Primary bone (skeletal/osseous) lymphoma (PBL) is rare, accounting for <5% of bone tumors and <1% of non-Hodgkin lymphoma. It is less common than secondary involvement of bone from disseminated lymphoma.
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Terminology
PBL is defined as the presence of lymphoma isolated to one bone without distant spread for ≥6 months after diagnosis.
Multifocal PBL is much less common and occurs when lymphoma is confined to two or more bones, again without distant spread for 6 months 1.
Epidemiology
PBL can affect any age group, with peak incidence in 50-60 year-olds. It is rare in children <10 years old. There is a slight male predominance (M:F = 1.5:1) 2.
Clinical presentation
Symptoms include localized pain and swelling, B-type symptoms, pathological fractures, and cord compression.
Pathology
Diffuse large B cell lymphoma (DLBCL) is the most common subtype. The bony pelvis and femur are the most common locations 3. In adults, it usually occurs in the axial skeleton as well as regions of persistent red marrow, mainly the proximal humerus and femur.
Radiographic features
Plain radiograph
PBL has non-specific features and the affected bone may be normal or affected by lytic, sclerotic or mixed pattern. The most common is a lytic pattern with permeative bone destruction and a wide zone of transition 1. Cortical thickening and periosteal reaction may also be seen.
MRI
Associated soft tissue masses are common. Bone marrow changes include 2:
T1: low signal
T2: high signal
Treatment and prognosis
Five-year survival rate has been reported as high as 95% with combined chemoradiotherapy 4. This is much better than other bone tumors 2.
Differential diagnosis
For plain film presentation of permeative bone destruction consider 1:
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