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Lymphangioleiomyomatosis (LAM) is an uncommon cystic lung disease caused by infiltration of the lung with smooth muscle cells that arise from an unknown source, spread via blood and lymphatics, and contain growth-activating mutations in tuberous sclerosis genes [1,2]. LAM occurs in patients with tuberous sclerosis complex (TSCLAM) and in a “sporadic” form in patients who do not have tuberous sclerosis (S-LAM) [3]. It is a slowly progressive lung disease that leads to respiratory failure over one to two decades. It is exclusively seen in women and is characterized by progressive proliferation of smooth muscle-like cells (LAM cells) in lung parenchyma and causes cystic destruction, chylous pleural effusions and recurrent pnuemothoraxes. LAM has historically been considered an estrogen dependent tumor and was treated with hormonal manipulation strategies such as antiestrogens, antiprogesterones and oophorectomy. Current insights into the pathophysiology of LAM have provided new treatment drugs such as sirolimus, a mTOR inhibitor, that is effective in stabilizing lung function. Since the advent of novel therapies, hormonal manipulation strategies have become less popular. We describe a case of LAM in a young woman who underwent oophorectomy and was followed closely for 16 years without worsening of her lung function.

Role of Hormonal Manipulations in Delaying the Progression of Lymphangioleiomyomatosis (LAM): A 16-Years Follow-Up Report after Oophorectomy