Individuals With Li-Fraumeni Syndrome Benefit From Surveillance
Surveillance of individuals with Li-Fraumeni syndrome, each of whom carry a germline TP53 pathogenic variant, is likely to improve a patient’s overall survival.
Surveillance of individuals with Li-Fraumeni syndrome, each of whom carry a germline TP53 pathogenic variant, is likely to improve a patient's overall survival, according to a study published in The Lancet Oncology.1
To update the surveillance protocol for carriers of TP53 pathogenic variants, which greatly increase one's risk of developing cancer at any time of life, researchers identified and enrolled 89 individuals from 39 unrelated families to a study that compared surveillance with non-surveillance outcomes.
Forty patients agreed to undergo surveillance; 49 declined; patients were, however, allowed to cross over groups at any time, and 19 crossed from non-surveillance to surveillance. Median surveillance time was 32 months.
Forty asymptomatic tumors were identified in 19 of the 59 total patients undergoing surveillance; 61 symptomatic tumors were identified in 43 of the 49 patients who initially refused surveillance.
Median 5-year overall survival was 88.8% for patients in the surveillance group, versus 59.6% for those in the non-surveillance group.
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The authors concluded that surveillance is feasible, and is associated with better overall survival than non-surveillance, for patients with Li-Fraumeni syndrome.
- Villani A, Shore A, Wasserman JD, et al. Biochemical and imaging surveillance in germline TP53 mutation carriers with Li-Fraumeni syndrome: 11 year follow-up of a prospective observational study. Lancet Oncol. 2016 Aug 5. doi: 10.1016/S1470-2045(16)30249-2 [Epub ahead of print]