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Oncology and Therapy
Published in: Oncology and Therapy 1/2016

Open Access 01-06-2016 | Review

Chordoma: The Quest for Better Treatment Options

Author: Christopher R. Heery

Published in: Oncology and Therapy | Issue 1/2016

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Abstract

Chordoma is an extremely rare cancer, with an incidence of about one case per million persons per year in the USA and Europe (about 300 and 450 cases per year, respectively). The estimated median overall survival of patients with chordoma is approximately 6–7 years, yielding a rough estimate of chordoma prevalence at about 2000 in the USA and 3000 in Europe. Primary tumor develops along the axial spine between the clivus and sacrum and develops from the residual embryonic notochord. Brachyury (T), a transcription factor required for normal embryonic development, is expressed in the notochord and overexpressed in almost all cases of chordoma. The primary treatment for chordoma is surgical excision with wide local margins, when possible. Radiotherapy also plays a significant role in the adjuvant setting and when surgery is not possible. Unfortunately, in the advanced and/or metastatic setting, where the role of surgery and/or radiation is less clear, treatment options are very limited. To date, there have been no randomized, controlled trials in chordoma that have resulted in defined agents of clinical benefit for systemic treatment. This review briefly describes the natural history and initial treatment of chordoma and focuses on treatment options for advanced disease and potential avenues of research that may lead to improved treatment options in the future.
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Metadata
Title
Chordoma: The Quest for Better Treatment Options
Author
Christopher R. Heery
Publication date
01-06-2016
Publisher
Springer Healthcare
Published in
Oncology and Therapy / Issue 1/2016
Print ISSN: 2366-1070
Electronic ISSN: 2366-1089
DOI
https://doi.org/10.1007/s40487-016-0016-0

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