Extramedullary Plasmacytoma of the Lung - Rather a Disseminated than a Localized Disease? - A Case Report of a Primary Pulmonary Plasmacytoma Showing Distinct Signs of Systemic Spread
Keywords:Primarypulmonary plasmacytoma, extramedullary plasmacytoma, multiple myeloma, cytogenetics.
Objective: We report an unusual case of a patient with a plasma cell tumor presenting first as a primary pulmonary plasmacytoma (PPP) but then showing distinct signs of systemic spread.
Case Summary: A 58-year-old woman presented with an extramedullary plasma cell tumor of the lung with additional affection of lymph nodes above and underneath the diaphragm, without apparent infiltration of the bone marrow by malignant plasma cells, but with evidence of a small proportion of clonal plasma cells in FACS analysis. Due to the fact that the patient showed excretion of lambda light chains in the urine in addition to the solid manifestations on multiple sites and minimal bone marrow involvement, we verified the systemic spread. This is in contrast to primary extramedullary plasmacytoma presenting as solitaire plasma cell tumors mostly occurring in the upper aerodigestive tract or extramedullary myeloma, which describes relapses or extramedullary progression of multiple myeloma emerging in various organs. We herein present all features of a, to our opinion, systemic disease by means of affection pattern, laboratory values, and bone marrow infiltration.
Conclusion: This unusual case demonstrates the presentation of an extramedullary plasma cell tumor that appeared as a PPP but showed distinct signs of dissemination as well as uncommon features such as a monoclonal component of IgM type. This example reveales that extramedullary manifestations of plasma cell dyscrasia should be surveyed carefully as they can conceal an underlying systemic disease.
Galieni P, Cavo M, Pulsoni A, et al. Clinical outcome of extramedullary plasmacytoma. Haematologica 2000; 85: 47-51.
Alexiou C, Kau RJ, Dietzfelbinger H, et al. Extramedullary plasmacytoma: tumor occurrence and therapeutic concepts. Cancer 1999; 85: 2305-14. http://dx.doi.org/10.1002/(SICI)1097-0142(19990601)85:11<2305::AID-CNCR2>3.3.CO;2-V
Zeiser R, Deschler B, Bertz H, Finke J and Engelhardt, M. Extramedullary vs medullary relapse after autologous or allogeneic hematopoietic stem cell transplantation (HSCT) in multiple myeloma (MM) and its correlation to clinical outcome. Bone Marrow Transplant 2004; 34: 1057-654. http://dx.doi.org/10.1038/sj.bmt.1704713
Laso FJ, Tabernero MD, Iglesias-Osma MC. Extramedullary plasmacytoma: a localized or systemic disease? Ann Intern Med1998; 128: 156.
Gordon J, Walker G. Plasmacytoma of the lung. Arch Pathol 1944; 37: 222-24.
Godziuk K, Kedra M, Rybojad P, Sagan, D. A rare case of solitary plasmacytoma mimicking a primary lung tumor. Ann Thorac Surg2009; 87: 25-26. http://dx.doi.org/10.1016/j.athoracsur.2008.11.066
Edelstein E, Gal AA, Mann KP, Miller JI Jr., Mansour KA. Primary Solitary Endobronchial Plasmacytoma. Ann Thorac Surg 2004; 78: 1448-9. http://dx.doi.org/10.1016/S0003-4975(03)01474-7
The International Myeloma Working Group. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: a report of the International Myeloma Working Group. Br J Haematol 2003; 121: 749-757. http://dx.doi.org/10.1046/j.1365-2141.2003.04355.x
Koss MN, Hochholzer L, Moran CA, Frizzera G. Pulmonary plasmacytomas: a clinicopathological and immunohistochemical study of 5 cases. Ann Diagn Pathol 1998; 2: 1-11. http://dx.doi.org/10.1016/S1092-9134(98)80029-4
Terpos E, Rezvani K, Basu S, et al. Plasmacytoma relapses in the absence of systemic progression post-high-dose therapy for multiple myeloma. Eur J Haematol 2005; 75: 376-83. http://dx.doi.org/10.1111/j.1600-0609.2005.00531.x
Kintzer JS, Rosenow EC, Kyle RA. Thoracic and pulmonary abnormalities in multiple myeloma. A review of 958 cases. Arch Intern Med 1978; 138: 727-30. http://dx.doi.org/10.1001/archinte.1978.03630290039015
Luh SP, Lai YS, Tsai CH and Tsao TC. Extramedullary plasmacytoma (EMP): Report of a case manifested as a mediastinal mass and multiple pulmonary nodules and review of literature. World J Surg Oncol 2007; 5: 123. http://dx.doi.org/10.1186/1477-7819-5-123
Hayes-Lattin B, Blanke CD, Deloughery TG. Pulmonary and intracerebral plasmacytomas in a patient without multiple myeloma: a case report. Am J Hematol 2003; 73: 131-4. http://dx.doi.org/10.1002/ajh.10330
Nonomura A, Mizukami Y, Shimizu J, et al. Primary extramedullary plasmacytoma of the lung. Intern Med 1992; 31: 1396-400. http://dx.doi.org/10.2169/internalmedicine.31.1396
López-Anglada L, Gutiérrez NC, García JL, et al. p53 may drive the clinical evolution and treatment response in multiple myeloma. Eur J Haematol 2010; 84: 359-61. http://dx.doi.org/10.1111/j.1600-0609.2009.01399.x
Damaj G, Mohty M, Vey N, et al. Features of extramedullary and extraosseous multiple myeloma: a report of 19 patients from a single center. Eur J Haematol 2004; 73: 402-6. http://dx.doi.org/10.1111/j.1600-0609.2004.00331.x
Chang H, Sloan S, Li D, Keith Stewart A. Multiple myeloma involving central nervous system: high frequency of chromosome 17p13.1 (p53) deletions. Br J Haematol 2004; 127: 280-4. http://dx.doi.org/10.1111/j.1365-2141.2004.05199.x
Kremer M, Ott G, Nathrath M, et al. Primary extramedullary plasmacytoma and multiple myeloma: phenotypic differences revealed by immunohistochemical analysis. J Pathol 2005; 205: 92-101. http://dx.doi.org/10.1002/path.1680
Chang H, Bartlett ES, Patterson B, Chen CI, Yi QL. The absence of CD56 on malignant plasma cells in the cerebrospinal fluid is the hallmark of multiple myeloma involving central nervous system. Br J Haematol 2005; 129:539-41. http://dx.doi.org/10.1111/j.1365-2141.2005.05493.x
Sahara N, Takeshita A, Shigeno K, et al. Clinicopathological and prognostic characteristics of CD56-negative multiple myeloma. Br J Haematol 2002; 117: 882-5. http://dx.doi.org/10.1046/j.1365-2141.2002.03513.x
Dierlamm T, Laack E, Dierlamm J, et al. IgM myeloma: a report of four cases. Ann Hematol 2002; 81: 136-9. http://dx.doi.org/10.1007/s00277-001-0420-9
De Gramont A, Grosbois B, Michaux JL, et al. IgM myeloma: 6 cases and a review of the literature. La Revue de Medicine Interne 1990; 11: 13-18