NKX2.2 (EP336)

NKX2.2 is a homeodomain transcription factor that plays a role in neuroendocrine and glial differentiation.1 NKX2.2 is upregulated in Ewing’s sarcoma as a result of the oncogenic EWS-FLI1 fusion protein.2,3 As one of many small round blue cell tumors, Ewing’s sarcoma can be difficult to diagnose due to the characteristic undifferentiated morphology.4 NKX2.2 has proven its utility as a sensitive maker for distinguishing Ewing’s sarcoma from other round blue cell tumors when used in a panel.1,5