Correspondence

Growth of a Meningioma in a Transsexual Patient after Estrogen–Progestin Therapy

N Engl J Med 2007; 357:2411-2412December 6, 2007

Article

To the Editor:

A 28-year-old male-to-female transsexual patient presented with severe headache and visual disturbances; clinical examination showed visual impairment, with bitemporal inferior visual-field defects, papilledema of the left eye, and optic atrophy of the right eye. The patient was euphoric and confused. Personality changes noted during the previous 4 months had been attributed by family members to the new gender identity of the patient. The patient had been taking a feminizing endocrine regimen of ethinyl estradiol (100 μg per day orally) and cyproterone acetate (100 mg per day orally) for the previous 5 years. After 2 years of hormone treatment, the patient underwent gonadectomy for sex reassignment, and estradiol-17-undecanoate (100 mg twice weekly administered intramuscularly) was added to the patient's therapy for the following 2 years. A cerebral magnetic resonance imaging (MRI) scan obtained 3 years before presentation to evaluate an increased prolactin level (42 ng per milliliter) was negative (Figure 1AFigure 1MRI Scans and Histologic Findings.). On admission, a contrast-enhanced MRI scan revealed a giant olfactory-groove meningioma (Figure 1B). After a radical tumor resection, the histologic diagnosis was meningothelial meningioma (World Health Organization grade I), which was negative for estrogen receptors, with a Ki-67 index of 5% and an MIB-1 index of 3.4% per high-power field (Figures 1C and 1D, respectively). At 1 year of follow-up, the patient was continuing with the hormone therapy at a lower dose (50 μg of ethinyl estradiol per day and 100 mg of spironolactone per day), and a contrast-enhanced MRI scan showed no recurrence of the tumor. The patient's behavioral changes had regressed, and the visual impairments were ameliorated.

Cross-sex hormonal therapy is an important component of the endocrine regimen in transsexual people. Reported adverse effects in this population include venous thromboembolytic disease,1 breast cancer,2 lactotroph hyperplasia,3 and an increase in prolactin levels with possible growth of prolactinomas.4 The role of sex hormones in the development of intracranial meningioma has been proposed as one hypothesis to explain the overabundance of such tumors in women. The risk of meningioma is increased among postmenopausal women who have a history of using hormone-replacement therapy and among women who have used long-acting contraceptives.5 In this case, a causal association between the growth of a meningioma and the hormone therapy was suggested by the negative cerebral MRI scan obtained 3 years before presentation. This report of abrupt growth of an intracranial meningioma after use of high doses of steroid therapy in a transsexual patient should prompt clinicians caring for transsexual patients to consider the possibility of such an event.

Roberto Gazzeri, M.D.
Marcelo Galarza, M.D.
San Giovanni-Addolorata Hospital, 00100 Rome, Italy
robertogazzeri@gmail.com

Giovanni Gazzeri, M.D.
San Filippo Neri Hospital, 00100 Rome, Italy

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Citing Articles (10)

Citing Articles

1. 1

   Thomas J. Gruber, Andrew J. Fabiano, George Deeb, Shashikant B. Lele, Robert A. Fenstermaker. (2011) Intracranial Meningiomas in Patients with Uterine Sarcoma Treated with Long-Term Megestrol Acetate Therapy. World Neurosurgery 76:5, 477.e16-477.e20
   CrossRef

2. 2

   Lucía Cea-Soriano, Tilo Blenk, Mari-Ann Wallander, Luis A. García Rodríguez. (2011) Hormonal therapies and meningioma: Is there a link?. Cancer Epidemiology
   CrossRef

3. 3

   Anne Cowppli-Bony, Ghislaine Bouvier, Marjory Rué, Hugues Loiseau, Anne Vital, Pierre Lebailly, Pascale Fabbro-Peray, Isabelle Baldi. (2011) Brain tumors and hormonal factors: review of the epidemiological literature. Cancer Causes & Control 22:5, 697-714
   CrossRef

4. 4

   Helene Cebula, Trang Q. Pham, Patrick Boyer, Sébastien Froelich. (2010) Regression of meningiomas after discontinuation of cyproterone acetate in a transsexual patient. Acta Neurochirurgica 152:11, 1955-1956
   CrossRef

5. 5

   Amy R. deIpolyi, Seunggu J. Han, Andrew T. Parsa. (2010) Development of a symptomatic intracranial meningioma in a male-to-female transsexual after initiation of hormone therapy. Journal of Clinical Neuroscience 17:10, 1324-1326
   CrossRef

6. 6

   Patrick Y. Wen, Eudocia Quant, Jan Drappatz, Rameen Beroukhim, Andrew D. Norden. (2010) Medical therapies for meningiomas. Journal of Neuro-Oncology 99:3, 365-378
   CrossRef

7. 7

   A. M. G. Goncalves, P. Page, V. Domigo, J.- F. Meder, C. Oppenheim. (2010) Abrupt Regression of a Meningioma after Discontinuation of Cyproterone Treatment. American Journal of Neuroradiology 31:8, 1504-1505
   CrossRef

8. 8

   Andrew D. Norden, Jan Drappatz, Patrick Y. Wen. (2009) Advances in meningioma therapy. Current Neurology and Neuroscience Reports 9:3, 231-240
   CrossRef

9. 9

   Roberto Gazzeri, Marcelo Galarza, Giovanni Gazzeri. (2008) Giant olfactory groove meningioma: ophthalmological and cognitive outcome after bifrontal microsurgical approach. Acta Neurochirurgica 150:11, 1117-1126
   CrossRef

10. 10

    (2008) Current awareness: Pharmacoepidemiology and drug safety. Pharmacoepidemiology and Drug Safety 17:6, i-xvi
    CrossRef