Li-Fraumeni Syndrome Cancer Surveillance Strategy Considerations for Glioblastoma Multiforme

Abstract

Sporadic or inherited deficiencies in the production or activity of the tumor suppressor P53 lead to Li-Fraumeni Syndrome (LFS), a multi-organ tumorigenic condition. Glioblastoma multiforme (GBM), a tumor that commonly presents with a median age of 64, has a higher chance of appearing in much younger patients who have LFS [9]. Since the implementation of the 2016 Toronto Protocol to increase cancer surveillance in LFS patients, three cases of LFS-GBM have been discussed [11-13]. Here, we report a case of LFS in an 18-year-old male who had a seizure due to a GBM that had evaded a full-body MRI six months prior. Furthermore, we discuss the potential quality of life (QOL) benefits of providing patients with a shorter brain MRI screening interval: better survival outcomes and peace of mind. Though there may be a rise in the financial cost with an increase in the number of MRI scans, the prevalence of aggressive tumors that must be treated early for a better prognosis warrants more frequent screening. Furthermore, we address the importance of expanding clinical knowledge on GBM in the LFS setting as well as addressing the benefits of the protocol through statistical studies.