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Chordoid Glioma: A Neoplasm Unique to the Hypothalamus and Anterior Third Ventricle

Martin G. PomperGo,a, Theodore J. Passea, Peter C. Burgera, Bernd W. Scheithauera and Daniel J. Brata

a From the Departments of Radiology (M.G.P., T.J.P.) and Pathology (P.C.B., D.J.B.), The Johns Hopkins University School of Medicine, Baltimore, MD, and The Mayo Clinic (B.W.S.), Rochester MN.



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FIG 1. Representative appearance of chordoid glioma on MR image. Note the location of the mass in the hypothalamus/third ventricle region, dense enhancement, and greatest dimension in the superoinferior orientation.

A, Sagittal contrast-enhanced spin-echo MR image (500/15) of Patient 1, a 59-year-old man. Note hypothalamic involvement (arrow).

B, Coronal contrast-enhanced spin-echo MR image (500/15) of Patient 1, a 59-year-old man. Note hypothalamic involvement (arrow).

C, Unenhanced sagittal spin-echo MR image (500/14) of Patient 3, a 36-year-old man. Arrows demarcate the suprasellar mass.

D, Contrast-enhanced sagittal spin-echo MR image (500/14) of Patient 3, a 36-year-old man. Note the posterior displacement of the infundibulum (arrow).



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FIG 2. CT scans of Patient 2, a 41-year-old woman with chordoid glioma.

A, Unenhanced axial CT scan reveals a hyperdense mass (arrow) that enhances uniformly in the suprasellar region.

B, Contrast-enhanced axial CT scan reveals a hyperdense mass that enhances uniformly (arrow) in the suprasellar region.



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FIG 3. Images of Patient 5, a 35-year-old woman with chordoid glioma.

A, Axial T1-weighted spin-echo MR image (450/20) reveals an isointense hypothalamic/third ventricular mass (arrows).

B, Axial fluid-attenuated inversion recovery MR image (8912/142; inversion time, 2200 ms) reveals an iso- to slightly hyperintense hypothalamic/third ventricular mass (large arrow). Note vasogenic edema within the basal ganglia (small arrows), posterior limbs of the internal capsules, and lateral geniculate ganglia of the thalami (arrowheads).

C, Axial fluid-attenuated inversion recovery MR image (8912/142; inversion time, 2200 ms) obtained at a level approximately 1.5 cm inferior to that shown in B. Note splaying of the cerebral peduncles (large arrows) and bilateral involvement of the optic tracts (small arrows).



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FIG 4. Image of Patient 1, a 59-year-old man with chordoid glioma. Contrast-enhanced coronal T1-weighted spin-echo MR image (500/15) reveals a densely enhancing hypothalamic/third ventricular mass with a central nonenhancing component representing a small cyst or necrosis (arrow)



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FIG 5. Photomicrographs of chordoid glioma (Patient 5).

A, Hematoxylin and eosin stain of chordoid glioma reveals cords and clusters of eosinophilic epithelioid tumor cells (large arrows) dispersed within a slightly bluish mucinous matrix. A lymphoplasmacytic infiltrate is consistently present (small arrows).

B, Glial fibrillary acidic protein stain of chordoid glioma tumor cells shows strong, diffuse, cytoplasmic immunoreactivity.