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{{SI}}
{{SI}}                                                                  
{{CMG}} {{AE}} {{MV}}
{{SK}} Synonym 1; Synonym 2; Synonym 3
==Overview==


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


==Historical Perspective==
*Primary lymphoma of the bone was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
*In [year], [gene] mutations were first identified in the pathogenesis of primary lymphoma of the bone.
*In [year], the first [discovery] was developed by [scientist] to treat/diagnose primary lymphoma of the bone.
==Classification==
==Classification==
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.
*Primary lymphoma of the bone may be classified according to [classification method] into [number] subtypes/groups:
 
:*[group1]
:*[group2]
:*[group3]
*Other variants of primary lymphoma of the bone include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
==Pathophysiology==
==Pathophysiology==
PLB most commonly are large [[cell]] or mixed small and [[large cell lymphoma]]s 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]].
*The pathogenesis of primary lymphoma of the bone is characterized by [feature1], [feature2], and [feature3].
 
*The [gene name] gene/Mutation in [gene name] has been associated with the development of primary lymphoma of the bone, involving the [molecular pathway] pathway.
The [[femur]] is the most common site and is affected in 25% of cases. Vertebral involvement is not unusual.
*On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of primary lymphoma of the bone.
 
*On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of primary lymphoma of the bone.
==Causes==
* Primary lymphoma of the bone may be caused by either [cause1], [cause2], or [cause3].
* Primary lymphoma of the bone is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
* 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 [clinical feature 1], [clinical feature 2], and [clinical feature 3], such as:
:*[Differential dx1]
:*[Differential dx2]
:*[Differential dx3]
==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of primary lymphoma of the bone is approximately [number or range] per 100,000 individuals worldwide.
* In [year], the incidence of primary lymphoma of the bone was estimated to be [number or range] cases per 100,000 individuals in [location].
===Age===
===Age===
The peak prevalence is among patients in the 6th to 7th decades of life.
*Patients of all age groups may develop primary lymphoma of the bone.
 
==Prognosis==
*Primary lymphoma of the bone is more commonly observed among patients aged [age range] years old.
Distinguishing primary bone lymphoma from other bone tumors is important because PLB has a better response to therapy and a better prognosis.
*Primary lymphoma of the bone is more commonly observed among [elderly patients/young patients/children].
 
==Diagnosis==
===Gender===
===Symptoms===
*Primary lymphoma of the bone affects men and women equally.
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>
 
*[Gender 1] are more commonly affected with primary lymphoma of the bone than [gender 2].
===Imaging===
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
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.
 
===Race===
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.
*There is no racial predilection for primary lymphoma of the bone.
*Primary lymphoma of the bone usually affects individuals of the [race 1] race.
*[Race 2] individuals are less likely to develop primary lymphoma of the bone.
==Risk Factors==
*Common risk factors in the development of primary lymphoma of the bone are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
== 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, [#%] of patients with primary lymphoma of the bone may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
*Common complications of primary lymphoma of the bone include [complication 1], [complication 2], and [complication 3].
*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 ==
===Diagnostic Criteria===
*The diagnosis of primary lymphoma of the bone is made when at least [number] of the following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]
=== Symptoms ===
*Primary lymphoma of the bone is usually asymptomatic.
*Symptoms of primary lymphoma of the bone may include the following:
:*[symptom 1]
:*[symptom 2]
:*[symptom 3]
:*[symptom 4]
:*[symptom 5]
:*[symptom 6]
=== Physical Examination ===
*Patients with primary lymphoma of the bone usually appear [general appearance].
*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.


==Treatment==
*A  [positive/negative] [test name] is diagnostic of primary lymphoma of the bone.
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.
*An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of primary lymphoma of the bone.
*Other laboratory findings consistent with the diagnosis of primary lymphoma of the bone include [abnormal test 1], [abnormal test 2], and [abnormal test 3].
===Imaging Findings===
*There are no [imaging study] findings associated with primary lymphoma of the bone.
*[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].
==References==
==References==
{{reflist|2}}
{{Reflist|2}}
 
{{WH}}
[[Category:Pick One of 28 Approved]]
{{WS}}
 
[[Category:Disease]]
[[Category:Oncology]]
[[Category:Orthopedics]]

Revision as of 19:50, 2 May 2016

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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

Historical Perspective

  • Primary lymphoma of the bone was first discovered by [scientist name], a [nationality + occupation], in [year] during/following [event].
  • In [year], [gene] mutations were first identified in the pathogenesis of primary lymphoma of the bone.
  • In [year], the first [discovery] was developed by [scientist] to treat/diagnose primary lymphoma of the bone.

Classification

  • Primary lymphoma of the bone may be classified according to [classification method] into [number] subtypes/groups:
  • [group1]
  • [group2]
  • [group3]
  • Other variants of primary lymphoma of the bone include [disease subtype 1], [disease subtype 2], and [disease subtype 3].

Pathophysiology

  • The pathogenesis of primary lymphoma of the bone is characterized by [feature1], [feature2], and [feature3].
  • The [gene name] gene/Mutation in [gene name] has been associated with the development of primary lymphoma of the bone, involving the [molecular pathway] pathway.
  • On gross pathology, [feature1], [feature2], and [feature3] are characteristic findings of primary lymphoma of the bone.
  • On microscopic histopathological analysis, [feature1], [feature2], and [feature3] are characteristic findings of primary lymphoma of the bone.

Causes

  • Primary lymphoma of the bone may be caused by either [cause1], [cause2], or [cause3].
  • Primary lymphoma of the bone is caused by a mutation in the [gene1], [gene2], or [gene3] gene[s].
  • 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 [clinical feature 1], [clinical feature 2], and [clinical feature 3], 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.
  • In [year], the incidence of primary lymphoma of the bone was estimated to be [number or range] cases per 100,000 individuals in [location].

Age

  • Patients of all age groups may develop primary lymphoma of the bone.
  • Primary lymphoma of the bone is more commonly observed among patients aged [age range] years old.
  • Primary lymphoma of the bone is more commonly observed among [elderly patients/young patients/children].

Gender

  • Primary lymphoma of the bone affects men and women equally.
  • [Gender 1] are more commonly affected with primary lymphoma of the bone than [gender 2].
  • The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.

Race

  • There is no racial predilection for primary lymphoma of the bone.
  • Primary lymphoma of the bone usually affects individuals of the [race 1] race.
  • [Race 2] individuals are less likely to develop primary lymphoma of the bone.

Risk Factors

  • Common risk factors in the development of primary lymphoma of the bone are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].

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, [#%] of patients with primary lymphoma of the bone may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
  • Common complications of primary lymphoma of the bone include [complication 1], [complication 2], and [complication 3].
  • 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

Diagnostic Criteria

  • The diagnosis of primary lymphoma of the bone is made when at least [number] of the following [number] diagnostic criteria are met:
  • [criterion 1]
  • [criterion 2]
  • [criterion 3]
  • [criterion 4]

Symptoms

  • Primary lymphoma of the bone is usually asymptomatic.
  • Symptoms of primary lymphoma of the bone may include the following:
  • [symptom 1]
  • [symptom 2]
  • [symptom 3]
  • [symptom 4]
  • [symptom 5]
  • [symptom 6]

Physical Examination

  • Patients with primary lymphoma of the bone usually appear [general appearance].
  • 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.
  • A [positive/negative] [test name] is diagnostic of primary lymphoma of the bone.
  • An [elevated/reduced] concentration of [serum/blood/urinary/CSF/other] [lab test] is diagnostic of primary lymphoma of the bone.
  • Other laboratory findings consistent with the diagnosis of primary lymphoma of the bone include [abnormal test 1], [abnormal test 2], and [abnormal test 3].

Imaging Findings

  • There are no [imaging study] findings associated with primary lymphoma of the bone.
  • [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].

References

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