Clinical outcome of extramedullary plasmacytoma

Background and Ojectives. The aim of this study was a retrospective analysi s of the presenting feb tures of extramedullary plasmacytoma, its response to therapy and its clinical course. Design and Methods. Forty-six cases diagnosed between August 1970 and June 1993 were carefully reviewed. The follow-up was continued until June;1998 a nd the median observation time was 118 months. Results. The disease was most frequently localized in the upper airways (37 /46; 80%), with the mass limited to a single site in all but seven patients in whom two contiguous sites were involved. Other localizations were the l ymph nodes, thyroid, skin, stomach, and brain. The clinical symptoms were r elated to the site of presentation, and the median time between appearance and diagnosis was 7.5 months. The median age at diagnosis was 55 years (ran ge 16-80), with 14 patients (30%) being under 50 years old. The disorder wa s approximately twice as common in males as in females. Ten patients (21%) had a monoclonal component. The therapeutic strategy varied, although the m ost frequent form :of treatment was local radiotherapy. Thirty-nine patient s (85%) achieved complete remission (CR), Rye (11%) a partial remission (PR ) and two (4%) did riot respond to therapy (NR). Local recurrence (LR) br r ecurrence at other sites (ROS) occurred in 7.5% and 10%, respectively. Seve n patients (15%) developed multiple myeloma (MM), characterized by multiple sites of osteolysis in almost all cases with soft tissue involvement in so me of them. The 15 year survival rate was 78%. Interpretation and Conclusions. This review of a relatively large series of patients confirms the favorable prognosis of EMP when treated locally by i rradiation and/or surgery. (C) 2000 Ferrata Storti Foundation.