Strike a Chord (abstract)

Optic pathway gliomas are traditionally observed until clinical signs indicate visual compromise. The patient's general and neuro-ophthalmic examinations did not support surgical intervention for this suspected optic pathway glioma. Upon insistence of the patient, she was taken to the operating room, and an excisional biopsy was performed using a right fronto-temporal craniotomy approach. Surgical biopsy specimen showed a chordoid glioma of the third ventricle, WHO Grade II. Post-operative visual examination was stable, with no visual field deficits. Chordoid glioma is a term used to describe a low-grade neoplasm that arises from the anterior third ventricle in the central nervous system. It was first described in 1998 (1). There are fewer than 100 pathology-confirmed cases of chordoid glioma published in the literature. Clinically, the mean age of diagnosis is 48 years of age, with the most common symptoms being headache, visual symptoms, mental status changes and memory deficits (2). A small number present with obstructive hydrocephalus, and about 10% have endocrine dysfunction (2). Although radiological characteristics of the tumor are variable, computed tomography imaging most often shows a hyperdense homogenously enhancing lesion (2). MRI shows a well-circumscribed ovoid mass, iso-intense on T1-weighted sequences, with uniform and intense enhancement (2, 3). Pathologically, the origin of chordoid glioma is controversial, but histopathologic evidence suggests that the tumor originates from the area of lamina terminalis (4). Histopathology shows cords and clusters of epithelioid cells with eosinophilic cytoplasm and uniform nuclei. The underlying background is basophilic and myxoid in nature, with lymphoplasmacytic infiltrates. The tumor traditionally does not infiltrate surrounding tissues (4).