title: [Characteristics of medulloblastoma in children under age of three years].
abstract: We present a series of 51 medulloblastoma in children under three years, collected in N.N. Burdenko Neurosurgical Institute from 2000 to 2010. 57% of the tumors showed desmoplastic/nodular histology. Performed fluorescence in situ hybridization (FISH) analysis revealed the MYC oncogene amplification in 4%, the MYCN oncogene amplification - in 8%, isochromosome 17q - in 16% of cases. 9q deletion was found in 8% of desmoplastic/ nodular medulloblastomas. Our results showed that desmoplastic/nodular medulloblastoma has a positive predictive value for progression-free survival. Another feature of a biology of medulloblastomas in children younger than three years is the lack of nuclear accumulation of beta-catenin, and 6q deletion. Medulloblastomas with MYCN oncogene amplification often exhibit desmoplastic/nodular histology and a relatively favorable outcome. The most unfavorable prognostic marker is the MYC oncogene amplification, which in our series of 100% combined with the large cell/anaplastic medulloblastoma and isochromosome 17q - such tumors should be included in the "high risk" protocol.
authors: M V
authors: MV
authors: Ryzhova
authors: O G
authors: OG
authors: Zheludkova
authors: É V
authors: ÉV
authors: Kumirova
authors: L V
authors: LV
authors: Shishkina
authors: T N
authors: TN
authors: Panina
authors: S K
authors: SK
authors: Gorelyshev
authors: E A
authors: EA
authors: Khukhlaeva
authors: N A
authors: NA
authors: Mazerkina
authors: K B
authors: KB
authors: Matuev
authors: O A
authors: OA
authors: Medvedeva
authors: E M
authors: EM
authors: Tarasova
authors: B V
authors: BV
authors: Kholodov
authors: O Iu
authors: OIu
authors: Kapitul'skaia
mesh_terms: D002528:Cerebellar Neoplasms / Q000235:genetics* / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D002675:Child, Preschool; D002869:Chromosome Aberrations / Q000706:statistics & numerical data; D005260:Female; D016259:Genes, myc / Q000235:genetics; D006801:Humans; D017404:In Situ Hybridization, Fluorescence; D007223:Infant; D053208:Kaplan-Meier Estimate; D008297:Male; D008527:Medulloblastoma / Q000235:genetics* / Q000401:mortality / Q000473:pathology* / Q000601:surgery; D000071447:N-Myc Proto-Oncogene Protein; D009687:Nuclear Proteins / Q000096:biosynthesis; D015513:Oncogene Proteins / Q000096:biosynthesis
chemical_list: C459801:MYCN protein, human; D000071447:N-Myc Proto-Oncogene Protein; D009687:Nuclear Proteins; D015513:Oncogene Proteins