Lymphangioleiomyomatosis
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Lymphangioleiomyomatosis (LAM) is a rare multi-system disorder that can occur either sporadically or in association with the tuberous sclerosis complex (TSC) and is often considered a forme fruste of TSC.
Epidemiology
It almost exclusively affects women of child-bearing age 7. The estimated incidence is 1:400,000 4.
Clinical presentation
Patients usually present with exertional dyspnoea and recurrent episode pneumothorax are common 8.
Pathology
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.
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.
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-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.
-
general/radiograph
- 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: most common abdominal finding 2,3
- splenic cysts
- chylous ascites
- lymphangioleiomyomas
- uterine fibroids 2
- abdominal lymphadenopathy 3
Neck
Treatment and prognosis
Despite treatment with agents such as medroxyprogesterone and tamoxifen 6, LAM tends to be progressive with most of the disease severity due to pulmonary disease.
Complications
Thoracic
- recurrent pneumothorax can occur in up to 80% of cases
- thoracic chylous collections
- haemoptysis (occasional) 4
Abdominal
- pelvic lymphatic obstruction
Differential diagnosis
For pulmonary manifestations, the primary differential to be considered is:
-
Langerhans cell histiocytosis (LCH)
- tends to happen in children and young adults with a history of heavy cigarette smoking
- mid to upper lobe distribution with preservation of the costophrenic angles
- cysts in LCH tend to be more irregular in contour
- has much more favourable prognosis compared with LAM
- lymphocytic interstitial pneumonitis (LIP): smooth cysts with ground glass attenuation and nodules, often associated with autoimmune disease
-<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>Clinical presentation</h4><p>Patients usually present with exertional dyspnoea and recurrent episode pneumothorax are common <sup>8</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><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>Clinical presentation</h4><p>Patients usually present with exertional dyspnoea and recurrent episode pneumothorax are common <sup>8</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><ul>
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