Source:http://linkedlifedata.com/resource/pubmed/id/19915490
Switch to
| Predicate | Object |
|---|---|
| rdf:type | |
| lifeskim:mentions | |
| pubmed:issue |
12
|
| pubmed:dateCreated |
2009-11-26
|
| pubmed:abstractText |
Atypical teratoid/rhabdoid tumors are malignant embryonal tumors characterized by the presence of rhabdoid cells, genetic alterations affecting the SMARCB1 gene (hSNF5/INI1), and a poor prognosis. Whether INI1 plays a role in the pathogenesis of other central nervous system tumors is uncertain. We report on cases of 2 young children with unusual intracranial nonrhabdoid neuroectodermal tumors within and around the third or fourth ventricle that are characterized by cribriform strands and trabeculae and well-defined epithelial membrane antigen-immunopositive surfaces and show INI1 protein loss. Histological and immunohistochemical features did not correspond to established tumor types, including atypical teratoid/rhabdoid tumors, medulloepithelioma, choroid plexus carcinoma, and ependymoma. Fluorescence in situ hybridization analyses failed to identify chromosomal alterations affecting the SMARCB1 locus, but sequencing revealed a homozygous 4-bp duplication in exon 4 (492duplCCTT) in one of the tumors. Both children responded well to conventional adjuvant therapy protocols and are alive and in complete remission longer than 5 years postoperatively. We suggest that cribriform neuroepithelial tumor (CRINET) is a nonrhabdoid ventricular tumor that shows loss of tumoral INI1 protein and has a relatively favorable prognosis.
|
| pubmed:language |
eng
|
| pubmed:journal | |
| pubmed:citationSubset |
IM
|
| pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Chromosomal Proteins, Non-Histone,
http://linkedlifedata.com/resource/pubmed/chemical/DNA-Binding Proteins,
http://linkedlifedata.com/resource/pubmed/chemical/SMARCB1 protein, human,
http://linkedlifedata.com/resource/pubmed/chemical/Transcription Factors
|
| pubmed:status |
MEDLINE
|
| pubmed:month |
Dec
|
| pubmed:issn |
0022-3069
|
| pubmed:author |
pubmed-author:BergmannMarkusM,
pubmed-author:FrühwaldMichael CMC,
pubmed-author:GeskStefanS,
pubmed-author:HasselblattMartinM,
pubmed-author:KosturEE,
pubmed-author:OyenFlorianF,
pubmed-author:PaulusWernerW,
pubmed-author:SchmidHansjörgH,
pubmed-author:SchneppenheimReinhardR,
pubmed-author:SiebertReinerR,
pubmed-author:WredeBrigitteB
|
| pubmed:issnType |
Print
|
| pubmed:volume |
68
|
| pubmed:owner |
NLM
|
| pubmed:authorsComplete |
Y
|
| pubmed:pagination |
1249-55
|
| pubmed:meshHeading |
pubmed-meshheading:19915490-Antineoplastic Combined Chemotherapy Protocols,
pubmed-meshheading:19915490-Brain Neoplasms,
pubmed-meshheading:19915490-Child, Preschool,
pubmed-meshheading:19915490-Chromosomal Proteins, Non-Histone,
pubmed-meshheading:19915490-Combined Modality Therapy,
pubmed-meshheading:19915490-DNA-Binding Proteins,
pubmed-meshheading:19915490-Female,
pubmed-meshheading:19915490-Fourth Ventricle,
pubmed-meshheading:19915490-Humans,
pubmed-meshheading:19915490-Immunohistochemistry,
pubmed-meshheading:19915490-In Situ Hybridization, Fluorescence,
pubmed-meshheading:19915490-Infant,
pubmed-meshheading:19915490-Male,
pubmed-meshheading:19915490-Neoplasms, Neuroepithelial,
pubmed-meshheading:19915490-Neurosurgical Procedures,
pubmed-meshheading:19915490-Prognosis,
pubmed-meshheading:19915490-Radiotherapy,
pubmed-meshheading:19915490-Third Ventricle,
pubmed-meshheading:19915490-Transcription Factors
|
| pubmed:year |
2009
|
| pubmed:articleTitle |
Cribriform neuroepithelial tumor (CRINET): a nonrhabdoid ventricular tumor with INI1 loss and relatively favorable prognosis.
|
| pubmed:affiliation |
Institute of Neuropathology, University Hospital Münster, Münster, Germany. hasselblatt@uni-muenster.de
|
| pubmed:publicationType |
Journal Article,
Case Reports,
Research Support, Non-U.S. Gov't
|