Lymphangioleiomyomatosis

Case contributed by Dr Kenny Sim

Presentation

Acute dyspneoa. No history of trauma.

Patient Data

Age: 30-35
Gender: Female
Modality: X-ray

Bilateral large pneumothoraces greater on the left than the right. Mediastinal structures appear midline. No displaced rib fractures identified.

Modality: CT

Bilateral apically directed pigtail intercostal catheters in situ with only small pneumothoraces bilaterally. Small volume subcutaneous emphysema is related to the intercostal catheters.

There are multiple thin walled cysts scattered throughout both lungs involving all lung zones, measuring from a few millimetres to 14mm. No pulmonary nodules identified. Minor dependent changes present, greater on the left. There are also tiny bilateral pleural effusions, slightly greater on the left. The thoracic duct is not overtly dilated.

There is a small, well-defined focal collection of air measuring 13mm contiguous with the right posterolateral aspect of the trachea at the thoracic inlet.

There are no enlarged hilar, mediastinal or axillary lymph nodes. Heart size is normal and there is no pericardial effusion.

On the most inferior image through the upper abdomen, well-defined fluid density is seen around the origin of the SMA consistent with nodal involvement by lymphangioleiomyomatosis.

Within segment 8 of the liver, there is a 6 mm hypodensity. No renal lesion identified within the imaged portions of the kidneys. The adrenal and imaged pancreas are also unremarkable on arterial phase imaging. A thin rim of ascites surrounds the liver.

Conclusion:

  1. Bilateral pigtail catheters with small residual pneumothoraces.
  2. Cystic lung disease with no pulmonary nodules is consistent with lymphangioleiomyomatosis. No renal lesion appreciated although the kidneys have been incompletely imaged.
  3. Small bilateral pleural effusions may relate to the pneumothoraces.
  4. A small volume of free intraperitoneal fluid is of uncertain significance.
  5. Fluid density around the origin of the SMA is consistent with nodal involvement by lymphangioleiomyomatosis.

The patient underwent a right VATS pleurodesis and right upper lobe wedge resection.

Pathology demonstrated:

MICROSCOPIC DESCRIPTION:

Sections of lung show randomly dispersed areas of interstitial smooth muscle proliferation composed of bland spindle cells. Several cystically dilated air-spaces contain a similar smooth muscle proliferation within the cyst wall. A subpleural bulla is also present. There is no evidence of atypia or malignancy. Immunohistochemical results show spindle cells stain: SMA+, HMB45-, MelanA- and Synaptophysin-. The histological features are those of lymphangioleiomyomatosis. Clinical and radiological correlation is recommended.

DIAGNOSIS:

Right lung, upper lobe, wedge resection: Lymphangioleiomyomatosis.

Case Discussion

Lymphangioleiomyomatosis is a rare multi-system disease that can occur sporadically or be associated with tuberous sclerosis. There is abnormal smooth muscle proliferation with resulting obstruction of lymphatics, venules and small airways. In the lungs, there is development of thin walled cysts, with surrounding normal lung parenchyma. It can be associated with recurrent pneumothoraces as well as chylous pleural effusions.

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Case Information

rID: 38434
Case created: 19th Jul 2015
Last edited: 15th Feb 2017
System: Chest
Inclusion in quiz mode: Included

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