Solitary Plasmacytoma

Updated: May 15, 2018
  • Author: Suzanne R Fanning, DO; Chief Editor: Emmanuel C Besa, MD  more...
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Overview

Practice Essentials

Solitary plasmacytoma (SP) is an early-stage plasma cell malignancy that is in between monoclonal gammopathy of undetermined significance (MGUS) and multiple myeloma (MM) along the spectrum of plasma cell disorders. [1] . A plasmacytoma is a discrete, solitary mass of neoplastic monoclonal plasma cells. SPs can be divided into 2 groups according to location:

  • Solitary bone plasmacytoma (SBP) – These occur most commonly in the vertebrae

  • Extramedullary plasmacytoma (EMP) – These encompass all nonosseus SPs; they can occur in any soft tissue but the upper airway tract, including the sinuses, is the most common site.  

For a diagnosis of SP, the International Myeloma Working Group (IMWG) requires all four of the following criteria be met [1] :

  • Biopsy-proven solitary lesion of bone or soft tissue with evidence of clonal plasma cells
  • Normal bone marrow with no evidence of clonal plasma cells
  • Normal skeletal survey and MRI (or CT) of the spine and pelvis, except for the primary solitary lesion 
  • Absence of end-organ damage, such as CRAB (hypercalcemia, renal failure, anemia, and osteolytic bone lesions), that can be attributed to a lympho-plasma cell proliferative disorder

Patients with an apparent SP who have less than 10% clonal marrow involvement are considered to have SP with minimal marrow involvement. SP with 10% or greater clonal plasma is diagnosed as multiple myeloma.

SP is a highly radiosensitive tumor and most patients undergo radiation therapy as the first line of treatment, resulting in an excellent local control rate. [2] The risk of recurrence or progression to myeloma within 3 years is approximately 10% for patients with SP versus 20% to 60% for patients who have SP with minimal marrow involvement. [1]

For patient education information, see Myeloma.

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Pathophysiology

A plasmacytoma can arise in any part of the body. A solitary bone plasmacytoma (SBP) arises from the plasma cells located in the bone marrow, whereas an extramedullary plasmacytoma (EMP) is thought to arise from plasma cells located in mucosal surfaces. [3] Both represent a different group of neoplasms in terms of location, tumor progression, and overall survival rate. [4, 5] Some authors suggest that SBPs represent marginal cell lymphomas with extensive plasmacytic differentiation. [5] Both SBP and extramedullary plasmacytoma (EMP) do, however, share many of the biologic features of other plasma cell disorders.

Cytogenetic studies show recurrent losses in chromosome 13, chromosome arm 1p, and chromosome arm 14q, as well as gains in chromosome arms 19p, 9q, and 1q. [6] Interleukin-6 (IL-6) is still considered the principal growth factor in the progression of plasma cell disorders. [7]

The specific roles of surface markers, adhesion molecules, and angiogenesis in solitary plasmacytoma need to be studied further.

In a study by Kumar et al, high-grade angiogenesis in SBP was associated with increased progression to multiple myeloma and shorter progression-free survival. Some have postulated that SBP may be considered an intermediate step in the evolution from monoclonal gammopathy of undetermined significance to multiple myeloma. [7, 8]

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Etiology

No definite cause has been found for solitary bone plasmacytoma (SBP). Because of its presentation in the mucosa of the aerodigestive tract (>80%), the etiology of extramedullary plasmacytoma (EMP) may be related to chronic stimulation of inhaled irritants or viral infection. [9]

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Epidemiology

Solitary plasmacytoma (SP) has an annual incidence of less than 450 cases in the United States. [2]  Solitary bone plasmacytoma (SBP) affects fewer than 5% of patients with plasma cell disorders. [10, 7] Extramedullary plasmacytoma (EMP) represents approximately 3% of all plasma cell neoplasms. [11]

Population studies have reported a male predominance in the SP with a male-to-female ratio of 1.6:1. In studies focusing primarily on SBP, the male-to-female ratio ranges from 1.5–2.4:1. Among EMP patients, a 3:1 male predominance has been reported in sites involving upper airway tract. [2]

SP primarily affects adults over 40 years old, and its incidence increases with age. [2]  The median age of patients with either SBP or EMP is 55 years. This median age is 10 years younger than in patients with multiple myeloma. [12, 3, 13, 14, 15]

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Prognosis

Solitary bone plasmacytoma (SBP)

Solitary bone plasmacytoma (SBP) progresses to multiple myeloma at a rate of 65-84% at 10 years and 65-100% at 15 years. The median onset of conversion to multiple myeloma is 2-5 years with a 10-year disease-free survival rate of 15-46%. The overall median survival time is 10 years. [13]

Kyle described the following 3 patterns of treatment failure [16] :

  • Development of multiple myeloma (54%)
  • Local recurrence (11%)
  • Development of new bone lesions in the absence of multiple myeloma (2%)

Prognostic features for conversion of  SBP to multiple myeloma, although controversial, include the following [17, 18, 19] :

  • Lesion size of at least 5 cm
  • Patient age 40 years and older
  • High M protein levels
  • Persistence of M protein after treatment
  • Spine lesions

In a study by Wilder et al, 10-year myeloma-free survival in patients whose M protein resolved within 1 year after radiation therapy was 91%, versus 29% in patients whose M-protein persisted beyond that time. [20]

Extramedullary plasmacytoma (EMP)

Patients with EMP have a 5-year survival rate of about 82% and 10-year disease-specific survival rate greater than 50%. [11]  The rate of progression to multiple myeloma is lower than in SBP, ranging from 11% to 30% at 10 years. [13]  Patients with EMP that progressed to multiple myeloma had a 5-year survival rate of 100%, compared with 33% for those with SBP. [21]  In a review of 721 cases by Alexiou and colleagues, after treatment, approximately 65% of patients were free of recurrence and did not progress to multiple myeloma, 22% experienced recurrence, and 15% of cases evolved to multiple myeloma. [14]

In one study, local control following radiation therapy was achieved in 83% of patients with low-grade histology versus 17% of patients with intermediate- to high-grade tumors. [22]

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