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Pathology - Extrahepatic bile duct carcinoma
Definition: A malignant epithelial tumor that develops in an extrahepatic bile duct.
Epidemiology • Incidence is rare and does not vary by geography. Aetiology • PSC. • Infestation of liver fluke. • Choledochal cysts. 2 Choledocholithiasis doesn't appear to be important. Carcinogenesis • Mutations in KRAS and TP53 are identified.
Presentation: • Obstructive jaundice. • Fever and rigors can be caused by superimposed cholangitis. Macroscopy reveals a tumor in the bile duct, which can be polypoid, stenosing, or diffusely infiltrative.
Histopathology: • The majority of adenocarcinomas are well or moderately differentiated, with malignant epithelial cells forming glandular structures similar to bile ducts.
Prognosis: Patients with resectable tumors and clear surgical margins typically have a 5-year survival rate of 20-40%. • Tumors associated with PSC have a low 5-year survival rate (<10%).< />pan>


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