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

Unusual location of chordoma metastasis.
 

Author(s): Marta Paulina Wiącek, Krystian Kaczmarek, Adam Sulewski, Łukasz Kubaszewski, Jacek Kaczmarczyk

Journal:

 

Chordoma is a rare tumor originating from the remnants of the notochord. The tumor is known as locally aggressive with a strong tendency for recurrence rather than metastasis.

Last Updated: 19 Jun 2014

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A rare case of chordoma and craniopharyngioma treated by an endoscopic endonasal, transtubercular transclival approach.
 

Author(s): Maurizio Iacoangeli, Alessandro Dı Rienzo, Roberto Colasanti, Marina Scarpelli, Maurizio Gladi, Lorenzo Alvaro, Niccolò Nocchi, Massimo Scerrati

Journal: Turk Neurosurg. 2014 ;24(1):86-9.

 

Co-occurrence of different brain tumors is rarely observed, being more frequent in patients affected by genetic syndromes like phacomatoses. Different histological types of intracranial lesions may present at different times in the clinical history of the patient or, more rarely, ...

Last Updated: 18 Feb 2014

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Molecular profiling of chordoma.
 

Author(s): Stefanie Scheil-Bertram, Roland Kappler, Alexandra von Baer, Erich Hartwig, Michael Sarkar, Massimo Serra, Silke Brüderlein, Bettina Westhoff, Ingo Melzner, Birgit Bassaly, Jochen Herms, Heinz-Hermann Hugo, Michael Schulte, Peter Möller

Journal: Int. J. Oncol.. 2014 Apr;44(4):1041-55.

 

The molecular basis of chordoma is still poorly understood, particularly with respect to differentially expressed genes involved in the primary origin of chordoma. In this study, therefore, we compared the transcriptional expression profile of one sacral chordoma recurrence, two chordoma ...

Last Updated: 19 Feb 2014

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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

Ion Irradiation of Sacrococcygeal Chordoma
 

Status: Recruiting

Condition Summary: Sacral Chordoma

 

Last Updated: 2 Dec 2014

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Proton Therapy for Chordomas and/or Chondrosarcomas
 

Status: Recruiting

Condition Summary: Chordomas; Chondrosarcomas

 

Last Updated: 23 Sep 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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