Guidelines for newly diagnosed mobile spine and sacral chordoma
 The treatment recommendations you find here were developed by the Chordoma Global Consensus GroupA multidisciplinary, international group of over 60 doctors who have extensive experience caring for chordoma patients. The group is responsible for developing and publishing consensus guidelines, based on all available medical and scientific evidence, for the treatment of primary and recurrent chordoma. – a multidisciplinaryTreatment that involves a team of physicians from various disciplines. In the case of chordoma, these disciplines include sarcoma or bone pathology, radiology, spine surgery or skull base surgery, otolaryngology, radiation oncology, medical oncology, and palliative care., 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 spineThe parts of the spine not including the sacrum. The cervical spine (neck), thoracic spine (upper back), and lumbar spine (lower back) are the parts of the 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
Your doctors will need to do some additional tests before you have surgery. Even if you have already had CTA type of imaging scan that is used to help diagnose chordoma and can also be used to help guide the needle during a biopsy. They are also referred to as CT scans or “CAT” scans. or MRIA type of imaging scan that is used initially to help diagnose chordoma, as well as during follow up to check for recurrence or metastasis. 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.
Additionally, if a biopsyA procedure that uses a needle to remove a small tissue sample from the tumor to be tested in order to make a diagnosis. 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 surgeryEach patient’s recovery after surgery will differ. Pain is common, so a pain management specialist should be part of your care team. |
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 sacrumThe five vertebrae at the base of the spine near the pelvis, and between the two hip bones. These bones are commonly referred to as S1-S5. and likely side effects from 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 |
|
Mobile spine tumors
The goal of surgery for mobile spine chordomas is to achieve a wide resectionRemoval of the entire tumor with at least 1 millimeter of healthy tissue surrounding the tumor., 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 resectionDuring surgery, removal of the entire tumor in one piece without cutting it into smaller pieces. 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 resectionVisible tumor tissue has been left behind after surgery. 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 surgeryEach patient’s recovery after surgery will differ. Pain is common, so a pain management specialist should be part of your care team. |
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 EquivalentsThe abbreviation for gray equivalents, which refers to the unit of measurement for an absorbed dose of radiation. (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, brainstemThe brainstem is the lower part of the brain connected to the spinal cord. The brainstem relays all signals to and from the brain and the body and is responsible for maintaining consciousness, breathing, and heartbeat., nerves, or spinal cord. Radiation that is highly focused is called conformal radiationTypes of radiation that can focus the beam of radiation very accurately on the tumor while minimizing the amount of radiation that reaches the surrounding healthy tissue.. 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 radiationRadiation that is delivered from outside the body. called particle therapyA type of external beam radiation that uses beams of protons, neutrons, or positive ions for the treatment of cancer. 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 therapyA type of particle therapy that uses beams of protons to kill cancer cells. and carbon ion therapyA type of particle therapy that uses beams of charged carbon ions to kill cancer cells. Carbon ion therapy can deliver high doses of radiation to a tumor while sparing surrounding normal tissue..
In some cases, highly focused photonA type of external beam radiation that uses x-rays to kill cancer cells. 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 guidanceThe use of frequent imaging, such as MRI or CT, during radiation treatments to help direct the radiation to the right place..
Another method of delivering radiation, called brachytherapyA type of radiation therapy in which a small amount of radioactive material is placed inside the body to kill cancer cells., 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 radiationSide 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. |
Consider palliative carePalliative care, also called supportive careCare given to improve the quality of life of patients who have a serious or life-threatening disease., 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 hospiceA specific type of supportive care that is provided to patients who are near the end of life and have stopped treatments meant to cure or control their disease. The main goal is to help patients feel as comfortable as possible, and to support both patients and family members through the end of life process. If a treatment option becomes available, patients can be taken out of hospice care and receive that treatment. 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 recurrenceTumor that has grown back after initial treatment. Recurrences can be isolated or multifocal, local or regional., 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. |
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.