PedAM
Pediatric Disease Annotations & Medicines
| Disease | li-fraumeni syndrome |
| Phenotype | C1261473|sarcoma |
| Sentences | 1 |
| PubMedID- 25745605 | Afamilies were classified according to the clinical definitions of li-fraumeni syndrome (lfs, proband with sarcoma at 45 years and a first-degree relative with tumor at 45 and another close relative with tumor at 45, or sarcoma at any age) or li-fraumeni-like syndromes; lfl(b), proband with any childhood cancer or sarcoma, brain tumor or adrenocortical carcinoma at 45 years, with one first- or second-degree relative with typical lfs cancer, including sarcoma, breast cancer, brain tumor, leukemia, or adrenocortical carcinoma at any age, plus one first- or second-degree relative in the same lineage with any cancer diagnosed under age 60; lfl(c), proband with tumor belonging to lfs tumor spectrum (soft-tissue sarcoma, osteosarcoma, brain tumor, pre-menopausal breast cancer, adrenocortical carcinoma, leukemia, lung bronchoalveolar cancer) before age 46 years and at least one first- or second-degree relative with lfs tumor (except breast cancer if proband has breast cancer) before age 56 years or with multiple tumors; or proband with multiple tumors (except multiple breast tumors), two of which belong to lfs tumor spectrum and first of which occurred before age 46 years; or patient with adrenocortical carcinoma or choroid plexus tumor, irrespective of family history. |
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