What You Need to Know About Chordoma Cancer
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rarediseases.org/rare-diseases/chordoma/Chordomas. (n.d.).
cedars-sinai.org/health-library/diseases-and-conditions/c/chordomas.htmlSurvival rates for bone cancer. (2022).
cancer.org/cancer/bone-cancer/detection-diagnosis-staging/survival-statistics.htmlTreatment for advanced or metastatic disease. (n.d.).
chordomafoundation.org/treatment/advanced-and-metastatic-disease/Understanding chordoma. (n.d.).
chordomafoundation.org/learn/understanding-chordoma/Our experts continually monitor the health and wellness space, and we update our articles when new information becomes available. Current Version Oct 14, 2022 By Corey Whelan Edited By Tim Jewell Medically Reviewed By Julie Scott, DNP, ANP-BC, AOCNP Copy Edited By Stassi Myer - CE Share this articleMedically reviewed by Julie Scott, DNP, ANP-BC, AOCNP — By Corey Whelan on October 14, 2022
What Is Chordoma Cancer
Medically reviewed by Julie Scott, DNP, ANP-BC, AOCNP — By Corey Whelan on October 14, 2022Chordoma is a rare type of cancer that affects the spine and bones of the skull. Around 300 people are diagnosed with this condition in the United States every year. Chordoma is categorized as a type of cancer called sarcoma — cancer that affects bone and soft tissue. The American Cancer Society categorizes chordoma as a type of bone cancer. Chordoma tumors commonly form in the spine in these locations:Sacrum: the triangular bone in the lower back that sits between each hip boneClivus: the bone at the base of the skull near the spineCoccyx: the bottom portion of the spine also known as the tailbone Overall survival for chordoma cancer is comparable to other types of bone cancers and, in some cases, better. The stage of the cancer and how far it’s spread (metastasis) may make a big difference when it comes to each person’s longevity and survival rates. Let’s get into the causes, symptoms, diagnosis, and treatment for chordoma cancer.What causes chordoma cancer
Chordoma tumors form from notochordal cells — remnants of the notochord, a structure in an embryo that’s replaced by the spine during the first trimester of development. The exact reason why malignancies form in notochordal cells isn’t known or understood. Around 95% of people with chordoma cancer have an SNP (snip) variation in the DNA sequence of their brachyury gene. This SNP variation doesn’t cause chordoma but increases the risk of developing it. Approximately 2 out of every 1 million people with this genetic variation get chordoma cancer. There are a few instances of families who have several members with chordoma. This rare occurrence appears to be associated with having an extra copy of the brachyury gene. Children born with the rare genetic condition tuberous sclerosis complex (TSC) are at a higher risk for developing chordoma cancer than most people without this condition. It’s thought that changes to the genes responsible for TSC may also heighten someone’s risk for chordoma cancer. More research is needed on the hereditary and genetic causes of chordoma. There aren’t any lifestyle factors that cause this condition or increase the risk for it.What are the symptoms of chordoma cancer
Chordoma symptoms vary based on the size and location of the tumor. Chordoma tumors usually grow slowly but can become large. As they grow, chordoma tumors may push on the brain or parts of the spine. You may be able to feel a large chordoma from outside your skin, especially if the tumor is in your lower spine. The range of possible symptoms may include:pain in the face, neck, head, and lower backnumbness and tinglingvision problems, including double visionparalysis of facial nervesheadachesvomitingtrouble swallowingproblems with bowel or bladder controlsexual (erectile) dysfunctionleg pain Chordoma cancer may also metastasize to other parts of the body, such as:lymph nodeslungsliverother bones When it metastasizes elsewhere in the body, other symptoms you may experience can include:widespread painfatigueloss of energyweaknessHow is it diagnosed
Because chordoma symptoms can be mistaken for symptoms of other conditions that can be either benign (noncancerous) or malignant (cancerous), you’ll need to be diagnosed through imaging tests and a biopsy. A healthcare professional will do a physical exam to check for lumps, especially along your spinal column. Imaging tests that may be used include:X-raycomputerized tomography (CT scan)magnetic resonance imaging (MRI) A biopsy uses a sample of tissue from a lump or area that’s suspected to be cancerous to make a definitive diagnosis of chordoma. There are two possible types of biopsy done for this condition:Needle aspiration: A hollow needle is inserted into the lump and used to extract tissue from the biopsy for analysis at a lab. No cuts or surgery are required for this type, and it’s often done when the lump is close to the skin’s surface.Surgical biopsy: A doctor cuts open the area that’s suspected to be cancerous and removes possibly cancerous tissue to be analyzed at a lab. This method may be used if the cancer is impossible to reach with a needle or isn’t close to the surface of the skin.Can chordoma cancer be cured
Your health, age, and other factors, such as tumor size and spread, are important considerations that will impact your treatment plan and possibly of being fully cured. Around 30%–40% of chordoma tumors eventually metastasize to other parts of the body, but this doesn’t mean that they’re not treatable. Surgical removal of chordoma tumors is usually the first step of treatment for this condition. The location of chordomas in the spine or bones of the skull base can make them challenging to remove. Several surgeries may be required along with rounds of high dose radiation before the cancer is fully treated. Chordomas can also recur in the same area of the body, requiring new rounds of treatment. Ongoing testing every few months after successful treatment is often needed to check for recurrence and spread. Chemotherapy and targeted drug therapy are also being looked at as possible treatments for this condition. New types of radiation therapy techniques that better protect the healthy tissue surrounding the tumor are also being examined.How long can you live with chordoma
If you or a loved one is diagnosed with chordoma, your first thoughts may be about the survival rate. Data about relative 5-year survival rates are gathered and maintained by the National Cancer Institute (NCI) and reported in the Surveillance, Epidemiology, and End Results (SEER) database. Knowing the 5-year relative survival rates for chordoma can be helpful, but it’s important to understand what exactly these rates mean. Keep in mind that survival data is based on the experiences of people who were diagnosed more than 5 years ago. New treatments, investigational therapies, and individualized factors like age and overall health are not always represented in these figures. Five-year relative survival rates are also not set in stone. Many people with this condition tend to live far longer than 5 years — your experience may vary significantly from the rates reported in these studies.Chordoma cancer 5-year survival rates
Here are the most recent 5-year relative survival rates available for chordoma cancer based on people who were diagnosed between 2011–2017:Localized (cancer has not spread): 86%Regional (cancer has spread into nearby structures, bones, or lymph nodes): 85%Distant (cancer has metastasized to distant parts of the body): 61%All stages combined: 79%The takeaway
Chordoma cancer is a rare form of sarcoma that occurs in the base of the skull or spine. The first-line treatment for this condition is usually surgery to remove as much of the tumor as possible. If a doctor suspects you have a chordoma or any other type of bone cancer, you’ll undergo imaging tests and a biopsy. The 5-year relative survival rate for this condition is favorable, ranging from 86% for localized cancers to 61% for those that have metastasized to distant parts of the body. Last medically reviewed on October 14, 2022How we vetted this article
SourcesHistoryHealthline has strict sourcing guidelines and relies on peer-reviewed studies, academic research institutions, and medical associations. We avoid using tertiary references. You can learn more about how we ensure our content is accurate and current by reading our editorial policy.Chordoma. (n.d.).rarediseases.org/rare-diseases/chordoma/Chordomas. (n.d.).
cedars-sinai.org/health-library/diseases-and-conditions/c/chordomas.htmlSurvival rates for bone cancer. (2022).
cancer.org/cancer/bone-cancer/detection-diagnosis-staging/survival-statistics.htmlTreatment for advanced or metastatic disease. (n.d.).
chordomafoundation.org/treatment/advanced-and-metastatic-disease/Understanding chordoma. (n.d.).
chordomafoundation.org/learn/understanding-chordoma/Our experts continually monitor the health and wellness space, and we update our articles when new information becomes available. Current Version Oct 14, 2022 By Corey Whelan Edited By Tim Jewell Medically Reviewed By Julie Scott, DNP, ANP-BC, AOCNP Copy Edited By Stassi Myer - CE Share this articleMedically reviewed by Julie Scott, DNP, ANP-BC, AOCNP — By Corey Whelan on October 14, 2022