Background: The β chain of the interleukin 2/15 receptor (IL-2/15Rβ) is induced by the expression of the EWS–WT1. A case of desmoplastic small round cell tumour (DSRCT) expressing only an unusual EWS-WT1 treated by us is reported here.
Aim: To characterise an unusual form of EWS–WT1.
Methods: Frozen tissue sections of the axillary tumour were examined using a laser-assisted microdissection technique and reverse transcriptase polymerase chain reaction.
Results: The novel fusion of exon 8 of EWS and the defective exon 10 of WT1 (−KTS) was detected. Although it was an unusual form, the coexpression of the present EWS–WT1, IL-2/15Rβ and Janus kinase (JAK1) mRNA was detected in the tumour cells. IL-2 and signal transducers and activators of transcription (STAT5) mRNA were detected in both tumour and stromal cells.
Conclusion: The induction of the IL-2/15 receptor signalling pathway may contribute to tumorigenesis in DSRCT through a paracrine or an autocrine system, even though the EWS–WT1 was an unusual form.
- DSRCT, desmoplastic small round cell tumour
- EWS, Ewing’s sarcoma gene
- IL-2/15Rβ, interleukin 2/15 receptor β chain
- JAK, Janus kinase
- RT-PCR, reverse transcriptase polymerase chain reaction
- STAT5, signal transducers and activators of transcription
- WT1, Wilms’s tumour gene
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Competing interests: None declared.
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