Chordomas: Nasopharyngeal Presentation and Atypical Long Survival

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Abstract
Atypical clinical and pathological features of 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 attention 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.

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