About Chordoma

Overview

Chordoma (also called notochordal sarcomas) is a rare type of cancerous bone tumor that develops from cells of the notochord, which is a structure present in the developing embryo and important for the development of the spine. Chordomas can occur anywhere along the spine, from the base of the skull to the tailbone. They grow slowly, gradually extending into the surrounding bone and soft tissue. About half of all chordomas occur at the bottom of the spine (sacrum), about one third occur at the base of the skull, and the remaining cases form in other parts of the lower back or spine. The symptoms experienced by individuals with chordoma depend on its location. A chordoma at the base of the spine may cause problems with bladder and bowel function, while a chordoma at the base of the skull may lead to double vision and headaches. The cause of chordoma remains unknown in many cases, although recent studies have shown that changes in a gene called T gene have been associated with chordomas in some families.

Causes and Risk Factors

While the exact causes of chordoma are not fully understood, researchers have identified some factors that may contribute to its development.

Risk factors of chondromas include:

• Increasing Age: Chordoma predominantly affects middle-aged and older adults over 40 years of age.
• Genetic predispositions: About 80% of individuals with chordoma have a genetic variation in a gene responsible for spinal development. However, many people in the general population also have this variant and do not develop chordoma.

Symptoms

The symptoms of chordoma can vary depending on the location and size of the tumor. In general, chrodoma is associated with symptoms that include numbness, weakness, or pain along the back, arms and legs. Symptoms of chordoma at or near the base of the skull include:

• Vision issues, including double vision and blurry vision
• Headaches
• Facial numbness and pain
• Difficulty swallowing
• With more progression: Facial weakness or drooping due to facial nerve paralysis

Symptoms of chordoma at or near the tailbone inlcude:

• Numbness or tingling in the legs
• Weakness in the legs
• A lump near the tailbone
• Low back pain or tailbone pain
• Bladder or bowel Issues
• Difficulty walking
• Erectile dysfunction

In some cases, chordomas can spread (metastasize) to other parts of the body, including lymph nodes, lungs, liver, or other bones. This can result in widespread pain, fatigue, loss of energy, and weakness.

Diagnosis

To diagnose chordoma, healthcare providers may perform several examinations, tests, and procedures. These include:

• Physical exam and health history: The healthcare provider will examine the body for any physical signs of a medical problem, such as pain, swelling, or the presence of masses. They will also ask about the patient's health habits and past illnesses and treatments.
• Magnetic resonance imaging (MRI): This procedure uses a magnet, radio waves, and a computer to create detailed pictures of areas of the body, including the spine. It can help determine the location of a chordoma tumor and whether it has invaded surrounding tissues.
• Computed tomography (CT) scan: This procedure creates detailed pictures of areas inside the body, such as the brain and spine, from different angles. It can provide additional information about whether a tumor has spread. Doctors may also recommend scanning other areas like the pelvis, chest, and abdomen to check for spread.
• Biopsy: If MRI and CT scans indicate the presence of chordoma, a doctor may perform a biopsy to confirm the diagnosis. This involves removing a small sample of the tumor and examining it under a microscope.
• Bone scan: This procedure checks for rapidly dividing cells, such as cancer cells, in the bone. It can help determine if chordoma has spread.

This information gathered will help determine the stage of chordoma, which is based on whether cancer cells have spread within the spine or to other parts of the body. Staging may be used to plan treatment.

It's important to follow up with your healthcare provider if any symptoms worsen or change after a physical exam or any other tests. They will guide you through the diagnostic process and provide appropriate care based on your individual situation.

Treatment Options

The goals of treatment for chordoma are to achieve complete removal of the tumor if possible, control local recurrence, and improve overall survival. Treatment options include:

• Surgery: Surgical resection is the mainstay of treatment for chordoma. The goal of surgery is to completely remove the tumor without causing harm to surrounding structures. Complete removal, known as en bloc resection, provides the best chance for local control and long-term survival. Surgical margins, which refer to the extent of tumor removal, are an important prognostic factor.
• Radiation therapy: Radiation therapy uses high-energy beams to kill cancer cells and shrink tumors. It can be used before surgery (neoadjuvant) to reduce tumor size or after surgery (adjuvant) to destroy any remaining cancer cells. An example of a type of radiation therapy that may be used for chordoma is proton therapy.
• Targeted therapy: Chordomas are generally resistant to traditional chemotherapy drugs. Targeted therapies are newer treatments being researched. These therapies specifically target certain molecules or pathways involved in cancer growth and progression. More studies are needed to determine their effectiveness in Chordoma treatment.
• Immunotherapy: Immunotherapy is a type of treatment that helps the immune system recognize and attack cancer cells. Immunotherapy is being researched for chordoma.
• Health behavior changes: Making certain lifestyle changes can also support chordoma treatment and overall well-being. These changes may include maintaining a healthy diet, engaging in regular physical activity as tolerated, managing stress levels, and getting enough restful sleep.

It's important to note that there is no single treatment that is suitable for every person with chordoma. The choice of treatment depends on various factors such as the individual's overall health, tumor location, and stage of the disease. A comprehensive approach involving a team of healthcare professionals is crucial for developing an individualized treatment plan that maximizes outcomes for patients with chordoma.

Progression or Complications

Chordomas are initially slow-growing but known for their locally aggressive growth pattern and high rates of local recurrence. Chordomas can recur in the same area even after successful treatment. Around 30% to 40% of chordoma tumors eventually spread to other parts of the body, a process known as metastasis. However, this does not mean that chordomas are untreatable.

Complications associated with chordoma include:

• Cerebrospinal fluid leakage: This occurs when there is a leakage of the fluid that surrounds the brain and spinal cord. In some cases, cerebrospinal fluid leakage can lead to infections.
• Intracranial infection: Chordomas located in the skull base can sometimes lead to infections within the cranial cavity.
• Cranial nerve defect: Chordomas near the skull base can affect cranial nerves, leading to various cranial nerve deficits.
• Respiratory dysfunction: In some cases, chordomas can cause respiratory problems.
• Carotid artery injury: The carotid artery, which supplies blood to the head and neck, can be damaged by chordomas in rare instances.

It's crucial for individuals with chordoma to work closely with their healthcare team to determine the most appropriate treatment plan based on their specific situation. As with any medical condition or treatment approach, it's always recommended to consult a healthcare professional for personalized advice and guidance tailored to individual needs. Regular follow-up tests are often necessary to monitor for any signs of recurrence or spread.