Table 1.

Criteria for TP53 Testing in Cancer Patients

Clinical Criteria for Li-Fraumeni Syndrome
Classical criteria (all must apply)A patient with a sarcoma diagnosed before age 45 yr
A first-degree relative with any cancer before age 45 yr
A first- or second-degree relative with any cancer before age 45 yr or a sarcoma at any age
Revised Chompret criteria (only one must apply)Familial presentation:
Proband with an LFS spectrum tumora prior to age 46 yr and at least 1 first- or second-degree relative with an LFS tumor (except breast cancer, if the proband has breast cancer) before the age of 56 yr or with multiple tumors
Multiple tumors:
Proband with multiple malignancies (except 2 breast cancers), of which at least 2 belong to the LFS spectrum, before the age of 46 yr
Rare tumors:
Patients with ACC, choroid plexus carcinoma, or embryonal anaplastic subtype rhabdomyosarcoma independent of family history
Early-onset breast cancer:
Patients with breast cancer before the age of 31 yr
Clinical Criteria for Li-Fraumeni Syndrome
Classical criteria (all must apply)A patient with a sarcoma diagnosed before age 45 yr
A first-degree relative with any cancer before age 45 yr
A first- or second-degree relative with any cancer before age 45 yr or a sarcoma at any age
Revised Chompret criteria (only one must apply)Familial presentation:
Proband with an LFS spectrum tumora prior to age 46 yr and at least 1 first- or second-degree relative with an LFS tumor (except breast cancer, if the proband has breast cancer) before the age of 56 yr or with multiple tumors
Multiple tumors:
Proband with multiple malignancies (except 2 breast cancers), of which at least 2 belong to the LFS spectrum, before the age of 46 yr
Rare tumors:
Patients with ACC, choroid plexus carcinoma, or embryonal anaplastic subtype rhabdomyosarcoma independent of family history
Early-onset breast cancer:
Patients with breast cancer before the age of 31 yr

Abbreviations: ACC, adrenocortical carcinoma; LFS, Li-Fraumeni syndrome.

aLFS spectrum tumors: premenopausal breast cancer, soft-tissue sarcoma, brain tumor, and adrenocortical carcinoma.

Table 1.

Criteria for TP53 Testing in Cancer Patients

Clinical Criteria for Li-Fraumeni Syndrome
Classical criteria (all must apply)A patient with a sarcoma diagnosed before age 45 yr
A first-degree relative with any cancer before age 45 yr
A first- or second-degree relative with any cancer before age 45 yr or a sarcoma at any age
Revised Chompret criteria (only one must apply)Familial presentation:
Proband with an LFS spectrum tumora prior to age 46 yr and at least 1 first- or second-degree relative with an LFS tumor (except breast cancer, if the proband has breast cancer) before the age of 56 yr or with multiple tumors
Multiple tumors:
Proband with multiple malignancies (except 2 breast cancers), of which at least 2 belong to the LFS spectrum, before the age of 46 yr
Rare tumors:
Patients with ACC, choroid plexus carcinoma, or embryonal anaplastic subtype rhabdomyosarcoma independent of family history
Early-onset breast cancer:
Patients with breast cancer before the age of 31 yr
Clinical Criteria for Li-Fraumeni Syndrome
Classical criteria (all must apply)A patient with a sarcoma diagnosed before age 45 yr
A first-degree relative with any cancer before age 45 yr
A first- or second-degree relative with any cancer before age 45 yr or a sarcoma at any age
Revised Chompret criteria (only one must apply)Familial presentation:
Proband with an LFS spectrum tumora prior to age 46 yr and at least 1 first- or second-degree relative with an LFS tumor (except breast cancer, if the proband has breast cancer) before the age of 56 yr or with multiple tumors
Multiple tumors:
Proband with multiple malignancies (except 2 breast cancers), of which at least 2 belong to the LFS spectrum, before the age of 46 yr
Rare tumors:
Patients with ACC, choroid plexus carcinoma, or embryonal anaplastic subtype rhabdomyosarcoma independent of family history
Early-onset breast cancer:
Patients with breast cancer before the age of 31 yr

Abbreviations: ACC, adrenocortical carcinoma; LFS, Li-Fraumeni syndrome.

aLFS spectrum tumors: premenopausal breast cancer, soft-tissue sarcoma, brain tumor, and adrenocortical carcinoma.

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