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

Man With Recurring Chordoma and Progressive Disease Despite Radiotherapy and Radical Resection.
 

Author(s): Monica Meneses-Medina, Ana C Ceja-Bojorge, Anamari Perochena-Gonzalez, Shaddai Urbina-Ramírez, Jazmin de Anda-Gonzalez, María T Bourlon

Journal: Oncology (Williston Park, N.Y.). 2016 Feb;30(2):180-4, 186.

 

Last Updated: 19 Feb 2016

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Combined PDGFR and HDAC Inhibition Overcomes PTEN Disruption in Chordoma.
 

Author(s): Dae-Hee Lee, Ying Zhang, Amin B Kassam, Myung-Jin Park, Paul Gardner, Daniel Prevedello, Stephanie Henry, Craig Horbinski, Jan H Beumer, Hussein Tawbi, Brian J Williams, Mark E Shaffrey, Merrill J Egorin, Roger Abounader, Deric M Park

Journal:

 

The majority of chordomas show activation of the platelet-derived growth factor receptor (PDGFR). Based on in vitro intertumoral variation in response to recombinant PDGF protein and PDGFR inhibition, and variable tumor response to imatinib, we hypothesized that chordomas resistant ...

Last Updated: 7 Aug 2015

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Expression of programmed cell death ligand 1 (PD-L1) and prevalence of tumor-infiltrating lymphocytes (TILs) in chordoma.
 

Author(s): Yong Feng, Jacson Shen, Yan Gao, Yunfei Liao, Gregory Cote, Edwin Choy, Ivan Chebib, Henry Mankin, Francis Hornicek, Zhenfeng Duan

Journal: Oncotarget. 2015 May;6(13):11139-49.

 

Chordomas are primary malignant tumors of the notochord that are resistant to conventional chemotherapy. Expression of programmed cell death ligand 1 (PD-L1), prevalence of tumor-infiltrating lymphocytes (TILs), and their clinical relevance in chordoma remain unknown. We evaluated ...

Last Updated: 1 Jun 2015

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

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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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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Symptoms, Diagnosis, and Treatment

There are currently no related results available in Genetics Home Reference.

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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Last Updated: 22 Mar 2016

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