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Purpose Long-term use of cyproterone acetate (CPA) is associated with an increased risk of developing an intracranial meningioma. Discontinuation of CPA most often induce stabilization or regression of the tumor. The exact mechanism of regression is unknown as well as the reason why some meningiomas are still growing after CPA discontinuation.We are reporting four patients with multiple meningiomas, showing opposite tumor evolutions after stopping the CPA highlighting the underlying histologic and genetic features.MethodsPatients presenting several meningiomaswith opposite evolutions following the discontinuation of CPA were identified. The clinical and radiological data’s were reviewed. A retrospective volumetric analysis of the meningiomas was performed. All the growing meningiomas were operated. Each tumor was characterized histopathologically and by molecular and genetic analyses.ResultsFour female withmultiple meningiomas and opposite tumor volume evolution after CPA discontinuation were identified. The histopathological results found fibroblastic meningiomas for tumorsgrowinglocated in the convexity and a morefibrousmeningioma in the skull-base tumor which decreased. Meningothelial and transitional meningiomaswere found in two skull-base growing meningiomas.The molecular characterization found twoNF2-mutations among the 4 growing meningiomas. In one patient who presented both patterns, the shrinking skull-basetumor harbored a PIK3CA-mutation whereas the growing tumor, a NF2-mutation.ConclusionTo our knowledge, this is the first report of such an atypical tumor evolution of CPA-associated meningiomatosis after CPA discontinuation in the same patient. Underlying biological mechanisms explaining this observationespecially, the close relationship between mutational landscapes and the meningeal embryologyin CPA-related meningiomasrequire further research.

Background: Associations between progestins and meningiomas is now well established. While the link between cyproterone acetate (CA) and meningioma was thoroughly studied, there is far less available data regarding the link between chlormadinone acetate (CHA) or nomegestrol acetate (NA) and risk of intracranial meningiomaMethods: We are presenting a series of 28 patients diagnosed with single or multiple meningiomas while treated with CHA-NA, in which the clinical and radiological course were analyzed after treatment discontinuation.Results: 28 women, with a mean age of 56 years old, were diagnosed with one or multiple meningioma while being treated with either CHA or NA. After stopping treatment, 89.3% showed either tumor shrinkage or tumor stabilization on follow-up MRIs. Multiple meningiomas were more likely observed in patients with long periods of treatment (>10 years, p 0.03) and seem to have a better clinical course (p 0.01). Most of the lesions were located on the skull base (55.4%). Four patients with multiple meningiomas showed discordant tumors evolution, with some tumors growing while others were decreasing. Most of the growing meningiomas were either convexity or midline lesions and more posteriorly located. Conclusion: Our study demonstrated a significant percentage of tumor diminution or stabilization after NA and CHA discontinuation. Therefore, treatment discontinuation with close monitoring should be the first measure taken if urgent surgery is not indicated. However, our results seem to be less encouraging than previously described in patients treated by CA, with more patients showing tumor growth despite treatment discontinuation. Further studies are needed to differentiate the effect of the different progestins treatment on meningiomas.