Chordoma Foundation


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Treatment

Chordomas are slow growing, yet aggressive and life-threatening tumors.  Outcomes for chordoma patients vary widely and are highly dependent on the course of treatment received and the individual tumor’s behavior. Modern treatment approaches can significantly prolong patient survival, and, with appropriate care, many patients can be cured.

Because the course of treatment received is so important for a patient’s outcome, we aim to provide the information patients need to make educated treatment decisions and obtain the best care possible. This page summarizes current treatment options available to chordoma patients and lays out points to consider when making treatment decisions.

The information provided herein does not replace advice from your doctor. It is important to remember that each patient’s situation is unique and only your doctor can tell you what treatments are right for you.

Key principles  |  Treatment overview  |  Surgery  |  Radiation  |  Systemic therapy

Key Principles


Find experienced doctors

Chordoma is a challenging disease to manage and treatment often involves highly complex procedures that require extensive sub-specialty training and experience to master. Furthermore, treatment of chordoma requires multiple specialists who must communicate and work closely together to coordinate your care. It is therefore very important to find experienced doctors who routinely treat chordoma as part of a multi-disciplinary team. Our doctor directory can help you find specialists who are experienced in treating chordoma.

Get multiple opinions

It’s always a good idea to consult with multiple qualified doctors for any major medical procedure. Good doctors are not offended by this reasonable step, as second opinions are standard practice, especially for complex diseases like chordoma.

For each chordoma patient there may be multiple viable treatment options, each with different risks and benefits. It is possible, even likely, that different doctors will recommend different approaches. Before making a decision, it is important to understand your options and the consequences of each approach. If you get conflicting recommendations from two specialists, then consider getting a third or fourth opinion. Ultimately, you will have to select the doctor and the approach that you are most comfortable with.

Don’t rush

The doctor and treatment plan you choose will have a major impact on your outcome. It is therefore important not to rush to a decision without understanding your options. In general, chordomas are slow growing, so, in most cases, patients have time to gather information and seek multiple opinions. Ask your doctors how soon you need to be treated, and do not delay treatment if you are advised it would be unsafe.

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Treatment overview


NCCN Guidelines® for Bone Cancer Treatment

We recommend that all patients read the National Comprehensive Cancer Network’s guidelines for the diagnosis and treatment of chordoma before planning treatment. You will be asked to create a free account to access the latest version.

Surgery is the mainstay of treatment for chordomas.  The goal of surgery is to remove as much of the tumor as possible without causing unacceptable harm. For some patients, radiation therapy can reduce the risk of recurrence after surgery and prolong survival. For patients who are not candidates for surgery, radiation therapy is sometimes used as the primary treatment. Chordomas are resistant to radiation, meaning that very high doses of radiation are required to control these tumors. Because of their proximity to vital anatomy such as the brain and spinal cord, which cannot tolerate high doses of radiation, specialized forms of radiation are used to focus radiation on the tumor while avoiding surrounding tissue.

Even after surgery and/or radiation, chordomas tend to return in the same location or in the areas around the original tumor. Many patients undergo multiple surgeries over several years to treat these local recurrences. For patients with advanced or inoperable disease, chemotherapy may slow or temporarily stop the progression of the tumor. Because chordomas are slow growing, standard cytotoxic chemotherapy agents that kill fast-growing cells are generally ineffective, however some chordomas patients have been reported to respond to molecularly targeted cancer drugs. There are currently no drugs approved by the FDA to treat chordoma.

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Surgery


Regardless of the location of one’s tumor, surgery is required for virtually all chordoma patients. The extent of surgical resection has a major impact on one’s outcome. Complete resection during one’s first surgery offers the best chance of long-term remission and survival. However, because chordomas occur near vital structures such as the spinal cord, brainstem, nerves, and arteries, complete resection is not always possible, or may cause significant loss of quality of life that patients find unacceptable. Below are points to consider regarding surgery for chordomas of the skull base, spine and sacrum.

Sacral and Spinal Chordomas

For chordomas of the sacrum and mobile spine, complete en bloc  resection (removing the tumor in one piece) with tumor-free margins is the goal of surgery. Intralesional resection (removing the tumor in pieces) is associated with a high rate of recurrence and diminished survival. [1] [2] In a 2009 review article of the treatment of sacral and spinal chordomas in the Journal of the American Academy of Orthopaedic Surgeons, Scuibba and colleagues at Johns Hopkins University write,

The importance of obtaining wide tumor-free margins when possible cannot be underestimated. Numerous studies demonstrate a direct correlation between the extent of surgical resection and the length of recurrence-free survival. [3]

Surgery for sacral chordomas may involve removal a portion of the sacrum or the entire sacrum depending on the location and size of the tumor. Depending on the extent of the tumor, these procedures may require a combination of surgical approaches to access the tumor from different angles. In order to achieve wide margins, these procedures often involve the intentional sacrifice of sacral nerves, which may result in motor, sensory, sphincter, or sexual dysfunction. The extent of dysfunction depends on which sacral nerves must be sacrificed. In general, the higher the tumor extends in the sacrum, the more sacral nerves must be sacrificed, and the more dysfunction will be experienced.

Surgery for spinal chordomas involves removal of one or more vertebral bodies – a procedure called a “spondylectomy.” These procedures often require a combination of surgical approaches (from the front and back), and may need to be carried out in multiple stages.

For both spinal and sacral chordomas, mechanical and soft tissue reconstruction are usually required after removal of the tumor and involved vertebral bodies. Due to the complexity of surgery and reconstruction, a multidisciplinary surgical team may be needed, including specialists in surgical oncology, neurosurgery, orthopaedic surgery, vascular surgery, and plastic surgery. This operation should be led by a neurosurgeon or orthopaedic surgeon who specializes in complex spine surgery.

Skull Base Chordomas

The goal of surgery for skull base chordomas is gross total resection; in other words, complete removal of all visible tumor.[4] [5] A meta-analysis of all published studies between 1999 and 2010 concluded that

complete resection is associated with improved progression free survival and overall survival in patients with skull base chordomas compared with subtotal resection.

Specifically, patients who have a subtotal resection are 3.83 times more likely to experience a recurrence and 5.85 times more likely to die at 5 years versus patients with complete resection.[6]

Due to the location of skull base tumors within the head, en bloc resection (removing the tumor in one piece) is rarely possible, however gross total resection can be achieved with an intralesional approach. When en-bloc resection or gross total resection is not possible, maximum safe resection is advocated.

There are a broad range of surgical approaches available to access skull base chordomas, and staged surgeries combining multiple approaches are often necessary. Selection of the surgical strategy for each patient is highly dependent on the location of the tumor within the skull base, and the surgeon’s preference. For each patient different approaches could have different benefits, and could carry different risks.  For some approaches, a neurosurgeon may work in tandem with an otolaryngologist-head and neck surgeon (ear nose and throat [ENT] surgeon). Regardless of the approach, it is important that the surgeon(s) be experienced in skull base surgery, which is a distinct sub-specialty for neurosurgeons and ENT surgeons.

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Radiation


Once the tumor is removed, if complete resection is not achieved, radiation therapy is usually recommended to reduce the likelihood of re-growth of residual tumor.[7] For patients who have a complete resection radiation is often advised, however the benefit of radiation for this group of patients is debated.[6] [3]

Chordomas can only be controlled with very high doses of radiation – doses that would permanently damage normal tissue. Because of their proximity to vital anatomy such as the brain and spinal cord, which cannot tolerate doses of radiation required to kill chordoma, radiotherapy for chordoma must be highly conformal, meaning focused on the tumor while avoiding surrounding tissue. Several different types of conformal radiotherapy are available.

A type of radiation called proton beam therapy is most often recommended for chordoma patients because it allows delivery of very high doses of radiation to the tumor while minimizing doses to tissues just millimeters away. Another type of particle beam radiation called carbon ion therapy has similar properties to proton beam therapy, but is only available at a small number of centers.

Certain other types of conformal radiation, such as radiosurgery (including Gamma Knife and CyberKnife) and intensity modulated radiotherapy (IMRT) can also be effective, depending on the size and location of the tumor.[6] No direct comparison trials have been performed to establish the optimal form of radiation. However, there is consensus that conventional photon radiation is not beneficial for chordoma patients. [7]

While radiation is usually well tolerated, side effects are possible. For skull base tumors, pituitary dysfunction is common after radiation. Depending on the area radiated, there may also be a risk of damage to the optic nerve, cranial nerves and brain stem, which could cause blindness or paralysis. For tumors of the spine, radiation can also damage to the spinal cord, causing paralysis. Radiation for sacral tumors can cause radiation proctitis, which is damage or inflammation to the colon.

If a tumor returns after initial irradiation, it may or may not be possible to have radiation therapy again. This is because every tissue in the body has a certain life-time maximum tolerance for radiation, beyond which serious injury will occur. Often, delivering enough radiation to effectively treat chordoma exposes vital surrounding structures  to a dose of radiation close to this lifetime maximum. Delivering more radiation to those structures after initial treatment could be very dangerous. The ability to have repeated radiation, therefore, depends on the location of the original tumor, the location of the recurrent tumor, the dose and distribution of radiation that was given initially, and other factors.[5]

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Systemic Therapy


If surgery and/or radiation are not possible, systemic therapy can be used to slow the progression of the disease. Because chordomas tend to be slow growing, they are generally resistant to conventional cytotoxic chemotherapy drugs, which target rapidly dividing cells. There have been a number of published reports of responses to various targeted drugs, however evidence supporting the use of any existing chemotherapeutic agents is limited.

Clinical Trials

Clinical trials can offer patients access to promising new treatments before they become available to the public. In addition to having the potential to benefit from the latest treatments, chordoma patients who participate in clinical trials will help researchers and physicians learn about chordoma and how to treat it more effectively. Before undergoing chemotherapy, it is worthwhile to discuss clinical trial options with your doctor. More information about clinical trials and a list of clinical trials open to chordoma patients is available on our clinical trials page.

To receive email updates about clinical trials that are open to chordoma patients, click here.

Personalized Oncology Services

Increasingly, oncologists are attempting to customize therapies to the unique biology of each patient’s individual tumor (“personalized medicine”) in the hope that the treatments will be more effective and have fewer side effects. Your doctor may perform tests on your tumor to determine which targeted therapies make the most sense for you. In addition, a number of companies offer tumor-profiling services intended to help inform a rational decision about targeted therapies, however the utility of these services is unproven.

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Reviewed 3/1/2012 by:


The information provided herein is not intended to be a substitute for professional medical advice, diagnosis or treatment. Always seek the advice of your physician with any questions you may have regarding your medical care. Never disregard professional medical advice or delay in seeking it because of something you have read on this Website.

References

  1. P. Bergh, L.G. Kindblom, B. Gunterberg, F. Remotti, W. Ryd, and J.M. Meis-Kindblom, "Prognostic factors in chordoma of the sacrum and mobile spine: a study of 39 patients.", Cancer, 2000. http://www.ncbi.nlm.nih.gov/pubmed/10813725
  2. S. Boriani, S. Bandiera, R. Biagini, P. Bacchini, L. Boriani, M. Cappuccio, F. Chevalley, A. Gasbarrini, P. Picci, and J.N. Weinstein, "Chordoma of the mobile spine: fifty years of experience.", Spine, 2006. http://www.ncbi.nlm.nih.gov/pubmed/16481964
  3. D.M. Sciubba, J.J. Cheng, R.J. Petteys, K.L. Weber, D.A. Frassica, and Z.L. Gokaslan, "Chordoma of the sacrum and vertebral bodies.", The Journal of the American Academy of Orthopaedic Surgeons, 2009. http://www.ncbi.nlm.nih.gov/pubmed/19880681
  4. M. Koutourousiou, C.H. Snyderman, J. Fernandez-Miranda, and P.A. Gardner, "Skull base chordomas.", Otolaryngologic clinics of North America, 2011. http://www.ncbi.nlm.nih.gov/pubmed/21978899
  5. B.P. Walcott, B.V. Nahed, A. Mohyeldin, J. Coumans, K.T. Kahle, and M.J. Ferreira, "Chordoma: current concepts, management, and future directions.", The Lancet. Oncology, 2012. http://www.ncbi.nlm.nih.gov/pubmed/22300861
  6. S. Di Maio, N. Temkin, D. Ramanathan, and L.N. Sekhar, "Current comprehensive management of cranial base chordomas: 10-year meta-analysis of observational studies.", Journal of neurosurgery, 2011. http://www.ncbi.nlm.nih.gov/pubmed/21819197
  7. M. Amichetti, M. Cianchetti, D. Amelio, R.M. Enrici, and G. Minniti, "Proton therapy in chordoma of the base of the skull: a systematic review.", Neurosurgical review, 2009. http://www.ncbi.nlm.nih.gov/pubmed/19319583