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: Synonym 1; Synonym 2; Synonym 3

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 [scientist name], a [nationality + occupation], in [year] during/following [event].

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

• The 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.

Causes

• The most common cause of primary lymphoma of the bone is

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:

• [Differential dx1]
• [Differential dx2]
• [Differential dx3]

Epidemiology and Demographics

• The prevalence of primary lymphoma of the bone is approximately [number or range] 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 adults and elderly patients.

Gender

• Primary lymphoma of the bone affects men and women equally.

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 remain asymptomatic for [duration/years].
• Early clinical features include [manifestation 1], [manifestation 2], and [manifestation 3].
• 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 [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with primary lymphoma of the bone is approximately [#%].

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:

• [finding 1]
• [finding 2]
• [finding 3]
• [finding 4]
• [finding 5]
• [finding 6]

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

• [Imaging study 1] is the imaging modality of choice for primary lymphoma of the bone.
• On [imaging study 1], primary lymphoma of the bone is characterized by [finding 1], [finding 2], and [finding 3].
• [Imaging study 2] may demonstrate [finding 1], [finding 2], and [finding 3].

Other Diagnostic Studies

• Primary lymphoma of the bone may also be diagnosed using [diagnostic study name].
• Findings on [diagnostic study name] include [finding 1], [finding 2], and [finding 3].

Treatment

Medical Therapy

• There is no treatment for primary lymphoma of the bone; the mainstay of therapy is supportive care.

• The mainstay of therapy for primary lymphoma of the bone is [medical therapy 1] and [medical therapy 2].
• [Medical therapy 1] acts by [mechanism of action1].
• Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].

Surgery

• Surgery is the mainstay of therapy for primary lymphoma of the bone.
• [Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of primary lymphoma of the bone.
• [Surgical procedure] can only be performed for patients with [disease stage] primary lymphoma of the bone.

Prevention

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

• Effective measures for the primary prevention of primary lymphoma of the bone include [measure1], [measure2], and [measure3].

• Once diagnosed and successfully treated, patients with primary lymphoma of the bone are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].