Lymphangioleiomyomatosis

Case contributed by RMH Report Writing

Presentation

Acute shortness of breath in a previously well young lady.

Patient Data

Age: 32-year-old
Gender: Female
X-ray

Chest radiograph

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

CT

CT Chest

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 poster lateral aspect of the trachea at the thoracic inlet.

The 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.

No suspicious osseous lesion.

Conclusion:  Bilateral pigtail catheters with small residual pneumothoraces.

Cystic lung disease with no pulmonary nodules is consistent with lymphangioleiomyomatosis. No renal lesion appreciated although the kidneys have been incompletely imaged.

Small bilateral pleural effusions may relate to the pneumothoraces.

A small volume of free intraperitoneal fluid is of uncertain significance.

Fluid density around the origin of the SMA is consistent with nodal involvement by lymphangioleiomyomatosis.

The patient had right upper lobe wedge resection.

PATHOLOGY

MACROSCOPIC DESCRIPTION: "Right upper lobe wedge": A lung wedge resection 95x40x35mm. Sections show multiloculated subpleural blebs up to 25mm in maximal dimension. 

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

Relevant aspects of lymphangioleiomyomatosis:

  • almost exclusively affects women of child-bearing age
  • it is a multi-system disorder and can affect many organs
  • recurrent pneumothorax can occur in up to 80% of cases
  • in the lungs it manifests as multiple thin-walled cysts throughout the lungs (usually with a uniform distribution)
  • for pulmonary manifestations, the primary differential to be considered is Langerhans cell histiocytosis (LCH) which tends to happen in children and young adults with a history of heavy cigarette smoking
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Case information

rID: 38492
Case created: 21st Jul 2015
Last edited: 27th Mar 2017
System: Chest
Tag: rmh
Inclusion in quiz mode: Included

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