Chordoid glioma: A neoplasm unique to the hypothalamus and anterior third ventricle

BACKGROUND AND PURPOSE: Chordoid glioma is a new clinicopathologic entity t hat occurs in the region of the hypothalamus/anterior third ventricle, The aims of this study were to describe the characteristic radiographic feature s of chordoid glioma, identify specific imaging features that may enable di fferentiation of chordoid glioma from other suprasellar tumors, and increas e neuroradiologists' awareness of this newly described tumor, facilitating prospective diagnosis. METHODS: CT scans and/or MR images of six patients with chordoid glioma wer e reviewed retrospectively to determine whether any characteristic radiogra phic features would emerge. Reports of the clinical presentation, pathologi c findings, and radiographic findings of another six patients were reviewed and included, for a total patient population of 12 (mean age +/- SD, 46 +/ - 13 years). RESULTS: Imaging features were strikingly similar for all tumors. In each c ase, the mass was ovoid, was well circumscribed, was located in the region of the hypothalamus/anterior third ventricle, and enhanced uniformly and in tensely. Tumors were hyperdense to gray matter on CT scans and were isointe nse on T1-weighted MR images and slightly hyperintense on long-TR MR images . In two patients, vasogenic edema extended into the optic tracts, and in t hree, there was hydrocephalus. CONCLUSION: Chordoid glioma is a recently described unique histopathologic entity that has been added to the World Health Organization glioma classifi cation scheme and must be included in the differential diagnosis of a supra sellar mass. Distinctive imaging features are its location, ovoid shape, hy perdensity on CT scans, and uniform intense contrast enhancement.