title: Prognostic significance of chromosome 17p deletions in childhood primitive neuroectodermal tumors (medulloblastomas) of the central nervous system.
abstract: Deletions in the short arm of chromosome 17 (17p) are the most common genetic abnormality in primitive neuroectodermal tumors of the posterior fossa/medulloblastoma (PNET/Mb). The biological consequences of these deletions are not known for children with PNET/Mb; however, the presence of a tumor suppressor gene located in 17p, distinct from p53, has been implicated in tumorigenesis. Two recent studies suggest that 17p deletions in PNET/Mb are associated with a poor prognosis. To address this question, we identified deletions of chromosome 17p by cytogenetic and/or molecular biology methods in tumor biopsy samples from 56 patients with PNET/Mb. Associations between clinical characteristics or survival outcomes and 17p status were examined by multivariate analysis. Forty-one percent of PNET/Mb cases had a deletion of 17p. No significant association was found between 17p deletion and shorter survival duration or higher metastatic stage. Multivariate analysis did not find independent prognostic significance for 17p deletions after accounting for the effects of significant clinical variables. A larger study of the prognostic value of 17p deletion should be considered; however, clinical use of this factor to distinguish high-risk from standard-risk PNET/Mb populations is not warranted at this time.
authors.affiliation: Divisions of Human Genetics, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
authors.forename: J A
authors.initials: JA
authors.lastname: Biegel
authors.forename: A J
authors.initials: AJ
authors.lastname: Janss
authors.forename: C
authors.initials: C
authors.lastname: Raffel
authors.forename: L
authors.initials: L
authors.lastname: Sutton
authors.forename: L B
authors.initials: LB
authors.lastname: Rorke
authors.forename: J M
authors.initials: JM
authors.lastname: Harper
authors.forename: P C
authors.initials: PC
authors.lastname: Phillips
mesh_terms: D000367:Age Factors; D001932:Brain Neoplasms / Q000235:genetics* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D002528:Cerebellar Neoplasms / Q000235:genetics / Q000401:mortality / Q000473:pathology / Q000601:surgery; D002675:Child, Preschool; D002872:Chromosome Deletion; D002874:Chromosome Mapping; D002886:Chromosomes, Human, Pair 17; D005260:Female; D006801:Humans; D008297:Male; D008527:Medulloblastoma / Q000235:genetics* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D015999:Multivariate Analysis; D009367:Neoplasm Staging; D018242:Neuroectodermal Tumors, Primitive / Q000235:genetics* / Q000401:mortality / Q000473:pathology / Q000601:surgery; D011379:Prognosis; D016019:Survival Analysis