• soft tissue sarcoma;
  • childhood;
  • genetic predisposition;
  • Li–Fraumeni syndrome;
  • neurofibromatosis


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.