Cholangiocarcinoma Wikipedia. Cholangiocarcinoma, also known as bile duct cancer, is a form of cancer that is composed of mutatedepithelial cells or cells showing characteristics of epithelial differentiation that originate in the bile ducts which drain bile from the liver into the small intestine. Blumgart Hepatobiliary Surgery Pdf' title='Blumgart Hepatobiliary Surgery Pdf' />Other biliary tract cancers include gallbladder cancer and cancer of the ampulla of Vater. Cholangiocarcinoma is a relatively rare neoplasm that is classified as an adenocarcinoma a cancer that forms glands or secretes significant amounts of mucins. It has an annual incidence rate of 12 cases per 1. Western world,1 but rates of cholangiocarcinoma have been rising worldwide over the past few decades. Prominent signs and symptoms of cholangiocarcinoma include abnormal liver function tests, abdominal pain, jaundice, and weight loss. Other symptoms such as generalized itching, fever, and changes in color of stool or urine may also occur. ORIGINAL. Manejo nutricional del paciente postciruga de vas biliares y pncreas. Nutritional management of the patient after surgery of the biliary tract and. Original Article. Cisplatin plus Gemcitabine versus Gemcitabine for Biliary Tract Cancer. Juan Valle, M. D., Harpreet Wasan, M. D., Daniel H. Palmer, M. D., Ph. D., David. Mtt Milano Font. Rak drg ciowych ac. Blumgart Hepatobiliary Surgery Pdf Books' title='Blumgart Hepatobiliary Surgery Pdf Books' />The disease is diagnosed through a combination of blood tests, imaging, endoscopy, and sometimes surgical exploration, with confirmation obtained after a pathologist examines cells from the tumor under a microscope. Known risk factors for cholangiocarcinoma include primary sclerosing cholangitis an inflammatory disease of the bile ducts,3 infection with the parasitic liver flukes. Opisthorchis viverrini or Clonorchis sinensis, some congenital liver malformations, and exposure to Thorotrast thorium dioxide, a chemical formerly used in medical imaging. However, most people with cholangiocarcinoma have no identifiable risk factors. Cholangiocarcinoma is considered to be an incurable and rapidly lethal cancer unless both the primary tumor and any metastases can be fully removed by surgery. No potentially curative treatment exists except surgery, but most people have advanced stage disease at presentation and are inoperable at the time of diagnosis. People with cholangiocarcinoma are generally managed though not cured with chemotherapy, radiation therapy, and other palliative care measures. These are also used as additional therapies after surgery in cases where resection has apparently been successful or nearly so. Signs and symptomseditThe most common physical indications of cholangiocarcinoma are abnormal liver function tests, jaundice yellowing of the eyes and skin occurring when bile ducts are blocked by tumor, abdominal pain 3. To some extent, the symptoms depend upon the location of the tumor patients with cholangiocarcinoma in the extrahepatic bile ducts outside the liver are more likely to have jaundice, while those with tumors of the bile ducts within the liver more often have pain without jaundice. Blood tests of liver function in patients with cholangiocarcinoma often reveal a so called obstructive picture, with elevated bilirubin, alkaline phosphatase, and gamma glutamyl transferase levels, and relatively normal transaminase levels. Such laboratory findings suggest obstruction of the bile ducts, rather than inflammation or infection of the liver parenchyma, as the primary cause of the jaundice. CA1. Risk factorseditAlthough most patients present without any known risk factors evident, a number of risk factors for the development of cholangiocarcinoma have been described. In the Western world, the most common of these is primary sclerosing cholangitis PSC, an inflammatory disease of the bile ducts which is closely associated with ulcerative colitis UC. Epidemiologic studies have suggested that the lifetime risk of developing cholangiocarcinoma for a person with PSC is on the order of 1. Casting Simulation Software Open Source there. The mechanism by which PSC increases the risk of cholangiocarcinoma is not well understood. Certain parasitic liver diseases may be risk factors as well. Colonization with the liver flukes. Opisthorchis viverrini found in Thailand, Laos PDR, and Vietnam1. Clonorchis sinensis found in China, Taiwan, eastern Russia, Korea, and Vietnam1. Patients with chronic liver disease, whether in the form of viral hepatitis e. B or hepatitis C,1. HIV infection was also identified in one study as a potential risk factor for cholangiocarcinoma, although it was unclear whether HIV itself or other correlated and confounding factors e. C infection were responsible for the association. Infection with the bacteria Helicobacter bilis and Helicobacter hepaticus species can cause biliary cancer. Congenital liver abnormalities, such as Carolis syndrome a specific type of five recognized choledochal cysts, have been associated with an approximately 1. The rare inherited disorders Lynch syndrome II and biliary papillomatosis have also been found to be associated with cholangiocarcinoma. The presence of gallstones cholelithiasis is not clearly associated with cholangiocarcinoma. However, intrahepatic stones called hepatolithiasis, which are rare in the West but common in parts of Asia, have been strongly associated with cholangiocarcinoma. Exposure to Thorotrast, a form of thorium dioxide which was used as a radiologic contrast medium, has been linked to the development of cholangiocarcinoma as late as 3. Thorotrast was banned in the United States in the 1. Pathophysiologyedit. Digestive system diagram showing bile duct location. Cholangiocarcinoma can affect any area of the bile ducts, either within or outside the liver. Tumors occurring in the bile ducts within the liver are referred to as intrahepatic, those occurring in the ducts outside the liver are extrahepatic, and tumors occurring at the site where the bile ducts exit the liver may be referred to as perihilar. A cholangiocarcinoma occurring at the junction where the left and right hepatic ducts meet to form the common hepatic duct may be referred to eponymously as a Klatskin tumor. Although cholangiocarcinoma is known to have the histological and molecular features of an adenocarcinoma of epithelial cells lining the biliary tract, the actual cell of origin is unknown. Recent evidence has suggested that the initial transformed cell that generates the primary tumor may arise from a pluripotent hepatic stem cell. Cholangiocarcinoma is thought to develop through a series of stages from early hyperplasia and metaplasia, through dysplasia, to the development of frank carcinoma in a process similar to that seen in the development of colon cancer. Chronic inflammation and obstruction of the bile ducts, and the resulting impaired bile flow, are thought to play a role in this progression. Histologically, cholangiocarcinomas may vary from undifferentiated to well differentiated. They are often surrounded by a brisk fibrotic or desmoplastic tissue response in the presence of extensive fibrosis, it can be difficult to distinguish well differentiated cholangiocarcinoma from normal reactive epithelium. There is no entirely specific immunohistochemical stain that can distinguish malignant from benign biliary ductal tissue, although staining for cytokeratins, carcinoembryonic antigen, and mucins may aid in diagnosis. Most tumors 9. DiagnosiseditMicrograph of an intrahepatic, i. Histologically, this is a cholangiocarcinoma as 1 atypical bile duct like cells left of image extend from the tumor in an interlobular septum the normal anatomical location of bile ducts, and 2 the tumor has the abundant desmoplastic stroma often seen in cholangiocarcinomas. A portal triad upper left of image has a histologically normal bile duct.