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Journal of Medical Case Reports
Published in: Journal of Medical Case Reports 1/2016

Open Access 01-12-2016 | Case report

Chordoma dedifferentiation after proton beam therapy: a case report and review of the literature

Authors: Joseph Frankl, Cassi Grotepas, Baldassare Stea, G. Michael Lemole, Alexander Chiu, Rihan Khan

Published in: Journal of Medical Case Reports | Issue 1/2016

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Abstract

Background

Chordoma is a rare invasive bone tumor that may occur anywhere along the neuraxis. A total of three primary histological varieties have been identified: conventional, chondroid, and dedifferentiated.

Case presentation

We report a case of an 8-year-old white girl who presented with conventional chordoma, was treated with surgical resection and mixed proton and photon beam therapy, and had a recurrence in the resection cavity 2.5 years later with dedifferentiated morphology. The recurrent tumor did not express brachyury, a recently identified protein specific to tissue of notochordal origin.

Conclusions

The short time period between radiation therapy and dedifferentiation, low dose of photons, and rarity of dedifferentiated skull base chordomas in pediatric patients should alert clinicians to the possibility of chordoma dedifferentiation after proton beam therapy.
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Metadata
Title
Chordoma dedifferentiation after proton beam therapy: a case report and review of the literature
Authors
Joseph Frankl
Cassi Grotepas
Baldassare Stea
G. Michael Lemole
Alexander Chiu
Rihan Khan
Publication date
01-12-2016
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2016
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-016-1076-3

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