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Primitive neuroectodermal tumour of the tongue

Abstract Peripheral primitive neuroectodermal tumours, which belong to the Ewing sarcoma tumour family, are extremely rare. These tumours are highly aggressive and are known to have a poor prognosis. Immunostaining with at least two neural markers and evidence of an abnormal t(11;22)(q24;q12) translocation are hallmark features in this diagnosis. We present the first reported case of peripheral primitive neuroectodermal tumour to occur in the tongue.