
pmid: 19294331
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.A retrospective chart review was completed of the three cases of primary familial oronasopharyngeal chordoma treated at the University of Utah.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.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.
Adult, Notochord, Nasopharyngeal Neoplasms, Magnetic Resonance Imaging, Skull Base Neoplasms, Neurosurgical Procedures, Oropharyngeal Neoplasms, Treatment Outcome, Cranial Fossa, Posterior, Child, Preschool, Nasopharynx, Chordoma, Humans, Female, Child, Tomography, X-Ray Computed, Retrospective Studies
Adult, Notochord, Nasopharyngeal Neoplasms, Magnetic Resonance Imaging, Skull Base Neoplasms, Neurosurgical Procedures, Oropharyngeal Neoplasms, Treatment Outcome, Cranial Fossa, Posterior, Child, Preschool, Nasopharynx, Chordoma, Humans, Female, Child, Tomography, X-Ray Computed, Retrospective Studies
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