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Chompret diagnostic criteria for Li-Fraumeni syndrome and TP53 testing[1,2]

Chompret diagnostic criteria for Li-Fraumeni syndrome and TP53 testing[1,2]
One of the following:
• A proband who has:
- A tumor belonging to the LFS tumor spectrum (soft tissue sarcoma, osteosarcoma, premenopausal breast cancer, brain tumor, adrenocortical carcinoma, leukemia, or lung bronchoalveolar cancer) before age 46 years AND
- At least 1 first- or second-degree relative with an LFS tumor (except breast cancer if the proband has breast cancer) before age 56 years or with multiple tumors;
• A proband with multiple tumors (except multiple breast tumors), 2 of which belong to the LFS tumor spectrum and the first of which occurred before age 46 years;
• A proband who is diagnosed with adrenocortical carcinoma or choroid plexus tumor, irrespective of family history
LFS: Li-Fraumeni syndrome.
References:
  1. Tinat J, Bougeard G, Baert-Desurmont S, et al. 2009 Version of the Chompret criteria for Li Fraumeni syndrome. J Clin Oncol 2009; 27:e108.
  2. Li-Fraumeni Syndrome. Schneider K, Garber J. In: Pagon RA, Bird TC, Dolan CR, Stephens K, editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-1999 Jan 19 [updated 2010 Feb 9].
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