Chordoid glioma of the third ventricle

Chordoid glioma is a rare tumour (World Health Organisation grade II) originating from the third ventricle with both glial and chordoid features. It was first described by Brat in 1998. Since there is no detailed information available on the outcome after surgery and adjuvant treatment, we reviewed the literature.

A literature search through PUBMED revealed 50 cases of chordoid glioma. Most reports were found in pathology journals. Information on the postoperative course was sometimes very limited. We reviewed the available literature and studied in detail the presenting symptoms, mortality and postoperative complications in relation to the extent of resective surgery, as well as the importance of adjuvant treatment.

Mortality in the immediate postoperative period is 32% and is higher after gross total resection as compared to subtotal resection. Non-fatal postoperative complications are hypothalamic disorders and mental alterations. Gross total resection is the treatment of choice since no recurrence has been reported after macroscopically complete resection, but this is often difficult because of the location and adherence to the hypothalamus. The role of postoperative radiotherapy is uncertain. There is some indication that radiosurgery with or without conventional irradiation is superior to conventional radiation alone. Planned subtotal resection followed by stereotactic radiosurgery can be a safe and effective alternative in a patient in whom gross total resection is considered to be too risky. There is no report on the use of chemotherapy in the treatment of chordoid gliomas. More information about the optimal treatment strategy is needed, and more reports are also needed.