Proximal-type epithelioid sarcoma in the maxillary sinus: an unusual presentation

Epithelioid sarcoma (ES) is a rare histological type of soft tissue sarcoma presenting as a subcutaneous or deep dermal mass in the distal extremities of young adults. Recently, a more aggressive, so called 'proximal-type' ES has been described. The literature is limited on the clinical features and management of ES originating in the head and neck area. We here report a case of 16-year-old female who initially presented with progressive swelling and pain in the left cheek. On physical and radiographic examination, a malignant neoplasm was found in the left maxillary sinus with bony invasion. The definite diagnosis of proximal-type ES was based on the pathological and immunohistochemical characteristics. A subtotal maxillectomy with wide margins was performed on this patient. The patient survived uneventfully for three years. This is the first report of a proximal-type ES found in the maxillary sinus.