Abstract
Primary bone lymphoma (PBL) is a subtype of lymphoma that exclusively affects skeletal tissue. Despite the relatively common involvement of skeletal structures as a manifestation of non-Hodgkin's lymphoma (NHL), primary and exclusive involvement of the skeletal system is rare. The prevalence of PBL is estimated to be 3–7% amongst primary bone tumors and less than 2% amongst all lymphomas in adults. However, the definition of primary bone lymphoma has been inconsistent over time. Within our institution, we identified four cases of primary bone lymphoma based on diagnostic criteria formed from the general consensus of multiple organizations, including the World Health Organization (WHO) and International Extranodal Lymphoma Study Group (IELSG). Here, we discuss the distinct characteristics amongst these cases in addition to performing a systematic review of current literature regarding this lymphoproliferative entity.
Highlights
Primary bone lymphoma (PBL) is a subtype of lymphoma that exclusively affects skeletal tissue
In the retrospective study conducted by the International Extranodal Lymphoma Study group, authors defined that only cases with a clear bone origin are classified as primary bone lymphomas, i.e., either a single bony lesion, with or without involvement of regional lymph nodes or multiple bony lesions without nodal or visceral disease, defined as “multifocal osseous lymphoma” or “polyostotic lymphoma”. is definition corroborates with the prior World Health Organization (WHO) classification; the general consensus amongst clinicians seems to be in line with these criteria
Discussion e exact definition of PBL has remained a controversial topic throughout medical literature. e general consensus based on old WHO classifications and recommendations provided by the abovementioned International Extranodal Lymphoma Study Group (IELSG) 14 study has been to classify patients with osseous lesions that have no evidence of regional lymph node or distal visceral involvement within the category of PBL. ere are currently no clearly defined risk factors associated with the development of this malignancy
Summary
Primary bone lymphoma (PBL) is a subtype of lymphoma that exclusively affects skeletal tissue. Pathology results of the bone biopsy were consistent with aggressive large B-cell non-Hodgkin’s lymphoma (NHL). MRI of the right femur showed an abnormal intramedullary signal and enhancement involving the distal femur with associated abnormal periosteal soft tissue, suggestive of an underlying lymphoma.
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