Clinical features, management and prognosis of multifocal primary bone lymphoma: a retrospective study of the international extranodal lymphoma study group (the IELSG 14 study)

Abstract

Summary'Multifocal bone lymphoma' or 'polyostotic lymphoma' is a neoplasm with exclusive multifocal involvement of the skeleton, without affecting lymph nodes or other soft tissues. Knowledge on this uncommon condition is limited because the related literature is sparse and fragmentary. We reviewed cases of multifocal bone diffuse large B-cell lymphoma (MB-DLBCL) registered in a clinico-pathological database of the International Extranodal Lymphoma Study Group that includes 499 cases of bone lymphoma. Clinical features, management and prognosis of 37 MB-DLBCL patients and 63 'controls' (stage-IV DLBCL and skeletal involvement) were analysed. Presentation and treatment of MB-DLBCL and controls were identical. At a median follow-up of 52 months (10-189), MB-DLBCL patients exhibited a significantly better response rate (92% vs. 65%; P = 0Á002), progression-free survival (5-year: 56 AE 9% vs. 34 AE 6%; P = 0Á003) and overall survival (5-year: 74 AE 8% vs. 36 AE 7%; P = 0Á002). Among MB-DLBCL patients, the use of post-chemo radiotherapy was associated with better overall survival (5-year: 83 AE 12% vs. 55 AE 16%; P = 0Á003). Two MB-DLBCL patients (5Á4%) with spine and skull involvement experienced central nervous system (CNS) relapse. Thus, MB-DLBCL patients exhibit a significantly better prognosis compared to patients with advanced-stage DLBCL, and should be treated with conventional anthracycline-based chemotherapy, keeping intensified treatment for relapsing cases, considering involved-field radiotherapy, and CNS prophylaxis in high-risk patients.Keywords: primary bone lymphoma, polyostotic lymphoma, osteolymphoma, multifocal bone lymphoma.Primary bone lymphoma is a rare disease, accounting for about 3% of all primary bone malignancies, <5% of extranodal lymphomas and 1-2% of all lymphomas in adults (Limb et al, 1994;Fletcher, 2006;Maruyama et al, 2007;Ramadan et al, 2007;Jawad et al, 2010). This lymphoma arises more often in the femur, spine, and pelvic bones, but,

Primary bone diffuse large B-cell lymphoma: a retrospective evaluation on 76 cases from French institutional and LYSA studies

“…Several retrospective studies reported PB lymphomas separately and suggested that these topographic variants may have a seemingly favorable prognostic particularly when combining chemotherapy and radiation therapy (Table 2). [7,11,13,27] However, these studies report heterogeneous data and while DLBCL is the most frequently described histological subtype, other lymphomas are reported including follicular or anaplastic types. In contrast to most studies (Table 2), we excluded from our analysis non-DLBCL lymphomas.…”

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

“…For instance, in the IELSG-14 study, the addition of radiotherapy following chemotherapy, or the use of larger radiation fields and doses, were not associated with an improved outcome in patients treated with chemoradiotherapy (9). Although previous studies have obtained contradicting results, chemoradiotherapy is likely to be superior to single modality therapy, which may be advantageous for localized unifocal bulky sites of disease, which allows a shorter chemotherapy course (1,30,35), or for multifocal disease in relapsing cases (36). Anthracycline-containing chemotherapy has become the standard therapy of DLBCL, while numerous studies have demonstrated increased long term remission rates with the incorporation of rituximab (33,(37)(38)(39)(40)(41)(42).…”

Other Iatrogenic Immunodeficiency-associated Lymphoproliferative Disorder Presenting as Primary Bone Lymphoma in a Patient with Rheumatoid Arthritis

“…Conversely, the five-year OS and PFS rates of multifocal bone DLBCL, such as this case, were reported to be 36.7-74% and 13.4-34%, respectively (1,2). Several studies have identified predictors of the prognosis of PBL.…”

“…It accounts for 5% of extranodal lymphomas (1)(2)(3)(4)(5)(6), less than 1% of all non-Hodgkin's lymphomas (3,6,7), and 3% of all primary malignant bone tumors (2,5,8). Patients with rheumatoid arthritis (RA) tend to develop lymphoproliferative disorders (LPD) at a frequency of 2.0-5.5 times higher than that in the general population.…”