At this time, there is no standard treatment or cure for LFS or a germline TP53 gene mutation. With some exceptions, cancers in people with LFS are treated the same as for cancers in other patients, but research continues on how to best manage those cancers involved in LFS.
Research has indicated that those individuals with LFS appear to be an elevated risk for radiation-induced cancers, so the use of radiotherapy should be approached with caution. For this reason, computed tomography (CT) scans and other diagnostic techniques involving ionizing radiation should be limited. However, radiation therapy should not be avoided if the benefits outweigh the risks.
Since those living with LFS are susceptible to the development of a number of different cancers, individuals should ensure that they incorporate simple measures into a healthylifestyle, such as sun protection and the avoidance of tobacco products.
It has been widely accepted that early cancer detection can greatly increase overall survival, and those diagnosed with LFS should seek to adhere to preventive screening. An expert panel of LFS researchers, oncologists, and genetic counselors has published surveillance recommendations that utilize whole body MRI screening for patients that fit the definition of Li-Fraumeni syndrome. This should be offered as soon as the diagnosis of LFS is established. In brief, the screening recommendations involve:
Children (birth to age 18 years)
General assessment
Complete physical exam every 3-4 months
Prompt assessment with primary care physician for any medical concerns
Also noted, for families in which breast cancer has already made an appearance at or around age 20 – awareness and screening can be considered 5 to 10 years before the earliest age of onset known. The same is recommended for gastrointestinal cancers – consider screening 5 years before the earliest known onset of a gastrointestinal cancer in the family.
