Biphenotypic sarcoma with characteristics of both a Ewing sarcoma and a desmoplastic small round cell tumor.
Published
Journal Article
BACKGROUND: The EWS gene, a transcription factor of unknown function, is involved in chromosomal translocations associated with a wide variety of tumors, particularly small round blue cell tumors such as Ewing sarcoma. It has previously been reported that desmoplastic small round blue cell tumor (DSRBCT) frequently has an associated t(11;22) abnormality resulting from fusion of the EWS and WT-1 genes. PROCEDURE: We report a case of a small round blue cell tumor with characteristics of both Ewing sarcoma and DSRBCT with a t(11;22) translocation leading to fusion of the EWS and FLI1genes. RESULTS: The translocation point and fusion products were confirmed by polymerase chain reaction amplification and restriction fragment mapping of the products. CONCLUSIONS: The biphenotypic nature of this case and the apparent promiscuity of the EWS gene in tumor-associated translocations coupled with other reports of biphenotypic childhood sarcomas has potential implications for the relationship between small round blue cell tumors and the mechanism of EWS/FLI1 oncogenesis.
Full Text
Duke Authors
Cited Authors
- Rosoff, PM; Hatcher, S; West, DC
Published Date
- June 2000
Published In
Volume / Issue
- 34 / 6
Start / End Page
- 407 - 412
PubMed ID
- 10842247
Pubmed Central ID
- 10842247
International Standard Serial Number (ISSN)
- 0098-1532
Digital Object Identifier (DOI)
- 10.1002/(sici)1096-911x(200006)34:6<407::aid-mpo5>3.0.co;2-x
Language
- eng
Conference Location
- United States