Impact of Age, Menopause, and Sirolimus on Spontaneous Pneumothoraces in Lymphangioleiomyomatosis

Abstract

Lymphangioleiomyomatosis (LAM) is a rare neoplasm, predominantly seen among young women, involving proliferation and metastatic infiltration of the lungs by atypical smooth muscle-like cells. LAM is caused by mutations in the tuberous sclerosis complex (TSC) genes that lead to constitutive activation of the mechanistic target of rapamycin (mTOR) pathway.1 Sirolimus, an oral mTOR inhibitor, has been shown to stabilize lung function decline in patients with LAM2 and has become the first-line treatment option for qualifying patients.