ABSTRACT
In 1969, Li and Fraumeni published a paper that led to the identification of a new
hereditary cancer syndrome known as Li-Fraumeni Syndrome (LFS) (1). They reported
four families with an unusual clustering of childhood soft tissue sarcomas, young onset
breast cancer, and a variety of other tumors, and suggested this might represent a new
familial syndrome; they followed those findings with a systematic survey of the cancer
family history of over 600 U.S. children with rhabdomyosarcoma (2) that revealed
additional components of the syndrome including adrenal cortical carcinoma and brain
tumors. The syndrome was distinctly different from most familial cancer aggregates in
the diversity of tumor types and young ages of cancer diagnosis.
