Recent advances in the molecular biology and treatment of biliary trac
t cancer are highlighted in this review. Mutations in an oncogene (K-r
as) and a tumor suppressor gene (p53) are increasingly reported in ass
ociation with biliary tract cancer, and their presence may indicate a
poor prognosis. The identification of a point mutation in cyclin-depen
dent kinase 4 inhibitor p1 6INK4 in vivo and in vitro suggests that bi
liary tract carcinogenesis is associated with the inactivation of impo
rtant cell-cycle proteins. Several institutions not only in Japan but
also in the Western hemisphere report the results of extended resectio
ns including hepatectomies in combination with local resections for pr
oximal cholangiocarcinoma. In the selected group of patients curativel
y resected (negative margins), this appears to translate into improved
median and long-term survivals. Likewise, complete resection of gallb
lader cancer without microscopic residual disease is associated with l
ong median survival rates, even in advanced stages. Radiation therapy
has been used in patients with positive margins as adjuvant treatment
or as palliation of unresectable biliary tract carcinoma. A detailed r
eview of several recent series reporting the results of radiotherapy i
n these two settings suggests that radiotherapy currently does not con
fer a definite improvement in survival.