Meningiomas in lymphangioleiomyomatosis

Context Lymphangioleiomyomatosis (LAM), a cystic lung disease associated with progressive respiratory failure, is found predominantly in women of childbearing age and therefore has been treated with progesterone and other hormonal agents. However, meningiomas have progesterone receptors, and progesterone is believed to be a mitogen for meningioma cells in culture. Since 30% to 40% of patients with tuberous sclerosis complex (TSC) have LAM, we routinely screen patients with LAM for brain lesions found in TSC. Objective To determine the prevalence of meningiomas in women with LAM. Design and Setting Analysis of results from ongoing routine screening protocols initiated in December 1995 at the National Heart, Lung, and Blood Institute. Patients Two hundred fifty women with sporadic LAM who were referred for screening by magnetic resonance imaging (MRI) and/or computed tomography (CT) of the brain. Main Outcome Measures Presence of meningiomas on MRI and/or CT scans. Results Eight women with LAM (3 with and 5 without a diagnosis of TSC) had lesions on MRI scans compatible with meningiomas. Five of the patients had been treated with progesterone. Multiple meningiomas were observed in 2 patients. Conclusions Women with LAM appear to have a high prevalence of meningiomas. We recommend that patients with LAM be screened for meningiomas regardless of diagnosis of TSC. In view of the lack of a documented effect of progesterone on progression of lung disease in LAM and the reported mitogenic response of meningiomas to progesterone, we recommend that the drug not be given to LAM patients with an MRI result consistent with diagnosis of meningioma.