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: John Fani Srour, M.D.; Maria Fernanda Villarreal, M.D. [2]

Synonyms and keywords: PLB; Reticulum cell lymphoma

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; Hodgkin disease accounting for only 6% of cases. Primary lymphoma of the bone was first discovered by Parker and Jackson in 1939. The pathogenesis of primary lymphoma of the bone is characterized by excessive osteoclast activation. Primary lymphoma of the bone is commonly located in bone marrow, axial skeleton, or femur (most common). Primary lymphoma of the bone may be classified according to location into solitary, multifocal, distant, and visceral subtypes. Mutations in BCL2 and BCL6 has been associated with the development of primary lymphoma of the bone. Primary lymphoma of the bone is rare. The prevalence of primary lymphoma of the bone is approximately 0.01 per 100,000 individuals worldwide. Primary lymphoma of the bone is more commonly observed among middle aged adults and elderly patients. MRI is the imaging modality of choice for primary lymphoma of the bone. The mainstay of therapy for primary lymphoma of the bone is chemotherapy.

Historical Perspective

• Primary lymphoma of the bone was first discovered by Parker and Jackson in 1939.

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

Pathogenesis

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

• Excessive osteoclast activation
• Commonly located in bone marrow, axial skeleton, or femur
• The femur is the most common site of affection
• The most common site affected is the metaphysis

Genetics

• Mutations in BCL2 and BCL6 has been associated with the development of primary lymphoma of the bone.

Gross Pathology

• On gross pathology, characteristic findings of primary lymphoma of the bone include:

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

Microscopic Pathology

• 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 accounts for less than 5% of all primary bone tumors.

Prevalence

• 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 metastasis.
• The most common complication of primary lymphoma of the bone is a pathological fracture.
• Prognosis is generally good, and the 5-year survival rate of patients with primary lymphoma of the bone is commonly >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 sweats

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 β2 microglobulin

Imaging Findings

• MRI is the imaging modality of choice for the diagnosis of primary lymphoma of the bone.

MRI

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

• Abnormalities in bone marrow
• Soft tissue mass

X ray

• On conventional radiograph, the following non-specific findings of primary lymphoma of the bone may be found:

• Solitary metadiaphyseal lesion
• Layered periosteal reaction
• Affected bone may be normal or affected by lytic, sclerotic or mixed pattern
• Most commonly, a lytic pattern with permeative bone destruction and a wide zone of transition

Treatment

Medical Therapy

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

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.