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Background. Childhood soft tissue sarcomas are known to occur in a number of genetic syndromes. This study assesses the proportion of soft tissue sarcoma diagnosed in childhood associated with genetic predisposition to cancer. Methods. Information on the occurrence of neoplastic disease was collected for 151 of 179 families of a population-based series of children with soft tissue sarcoma. Results. Considering the index 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. One proband had double primary syndrome cancers. One other family had a sibling pair of childhood cancers, seven families had cancer which had occurred in childhood in other relatives, and three families had adult-onset sarcomas in more distant relatives. In another 16 families, one parent or the other had developed a possible syndrome cancer, or had developed cancer when younger than 60 years of age. Two families showed striking clusters of stomach cancer. Five case children were thought to have been affected with neurofibromatosis. Conclusions. Genetic predisposition to cancer was thought to be present in 7% to 33% of families interviewed.

Previous research has identified families with a higher-than-average risk of cancer. These families were first identified on the basis of soft-tissue sarcomas occurring in the children. Soft tissue sarcomas include entities such as rhabdomyosarcoma and fibrosarcoma. To extend the knowledge of such cancer-prone families, a study was undertaken of 754 first-degree relatives of 177 children with soft-tissue sarcoma. A total of 40 cancers were recorded among the relatives, in contrast to the 25 which might be expected in a population of this size. There was no significant excess among the fathers of the index cases, and only a borderline excess among the mothers. Siblings of the index cases, however, had a roughly five-fold increase in the risk of cancer. Breast cancers were the most common among the 40 cancers identified in the relatives; carcinomas of the trachea and lung, and colon and rectum were quite common in childhood. These results suggest that soft tissue sarcomas in children are likely to have a strong genetic component. Furthermore, the occurrence of more common cancers in the families of children with relatively uncommon soft tissue sarcomas may provide insight into some of the fundamental mechanisms of cancer development. (Consumer Summary produced by Reliance Medical Information, Inc.)