CHORDOID GLIOMA OF THE THIRD VENTRICLE

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Contents

Definition

Chordoid glioma of the 3rd ventricle is a ventricular tumor of adulthood characterized by clusters and cords of epithelioid, GFAP-expressing tumor cells within a variably mucinous stroma typically containing a lymphoplasmacytic infiltrate. Chordoid glioma of the 3rd ventricle corresponds to WHO Grade II glioma.

Epidemiology

Chordoid glioma of the 3rd ventricle is a rare tumor, but should be considered within the differential diagnosis of a contrast-enhancing third-ventricular mass.

Localization

Chordoid gliomas of the 3rd ventricle arise in the anterior portion of the 3rd ventricle. Their site of origin has not been well defined.

Clinical presentation

Patients most commonly present with signs and symptoms of obstructive hydrocephalus. Evidence of hypothalamic disturbance, compression of the visual system, or psychiatric or memory disturbance may also occur.

Imaging

On MRI, chordoid gliomas of the 3rd ventricle appear as uniformly contrast-enhancing, well-circumscribed lesions of the 3rd ventricle. These lesions may appear to be contiguous with the hypothalamus or supresellar structures.


Pathology

Macroscopy

These lesions present as firm nodules of variable size bulging into the ventricular lumen.

Histopathology

Chordoid gliomas of the 3rd ventricle are solid neoplasms composed of clusters and cords of epithelioid, GFAP-expressing tumor cells within a variably mucinous stroma typically containing a lymphoplasmacytic infiltrate. Mitoses are absent or rare.

Molecular genetics

No genetic abnormality underlying the development of these lesions has been delineated.

Prognosis

Because of their location and intimate association with the roof of the hypothalamus, these lesions often evade complete resection. Treatment often involves surgical biopsy and debulking, placement of a ventriculo-peritoneal shunt for CSF diversion (often with septostomy to allay the need for bilateral shunts), and adjuvant chemotherapy or radiation.

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