Orbital invasion by an intracranial chordoma

A 26-year-old woman experienced bilateral hearing loss, progressive nasal obstruction, and rhinorrhea. Examination disclosed a retropharyngeal mass. A needle biopsy specimen of the mass showed that it was a chordoma. The patient underwent surgery to remove the mass and received a postoperative course of radioactive cobalt. She did well for 18 months, at which time proptosis gradually developed in her right eye. Although the optic nerve heads and visual fields appeared normal, roentgenograms showed a large lesion involving the anterior and middle cranial fossae and destruction of the right posterior ethmoid sinus and right superior orbital fissure. Shortly after completing a course of methotrexate therapy (total dose, 89 mg), the patient experienced sudden pain and visual loss in her right eye. Surgical decompression of the orbit failed to restore light perception. The following year, her left eye became involved. A transfrontal craniotomy and extradural orbital decompression provided only temporary improvement. Four months later, left lateral rectus muscle palsy developed and her visual acuity decreased to 6/60 (20/200). Radiation therapy (400 rads per week; total dose, 3,200 rads) and treatment with methotrexate, vincristine sulfate, and prednisone did not improve her condition. At the time of her death, six years after the first symptom appeared, the patient was blind in both eyes, almost completely deaf, and suffered from severe dysphagia.