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Diagnosis and management of solitary extramedullary plasmacytoma

S Vincent Rajkumar, MD
Section Editors
Robert A Kyle, MD
Thomas F DeLaney, MD
Deputy Editor
Rebecca F Connor, MD


Plasma cell neoplasms (plasma cell dyscrasias) are a group of entities characterized by the neoplastic proliferation of a single clone of plasma cells, typically producing a monoclonal immunoglobulin. Plasma cell neoplasms can present as a single lesion (solitary plasmacytoma) or as multiple lesions (multiple myeloma). Solitary plasmacytomas most frequently occur in bone (plasmacytoma of bone), but can also be found outside bone in soft tissues (extramedullary plasmacytoma) [1-4]. Why some patients develop multiple myeloma and others a single plasmacytoma is not understood, but might be related to differences in cellular adhesion molecules or chemokine receptor expression profiles of the malignant plasma cells [5].

Solitary extramedullary plasmacytomas (SEPs, solitary extraosseous plasmacytoma) are plasma cell tumors that arise outside of the bone marrow. They are solitary lesions, and are most often located in the head and neck region, mainly in the upper aerodigestive tract, but may also occur in the gastrointestinal tract, urinary bladder, central nervous system, thyroid, breast, testes, parotid gland, lymph nodes, and skin.

SEP refers to a solitary non-osseus plasma cell neoplasm in the absence of any other sign of multiple myeloma. The diagnosis and management of SEP will be discussed here. Note that extramedullary plasmacytomas can arise in patients with multiple myeloma at any time during the course of the disease, and should not be confused with SEP. The diagnosis and treatment of other plasma cell disorders (eg, solitary plasmacytoma of bone, multiple myeloma, primary AL amyloidosis, monoclonal gammopathy of undetermined significance) are discussed separately. (See "Diagnosis and management of solitary plasmacytoma of bone" and "Clinical features, laboratory manifestations, and diagnosis of multiple myeloma" and "Clinical presentation, laboratory manifestations, and diagnosis of immunoglobulin light chain (AL) amyloidosis (primary amyloidosis)" and "Diagnosis of monoclonal gammopathy of undetermined significance".)


SEPs account for approximately 3 percent of plasma cell malignancies [6-8]. The median age at diagnosis is 55 to 60 years, and approximately two-thirds of patients are male [9].


Most patients present with symptoms related to the location of the mass. Approximately 80 percent involve the upper respiratory tract (ie, oronasopharynx and paranasal sinuses), producing epistaxis, nasal discharge (rhinorrhea), or nasal obstruction [10-14]. Less common sites of involvement include the gastrointestinal tract [15], liver [16], lymph nodes [17,18], testes [19], skin, and central nervous system [20]. Primary plasmacytoma of the lung often presents as a pulmonary nodule or hilar mass with or without hemoptysis [21]. Regional lymph nodes may be involved.

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Literature review current through: Nov 2017. | This topic last updated: Nov 21, 2016.
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