Cancer risks in LKB1 germline mutation carriers.

ABSTRACT:

Background and aims

Germline mutations in the LKB1 gene are known to cause Peutz-Jeghers syndrome, which is an autosomal dominant disorder characterised by hamartomatous polyposis and mucocutaneous pigmentation. This syndrome is associated with an increased risk of malignancies in different organs but there is a lack of data on cancer range and risk in LKB1 germline mutation carriers.

Patients and methods

The cumulative incidence of cancer in 149 Peutz-Jeghers syndrome patients with germline mutation(s) in LKB1 was estimated using Kaplan-Meier time to cancer onset analyses and compared between relevant subgroups with log rank tests.

Results

Thirty two cancers were found in LKB1 mutation carriers. Overall cancer risks at ages 30, 40, 50, 60, and 70 years were 6%, 18%,

SUBMITTER: Mehenni H 

PROVIDER: S-EPMC1856321 | biostudies-literature | 2006 Jul

REPOSITORIES: biostudies-literature