A LACK OF NEUROBLASTOMA IN DOWN-SYNDROME - A STUDY FROM 11 EUROPEAN COUNTRIES

An epidemiological investigation in 11 European countries comprising a total childhood population of 54.1 million children and using 8 separ ate data sources was conducted to evaluate the occurrence of neuroblas toma in Down syndrome (DS). No cases of DS were detected among 6724 in fants and children with neuroblastoma, although more than five were ex pected. This highly significant result (P = 0.0045 according to the Po isson test) is consistent with data in the literature, which contains only two poorly detailed cases in epidemiological studies and one gang lioneuroma in a DS mosaic patient. Like other tumors, such as leukemia s, testicular germ cell tumors and lymphomas are in excess in DS patie nts; the lack of neuroblastomas does not reflect a general decreased i ncidence of cancer but rather a specific underrepresentation of this p recise tumor. S-100 b protein, the gene for which maps to the long arm of chromosome 21, (a) is overproduced in DS patients, (b) produces gr owth inhibition and differentiation of neural cells in vitro, (c) is a bundant in good-prognosis neuroblastomas, and (d) has been shown to in duce growth inhibition and differentiation and cell death in several h uman and murine neuroblastoma cell lines and could be responsible for this variation. Additional epidemiological and experimental studies ar e warranted to confirm our interpretation of these data.