Materiały źródłowe

• #1 Cholangiocarcinoma: Symptoms, Causes, Types, Staging & Treatment PACE Hospitals – Best Hospitals in Hitech City, Hyderabad, India | Near Madhapur, Kukatpally, KPHB, Kondapur, Gachibowli, Jubilee Hills, Banjara HillsPACE Hospitals Contact

  https://www.pacehospital.com/cholangiocarcinoma-bile-duct-cancer-symptoms-causes-types-staging-treatment

  Hilar Cholangiocarcinoma (hCCA) or Klatskin Tumor is the most common subtype, accounting for 50–60% of cases, and arises at the confluence of the right and left hepatic ducts, often leading to bile duct obstruction and early-onset jaundice. It is also called perihilar cholangiocarcinoma (pHCC). hCCA typically presents as a locally advanced disease, making surgical resection challenging, and frequently invades adjacent vascular structures, further complicating curative treatment.

• #2 Cholangiocarcinoma – Clinical Features – Investigations – TeachMeSurgery

  https://teachmesurgery.com/hpb/gall-bladder/cholangiocarcinoma/

  The most common site for bile duct cancers is at the bifurcation of the right and left hepatic ducts, termed Klatskin tumours or hilar cholangiocarcinoma, and account for 60% of cholangiocarcinoma. […] These tumours generally invade local structures, such as the portal vein and hepatic arteries, and metastasise to local lymph nodes, before spreading to the peritoneal cavity, lung, and liver. […] Intrahepatic cholangiocarcinoma is generally asymptomatic until a late stage in the disease, whereas extrahepatic cholangiocarcinoma will typically present early. […] Patients will present with jaundice, along with associated pruritus, steatorrhoea, non-specific abdominal pain, or dark urine. In advanced disease, weight loss and lethargy can occur. […] On examination, jaundice and cachexia are often evident.

• #3 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The early symptoms of hilar cholangiocarcinoma are often non-specific. Abdominal pain or discomfort, anorexia, weight loss and pruritus are the most common but are seen only in about one-third of patients. Most patients have few symptoms and come to attention because of jaundice or abnormalities on routine blood testing. […] The majority of patients with unresectable bile duct cancer die within 12 months of diagnosis, often from hepatic failure or infectious complications secondary to biliary obstruction. The prognosis has been considered worse for lesions involving the biliary confluence compared with that for distal lesions. […] It has been suggested that the hilar cholangiocarcinoma is a relatively slow-growing, predominantly locally invasive tumor. However, metastatic disease is not uncommon and disease progression can be rapid in some patients.

• #4

  https://link.springer.com/article/10.1007/s11684-010-0130-6

  Hilar cholangiocarcinoma is a slowly growing tumor and tends to spread longitudinally along the bile ducts with neural, perineural, and subepithelial extension. […] Lymph node invasion can be found in 30%50% patients at the time of diagnosis, but blood-born metastases are rare and usually occur at late stages.

• #5 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The early symptoms of hilar cholangiocarcinoma are often non-specific. Abdominal pain or discomfort, anorexia, weight loss and pruritus are the most common but are seen only in about one-third of patients. Most patients have few symptoms and come to attention because of jaundice or abnormalities on routine blood testing. […] The majority of patients with unresectable bile duct cancer die within 12 months of diagnosis, often from hepatic failure or infectious complications secondary to biliary obstruction. The prognosis has been considered worse for lesions involving the biliary confluence compared with that for distal lesions. […] It has been suggested that the hilar cholangiocarcinoma is a relatively slow-growing, predominantly locally invasive tumor. However, metastatic disease is not uncommon and disease progression can be rapid in some patients.

• #6 Hilar cholangiocarcinoma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/hilar-cholangiocarcinoma?content_id=CON-20202299

  Hilar cholangiocarcinoma is the most common type of bile duct cancer, but it is still a rare form of cancer. Common symptoms include yellowing of the skin and the whites of the eyes, weight loss, stomach pain, and itching. […] Hilar cholangiocarcinoma may not cause symptoms at first. Symptoms typically appear when the cancer grows and blocks the bile ducts. Signs and symptoms of hilar cholangiocarcinoma may include: Yellowing of the skin and the whites of the eyes, known as jaundice. Itching. Dark urine. Clay-colored stools. Stomach pain. Fatigue. Weight loss. […] The stages of hilar cholangiocarcinoma range from 1 to 4. A stage 1 hilar cholangiocarcinoma is small and confined to the bile duct. As the cancer gets larger or spreads beyond the area it started in, the stages get higher. A stage 4 hilar cholangiocarcinoma has spread to many nearby lymph nodes or to other parts of the body.

• #7 Klatskin Tumors (Hilar Cholangiocarcinoma)

  https://my.clevelandclinic.org/health/diseases/hilar-cholangiocarcinoma

  Hilar cholangiocarcinoma is a form of extrahepatic bile duct cancer. In this condition, you have cancerous tumors on part of your bile duct, outside of your liver. These tumors are known as Klatskin tumors. […] This condition affects your hilum. Thats the area right outside your liver where your left and right bile ducts come together to form your common bile duct. Cancerous tumors on your bile ducts block the flow of bile. When that happens, you may have symptoms like: Abdominal pain. Fatigue. Nausea. Unexplained weight loss. Yellow skin or yellow whites of your eyes from jaundice. […] Managing symptoms may be challenging, so dont hesitate to ask your healthcare provider for help.

• #8 Symptoms of bile duct cancer | Cancer Research UK

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

  Bile duct cancer may not cause any symptoms in the early stages. If you do have symptoms, they can include: yellowing of the skin and the white of your eyes – this is called jaundice […] The symptoms of bile duct cancer depend on where the cancer is in the bile ducts. The perihilar and distal bile ducts are outside the liver. Cancer that starts in these ducts, or that blocks the drainage of bile from the liver, can cause jaundice. […] Cancers that start here might make you feel generally unwell. But they often dont cause any specific symptoms until they are more advanced. When they do cause symptoms they can include: weight loss, abdominal pain. […] If a cancer blocks the bile ducts it can stop bile flowing into the bowel. The bile goes into your bloodstream and tissues instead. This makes your skin and the white of your eyes turn yellow. […] Unexplained weight loss can be a symptom of bile duct cancer. […] Pain in the abdomen is a common symptom of bile duct cancer. The pain is usually on your right hand side, just below your ribs.

• #9 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The early symptoms of hilar cholangiocarcinoma are often non-specific. Abdominal pain or discomfort, anorexia, weight loss and pruritus are the most common but are seen only in about one-third of patients. Most patients have few symptoms and come to attention because of jaundice or abnormalities on routine blood testing. […] The majority of patients with unresectable bile duct cancer die within 12 months of diagnosis, often from hepatic failure or infectious complications secondary to biliary obstruction. The prognosis has been considered worse for lesions involving the biliary confluence compared with that for distal lesions. […] It has been suggested that the hilar cholangiocarcinoma is a relatively slow-growing, predominantly locally invasive tumor. However, metastatic disease is not uncommon and disease progression can be rapid in some patients.

• #10 Cholangiocarcinoma (bile duct cancer) – Liver Foundation

  https://liver.org.au/your-liver/liver-cancer/cholangiocarcinoma-bile-duct-cancer/

  Most people don’t realise they have cholangiocarcinoma because it doesn’t cause any symptoms at first. […] Sometimes people may notice symptoms including: Chills and/or fever, night sweats; Light-coloured poo; Dark urine; Feeling very tired and weak; Being very itchy (also called pruritus); Losing your appetite; Losing weight without trying to; Feeling nauseous; Pain in the upper right belly that may radiate to the back; Yellow skin and eyes (jaundice). […] Cholangiocarcinoma can be quite difficult to treat successfully because usually it’s already spread by the time people are diagnosed.

• #11 Cholangiocarcinoma (bile duct cancer) – Liver Foundation

  https://liver.org.au/your-liver/liver-cancer/cholangiocarcinoma-bile-duct-cancer/

  Most people don’t realise they have cholangiocarcinoma because it doesn’t cause any symptoms at first. […] Sometimes people may notice symptoms including: Chills and/or fever, night sweats; Light-coloured poo; Dark urine; Feeling very tired and weak; Being very itchy (also called pruritus); Losing your appetite; Losing weight without trying to; Feeling nauseous; Pain in the upper right belly that may radiate to the back; Yellow skin and eyes (jaundice). […] Cholangiocarcinoma can be quite difficult to treat successfully because usually it’s already spread by the time people are diagnosed.

• #12 Klatskin Tumors (Hilar Cholangiocarcinoma)

  https://my.clevelandclinic.org/health/diseases/hilar-cholangiocarcinoma

  Hilar cholangiocarcinoma is a form of extrahepatic bile duct cancer. In this condition, you have cancerous tumors on part of your bile duct, outside of your liver. These tumors are known as Klatskin tumors. […] This condition affects your hilum. Thats the area right outside your liver where your left and right bile ducts come together to form your common bile duct. Cancerous tumors on your bile ducts block the flow of bile. When that happens, you may have symptoms like: Abdominal pain. Fatigue. Nausea. Unexplained weight loss. Yellow skin or yellow whites of your eyes from jaundice. […] Managing symptoms may be challenging, so dont hesitate to ask your healthcare provider for help.

• #13 Cholangiocarcinoma (bile duct cancer) – Liver Foundation

  https://liver.org.au/your-liver/liver-cancer/cholangiocarcinoma-bile-duct-cancer/

  Most people don’t realise they have cholangiocarcinoma because it doesn’t cause any symptoms at first. […] Sometimes people may notice symptoms including: Chills and/or fever, night sweats; Light-coloured poo; Dark urine; Feeling very tired and weak; Being very itchy (also called pruritus); Losing your appetite; Losing weight without trying to; Feeling nauseous; Pain in the upper right belly that may radiate to the back; Yellow skin and eyes (jaundice). […] Cholangiocarcinoma can be quite difficult to treat successfully because usually it’s already spread by the time people are diagnosed.

• #14 Klatskin Tumors (Hilar Cholangiocarcinoma)

  https://my.clevelandclinic.org/health/diseases/hilar-cholangiocarcinoma

  Hilar cholangiocarcinoma is a form of extrahepatic bile duct cancer. In this condition, you have cancerous tumors on part of your bile duct, outside of your liver. These tumors are known as Klatskin tumors. […] This condition affects your hilum. Thats the area right outside your liver where your left and right bile ducts come together to form your common bile duct. Cancerous tumors on your bile ducts block the flow of bile. When that happens, you may have symptoms like: Abdominal pain. Fatigue. Nausea. Unexplained weight loss. Yellow skin or yellow whites of your eyes from jaundice. […] Managing symptoms may be challenging, so dont hesitate to ask your healthcare provider for help.

• #15 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The early symptoms of hilar cholangiocarcinoma are often non-specific. Abdominal pain or discomfort, anorexia, weight loss and pruritus are the most common but are seen only in about one-third of patients. Most patients have few symptoms and come to attention because of jaundice or abnormalities on routine blood testing. […] The majority of patients with unresectable bile duct cancer die within 12 months of diagnosis, often from hepatic failure or infectious complications secondary to biliary obstruction. The prognosis has been considered worse for lesions involving the biliary confluence compared with that for distal lesions. […] It has been suggested that the hilar cholangiocarcinoma is a relatively slow-growing, predominantly locally invasive tumor. However, metastatic disease is not uncommon and disease progression can be rapid in some patients.

• #16 Cholangiocarcinoma Clinical Presentation: History, Physical, Causes

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

  Signs and symptoms of cholangiocarcinoma include the following: Jaundice, Clay-colored stools, Bilirubinuria (dark urine), Pruritus, Weight loss, Abdominal pain. […] Jaundice is the most common manifestation of bile duct cancer and, in general, is best detected in direct sunlight. The obstruction and subsequent cholestasis tend to occur early if the tumor is located in the common bile duct or common hepatic duct. Jaundice often occurs later in perihilar or intrahepatic tumors and is often a marker of advanced disease. The excess of conjugated bilirubin is associated with bilirubinuria and acholic stools. […] Pruritus usually is preceded by jaundice, but itching may be the initial symptom of cholangiocarcinoma. Pruritus may be related to circulating bile acids. […] Weight loss is a variable finding. It may be present in one third of patients at the time of diagnosis. […] Abdominal pain is relatively common in advanced disease. It often is described as a dull ache in the right upper quadrant.

• #17 Hilar cholangiocarcinoma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/hilar-cholangiocarcinoma?content_id=CON-20202299

  Hilar cholangiocarcinoma is the most common type of bile duct cancer, but it is still a rare form of cancer. Common symptoms include yellowing of the skin and the whites of the eyes, weight loss, stomach pain, and itching. […] Hilar cholangiocarcinoma may not cause symptoms at first. Symptoms typically appear when the cancer grows and blocks the bile ducts. Signs and symptoms of hilar cholangiocarcinoma may include: Yellowing of the skin and the whites of the eyes, known as jaundice. Itching. Dark urine. Clay-colored stools. Stomach pain. Fatigue. Weight loss. […] The stages of hilar cholangiocarcinoma range from 1 to 4. A stage 1 hilar cholangiocarcinoma is small and confined to the bile duct. As the cancer gets larger or spreads beyond the area it started in, the stages get higher. A stage 4 hilar cholangiocarcinoma has spread to many nearby lymph nodes or to other parts of the body.

• #18 Cholangiocarcinoma: Symptoms, Causes, Types, Staging & Treatment PACE Hospitals – Best Hospitals in Hitech City, Hyderabad, India | Near Madhapur, Kukatpally, KPHB, Kondapur, Gachibowli, Jubilee Hills, Banjara HillsPACE Hospitals Contact

  https://www.pacehospital.com/cholangiocarcinoma-bile-duct-cancer-symptoms-causes-types-staging-treatment

  Cholangiocarcinoma is often asymptomatic in its early stages, leading to a delayed diagnosis. Symptoms may differ based on the tumor location. […] Symptoms of hilar and distal cholangiocarcinoma are more likely to present with bile duct obstruction. Some of the symptoms include: Painless Jaundice: Jaundice is one of the most common presenting feature. Pruritus: Pruritus occurs due to bile salts accumulation in the skin. Dark Urine and Pale Stools: Disrupted in bilirubin metabolism may result in dark urine and pale stools. Abdominal Pain: Pain in the abdomen is more common in distal cholangiocarcinoma. Cholangitis: In case of any infection, cholangitis is observed which is characterized by fever, chills, and sepsis. Anorexia and Nutritional Deficiencies: Cholestasis and malabsorption lead to nutritional deficiency and anorexia in patients with cholangiocarcinoma.

• #19 Klatskin Tumor | OncoLink

  https://www.oncolink.org/cancers/gastrointestinal/cholangiocarcinoma/support-and-resources/klatskin-tumor

  Symptoms are often caused by the tumor blocking drainage from the bile duct. These include: Jaundice, is the buildup of bilirubin that leads to yellowing of the skin and eyes. […] Jaundice is the most common symptom of cholangiocarcinomas. If you are jaundiced or having nonstop or worsening abdominal pain, then imaging of your abdomen should be done to find out the cause. Lab tests are also done to check the liver function tests (AST, ALT, and total bilirubin).

• #20 Bile Duct Cancer Symptoms | Bile Duct Cancer Signs | American Cancer Society

  https://www.cancer.org/cancer/types/bile-duct-cancer/detection-diagnosis-staging/signs-symptoms.html

  Bile duct cancer doesnt usually cause signs or symptoms until later in the course of the disease, but sometimes symptoms can appear sooner and lead to an early diagnosis. […] When bile duct cancer does cause symptoms, it’s usually because a bile duct is blocked. Symptoms tend to depend on whether the cancer is in ducts inside the liver (intrahepatic) or in ducts outside the liver (extrahepatic). […] Jaundice is the most common symptom of bile duct cancer, but most of the time, jaundice isn’t caused by cancer. […] Most people with bile duct cancer experience some degree of itching. […] If bilirubin cant move into your intestine because it is blocked by the cancer inside the bile ducts, the color of your stool might be lighter. […] Early bile duct cancers seldom cause pain, but bigger tumors may cause belly pain, especially below the ribs on the right side. […] People with bile duct cancer may not feel hungry and may lose weight without trying to do so. […] Some people with bile duct cancer develop fevers. […] Keep in mind: Bile duct cancer is rare. These symptoms are far more likely to be caused by something other than bile duct cancer.

• #21 Cholangiocarcinoma: Symptoms, Causes, Types, Staging & Treatment PACE Hospitals – Best Hospitals in Hitech City, Hyderabad, India | Near Madhapur, Kukatpally, KPHB, Kondapur, Gachibowli, Jubilee Hills, Banjara HillsPACE Hospitals Contact

  https://www.pacehospital.com/cholangiocarcinoma-bile-duct-cancer-symptoms-causes-types-staging-treatment

  Cholangiocarcinoma is often asymptomatic in its early stages, leading to a delayed diagnosis. Symptoms may differ based on the tumor location. […] Symptoms of hilar and distal cholangiocarcinoma are more likely to present with bile duct obstruction. Some of the symptoms include: Painless Jaundice: Jaundice is one of the most common presenting feature. Pruritus: Pruritus occurs due to bile salts accumulation in the skin. Dark Urine and Pale Stools: Disrupted in bilirubin metabolism may result in dark urine and pale stools. Abdominal Pain: Pain in the abdomen is more common in distal cholangiocarcinoma. Cholangitis: In case of any infection, cholangitis is observed which is characterized by fever, chills, and sepsis. Anorexia and Nutritional Deficiencies: Cholestasis and malabsorption lead to nutritional deficiency and anorexia in patients with cholangiocarcinoma.

• #22 Cholangiocarcinoma- Symptoms, Types, Causes, & More

  https://www.impactguru.com/info/cholangiocarcinoma/

  Symptoms of cholangiocarcinoma can differ depending on the location and size of the tumor. Common symptoms may include jaundice, characterized by yellowing of the skin eyes due to the accumulation of bile pigments, itching, abdominal pain or discomfort, unexplained weight loss, fever, and fatigue. […] Jaundice may be accompanied by intense itching, also known as pruritus. This occurs due to the accumulation of bile salts in the skin, which irritate nerve endings and cause itching. […] Cholangiocarcinoma can cause pain in the abdomen, mainly in the upper right quadrant, where the liver is located. The pain can vary from a persistent dull ache to an intermittent sharp sensation, depending on the size and location of the tumor. […] Losing weight is a common indication of several cancers, including cholangiocarcinoma. As the cancer progresses, it can cause a loss of appetite and unintended weight loss.

• #23 Hilar cholangiocarcinoma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/hilar-cholangiocarcinoma?content_id=CON-20202299

  Hilar cholangiocarcinoma is the most common type of bile duct cancer, but it is still a rare form of cancer. Common symptoms include yellowing of the skin and the whites of the eyes, weight loss, stomach pain, and itching. […] Hilar cholangiocarcinoma may not cause symptoms at first. Symptoms typically appear when the cancer grows and blocks the bile ducts. Signs and symptoms of hilar cholangiocarcinoma may include: Yellowing of the skin and the whites of the eyes, known as jaundice. Itching. Dark urine. Clay-colored stools. Stomach pain. Fatigue. Weight loss. […] The stages of hilar cholangiocarcinoma range from 1 to 4. A stage 1 hilar cholangiocarcinoma is small and confined to the bile duct. As the cancer gets larger or spreads beyond the area it started in, the stages get higher. A stage 4 hilar cholangiocarcinoma has spread to many nearby lymph nodes or to other parts of the body.

• #24

  https://www.parkwayshenton.com.sg/conditions-diseases/bile-duct-cancer/symptoms-causes

  Symptoms of bile duct cancer include: Abdominal pain on the right, just below the ribs […] Dark urine […] Fatigue […] Fever […] Itchy skin […] Jaundice […] Pale or light-coloured stools […] Nausea and vomiting […] Unexplained weight loss. […] In some patients, the resulting tumour from bile duct cancer can obstruct the bile duct and cause infection of the bile drainage system (cholangitis). […] Bile duct cancer may also lead to cirrhosis, causing scarring and destruction of liver cells, especially among patients with primary sclerosing cholangitis.

• #25 Cholangiocarcinoma: Symptoms, Causes, Types, Staging & Treatment PACE Hospitals – Best Hospitals in Hitech City, Hyderabad, India | Near Madhapur, Kukatpally, KPHB, Kondapur, Gachibowli, Jubilee Hills, Banjara HillsPACE Hospitals Contact

  https://www.pacehospital.com/cholangiocarcinoma-bile-duct-cancer-symptoms-causes-types-staging-treatment

  Cholangiocarcinoma is often asymptomatic in its early stages, leading to a delayed diagnosis. Symptoms may differ based on the tumor location. […] Symptoms of hilar and distal cholangiocarcinoma are more likely to present with bile duct obstruction. Some of the symptoms include: Painless Jaundice: Jaundice is one of the most common presenting feature. Pruritus: Pruritus occurs due to bile salts accumulation in the skin. Dark Urine and Pale Stools: Disrupted in bilirubin metabolism may result in dark urine and pale stools. Abdominal Pain: Pain in the abdomen is more common in distal cholangiocarcinoma. Cholangitis: In case of any infection, cholangitis is observed which is characterized by fever, chills, and sepsis. Anorexia and Nutritional Deficiencies: Cholestasis and malabsorption lead to nutritional deficiency and anorexia in patients with cholangiocarcinoma.

• #26 Cholangiocarcinoma (bile duct cancer) – Liver Foundation

  https://liver.org.au/your-liver/liver-cancer/cholangiocarcinoma-bile-duct-cancer/

  Most people don’t realise they have cholangiocarcinoma because it doesn’t cause any symptoms at first. […] Sometimes people may notice symptoms including: Chills and/or fever, night sweats; Light-coloured poo; Dark urine; Feeling very tired and weak; Being very itchy (also called pruritus); Losing your appetite; Losing weight without trying to; Feeling nauseous; Pain in the upper right belly that may radiate to the back; Yellow skin and eyes (jaundice). […] Cholangiocarcinoma can be quite difficult to treat successfully because usually it’s already spread by the time people are diagnosed.

• #27 Cholangiocarcinoma (Bile Duct Cancer)

  https://my.clevelandclinic.org/health/diseases/21524-cholangiocarcinoma

  Cholangiocarcinoma symptoms dont usually start until the cancer advances and blocks a bile duct. Symptoms of bile duct cancer include: Abdominal pain. Fever. Fatigue. Itchy skin. Jaundice (skin and whites of eyes turn yellow). Dark urine. Light-colored or greasy stools. Nausea and vomiting. Unexplained weight loss. […] Cholangiocarcinoma is an aggressive cancer, which means it spreads fast. Most people receive a cholangiocarcinoma diagnosis after its already spread outside of their bile ducts. At this point, bile duct cancer is difficult to treat, and the prognosis (chance of recovery) is usually poor. […] The outlook (prognosis) for people with cholangiocarcinoma is usually poor. The five-year survival rate for cholangiocarcinoma that hasnt spread outside of the bile ducts ranges from 18% to 23%. That number drops to 2% to 3% for cancer thats spread beyond bile ducts.

• #28 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/3374/html

  Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6th decade of life. Patients typically present with abdominal pain, pruritis, weight loss, and jaundice. HC generally presents in the 6th decade of life. Patients commonly present with jaundice, abdominal pain and weight loss. Fatigue, pruritus, nausea, dark urine and clay colored stools are also often seen. Over 80% of proximal biliary obstructions are secondary to HC. The remaining 15-20% are caused by benign strictures secondary to inflammatory disease, sclerosing cholangitis, stone disease, and gallbladder cancer invading the hepatoduodenal ligament. Both benign and malignant biliary strictures present with similar clinical features. Moreover, bilirubin and serum tumor marker levels do not reliably distinguish malignant and benign biliary strictures. Less than one half of HC are resectable. Accurate radiological staging of these lesions is difficult secondary to the complexity of the hilar region, proximity to major vessels, and small tumor sizes. Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. Five-year survival after surgical resection of HC ranges from 10% to 40% however, recurrence can be as high as 50-70% even after R0 resection. Lymph node invasion, tumor grade and negative margins are important prognostic indicators.

• #29 Cholangiocarcinoma – Clinical Features – Investigations – TeachMeSurgery

  https://teachmesurgery.com/hpb/gall-bladder/cholangiocarcinoma/

  The most common site for bile duct cancers is at the bifurcation of the right and left hepatic ducts, termed Klatskin tumours or hilar cholangiocarcinoma, and account for 60% of cholangiocarcinoma. […] These tumours generally invade local structures, such as the portal vein and hepatic arteries, and metastasise to local lymph nodes, before spreading to the peritoneal cavity, lung, and liver. […] Intrahepatic cholangiocarcinoma is generally asymptomatic until a late stage in the disease, whereas extrahepatic cholangiocarcinoma will typically present early. […] Patients will present with jaundice, along with associated pruritus, steatorrhoea, non-specific abdominal pain, or dark urine. In advanced disease, weight loss and lethargy can occur. […] On examination, jaundice and cachexia are often evident.

• #30 Cholangiocarcinoma (Bile duct cancer) | Doctor

  https://patient.info/doctor/cholangiocarcinoma

  Most patients develop symptoms only once the disease had advanced. […] Intrahepatic cholangiocarcinomas present with non-specific symptoms such as abdominal pain, night sweats, and cachexia. […] Jaundice is an early feature in perihilar tumours, sometimes with hepatomegaly. […] Fever is more common in perihilar tumours. […] Abdominal pain, localised to the right upper quadrant, especially in advanced disease. […] Weight loss is variable. […] Pale-coloured stools, passage of dark urine, upper gastrointestinal pain (dull ache in the upper right quadrant), weight loss, anorexia and general malaise are common features. […] Pruritus may be the presenting symptom predating jaundice on occasions. […] The presence of a palpable gallbladder (Courvoisier’s sign) may occur with tumours distal to the cystic duct.

• #31 Cholangiocarcinoma (bile duct cancer) – Liver Foundation

  https://liver.org.au/your-liver/liver-cancer/cholangiocarcinoma-bile-duct-cancer/

  Most people don’t realise they have cholangiocarcinoma because it doesn’t cause any symptoms at first. […] Sometimes people may notice symptoms including: Chills and/or fever, night sweats; Light-coloured poo; Dark urine; Feeling very tired and weak; Being very itchy (also called pruritus); Losing your appetite; Losing weight without trying to; Feeling nauseous; Pain in the upper right belly that may radiate to the back; Yellow skin and eyes (jaundice). […] Cholangiocarcinoma can be quite difficult to treat successfully because usually it’s already spread by the time people are diagnosed.

• #32 Controlling symptoms of advanced bile duct cancer | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/bile-duct-cancer/treatment/controlling-symptoms

  Feeling tired is the most common symptom of advanced cancer. Other symptoms depend on where in the body the cancer has spread. […] Unfortunately, most people have advanced bile duct cancer when they are diagnosed. This is because bile duct cancer usually doesn’t cause symptoms early on. When the cancer is advanced you may: have yellowing of your skin and whites of your eyes (jaundice) – this can also make your skin itchy, have abdominal (tummy) pain, feel or be sick, lose your appetite and lose weight. […] Advanced bile duct cancer might give you pain in your abdomen. Let your doctor or nurse know if you have pain so they can give you painkillers. You should also tell them how well the painkillers are working. […] Sickness when you have advanced bile duct cancer can be caused by lots of different things. This includes the cancer itself and some painkillers. A blocked bile duct can also cause sickness. […] You might not feel like eating and may lose weight. If you find you have a loss of appetite, some of the following suggestions might help.

• #33 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The physical findings are often nonspecific but may provide some useful information. Jaundice will usually be obvious. Patients with pruritus often have multiple excoriations of the skin. The liver may be enlarged and firm as a result of biliary tract obstruction. […] Lobar atrophy is not uncommon in patients with hilar cholangiocarcinoma, and its importance cannot be overemphasized, since it often influences therapy. Long-standing biliary obstruction alone may cause moderate atrophy, whereas concomitant portal venous compromise induces rapid and severe atrophy of the involved segments.

• #34 Understanding Hilar Cholangiocarcinoma: Causes, Symptoms, Diagnosis, and Treatment

  https://www.doctorshubnepal.com/diseases-conditions/hilar-cholangiocarcinoma

  Hilar cholangiocarcinoma, commonly referred to as Klatskin tumors, is a rare and aggressive form of bile duct cancer. […] The progression of hilar cholangiocarcinoma is often insidious, and symptoms might not become apparent until the disease has reached an advanced stage. […] The symptoms of hilar cholangiocarcinoma are often vague and can overlap with other conditions. Key symptoms include: 1. Jaundice: Yellowing of the skin and eyes due to bile duct obstruction. 2. Abdominal Pain: Persistent pain in the upper right side of the abdomen. 3. Weight Loss: Unexplained weight loss despite normal eating habits. 4. Pruritus: Severe itching caused by bile salt accumulation. 5. Dark Urine and Pale Stools: Changes in urine and stool color due to bile duct blockage. 6. Fever: Associated with infection or inflammation. 7. Nausea and vomiting: Often linked to bile duct obstruction and digestive issues. 8. Enlarged Liver: Detected during physical examinations or imaging tests. 9. Fatigue: Persistent tiredness that affects daily activities.

• #35 Cholangiocarcinoma – Clinical Features – Investigations – TeachMeSurgery

  https://teachmesurgery.com/hpb/gall-bladder/cholangiocarcinoma/

  The most common site for bile duct cancers is at the bifurcation of the right and left hepatic ducts, termed Klatskin tumours or hilar cholangiocarcinoma, and account for 60% of cholangiocarcinoma. […] These tumours generally invade local structures, such as the portal vein and hepatic arteries, and metastasise to local lymph nodes, before spreading to the peritoneal cavity, lung, and liver. […] Intrahepatic cholangiocarcinoma is generally asymptomatic until a late stage in the disease, whereas extrahepatic cholangiocarcinoma will typically present early. […] Patients will present with jaundice, along with associated pruritus, steatorrhoea, non-specific abdominal pain, or dark urine. In advanced disease, weight loss and lethargy can occur. […] On examination, jaundice and cachexia are often evident.

• #36 Cholangiocarcinoma (bile duct cancer) – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/cholangiocarcinoma/symptoms-causes/syc-20352408

  Cholangiocarcinoma is often diagnosed when it’s advanced, making successful treatment difficult to achieve. […] Signs and symptoms of cholangiocarcinoma include: Yellowing of your skin and the whites of your eyes (jaundice), Intensely itchy skin, White-colored stools, Fatigue, Abdominal pain on the right side, just below the ribs, Losing weight without trying, Fever, Night sweats, Dark urine. […] See your doctor if you have persistent fatigue, abdominal pain, jaundice, or other signs and symptoms that bother you.

• #37 Cholangiocarcinoma (bile duct cancer) – Liver Foundation

  https://liver.org.au/your-liver/liver-cancer/cholangiocarcinoma-bile-duct-cancer/

  Most people don’t realise they have cholangiocarcinoma because it doesn’t cause any symptoms at first. […] Sometimes people may notice symptoms including: Chills and/or fever, night sweats; Light-coloured poo; Dark urine; Feeling very tired and weak; Being very itchy (also called pruritus); Losing your appetite; Losing weight without trying to; Feeling nauseous; Pain in the upper right belly that may radiate to the back; Yellow skin and eyes (jaundice). […] Cholangiocarcinoma can be quite difficult to treat successfully because usually it’s already spread by the time people are diagnosed.

• #38 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The early symptoms of hilar cholangiocarcinoma are often non-specific. Abdominal pain or discomfort, anorexia, weight loss and pruritus are the most common but are seen only in about one-third of patients. Most patients have few symptoms and come to attention because of jaundice or abnormalities on routine blood testing. […] The majority of patients with unresectable bile duct cancer die within 12 months of diagnosis, often from hepatic failure or infectious complications secondary to biliary obstruction. The prognosis has been considered worse for lesions involving the biliary confluence compared with that for distal lesions. […] It has been suggested that the hilar cholangiocarcinoma is a relatively slow-growing, predominantly locally invasive tumor. However, metastatic disease is not uncommon and disease progression can be rapid in some patients.

• #39

  https://link.springer.com/article/10.1007/s11684-010-0130-6

  Hilar cholangiocarcinoma is a slowly growing tumor and tends to spread longitudinally along the bile ducts with neural, perineural, and subepithelial extension. […] Lymph node invasion can be found in 30%50% patients at the time of diagnosis, but blood-born metastases are rare and usually occur at late stages.

• #40

  https://link.springer.com/article/10.1007/s11684-010-0130-6

  Hilar cholangiocarcinoma is a slowly growing tumor and tends to spread longitudinally along the bile ducts with neural, perineural, and subepithelial extension. […] Lymph node invasion can be found in 30%50% patients at the time of diagnosis, but blood-born metastases are rare and usually occur at late stages.

• #41 Cholangiocarcinoma – Clinical Features – Investigations – TeachMeSurgery

  https://teachmesurgery.com/hpb/gall-bladder/cholangiocarcinoma/

  The most common site for bile duct cancers is at the bifurcation of the right and left hepatic ducts, termed Klatskin tumours or hilar cholangiocarcinoma, and account for 60% of cholangiocarcinoma. […] These tumours generally invade local structures, such as the portal vein and hepatic arteries, and metastasise to local lymph nodes, before spreading to the peritoneal cavity, lung, and liver. […] Intrahepatic cholangiocarcinoma is generally asymptomatic until a late stage in the disease, whereas extrahepatic cholangiocarcinoma will typically present early. […] Patients will present with jaundice, along with associated pruritus, steatorrhoea, non-specific abdominal pain, or dark urine. In advanced disease, weight loss and lethargy can occur. […] On examination, jaundice and cachexia are often evident.

• #42

  https://link.springer.com/article/10.1007/s11684-010-0130-6

  Hilar cholangiocarcinoma is a slowly growing tumor and tends to spread longitudinally along the bile ducts with neural, perineural, and subepithelial extension. […] Lymph node invasion can be found in 30%50% patients at the time of diagnosis, but blood-born metastases are rare and usually occur at late stages.

• #43 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/3374/html

  Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6th decade of life. Patients typically present with abdominal pain, pruritis, weight loss, and jaundice. HC generally presents in the 6th decade of life. Patients commonly present with jaundice, abdominal pain and weight loss. Fatigue, pruritus, nausea, dark urine and clay colored stools are also often seen. Over 80% of proximal biliary obstructions are secondary to HC. The remaining 15-20% are caused by benign strictures secondary to inflammatory disease, sclerosing cholangitis, stone disease, and gallbladder cancer invading the hepatoduodenal ligament. Both benign and malignant biliary strictures present with similar clinical features. Moreover, bilirubin and serum tumor marker levels do not reliably distinguish malignant and benign biliary strictures. Less than one half of HC are resectable. Accurate radiological staging of these lesions is difficult secondary to the complexity of the hilar region, proximity to major vessels, and small tumor sizes. Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. Five-year survival after surgical resection of HC ranges from 10% to 40% however, recurrence can be as high as 50-70% even after R0 resection. Lymph node invasion, tumor grade and negative margins are important prognostic indicators.

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

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

  Cholangiocarcinomas correspond to ~15% of all primary liver tumors and to ~3% of all gastrointestinal malignancies. They usually present in the elderly, with a mean age of 65 years. Typically, the presentation is with painless jaundice. The most important factor in prognosis is whether or not the tumor can be resected. Unfortunately, when discovered, most cases are too advanced for curative resection. Even with resection, the prognosis is poor, with a 5-year survival of only 10-44%, with the prognosis favoring extrahepatic tumors (around 30% 5-year survival vs 15% for intrahepatic tumors). […] The pattern of metastatic spread includes intrahepatic vascular involvement with numerous local metastases, regional lymph nodes (50% at autopsy), and hematogenous (50% at autopsy) to lungs, bones, especially vertebrae, adrenal glands, and brain.

• #45 How Aggressive Is Bile Duct Cancer?

  https://www.healthline.com/health/cancer/how-aggressive-is-bile-duct-cancer

  Doctors group bile duct cancer into five major stages (0 to 4) based on the size of the tumor and how much the cancer has spread. […] Bile duct cancer usually first spreads to a nearby blood vessel. It then spreads to nearby organs or tissues such as your liver, intestines, stomach, diaphragm, lymph nodes. […] In later stages, bile duct cancer can spread to more distant parts of the body, such as your lung (most common), abdominal lining, bones.

• #46 Hilar cholangiocarcinoma | Beacon Health System

  https://www.beaconhealthsystem.org/library/diseases-and-conditions/hilar-cholangiocarcinoma?content_id=CON-20202299

  Hilar cholangiocarcinoma is the most common type of bile duct cancer, but it is still a rare form of cancer. Common symptoms include yellowing of the skin and the whites of the eyes, weight loss, stomach pain, and itching. […] Hilar cholangiocarcinoma may not cause symptoms at first. Symptoms typically appear when the cancer grows and blocks the bile ducts. Signs and symptoms of hilar cholangiocarcinoma may include: Yellowing of the skin and the whites of the eyes, known as jaundice. Itching. Dark urine. Clay-colored stools. Stomach pain. Fatigue. Weight loss. […] The stages of hilar cholangiocarcinoma range from 1 to 4. A stage 1 hilar cholangiocarcinoma is small and confined to the bile duct. As the cancer gets larger or spreads beyond the area it started in, the stages get higher. A stage 4 hilar cholangiocarcinoma has spread to many nearby lymph nodes or to other parts of the body.

• #47 Cholangiocarcinoma Stage 4 – Metastatic Cholangiocarcinoma

  https://massivebio.com/cholangiocarcinoma-stage-4/

  Cholangiocarcinoma symptoms include abdominal pain, jaundice, weak stool, and discoloration in the urine. […] If cholangiocarcinoma is suspected in an ultrasound, tomography or MRI, the diagnosis must be confirmed by performing a needle biopsy under the guidance of an ultrasound or tomography. […] Metastatic Cholangiocarcinoma (Stage IV) is an advanced stage of cancer and is examined in two groups: The first group is when the tumor has spread to the main blood vessel and lymph nodes. The second group is when the tumor has spread to the lymph nodes away from the tumor or has spread to other distant parts of the body.

• #48 Stage 4 bile duct cancer: Symptoms, treatment, and outlook

  https://www.medicalnewstoday.com/articles/bile-duct-cancer-stage-4

  Stage 4 bile duct cancer is the most advanced stage of this disease. Treatment involves managing symptoms and improving quality of life. […] A diagnosis of bile duct cancer is more likely when the disease is in the later stages and when the tumor starts to cause symptoms. In the early stages, people often have no symptoms, while stage 4 is the most advanced stage of bile duct cancer. At this stage, the tumor has metastasized, or spread, to other parts of the body. […] The signs and symptoms of bile duct cancer are due to the disruption of typical bile passage through the bile ducts. The body needs bile to digest fat properly. […] Another major symptom of bile duct cancer is jaundice. When bile cannot enter the digestive system, it stays in the body, causing bilirubin levels to build up and leading to jaundice. Jaundice causes the skin and whites of the eyes to develop a yellowish tinge.

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

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

  Given the complexity of diagnosis and staging, each case of suspected hCCA should be discussed in a multidisciplinary team (MDT) meeting. […] Surgical resection is the only potentially curative treatment option, with reported 5-year survival from 25%-40% in patients undergoing R0 resection. […] The choice of resection in Bismuth-Corlette III and selected cases of Bismuth-Corlette IV is dictated by the extent of biliary involvement, lobar atrophy, vascular involvement, side of biliary dominance and hilar anatomical variations. […] While surgical resection remains the primary treatment for hCCA, a significant majority present with irresectable disease due to extensive biliary and vascular involvement at the hepatic hilus and underlying parenchymal liver disease such as PSC. […] After complete surgical resection, almost 60% of patients in the high-risk group develop local recurrence. […] The main aim of palliative treatment is to improve the quality of life by minimizing the number of hospitalizations.

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

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

  Given the complexity of diagnosis and staging, each case of suspected hCCA should be discussed in a multidisciplinary team (MDT) meeting. […] Surgical resection is the only potentially curative treatment option, with reported 5-year survival from 25%-40% in patients undergoing R0 resection. […] The choice of resection in Bismuth-Corlette III and selected cases of Bismuth-Corlette IV is dictated by the extent of biliary involvement, lobar atrophy, vascular involvement, side of biliary dominance and hilar anatomical variations. […] While surgical resection remains the primary treatment for hCCA, a significant majority present with irresectable disease due to extensive biliary and vascular involvement at the hepatic hilus and underlying parenchymal liver disease such as PSC. […] After complete surgical resection, almost 60% of patients in the high-risk group develop local recurrence. […] The main aim of palliative treatment is to improve the quality of life by minimizing the number of hospitalizations.

• #51 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The early symptoms of hilar cholangiocarcinoma are often non-specific. Abdominal pain or discomfort, anorexia, weight loss and pruritus are the most common but are seen only in about one-third of patients. Most patients have few symptoms and come to attention because of jaundice or abnormalities on routine blood testing. […] The majority of patients with unresectable bile duct cancer die within 12 months of diagnosis, often from hepatic failure or infectious complications secondary to biliary obstruction. The prognosis has been considered worse for lesions involving the biliary confluence compared with that for distal lesions. […] It has been suggested that the hilar cholangiocarcinoma is a relatively slow-growing, predominantly locally invasive tumor. However, metastatic disease is not uncommon and disease progression can be rapid in some patients.

• #52 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. Palliative treatment is particularly indicated in the presence of cholangitis and pruritus but is often also offered for high-grade jaundice and abdominal pain. […] 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. […] Not every patient with unresectable HC needs palliative intervention. Patients with complications of cholangitis and intractable pruritus are definite candidates for palliation. Palliation is also often performed in patients with abdominal pain and high bilirubin with the hope of ameliorating their pain and sense of well-being, respectively.

• #53 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The physical findings are often nonspecific but may provide some useful information. Jaundice will usually be obvious. Patients with pruritus often have multiple excoriations of the skin. The liver may be enlarged and firm as a result of biliary tract obstruction. […] Lobar atrophy is not uncommon in patients with hilar cholangiocarcinoma, and its importance cannot be overemphasized, since it often influences therapy. Long-standing biliary obstruction alone may cause moderate atrophy, whereas concomitant portal venous compromise induces rapid and severe atrophy of the involved segments.

• #54 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The early symptoms of hilar cholangiocarcinoma are often non-specific. Abdominal pain or discomfort, anorexia, weight loss and pruritus are the most common but are seen only in about one-third of patients. Most patients have few symptoms and come to attention because of jaundice or abnormalities on routine blood testing. […] The majority of patients with unresectable bile duct cancer die within 12 months of diagnosis, often from hepatic failure or infectious complications secondary to biliary obstruction. The prognosis has been considered worse for lesions involving the biliary confluence compared with that for distal lesions. […] It has been suggested that the hilar cholangiocarcinoma is a relatively slow-growing, predominantly locally invasive tumor. However, metastatic disease is not uncommon and disease progression can be rapid in some patients.

• #55 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. Palliative treatment is particularly indicated in the presence of cholangitis and pruritus but is often also offered for high-grade jaundice and abdominal pain. […] 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. […] Not every patient with unresectable HC needs palliative intervention. Patients with complications of cholangitis and intractable pruritus are definite candidates for palliation. Palliation is also often performed in patients with abdominal pain and high bilirubin with the hope of ameliorating their pain and sense of well-being, respectively.

• #56 Cholangiocarcinoma (Bile Duct Cancer)

  https://my.clevelandclinic.org/health/diseases/21524-cholangiocarcinoma

  Cholangiocarcinoma symptoms dont usually start until the cancer advances and blocks a bile duct. Symptoms of bile duct cancer include: Abdominal pain. Fever. Fatigue. Itchy skin. Jaundice (skin and whites of eyes turn yellow). Dark urine. Light-colored or greasy stools. Nausea and vomiting. Unexplained weight loss. […] Cholangiocarcinoma is an aggressive cancer, which means it spreads fast. Most people receive a cholangiocarcinoma diagnosis after its already spread outside of their bile ducts. At this point, bile duct cancer is difficult to treat, and the prognosis (chance of recovery) is usually poor. […] The outlook (prognosis) for people with cholangiocarcinoma is usually poor. The five-year survival rate for cholangiocarcinoma that hasnt spread outside of the bile ducts ranges from 18% to 23%. That number drops to 2% to 3% for cancer thats spread beyond bile ducts.

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

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

  Cholangiocarcinoma (CCA) constitutes a diverse group of malignancies emerging in the biliary tree. 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. […] The most frequent symptom of pCCA and dCCA is jaundice due to biliary tract obstruction. In iCCA, jaundice is less frequent and mostly associated with advanced disease. Other symptoms of advanced disease include asthenia, abdominal pain, malaise, nausea, anorexia and weight loss. iCCA is an incidental finding in around 20-25% of cases. […] The current diagnosis of CCA by non-invasive approaches is not accurate enough, and histological confirmation is necessary. […] Despite advances in CCA awareness, knowledge, diagnosis and therapies, patient prognosis has not improved substantially in the past decade, with 5-year survival (7-20%) and tumour recurrence rates after resection still disappointing.

• #58 Cholangiocarcinoma – StatPearls – NCBI Bookshelf

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

  Cholangiocarcinoma is an aggressive malignancy that originates from the biliary epithelium, both inside and outside the liver. This neoplasm is difficult to diagnose because obtaining a tissue sample for confirmation is not always possible. Unfortunately, cholangiocarcinoma is often locally invasive or metastatic by the time it is detected. The clinical presentation of cholangiocarcinoma will vary with the location and size of the tumor. Diagnosing cholangiocarcinoma can be difficult, particularly for extrahepatic lesions; available biopsy techniques lack diagnostic sensitivity. All patients with suspected or confirmed cholangiocarcinoma should be evaluated for distant metastatic disease; almost 75% of patients have nonresectable or metastatic disease at presentation. The overall prognosis of cholangiocarcinoma is poor, given the aggressive nature of the tumor and the usually advanced stage at presentation. Intrahepatic cholangiocarcinoma typically presents with nonspecific symptoms, including abdominal pain, weight loss, and fatigue. Jaundice and cholangitis may occur in the presence of biliary obstruction. Frequently, symptoms occur late in the disease course; tumors can be large at the time of diagnosis. Extrahepatic cholangiocarcinomas are symptomatic earlier in their clinical course due to biliary obstruction, leading to jaundice, pruritus, clay-colored stools, and dark-colored urine. There may be a palpable mass and ascites in advanced disease. The clinical presentation of cholangiocarcinoma will depend on the location and size of the tumor.

• #59 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. Palliative treatment is particularly indicated in the presence of cholangitis and pruritus but is often also offered for high-grade jaundice and abdominal pain. […] 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. […] Not every patient with unresectable HC needs palliative intervention. Patients with complications of cholangitis and intractable pruritus are definite candidates for palliation. Palliation is also often performed in patients with abdominal pain and high bilirubin with the hope of ameliorating their pain and sense of well-being, respectively.

• #60 SSA – POMS: DI 23022.704 – Cholangiocarcinoma – 08/09/2023

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022704

  The prognosis for people with cholangiocarcinoma is usually poor. The five-year survival rate for bile duct cancer that hasn’t spread outside of the bile ducts is 10% to 15%. This rate drops to 2% if the cancer spreads to areas of the body that are far from the bile ducts, such as the lungs. However, newer treatments mean these rates will improve over time.

• #61 Cholangiocarcinoma (Bile Duct Cancer)

  https://my.clevelandclinic.org/health/diseases/21524-cholangiocarcinoma

  Cholangiocarcinoma symptoms dont usually start until the cancer advances and blocks a bile duct. Symptoms of bile duct cancer include: Abdominal pain. Fever. Fatigue. Itchy skin. Jaundice (skin and whites of eyes turn yellow). Dark urine. Light-colored or greasy stools. Nausea and vomiting. Unexplained weight loss. […] Cholangiocarcinoma is an aggressive cancer, which means it spreads fast. Most people receive a cholangiocarcinoma diagnosis after its already spread outside of their bile ducts. At this point, bile duct cancer is difficult to treat, and the prognosis (chance of recovery) is usually poor. […] The outlook (prognosis) for people with cholangiocarcinoma is usually poor. The five-year survival rate for cholangiocarcinoma that hasnt spread outside of the bile ducts ranges from 18% to 23%. That number drops to 2% to 3% for cancer thats spread beyond bile ducts.

• #62 SSA – POMS: DI 23022.704 – Cholangiocarcinoma – 08/09/2023

  https://secure.ssa.gov/apps10/poms.nsf/lnx/0423022704

  The prognosis for people with cholangiocarcinoma is usually poor. The five-year survival rate for bile duct cancer that hasn’t spread outside of the bile ducts is 10% to 15%. This rate drops to 2% if the cancer spreads to areas of the body that are far from the bile ducts, such as the lungs. However, newer treatments mean these rates will improve over time.

• #63 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/3374/html

  Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6th decade of life. Patients typically present with abdominal pain, pruritis, weight loss, and jaundice. HC generally presents in the 6th decade of life. Patients commonly present with jaundice, abdominal pain and weight loss. Fatigue, pruritus, nausea, dark urine and clay colored stools are also often seen. Over 80% of proximal biliary obstructions are secondary to HC. The remaining 15-20% are caused by benign strictures secondary to inflammatory disease, sclerosing cholangitis, stone disease, and gallbladder cancer invading the hepatoduodenal ligament. Both benign and malignant biliary strictures present with similar clinical features. Moreover, bilirubin and serum tumor marker levels do not reliably distinguish malignant and benign biliary strictures. Less than one half of HC are resectable. Accurate radiological staging of these lesions is difficult secondary to the complexity of the hilar region, proximity to major vessels, and small tumor sizes. Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. Five-year survival after surgical resection of HC ranges from 10% to 40% however, recurrence can be as high as 50-70% even after R0 resection. Lymph node invasion, tumor grade and negative margins are important prognostic indicators.

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

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

  Patients commonly present with cachexia, fatigue, and/or painless jaundice secondary to biliary duct obstruction. Unfortunately, painless jaundice at presentation often represents locally advanced or metastatic disease. About 10% of patients will have concomitant cholangitis at presentation. […] Unfortunately, most patients present with locally advanced or metastatic disease and must rely on systemic therapy as their primary treatment. However, even with current systemic therapy, survival is still poor. […] More than one-half of patients with HC already have locally advanced or metastatic disease at the time of diagnosis. Systemic therapy options for these patients are limited and do little to improve long-term survival. […] Unfortunately, given the aggressive nature of HC, 50–70% of patients will develop recurrence after surgery.

• #65 Cholangiocarcinoma – Clinical Features – Investigations – TeachMeSurgery

  https://teachmesurgery.com/hpb/gall-bladder/cholangiocarcinoma/

  Long term survival is generally poor and treatment should be aimed at good symptom relief and palliative management. The average survival is 12-18 months from diagnosis, as most patients have unresectable disease at the time of presentation. […] In patients undergoing aggressive surgery, 5 year survival rates are 10-40%. Survival appears to be better for distal tumours, at up to 54% at 5 years.

• #66 Cholangiocarcinoma (Bile duct cancer) | Doctor

  https://patient.info/doctor/cholangiocarcinoma

  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.

• #67 Hilar cholangiocarcinoma: diagnosis, treatment options, and management – Soares – Hepatobiliary Surgery and Nutrition

  https://hbsn.amegroups.org/article/view/3374/html

  Hilar cholangiocarcinoma (HC) is a rare disease with a poor prognosis which typically presents in the 6th decade of life. Patients typically present with abdominal pain, pruritis, weight loss, and jaundice. HC generally presents in the 6th decade of life. Patients commonly present with jaundice, abdominal pain and weight loss. Fatigue, pruritus, nausea, dark urine and clay colored stools are also often seen. Over 80% of proximal biliary obstructions are secondary to HC. The remaining 15-20% are caused by benign strictures secondary to inflammatory disease, sclerosing cholangitis, stone disease, and gallbladder cancer invading the hepatoduodenal ligament. Both benign and malignant biliary strictures present with similar clinical features. Moreover, bilirubin and serum tumor marker levels do not reliably distinguish malignant and benign biliary strictures. Less than one half of HC are resectable. Accurate radiological staging of these lesions is difficult secondary to the complexity of the hilar region, proximity to major vessels, and small tumor sizes. Despite the limited data, chemotherapy is indicated for patients with unresectable tumors and adequate functional status. Five-year survival after surgical resection of HC ranges from 10% to 40% however, recurrence can be as high as 50-70% even after R0 resection. Lymph node invasion, tumor grade and negative margins are important prognostic indicators.

• #68 Hilar cholangiocarcinoma: diagnosis and staging

  https://pmc.ncbi.nlm.nih.gov/articles/PMC2043098/

  The early symptoms of hilar cholangiocarcinoma are often non-specific. Abdominal pain or discomfort, anorexia, weight loss and pruritus are the most common but are seen only in about one-third of patients. Most patients have few symptoms and come to attention because of jaundice or abnormalities on routine blood testing. […] The majority of patients with unresectable bile duct cancer die within 12 months of diagnosis, often from hepatic failure or infectious complications secondary to biliary obstruction. The prognosis has been considered worse for lesions involving the biliary confluence compared with that for distal lesions. […] It has been suggested that the hilar cholangiocarcinoma is a relatively slow-growing, predominantly locally invasive tumor. However, metastatic disease is not uncommon and disease progression can be rapid in some patients.

• #69 Cholangiocarcinoma – Clinical Features – Investigations – TeachMeSurgery

  https://teachmesurgery.com/hpb/gall-bladder/cholangiocarcinoma/

  Long term survival is generally poor and treatment should be aimed at good symptom relief and palliative management. The average survival is 12-18 months from diagnosis, as most patients have unresectable disease at the time of presentation. […] In patients undergoing aggressive surgery, 5 year survival rates are 10-40%. Survival appears to be better for distal tumours, at up to 54% at 5 years.

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