Retinoblastoma: Epidemiologic Characteristics

Abstract

Nationwide study of 269 death certificates for United States children with retinoblastoma, 1960–67, revealed a peak in mortality at two to three years of age that was 2 1/2 times greater in Negroes than in whites. Nationally, an estimated 81 per cent of children with the neoplasm survived. Study of 1623 hospital records of children with retinoblastoma revealed an excess of mental retardation: 21 cases observed vs. 4.7 expected. Associated malformations in several cases suggested D-deletion syndrome, which has been described by others in six children with retinoblastoma. In accord with previous studies, radiotherapy was associated with an excess of osteosarcoma, soft-tissue neoplasms or carcinoma of the skin. In addition, 11 children had second primary cancers not attributable to radiotherapy, a seeming excess over normal expectation. The cell types were diverse, but three were osteosarcomas, suggesting an innate susceptibility that may add to the risk of radiotherapy.