CASE 15287 Published on 12.12.2017

Chordoid glioma of the third ventricle

Section

Neuroradiology

Case Type

Clinical Cases

Authors

Carlos Fernández Cabrera, María Conde Martín, Patricia Martín Medina, Laura Koren, Ana Ramos González

H.U 12 de Octubre,University Hospital 12 de Octubre,Radiodiagnostico; Avenida de Andalucia 28041 Madrid, Spain; Email:carlosfc89@gmail.com
Patient

54 years, female

Categories
Area of Interest Neuroradiology brain ; Imaging Technique MR, MR-Diffusion/Perfusion
Clinical History
A previously healthy 54-year-old woman was referred with a 14-months history of headaches and right facial paraesthesias.
Imaging Findings
CT examination showed the presence of an isodense suprasellar solid mass involving the third ventricle (Fig. 1), so a brain MRI was performed.

Brain MRI revealed a solid polylobulated mass with perilesional vasogenic oedema centered on the third ventricle anterior recess, measuring about 2, 5 cm in the craniocaudal axis. The mass appeared to invade the hypothalamus and displaced the floor of the third ventricle, but remained separate from the pituitary stalk.

The tumour was slightly heterogeneous, predominantly isointense at T1 (Fig. 2) and hyperintense at T2 and FLAIR-weighted MRI sequences (Fig. 3). After paramagnetic contrast intravenous injection, the lesion demonstrated intense enhancement (Fig. 4). No diffusion restriction was demonstrated (Fig. 5). Perfusion imaging using T2*-weighted gradient-echo sequence (Fig. 6) showed no significant increase of relative cerebral brain volume (rCBV) compared to healthy white matter (the maximum rCBV was 1, 5).
Discussion
Chordoid glioma is a rare, slow growing neoplasm (WHO grade II) containing both glial and chordoid histologic elements. It arises from the anterior third ventricle and is frequently adherent to the hypothalamus. They are believed to originate from the ependymal cells of the lamina terminalis [1].
Patients typically present with symptoms related to the local mass effect of the tumour such us headaches, obstructive hydrocephalus and hypothalamic dysfunction [2].

The best imaging diagnostic tool for evaluation of suprasellar masses is brain MRI. Intraventricular third ventricular masses constitute an uncommon subset of suprasellar masses, as in this case. These tumours may arise from choroid plexus, like primary choroid plexus tumours (papilloma and carcinoma), which usually have a papillary or lobulated appearance and may show calcifications and foci of haemorrhage.
Rarely, purely intraventricular neoplasms such as meningioma, craniopharyngioma and chordoid glioma may be found [3]. At MRI, chordoid glioma appears as a well-defined ovoid mass that is isointense on T1-weighted images and enhances intensely with gadolinium. Multiplanar imaging helps the radiologist to depict the typical hypothalamic involvement and the posterior infundibulum displacement. The greatest diameter of this tumour is usually observed in the supero-inferior orientation. Intratumoural haemorrhage or necrosis has not been reported, and calcification is rare. There has been only two case reports describing the perfusion MR imaging features of chordoid glioma. Grand et al. [4] have reported that the CBV of the tumour is not elevated compared to that of white matter. By contrast, So Yeon Ki et al. [5] have recently reported an elevated maximum CBV within the tumour (rCBV 6, 95). We found no significant increase of rCBV within the tumour.
In contrast, calcification may be present in approximately 50% of meningiomas, which may also show cystic areas and usually demonstrate high blood volumen at perfusion-weighted imaging. Intrinsic third ventricle craniopharyngiomas are rare and usually appear as heterogeneous masses of variable intensity (often hyperintense on T1WE and hypointense on T2WE), which may help us to differentiate them from other intraventricular neoplasms [6].

Clinical outcome for chordoid glioma cases reported so far have been poor, which is partially due to the deep location of the tumour, and also due to typical tumour features such as solid consistency and adherence to critical neurovascular structures, which often render radical resection difficult or impossible [7].

When confronted with a suprasellar mass with the imaging features described, the neuroradiologist must consider chordoid glioma in the differential diagnosis.
Differential Diagnosis List
Chordoid glioma of the third ventricle.
Intrinsic third ventricular craniopharyngioma.
Primary choroid plexus papilloma of the third ventricle.
Chordoid glioma of the third ventricle.
Intraventricular meningioma.
Final Diagnosis
Chordoid glioma of the third ventricle.
Case information
URL: https://eurorad.org/case/15287
DOI: 10.1594/EURORAD/CASE.15287
ISSN: 1563-4086
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