Natural History

The vast majority of patients with unresectable bile duct cancer die within six months to a year of diagnosis, usually from liver failure or infectious complications secondary to biliary obstruction (2,4,12,13). The prognosis has been considered worse for lesions affecting the confluence of the bile ducts and better for lesions of the distal bile duct close to the papilla, which probably reflects the greater complexity and difficulty in effectively managing proximal lesions more so than differences in biologic behavior. Indeed, it has been shown that location within the biliary tree (proximal vs. distal) has no impact on survival provided that complete resection is achieved (5). On the other hand, anatomic site-related differences in biological

behavior may well exist but are not well-defined, and their clinical relevance remains unclear. IHC, unlike tumors of the extrahepatic bile ducts, rarely cause jaundice or biliary tract-related sepsis. Patients often present with advanced lesions due to the absence of symptoms with small intrahepatic tumors. Multifocal hepatic disease, likely the result of intrahepatic vascular spread, is not uncommon. Patients with disease not amenable to resection usually die of hepatic failure within 12 months, secondary to diffuse liver involvement, or inanition related to advanced malignant disease.

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