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 209 free, full-text research articles on human participants. First 3 results:

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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C-reactive protein as a prognostic factor in patients with chordoma of lumbar spine and sacrum--a single center pilot study.
 

Author(s): Gerhard Martin Hobusch, Florian Bodner, Sonja Walzer, Rodrig Marculescu, Philipp T Funovics, Irene Sulzbacher, Reinhard Windhager, Joannis Panotopoulos

Journal:

 

This is a retrospective, diagnostic study, level IV.

Last Updated: 19 Apr 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 16 free, full-text review articles on human participants. First 3 results:

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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Brachyury: a diagnostic marker for the differential diagnosis of chordoma and hemangioblastoma versus neoplastic histological mimickers.
 

Author(s): Valeria Barresi, Antonio Ieni, Giovanni Branca, Giovanni Tuccari

Journal: Dis. Markers. 2014 ;2014():514753.

 

Brachyury is a transcription factor which is required for posterior mesoderm formation and differentiation as well as for notochord development during embryogenesis. Due to its expression in the neoplastic cells of chordoma, a malignant tumour deriving from notochordal remnants, but ...

Last Updated: 4 Mar 2014

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From notochord formation to hereditary chordoma: the many roles of Brachyury.
 

Author(s): Yutaka Nibu, Diana S José-Edwards, Anna Di Gregorio

Journal: Biomed Res Int. 2013 ;2013():826435.

 

Chordoma is a rare, but often malignant, bone cancer that preferentially affects the axial skeleton and the skull base. These tumors are both sporadic and hereditary and appear to occur more frequently after the fourth decade of life; however, modern technologies have increased the ...

Last Updated: 10 May 2013

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

 
 
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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: 5 Dec 2015

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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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