Chordoma: Understanding Symptoms & Treatment Options

Chordoma: Understanding Symptoms & Treatment Options Chordoma is a rare cancer that happens in the skull base and spine bones. This article will cover the main parts of this disease, like symptoms and treatment. Knowing about chordoma helps with early detection and better care for patients, their families, and doctors.

Since chordoma is rare, it’s key to know its symptoms. Symptoms like neck or spine pain and nerve problems can seem like other illnesses. This makes it important to be aware. Finding chordoma needs special tests and biopsies, helped by experts like the Chordoma Foundation.

This article looks at treatment options, including surgery, radiation, and drugs. It shares new research and methods to help improve chordoma treatment and outlook.


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What is Chordoma?

Chordoma is a rare cancer that mainly affects the bones at the base of the skull and spine. It comes from leftover parts of the notochord, which is there during the early stages of growth. The American Cancer Society says chordoma cancer is very rare, happening to about one in a million people each year.

It’s important to know about it because it’s a serious condition.

Chordoma usually grows in three places:


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  1. Skull base
  2. Cervical spine (neck area)
  3. Sacral region (lower spine)

Studies show that chordomas happen when cells from the notochord don’t go away and change. The notochord is key in early growth, helping make the spine.

Chordoma grows slowly but can spread a lot. It’s a rare tumor but hard to treat because it’s near important nerves.

Doctors work hard to find it early and plan the best treatment. Knowing how it starts and grows helps fight this serious illness.

Common Symptoms of Chordoma

Knowing the signs of chordoma early is key to managing it well. Chordoma symptoms can show up in many ways, based on where and how big the tumor is. Here are some common signs to watch for.

Neck and Spine Pain

One big sign of chordoma is pain in the neck and spine. This pain comes from the tumor pressing on the spine or spinal cord. People often feel a dull, aching pain that gets worse over time.

This pain can make life hard. Sometimes, doctors think it’s from another spinal issue. So, it’s important to get a full check-up to find the real cause.

Nerve Dysfunction

Chordoma can also cause nerve problems. This might mean muscles feel weak, you lose feeling, or moving is hard. If the tumor gets bigger, it can press on the spinal cord, making these problems worse.

These issues are often talked about in medical journals and groups for patients. Getting medical help quickly is important to stop nerve problems from getting worse.

Other Notable Symptoms

Other symptoms of chordoma include headaches and problems with bowel or bladder function. These signs show up as the tumor puts pressure on nearby parts. Spotting these signs early can help doctors diagnose and treat chordoma faster, which might make things better for patients.

Causes of Chordoma

Researchers have looked into what causes chordoma. They found both genes and environment might play a part. But, we still don’t fully understand it. They’ve made good progress in finding out why it happens.

Genetic Factors

The brachyury gene, also known as the T gene, is key in chordoma causes. It’s linked to chordoma, especially when it runs in families. Studies show that changes in this gene make getting chordoma more likely.

Experts at places like the National Cancer Institute have checked this out. They used genetic maps to prove the link.

Environmental Influences

Some think environmental factors might also cause chordoma. But, we haven’t found any clear causes yet. Scientists are looking into work hazards, chemicals, and lifestyle choices. But, they’re focusing more on genes because we know more about them.

Factors Details
Genetic Mutations in the brachyury gene observed in familial cases of chordoma.
Environmental No conclusive evidence yet; possible factors include occupational hazards and chemical exposures.

Diagnostic Methods for Chordoma

Diagnosing chordoma takes several steps to make sure it’s right. Key steps include using advanced imaging and doing biopsies. These steps are very important for finding and understanding the chordoma.

Imaging Techniques

Doctors use imaging to find chordoma. MRI shows soft tissues well, helping to see the tumor’s size and where it is. CT scans show bones clearly, which is important for knowing the disease’s full picture.

These scans help doctors spot chordoma’s signs. They show where the tumor is in the skeleton and its special features. Following guidelines, these scans help make a first chordoma diagnosis. This makes sure treatments can hit the right target.

Biopsy Procedures

After seeing the tumor with MRI and CT scans, a biopsy is done to confirm the diagnosis. Surgeons and oncologists work together to take a tissue sample from the tumor.

A pathologist looks at the biopsy sample to see if it’s chordoma. This is key to telling chordoma apart from other tumors. Knowing the chordoma’s details helps plan the best treatment.

Diagnostic Method Primary Use Advantages Limitations
MRI Soft tissue imaging High resolution, detailed tumor visualization Expensive, contraindicated for patients with certain implants
CT Scans Bone imaging Clear images of bone involvement Radiation exposure, less soft tissue detail
Biopsy Tissue sample analysis Definitive diagnosis, histological confirmation Invasive, risk of complications

Chordoma Treatment Options

Managing chordoma means using different treatments based on the patient and the tumor. Places like Memorial Sloan Kettering Cancer Center and Mayo Clinic use a team approach. This helps in treating chordoma better.

  • Surgery: Surgery is often the first step. The goal is to remove as much of the tumor as possible. Surgeons work hard to leave no cancer behind. Thanks to new surgery methods, more people are getting better.
  • Radiation Therapy: This is used when surgery can’t be done or as extra help. New types like proton beam therapy and stereotactic radiosurgery are very precise. They help control cancer and lower the chance of it coming back.
  • Emerging Treatments: Researchers are looking into new treatments like targeted therapies and immunotherapy. There are ongoing studies to see if these can work better and be safer.

It’s important to have a team of experts like neurologists, oncologists, and radiologists working together. Following guidelines from places like the National Comprehensive Cancer Network (NCCN) helps make sure patients get the best care for chordoma.

Surgical Interventions

Surgery is key for chordoma treatment. It aims to remove the tumor fully. This helps prevent it from coming back and improves the patient’s chances of getting better.

Types of Surgery

En bloc resection is a top choice for its success. It removes the tumor and some healthy tissue around it in one go. This lowers the risk of the tumor spreading. Studies in the Journal of Neurosurgery show it gives better long-term results than removing the tumor in pieces.

This method also tries to save important nerve functions. It does this by touching less the areas around the tumor.

Risks and Recovery

Chordoma surgery has risks like infection, nerve harm, and leaks of cerebrospinal fluid. Watching over the patient after surgery is very important. Some patients bounce back quickly, but others may need more time to fully recover.

Recovery varies based on how much surgery was needed. It often includes physical therapy, managing pain, and check-ups to catch any signs of the tumor coming back. Knowing what to expect helps patients get ready for their recovery.

Radiation Therapy for Chordoma

Radiation therapy is key in treating chordoma, especially when surgery can’t remove the whole tumor. New advances in radiation have greatly helped chordoma patients live longer.

Proton Beam Therapy

Proton beam therapy is a new way to give radiation that’s great for chordoma. It targets the tumor without harming nearby healthy tissues. This makes it safer and more effective.

Studies at The University of Texas MD Anderson Cancer Center show it works well. It stops chordoma from growing and helps patients live longer.

Traditional Radiation

Traditional radiation uses X-rays to kill chordoma cells. It’s been a main treatment for a long time. But, it’s not as precise as proton therapy and can harm healthy tissues.

Still, it’s an option for patients who can’t get proton therapy. The American Society for Radiation Oncology says it can still make a big difference in survival rates.

Aspect Proton Beam Therapy Traditional Radiation
Precision Level High Moderate
Side Effects Minimal More Pronounced
Chordoma Survival Rate Improvement Notable Substantial
Accessibility Limited Widespread

Medications and Drug Therapies

Chordoma treatment goes beyond surgery and radiation. It includes many medications and drug therapies. These help target cancer cells and ease symptoms. We’ll look at the latest options, like targeted therapies and pain relief strategies, based on research and trials.

Targeted Therapies

Targeted therapies are key in fighting chordoma. They aim at specific molecules in cancer cells. This makes them more effective than old treatments.

New research has found promising targets. This led to new drugs being tested in trials. These drugs block growth pathways or proteins, slowing the cancer’s growth with fewer side effects.

Pain Management

Managing pain is vital for chordoma patients. They often face a lot of pain. There are many drugs to help, from non-opioid painkillers to new treatments.

Researchers are always finding new ways to ease pain in trials. They test new drugs or ways to deliver them. The goal is to give patients more relief and a better life. This shows how important pain management is in treating chordoma.

FAQ

What are the common symptoms of chordoma?

People with chordoma often feel neck and spine pain. They might also have nerve problems like weak muscles or losing feeling. Some have headaches or trouble with their bowels or bladder.

How is chordoma diagnosed?

Doctors use MRI and CT scans to see the tumor. They might take a biopsy to check the tissue under a microscope.

Are there genetic factors involved in chordoma development?

Yes, genes play a part in chordoma. Mutations in the brachyury (T) gene are linked to it. But, we're still learning more.


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