ABSTRACT

Chordoid glioma likely derives from ependymal cells or tanycytes, as chordoid glioma tumor cells often demonstrate features of ependymal differentiation on electron microscopy, including basal lamina, microvilli, and intermediate filaments. Angiocentric glioma is postulated to originate from astrocytic and ependymal lineages, or radial glia or neuronal lineages. Diffuse midline glioma arises from glial cells located in the pons, thalamus, and spinal cord, as well as the third ventricle, hypothalamus, pineal region, and cerebellum. Chordoid glioma is a homogeneous tumor of uncertain histogenesis with distinct clinicopathologic features. Angiocentric glioma often presents with intractable seizures, headache, decreasing visual acuity, blank stares, episodes of stomach sensation, and speech arrest. Surgical resection represents a preferred option for treating chordoid glioma, whereas radiotherapy is reserved as adjuvant therapy for the management of residual tumor following resection. Chordoid glioma is a low-grade tumor with a poor prognosis due to its location and the difficulty in performing complete surgical resection without causing severe hypothalamic damage.