title: 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: '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 = 0·002), progression-free survival (5-year: 56 ± 9% vs. 34 ± 6%; P = 0·003) and overall survival (5-year: 74 ± 8% vs. 36 ± 7%; P = 0·002). Among MB-DLBCL patients, the use of post-chemo radiotherapy was associated with better overall survival (5-year: 83 ± 12% vs. 55 ± 16%; P = 0·003). Two MB-DLBCL patients (5·4%) 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.
authors.affiliation: Unit of Lymphoid Malignancies, Department of Onco-Haematology, San Raffaele Scientific Institute, Milan, Italy.
authors.forename: Carlo
authors.initials: C
authors.lastname: Messina
authors.forename: Andrés J M
authors.initials: AJ
authors.lastname: Ferreri
authors.forename: Silvia
authors.initials: S
authors.lastname: Govi
authors.forename: Marta
authors.initials: M
authors.lastname: Bruno-Ventre
authors.forename: Elías A
authors.initials: EA
authors.lastname: Gracia Medina
authors.forename: David
authors.initials: D
authors.lastname: Porter
authors.forename: John
authors.initials: J
authors.lastname: Radford
authors.forename: Dae S
authors.initials: DS
authors.lastname: Heo
authors.forename: Hee Y
authors.initials: HY
authors.lastname: Park
authors.forename: Barbara
authors.initials: B
authors.lastname: Pro
authors.forename: Jayasingham
authors.initials: J
authors.lastname: Jayamohan
authors.forename: Carlo
authors.initials: C
authors.lastname: Visco
authors.forename: Lydia
authors.initials: L
authors.lastname: Scarfò
authors.forename: Emanuele
authors.initials: E
authors.lastname: Zucca
authors.forename: Mary
authors.initials: M
authors.lastname: Gospodarowicz
authors.forename: David
authors.initials: D
authors.lastname: Christie
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D001859:Bone Neoplasms / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy / Q000628:therapy*; D016022:Case-Control Studies; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D016403:Lymphoma, Large B-Cell, Diffuse / Q000188:drug therapy / Q000473:pathology* / Q000532:radiotherapy / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult
keywords: multifocal bone lymphoma; osteolymphoma; polyostotic lymphoma; primary bone lymphoma