Primary Bone Lymphoma: A Review of the Literature with Emphasis on Histopathology and Histogenesis

Primary bone lymphoma (PBL) is a rare neoplasm of malignant lymphoid cells presenting with one or more bone lesions without nodal or other extranodal involvement. It accounts for approximately 1% of all lymphomas and 7% of malignant primary bone tumors. Diffuse large B-cell lymphoma (DLBCL), not oth...

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Bibliographic Details
Published in:Diseases 2023-03, Vol.11 (1), p.42
Main Authors: Kanavos, Theofilos, Birbas, Effrosyni, Papoudou-Bai, Alexandra, Hatzimichael, Eleftheria, Kitsouli, Aikaterini, Karpathiou, Georgia, Kanavaros, Panagiotis
Format: Article
Language:English
Subjects:
B-cell lymphoma
B-cells
Biopsy
bone
Bone lesions
Bone marrow
Bone mass
Bone tumors
Chemoradiotherapy
Diagnosis
Edema
Epidemiology
Femur
Fractures
Gene expression
Histogenesis
Humerus
immunohistochemistry
Literature reviews
Lymphocytes B
Lymphoid cells
Lymphoma
Lymphomas
Magnetic resonance imaging
miRNA
Pelvis
Physiological aspects
Prognosis
Review
Skeleton
Tibia
Tomography
Tumor necrosis factor-TNF
Tumors
Ultrasonic imaging
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Summary:Primary bone lymphoma (PBL) is a rare neoplasm of malignant lymphoid cells presenting with one or more bone lesions without nodal or other extranodal involvement. It accounts for approximately 1% of all lymphomas and 7% of malignant primary bone tumors. Diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS) represents the predominant histological type and constitutes over 80% of all cases. PBL may occur at all ages with a typical diagnosis age of 45-60 years and a slight male predominance. Local bone pain, soft tissue edema, palpable mass and pathological fracture are the most common clinical features. Diagnosis of the disease, which is frequently delayed due to its non-specific clinical presentation, is based on the combination of clinical examination and imaging studies and confirmed by combined histopathological and immunohistochemical examination. PBL can develop in any part of the skeleton, although it occurs most commonly in the femur, humerus, tibia, spine and pelvis. The imaging appearance of PBL is highly variable and unspecific. In terms of the cell-of-origin, most cases of primary bone DLBCL (PB-DLBCL), NOS belong to the germinal center B-cell-like subtype and specifically originate from germinal center centrocytes. PB-DLBCL, NOS has been considered a distinct clinical entity based on its particular prognosis, histogenesis, gene expression and mutational profile and miRNA signature. PBL carries a favorable prognosis, especially when treated with combined chemoradiotherapy.
ISSN:2079-9721
2079-9721
DOI:10.3390/diseases11010042