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Cancer Genetics

Li-Fraumeni Syndrome Criteria

07/13/2000

Question:

One more Li Fraumeni question: I don`t understand why relatives from both sides of the family "count" in the LFS criteria. Don`t the first-degree and second-degree relatives have to come from the same side of the family?

Answer:

The criteria for Li-Fraumeni syndrome have been modified over the years. The criteria currently recommended for usage by the National Comprehensive Cancer Network (NCCN) may be found in the British Journal of Cancer, volume 76, pages 1-14, from 1997.

The criteria for Li-Fraumeni syndrome are as follows:

Classic Li-Fraumeni syndrome -- 1) member of kindred with a known p53 mutation 2) a proband under age 45 yr with a sarcoma having a first-degree relative under age 45 yr with any cancer and an additional first- or second-degree relative in the same lineage with any cancer under age 45 yr, or a sarcoma at any age.

[If you notice, these criteria specify that the affected relatives must be in the same lineage.]

There are also Li-Fraumeni-Like syndrome criteria. A proband with any childhood tumor or sarcoma, or brain tumor or adrenocortical carcinoma under age 45, plus a first- or second-degree relative with a typical Li-Fraumeni syndrome tumor at any age, plus another first- or second-degree relative with any cancer under age 60.

Cancers associated with Li-Fraumeni syndrome include acute leukemia, premenopausal breast cancer, brain tumor, adrenocortical carcinoma, bone and soft-tissue sarcomas.

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Response by:

Judith A Westman, MD Judith A Westman, MD
Associate Professor, Clinical Internal Medicine, Pediatrics and Medical Biochemistry
College of Medicine
The Ohio State University