Chordoid glioma: clinicopathologic profile and differential diagnosis of an uncommon tumor

Abstract

Chordoid glioma is an uncommon low-grade brain neoplasm arising in the third ventricular region, predominantly in middle-aged women. It characteristically shows chordoma-like histologic features and glial fibrillary acidic protein immunoreactivity. We present a case of chordoid glioma in a previously healthy 56-year-old woman admitted to our hospital because of a cranial trauma subsequent to an incidental fall. Radiologic examinations revealed a well-demarcated, partially cystic, enhancing mass at the level of the lamina terminalis. The lesion was surgically removed. The patient remained alive and well 8 months after the surgery. Histologically, the tumor consisted of clusters and cords of epithelioid cells embedded in a mucinous matrix. Lymphoplasmacytic infiltrates and Russell bodies were prominent. Immunohistochemically, the tumor cells were positive for glial fibrillary acidic protein, neurofilaments, and neuron-specific enolase, suggesting a divergent neuronal and glial differentiation. The Ki-67 index was low. The clinicopathologic profile and the differential diagnosis of this tumor are discussed.