Objective: To summarize the clinical features of extraorbital inflammatory myofibroblastic tumor (IMT) of the head and neck.
Methods: Fourteen cases of extraorbital IMT treated in recent 20 years were analyzed retrospectively.
Results: Of the 14 patients, 9 cases with limited lesion in maxilla (n = 5), mandible (n = 2) or neck (n = 2) underwent local resection, and no recurrences were found after 1.5 to 20.0 years; 3 cases diagnosed as maxillary IMT involved in orbit, hard palate or pterygopalatine fossa received conservative therapy (prednisone, prednisone plus radiotherapy or prednisone plus chemotherapy), and no disease progression was found after 6, 9 or 2 years respectively; and 1 case diagnosed as maxillary IMT involved in orbit and pterygopalatine fossa was confirmed with cervical metastases after two operations and died of brain invasion within 17 months. One patient with localized lesion around the common carotid artery was treated with prednisone and had no disease progression with a 2-year follow-up.
Conclusions: Extraorbital IMT of the head and neck is a rare clinical entity. Pathology examination is required for final diagnosis. Corticosteroid administration may be a choice of treatments, and radical resection should be taken selectively for limited lesions.