Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
3
pubmed:dateCreated
2004-9-27
pubmed:abstractText
Cerebellar liponeurocytoma, a rare, newly identified CNS neoplasm of adults, is characterized by advanced neuronal/neurocytic and focal lipomatous differentiation, low proliferative potential and a favorable clinical prognosis. Despite the different age distribution and benign biological behavior, the cerebellar liponeurocytoma shares several features with the cerebellar medulloblastoma, which may include an origin from the periventricular matrix of the fourth ventricle or the external granular layer of the cerebellum. To establish the genetic profile of cerebellar liponeurocytomas, we have formed an international consortium and collected tumor samples from 20 patients. DNA sequencing revealed TP53 missense mutations in 4 (20%) of 20 cerebellar liponeurocytomas, a frequency higher than in medulloblastomas. There was no case with PTCH, APC, or beta-catenin mutations, each of which may be present in subsets of medulloblastomas. Isochromosome 17q, a genetic hallmark of classic medulloblastomas, was not observed in any of the cases investigated by FISH analysis. cDNA array analyses were carried out on 4 cerebellar liponeurocytomas, 4 central neurocytomas, and 4 classic medulloblastomas. Cluster analysis of the cDNA expression data of 1176 genes grouped cerebellar liponeurocytomas close to central neurocytomas, but distinct from medulloblastomas. These results suggest cerebellar liponeurocytoma as a distinct tumor entity that is genetically different from medulloblastoma. Furthermore, the cDNA expression array data suggest a relationship to central neurocytomas, but the presence of TP53 mutations, which are absent in central neurocytomas, suggests that their genetic pathways are different.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jul
pubmed:issn
1015-6305
pubmed:author
pubmed-author:BergmannMarkusM, pubmed-author:BrandnerSebastianS, pubmed-author:ChimelliLeilaL, pubmed-author:ContiRenatoR, pubmed-author:DavisDaron GDG, pubmed-author:Del BigioMarc RMR, pubmed-author:EllisonDavid WDW, pubmed-author:GiangasperoFeliceF, pubmed-author:González-CámporaRicardoR, pubmed-author:HaraAkiraA, pubmed-author:HeppnerFrank LFL, pubmed-author:HorstmannSonjaS, pubmed-author:HuangHerveH, pubmed-author:JacksonThadT, pubmed-author:KleihuesPaulP, pubmed-author:MarkesberyWilliam RWR, pubmed-author:MasuokaJunJ, pubmed-author:MontagnaNádiaN, pubmed-author:OhgakiHirokoH, pubmed-author:PerryArieA, pubmed-author:QianJiangJ, pubmed-author:RadhakrishnanV VVV, pubmed-author:RainovNikolai GNG, pubmed-author:ReifenbergerGuidoG, pubmed-author:SöylemezogluFigenF, pubmed-author:TaddeiGian LGL, pubmed-author:Uro-CosteEmmanuelleE, pubmed-author:WellerRoy ORO
pubmed:issnType
Print
pubmed:volume
14
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
281-9
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
2004
pubmed:articleTitle
Genetic and expression profiles of cerebellar liponeurocytomas.
pubmed:affiliation
International Agency for Research on Cancer, Lyon, France.
pubmed:publicationType
Journal Article, Comparative Study, Research Support, Non-U.S. Gov't, Multicenter Study