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Li-Fraumeni syndrome
Background
Li-Fraumeni syndrome (LFS) is a rare inherited condition that predisposes to a wide range of childhood and adult cancers, particularly sarcomas (a group of cancers that affect the connective tissues such as bone, muscle, nerves, fatty tissue, and cartilage). LFS occurs in both males and females. The syndrome was first described in 1969 by Dr Frederick Li and Dr Joeseph Fraumeni. LFS is specifically associated with sarcomas, which are named after the connective tissue they arise from, for example, cancers arising from bone are called osteosarcomas, from cartilage are called chondrosarcomas and from blood vessels are called angiosarcoma. Cancers of the brain, breast, blood (leukaemia; see entry Leukaemia and other Allied Blood disorders) and the adrenal gland (adrenocortical carcinomas) also occur commonly in families with LFS, but other cancers can also occur.
