Publication:

An unusual case of maxillary sinus involvement by a malignant peripheral nerve sheath tumour

Malignant peripheral nerve sheath tumours (MPNSTs) are uncommon aggressive soft-tissue sarcomas, accounting for approximately 5–10% of all sarcomas. Their occurrence in the head and neck region is rare, and involvement of the paranasal sinuses, particularly the maxillary sinus is exceedingly uncommon. Here we report a case of a middle-aged patient who presented with pain and swelling over left cheek, hard palate and nasal obstruction since 3 months. Clinical examination revealed a spherical swelling of size 3×2 cm present over left cheek with indistinct borders, another spherical swelling of size 3×2 cm on left side of hard palate. Patient underwent multidisciplinary evaluation, leading to surgical resection. Histopathological examination proved as low grade malignant peripheral nerve sheath tumour with Immunohistochemistry positivity to Vimentin. Patient underwent adjuvant radiotherapy and post-treatment follow-up showed no evidence of recurrence. This case emphasizes the significance of a broad differential, thorough histopathologic assessment, and coordinated multidisciplinary care to optimize outcomes in such rare presentations. Limited cases in the literature make diagnosis and management particularly challenging.