Author/Authors :
Shimizu، نويسنده , , Yukitoshi and Mitsui، نويسنده , , Tetsuo and Kawakami، نويسنده , , Takako and Ikegami، نويسنده , , Tohru and Kanazawa، نويسنده , , Chikako and Katsuura، نويسنده , , Michihiko and Obata، نويسنده , , Kazuya and Yamagiwa، نويسنده , , Iwao and Hayasaka، نويسنده , , Kiyoshi، نويسنده ,
Abstract :
We report here a 15-year-old boy with an intraabdominal desmoplastic small round cell tumor (DSRCT). Cytogenetic analysis of the tumor cells showed the chromosomal translocation (11;22). Reverse-transcriptase polymerase chain reaction and sequencing analysis revealed a chimeric transcriptional message of the EWS gene exon 10 fused to the WT1 gene exon 8. The typical chimeric transcript seen in DSRCT is an in-frame fusion of EWS exon 7 to WT1 exon 8. The tumor in this case had a novel and longer chimeric transcript, which should be a potent transcription factor. Genetic analysis is a very powerful and specific aid in the differential diagnosis of small round cell tumors.
