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A Lack of Neuroblastoma in Down Syndrome: A Study from 11 European Countries¹

Laboratory of Pathology, Centre Hospitalier, 19000 Tulle, France [D. S.]; Epidemiology for Cancer Prevention, IARC, 69372 Lyon, France [A. J. S.]; Pediatric Hematology Oncology, Odense University Hospital, DK-5000 Odense C. Denmark [N. L. T. C.]; Childhood Cancer Research Group, Department of Pediatrics, Oxford OX6HJ, United Kingdom [C. A. S.]; Pediatric Hematology Oncology, Médecine Infantile G, Centre Hospitalier Universitaie Purpan, 31059 Toulouse, France [H. R.]; Pediatric Hematology Oncology, Klinik für Kinderheilkunde, D-5000 Cologne, Germany [B. H.]; Department of Hematology Oncology, Giannina Gaslini Children's Hospital, 16148 Genova, Italy [B. d. B.]; Pediatric Hematology Oncology, Emma Kinder Ziekenhuis, Academic Medical Center, 1105AZ Amsterdam, the Netherlands [J. d. K.]; Pediatric Oncology, University Hospital La Timone, 13385 Marseille, France [C. C.]; Childhood Cancer Research Unit, Department of Pediatrics, Karolinska Hospital, S-17176 Stockholm, Sweden [P. K.]; The Cancer Registry of Norway, Institute for Epidemiological Research Montebello, 0310 Oslo, Norway [F. L.]; Pediatric Hematology Oncology, Sophia Children's Hospital, 60 3015 GJ Rotterdam, the Netherlands [F. G. A. J. H-C.]; Pediatric Hematology Oncology, Centre Hospitalier Universitaire, Vaudois, 1011 Lausanne, Switzerland [D. B.]; Pediatric Hematology Oncology, Inselspital, 3010 Bern, Switzerland [N. v. d. W.]; West Middlands Regional Children's Tumour Research Group, Birmingham Children's Hospital, Birmingham B16 8ET, United Kingdom [S. P.]; Pediatric Hematology Oncology, Institut Gustave Roussy, Rue Camille Desmoulins, 94805 Villejuif, France [O. H.]; Pediatric Hematology Oncology, University Hospital Nijmegen, 6500 HB Nijmegen, the Netherlands [R. J. J. L.]; Pediatric Hematology Oncology, Beatrix Children's Hospital, 9700 RB Groningen, the Netherlands [W. A. K.]; and Pediatric Hematology Oncology, University Hospital, 54511 Nancy, France [D. S.]

An epidemiological investigation in 11 European countries comprising a total childhood population of 54.1 million children and using 8 separate data sources was conducted to evaluate the occurrence of neuroblastoma in Down syndrome (DS). No cases of DS were detected among 6724 infants and children with neuroblastoma, although more than five were expected. This highly significant result (P = 0.0045 according to the Poisson test) is consistent with data in the literature, which contains only two poorly detailed cases in epidemiological studies and one ganglioneuroma in a DS mosaic patient. Like other tumors, such as leukemias, testicular germ cell tumors and lymphomas are in excess in DS patients; the lack of neuroblastomas does not reflect a general decreased incidence of cancer but rather a specific underrepresentation of this precise 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 growth inhibition and differentiation of neural cells in vitro, (c) is abundant in good-prognosis neuroblastomas, and (d) has been shown to induce growth inhibition and differentiation and cell death in several human and murine neuroblastoma cell lines and could be responsible for this variation. Additional epidemiological and experimental studies are warranted to confirm our interpretation of these data.

¹ This study was supported by a grant from Ligue pour la Lutte contre le Cancer, Division Corrézienne. The Childhood Cancer Research Group was supported by the Department of Health, the Scottish Home and Health Department, and the Cancer Research Campaign.

² To whom requests for reprints should be addressed, at Laboratory of Pathology, Centre Hospitalier, 19000 Tulle, France. Phone: (33) 555-29-79-13; Fax: (33) 555-29-79-31.

³ Deceased.

Received 8/28/97. Accepted 12/ 2/97.

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