Background: Meningiomas are the most frequent primary brain tumor in adults. Evidence suggests that female sex hormones play a role in the meningioma tumorigenesis. In particular, progesterone, has a receptor (PR) that is highly expressed in the majority of grade I meningiomas. Multiple meningiomas (diffuse meningiomatosis) are less frequent, but have a higher female predominance and a higher PR expression. They are, therefore, attractive candidates for anti-PR therapy.
Methods: We treated three consecutive women with multiple meningiomas with mifepristone (RU 486). It is a synthetic steroid with high affinity for both progesterone and glucocorticoid receptors.
Results: The treatment was well tolerated, and we observed an important and long-lasting clinical (3/3) and radiological response (2/3) or stabilisation. All the three patients are now stable after five to nine years of treatment.
Conclusions: These encouraging results strongly support a prospective clinical trial in this preselected population.