PATTERNS OF CANCER IN THE FAMILIES OF CHILDREN WITH SOFT-TISSUE SARCOMA

Background. Childhood soft tissue sarcomas are known to occur in a num ber of genetic syndromes. This study assesses the proportion of soft t issue sarcoma diagnosed in childhood associated with genetic predispos ition to cancer, Methods. Information on the occurrence of neoplastic disease was collected for 151 of 179 families of a population-based se ries of children with soft tissue sarcoma. Results. Considering the in dex child as the proband, 5 of the 151 families manifested the classic Li-Fraumeni cancer family syndrome according to standard criteria and a further 10 families showed features consistent with the syndrome. O ne proband had double primary syndrome cancers. One other family had a sibling pair of childhood cancers, seven families had cancer which ha d occurred in childhood in other relatives, and three families had adu lt-onset sarcomas in more distant relatives. In another 16 families, o ne parent or the other had developed a possible syndrome cancer, or ha d developed cancer when younger than 60 years of age. Two families sho wed striking clusters of stomach cancer. Five case children were thoug ht to have been affected with neurofibromatosis. Conclusions. Genetic predisposition to cancer was thought to be present in 7% to 33% of fam ilies interviewed.