Abstract
Lynch syndrome is an autosomal dominant inherited disease characterized by an elevated risk of a variety of cancers. Presently, we can expect good prognoses of colon, endometrial, and gastric cancers in patients with Lynch syndrome due to the effectiveness of surveillance for these cancers and the relatively low malignant potentials of these cancers. However, in order to increase a patient’s life expectancy, a surveillance of other Lynch syndrome-associated cancers is also necessary. Biliary tract cancer has a high incidence in the general population in eastern Asia and it is an aggressive malignancy with a poor prognosis. The biliary tract may become one of the important target organs of cancer surveillance strategies in Japan for patients with Lynch syndrome.