Diagnosis and management of solitary plasmacytoma of bone
- S Vincent Rajkumar, MD
S Vincent Rajkumar, MD
- Edward W. and Betty Knight Scripps Professor of Medicine
- Mayo Clinic
- Section Editors
- Robert A Kyle, MD
Robert A Kyle, MD
- Section Editor — Plasma Cell Disorders
- Professor of Medicine
- Mayo Medical School
- Thomas F DeLaney, MD
Thomas F DeLaney, MD
- Section Editor — Bone and Soft Tissue Tumors
- Professor of Radiation Oncology
- Harvard Medical School
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 [MM]). Solitary plasmacytomas most frequently occur in bone (plasmacytoma of bone), but can also be found outside bone in soft tissues (extramedullary plasmacytoma) [1,2]. Why some patients develop MM and others plasmacytoma is not understood, but might be related to differences in cellular adhesion molecules or chemokine receptor expression profiles of the malignant plasma cells .
Solitary plasmacytoma of bone (SPB, also called osseous plasmacytoma) is a localized tumor in the bone comprised of a single clone of plasma cells in the absence of other features of MM (ie, anemia, hypercalcemia, renal insufficiency, or multiple lytic bone lesions) [4,5].
The diagnosis and management of SPB will be reviewed here. The diagnosis and treatment of other plasma cell disorders (eg, solitary extramedullary plasmacytoma, MM, primary AL amyloidosis, monoclonal gammopathy of undetermined significance) are discussed separately. (See "Diagnosis and management of solitary extramedullary plasmacytoma" and "Clinical features, laboratory manifestations, and diagnosis of multiple myeloma" and "Overview of the management of multiple myeloma" and "Overview of amyloidosis" and "Diagnosis of monoclonal gammopathy of undetermined significance".)
Approximately 5 percent of all cases of plasma cell disorders are solitary plasmacytoma of bone (SPB) . In the United States, the incidence is approximately 0.15 cases/100,000 person-years accounting for approximately 450 new cases per year. The incidence is highest in Blacks and lowest in Asians and Pacific Islanders. Men are diagnosed twice as frequently as women. The median age at diagnosis is 55 to 65 years, which compares with a median age at diagnosis of 71 years for patients with multiple myeloma (MM) [6-8]. SPB has been reported in patients as young as 15 years [9,10]. Although an increased risk of plasma cell dyscrasia has been reported in first-degree relatives of patients with monoclonal gammopathy of undetermined significance and patients with MM , there are no data on familial predisposition in solitary plasmacytoma.
Most patients present with skeletal pain or a pathologic fracture of the affected bone. Patients with vertebral involvement may have severe back pain or neurologic compromise (eg, cord compression). Less commonly, solitary plasmacytoma of bone (SPB) can extend into the surrounding soft-tissue, resulting in a palpable mass.
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- CLINICAL FEATURES
- Solitary plasmacytoma of bone (SPB)
- SPB with minimal marrow involvement
- SPB meeting criteria for multiple myeloma
- DIFFERENTIAL DIAGNOSIS
- Multiple myeloma
- POEMS syndrome
- Metastatic carcinoma
- Patients with multiple plasmacytomas
- Monoclonal protein and abnormal free light chain ratio
- SUMMARY AND RECOMMENDATIONS