Background: Chordoid glioma of the third ventricle is a rare type of brain tumor that was recently categorized as a novel tumor entity. Despite low-grade histologic features, the clinical outcome in reported cases was poor.
Case description: A 61-year-old woman presented to our institution with a history of syncope. On presentation, she was alert and oriented, and her systemic examination was unremarkable. Computed tomographic scan showed a well-circumscribed, slightly hyperdense mass with calcification and a cystic component in the anterior part of the third ventricle. The mass was homogenously enhancing after the intravenous administration of contrast material, and its maximum diameter was 3.5 cm. The preoperative diagnosis was craniopharyngioma. Because the tumor seemed to invade the hypothalamus bilaterally, the operative plan was to reduce the tumor volume, followed by radiosurgery. The patient underwent partial removal of the tumor via a bifrontal basal interhemispheric approach. The histologic and immunohistochemical findings indicated CG. Surprisingly, tumor cells showed NFP expression. The residual tumor was treated by GKRS and showed no regrowth at 1-year follow-up.
Conclusions: Chordoid glioma is considered a glial neoplasm with distinct morphological and clinicopathologic features, but there may also be other unknown characteristics because of its rarity. To the best of our knowledge, this is the second reported case of CG with calcification and, at the same time, the second case with NFP expression in the English literature. Calcification and expression of NFP should not exclude CG in the differential diagnosis of a third ventricular tumor. The authors also suggest that the combination of microsurgery and GKRS is a safe and effective treatment strategy for CG.