What Is It?

A chordoma is a rare tumor that is derived from primitive remnants of a developmental structure called the notochord. The notochord is present during early development and helps to guide the development of the nervous system and vertebral column in vertebrates. Rarely, remnants of this structure, which normally is not present in the body after early development, can give rise to this slow-growing tumor.

Chordomas can occur anywhere along the vertebral column and base of the skull. However, the two most common locations by far are the sacrum (the bottom part of the spinal column and a part of the pelvis) and the clivus. The clivus is a bone of the skull, located at the base of the skull just in front of the brain stem and just behind the nasal cavities. Therefore, while not technically a brain tumor, clival chordomas (the ones originating in the clivus) grow very close to the brain stem and the cranial nerves that pass through the area. Because of its location at the base of the skull, this is one of many tumors which are often called skull base tumors.

While the pathology of these rare tumors are generally relatively benign, they can be quite aggressive with destruction of local tissues. The erode the bone and adjacent soft tissues and can cause damage to the nerves and brain stem as they enlarge.

What Types of Symptoms Are Typical?

Sacral tumors often cause back pain and/or neurological symptoms such as leg pain and weakness.

Clival chordomas sit in a region which can affect both the nerves coming out of the brain stem, the cranial nerves, and the brain stem itself. While presentation can vary, common presenting symptoms include double vision (due to disruption of the nerves that control eye movements), dysfunction of eye movements, and headache.

As they enlarge, other cranial nerves can become involved, often causing numbness or tingling of the face. Very large tumors can compress the brain stem, leading to more severe neurological impairment.

How Is The Diagnosis Typically Made?

After a consistent history and physical, most brain tumors require a CT scan or MRI scan to image the brain and surrounding structures. The typical location in the clivus and other factors make this tumor fairly easy to diagnose by these studies. However, as with any tumor, obtaining a tissue sample for pathological analysis is needed to make a definitive diagnosis and to distinguish it from other tumors that can occur in the area, such as skull base chondrosarcoma.

What Are Some Common Treatments?

Although each case is different, many chordoma patients are recommended to have surgery. This is done through a number of different types of skull base approaches, including endonasal transsphenoidal surgery in some centers. How much tumor can be removed depends on the size and invasiveness of the tumor. Because these tumors do tend to recur frequently, the more tumor that can be removed safely, the better chance for longer intervals until the tumor recurs. If residual tumor is left after surgery, radiation treatments are often recommended to help slow or prevent these recurrences. Each specific case is different and should be discussed with each patient's physician to decide what treatment options are best.

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Important Note: This site is not intended to offer medical advice. Every patient is different, and only your personal physician can help to counsel you about what is best for your situation. What we offer is general reference information about various disorders and treatments for your education.

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