Chordomas: nasopharyngeal presentation and atypical long survival.

Atypical clinical and pathological features of a cranio-cervical chordomas have prompted a review of these neoplasms at the University of Michigan Medical Center. Contrary to prior investigations, 92% of the spheno-occipital chordomas reported in this study clinically presented with a nasopharyngeal or intranasal mass. This high incidence indicates the requirement for an otorhinologic examination on all patients presenting with the neuro-ophthalmologic syndrome and roentgenologic findings characteristic of chordomas in this anatomical region. Special attenion is called to a histologically distinctive variant of chordoma - the "chondroid chordoma.' Apparently restricted to the cranio-cervical region, this variant has been associated with long survival.