Extramedullary Plasmacytoma Clinical Presentation

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

History

See Physical.

  • Solitary bone plasmacytoma (SBP)
    • The most common symptom of solitary bone plasmacytoma (SBP) is pain at the site of the skeletal lesion due to bone destruction by the infiltrating plasma cell tumor. [5, 19]
    • Compression fractures of the thoracic and lumbar vertebral bodies usually result in severe spasms and back pain. Patients with important vertebral involvement may also have evidence of nerve root or spinal cord compression. [5] Spinal cord compression represents an emergency that requires immediate diagnosis and treatment to avoid permanent neurologic damage (eg, paraplegia, bowel and bladder dysfunction, chronic pain).
    • Pleuritic pain from pathologic rib and clavicular fractures are associated with marked local tenderness.
  • Solitary extramedullary plasmacytoma (EMP)
    • Extramedullary plasmacytoma (EMP) presents as a mass growing in the aerodigestive tract in 80-90% of patients, often with spread to lymph nodes, although other sites are affected as well.
    • Common complaints include swelling, headache, nasal discharge, epistaxis, nasal obstruction, sore throat, hoarseness, dysphonia, dysphagia, dyspnea, epigastric pain, and hemoptysis. [6, 20, 21, 22]
    • Symptoms from extramedullary plasmacytoma (EMP) in other tissues are associated with the site of the tumor, tumor size, and compression and/or involvement of the surrounding structures.
    • Extramedullary plasmacytoma (EMP) involving the lung most commonly presents as a pulmonary nodule or hilar mass.
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Physical

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  • Solitary bone plasmacytoma (SBP)
    • Solitary bone plasmacytoma (SBP) may involve any bone, but it has a predisposition for the red marrow–containing axial skeleton. Spinal disease is observed in 34-72% of cases. The thoracic vertebrae are most commonly involved, followed by lumbar, sacral, and cervical vertebrae. [4] The rib, sternum, clavicle, or scapula is involved in 20% of cases. [23] Physical findings are related to the site of involvement, presenting as a painful mass, pathologic fracture, or root or spinal cord compression syndrome.
    • Patients with long bone involvement may present with pathologic fracture. [4]
    • Occasionally, patients with solitary bone plasmacytoma (SBP) may present with peripheral polyneuropathy. [24, 25] or with features that are consistent with POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes). [26]
  • Extramedullary plasmacytoma (EMP)
    • Although extramedullary plasmacytoma (EMP) can occur in any site, 80-90% of tumors develop in the head and neck area, especially in the aerodigestive tract.
    • Approximately 80% of cases involve the paranasal sinuses, pharynx, nasal cavity, or gums and oral mucosa. [4, 6, 9, 17, 18] A mass (plasmacytoma) in these areas is the most common finding, with compression or invasion of the surrounding structures. Patients with tumors involving the base of the skull may present with cranial nerve palsies.
    • Case reports of involvement of the urinary bladder, central nervous system, orbit, gastrointestinal tract, liver, spleen, pancreas, lung, breast, skin, testis, parotid gland, mediastinum, and thyroid gland (associated with goiter and Hashimoto thyroiditis) exist. [4, 6, 9, 17, 18, 27]
    • In 30-40% of cases, local lymph nodes are involved at presentation or upon relapse. [16]
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Causes

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  • 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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