Extending this theory, TSC1- or TSC2-deficient cardiac NC may be the cell of origin for cardiac rhabdomyomas and even pulmonary LAM lesions (due to the involvement in aorticopulmonary septum development), and dysfunctional trunk NC may be responsible for “confetti” skin lesions and hypomelanotic macules. Here, TSC2 is linked to lymphangioleiomyomatosis.