Immunotherapy has revolutionized the treatment of cancers in the last 15 years, demonstrating that a patient’s very own immune system can seek and destroy a tumor even in its earlier stages. One of the most exciting findings from this research is the possibility that the immune system responds against non-inherited (spontaneous) mutations in the TP53 gene.
Based on this promising evidence, the Li-Fraumeni Syndrome Association (LFSA) has set forth a scientific agenda that prioritizes understanding how the immune system responds to cancers that arise from inherited (germline) variants in the TP53 gene. One of the first outcomes from this agenda is a recent study led by the late Pierre Hainaut of the LFSA Medical and Scientific Advisory Board (MSAB), Neoantigenic properties of TP53 variants influence cancer risk in individuals with Li-Fraumeni syndrome.
This work demonstrates that the age of onset of cancers can be reliably predicted from the immune system’s response against tumors driven by germline variants in the TP53 gene. Therefore, the immune system is actively surveilling cancers in patients with LFS, confirming the observations in the spontaneous case. This research indicates that immunotherapy may offer especially strong benefit against cancers in individuals with Li-Fraumeni syndrome.
