[Current understanding and perspectives of lymphangioleiomyomatosis].

Abstract excerpt

Lymphangioleiomyomatosis (LAM) is a slowly progressive neoplastic disease characterized with proliferation of abnormal smooth muscle-like cells (LAM cells) in the lungs and along axial lymphatics. Proliferation of LAM cells are considered to be driven by dysregulated mTORC1 signaling, that is caused by mutations in either the TSC1 or TSC2 gene in LAM cells. The MILES trial has successfully demonstrated that...

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