Multiple meningiomas are rare benign tumors (1.5% of all meningiomas). They are either sporadic or associated with neurofibromatosis. Their long term morbidity is high due to the frequency of surgical procedures needed to overcome the absence of efficient adjuvant therapy. We present a cohort of patients in whom we strongly suspect cyproterone acetate to be responsible for the development and progression of multiple meningiomas.
Patients and methods: We report 9 female patients (3362 yo, mean: 46 yo) with multiple meningiomas (2 to 11) without any clinical evidence of neurofibromatosis. All patients were treated with cyproterone acetate (50 mg/day) for various indications for a time period ranging from 10 to 20 years.
Results: A rapid onset of clinical symptoms was observed in 6 patients with rapid decreased visual acuity in 5 patients, suggesting rapidly progressive meningiomas. Lesions were preferentially located on the skull base. Cyprotenone acetate was stopped at the time of diagnosis in 2 patients. Six patients were followed radiologically for a period exceeding 5 months (8 to 81) before treatment withdrawal. A significant increase in tumor size and/or the development of new lesions was observed in all cases. In six patients, the follow-up period after treatment withdrawal was more than 5 months (5 to 32 months, mean: 17 months) and no clinical nor radiological progression was observed.
Discussion: We strongly suspect cyproterone acetate as a promoting factor in the development of multiple meningiomas in concert with a particular endocrine status. We could face a particular histopathologic entity considering the preferential skull base location of lesions and the unusual absence of progression after treatment withdrawal.
Conclusion: Although undescribed in the literature, the possibility of a relationship between multiple meningiomas and cyproterone acetate, needs to be further investigated.
03 - 07 May 2008
European Society of Endocrinology