Treatment for advanced and metastatic disease

Chordomas are considered advanced when an isolated recurrenceA single recurrent tumor at or near the site of the original tumor. cannot be treated with high-dose radiation or when the recurrenceTumor that has grown back after initial treatment. Recurrences can be isolated or multifocal, local or regional. is multifocal. Multifocal recurrences involve multiple tumors in the area of the original tumor location. Metastatic chordoma is one or more tumors that have spread to other parts of the body.

Advanced disease is very unlikely to be cured and currently there is no known cure for metastatic diseaseWhen tumors have spread to other parts of the body from the original tumor site.. However, multiple treatment options are available to control or temporarily stop tumor growth, as well as to alleviate symptoms. These include debulkingSurgical removal of part of a tumor. surgery, low-dose radiation, ablative therapies, and systemic therapies. With the right care, your tumor may be controlled for many years while also maintaining a good quality of life.

This flowchart illustrates the possible treatment options for advanced and metastatic disease as discussed in the sections below.

View or download the flowchart as a PDF »

Debulking surgery

In a debulking surgery, some of the tumor is removed in order to help relieve or avoid symptoms caused by compression of important structures such as nerves, spinal cord, or the 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.. It can also be used to separate healthy tissues from your tumor so that you can more safely undergo radiation if necessary. Debulking surgery is recommended only in certain cases because it is not curativeTreatments that are given for the purpose of curing a disease or providing long-term survival. and the chance of serious side effects increases with each surgery.

How do I decide?

Every patient’s situation is unique. If you have advanced or metastatic disease, it is important to discuss all of these options in detail with your medical team and your family, weighing the risks and benefits of each option for your situation. The treatment options listed here involve various types of doctors, so it is important to consult with doctors who specialize in these treatments and have experience treating chordoma patients. For some patients, just one type of treatment might be the best decision, while for others a combination of treatments might provide the best option.

Low-dose radiation

Low-dose radiation may be given alone or following a debulking surgery. It is used to slow tumor progression or relieve symptoms. Your doctor should consider all previous radiation treatments and the location of the recurrent tumor to determine whether radiation is safe for you.

It may also be possible for you to have stereotactic radiosurgeryA type of external beam radiation that uses special equipment to position a patient and precisely deliver radiation to tumors in or near the brain, over a small number of treatments. (SRS) or stereotactic body radiation therapyA type of external beam radiation that uses special equipment to position a patient and precisely deliver radiation to tumors in all parts of the body except the brain, over a small number of treatments. (SBRT), which are both types of hypofractionated radiation. When hypofractionationA radiation treatment technique that gives larger doses of radiation over a smaller number of sessions. The total amount of radiation given is less than when standard fractionation is used, but the effect is the same. is used, the total amount of radiation given is less but the effect is the same as standard fractionation.

Ablative therapy

There is some evidence to suggest that ablative therapies like cryoablationA type of ablative therapy that uses a needle to deliver extreme cold to a tumor in order to kill cancer cells., radiofrequency ablationA procedure that uses a needle to deliver energy to the tumor, causing it to heat up, and killing the cancer cells within it. (RFA), or high-intensity focused ultrasoundA type of ablative therapy that destroys cancer cells with high-frequency sound waves delivered from outside of the body. (HIFU) can be used to help manage symptoms caused by recurrent tumors. Cryoablation destroys cancer cells with extreme cold, while RFA uses heat. Both are delivered through small probes inserted directly into the tumor. HIFU destroys cancer cells with high-frequency sound waves delivered from outside of the body. More research is needed to determine how effective these procedures are for treating chordoma, but they are options you can discuss further with your doctors.

Systemic therapy

There are no drugs currently approved by any government regulatory agencies for the treatment of chordoma. However, evidence has shown that certain types of systemic therapies such as targeted therapy and immunotherapySystemic therapies that are designed to teach the immune system how to find and destroy cancer cells. that are commonly used to treat other cancers may also help patients with recurrent chordoma.

Conventional chemotherapyA type of systemic therapy that is designed to kill rapidly dividing cancer cells. is typically not effective in treating chordoma. However, some patients with poorly differentiatedA histological subtype of chordoma that is more aggressive and usually grows faster than the conventional type. It is more common in children and young adults, and is often characterized by loss of the INI1 protein. or dedifferentiatedA histological subtype of chordoma that is more aggressive and usually grows faster than conventional chordomas. Dedifferentiated chordomas occur in only 5 percent of patients, can have loss of the INI1 gene, and are more common in pediatric patients. chordoma have benefitted from sarcomaCancer of bone and connective tissue such as cartilage, fat, muscle, and blood vessels. Chordoma is a type of sarcoma. chemotherapy regimens.

Chordoma Foundation Medical Advisory Board recommendations

Although each patient’s situation is unique, in general, our Medical Advisory Board (MAB) recommends that patients considering systemic therapyThe use of drugs that spread through the body to kill cancer cells. Also called chemotherapy or drug therapy. treatment options do so in the following order of priority, in consultation with a chordoma-experienced medical oncologist:

1. Chordoma-specific clinical trials
Start by considering trials designed specifically for chordoma patients. These trials are likely to have strong scientific justification and be conducted by teams with significant experience caring for chordoma patients.

2. Other relevant clinical trials recommended by an experienced physician
If you are not eligible for any chordoma-specific trials, ask your oncologist about other clinical trials that may be open to you.

3. Off-labelThe practice of prescribing drug treatments that are not approved by government agencies to treat a particular disease. Doctors are allowed to prescribe drugs off-label if they believe it is in the best interest of the patient. therapy with evidence of clinical benefit for chordoma patients
While there are currently no drugs approved for the treatment of chordoma, some drugs that are approved for the treatment of other cancers have shown modest activity in chordoma patients. In some countries, doctors can choose to prescribe these drugs to chordoma patients if they believe they will benefit the patient. This is called off-label use. If you are not eligible for any clinical trials, consult with your oncologist about off-label use of approved drugs that have been used to treat chordoma patients.

Consider palliative care

Palliative 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 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.

References and further information

Stacchiotti S, Gronchi A, Fossati P, et al. Best practices for the management of local-regional recurrent chordoma: a position paper by the Chordoma Global Consensus Group. Ann Oncol. 2017 Jun 1;28(6):1230-1242. doi: 10.1093/annonc/mdx054.

The information on this page was developed by the Chordoma Foundation in consultation with members of 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.. 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.