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{{SI}}
{{SI}}                                                                  
{{CMG}}; {{AE}} John Fani Srour, M.D.; {{MV}}
{{SK}} 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]] [[Tumor|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 [[Viscus|visceral]] subtypes. [[Mutation|Mutations]] in BCL2 and [[BCL6]] [[Gene|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 [[Adult|adults]] and [[Old age|elderly]] [[Patient|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.<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>
 
==Classification==
Primary lymphoma of the bone may be [[Classification|classified]] according to location into 4 groups:<ref name="radio">Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016 </ref>
*PBL 1: [[solitary]] [[bone]] [[lymphoma]]
*PBL 2: multifocal [[Bone|bony]] [[Lesion|lesions]]
*PBL 3: cases with distant [[Lymph node|nodal]] [[disease]]
*PBL 4: cases with [[Viscus|visceral]] [[disease]]
 
==Pathophysiology==
* 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>
 
* [[Mutation|Mutations]] in [[BCL2]] and [[BCL6]] have been associated with the development of primary lymphoma of the bone.
 
* 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.


{{CMG}}; '''Associate-Editor-In-Chief:''' John Fani Srour, M.D.
* 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>


==Overview==
**Fish [[flesh]] appearance of [[lymphoma]]
Primary [[lymphoma]] of the bone (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 6% of cases.
**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==
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 [[Disease|diseases]] that cause [[bone pain]], [[fever]], and limited range of motion such as:<ref name="radio">Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016 </ref>
*[[Osteosarcoma]]
*[[Bone metastasis]]
*[[Spindle neuron|Spindle cell]] [[sarcoma]]
*[[Myeloid]] [[sarcoma]]


==Epidemiology and Demographics==
==Epidemiology and Demographics==
===Age===
* Primary lymphoma of the bone accounts for less than 5% of all primary [[bone]] [[Tumor|tumors]].
The peak prevalence is among patients in the 6th to 7th decades of life.
 
* 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 [[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.
 
==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>
 
== Screening ==
There is insufficient evidence to recommend routine [[Screening (medicine)|screening]] for primary lymphoma of the bone.
 
== Natural History, Complications, and Prognosis==
*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]].
*If left untreated, [[Patient|patients]] with primary lymphoma of the bone may progress to develop [[metastasis]].
 
* The most common [[Complication (medicine)|complication]] of primary lymphoma of the bone is a [[Bone fracture|pathological fracture]].
 
* 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 ==
=== Diagnostic Study of Choice ===
[[MRI]] is the [[imaging]] modality of choice for the diagnosis of primary lymphoma of the bone.


==Pathology==
=== History and Symptoms ===
PLB most commonly are large cell or mixed small and large cell lymphomas of the [[B-cell]] lineage. On the basis of the current [[WHO classification]], the majority of cases would be characterized as [[diffuse large B-cell lymphoma]]. [[T-cell]] primary bone lymphomas are rare and almost all lymphomas will express common [[leukocyte]] antigen and B-cell markers CD20 and CD79a.
[[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]]
*Limited [[range of motion]]  
*Morning stiffness
*[[Low grade fever]]
*[[Night sweats]]


The [[femur]] is the most common site and is affected in 25% of cases. Vertebral involvement is not unusual.
=== Physical Examination ===
[[Patient|Patients]] with primary lymphoma of the bone usually appear [[Pallor|pale]] or [[Malnutrition|malnourished]]. [[Physical examination]] may be remarkable for:
*[[edema|Swelling]] of the affected area
*Limited [[range of motion]]
*[[Gait]] disturbances
*[[Palpation|Palpable]] [[mass]]


==Classification==
=== Laboratory Findings ===
Although the original criteria for PLB described by Coley implied the involvement of a solitary bone, an expansion was suggested by Ostrowski et al in 1986 when they sub-classified osseous lymphoma into four groups. Group 2 includes cases in which more than one bone was affected but no nodal or visceral disease was present.  
There are no specific [[Medical laboratory|laboratory]] findings associated with primary lymphoma of the bone. In some cases, non-specific findings may include:<ref name="radio">Primary lymphoma of the bone. Radiopedia. http://radiopaedia.org/articles/primary-bone-lymphoma Accessed on May 1st 2016 </ref>
*Elevated [[LDH]]
*Elevated β2 microglobulin
 
===Electrocardiogram===
There are no [[The electrocardiogram|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:<ref name="radio" />
*Solitary metadiaphyseal lesion
*Layered [[periosteum|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 [[Computed tomography|CT scan]] findings associated with primary lymphoma of the bone.


==Diagnosis==
===MRI===
===Symptoms===
On [[MRI]], findings of primary lymphoma of the bone include:
Primary lymphoma of bone manifests with insidious bone pain, local swelling, a palpable mass, and systemic symptoms such as [[weight loss]] and [[fever]]. Vertebral involvement can cause [[radicular]] symptoms and, in some case, lead to compression of the [[spinal cord]].<br>
*Abnormalities in [[bone marrow]]
*[[Soft tissue]] [[mass]]


===Imaging===
=== Other Imaging Findings ===
The radiographic appearances of PLB are variable, and because the lesion can appear near normal on plain radiographs,[[ bone scan]] or [[magnetic resonance imaging]] (MRI) should be used.
There are no other [[imaging]] findings associated with primary lymphoma of the bone.


The presence of a solitary metadiaphyseal lesion with a layered periosteal reaction on plain radiographs and a soft-tissue mass on MRI is highly suggestive of lymphoma. However, diffuse multifocal osseous involvement and variable radiographic appearances of primary bone lymphoma are well known features.
=== Other Diagnostic Studies ===
There are no other [[Diagnosis|diagnostic]] studies associated with primary lymphoma of the bone.


==Treatment==
== Treatment ==
Treatment for PLB often involves [[radiation therapy]] to control the tumor in the affected bone. In certain instances, surgical intervention for control of the primary bone lesion may be desirable or necessary. Chemotherapeutic regimens are employed in cases of diffuse osseous involvement.
=== 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 ===
[[Surgery|Surgical]] intervention is not recommended among [[Patient|patients]] with primary lymphoma of the bone.
=== Primary Prevention ===
There are no established measures for the [[Prevention (medical)|primary prevention]] of primary lymphoma of the bone.


==Prognosis==
=== Secondary Prevention ===
Distinguishing primary bone lymphoma from other bone tumors is important because PLB has a better response to therapy and a better prognosis.<br>
There are no established measures for the [[Prevention (medical)|secondary prevention]] of primary lymphoma of the bone.


==References==
==References==
{{reflist|2}}
{{Reflist|2}}
[[Category:Oncology]]
 


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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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