The goal of this review is to demonstrate the effective interaction of epidemiologic methods
and molecular genetics in the identification of familial cancer predisposition. The example …

Abstract The Li-Fraumeni cancer family syndrome is manifested by susceptibility to breast
cancer, sarcomas, and other neoplasms in children and young adults. The present study …

Germ-line mutations of the tumor-suppressor gene p53 have been observed in some
families with the Li-Fraumeni syndrome (LFS), a familial cancer syndrome in which affected …

Genetic anticipation is the increased incidence, earlier onset, or increased severity of a
disease in successive generations. Before the biological basis of anticipation had been …

Germ line p53 point mutations have been reported for some families with Li-Fraumeni
syndrome, a syndrome characterized by a dominantly inherited increased susceptibility for …

Abstract The Li-Fraumeni familial cancer syndrome was initially described in 1969 in a
retrospective epidemiologic review of more than 600 pediatric sarcoma patients. The clinical …

In 1969 Li and Fraumeni identified, from a systematic study of 648 children with
rhabdomyosarcomas, five families in which a sibling or a cousin was affected by sarcoma (Li …

Li-Fraumeni syndrome is a rare autosomal dominant susceptibility to a variety of cancers
including carcinomas of the breast and the adrenal cortex, tumors of brain and muscle …

Abstract Background: Li-Fraumeni syndrome is a dominantly inherited disorder
characterized by early-onset breast cancer, sarcomas, and other cancers in children and …

Genetic susceptibility to cancer has been the focus of much recent research. A number of
clearly defined, inherited conditions are known to predispose to specific cancers, for …