Epidemiology
About 5,000 cases per year in US
Most common location is at bifurcation of bile duct (Klatskin tumor, 65-70%)
Also occurs in the ampulla of Vater (25-30%) and intrahepatic bile duct (5-10%)
Risk factors
Primary sclerosing cholangitis
Schistosomiasis (liver fluke infestation)
Ulcerative colitis
Prior history of colon cancer
Pathology
Most tumors are adenocarcinomas
Sclerosing: associated with desmoplastic reaction, poor prognosis
Papillary: favorable prognosis
Tumor markers: CEA, CA19-9 elevation, no AFP elevation (helps to differentiate from HCC)
Treatment
Resection is the only cure (fewer than 1/3 of patients have resectable disease)
Neoadjuvant chemoradiation followed by liver transplant appears to improve survival in highly selected patients
Distal tumors are more frequently resectable
2010 trial showed survival improvement with cisplatin + gemcitabine vs. gemcitabine alone in locally advanced tumors (PMID 20375404)
No prospective CRT studies exist
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