Abstract

Primitive neuroectodermal tumours (PNETs) belong to the Ewing’s sarcoma family of tumours and are composed of small round cells. Since their incidence is rare, their management is challenging. Diagnosis is based on radiology, histopathology, immunohistochemistry, cytogenetics and ultrastructural studies. In this case report, we present a unique case of a right maxillary tumour, which later turned out to be a PNET. Management and controversies in dealing with this tumour are discussed in detail.

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