Table 2 Classification schemes for Li-Fraumeni syndrome

Classic LFS [29]

Proband diagnosed with sarcoma before 45 years of age, AND

A first-degree relative with cancer before 45 years of age, AND

Another first- or second-degree relative with any cancer diagnosed under 45 years of age or with sarcoma at any age

Chompret's [112, 113]

I. Proband with sarcoma, brain tumor, breast cancer, or adrenocortical carcinoma before age 36 years, AND at least one first or second degree relative with cancer (other than breast cancer if the proband has breast cancer) under the age of 46 years or a relative with multiple primaries at any age

II. Proband with multiple primary tumors, two of which are sarcoma, brain tumor, breast cancer, and/or adrenocortical carcinoma,

with the initial cancer occurring before the age of 36 years, regardless of the family history

III. Proband with adrenocortical carcinoma at any age of onset, regardless of the family history