Chordoma

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Diagnosing and Treating a Chordoma

A person suspected of having a chordoma will have an initial examination that includes a detailed medical history, blood and urine tests, and a basic neurological examination to check for alertness, coordination, eye movement, muscle strength, and reflexes. Other tests, such as an ophthalmological exam, may be performed depending on symptoms. It is vital that an individual with a chordoma seek care from a highly trained, experienced multidisciplinary team, including neurosurgeons and neuro-oncologists at a major medical center where the physicians have proven skills in diagnosis and treatment. Since symptoms develop gradually and chordomas are generally slow-growing, by the time an individual goes to a doctor with symptoms, the chordoma may be quite large. In fact, an individual with a very large chordoma may even be able to feel a lump along the spine.

Also, since symptoms of a chordoma are common to other conditions, an accurate diagnosis will include extensive testing and expertise. The Weill Cornell Medicine Brain and Spine Center’s specially trained team will have the knowledge and tools to confirm a diagnosis and then embark on giving their patient the best care possible.

One of the tools used to diagnose a chordoma is an MRI scan, which uses magnetic fields and radio-frequency waves to create an image that can show the presence of a tumor and some of its characteristics, such as its size, precise location, and whether it has spread. If the tumor has not spread, and depending on other factors such as location, a needle biopsy may be performed to collect a small sample to be examined under a microscope. If a surgical team decides to perform a needle biopsy of the tumor, they may be prepared to also immediately remove the chordoma once the diagnosis is confirmed by a pathologist. This is because the procedure itself may cause cells to spread along the path of the needle. For this reason, clival chordomas are rarely biopsied.

Diagnostic Tests

Reaching an accurate diagnosis is critical to ensure optimal outcome and require unique, specific tests. Tests we offer include:

  • Dynamic hormone testing
  • High-resolution pituitary MRI
  • Inferior petrosal sinus sampling
  • Office endonasal endoscopy
  • Visual field and OCT testing
  • CT angiogram

 

Treatment

Treatment of a chordoma depends on its size and location, as well as the health of an individual. Successful treatment demands a medical team with extensive experience since removal and follow-up management of a chordoma is a delicate, complicated matter, and requires special attention to preserve neurological function. The usual treatment options are surgery, stereotactic radiotherapy and sometimes chemotherapy

The most effective treatment for chordoma is surgery and the goal is to remove most or all of the tumor. Advanced surgical methods allow neurosurgeons to approach clival tumors through the nostrils, which requires no incision and leaves no facial scarring. (See more about Surgery for a Chordoma.) For chordomas of the spine, neurosurgeons may use advanced minimally invasive techniques to approach and resect (remove) the tumor.

Stereotactic radiosurgery (SRS) may give more rapid results than traditional radiation therapy. Stereotactic radiosurgery, which may be performed using a proton beam device or linear accelerator (LINAC) or with machines known as CyberKnife and Gamma Knife, is not traditional surgery. Instead, this procedure uses a highly focused beam of radiation to target tumor cells while avoiding healthy tissue. The Stereotactic Radiosurgery Program at the Weill Cornell Brain and Spine Center offers the latest in advanced treatment for chordoma. (See more about our Stereotactic Radiosurgery Program.)

Chemotherapy is the use of anti-cancer (cytotoxic) drugs to destroy cancer cells. Chemotherapy has not been shown to be very effective in treating chordomas. However, it may sometimes be used to try to control a chordoma that has recurred or spread elsewhere in the body.

Close monitoring is important following treatment for chordomas, since they have a high recurrence rate. Imaging scans may be routinely ordered, and individuals who have had chordomas should let a doctor know if they experience any new symptoms between check-ups.

 

Reviewed by: Theodore Schwartz, MD

Last reviewed: March 2018