The differentiation abnormalities are a hallmark of the central nervous system and peripheral lesions of the tuberous sclerosis complex (TSC), which is a genetic disorder resulting from the loss of TSC1/2 function, manifesting in the form of brain tumors with aberrant glioneuronal differentiation, pulmonary lymphangioleiomyomatosis (LAM), and renal angiomyolipomas4. Here, TSC1 is linked to lymphangioleiomyomatosis.