AUTHORS' REPLY

✍ Scribed by McMANUS, AIDAN P.; GUSTERSON, BARRY A.; PINKERTON, C. ROSS; SHIPLEY, JANET M.

• Publisher: John Wiley and Sons
• Year: 1996
• Tongue: English
• Weight: 113 KB
• Volume: 180
• Category: Article
• ISSN: 0022-3417
• DOI: 10.1002/(sici)1096-9896(199612)180:4<462::aid-path695>3.0.co;2-v

✦ Synopsis

We have read with some interest the recent review of the molecular pathology of small round cell tumours by McManus et al.' In the section on desmoplastic small round cell tumour (DSRCT), the authors report that the EWS/WTI gene fusion has been described in a case of undifferentiated carcinoma of unknown primary site.2 We should note that in the same article the final pathological diagnosis provided was DSRCT. Recent studies by us and others have confirmed the high specificity and sensitivity of the EWS/WTI gene fusion for DSRCT.3-7 In aggregate, I1 of 12 DSRCTs tested have shown the EWS/WTI gene fusion, compared to none of 125 various other primitive paediatric sarcomas. The single negative case of DSRCT may well have been due to lack of tuinour in the sample submitted for molecular analysis. We have also detected the EWSIWTI gene fusion in another 12 unpublished cases of DSRCT. The data thus support the high specificity of this gene fusion for DSRCT.