PMID- 23659115 OWN - NLM STAT- MEDLINE DCOM- 20130625 LR - 20180404 IS - 0042-8817 (Print) IS - 0042-8817 (Linking) VI - 77 IP - 1 DP - 2013 TI - Characteristics of medulloblastoma in children under age of three years. PG - 3-10; discussion 11 AB - 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. FAU - Ryzhova, M V AU - Ryzhova MV FAU - Zheludkova, O G AU - Zheludkova OG FAU - Kumirova, E V AU - Kumirova EV FAU - Shishkina, L V AU - Shishkina LV FAU - Panina, T N AU - Panina TN FAU - Gorelyshev, S K AU - Gorelyshev SK FAU - Khukhlaeva, E A AU - Khukhlaeva EA FAU - Mazerkina, N A AU - Mazerkina NA FAU - Matuev, K B AU - Matuev KB FAU - Medvedeva, O A AU - Medvedeva OA FAU - Tarasova, E M AU - Tarasova EM FAU - Kholodov, B V AU - Kholodov BV FAU - Kapitul'skaia, O Iu AU - Kapitul'skaia OIu LA - eng LA - rus PT - Journal Article PL - Russia (Federation) TA - Zh Vopr Neirokhir Im N N Burdenko JT - Zhurnal voprosy neirokhirurgii imeni N. N. Burdenko JID - 7809757 RN - 0 (MYCN protein, human) RN - 0 (N-Myc Proto-Oncogene Protein) RN - 0 (Nuclear Proteins) RN - 0 (Oncogene Proteins) SB - IM MH - Cerebellar Neoplasms/*genetics/mortality/*pathology/surgery MH - Child, Preschool MH - Chromosome Aberrations/statistics & numerical data MH - Female MH - Genes, myc/genetics MH - Humans MH - In Situ Hybridization, Fluorescence MH - Infant MH - Kaplan-Meier Estimate MH - Male MH - Medulloblastoma/*genetics/mortality/*pathology/surgery MH - N-Myc Proto-Oncogene Protein MH - Nuclear Proteins/biosynthesis MH - Oncogene Proteins/biosynthesis EDAT- 2013/05/11 06:00 MHDA- 2013/06/26 06:00 CRDT- 2013/05/11 06:00 PHST- 2013/05/11 06:00 [entrez] PHST- 2013/05/11 06:00 [pubmed] PHST- 2013/06/26 06:00 [medline] PST - ppublish SO - Zh Vopr Neirokhir Im N N Burdenko. 2013;77(1):3-10; discussion 11.