Paranasal Sinus Malignant Peripheral Nerve Sheath Tumour: A Rare Case Report.

Abstract

Malignant Peripheral Nerve Sheath Tumour (MPNST) is a neoplastic lesion rarely reported in literature. In approximately 5% of cases it is associated with Neurofibromatosis type 1 syndrome. Pathognomic features of MPNST include slow growth rate, aggressive nature, nearly circumscribed, unencapsulated arising from non myelinated Schwann cells. In this case report we elaborate probable molecular pathogenesis, clinical features, histopathology (HPE) and radiological findings in a unique case of MPNST. A 52year old female patient presented with Right cheek swelling, Loss of sensations over right maxillary region, U/L nasal obstruction and watery nasal discharge, palatal buldge and intermittent pain over right maxillary region and generalised headache. Following Magnetic resonance imaging studies (MRI scan) of paranasal sinuses, Biopsy was taken from maxillary mass and palatal swelling. HPE report was suggestive of Spindle cell proliferation against myxoid stroma. Positron Emmision Tomography (PET-Scan) was done and Biopsy specimen was subjected for Immunohistochemistry staining (IHC). After confirmation of MPNST on IHC, patient was referred to skull base surgeon for complete excision of the tumour and reconstruction.