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.

http://www.chordomafoundation.org/

Last Updated: 5 Aug 2013

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Chordoma Support Group

Chordoma Support Group is an international, online, support group for all those affected by chordoma to offer each other friendship, support and information.

http://www.chordomasupport.org

Last Updated: 31 May 2011

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General Support Organizations

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How do you compare to others with this condition?

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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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Chordoma Support Group

Chordoma Support Group is an international, online, support group for all those affected by chordoma to offer each other friendship, support and information.

http://www.chordomasupport.org

Last Updated: 31 May 2011

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General Support Organizations

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

Chemotherapy of skull base chordoma tailored on responsiveness of patient-derived tumor cells to rapamycin.
 

Author(s): Lucia Ricci-Vitiani, Daniele Runci, Quintino Giorgio D'Alessandris, Tonia Cenci, Maurizio Martini, Federico Bianchi, Giulio Maira, Louis Stancato, Ruggero De Maria, Luigi Maria Larocca, Roberto Pallini

Journal: Neoplasia. 2013 Jul;15(7):773-82.

 

Skull base chordomas are challenging tumors due to their deep surgical location and resistance to conventional radiotherapy. Chemotherapy plays a marginal role in the treatment of chordoma resulting from lack of preclinical models due to the difficulty in establishing tumor cell lines ...

Last Updated: 1 Jul 2013

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Chordoma characterization of significant changes of the DNA methylation pattern.
 

Author(s): Beate Rinner, Andreas Weinhaeusel, Birgit Lohberger, Elke Verena Froehlich, Walter Pulverer, Carina Fischer, Katharina Meditz, Susanne Scheipl, Slave Trajanoski, Christian Guelly, Andreas Leithner, Bernadette Liegl

Journal: PLoS ONE. 2013 ;8(3):e56609.

 

Chordomas are rare mesenchymal tumors occurring exclusively in the midline from clivus to sacrum. Early tumor detection is extremely important as these tumors are resistant to chemotherapy and irradiation. Despite continuous research efforts surgical excision remains the main treatment ...

Last Updated: 27 Mar 2013

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High-resolution whole-genome analysis of skull base chordomas implicates FHIT loss in chordoma pathogenesis.
 

Author(s): Roberto Jose Diaz, Mustafa Guduk, Rocco Romagnuolo, Christian A Smith, Paul Northcott, David Shih, Fitim Berisha, Adrienne Flanagan, David G Munoz, Michael D Cusimano, M Necmettin Pamir, James T Rutka

Journal: Neoplasia. 2012 Sep;14(9):788-98.

 

Chordoma is a rare tumor arising in the sacrum, clivus, or vertebrae. It is often not completely resectable and shows a high incidence of recurrence and progression with shortened patient survival and impaired quality of life. Chemotherapeutic options are limited to investigational ...

Last Updated: 28 Sep 2012

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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 11 free, full-text review articles on human participants. First 3 results:

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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Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review.
 

Author(s): Simon G Launay, Bruno Chetaille, Fanny Medina, Delphine Perrot, Serge Nazarian, Jérôme Guiramand, Laurence Moureau-Zabotto, François Bertucci

Journal:

 

Chordomas are very rare low-grade malignant bone tumors that arise from the embryonic rests of the notochord. They are characterized by slow growth and long history with frequent local relapses, and sometimes metastases. While chemotherapy is not efficient, imatinib has shown antitumor activity.

Last Updated: 24 Oct 2011

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Total resection of inferiorly located sacral chordoma with posterior only approach: case report and review of the literature.
 

Author(s): Hakan Sabuncuoglu, Selcuk Ozdogan, Handan Dogan, Omur Ataoglu, Erdener Tımurkaynak

Journal: Turk Neurosurg. 2010 Oct;20(4):527-32.

 

Chordoma is a primary sacral neoplasm of ectodermal origin and makes up %1- 4 of all primary bone tumors. It is usually present on the midline cerebrospinal axis and the most common locations are the spheno-clival region and the sacrum. The treatment of primary sacral tumors represents ...

Last Updated: 21 Oct 2010

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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

Last Updated: 15 Apr 2014

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Nilotinib With Radiation for High Risk Chordoma
 

Status: Recruiting

Condition Summary: Chordoma

 

Last Updated: 26 Mar 2014

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High Dose Intensity Modulated Proton Radiation Treatment +/- Surgical Resection of Sarcomas of the Spine, Sacrum and Base of Skull
 

Status: Recruiting

Condition Summary: Chordoma of Spine; Chordoma of Sacrum; Chordoma of Base of Skull; Chondrosarcoma of the Spine; Chondrosarcoma of the Sacrum

 

Last Updated: 8 Apr 2014

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