A surveillance clinic for children and adolescents with, or at risk of, hereditary cancer predisposition syndromes

Hereditary cancer predisposition syndromes (HCPS) account for at least 10% of paediatric cancers.1 Li‐Fraumeni syndrome (LFS) is a dominant HCPS caused by mutations in the TP53 gene and is associated with an 80–90% lifetime risk of cancer, commencing in infancy.2 Children of affected individuals are at 50% risk of inheriting the family mutation.

Citation:
Leedman N, Princehorn M, Gottardo N, Franklin C, D'Souza R, Kiraly-Borri CE. A surveillance clinic for children and adolescents with, or at risk of, hereditary cancer predisposition syndromes. Med J Aust. 2021.

Keywords:
Adolescence; Early detection of cancer; Genetic counseling; Genetic testing; Physician-patient relations; Preventive medicine; Risk factors

Abstract:
Hereditary cancer predisposition syndromes (HCPS) account for at least 10% of paediatric cancers.1 Li‐Fraumeni syndrome (LFS) is a dominant HCPS caused by mutations in the TP53 gene and is associated with an 80–90% lifetime risk of cancer, commencing in infancy.2 Children of affected individuals are at 50% risk of inheriting the family mutation.