Malignant nerve sheath tumors are uncommon and account for <10% of all sarcomas. They are rare high-grade sarcomas arising from peripheral nerves or cells of the peripheral nerve sheath. Its incidence is even uncommon and rare in salivary gland, especially parotid gland. We hereby report a case of Malignant Peripheral Nerve Sheath tumor (MPNST) of Parotid Gland. A 40 years old male presented with history of parotid swelling of 15 years duration and rapid increase in size since one year. Patient underwent surgery and specimen was sent for histopathological examination. Grossly, the specimen was encapsulated and well circumscribed. Cut surface showed two nodular greyish white areas with necrosis. Histomorphology showed a spindle cell neoplasm with features of malignant peripheral nerve sheath tumor. Neural origin of the tumor was confirmed by immunohistochemistry. The patient is under follow up and disease free. We report this case due to its rarity and also to make aware the surgical pathologists to keep MPNST as a differential diagnosis in the evaluation of malignant spindle cell neoplasm of parotid and there is an important role of immunohistochemistry in reaching a diagnosis.
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