Li-Fraumeni Syndrome
- Symptoms
What are the cancer risks associated with LFS?
|
Cancer type |
LFS risk (up to age 20) |
General population risk (up to age 20) |
|
Overall cancer | Females: 15 - 27%
Males: 25 - 39% | < 0.4% |
Soft tissue cancer
(sarcoma) | Females: 2.5 - 5%
Males: 6 - 12% | 0.02% |
Bone cancer
(osteosarcoma) | Females: 2.5 - 7.5%
Males: 7.5 - 12.5% | Rare |
Brain
(various) | Up to 8% | 0.04% |
Cancer of the adrenal cortex
(adrenocortical carcinoma) | Up to 9% | Rare |
The risk of other cancers including leukaemia, Wilms tumour, neuroblastoma, and lung, colorectal, stomach and pancreatic cancers may also be increased (risk figures not quantified).
Note: The conditions associated with LFS and their risk estimates may change as more information is available. Published literature may overestimate cancer risks. These figures are based on retrospective analysis which may be biased towards families with an excess of cancer diagnoses.
|
Cancer type |
LFS risk (up to age 70) |
General population risk (up to age 70) |
|
Overall cancer (female) | > 95% | 22% |
|
Overall cancer (male) | > 90% | 23% |
|
Breast (female) | 85% | 13% |
|
Soft tissue cancer (sarcoma) | 50% | Rare |
|
Bone cancer (osteosarcoma) | 10% | Rare |
|
Brain cancer | 20 - 30% | Rare |
|
Colorectal cancer | Up to 24% | 4% |
|
Gastric cancer | 1 - 16% | ~1% |
|
Cancer of the adrenal cortex (adrenocortical carcinoma) | Increased | Rare |
|
Others: Lung, prostate, renal, melanoma and pancreatic cancers | Increased | - |
|
Risk of second primary cancer | 50% by 10 years | 8% |
Note: The conditions associated with LFS and their risk estimates may change as more information is available. Published literature may overestimate cancer risks. These figures are based on retrospective analysis which may be biased towards families with an excess of cancer diagnoses.
