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

Author:

Kanavos Theofilos1ORCID,Birbas Effrosyni1ORCID,Papoudou-Bai Alexandra2,Hatzimichael Eleftheria3ORCID,Kitsouli Aikaterini1,Karpathiou Georgia4ORCID,Kanavaros Panagiotis1

Affiliation:

1. Department of Anatomy-Histology-Embryology, Faculty of Medicine, School of Health Sciences, University of Ioannina, 45110 Ioannina, Greece

2. Department of Pathology, Faculty of Medicine, School of Health Sciences, University of Ioannina, 45110 Ioannina, Greece

3. Department of Hematology, Faculty of Medicine, School of Health Sciences, University of Ioannina, 45500 Ioannina, Greece

4. Department of Pathology, University Hospital of Saint-Etienne, 42055 Saint-Etienne, France

Abstract

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.

Publisher

MDPI AG

Subject

General Medicine

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