EU2019 Society for Endocrinology: Endocrine Update 2019 Poster Presentations (73 abstracts)
Case history: A 58 year old woman presented to her GP when her family members noted prominence of her left eye. She had a background history of polycystic ovary syndrome (PCOS). Her main symptom of PCOS was hirsutism. She had been treated initially with the combined oral contraceptive pill Dianette (ethinyloestradiol/cyproterone) but had also been treated intermittently with varying doses of 25100 mg cyproterone acetate (CPA) 10 days/cycle. Post menopause she took 25mg daily CPA for 4 years. In total she took CPA for 34 years.
Investigations: She underwent MRI brain which showed a left sphenoid wing meningioma with a soft tissue component in the middle cranial fossa, a separate focus of meningioma in the right olfactory groove and proptosis.
Results and treatment: Patient underwent craniotomy, resection and orbital reconstruction with calvarial bone grafting. Histology was consistent with a WHO Grade 1 (benign) meningioma with extensive bony infiltration. The tumour showed extensive strong nuclear positivity for progesterone receptor (>90% nuclei) but oestrogen receptor. Follow up MRI showed complete macroscopic soft tissue clearance of the meningioma. The management plan is to monitor for recurrence for 10 years.
Conclusions and points for discussion: Meningiomas are among the most common tumours of the central nervous system. 70% express progesterone receptors but less than 30% express oestrogen receptors. Meningioma is a rare side effect of progestogen therapy. The relationship appears to be related to the dose and duration of therapy. In a cohort study the incidence of meningioma was 11× higher in those taking >25 mg daily of CPA (which has progestogenic activity), with an incidence rate of 60/100,000 person years. There were no cases amongst low dose users. Progestin-associated meningiomas were also more frequently multiple meningiomas. Duration of treatment ranged from 2 to 27 years. None of the reported cases had a fatal outcome. Most case reports have been in women, although there have also been some in men, and there have been 9 case reports in transsexual women. Cyproterone acetate is a commonly used medication in women with PCOS and lower doses (e.g. 12.525 mg/day 10 days/cycle or 25 mg alternate days in post-menopausal women) are effective in controlling hirsutism. We recommend using the lowest efficacious dose of CPA in treating androgenic symptoms and to frequently assess the ongoing need for treatment, in order to minimise the rare but recognised risk of meningioma associated with this therapy.