PRIMARY CRANIOFACIAL CHORDOMA - CASE-REPORT

A 37-YEAR-OLD MAN presented with right facial pain and a nonpalpable m ass over the malar eminence. An incisional biopsy via the intraoral ro ute was performed and interpreted as a vascular malformation with dege nerative changes. His symptoms persisted, and a repeat biopsy was sugg estive of an epithelioid nerve sheath tumor. Total resection of the tu mor was planned to include the infraorbital and malar regions, the inf ratemporal fossa, and the pterygopalatine fossa. At surgery, the tumor was removed with tumor-free margins obtained along the course of the maxillary nerve just before its entrance into the cavernous sinus. The pathological findings and the immunohistochemistry demonstrated a typ ical chordoma with no chondroid or sarcomatous dedifferentiation. We t hink that with greater use of immunohistochemical markers and electron microscopy, patients with chordoma in this location may be diagnosed promptly and accurately.