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Chordoma

A chordoma is a rare type of cancerous bone tumor that can occur at any part of the spine or in the skull

Prevalence

Unknown

US Estimated

Unknown

Europe Estimated

Age of Onset

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ICD-10

C76.7

Inheritance

Autosomal dominant

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Autosomal recessive

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Mitochondrial/Multigenic

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X-linked dominant

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X-linked recessive

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Rare View

A chordoma is a rare type of cancerous tumor that can occur anywhere along the spine, from the base of the skull to the tailbone. Chordomas grow slowly, gradually extending into the bone and soft tissue around them. The tumors that arise from embryonic notochordal remnants along the length of the neuraxis at developmentally active sites. The median survival in the United States is about 7 years, with overall survival rates of ~68% at 5 years and ~40% at 10 years. Tumor size and disease stage can influence conditional survival for patients with chordoma.

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5 Facts you should know

FACT

1

A rare slow-growing neoplasm thought to arise from cellular remnants of the notochord

FACT

2

Chordomas typically present in adults between the ages of 40 and 70 and can occur anywhere along the spine

FACT

3

About half of all chordomas occur in the sacrum; about one third occur in the clivus

FACT

4

A chordoma that occurs at the base of the spine may cause problems with bladder and bowel function

FACT

5

The annual incidence of chordoma is approximately 1 in one million (300 new patients each year)

Chordoma is also known as...

Chordoma

Spinal cancer

What’s your Rare IQ?

This famous NFL running back was diagnosed with a chordoma in 1998, and it's recurrence ultimately caused his death.

Common signs & symptoms

Abnormality of the head

Head abnormality

Autosomal dominant inheritance

Abnormality of the vertebral column

Abnormality of the spine

Current treatments

Chordomas are treated with surgery because these tumors continuously grow, although they grow slowly. If the chordoma is not removed, it may wear away the bone and adjacent soft tissue, causing destruction of surrounding tissues.[4] The surgery aims to remove as much of the tumor as possible. The extent of the surgery, or the amount of tumor that may be removed, depends on the location of the tumor and how close it is to critical structures in the brain. In some cases, surgery is followed by radiation therapy to destroy any cancer cells that may remain after surgery, especially when the tumor cannot be removed completely.[5] Several studies have shown that carbon ion therapy or  proton beam radiation may control tumor growth and improve survival.[6]

Radical resections of tumors (removal of all the tumor) with clean margins (with no remaining of the tumor) are associated with a longer period of being disease-free. If the tumor cannot be removed completely, because of the location and closeness to critical delicate structures, the addition of radiation therapy decreases the recurrence of the tumor. Frequent follow-up is needed because of the high rate of recurrence of these tumors. Tumor recurrence identified early is easier to treat. The time in between follow-up visits, including repeat MRI or CT scans, depends on the completeness of the resection. Because residual tumor shortens the recurrence time, patients with known or suspected residual tumor need to be evaluated more frequently.[4]

Top Clinical Trials

TitleDescriptionPhasesStatusInterventionsMore Information
Proton Beam Therapy for Chordoma PatientsThe goal of this clinical research study is to learn if proton beam therapy, with or without photon beam radiation therapy, is effective in the treatment of skull base chordoma. Phase 2Active, not recruitingRadiation: Proton Beam Therapy|Radiation: Photon Beam TherapyClick here for more information
Nivolumab and Relatlimab in Treating Participants With Advanced ChordomaThis phase II trial studies how well nivolumab and relatlimab work in treating participants with chordoma that has spread to other places in the body.Phase 2RecruitingBiological: Nivolumab|Biological: RelatlimabClick here for more information
BN Brachyury and Radiation in ChordomaThe goal of this study is to determine if the combination of BN-Brachyury plus radiation therapy can induce objective radiographic response rate (ORR) in patients, using a Simon 2-stage optimal design. In stage 1, a minimum of threshold of activity will be needed to proceed to stage 2.Phase 2Active, not recruitingBiological: BN-Brachyury plus radiationClick here for more information
Talimogene Laherparepvec, Nivolumab and Trabectedin for SarcomaThis is a Phase 2 study using talimogene laherparepvec, nivolumab, and trabectedin as first, second or third line therapy for advanced sarcoma, including desmoid tumor and chordoma.Phase 2RecruitingDrug: Talimogene Laherparepvec [IMLYGIC]|Drug: Nivolumab |Drug: TrabectedinClick here for more information
Study of Nivolumab and Ipilimumab in Children and Young Adults With INI1-Negative CancersThis clinical trial is studying two immunotherapy drugs (nivolumab and ipilimumab) given together as a possible treatment for INI1-negative tumors.Phase 2RecruitingDrug: Nivolumab|Drug: IpilimumabClick here for more information
A Phase 1/2 Safety Study of Intratumorally Dosed INT230-6Phase 1|Phase 2RecruitingDrug: INT230-6|Biological: anti-PD-1 antibody|Biological: anti-CTLA-4 antibodyClick here for more information
Nivolumab and Ipilimumab in Treating Patients With Rare TumorsThis study evaluates the intratumoral administration of escalating doses of a novel, experimental drug, INT230-6. Sponsor also plans to test INT230-6 in combination with anti-PD-1 and anti-CTLA-4 antibodies.Phase 2RecruitingProcedure: Biospecimen Collection|Biological: Ipilimumab|Biological: NivolumabClick here for more information
A Phase II, Multicenter Study of the EZH2 Inhibitor Tazemetostat in Adult Subjects With INI1-Negative Tumors or Relapsed/Refractory Synovial SarcomaThis is a Phase II, multicenter, open-label, single arm, 2-stage study of tazemetostat 800 mg BID (twice daily) and 1600 mg QD (once daily). Phase 2RecruitingDrug: TazemetostatClick here for more information
Intravenous TAEK-VAC-HerBy Vaccine Alone and in Combination Treatment in HER2 Cancer PatientsA Phase 1/2 open label trial of intravenous administration of TAEK-VAC-HerBy vaccine in patients with advanced HER2- expressing cancer.Phase 1|Phase 2RecruitingBiological: TAEK-VAC-HerByClick here for more information

Top Treatments in Research

AgentClass/Mechanism of ActionDevelopment StatusCompanyClinical StudiesMore Information
Proton Beam Therapy|Radiation: Photon Beam TherapyPhoton Beam TherapyPhase 2MD Anderson Cancer CenterClick here for more informationClick here for more information
Biological: Nivolumab|Biological: RelatlimabNivolumab is a human recombinant monoclonal immunoglobulin G4 antibody to the programmed cell death receptor-1 (PD-1) which has distinctive immunomodulatory activity and is used in cancer immunotherapy. Relatlimab (RELA), a human IgG4 LAG-3-blocking antibody, restores effector function of exhausted T cellsPhase 2Sponsor:
Jonsson Comprehensive Cancer Center
Collaborator:
Bristol-Myers Squibb
Click here for more informationClick here for more information
Biological: BN-Brachyury plus radiationBrachyury is highly expressed in all cells in nearly every chordoma tumor. Knock down of brachyury in chordoma cell lines induces growth arrest and apoptosis. BN-Brachyury utilizes a prime-boost vaccination regimen that has been optimized to include the gene for brachyury and other molecules known to increase immune activation.Phase 2Bavarian NordicClick here for more informationClick here for more information
Drug: Talimogene Laherparepvec (IMLYGIC) |Drug: Nivolumab|Drug: TrabectedinTalimogene laherparepvec (TVEC) is a type I herpes simplex virus genetically modified to preferentially replicate in tumor cells, enhance antigen loading of MHC class I molecules and express granulocyte-macrophage colony-stimulating factor to increase tumor-antigen presentation by dendritic cells. Nivolumab is a human recombinant monoclonal immunoglobulin G4 antibody to the programmed cell death receptor-1 (PD-1) which has distinctive immunomodulatory activity and is used in cancer immunotherapy. Trabectedin is classified as an alkylating agent.Phase 2Sarcoma Oncology Research Center, LLCClick here for more informationClick here for more information
Nivolumab|Drug: IpilimumabIpilimumab is a monoclonal antibody medication that works to activate the immune system by targeting CTLA-4, a protein receptor that downregulates the immune system.Phase 2Dana-Farber Cancer InstituteClick here for more informationClick here for more information
Drug: INT230-6|Biological: anti-PD-1 antibody|Biological: anti-CTLA-4 antibodyINT230-6 is a formulation consisting of a proprietary amphiphilic cell penetration enhancer molecule, 8-((2-hydroxybenzoyl)amino)octanoate, also referred to as SHAO, combined with cisplatin and vinblastine.Phase 2Intensity Therapeutics, Inc.Click here for more informationClick here for more information
Biological: Ipilimumab|Biological: NivolumabNivolumab is a human recombinant monoclonal immunoglobulin G4 antibody to the programmed cell death receptor-1 (PD-1) which has distinctive immunomodulatory activity and is used in cancer immunotherapy. Ipilimumab is a monoclonal antibody medication that works to activate the immune system by targeting CTLA-4, a protein receptor that downregulates the immune system.Phase 2National Cancer Institute (NCI)Click here for more informationClick here for more information
TazemetostatTazemetostat is a first-in-class, small molecule enhancer of zeste homolog 2 (EZH2) inhibitor.Phase 2Epizyme, Inc.Click here for more informationClick here for more information
Biological: TAEK-VAC-HerByTAEK-VAC-HerBy vaccine may induce a cytotoxic T-lymphocyte (CTL)-mediated immune response against tumor cells expressing the HER-2/neu antigen, which may result in the inhibition of proliferation in Her-2/neu-expressing tumor cells.Phase 2Bavarian NordicClick here for more informationClick here for more information

Medline Plus, National Library of Medicine