Diagnosis and management of solitary plasmacytoma of bone
- 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 [MM]). Solitary plasmacytomas most frequently occur in bone (plasmacytoma of bone), but can also be found outside bone in soft tissues (extramedullary plasmacytoma) [1-4]. Why some patients develop MM and others 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 plasmacytoma of bone (SPB, also called osseous plasmacytoma) is a localized tumor in the bone comprised of a single clone of plasma cells in the absence of other features of MM (ie, anemia, hypercalcemia, renal insufficiency, or multiple lytic bone lesions) [6,7].
The diagnosis and management of SPB will be reviewed here. The diagnosis and treatment of other plasma cell disorders (eg, solitary extramedullary plasmacytoma, MM, primary AL amyloidosis, monoclonal gammopathy of undetermined significance) are discussed separately. (See "Diagnosis and management of solitary extramedullary plasmacytoma" and "Clinical features, laboratory manifestations, and diagnosis of multiple myeloma" and "Overview of the management of multiple myeloma" and "Overview of amyloidosis" and "Diagnosis of monoclonal gammopathy of undetermined significance".)
Approximately 5 percent of all cases of plasma cell disorders are SPB . In the United States, the incidence is approximately 0.15 cases/100,000 person-years accounting for approximately 450 new cases per year. The incidence is highest in Blacks and lowest in Asians and Pacific Islanders. Men are diagnosed twice as frequently as women. The median age at diagnosis is 55 to 65 years, which compares with a median age at diagnosis of 71 years for patients with multiple myeloma (MM) [8-10]. SPB has been reported in patients as young as 15 years [11,12]. Although an increased risk of plasma cell dyscrasia has been reported in first-degree relatives of patients with monoclonal gammopathy of undetermined significance and patients with MM , there are no data on familial predisposition in solitary plasmacytoma.
Most patients present with skeletal pain or a pathologic fracture of the affected bone. Patients with vertebral involvement may have severe back pain or neurologic compromise (eg, cord compression). Less commonly, SPB can extend into the surrounding soft-tissue, resulting in a palpable mass.
- 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.
- Swerdlow SH, Campo E, Pileri SA, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016; 127:2375.
- World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, Swerdlow SH, Campo E, Harris NL, et al. (Eds), IARC Press, Lyon 2008.
- 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.
- Swerdlow SH, Campo E, Harris NL, et al. (Eds). World Health Organization Classification of Tumours of Haematopoietic and Lymphoid Tissues, IARC Press, Lyon 2008.
- 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.
- Greenberg AJ, Rajkumar SV, Vachon CM. Familial monoclonal gammopathy of undetermined significance and multiple myeloma: epidemiology, risk factors, and biological characteristics. Blood 2012; 119:5359.
- Bataille R, Sany J. Solitary myeloma: clinical and prognostic features of a review of 114 cases. Cancer 1981; 48:845.
- Ozsahin M, Tsang RW, Poortmans P, et al. Outcomes and patterns of failure in solitary plasmacytoma: a multicenter Rare Cancer Network study of 258 patients. Int J Radiat Oncol Biol Phys 2006; 64:210.
- Warsame R, Gertz MA, Lacy MQ, et al. Trends and outcomes of modern staging of solitary plasmacytoma of bone. Am J Hematol 2012; 87:647.
- Dimopoulos MA, Hillengass J, Usmani S, et al. Role of magnetic resonance imaging in the management of patients with multiple myeloma: a consensus statement. J Clin Oncol 2015; 33:657.
- Wilder RB, Ha CS, Cox JD, et al. Persistence of myeloma protein for more than one year after radiotherapy is an adverse prognostic factor in solitary plasmacytoma of bone. Cancer 2002; 94:1532.
- Moulopoulos LA, Dimopoulos MA, Weber D, et al. Magnetic resonance imaging in the staging of solitary plasmacytoma of bone. J Clin Oncol 1993; 11:1311.
- Pertuiset E, Bellaiche L, Lioté F, Laredo JD. Magnetic resonance imaging of the spine in plasma cell dyscrasias. A review. Rev Rhum Engl Ed 1996; 63:837.
- Kim PJ, Hicks RJ, Wirth A, et al. Impact of 18F-fluorodeoxyglucose positron emission tomography before and after definitive radiation therapy in patients with apparently solitary plasmacytoma. Int J Radiat Oncol Biol Phys 2009; 74:740.
- 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.
- Paiva B, Chandia M, Vidriales MB, et al. Multiparameter flow cytometry for staging of solitary bone plasmacytoma: new criteria for risk of progression to myeloma. Blood 2014; 124:1300.
- Hill QA, Rawstron AC, de Tute RM, Owen RG. Outcome prediction in plasmacytoma of bone: a risk model utilizing bone marrow flow cytometry and light-chain analysis. Blood 2014; 124:1296.
- Holland J, Trenkner DA, Wasserman TH, Fineberg B. Plasmacytoma. Treatment results and conversion to myeloma. Cancer 1992; 69:1513.
- Galieni P, Cavo M, Avvisati G, et al. Solitary plasmacytoma of bone and extramedullary plasmacytoma: two different entities? Ann Oncol 1995; 6:687.
- Dimopoulos MA, Goldstein J, Fuller L, et al. Curability of solitary bone plasmacytoma. J Clin Oncol 1992; 10:587.
- Burt M, Karpeh M, Ukoha O, et al. Medical tumors of the chest wall. Solitary plasmacytoma and Ewing's sarcoma. J Thorac Cardiovasc Surg 1993; 105:89.
- McLain RF, Weinstein JN. Solitary plasmacytomas of the spine: a review of 84 cases. J Spinal Disord 1989; 2:69.
- Mayr NA, Wen BC, Hussey DH, et al. The role of radiation therapy in the treatment of solitary plasmacytomas. Radiother Oncol 1990; 17:293.
- 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.
- Reed V, Shah J, Medeiros LJ, et al. Solitary plasmacytomas: outcome and prognostic factors after definitive radiation therapy. Cancer 2011; 117:4468.
- Bolek TW, Marcus RB Jr, Mendenhall NP. Solitary plasmacytoma of bone and soft tissue. Int J Radiat Oncol Biol Phys 1996; 36:329.
- Avilés A, Huerta-Guzmán J, Delgado S, et al. Improved outcome in solitary bone plasmacytomata with combined therapy. Hematol Oncol 1996; 14:111.
- 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.
- de Waal EG, Leene M, Veeger N, et al. Progression of a solitary plasmacytoma to multiple myeloma. A population-based registry of the northern Netherlands. Br J Haematol 2016.
- Jackson A, Scarffe JH. Upper humeral cortical thickness as an indicator of osteopenia: diagnostic significance in solitary myeloma of bone. Skeletal Radiol 1991; 20:363.
- Kumar S, Fonseca R, Dispenzieri A, et al. Prognostic value of angiogenesis in solitary bone plasmacytoma. Blood 2003; 101:1715.
- Dingli D, Kyle RA, Rajkumar SV, et al. Immunoglobulin free light chains and solitary plasmacytoma of bone. Blood 2006; 108:1979.
- Liebross RH, Ha CS, Cox JD, et al. Solitary bone plasmacytoma: outcome and prognostic factors following radiotherapy. Int J Radiat Oncol Biol Phys 1998; 41:1063.
- CLINICAL FEATURES
- Solitary plasmacytoma of bone (SPB)
- SPB with minimal marrow involvement
- SPB meeting criteria for multiple myeloma
- DIFFERENTIAL DIAGNOSIS
- Multiple myeloma
- POEMS syndrome
- Metastatic carcinoma
- Patients with multiple plasmacytomas
- Monoclonal protein and abnormal free light chain ratio
- SUMMARY AND RECOMMENDATIONS