Meningiomas in Lymphangioleiomyomatosis

Abstract

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.

Abrupt Regression of a Meningioma after Discontinuation of Cyproterone Treatment: Fig 1.

“…6,7 The risk of meningioma is increased among postmenopausal women who have used hormonal treatment replacement therapy and among women who have used longacting contraceptives. 7 The suspicion that high daily doses of cyproterone acetate for long periods could be responsible for the development of meningiomas, and especially multiple meningiomas, was reported in 2008.…”

European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis

“…Screening for meningioma in patients with sporadic LAM Patients with LAM have an increased risk of meningioma [9], the growth of which may be promoted by progesterone. These lesions should be identified especially in patients receiving progesterone.…”

“…Extra pulmonary lymphadenopathy and cystic masses of the axial lymphatics termed lymphangioleiomyomas can result in abdominal and pelvic lymphatic obstruction [7]. LAM is often associated with angiomyolipoma in the kidneys [8], and an increased frequency of meningioma [9]. LAM varies in clinical features and rate of progression: this together with an absence of clear prognostic factors results in patients being given conflicting information about prognosis.…”

Meningioma in untreated congenital adrenal hyperplasia: a relationship?

“…For instance, during the luteal phase of the menstrual cycle, a period of relative progesterone excess, increased growth of meningioma can be observed (cyclic vision problems) [9]. Another argument for the involvement of progestins in the development or growth of meningiomas is the higher incidence of meningiomas in women with lymphangioleiomyomatosis (LAM), a lung disease that is often treated with progesterone [10]. Direct evidence for such a role of progesterone in the pathogenesis of meningioma is still lacking.…”