Clivus Chordoma

Chordomas are rare lesions comprising 0.1–0.2% of primary intracranial tumors. Most authors still differ as to whether these tumors are histologically benign or malignant. However, they are characterized by local aggressiveness and tendency to recur.

We are presenting our experience in 32 cases of clival chordomas, treated surgically. There were 22 females with age ranging from 19–57 years ( mean, 35 years ) and 10 males ranging in age from 18–61 years (average, 41 years). Mean presentation features were increased intracranial pressure and diplopia, and the abducent nerve was the most common affected nerve.

We achieved gross total resection in 20 patients and subtotal resection in 10 patients. We used transnasal approach in 6 cases, maxillotomy in 8 cases, transbasal subfrontal in 3 cases, transpetrosal in 3 cases, and retrosigmoid approach in 12 cases. Perioperative mortality was encountered in one patient who developed respiratory arrest 12 hours after surgery. Morbidity was encountered in four patients who experienced increased neurological deficits and in one patient with CSF leak.

Following surgery, 24 patients received fractionated radiotherapy and 8 patients received Gamma radiosurgery.

Follow-up period ranged from 19 months to 120 months (mean of 67.4 months). Eight cases recurred after surgery and radiotherapy; four of these died 2–5 years after second surgery. Two patients refused further surgery. One case recurred after Gamma radiosurgery requiring a second operation . The overall result of the 32 patients was: 20 patients are still alive and well, 10 are in fair condition, 4 are in poor condition, and 6 died in the long follow-up period.

We believe that radical surgery has an important role to play in almost all cases. Postoperatively, the residual tumor should receive complementary treatment by external beam radiation, radiosurgery, or proton beam.