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| Year : 2019 | Volume
: 67
| Issue : 4 | Page : 1178-1179 |
Chordoid Glioma of the Third Ventricle
Rajeshwari K Muthusamy, Sangita S Mehta
Department of Pathology, Kovai Medical Center and Hospital, Coimbatore, Tamil Nadu, India
| Date of Web Publication | 10-Sep-2019 |
Correspondence Address:
Dr. Rajeshwari K Muthusamy
Department of Pathology, Kovai Medical Center and Hospital, Coimbatore - 641 014, Tamil Nadu
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.266267
How to cite this article:
Muthusamy RK, Mehta SS. Chordoid Glioma of the Third Ventricle. Neurol India 2019;67:1178-9 |
Sir,
A 52-year-old woman presented with a history of worsening headache for 5 days and recurrent giddiness on/off for a period of 5 years. Her blood investigations, vision, and endocrine function tests were normal. Magnetic resonance imaging (MRI) revealed a heterogeneous lesion measuring 3.7 × 2.5 × 2.2 cm in the suprasellar region with a cystic component. Solid portion showed intense heterogeneous post-contrast enhancement [Figure 1]. The lesion showed mass effect over the floor of the third ventricle causing its dilatation. Pituitary gland was seen separately from the lesion. She underwent subtotal excision of the tumor. Histology revealed sheets, fascicles, and cords of stellate to epithelioid cells in an abundant myxoid stroma. There was spillage of lymphocytes and plasma cells amidst tumor cells [Figure 2]a and [Figure 2]b. Mitotic figures were rare (<1 per 10 High Power Field). Differential diagnosis considered were chordoid glioma, chordoid meningioma, and metastatic carcinoma. The tumor cells were diffusely and strongly immunoreactive to glial fibrillary acidic protein (GFAP) [Figure 3]a, vimentin [Figure 3]b, and scattered reactivity to TTF-1 [Figure 3]c. Cells were negative for cytokeratin, Epithelial Membrane Antigen (EMA) and S-100. The MIB-1 proliferation index [Figure 3]d was very low (<2%). Based on histology and immunomarkers, a diagnosis of chordoid glioma of the third ventricle was made [Table 1]. Radiotherapy was suggested for the residual lesion but was denied by the patient. Chordoid glioma (World Health Organization Grade II) is a rare slow growing tumor that arises in midline and occupies the anterior portion of the third ventricle.[1] It is hypothesized that the anatomic site of origin can be traced to the specialized ependyma containing tanycytes covering the organum vasculosum of the lamina terminalis.[2] This uncommon tumor is usually seen in adults with a female preponderance and mean age of 46 years.[3] Wanschitz et al.,[3] in 1995, reported a solid tumor localized to third ventricle in a 24-year-old woman as meningioma with peculiar expression of GFAP. Subsequently, Brat et al.[4] described chordoid glioma as a novel clinicopathological entity based on a series of 8 cases. The authors determined that the tumor was of glial origin. Radiologically, chordoid glioma typically demonstrates solid circumscribed mass in the third ventricle with uniform contrast enhancement. Cystic change is uncommon,[4] which was present in our case. Histopathologically, it is a low grade tumor characterized by cords and clusters of plump cells in a prominent basophilic myxoid background. Lymphoplasmacytic infiltrate and Russell bodies are usually seen.[4] Atypia, necrosis, and microvascular proliferation are usually absent. Infiltration into the surrounding brain tissue is not a feature. | Figure 1: Coronal and Sagittal images show solid and cystic component well with uniform post-contrast T1 MPRAGE enhancement
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 | Figure 2: Fascicles and cords of cells amidst myxoid stroma (a)10× and (b) 40×
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 | Figure 3: Intense staining of tumor cells to GFAP (a) and vimentin (b), and scattered positivity to TTF-1 (c) and Ki-67 (d) (40×)
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 | Table 1: Salient features of differential diagnosis of supratentorial myxoid tumors
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Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| » References | |  |
| 1. | Brat DJ, Scheithauer BW. Chordoid glioma of the third ventricle. In: Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. WHO Classification of tumours of the central nervous system. 4 th ed. Lyon: International Agency for Research on cancer; 2007. p. 90-1.  |
| 2. | Leeds NE, Lang FF, Ribalta T, Sawaya R, Fuller GN. Origin of chordoid glioma of the third ventricle. Arch Pathol Lab Med 2006;130:460-4. |
| 3. | Wanschitz J, Schmidbauer M, Maier H, Rossler K, Vorkapic P, Budka H. Suprasellar meningioma with expression of glial fibrillary acidic protein: A peculiar variant. Acta Neuropathol 1995;90:539-44. |
| 4. | Brat DJ, Scheithauer BW, Staugaitis SM, Cortez SC, Brecher K, Burger PC. Third ventricular chordoid glioma: A distinct clinicopathologic entity. J Neuropathol Exp Neurol 1998;57:283-90. |
[Figure 1], [Figure 2], [Figure 3]
[Table 1]
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