Primary chordoma of the nose

摘要

Chordoma as a rare malignant tumor arising from remnants of the notochord, accounts for 4% of malignant bone tumors. It occurs twice as frequently in men compared with women and is uncommon in individuals less than 40 years of age. Topographic distribution is spheno-occipital (35% to 44%), vertebral (15%), and sacrococcygeal (42% to 50 % ) . Unusual locations include the mandible and the maxilla, and lesions in these locations have been called ectopic or dental chordomas. Some chordomas with nasal and paranasal presentations have been reported, but primary chordoma of the nose occurs rarely. To our knowlege only two cases have been reported so far according to the literature. In this paper, we present primary chordoma of the nose, its characteristics and our experiences with it.