Definition

Chordoma is an uncommon, slow-growing tumor that is thought to arise from cellular remnants of the embryonic spine called the notochord. Chordomas are found most frequently at the base of the spine (the sacrum) and intracranially at the clivus (see figure) but they may occur anywhere along the spine.

Causes

No real cause for chordomas is known but a genetic basis has been described for some chordomas.

They have also been reported in families with tuberous sclerosis, and certain types of childhood brain tumors

No environmental factors, trauma, or diet are known to be associated with chordomas.

Risks

Risk factors are things associated with an increased chance of developing a disease or condition. There are no known risk factors for chordomas.

While chordomas are generally slow-growing tumors, they tend to cause local destruction of the bone and grow into nearby tissues and organs. They frequently come back after initial treatment. Distant spread (metastasis) is rare but not unknown.

Symptoms

Symptoms depend on the location of the chordoma.

Clival chordomas most commonly present with:

  • double vision
  • headache
  • facial pain or paralysis
  • hearing changes
  • swallowing problems
  • hoarseness

Sacral chordomas may not cause symptoms until they are quite large but common symptoms are:

  • back and/or lower extremity pain
  • leg weakness
  • rectal dysfunction
  • urinary incontinence
  • erectile dysfunction
  • palpable sacral mass

Diagnosis

  • MRI and/or CT of the brain
  • Neurological examination
  • Tissue biopsy

Treatment

  • Surgery is the primary treatment. Sometimes this requires an open cranial procedure. As minimally invasive, transnasal approaches have evolved, many can now be removed through the nose using endoscopic assistance.
  • In most cases, as complete a surgical resection as is possible within the limits of safety, offers the best chance of long-term control.
  • Chordomas are fairly resistant to radiation and high doses are often needed for disease control. Since chordomas are usually close to critical neurological structures such as the brain stem and cranial nerves, there is a limit as to how much radiation can safely be delivered. Therefore, highly focused radiation such as sterotactic radiosurgery or proton beam therapy may be more effective than conventional x-ray radiation
  • Frequent follow-up is required because of the high rate of recurrence of these tumors, especially when a gross total resection was not achieved.

Prevention 

There is no known way to prevent chordomas.

Case Study

A 66 year old woman began to develop double vision, which was worse when she looked to the right. She also noticed a muffled sound in the right ear with diminished hearing and discomfort in the right side of her jaw. Her work up led to MRI and CT which showed a lesion in the lateral clivus. Various management options were considered. As the diagnosis was unclear, a tissue biopsy was strongly recommended. Dr. Joshua Bederson and Dr. Eric Genden performed an extended transnasal microendoscopic partial resection. The final pathology revealed a chordoma and the patient was referred for radiation to treat the residual tumor. Aside from some post-operative headaches, changes in her sense of smell, and radiation related fatigue she has done well and resumed most of her former activities. She continues to be monitored on a regular basis.

If you want to learn more about Chordomas call the Mount Sinai Department of Neurosurgery at 212-241-2377.

Image from: http://en.wikipedia.org/wiki/File:Clivus.png#file (Permission is granted to copy, distribute and/or modify this document under the terms of the GNU Free Documentation License, Version 1.2 or any later version published by the Free Software Foundation; with no Invariant Sections, no Front-Cover Texts, and no Back-Cover Texts.)