Primary chordoma of the maxillary sinus

A case of primary chordoma of the maxillary sinus is presented. The embryology of the notochord is reviewed and it is suggested that the frequently used adjective "ectopic" is a misnomer. The literature of primary paranasal sinus chordomas is reviewed and the clinicopathological features described. It is emphasized that these lesions can mimic other benign and malignant processes both clinically, radiographically and histologically. The treatment and prognosis is more favorable than the more common craniocervical chordoma.