Primary non-Hodgkin’s lymphoma of bone: a clinicopathological investigation of 60 cases

Abstract

A retrospective analysis of patients presenting with primary lymphoma of bone (PLB) was performed to determine clinical factors affecting prognosis in relation to histological subtype and treatment outcome. Data from 106 patients, presenting with a PLB between 1943 and 1996, were retrieved from the files of the Netherlands Committee on Bone Tumours and Leiden University Medical Centre. The lymphomas were reclassified according to the REAL and updated Kiel classification. The clinical presentation, survival and prognostic factors were investigated. Sixty patients had sufficient clinical information and adequate follow-up to be included in the study. All 33 PLB that could be immunophenotyped were of B cell origin. According to the REAL classification, most PLB were large (B) cell lymphomas (92%) and according to the Kiel classification 45% of the tumours were centroblastic multilobated. PLB presented most often in the long bones (48%), with Ann Arbor stage I (46%), II (16%), IV (16%) and unknown (20%). Stage IV disease was exclusively caused by the presence of multiple bone lesions. Notwithstanding the heterogeneous treatment, the 5-year overall survival was 61%; 46% of patients were progression free at 5 years. Patients at presentation older than 60 had a worse overall survival (76% vs 37%, P ‫؍‬ 0.0002) and a worse progression-free period (58% vs 28%, P ‫؍‬ 0.0073). Patients with the immunoblastic subtype had a worse survival than the centroblastic mono/polymorphic subtype or the centroblastic multilobated subtype (P ‫؍‬ 0.015). Primary lymphoma of bone represents an uncommon bone tumour with a relatively homogeneous morphology and clinical behaviour. Compared to other aggressive lymphomas, PLB have a favourable prognosis.

A 38-year-old Man with Left Knee Pain

“…The majority of these tumors are nonHodgkin's lymphomas, and as observed in our patient, as much as 92% are diffuse large B cell lymphomas (historically referred to as reticulum cell sarcomas) [6,14]. Peak age at presentation is during the fifth decade, with most occurring after skeletal maturity [18].…”

“…Some series therefore have restricted the diagnosis to patients presenting with an isolated lesion of bone, although the most common site of spread is to additional osseous sites [6]. Furthermore, exclusion of patients with greater than Stage IE disease may confer the false connotation of a localized process to what is, in terms of staging, relapse, and patient survival, a systemic disease; the variable inclusion and exclusion of patients with distant sites of osseous or other systemic disease at presentation make analysis of the outcomes for the disease difficult.…”

“…The lesions are located in the lower extremity in 30% of cases, with the femur and pelvis representing the most common sites, followed by the spine and tibia [6,11], with a predilection for the metadiaphyseal region. Radiographically, primary lymphomas of bone may have a blastic or mixed appearance.…”

An Immunophenotypic and Molecular Study of Primary Large B-Cell Lymphoma of Bone

“…Clinically defined as "lymphoma presenting in an osseous site with no evidence of disease elsewhere for at least six months after diagnosis," it was first established as a clinical entity in 1939 (3). The majority of PNHLB have been more recently characterized as diffuse large B-cell lymphomas (DLBCL) using the REAL classification (4,5). Although many studies have reported the clinical, radiologic, and histopathologic parameters of PNHLB, few have addressed the molecular features to any significant extent (1, 4, 6 -10), likely because of their rarity and the paucity of tissue available in small biopsy specimens.…”

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

“…Radiotherapy was established as the standard treatment in earlier years (19,20). However, with the development of highly effective chemotherapy regimens and particularly the advent of novel agents, such as the anti-CD20 monoclonal antibody rituximab, the role of radiotherapy has been increasingly questioned by certain researchers (2,(25)(26)(27).…”