Cancer Predisposition: Li-Fraumeni Syndrome in Pediatrics, Nurses Can Make A Difference in Quality of Life and Long-Term Survival Through Screening and Education (201-1)

10:45 – 11:00 am Wednesday, September 2

Oncology nurses provide care for patients and families with multiple early-onset cancers. This session will review Li-Fraumeni Syndrome (LFS), a genetic cancer predisposition syndrome. It is recognized as a mutation in the TP53 gene, the most frequently mutated gene in sporadic cancers. LFS is defined as any germline mutation in the TP53 tumor suppressor gene that is passed down in an autosomal dominant pattern.

In response to stress signals, the tumor suppressor gene controls a wide range of processes including apoptosis, DNA replication and repair, and regulatory control over the cell cycle. There are two different types of criteria used to diagnose LFS, the classical and the Chompret criteria. It is important to understand the history and genetics of LFS, along with the criteria used to diagnose it. There are certain disease types that are associated with LFS, and the prevalence of these cancers varies based on age. Cumulative cancer risk associated with LFS has been estimated to be approximately 50% by age 40 years and up to 90% by age 60 years. Patients are recommended to follow regular screening guidelines, which have been shown to improve overall survival. Multiple screening guideline protocols are utilized, including that of the MD Anderson Children’s Cancer Hospital pediatric LEAD clinic. Effective surveillance is critical to increasing patients’ overall survival. LFS can have a significant psychological impact on the children and families that it affects. Oncology nurses can help make a difference in the lives of these patients through increasing their quality of life and long-term survival with regular screening and education. The goal of this session is to educate and empower pediatric oncology nurses so that they can make a difference in this unique population.

Heather Meador, MSN APRN CPNP CPHON®
Whitney Throckmorton, MPAS PA-C
CNE Hours: