Vol. 8, Issue 4, Part A (2025)

Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, is a highly aggressive cancer that develops from mesenchymal tissues. Although it most often appears in soft tissue-and occasionally in bone-it is rarely seen in the oral cavity and is extremely uncommon in the maxillary sinus. Histopathological diagnosis of UPS in this region is often difficult due to its varied microscopic appearance, which can closely mimic a number of other benign and malignant lesions.
In this paper, we report a case of undifferentiated pleomorphic sarcoma in the maxillary sinus of a 32-year-old Indian man who was referred to the Otorhinolaryngology department of JNMCH, AMU, Aligarh due to severe pain on right side of face, severe headache and right nasal obstruction. Functional endoscopic sinus surgery was done and in biopsy the diagnosis of spindle cell neoplasm was made. Tumor was excised in ENT department and using a routine histopathological and immuno-histochemical workup, a diagnosis of undifferentiated pleomorphic sarcoma was rendered. This case report emphasises on the diagnostic approach to undifferentiated pleomorphic sarcoma.