Rhabdomyosarcoma in Children: Epidemiologic Study and Identification of a Familial Caneer Syndrome

Abstract

To study the origins of childhood rhabdomyosarcoma, an examination was made of the 418 death certificates of U.S. children who died of this neoplasm, 1960–64, and of 280 medical charts from 17 hospital centers. Of exceptional interest was the presence in 5 families of a second child with a soft-tissue sarcoma, 3 sibs (vs. 0.06 expected by chance), and 2 cousins. The parents, grandparents, and other relatives of children in these families had a high frequency of carcinoma of the breast and diverse neoplasms (e.g., acute leukemia and carcinomas of the lung, pancreas, and skin) at relatively young ages, suggesting a new familial syndrome of multiple primary cancers. Additional components of the syndrome were implicated by the occurrence of adrenocortical carcinoma and brain tumor in the first-degree relatives of 2 other children with rhabdomyosarcoma. While suggesting the role of inheritance, the familial patterns seen with rhabdomyosarcoma may result from an interaction of genetic and environmental (?viral) factors. The oncogenic agents and mechanisms in human cancer may be identified by the use of such family aggregations for laboratory studies and further epidemiologic studies. Like most childhood neoplasms, rhabdomyosarcoma showed a peak mortality before 4 years of age and occurred slightly more often in males. This neoplasm was diagnosed in 29 children in the hospital series before 1 year of age and in 9 within 1 month of birth; this indicates that rhabdomyosarcoma may arise in vtero. Unlike most neoplasms of early inception, however, no association with congenital defects was detected. Rhabdomyosarcoma also showed no variations in time and/or space that might reflect environmental influences, such as viral or chemical agents which can induce this neoplasm in laboratory animals.