Chordoma

Common Name(s)

Chordoma

A chordoma is a rare tumor that develops from cells of the notochord, a structure that is present in the developing embryo and is important for the development of the spine.  The notochord usually disappears before birth, though a few cells may remain embedded in the bones of the spine or at the base of the skull.   A chordoma occurs when these notochord cells start to grow into a tumor and spread into surrounding bone.  Approximately one third of chordomas occur at the base of the skull in a bone called the clivus and the most common symptoms are double vision (diplopia) and headaches.   The average age of diagnosis for chordomas of the skull base is 38 years.    Treatment often begins with surgery to remove as much of the tumor as possible, and radiation therapy may follow surgery to destroy any remaining tumor cells.  Chordomas often grow back in the original location after treatment (known as a recurrence); they do not often spread to distant parts of the body (metastasize).   Though the cause of chordomas is unknown, there is currently a study aimed at identifying a potential genetic predisposition to this disease; more information on this study can be found on the National Cancer Institute's web site.
 

Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Chordoma" for support, advocacy or research.

Chordoma Foundation

The Chordoma Foundation is a nonprofit organization working to improve the lives of chordoma patients by accelerating research to develop effective treatments for chordoma, and by helping patients to get the best care possible.

Last Updated: 5 Aug 2013

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Chordoma" for support, advocacy or research.

Chordoma Foundation

The Chordoma Foundation is a nonprofit organization working to improve the lives of chordoma patients by accelerating research to develop effective treatments for chordoma, and by helping patients to get the best care possible.

http://www.chordomafoundation.org/

Last Updated: 5 Aug 2013

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Chordoma" returned 211 free, full-text research articles on human participants. First 3 results:

Diagnostic Clues and Treatment of Intradural Cranial Chordoma.
 

Author(s): Han-Hai Zeng, Xiao-Bo Yu, Lin-Feng Fan, Gao Chen

Journal: Chin. Med. J.. 2016 12;129(23):2895-2896.

 

Last Updated: 30 Nov 2016

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Surgical spacer placement prior carbon ion radiotherapy (CIRT): an effective feasible strategy to improve the treatment for sacral chordoma.
 

Author(s): Cobianchi Lorenzo, Peloso Andrea, Vischioni Barbara, Panizza Denis, Fiore Maria Rosaria, Fossati Piero, Vitolo Viviana, Iannalfi Alberto, Ciocca Mario, Silvia Brugnatelli, Dominioni Tommaso, Dario Bugada, Maestri Marcello, Alessiani Mario, Valvo Francesca, Orecchia Roberto, Dionigi Paolo

Journal:

 

Sacral chordoma (SC) is a neoplasm arising from residual notochordal cells degeneration. SC is difficult to manage mainly because of anatomic location and tendency to extensive spread. Carbon ion radiotherapy (CIRT) is highly precise to selectively deliver high biological effective ...

Last Updated: 10 Aug 2016

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Thoracic intramedullary chordoma without bone involvement: a rare clinical entity.
 

Author(s): Mohd Faheem, Qazi Zeeshan, Balkrishna Ojha, Preeti Agrawal

Journal:

 

An 8-year-old boy presented with a 1-year history of low backache, followed by paraparesis and urinary incontinence. MRI of the thoracic spine revealed an intramedullary, intensely contrast-enhancing lesion extending from T11 to L1 vertebral level, consistent with astrocytoma, ependymoma ...

Last Updated: 29 Jul 2016

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Chordoma" returned 18 free, full-text review articles on human participants. First 3 results:

Safety and Local Control of Radiation Therapy for Chordoma of the Spine and Sacrum: A Systematic Review.
 

Author(s): Brenton Pennicooke, Ilya Laufer, Arjun Sahgal, Peter P Varga, Ziya L Gokaslan, Mark H Bilsky, Yoshiya J Yamada

Journal: Spine. 2016 Oct;41 Suppl 20():S186-S192.

 

Systematic literature review.

Last Updated: 10 Aug 2016

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

Author(s): Joseph Frankl, Cassi Grotepas, Baldassare Stea, G Michael Lemole, Alexander Chiu, Rihan Khan

Journal:

 

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.

Last Updated: 12 Oct 2016

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Spine extra-osseous chordoma mimicking neurogenic tumors: report of three cases and review of the literatures.
 

Author(s): Jian Yang, Xinghai Yang, Wujun Miao, Qi Jia, Wei Wan, Tong Meng, Zhipeng Wu, Xiaopan Cai, Dianwen Song, Jianru Xiao

Journal:

 

For a long time, chordoma has been known as an osseous tumor mainly found at the clivus and sacrococcygeal region. However, spine extra-osseous chordoma (SEC) with a better prognosis than the classic type has been neglected. According to our literature review, only several case reports ...

Last Updated: 5 Aug 2016

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Ion Irradiation of Sacrococcygeal Chordoma
 

Status: Recruiting

Condition Summary: Sacral Chordoma

 

Last Updated: 1 May 2017

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CDK4/6 Inhibition in Locally Advanced/Metastatic Chordoma
 

Status: Not yet recruiting

Condition Summary: Chordoma

 

Last Updated: 11 Apr 2017

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Last Updated: 16 Mar 2017

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