Lymphangioleiomyomatosis
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Lymphangioleiomyomatosis (LAM) is a rare multi-system disorder whichthat can occur either sporadically or in association with the tuberous sclerosis complex (TSC) and is often often considered a forme fruste of TSC.
Epidemiology
It almost exclusively affects women of child bearing-bearing age7. The estimated incidence is 1:400,0004.
Pathology
The disease is characterised by the persistence of dilated lymphatics and interstitial proliferation of abnormal smooth muscle whichthat in turn can obstruct venules, lymphatics, and small airways.
In the chest, there are two phases of proliferation in lymphangioleiomyomatosis. The early phase is characterised by proliferation of immature muscle cells whichthat cover alveolar walls, bronchioles, pleura and vessels, including lymphatic routes. In the late phase there is a development of cystic spaces and wider proliferation of muscle cells throughout the lung.
Radiographic features
Lymphangioleiomyomatosis 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 ana 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/radiograph- chylothorax: chylous pleural effusion
- evidence of hyperinflation
- diffuse bilateral reticulonodular densities
- recurrentpneumothoracesin complicated cases
-
HRCT
- thin walled cysts of variable sizes surrounded by normal lung parenchyma
-cancan 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: most common
abdominalabdominal finding 2,3 - splenic cysts
- chylous ascites
- lymphangioleiomyomas
- uterine fibroids 2
- abdominal lymphadenopathy 3
Neck
Complications
Thoracic
- recurrent pneumothorax
cancan occur in up to 80% of cases - thoracic chylous collections
- haemoptysis (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 mainprimary differential to be considered is:
-
Langerhans cell histiocytosis (LCH)
which- tends to happen in children and
youngyoung adults with a history ofheavyheavy cigarette smoking. It has a mid -
mid to upper lobe distribution with
preservationpreservation ofcostophrenic anglescostophrenic angle.In addition, the - cysts in LCH tend to be more irregular in contour
. LCH has much -
has much more favourable
prognosis comparedprognosis compared with LAM.
- tends to happen in children and
-<p><strong>Lymphangioleiomyomatosis (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 complex</a> (TSC) and is often considered a <a 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>Pathology</h4><p>The disease is characterised by persistence of dilated lymphatics and interstitial proliferation of abnormal smooth muscle which in turn can obstruct venules, lymphatics, and small airways.</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>Radiographic features</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>- +<p><strong>Lymphangioleiomyomatosis (LAM)</strong> is a rare multi-system disorder that can occur either sporadically or in association with the <a href="/articles/tuberous-sclerosis">tuberous sclerosis complex</a> (TSC) and is often considered a <a 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>Pathology</h4><p>The disease is characterised by the persistence of dilated lymphatics and interstitial proliferation of abnormal smooth muscle that in turn can obstruct venules, lymphatics, and small airways.</p><p>In the chest, there are two phases of proliferation in lymphangioleiomyomatosis. The early phase is characterised by proliferation of immature muscle cells that cover alveolar walls, bronchioles, pleura and vessels, including lymphatic routes. In the late phase there is a development of cystic spaces and wider proliferation of muscle cells throughout the lung.</p><h4>Radiographic features</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 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>
-<strong>general/plain film</strong><ul>- +<strong>general/radiograph</strong><ul>
-<li>recurrent <a href="/articles/pneumothorax">pneumothoraces </a>in complicated cases</li>- +<li>recurrent <a href="/articles/pneumothorax">pneumothoraces </a>in complicated cases</li>
-<li>thin walled cysts of variable sizes surrounded by normal lung parenchyma-can be seen throughout the lung</li>- +<li>thin walled cysts of variable sizes surrounded by normal lung parenchyma can be seen throughout the lung</li>
-<a href="/articles/renal-angiomyolipoma">renal angiomyolipomas</a>: most common abdominal finding <sup>2,3</sup>- +<a href="/articles/renal-angiomyolipoma">renal angiomyolipomas</a>: most common abdominal finding <sup>2,3</sup>
-<li>recurrent pneumothorax can occur in up to 80% of cases</li>- +<li>recurrent pneumothorax can occur in up to 80% of cases</li>
-</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 happen in children and young adults with history of heavy cigarette smoking. It has a mid to upper lobe distribution with preservation of costophrenic angles. In addition, the cysts in LCH tend to be more irregular in contour. LCH has much more favourable prognosis compared with LAM.</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 primary differential to be considered is:</p><ul><li>
- +<a href="/articles/pulmonary-langerhans-cell-histiocytosis">Langerhans cell histiocytosis (LCH)</a> <ul>
- +<li>tends to happen in children and young adults with a history of heavy cigarette smoking</li>
- +<li>mid to upper lobe distribution with preservation of costophrenic angle.</li>
- +<li>cysts in LCH tend to be more irregular in contour</li>
- +<li>has much more favourable prognosis compared with LAM</li>
- +</ul>
- +</li></ul>
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