Pediatric Primary Bone Lymphoma-Diffuse Large B-Cell Lymphoma: Morphologic and Immunohistochemical Characteristics of 10 Cases

Abstract

Most primary bone lymphomas (PBLs) are diffuse large B-cell lymphomas (DLBCLs). Pediatric PBL-DLBCL has a favorable prognosis but remains poorly characterized. Herein, 10 such cases are detailed. They involved 11- to 20-year-old males with bone lesions that were often painful. They were diagnosed often after months to years of symptoms, suggesting an indolent disease. All were successfully treated with chemotherapy with or without radiotherapy (0.5- to 24-year followup). Biopsy revealed that the lymphomas were paratrabecular or diffuse and were medium- to large-sized with round to irregular nuclei, dispersed chromatin, indistinct to small nucleoli, and abundant cytoplasm. Other features included varying levels of necrosis, cytoplasmic retraction, and myeloid hyperplasia. All cases marked as mature B cells, and most were CD10+ (7/10). Typical centroblastic morphologic features with nucleoli were rare, multilobated nuclei were uncommon, and CD10 negativity did not predict poor prognosis, unlike in the adult PBL-DLBCL. These findings suggest that pediatric and adult PBL-DLBCLs are distinct entities.

Primary sternum diffuse large B-cell lymphoma: A case report and review of the literature

“…Diffuse large B-cell lymphoma (DLBCL) is the most common histological type of PBL, and the majority of patients have limited-stage disease (stages IE-IIE) at presentation (4,(6)(7)(8)(9). The most common symptoms at presentation are pain, swelling and pathologic fractures (PFs), however, systemic symptoms, such as fever, night sweats and weight loss, occur less frequently in PBL when compared with other NHLs (10).…”

Primary Bone Lymphoma of Humerus Diagnosed by FNAC- A Rare Case Report

“…Primary bone lymphoma is rare accounting for less than 1 % of all Non-Hodgkin's lymphoma [4][5][6][7]. The criteria for diagnosis of PLB is suggested by Coley are as follows: 'Lymphoma presenting in an osseous site with no evidence of disease elsewhere for at least 6 months after diagnosis' [7][8][9].…”

“…The criteria for diagnosis of PLB is suggested by Coley are as follows: 'Lymphoma presenting in an osseous site with no evidence of disease elsewhere for at least 6 months after diagnosis' [7][8][9]. The presence of regional lymph node involvement does not exclude a diagnosis of PLB, but a histological examination of the lymph node is necessary [4,6,9,10]. Although the original criteria of Coley implied the involvement of solitary bone.…”

“…Group 1 consists of solitary primary bone lymphoma and Group 2 encompasses cases in which more than one bone is affected but no nodal or visceral disease is present. Group 3 includes cases with distant nodal disease and Group 4 with visceral disease [6,7,9,10].…”

“…Vertebral involvement is not unusual [3,7,9]. In the long bones, lymphoma is usually metaphyseal or diaphyseal in location, rarely involving the epiphysis [6,8,10].…”