Linked Life Data

16704491

Source:http://linkedlifedata.com/resource/pubmed/id/16704491

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
6
pubmed:dateCreated
2006-5-17
pubmed:abstractText
Malignant rhabdoid tumor (MRT) of the soft tissue is a rare and highly aggressive tumor that occurs in infancy or childhood. It predominantly involves a deep axial location such as the neck or paraspinal region. Microscopically, the tumor is composed of a diffuse proliferation of rounded or polygonal cells with eccentric nuclei, prominent nucleoli and glassy eosinophilic cytoplasm containing hyaline-like inclusion bodies, arranged in sheets and nests. These characteristic 'rhabdoid cells' are also present in certain soft-tissue sarcomas such as synovial sarcoma, extraskeletal myxoid chondrosarcoma and leiomyosarcoma. The existence of rhabdoid cells in these other sarcomas is correlated with a worse prognosis for the patients. Cytogenetic and molecular analyses have shown abnormalities in the long arm of chromosome 22 and alteration of the hSNF5/INI1 (SMARCB1) gene in renal, extrarenal and intracranial MRT. This gene alteration has been considered to be a specific molecular event in MRT, but a recent study has also demonstrated frequent alteration of this gene in proximal-type epithelioid sarcoma (ES). Both MRT of soft tissue and proximal-type ES show immunoreactivity for vimentin, cytokeratin and epithelial membrane antigen. The tumor cells of proximal-type ES are also occasionally positive for CD34 and beta-catenin, whereas MRT of soft tissue has no immunoreaction for these markers. Detailed clinicopathological and immunohistochemical evaluations are necessary to distinguish MRT of soft tissue from proximal-type ES, because these tumors demonstrated a similar morphology and the same gene alteration.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jun
pubmed:issn
1320-5463
pubmed:author
pubmed:issnType
Print
pubmed:volume
56
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
287-95
pubmed:dateRevised
2006-11-15
pubmed:meshHeading
pubmed:year
2006
pubmed:articleTitle
Extrarenal rhabdoid tumors of soft tissue: clinicopathological and molecular genetic review and distinction from other soft-tissue sarcomas with rhabdoid features.
pubmed:affiliation
Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. oda@surgpath.med.kyushu-u.ac.jp
pubmed:publicationType
Journal Article, Review, Research Support, Non-U.S. Gov't