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li-fraumeni-syndrome
Li-Fraumeni Syndrome
Li-Fraumeni syndrome (LFS) is caused by germline TP53 mutations; spectrum of sarcoma, brain tumors, breast cancer, and adrenocortical carcinoma; radiation avoidance is critical; whole-body MRI annual surveillance; TP53 is the most commonly mutated gene in all cancers.
Entry Metadata
| Field | Value |
|---|---|
| ID | li-fraumeni-syndrome |
| Name | Li-Fraumeni Syndrome |
| Status | draft |
| Last reviewed | 2026-06-07 |
| Atlas | 01-human |
| Scale | 07-system |
Cross-Atlas Connections
Sources
- Bougeard G, Renaux-Petel M, Flaman JM, et al. Revisiting Li-Fraumeni Syndrome From TP53 Mutation Carriers. J Clin Oncol. 2015;33(21):2345-2352. · PubMed 26014290
- 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;17(9):1295-1305. · PubMed 27501770