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High-resolution CT imaging of the chest shows multiple bizarrely-shaped cysts within the lung parenchyma. They are randomly distributed, and have no prediliction for the upper or lower lung zones. This is the typical appearance of lymphangioleiomyomatosis (LAM). This patient, however, had tuberous sclerosis and a renal angioleiomyolipoma; therefore this represents tuberous sclerosis-related lymphangioleiomyomatosis (TS-LAM). LAM only occurs in females, TS-LAM can infrequently occur in males (with tuberous sclerosis). The differential diagnosis includes histiocytosis X.
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