Life expectancy has dramatically expanded, the global population has aged unprecedentedly and the number of centenarians has significantly increased. The analysis of autopsy series, cancer registries data, vital statistics and surveys specific to this age group allows unique observations with respect to incidence and prevalence, cause of death by cancer, frequency of primary tumours, metastatic patterns, occurrence of incidental cancers and of multiple primary tumours. Data analysis demonstrates how cancer incidence and cause of death present a threefold decrease after age 90 and reach 0-4% above age 100. In addition, the number of metastatic sites are remarkably less and incidental malignant tumours or multiple primary cancers are more frequent, indicating that cancer in centenarians carries a more indolent behaviour. The unique features of malignant tumours in this population is hereby presented and discussed following a systematic review of the available literature. Cancer in the very elderly is relatively uncommon as a disease and as a cause of death. It is characterized by a slow growth and a modest life-threatening potential.
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