Multiple osteolytic lesions due to Double-Expressor Primary non-Hodgkin Lymphoma of the Bone

Abstract

Primary non-Hodgkin lymphoma of the bone (PLB) is a rare type of non-Hodgkin's lymphoma (NHL) that affects the skeletal system with or without regional lymph node involvement. We present the case of a 74-year-old female patient with pain due to multifocal osteolytic lesions. The diagnosis of diffuse large B-cells (non-GCB) phenotype was made by clinical, laboratory, histopathological examination accompanied by an extensive immunohistochemical profile of one of the skeletal lesions.

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

“…The most common symptom of PBL is local bone pain in the affected area, which is not relieved by rest and has been characterized as insidious, intermittent and progressively worsening [ 2 , 3 , 4 , 7 , 9 , 15 , 18 , 19 , 20 , 21 , 22 , 23 ]. Other less common manifestations include soft tissue edema, palpable mass, pathological fracture, restricted range of motion in the involved articulation and “B” symptoms, namely fever, night sweats and unintentional weight loss [ 2 , 3 , 4 , 8 , 11 , 18 , 19 , 22 , 23 , 24 , 25 ]. The latter are present in a minority of patients and are less frequent in PBL compared to systemic lymphomas [ 2 , 24 ].…”

“…PBL can develop in any part of the skeleton, although is most common in the femur, humerus, tibia, spine and pelvis [ 2 , 3 , 4 , 7 , 8 , 9 , 10 , 17 , 20 , 21 , 22 , 24 , 31 ]. Other less common sites of occurrence include the skull, forearm, scapula, clavicle, patella, hands and feet [ 3 , 4 , 9 , 10 , 17 , 21 , 25 ].…”

“…The histological and immunohistochemical appearance of PBL has been reported in a number of studies [ 2 , 3 , 4 , 7 , 8 , 9 , 14 , 15 , 16 , 18 , 19 , 20 , 22 , 23 , 24 , 25 , 26 , 27 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 , 69 , 70 , 71 , 72 , 73 , 74 ,…”

“…The findings of articles published before 2006 [ 16 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 , 56 , 57 , 58 , 59 , 60 , 61 , 62 , 63 , 64 , 65 , 66 , 67 , 68 ] were summarized in our previous review about PBL [ 78 ]. Herein, we analyze the histological and immunohistochemical features of PBL described in subsequent studies [ 2 , 3 , 4 , 7 , 8 , 9 , 14 , 15 , 18 , 19 , 20 , 22 , 23 , 24 , 25 , 26 , 27 , 69 , 70 , 71 , 72 ...…”

“…PBL must be distinguished from the secondary bone involvement of systemic lymphomas, which occurs in 16–20% of lymphoma patients and carries a poor prognosis, whereas PBL is considered to have the most favorable prognosis of all malignant primary bone tumors [ 4 , 5 , 6 ]. Diagnosis of PBL, which is frequently delayed due to its non-specific clinical manifestations and equivocal radiographic findings, is based on the combination of clinical examination and imaging studies and confirmed by histopathological examination with immunohistochemical staining [ 7 , 8 , 9 ]. The present review summarizes the epidemiological, clinical, radiological and histological features, as well as the etiology, histogenesis, treatment and prognosis of PBL.…”