Chordoma

Chordomas often present late with neurological or ophthalmological symptoms, but occasionally they present earlier with nasal symptoms of obstruction or epistaxis. CT scanning and MRI are important in assessing their extent. Biopsy must differentiate it from a chondrosarcoma. Large lesions often require a transfacial approach, access via a Le Fort I osteotomy, or lower lesions by a mandibular split. Some anterior le-

Fort Iii Osteotomy
Fig. 15.46 Peroperative view using computer-aided surgery in a recurrent chordoma removed because of deteriorating vision. Note its faint yellow color.
Nerves Middle Turbinate

sions based on the anterior aspect of the sphenoid can largely be removed endoscopically (Fig. 15.45 a, b), although it is relatively unusual to be able to excise them completely (Fig. 15.46). This approach normally involves a bilateral sphenoid sinusotomy type III when the sphenoidotomy is extended to the floor of the sinus and laterally to the vital structures. The carotid arteries and optic nerves can almost be skeletonized using a long-shanked drill and a well-irrigated coarse diamond burr.

Chordomas are moderately radiosensitive and radiotherapy is often done in conjunction with surgery. Cure is rare as recurrence is common, and while the long-term prognosis is poor they often grow slowly and patients can live for several years.

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