Primary lymphoma of the bone

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: PLB

Overview

Primary lymphoma of the bone (also known as "PLB" ) is an uncommon malignancy that accounts for less than 5% of all primary bone tumors. The majority of cases are of the non-Hodgkin type, with Hodgkin disease accounting for only 6% of cases.

Historical Perspective

• Primary lymphoma of the bone was first discovered by Ostrowski in 1986.

Classification

• Primary lymphoma of the bone may be classified according to location into 4 groups:
• PBL 1: solitary bone lymphoma
• PBL 2: multifocal bony lesions
• PBL 3: cases with distant nodal disease
• PBL 4: cases with visceral disease

Pathophysiology

• The pathogenesis of primary lymphoma of the bone is characterized by

• Osteoclast activating factors
• Commonly located in bone marrow, axial skeleton, or femur
• The femur is the most common site and is affected

• The mutation in BCL2 and BCL6, has been associated with the development of primary lymphoma of the bone.
• On gross pathology, characteristic findings of primary lymphoma of the bone, include:

• Fish flesh appearance of lymphoma
• Extraosseous extension and indistinct margins

• On microscopic histopathological analysis, characteristic findings of primary lymphoma of the bone, include:

• Diffuse growth pattern
• Infiltrating between bone trabeculae
• Large atypical cells
• Abundant cytoplasm
• Large bizarre cells, centroblasts, and immunoblasts
• Positive CD20 and CD79a

Causes

• There are no established causes for primary lymphoma of the bone.

Differentiating Primary Lymphoma of the Bone from Other Diseases

• Primary lymphoma of the bone must be differentiated from other diseases that cause bone pain, fever, and limited range of motion, such as:

• Osteosarcoma
• Bone metastasis
• Spindle cell sarcoma
• Myeloid sarcoma

Epidemiology and Demographics

• Primary lymphoma of the bone is rare
• The prevalence of primary lymphoma of the bone is approximately 0.01 per 100,000 individuals worldwide.

Age

• Primary lymphoma of the bone is more commonly observed among patients aged 50 to 70 years old.
• Primary lymphoma of the bone is more commonly observed among middle aged adults and elderly patients.

Gender

• Primary lymphoma of the bone affects men more frequently than women.

Race

• There is no racial predilection for primary lymphoma of the bone.

Risk Factors

• The most common risk factors in the development of primary lymphoma of the bone is previous exposure to radiation.

Natural History, Complications and Prognosis

• The majority of patients with primary lymphoma of the bone are symptomatic at the time of diagnosis.
• Early clinical features include bone pain, or palpable mass.
• If left untreated, patients with primary lymphoma of the bone may progress to develop
• Common complications of primary lymphoma of the bone, include:

• Prognosis is generally good, and the 5-year survival rate of patients with primary lymphoma of the bone is approximately > 80%.

Diagnosis

Symptoms

• Symptoms of primary lymphoma of the bone may include the following:

• Bone pain
• Limited range of motion
• Morning stiffness
• Low grade fever
• Night sweating

Physical Examination

• Patients with primary lymphoma of the bone usually appear pale or malnourished.
• Physical examination may be remarkable for:

• Swelling
• Limited range of motion
• Gait disturbances
• Palpable mass

Laboratory Findings

• There are no specific laboratory findings associated with primary lymphoma of the bone.
• In some cases, findings may include:

• Elevated LDH
• Elevated B2 microglobulin

Imaging Findings

• MRI is the imaging modality of choice for primary lymphoma of the bone.
• On conventional radiograph, findings of primary lymphoma of the bone, include:

• Non-specific features
• Affected bone may be normal or affected by lytic, sclerotic or mixed pattern.
• The most common is a lytic pattern with permeative bone destruction and a wide zone of transition

• On MRI, findings of primary lymphoma of the bone, include:

• Abnormalities in bone marrow

Treatment

Medical Therapy

• The mainstay of therapy for primary lymphoma of the bone is chemotherapy.
• Response to chemotherapy or radiotherapy can be monitored with imaging findings.

Surgery

• Surgery is not recommended among patients with primary lymphoma of the bone.

Prevention

• There are no primary preventive measures available for primary lymphoma of the bone.