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Five percent of childhood cancers reflect well-known hereditary cancer disposition syndromes. Hereditary cancer disposition syndromes should be considered when the cancer in question frequently is hereditary, when several related cancers occur in the same family, or in the presence of congenital malformations. Genetic counselling requires specialist training and should focus on the risk of developing cancer among family members, the risk of a second cancer in the patient, the possibility of prenatal diagnosis of the cancer disposition syndrome, and the advantages of prophylaxis and of early cancer diagnosis through screening.

MeSH