Spectrum and Excess Risk of Gastrointestinal Tumors in Li-Fraumeni Syndrome.

ABSTRACT: Li-Fraumeni syndrome (LFS), linked to heterozygous germline pathogenic/likely pathogenic variants in TP53, confers exceptionally high cancer risk, including core cancers (sarcoma, breast, adrenocortical, and brain cancer) among many other cancer types.¹ Colorectal cancer (CRC) is most common after the core and hematologic cancers, accounting for ∼2.8% of diagnoses. Stomach and esophageal cancers constitute another 1.3% (TP53 Database; R20, July 2019: https://tp53.isb-cgc.org).².

SUBMITTER: Hatton JN 

PROVIDER: S-EPMC10922060 | biostudies-literature | 2024 Mar

REPOSITORIES: biostudies-literature