Lymphangioleiomyomatosis
Updates to Article Attributes
LymphangiomyomatosisLymphangioleiomyomatosis (LAM) is a rare multi-system disorder which can occur either sporadically or in association with the tuberous sclerosis (TScomplex (TSC) and is often considered a forme frustecomplex-approximately 1% of those with TSTSC.
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 persistence 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 lymphangiomyomatosislymphangioleiomyomatosis. 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
LymphangiomyomatosisLymphangioleiomyomatosis 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 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.
Additional features
-
general/plain film:
- chylothorax: chylous pleural effusion
- evidence of hyperinflation
- diffuse bilateral reticulonodular densities
- recurrent pneumothoraces in complicated cases
-
HRCT:
- thin walled cysts of variable sizes surrounded by normal lung parenchyma-can be seen throughout the lung
- interlobular septal thickening
- may show a dilated thoracic duct
- haemorrhages may be seen as areas of increased attenuation
Abdomen and pelvis:
- renal angiomyolipomas 2-3 -commonest abdominal finding 3
- splenic cysts
- chylous ascites
lymphangioleiyomyomaslymphangioleiomyomas- uterine fibroids 2
- abdominal lymphadenopathy 3
Neck:
Complications
Thoracic:
- recurrent pneumothorax
: cancan 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 Langerhans cell histiocytosis (LCH) 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><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>- +<p><strong>Lymphangioleiomyomatosis (LAM)</strong> is a rare multi-system disorder which can occur either sporadically or in association with the <a title="Tuberous sclerosis complex" href="/articles/tuberous-sclerosis">tuberous sclerosis complex</a> (TSC) and is often considered a <a title="Forme fruste" href="/articles/forme-fruste">forme fruste</a> of TSC.</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 lymphangioleiomyomatosis. 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>Lymphangioleiomyomatosis 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>
-<li><a href="/articles/lymphangioleiyomyomas">lymphangioleiyomyomas</a></li>- +<li><a href="/articles/lymphangioleiyomyomas">lymphangioleiomyomas</a></li>
-<li>recurrent pneumothorax: can occur in up to 80% of cases</li>- +<li>recurrent pneumothorax can occur in up to 80% of cases</li>
-<a 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">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>- +</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>
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