Treatment guidelines for locally recurrent chordoma

The treatment recommendations you find here were developed by the – 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.

Download or request a hard copy of the 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 recurrenceTumor 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 recurrent chordoma

More than half of all chordoma tumors grow again after initial treatment. This is called a recurrence. It is not unusual for chordoma patients to have a recurrence several years after initial treatment. Many patients have more than one recurrence over time.

If the tumor grows back in the same place as the original tumor, this is known as a local recurrenceTumor that has grown back in the same location after treatment.. If it grows back in an area close to where the original tumor grew, this is called a regional recurrenceTumor that has grown back in the area adjacent to where the primary tumor was located.. A regional recurrence is the result of tumor spreading to an area in direct physical contact with the site of the original tumor, such as nearby bones or muscles. When tumor spreads beyond the site of the original tumor to other areas of the body, this is called metastasis or metastatic diseaseWhen tumors have spread to other parts of the body from the original tumor site..

There is no way to know for sure if a chordoma will recur because several factors affect your chance of having a recurrence. These include the size of your original tumor, how much of the tumor was removed during your initial surgery, your age, and the nature of your initial surgery and/or radiation.

Chordomas that recur are difficult to control and are rarely curable. However, there are certain circumstances in which a recurrent chordoma might be cured with appropriate treatment. For those whose chordomas are not currently curable, there are treatment options that can help you live longer with good quality of life.

It is critical to know if a cure is possible or if the best outcomeThe health condition of a person after being diagnosed and treated for a particular disease. Outcome is usually measured at different time points. For example: one-, five-, or ten-year outcome. that can be expected is to extend life or improve quality of life. This knowledge can help you and your medical team pursue a treatment plan that offers the best outcome possible, while avoiding treatments that can expose you to unnecessary or unacceptable risks.

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A Chordoma Foundation Patient Navigator can help you learn about recurrent chordoma and understand the treatment guidelines outlined below so you can have informed discussions with your care team. Our Patient Navigators are available Monday through Friday from 8 AM to 5 PM Eastern Time.

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Finding the right medical team

When confronting a recurrence, 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 a recurrence.

It is important to be evaluated and treated by a multidisciplinary teamTreatment 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:

Radiology
Pathology
Surgery
Radiation oncology
Medical oncology
Palliative care

Individualized treatment plan

Each patient’s situation is different, so you should talk with your doctors about the course of treatment that makes the most sense for you. Your doctors can help you understand all the treatment options that are available to you, and create an individualized treatment plan based on your choices.

Value of multiple opinions

Getting multiple opinions from doctors who have experience with chordoma can help you make the most informed decision about your treatment. The Chordoma Foundation Doctor Directory provides information for doctors who have this experience.

Search our Doctor Directory to find chordoma experts »

Diagnosing a recurrence

In some cases, worsening or new symptoms are the first sign of a recurrence. Other times, new tumor growth is seen on routine follow-up scans. In either situation, your doctor will need to do more tests to learn more about what is happening.

The following flow chart illustrates the steps to take when a recurrence is suspected, as discussed in the sections below.

View/download flow chart as a PDF »

Imaging

Your doctor will need to order imaging tests to help determine if your chordoma has recurred. If it has, the imaging will provide more information about the new tumor growth, such as the size and exact location.

The first imaging test that should be done is magnetic resonance imagingA type of imaging scan that is used initially to help diagnose chordoma, as well as during follow up to check for recurrence or metastasis., or MRI. Your MRI should be done with contrastA dye or other substance that is injected into a vein to help areas of the body show up more clearly on scans like MRIs and CTs.. Contrast agents are special liquids injected into a vein which help organs and tissues appear more clearly on MRI images. Computed tomographyA 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 CT, scans may also be ordered.

Biopsy for recurrence

If your doctor cannot determine from imaging tests whether your tumor has recurred, 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. 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 core-needle biopsyA 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 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., both of which may require different treatment approaches. Poorly differentiated chordomas often lose expression of a protein called INI1A 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 conventional chordomaThe 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 recurred 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.

Questions to ask about treatment

To help you get the best care possible, we developed lists of questions with the input of chordoma patients and caregivers that can help you think about what you want to ask your doctors.

View question lists »

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.

Important points to consider

For any tumor location, the decision to have surgery alone, surgery plus radiation, or radiation alone should be made together with your doctors, based on your individual situation. There is currently no published data to support the same recommendations for all patients.
If you previously had high-dose radiation near the location of your recurrence, your doctors should take particular caution when planning for surgery because there is a higher risk of complications and wound healing with tissue that has been treated with radiation.
Treatments for recurrent chordoma may cause serious side effects, so it is important to carefully consider and discuss with your treatment team the risks and benefits of any treatment option, along with how the treatment will impact your overall health and quality of life.

The following flow chart illustrates the steps to take when determining your treatment options, as discussed in the sections below.

View/download flow chart as a PDF »

Curative treatments versus treatments to delay tumor progression

When a recurrence is confirmed, it is important for your doctors to perform a full evaluation to help determine your options for treatment and whether the goal of treatment is to cure the disease or to delay tumor progression as long as possible. You and your doctors should consider a number of factors when making these determinations, including:

Where the tumor has recurred
What treatments you have had in the past
What side effects might be caused by the treatment
What risks would be acceptable to you

Factors such as your age, other health conditions you have, and your ability to care for yourself and perform daily activities are also important for you and your treatment team to discuss.

A cure for recurrent chordoma – meaning that the tumor is permanently controlled – is only possible in a small number of cases. However, it is important to recognize when the potential for a cure exists, and use this knowledge to help guide treatment decisions. The two most important factors that determine whether the goal of treatment is to cure the disease, which is also called curative intentTreatments that are given for the purpose of curing a disease or providing long-term survival., or whether the goal is to delay tumor progression as long as possible are:

The extent of the recurrence
Whether high-dose radiation is possible

Extent of recurrence

In order for your doctors to determine exactly where the tumor has recurred and to what degree, you will need to have more extensive imaging scans. A CT scan of your whole body should be done, as well as an MRI of your entire spine, to determine whether the tumor has spread. Your doctors should also compare your new MRI and CT images to those that were taken after your last treatments. This will help them determine how much of what is seen in the new images is recurrent tumor, and what might be treatment sequelaeA chronic condition or injury resulting from treatment for a disease. (changes that are a result of treatment).

The results of this pre-treatment imaging will show your doctors whether you have:

An isolated recurrenceA single recurrent tumor at or near the site of the original tumor., which is a single tumor at or near the site of the original tumor
A multifocal recurrenceMultiple tumors at or near the site of the original tumor that have regrown after treatment., which is multiple tumors at or near the site of the original tumor
Or metastatic disease, which is one or more tumors in other parts of the body in addition to the recurrent tumor

An isolated recurrence may be curable under certain conditions. In this case, high-dose radiation and surgery should be considered first to give you the best chance for a cure.

Multifocal recurrences are very unlikely to be cured and currently there is no known cure for metastatic disease. However, multiple treatment options are available to control or temporarily stop tumor growth, as well as to alleviate symptoms. In many cases, these options can control the disease for multiple years while allowing patients to maintain their quality of life.

If the tumor does not appear to be growing and you are not experiencing symptoms, your doctors might recommend a period of observation followed by more imaging to determine what treatment is necessary.

Possibility of high-dose radiation

For patients with isolated recurrences, high-dose radiation given with or without surgery provides the best chance of achieving a cure or long-term disease control. 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) to the entire tumor is required, so if you have an isolated recurrence an important first step is for your doctors to determine whether this amount of radiation can be safely delivered.

There is a lifetime maximum amount of radiation that healthy tissues like bone, nerves, and arteries can receive without being harmed. If you have never had radiation, you can very likely receive high-dose radiation to treat new tumor growth.

If you have had radiation in the past and the recurrence is in an area that was radiated, it will likely not be safe for you to have high-dose radiation again. However, if the recurrence is outside or at the edge of the area that was previously radiated, it may be possible to have additional high-dose radiation. Every case is unique, so it is important for a radiation oncologist with experience treating chordoma to review your previous radiation plans in relation to the current tumor growth.

For patients with multifocal recurrences, metastatic disease, or if radiation cannot be given at a dose of at least 74 GyE, a lower dose of radiation may still be able to help control growth of the tumor or alleviate symptoms.

Tests to get before treatment

No matter the extent of your recurrence, your doctors should perform a complete physical and neurological assessment to help prepare for treatment. This includes discussing any symptoms you are experiencing and how fast they are progressing. When evaluating your pain levels, your doctor should note what pain is related to previous treatments and what is related to the new tumor growth. If you have a skull baseThe bones at the bottom part of the skull that separate the brain from other structures. The clivus is one of the bones of the skull base. chordoma, you might also need evaluations of your vision, hearing, and endocrine system function. These tests will help your doctors determine the effects of the new tumor growth and consider this information in treatment planning.

The treatment recommendations for isolated recurrences differ based on whether your tumor is located in the skull base, the 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., or 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.. The next sections discuss in detail the pathways that determine the recommendations for each location.

Go to guidelines for recurrent skull base tumors »

Go to guidelines for recurrent mobile spine and sacral tumors »

Palliative and supportive 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.