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Plasmacytoma

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Title: Plasmacytoma  
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Plasmacytoma

Plasmacytoma
Micrograph of a plasmacytoma. H&E stain.
Classification and external resources
Specialty Hematology and oncology
ICD-10 C90.2
ICD-9-CM 238.6
ICD-O M9731/3, M9734/3
DiseasesDB 8628
eMedicine med/2532
MeSH D010954

Plasmacytoma refers to a


  • Educational Resource on Plasmacytomas
  • The Internet Journal of Otorhinolaryngology
  • The Doctor's Doctor
  • Overview at National Cancer Institute

External links

  1. ^ a b c d
  2. ^ a b c d
  3. ^ a b c d e
  4. ^ "plasmacytoma" at Dorland's Medical Dictionary
  5. ^
  6. ^
  7. ^
  8. ^
  9. ^
  10. ^
  11. ^ a b c d
  12. ^ a b
  13. ^
  14. ^ a b

References

See also

Gallery

Most cases of SPB progress to multiple myeloma within 2–4 years of diagnosis, but the overall median survival for SPB is 7–12 years. 30–50% of EP cases progress to multiple myeloma with a median time of 1.5–2.5 years. 15–45% of SPB and 50–65% of EP are disease free after 10 years.[3]

Prognosis

Radiotherapy is the main choice of treatment for both SPB and EP, and local control rates of >80% can be achieved. This form of treatment can be used with curative intent because plasmacytoma is a radiosensitive tumor. Surgery is an option for EP, but for cosmetic reasons it is generally used when the lesion is not present within the head and neck region.[3][11][14]

Treatment

An important distinction to be made is that a true plasmacytoma is present and not a systemic plasma cell disorder, such as multiple myeloma. The difference between plasmacytoma and multiple myeloma is that plasmacytoma lacks increased blood calcium, renal insufficiency, anemia and multiple bone lesions (collectively termed CRAB).[11]

Skeletal surveys are used to ensure there are no other primary tumors within the axial skeleton. MRI can be used to assess tumor status and may be advantageous in detecting primary tumors that are not detected by plain film radiography. PET-CT may also be beneficial in detecting extramedullary tumours in individuals diagnosed with SPB. CT imaging may be better than plain film radiography for assessing bone damage.[11][12]

Bone marrow biopsies are performed to ensure the disease is localised; and in SPB or EP there will not be an increase of monoclonal plasma cells. Tissue biopsies of SPB and EP are used to assess the phenotype of the plasma cells. Histological analyses can be performed on these biopsies to see what cluster of differentiation (CD) markers are present and to assess monoclonality of the cells. CD markers can aid in the distinction of EP from lymphomas.[3][14]

Serum protein electrophoresis separates the proteins in the liquid part of the blood (serum), allowing the analysis of antibodies. Normal blood serum contains a range of antibodies and are said to be polyclonal, whereas serum from a person with plasmacytoma may show a monoclonal spike. This is due to an outgrowth of a single type of plasma cell that forms the plasmacytoma and produces a single type of antibody. The plasma cells are said to be monoclonal and the excessively produced antibody is known as monoclonal protein or paraprotein.[13] Paraproteins are present in 60% of SPB and less than 25% of EP.[2]

The diagnosis of plasmacytoma uses a diverse range of interdisciplinary techniques including serum protein electrophoresis, bone marrow biopsy, urine analysis for Bence Jones protein and complete blood count, plain film radiography, MRI and PET-CT.[11][12]

Serum protein electrophoresis of an individual with polyclonal antibodies (top) and an individual with a large paraprotein (bottom).

Diagnosis

For SPB the most common presenting symptom is that of pain in the affected bone. Back pain and other consequences of the bone lesion may occur such as spinal cord compression or pathological fracture. Around 85% of EP presents within the upper respiratory tract mucosa, causing possible symptoms such as epistaxis, rhinorrhoea and nasal obstruction. In some tissues it may be found as a palpable mass.[1][2][3]

Signs and symptoms

Plasmacytomas are a rare form of cancer. SPB is the most common form of the disease and accounts for 3-5% of all plasma cell malignancies. The median age at diagnosis for all plasmacytomas is 55. Both SPB and EP are more prevalent in males; with a 2:1 male to female ratio for SPB and a 3:1 ratio for EP.[2]

Epidemiology

The skeletal survey (other than the single bone lesion). The criteria for EP are the same but the tumor is located in soft tissue. No bone lesions should be present. Criteria for multiple solitary plasmacytomas (+/- recurrent) are the same except either multiple solitary bone or soft tissue lesions must be present. They may occur as multiple primary tumors or as a recurrence from a previous plasmacytoma.

Plasmacytoma is a tumor of plasma cells. The cells are identical to those seen in multiple myeloma, but they form discrete masses of cells in the skeleton (solitary plasmacytoma of bone; SPB) or in soft tissues (extramedullary plasmacytoma; EP). They do not present with systemic disease, which would classify them as another systemic plasma cell disorder.[10]

Classification

There can be some ambiguity when using the word. "Plasmacytoma" is sometimes equated with "plasma cell dyscrasia" or "solitary myeloma".[4] It is often used as part of the phrase "solitary plasmacytoma".[5][6][7] or as part of the phrase "extramedullary plasmacytoma".[8][9] In this context, "extramedullary" means outside of the bone marrow.

Terminology

Contents

  • Terminology 1
  • Classification 2
  • Epidemiology 3
  • Signs and symptoms 4
  • Diagnosis 5
  • Treatment 6
  • Prognosis 7
  • Gallery 8
  • See also 9
  • References 10
  • External links 11

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