Plasmacytomas occasionally may arise in extraosseous sites, of which the upper respiratory tract is the most common. In our series of 256 nonepithelial tumors involving the nasal cavity, paranasal sinuses and nasopharynx, 10 (4%) were apparently primary extramedullary plasmacytomas. Of our patients with adequate follow-up information, four died of disseminated disease 2, 4, 5 and 6 years after the initial diagnosis. One was alive 6 years after diagnosis with locally persistent tumor. Another was living without recurrence 12 years later. Based on our findings and on the cases reported in the literature, extramedullary plasmacytomas arising in the head and neck area may evolve into one of several different patterns. 1) Some patients have localized disease which is apparently controlled (by surgery, radiotherapy or both) and which never recurs locally or becomes disseminated. 2) In some cases, the tumor recurs locally and is controlled by further therapy. 3) Other patients have a locally persistent and aggressive lesion which cannot be eradicated and which eventually leads to the patient's death by uncontrolled local growth. 4) Still other patients eventually develop evidence of plasma cell neoplasms elsewhere in the body and/or multiple myeloma.