Treatment for advanced and metastatic disease
|The treatment recommendations you find here were developed by the A 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 multidisciplinary, international group of more than 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 Annals of Oncology in June 2017. The full paper can be accessed here.
You can also find these recommendations in our Expert Recommendations for the Treatment of Recurrent Chordoma booklet.
A series of flowcharts helps to illustrate the treatment guidelines and walk you through the suggested tests, treatment options, and follow-up steps recommended for different Tumor that has grown back after initial treatment. Recurrences can be isolated or multifocal, local or regional. scenarios. You’ll find the flowcharts and supporting information throughout the page below. You can also download the collection of flowcharts as a PDF.
Understanding advanced and metastatic disease
Chordomas are considered advanced when an A single recurrent tumor at or near the site of the original tumor. cannot be treated with high-dose radiation or when the recurrence 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 When 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 Surgical 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.
Finding the right medical team
When confronting advanced or metastatic disease, finding the right medical team is a critical first step. Depending on your situation, the medical center where you were treated initially may or may not be best suited to diagnose and treat your case.
It is important to be evaluated and treated by a Treatment 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. of specialists who have substantial experience treating chordoma. Teams with this experience are typically only found at larger hospitals, sometimes called referral centers, which see large numbers of patients.
Your care team should include specialists with experience diagnosing and treating chordoma in the following areas:
- Radiation oncology
- Medical oncology
- Palliative care
Biopsy for metastatic disease
If your tumor has spread to other areas of the body, a A procedure that uses a needle to remove a small tissue sample from the tumor to be tested in order to make a diagnosis. may be recommended, if it can be done safely. A biopsy may also be done if your tumor is growing unusually fast or if your doctors think you may have developed a new form of cancer that is not chordoma. A A type of biopsy performed with a wide needle. Also called a core biopsy. is recommended and should be performed by doctors who have experience with chordoma. The tissue sample should be examined by a pathologist with experience diagnosing chordoma.
If your tumor is growing quickly, the tissue sample should be analyzed to determine if the tumor has become A 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 A 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., both of which may require different treatment approaches. Poorly differentiated chordomas often lose expression of a protein called A protein that regulates the growth of certain tumor cells. Poorly differentiated chordoma tumors are characterized by loss of INI1. Also called SMARCB1., and sometimes dedifferentiated and The most common histological subtype of chordoma, also called classic chordoma. It is composed of a unique cell type that looks like notochord cells and can show areas of chondroid dedifferentiation. can as well.
If your doctors are unsure whether your tumor has spread and you are not experiencing any symptoms, it may be possible to wait and do imaging of the area again after a period of observation rather than performing a biopsy.
Medical Advisory Board recommendations
The Chordoma Foundation Medical Advisory Board suggests that chordoma patients under 35 years of age, or whose tumors are growing unusually fast, have their tumors tested for loss of INI1, either through immunohistochemistry (IHC) or genomic testing. If tissue is not available for testing, the possibility of a biopsy to obtain tissue for this testing should be discussed with a doctor who has experience treating chordoma, carefully weighing the potential risks of biopsy. Interpretation of the testing should be done by a pathologist who has experience diagnosing chordoma.
Determining treatment options
There are a number of factors that will affect what treatment options are available to you. Each patient’s situation is unique and must be evaluated individually. Decisions about treatment should balance side effects, quality of life, and how well the treatment is expected to work. This will help ensure that you get the best treatment for your tumor with as little impact on your quality of life as possible.
This flowchart illustrates the possible treatment options for advanced and metastatic disease as discussed in the sections below.
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 The 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 Treatments 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 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 A 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 A 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 A 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.
There is some evidence to suggest that ablative therapies like A type of ablative therapy that uses a needle to deliver extreme cold to a tumor in order to kill cancer cells., A 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 A 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.
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 Systemic 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 A 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 differentiated or dedifferentiated chordoma have benefitted from Cancer of bone and connective tissue such as cartilage, fat, muscle, and blood vessels. Chordoma is a type of sarcoma. chemotherapy regimens.
Dealing with pain and other side effects
Palliative care, also called Care 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 A 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
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.