Nasal and paranasal presentations of chordomas.

Eight patients with chordomas whose initial manifestations were caused by nasal and paranasal extension of the tumor are presented. Their ages ranged from 24 to 51 years. Disturbances of vision developed subsequently in all eight patients, and headache was present in each patient. Roentgenograms showed bony erosion of the sphenoid sinus or a nasopharyngeal mass. Because chordomas can be confused with chondrosarcomas histopathologically, differentiation is important. Treatment consists of surgical debulking or radiotherapy (or both). Despite such treatment, six patients died from their disease. The average survival was five years. This emphasizes the well-known poor prognosis for patients with clival chordomas.