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Items: 4

1.

Intrahepatic Cholangiocarcinoma

A carcinoma that arises from the intrahepatic bile duct epithelium in any site of the intrahepatic biliary tree. Grossly, the malignant lesions are solid, nodular, and grayish. Morphologically, the vast majority of cases are adenocarcinomas. Signs and symptoms include malaise, weight loss, right upper quadrant abdominal pain, and night sweats. Early detection is difficult and the prognosis is generally poor. [from NCI]

MedGen UID:
87521
Concept ID:
C0345905
Neoplastic Process
2.

Cholangiocarcinoma

Cholangiocarcinoma is a primary cancer originating in the biliary epithelium i.e., the cholangiocytes, of the extrahepatic and intrahepatic biliary ducts. It is extremely invasive, develops rapidly, often metastasizes, and has a very poor prognosis. They are slow growing tumors which spread longitudinally along the bile ducts with neural, perineural and subepithelial extension. [from HPO]

MedGen UID:
60210
Concept ID:
C0206698
Neoplastic Process
3.

Cholangiocarcinoma, susceptibility to

Carcinomas of the biliary tract are aggressive malignancies, with 5-year survival of less than 10%. These carcinomas arise throughout the biliary tree and are anatomically classified as either intrahepatic or extrahepatic cholangiocarcinomas. Gallbladder carcinomas also arise from the biliary tree but have distinct natural histories compared to cholangiocarcinomas, suggesting different underlying tumor biology. Cholangiocarcinoma incidence varies widely between geographic regions, reflecting the impact of different underlying etiologies. In endemic areas, liver fluke infections by O. viverrini and Clonorchis sinensis, both group I carcinogens, represent the major risk factor for cholangiocarcinomas. In nonendemic regions, other risk factors, including choledochal cysts (603003), hepatolithiasis, and primary sclerosing cholangitis (613806), are likely contributors (summary by Chan-on et al., 2013). Overall, the majority of patients lack such identifiable risk factors (summary by Jiao et al., 2013). [from OMIM]

MedGen UID:
816486
Concept ID:
C3810156
Finding
4.

Hilar Cholangiocarcinoma

Klatskin tumour is an extra-hepatic cholangiocarcinoma arising in the junction of the main right or left hepatic ducts to form the common hepatic duct. Klatskin tumours occur in the hepatic duct bifurcation. Patients are usually asymptomatic until advanced stages of the disease where jaundice is the principle manifestation. Abdominal pain, weight loss and malaise are other manifestations. Metastasis to regional lymph nodes is frequent. In 90% of cases Klatskin tumours occur sporadically but certain risk factors have been associated with the disease. [from SNOMEDCT_US]

MedGen UID:
61664
Concept ID:
C0206702
Neoplastic Process
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