TABLE 1.
Clinical Criteria for Classic LFS, LFS-like Criteria, and Chompret Criteria
| Classic LFS Criteria:21 |
|---|
| • Proband diagnosed with a sarcoma before age 45 |
| • A first-degreea relative with cancer diagnosed before age 45 |
| • A first- or second-degreeb relative on the same side of the family with cancer diagnosed before age 45 or a sarcoma at any age |
| LFS-like Criteria Birch:84 |
| • Proband with any childhood cancer or sarcoma, brain tumor, or adrenocortical carcinoma diagnosed before age 45 |
| • First- or second-degree relative with a typical LFS cancer (sarcoma, breast cancer, brain tumor, leukemia, or adrenocortical carcinoma) diagnosed at any age and |
| • A first- or second-degree relative on the same side of the family with any cancer diagnosed under age 60 |
| Eeles definition of LFL:84 |
| • Two first- or second-degree relatives with LFS related malignancies at any age |
| Chompret criteria for LFS:29 |
| • Proband diagnosed with a narrow-spectrum cancer (sarcoma, brain tumor, breast cancer, or adrenocortical carcinoma) before age 36 and at least one first- or second-degree |
| • A proband with multiple primary tumors, two of which belong to the narrow spectrum and the first of which occurred before age 36, regardless of family history |
| • A proband with adrenocortical carcinoma, regardless of age at diagnosis or family history |
Abbreviations: LFS, Li-Fraumeni syndrome: LFL, Li-Fraumeni-like.
a
First-degree relative is defined as a parent, sibling, or child.
b
Second-degree relative is defined as a grandparent, aunt, uncle, niece, nephew, or grandchild.