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CASE REPORT
Year : 2010  |  Volume : 58  |  Issue : 4  |  Page : 659-661

Contrasting behavior of glio-ependymal cysts: A report of two cases and literature review


1 Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Histopathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India

Date of Acceptance01-Feb-2010
Date of Web Publication24-Aug-2010

Correspondence Address:
Alok Umredkar
Department of Neurosurgery, Postgraduate Institute of Medical Education and Research, Chandigarh
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.68702

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 » Abstract 

Cystic lesions of the brain may present with seizures or headache due to raised intracranial pressure. These cysts when associated with developmental brain anomalies may turn out to be pathologic surprises. In the present communication, two patients with glio-ependymal cysts were described with contrasting symptomatologies and surgical management. Non-enhancing cystic lesions of the brain, without mural nodule, may turn out to be glio-ependymal cysts, requiring total surgical excision or marsupilization.


Keywords: Cyst, ependymal cyst, neuroepithelial cyst


How to cite this article:
Umredkar A, Mohindra S, Gupta R, Bal AK. Contrasting behavior of glio-ependymal cysts: A report of two cases and literature review. Neurol India 2010;58:659-61

How to cite this URL:
Umredkar A, Mohindra S, Gupta R, Bal AK. Contrasting behavior of glio-ependymal cysts: A report of two cases and literature review. Neurol India [serial online] 2010 [cited 2019 Sep 22];58:659-61. Available from: http://www.neurologyindia.com/text.asp?2010/58/4/659/68702



 » Introduction Top


Intracranial cysts may be intracerebral or extracerebral. Arachnoid and subarachnoid cysts are among the common extracerebral cysts. These cysts may be of developmental, traumatic or inflammatory origin. The cysts, lined by epithelial, ependymal cells are described under various names: ependymal, glio-ependymal, neuroepithelial, choroidal epithelial and epithelial cysts. [1] We report the clinical details of two young patients with glio-ependymal cysts.


 » Case Reports Top


Case 1

A 25-year-old female presented with recurrent episodes of seizures for the past 2 years. Seizures were generalized tonic-clonic in nature and were partially controlled with antiepileptic drugs (AED). She had early morning headache for the past 1 year and developed altered sensorium four days prior to presentation, without any focal signs. Computerized tomography (CT) scan showed a well-defined non-enhancing cystic lesion in right frontal lobe. Magnetic resonance imaging (MRI) confirmed the CT findings and ruled out the presence of any associated mural nodule [Figure 1]a-c.
Figure 1 : (a) Axial section of noncontrast T1 weighted MRI scan showing cystic lesion within right frontal lobe of case 1. (b) Sagittal section showing similar cyst in case 1. (c) Axial section of contrast-enhanced MRI scan showing no contrast enhancement of cystic lesion and absence of any mural nodule of case 1. (d) Photomicrograph showing a cyst lined by a single layer of columnar epithelium and the wall composed of glial tissue (hematoxylin and eosin, ×40) in case 1

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She underwent right frontal craniotomy and excision of the cysts. Intraoperatively, the cysts were thin membranous, well defined, easily separable from brain parenchyma, containing clear fluid. Postoperative course was uneventful and she remains symptom-free at 1 year of follow-up. The surgical specimen revealed a thick layer of glial/connective tissue lined by a single layer of columnar epithelium. The epithelium was diffusely positive for S-100 protein and glial fibrillary acidic protein (GFAP), confirming glio-ependymal cyst [Figure 1]d.

Case 2

A 25-year-old male presented in a decerebrating state in the neuro-surgical emergency. The MRI scans [Figure 2]a-c brought by the patient done showed a large cystic lesion in left temporal lobe. She had an emergency twist drill tap of the cyst, which showed clear cerebrospinal fluid (CSF) like fluid. After 12 hours of the life-saving cyst tap, patient had improvement in his conscious state, but remained in altered sensorium. Left temporal craniotomy and mid-temporal gyrus resection was performed so as to lay open the cyst. The cyst wall was biopsied which showed a layer of glial tissue lined by columnar epithelium. Definitive pathologic diagnosis was glio-ependymal cyst. Postoperatively, the patient continued to improve and remains asymptomatic at 18 months of follow-up and the imaging showed no residual or recurrence lesion [Figure 2]d.
Figure 2 : (a) Axial section of T2 weighted scan showing a large left sided temporal horn cyst with intense brain edema in case 2. (b) Axial section of T1 weighted scan with similar findings in case 2. (c) Sagittal section of contrast-enhanced scan ruling out any mural nodule in case 2. (d) Contrast T1 weighted scan showing no lesion residual or recurrence of lesion after 18 months of surgery in case 2

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 » Discussion Top


Glio-ependymal cysts, also known as neuroepithelial cysts, are thought to arise from ectopic rests of primitive neuroglial tissue, and hence, can arise anywhere in the neuraxis [2],[3] [Table 1]. Generally, these cysts present in the second or third decade, [4] but have been reported at all ages and even in intrauterine period. [3],[5],[6] Clinical presentations include seizures, [7],[8] symptoms due to mass effect [3],[7],[8] and some are incidental. [9],[10],[11]
Table 1 : Reported cases of glio-ependymal cysts

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The cyst wall is composed of an inner glial layer with a luminal ependymal lining and an outer fibrous layer with no external lining cells. The lining ependymal cells have vacuoles, bleb-like protrusion, normal and abnormal cilia, and microvilli, the latter are sometimes distended and detached into the lumen. These cysts have neither pinocytic vesicles nor a basement membrane. The glial layer contains astrocytes, glial bundle and ependymal cells. This cyst appears to have originated from a leptomeningeal neuroglial heterotopia and may represent a continued proliferative growth rather than degenerative change of the heterotopias. [9]

On imaging, glio-ependymal cysts appear as nonenhancing CSF containing unilocular thin-walled cysts, found both in intra-axial and extra-axial locations. They are difficult to be differentiated from arachnoid cyst, enterogenous cyst, ependymal cyst, dermoid cyst, encephalocele and congenital dysplasia of sphenoid wing in neurofibromatosis 1. [3] MR proton spectroscopy remains an inconclusive diagnostic tool till the present time; however, resonance of N-acetyl group of the compound may be a subtle indicator of glial tissue within ependymal lining. [12]

Associated congenital brain malformations have been documented in the antenatal period, [6],[13] adolescents and elderly. Clinical presentation of raised intracranial pressure may be attributed to the cysts per se while associated developmental anomalies may account for seizures.

Treatment may not be warranted in incidentally detected glio-ependymal cysts, but treatment may be required for symptomatic cysts. Surgical management may be considered superior to AEDs. The proliferative nature of these lesions justifies their surgical removal. [13] Surgical intervention may include cysto-peritoneal shunt, [3] cysto-arachnoid shunt, [7] partial excision, [13] marsupilization, [1] fenestration or total excision. [4],[8],[14] Surgical outcome remains satisfactory without recurrence or repeated surgeries after complete removal.

 
 » References Top

1.Monaco P, Filippi S, Tognetti F, Calbucci F. Glioependymal cyst of the cerebellopontine angle. J Neurol Neurosurg Psychiatry 1995;58:109-10.  Back to cited text no. 1      
2.Balasubramaniam C, Balasubramaniam V, Santosh V. Intramedullary glioependymal cyst and tethered cord in an infant. Childs Nerv Syst 2004;20:496-8.   Back to cited text no. 2      
3.Obaldo RE, Shao L, Lowe LH. Congenital glioependymal cyst presenting with severe proptosis. AJNR Am J Neuroradiol 2007;28:999-1000.  Back to cited text no. 3      
4.Tsuchida T, Kawamoto K, Sakai N, Tsutsumi A. Glioependymal cyst in the posterior fossa. Clin Neuropathol 1997;16:13-6.  Back to cited text no. 4      
5.Hassan J, Sepulveda W, Teixeira J, Cox PM. Glioependymal and arachnoid cysts: unusual causes of early ventriculomegaly in utero. Prenat Diagn 1996;16:729-33.  Back to cited text no. 5      
6.Mόhler MR, Hartmann C, Werner W, Meyer O, Bollmann R, Klingebiel R. Fetal MRI demonstrates glioependymal cyst in a case of sonographic unilateral ventriculomegaly. Pediatr Radiol 2007;37:391-5.  Back to cited text no. 6      
7.Boockvar JA, Shafa R, Forman MS, O'Rourke DM. Symptomatic lateral ventricular ependymal cysts: criteria for distinguishing these rare cysts from other symptomatic cysts of the ventricles: case report. Neurosurgery 2000;46:1229-32.   Back to cited text no. 7      
8.Pawar SJ, Sharma RR, Mahapatra AK, Dev EJ. Giant ependymal cyst of the temporal horn -- an unusual presentation. Case report with review of the literature. Pediatr Neurosurg 2001;34:306-10.  Back to cited text no. 8      
9.Ho KL, Chason JL. A glioependymal cyst of the cerebellopontine angle. Immunohistochemical and ultrastructural studies. Acta Neuropathol 1987;74:382-8.  Back to cited text no. 9      
10.Pelkey TJ, Ferguson JE 2nd, Veille JC, Alston SR. Giant glioependymal cyst resembling holoprosencephaly on prenatal ultrasound: case report and review of the literature. Ultrasound Obstet Gynecol 1997;9:200-3.  Back to cited text no. 10      
11.Wu CL, Tsai CY, Lin LS, Lee CC. Subarachnoid glioependymal cyst: a case report. Zhonghua Yi Xue Za Zhi (Taipei) 1994;53:116-9.  Back to cited text no. 11      
12.Shukla-Dave A, Gupta RK, Roy R, Husain N, Paul L, Venkatesh SK, et al. Prospective evaluation of in vivo proton MR spectroscopy in differentiation of similar appearing intracranial cystic lesions. Magn Reson Imaging 2001;19:103-10.  Back to cited text no. 12      
13.Tange Y, Aoki A, Mori K, Niijima S, Maeda M. Interhemispheric glioependymal cyst associated with agenesis of the corpus callosum--case report. Neurol Med Chir (Tokyo) 2000;40:536-42.  Back to cited text no. 13      
14.Frazier J, Garonzik I, Tihan T, Olivi A. Recurrent glioependymal cyst of the posterior fossa: an unusual entity containing mixed glial elements. Case report. J Neurooncol 2004;68:13-7.  Back to cited text no. 14      


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1]

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