Diagnosis and management of solitary extramedullary plasmacytoma
- S Vincent Rajkumar, MD
S Vincent Rajkumar, MD
- Edward W. and Betty Knight Scripps Professor of Medicine
- Mayo Clinic
- Section Editors
- Robert A Kyle, MD
Robert A Kyle, MD
- Section Editor — Plasma Cell Disorders
- Professor of Medicine
- Mayo Medical School
- Thomas F DeLaney, MD
Thomas F DeLaney, MD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Radiation Oncology
- Harvard Medical School
Plasma cell neoplasms (plasma cell dyscrasias) are a group of entities characterized by the neoplastic proliferation of a single clone of plasma cells, typically producing a monoclonal immunoglobulin. Plasma cell neoplasms can present as a single lesion (solitary plasmacytoma) or as multiple lesions (multiple myeloma). Solitary plasmacytomas most frequently occur in bone (plasmacytoma of bone), but can also be found outside bone in soft tissues (extramedullary plasmacytoma) [1,2]. Why some patients develop multiple myeloma and others a single plasmacytoma is not understood, but might be related to differences in cellular adhesion molecules or chemokine receptor expression profiles of the malignant plasma cells .
Solitary extramedullary plasmacytomas (SEP, solitary extraosseous plasmacytoma) are plasma cell tumors that arise outside of the bone marrow. They are solitary lesions, and are most often located in the head and neck region, mainly in the upper aerodigestive tract, but may also occur in the gastrointestinal tract, urinary bladder, central nervous system, thyroid, breast, testes, parotid gland, lymph nodes, and skin.
SEP refers to a solitary non-osseus plasma cell neoplasm in the absence of any other sign of multiple myeloma. The diagnosis and management of SEP will be discussed here. Note that extramedullary plasmacytomas can arise in patients with multiple myeloma at any time during the course of the disease, and should not be confused with SEP. The diagnosis and treatment of other plasma cell disorders (eg, solitary plasmacytoma of bone, multiple myeloma, primary AL amyloidosis, monoclonal gammopathy of undetermined significance) are discussed separately. (See appropriate topic reviews).
Solitary extramedullary plasmacytomas (SEP) account for approximately 3 percent of plasma cell malignancies [4-6]. The median age at diagnosis is 55 to 60 years, and approximately two-thirds of patients are male .
Most patients present with symptoms related to the location of the mass. Approximately 80 percent involve the upper respiratory tract (ie, oronasopharynx and paranasal sinuses), producing epistaxis, nasal discharge (rhinorrhea), or nasal obstruction [8-12]. Less common sites of involvement include the gastrointestinal tract , liver , lymph nodes [15,16], testes , skin, and central nervous system . Primary plasmacytoma of the lung often presents as a pulmonary nodule or hilar mass with or without hemoptysis . Regional lymph nodes may be involved.
- World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Haematopoietic and Lymphoid Tissues, Jaffe ES, Harris NL, Stein H, Vardiman JW (Eds), IARC Press, Lyon 2001.
- Soutar R, Lucraft H, Jackson G, et al. Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Br J Haematol 2004; 124:717.
- Hughes M, Doig A, Soutar R. Solitary plasmacytoma and multiple myeloma: adhesion molecule and chemokine receptor expression patterns. Br J Haematol 2007; 137:486.
- Dimopoulos MA, Moulopoulos LA, Maniatis A, Alexanian R. Solitary plasmacytoma of bone and asymptomatic multiple myeloma. Blood 2000; 96:2037.
- Dores GM, Landgren O, McGlynn KA, et al. Plasmacytoma of bone, extramedullary plasmacytoma, and multiple myeloma: incidence and survival in the United States, 1992-2004. Br J Haematol 2009; 144:86.
- Shih LY, Dunn P, Leung WM, et al. Localised plasmacytomas in Taiwan: comparison between extramedullary plasmacytoma and solitary plasmacytoma of bone. Br J Cancer 1995; 71:128.
- Frassica DA, Frassica FJ, Schray MF, et al. Solitary plasmacytoma of bone: Mayo Clinic experience. Int J Radiat Oncol Biol Phys 1989; 16:43.
- Pavithran K, Doval DC, Rao CR, et al. Pediatric solitary plasmacytoma. Acta Oncol 1997; 36:83.
- Boos N, Goytan M, Fraser R, Aebi M. Solitary plasma-cell myeloma of the spine in an adolescent. Case report of an unusual presentation. J Bone Joint Surg Br 1997; 79:812.
- Bataille R, Sany J. Solitary myeloma: clinical and prognostic features of a review of 114 cases. Cancer 1981; 48:845.
- 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.
- Gerry D, Lentsch EJ. Epidemiologic evidence of superior outcomes for extramedullary plasmacytoma of the head and neck. Otolaryngol Head Neck Surg 2013; 148:974.
- Rajkumar SV, Dispenzieri A, Kyle RA. Monoclonal gammopathy of undetermined significance, Waldenström macroglobulinemia, AL amyloidosis, and related plasma cell disorders: diagnosis and treatment. Mayo Clin Proc 2006; 81:693.
- Mayr NA, Wen BC, Hussey DH, et al. The role of radiation therapy in the treatment of solitary plasmacytomas. Radiother Oncol 1990; 17:293.
- Holland J, Trenkner DA, Wasserman TH, Fineberg B. Plasmacytoma. Treatment results and conversion to myeloma. Cancer 1992; 69:1513.
- Tsang RW, Gospodarowicz MK, Pintilie M, et al. Solitary plasmacytoma treated with radiotherapy: impact of tumor size on outcome. Int J Radiat Oncol Biol Phys 2001; 50:113.
- Knobel D, Zouhair A, Tsang RW, et al. Prognostic factors in solitary plasmacytoma of the bone: a multicenter Rare Cancer Network study. BMC Cancer 2006; 6:118.
- Bolek TW, Marcus RB Jr, Mendenhall NP. Solitary plasmacytoma of bone and soft tissue. Int J Radiat Oncol Biol Phys 1996; 36:329.
- Galieni P, Cavo M, Avvisati G, et al. Solitary plasmacytoma of bone and extramedullary plasmacytoma: two different entities? Ann Oncol 1995; 6:687.
- Tirumani SH, Shinagare AB, Jagannathan JP, et al. MRI features of extramedullary myeloma. AJR Am J Roentgenol 2014; 202:803.
- Swerdlow SH, Campo E, Harris NL, et al. (Eds). World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC Press, Lyon 2008.
- Kyle RA, Rajkumar SV. Criteria for diagnosis, staging, risk stratification and response assessment of multiple myeloma. Leukemia 2009; 23:3.
- Rajkumar SV, Dimopoulos MA, Palumbo A, et al. International Myeloma Working Group updated criteria for the diagnosis of multiple myeloma. Lancet Oncol 2014; 15:e538.
- Creach KM, Foote RL, Neben-Wittich MA, Kyle RA. Radiotherapy for extramedullary plasmacytoma of the head and neck. Int J Radiat Oncol Biol Phys 2009; 73:789.
- Tournier-Rangeard L, Lapeyre M, Graff-Caillaud P, et al. Radiotherapy for solitary extramedullary plasmacytoma in the head-and-neck region: A dose greater than 45 Gy to the target volume improves the local control. Int J Radiat Oncol Biol Phys 2006; 64:1013.
- Reed V, Shah J, Medeiros LJ, et al. Solitary plasmacytomas: outcome and prognostic factors after definitive radiation therapy. Cancer 2011; 117:4468.
- Avilés A, Huerta-Guzmán J, Delgado S, et al. Improved outcome in solitary bone plasmacytomata with combined therapy. Hematol Oncol 1996; 14:111.
- Dimopoulos MA, Goldstein J, Fuller L, et al. Curability of solitary bone plasmacytoma. J Clin Oncol 1992; 10:587.
- Knowling MA, Harwood AR, Bergsagel DE. Comparison of extramedullary plasmacytomas with solitary and multiple plasma cell tumors of bone. J Clin Oncol 1983; 1:255.