Presentation
Recurrent pneumothorax.
Patient Data
Age: 40 years
Gender: Female
From the case:
Lymphangioleiomyomatosis
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- 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.
From the case:
Lymphangioleiomyomatosis
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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.