Malignant peripheral nerve sheath tumor of the parotid gland

Malignant peripheral nerve sheath tumors originating from the parotid gland are extremely rare. A 76-year-old male patient underwent an incisional biopsy for an ulcerated mass in the anteroinferior aspect of the left auricle. The diagnosis was made as malignant mesenchymal tissue sarcoma, but the patient refused treatment. Upon progressive growth of the mass within two months, he underwent a partial parotid gland resection. After three months, he was referred to our clinic with an aggressively growing parotid mass. Total parotidectomy and radical neck dissection were performed. Histopathological diagnosis was malignant peripheral nerve sheath tumor. Following radiotherapy, he has been under follow-up for five years.