Extramedullary Plasmacytoma Workup

Updated: Dec 01, 2015
  • Author: Suzanne R Fanning, DO; Chief Editor: Emmanuel C Besa, MD  more...
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Workup

Laboratory Studies

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  • Solitary bone plasmacytoma (SBP)
    • Although levels are lower than in multiple myeloma, electrophoresis reveals a monoclonal protein in the serum or urine in 24-72% of patients. [28, 29, 30, 31, 32, 33, 34, 35]
    • Uninvolved immunoglobulin levels are usually within the reference range.
    • Peripheral blood cell count, renal function, and calcium are within the reference range.
  • Extramedullary plasmacytoma (EMP)
    • Protein electrophoresis shows a monoclonal component in 14-25% of cases. [4, 6, 18] In a series of 46 patients by Galieni and colleagues, all patients had normal uninvolved immunoglobulins. [6]
    • Peripheral blood cell count, renal function, and calcium are within the reference range.
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Imaging Studies

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  • Solitary bone plasmacytoma (SBP)
    • On plain radiographs, solitary bone plasmacytoma (SBP) classically has a lytic appearance with clear margins and a narrow zone of transition to healthy surrounding bone. [5] Rare occurrences of a cyst, a trabeculated lesion resembling a giant cell tumor or an aneurysmal bone cyst, and sclerotic lesions have been described. [36] The sclerotic lesion is associated with polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome. [37]
    • On MRI, the solitary bone plasmacytoma (SBP) exhibits abnormal signal intensity (low on T1-weighted imaging and high on T2-weighted or short tau inversion recovery [STIR] images) that, in the appropriate clinical setting, is consistent with solitary bone plasmacytoma (SBP). [38]
  • Extramedullary plasmacytoma (EMP)
    • Radiographic assessment shows local bone destruction in most patients with nasal cavity or maxillary sinus involvement. [18]
    • Computed tomography (CT) scanning, MRI, and complete endoscopic examination of the aerodigestive and gastrointestinal tracts are required to determine the exact extent of the tumor and its potential for resectability. [17] These lesions may be associated with variable mass effect, infiltration and/or destruction of adjacent bone, muscle, fat, or vascular encasement. [39]
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Histologic Findings

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  • Solitary bone plasmacytoma (SBP): Biopsy of the lesion reveals infiltration of the bone by monoclonal plasma cells.
  • Extramedullary plasmacytoma (EMP): Biopsy of the soft-tissue lesion shows infiltration by monoclonal plasma cells. [40]
    • In extramedullary plasmacytoma (EMP), the soft-tissue lesion commonly exhibits submucosal growth, requiring deep biopsy, open biopsy, or complete excision depending on the tumor location. [17]
    • Histologically, extramedullary plasmacytoma (EMP) may be classified as low, intermediate, or high grade. [41]
    • Bone marrow biopsy shows less than 5% plasma cells without evidence of clonality. [6]
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Staging

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  • Wiltshaw classified soft-tissue plasmacytoma into 3 clinical stages, as follows [9] :
    • Stage I – Limited to an extramedullary site
    • Stage II – Involvement of regional lymph nodes
    • Stage III – Multiple metastasis (although it is no longer a solitary plasmacytoma)
  • The therapeutic and prognostic value of this classification needs further evaluation.
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