In the current study, all the 10 patients with clinical diagnosis of TSC-LAM were found to carry TSC1/2 mutations and 8 of them carried biallelic inactivation of TSC1/2, suggesting that it is less like that a non-TSC1/2 tumor suppressor gene might have participated in the initiation of TSC or TSC-LAM, consistent with the opinion from Lam HC et al. that “the possibility of a third germline TSC-causing gene is unlikely” in patients with TSC [26,27,29]. This evidence concerns the gene TSC1 and tuberous sclerosis.