Document Detail


Extrarenal rhabdoid tumors of soft tissue: clinicopathological and molecular genetic review and distinction from other soft-tissue sarcomas with rhabdoid features.
MedLine Citation:
PMID:  16704491     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
Yoshinao Oda; Masazumi Tsuneyoshi
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Pathology international     Volume:  56     ISSN:  1320-5463     ISO Abbreviation:  Pathol. Int.     Publication Date:  2006 Jun 
Date Detail:
Created Date:  2006-05-17     Completed Date:  2006-08-10     Revised Date:  2006-11-15    
Medline Journal Info:
Nlm Unique ID:  9431380     Medline TA:  Pathol Int     Country:  Australia    
Other Details:
Languages:  eng     Pagination:  287-95     Citation Subset:  IM    
Affiliation:
Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan. oda@surgpath.med.kyushu-u.ac.jp
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MeSH Terms
Descriptor/Qualifier:
Child
Child, Preschool
Chromosomal Proteins, Non-Histone
Cytogenetics
DNA-Binding Proteins / analysis,  genetics
Diagnosis, Differential
Humans
Infant
Molecular Biology
Mutation
Rhabdoid Tumor / chemistry,  genetics,  pathology*
Sarcoma / chemistry,  genetics,  pathology*
Soft Tissue Neoplasms / chemistry,  genetics,  pathology*
Transcription Factors / analysis,  genetics
Tumor Markers, Biological / analysis
Chemical
Reg. No./Substance:
0/Chromosomal Proteins, Non-Histone; 0/DNA-Binding Proteins; 0/SMARCB1 protein, human; 0/Transcription Factors; 0/Tumor Markers, Biological

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