Clinical and pathological features of intracranial chordoma with orbital involvement in a child.

We examined a 44-month-old girl with proptosis of the left eye. Magnetic resonance imaging showed a dumbbell-shaped mass involving the cavernous sinus with orbital extension. Biopsy and immunohistochemistry showed the tumor to be a primitive chordoma. Intracranial chordoma should be considered in the differential diagnosis of children with orbital lesions. Immunohistochemistry is helpful in differentiating chordomas from other primitive round-cell or spindle-cell tumors.