These features include: 1) TSC1/TSC2 gene mutations have been identified in lung and other tissues from sporadic LAM patients [7], [8], [9], [10], [11]; 2) activation of the mTOR (mammalian Target Of Rapamycin) pathway occurs in abnormal LAM and TSC tissues [12], [13]; 3) LAM is a major feature of TSC [14] and cystic lung disease consistent with early LAM is present in 30–40% of women with TSC [15], [16]; 4) kidney angiomyolipomas are a major feature of TSC and occur in 40–50% of individuals with sporadic LAM [5], [17]. Here, TSC2 is linked to lymphangioleiomyomatosis.