Chordoma

A rare, slow-growing bone cancer. It starts in the skull base, the spine, or the tailbone. What the name means, and why the first operation matters more than most. Which signs mean getting help now, and what treatment actually involves.

The short version

Chordoma is a rare bone cancer. It grows in the bones down the middle of the body: the base of the skull, the spine, and the tailbone.

It starts from cells left over from before you were born. Those cells helped build your spine. In most people they disappear. In a few people some stay, and very rarely one turns into this tumor.

It usually grows slowly. That is why it is often found late. Unless your team has told you that something is urgent, it is also why most people have time to get to the right team before deciding anything.

The first treatment matters more than it would for most tumors. Getting to a center that treats chordoma, before a biopsy, changes what is possible later.

Some signs mean getting help the same day, and a few mean going in now. They have their own section below.

What is a chordoma?

It is cancer. The word written on your paperwork will be malignant. Cleveland Clinic puts it plainly: every kind of chordoma counts as cancer.

It belongs to a family called sarcoma. Those are cancers that start in bone or the soft tissue around it, rather than in an organ.

It starts from the notochord. Before you were born, a soft rod ran down the middle of your body. It helped your spine form. It normally disappears well before birth. In a small number of people a few of those cells stay behind, in the bones of the skull base or the spine.

That is why this tumor sits in the middle. The rod was the midline, so its leftovers are in the midline, so the tumor is too. Almost everything else on this page follows from that one fact.

It is rare. About 300 people are told they have one each year in the United States, which works out at about one person in a million.

Is it cancer? What does its grade mean?

Yes, it is cancer. And there is no simple grade number for it.

That surprises people, because grade is the first thing many readers look for. Here is the honest picture.

The book that names brain and spine tumors lists chordoma and prints no grade beside it. That book is the WHO classification. If your report says WHO, it means the same book. It sorts this tumor into a family called notochordal tumors. It does not give it a 1, 2, 3 or 4 the way it does for many others.

Clinical references call it low grade. That phrase describes the appearance of the cells under a microscope, and nothing else. Cells resembling normal ones earn that label.

Low grade here does not mean mild, and this is the part that matters. The same references that call it low grade say in the same sentence that it grows into the bone and tissue right beside it, and that it often comes back. Slow is not the same as harmless.

So grade is not the useful number on your report. What your team actually works from is where it sits, how much of it can be taken out, and what is next to it. Those come further down.

If you see a Roman numeral on older paperwork, that is only an older way of writing a grade. The rules changed in 2021 and ordinary numbers are used now.

Where does it grow, and why does it cause these symptoms?

Three places, all down the middle. The base of the skull, the spine, and the sacrum, which is the bone at the bottom of your spine behind your hips.

About a third start at the skull base, about a third at the tailbone end, and the rest in the spine in between. Different studies count these a little differently. Treat them as rough shares.

Your symptoms come from what the tumor is pressed against, not from the tumor itself. That is the whole of it. A tumor this size in a roomy place might cause nothing for years. The same tumor against a nerve causes trouble early.

Looking it up by place instead of by name. Where your tumor is, and what that changes starts from the spot rather than the diagnosis. Two parts of it are written for the head: its list of places, and the signs it says to call about. It says so both times. The rest of it works wherever your tumor is. If yours is at the base of your skull, the entry called the floor of the skull is the one that names the word your report may use.

At the base of the skull

The bone it grows in sits in a crowded place. It is called the clivus. It lies in front of the brain stem and behind the back of your throat. The nerves that move your eyes, carry feeling to your face, and run your voice and swallowing all pass close by.

Double vision is often the first thing people notice, and there is a reason it is that one. One of the nerves that moves the eye runs a long way along the surface of that exact bone before it gets where it is going. A long exposed run means it is often the first to be squeezed. That nerve turns one eye outward, so when it stops working properly you see two of things, usually worse when you look to the side.

Other things come from the same crowd of nerves: headache, numbness or pain in the face, changes in hearing, a voice that changes, and trouble swallowing. A blocked or stuffy nose that does not clear up can be part of it too.

Sometimes it blocks the fluid around the brain. The brain sits in a watery fluid that flows past it and drains away. A tumor sitting across that path can block the drain, and the pressure inside your head then climbs. That can bring headaches, changes in how you think or behave, and trouble focusing your eyes.

In the spine and at the tailbone

Lower down, the tumor presses on the cord and on the nerve roots coming off it. That brings pain in the back or neck, pain running down a leg, numbness or tingling, and weakness.

At the tailbone end, the nerves it meets are the ones that run your bladder, your bowel, and sexual function. So the symptoms there are different, and they are ones people find hard to raise: trouble peeing or pooping, numbness through the area you sit on, and changes in sex. Sometimes a lump can be felt.

Tumors down there are often quiet until they are large, because there is more room before anything gets pressed.

What symptoms does it cause?

They are rarely obvious, and that is not your fault. One reference says plainly that the symptoms of chordoma are not specific. They look like ordinary problems: back pain, a stuffy nose, vision that is a bit off.

The ones people most often notice:

• Double vision, or other changes in how your eyes work together.
• Headache, often felt at the back of the head or behind an eye.
• Numbness or pain in the face.
• Changes in hearing, voice, or swallowing.
• A blocked nose that does not clear.
• Pain in the back or neck, or pain running down a leg.
• Numbness, tingling, or weakness in an arm or a leg.
• Trouble controlling peeing or pooping, or numbness where you sit.

Many people are unwell for months before anyone lands on the answer. That is normal for this tumor and it is written about openly. It is slow, it is rare, and its early signs belong to a dozen more common things.

Something being slow is not a reason to leave it. If any of these are new, or are getting worse, the Chordoma Foundation's advice is simply to tell your doctors.

The chordoma signs that mean getting help now

Where this advice comes from is worth knowing before you read it. We looked through the chordoma organizations, and the chordoma pages on the big hospital sites, and not one page about this tumor says when to head for an emergency room. So nothing below is a chordoma rule. Each one comes from a page about the warning sign itself, and each is credited to the people who wrote it.

New weakness in your legs, numbness through your legs or belly, or losing control of peeing or pooping is an emergency. Call 911, or get to the closest emergency room. Pressure on the spinal cord is treated as an emergency in cancer care, and the American Cancer Society warns that without fast treatment a person can be left paralyzed. Of everything here, this is where waiting costs most.

The same signs at the tailbone end have their own name, and the same answer. Doctors call it cauda equina syndrome, and it is worth saying that name out loud if you ever have to describe this at a front desk. When the nerves at the bottom of the spine are squeezed, Cleveland Clinic says to visit an emergency room immediately. Getting treated sooner lowers the chance of lasting trouble. More than one in four people with a tumor in the sacrum have either muscle weakness or this nerve picture, so it is not a rare thing to warn about.

Double vision that comes on suddenly is different from double vision that creeps in. Cleveland Clinic says to go to an emergency room for double vision that does not settle within a few hours. The same goes for double vision that comes with eye pain, dizziness, weakness, slurred speech or confusion. With this tumor the slow pattern is the usual one, and that means phoning your team instead. Both halves are true, and they are not the same situation.

Signs of rising pressure inside the head are an emergency in their own right. Cleveland Clinic says so plainly and says to go to the nearest hospital. Bad headaches that are worse in the morning or lying down, throwing up with them, being much less alert than usual, or new trouble with your vision all belong on that list.

Before you read the list below, two things about it. It is written for brain tumors in general, so some of it will not be your situation: this tumor does not usually cause seizures, and it does not swell the brain around it the way a tumor growing inside the brain does. If you have ever had a seizure, that ambulance rule still covers you. The parts about pressure building up, and about a fever while you are having chemotherapy, do apply. So does the fever rule for the weeks after an operation, even though yours may have gone through your nose rather than through an opening in the skull.

When to call for help right now

Some things should not wait for the next appointment.

Call an ambulance for any of these. Call 911, or your local emergency number:

• A first ever seizure.
• A seizure that lasts more than five minutes.
• One seizure right after another, with no waking up in between.
• Someone who cannot be woken.
• Trouble breathing, or someone who seems to be choking.
• Suddenly not being able to speak, move one side, or see.

A seizure like the ones you usually have, that stops on its own, is not an ambulance call. What to do during a seizure draws the line.

Call your team the same day for any of these:

• A headache much worse than usual, or one that wakes you from sleep.
• Throwing up again and again.
• New weakness in the face, an arm or a leg.
• New trouble speaking, or understanding what people say.
• New confusion.
• Sleeping much more than being awake.
• A sudden change in your vision.

If you are having chemotherapy, a fever is its own rule. It means calling your team right away, at any hour, not waiting for the morning. An infection during chemotherapy can get dangerous fast, and it is treatable when it is caught early. The fever rule explains why, and what number your team will give you.

In the weeks after brain surgery, a fever is its own rule too, especially with a stiff neck or a bad headache. What comes after a craniotomy has the rest of that list.

If you have a shunt, the signs it has stopped working are their own rule. For you, everything on the same-day list above is a right-away call instead, and so are a few other signs. Get help right away, at any hour: call your team, or go to the emergency department. Signs a shunt is not working has the full list.

If you are not sure whether to call, call. Nobody on that team minds a phone call that turns out to be nothing.

Same day means today, not the next clinic appointment. Ask your team now for the after-hours number, and put it somewhere you will find it at two in the morning rather than somewhere sensible.

What to do during a seizure has the step-by-step, and getting help now is the short version to keep on a phone.

How do doctors find out it is this?

A scan can suggest it. Only tissue can name it.

You will usually have both an MRI and a CT. They answer different questions. The MRI is the best way to see the tumor itself and what it is touching, such as nerves and blood vessels. The CT scan shows what it has done to the bone. Teams normally want both.

Then a small piece is taken and looked at. That is a biopsy, and for this tumor how it is done matters as much as that it is done.

If you read one paragraph here, read this one. Chordoma can shed cells along the track a needle makes. The Chordoma Foundation says a biopsy for a tumor in the spine or sacrum should be done from the back, with the needle inside a tube, to stop cells spreading along its path. Surgeons call that seeding. The track itself is normally cut out later, during the operation.

The Chordoma Foundation also says to avoid having a biopsy or surgery outside a referral center. A biopsy that is not carefully planned can make the tumor harder to treat later.

So the single most useful move is about where you go, not how fast. The advice is to get to a hospital or network that has experience with chordoma as soon as it is suspected. That is not an instruction to rush to an emergency room. It is an instruction about which door to walk through first.

One lab test names it. Chordoma cells carry a protein called brachyury (say: bra-KEE-ur-ee). Nearly all chordomas have it and the look-alikes do not, so staining for it is what separates this tumor from the things it resembles.

What do the words on my report mean?

Your report describes cells, not your future. Here are the words most likely to be on it.

Physaliphorous cells. A long word for a simple observation. Cleveland Clinic describes them as looking bubbly. Seeing them is part of how the diagnosis is made.

Brachyury, or TBXT. The protein above, and the gene that makes it. A positive result supports chordoma and is the main thing that rules out its look-alikes.

Triple positive. An older shorthand. This tumor was first described as staining for three things at once. Brachyury is the modern, decisive one.

Margin. The healthy tissue taken out around the tumor. Your report may say:

• Wide. The whole tumor came out with at least a millimeter of healthy tissue all round it. That is the best result.
• Marginal. Less than a millimeter of healthy tissue, but nothing visible left behind.
• Intralesional. Visible tumor was left, or the tumor was cut into and cells spilled.

The type. Most chordomas are the conventional kind. Two rarer kinds behave differently: one called poorly differentiated, and one called dedifferentiated.

One gene result changes the plan, and it is worth asking about. The poorly differentiated kind has lost a gene called SMARCB1, also written INI1. The Chordoma Foundation's medical advisory board suggests testing for that loss in anyone under 35, or whose tumor is growing unusually fast. Those tumors are sometimes treated differently.

The rules for naming brain tumors changed in 2021. If your paperwork was written before that, some of its words are no longer used.

This matters more than it sounds. People read an old report next to a new one, see two different names, and think somebody made a mistake. Usually nobody did. The tumor did not change. The way doctors write it down changed.

Three changes affect almost every report.

• Roman numeral grades, such as grade II or grade IV on an older report. These are now written with ordinary numbers instead: grade 2, grade 4. Nothing about the grade itself changed. Only the way of writing it did.
• Gene results are now part of the name. Older reports named a tumor mostly by how the cells looked. A name written now usually has a gene result built into it. That is why a newer report can look longer and more technical than an older one about the same tumor. You may also see NOSNOS: Short for "not otherwise specified". It means the gene tests needed to name the tumor fully were not done or could not be done. Ask your team whether they can be. See the glossary or NECNEC: Short for "not elsewhere classified". The tests were done, but the results do not match any named tumor type. Ask your team what it means for your plan. See the glossary written next to your diagnosis. These say something about the testing, not about how serious the tumor is. NOS means the tests needed to be more exact were not available. NEC means testing was done, but the results did not fit any defined group neatly.

Seeing an older name on your own report does not mean it was wrong. It means it was written under the old rules. If you are holding an old report and a new one and they do not match, that is a good thing to take to your team and ask about.

Older names, and names that are not this tumor

Chondroid chordoma is not a separate diagnosis any more. It was moved to sit inside conventional chordoma. If that phrase is on your paperwork, nothing about your tumor changed. The way of grouping it did.

You may see "chordoma, NOS" or "malignant chordoma" on a form. Neither is a different disease. All chordoma is cancer, so "malignant" adds nothing, and the NOS part is the one explained just above.

Parachordoma is an old name and it is not this tumor. It was used decades ago for something that is now grouped with a different family altogether. It does not carry brachyury, which is how the two are told apart.

Some names sound like this one and are something else entirely:

• Chondrosarcoma. The important one. It also turns up at the skull base and can look similar. It starts in cartilage rather than notochord leftovers, it usually sits off to the side rather than in the midline, and it does not carry brachyury.
• Chordoid meningioma and chordoid gliomaglioma: A tumor that starts in the glial cells. Those are the support cells of the brain and spinal cord. Gliomas are given a grade from 1 to 4. The grade says how the tumor is expected to act. See the glossary. Both share part of the word and neither is a chordoma. One is a kind of meningioma; the other grows in a fluid space inside the brain.
• Ecchordosis physaliphora and benign notochordal cell tumor. Both are non-cancerous leftovers of the same rod this tumor comes from. The first is described as a benign lesion, and the second is usually just watched rather than treated. Being told you have a small notochord remnant is not being told you have cancer.

If two of your reports disagree, bring them both to your team. That is a good question to raise, not an awkward one.

How is it usually treated?

For most people it is surgery, then radiation. Drugs have a smaller part in this tumor than in most cancers.

Where the tumor is changes what surgery can aim for, and the two are genuinely different. This is the part most likely to be flattened into one rule, so it is split here.

If it is in your spine or at your tailbone

The aim is to take it out in one piece, with a rim of healthy tissue all the way round. Surgeons call that en bloc removal.

The reason is the seeding above. Taking it out in pieces, or cutting into it, can spill cells into the space around it, and those cells can grow.

At the tailbone end this is a hard trade and you should hear it early. The nerves that run your bladder, bowel and sexual function pass right through there. How high up the tumor sits decides how much of that function is at risk, and your team can usually tell you beforehand. The Chordoma Foundation is direct about it: people can be left with loss of bladder and bowel control, changes in sexual function, nerve pain, and trouble moving.

That is not the end of the story. Some of it can come back over time, and there are specialists whose whole job is helping with exactly these problems. Ask to meet them early rather than late.

If it is at the base of your skull

Here, taking it out in one piece is often not possible, and that is not a failure. The expert consensus group says so directly: at the skull base, removal may have to be piecemeal. There is brain stem, there are big blood vessels, and there are the nerves for your eyes, face and swallowing, all in the way.

Most of these operations go through the nose. A surgeon passes a small scope through the nostril and works through that natural corridor. Some tumors are reached from the side of the head instead.

Radiation, for both

Chordoma needs an unusually high dose, and that single fact explains most of what follows.

The trouble is what sits next to it. The dose this tumor needs is more than the brain stem, the nerves to your eyes, or the spinal cord can safely take. So the beam has to be shaped very tightly, or it has to stop.

That is the real argument for proton beams. Protons put most of their energy at a set depth and then stop, instead of carrying on through. Carbon ion beams work in a similar way.

Be careful with the word better. The Chordoma Foundation recommends the same dose target for particle beams and for X-ray treatments alike. The requirement is the dose, not the machine. Reviews comparing them rate the evidence as low or very low quality, and found no clear difference in how long people lived. Proton therapy goes through that question properly.

Carbon ion is not offered in many places. The Chordoma Foundation's own list of countries offering it does not include the United States. That is one organization's list, and it may not be current, so ask your team if it matters to you.

Practically, it is a long haul. Around 35 to 40 sessions over six to eight weeks is usual. For many people that means moving near a center, or a long daily commute, for about two months. Radiation therapy covers what the daily visits are actually like.

Drugs, and trials

Standard chemotherapy does not usually work on this tumor, and no drug has been approved specifically for it. Doctors sometimes use drugs approved for other cancers.

When a drug does help, help usually means the tumor stops growing. It rarely means the tumor shrinks. That is worth knowing before you start, because otherwise a stable scan reads like failure when it is the goal.

The rare poorly differentiated kind is the exception, and chemotherapy is sometimes part of the plan there.

Clinical trials matter more here than for more common cancers, simply because the standard drug options are thin.

A tumor board is a meeting where specialists look at one person's case together: surgeons, cancer doctors, the lab, and the people who read scans. It tends to happen when a case is complicated or something is hard to read.

You do not go to the meeting. Afterwards, the doctor who knows you best explains what was said.

What they decide is advice, not an order. The choice is still yours. Because you are not in the room, tell your team what matters to you before it happens, so it is taken into account.

What is treatment actually like, and what is normal afterwards?

After surgery through the nose. Your nose will be blocked, crusty and sore for a while, and that is expected. Rinses and follow-up visits with an ear, nose and throat specialist are part of the plan rather than a sign something is wrong.

One thing after that operation needs its own rule. The fluid around your brain sits just behind where the surgeon worked, and it can leak.

• Clear, watery fluid running from your nose or down the back of your throat means calling your surgery team, especially in the first week or two. It is not mucus, and people often notice a salty taste, or that it is worse when they stand up.
• Do not sit on it, because a leak that keeps going can let infection reach the fluid around the brain. Cleveland Clinic calls that infection a medical emergency that needs treating immediately. A fever, a stiff neck, or bright light hurting your eyes means going to an emergency room, not waiting to see how the night goes.

After surgery lower down, expect weeks of limited movement, and a rehabilitation plan rather than a single recovery date. If bladder or bowel function changed, a specialist should be involved from the start.

During radiation, tiredness builds slowly over the weeks and is usually worst near the end and for a while afterwards. Skin over the treated area can get sore. Where the beam passes near your ears, mouth or throat, expect effects there.

Scans do not happen right after radiation. Teams normally wait a couple of months before imaging, because the area needs time to settle before a picture means anything.

Everyday life

Tiredness trips people up. It is not the same as being short of sleep, and rest does not entirely fix it. Studies have shown that physical activity, during treatment and after it, can bring it down.

Pain is the symptom people report most often, and it comes in two kinds. Pain from damaged nerves feels like burning, tingling or numbness, and it needs different medicines from ordinary pain. Say which kind yours feels like, because it changes the answer. If numbness or weakness is new, that is a different thing: it is the rule in the section further up.

If nerves near the skull base were affected, there is a specialist for each problem: eye specialists for double vision, including prisms and exercises; speech and swallowing therapists; audiologists for hearing.

If nerves lower down were affected, bladder and bowel programs exist and work. So does pelvic floor physical therapy. These are not things you have to work out alone, and they are not things to be embarrassed about raising.

Work. Some people go back to the job they had, some change what they do, and some cannot go back. In the United States, cancer counts as a disability under the law, which means you can ask for reasonable adjustments. You do not have to explain your diagnosis to ask. Saying you have a medical condition that may need adjustments is enough to start that conversation.

Money and travel are a real part of this, particularly if treatment means living near a proton center for two months. The Chordoma Foundation's navigators help with exactly that, and their number is at the bottom of this page.

Follow-up scans, and what to do while you wait

Follow-up for this tumor goes on for years, often fifteen or twenty of them. That is not because anyone expects the worst. It is because chordoma can come back late, and a late return that is found early is a much better problem.

We are not going to print a schedule, and the reason is honest: the published ones disagree. The Chordoma Foundation's own two pages give different intervals, and the expert consensus group says plainly there is not enough data to recommend one. Ask your team what yours is, and ask them to write it down.

Waiting for scan results is its own thing. Nearly everyone finds it hard and it tends to get worse in the days just before. Follow-up scans and waiting for results cover what helps.

If it comes back

More than half of these tumors grow again after the first round of treatment. Better to read that here, calmly, than to meet it unprepared later on.

Usually it returns to the very spot it started from, and there is a reason that is worth understanding. This tumor spreads by pushing into what it is touching, rather than by traveling through the blood or the lymph system. So it turns up where it was, not somewhere new.

It can spread further afield, to places such as the lungs, but that is usually not the first thing that happens.

Coming back does not mean nothing can be done. If it is in one spot, surgery and high dose radiation may both be options again. If it is in several, the aim changes to holding it steady and keeping you comfortable, and there are several ways to do that. Once it has spread widely, doctors do not expect to get rid of it completely, and they say so; what they work on then is control and how you feel.

Ask what the aim of each treatment is. For this tumor the answer is often control rather than removal, and knowing which one you are being offered changes how you read the result.

Did I cause this?

Almost everybody asks this, usually in the first week, usually in private. It is worth answering plainly.

For most brain tumors, nobody knows the cause. That is the honest state of it. The exact cause of most brain and spinal cord tumors is not fully understood, and there are very few well established risk factors. Tumors start when genes that control how cells grow and divide are changed, and in most cases those changes happen by chance during a person's life. Not because of something done or not done.

Cell phones. They give off a kind of radiation called radiofrequency. It is not the same as the radiation in an x-ray. The energy is too low to damage DNA, and it is not known to cause cancer. The evidence so far is that using a cell phone does not cause brain tumors.

Stress. There is no scientific evidence that stress causes brain tumors, and no data linking stress to a higher chance of getting one. A hard year before a diagnosis is a hard year. It is not a cause.

Something you ate, or did not eat. No food and no diet is an established cause of a brain tumor. It is not on the list of known risk factors at all.

Something you passed on, or caught. Brain tumors are not contagious. Most are not inherited either. A small number of people do have an inherited condition that raises the risk, and if that applies to you, your team will raise it and offer to talk it through.

If you are turning this over at three in the morning, that is ordinary and it happens to nearly everyone. It is also worth saying out loud to somebody on your team, because they have answered it many times and they would rather you asked.

For this tumor there are two things worth adding.

Almost nobody inherits this. A handful of families worldwide carry an extra copy of the brachyury gene, and even there the wording matters: MedlinePlus says people with that duplication inherit an increased risk of the condition, not the condition itself.

A common gene finding is not the same thing. More than 95 percent of people with chordoma carry a tiny common variation in that same gene. It is far too common to be a cause on its own, and the Chordoma Foundation says outright that it does not by itself cause chordoma. It is not something you passed on, and it is not something you did.

What might happen over time

For the person caring for someone with this

If you are the one driving to the appointments, keeping track of the pills, and lying awake at night worrying about them, this part is for you.

You are allowed to ask questions. In one study, families said they were afraid of annoying the doctor by asking too much. One of them was told by a doctor to stop playing doctor. Ask anyway. Write your questions down before you go, and bring a notepad. Two people in the room hear more than one.

Ask who your first call is. Care comes from a lot of people at once, and it is easy to end up as the only one holding it together. Ask the team: who do I call first? Some teams have one person whose job this is, often a nurse. Get the name and the number.

Get two numbers, and know which one to use. One for work hours, one for nights and weekends. Then ask what counts as "call us today" and what counts as "call an ambulance" for this tumor, and write the answer down where you can see it. If you are not sure which it is, call. Nobody on that team will mind.

If they have seizures, read about them before you need to. What to do when someone has a seizure is the one to know by heart. Living with seizures is about the rest of the time: what is safe, what to change, and driving.

Ask to be shown anything you are sent home to do. In that same study, families were sent home to change dressings and give medicines with no training at all. That is not fair to you. Before you leave the hospital, ask someone to show you. Then do it once while they watch.

Say what you notice. You may see a change before they do. Changes in thinking, mood and behavior can show up to the people close by first. Telling the team is not going behind their back. It is something the team cannot learn any other way.

Look after yourself, and let people help. Eat, sleep, and keep your own appointments. Take a little of the day back for yourself. When someone asks what they can do, give them a real job: a meal, a ride, an hour with the kids. Most people want to help and do not know how to offer.

You can talk to somebody about all of this today. See where to get help.

Chordoma adds three things to that list.

You may be having a harder time than they are, and that is documented. The Chordoma Foundation reports that caregivers say they struggle emotionally at a higher rate than patients do. In a survey of families living with this tumor, the thing caregivers reported most often was grief. Not stress. Grief. If that lands, it is because it is common, and it is not a sign you are doing this badly.

There is a support group for you specifically. The Chordoma Foundation runs a monthly online group for caregivers, separate from the one for patients. It is free and someone trained runs it. Being in a room with people who already understand saves a lot of explaining.

The most useful thing early on is often logistics, not comfort. Getting to an experienced center, before a biopsy, is the thing that changes this person's options most. If you have the energy to make phone calls when they do not, that is where it counts.

What to ask your team

• Where exactly is my tumor, and what is it touching?
• Have you treated chordoma before, and how often does this center see it?
• Should I be seen at a specialist center before anything else is done?
• If I need a biopsy, who will do it, and how will you avoid seeding the track?
• Is the goal of surgery to take it out in one piece? If not, why not?
• What does the edge, or margin, need to look like for you to be satisfied?
• If the tumor is near my tailbone, what will this cost me in bladder, bowel and sexual function, and can you predict that beforehand?
• Will I need radiation, what kind, and where would I have to go for it?
• How many weeks of treatment, and will I need to move to be near it?
• What is this treatment trying to achieve: removing it, or controlling it?
• Should my tumor be tested for the SMARCB1, or INI1, gene?
• How often will I be scanned, and for how many years?
• Which changes should I report to you between visits?
• Who is my first call if something changes?

Where to get support

• The Chordoma Foundation's navigators, on 1-888-502-6109, Monday to Friday, 8am to 5pm Eastern. It is free, confidential, and open to anyone affected by chordoma, including family and friends. They help with finding doctors, understanding treatment guidance, discounted lodging and travel, and appealing insurance decisions.
• Their peer program, which puts you in touch with someone who has been through this. It is open to family and friends too, not only to patients.
• Their monthly online groups, one for patients and survivors and a separate one for caregivers and the people around them.
• Getting help now, for the moments when you would rather talk to a person than read anything at all.