Chordoma

Chordoma: A Rare Bone Cancer

Chordoma is a rare type of cancer that affects the bones, most commonly the bones of the spine or the skull. It often starts where the skull meets the spine (skull base) or at the bottom of the spine (sacrum).

This cancer begins in cells that were part of a group of cells crucial for the development of the spine's cushioning discs. Usually, these cells disappear before birth or shortly after. Sometimes, a few linger, and in rare cases, these remaining cells can become cancerous, forming chordoma.

Chordoma is most frequently diagnosed in adults between 40 and 60 years old, but it can occur at any age. The cancer typically grows slowly. Treatment can be challenging because chordomas are often located near vital structures like the spinal cord, arteries, nerves, or the brain itself.

Diagnosing Chordoma:

Doctors use several methods to diagnose chordoma:

• Biopsy: A biopsy involves removing a small tissue sample of the suspected cancerous area. This sample is sent to a lab where specially trained doctors (pathologists) examine it under a microscope to see if cancer cells are present. Carefully planning the biopsy is crucial to avoid hindering future surgery to remove the tumor. It's important to seek a team of specialists experienced in chordoma treatment to ensure the biopsy is performed in a way that doesn't interfere with future procedures.

• Imaging Tests: Doctors often use imaging tests like MRI or CT scans to get a clearer picture of the chordoma and see if it has spread beyond the spine or skull base.

Treating Chordoma:

Once diagnosed, a personalized treatment plan is developed by a team of specialists, including oncologists (cancer doctors), otolaryngologists (ear, nose, and throat specialists), radiation oncologists, and possibly surgeons, endocrinologists, ophthalmologists, and physical therapists as needed. The plan depends on factors like the tumor's size, location, and whether it has affected surrounding tissues.

Treatment Options (Sacral Chordoma):

If the chordoma is located in the lower part of the spine (sacrum), treatment may involve:

• Surgery: The goal is to remove as much of the tumor as possible while minimizing damage to healthy tissue around the tumor, which can be challenging due to the proximity of important nerves and blood vessels. If complete removal isn't possible, surgeons will aim to remove as much of the tumor as safely possible.

• Radiation Therapy: High-energy beams (like X-rays or protons) are used to target and destroy cancer cells. You lie on a table, and the machine moves around you, precisely directing radiation to the tumor. Radiation therapy can be used before or after surgery to shrink the tumor or kill any remaining cancer cells. Modern radiation techniques, like proton therapy, allow for higher doses while better protecting healthy tissues, potentially improving treatment outcomes.

• Radiosurgery: A specialized form of radiation therapy that uses multiple beams of radiation to precisely target and destroy cancer cells in a small area. While each beam is relatively weak, the combined effect is a powerful dose concentrated on the tumor. Radiosurgery can be used before or after surgery.

• Targeted Therapy: Drugs that specifically target weaknesses within cancer cells are used to destroy them. This approach might be considered if the cancer has spread to other parts of the body.

Treatment Options (Skull Base Chordoma):

If the chordoma is located at the skull base, treatment may involve:

• Surgery: The initial approach often involves surgery to remove as much of the tumor as possible, carefully avoiding damage to nearby healthy tissue and structures like the brain or spinal cord. Complete removal may not be possible if the tumor is close to vital structures like the carotid artery. Minimally invasive techniques, such as endoscopic surgery through the nose, can be used to access and remove the tumor. A follow-up surgery might be necessary in some cases to fully remove remaining tumor or stabilize the area.

• Radiation Therapy: Radiation therapy is often used after surgery to eliminate any remaining cancer cells. Modern techniques like proton therapy and stereotactic radiosurgery allow for precise targeting and higher doses, which can be more effective in treating skull base chordoma.

• New Treatments: Ongoing research and clinical trials explore new treatments for skull base chordoma, including therapies that target specific vulnerabilities within chordoma cells. Discuss these options with your doctor if you are interested.

It's crucial to work closely with a team of experienced specialists throughout your chordoma treatment journey. This collaborative approach ensures the best possible outcomes.

Spinal cord tumors can cause various problems, especially as they grow. These tumors can affect the spinal cord itself, the nerves branching off it, the blood vessels, or the bones of the spine. This can lead to a range of symptoms:

Common Symptoms:

• Pain: A common early symptom is pain at the location of the tumor. This pain can spread to other areas, like the hips, legs, feet, or arms, and often gets worse over time. Sometimes, back pain that's worse at night is a sign.
• Numbness and Reduced Sensation: You might feel less sensitive to touch, heat, or cold.
• Muscle Weakness: This can range from mild to severe, affecting different parts of the body, including arms and legs. Difficulty walking, even to the point of falls, is another possible symptom.
• Bowel and Bladder Problems: Tumors can cause trouble controlling bowel movements or urination.

When to See a Doctor:

While back pain is very common, it's not always a sign of a tumor. Many things can cause back pain. However, it's important to see your doctor if your back pain:

• Is persistent and getting worse: If the pain continues and worsens over time.
• Isn't related to activity: Pain that doesn't go away when you rest or change positions.
• Gets worse at night: Pain that intensifies when you're sleeping.
• Develops after a history of cancer: If you've had cancer before, new back pain warrants a doctor's visit.
• Comes with other cancer-like symptoms: If you experience nausea, vomiting, or dizziness, along with back pain.

Urgent Medical Attention:

Seek immediate medical care if you experience:

• Progressive numbness or weakness in your arms or legs: If these symptoms develop or worsen quickly.
• Changes in bowel or bladder control: Any sudden or noticeable changes in how you use the bathroom.

Important Note: The progression of spinal tumors varies greatly depending on the type of tumor. This information is not a substitute for professional medical advice. If you have any concerns about back pain or other symptoms, it's crucial to see a doctor for proper diagnosis and treatment.

Back pain has many possible causes, and most cases aren't due to a tumor. However, early detection is crucial for spinal tumors. See your doctor if your back pain:

• Keeps happening and gets worse over time: This means the pain doesn't go away and is steadily increasing.
• Isn't connected to activity: The pain isn't triggered by moving or exercising.
• Gets worse at night: Pain that worsens when you're resting could be a sign of something more serious.
• Develops after you've had cancer: If you've had cancer in the past and now have new back pain, it's important to see a doctor.
• Comes with other cancer-like symptoms: These might include feeling sick to your stomach (nausea), throwing up (vomiting), or feeling dizzy.

Seek immediate medical attention if you notice:

• Increasing weakness or numbness in your arms or legs: If you're having trouble using your arms or legs, this needs to be checked quickly.
• Changes in how your bowels or bladder work: This includes problems with urination or bowel movements. Any unusual or new changes in these functions should be addressed right away.

It's important to remember that these are just some warning signs. If you have any concerns about your back pain, it's always best to talk to a doctor. They can properly evaluate your situation and provide the best advice for you.

Scientists don't fully understand why most spinal tumors happen. One theory is that faulty genes are involved. It's often unclear if these faulty genes are passed down from parents or if they develop later in life. These changes might be triggered by environmental factors, like exposure to specific chemicals. However, some spinal tumors are clearly connected to specific inherited conditions, like neurofibromatosis type 2 and von Hippel-Lindau disease. These inherited conditions significantly increase the risk of developing these tumors.

Certain inherited conditions increase the risk of spinal cord tumors. One such condition is neurofibromatosis type 2. This is a genetic disorder where non-cancerous (benign) tumors grow on or near nerves, particularly those connected to hearing. This can cause gradual hearing loss in one or both ears. Unfortunately, some people with neurofibromatosis type 2 also develop tumors within the spinal canal.

Another condition linked to spinal cord tumors is Von Hippel-Lindau disease. This is a rare, complex disorder affecting many parts of the body. It's characterized by the growth of tumors called hemangioblastomas, which are tumors of blood vessels, in the brain, the lining of the eye (retina), and the spinal cord. People with Von Hippel-Lindau disease can also develop tumors in other organs like the kidneys or adrenal glands. It's important to understand that these tumors are not always cancerous, but they can still cause problems due to their location and size.

Tumors in the spine can put pressure on the nerves that run through it. This pressure can cause problems with movement and feeling in the parts of the body below where the tumor is located. For example, someone might lose the ability to feel their legs or have trouble moving them. The tumor's pressure can also affect how the bowels and bladder work.

This nerve damage can sometimes be permanent. However, if a spinal tumor is found and treated quickly, it's possible to stop the damage from getting worse and even help the nerves heal.

The location of the tumor is important. If the tumor presses directly on the spinal cord, it can be a very serious problem, potentially even life-threatening. This is because the spinal cord is like a major highway for messages between the brain and the rest of the body. If the spinal cord is squeezed, the messages can't get through properly, leading to serious problems.

Sometimes, spinal tumors can be hard to spot because they're not very common, and their symptoms can be similar to other, more usual health problems. This is why it's crucial for you to tell your doctor everything about your health history and have a complete physical and neurological exam.

If your doctor thinks you might have a spinal tumor, several tests can help confirm the diagnosis and find out exactly where the tumor is:

1. MRI (Magnetic Resonance Imaging):

An MRI uses a powerful magnet and radio waves to create detailed pictures of your spine, spinal cord, and nerves. It's often the best test for finding tumors in the spinal cord and the area around it. Sometimes, a special dye (contrast agent) is injected into a vein in your arm. This dye helps make certain parts of the picture stand out more clearly.

Some people feel anxious or uneasy inside the MRI machine because it can be a bit cramped and make a loud thumping noise. Ear plugs are usually provided, and some machines have TVs or headphones to help. If you're very worried, talk to your doctor about a medicine to help you relax (a mild sedative). In rare cases, general anesthesia might be needed.

2. CT Scan (Computed Tomography):

A CT scan uses a narrow beam of X-rays to create detailed images of your spine. Sometimes, a special dye is injected to make any unusual changes in the spinal canal or spinal cord more visible. A CT scan isn't typically the first choice for diagnosing spinal tumors, but it can sometimes be helpful in addition to an MRI.

3. Biopsy:

The only way to know exactly what kind of tumor it is is to take a tiny sample of tissue (a biopsy) and look at it under a microscope. This helps doctors decide the best treatment plan.