ABSTRACT
Introduction Cancer results from complex interactions between multiple genes and environmental factors. In families with hereditary cancer, one single altered susceptibility gene is largely responsible for cancer risk. Most hereditary cancer syndromes are inherited in an autosomal dominant fashion. Individuals who inherit cancer susceptibility genes inherit a predisposition to cancer rather than the cancer itself. Some mutation carriers do not develop cancer, indicating that these altered genes are ‘incompletely penetrant’, and a mutation in the second allele is required for cancer to develop. This chapter gives a review of the main surveillance, surgery, and screening measures used in the most frequent hereditary cancer syndromes.
