A Rare Case of Primitive Neuroectodermal Tumor of Tongue.

Abstract

Primitive neuroectodermal tumor of the periphery is a rare entity and belongs to Ewing sarcoma family of tumors. It comprises 1% of all sarcomas and majority of these cases occur in the age group of less than 35 years. It is highly aggressive and patient often presents with distant metastasis leading to poor prognosis. Its characteristic features include t(11:22) translocation. A young female patient presented with growth in the tip of the tongue. Biopsy was done and histopathology using H&E and PAS staining revealed primitive neuroectodermal tumor. Immunohistochemistry helped to confirm the diagnosis. There was no evidence of distant metastasis at the time of presentation. The most accepted modality of treatment is wide surgical excision followed by chemoradiotherapy. The growth was excised and patient was treated with radiotherapy. The patient succumbed to liver metastasis following 10 months of treatment. Peripheral PNET usually affects the long bones like femur and in head and neck region, mandible is most frequently involved. This is a rare case of PNET of tongue with very few references from literature and hence makes it an interesting read.