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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.)

There has been speculation that increasing trends in incidence of childhood central nervous system tumors and infant neuroblastoma in the United States have been due to diagnostic improvements or reporting changes. To investigate whether or not such trends could be explained in this way in the U.K., the authors used population-based data from Northwest England to analyze incidence trends in childhood solid tumors. Cases were diagnosed during 1954–1998 and were grouped according to a morphology-based classification scheme. More than 95% of diagnoses were based on special histopathologic review. Tissue sections were retained, and diagnoses were rereviewed to ensure consistency in classification throughout the time period. Age-, gender- and period-specific incidence rates were calculated. Analyses were performed with chi-square tests and Poisson regression. There was an overall increase in the incidence of all childhood solid tumors of 0.9% each year. A temporal increase was found in childhood brain tumors characterized by, in particular, annual increases of 1% in pilocytic astrocytoma, 1% in primitive neuroectodermal tumors, and 2.3% in miscellaneous gliomas. The incidence of germ cell tumors increased at a rate of 2.6% each year. These increases could not be attributed to changes in diagnostic practice, and it is unlikely that the increases were due to changes in reporting practice. Further, the restriction of the increases to certain groups, with stable rates in others, argued against the changes being artifactual. The authors concluded that the increases in incidence were likely to be real. Cancer 2001;92:1967–76. © 2001 American Cancer Society.