LI-FRAUMENI-SYNDROME IN PEDIATRIC-PATIENTS WITH SOFT-TISSUE SARCOMA OR OSTEOSARCOMA

Family history of cancer and features of the Li-Fraumeni syndrome (LFS ) were investigated in 42 patients with soft tissue sarcoma or osteosa rcoma in a pediatric hospital in Mexico City, and compared with 42 non -cancer children. Si;rt subjects with cancer were found among 204 firs t-degree relatives of cancer patients while there were none among 183 first-degree relatives of non-cancer children. In three families, the proband had two affected relatives, and the type of neoplasia as well as the age of onset suggested the clinical diagnosis of LFS. Our resul ts show that 7.1% of our pediatric patients with soft tissue sarcoma o r osteosarcoma may belong to LFS families. The authors encourage pedia tric and adult oncologists to pay more attention to the history of can cer in nuclear families for eventual hereditary cancer syndrome identi fication and cancer prevention.