Prospective study of a family cancer syndrome

Risk of cancer was studied prospectively in four families described in 1969 as having diverse neoplasms, especially breast cancer and soft-tissue sarcoma. Between 1969 and 1981, in ten of 31 surviving family members, there developed 16 additional cancers (expected, 0.5). There were five breast carcinomas, four soft-tissue sarcomas, and seven other cancers. In eight patients, multiple primary cancers developed, including three softtissue sarcomas and one mesothelioma at sites of prior radiotherapy. (JAMA1982;247:2692-2694)