Multiple meningiomas in patients with Turner syndrome

Dear Editor, We report two cases of multiple meningiomas in patients with Turner syndrome. Turner (Ullrich-Turner) syndrome is the most common diagnosed sex chromosome abnormality in females, affecting 1 in 2,000 live-born girls, and is characterized by complete or partial X chromosome monosomy [16]. A recent cohort study demonstrated an increased incidence of meningiomas in patients with Turner syndrome [14]. As girls diagnosed with Turner syndrome receive hormone replacement therapy and meningiomas are well-known hormone-related tumors, a possible role of hormones has been proposed to explain this association. These two cases add additional information on the relationships among Turner syndrome, hormone therapy andmeningioma, stressing the possible roles of genetic and/or hormonal factors. In the first case, a 56-year-old female with a history of Turner syndrome was diagnosed with multiple meningiomas after a vaso-vagal episode: in the left pterional area (22.4 cm), in the right cerebellopontine angle (CPA) (2.95 cm) and at the floor of the middle cerebral fossa (0.8 cm). She had been under substitutive estrogen-progesterone hormonal therapy for 42 years. Three years later, the control MRI revealed a significant increase in size of the left pterional meningioma (21 %) associated with perilesional edema without associated symptoms. Subtotal resection of the left pterional meningioma was performed, while maintaining surveillance for the left CPA tumor in the absence of mass effect on the brainstem and the desire to preserved ipsilateral serviceable hearing. The final pathological diagnosis was grade I meningothelial meningioma, with a high expression of progesterone receptors. After a 9-year follow-up, the patient remained asymptomatic. MRI follow-up showed a slight increase of the APC meningioma (8.86 cm) and stability of the left temporal meningioma (1.83 cm) (Fig. 1, upper panel). In the second case, a 54-year-old patient with Turner syndrome was seen in our department for progressive right transmission deafness and tinnitus. The diagnosis magnetic resonance imaging (MRI) performed revealed multiple meningiomas: one right pterional, one left pterional and one parasagittal rolandic. As the patient was under estrogen therapy, hormonal substitution was stopped. On imaging follow-up the left pterional meningioma progressively increased in size with evidence of perilesional edema without clinical expression. This lesion was totally removed 4 years later because it had grown almost 40% in volume, and no residual tumor was found on imaging. Microscopically, this was a WHO Grade I meningioma with a high expression of progesterone receptors. The two others meningiomas where stable on 7-year MRI follow-up (Fig. 1, lower panel). To date, 13 cases of meningiomas in patients with Turner syndrome have been published in the literature, including our cases. Of those, three were multiple meningiomas (23 %) [4, 8, 13]. Sporadic multiple meningiomas account for 9 % of all meningiomas [7]. Multiple meningiomas were initially reported to be related to the NF2 gene mutation with a clonal spread across the meninges [15], but might also correspond to mosaic NF2 cases [3, 6, 11]. Apart from patients with Turner syndrome, loss of chromosome X has already been found in A. Amelot (*) :G. Lemaistre : P. Cornu :M. Kalamarides : M. Peyre Department of Neurosurgery, Batiment Babinski, Groupe Hospitalier Pitie-Salpetriere, APHP, 47-83 boulevard de l’Hopital, 75013 Paris, France e-mail: aymmed@hotmail.fr