INTRODUCTION

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 "Initial 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".)

EPIDEMIOLOGY

Primary lymphoma of bone (PLB) is a rare disease that accounts for less than 2 percent of all lymphomas in adults [1]. 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 percent of primary bone tumors [6] and 3 to 5 percent of all extranodal non-Hodgkin lymphomas [7,8].

Men are diagnosed slightly more frequently than women with a male:female ratio ranging from 1.2 to 1.8 [9-11]. 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) [9,11]. While PLB occurs in children, pediatric PLB is considered a distinct clinical entity and the approach to treatment differs from that of adults.

CLINICAL PRESENTATION

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 [10]. 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.