Presentation
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]
Perihilar bile duct cancer: These cancers are mostly present in the hilum region, at the end portion of the liver where the left and right bile ducts move outside the liver. [news-medical.net]
Entire Body System
- 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]
- Falling
They fall into two broad categories based on the origin of tumor growth: intrahepatic cholangiocarcinoma and extrahepatic cholangiocarcinoma. 1 Intrahepatic cholangiocarcinoma arises from tumors that grow in the small ducts within the liver and extrahepatic [cancerquest.org]
D espite the need for cumulative evidence, clinical practice can fall short of that ideal. “Sometimes all you have is just one of these,” Dr. Izzy says—imaging suggesting a malignant stricture, but nothing else. [captodayonline.com]
Thus, although multiple studies report rising incidence rates of iCCA and falling rates of extrahepatic CCA, we do not know what is happening with incidence/mortality rates of pCCA, the commonest form of CCA, which could have been incorrectly coded as [onlinelibrary.wiley.com]
Gastrointestinal
- Overeating
My journey included five recurrences with eight tumors, over 30 hours of surgery, 52 chemotherapy treatments and many radiation sessions. [curetoday.com]
A small device that makes soundwaves is then passed over the area. The soundwaves produce a picture on a computer. The test is painless and only takes a few minutes. [macmillan.org.uk]
Cholangiocarcinoma is a rare cancer of the bile ducts with a dire prognosis that has been rising in incidence in the U.S. over the past 30 years. [targetcancerfoundation.org]
In the United States, incidence had been on the rise over the last 40 years, with 2000-3000 new cases each year. [radiopaedia.org]
And when they seek medical care, “it’s almost over.” The five-year survival rate without any treatment is dismal—less than 10 percent. [captodayonline.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]
Skin
- 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]
Treatment
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]
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]
They create a treatment plan that will best serve the patient. [cancercenter.gwu.edu]
Prognosis
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]
Prognosis Surgical resection improves prognosis, but complete removal of cancer at an advanced stage is hardly possible. Chemotherapy, radiotherapy, and immunotherapy show little benefits. [atlasgeneticsoncology.org]
Etiology
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]
Epidemiology
Going forward, diagnoses and epidemiological data need to be recorded uniformly and accurately. [onlinelibrary.wiley.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]
Definition Epidemiology References: [4] [6] Epidemiological data refers to the US, unless otherwise specified. [amboss.com]
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]
Pathophysiology
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
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]