Chordoma is a rare form of cancer found in the bone. It belongs to a group of tumors called sarcomas. They are found in the spine (especially in the sacrum or neck) or skull (especially in the middle of the skull base at areas called the sella and clivus). Chordomas tend to grow slowly and may be present for many years before they produce symptoms.
Most patients develop symptoms in their 50s or 60s. About one-third have their chordoma in the clivus, and these usually present at a younger age. Chordomas are more common in males.
Chordomas can be difficult to treat due to their close location to the brainstem and its cranial nerves, the spinal cord and its nerve roots, and blood vessels. Outlook can be unfavorable due to the local growth and invasive nature of a chordoma which leads to bone destruction.
Recurrence or regrowth of the tumor at its original site is not uncommon. About one-third of patients may eventually develop distant metastases (chordoma deposits spreading to other organs such as lung or liver).
Signs and Symptoms
Symptoms from a cranial chordoma are primarily a result of compression of nearby structures (brainstem and cranial nerves). Some of the more common symptoms of chordomas include:
- Abnormal movement of the eyes
- Voice or speech changes
- Double vision
- Frequent headaches
- Inability to feel or move your face
- Difficultly swallowing
- Pain in the neck
- Weakness, numbness or tingling in the extremities
- Endocrine (hormone) problems
Chordomas are thought to arise from remnants (remains) of the notochord. The notochord is a critical structure leading to the development of the spine. It should normally disappear during development of the embryo.
An exact cause for chordoma is unknown. Most are thought to occur randomly. Occasionally they are found in families, suggesting certain genetic mutations may be associated with an increased risk. Chordomas are seen more often in patients who have tuberous sclerosis.
Many other conditions can cause the same symptoms, so it is important to check with your doctor for an accurate diagnosis. Only a doctor experienced in recognizing the exact combination of symptoms that indicate a brain tumor can make a firm diagnosis.
Some chordomas may be found by accident before they produce any symptoms. More often, the development of symptoms leads to a physician visit. Diagnosis begins with a medical history and neurological examination. Other tests are needed to establish and confirm the diagnosis. These include: MRI scan of the brain (or spine), CT of the brain (or spine) or a biopsy. Occasionally an image-guided biopsy is done to make the diagnosis. At times MRI or CT may be combined with angiography to provide more detail about the arteries.
A customized treatment plan is put together for you based on:
- The location and size of your chordoma
- Your age and general health
- Your tolerance for treatment
- Your preferences
Treatment for a chordoma may include one or a combination of the following options:
- Surgery Skull base surgery is the primary treatment option for a cranial chordoma. However, the tumor can be large, difficult to access and close to extremely important structures.
- Radiation therapy This treatment is often employed, but chordoma is not particularly sensitive to radiation in comparison with many other types of tumors. It is usually used as an adjunct (supplement) to surgery. Stereotactic radiosurgery, proton beam therapy or intensity-modulated radiotherapy are techniques which may be employed.
- Molecular targeted therapies There has been some success with targeted therapy for advanced or recurrent chordoma. Recently, brachyury vaccine has shown potential promise for treatment.