primäres Lymphom des Schädelknochens
primäres Lymphom des Schädelknochens
primäres Lymphom des Knochens Radiopaedia • CC-by-nc-sa 3.0 • de
Primary bone (skeletal/osseous) lymphoma (PBL) is a less common manifestation of lymphoma than secondary involvement from disseminated lymphoma. It is rare, accounting for <5% of bone tumors and <1% of non-Hodgkin lymphoma.
Terminology
PBL is defined as the presence of lymphoma isolated to one bone without distant spread for six months after diagnosis. Multifocal PBL is less common and occurs with lymphoma is confined to two or more bones, again without distant spread for six months .
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) .
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 .
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 .
MRI
Associated soft tissue masses are common. Bone marrow changes include :
- T1: low signal
- T2: high signal
Treatment and prognosis
Five-year survival rate has been reported at ~ 80%, much better than other bone tumors .
Differential diagnosis
For plain film presentation of permeative bone destruction consider :
Siehe auch:
Assoziationen und Differentialdiagnosen zu primäres Lymphom des Schädelknochens:
