Chordoma Foundation

Guidelines for newly diagnosed mobile spine and sacral chordoma

The treatment recommendations you find here were developed by the Chordoma Global Consensus Group – a multidisciplinary, international group of over 60 doctors who specialize in caring for chordoma patients. The Chordoma Foundation and the European Society for Medical Oncology brought this group together to define the recommendations for treating chordoma based on all available medical and scientific evidence. The resulting consensus guidelines were published in the medical journal The Lancet Oncology in February 2015.

You can also find these recommendations in our Expert Recommendations for the Diagnosis and Treatment of Chordoma booklet.

Download or request a hard copy of the booklet »

Your first treatments will have a big impact on your life after treatment and the chances of the tumor coming back. In most cases, surgery is recommended for newly diagnosed mobile spine and sacral chordoma. Radiation therapy is generally recommended as well, to help lower the chance of the tumor coming back. A radiation oncologist should be part of your care team from the beginning, to plan with your surgeons for any radiation treatment you will receive before, during, or after surgery.

Surgery

Tumor Donation ProgramYour doctors will need to do some additional tests before you have surgery. Even if you have already had CT or MRI scans, you may need more extensive imaging. This will help your surgeon see the tumor better and plan your surgery. The way surgery is performed is very important.

Learn more about surgery for chordoma »

Sacral tumors

The goal of surgery for sacral chordomas is to completely remove the tumor in one piece (en-bloc) with wide margins of normal tissue surrounding it. Removing the tumor in more than one piece should be avoided if at all possible. The surgeon must be very careful to avoid disturbing or spilling the contents of the tumor during surgery because this can cause it to re-grow or spread.

Read more about surgical margins »

Additionally, if a biopsy was performed, your surgeon should plan to take out the tissue that
was touched by the biopsy needle to remove any tumor cells that may have been left behind.

After surgery to remove the tumor, plastic and reconstructive surgery will likely be required to repair or replace bone or tissue lost during surgery. This should be planned at the time of initial surgery to reduce complications.

What to expect after surgery

Each patient’s recovery after surgery will differ. Pain is common, so a pain management specialist should be part of your care team.

Learn more »

In some cases, surgery for sacral tumors can cause serious side effects including loss of bowel and bladder control, sexual function impairment, and movement problems. Surgeons
can usually predict how severe these side effects will be depending on the location of the tumor and which nerves are involved (see the table below).

If your tumor is located where surgeons cannot reach it or if the side effects of surgery are very serious and unacceptable to you, radiation may be recommended as the only treatment instead of surgery.

However, radiation alone is less likely to successfully control the tumor than surgery and radiation together. Additionally, the high dose radiation needed for treatment can also cause serious side effects later. Talk with your doctors about your options to decide what treatment plan is best for you.

Doctors do not always agree on whether patients whose tumors can be removed by surgery should choose radiation therapy instead of surgery. Therefore, before having treatment you should talk with your doctors about all of your options and understand the risks and benefits of each type of treatment. It is a good idea to get multiple opinions from doctors who have experience treating chordoma patients.

Recommended primary treatment for sacral tumors

The table below shows the recommended primary treatment for chordoma in each part of the sacrum and likely side effects from surgery.

Sacral vertebra Recommended treatment Side effects of surgery
S1 Radiation is an advisable alternative to surgery Side effects are very serious
S2 Dependent on patient preferences and quality of life considerations Serious side effects are likely
S3 Surgery Is S2 nerve roots are not damaged, about 40 percent of people recover from side effects
S4 and below Surgery Most important functions can be preserved

 

Find an expert

To help you find the best care possible we have organized a list of chordoma experts you can search by specialty, location, and key terms.

Search our Doctor Directory »

Mobile spine tumors

The goal of surgery for mobile spine chordomas is to achieve a wide resection, completely removing the tumor in one piece (en-bloc) with wide margins, whenever possible. Removing the tumor in more than one piece should be avoided if at all possible. The surgeon must be very careful to avoid disturbing or spilling the contents of the tumor during surgery because this can cause it to re-grow or spread.

Read more about surgical margins »

Additionally, if a biopsy was performed, your surgeon should plan to take out the tissue that was touched by the biopsy needle to remove any tumor cells that may have been left behind.

An en-bloc resection might not be possible if the tumor has extended into the neck, chest, or behind the abdomen. In this case there may be tumor tissue left behind, and radiation following surgery should be considered. Sometimes radiation may be recommended before as well as after surgery, especially when an incomplete resection is likely. Radiation therapy alone rather than surgery should be considered if the tumor cannot be safely removed, or if the potential side effects of surgery are unacceptable to you.

After surgery to remove the tumor, plastic and reconstructive surgery will likely be required to repair or replace bone or tissue lost during surgery. This should be planned at the time of initial surgery to reduce complications.

What to expect after surgery

Each patient’s recovery after surgery will differ. Pain is common, so a pain management specialist should be part of your care team.

Learn more »

 

Radiation

It is important to have a detailed discussion with your radiation oncologist to understand the type of radiation therapy that is best for you and the short- and long-term side effects of the treatment you can expect.

The most important thing to know about radiation is that high doses are required to control chordoma. Specifically, a dose of at least 74 Gray Equivalents (GyE) is recommended. This dose should be given to any visible tumor as well as any areas where your doctors believe there may be microscopic tumor remaining after surgery. Even if the tumor was completely removed there could still be microscopic tumor cells nearby, and these can grow into tumors if they are not radiated. If an en-bloc resection is achieved the dose of radiation to the areas surrounding where the tumor was can be limited to 70 GyE.

The amount of radiation required to treat chordoma is higher than what healthy tissue can handle. For this reason, the radiation dose must be focused on the tumor while avoiding important nearby structures such as the brain, brainstem, nerves, or spinal cord. Radiation that is highly focused is called conformal radiation. Your radiation oncologist should plan radiation therapy to deliver the necessary dose to the tumor without causing harm to surrounding tissues.

Types of radiation

A type of external beam radiation called particle therapy is generally recommended for treating chordoma because it can be focused most precisely. Two different types of particles are commonly used: protons and carbon ions. It is not known whether there is any difference in effectiveness between proton beam therapy and carbon ion therapy.

See a list of proton beam and carbon ion centers across the world from the Particle Therapy Co-Operative Group »

In some cases, highly focused photon radiation can be a suitable alternative to particle therapy as long as a high enough dose can be delivered to the target area without damaging healthy tissue. Sometimes it may be helpful to combine photon radiation and particle therapy. For all types of external beam radiation, imaging is needed every day of treatment to make sure that the radiation is going to exactly the right place. This technique is called image guidance.

Another method of delivering radiation, called brachytherapy, involves inserting a small amount of radioactive material inside the body during surgery. This method is used infrequently, but can sometimes be helpful for delivering a high enough dose of radiation to the area near the brainstem or spinal cord. When it is used, it is usually given in combination with external beam radiation.

Learn more about the types of radiation used in chordoma treatment »

What matters most about radiation is that a high enough dose is delivered to the area that needs it while a safe, lower dose is delivered to important nearby structures. Whether the right dose can be delivered with a particular type of radiation depends on a number of factors, including the shape of the area being radiated and the location of important structures that must be avoided. In general, the more precisely the radiation can be focused (the more conformal it is) the better.

What to expect during radiation

Side effects from radiation are possible and can vary depending on where your tumor is located. Your radiation oncologist will help get you ready for treatment by preparing your treatment plan and discussing possible side effects.

Learn more »

 

 

Consider palliative care

Palliative care, also called supportive care, can improve the quality of life and well-being of patients dealing with a serious illness by preventing and treating symptoms of the disease or the side effects of its treatment. Palliative care is often confused with hospice care or end of life care, but they are not the same. Hospice care is intended for the end of life period, generally for patients expected to live for less than six months, while palliative care is recommended for patients at any stage of a life-threatening or chronic illness.

Chordoma experts recommend that palliative care be included in all chordoma patients’ care plans from the time of diagnosis, through all stages of treatment, as well as after treatment ends. No matter what treatment you have for your recurrence, palliative care can help address pain, mobility and functional issues, mental and emotional health, nutrition, and many other concerns to help you live well while managing your chordoma.

Read more about palliative care »

 

The information on this page was developed by the Chordoma Foundation in consultation with members of the Chordoma Global Consensus Group. We would like to thank the members of the Chordoma Global Consensus Group for providing their expertise in the development of the original consensus guidelines and their review of this educational content.


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

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