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Coexpression of an unusual form of the EWS–WT1 fusion transcript and interleukin 2/15 receptor ßmRNA in a desmoplastic small round cell tumour

¹ Department of Pathology, Nihon University School of Medicine, Tokyo, Japan
² Pathology Laboratory, Nihon University Itabashi Hospital, Tokyo
³ Department of Thoracic Surgery, Nihon University School of Medicine

Correspondence to:
Dr N Nemoto
Pathology Laboratory, Nihon University Itabashi Hospital, 30-1 Oyaguchi-kamimachi Itabasi-ku, Tokyo 173-8610, Japan; nemo{at}med.nihon-u.ac.jp
ABSTRACT
Background: The ß chain of the interleukin 2/15 receptor (IL-2/15Rß) 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-2/15Rß 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.

Abbreviations: DSRCT, desmoplastic small round cell tumour; EWS, Ewing’s sarcoma gene; IL-2/15Rß, interleukin 2/15 receptor ß chain; JAK, Janus kinase; RT-PCR, reverse transcriptase polymerase chain reaction; STAT5, signal transducers and activators of transcription; WT1, Wilms’s tumour gene