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==Pathophysiology==
==Pathophysiology==
===Pathogenesis===
* The [[pathogenesis]] of primary lymphoma of the bone is characterized by excessive [[osteoclast]] activation.<ref name="pathology">Sheren Yones, M.D. Primary Lymphoma of the Bone. Pathology Outlines. http://www.pathologyoutlines.com/topic/bonelymphomageneral.html</ref>
The [[pathogenesis]] of primary lymphoma of the bone is characterized by excessive [[osteoclast]] activation.<ref name="pathology">Sheren Yones, M.D. Primary Lymphoma of the Bone. Pathology Outlines. http://www.pathologyoutlines.com/topic/bonelymphomageneral.html</ref>
===Genetics===
[[Mutation|Mutations]] in [[BCL2]] and [[BCL6]] have been associated with the development of primary lymphoma of the bone.
===Gross Pathology===
Common locations of primary lymphoma of the bone are [[bone marrow]], [[axial skeleton]], or [[femur]]. The [[metaphysis]] of the [[femur]] is the most commonly affected site. On [[gross pathology]], characteristic findings of primary lymphoma of the bone include:<ref name="pathology">Sheren Yones, M.D. Primary Lymphoma of the Bone. Pathology Outlines. http://www.pathologyoutlines.com/topic/bonelymphomageneral.html</ref>
*Fish [[flesh]] appearance of [[lymphoma]]
*Extraosseous extension and indistinct margins


===Microscopic Pathology===
* [[Mutation|Mutations]] in [[BCL2]] and [[BCL6]] have been associated with the development of primary lymphoma of the bone.
On [[microscopic]] [[Histopathology|histopathological]] [[analysis]], characteristic findings of primary lymphoma of the bone include:<ref name="pathology">Sheren Yones, M.D. Primary Lymphoma of the Bone. Pathology Outlines. http://www.pathologyoutlines.com/topic/bonelymphomageneral.html</ref>
 
*Diffuse growth pattern
* Common locations of primary lymphoma of the bone are [[bone marrow]], [[axial skeleton]], or [[femur]]. The [[metaphysis]] of the [[femur]] is the most commonly affected site.
*Infiltrating between [[Cancellous bone|bone trabeculae]]
 
*Large atypical [[Cell (biology)|cells]]
* On [[gross pathology]], characteristic findings of primary lymphoma of the bone include:<ref name="pathology">Sheren Yones, M.D. Primary Lymphoma of the Bone. Pathology Outlines. http://www.pathologyoutlines.com/topic/bonelymphomageneral.html</ref>
*Abundant [[cytoplasm]]
 
*Large bizarre [[Cell (biology)|cells]], centroblasts, and [[Immunoblast|immunoblasts]]
**Fish [[flesh]] appearance of [[lymphoma]]
*Positive [[CD20]] and [[CD79a]]
**Extraosseous extension and indistinct margins
 
* On [[microscopic]] [[Histopathology|histopathological]] [[analysis]], characteristic findings of primary lymphoma of the bone include:<ref name="pathology">Sheren Yones, M.D. Primary Lymphoma of the Bone. Pathology Outlines. http://www.pathologyoutlines.com/topic/bonelymphomageneral.html</ref>
 
**Diffuse growth pattern
**Infiltrating between [[Cancellous bone|bone trabeculae]]
**Large atypical [[Cell (biology)|cells]]
**Abundant [[cytoplasm]]
**Large bizarre [[Cell (biology)|cells]], centroblasts, and [[Immunoblast|immunoblasts]]
**Positive [[CD20]] and [[CD79a]]


==Causes==
==Causes==
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==Epidemiology and Demographics==
==Epidemiology and Demographics==
Primary lymphoma of the bone accounts for less than 5% of all primary [[bone]] [[Tumor|tumors]].
* Primary lymphoma of the bone accounts for less than 5% of all primary [[bone]] [[Tumor|tumors]].
===Prevalence===
 
The [[prevalence]] of primary lymphoma of the bone is approximately 0.01 per 100,000 individuals worldwide.
* 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 [[Patient|patients]] aged 50 to 70 years old.
* Primary lymphoma of the bone is more commonly observed among [[Patient|patients]] aged 50 to 70 years old.
 
* Primary lymphoma of the bone affects [[Male|men]] more frequently than [[Female|women]].
 
* There is no racial predilection for primary lymphoma of the bone.


===Gender===
Primary lymphoma of the bone affects [[Male|men]] more frequently than [[Female|women]].
===Race===
There is no racial predilection for primary lymphoma of the bone.
==Risk Factors==
==Risk Factors==
The most common [[risk factor]] in the development of primary lymphoma of the bone is previous exposure to [[radiation]].<ref name="radio">Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016 </ref>
The most common [[risk factor]] in the development of primary lymphoma of the bone is previous exposure to [[radiation]].<ref name="radio">Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016 </ref>
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== Natural History, Complications, and Prognosis==
== Natural History, Complications, and Prognosis==
===Natural History===
*The majority of [[Patient|patients]] with primary lymphoma of the bone are [[symptomatic]] at the time of [[diagnosis]].<ref name="radio">Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016 </ref>
*The majority of [[Patient|patients]] with primary lymphoma of the bone are [[symptomatic]] at the time of [[diagnosis]].<ref name="radio">Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016 </ref>
*Early [[clinical]] features include [[bone pain]] or a [[Palpation|palpable]] [[mass]].
*Early [[clinical]] features include [[bone pain]] or a [[Palpation|palpable]] [[mass]].
*If left untreated, [[Patient|patients]] with primary lymphoma of the bone may progress to develop [[metastasis]].
*If left untreated, [[Patient|patients]] with primary lymphoma of the bone may progress to develop [[metastasis]].
===Complications===
 
The most common [[Complication (medicine)|complication]] of primary lymphoma of the bone is a [[Bone fracture|pathological fracture]].
* The most common [[Complication (medicine)|complication]] of primary lymphoma of the bone is a [[Bone fracture|pathological fracture]].
===Prognosis===
 
The [[prognosis]] of primary lymphoma of the bone is generally good; the 5-year survival rate of [[Patient|patients]] with primary lymphoma of the bone is commonly >80%.<ref name="pmid12298430">{{cite journal |vauthors=Choi JY, Hahn JS, Suh CO, Yang WI |title=Primary lymphoma of bone--survival and prognosis |journal=Korean J. Intern. Med. |volume=17 |issue=3 |pages=191–7 |year=2002 |pmid=12298430 |pmc=4531681 |doi= |url=}}</ref>
* The [[prognosis]] of primary lymphoma of the bone is generally good; the 5-year survival rate of [[Patient|patients]] with primary lymphoma of the bone is commonly >80%.<ref name="pmid12298430">{{cite journal |vauthors=Choi JY, Hahn JS, Suh CO, Yang WI |title=Primary lymphoma of bone--survival and prognosis |journal=Korean J. Intern. Med. |volume=17 |issue=3 |pages=191–7 |year=2002 |pmid=12298430 |pmc=4531681 |doi= |url=}}</ref>


== Diagnosis ==  
== Diagnosis ==  
=== Symptoms ===
=== Diagnostic Study of Choice ===
[[MRI]] is the [[imaging]] modality of choice for the diagnosis of primary lymphoma of the bone.
 
=== History and Symptoms ===
[[Symptom|Symptoms]] of primary lymphoma of the bone may include the following:<ref name="radio">Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016 </ref>
[[Symptom|Symptoms]] of primary lymphoma of the bone may include the following:<ref name="radio">Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016 </ref>
*[[Bone pain]]
*[[Bone pain]]
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*Elevated β2 microglobulin
*Elevated β2 microglobulin


===Imaging Findings===
===Electrocardiogram===
[[MRI]] is the [[imaging]] modality of choice for the diagnosis of primary lymphoma of the bone.
There are no [[The electrocardiogram|ECG]] findings associated with primary lymphoma of the bone.
====MRI====
 
On [[MRI]], findings of primary lymphoma of the bone include:
=== X-ray ===
*Abnormalities in [[bone marrow]]
On conventional radiograph, the following non-specific findings of primary lymphoma of the bone may be found:<ref name="radio" />
*[[Soft tissue]] mass
====X ray====
On conventional radiograph, the following non-specific findings of primary lymphoma of the bone may be found:<ref name="radio">Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016 </ref>
*Solitary metadiaphyseal lesion
*Solitary metadiaphyseal lesion
*Layered [[periosteum|periosteal]] reaction
*Layered [[periosteum|periosteal]] reaction
*Affected bone may be normal or affected by lytic, sclerotic or mixed pattern
*Affected bone may be normal or affected by lytic, sclerotic or mixed pattern
*A lytic pattern with permeative bone destruction and a wide zone of transition
*A lytic pattern with permeative bone destruction and a wide zone of transition
=== Echocardiography or Ultrasound ===
There are no [[echocardiography]]/[[ultrasound]] findings associated with primary lymphoma of the bone.
===CT scan===
There are no [[Computed tomography|CT scan]] findings associated with primary lymphoma of the bone.
===MRI===
On [[MRI]], findings of primary lymphoma of the bone include:
*Abnormalities in [[bone marrow]]
*[[Soft tissue]] [[mass]]
=== Other Imaging Findings ===
There are no other [[imaging]] findings associated with primary lymphoma of the bone.
=== Other Diagnostic Studies ===
There are no other [[Diagnosis|diagnostic]] studies associated with primary lymphoma of the bone.


== Treatment ==
== Treatment ==
=== Medical Therapy ===
=== 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 studies.  
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]] studies.  
=== Surgery ===
=== Surgery ===
Surgical intervention is not recommended among patients with primary lymphoma of the bone.
[[Surgery|Surgical]] intervention is not recommended among [[Patient|patients]] with primary lymphoma of the bone.
=== Prevention ===
=== Primary Prevention ===
There are no primary or secondary preventive measures available for primary lymphoma of the bone.
There are no established measures for the [[Prevention (medical)|primary prevention]] of primary lymphoma of the bone.
 
=== Secondary Prevention ===
There are no established measures for the [[Prevention (medical)|secondary prevention]] of primary lymphoma of the bone.


==References==
==References==

Latest revision as of 16:36, 2 May 2019

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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 the 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 genes have 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.[1]

Classification

Primary lymphoma of the bone may be classified according to location into 4 groups:[2]

Pathophysiology

  • Mutations in BCL2 and BCL6 have been associated with the development of primary lymphoma of the bone.
    • Fish flesh appearance of lymphoma
    • Extraosseous extension and indistinct margins

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:[2]

Epidemiology and Demographics

  • Primary lymphoma of the bone accounts for less than 5% of all primary bone tumors.
  • 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 patients aged 50 to 70 years old.
  • Primary lymphoma of the bone affects men more frequently than women.
  • There is no racial predilection for primary lymphoma of the bone.

Risk Factors

The most common risk factor in the development of primary lymphoma of the bone is previous exposure to radiation.[2]

Screening

There is insufficient evidence to recommend routine screening for primary lymphoma of the bone.

Natural History, Complications, and Prognosis

  • The prognosis of primary lymphoma of the bone is generally good; the 5-year survival rate of patients with primary lymphoma of the bone is commonly >80%.[1]

Diagnosis

Diagnostic Study of Choice

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

History and Symptoms

Symptoms of primary lymphoma of the bone may include the following:[2]

Physical Examination

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

Laboratory Findings

There are no specific laboratory findings associated with primary lymphoma of the bone. In some cases, non-specific findings may include:[2]

  • Elevated LDH
  • Elevated β2 microglobulin

Electrocardiogram

There are no ECG findings associated with primary lymphoma of the bone.

X-ray

On conventional radiograph, the following non-specific findings of primary lymphoma of the bone may be found:[2]

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

Echocardiography or Ultrasound

There are no echocardiography/ultrasound findings associated with primary lymphoma of the bone.

CT scan

There are no CT scan findings associated with primary lymphoma of the bone.

MRI

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

Other Imaging Findings

There are no other imaging findings associated with primary lymphoma of the bone.

Other Diagnostic Studies

There are no other diagnostic studies associated with primary lymphoma of the bone.

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 studies.

Surgery

Surgical intervention is not recommended among patients with primary lymphoma of the bone.

Primary Prevention

There are no established measures for the primary prevention of primary lymphoma of the bone.

Secondary Prevention

There are no established measures for the secondary prevention of primary lymphoma of the bone.

References

  1. 1.0 1.1 Choi JY, Hahn JS, Suh CO, Yang WI (2002). "Primary lymphoma of bone--survival and prognosis". Korean J. Intern. Med. 17 (3): 191–7. PMC 4531681. PMID 12298430.
  2. 2.0 2.1 2.2 2.3 2.4 2.5 2.6 Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016
  3. 3.0 3.1 3.2 Sheren Yones, M.D. Primary Lymphoma of the Bone. Pathology Outlines. http://www.pathologyoutlines.com/topic/bonelymphomageneral.html


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