- Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University, Montreal, Quebec, Canada
Department of Neurology and Neurosurgery, Montreal Neurological Institute and Hospital, McGill University, Montreal, Quebec, Canada
DOI:10.4103/2152-7806.80118Copyright: y© 2011 Al Hinai QS. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
How to cite this article: Al Hinai QS, Petrecca K. Rarest of the rare: Chordoid glioma infiltrating the optic chiasm. Surg Neurol Int 28-Apr-2011;2:53
How to cite this URL: Al Hinai QS, Petrecca K. Rarest of the rare: Chordoid glioma infiltrating the optic chiasm. Surg Neurol Int 28-Apr-2011;2:53. Available from: http://sni.wpengine.com/surgicalint_articles/rarest-of-the-rare-chordoid-glioma-infiltrating-the-optic-chiasm/
Background:Chordoid glioma is a rare brain tumor typically located within the anterior third ventricle. It is a well-circumscribed, non-infiltrative tumor that grows as a mass within the ventricle.
Case Description:We present the case of a 50-year-old woman with a chordoid glioma located in the anterior third ventricle. Unusually, MRI revealed an enlarged optic chiasm. Histological sampling of the chiasm revealed tumor invasion.
Conclusion:Involvement of the optic apparatus is generally thought to be an imaging feature that can be used to distinguish chordoid gliomas from optic/hypothalamic gliomas. This case provides the first reported exception to this dogma.
Keywords: Chordoid glioma, optic apparatus invasion, third ventricle tumor
Chordoid glioma is a rare tumor of the anterior third ventricle.[
A 50-year-old woman presented with short-term memory loss and occasional headaches. A CT scan revealed a large suprasellar mass that was isodense and uniformly enhancing. Small foci of calcification were evident. MRI demonstrated that the tumor was located in the anterior third ventricle exerting rostral pressure on the fornices. Suprachiasmatic and retrochiasmatic components were also evident [
Sagittal (a) and axial (b) gadolinium enhanced images. (c) T2-weighted axial image. (d) Coronal gadolinium enhanced image. (e) T2-weighted axial image showing involvement of the optic chiasm. (f) High magnification image from (e) revealing hyperintense signal in the optic chiasm (OC). (g) High magnification image from (d) revealing enhancement within the right optic chiasm (Rt OC)
A right pterional craniotomy and orbital osteotomy was performed. Upon dissection of the optic apparatus it was apparent that the optic chiasm was involved in the tumoral process. It was grossly enlarged with an infrachiasmatic growth caudal to the right optic nerve [
Pathological examination revealed cords of epithelioid cells separated by a mucinous background. Cells were positive for glial fibrillary acidic protein. Epidermal growth factor receptor positive cells were also identified. A single mitotic cell was found. A CD45-positive lymphocytic infiltrate was also seen. These findings are consistent with a chordoid glioma.
The patient and family were informed regarding the diagnosis, prognosis, risks of resection, and radiotherapy. The patient deferred further treatment until evidence of growth on imaging or worsening symptoms. The tumor has remained unchanged over 18 months and the patient remains unchanged neurologically.
Chordoid glioma is a rare tumor of the anterior third ventricle. It is only recently characterized and not typically included in the differential diagnosis of suprachiasmatic and third ventricular tumors. To date, 65 cases have been reported.[
Lesions commonly seen in this location include craniopharyngiomas, optic/hypothalamic gliomas, germ cell tumors, meningiomas, Rathke's cleft cysts, hamartomas, and pituitary macroadenomas. Imaging features that can help to distinguish chordoid gliomas from other lesions in this location include displacement of the infundibulum and involvement of the optic apparatus. Chordoid gliomas displace the infundibulum posteriorly. In contrast, Rathke's cleft cysts and tuber cinereum hamartomas displace the infundibulum anteriorly.[
Surgical reports have consistently confirmed the lack of optic apparatus involvement observed on MR imaging. Ricoy et al.[
Of the 65 reported cases of chordoid glioma, none have been shown to involve the optic chiasm. This is the first demonstration of a chordoid glioma that involves the optic chiasm on imaging with intraoperative confirmation. We thus propose that the presence of optic chiasm involvement should not be used as a feature to exclude chordoid glioma from the differential diagnosis of suprasellar/anterior third ventricular tumors.
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