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Acta Neurochirurgica

Erschienen in:

01.08.2009 | Clinical Article

Oronasopharyngeal chordomas

verfasst von: Jeroen R. Coppens, H. Ric Harnsberger, Michael A. Finn, Pramod Sharma, William T. Couldwell

Erschienen in: Acta Neurochirurgica | Ausgabe 8/2009

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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.

Findings

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.

Conclusion

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.

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Titel: Oronasopharyngeal chordomas
verfasst von: Jeroen R. Coppens
H. Ric Harnsberger
Michael A. Finn
Pramod Sharma
William T. Couldwell
Publikationsdatum: 01.08.2009
Verlag: Springer Vienna
Erschienen in: Acta Neurochirurgica / Ausgabe 8/2009
Print ISSN: 0001-6268
Elektronische ISSN: 0942-0940
DOI: https://doi.org/10.1007/s00701-009-0253-1

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