Lymphangioleiomyomatosis
Updates to Article Attributes
Lymphangiomyomatosis (LAM) is a rare multi-system disorder which can occur either sporadically or in association with the tuberous sclerosis (TS) complex - approximately-approximately 1% of those with TS.
Epidemiology
It almost exclusively affects women of child bearing age 7. The estimated incidence is 1:400,000 4.
Pathology
The disease is characterised by persistancepersistence of dilated lymphatics and interstitial proliferation of abnormal smooth muscle which in turn can obstruct venules and lymphatics.
In the chest, there are two phases of proliferation in lymphangiomyomatosis. The early phase is characterised by proliferation of immature muscle cells which cover alveolar walls, bronchioles, pleura and vessels, including lymphatic routes. In the late phase there is development of cystic spaces and wider proliferation of muscle cells throughout the lung.
Radiographic features
Lymphangiomyomatosis is a multi-system disorder and can affect many organs.
Bone
May show massive osteolysis with little or no periosteal reaction. Often multifocal disease
Chest
Multiple thin walled cysts throughout the lungs (usually with aan uniform distribution). These are present in nearly all cases. If the cysts are small they may be seen as diffuse coarse interstitial markings on plain film.
Additional features
-
general
/ plain/plain film:-
chylothorax
-: chylous pleural effusion - evidence of hyperinflation
- diffuse bilateral reticulonodular densities
- recurrent pneumothoraces in complicated cases
-
chylothorax
-
HRCT:
- thin walled cysts of variable sizes surrounded by normal lung parenchyma
- can-can be seen throughout the lung - interlobular septal thickening
- may show a dilated thoracic duct
- haemorrhages may be seen as areas of increased attenuation
- thin walled cysts of variable sizes surrounded by normal lung parenchyma
Abdomen and pelvis:
-
renal angiomyolipomas 2-3
- commonest-commonest abdominal finding 3 - splenic cysts
- chylous ascites
- lymphangioleiyomyomas
- uterine fibroids 2
- abdominal lymphadenopathy 3
Neck:
Complications
Thoracic:
- recurrent pneumothorax
-: can occur in up to 80% of cases - thoracic chylous collections
-
haemoptysis
- occasional-occasional 4
Abdominal:
- pelvic lymphatic obstruction
Treatment and prognosis
Despite treatment with agents such as medroxyprogesterone and tamoxifen 6, LAM tends to be progressive with most of disease severity due to pulmonary disease.
Differential diagnosis
For pulmonary manifestations the main differential to be considered is LangehansLangerhans cell histiocytosis (LCH) which tends to have a mid to upper lobe distribution with presevation preservation of costophenic costophrenic angles. In addition, the cysts in LCH tend to be more irregular in contour.
-<p><strong>Lymphangiomyomatosis (LAM)</strong> is a rare multi-system disorder which can occur either sporadically or in association with the <a href="/articles/tuberous-sclerosis">tuberous sclerosis (TS) </a>complex - approximately 1% of those with TS. </p><h4>Epidemiology</h4><p>It almost exclusively affects women of child bearing age <sup>7</sup>. The estimated incidence is 1:400,000 <sup>4</sup>.</p><h4><strong>Pathology</strong></h4><p>The disease is characterised by persistance of dilated lymphatics and interstitial proliferation of abnormal smooth muscle which in turn can obstruct venules and lymphatics.</p><p>In the chest, there are two phases of proliferation in lymphangiomyomatosis. The early phase is characterised by proliferation of immature muscle cells which cover alveolar walls, bronchioles, pleura and vessels, including lymphatic routes. In the late phase there is development of cystic spaces and wider proliferation of muscle cells throughout the lung.</p><h4><strong>Radiographic features</strong></h4><p>Lymphangiomyomatosis is a multi-system disorder and can affect many organs. </p><h5>Bone </h5><p>May show massive osteolysis with little or no periosteal reaction. Often multifocal disease</p><h5>Chest </h5><p>Multiple thin walled cysts throughout the lungs (usually with a uniform distribution). These are present in nearly all cases. If the cysts are small they may be seen as diffuse coarse interstitial markings on plain film.</p><h6>Additional features </h6><ul>- +<p><strong>Lymphangiomyomatosis (LAM)</strong> is a rare multi-system disorder which can occur either sporadically or in association with the <a href="/articles/tuberous-sclerosis">tuberous sclerosis (TS) </a>complex-approximately 1% of those with TS. </p><h4>Epidemiology</h4><p>It almost exclusively affects women of child bearing age <sup>7</sup>. The estimated incidence is 1:400,000 <sup>4</sup>.</p><h4><strong>Pathology</strong></h4><p>The disease is characterised by persistence of dilated lymphatics and interstitial proliferation of abnormal smooth muscle which in turn can obstruct venules and lymphatics.</p><p>In the chest, there are two phases of proliferation in lymphangiomyomatosis. The early phase is characterised by proliferation of immature muscle cells which cover alveolar walls, bronchioles, pleura and vessels, including lymphatic routes. In the late phase there is development of cystic spaces and wider proliferation of muscle cells throughout the lung.</p><h4><strong>Radiographic features</strong></h4><p>Lymphangiomyomatosis is a multi-system disorder and can affect many organs. </p><h5>Bone </h5><p>May show massive osteolysis with little or no periosteal reaction. Often multifocal disease</p><h5>Chest </h5><p>Multiple thin walled cysts throughout the lungs (usually with an uniform distribution). These are present in nearly all cases. If the cysts are small they may be seen as diffuse coarse interstitial markings on plain film.</p><h6>Additional features </h6><ul>
-<strong>general / plain film</strong><ul>- +<strong>general/plain film:</strong><ul>
-<a href="/articles/chylothorax">chylothorax </a>- chylous pleural effusion</li>- +<a href="/articles/chylothorax">chylothorax</a>: chylous pleural effusion</li>
-<strong>HRCT</strong><ul>-<li>thin walled cysts of variable sizes surrounded by normal lung parenchyma - can be seen throughout the lung</li>- +<strong>HRCT:</strong><ul>
- +<li>thin walled cysts of variable sizes surrounded by normal lung parenchyma-can be seen throughout the lung</li>
-</ul><h5>Abdomen and pelvis</h5><ul>- +</ul><h5>Abdomen and pelvis:</h5><ul>
-<a href="/articles/renal-angiomyolipoma">renal angiomyolipomas</a> <sup>2-3 </sup>- commonest abdominal finding <sup>3</sup>- +<a href="/articles/renal-angiomyolipoma">renal angiomyolipomas</a> <sup>2-3 </sup>-commonest abdominal finding <sup>3</sup>
-</ul><h5>Neck </h5><ul><li><a href="/articles/cystic_hygroma">cystic hygroma</a></li></ul><h4>Complications</h4><h5>Thoracic</h5><ul>-<li>recurrent pneumothorax - can occur in up to 80 % of cases</li>- +</ul><h5>Neck:</h5><ul><li><a href="/articles/cystic-hygroma-1">cystic hygroma</a></li></ul><h4>Complications</h4><h5>Thoracic:</h5><ul>
- +<li>recurrent pneumothorax: can occur in up to 80% of cases</li>
-<a title="Haemoptysis" href="/articles/haemoptysis-1">haemoptysis</a> - occasional <sup>4</sup>- +<a href="/articles/haemoptysis-1">haemoptysis</a>-occasional <sup>4</sup>
-</ul><h5>Abdominal</h5><ul><li>pelvic lymphatic obstruction</li></ul><h4>Treatment and prognosis</h4><p>Despite treatment with agents such as medroxyprogesterone and tamoxifen <sup>6</sup>, LAM tends to be progressive with most of disease severity due to pulmonary disease.</p><h4>Differential diagnosis</h4><p>For pulmonary manifestations the main differential to be considered is <a href="/articles/pulmonary-langerhans-cell-histiocytosis">Langehans cell histiocytosis (LCH)</a> which tends to have a mid to upper lobe distribution with presevation of costophenic angles. In addition the cysts in LCH tend to be more irregular in contour. </p>- +</ul><h5>Abdominal:</h5><ul><li>pelvic lymphatic obstruction</li></ul><h4>Treatment and prognosis</h4><p>Despite treatment with agents such as medroxyprogesterone and tamoxifen <sup>6</sup>, LAM tends to be progressive with most of disease severity due to pulmonary disease.</p><h4>Differential diagnosis</h4><p>For pulmonary manifestations the main differential to be considered is <a href="/articles/pulmonary-langerhans-cell-histiocytosis">Langerhans cell histiocytosis (LCH)</a> which tends to have a mid to upper lobe distribution with preservation of costophrenic angles. In addition, the cysts in LCH tend to be more irregular in contour. </p>
Unable to process the form. Check for errors and try again.