Journal of Clinical Neuroscience 16 (2009) 1073–1074
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Case Reports
Intracerebral cystic rhabdoid meningioma Debnarayan Dutta a,*, Hsueh Ni Lee a, Anusheel Munshi a, Tejpal Gupta a, Subhada Kane b, Epari Sridhar b, Rakesh Jalali a a b
Department of Radiation Oncology, Tata Memorial Hospital, Dr E Borges Road, Parel, Mumbai 400012, India Department of Pathology, Tata Memorial Hospital, Mumbai, India
a r t i c l e
i n f o
Article history: Received 24 April 2008 Accepted 24 July 2008
Keywords: Cystic lesion Intracerebral meningioma Rhabdoid meningioma
a b s t r a c t A 36-year-old woman presented to our hospital with a short history of intermittent headaches. An MRI of the brain revealed a left temporal intracerebral cystic lesion with rim enhancement. Histopathology showed a malignant tumour with features of rhabdoid differentiation. Immunohistochemistry revealed that vimentin, epithelial membrane antigen and S-100 were positive, and that glial fibrillary acidic protein and the chromosome deletion 1p/19q were negative. The patient was diagnosed as having an intracerebral cystic rhabdoid meningioma. She was treated with surgery and post-operative radiotherapy. Cystic intracerebral rhabdoid meningiomas are rare. We discuss the clinical picture of this patient with reference to the published literature on this uncommon diagnosis. Ó 2008 Elsevier Ltd. All rights reserved.
1. Case report
2. Discussion
Meningiomas are among the most varied of all intracranial lesions. They present with a range of symptoms, as well as imaging and histopathological features, explaining why they are often called the ‘great masqueraders’. A 36-year-old female patient presented to our facility with intermittent episodes of headache and a gradual diminution of vision over four months. MRI scans of the brain revealed a 6.5 cm 4.5 cm well-circumscribed cystic lesion in the left temporal region, which was associated with a solid nodule (Fig. 1). The mass was hypointense on both T1-weighted and T2 fluid-attenuated inversion recovery (FLAIR) images. On T1 sequences with contrast, both the nodule and the cyst wall showed intense enhancement. The cyst appeared intracerebral, without dural attachment, and had minimal edema. The patient underwent near-total excision of the mass. Histopathology was suggestive of a high-grade malignant tumor infiltrating the brain, showing rhabdoid morphology with a dense lymphocytic background suggestive of a rhabdoid meningioma (Supplementary Fig. 1). Immunohistochemistry showed tumor cell positivity for vimentin and S-100, and focal positivity for epithelial membrane antigen (EMA), progesterone receptor and p53 protein. Glial fibrillary acidic protein (GFAP) immunostaining and 1p/19q deletion chromosomal studies were negative (Supplementary Fig. 1). Positron emission tomography scans of the whole body revealed that there was increased uptake in the involved area of brain only, which was suggestive of a primary rhabdoid meningioma. The final impression was that of an intracerebral cystic rhabdoid meningioma. She was treated with post-operative adjuvant radiotherapy and is currently undergoing routine follow-up.
Meningiomas are one of the most common dural-based extraaxial tumors seen in adults, and account for approximately 25% to 30% of all intracranial tumors.1 Cystic intraparenchymal meningiomas are rare, with only a few case reports in the literature.2 The most common locations for cystic meningiomas are the cerebral convexity and the sphenoid ridge.3 Meningiomas are dural-based lesions, and usually arise from the meningothelial cells that line the arachnoid membrane.3 Meningiomas without dural attachment are postulated to arise from the ectopic meningothelial cells within the choroid plexus, the tela choroidea or the pia mater stroma.2 Intra-axial meningiomas may arise from the pia-arachnoid stromal cells that invest the perforating blood vessels as they enter the surface of the brain. These meningiomas appear as a hyperintense mass on T1weighted MRI, while on T2-weighted FLAIR sequences they appear as a hypointense mass, with brilliant contrast enhancement seen either within the brain parenchyma or the sylvian cistern.2 Supratentorial meningiomas without dural attachment have been classified into five types.4 In this particular instance, as the cystic lesion in the left parietal region had rim enhancement, it would be classified as a type III cystic meningioma. These intracerebral meningiomas occur in younger patients, compared to extraaxial meningiomas.5 They are usually benign tumors with a favorable prognosis, with fibroblastic meningiomas constituting the major subtype.5 Although rare, intracerebral meningiomas may present with intratumoral cysts. There is debate regarding the mechanism of cyst formation within meningiomas.6 These cysts may be formed by absorption of hemorrhage, secretion – especially by clear cell meningiomas – or from necrosis of the tumor.6 Cystic meningiomas are frequently confused with glial or metastatic tumors. Intracerebral ‘‘cystic” meningiomas, and rhabdoid meningiomas with a cystic component, are rarely described.7
* Corresponding author. Tel.: +91 22 2417 7162; fax: +91 22 2414 6937. E-mail address:
[email protected] (D. Dutta).
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Case Reports / Journal of Clinical Neuroscience 16 (2009) 1073–1074
Fig. 1. MRI scans of the cystic intracerebral mass. (A) Unenhanced axial T1-weighted MRI showing a hypointense nodule; (B) Contrast-enhanced axial T1-weighted MRI showing peripheral ring and nodule enhancement. (C) T2-weighted axial MRI showing hyperintensity of the cyst fluid and surrounding edema; and (D) T2-weighted fluid attenuated inversion recovery (FLAIR) axial sequence showing hyperintensity of the rim and nodule and the surrounding edema.
Unlike classical meningiomas, rhabdoid meningiomas are aggressive. They have a higher probability of recurrence, poorer overall survival and occur in younger patients.7,8 Histologically, rhabdoid meningiomas exhibit anaplasia, high mitotic activity, and a markedly increased MIB-1 (mindbomb homolog 1 [Drosophila]) labeling index (LI), which is a marker of cell proliferation. In addition, cells are abundantly vimentin-positive, whorled with a fibrillary cytoplasm. EMA is variably positive, but GFAP, S-100, desmin and myogenin are universally negative.9 Rhabdoid meningiomas may occasionally occur as intracerebral tumors. However, radiological features of intracerebral rhabdoid meningiomas are similar to those for classical meningiomas. Intracerebral rhabdoid tumors are hyperintense on unenhanced T1-weighted MRI scans, hypointense on T2-weighted FLAIR images and heterogeneously contrast-enhancing.8 Rhabdoid meningiomas are treated with surgery and post-operative radiotherapy. Chemotherapy has been tried in these tumors without any significant survival benefit. Despite adequate surgical excision and radiotherapy, recurrence is the rule; recurrences do not necessarily occur in the primary site. Re-excision of the tumor or palliative chemotherapy are the available treatment options in such instances. In view of the paucity of clinical experience, intracerebral rhabdoid meningiomas are usually managed with the same schedule. The meningioma in our patient had three unusual features; the rhabdoid variant, intracerebral location and the
doi:10.1016/j.jocn.2008.07.085
intralesional cyst. While the latter two features bear a good prognosis for meningiomas in general, the rhabdoid variant, however, strongly overrides this and indicates a poor outcome for this patient. Supplementary data Supplementary data associated with this article can be found, in the online version, at doi:10.1016/j.jocn.2008.07.085. References 1. Claus EB, Bondy ML, Schildkraut JM. Epidemiology of intracranial meningioma. Neurosurg 2005;57:1088–95. 2. Mamourian AC, Lewandowski AE, Towfighi J. Cystic intraparenchymal meningioma in a child: case report. Am J Neuroradiol 1991;12:366–7. 3. Ismail H, Zorludemir L. Intraparenchymal meningioma. J Clin Neurosci 2005;12:605–8. 4. Jung TY, Jung S, Shin SR, et al. Clinical and histopathological analysis of cystic meningiomas. J Clin Neurosci 2005;12:651–5. 5. Zhang J, Chi L, Meng B, et al. Meningioma without dural attachment: case report, classification, and review of the literature. Surg Neurol 2007;67:535–9. 6. Sridhar K, Ravi R, Ramamurthi B, et al. Cystic meningiomas. Surg Neurol 1995;43:235–9. 7. Liu M, Liu Y, Li X, et al. Cystic meningioma. J Clin Neurosci 2007;14:856–9. 8. Han J, Seol H, Kim D, et al. A case of rhabdoid meningioma. J Korean Neurosurg Soc 2006;391:144–7. 9. Hojo H, Abe M. Rhabdoid papillary meningioma. Am J Surg Pathol 2001;25:964–9.
