Journal of Cytology
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Year : 2015  |  Volume : 32  |  Issue : 1  |  Page : 30-32

Upregulation of NKX2.2, a target of EWSR1/FLI1 fusion transcript, in primary renal Ewing sarcoma

Department of Pathology, Teikyo University, Chiba Medical Center, Ichihara, Chiba, Japan

Correspondence Address:
Dr. Kazuto Yamazaki
3426-3, Anesaki, Ichihara, Chiba, 299-0111
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0970-9371.155229

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Renal Ewing sarcoma (ES) is a rare malignant tumor characterized by fusion of the EWSR1 gene with a member of the ETS family of oncogenes, arising at a specific chromosomal translocation. Diagnosis of ES can be problematic, especially from cytological or small bioptical specimens because the differential diagnoses comprising a diverse group of small round blue cell tumors (SRBCTs). We report a case of primary renal ES in a young male, which had a t(11;22) (q24;q12) chromosome translocation encoding a type2 EWSR1/FLI1 fusion transcript. The tumor cells showed diffuse cytoplasmic immunoreactivity for CD99 and diffuse nuclear immunoreactivity for NKX2.2, an important oncogenic transcriptional target of EWSR1/FLI1, not only in the histological, but also in the cytological specimens. From the results of this case, we speculate that NKX2.2, in combination with CD99, may be a useful immunocytochemical marker to distinguish renal ES from other SRBCTs of kidney.

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