title: Recurrent extramedullary plasmacytoma in asymptomatic multiple myeloma: a case report.
abstract: BACKGROUND
  The gross majority of extramedullary plasmacytomas arise in the lymphatic tissue of the upper respiratory tract. On average, one third of patients with a solid plasmacytoma will develop multiple myeloma, resulting in a worse clinical outcome. We describe a case of rapid recurrent extramedullary plasmacytomas in the background of an asymptomatic multiple myeloma.
  
  
  METHODS
  A 71-year-old, white Caucasian woman presented with three extramedullary plasmacytomas occurring within a short time period. The third plasmacytoma was accompanied by progressive cervical pain and swallow dysfunction. Additional immunostaining test results were negative for CD56 and showed high MIB-1 expression in the extramedullary plasmacytoma and low MIB-1 expression in the bone marrow. A conventional swallow X-ray did not show any obstruction, however a magnetic resonance imaging scan of her cervical backbone revealed an extramedullary plasmacytoma, threatening her spinal cord. A short course of radiation therapy alleviated her pain and during almost a two-year follow-up period, the multiple myeloma remained asymptomatic, despite the rise in immunoglobulin A lambda levels. After the appearance of the third plasmacytoma, systemic chemotherapy was started to prevent the development of a fourth plasmacytoma, despite the asymptomatic character of the multiple myeloma.
  
  
  CONCLUSIONS
  In this case report we describe the rapid appearance of extramedullary plasmacytomas in the background of an asymptomatic multiple myeloma. An immunohistochemical analysis was negative for CD56 and showed high MIB-1 expression in the extramedullary plasmacytoma and low MIB-1 expression in the bone marrow, contributing to the potential underlying pathophysiology of the recurrent extramedullary plasmacytomas and their genetic changes. Systemic chemotherapy was started and no fourth extramedullary plasmacytoma has developed since.
authors.affiliation: Department of Hematology, Internal Medicine, Maastricht University Medical Center, Debyelaan 25, 6229 HX, Maastricht, The Netherlands. saskia.schols@mumc.nl.
authors.forename: Saskia E M
authors.initials: SE
authors.lastname: Schols
authors.affiliation: Department of Internal Medicine, Máxima Medical Centre, De Run 4600, 5504 DB, Veldhoven, The Netherlands. l.tick@mmc.nl.
authors.forename: Lidwine L W
authors.initials: LL
authors.lastname: Tick
mesh_terms: D000368:Aged; D000970:Antineoplastic Agents / Q000627:therapeutic use; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008279:Magnetic Resonance Imaging; D008558:Melphalan / Q000627:therapeutic use; D009101:Multiple Myeloma / Q000175:diagnosis* / Q000628:therapy; D009364:Neoplasm Recurrence, Local; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis* / Q000628:therapy; D010954:Plasmacytoma / Q000175:diagnosis* / Q000628:therapy; D011241:Prednisone / Q000627:therapeutic use
chemical_list: D000970:Antineoplastic Agents; D007166:Immunosuppressive Agents; D008558:Melphalan; D011241:Prednisone