Solitary bone plasmacytoma
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At the time the article was created Bruno Di Muzio had no recorded disclosures.View Bruno Di Muzio's current disclosures
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Solitary bone plasmacytomas are an uncommon plasma cell tumor which are localized to bone. They may involve any bone, but they have a predisposition for the red marrow-containing axial skeleton:
- spinal disease is observed in ~50% (range 34-72%) of cases
- the thoracic vertebrae are most commonly involved, followed by lumbar, sacral, and cervical vertebrae
- the rib, sternum, clavicle, or scapula are involved in 20% of cases
Solitary bone plasmacytoma is one of the two subgroups of plasmacytoma, the other being extramedullary plasmacytoma.
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- peak incidence is in 4th to 6th decades
- more common in males ~2:1
Physical findings are related to the site of involvement, presenting as a painful mass, pathologic fracture, or root or spinal cord compression syndrome.
The most common symptom of solitary bone plasmacytoma is a pain at the site of the skeletal lesion due to bone destruction by the infiltrating plasma cell tumor.
Patients with long bone involvement may present with pathologic fracture.
Diagnostic criteria for solitary bone plasmacytoma has been considered as follows 1:
- single area of destruction due to clonal plasma cells
- bone marrow plasma cell infiltration <5% of all nucleated cells
- absence of osteolytic bone lesions or other tissue involvement (i.e. no evidence of myeloma)
- absence of anemia, hypercalcemia or renal impairment (usually attributable to myeloma)
- low or absent serum/urine monoclonal protein
- preserved levels of uninvolved immunoglobulins
Solitary expansile lytic lesion with thinning and destruction of the cortex, and bubbly/trabeculated appearance. Characteristically, the absence of sclerotic reaction is seen.
Expansile lytic lesion with thinned out cortex and characteristic 'mini-brain' appearance has been described in solitary vertebral lesions.
A mini-brain appearance has also been described on MRI 3. It is seen as curvilinear low signal intensity areas within the lesion, giving an appearance of sulci in the brain. In fact, this appearance is so characteristic that it may obviate the need for a diagnostic biopsy.
Signal characteristics include:
- T1: hypo- to isointense
- T2: iso- to hyperintense to muscle
- T1 C+ (Gd): variable enhancement
Treatment and prognosis
Excision of the tumor and its extent is done, with chemoradiotherapy as an adjuvant. Local recurrence is less than 5% and dissemination is seen in 35-70% of patients. The disease may progress to multiple myeloma, with a bad prognosis.
General imaging differential considerations include:
- 1. Dimopoulos MA, Kiamouris C, Moulopoulos LA. Solitary plasmacytoma of bone and extramedullary plasmacytoma. Hematol. Oncol. Clin. North Am. 1999;13 (6): 1249-57. - Pubmed citation
- 2. Ooi GC, Chim JC, Au WY et-al. Radiologic manifestations of primary solitary extramedullary and multiple solitary plasmacytomas. AJR Am J Roentgenol. 2006;186 (3): 821-7. doi:10.2214/AJR.04.1787 - Pubmed citation
- 3. Major NM, Helms CA, Richardson WJ. The "mini brain": plasmacytoma in a vertebral body on MR imaging. AJR Am J Roentgenol. 2000;175 (1): 261-3. AJR Am J Roentgenol (full text) - Pubmed citation