Primary extramedullary plasmacytoma of the salivary glands.

Primary extramedullary plasmacytomas (PEMP) are uncommon plasma cell neoplasms that generally occur in the submucosal tissue of the upper airway. Salivary gland PEMP is an extremely rare condition. This report describes a unique case of PEMP in the parotid gland associated with pulmonary amyloidosis. A review of all salivary gland PEMP's suggests that they behave similarly to PEMP's in other locations. The treatment of choice for localized disease is radiotherapy which should include regional lymphatics. Local control can be achieved in the majority of cases. The minority of patients develop systemic metastases and die from their disease. Chemotherapy can control disseminated disease and may induce remission.