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

Extended ultrastructural characterization of chordoma cells: the link to new therapeutic options.
 

Author(s): Dagmar Kolb, Elisabeth Pritz, Bibiane Steinecker-Frohnwieser, Birgit Lohberger, Alexander Deutsch, Thomas Kroneis, Amin El-Heliebi, Gottfried Dohr, Katharina Meditz, Karin Wagner, Harald Koefeler, Gerd Leitinger, Andreas Leithner, Bernadette Liegl-Atzwanger, Dagmar Zweytick, Beate Rinner

Journal:

 

Chordomas are rare bone tumors, developed from the notochord and largely resistant to chemotherapy. A special feature of this tumor is the heterogeneity of its cells. By combining high pressure freezing (HPF) with electron tomography we were able to illustrate the connections within ...

Last Updated: 6 Dec 2014

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Identification of miR-140-3p as a marker associated with poor prognosis in spinal chordoma.
 

Author(s): Ming-Xiang Zou, Wei Huang, Xiao-Bin Wang, Guo-Hua Lv, Jing Li, You-Wen Deng

Journal:

 

To investigate the expression profile of miR-140-3p in formalin-fixed paraffin-embedded (FFPE) tissues of spinal chordoma, and its correlation with the prognosis of spinal chordoma patients.

Last Updated: 8 Sep 2014

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Natural history and surgical treatment of chordoma: a retrospective cohort study.
 

Author(s): Samuel Aguiar Júnior, Wesley Pereira Andrade, Glauco Baiocchi, Gustavo Cardoso Guimarães, Isabela Werneck Cunha, Daniel Alvarez Estrada, Sergio Hideki Suzuki, Luiz Paulo Kowalski, Ademar Lopes

Journal: Sao Paulo Med J. 2014 ;132(5):297-302.

 

Chordoma is a rare tumor with a high risk of locoregional recurrences. The aim of this study was analyze the long-term results from treating this pathological condition.

Last Updated: 4 Sep 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 12 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 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: 24 Jul 2015

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Nilotinib With Radiation for High Risk Chordoma
 

Status: Recruiting

Condition Summary: Chordoma

 

Last Updated: 5 May 2015

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