Two recent studies have addressed paucity of cancer among people with Down syndrome. Based on a literature review in 1998, Satge and colleagues reported an underrepresentation of certain tumors in adults with Down syndrome. Their examination of the literature suggested that some tumors may be in excess (lymphomas, gonadal and extragonadal germ cell tumors, and possibly retinoblastomas and pancreatic and bone tumors). Other tumors were underrepresented, particularly neuroblastomas in children and common epithelial tumors in adults.

Hasle and coworkers identified 2,814 individuals with Down syndrome using the Danish Cytogenetic Register and linked these data to the Danish Cancer Registry. Cases in this study were followed an average of 17 years. The risk for solid tumors in persons with Down syndrome was decreased -- 24 tumors were observed, while 47.8 were expected. Higher than expected numbers of testicular cancers, ovarian cancers, and retinoblastomas were seen, but these findings were not statistically significant.