Chordoid glioma of the third ventricle [Neurosurgery Wiki]

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====== Chordoid glioma of the third ventricle ======

Chordoid glioma of the [[third ventricle]] located exclusively in the mid-anterior portion of the [[third ventricle]] along the anterior aspect of the hypothalamic wall near the [[hypothalamus]] and optic nerves, with the histological features of a [[chordoma]] and immuno-labeling for [[glial fibrillary acidic protein]]
((Al-Zubidi N, McGlynn MM, Chévez-Barrios P, Yalamanchili S, Lee AG.
Neuro-Ophthalmologic Features of Chordoid Glioma. J Neuroophthalmol. 2013 Oct 9. 
[Epub ahead of print] PubMed PMID: 24113763.)).

The term "chordoid glioma" was introduced to denote a circumscribed, apparently, low-grade neoplasm arising in or preferentially involving the [[third ventricle]] of middle-aged women
((Vajtai I, Varga Z, Scheithauer BW, Bodosi M. Chordoid glioma of the third
ventricle: confirmatory report of a new entity. Hum Pathol. 1999 Jun;30(6):723-6.
PubMed PMID: 10374785.)).

===== Epidemiology =====

A rare (only ≈ 80 case reports).
Primarily a tumor of adulthood. Female:male ratio=3:1. Solid, enhancing mass on imaging. Mitotic activity is absent in most. GFAP immunostaining is common, [[S100]] reactivity is variable. Histologically similar appearing to [[chordoid meningioma]], which lacks GFAP staining. Attachment to the wall of third ventricle ([[hypothalamus]]) may prevent total removal.

===== Diagnosis =====

The MR appearances possess unique features which are very helpful in diagnosis of chordoid glioma, but to make a definite diagnosis is based on clinical and histopathological features and immunohistochemical staining
((Shi HY, Tian Y, Shi YZ, Wang JD, Lu GM. [The MRI findings in chordoid glioma
of the third ventricle]. Zhonghua Yi Xue Za Zhi. 2013 Jan 29;93(5):373-5.
Chinese. PubMed PMID: 23660212.)).
----
Characterized by clusters and chords of epithelial cells expressing [[GFAP]], within a mucinous stroma often containing a lymphoplasmacytic infiltrate.


===== Treatment =====

Low dose gamma knife radiosurgery after less invasive microsurgery is both safe and effective for the control of chordoid glioma of the third ventricle over a very long follow-up period
((Kobayashi T, Tsugawa T, Hashizume C, Arita N, Hatano H, Iwami K, Nakazato Y,
Mori Y. Therapeutic approach to chordoid glioma of the third ventricle. Neurol
Med Chir (Tokyo). 2013;53(4):249-55. PubMed PMID: 23615418.)).

===== Outcome =====
Slow-growing, noninvasive, benign tumor ([[WHO grade II]])