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]

• Malaise

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

  Signs and symptoms include malaise, weight loss, right upper quadrant abdominal pain, and night sweats. [ebi.ac.uk]

  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]

  Diagrams / tables Images hosted on other servers: Morphologic classification Clinical features Asymptomatic in early stages (19 - 40% incidentally identified) ( J Am Coll Surg 2001;193:384 ) Nonspecific symptoms in late stages, such as abdominal pain, malaise [pathologyoutlines.com]

  In an advanced stage, abdominal pain, fever, general malaise, and weight loss can occur. On ultrasound imaging, there are no specific features for intrahepatic cholangiocarcinomas to distinguish them from other intrahepatic tumors. [atlasgeneticsoncology.org]

• 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]

• 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]

• Alopecia

  […] control rate, best overall response, and progression-free survival were secondary endpoints. 8 In the interim analysis, the most common treatment-emergent adverse events (all grades) included hyperphosphatemia (73%), fatigue (49%), stomatitis (45%), alopecia [qedtx.com]

• Dark Urine

  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]

  Check with your doctor if you have any of the following: jaundice (yellowing of the skin or whites of the eyes) dark urine clay colored stool pain in the abdomen fever itchy skin nausea and vomiting weight loss for an unknown reason There are no routine [cancer.gov]

  Dark urine. Clay-colored stool. Pain in the abdomen. Fever. Itchy skin. Nausea and vomiting. Weight loss for an unknown reason. Check with your physician if you have any of these symptoms. [mysouthernhealth.com]

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

  Jaundice symptoms are also seen in the urine and stool; jaundiced individuals often release very dark urine and pale-colored stool. [loyolamedicine.org]

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]