Cancer mortality in relatives of desmoid sarcoma patients

Abstract

Desmoid sarcoma patients from the U.S. and Canada, diagnosed under the age of 16 years and referred to the University of Texas, M.D. Anderson Hospital - MDAH - between 1944 and 1975 were surveyed. Family information was collected for grandparents, parents, parental siblings, proband offspring and siblirtgs. Expected mortality was calculated by applying age-race-sex specific U.S. mortality rates to the person-years at risk. Standardized mortality ratio's - SMR- were calculated. Among 429 relatives of 26 desmoid sarcoma probands no overali cancer excess was found, observed/expected = 13/24.7. The risk among parents, the ones more informative for genetic analysis were also not significant, observed/expected = 2/1.83. An updated follow-up of incidence data of the study population and of the general population is suggested in the years to come, and in doing so, the inclusion of all familly members history should pull the results to a number closer to reality.