title: Familial cancer or cancer family syndrome. Report on a cancer family and consideration of genetic mechanisms.
abstract: We present a family with an unusually high incidence of cancer in four generations. Complete information could be obtained for the first three generations with 73 persons. Tumor incidence was 2/8 in the first generation, 14/22 in the second, and 11/44 in the third generation. A formal analysis, however, according to the four criteria of cancer family syndrome: increased frequency of adenocarcinomas of multiple anatomical sites, multiple primary malignant neoplasms, early age of onset, and autosomal dominant mode of inheritance, revealed that in this family multiple primaries are virtually absent and that tumors do not occur at a particularly early age. We therefore consider this family shows that familial clustering of tumors may follow an autosomal dominant pattern of inheritance even when the strict criteria of cancer family syndrome are not fulfilled. We conclude that the phenomenon of cancer families is not confined to one or two distinct clinical types.
mesh_terms: D000230:Adenocarcinoma / Q000235:genetics; D000328:Adult; D000367:Age Factors; D000368:Aged; D005260:Female; D005799:Genes, Dominant; D006801:Humans; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000235:genetics*; D010375:Pedigree; D013577:Syndrome