We reviewed 17 cases of pulmonary lymphangioleiomyomatosis diagnosed by open lung biopsy. All patients were women with a mean age of 39.6 years. Tuberous sclerosis was diagnosed in two cases. The patients were submitted to high-resolution computed tomography that showed diffuse thin walled cystic lesions of less than 60 mm in diameter. Associated pneumothorax was seen in two cases. The main high-resolution computed tomography findings were diffuse, rounded, cystic lesions, generally of less than 20 mm in diameter.
Keywords: Lymphangioleiomyomatosis, Cystic lung diseases, High-resolution computed tomography
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