Notochordoma

Notochordoma is another name for chordoma, a very rare cancer that grows from tiny “leftover” cells of the notochord, an early structure that helps form the spine in the embryo. After birth, most notochord cells disappear, but a few can stay inside the bones of the spine or skull base and, many years later, can turn into this slow-growing bone cancer.

Notochordoma is a term people sometimes use when they mean chordoma. Chordoma is a rare, slow-growing cancer that starts from leftover notochord cells (cells that helped form the spine before birth). It usually grows in the skull base (clivus), spine, or sacrum (tailbone area). Even though it can grow slowly, it can invade nearby nerves and bone, and it can come back after treatment, so care is usually done by a specialist team.

Chordoma usually grows in three main places: at the base of the skull (clivus), along the mobile spine (neck, chest, lower back), and at the bottom of the spine (sacrum and coccyx). It grows slowly but can be dangerous because it sits next to the brain, spinal cord, and important nerves, so even a “small” tumor can cause serious problems.

Other Names

Doctors and books may use several names for the same disease. These words are closely related:

• Chordoma – the most common and preferred medical name today.

• Notochordoma – older or alternative term that highlights that the tumor comes from notochord cells.

• Notochordal sarcoma – another synonym; “sarcoma” means a cancer that starts from bone, cartilage, or soft tissue.

• Notochordal tumor with malignant behavior – used in some pathology texts to stress that it has notochord-type cells under the microscope.

All these names describe the same family of cancers that arise from notochord remnants along the spine and skull base.

Types of Notochordoma (Chordoma)

Doctors describe chordoma by where it grows and by how it looks under the microscope.

• Skull base (clival) chordoma – grows at the base of the skull, usually in a bone called the clivus. It often causes headaches, double vision, or problems with eye movements because it presses on cranial nerves.

• Mobile spine chordoma – grows in the vertebrae of the neck, chest, or lower back (cervical, thoracic, lumbar). It can cause back or neck pain, weakness, and numbness in arms or legs.

• Sacrococcygeal chordoma – grows at the bottom of the spine (sacrum and coccyx). It often causes deep low-back or buttock pain, and changes in bowel or bladder control.

• Extra-axial (extra-spinal) chordoma – very rare tumors that appear outside the usual skull-base or spine area, such as in soft tissues nearby.

Histologic (microscope) types:

• Conventional (classic) chordoma – the most common type (around 80–90%). Cells look “bubbly” (physaliferous) in a jelly-like background.

• Chondroid chordoma – a variant that looks partly like cartilage (chondrosarcoma) and partly like classic chordoma; it often appears in the skull base.

• Dedifferentiated / poorly differentiated chordoma – rare, more aggressive types where part of the tumor looks very high-grade and grows faster, with higher risk of spread.

Causes and Risk Factors

For chordoma, doctors do not know one single clear cause. There are no proven lifestyle, food, or environmental triggers. Most cases seem to happen by chance, but several risk factors and mechanisms are known.

1. Remnant notochord cells staying after birth
   Chordoma can only happen if some notochord cells remain inside the bones of the skull base or spine. These “leftover” cells are normal at first, but in rare people they later change into cancer cells.

2. Changes (mutations) in the TBXT (brachyury) gene inside the tumor
   Many chordomas show extra copies or increased activity of a gene called TBXT, which makes the brachyury protein. This gene helps control notochord development, and when it is abnormal, it can push cells toward tumor growth.

3. Inherited TBXT gene duplication in some families
   In a few families, several relatives have chordoma, and they share an inherited extra copy (duplication) of the TBXT gene. This family change makes chordoma more likely but still does not guarantee it.

4. Other rare inherited gene changes
   Studies suggest that some people carry rare variants in other genes linked to cell growth and DNA repair. These variants may increase chordoma risk slightly, but they are uncommon and still under research.

5. Tuberous sclerosis complex (TSC1 or TSC2 variants)
   Children with tuberous sclerosis complex (a genetic condition affecting many organs) have a higher chance of developing chordoma than other children, likely because TSC1 or TSC2 gene changes disturb cell-growth control.

6. General DNA damage over a lifetime
   Like many cancers, random DNA damage that builds up in notochord remnants over many years may turn normal cells into tumor cells, even when no single outside “cause” can be found.

7. Older age
   Chordoma usually appears in adults between about 40 and 70 years, and incidence peaks in older age groups, which suggests that time and accumulated genetic damage play a role.

8. Male sex
   Men are affected more often than women (about 1.5–2 times more), so being male is a recognized risk factor, although the reason is not fully understood.

9. Being from some racial or ethnic groups
   Population studies show chordoma is more common in White people, and patterns may differ by race and country. This suggests genetic background may influence risk.

10. Large pool of at-risk notochord cells in skull base and sacrum
    Notochord cells tend to be left behind especially in the clivus and sacrococcygeal area, which explains why chordoma is most common there. The more remnants, the more “targets” for mutations.

11. Abnormal cell-growth signaling pathways
    Studies show many chordomas have overactive pathways like PI3K–AKT–mTOR and receptor tyrosine kinases (PDGFR, EGFR, KIT). These make cells grow and survive more than they should and may help drive tumor formation.

12. Loss of tumor-suppressor genes (for example, CDKN2A / CDKN2B)
    Deletions of genes that normally slow down cell division, such as CDKN2A and CDKN2B, are common in chordoma. When these “brakes” are lost, cells can divide in an uncontrolled way.

13. Very large tumor size at diagnosis (as a marker of long growth)
    Many chordomas are large when first found, meaning they have been growing quietly for years. Large size reflects long-term unchecked growth caused by the genetic changes described above.

14. Dedifferentiated / poorly differentiated histology
    Some tumors change from conventional to more aggressive subtypes (dedifferentiated, poorly differentiated). This shift is linked to extra mutations that give the cells more aggressive behavior.

15. Local micro-environment in bone and cartilage
    Notochord remnants sit inside bone and cartilage, which supply growth factors and support tissues. This environment may help mutated cells survive and grow into chordoma.

16. Possible second cancers or genetic syndromes in the family
    Some people with familial chordoma also have other tumors, suggesting a shared inherited weakness in DNA repair or growth control across tissues.

17. Very rare childhood cases linked to strong inherited factors
    When chordoma appears in very young children, doctors strongly suspect a genetic cause (such as TBXT duplication or TSC), because random age-related changes are less likely at that age.

18. Lack of proven lifestyle or environmental causes
    It is also important to know what does not cause chordoma: no clear link with smoking, alcohol, diet, trauma, or common workplace exposures has been proven. This suggests internal genetic factors are more important.

19. Possible (but unproven) contribution from prior radiation
    For many bone tumors, high-dose radiation to the area is a risk factor. For chordoma, this link is not clearly proven but is sometimes considered in individual cases.

20. Chance (“sporadic”) events in a single cell
    Most chordomas are called sporadic, meaning they happen in people with no family history and no known trigger. In these cases, the “cause” is most likely a random mix of DNA changes over time in one notochord cell.

Common Symptoms of Notochordoma

Symptoms depend on where the tumor is and how big it has grown. Many symptoms are due to pressure on the spinal cord, brain stem, or nearby nerves.

1. Deep, persistent pain near the tumor
   The most common symptom is slow, constant pain in the skull base, neck, back, or sacrum where the tumor sits. Pain often worsens over months or years and may be worse at night or with movement.

2. Headaches (especially for skull-base tumors)
   A clival chordoma can cause headaches because it increases pressure at the base of the skull and irritates nearby structures and nerves.

3. Neck pain and stiffness
   Tumors in the upper spine or skull base can cause neck pain and reduced range of motion as the mass presses on joints, muscles, and ligaments.

4. Back or sacral pain
   Sacral chordomas often cause dull, deep pain in the lower back, buttocks, or tailbone region, sometimes radiating down the legs.

5. Weakness in arms or legs
   When the tumor compresses the spinal cord or nerve roots, people may notice weakness, heaviness, or clumsiness in the limbs.

6. Numbness or tingling
   Damage or pressure on sensory nerves can cause “pins and needles,” numbness, or burning feelings in the face, arms, trunk, or legs, depending on the tumor level.

7. Trouble walking or balance problems
   Spinal cord compression can affect coordination and balance. People may stumble, feel unsteady, or need support to walk.

8. Double vision or eye-movement problems
   Skull-base chordomas can press on nerves that control eye muscles (especially cranial nerve VI), causing double vision or difficulty moving the eyes normally.

9. Facial numbness or weakness
   Pressure on cranial nerves can lead to numbness, tingling, or weakness in the face, or difficulty closing the eye or moving facial muscles.

10. Difficulty swallowing or speaking
    A skull-base tumor can affect nerves that control swallowing and speech, causing choking, hoarseness, or changes in voice.

11. Changes in hearing or ringing in the ears
    If nearby hearing or balance nerves are compressed, people may notice hearing loss, buzzing, or a feeling of fullness in the ear.

12. Bladder problems (difficulty starting, incontinence, or retention)
    Sacral chordomas can damage nerves that control the bladder, leading to trouble starting urination, leakage, or not being able to empty fully.

13. Bowel changes (constipation or incontinence)
    Pressure on sacral nerves can cause constipation, loss of bowel control, or a feeling of incomplete emptying.

14. Visible or palpable mass in the sacrum or neck
    Some people notice a lump or swelling in the lower back, buttock, or neck area where the tumor extends toward the skin.

15. Fatigue and general unwell feeling
    Long-term pain, nerve problems, and the stress of a chronic tumor can lead to tiredness, poor sleep, and reduced activity, even though routine blood tests may look normal.

Diagnostic Tests for Notochordoma

Doctors combine your story, physical exam, imaging scans, and tissue tests to diagnose chordoma. There is no single blood test that can confirm it.

Physical Examination (Basic Clinical Checks)

1. General physical and neurologic examination
   The doctor asks about symptoms and checks strength, reflexes, sensation, balance, and coordination. This helps locate which part of the brain, spinal cord, or nerves might be pressed by a tumor.

2. Cranial nerve examination (for skull-base tumors)
   For suspected skull-base chordoma, the doctor carefully tests eye movements, facial sensation, facial strength, swallowing, hearing, and tongue movement to see which cranial nerves are affected.

3. Spine and sacral region inspection and palpation
   For spinal or sacral tumors, the doctor looks and feels along the spine and tailbone area for tenderness, swelling, or a mass, and checks range of motion of the neck and back.

Manual Clinical Tests (Hands-On Bedside Tests)

4. Manual muscle strength testing
   The doctor asks you to push or pull against resistance with your arms and legs. Weakness in certain muscle groups points to pressure on specific nerve roots or parts of the spinal cord.

5. Sensory testing (touch, pain, and vibration)
   Light touch, pinprick, and vibration are checked along the skin. Areas of numbness or abnormal feeling help map which nerves are involved and where the tumor might be pressing.

6. Straight leg raise and nerve tension tests
   For sacral or lumbar tumors, lifting the leg while lying down can stretch the sciatic nerve. Pain shooting down the leg suggests nerve root compression from a mass like chordoma or a large disc.

Laboratory and Pathological Tests

7. Basic blood tests (CBC, kidney and liver function)
   Routine blood tests are usually normal in chordoma but are used to check general health, look for anemia, and plan surgery or radiation. There are no specific blood tumor markers for chordoma.

8. Inflammatory markers (ESR, CRP)
   These tests may be slightly raised in some patients but are not specific. Doctors sometimes use them to rule out infection or other inflammatory conditions when evaluating back pain.

9. Image-guided core needle biopsy
   To confirm the diagnosis, doctors usually take a small cylinder of tissue from the tumor using a needle guided by CT or other imaging. The sample is then sent to the lab for detailed study.

10. Open surgical biopsy (when needle biopsy is not possible)
    In some cases, especially at the skull base, a surgeon must perform a small operation to remove a piece of the tumor safely. The tissue gives the pathologist enough material to make an accurate diagnosis.

11. Histopathology with H&E staining
    Under the microscope, chordoma shows lobules of “bubbly” physaliferous cells in a mucoid (gel-like) background. This classic picture on standard hematoxylin and eosin (H&E) stain supports the diagnosis.

12. Immunohistochemistry for brachyury
    Brachyury is a key protein that marks notochord-type cells. Strong nuclear staining for brachyury is highly specific and sensitive for chordoma and helps distinguish it from other tumors in the same area.

13. Immunohistochemistry panel (cytokeratin, EMA, S100, GFAP as needed)
    Chordoma cells usually stain positive for epithelial markers like cytokeratins and EMA, and often S100. A panel of these markers plus brachyury helps separate chordoma from chondrosarcoma and other skull-base or spinal tumors.

Electrodiagnostic Tests

14. Nerve conduction studies (NCS)
    When limb weakness or numbness is present, NCS can help show whether peripheral nerves are damaged or slowed by compression from the tumor, and can help distinguish root compression from other nerve diseases.

15. Electromyography (EMG)
    EMG measures electrical activity in muscles. It can show ongoing nerve injury from long-term compression by a spinal or sacral chordoma, helping guide how urgent decompression might be.

16. Somatosensory evoked potentials (SSEP) in selected cases
    In some centers, SSEP tests are used before or during surgery to monitor spinal cord function, especially when the tumor lies close to critical neural pathways.

Imaging Tests (Scans)

17. Plain X-ray of skull base or spine
    X-rays can sometimes show bone destruction, abnormal bone shape, or a lytic mass, especially in the sacrum. However, small or early chordomas may be missed, so more advanced scans are almost always needed.

18. CT scan (computed tomography)
    CT shows detailed bone changes, such as destruction of the clivus or sacrum and calcifications inside the tumor. It is very helpful for surgical planning and for guiding biopsies.

19. MRI scan with and without contrast (key imaging test)
    MRI is the best test to see chordoma. It shows the size of the tumor, how it spreads into the brain, spinal cord, or soft tissues, and how close it is to nerves and vessels. Chordomas often show a “honeycomb” pattern of contrast enhancement.

20. PET-CT or bone scan to look for spread (metastasis)
    PET-CT or bone scans may be used to look for distant spread to lungs, bones, or other organs, especially in advanced or recurrent cases. This helps with staging and treatment planning.

21. Follow-up MRI for monitoring
    After treatment, repeated MRI scans are used to check for tumor regrowth or spread, because chordomas have a high chance of coming back in the same area.

Non-pharmacological treatments (therapies and others)

1. Specialist team care (sarcoma/skull-base/spine team) helps because chordoma needs surgeons, radiation experts, and rehab working together. The purpose is safer surgery and better long-term control. The mechanism is simple: the team plans one clear strategy (how to remove tumor, how to protect nerves, and how to use high-dose radiation) instead of piece-by-piece decisions.

2. Maximal safe tumor removal (complete resection when possible) is often the main step because chordoma does not respond well to standard chemo. The purpose is to remove as much tumor as possible with clean edges. The mechanism is physical: removing the tumor lowers the number of cancer cells and can reduce pressure on nerves and brain/spinal structures.

3. High-dose radiation after surgery is commonly used to kill leftover microscopic tumor cells. The purpose is to reduce the chance the tumor grows back. The mechanism is that radiation damages tumor DNA so cells cannot keep dividing. Many centers use radiation because chordoma can recur if even small cells remain.

4. Pre-operative radiation (selected cases) may be used in some sacral or spine tumors. The purpose is to weaken tumor cells before surgery and sometimes make margins easier. The mechanism is the same DNA damage effect, and the pre-surgery timing may help when the tumor is hard to remove completely in one piece.

5. Proton beam radiation is used often for chordoma because it can deliver high dose while sparing nearby brainstem, spinal cord, or bowel. The purpose is strong tumor control with fewer side effects. The mechanism is the proton “dose drop-off,” which reduces dose beyond the tumor.

6. Carbon ion radiation (where available) is another focused, high-energy option used in some countries/centers. The purpose is better control for radio-resistant tumors or hard locations. The mechanism is more “biologic punch” to DNA damage than standard photons, which may help in chordoma care plans.

7. Stereotactic radiosurgery or stereotactic body RT (selected small targets) may be used for small residual or recurrent spots. The purpose is a very focused boost dose. The mechanism is delivering high precision radiation in fewer sessions to reduce spill into nerves and vital tissues.

8. Careful imaging follow-up schedule (MRI/CT monitoring) is a treatment strategy, not just “checking.” The purpose is to catch recurrence early when surgery or radiation is easier. The mechanism is early detection: small regrowth can be treated sooner before it causes nerve damage or spreads.

9. Physical therapy (PT) helps after surgery or radiation to restore strength, walking, balance, and posture. The purpose is better daily function and less disability. The mechanism is guided exercise that retrains muscles and protects the spine while healing, reducing stiffness and weakness that can happen after major treatment.

10. Occupational therapy (OT) focuses on normal life skills (bathing, dressing, work setup). The purpose is independence. The mechanism is training, tools, and home/work changes that reduce strain on healing spine or pelvis and improve safety, especially if nerves were affected.

11. Speech and swallow therapy (skull-base tumors) can be important if surgery/radiation affects swallowing, voice, or speech. The purpose is safer eating and better communication. The mechanism is exercises and strategies that retrain muscles and reduce aspiration risk when cranial nerves are irritated.

12. Pain rehabilitation program (multimodal, not only pills) can include movement, pacing, relaxation, and nerve-friendly positions. The purpose is less pain and more activity. The mechanism is reducing pain sensitization and improving muscle support around the tumor area after surgery/radiation.

13. Psychological support (CBT, counseling) helps with anxiety, fear of recurrence, and long treatment stress. The purpose is better coping and sleep. The mechanism is changing unhelpful thought loops, building routines, and lowering stress hormones that can worsen pain and fatigue during cancer care.

14. Nutrition counseling supports healing and weight stability (important for wound healing and strength). The purpose is fewer complications and better energy. The mechanism is adequate protein, calories, vitamins, and hydration, adjusted for nausea, mouth/throat problems, or bowel changes from treatment.

15. Smoking cessation improves wound healing and lung function and lowers complications after surgery. The purpose is safer recovery and better overall health. The mechanism is improved blood flow and oxygen delivery to tissues, which helps surgical sites and reduces infection risk.

16. Prehabilitation (“prehab”) before surgery means strengthening, breathing exercises, and nutrition before the operation. The purpose is faster recovery and fewer complications. The mechanism is building physical reserve so the body handles surgery stress better, especially in major sacral or skull-base operations.

17. Pelvic floor rehab (some sacral tumors) may help bladder/bowel control issues. The purpose is improved continence and quality of life. The mechanism is training and strengthening specific muscles and learning techniques to manage nerve-related bowel/bladder changes after sacral surgery.

18. Assistive devices (brace, cane, walker, seating supports) can reduce falls and pain. The purpose is safe movement. The mechanism is mechanical support: stabilizing spine/pelvis during healing and taking pressure off painful areas while you rebuild strength.

19. Palliative care early (supportive care) is not “giving up.” The purpose is better symptom control (pain, fatigue, nausea) alongside active treatment. The mechanism is a specialist team that adjusts meds, sleep, mood, and daily plans so you can tolerate surgery/radiation/systemic therapy better.

20. Clinical trial participation (when available) can give access to new targeted or immune therapies. The purpose is more options in advanced or recurrent disease. The mechanism is receiving treatments studied specifically for chordoma biology (targets like PDGFR/EGFR and others), with close monitoring.

Drug treatments

Important: As of current common guidance, many drugs used for chordoma are not specifically FDA-approved for chordoma, but may be used off-label or in trials when surgery/radiation are not enough. Dosing must be chosen by an oncologist based on your case and the drug label.

1. Imatinib (Gleevec) – TKI: Often considered when the tumor shows PDGFR pathway activity. Purpose: slow growth and sometimes improve pain more than shrink the tumor. Mechanism: blocks certain tyrosine kinases (including PDGFR) involved in tumor signaling. Typical label dosing depends on approved cancer type; tablets are taken by mouth with food/water; your doctor individualizes dosing for chordoma. Side effects can include fluid retention, nausea, muscle cramps, and low blood counts.

2. Erlotinib (Tarceva) – EGFR inhibitor: Used in some advanced chordoma cases when EGFR signaling is relevant. Purpose: slow tumor activity when other options are limited. Mechanism: blocks EGFR tyrosine kinase signaling that can support tumor growth. Label dosing is oral and taken on an empty stomach in its approved uses; chordoma dosing is individualized. Side effects often include rash and diarrhea; rare serious lung or liver problems can occur.

3. Sunitinib (Sutent) – multi-kinase TKI: Sometimes used in chordoma based on small studies and expert experience. Purpose: slow progression by blocking growth signals and blood vessel growth. Mechanism: inhibits VEGFR/PDGFR and related kinases. Label dosing uses cycles in approved cancers (e.g., “weeks on/weeks off” schedules), but chordoma plans vary. Side effects can include fatigue, high blood pressure, hand-foot syndrome, and thyroid changes.

4. Sorafenib (Nexavar) – multi-kinase TKI: Used in some chordoma patients when disease is advanced. Purpose: slow tumor growth and limit tumor blood supply. Mechanism: blocks RAF kinases and VEGFR pathways. Label dosing is oral (commonly twice daily in approved uses), but chordoma use is individualized. Side effects can include hand-foot skin reaction, diarrhea, high blood pressure, and bleeding risk.

5. Pazopanib (Votrient) – VEGFR TKI: Considered in selected advanced bone/soft-tissue tumor settings and sometimes for chordoma. Purpose: reduce tumor blood vessel support and slow progression. Mechanism: blocks VEGFR, PDGFR, and c-KIT signaling. Label dosing is oral once daily in its approved uses; liver monitoring is important. Side effects can include liver injury risk, high blood pressure, diarrhea, and hair/skin color changes.

6. Regorafenib (Stivarga) – multi-kinase TKI: Sometimes considered when other TKIs fail. Purpose: slow progression in heavily treated disease. Mechanism: blocks multiple kinases involved in tumor growth and angiogenesis. Label dosing is oral with a schedule (days on, days off) in approved cancers; chordoma dosing is clinician-chosen. Side effects include hand-foot skin reaction, liver toxicity risk, fatigue, and hypertension.

7. Dasatinib (Sprycel) – TKI: Studied because it hits PDGFR/Src family pathways that may matter in chordoma biology. Purpose: slow growth in selected cases. Mechanism: kinase inhibition affecting tumor signaling and invasion pathways. Label dosing is oral once daily in approved leukemia uses; chordoma use is off-label. Side effects can include low blood counts, fluid around lungs, and bleeding risk.

8. Everolimus (Afinitor) – mTOR inhibitor: Sometimes used alone or combined in complex cases based on pathway testing. Purpose: reduce cell growth signals and slow progression. Mechanism: inhibits mTOR, a key growth pathway in cells. Label dosing is oral daily in approved cancers; chordoma dosing varies. Side effects may include mouth sores, infections, high blood sugar/lipids, and lung inflammation.

9. Temsirolimus (Torisel) – mTOR inhibitor (IV): Used in selected advanced settings or combinations in some reports. Purpose: slow tumor growth when oral options are limited. Mechanism: mTOR pathway inhibition. Label dosing is IV weekly in its approved use; chordoma use is individualized and needs close monitoring. Side effects can include rash, mouth sores, high blood sugar/lipids, and infection risk.

10. Sirolimus (Rapamune) – mTOR inhibitor (oral): Sometimes used in combinations (specialist-directed) to target mTOR signaling. Purpose: slow tumor growth signaling, usually as an add-on strategy. Mechanism: mTOR inhibition. Label use is as an immunosuppressant (transplant), so infection risk is important. Side effects can include high cholesterol/triglycerides, mouth sores, slow wound healing, and infections.

11. Pembrolizumab (Keytruda) – PD-1 immunotherapy (IV): Considered when biomarkers (like high TMB/MSI-H) or clinical context suggests benefit, though chordoma evidence is still emerging. Purpose: help the immune system attack tumor cells. Mechanism: blocks PD-1 “brakes” on T-cells. Label dosing is IV on a schedule (e.g., every few weeks). Side effects can be immune-related (thyroid, lungs, colon, liver).

12. Nivolumab (Opdivo) – PD-1 immunotherapy (IV): Another immune checkpoint option sometimes tried in advanced disease or trials. Purpose: immune-based tumor control. Mechanism: PD-1 blockade to increase T-cell activity. Label dosing is IV; schedules vary by indication. Side effects can include immune-mediated inflammation (skin, gut, lung, endocrine organs).

13. Ipilimumab (Yervoy) – CTLA-4 immunotherapy (IV): Sometimes combined with PD-1 therapy in certain cancers and can be considered only by specialists/trials for rare tumors. Purpose: stronger immune activation in selected cases. Mechanism: blocks CTLA-4 immune checkpoint. Label dosing is IV, often in limited number of doses. Side effects can be stronger immune reactions (colitis, hepatitis, endocrine issues).

14. Cetuximab (Erbitux) – EGFR antibody (IV): Used in EGFR-driven cancers and sometimes considered in chordoma research/selected cases. Purpose: block EGFR signaling at the cell surface. Mechanism: antibody binds EGFR and reduces downstream growth signaling. Label dosing is IV with premedication due to infusion reactions. Side effects include acne-like rash, low magnesium, and infusion reactions.

15. Bevacizumab (Avastin) – anti-VEGF (IV): Sometimes used to reduce tumor blood vessel support in selected cancers and investigational settings. Purpose: slow angiogenesis (new blood vessels). Mechanism: binds VEGF so tumors get less blood-supply signaling. Label dosing is IV. Side effects can include high blood pressure, bleeding, clotting risk, protein in urine, and wound-healing issues (important around surgery).

16. Denosumab (Xgeva) – bone protection (SC injection): This does not treat chordoma directly, but may help if there is bone damage, risk of skeletal events, or certain bone-related complications (specialist decision). Purpose: reduce bone breakdown. Mechanism: blocks RANKL, lowering osteoclast activity. Label dosing is injections every 4 weeks in approved uses. Side effects include low calcium and rare jaw bone problems; dental checks matter.

17. Zoledronic acid (Zometa) – bone protection (IV): Also supportive, not chordoma-specific. Purpose: reduce bone complications and treat high calcium of malignancy if it happens. Mechanism: bisphosphonate that slows bone resorption. Label dosing is IV, with kidney monitoring. Side effects can include flu-like symptoms, kidney strain, and rare jaw osteonecrosis; hydration and dental care matter.

18. Dexamethasone (a corticosteroid) – symptom control: Used for swelling, nerve irritation, or inflammation around tumor areas, especially brain/spine symptoms (doctor-directed). Purpose: reduce pressure symptoms and pain. Mechanism: strong anti-inflammatory effect that reduces edema. Dosing is individualized and usually short-term when possible. Side effects include high sugar, mood changes, sleep problems, infection risk, and stomach irritation.

19. Morphine (or other opioid pain medicines) – pain control: Supportive but often necessary in advanced disease. Purpose: control severe cancer pain so you can move, sleep, and participate in therapy. Mechanism: binds opioid receptors to reduce pain signal transmission. Dosing and timing are individualized; constipation prevention is usually needed. Side effects include constipation, drowsiness, nausea, and breathing risk if misused—only use as prescribed.

20. Ondansetron (anti-nausea) – supportive care: Helps nausea from radiation, pain medicines, or systemic therapy. Purpose: maintain hydration and nutrition. Mechanism: blocks serotonin (5-HT3) signals that trigger vomiting. Taken before treatments or as needed per clinician. Side effects can include constipation and headache; rare heart rhythm effects in high-risk people, so doctors adjust as needed.

Dietary molecular supplements (supportive, not a cure)

1. Vitamin D may be used if levels are low, to support bone strength and muscle function. Typical doses depend on blood tests; many people need daily maintenance, while deficiency needs higher short-term dosing. Mechanism: supports calcium absorption and bone remodeling. Avoid mega-doses without testing because excess can raise calcium too much.

2. Calcium can support bone health, especially if using bone-targeted drugs or if intake is low. Dose depends on diet and blood calcium levels. Mechanism: mineral for bone structure and nerve/muscle function. Too much can cause constipation or kidney stones in prone people, so balance food plus supplements carefully with clinician advice.

3. Omega-3 (EPA/DHA) may help with inflammation balance and maintaining weight/appetite in some cancer patients. Typical doses vary; take with food. Mechanism: affects cell membrane signaling and inflammatory mediators. High doses can increase bleeding tendency, so tell your doctor, especially if you are on blood thinners or near surgery.

4. Protein powder (whey/plant) can help meet protein goals when chewing/swallowing is hard (skull base treatment) or appetite is low. Dose is based on daily protein targets. Mechanism: provides amino acids for muscle repair and wound healing. Choose products with simple ingredients; if kidney disease exists, your clinician may limit protein.

5. Creatine monohydrate can support strength during supervised rehab in some people. Typical dose is small daily maintenance after a short loading phase (optional). Mechanism: increases phosphocreatine stores for short-burst muscle energy. Avoid if your doctor is concerned about kidney function or if you are dehydrated often during treatment.

6. Magnesium may be needed if levels drop (this can happen with some EGFR drugs like cetuximab). Dose depends on blood magnesium and bowel tolerance. Mechanism: supports nerve and muscle function and helps prevent cramps. Too much can cause diarrhea, so start low and monitor.

7. Zinc may help taste changes and wound healing if deficient. Typical dose is modest and time-limited unless deficiency is proven. Mechanism: supports immune and skin repair enzymes. Too much zinc for long periods can lower copper and cause anemia/nerve issues, so avoid high chronic dosing without labs.

8. Vitamin B12 can be useful if you have low B12 or nerve symptoms from deficiency. Dose depends on labs (oral or injection). Mechanism: supports nerve insulation and red blood cell production. It is usually safe, but taking it “just because” can hide other causes of anemia, so check levels when possible.

9. Folate (folic acid or methylfolate) may help only if folate is low. Dose is usually daily. Mechanism: supports DNA repair and red blood cell formation. Do not take high folate without checking B12, because folate can improve anemia while nerve damage from B12 deficiency continues silently.

10. Probiotics (selected strains) may help bowel regularity during stress, pain meds, or diet changes. Dose depends on product. Mechanism: supports gut microbiome balance. If you are severely immunosuppressed, have a central line, or are very low in white cells, ask your doctor first because rare infections can occur.

Immunity booster / regenerative / stem-cell support” medicines

1. Filgrastim (G-CSF) is used when white blood cells (neutrophils) drop from cancer treatment. Purpose: reduce infection risk and keep treatment on schedule. Mechanism: stimulates the bone marrow to make neutrophils. Dosing is individualized by weight and lab counts. Side effects can include bone pain and rare spleen issues, so labs and symptoms must be monitored.

2. Pegfilgrastim (long-acting G-CSF) has a similar purpose but lasts longer, so it may be given once per cycle depending on the regimen. Mechanism: sustained stimulation of neutrophil production. Dosing and timing depend on the chemo plan. Side effects include bone pain and rare serious reactions; your oncology team decides if you need it.

3. Sargramostim (GM-CSF) may be used in special situations to support certain white cell lines. Purpose: immune cell recovery support. Mechanism: stimulates marrow to produce granulocytes and macrophage-related cells. Dosing is individualized. Side effects can include fever, bone pain, and fluid shifts, so it is used with careful monitoring by clinicians.

4. Epoetin alfa (ESA for anemia) may be used in selected cancer-related anemia cases. Purpose: reduce transfusion need and improve fatigue from low hemoglobin. Mechanism: stimulates red blood cell production. Dosing depends on hemoglobin and cancer setting. ESAs can increase clot risk and are not used in every patient, so it must be a careful oncology decision.

5. Plerixafor (stem-cell mobilizer) is used when doctors need to collect blood stem cells for transplant in certain diseases (not routine for chordoma). Purpose: move stem cells from marrow into blood for collection. Mechanism: blocks CXCR4 interaction, releasing stem cells. Dosing is clinician-controlled. This is only relevant if your team is doing a transplant-type plan for another reason.

6. Pembrolizumab or nivolumab as “immune boost” (checkpoint therapy) is not a vitamin-type booster; it is a prescription immune therapy used only when the cancer plan supports it. Purpose: help immune cells recognize and attack tumor cells. Mechanism: PD-1 blockade. Dosing is IV. Side effects can be serious immune inflammation, so this must be supervised by oncology.

 Surgeries (procedures and why it is done)

1. En bloc resection (removing tumor in one piece when possible) is done to achieve “clean margins” and reduce recurrence risk. It is often the goal for sacral or mobile spine tumors when anatomy allows. It may require complex reconstruction afterward because bone and soft tissue are removed with the tumor.

2. Endoscopic endonasal skull-base surgery (through the nose) can be used for some clival chordomas. It is done to reach the tumor with less external cutting and to protect brain structures. The approach depends on tumor size and extension; surgeons balance removal against nerve and vessel safety.

3. Craniofacial/skull-base open resection is used when the tumor extends beyond what endoscopic routes can safely reach. It is done to remove more tumor and reduce pressure on the brainstem and cranial nerves. The trade-off is larger surgery and longer recovery, so it’s chosen when benefit outweighs risk.

4. Sacrectomy (partial or total) removes chordoma in the sacrum. It is done to achieve local control when the tumor sits in the tailbone area. Because sacral nerves control bowel, bladder, and sexual function, the team discusses expected nerve outcomes clearly before surgery and plans rehab afterward.

5. Spinal stabilization and reconstruction (hardware, grafts, flaps) is done when tumor removal weakens the spine/pelvis or leaves a large defect. Purpose: protect the spinal cord/nerves, allow safe movement, and help wound healing. Mechanism: mechanical support (rods/screws) plus soft-tissue coverage to reduce infection and breakdown.

Preventions (mostly preventing recurrence and complications)

1. Get treated at a center experienced in chordoma to reduce incomplete surgery and suboptimal radiation dosing.

2. Follow the planned radiation course fully if your team recommends it, because dose and completeness matter for control.

3. Keep all follow-up scans on schedule so recurrence is found early.

4. Report new nerve symptoms quickly (new numbness, weakness, swallowing trouble) to prevent permanent damage.

5. Do PT/rehab consistently to prevent stiffness, falls, and loss of function.

6. Stop smoking to prevent wound problems and improve overall recovery.

7. Protect bone health (vitamin D/calcium if deficient; weight-bearing as allowed) to prevent fractures and weakness.

8. Dental check before bone-targeted drugs (denosumab/zoledronic acid) to reduce jaw complications.

9. Manage blood pressure and liver tests if using TKIs, to prevent dangerous side effects.

10. Ask about clinical trials early (not only after many treatments) to access better-matched therapies sooner.

When to see doctors

See a doctor urgently if you have new severe headache, new weakness/numbness, trouble walking, new bladder or bowel control problems, trouble swallowing, or vision changes, because chordoma can press on nerves and critical structures. Also contact your cancer team quickly for fever, shortness of breath, uncontrolled diarrhea, yellow eyes/skin, or severe rash, especially if you are on targeted therapy or immunotherapy.

What to eat and what to avoid

1. Eat enough protein (eggs, fish, chicken, lentils, yogurt) to help healing and strength.

2. Choose high-fiber foods (vegetables, oats, fruits) to prevent constipation from pain medicines.

3. Drink enough water unless your doctor limits fluids, because dehydration worsens fatigue and constipation.

4. Use soft, easy foods if swallowing is hard (soups, smoothies, mashed foods) and ask for swallow therapy.

5. Prefer healthy fats (olive oil, nuts, fish) to maintain weight when appetite is low.

6. Avoid alcohol excess, especially with liver-risk TKIs, because it can stress the liver.

7. Avoid grapefruit with many cancer drugs because it can change drug levels (ask your pharmacist for your exact list).

8. Limit ultra-processed foods when possible, because they can displace needed nutrients during recovery.

9. Avoid raw/unsafe foods (unpasteurized, raw eggs/seafood) if your white blood cells are low.

10. Do not start supplements without telling your doctor because some can increase bleeding or interact with TKIs/immune therapy.

FAQs

1. Is notochordoma the same as chordoma? Many people use it to mean chordoma; chordoma is the recognized diagnosis name in most medical references.

2. Is chordoma cancer? Yes, it is a rare malignant tumor that can invade nearby tissue and sometimes spread.

3. Where does it usually occur? Most commonly at the skull base, spine, or sacrum.

4. Why is surgery important? Removing the tumor (often as completely as safely possible) is a major way to control the disease.

5. Why do many patients need radiation too? Radiation helps kill leftover tumor cells after surgery and lowers recurrence risk.

6. What type of radiation is often used? High-dose, highly focused radiation such as proton therapy is common in many centers.

7. Does chemotherapy usually work? Traditional chemotherapy often has limited benefit; specialists may use targeted or immune drugs in advanced cases.

8. Is there an FDA-approved drug specifically for chordoma? Many systemic drugs used are off-label or in trials; treatment is individualized by specialists.

9. Can chordoma come back after treatment? Yes, recurrence is a known risk, so long-term imaging follow-up matters.

10. What symptoms should make me worry? New weakness, numbness, bowel/bladder issues, swallowing trouble, or vision problems need prompt evaluation.

11. Do targeted drugs cure chordoma? Usually they aim to slow growth or control symptoms rather than cure; response differs by person.

12. What are common targeted drug side effects? Many TKIs can cause fatigue, diarrhea, skin problems, high blood pressure, and liver issues, so monitoring is needed.

13. What are immune therapy risks? Checkpoint drugs can cause immune attacks on organs (thyroid, lungs, gut, liver), so new symptoms must be reported early.

14. Can diet cure chordoma? Diet cannot cure chordoma, but good nutrition helps healing, strength, and treatment tolerance.

15. Should I consider a clinical trial? If available, trials can offer therapies designed around chordoma biology and are worth discussing early.

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.