Neurology India
Open access journal indexed with Index Medicus
  Users online: 42  
 Home | Login 
  About Current Issue Archive Ahead of print Search Instructions Online Submission Subscribe Etcetera Contact  
  Navigate Here 
 Search
 
  
 Resource Links
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (1,859 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

 
  In this Article
   References
   Article Figures

 Article Access Statistics
    Viewed2136    
    Printed68    
    Emailed1    
    PDF Downloaded50    
    Comments [Add]    
    Cited by others 2    

Recommend this journal

 


 
Table of Contents    
LETTER TO EDITOR
Year : 2011  |  Volume : 59  |  Issue : 3  |  Page : 469-471

Chordoid glioma of the third ventricle: A case report with review of literature


1 Department of Pathology, Global Hospital and Health City, Chennai, India
2 Department of Pathology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, India
3 Department of Neurosurgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Raebareli Road, Lucknow, India

Date of Submission06-Feb-2011
Date of Decision06-Feb-2011
Date of Acceptance01-Apr-2011
Date of Web Publication7-Jul-2011

Correspondence Address:
Mukul Vij
Department of Pathology, Global Hospital and Health City, Chennai
India
Login to access the Email id


DOI: 10.4103/0028-3886.82740

PMID: 21743188

Get Permissions



How to cite this article:
Vij M, Jaiswal S, Jaiswal AK, Jain M, Behari S. Chordoid glioma of the third ventricle: A case report with review of literature. Neurol India 2011;59:469-71

How to cite this URL:
Vij M, Jaiswal S, Jaiswal AK, Jain M, Behari S. Chordoid glioma of the third ventricle: A case report with review of literature. Neurol India [serial online] 2011 [cited 2014 Dec 21];59:469-71. Available from: http://www.neurologyindia.com/text.asp?2011/59/3/469/82740


Sir,

A 48-year-old male presented with gradual diminution of vision of 1 month duration and recurrent episodes of focal seizures involving right lower limb of 20 days duration. He became less communicative and developed bladder and bowel incontinence 4 days before admission. On examination, the patient was conscious, but preferring to sleep, and was following commands on coaxing. Cranial nerves and ocular fundi were normal. Tone on the right side was increased. There were no motor, sensory, or cerebellar deficits. His hematological and biochemical parameters were normal. Contrast computer tomography (CT) scan showed a mass lesion in the third ventricle. The tumor was isodense with homogenous enhancement [Figure 1]a. Magnetic resonance imaging (MRI) revealed a mass lesion in the third ventricle, iso to hypointense in T1-weighted images and iso to hyperintense on T2-weighted images with homogenous contrast enhancement and perilesional edema [Figure 1]b. Right pterional craniotomy and tumor decompression was performed through lamina terminalis. Intraoperatively, the tumor was yellowish, firm, and minimally vascular. Partial excision of the tumor was done. The patient had uneventful postoperative course and at follow-up of 10 months was doing well.
Figure 1: (a) Contrast CT scan axial section showing enhancing mass in the third ventricle; (b) contrast MRI scan coronal section showing homogenously enhancing mass in the third ventricle

Click here to view


The tumor consisted of clusters and singly scattered oval-to-polygonal epithelioid cells with abundant eosinophilic cytoplasm having relatively uniform centric or eccentrically placed round-to-oval hyperchromatic nuclei and inconspicuous nucleoli with abundant extracellular pools of mucin [Figure 2]a. Mitotic figures were not discernible. Occasional small foci of tumor showed sparse infiltrates of mature lymphocytes and plasma cells with Russell bodies. Alcian blue stain highlighted the background mucinous matrix [Figure 2]b. Immunohistochemistry tests for the cell proliferation marker Ki-67, glial fibrillary acidic protein (GFAP), vimentin, CD-34, epithelial membrane antigen (EMA), pan cytokeratin, S100, neurofilament protein (NFP) and synaptophysin were performed. Immunohistochemistry tests for GFAP [Figure 2]c, CD-34 [Figure 2]d and cytokeratin (CK) [Figure 2]e were positive within tumor cells. There was variable focal positivity for S100 and EMA [Figure 2]f. Immunohistochemistry tests for NFP and synaptophysin were negative. The Ki-67 proliferation index was very low ( <1%). Based on these findings, a diagnosis of chordoid glioma (CG) of the third ventricle was made.
Figure 2: Photomicrographs of chordoid glioma. (a) H and E (x200) stain shows cord like arrangement of epithelioid cells in myxomatous background. (b) Alcian blue highlights myxoid background. (c) Immunohistochemistry showing positivity for GFAP, (d) CD-34, (e) EMA, (f) CK

Click here to view


World Health Organization (WHO) defines CG as a non-invasive glial tumor of the third ventricle, located in third ventricle and histologically characterized by clusters and cords of epithelioid GFAP expressing tumor cells within variably mucinous stroma. [1] Although the incidence of CG cannot yet be evaluated because of its recent description, it seems to be an uncommon tumor.[1],[2] The precise histogenesis of CG remains uncertain. Immunohistochemical positivity for GFAP and vimentin favors a glial origin. Histopathologic features of CG have been similar in all the reported cases: Clusters and cords of epithelioid cells embedded in a mucinous matrix with prominent lymphoplasmacytic infiltrates. [3],[4] Common immunohistochemical results include a low Ki-67 index; diffuse GFAP, vimentin, and CD-34 positivity; focal cytokeratin and EMA positivity; inconsistent S100 protein positivity; and negativity for neuronal markers (synaptophysin, NF, and NSE). Few cases, however, were surprisingly positive for NF and NSE. [2],[3],[4],[5]

From a histological point of view, the most difficult differential diagnosis is between chordoma and chordoid meningioma. The absence of bone infiltration and of physaliphorous cells (typical features of chordoma) and the lack of psammoma bodies, cellular whorls, and nuclear pseudoinclusions (common in meningioma) can help in making the diagnosis. Immunohistochemistry may be decisive. Meningioma is EMA positive and GFAP negative, and chordoma is positive for EMA, cytokeratins, and S100 and negative for GFAP. CG, on the other hand, is typically immunopositive with GFAP and CD-34, and, in some cases, focally positive with EMA, cytokeratins, and S100. [1],[2],[3],[4],[5],[6]

Although CG is considered to be a low-grade tumor, the anatomic origin in the rostral third ventricular region in close juxtaposition to hypothalamic structures increases the risk of surgical morbidity and mortality. Incompletely resected tumors continue to enlarge slowly, and some patients experience a poor outcome. [7],[8],[9] Gross total surgical resection, when possible, appears to be the treatment of choice, although, as noted, the proximity to vital central nervous system structures increases the possibility of significant morbidity.

 
  References Top

1.Brat DJ, Scheithauer BW, Cortez SC, Reifenberger G, Burger PC. Chordoid glioma of the third ventricle. In: Kleihuis P, Cavenee WK, editors. Pathology and genetics of tumours of the nervous system. 2 nd ed. Lyon: International Agency for Research; 2000. p. 90-1.  Back to cited text no. 1
    
2.Vanhauwaert DJ, Clement F, Van Dorpe J, Deruytter MJ. Chordoid glioma of the third ventricle. Acta Neurochir 2008;150:1183-91.  Back to cited text no. 2
    
3.Buccoliero AM, Caldarella A, Gallina P, Di Lorenzo N, Taddei A, Taddei GL. Chordoid glioma: Clinicopathologic profile and differential diagnosis of an uncommon tumor. Arch Pathol Lab Med 2004;128:141-5.  Back to cited text no. 3
    
4.Gallina P, Pansini G, Mouchaty H, Mura R, Buccoliero AM, Di Lorenzo N. Anincidentally detected third ventricle chordoid glioma. Neurol India 2007;55:406-7.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.Iwami K, Arima T, Oooka F, Fukumoto M, Takagi T, Takayasu M. Chordoid glioma with calcification and neurofilament expression: Case report and review of the literature. Surg Neurol 2009;71:115-20.  Back to cited text no. 5
    
6.Sangoi AR, Dulai MS, Beck AH, Brat DJ, Vogel H. Distinguishing chordoid meningiomas from their histologic mimics: An immunohistochemical evaluation. Am J Surg Pathol 2009;33:669-81.  Back to cited text no. 6
    
7.Kurian KM, Summers DM, Statham PF, Smith C, Bell JE, Ironside JW. Third ventricular chordoid glioma: Clinicopathological study of two cases with evidence for a poor clinical outcome despite low grade histological features. Neuropathol Appl Neurobiol 2005;31:354-61.  Back to cited text no. 7
    
8.Jung TY, Jung S. Third ventricular chordoid glioma with unusual aggressive behavior. Neurol Med Chir 2006;46:605-8.  Back to cited text no. 8
    
9.Ortega-Martínez M, Cabezudo JM, Bernal-García LM, Fernández-Portales I, Gómez-Perals L, Gómez de Tejada R, et al. Chordoid glioma of the III ventricle. Case report and revision of the literature. Neurocirugia 2007;18:115-22.  Back to cited text no. 9
    


    Figures

  [Figure 1], [Figure 2]

This article has been cited by
1 Chordoid glioma of the third ventricle: Four cases including one case with papillary features
Ni, H.-C. and Piao, Y.-S. and Lu, D.-H. and Fu, Y.-J. and Ma, X.-L. and Zhang, X.-J.
Neuropathology. 2013; 33(2): 134-139
[Pubmed]
2 Chordoid glioma of the third ventricle: Four cases including one case with papillary features
Hai-Chun Ni,Yue-Shan Piao,De-Hong Lu,Yong-Juan Fu,Xiao-Li Ma,Xiao-Juan Zhang
Neuropathology. 2013; 33(2): 134
[Pubmed]



 

Top
Print this article  Email this article
   
Online since 20th March '04
Published by Medknow