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Biphenotypic sarcoma with characteristics of both a Ewing sarcoma and a desmoplastic small round cell tumor.

Publication ,  Journal Article
Rosoff, PM; Hatcher, S; West, DC
Published in: Med Pediatr Oncol
June 2000

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.

Duke Scholars

Published In

Med Pediatr Oncol

DOI

ISSN

0098-1532

Publication Date

June 2000

Volume

34

Issue

6

Start / End Page

407 / 412

Location

United States

Related Subject Headings

  • Translocation, Genetic
  • Tomography, X-Ray Computed
  • Sarcoma, Ewing
  • Sarcoma
  • Reverse Transcriptase Polymerase Chain Reaction
  • Photomicrography
  • Phenotype
  • Oncology & Carcinogenesis
  • Humans
  • Fibromatosis, Aggressive
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Rosoff, P. M., Hatcher, S., & West, D. C. (2000). Biphenotypic sarcoma with characteristics of both a Ewing sarcoma and a desmoplastic small round cell tumor. Med Pediatr Oncol, 34(6), 407–412. https://doi.org/10.1002/(sici)1096-911x(200006)34:6<407::aid-mpo5>3.0.co;2-x
Rosoff, P. M., S. Hatcher, and D. C. West. “Biphenotypic sarcoma with characteristics of both a Ewing sarcoma and a desmoplastic small round cell tumor.Med Pediatr Oncol 34, no. 6 (June 2000): 407–12. https://doi.org/10.1002/(sici)1096-911x(200006)34:6<407::aid-mpo5>3.0.co;2-x.
Rosoff PM, Hatcher S, West DC. Biphenotypic sarcoma with characteristics of both a Ewing sarcoma and a desmoplastic small round cell tumor. Med Pediatr Oncol. 2000 Jun;34(6):407–12.
Rosoff, P. M., et al. “Biphenotypic sarcoma with characteristics of both a Ewing sarcoma and a desmoplastic small round cell tumor.Med Pediatr Oncol, vol. 34, no. 6, June 2000, pp. 407–12. Pubmed, doi:10.1002/(sici)1096-911x(200006)34:6<407::aid-mpo5>3.0.co;2-x.
Rosoff PM, Hatcher S, West DC. Biphenotypic sarcoma with characteristics of both a Ewing sarcoma and a desmoplastic small round cell tumor. Med Pediatr Oncol. 2000 Jun;34(6):407–412.

Published In

Med Pediatr Oncol

DOI

ISSN

0098-1532

Publication Date

June 2000

Volume

34

Issue

6

Start / End Page

407 / 412

Location

United States

Related Subject Headings

  • Translocation, Genetic
  • Tomography, X-Ray Computed
  • Sarcoma, Ewing
  • Sarcoma
  • Reverse Transcriptase Polymerase Chain Reaction
  • Photomicrography
  • Phenotype
  • Oncology & Carcinogenesis
  • Humans
  • Fibromatosis, Aggressive