What is a Chordoma?

Chordoma is one of the rarest types of bone cancer and often manifests in the skull or spine. The diagnosis of such an unusual tumor can come as an unsettling surprise.

It is not uncommon to feel overwhelmed with the information and new terminology presented during the initial discussions with your medical team. Continue reading to gain a better understanding of this condition at your own pace as you embark on your journey towards treatment.

Chordoma is a rare and slow-growing type of bone cancer. It most often develops in the large triangle-shaped bone at the bottom of the spine (sacrum) or at the base of the skull. When at the sacrum, it may be referred to as a "sacral chordoma". Because they can also develop at the skull base and are located close to brain tissue, chordomas are sometimes thought of as brain tumors, even though they do not originate from brain tissue itself.

Chordomas arise in approximately 1 in 1 million people per year and are most frequently diagnosed in individuals between 50 and 60 years of age. They are exceptionally rare among children. When affecting young female patients, chordomas appear more often at the skull base. Although many genes have been identified that can increase the risk of chordomas, instances of this type of tumor running in families are exceedingly rare.

Although chordomas are known to grow slowly, they are malignant tumors that can cause significant damage to bones and surrounding tissues over time. Chordomas also tend to recur after treatment. In many cases, recurrence occurs late and can appear 5 or even 10 years following treatment. Thus, many patients will require long-term follow-up with their medical team. 

Depending on the location and size, chordomas may cause no symptoms (asymptomatic) in some patients or severe symptoms in others. Common symptoms include:

• Headache: Chordomas can put pressure on the brain, blood vessels, or nerves.
• Vision changes: Chordomas that occur in the skull base can press on the eyes or optic nerves, leading to vision disturbances, double vision, and sometimes blindness.
• Weakness or numbness in the limbs: Chordomas in the spinal column can press on nerves that control movement and sensation, leading to weakness or numbness in the arms or legs.
• Back or neck pain radiating to the limbs: Chordomas can cause pain in the back or neck. The pain can also radiate to the limbs if they press on or squeeze a nerve.
• Bowel or bladder disturbances: Chordomas in the sacral region, at the base of the spine, can cause difficulties in urinating or defecating by compressing the nerves that control these body functions.

If you have any concerning symptoms, it's important to see a doctor for evaluation. Even though they are rare, a chordoma is a serious condition that can cause worse symptoms if left untreated. Diagnosis typically begins with a detailed medical history and examination. Your doctor will likely ask about the timing and characteristics of your symptoms and will perform a physical and neurological examination.

Certain symptoms, such as a headache or back pain, are extremely common and it is unlikely that a chordoma will be suspected if you are experiencing these symptoms in isolation. On the other hand, neurological deficits, such as weakness or numbness, are more concerning and may prompt further testing. Imaging tests such as an MRI to provide a detailed picture of soft tissues or a CT scan that helps to visualize bony structures can determine the location and size of the tumor.

If a tumor is found, a biopsy may be performed to confirm the diagnosis. A biopsy is a procedure in which a small sample of tissue is removed from the tumor and examined under a microscope. The specimen can also undergo further testing to check for other molecular features. A special physician called a pathologist will perform these tests and prepare a report. This pathology report will help to determine the type of tumor which is essential in planning treatment.

In some cases, additional tests may be ordered to determine if the tumor has spread to other parts of the body. This may include a positron emission tomography (PET) scan, which uses a small amount of radioactive material to help identify any areas of increased activity that could indicate the presence of cancer cells, or a bone scan, which can help to detect if the tumor has spread to the bones.

Treatment for chordomas typically involves a combination of surgery, radiation therapy, and sometimes chemotherapy. The specific treatment plan will depend on the location and size of the tumor, as well as your overall health and preferences.

Surgery is often the main treatment for chordomas. The goal of surgery is to remove as much of the tumor as possible without causing damage to surrounding normal tissue. Surgery can be complex, especially if the tumor is in a difficult-to-reach area of the skull or spine.

In some cases, a combination of different surgical approaches may be needed, such as open surgery (a traditional surgical approach with larger incisions) and endoscopic surgery (minimally invasive surgery using small incisions and specialized instruments to access the tumor and perform the procedure).

Radiation therapy may be used after surgery to eliminate any remaining cancer cells. Radiation therapy is delivered by a machine that targets high-energy x-rays or protons to the tumor. Chemotherapy may be used in addition to or instead of radiation therapy, especially for chordomas that have spread to other parts of the body. Clinical trials are underway to assess the effectiveness and safety of new drugs for chemotherapy.

In cases of recurring chordomas, the treatment options available may be limited and will depend on the location of the recurrent tumor. Note that treatment for chordomas may cause side effects, including fatigue, nausea, hair loss, and skin irritation. Managing these side effects is also part of the treatment process. A specialized team of doctors with experience in treating chordomas can provide you with more information about your specific case and help you to make an informed decision about the best treatment approach for you.

• Chordomas are slow-growing, locally invasive malignant tumors that often recur but may also spread to other parts of the body.
• Chordomas usually occur in the skull base, sacrum, and spine and may cause a variety of symptoms.
• Diagnosis may include a CT and MRI scan to visualize the location and size of the tumor, a PET scan to check for metastases, and a biopsy to confirm the diagnosis.
• Surgery is the primary treatment method and is sometimes accompanied by radiotherapy, and rarely chemotherapy.

• National Cancer Institute: Chordoma
• Chordoma Foundation
• NIH Genetic & Rare Diseases (GARD) Center: Chordoma
• Cancer Net
• American Cancer Society