Clinical features of biliary tract cancer in Japanese individuals with Lynch syndrome.

ABSTRACT:

Background

Biliary tract cancer (BTC) is a Lynch syndrome (LS)-associated cancer with a high mortality rate. This study aimed to clarify the clinical features of BTC in individuals with LS and to discuss its management.

Methods

We obtained data from genetically verified Japanese individuals with LS who were diagnosed at a single institution, between January 2003 and April 2021. Moreover, 21 individuals with sporadic BTC (n=15) and LS associated BTC (n=6) underwent microsatellite instability (MSI) testing.

Results

Among 92 individuals with LS, 6 individuals with MLH1 variants developed BTCs (10 lesions, male/female, 2:1). The median age at diagnosis of initial BTC was 69 years (range, 34-78 years). Histological examination revealed a predominance of differentiat

SUBMITTER: Kanaya N 

PROVIDER: S-EPMC9660066 | biostudies-literature | 2022 Oct

REPOSITORIES: biostudies-literature