Epiphyseal Primary Diffuse Large B-Cell Lymphoma of Bone

Abstract

Primary lymphoma of bone (PLB) confined to the epiphysis has only been described in four other patients. Due to the rarity of this entity, diagnosis has often been delayed, leading to mismanagement with adverse clinical consequences. We report a fifth case of primary epiphyseal lymphoma of bone located in the left distal medial femoral epiphysis of a 13-year-old boy. Radiographic and histologic features of PLB are discussed, along with a review of the literature and pitfalls of misdiagnosis. The patient initially presented with six months of progressive left knee pain with an associated loss of passive range of motion. Imaging revealed a mixed radiolucent lesion within the left distal medial femoral epiphysis with cortical breakthrough. A core biopsy was performed revealing a blue round cell tumor. Thanks to modern immunohistochemistry techniques, a diagnosis of primary lymphoma of bone was quickly made. The patient thus avoided further surgical intervention and received the appropriate treatment of chemotherapy, with subsequent rapid resolution of the lesion. This case highlights the necessity of including primary lymphoma of bone in all epiphyseal lesion differential diagnoses, especially in the pediatric patient population when aggressive radiographic features are present.

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

“…The clinical features of PBL are generally unspecific, frequently leading to a delay in diagnosis, and, thus, a high index of suspicion is required [ 7 , 18 ]. 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 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 ].…”

“…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 ...…”

“…Tissue samples can be obtained either via image-guided percutaneous fine-needle, core-needle or open biopsy. Kenan et al [ 19 ] consider core biopsy the preferred method since it ensures an appropriate representative specimen and minimal damage to the surrounding tissue. Collecting a sufficient amount of tissue is of great significance for PBL cases because of the extensive tissue necrosis and damage during cytological preparation that characterize lymphomas and result in reduced diagnostic accuracy [ 19 ].…”