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

Clival chordoma: long-term clinical outcome in a single center.
 

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

Journal: Medicine (Baltimore). 2018 Sep;97(36):e12207.

 

The treatment of clival chordoma remains highly challenging. This difficulty is enhanced by the very small likelihood of a successful complete surgical resection or nonsurgical treatment of chordoma. Additionally, no effective means of interdisciplinary treatment for chordoma have ...

Last Updated: 31 Dec 1969

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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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Clival chordoma with drop metastases.
 

Author(s): Faimee Erwan Muhamat Nor, Vijayadwaja Desai, Lee Lian Chew

Journal:

 

Chordoma is a rare midline malignant tumor arising from embryonic remnants of the primitive notochord. The base of the skull is the second most common site of disease after the sacrococcygeal region. Intracranial chordoma constitutes about 30-35% of chordoma cases. Metastasis from ...

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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Chordoma of the Head and Neck: A Review.
 

Author(s): Jason K Wasserman, Denis Gravel, Bibianna Purgina

Journal: Head Neck Pathol. 2018 Jun;12(2):261-268.

 

Chordoma is a rare malignant bone tumor that can arise anywhere along the central neural axis and many involve head and neck sites, most commonly the skull base. The relative rarity of these tumors, combined with the complex anatomy of the head and neck, pose diagnostic challenges ...

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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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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