The diagnosis of hilar cholangiocarcinoma is usually made on evaluation of obstructive jaundice or elevated liver enzymes. Biliary cancers may be clinically silent for long periods of time and it may be many months before a patient bearing such a tumor presents with overt clinical features. Progressive and unremitting jaundice is usually the predominant clinical feature, and diagnostic investigations are largely related to elucidation of the cause of biliary tract obstruction. A minority of patients will present with abdominal pain that may be mistakenly attributed to gallstone disease. While gallstones or even common bile duct stones may coexist with bile duct cancer, in the absence of certain predisposing conditions (e.g., PSC, oriental cholangiohep-atitis), it is uncommon for choledocholithiasis to cause obstruction at the biliary confluence. It is therefore imperative to fully investigate and delineate the level and nature of any obstructing lesion causing jaundice to avoid missing the diagnosis of carcinoma.
Most patients are referred after having had some studies done elsewhere, usually a computed tomography (CT) scan and some form of direct cholangiography [percutaneous transhe-patic cholangiography (PTC) or endoscopic retrograde cholangiopancreatography (ERCP)]. These studies are often inadequate for full assessment of the tumor extent. In addition, biopsies or brushings are frequently taken at the time of cholangiography (or even at the time of exploration in some cases) but are often nondiagnostic. In the authors' view, histologic confirmation of malignancy is not mandatory prior to exploration. With no prior suggestive history (i.e., prior biliary tract operation, PSC, hepatolithiasis), the finding of a focal stenotic lesion combined with the appropriate clinical presentation are sufficient for a presumptive diagnosis of hilar cholangiocarcinoma, which is correct in most instances (39). Once a diagnosis of cholangiocar-cinoma is suggested, radiographic studies are crucial to determine the extent of the tumor to appropriately forge a therapeutic plan.
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