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A 48-year-old woman presented chronic headache and serous otitis with feeling of fullness of the right ear. She had no medical or surgery history. ENT and clinical neurological examination revealed no other anomaly. She didn't take any treatment except Nomegestrol Acetate (NA) contraception for 15 years. A Cerebral Magnetic Resonance Imaging (MRI) was finally performed.
What is the diagnosis?
A large meningioma of the skull base invading petrous bone and infratemporal fossa (Fig. 1 A and B) .
Fig. 1A and B. MRI showed a bulky right petroclival meningioma invading the two cavernous sinuses and the right infratemporal fossa. The lesion extends to the sellar area and to the ethmoid and sphenoid sinuses.
This patient didn't have any risk factor of meningioma except the use of NA. This drug is a synthetic progestin, a derivate of 19-nor-progesterone, as Cyproterone Acetate. It is now well established that the long-term use of high doses of Cyproterone Acetate is prone to promote meningiomas development. In 2019, the first cases of meningiomas responsiveness to NA cessation were reported [
]. Derivatives of 19-nor-progesterone are characterised by high selectivity of action on progesterone receptor. From a pathophysiological point of view, the association between these drugs and meningioma occurrence can be explained by the strong expression of progesterone receptors in meningiomas as reflected by the association between meningioma and breast cancer. As NA has an antigonadotropic activity, another mechanism could be the enhanced expression of the gonadotropin-releasing-hormone (GnRH), given the loss of negative feedback of testosterone and estrogen on GnRH secretion at the level of the hypothalamus. Recently, mutations of PIK3CA and TRAF7 were shown to be associated with progestin-associated meningiomas [
]. In our case, we decided to stop immediately and definitively NA. Despite the voluminous character of the tumour, we applied a “discontinuation and scan strategy” without surgical intervention. This conservative approach is driven by similar observation in literature; Bernat et al. recently reported prompt regression of a large and symptomatic giant olfactory groove meningioma after discontinuation of Cyproterone Acetate [
All procedures performed in studies involving human participants were in accordance with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.
Informed consent
Written informed consent was obtained from the patient for being included in the study.
Declaration of Competing Interest
The authors declare that they have no conflict of interest.
Acknowledgements
None.
References
Passeri T
Champagne P-O
Bernat A-L
et al.
Spontaneous regression of meningiomas after interruption of nomegestrol acetate: a series of three patients.
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