Malignant myoepithelioma of the maxilla posing a diagnostic dilemma

Malignant myoepithelioma is an extremely rare tumor of the salivary glands. Myoepitheliomas make up less than 1% of all salivary gland tumors. We report of a case of malignant myoepithelioma of the upper jaw in a 55-year old man. The tumor primarily arose from the maxillary sinus and secondarily involved the palate. A diagnosis of pleomorphic adenoma was interpreted, following fine needle aspiration cytology and spindle cell tumor, following incision biopsy with a dilemma. The surgery consisted of subtotal maxillectomy preserving the infra orbital rim and posterior wall of the maxillary sinus, with wide local resection. Neck dissection was not carried out. The final histologic diagnosis of the tumor proper was made out to be malignant myoepithelioma. Three different diagnoses were made in dilemma to reach out the final diagnosis, which was confirmed by immunohistochemistry. The case presented and discussed, underscores the importance of diagnosis and treatment of this rare clinicopathologic entity.