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Detection of chimeric transcripts in desmoplastic small round cell tumor and related developmental tumors by reverse transcriptase polymerase chain reaction. A specific diagnostic assay.
Am J Pathol. 1995 Dec; 147(6):1584-91.AJ

Abstract

Desmoplastic small round cell tumor is a recently described entity associated with fusion of the EWS and WT1 genes and with expression of a chimeric transcript. To investigate the structure and potential diagnostic utility of the detection of EWS-WT1 chimeric RNA in desmoplastic small round cell tumor, 12 examples of this entity and 49 other tumors that enter in its differential diagnosis were studied by reverse transcriptase polymerase chain reaction for the presence of EWS-WT1, EWS-FLI-1, PAX3-FKHR, and PAX7-FKHR chimeric transcripts. EWS-WT1 was detected in 11 of 12 desmoplastic small round cell tumors but not in any other tumor type studied, including 17 Wilms' tumors, 10 Ewing's sarcomas/primitive neuroectodermal tumors, 13 alveolar rhabdomyosarcomas, and 9 embryonal rhabdomyosarcomas. One desmoplastic small round cell tumor was found to have a variant EWS-WT1 chimeric product that included exon 8 of EWS EWS-FLI-1 chimeric RNA was present in all Ewing's sarcoma/primitive neuroectodermal tumor and not identified in any other tumor types, including desmoplastic small round cell tumor. PAX3/PAX7-FKHR chimeras were present in 9 of 13 alveolar rhabdomyosarcomas but not in any other tumors. Detection of chimeric transcripts by reverse transcriptase polymerase chain reaction is a very specific aid in differential diagnosis of developmental tumors and further establishes desmoplastic small round cell tumor as a distinct entity.

Authors+Show Affiliations

Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, New York 10021, USA.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

7495283

Citation

de Alava, E, et al. "Detection of Chimeric Transcripts in Desmoplastic Small Round Cell Tumor and Related Developmental Tumors By Reverse Transcriptase Polymerase Chain Reaction. a Specific Diagnostic Assay." The American Journal of Pathology, vol. 147, no. 6, 1995, pp. 1584-91.
de Alava E, Ladanyi M, Rosai J, et al. Detection of chimeric transcripts in desmoplastic small round cell tumor and related developmental tumors by reverse transcriptase polymerase chain reaction. A specific diagnostic assay. Am J Pathol. 1995;147(6):1584-91.
de Alava, E., Ladanyi, M., Rosai, J., & Gerald, W. L. (1995). Detection of chimeric transcripts in desmoplastic small round cell tumor and related developmental tumors by reverse transcriptase polymerase chain reaction. A specific diagnostic assay. The American Journal of Pathology, 147(6), 1584-91.
de Alava E, et al. Detection of Chimeric Transcripts in Desmoplastic Small Round Cell Tumor and Related Developmental Tumors By Reverse Transcriptase Polymerase Chain Reaction. a Specific Diagnostic Assay. Am J Pathol. 1995;147(6):1584-91. PubMed PMID: 7495283.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Detection of chimeric transcripts in desmoplastic small round cell tumor and related developmental tumors by reverse transcriptase polymerase chain reaction. A specific diagnostic assay. AU - de Alava,E, AU - Ladanyi,M, AU - Rosai,J, AU - Gerald,W L, PY - 1995/12/1/pubmed PY - 1995/12/1/medline PY - 1995/12/1/entrez SP - 1584 EP - 91 JF - The American journal of pathology JO - Am J Pathol VL - 147 IS - 6 N2 - Desmoplastic small round cell tumor is a recently described entity associated with fusion of the EWS and WT1 genes and with expression of a chimeric transcript. To investigate the structure and potential diagnostic utility of the detection of EWS-WT1 chimeric RNA in desmoplastic small round cell tumor, 12 examples of this entity and 49 other tumors that enter in its differential diagnosis were studied by reverse transcriptase polymerase chain reaction for the presence of EWS-WT1, EWS-FLI-1, PAX3-FKHR, and PAX7-FKHR chimeric transcripts. EWS-WT1 was detected in 11 of 12 desmoplastic small round cell tumors but not in any other tumor type studied, including 17 Wilms' tumors, 10 Ewing's sarcomas/primitive neuroectodermal tumors, 13 alveolar rhabdomyosarcomas, and 9 embryonal rhabdomyosarcomas. One desmoplastic small round cell tumor was found to have a variant EWS-WT1 chimeric product that included exon 8 of EWS EWS-FLI-1 chimeric RNA was present in all Ewing's sarcoma/primitive neuroectodermal tumor and not identified in any other tumor types, including desmoplastic small round cell tumor. PAX3/PAX7-FKHR chimeras were present in 9 of 13 alveolar rhabdomyosarcomas but not in any other tumors. Detection of chimeric transcripts by reverse transcriptase polymerase chain reaction is a very specific aid in differential diagnosis of developmental tumors and further establishes desmoplastic small round cell tumor as a distinct entity. SN - 0002-9440 UR - https://www.unboundmedicine.com/medline/citation/7495283/Detection_of_chimeric_transcripts_in_desmoplastic_small_round_cell_tumor_and_related_developmental_tumors_by_reverse_transcriptase_polymerase_chain_reaction__A_specific_diagnostic_assay_ L2 - https://www.ncbi.nlm.nih.gov/pmc/articles/pmid/7495283/ DB - PRIME DP - Unbound Medicine ER -