Multiple Skull Base Meningiomata in a Transgender Patient: Case Report and Literature Review

Introduction: There is increasing evidence to show that skull base meningioma development is associated with the use of exogenous sex hormones. Patients on long-standing hormone replacement therapy (HRT), particularly trans-sexual patients are at risk for meningioma development and progressive growth. Here we present a case of a male-to-female transsexual patient who had been on long-term HRT for an extended period of time with both patients requiring surgical treatment at our institution due to multiple growing meningiomas.

Case Description: We present a case of a 67-year-old patient genetic male (male-to-female transsexual) who presented initially 14 years after a sex-change operation with visual impairment. The patient had been on long-term cyproterone acetate and estrogen supplementation. The initial radiological imaging demonstrated a left anterior clinoid meningioma and a small olfactory groove meningioma. The clinoid meningioma was resected, and the patient was followed up clinically.

She presented to our unit 4 years after with worsening vision and some cognitive impairment, and subsequent imaging showed a left middle cerebral aneurysm and significantly larger meningiomata i.e., small left clinoid meningioma, large olfactory groove meningioma and a right tentorial meningioma. The olfactory groove meningioma, as well as the recurrent clinoid meningioma was resected.

The histological diagnosis for the clinoid meningioma was WHO Grade I (transitional meningioma) – similar to the previous histology results of the same lesion. The olfactory groove meningioma was diagnosed as a chordoid (WHO Grade II) meningioma. On Ki67 immunolabeling, the nuclear proliferation index was 6 to 8%.

Immunohistochemical (IH) analysis of the chordoid meningioma showed both estrogen and progesterone receptor expression. Intratumoral heterogeneity was noted—Cluster 1 (estrogen receptor: positive in 60% tumor cells, progesterone receptor: positive in 90% tumor cells) and Cluster 2 (estrogen receptor: positive in 15% tumor cells, progesterone receptor: positive in 90% tumor cells).

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Conclusion: Approximately 0.6% of the population identify as transgender, although it is suspected that the number might be much higher.

Explanation about the risks of developing multiple meningiomata, radiological screening as part of the follow-up protocol and possible use of alternative medications should be considered as integral components of the management plan for trans-sexual patients (possibly, in specialist clinics).

Long-standing use of HRTs and presentation with neurological symptoms are risk factors that should be justifiably highlighted in these patients.

No conflict of interest has been declared by the author(s).