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

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.

Conditional survival of patients with primary bone lymphoma of the spine: how survival changes after initial diagnosis

“…PBL typically portends a favorable prognosis ( 2 , 10 ). Indeed, it is deemed to have the most favorable prognosis among all primary malignant bone neoplasms and better prognosis than secondary bone lymphoma ( 1 , 21 ).…”

“…And the estimated 5-year survival rate (OS) for PBL patients has been reported to fall within a range of approximately 55%-75% ( 4 , 5 ). Non-Hodgkin lymphoma (NHL) constitutes the predominant category of PBLs with diffuse large B-cell lymphoma (DLBCL) accounting for more than 80% of all instances ( 2 , 6 ). PBL can occur across all age groups, with a prevalent diagnostic age range of 45-60 years, and males are predominantly affected compared to females ( 2 , 7 , 8 ).…”

“…Non-Hodgkin lymphoma (NHL) constitutes the predominant category of PBLs with diffuse large B-cell lymphoma (DLBCL) accounting for more than 80% of all instances ( 2 , 6 ). PBL can occur across all age groups, with a prevalent diagnostic age range of 45-60 years, and males are predominantly affected compared to females ( 2 , 7 , 8 ). PBL of the spine is an uncommon entity, typically arising as metastatic disease from additional sites ( 2 ).…”

“…PBL can occur across all age groups, with a prevalent diagnostic age range of 45-60 years, and males are predominantly affected compared to females ( 2 , 7 , 8 ). PBL of the spine is an uncommon entity, typically arising as metastatic disease from additional sites ( 2 ). The clinical manifestations of spinal PBL are commonly non-specific, often resulting in a diagnostic delay, and usually necessitating a biopsy for a definitive diagnosis ( 5 , 9 – 11 ).…”

“…Owing to the scarcity of the PBL of the spine, there is a scarcity of comprehensive data on the optimal treatment strategy and prognosis. The prevailing modalities encompass chemotherapy or immunochemotherapy with or without radiotherapy; while surgical intervention are generally restricted ( 2 ). Furthermore, the late complications after chemotherapy in such patients are currently under scrutiny, including bone loss and the development of fragility fractures ( 12 ).…”