Primary pulmonary lymphoma
Updates to Article Attributes
Primary pulmonary lymphomas refer to clonal lymphoid proliferation affecting the lungs without any detectable extrapulmonary involvement. It is a much rarer type ofthan secondary pulmonary lymphoma and is most frequently represented by lymphoma of B-cell lineage - often marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type.
Epidemiology
Primary pulmonary lymphoma is a very rare entity and is thought to only represent only 3-4% of extranodal non-Hodgkin lymphoma, <1% of all non-Hodgkin lymphoma, and 0.5-1% of all primary pulmonary malignancies 1-6.
Radiographic features
The diagnosis of primary pulmonary lymphoma requires the following criteria at the time(as of initial writing (DecemberDecember 2012) 6:
- involvement of lung, bronchus, or both, without evidence of mediastinal lymphadenopathy or mass on a chest radiograph
- no previous extrathoracic lymphoma
- no evidence of extrathoracic lymphoma or lymphatic leukaemia at the time of diagnosis
- no extrathoracic disease outside the thorax for least three months following the initial diagnosis within the lung
When the lung is the principal site of lymphoma, the definition also includes:
- multifocal mucosa-associated lymphoid tissue (MALT) non-Hodgkin lymphoma
- pulmonary involvement with satellite nodes (hilar or mediastinal)
- multiorgan involvement by lymphomatoid granulomatosis, the clonal nature of which is controversial
Treatment and prognosis
This entity of lymphoma is thought to carry a comparatively good prognosis 4,5.
-<p><strong>Primary pulmonary lymphomas</strong> refer to clonal lymphoid proliferation affecting the lungs without any detectable extrapulmonary involvement. It is a much rarer type of <a href="/articles/pulmonary-lymphoma">pulmonary lymphoma</a> and is most frequently represented by lymphoma of B-cell lineage - often marginal zone B-cell lymphoma of <a href="/articles/inguinal-canal-walls-mnemonic">mucosa-associated lymphoid tissue (MALT)</a> type.</p><h4>Epidemiology</h4><p>Primary pulmonary lymphoma is a very rare entity and is thought to only represent only 3-4% of extranodal non-Hodgkin lymphoma, <1% of all <a href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphoma</a>, and 0.5-1% of all primary pulmonary malignancies <sup>1-6</sup>.</p><h4>Radiographic features</h4><p>The diagnosis of primary pulmonary lymphoma requires the following criteria at the time of initial writing (December 2012) <sup>6</sup>:</p><ul>-<li>involvement of lung, bronchus or both, without evidence of <a href="/articles/mediastinal-lymph-node-enlargement">mediastinal lymphadenopathy</a> or <a href="/articles/mediastinal-mass">mass</a> on a chest radiograph</li>- +<p><strong>Primary pulmonary lymphomas</strong> refer to clonal lymphoid proliferation affecting the lungs without any detectable extrapulmonary involvement. It is a much rarer than secondary <a href="/articles/pulmonary-lymphoma">pulmonary lymphoma</a> and is most frequently represented by lymphoma of B-cell lineage - often marginal zone B-cell lymphoma of <a href="/articles/inguinal-canal-walls-mnemonic">mucosa-associated lymphoid tissue (MALT)</a> type.</p><h4>Epidemiology</h4><p>Primary pulmonary lymphoma is a very rare entity and is thought to represent only 3-4% of extranodal non-Hodgkin lymphoma, <1% of all <a href="/articles/non-hodgkin-lymphoma">non-Hodgkin lymphoma</a>, and 0.5-1% of all primary pulmonary malignancies <sup>1-6</sup>.</p><h4>Radiographic features</h4><p>The diagnosis of primary pulmonary lymphoma requires the following criteria (as of December 2012) <sup>6</sup>:</p><ul>
- +<li>involvement of lung, bronchus, or both, without evidence of <a href="/articles/mediastinal-lymph-node-enlargement">mediastinal lymphadenopathy</a> or <a href="/articles/mediastinal-mass">mass</a> on a chest radiograph</li>
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