At-Risk Populations for Osteosarcoma: The Syndromes and Beyond

Table 2

Li-Fraumeni diagnosis and screening criteria.

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Classic Li-Fraumeni Criteria

Proband diagnosed with a sarcoma before age 45

First-degree relative with any cancer before age 45

First- or second-degree relative with any cancer before age 45 or a sarcoma at any age

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Li-Fraumeni-like Criteria

Proband with any childhood cancer, sarcoma, brain tumor, or adrenocortical carcinoma before age 45

First- or second degree relative with sarcoma, brain tumor, adrenocortical carcinoma, or leukemia at any age

First- or second-degree relative with any cancer before age 60

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2009 Chompret Criteria for TP53 testing

(I) Proband with tumor belonging to LFS tumor spectrum (e.g., soft tissue sarcoma, osteosarcoma, brain tumor, premenopausal breast cancer, adrenocortical carcinoma, leukemia, and 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

(II) 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

(III) Patient with adrenocortical carcinoma or choroid plexus tumor, irrespective of family history

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