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

A giant lumbar chordoma: A case report.
 

Author(s): Yibiao Zhou, Bolin Hu, Zhiwei Wu, Hanxiong Cheng, Min Dai, Bin Zhang

Journal: Medicine (Baltimore). 2018 Jun;97(25):e11128.

 

Chordomas are malignant neoplasms derived from incomplete regression of notochordal tissue along the craniococcygeal axis.It is rare for Chordoma arising from the lumbar spine and the traditional long-term prognosis is typically poor.

Last Updated: 31 Dec 1969

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The driver landscape of sporadic chordoma.
 

Author(s): Patrick S Tarpey, Sam Behjati, Matthew D Young, Inigo Martincorena, Ludmil B Alexandrov, Sarah J Farndon, Charlotte Guzzo, Claire Hardy, Calli Latimer, Adam P Butler, Jon W Teague, Adam Shlien, P Andrew Futreal, Sohrab Shah, Ali Bashashati, Farzad Jamshidi, Torsten O Nielsen, David Huntsman, Daniel Baumhoer, Sebastian Brandner, Jay Wunder, Brendan Dickson, Patricia Cogswell, Josh Sommer, Joanna J Phillips, M Fernanda Amary, Roberto Tirabosco, Nischalan Pillay, Stephen Yip, Michael R Stratton, Adrienne M Flanagan, Peter J Campbell

Journal:

 

Chordoma is a malignant, often incurable bone tumour showing notochordal differentiation. Here, we defined the somatic driver landscape of 104 cases of sporadic chordoma. We reveal somatic duplications of the notochordal transcription factor brachyury (T) in up to 27% of cases. These ...

Last Updated: 31 Dec 1969

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Targeting the Cohesive Cluster Phenotype in Chordoma via β1 Integrin Increases Ionizing Radiation Efficacy.
 

Author(s): William L Harryman, Jaime M C Gard, Kelvin W Pond, Skyler J Simpson, Lucas H Heppner, Daniel Hernandez-Cortes, Andrew S Little, Jennifer M Eschbacher, Anne E Cress

Journal: Neoplasia. 2017 Nov;19(11):919-927.

 

Chordoma is a rare, radiation-resistant, skull-base and spinal tumor with high local recurrence containing mixed cell-adhesion phenotypes. We characterized DNA damage response (DDR) signaling (γH2AX, pKAP1, pATM) and survival response to ionizing radiation (IR) in human chordoma ...

Last Updated: 31 Dec 1969

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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:

An unusual case of oropharyngeal chordoma: A case report and literature review.
 

Author(s): Xiang Li, Yufan Wang, Feng Wang, Bowen Li, Shuai Sun, Hongyu Yang

Journal: Medicine (Baltimore). 2017 Dec;96(48):e8963.

 

Chordomas are rare malignant neoplasms derived from incomplete regression of notochordal tissue along the cranio-coccygeal axis. Chordomas that develop in an atypical position are called ectopic chordomas, such as oropharyngeal chordomas (OCs). OCs are exceedingly rare; only 11 cases ...

Last Updated: 31 Dec 1969

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Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group.
 

Author(s): S Stacchiotti, A Gronchi, P Fossati, T Akiyama, C Alapetite, M Baumann, J Y Blay, S Bolle, S Boriani, P Bruzzi, R Capanna, A Caraceni, R Casadei, V Colia, J Debus, T Delaney, A Desai, P Dileo, S Dijkstra, F Doglietto, A Flanagan, S Froelich, P A Gardner, H Gelderblom, Z L Gokaslan, R Haas, C Heery, N Hindi, P Hohenberger, F Hornicek, R Imai, L Jeys, R L Jones, B Kasper, A Kawai, M Krengli, A Leithner, I Logowska, J Martin Broto, D Mazzatenta, C Morosi, P Nicolai, O J Norum, S Patel, N Penel, P Picci, S Pilotti, S Radaelli, F Ricchini, P Rutkowski, S Scheipl, C Sen, E Tamborini, K A Thornton, B Timmermann, V Torri, P U Tunn, M Uhl, Y Yamada, D C Weber, D Vanel, P P Varga, C L A Vleggeert-Lankamp, P G Casali, J Sommer

Journal: Ann. Oncol.. 2017 Jun;28(6):1230-1242.

 

Chordomas are rare, malignant bone tumors of the skull-base and axial skeleton. Until recently, there was no consensus among experts regarding appropriate clinical management of chordoma, resulting in inconsistent care and suboptimal outcomes for many patients. To address this shortcoming, ...

Last Updated: 31 Dec 1969

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Management of Locally Recurrent Chordoma of the Mobile Spine and Sacrum: A Systematic Review.
 

Author(s): Tamir Ailon, Radmehr Torabi, Charles G Fisher, Laurence D Rhines, Michelle J Clarke, Chetan Bettegowda, Stefano Boriani, Yoshiya J Yamada, Norio Kawahara, Peter P Varga, John H Shin, Arjun Saghal, Ziya L Gokaslan

Journal: Spine. 2016 Oct;41 Suppl 20():S193-S198.

 

Systematic review.

Last Updated: 31 Dec 1969

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Clinical Trial Information This information is provided by ClinicalTrials.gov

BN Brachyury and Radiation in Chordoma
 

Status: Not yet recruiting

Condition Summary: Chordoma

 

Last Updated: 7 Aug 2018

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CDK4/6 Inhibition in Locally Advanced/Metastatic Chordoma
 

Status: Recruiting

Condition Summary: Chordoma

 

Last Updated: 19 Dec 2017

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Afatinib in Locally Advanced and Metastatic Chordoma
 

Status: Recruiting

Condition Summary: Chordoma

 

Last Updated: 6 Jul 2018

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