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Moss J, DeCastro R, Patronas NJ, Taveira-DaSilva A. Meningiomas in Lymphangioleiomyomatosis. JAMA. 2001;286(15):1879–1881. doi:https://doi.org/10.1001/jama.286.15.1879
Author Affiliations: Pulmonary-Critical Care Medicine Branch, National Heart, Lung, and Blood Institute (Drs Moss and Taveira-DaSilva and Ms DeCastro), and Department of Diagnostic Radiology, Clinical Center (Dr Patronas), National Institutes of Health, Bethesda, Md.
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
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.
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