One of a kind-chordoid glioma in the fourth ventricle: a case report and literature review

Abstract

Chordoid glioma (CG) is a rare brain tumor that is known for its characteristic location in the third ventricle. A wide spectrum of radiological presentations has been described, with few common features among them. Its radiological diagnosis is mainly suggested by location. However, several cases of CG with atypical locations have been described, illustrating that CG is not limited to the third ventricle, and should be considered in the list of radiological differential diagnosis for intraventricular masses. We present here a case of CG that was found in the fourth ventricle.

Keywords: Chordoid glioma; fourth ventricle; intraventricular tumor; magnetic resonance imaging.

Fig. 1.

(a and b) MRI study shows a T1-weighted hypointense, T2-weighted hyperintense intraventricular lesion in the fourth ventricle, complicated by moderate obstructive hydrocephalus. (c and g) Ring enhancement and mural nodules are seen. (d and f) Intralesional fluid–fluid level with susceptibility artifacts indicates tumor hemorrhage. (e) No restricted diffusion is demonstrated.

Fig. 2.

Photomicrographs of the tumor. (a) Hematoxylin and Eosin stained slides show cords and clusters of eosinophilic abundant tumor cytoplasm on myxoid basophilic matrix. (b) Immunostained slide for glial nucleic marker Olig2 shows strong expression in neoplastic cells. (c) Negative immunostaining for Brachyury, a sensitive and fairly specific diagnostic marker for chordoma. (d) Immunostaining for somatostatin receptor (SSTR) is also negative.

Fig. 3.

Two-year postoperative MRI shows ∼1 cm residual intraventricular tumor tissue at lower part of the cerebral aqueduct.