Primary pulmonary lymphoma
Disclosures
- updated 27 Nov 2022:
Nothing to disclose
Updates to Article Attributes
Body
was changed:
Primary pulmonary lymphomas refer to clonal lymphoid proliferation affecting the lungs without any detectable extrapulmonary involvement. It is much rarer than 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 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 (as of December 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 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>-<li>no previous extrathoracic lymphoma</li>-<li>no evidence of extrathoracic lymphoma or lymphatic leukaemia at the time of diagnosis</li>-<li>no extrathoracic disease outside the thorax for least three months following the initial diagnosis within the lung</li>-</ul><p>When the lung is the principal site of lymphoma, the definition also includes:</p><ul>-<li>multifocal <a href="/articles/balt-lymphoma-1">mucosa-associated lymphoid tissue (MALT) non-Hodgkin lymphoma</a>-</li>-<li>pulmonary involvement with satellite nodes (hilar or mediastinal)</li>-<li>multiorgan involvement by <a href="/articles/lymphomatoid-granulomatosis">lymphomatoid granulomatosis</a>, the clonal nature of which is controversial</li>- +<p><strong>Primary pulmonary lymphomas</strong> refer to clonal lymphoid proliferation affecting the lungs without any detectable extrapulmonary involvement. It is 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>
- +<li>no previous extrathoracic lymphoma</li>
- +<li>no evidence of extrathoracic lymphoma or lymphatic leukaemia at the time of diagnosis</li>
- +<li>no extrathoracic disease outside the thorax for least three months following the initial diagnosis within the lung</li>
- +</ul><p>When the lung is the principal site of lymphoma, the definition also includes:</p><ul>
- +<li>multifocal <a href="/articles/balt-lymphoma-1">mucosa-associated lymphoid tissue (MALT) non-Hodgkin lymphoma</a>
- +</li>
- +<li>pulmonary involvement with satellite nodes (hilar or mediastinal)</li>
- +<li>multiorgan involvement by <a href="/articles/lymphomatoid-granulomatosis">lymphomatoid granulomatosis</a>, the clonal nature of which is controversial</li>
Images Changes:
Image 1 CT (lung window) ( create )
Unable to process the form. Check for errors and try again.