ORGAN SITE SPECIFICITY FOR CANCER IN CHROMOSOMAL INSTABILITY DISORDERS

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  • 1 January 1982
    • journal article
    • research article
    • Vol. 42  (8) , 3252-3254
Abstract
DNA rearrangement rather than point mutation is an emerging hypothesis for human carcinogenesis. Although there is no direct evidence for this hypothesis, indirect evidence is provided by cancer cytogenetics and genetics. It has been suggested that patients with Bloom''s syndrome, a disorder of spontaneous chromosomal rearrangement, develop the common fatal internal cancers and thus that genetic rearrangements, rather than chemical mutagens, cause most internal human cancers. To test this observation age- and sex adjusted cancer incidence rate ratios were derived for specific organ sites in patients with 3 chromosomal instability disorders (Bloom''s syndrome, xeroderma pigmentosum, and dyskeratosis congenital); the increased incidence of cancer in all 3 disorders is limited to specific and often uncommon organ sites. Chromosomal instability disorders apparently do not predispose patients to the common fatal internal cancers. Although DNA rearrangement remains a promising concept in human carcinogenesis, the organ site specificity of cancers associated with Bloom''s syndrome, xeroderma pigmentosum and dyskeratosis congenita cannot be used as evidence to implicate this mechanism.