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Oronasopharyngeal chordomas

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An Erratum to this article was published on 26 May 2009

An Erratum to this article was published on 26 May 2009

An Erratum to this article was published on 26 May 2009

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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Acknowledgment

The authors thank Kristin Kraus, M.Sc., for her editorial assistance in preparing this paper.

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Authors and Affiliations

  1. Department of Neurosurgery, University of Utah, Salt Lake City, UT, 84132, USA

    Jeroen R. Coppens, Michael A. Finn & William T. Couldwell

  2. Department of Radiology, University of Utah, Salt Lake City, UT, 84132, USA

    H. Ric Harnsberger

  3. Ear, Nose, and Throat Center, Salt Lake City, UT, 84102-1307, USA

    Pramod Sharma

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  1. Jeroen R. Coppens
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  2. H. Ric Harnsberger
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  3. Michael A. Finn
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Correspondence to William T. Couldwell.

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An erratum to this article can be found at http://dx.doi.org/10.1007/s00701-009-0303-8

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Coppens, J.R., Ric Harnsberger, H., Finn, M.A. et al. Oronasopharyngeal chordomas. Acta Neurochir 151, 901–907 (2009). https://doi.org/10.1007/s00701-009-0253-1

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