Primary bone lymphoma

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Primary bone (skeletal/osseous) lymphoma (PBL) is a less common manifestation of lymphoma than secondary involvement from disseminated lymphoma. It is a rare, ~~accounting~~ accounting for <5% of bone tumours and <1% of non-Hodgkin lymphoma.

Terminology

PBL is defined as the presence of lymphoma isolated to one bone without distant spread for six months after diagnosis. Multifocal PBL is less common and occurs with lymphoma is confined to two or more bones, again without distant spread for six months ¹.

Epidemiology

PBL can affect any age group, with peak incidence in 50-to-60 year olds. It is rare in children <10 years old. There is a ~~slightly~~slight male predominance (M:F = 1.5:1) ².

Clinical presentation

Symptoms include localised pain and swelling; , B-type symptoms; , pathological fractures;, and cord compression.

Pathology

~~Lymphoma of the non-Hodgkin type~~Diffuse large B cell lymphoma (DLBCL) is the most common subtype. The bony pelvis and femur are the most common locations ³.

Radiographic features

Plain filmradiograph

PBL has non-specific features and the affected bone may be normal~~, lytic~~ or affected by lytic, sclerotic or mixed pattern. The most common ~~pattern~~ is a lytic pattern with permeative bone destruction ~~with~~ and a wide zone of transition ¹.

MRI

Associated soft tissue masses are common. Bone marrow changes include ²:

T1: low signal
T2: high signal

Treatment and prognosis

Five-year survival rate has been reported at ~80%, much better than other bone tumours².

Differential diagnosis

For plain film presentation of permeative bone destruction consider ¹:

-<p><strong>Primary bone (skeletal/osseous) lymphoma (PBL)</strong> is a less common manifestation than secondary involvement from disseminated <a title="lymphoma" href="/articles/lymphoma">lymphoma</a>. It is a rare, accounting for <5% of <a title="Bone tumours" href="/articles/bone-tumours">bone tumours</a> and <1% of <a title="non-Hodgkin lymphoma" href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphoma</a>. </p><h4>Terminology</h4><p>PBL is defined as the presence of lymphoma isolated to one bone without distant spread for six months after diagnosis. Multifocal PBL is less common and occurs with lymphoma is confined to two or more bones, again without distant spread for six months <sup>1</sup>. </p><h4>Epidemiology</h4><p>PBL can affect any age group, with peak incidence in 50-to-60 year olds. It is rare in children <10 years old. There is a slightly male predominance (M:F = 1.5:1) <sup>2</sup>. </p><h4>Clinical presentation</h4><p>Symptoms include localised pain and swelling; B-type symptoms; <a title="Pathological fractures" href="/articles/pathological-fracture">pathological fractures</a>; and <a title="Cord compression" href="/articles/spinal-cord-compression">cord compression</a>. </p><h4>Pathology</h4><p>Lymphoma of the non-Hodgkin type is the most common subtype. </p><h4>Radiographic features</h4><h5>Plain film</h5><p>PBL has non-specific features and may be normal, lytic, sclerotic or mixed. The common pattern is a lytic pattern with permeative bone destruction with a wide zone of transition <sup>1</sup>. </p><h5>MRI</h5><p>Associated soft tissue masses are common. Bone marrow changes include <sup>2</sup>:</p><ul>
+<p><strong>Primary bone (skeletal/osseous) lymphoma (PBL)</strong> is a less common manifestation of lymphoma than <a href="/articles/secondary-involvement-of-the-bone-with-lymphoma">secondary involvement</a> from disseminated <a href="/articles/lymphoma">lymphoma</a>. It is rare, accounting for <5% of <a href="/articles/bone-tumours">bone tumours</a> and <1% of <a href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphoma</a>. </p><h4>Terminology</h4><p>PBL is defined as the presence of lymphoma isolated to one bone without distant spread for six months after diagnosis. Multifocal PBL is less common and occurs with lymphoma is confined to two or more bones, again without distant spread for six months <sup>1</sup>. </p><h4>Epidemiology</h4><p>PBL can affect any age group, with peak incidence in 50-to-60 year olds. It is rare in children <10 years old. There is a slight male predominance (M:F = 1.5:1) <sup>2</sup>. </p><h4>Clinical presentation</h4><p>Symptoms include localised pain and swelling, <a title="B-type symptoms" href="/articles/b-type-symptoms">B-type symptoms</a>, <a href="/articles/pathological-fracture">pathological fractures</a>, and <a href="/articles/spinal-cord-compression">cord compression</a>. </p><h4>Pathology</h4><p>Diffuse large B cell lymphoma (DLBCL) is the most common subtype. The <a href="/articles/bony-pelvis">bony pelvis</a> and <a href="/articles/femur">femur</a> are the most common locations <sup>3</sup>.</p><h4>Radiographic features</h4><h5>Plain radiograph</h5><p>PBL has non-specific features and the affected bone may be normal or affected by lytic, sclerotic or mixed pattern. The most common is a lytic pattern with <a title="Permeative process in bone" href="/articles/permeative-process-in-bone">permeative bone destruction</a> and a wide zone of transition <sup>1</sup>. </p><h5>MRI</h5><p>Associated soft tissue masses are common. <a title="Bone marrow" href="/articles/bone-marrow">Bone marrow</a> changes include <sup>2</sup>:</p><ul>
~~-</ul><h4>Differential diagnosis</h4><p>For plain film presentation of permeative bone destruction consider 1:</p><ul>~~
~~-<li><a title="Osteomyelitis" href="/articles/osteomyelitis">infection</a></li>~~
~~-<li><a title="Eosinophilic granuloma" href="/articles/skeletal-manifestations-of-langerhans-cell-histiocytosis">eosinophilic granuloma</a></li>~~
~~-<li><a title="Ewing sarcoma" href="/articles/ewing-sarcoma">Ewing sarcoma</a></li>~~
~~-<li><a title="Skeletal metastases" href="/articles/skeletal-metastases">skeletal metastases</a></li>~~
+</ul><h4>Treatment and prognosis</h4><p>Five-year survival rate has been reported at ~80%, much better than other <a title="Bone tumours" href="/articles/bone-tumours">bone tumours</a> <sup>2</sup>.</p><h4>Differential diagnosis</h4><p>For plain film presentation of permeative bone destruction consider <sup>1</sup>:</p><ul>
+<li><a href="/articles/osteomyelitis">infection</a></li>
+<li><a href="/articles/langerhans-cell-histiocytosis-skeletal-manifestations">eosinophilic granuloma</a></li>
+<li><a href="/articles/ewing-sarcoma">Ewing sarcoma</a></li>
+<li><a href="/articles/skeletal-metastases">skeletal metastases</a></li>

References changed:

1. Lim CY, Ong KO. Imaging of musculoskeletal lymphoma. Cancer Imaging. 2013;13 (4): 448-57. <a href="http://dx.doi.org/10.1102/1470-7330.2013.0036">doi:10.1102/1470-7330.2013.0036</a> - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3864222">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/24334414">Pubmed citation</a><span class="auto"></span>
2. Krishnan A, Shirkhoda A, Tehranzadeh J et-al. Primary bone lymphoma: radiographic-MR imaging correlation. Radiographics. 2003;23 (6): 1371-83. <a href="http://dx.doi.org/10.1148/rg.236025056">doi:10.1148/rg.236025056</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/14615550">Pubmed citation</a><span class="auto"></span>
3. Maruyama D, Watanabe T, Beppu Y et-al. Primary bone lymphoma: a new and detailed characterization of 28 patients in a single-institution study. Jpn. J. Clin. Oncol. 2007;37 (3): 216-23. <a href="http://dx.doi.org/10.1093/jjco/hym007">doi:10.1093/jjco/hym007</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/17472971">Pubmed citation</a><span class="auto"></span>

Tags changed:

oncology
bone tumour

Systems changed:

Musculoskeletal
Haematology

Images Changes:

Image 1 X-ray (Frontal) ( create )

Image 2 X-ray (AP pelvis) ( create )

Caption was changed:

Case 42

Position was set to .

Image 3 MRI (T1) ( create )

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