Multiple myeloma (MM) is a hematological disease characterized by the malignant proliferation of plasma cells. MM can be concomitant with plasmacytoma, at diagnosis or during relapse. Extramedullary plasmacytoma (EMP) is rare and is encountered most frequently in the upper respiratory tract and nasopharynx. It is of much less frequent occurrence in the intraabdominal and thoracic regions. EMPs tend to have a poor prognosis with a characteristic of high relapse/refractory disease rates and a relatively short overall survival, despite the use of various novel medications. Here, we present a case of relapse of MM concomitant with a large EMP surrounding the aorta, which is an extremely rare pattern of involvement. Our case showed nearly complete remission with an aggressive chemotherapy regimen. MR imaging served as a guidepost in both the diagnosis and the post-treatment follow-up.
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1. Kyle RA, Rajkumar SV. Multiple myeloma. Blood. 2008;111:2962-72.
2. Blade J, Fernandez de Larrea C, Rosinol L, et al. Soft-tissue plasmacytomas in multiple myeloma: incidence, mechanisms of extramedullary spread, and treatment approach. J Clin Oncol. 2011;29:3805-12.
3. Sunnetcioglu A, Ekin S, Bayram I, et al. Endobronchial plasmacytoma in patient with multiple myeloma. Clin Respir J. 2017;11:1057-9.
4. Weinstock M, Ghobrial IM. Extramedullary multiple myeloma. Leukemia & lymphoma. 2013;54:1135-41.
5. Demircioğlu S, Sönmez GM, Dogan A, et al. Incidence of extramedullary myeloma in multiple myeloma patients. Van Med J. 2019;26:337-41.
6. Ghimire KB, Rajkumar SV, Dispenzieri A, et al. Incidence and Survival Outcomes Of Extramedullary Myeloma. Blood. 2013;122:3141.
7. Usmani SZ, Heuck C, Mitchell A, et al. Extramedullary disease portends poor prognosis in multiple myeloma and is over-represented in high-risk disease even in the era of novel agents. Haematologica. 2012;97:1761-7.
8. Gerry D, Lentsch EJ. Epidemiologic evidence of superior outcomes for extramedullary plasmacytoma of the head and neck. Otolaryngol Head Neck Surg. 2013;148:974-81.
9. Ooi GC, Chim JC, Au WY, et al. Radiologic manifestations of primary solitary extramedullary and multiple solitary plasmacytomas. AJR Am J Roentgenol. 2006;186:821-7.
10. Weinstock M, Aljawai Y, Morgan EA, et al. Incidence and clinical features of extramedullary multiple myeloma in patients who underwent stem cell transplantation. Br J Haematol. 2015;169:851-8.
11. Varettoni M, Corso A, Pica G, et al. Incidence, presenting features and outcome of extramedullary disease in multiple myeloma: a longitudinal study on 1003 consecutive patients. Ann Oncol. 2010;21:325-30.
12. Touzeau C, Moreau P. How I treat extramedullary myeloma. Blood. 2016;127:971-6.
13. Bartel TB, Haessler J, Brown TL, et al. F18-fluorodeoxyglucose positron emission tomography in the context of other imaging techniques and prognostic factors in multiple myeloma. Blood. 2009;114:2068-76.
14. Pour L, Sevcikova S, Greslikova H, et al. Soft-tissue extramedullary multiple myeloma prognosis is significantly worse in comparison to bone-related extramedullary relapse. Haematologica. 2014;99:360-4.