MOLECULAR EPIDEMIOLOGY STUDY OF A SUSPECTED COMMUNITY CLUSTER OF CHILDHOOD CANCERS

We investigated the report of a community cluster of cancers in 33 chi ldren, which included two siblings known to have dominantly inherited Li-Fraumeni syndrome and a germline p53 mutation. After defining crite ria for inclusion in the cluster, the 12 eligible childhood cancer pro bands diagnosed between 1980 and 1989 were not excessive (expected, te n cases). The corresponding childhood cancer mortality rates for the c ommunity fluctuated between 1950 and 1989 and were not increased overa ll. However, three additional probands had family histories of childho od cancer that suggested a forme fruste oi Li-Fraumeni syndrome. The e pi demiological data suggested a geographic cluster of this rare hered itary disorder, but absence of germline p53 mutation in the three othe r multicase families indicates genetic heterogeneity. Laboratory studi es can assist analyses of suspected clusters, although investigations of geographic clusters of hereditary cancers raise complex issues of c onfidentiality and protection of affected individuals, their families, and the community. (C) 1997 Wiley-Liss, Inc.