title: Oronasopharyngeal chordomas.
abstract: BACKGROUND
  Chordomas are rare tumors derived from notochordal remnants. The authors report on a series of three cases of primary familial oronasopharyngeal chordomas treated at our institution.
  
  
  METHODS
  A retrospective chart review was completed of the three cases of primary familial oronasopharyngeal chordoma treated at the University of Utah.
  
  
  RESULTS
  All three patients (100%) were neurologically intact and presented with nasal obstruction. The patients ranged in age from 5 to 65 years and were first-degree relatives. None of the patients had bony erosion of the skull base on imaging, and all of the patients' tumors connected with the skull base via a tract. All three patients were treated with a wide excision combined with drilling of the involved skull base. They all tolerated the procedure without any complications and remain tumor free with a follow-up of 12 months to 4.5 years.
  
  
  CONCLUSIONS
  Primary oronasopharyngeal chordomas are rare tumors that may present without bony erosion of the skull base. A wide excision with drilling of the involved bony structures may offer an oncologic cure.
authors.affiliation: Department of Neurosurgery, University of Utah, Salt Lake City, UT 84132, USA. jeroen.coppens@hsc.utah.edu
authors.forename: Jeroen R
authors.initials: JR
authors.lastname: Coppens
authors.forename: H
authors.initials: H
authors.lastname: Ric Harnsberger
authors.forename: Michael A
authors.initials: MA
authors.lastname: Finn
authors.forename: Pramod
authors.initials: P
authors.lastname: Sharma
authors.forename: William T
authors.initials: WT
authors.lastname: Couldwell
mesh_terms: D000328:Adult; D002648:Child; D002675:Child, Preschool; D002817:Chordoma / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D003388:Cranial Fossa, Posterior / Q000000981:diagnostic imaging / Q000196:embryology / Q000473:pathology / Q000601:surgery; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D009303:Nasopharyngeal Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D009305:Nasopharynx / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D019635:Neurosurgical Procedures; D009672:Notochord / Q000196:embryology; D009959:Oropharyngeal Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D012189:Retrospective Studies; D019292:Skull Base Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology* / Q000601:surgery; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome