Chordoma is a rare type of bone cancer that grows in the bones of the spine or at the base of the skull. It starts from leftover cells of the notochord, a soft rod that helps form the spine in the growing baby before birth. Chordoma usually grows slowly, but it can press on the brain, spinal cord, or nearby nerves and can come back after treatment.
Chordoma is a rare, slow-growing cancer that starts from tiny “leftover” cells of the notochord, usually along the spine or at the base of the skull. It tends to press on nearby nerves and organs and often comes back in the same area, even after treatment. The main proven treatments are expert surgery plus high-dose, very precise radiation, and drug treatments are usually used in clinical trials or specialist centers.
Chordoma is a cancer in the sarcoma family, which means it starts in supporting tissues like bone, cartilage, and soft tissue. Even though it often looks low-grade under the microscope, it behaves in an aggressive way in the area where it grows. It may invade nearby bone and soft tissue and sometimes spreads to other parts of the body, such as the lungs or other bones.
Other names for chordoma
Doctors and cancer centers sometimes use other names for chordoma. One name is notochordal sarcoma, which means a cancer that comes from notochord cells. This name reminds us of the origin of the tumor in the early spine.
Chordoma can also be called primary notochordal bone cancer, because it begins in bone and is not a spread from another cancer. Some sources simply say notochordal tumor or malignant notochordal tumor, again pointing to the same tissue of origin in the embryo.
Doctors also use names based on where the tumor is in the body. A tumor at the base of the skull is often called skull base chordoma or clival chordoma. A tumor at the bottom of the spine is called sacral chordoma, and one in the middle part of the spine may be called mobile spine chordoma. These names describe the main location but still refer to the same disease.
Types of chordoma
There are several main types of chordoma. One way to divide them is by what the cells look like under a microscope. The most common type is classic or conventional chordoma. In this type, the tumor cells are arranged in lobules, with small round nuclei and a “bubbly” or vacuolated look in the cell body, sometimes called “physaliferous” cells.
Another histologic type is chondroid chordoma. In this type, the tumor shows features of both chordoma and cartilage-type tumor (chondrosarcoma). It often arises in the skull base. Chondroid chordoma may have a somewhat better outlook than classic chordoma in some series, but it is still a cancer that needs expert treatment.
A third type is dedifferentiated chordoma. This type is rare but more aggressive. It has areas of typical chordoma mixed with high-grade, faster-growing cancer cells that may behave more like other aggressive sarcomas. Dedifferentiated chordoma tends to grow faster and spread more often than classic chordoma.
Chordoma is also grouped by location. Skull base (clival) chordomas grow in the bone behind the nose and at the base of the skull, near the brainstem and cranial nerves. Mobile spine chordomas grow in the bones of the neck, chest, or lower back spine. Sacral chordomas grow at the base of the spine near the tailbone and are the single most common location overall.
Causes and risk factors of chordoma
The exact cause of chordoma is not fully known. For most people, no clear trigger is found. However, researchers have discovered some genetic and biologic changes that increase risk or seem to drive tumor growth.
TBXT (brachyury/T) gene duplication
In a small number of families, people who have an extra copy (duplication) of the TBXT gene, also called the T or brachyury gene, have a much higher chance of getting chordoma. This gene is important in forming the notochord in the embryo. When duplicated, it appears to push cells toward uncontrolled growth.Other changes in the TBXT (brachyury) gene
Even without a full duplication, changes in how the TBXT gene is expressed or amplified in tumor cells are found in many sporadic chordomas. These changes keep the brachyury protein turned on and support survival and growth of notochord-like tumor cells.Familial chordoma
Very rarely, several relatives in the same family develop chordoma. In many of these families, a TBXT gene duplication is present. This shows that inherited genetic factors can be a strong cause in these uncommon familial cases, even though most chordomas are not inherited.Notochord cell remnants
Chordoma always develops along the path where the notochord once was, such as the skull base and sacrum. After birth, small groups of notochord cells can remain in these bones. Over many years, these leftover cells can acquire DNA damage and eventually turn into cancer cells.DNA damage and genomic instability
Studies of chordoma tissue show many changes in chromosomes and genes, including gains and losses of pieces of DNA. Such genomic instability is a general cause of cancer and seems to play an important role in the step-by-step build-up of damage that turns a normal notochord cell into a chordoma cell.Loss of tumor-suppressor genes
Important “brake” genes, such as CDKN2A/CDKN2B and PTEN, are often partly or fully deleted or silenced in chordoma cells. When these genes are lost, the normal control of cell division is weakened, so cells can grow and divide more easily, which contributes to cancer formation.Activation of the PI3K–Akt–mTOR pathway
Many chordomas show abnormal activation of growth-signaling pathways, especially the PI3K–Akt–mTOR pathway. This pathway sends “grow and survive” signals inside the cell. When it is overly active, cells resist death and multiply more, helping the tumor to grow.Growth factor receptors (PDGFR, EGFR and others)
Chordoma cells often express high levels of growth factor receptors such as platelet-derived growth factor receptor (PDGFR) and epidermal growth factor receptor (EGFR). These proteins sit on the cell surface and receive growth signals; when overactive, they can drive cell growth and are being studied as drug targets.Epigenetic changes
Besides direct DNA mutations, chordomas show epigenetic changes, such as changes in DNA methylation and histone modification. These changes do not alter the DNA code but can switch genes on or off in a lasting way, supporting a cancer-like state in the cells.Association with tuberous sclerosis complex (TSC)
Some reports suggest a link between chordoma and tuberous sclerosis complex, a genetic disease caused by mutations in TSC1 or TSC2 genes. These genes normally help control mTOR signaling, and their loss can lead to abnormal cell growth, which may, in rare cases, favor chordoma formation.Male sex
Chordoma affects males about twice as often as females. Being male is therefore a risk factor, although we do not yet fully understand why. Hormonal or genetic differences between sexes may contribute to this pattern.Middle to older adult age
Chordoma can occur at almost any age, but it is most common in adults between about 40 and 70 years. This suggests that time and age-related accumulation of DNA damage in notochord remnants are important in the cause of the disease.Very rare childhood cases
A small number of chordomas are diagnosed in children. In these young patients, genetic causes such as TBXT changes may be especially important, and the disease often behaves differently than in adults.Family history of chordoma
Having a close family member with chordoma is a strong risk factor, though this situation is rare. Families with several cases often carry TBXT duplication or other shared genetic changes, which pass down the increased risk.General inherited cancer risk factors
In some people, chordoma may appear as part of a broader inherited tendency to cancer caused by mutations in DNA repair genes or growth control genes. While such syndromes are not common in chordoma, they illustrate that inherited defects in cell-control pathways can set the stage for this tumor.Spontaneous (sporadic) mutations
Most chordomas are “sporadic,” meaning they happen in people without a known family history. In these cases, random DNA errors that build up over a lifetime in notochord remnants probably play a major causal role, even though we do not see a single simple trigger.Chromosome copy number changes
Chordoma cells often show extra copies or losses of large chromosome regions, including 1p, 3p, 9p, and 10. These structural changes affect many genes at once, disturbing normal growth control and helping the tumor to start and progress.Abnormal cell cycle regulation
Many of the genetic changes in chordoma interfere with the cell cycle, the internal “clock” that controls when a cell divides. When this regulation is lost, notochord-derived cells may keep dividing when they should rest or die, which is a central cause of tumor formation.Unknown environmental factors (currently unproven)
At present, no specific environmental or lifestyle factor, such as diet or occupation, has been clearly proven to cause chordoma. Studies so far have not found a consistent link with common exposures, which means that if such factors exist, their effect is likely small compared with genetic and biologic causes.Chance and complex interaction of many factors
For many patients, chordoma likely develops from a mix of inherited tendency, random DNA damage over time, and complex changes in cell-signaling pathways. In these cases, we cannot point to a single simple cause. Instead, many small risks add up to allow a notochord cell to become cancerous.
Symptoms of chordoma
Because chordoma grows near the brain, spinal cord, or major nerves, most symptoms come from pressure on these structures. Symptoms also depend strongly on where the tumor sits along the spine or at the skull base.
Headache
Skull base (clival) chordomas often cause headaches. The tumor grows in the bone behind the nose and eyes and can press on the dura, brainstem, or nearby nerves. This pressure can lead to dull, deep headaches that may slowly get worse over time.Neck pain and stiffness
When chordoma grows near the top of the spine or at the skull base, it can cause chronic neck pain and stiffness. The tumor may irritate joints, ligaments, and nerves in the neck region, making turning or bending the neck uncomfortable or painful.Back or tailbone pain
Sacral chordomas, at the bottom of the spine, often cause deep aching pain in the lower back, buttocks, or tailbone. This pain may be worse when sitting and can be present for months or years before diagnosis, because the tumor grows slowly.Pain shooting into arms or legs (radicular pain)
If the tumor presses on spinal nerve roots, it can cause sharp, shooting pain that travels down an arm or leg, similar to sciatica. This kind of nerve pain is often described as burning, electric, or stabbing and may follow the path of a specific nerve.Weakness in arms or legs
Compression of the spinal cord or spinal nerves by a chordoma can lead to muscle weakness. A person may notice difficulty climbing stairs, gripping objects, or walking long distances. Weakness that slowly worsens over time is a key warning sign of spinal cord or nerve compression.Numbness or tingling
Pressure on sensory nerves can cause numbness, tingling, or a “pins and needles” feeling in the face, arms, legs, or around the groin and buttocks. These changes in sensation often match the nerve roots that are being compressed by the tumor.Problems with bowel control or constipation
Sacral chordomas can interfere with the nerves that control the bowel and rectum. People may notice constipation, difficulty passing stool, or loss of bowel control (incontinence). These symptoms suggest serious pressure on the lower spinal nerves.Urinary problems
The same sacral nerves also control bladder function. A chordoma in this area can cause difficulty starting urination, incomplete emptying, frequent infections, or loss of bladder control. Any new bladder problems with back or tailbone pain need careful evaluation.Sexual dysfunction
Sacral tumors may damage nerves responsible for sexual function. This can lead to erectile dysfunction in men or reduced sensation and sexual response in all patients. These changes may develop slowly and are sometimes overlooked until other symptoms are investigated.Double or blurred vision
Skull base chordomas often sit close to nerves that move the eyes. When these nerves are compressed, a person may see double images, have trouble focusing, or develop misalignment of the eyes. These visual symptoms are a common early sign of a clival chordoma.Facial numbness or weakness
Pressure on cranial nerves near the skull base can cause areas of numbness on the face or weakness of facial muscles. Patients may notice difficulty closing an eye, smiling evenly, or moving parts of the face normally.Difficulty swallowing or speaking
When a chordoma presses on nerves that control the throat and voice box, swallowing can become hard or painful, and speech may sound hoarse or nasal. Food or liquids may feel “stuck,” and choking episodes can occur, especially with clival tumors.Nasal blockage or mass in the back of the nose or throat
In some skull base chordomas, the tumor bulges into the nasal cavity or nasopharynx. People may notice chronic nasal blockage, nosebleeds, or a mass seen during nasal or throat examination or on imaging done for another reason.General tiredness and weight loss
As chordoma becomes more advanced, chronic pain, poor sleep, and the body’s reaction to cancer can cause fatigue and unintended weight loss. These symptoms are not specific to chordoma but often appear in people with long-standing or metastatic disease.Symptoms from spread (metastasis)
When chordoma spreads to the lungs, bones, or liver, it can cause new symptoms such as persistent cough, shortness of breath, bone pain in new areas, or discomfort in the upper abdomen. These signs usually appear late and point to advanced disease.
Diagnostic tests for chordoma
Diagnosing chordoma needs careful clinical examination, detailed imaging, and confirmation by studying a sample of the tumor under the microscope. Often, several different tests are used together so that doctors can be sure of the diagnosis and plan treatment safely.
Physical examination (examples of tests)
1. General physical and neurological examination
The doctor begins with a full physical and neurological exam. They check muscle strength, reflexes, sensation, balance, and coordination, and look for signs such as abnormal posture or visible swelling. This helps to locate where along the spine or skull base the tumor might be pressing on nerves.
2. Cranial nerve examination
For suspected skull base chordoma, the doctor carefully tests all cranial nerves. They check eye movements, facial power and feeling, hearing, swallowing, and tongue movement. Weakness or loss of function in certain cranial nerves can point to a clival mass like chordoma.
3. Spine inspection and palpation
The spine is inspected from the side and back for abnormal curves, lumps, or areas of tenderness. The doctor gently presses along the spine, sacrum, and tailbone to find painful spots. Local pain or a palpable mass in the sacral region can suggest a tumor such as chordoma.
4. Rectal and pelvic examination (for sacral tumors)
When a sacral chordoma is suspected, a rectal or pelvic exam may reveal a mass pressing from behind the rectum or vagina. This physical finding, together with tailbone pain and nerve symptoms, helps to confirm that there is a lesion in the sacral area that needs imaging.
Manual / bedside neurological tests
5. Manual muscle strength testing
The doctor asks the patient to push and pull with arms and legs against resistance. Weakness in certain muscle groups, such as foot dorsiflexion or hand grip, can show which nerve roots or spinal cord segments are being compressed by a spinal chordoma.
6. Sensory testing for light touch and pain
Using tools such as a cotton swab or pin, the examiner checks sensation across the body. Loss or change of feeling in a “saddle” area (inner thighs, buttocks) or along specific dermatomes (skin zones) can suggest pressure from a sacral or spinal tumor.
7. Reflex testing (deep tendon reflexes)
Reflexes at the knee, ankle, and other joints are checked with a small hammer. Reflexes that are very weak, absent, or very brisk in certain areas help show whether the spinal cord or specific nerve roots are affected by the tumor, guiding imaging and diagnosis.
8. Gait and balance assessment
The patient is asked to walk, stand with feet together, and sometimes walk on heels or toes. Difficulty with walking, unsteadiness, or need for support may indicate spinal cord compression or problems with brainstem pathways from a skull base chordoma.
Laboratory and pathological tests
9. Basic blood tests (supportive, not specific)
Routine blood tests such as complete blood count and blood chemistry are usually normal in chordoma and are not used to make the diagnosis directly. However, they are important to check overall health, look for anemia or organ problems, and get the patient ready for surgery or other treatment.
10. Needle or open biopsy of the tumor
A biopsy is the key test to confirm chordoma. Under imaging guidance (often CT), or during surgery, doctors take a small piece of the tumor. This tissue sample is then examined in the laboratory to identify the specific tumor type. Biopsy planning is crucial to avoid spreading tumor cells and to keep future surgery options safe.
11. Histopathology (microscopic examination)
Under the microscope, pathologists look for the classic features of chordoma: lobules of cells with bubbly, vacuolated cytoplasm (“physaliferous” cells) in a myxoid (gel-like) background. These features help distinguish chordoma from other bone tumors and are essential for a firm diagnosis.
12. Immunohistochemistry for brachyury
Brachyury is a protein made by the TBXT gene and is considered a very sensitive and specific marker for chordoma. Special stains on the biopsy tissue can show strong nuclear brachyury expression, which supports the diagnosis and helps separate chordoma from look-alike tumors such as chondrosarcoma.
13. Immunohistochemistry for cytokeratin, EMA, S100, and vimentin
Chordomas usually stain positive for epithelial markers such as cytokeratins and epithelial membrane antigen (EMA), as well as S-100 protein and vimentin. This combination of markers, together with brachyury positivity, gives a characteristic immunoprofile that confirms chordoma and helps rule out other bone and soft-tissue cancers.
14. Molecular testing for TBXT (brachyury) alterations
In some cases, especially with a family history, molecular tests on blood or tumor tissue look for TBXT gene duplication or other changes. Finding a TBXT duplication supports a genetic form of chordoma and may help with counseling of family members and future research on targeted therapies.
Electrodiagnostic tests
15. Electromyography (EMG)
EMG is a test that measures electrical activity in muscles. Small needles are placed in selected muscles, and their signals are recorded. Patterns of muscle denervation or weakness can show which nerve roots or parts of the spinal cord are affected by a sacral or spinal chordoma, and can help distinguish it from other nerve diseases.
16. Nerve conduction studies (NCS)
In NCS, mild electrical impulses are used to stimulate nerves, and the speed and strength of the nerve signals are measured. Slowed or reduced signals in particular nerves can confirm that they are being compressed or damaged by a nearby tumor such as chordoma, supporting imaging and exam findings.
Imaging tests
17. X-ray of the skull or spine
Plain X-rays are often the first imaging test. In chordoma, they may show bone destruction, erosion, or a lytic lesion in the clivus, spine, or sacrum. Although X-rays are not detailed enough to define the full tumor, they can raise suspicion and guide the need for more advanced imaging.
18. CT scan (computed tomography)
CT scans give detailed pictures of bone. In chordoma, CT often shows areas of bone destruction with a soft-tissue mass arising from the skull base, vertebra, or sacrum. CT is very useful to understand how much bone must be removed in surgery and to plan the safest surgical approach.
19. MRI scan (magnetic resonance imaging)
MRI is the most important imaging test for chordoma. It shows the size of the tumor, its exact relation to the spinal cord, brainstem, and nerves, and whether it extends into nearby soft tissues. MRI is used both at diagnosis and during follow-up after treatment to monitor for recurrence.
20. PET, PET-CT, or other functional imaging
Positron emission tomography (PET), often combined with CT, can help detect areas of active tumor and possible spread to distant sites such as lungs or other bones. While not always required, PET-CT or similar scans can be useful in staging advanced disease and in planning systemic therapy or radiation.
Non-Pharmacological Treatments (Therapies and Others)
Multidisciplinary chordoma center care
A multidisciplinary team means neurosurgeons, spine surgeons, radiation oncologists, medical oncologists, radiologists, pathologists, rehab doctors and nurses working together on one shared plan. For chordoma, this joined-up approach improves the chance of removing or controlling the tumor while protecting the brain, spinal cord and important nerves. It also makes it easier to join clinical trials, get second opinions and coordinate rehab, pain care and follow-up imaging in one place.High-dose photon radiotherapy
Standard external-beam radiotherapy with x-rays (photons) can be used after surgery or when surgery is not possible. In chordoma, very high doses are needed to control the tumor, so doctors use modern planning (like IMRT) to shape the beam around the tumor and spare the spinal cord and brainstem. Radiation damages the DNA inside tumor cells more than in normal tissue, so the cancer cells slowly die or stop dividing.Proton beam therapy
Proton beam therapy is a special type of radiotherapy where charged particles (protons) stop inside the tumor and deliver most of their energy there. For skull-base and spinal chordoma, this helps give a tumor-killing dose while lowering radiation to brain, eyes or spinal cord. This approach has become a key part of modern chordoma care in many centers, with better local control and fewer late side effects compared with older techniques.Carbon-ion or other particle therapy
Carbon-ion therapy uses heavier particles that deposit energy very sharply and may cause more complex DNA damage to tumor cells. Some centers in Europe and Asia use this for difficult skull-base and spinal chordomas, especially when tumors are large or close to nerves. Early results suggest good local control, but access is limited and long-term data are still developing.Stereotactic radiosurgery / stereotactic radiotherapy
Stereotactic radiosurgery (like Gamma Knife or CyberKnife) uses many tiny beams aimed very precisely from different angles. For small residual or recurrent chordomas, it can give a high dose to a tight spot in one or a few sessions. The goal is to “boost” control while protecting surrounding brain, cranial nerves or spinal cord. Planning has to be extremely careful because chordomas lie close to critical structures.Intensity-modulated radiotherapy (IMRT)
IMRT is a planning method used with photon beams. Computers change beam shape and intensity across the field so that high dose can “wrap around” the tumor and avoid normal tissues. In chordoma, IMRT is often combined with image guidance and sometimes proton boosts. It allows dose escalation while staying within safe limits for the spinal cord, brainstem, eyes or swallowing muscles.Physical therapy and mobility training
Chordoma and its treatments can weaken muscles, stiffen the neck or back and disturb balance. Physical therapists use stretching, strengthening and gait training to keep joints moving, protect posture and reduce fall risk. Gentle, regular exercise also helps fight fatigue, keeps lungs and heart strong and supports emotional health during long cancer treatment.Occupational therapy and daily-activity adaptation
Occupational therapists help patients adjust daily tasks like dressing, bathing, computer use and chores when pain, weakness or numbness make them hard. They may suggest adaptive tools, posture changes, pacing strategies and home modifications (like grab bars). This support aims to keep independence and safety while the medical team focuses on tumor control.Pain management with non-drug methods
Non-drug pain strategies include heat or cold packs, gentle stretching, massage from trained therapists, relaxation breathing, mindfulness, and sometimes TENS (mild electrical stimulation on the skin). These methods do not replace medical pain treatment but can reduce the amount of medication needed and give patients more control over day-to-day discomfort.Bracing and spinal support (neck or back brace)
In cervical, thoracic or sacral chordoma, the tumor or surgery can weaken bone. Short-term use of a neck collar or lumbar / sacral brace can stabilize the spine, reduce pain with movement and protect healing bone or hardware. The brace works by limiting motion in the affected segment so forces shift safely to stronger areas.Speech and swallowing therapy
Skull-base chordomas can affect the nerves that control speech and swallowing, and high-dose radiation can make the throat dry or stiff. Speech-language pathologists teach swallowing exercises, diet texture changes and voice strategies. Their goal is to lower choking risk, prevent weight loss and keep communication clear, even when cranial nerves are partly damaged.Balance and vestibular rehabilitation
Chordomas near the skull base or upper spine can disturb balance pathways or cause eye-movement problems. Special balance therapy trains the eyes, inner ear and muscles to work together again. This may include standing on soft surfaces, head-turning exercises and safe walking drills to cut the risk of falls during and after treatment.Psychological counseling and psycho-oncology
Living with a rare cancer is stressful and lonely. Psycho-oncology therapists help patients handle fear of recurrence, scan anxiety, body-image changes and family stress. Counseling, cognitive-behavioral therapy and relaxation techniques can reduce depression and anxiety, improve sleep and help patients stick with long, complex chordoma treatment plans.Palliative care and symptom management team
Palliative care is not only for end of life. For chordoma, palliative doctors and nurses help manage pain, fatigue, constipation, nausea, breathlessness and mood issues from the very beginning. They also support decision-making about treatment goals and quality of life. Studies show that early palliative care can improve both comfort and sometimes even survival in serious cancers.Nutritional counseling and medical nutrition therapy
A dietitian familiar with cancer helps maintain weight, muscle and energy. They adjust calories, protein, vitamins and fluid, and suggest foods that are easy to swallow or digest after skull-base or spinal surgery. Good nutrition supports wound healing, immune function and tolerance to radiation or systemic therapy.Gentle, supervised exercise (walking, yoga, stretching)
Moderate, tailored exercise can improve strength, mood and sleep in people with cancer. For chordoma, programs are adapted to spinal stability and nerve function, often starting with short walks, chair exercises or gentle yoga. Movement improves circulation, reduces inflammation and may ease treatment-related fatigue and stiffness.Smoking cessation and alcohol moderation support
Although chordoma is not clearly linked to smoking or alcohol, these habits worsen healing, heart and lung health and the safety of big spine or skull-base operations. Stopping smoking and limiting alcohol can lower surgical and radiation complications and support overall survival in cancer care.Support groups and patient-advocacy organizations
Because chordoma is rare, disease-specific support groups and foundations help patients find expert centers, clinical trials and emotional support. Sharing experiences with others facing the same diagnosis makes it easier to understand options, cope with uncertainty and plan for work, school and family.Regular MRI and long-term follow-up
Chordoma often comes back locally. Regular MRI scans of the involved area and sometimes whole spine imaging help detect recurrence when it is still small and treatable. Follow-up visits also monitor late side effects of surgery and radiation, such as nerve changes, hormone issues or bone weakness, so they can be managed early.Clinical-trial enrollment for new non-drug techniques
Some clinical trials test new radiation schedules, image-guided surgery tools, nerve-monitoring methods or patient-specific implants. Joining such trials at experienced centers can give access to cutting-edge technology while contributing to better future care for chordoma worldwide.
Drug Treatments for Chordoma
Important note: Right now, no medicine is officially approved specifically for chordoma. Systemic treatments are usually used in clinical trials or specialist centers, often based on how the tumor’s genes and receptors look. Doses below are typical adult doses from FDA labels for other cancers; real dosing for any person must be chosen and adjusted only by an oncology team.
Imatinib (tyrosine kinase inhibitor)
Imatinib blocks PDGFR, KIT and related signaling pathways, which are often active in chordoma cells. A typical adult cancer dose is about 400–800 mg by mouth once daily, with food, but chordoma regimens may differ. The purpose is to slow tumor growth and shrink soft-tissue parts of the mass. Mechanistically, imatinib stops kinase activity that tells cancer cells to grow and make new blood vessels. Common side effects include swelling, nausea, fatigue and low blood counts, so regular blood tests are needed.Sunitinib
Sunitinib is a multi-target tyrosine kinase inhibitor (VEGFR, PDGFR, KIT and others). In other cancers the usual schedule is 50 mg orally once daily for 4 weeks on, 2 weeks off, or continuous lower-dose schedules; for chordoma it is used mainly in trials. It aims to cut off tumor blood supply and signal pathways. Side effects include tiredness, diarrhea, hand–foot skin reactions, high blood pressure and low platelets, so heart and blood pressure monitoring are needed.Sorafenib
Sorafenib blocks RAF kinases and several VEGFR and PDGFR receptors, targeting both tumor cells and their blood vessels. Many cancer indications use 400 mg orally twice daily. It is sometimes tried for advanced chordoma when other TKIs fail. Main side effects are fatigue, diarrhea, rash, hand–foot syndrome, high blood pressure and liver toxicity, so liver tests and blood pressure checks are essential.Pazopanib
Pazopanib is an oral VEGFR, PDGFR and KIT inhibitor used in soft-tissue sarcomas, and has been explored in small chordoma series. The usual adult dose in other cancers is 800 mg once daily on an empty stomach. It aims to starve tumors of blood supply. Side effects include liver toxicity, high blood pressure, diarrhea, hair color changes and risk of bleeding or clotting, so liver tests and blood pressure must be monitored carefully.Afatinib
Afatinib is an irreversible inhibitor of EGFR and related receptors. EGFR signaling is active in many chordomas, so this drug is being investigated as a targeted therapy. The usual adult dose in lung cancer is 40 mg orally once daily. It works by blocking growth signals at the cell surface. Diarrhea, skin rash, mouth sores and nail changes are common, and dose reductions are often needed.Erlotinib
Erlotinib is another EGFR tyrosine kinase inhibitor given orally, often 150 mg once daily for lung cancer. In chordoma, EGFR inhibition is being tested for tumors that strongly express this receptor. Erlotinib blocks EGFR-driven growth and may make tumors more sensitive to radiation. Common side effects are acne-like rash, diarrhea, loss of appetite and fatigue.Gefitinib
Gefitinib is an oral EGFR inhibitor that has shown some activity in small chordoma datasets or in combination with other agents. Typical adult dosing for lung cancer is 250 mg once daily. It reduces EGFR signaling, which can slow cell division and enhance other therapies. Side effects are similar to other EGFR inhibitors: skin rash, diarrhea and mild liver test changes, requiring periodic blood tests.Regorafenib
Regorafenib blocks multiple kinases involved in angiogenesis and tumor growth (VEGFR, TIE2 and others). It is taken orally, often 160 mg once daily for 3 weeks on, 1 week off in colorectal and GIST cancers. For chordoma, it has been evaluated in small studies as a salvage option. It works by cutting blood supply and signaling pathways. Side effects include fatigue, high blood pressure, hand–foot skin reactions, diarrhea and liver enzyme rises.Dasatinib
Dasatinib targets SRC-family kinases and some PDGFR/KIT activity. In chordoma, SRC signaling may support invasion and resistance, so dasatinib is explored in trials or off-label in select cases. Typical adult dosing in leukemia is 100 mg once daily. It blocks enzymes that help tumor cells move and survive. Side effects include low blood counts, fluid around the lungs, bleeding risk and diarrhea, so close monitoring is necessary.Pemetrexed
Pemetrexed is an antifolate chemotherapy used in lung cancer and mesothelioma, and has shown signals of activity in advanced chordoma in case reports. Dosed intravenously every 3 weeks with folic acid and vitamin B12 support, it blocks enzymes needed for DNA building blocks, slowing tumor cell division. Side effects include low blood counts, fatigue, nausea and mouth sores. It is usually reserved for situations where targeted options are not suitable.Cisplatin (and platinum combinations)
Cisplatin is a classic chemotherapy drug that cross-links DNA strands so cells cannot divide. In chordoma it is sometimes used in combinations, especially in pediatric or aggressive cases, although overall chemo sensitivity is limited. Dosing is intravenous every few weeks with hydration. Side effects include nausea, kidney injury, hearing loss and nerve damage, so kidney function and hearing must be checked.Doxorubicin
Doxorubicin is an anthracycline that intercalates into DNA and generates free radicals. It is one of the backbone drugs in many sarcoma regimens and has been tried in chordoma, though overall benefit is modest. It is given by IV infusion every 3 weeks, with lifetime dose limits to protect the heart. Side effects include hair loss, low blood counts and possible heart weakening, requiring echocardiogram monitoring.Ifosfamide
Ifosfamide is an alkylating chemotherapy that damages DNA, sometimes combined with doxorubicin in resistant sarcomas, including rare use in chordoma. It is given intravenously with a protective drug (mesna) to guard the bladder. Side effects include low blood counts, kidney problems and confusion, so patients need hydration and careful monitoring.Everolimus
Everolimus inhibits mTOR, a key cell-growth pathway that may be active in chordoma cells. It is taken orally, usually 10 mg once daily in other cancers. By blocking mTOR, it can slow protein synthesis and cell growth and may also affect tumor blood vessels. Side effects include mouth ulcers, high blood sugar and high cholesterol, and increased infection risk.Temsirolimus
Temsirolimus is an intravenous mTOR inhibitor related to everolimus, given once weekly in some kidney cancers and sarcomas. For chordoma, it is sometimes studied alone or in combinations as a targeted therapy. It works through mTOR inhibition, slowing growth and angiogenesis. Typical side effects are fatigue, rash, mouth sores, low blood counts and raised blood sugar and lipids.Pembrolizumab (PD-1 inhibitor)
Pembrolizumab is an immune checkpoint inhibitor that blocks PD-1 on T cells so they can better attack tumors. In reports of recurrent chordoma, pembrolizumab has led to meaningful tumor shrinkage and months of disease control in some patients. Typical adult dosing is 200 mg IV every 3 weeks or 400 mg every 6 weeks in approved cancers. Side effects include immune-related inflammation of lungs, gut, liver, skin and hormones and must be managed quickly with steroids.Nivolumab (PD-1 inhibitor)
Nivolumab also blocks PD-1 and has shown responses in some chordoma patients, especially when tumors show immune cell infiltration and PD-L1 expression. Standard adult regimens in other cancers include 240 mg IV every 2 weeks or 480 mg every 4 weeks. It works by releasing immune “brakes” and letting T cells recognize tumor antigens like brachyury. Side effects are similar to pembrolizumab and require experienced management.Avelumab (PD-L1 inhibitor)
Avelumab targets PD-L1 on tumor and immune cells. Lab studies in chordoma show that avelumab can help natural killer cells kill chordoma cells, including cancer stem-like cells, through antibody-dependent cell-mediated cytotoxicity. In practice it is given intravenously every 2 weeks in approved cancers. Side effects include infusion reactions and immune-related inflammation of organs. Its use in chordoma remains mostly within trials.Cetuximab (EGFR monoclonal antibody)
Cetuximab is an antibody that binds EGFR on cell surfaces. An ongoing trial is testing cetuximab in metastatic chordoma with strong EGFR expression. Typically it is given as a loading infusion followed by weekly or bi-weekly IV doses. By blocking EGFR, it reduces growth and may increase radiosensitivity. Side effects include acne-like rash, infusion reactions and low magnesium.Experimental vaccines and combination immunotherapies
Several investigational approaches include vaccines targeting brachyury (a chordoma driver protein), combined checkpoint blockade and cell-based therapies. Doses and schedules vary by trial. These treatments aim to train the immune system to recognize chordoma cells or to overcome resistance to single-agent immunotherapy. Side effects can range from mild flu-like symptoms to serious immune reactions and must be managed in specialist centers.
Dietary Molecular Supplements (Supportive, Not Curative)
(Supplements do not replace surgery, radiation or oncologist-prescribed drugs. Evidence for chordoma-specific benefit is very limited; most data are from general cancer care. Always check with the oncology team before starting any supplement because of interactions.)
Vitamin D
Vitamin D supports bone health, muscle function and immune regulation. Low vitamin D is common in cancer and may worsen fatigue and bone loss from treatments. Typical adult supplementation ranges from 600–2000 IU daily, adjusted to blood levels. It works by binding vitamin D receptors, influencing cell growth, immune activity and calcium balance. While some studies suggest possible benefits for cancer outcomes, results are mixed, so vitamin D is mainly recommended to correct deficiency and support general health.Omega-3 fatty acids (EPA/DHA)
Omega-3 fatty acids from fish oil or algae may help reduce inflammation, support weight maintenance and improve appetite in some cancer patients. Common supplemental doses are around 1–3 g total EPA+DHA per day with meals, as tolerated. Mechanistically, omega-3s change cell-membrane composition and reduce pro-inflammatory eicosanoids, which may ease treatment-related inflammation and support heart and brain health. They can thin the blood slightly, so doctors should check for bleeding risks and drug interactions.Curcumin (from turmeric)
Curcumin is a plant compound with anti-inflammatory and antioxidant actions. It has been shown to affect EGFR, PDGFR and PI3K/AKT/mTOR pathways that are important in many cancers. Doses in studies vary widely (often 500–2000 mg/day of standardized extract) and absorption is a challenge. Curcumin may reduce cancer-related inflammation and enhance some drugs in lab models, but human cancer data are still evolving. It can interact with blood thinners and cause stomach upset in some people.Green tea extract (EGCG)
Green tea polyphenols, especially EGCG, have antioxidant and mild anti-cancer effects in lab studies, including effects on cell-signaling and angiogenesis. Supplement doses typically range from 200–400 mg EGCG per day, often divided. EGCG can scavenge free radicals and modulate signaling pathways, possibly supporting general cancer care, but high doses may stress the liver, so liver tests and medical supervision are important.Probiotics
Probiotics are “friendly” bacteria that support gut health. In cancer patients they may help reduce some forms of treatment-related diarrhea and support nutrient absorption, but they must be used carefully in people with very low white blood cells. Typical doses range from billions of colony-forming units daily in capsule or yogurt form. They act by stabilizing the microbiome and strengthening the gut barrier. Oncologists should approve their use, especially during intensive chemotherapy or immunotherapy.Vitamin C
Vitamin C is an antioxidant that also helps collagen and wound healing. Normal dietary intake plus moderate supplements (for example 200–500 mg/day) are usually enough for general health. High-dose IV vitamin C is being studied as an experimental cancer therapy, but evidence is still limited and it can interact with certain drugs. Mechanistically, vitamin C can act as an antioxidant or, at high doses, a pro-oxidant that stresses tumor cells.Zinc
Zinc is important for immune cell function, taste and wound healing. Mild zinc supplementation (for example 5–15 mg/day in adults) can help correct deficiency and support appetite and taste, which may be affected by skull-base radiation. Zinc acts as a cofactor in many enzymes and transcription factors. Too much zinc, however, can upset copper balance and cause nausea, so dosing should be modest and supervised.Selenium
Selenium is a trace mineral involved in antioxidant enzymes such as glutathione peroxidases. Some studies suggest low selenium may be linked with worse outcomes in some cancers, but evidence is not specific to chordoma. Common supplement doses are around 50–200 mcg/day, not higher unless a deficiency is proven. Excess selenium can be toxic, so oncologists should guide use.Medicinal mushroom extracts (e.g., beta-glucans)
Extracts from mushrooms such as reishi, shiitake or maitake contain beta-glucans that can modulate immune responses. Trials in some cancers suggest possible improvements in quality of life and immune markers, but data remain limited and not chordoma-specific. Typical doses vary by product. They act by stimulating parts of the innate immune system. Because supplements are not tightly regulated, quality and purity are important discussion points with the care team.Protein and amino-acid supplements
When eating enough protein is hard because of pain, swallowing problems or fatigue, whey or plant-based protein powders can help maintain muscle mass. Doses are usually 15–30 g of protein per serving, once or twice daily, adjusted by a dietitian. Adequate protein supports wound healing, immune function and recovery after surgery or radiation. The “mechanism” is simple: protein provides building blocks (amino acids) for muscles, organs and immune cells.
Immune-Boosting and Regenerative / Stem-Cell–Related Therapies
(These are supportive or experimental and not direct cures for chordoma. Many are used only under strict oncology supervision.)
Filgrastim (G-CSF) and related drugs
Filgrastim and similar medicines are lab-made forms of granulocyte colony-stimulating factor. They are given as injections, often around 5 mcg/kg/day in adults for a short period after chemotherapy, to help the bone marrow make more neutrophils (infection-fighting white blood cells). In chordoma, they are used when patients receive systemic therapy that suppresses the marrow, to cut the risk of dangerous infections and keep treatment on schedule.Epoetin alfa and related ESAs
Epoetin alfa and similar erythropoiesis-stimulating agents help the bone marrow make more red blood cells. Typical dosing is by injection every 1–3 weeks, adjusted to hemoglobin levels, in selected cancer patients with chemotherapy-related anemia. These drugs aim to reduce transfusions and improve energy but can increase the risk of blood clots and, in some situations, worse cancer outcomes, so their use is very carefully restricted.Autologous stem-cell support after high-dose therapy (rare in chordoma)
Some cancers are treated with very high-dose chemotherapy followed by reinfusion of the patient’s own blood-forming stem cells (autologous transplant). For chordoma this is uncommon and mostly experimental, but the concept is to allow more intense anti-tumor treatment while “rescuing” the bone marrow afterwards. Stem cells home back to the marrow and slowly rebuild blood cell production, but this approach has major risks and is only considered in very select research settings.Mesenchymal stem-cell-based approaches (research only)
Mesenchymal stem cells (MSCs) and their exosomes have immunomodulatory and tissue-repair properties, and they are being researched in many diseases, including cancers. For chordoma, scientists are more often studying cancer stem-like cells as targets rather than giving MSCs as treatment. Any regenerative or MSC-based therapy should only be used inside regulated clinical trials, because risks and true benefits in cancer are not yet clear.Immune checkpoint inhibitor combinations
Combining agents like nivolumab, pembrolizumab or avelumab with other drugs or radiation may “re-educate” the immune system and sustain anti-tumor responses. These regimens aim to overcome resistance by targeting multiple immune pathways. Because they can cause serious autoimmune-type side effects in organs such as lungs, liver, gut and endocrine glands, they must be prescribed and monitored only in experienced cancer centers.Targeting cancer stem-like cells in chordoma (emerging strategies)
Research shows that chordoma contains cancer stem-like cells that may drive recurrence and treatment resistance. New drugs and biological tools are being designed to block pathways linked to these cells (for example CXCR4 signaling or microRNAs that control epithelial–mesenchymal transition). These approaches are still at the laboratory or early clinical-trial stage, but the long-term goal is to make chordoma treatment more “curative” by eradicating the most resistant cells.
Surgeries for Chordoma
En bloc resection of sacral chordoma
For tumors in the sacrum, surgeons may perform an en bloc resection, removing the tumor in one piece with a rim of healthy tissue. The goal is to achieve negative margins, which lowers the chance of local recurrence. This is a major operation that can affect bowel, bladder and sexual function, so careful pre-operative planning and patient counseling are essential.Skull-base chordoma resection (often endoscopic endonasal)
Skull-base chordomas may be removed through the nose using an endoscopic endonasal approach, sometimes combined with open skull surgery. Surgeons work between the nasal passages and sinuses to reach the clivus and brainstem while preserving important nerves and blood vessels. The purpose is to take out as much tumor as safely possible and create a good target volume for high-dose proton or photon radiotherapy.Spinal chordoma resection with stabilization
In the mobile spine (cervical, thoracic, lumbar), surgeons may remove the tumor and affected vertebrae, then reconstruct the spine using metal rods, screws and cages. The procedure’s aim is to decompress the spinal cord and nerves, reduce pain and maintain or restore stable alignment so patients can stand and walk. This surgery is complex and often followed by proton or IMRT radiation.Debulking / intralesional resection for unresectable tumors
When en bloc removal would cause unacceptable harm (for example, severe brainstem damage), surgeons may “debulk” the tumor from the inside (intralesional resection). Although margins are not clean, reducing tumor volume can relieve pressure on nerves and make radiation more effective. The main purpose is symptom relief and better radiotherapy geometry, not cure.Revision and salvage surgery for local recurrence
Chordoma frequently recurs locally, even after good first surgery and radiation. At recurrence, experienced centers may offer repeat resection, sometimes with more advanced approaches or combined with intraoperative radiation or new reconstruction methods. The goal is to re-establish local control, relieve pain and protect neurological function, balancing potential benefit against increased risks from scar tissue and prior radiation.
Prevention – What Is and Is Not Possible
There is no proven way to completely prevent chordoma, because its exact causes are still unclear and mostly not related to lifestyle. However, the steps below support overall health, may lower general cancer risk and can help with earlier detection and safer treatment.
Maintain a healthy body weight and balanced diet to support immune function and healing.
Stay physically active within safe limits for your spine to keep muscles strong and reduce treatment-related fatigue.
Avoid tobacco in all forms; smoking increases surgical and radiation complications and harms heart and lung health.
Limit alcohol, which can interfere with healing, liver function and some medicines.
Use proper protective equipment and rules if you work with radiation or industrial chemicals that might affect general cancer risk.
Get regular medical check-ups and promptly report persistent deep back, neck or skull-base pain that does not improve with rest.
If there is a strong family history of rare tumors or known genetic syndromes, discuss genetic counseling with a specialist.
Keep vaccinations and general health maintenance up to date to reduce infection risks during treatment.
Avoid unnecessary high-dose diagnostic radiation (for example repeated CT scans) when safer imaging (like MRI) would do, in discussion with doctors.
Stay informed about chordoma research and clinical trials through reputable foundations and discuss options early with your care team.
When to See a Doctor
You should see a doctor urgently if you notice:
Constant or progressive deep neck, back or sacral pain that does not improve with rest or simple measures, especially if it wakes you at night.
New weakness, numbness or tingling in arms or legs, difficulty walking, frequent falls or loss of balance.
Problems with bladder or bowel control, or new trouble starting urination or severe constipation without a clear cause.
Double vision, trouble swallowing, slurred speech, facial numbness or hearing changes, which can signal skull-base involvement.
Sudden worsening pain, fever, or signs of infection during or after treatment.
For people already diagnosed with chordoma, contact your team promptly if any symptom suddenly worsens, if new neurological problems appear, or if you have troubling side effects from surgery, radiation or systemic drugs.
What to Eat and What to Avoid
(These tips support overall health during chordoma treatment; they do not directly “kill” the tumor.)
Eat: Plenty of colorful fruits and vegetables every day.
Avoid: Sugary drinks and sweets as everyday habits, because they add calories with little nutrition.Eat: Whole grains such as oats, brown rice and whole-grain bread for fiber and slow energy.
Avoid: Mostly refined white bread, pastries and ultra-processed snacks.Eat: Lean proteins like beans, lentils, fish, eggs, tofu and skinless poultry to support healing and muscle.
Avoid: Large portions of processed meats and deep-fried foods on a regular basis.Eat: Healthy fats from olive oil, nuts, seeds and fatty fish (for omega-3s).
Avoid: A lot of trans fats and repeatedly fried fast foods.Eat: Soft, moist foods (soups, stews, smoothies, yogurt) if swallowing is harder after skull-base treatment.
Avoid: Very dry, crumbly or sharp foods that are painful or unsafe to swallow.Eat: Foods rich in calcium and vitamin D (fortified milk, yogurt, certain fish) if approved by your team.
Avoid: Very high-dose calcium or vitamin D supplements without blood-level checks.Eat: Small, frequent meals or snacks if your appetite is low.
Avoid: Skipping many meals in a row; this can worsen weight loss and fatigue.Drink: Plenty of water and other low-sugar fluids to stay hydrated.
Avoid: Large amounts of sugary drinks and energy drinks, which can disturb blood sugar and sleep.Use cautiously: Herbal teas or supplements; always check for interactions with your oncologist.
Avoid: Starting new high-dose supplements during treatment without medical advice.General rule: Choose mostly plant-forward, minimally processed foods and adjust textures based on your swallowing and digestion, guided by a dietitian.
Frequently Asked Questions (FAQs)
Is chordoma cancer or a benign tumor?
Chordoma is a malignant (cancerous) tumor. It grows slowly but aggressively in its local area and can spread to other parts of the body. It needs specialist cancer care, not just simple observation, although small or hard-to-reach tumors may sometimes be watched very closely before treatment.What usually causes chordoma?
For most people, there is no clear cause. Chordoma develops from leftover notochord cells along the spine. A few families have genetic changes such as extra copies of the brachyury (T) gene, but most cases are random and not linked to lifestyle.Can chordoma be cured?
Some patients can live many years without evidence of disease, especially when the tumor is removed completely with clear margins and followed by high-dose proton or photon radiotherapy. However, chordoma often comes back locally, so doctors usually talk about long-term control rather than a guaranteed cure, and life-long follow-up is needed.Why is proton therapy talked about so much for chordoma?
Chordoma sits very close to the brainstem, spinal cord and nerves. Proton therapy can deliver a high dose to the tumor while lowering dose to these critical structures, which improves control and reduces some long-term side effects compared with older radiotherapy techniques.Is chemotherapy helpful in chordoma?
Traditional chemotherapy like cisplatin, doxorubicin or ifosfamide is often less effective in chordoma than in other sarcomas. It may be used in certain aggressive or pediatric cases or as part of clinical trials, but the main pillars of treatment remain surgery and high-dose radiation, with targeted and immune therapies being explored.What is targeted therapy for chordoma?
Targeted therapy uses drugs that block specific molecules such as PDGFR, VEGFR or EGFR that are active in chordoma cells. Examples include imatinib, sunitinib, sorafenib, pazopanib and afatinib. These drugs are usually used in clinical trials or specialist centers and can sometimes shrink or stabilize tumors, but responses vary and side effects still occur.What is immunotherapy for chordoma?
Immunotherapy tries to help the body’s immune system recognize and attack chordoma cells. Checkpoint inhibitors like pembrolizumab, nivolumab and avelumab block PD-1 or PD-L1 so T cells can stay active. Some patients with recurrent chordoma have had durable responses, but others do not respond, and side effects can be serious, so this approach is usually used in trials.Are there official treatment guidelines?
Yes. Multi-disciplinary groups supported by the Chordoma Foundation and ESMO have published consensus recommendations for diagnosis, first-line treatment and management of local-regional recurrence. These guidelines strongly recommend care at experienced centers and aggressive local therapy when safe.Can diet or supplements cure chordoma?
No. Diet and supplements cannot cure chordoma or replace surgery, radiation or evidence-based drugs. A healthy diet, maintaining weight and correcting deficiencies (like vitamin D) can support strength, healing and tolerance of treatment, but any supplement plan must be discussed with the oncology team to avoid harmful interactions.How often will I need scans after treatment?
Many centers recommend MRI of the treated area every few months in the first few years, then yearly if stable, sometimes supplemented with whole-spine imaging or CT scans, depending on the case. The exact plan is personalized based on tumor site, surgery type, radiation dose and any systemic therapy.Can children or teenagers get chordoma?
Yes, chordoma can occur at any age, but it is still rare in children and teenagers. Pediatric cases may behave somewhat differently and are treated at highly specialized centers with pediatric oncology and neurosurgery teams.Does chordoma spread (metastasize)?
Chordoma mainly causes problems by growing locally, but it can spread to lungs, liver, bone or other sites, especially after multiple local recurrences. Systemic therapies and clinical trials are more important when metastases appear, alongside continued control of the primary site.How long does chordoma treatment usually take?
Treatment is usually a long journey. Surgery requires weeks to months of recovery, and radiotherapy may last several weeks. Systemic therapies or clinical-trial drugs can continue for months or years as long as they work and side effects are manageable. Follow-up and rehabilitation often continue for life.Is it important to get a second opinion?
Because chordoma is so rare and complex, a second opinion at a specialized center can be very helpful. It can confirm the diagnosis, clarify whether complete resection and proton therapy are feasible, and open doors to clinical trials. Most doctors welcome this and will help share records and imaging.Where can I find trustworthy information and support?
Trusted resources include major cancer centers, published consensus guidelines and recognized patient-advocacy groups dedicated to chordoma. These organizations provide plain-language booklets, treatment center lists, clinical-trial information and peer support networks to help patients and families navigate this rare disease.
Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.
The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members
Last Updated: January 14, 2026.
