases.

• #1 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.

• #1 Cholangiocarcinoma: Practice Essentials, Pathophysiology, Epidemiology

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

  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. Incidence rates are particularly elevated in certain regions, such as South Korea, China, and Thailand.

• #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 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.

• #1 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. 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 The epidemiology and risk factors of hilar cholangiocarcinoma

  https://www.ekjm.org/journal/view.php?number=21148

  A hilar cholangiocarcinoma is a cholangiocarcinoma occurring at the confluence of the right and left hepatic bile ducts which is called as Klatskin tumor. Because hilar cholangiocarcinoma is the most common form of extrahepatic bile duct cancer (EBDC), the epidemiology and risk factors of hilar cholangiocarcinoma are similar to those of EBDC. In Korea, overall incidence of EBDC is 5.1/100,000 individuals without a significant change during past 10 years. Most of cases occur in patients over the age of 50 and the incidence is 1.78 times higher in men than women. […] Chronic biliary tract parasitic infection, such as Clonorchis sinensis and Opisthorchis viverrini, has been identified as a risk factor of EBDC and hilar cholangiocarcinoma. Other clear risk factors of EBDC are primary sclerosing cholangitis and choledochal cyst. However, there are no enough evidences whether primary sclerosing cholangitis and choledochal cyst are the definite risk factors of hilar cholangiocarcinoma or not.

• #1 The Epidemiology of Cholangiocarcinoma: Geographic Diversity in Disease Risk Factors | CCA News Online

  https://ccanewsonline.com/issues/2020/october-2020-vol-1-no-2/the-epidemiology-of-cholangiocarcinoma-geographic-diversity-in-disease-risk-factors

  In addition, the overall 5-year survival rate is 10%, with poorer survival seen in African-Americans, Native Americans, and Hispanic Americans. […] Dr Antwi pointed out that as in Europe, the incidence rate of intrahepatic CCA in the United States has also been increasing, and for the same reasons outlined by Dr Valle, with the rate of new cases of extrahepatic CCA remaining relatively stable. […] Furthermore, in the United States, according to Dr Antwi, a diagnosis of CCA is rare before the age of 40 years. The majority of patients with CCA are diagnosed after age 60 years; overall, 27% of individuals are diagnosed with intrahepatic CCA and 19% are diagnosed with extrahepatic CCA before age 60 years in the United States. […] It is difficult to compare epidemiologic data from the United States to other parts of the world, because the estimates were performed in different date ranges, Dr Antwi said. So, we are not looking at the same data, but one thing that can be claimed is that incidence rates across the globe do differ geographically, primarily due to the geographic diversity in risk factors, he concluded.

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

  https://hbsn.amegroups.org/article/view/13610/14587

  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. […] 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.

• #1 Perihilar or (Hilar) Cholangiocarcinoma: Interventional to Surgical Management | IntechOpen

  https://www.intechopen.com/chapters/64491

  Primary sclerosing cholangitis (PSC) is regarded as the prevalent risk factor for PHC in European nations and about 840% sufferers acquire this type of malignancy. Hepatobiliary flukes, together with Clonorchis sinensis and Opisthorchis viverrini, are the risk issues for cholangiocarcinoma in southeast Asian continent. Additional acknowledged risk factors for cholangiocarcinoma are intra-hepatic stones, biliary abnormalities such as Caroli disease, HCV infection, liver cirrhosis and acquaintance to thorium-containing contrast media. Cholangiocarcinoma affects more commonly in men than women, and the Asians are having higher incidence nearly twice over whites and blacks. […] HCCA is usually a tumor of the extrahepatic bile duct relating to the left and right main hepatic duct or their confluence. Biliary drainage in HCCA is oftentimes technically difficult as a consequence of difficulties linked to the degree of biliary obstruction. This could lead to some unfavorable situations, particularly in acute cholangitis. Therefore, the conclusion on the indication and techniques of biliary drainage in patients with HCCA ought to be meticulously assessed. PBD might have additional advantages in selected patients with the extreme lack of nutrition or biliary sepsis as well as in individuals considering the postponed surgical procedure depending upon on portal vein embolization or chemo-radio therapy.

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

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

  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. […] 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.

• #1 National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma

  https://www.wjgnet.com/1007-9327/full/v30/i9/1018.htm

  The causes of hCCA remain obscure in many patients. The role of genetic factors needs to be better defined. The estimated lifetime incidence of CCA with primary sclerosing cholangitis (PSC) ranges up to 20%. While PSC is a known risk factor for CCA, it is attributed to no more than 10% of CCA cases. Hepatobiliary flukes, specifically Opisthorchis viverrini and Clonorchis sinensis have been linked to the development of CCA in Southeast Asia, regardless of site. The presence of hepatitis B virus (commonly known as HBV) and hepatitis C virus (commonly known as HCV) has been linked to an increased risk of developing iCC. Studies do not confirm the association of HBV or HCV with hCCA. […] Patients with acute cholangitis can be drained with endoscopic or percutaneous biliary drainage. Palliative chemotherapy with cisplatin and gemcitabine has shown improved survival in patients with irresectable and recurrent hCCA.

• #1 Risk factors for intrahepatic and extrahepatic cholangiocarcinoma in the United States: A population-based study in SEER-Medicare | PLOS One

  https://journals.plos.org/plosone/article?id=10.1371/journal.pone.0186643

  Non-alcoholic fatty liver disease was associated with approximately 3-fold increased risks of ICC (OR = 3.52, 95% CI: 2.87-4.32) and ECC (OR = 2.93, 95% CI: 2.42-3.55). […] In the US, incidence rates of ICC have been increasing, even when accounting for misclassification of hilar cholangiocarcinoma (i.e., Klatskin tumors) as ICC instead of ECC. […] As metabolic conditions are increasing in prevalence, these could be increasingly important risk factors for both types of cholangiocarcinoma.

• #1 Systematic review of cholangiocarcinoma in Africa: epidemiology, management, and clinical outcomes | BMC Gastroenterology | Full Text

  https://bmcgastroenterol.biomedcentral.com/articles/10.1186/s12876-023-02687-6

  To date, no systematic review or meta-analysis of studies on CCA in Africa has been conducted. We address this gap by conducting a comprehensive systematic review on the epidemiology, management, and outcomes of CCA in Africa. […] The risk factors of CCA in Africa remain unclear, most occurring in absence of known risk factors. […] An Egyptian study has identified male gender (1.7:1), farming and rural residency, cirrhosis, hepatitis C infection (54%), Schistosomiasis (66.5%), chronic typhoid infection (52%) and gallstone disease as possible risk factors for hilar cholangiocarcinoma. […] CCA remains a late presenting disease in Africa. The best median survival outcome of 36 months were for patients who had undergone hepatectomy with caudate lobectomy. […] Eleven studies report on the management of CCA in Africa, 6 in Egypt, 2 in South Africa, 2 in Tunisia and 1 in Senegal.

• #1 Clinical manifestations and diagnosis of cholangiocarcinoma – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-cholangiocarcinoma

  Tumors involving the hepatic duct bifurcation (hilar cholangiocarcinomas) are collectively referred to as Klatskin tumors. […] Type IV tumors, defined as tumor invasion of the second order biliary radicles bilaterally, are associated with a higher rate of positive surgical margins and significantly poorer overall survival after resection compared with types I to III.

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

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

  A number of studies are examining the potential influence of commonly used drugs such as aspirin and lipid-lowering statins in the prevention of CCA. Notably, post-diagnosis aspirin usage has been found to be associated with a reduced risk of death among patients with CCA. […] There are currently no published genome-wide association studies (GWAS) in CCA, but an appropriately powered one is eagerly anticipated.

• #1 Palliation: Hilar cholangiocarcinoma

  https://www.wjgnet.com/1948-5182/full/v6/i8/559.htm

  Hilar cholangiocarcinomas are common tumors of the bile duct that are often unresectable at presentation. Palliation, therefore, remains the goal in the majority of these patients. […] Hilar cholangiocarcinomas (HC) or Klatskins tumors are the most common type of bile duct cancer throughout the world and constitute 46%-97% of all bile duct cancers. It has a particularly high prevalence in certain Asian countries, such as Thailand and China. […] Due to the critical nature and site of the disease, patients with HC suffer greatly from progressive jaundice, anorexia, pruritus, cholangitis and liver failure. Unfortunately, a majority of HC cases manifest late and are diagnosed at a stage when curative resection is not possible. […] Only approximately 20%-30% of patients with HC are diagnosed at a stage when surgical resection is possible.

• #1 Palliation: Hilar cholangiocarcinoma

  https://www.wjgnet.com/1948-5182/full/v6/i8/559.htm

  Multi-detector CT scan (MDCT) and magnetic resonance imaging (MRI) with MR Cholangiography continue to be the most two accurate modalities in the evaluation of the stages and resectability of HC. […] The accuracy of predicting hepatic artery invasion, portal vein involvement, lymph nodal metastasis and the extent of biliary ductal spread is approximately 85%-95% in both of these approaches. […] Not every patient with unresectable HC needs palliative intervention. Patients with complications of cholangitis and intractable pruritus are definite candidates for palliation. […] There are three established methods for the palliation of HC: endoscopic insertion of a stent, percutaneous placement of biliary drainage and surgical bypass. […] Endosonography-guided procedures have been evolving as alternatives to these standard techniques.

• #1 Klatskin tumor | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/klatskin-tumor-1?lang=us

  They are thought to account for up to 25% of all cholangiocarcinomas 1. […] MRCP with its 3D capability is superior to CT to delineate the degree and location of the biliary tree stricture. It is the gold standard method for diagnosis of hilar cholangiocarcinoma. Shouldering and abrupt tapering at the stricture site suggests the diagnosis. Identifying the exact location and involvement can help in preoperative classification.

• #1 National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma

  https://www.wjgnet.com/1007-9327/full/v30/i9/1018.htm

  A consensus meeting of national experts from all major national hepatobiliary centres in the country was held on May 26, 2023, at the Pakistan Kidney and Liver Institute Research Centre (PKLI RC) after initial consultations with the experts. The Pakistan Society for the Study of Liver Diseases (PSSLD) and PKLI RC jointly organised this meeting. This effort was based on a comprehensive literature review to establish national practice guidelines for hilar cholangiocarcinoma (hCCA). The consensus was that hCCA is a complex disease and requires a multidisciplinary team approach to best manage these patients. This coordinated effort can minimise delays and give patients a chance for curative treatment and effective palliation. […] The diagnostic and staging workup includes high-quality computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography. Brush cytology or biopsy utilizing endoscopic retrograde cholangiopancreatography is a mainstay for diagnosis. However, histopathologic confirmation is not always required before resection. Endoscopic ultrasound with fine needle aspiration of regional lymph nodes and positron emission tomography scan are valuable adjuncts for staging. The only curative treatment is the surgical resection of the biliary tree based on the Bismuth-Corlette classification. Selected patients with unresectable hCCA can be considered for liver transplantation. Adjuvant chemotherapy should be offered to patients with a high risk of recurrence. The use of preoperative biliary drainage and the need for portal vein embolisation should be based on local multidisciplinary discussions.

• #1 Hilar cholangiocarcinoma – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hilar-cholangiocarcinoma/cdc-20354548

  Hilar cholangiocarcinoma is a type of cancer that starts as a growth of cells in the bile ducts. […] Hilar cholangiocarcinoma is the most common type of bile duct cancer, but it is still a rare form of cancer. […] It’s not clear what causes most hilar cholangiocarcinomas. […] Hilar cholangiocarcinoma happens when cells in a bile duct develop changes in their DNA. […] Factors that may increase the risk of hilar cholangiocarcinoma include: Primary sclerosing cholangitis, older age, inflammatory bowel disease, liver cirrhosis, bile duct cysts, gallbladder conditions, and liver parasites. […] Hilar cholangiocarcinoma diagnosis often begins with a physical exam and a discussion of your health history. […] Imaging tests make pictures of the body. […] Tumor markers for hilar cholangiocarcinoma include carbohydrate antigen 19-9, also called CA 19-9, and carcinoembryonic antigen, also called CEA.

• #1 Cholangiocarcinoma

  https://www.ajmc.com/compendium/cholangiocarcinoma

  Surveillance Program Ineffective at Detecting Cholangiocarcinoma Early Enough for Benefit in Patients With PSC […] A new report consolidates existing research on hilar cholangiocarcinoma resection, but finds more work is needed to better evaluate the efficacy of robotic surgery in these patients. […] A new review article notes efforts to diagnose cholangiocarcinoma (CCA) early are hampered by a lack of biomarkers.

• #1 Cholangiocarcinoma: Epidemiology, Pathogenesis, and Prognosis | SpringerLink

  https://link.springer.com/10.1007/978-3-030-41683-6_13

  Cholangiocarcinoma is a malignancy arising from epithelial cells of the biliary tract characterized by its aggressive natural history, late diagnosis, and often fatal outcome. […] It is the most common biliary malignancy accounting for approximately 15-20% of all primary hepatobiliary cancers. […] There are substantial geographical variations in incidence with the highest rates reported in Southeast Asia. […] Patients with non-resectable advanced-stage cholangiocarcinoma have a dismal prognosis with a median survival of less than 24 months after diagnosis. […] If patients are diagnosed at an early stage, complete tumor resection is the only treatment that offers the best chance of cure and long-term survival. […] Unfortunately, despite R0 negative margin resections, long-term prognosis defined by survival and tumor recurrence remains poor in the presence of aggressive tumor-related factors.

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

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

  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 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. […] 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. […] In locally advanced disease, phase II trials have evaluated chemoradiotherapy with the goal of improved local control and potential downstaging for surgical resection.

• #1 Cholangiocarcinoma: Epidemiology, Pathogenesis, and Prognosis | SpringerLink

  https://link.springer.com/10.1007/978-3-030-41683-6_13

  Hilar cholangiocarcinoma: diagnosis, treatment options, and management. […] Prognostic predictability of American Joint Committee on Cancer 8th staging system for perihilar cholangiocarcinoma: Limited improvement compared with the 7th staging system. […] Hilar cholangiocarcinoma: tumor depth as a predictor of outcome. […] Prognostic factors and long-term outcomes of hilar cholangiocarcinoma: a single institution experience in China. […] Predictors of long term survival after hepatic resection for hilar cholangiocarcinoma: a retrospective study of 5-year survivors. […] The clinicopathological factors associated with prognosis of patients with resectable perihilar cholangiocarcinoma. […] Disease recurrence patterns after R0 resection of hilar cholangiocarcinoma. […] Predictive factors of early recurrence after R0 resection of hilar cholangiocarcinoma a single institution experience in China.

• #1 Multi-Disciplinary Care of Hilar Cholangiocarcinoma: Review of Guidelines and Recent Advancements

  https://www.mdpi.com/2072-6694/16/1/30

  Unfortunately, most patients will develop recurrent or metastatic disease afterwards. […] Given the tumor heterogeneity associated with CCA, research has focused on developing effective targeted therapies. […] The European Network for the Study of Cholangiocarcinoma (ENS-CC), the NCCN, and the ESMO guidelines recommend neoadjuvant chemoradiotherapy and liver transplant evaluation for patients with unresectable HC or HC in the setting of PSC. […] The neoadjuvant therapy regimen in this setting includes external beam radiotherapy (EBRT) and an infusion of 5-fluorouracil (5-FU). […] Although early initial studies demonstrated a high recurrence rate of 55%, further multicenter retrospective studies demonstrated a 5-year DFS of 65% as well as a 5-year intention-to-treat survival of 53%. […] The 5-year survival rate in patients with PSC who have undergone liver transplantation with neoadjuvant chemoradiotherapy is more variable, between 55–82%.

• #1 National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma

  https://www.wjgnet.com/1007-9327/full/v30/i9/1018.htm

  Consensus among national hepatobiliary experts convened at the Pakistan Kidney and Liver Institute Research Centre emphasized the complexity of hilar cholangiocarcinoma, advocating a multidisciplinary approach for optimal patient management. Diagnostic protocol includes imaging like computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography, while endoscopic retrograde cholangiopancreatography plays an important role in tissue acquisition. Surgical resection remains the best curative treatment option. For unresectable cases, liver transplantation is considered under strict selection criteria. Preoperative biliary drainage and portal vein embolisation decisions may be needed for selective cases. Adjuvant chemotherapy addresses the risk of recurrence. The role of Immunotherapy is emerging and offers improved survival for irresectable hilar cholangiocarcinoma.

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

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

  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 Cholangiocarcinoma: Epidemiology and risk factors – Bragazzi – Translational Gastrointestinal Cancer

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

  Cholangiocarcinoma (CCA) is a malignant tumour, arising from biliary epithelium at any portion of the biliary tree, characterized by a bad prognosis and poor response to current therapies. CCA is currently classified as intrahepatic (IH-CCA) or extrahepatic (EH-CCA). The distinction between IH-CCA and EH-CCA has become increasingly important, as the epidemiological features (i.e., incidence and risk factors), the biologic and pathologic characteristics and the clinical course are largely different. […] The epidemiologic data on CCA and its different forms are affected by the lack of worldwide uniform classification. In general, a number of biases and criticisms should be taken into consideration in evaluating the literature dealing with epidemiology and risk factors of CCA and especially of the two main forms, the IH- and EH-CCA: (I) in many cancer registries CCA is combined with other primitive liver cancers and the perihilar EH-CCA has been often considered as IH-CCA.

• #2 Clinical manifestations and diagnosis of cholangiocarcinoma – UpToDate

  https://www.uptodate.com/contents/clinical-manifestations-and-diagnosis-of-cholangiocarcinoma

  Cholangiocarcinomas (bile duct cancers) arise from the epithelial cells of the bile ducts. Although they are rare in the United States, these cancers are highly lethal because most are locally advanced at presentation. (See „Epidemiology, risk factors, anatomy, and pathology of cholangiocarcinoma.”) […] The epidemiology, pathogenesis, classification, and treatment of cholangiocarcinoma are discussed separately. […] Approximately 5 to 10 percent of cholangiocarcinomas are intrahepatic. Intrahepatic cholangiocarcinomas can originate from either small intrahepatic ductules (peripheral cholangiocarcinomas) or large intrahepatic ducts proximal to the bifurcation of the right and left hepatic ducts. […] Cancers arising in the perihilar region, which account for 60 to 70 percent of extrahepatic cholangiocarcinomas, have been further classified according to the pattern of involvement of the hepatic ducts (the Bismuth-Corlette classification).

• #2 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. […] 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. […] ICC represents 10%-15% of all primary liver tumors and is the second primary hepatic malignancy after hepatocellular carcinoma. […] ICC appears to be the most common biliary tract cancer and accounts for over two-thirds of all cholangiocarcinomas; however, the literature on the topic is ambiguous, with some studies indicating that ECC, including hilar cholangiocarcinoma, accounts for the large majority of all cholangiocarcinoma cases.

• #2 Cholangiocarcinoma: MedlinePlus GeneticsLock

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

  Cholangiocarcinoma affects 8,000 people each year in the United States. […] This type of cancer occurs much more frequently in Southeast Asian countries such as Thailand, where it is related to infection with a parasite that is common there. […] For unknown reasons, cholangiocarcinoma occurs slightly more often in men than in women.

• #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. […] They usually present in the elderly, with a mean age of 65 years. There may be a slight male predilection. […] Cholangiocarcinomas correspond to ~15% of all primary liver tumors and to ~3% of all gastrointestinal malignancies.

• #2 The epidemiology and risk factors of hilar cholangiocarcinoma

  https://www.ekjm.org/journal/view.php?number=21148

  A hilar cholangiocarcinoma is a cholangiocarcinoma occurring at the confluence of the right and left hepatic bile ducts which is called as Klatskin tumor. Because hilar cholangiocarcinoma is the most common form of extrahepatic bile duct cancer (EBDC), the epidemiology and risk factors of hilar cholangiocarcinoma are similar to those of EBDC. In Korea, overall incidence of EBDC is 5.1/100,000 individuals without a significant change during past 10 years. Most of cases occur in patients over the age of 50 and the incidence is 1.78 times higher in men than women. […] Chronic biliary tract parasitic infection, such as Clonorchis sinensis and Opisthorchis viverrini, has been identified as a risk factor of EBDC and hilar cholangiocarcinoma. Other clear risk factors of EBDC are primary sclerosing cholangitis and choledochal cyst. However, there are no enough evidences whether primary sclerosing cholangitis and choledochal cyst are the definite risk factors of hilar cholangiocarcinoma or not.

• #2 Epidemiology of cholangiocarcinoma

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

  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. […] In conclusion, the present analysis confirmed a global increase in ICC mortality and showed more favorable trends for ECC, with, however, some differences across countries.

• #2 Cholangiocarcinoma: Current Knowledge and New Developments

  https://www.gutnliver.org/journal/view.html?doi=10.5009/gnl15568

  During the last three decades, age-adjusted incidence rates (AAIR) of iCCA increased in Western Europe, while the incidence of extrahepatic CCA followed a stable to decreasing trend. […] Interestingly, AAIR of extrahepatic CCA in the United States had significantly increased throughout the last four decades while iCCA incidence remained overall stable. […] Throughout the last decade, annual mortality rates of iCCA in the United States decreased by 2.5%, while they increased by 9% in Europe. […] The male-to-female ratio of CCA is 1:1.21.5.2 Globally, the average age at diagnosis is 50 years. In Western industrialized nations, the median age at presentation is 65 years. It is uncommon before age 40 except in patients with primary sclerosing cholangitis (PSC).

• #2 Perihilar or (Hilar) Cholangiocarcinoma: Interventional to Surgical Management | IntechOpen

  https://www.intechopen.com/chapters/64491

  Primary sclerosing cholangitis (PSC) is regarded as the prevalent risk factor for PHC in European nations and about 840% sufferers acquire this type of malignancy. Hepatobiliary flukes, together with Clonorchis sinensis and Opisthorchis viverrini, are the risk issues for cholangiocarcinoma in southeast Asian continent. Additional acknowledged risk factors for cholangiocarcinoma are intra-hepatic stones, biliary abnormalities such as Caroli disease, HCV infection, liver cirrhosis and acquaintance to thorium-containing contrast media. Cholangiocarcinoma affects more commonly in men than women, and the Asians are having higher incidence nearly twice over whites and blacks. […] HCCA is usually a tumor of the extrahepatic bile duct relating to the left and right main hepatic duct or their confluence. Biliary drainage in HCCA is oftentimes technically difficult as a consequence of difficulties linked to the degree of biliary obstruction. This could lead to some unfavorable situations, particularly in acute cholangitis. Therefore, the conclusion on the indication and techniques of biliary drainage in patients with HCCA ought to be meticulously assessed. PBD might have additional advantages in selected patients with the extreme lack of nutrition or biliary sepsis as well as in individuals considering the postponed surgical procedure depending upon on portal vein embolization or chemo-radio therapy.

• #2 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 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). [...] The overall observed survival at 12, 36, and 60 months was 36.3%, 12.8%, and 8.1%, respectively. [...] Compared with nonsurgical intervention, surgery alone was associated with improved survival (HRadj: 0.28, 95% CI 0.26-0.31; p < 0.01). [...] Our study confirms the continued increase in ICC rates in recent years, particularly in the older population. Surgery remains the treatment modality with a proven increase in 5-year survival. Blacks experience worse mortality than the White population. Lymph node removal during surgery is associated with significantly better outcomes.

• #2 National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma

  https://www.wjgnet.com/1007-9327/full/v30/i9/1018.htm

  The causes of hCCA remain obscure in many patients. The role of genetic factors needs to be better defined. The estimated lifetime incidence of CCA with primary sclerosing cholangitis (PSC) ranges up to 20%. While PSC is a known risk factor for CCA, it is attributed to no more than 10% of CCA cases. Hepatobiliary flukes, specifically Opisthorchis viverrini and Clonorchis sinensis have been linked to the development of CCA in Southeast Asia, regardless of site. The presence of hepatitis B virus (commonly known as HBV) and hepatitis C virus (commonly known as HCV) has been linked to an increased risk of developing iCC. Studies do not confirm the association of HBV or HCV with hCCA. […] Patients with acute cholangitis can be drained with endoscopic or percutaneous biliary drainage. Palliative chemotherapy with cisplatin and gemcitabine has shown improved survival in patients with irresectable and recurrent hCCA.

• #2 Hilar cholangiocarcinoma – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hilar-cholangiocarcinoma/cdc-20354548

  Hilar cholangiocarcinoma is a type of cancer that starts as a growth of cells in the bile ducts. […] Hilar cholangiocarcinoma is the most common type of bile duct cancer, but it is still a rare form of cancer. […] It’s not clear what causes most hilar cholangiocarcinomas. […] Hilar cholangiocarcinoma happens when cells in a bile duct develop changes in their DNA. […] Factors that may increase the risk of hilar cholangiocarcinoma include: Primary sclerosing cholangitis, older age, inflammatory bowel disease, liver cirrhosis, bile duct cysts, gallbladder conditions, and liver parasites. […] Hilar cholangiocarcinoma diagnosis often begins with a physical exam and a discussion of your health history. […] Imaging tests make pictures of the body. […] Tumor markers for hilar cholangiocarcinoma include carbohydrate antigen 19-9, also called CA 19-9, and carcinoembryonic antigen, also called CEA.

• #2 Epidemiology and risk factors: intrahepatic cholangiocarcinoma

  https://pmc.ncbi.nlm.nih.gov/articles/PMC5411279/

  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. […] 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.

• #2 Intrahepatic and Hilar Cholangiocarcinomas: Epidemiology, Basic Principles of Treatment, and Clinical Data | Oncohema Key

  https://oncohemakey.com/intrahepatic-and-hilar-cholangiocarcinomas-epidemiology-basic-principles-of-treatment-and-clinical-data/

  The BismuthCorlette classification has long been used to categorize hilar cholangiocarcinoma based on the extent of hepatic ductal involvement in order to better guide surgical management. Hilar cholangiocarcinomas are divided into four types according to this system: Type I, tumor below the confluence of the left and right hepatic ducts; Type II, tumor reaching the confluence; Type III, tumor occluding the common hepatic and either the right hepatic ducts (Type IIIa) or left hepatic ducts (Type IIIb); Type IV, tumor that involves the confluence and both right and left hepatic ducts or tumors that are multicentric. […] A separate AJCC/UICC staging system has been developed for hilar cholangiocarcinoma in the 7th Edition. This is a significant change from prior AJCC systems in which hilar cholangiocarcinoma was staged as distal cholangiocarcinoma. The current staging system takes into account depth of bile duct invasion, hepatic extension, portal vein and hepatic artery involvement, and nodal and distant metastasis, though does not assess factors important in determining resectability, which are closely tied to disease stratification and outcomes.

• #2 Survival analysis of extrahepatic cholangiocarcinoma based on surveillance, epidemiology, and end results database

  https://www.ahbps.org/journal/view.html?volume=27&number=2&spage=151

  Cholangiocarcinoma (CCA) can be classified as intrahepatic CCA or extrahepatic CCA (eCCA). We intended to analyze and reports the survival outcomes for eCCA. […] In the United States, approximately 23,000 cases of CCA are diagnosed annually. Unfortunately, diagnosis is usually made at advanced stages due to non-specific early-stage symptoms and the lack of screening strategies. […] The incidence of cholangiocarcinoma is high in Southeast Asia. There are no recent US data regarding survival analyses of eCCA, significantly when differentiating between therapeutic interventions. […] Our study reported no differences in survival based on sex or race. Surgery with chemoradiotherapy remains the treatment modality with a demonstrated increase in 5-year survival. Black or American Indian/Alaska Native patients did not differ significantly in mortality compared with whites, Asians, or Pacific Islanders. Lymph node resection during surgery is associated with significantly better outcomes. dCCA has better outcomes than pCCA; lymph node dissection is equally beneficial for pCCA and dCCA.

• #2 National guidelines for the diagnosis and treatment of hilar cholangiocarcinoma

  https://www.wjgnet.com/1007-9327/full/v30/i9/1018.htm

  A consensus meeting of national experts from all major national hepatobiliary centres in the country was held on May 26, 2023, at the Pakistan Kidney and Liver Institute Research Centre (PKLI RC) after initial consultations with the experts. The Pakistan Society for the Study of Liver Diseases (PSSLD) and PKLI RC jointly organised this meeting. This effort was based on a comprehensive literature review to establish national practice guidelines for hilar cholangiocarcinoma (hCCA). The consensus was that hCCA is a complex disease and requires a multidisciplinary team approach to best manage these patients. This coordinated effort can minimise delays and give patients a chance for curative treatment and effective palliation. […] The diagnostic and staging workup includes high-quality computed tomography, magnetic resonance imaging, and magnetic resonance cholangiopancreatography. Brush cytology or biopsy utilizing endoscopic retrograde cholangiopancreatography is a mainstay for diagnosis. However, histopathologic confirmation is not always required before resection. Endoscopic ultrasound with fine needle aspiration of regional lymph nodes and positron emission tomography scan are valuable adjuncts for staging. The only curative treatment is the surgical resection of the biliary tree based on the Bismuth-Corlette classification. Selected patients with unresectable hCCA can be considered for liver transplantation. Adjuvant chemotherapy should be offered to patients with a high risk of recurrence. The use of preoperative biliary drainage and the need for portal vein embolisation should be based on local multidisciplinary discussions.

• #2 Cholangiocarcinoma: Epidemiology, Pathogenesis, and Prognosis | SpringerLink

  https://link.springer.com/10.1007/978-3-030-37482-2_13-1

  Cholangiocarcinoma is a malignancy arising from epithelial cells of the biliary tract characterized by its aggressive natural history, late diagnosis, and often fatal outcome. […] It is the most common biliary malignancy accounting for approximately 15-20% of all primary hepatobiliary cancers. […] There are substantial geographical variations in incidence with the highest rates reported in Southeast Asia. […] Cholangiocarcinogenesis is driven by a complex multistep process beginning with chronic biliary tract inflammation followed by exposure to carcinogenic molecules and overexpression of proliferative growth factors. […] Patients with non-resectable advanced-stage cholangiocarcinoma have a dismal prognosis with a median survival of less than 24 months after diagnosis. […] If patients are diagnosed at an early stage, complete tumor resection is the only treatment that offers the best chance of cure and long-term survival.

• #2 Cholangiocarcinoma: Epidemiology, Pathogenesis, and Prognosis | SpringerLink

  https://link.springer.com/10.1007/978-3-030-41683-6_13

  Cholangiocarcinoma is a malignancy arising from epithelial cells of the biliary tract characterized by its aggressive natural history, late diagnosis, and often fatal outcome. […] It is the most common biliary malignancy accounting for approximately 15-20% of all primary hepatobiliary cancers. […] There are substantial geographical variations in incidence with the highest rates reported in Southeast Asia. […] Patients with non-resectable advanced-stage cholangiocarcinoma have a dismal prognosis with a median survival of less than 24 months after diagnosis. […] If patients are diagnosed at an early stage, complete tumor resection is the only treatment that offers the best chance of cure and long-term survival. […] Unfortunately, despite R0 negative margin resections, long-term prognosis defined by survival and tumor recurrence remains poor in the presence of aggressive tumor-related factors.

• #2 Cholangiocarcinoma: Epidemiology, Pathogenesis, and Prognosis | SpringerLink

  https://link.springer.com/10.1007/978-3-030-37482-2_13-1

  Unfortunately, despite R0 negative margin resections, long-term prognosis defined by survival and tumor recurrence remains poor in the presence of aggressive tumor-related factors. […] Hilar cholangiocarcinoma: diagnosis, treatment options, and management. […] Prognostic factors and long-term outcomes of hilar cholangiocarcinoma: a single institution experience in China. […] Hilar cholangiocarcinoma: the Memorial Sloan-Kettering Cancer Center experience. […] Predictors of long term survival after hepatic resection for hilar cholangiocarcinoma: a retrospective study of 5-year survivors. […] The clinicopathological factors associated with prognosis of patients with resectable perihilar cholangiocarcinoma. […] Disease recurrence patterns after R0 resection of hilar cholangiocarcinoma. […] Predictive factors of early recurrence after R0 resection of hilar cholangiocarcinoma a single institution experience in China.

• #3 Palliation: Hilar cholangiocarcinoma

  https://www.wjgnet.com/1948-5182/full/v6/i8/559.htm

  Hilar cholangiocarcinomas are common tumors of the bile duct that are often unresectable at presentation. Palliation, therefore, remains the goal in the majority of these patients. […] Hilar cholangiocarcinomas (HC) or Klatskins tumors are the most common type of bile duct cancer throughout the world and constitute 46%-97% of all bile duct cancers. It has a particularly high prevalence in certain Asian countries, such as Thailand and China. […] Due to the critical nature and site of the disease, patients with HC suffer greatly from progressive jaundice, anorexia, pruritus, cholangitis and liver failure. Unfortunately, a majority of HC cases manifest late and are diagnosed at a stage when curative resection is not possible. […] Only approximately 20%-30% of patients with HC are diagnosed at a stage when surgical resection is possible.

• #3 Multi-Disciplinary Care of Hilar Cholangiocarcinoma: Review of Guidelines and Recent Advancements

  https://www.mdpi.com/2072-6694/16/1/30

  Cholangiocarcinoma (CCA) is a rare malignancy of the intrahepatic and extrahepatic biliary ducts. […] The incidence of CCAs (0.3–6 per 100,000) and mortality have been rising worldwide, particularly in South Asian countries including South Korea, China, and Thailand. […] HC represents 50–60% of all cholangiocarcinomas. […] The majority of HC tumors are sporadic, with an unknown etiology. […] Risk factors include choledochal cysts, choledocholithiasis or Caroli’s disease, cigarette smoking, alcohol, and systemic diseases like primary sclerosing cholangitis (PSC), cirrhosis, or chronic hepatitis B and C. […] The preponderance of HC in South Asia may be attributable to liver fluke infection. […] For patients with resectable disease, the standard of care is upfront surgery with adjuvant capecitabine.

• #3 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.

• #3 The Epidemiology of Cholangiocarcinoma: Geographic Diversity in Disease Risk Factors | CCA News Online

  https://ccanewsonline.com/issues/2020/october-2020-vol-1-no-2/the-epidemiology-of-cholangiocarcinoma-geographic-diversity-in-disease-risk-factors

  Cholangiocarcinoma (CCA) knows no geographic borders. Nevertheless, some geographical areas in the world are associated with increased risk for this type of cancer, according to recent research. […] Dr Valle presented an analysis of mortality rates globally for people with CCA, by age and by sex. The study showed that in the early 2000 years, the mortality rates for intrahepatic CCA were consistently below 1 patient per 100,000 population. […] By 2014, the mortality rates for men with intrahepatic CCA increased to 1 to 2 per 100,000 men in most European countries; the rates also increased for women compared with the early 2000s, but overall, the death rates were lower for women than for men. […] Dr Antwi presented an update on the epidemiologic data from the United States. […] According to Dr Antwi, the incidence of intrahepatic and extrahepatic CCA in the United States is higher in men than in women (ratio, 1.2 to 1.5, respectively).

• #3 Hilar cholangiocarcinoma – Overview – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/hilar-cholangiocarcinoma/cdc-20354548

  Hilar cholangiocarcinoma is a type of cancer that starts as a growth of cells in the bile ducts. […] Hilar cholangiocarcinoma is the most common type of bile duct cancer, but it is still a rare form of cancer. […] It’s not clear what causes most hilar cholangiocarcinomas. […] Hilar cholangiocarcinoma happens when cells in a bile duct develop changes in their DNA. […] Factors that may increase the risk of hilar cholangiocarcinoma include: Primary sclerosing cholangitis, older age, inflammatory bowel disease, liver cirrhosis, bile duct cysts, gallbladder conditions, and liver parasites. […] Hilar cholangiocarcinoma diagnosis often begins with a physical exam and a discussion of your health history. […] Imaging tests make pictures of the body. […] Tumor markers for hilar cholangiocarcinoma include carbohydrate antigen 19-9, also called CA 19-9, and carcinoembryonic antigen, also called CEA.

• #3 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. 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: Epidemiology, Pathogenesis, and Prognosis | SpringerLink

  https://link.springer.com/10.1007/978-3-030-37482-2_13-1

  Cholangiocarcinoma is a malignancy arising from epithelial cells of the biliary tract characterized by its aggressive natural history, late diagnosis, and often fatal outcome. […] It is the most common biliary malignancy accounting for approximately 15-20% of all primary hepatobiliary cancers. […] There are substantial geographical variations in incidence with the highest rates reported in Southeast Asia. […] Cholangiocarcinogenesis is driven by a complex multistep process beginning with chronic biliary tract inflammation followed by exposure to carcinogenic molecules and overexpression of proliferative growth factors. […] Patients with non-resectable advanced-stage cholangiocarcinoma have a dismal prognosis with a median survival of less than 24 months after diagnosis. […] If patients are diagnosed at an early stage, complete tumor resection is the only treatment that offers the best chance of cure and long-term survival.

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