Pulmonary involvement in tuberous sclerosis complex (TSC) resembles lymphangioleiomyomatosis (LAM) in its clinical, radiologic, and histopathological features. Typical high-resolution computed tomography (HRCT) findings are of bilateral thin-walled cysts symmetrically throughout both lungs, with associated increase in lung volumes. Atypical cases have been described; and we report a case of biopsy proven, focal unilateral disease.
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