Extramedullary Plasmacytomas of the Head and Neck

Abstract

Extramedullary plasmacytomas are solitary tumors consisting of neoplastic plasma cell proliferations that occur in locations other than bone. On initial presentation they must be differentiated from multiple myeloma. This may prove to be difficult because a varying percentage may be associated at a later date with the development of multiple myeloma. Solitary extramedullary plasmacytomas represent up to 4% of nonepithelial lesions of the upper respiratory tract. From 1970 to 1990, at West Virginia University Hospitals, seven patients with solitary extramedullary plasmacytoma were identified. In four of these patients the tumor was located in the head and neck, with one tumor located in each of the following sites: temporoparietal scalp, maxillary sinus, nasopharynx, and cervical region. One patient had extensive destruction of the temporal bone, with extension intracranially to the middle cranial fossa. No patient had multiple myeloma, nor did any develop. Diagnosis was based on a combination of histology along with special immunoperoxidase staining for Ig lambda and kappa light chains. This will be demonstrated and discussed. Treatment consisted of radiotherapy in three cases, with doses ranging from 3175 to 6000 rad. One patient, treated with surgical excision, experienced a relapse at a distant site 6 years later. All patients have maintained local control and have been followed for a minimum of 1 1/2 years, with an average of 3 years. We describe our experience with these tumors and present a pertinent review of the literature. While these tumors may present as aggressive locally destructive lesions, their management should be as organ-sparing as possible because excellent control can be achieved in the majority of cases. Patients should be followed for life because multiple myeloma may develop in a significant number.