Primary non-Hodgkin lymphoma of bone (PLB) presents as solitary or multiple, destructive bone lesions. The clinical presentation, diagnosis, and treatment of PLB will be discussed here.
A more general discussion of the approach to the patient with non-Hodgkin lymphoma is presented separately, as are discussions concerning the pathophysiology, diagnosis, and treatment of diffuse large B cell non-Hodgkin lymphoma, the most common tumor type found in this setting. (See "Clinical presentation and diagnosis of non-Hodgkin lymphoma" and "Evaluation and staging of non-Hodgkin lymphoma" and "Epidemiology, clinical manifestations, pathologic features, and diagnosis of diffuse large B cell lymphoma" and "Initial treatment of advanced stage diffuse large B cell lymphoma".)
Primary lymphoma of bone (PLB) is a rare disease that accounts for less than 2 percent of all lymphomas in adults . In the pediatric population it represents approximately 3 to 9 percent of non-Hodgkin lymphoma cases [2-5]. It is estimated that PLB accounts for 3 to 7 percent of primary bone tumors [6,7] and 3 to 5 percent of all extranodal non-Hodgkin lymphomas [8,9].
Men are diagnosed slightly more frequently than women with a male:female ratio ranging from 1.2 to 1.8 [10-13]. The vast majority of patients present over the age of 30 years (92 percent) with the largest cohort consisting of those over age 60 (56 percent) [10,12]. While PLB occurs in children, pediatric PLB is considered a distinct clinical entity and the approach to treatment differs from that of adults.
The majority of patients present with bone pain not relieved by rest. A palpable mass due to soft tissue extension of the bony disease is seen in about one-half of the cases . Swelling, pathologic fracture, cord compression and systemic "B" symptoms (ie, fever, weight loss, night sweats) may also be present at the time of diagnosis . Occasionally, symptoms may persist for many months before the patient seeks medical attention.