Background: Primary bone lymphoma (PBL) is a distinct clinicopathological entity. Although PBL has been reviewed in several small studies, few reflect recent improvements in primary treatment.
Methods: We used the British Columbia Cancer Agency Lymphoid Cancer Database to identify all patients with PBL (1983-2005). All were staged in a uniform manner and treated with era-specific protocols.
Results: We identified 131 patients with a median age of 63 years (18-87). One third had disease in long bones and another one third had disease in the spine, of which half presented with spinal cord compression. Patients with diffuse large-cell lymphoma (DLCL) (n=103, 79%) had 5- and 10-year overall survivals (OS) of 62% and 41%, respectively. Multivariate analysis identified three prognostic groups: age<60 with International Prognostic Index (IPI) 1-3 (n=43), age>or=60 with IPI 0-3 (n=23) and age>or=60 with IPI 4-5 (n=33), with markedly different 5-year OS of 90%, 61% and 25%, respectively (P<0.0001). Neither primary site nor pathological fracture at presentation had an impact on OS. The 3-year progression-free survival in patients who received rituximab plus combination chemotherapy with cyclophosphamide, doxorubicin, vincristine and prednisone (CHOPR) chemotherapy was 88% compared with 52% in those who received CHOP-like chemotherapy without rituximab (P=0.005). The 10-year OS for those with advanced-stage disease who received irradiation plus chemotherapy was 25% versus 56% for those who received chemotherapy alone (P=0.025). Patients received irradiation if spinal cord compression was present or residual disease at the end of chemotherapy was thought to require it.
Conclusions: PBL is usually of DLCL type and has an improved outcome with CHOPR. Younger patients with good IPI score have a favorable prognosis.