Lymphangioleiomyomatosis

Case contributed by Lukas Valkovic
Diagnosis probable

Presentation

Recurrent pneumothorax.

Patient Data

Age: 40 years
Gender: Female
ct
  • The initial HRCT demonstrates diffuse cystic changes in both lungs and adhesions in apical regions
  • CT 8 years after initial scan demonstrates marked encapsulated pleural effusions (the mediastinal window images do not show the interval progression of cystic diffuse changes).

VATS was performed with repeated pleurodesis.

mri

MRI shows multiple soft retroperitoneal cystic lesions with solid components which insinuate between normal structures with little mass effect.

Case Discussion

The original histology suggested metastatic gynecological origin. After repeated review histology confirmed lymphangioleiomyomatosis.

Lymphangioleiomyomas are a recognized feature of lymphangioleiomyomatosis and are most probably caused by lymphatic obstruction which is more commonly seen in sporadic LAM.

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