期刊
DISEASES
卷 11, 期 1, 页码 -出版社
MDPI
DOI: 10.3390/diseases11010042
关键词
bone; lymphomas; B-cells; immunohistochemistry
Primary bone lymphoma (PBL) is a rare form of malignant lymphoid cell tumor that presents with bone lesions without nodal or extranodal involvement. The most common histological type is diffuse large B-cell lymphoma (DLBCL), not otherwise specified (NOS), and it primarily occurs in the femur, humerus, tibia, spine, and pelvis. Diagnosis is often delayed due to non-specific clinical presentation, but can be confirmed through clinical examination, imaging studies, and histopathological and immunohistochemical examination. PBL has a favorable prognosis, especially when treated with combined chemoradiotherapy.
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.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据