There is limited evidence at present for the use of post-operative chemotherapy. [teachmesurgery.com]

It is slightly more prevalent in males than females (1.3:1.0) and usually presents in the fifth to seventh decade of life. Clinical manifestations are not usually noted until an advanced disease stage. [orpha.net]

These genetic changes, which are classified as germline mutations, are present in essentially all of the body's cells. However, no specific inherited changes have been found to be major risk factors for this disease. [ghr.nlm.nih.gov]

Presented June 3, 2017. 6. Diaz L, Marabelle A, Kim TW, et al: Efficacy of pembrolizumab in phase 2 KEYNOTE-164 and KEYNOTE-158 studies of microsatellite instability high cancers. 2017 ESMO Congress. Abstract 386P. Presented September 11, 2017. [ascopost.com]

• Painless Jaundice

  Painless jaundice (yellow/orange coloring of the skin and eyes) may be the only initial clue. The history often includes reviewing alcohol use, drug use, or recent illnesses that may be associated with hepatitis, or inflammation of the liver. [medicinenet.com]

  Distal cholangiocarcinomas, which can mimic pancreatic cancer with regards to presentation (painless jaundice with a distal common bile duct stricture), can be treated via pancreaticoduodenectomy (Whipple procedure). [healio.com]

• Collapse

  NorthShore is one of the few centers in the country performing radiofrequency (RF) ablation of obstructing bile duct cancers, which either block or have collapsed a bile duct, to restore normal bile flow. [northshore.org]

  It can involve bypass surgery to reroute the bile around the cancer or stents to hold open a bile duct being collapsed by cancer. Biliary drainage helps relieve signs and symptoms of cholangiocarcinoma. [drugs.com]

• Anorexia

  Other less common features include RUQ pain, early satiety, weight loss, anorexia and malaise. On examination, jaundice and cachexia are often evident. Courvoisier’s law can be applied in clinical assessment. [teachmesurgery.com]

• Abdominal Pain

  Clinical findings Obstruction, jaundice, abdominal pain, weight loss. Location Hilar, 50–60%; intrahepatic, 10%; extrahepatic 20–30%. [medical-dictionary.thefreedictionary.com]

  Biopsy People who have abdominal pain might have an exam of the gastrointestinal tract, called endoscopic retrograde cholangiopancreatography (ERCP). [upmc.com]

  Symptoms: Yellowing of your skin and whites of your eyes Weight loss Abdominal pain or discomfort Nausea Itchy skin Treatment Options Treatment depends on the size and location of the tumor, whether the cancer has spread, and the person’s overall health [bostonscientific.com]

  Cholangiocarcinoma [2] Extrahepatic cholangiocarcinoma Signs of cholestasis : jaundice, pale stools, dark urine, pruritus Abdominal pain and weight loss is usually a sign of late (unresectable) disease. [amboss.com]

• Loss of Appetite

  Common symptoms may include: Clay colored stools Jaundice, which is a yellowing of the skin and eyes Itching Abdominal pain that may extend to the back Loss of appetite Unexplained weight loss Fever Chills Your doctor will first ask about your medical [ucsfhealth.org]

  Other signs and symptoms of bile duct cancer include: Abdominal pain, particularly if the cancer has grown/enlarged or spread Itching Fever Weight loss Loss of appetite Risks Causes and Risk Factors of Cholangiocarcinoma Individuals with bile duct inflammation [loyolamedicine.org]

  This can cause: yellowing of the skin and whites of the eyes (jaundice) itchy skin pale stools and dark urine loss of appetite and weight loss persistent tiredness and feeling unwell tummy (abdominal) pain and swelling – some people feel a dull ache in [nhs.uk]

  Other cholangiocarcinoma symptoms may include weight loss, loss of appetite, weakness, loss of energy, and easy bruising or bleeding (factors that clot the blood are manufactured in the liver and loss of liver function may decrease the clotting factors [medicinenet.com]

  […] of appetite Pain in the upper right abdomen that may radiate to the back Weight loss Yellowing of the skin ( jaundice ) Exams and Tests Your health care provider will perform a physical exam. [slu.adam.com]

• Right Upper Quadrant Pain

  Right upper quadrant pain, fever, and rigors suggest cholangitis (this is unusual without drainage attempts). Cholangiocarcinoma usually presents after the disease is advanced. [gut.bmj.com]

• Pruritus

  Symptoms include post-hepatic jaundice and pruritus, with pale stools and dark urine. Other less common features include RUQ pain, early satiety, weight loss, anorexia and malaise. On examination, jaundice and cachexia are often evident. [teachmesurgery.com]

  Extrahepatic cholangiocarcinoma may manifest with signs of cholestasis (e.g., jaundice, dark urine, pruritus ) and a painless, enlarged gallbladder ( Courvoisier sign ). [amboss.com]

  Extra-hepatic CCA manifests with signs of cholestasis (jaundice, pale stools, dark urine, pruritus), malaise, weight loss and/or progressive weakness. [orpha.net]

  Palliative therapy Relief of biliary obstruction is warranted when symptoms such as pruritus and hepatic dysfunction outweigh other symptoms of the cancer. [healthy.kaiserpermanente.org]

  […] the permission of the Centre for Evidence-based Medicine. 3.0 DIAGNOSIS 3.1 Clinical features 5, 17 Most common presenting clinical features of perihilar or extrahepatic tumours are those of biliary obstruction: jaundice, pale stool, dark urine, and pruritus [gut.bmj.com]

• Enlargement of the Liver

  Additional rare but serious side effects may include enlargement of your liver, spleen, or gallbladder. [healthline.com]

• Hepatocellular Carcinoma

  Although narrowing the portal veins, or less frequently hepatic veins, is seen, unlike hepatocellular carcinoma, cholangiocarcinoma only rarely forms a tumor thrombus 2. [radiopaedia.org]

  Indeed, for hepatocellular carcinoma, which is typically dependent on an arterial supply, dose-response relationships have been reported ( 46, 47 ). [hbsn.amegroups.com]

  Cf Hepatocellular carcinoma. chol·an·gi·o·car·ci·no·ma ( kō-lan'jē-ō-kahr-si-nō'mă ) An adenocarcinoma, primarily in intrahepatic bile ducts, composed of ducts lined by cuboidal or columnar cells that do not contain bile, with abundant fibrous stroma. [medical-dictionary.thefreedictionary.com]

  Diagnosing Cholangiocarcinoma Symptoms of cholangiocarcinoma Symptoms of a bile duct tumor are similar to those of hepatocellular carcinoma (HCC), or advanced primary liver cancer, and may include: Abdominal pain Abdominal swelling or mass Fatigue Fluid [upmc.com]

When clinical trials show that a new treatment is better than the standard treatment, the new treatment may become the standard treatment. Patients may want to think about taking part in a clinical trial. [cancer.gov]

Treatment Options Treatment for bile duct cancer depends on the location, type and extent of the tumor. Patients need a multidisciplinary care plan that incorporates all potential therapies in the treatment decisions and plan. [froedtert.com]

They create a treatment plan that will best serve the patient. [cancercenter.gwu.edu]

Clonorchis sinensis, possibly ulcerative cholitis, cholecystitis; not associated with alcohol abuse Clinical Jaundice—71%, abdominal pain-49%, weight loss—44% Prognosis 53% 1-yr survival, 9% 3-yr, 4% 5-yr. [medical-dictionary.thefreedictionary.com]

Even with resection, the prognosis is poor with a five-year survival of only 10-44% 4, with prognosis favoring extrahepatic tumors (around 30% five-year survival vs. 15% for intrahepatic tumors). [radiopaedia.org]

Prognosis As proximal CCA is usually not diagnosed until a late stage of disease, prognosis is poor with 5-year survival rates of 20-50% after resection and almost 0% in unresectable tumors. [orpha.net]

Early detection is difficult and the prognosis is generally poor. [ NCIT : C4436 ] Synonyms: CCA, CC, Cholangiocar.- intra/extrahepatic, Cholangiocellular carcinoma, adult primary cholangiocarcinoma, cholangiocarcinoma, malignant, intrahepatic bile duct [ebi.ac.uk]

Etiology The etiology is unknown and most cases of CCA occur sporadically. [orpha.net]

Prognosis 53% 1-year survival; 4% 5-year survival. cholangiocarcinoma Bile duct cancer, cancer of bile ducts A rare–5/100,000/yr cancer of intrahepatic bile ducts seen > age 60 Etiology Anabolic steroids, Thorotrast,. [medical-dictionary.thefreedictionary.com]

Cholangiocarcinoma is difficult to cure and many cases are sporadic with no identifiable etiology or cause. [my.clevelandclinic.org]

PMID 16858537 Cholangiocarcinomas in Japanese and Thai patients: difference in etiology and incidence of point mutation of the c-Ki-ras proto-oncogene. [atlasgeneticsoncology.org]

Etiology Risk factors for cholangiocarcinoma [2] [3] [5] Primary sclerosing cholangitis Liver fluke infection (e.g., Clonorchis sinensis, Opisthorchis viverrini ) Choledocholithiasis (both with and without hepatic duct involvement) Chronic viral hepatitis [amboss.com]

Going forward, diagnoses and epidemiological data need to be recorded uniformly and accurately. [onlinelibrary.wiley.com]

Definition Epidemiology References: [4] [6] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]

[…] other hand, arises from tumors that grow in the bile ducts outside of the liver. 1 While, extrahepatic and intrahepatic cholangiocarcinoma produce similar symptoms, their risk factors, response to therapies, and origins are different. 1 Risk factors Epidemiological [cancerquest.org]

Summary Epidemiology The prevalence is unknown but it accounts for approximately 3% of gastrointestinal tumors and 10-15% of all hepatobiliary malignancies. Clinical description CCA can occur in the intra or extra-hepatic biliary tract. [orpha.net]

[…] stroma Exclusion of metastatic adenocarcinoma by thorough review of the patient's clinical history and application of an appropriate immunohistochemistry panel Terminology Also known as peripheral cholangiocarcinoma and intrahepatic bile duct carcinoma Epidemiology [pathologyoutlines.com]

Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology/Diagnosis/Management. 10th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 69. National Cancer Institute website. [medlineplus.gov]

In: Sleisenger and Fordtran's Gastrointestinal and Liver Disease: Pathophysiology, Diagnosis, Management. 10th ed. Philadelphia, Pa.: Saunders Elsevier; 2016. http://www.clinicalkey.com. Accessed Feb. 22, 2016. Rizvi S, et al. [mayoclinic.org]

[…] common in East Asia, due to endemic liver fluke infection More common in older patients (over 75% of patients are older than 65 at initial diagnosis) ( Gastroenterology 2009;136:1134 ) Slightly more common in males than females ( Hepatology 2011;54:173 ) Pathophysiology [pathologyoutlines.com]

Pathophysiology Cholangiocarcinomas arise from the intrahepatic or extrahepatic biliary epithelium. More than 90% are adenocarcinomas, and the remainder are squamous cell tumors. The etiology of most bile duct cancers remains undetermined. [emedicine.medscape.com]

Prevention There's no way to prevent cholangiocarcinoma (bile duct cancer). But you may reduce your risk of the disease if you: Stop smoking. Smoking is linked to an increased risk of cholangiocarcinoma. If you smoke, stop. [mayoclinic.org]

Causes, Risk Factors, and Prevention Learn about the risk factors for bile duct cancer and what you might be able to do to help lower your risk. Early Detection, Diagnosis, and Staging Know the signs and symptoms of bile duct cancer. [cancer.org]

Chemotherapy : Patients who undergo surgical resection of the tumor may be candidates for post-operative preventative chemotherapy. [rarediseases.org]

National Cancer Institute Liver and Bile Duct Cancer –Patient Version Centers for Disease Control and Prevention Clonorchis FAQs Tyson, El-Serag. Risk factors for cholangiocarcinoma. Hepatology 2011; 54: 173-84. [publichealth.va.gov]

Read more about preventing hepatitis C Hepatitis B A vaccine is available that provides immunisation against hepatitis B. [nhsinform.scot]