Notochordal sarcoma is another name doctors sometimes use for chordoma, a rare cancer that starts from tiny leftover pieces of the embryo’s notochord (a soft rod that later becomes part of the spine). It is a malignant (cancerous) bone tumor and belongs to the sarcoma family. These tumors grow slowly but can spread into nearby bone, nerves and soft tissues, so they are called “locally aggressive.” Most tumors sit in the midline of the body along the spine, usually at the base of the skull (clivus), the mobile spine, or the sacrum (tail bone).
Notochordal sarcoma is very rare. Studies suggest about 0.08 cases per 100,000 people each year, often in adults between 40 and 70 years old. Children can be affected, but this is uncommon. Because the tumor grows slowly and the early symptoms can be mild, diagnosis is often delayed until the tumor is large or pressing on nerves or the brainstem.
Modern classifications group these tumors under “notochordal tumours”, which include benign notochordal cell tumor (BNCT) and malignant chordoma. They share a special marker called brachyury (TBXT), a protein that helps confirm notochordal origin under the microscope.
Other names
Doctors and researchers often prefer the word chordoma instead of “notochordal sarcoma,” but they are describing the same malignant notochord-derived tumor in usual clinical use. Other names and closely related terms you may see in articles or reports include:
Chordoma – the standard medical name used in most guidelines and textbooks.
Malignant notochordal tumor – highlights that the tumor comes from notochord cells and is cancerous.
Axial chordoma / spinal chordoma / skull-base chordoma / sacrococcygeal chordoma – names that describe the main location of the tumor along the spine or skull base.
Notochordal tumor of bone – a broader phrase used in pathology to include both benign notochordal cell tumor and chordoma in the same family.
Types of notochordal sarcoma
Doctors can describe notochordal sarcoma (chordoma) in different ways: by microscope appearance (histologic type) and by tumor location.
Histologic (microscopic) types
Conventional (classic) chordoma
This is the most common type. Under the microscope, the tumor shows large cells with bubbles in the cytoplasm (“physaliphorous” cells) in a mucous-like background. It often grows slowly but invades bone and nearby structures.Chondroid chordoma
This type has areas that look like cartilage as well as typical chordoma cells. Some studies suggest a slightly better outlook than conventional chordoma, especially in skull-base tumors, but it is still a malignant tumor that can come back.Poorly differentiated chordoma
In this type, cells look more primitive and aggressive under the microscope. It is often linked to loss of a gene called SMARCB1/INI1 and tends to behave more aggressively, with higher risk of spread and recurrence.Dedifferentiated chordoma
Here, part of the tumor looks like usual chordoma, but other parts look like a high-grade sarcoma. This type grows faster and has a worse prognosis because it is more likely to spread.Benign notochordal cell tumor (BNCT) – related but not cancer
BNCT is a benign (non-cancer) lesion made of notochord-type cells, often found incidentally in the spine. Most BNCTs never cause symptoms. Very rare reports show BNCT and chordoma together in the same bone, so some experts think BNCT might sometimes be a “precursor” lesion, but the risk of change into cancer appears very low.
Types by location
Skull-base chordoma (clival chordoma)
This tumor grows at the base of the skull, near the brainstem and cranial nerves. It can cause headaches, double vision, and problems with eye movement or swallowing because of pressure on these nerves.Mobile-spine chordoma (cervical, thoracic, or lumbar)
These tumors appear in the vertebral bones of the neck, mid-back or lower back. They can cause back or neck pain, nerve root pain going into the arms or legs, and weakness or numbness from spinal cord or root compression.Sacrococcygeal chordoma (sacral chordoma)
This type arises in the sacrum or coccyx (tail bone). It may grow quite large before being noticed, causing low back or buttock pain, difficulty sitting, and bowel or bladder control problems.
Causes and risk factors
The exact cause of notochordal sarcoma (chordoma) is not fully known. Most cases happen without any clear trigger. Researchers have, however, found several genetic changes and risk patterns that seem to play a role.
Embryonic notochord remnants
The basic “cause” is that small remnants of the embryonic notochord remain trapped in the bones of the spine and skull. Very rarely, some of these remnants start to grow in an uncontrolled way and become chordoma. This is why tumors follow the midline of the spine.Brachyury (TBXT) gene changes
Many chordomas show extra copies or special variants (such as the rs2305089 SNP) in the TBXT gene, which codes for the brachyury protein. This gene is important in notochord development, and its abnormal activation seems to drive tumor growth.Chromosome 6q27 duplication
High-resolution genetic studies have found duplication of a region on chromosome 6q27 that includes the TBXT gene. This structural change likely increases brachyury levels and is thought to be a key step in tumor formation.Familial (inherited) chordoma
In some families, several members develop chordoma. Many of these families carry inherited duplication of the TBXT gene. This suggests that a strong genetic predisposition can greatly raise the risk, although familial cases remain rare.Benign notochordal cell tumor as a possible precursor
Some reports show benign notochordal cell tumor and chordoma in the same bone, and a few cases where chordoma seems to arise within a BNCT. This has led to the idea that, in some patients, a benign lesion may slowly change into malignant tumor, though this transformation appears very uncommon.Other genetic pathway changes (PI3K/AKT/mTOR, CDKN2A, EGFR, PDGFR)
Molecular studies show many chordomas have changes in growth and survival pathways, such as PI3K/AKT/mTOR, loss of CDKN2A, and abnormal activation of EGFR and PDGFR. These changes help the tumor cells grow and avoid normal cell death, although they may not be the first trigger.Epigenetic changes
Changes in how DNA is packaged and read (epigenetics), such as altered DNA methylation and histone modification, have been described in chordoma. These changes can switch genes on or off without changing the DNA code, helping tumor cells keep growing.MicroRNA and gene-expression changes
Some studies show altered patterns of small RNA molecules (microRNAs) and gene expression in chordoma tissue compared with normal bone. These patterns may support tumor survival and spread but are still being studied.Older age
Most patients are adults between 40 and 70 years. Age itself is not a direct cause, but cancer risk in many tissues increases with time, as more genetic errors accumulate in cells.Male sex
Chordoma is slightly more common in men than women. The reason for this difference is unclear, but it suggests that sex-related factors (hormonal or genetic) may modify risk.Skull-base notochord remnants
Notochord tissue normally disappears, but small rests can remain in the clivus at the base of the skull. These rests can later become the starting point for skull-base chordoma.Sacral notochord remnants
Similar notochord rests can be left behind in the sacrum and coccyx. These are the seeds from which sacral chordomas may grow many decades later.Chronic mechanical stress on the spine (possible, not proven)
Some experts suspect that long-term mechanical stress or micro-trauma to the spine might influence local bone and notochordal cells, but there is no strong direct proof. It is best to think of this as a possible modifying factor, not a confirmed cause.Radiation-related changes (rare and uncertain)
For many bone cancers, prior radiation is a known risk. For chordoma, evidence is very limited and not clear. A small number of reports suggest it could play a role in a few patients, but it is not considered a common cause.General cancer-related risk (smoking, toxins – no clear direct link)
Usual lifestyle risk factors for many cancers, such as smoking or some toxins, have not shown a strong, direct link to chordoma. Most guidelines still focus on the genetic and developmental origin rather than environment.Immune system and tumor micro-environment
Research suggests that immune cells and supporting tissue around the tumor can help or hinder growth. In chordoma, patterns of immune cells and signals in the tumor micro-environment may allow tumor cells to escape immune attack.Incomplete surgical removal in earlier lesions
When a tumor is not completely removed and microscopic disease is left behind, it can regrow locally and appear as a new or larger mass. This is not a cause of the first tumor but is a cause of recurrence.Benign notochordal lesions followed by long-term imaging only
Because BNCTs are usually just watched, the very small number that transform (if transformation truly occurs) might present later as chordoma. This highlights the importance of follow-up when BNCT is diagnosed.Genetic background and ethnicity (research in progress)
Some genetic studies suggest that certain inherited variants may slightly change risk in different populations, but data are still limited and no clear high-risk ethnic group has been defined.“Unknown cause” in most patients
Even with all this research, most individuals with notochordal sarcoma have no identifiable external cause. For many patients, the tumor likely results from a mix of random genetic changes plus the fact that notochord cells were left in that spot during development.
Symptoms
Symptoms depend mainly on the location and size of the tumor. Because growth is slow, early symptoms can be mild or vague.
Persistent deep pain at the tumor site
Many people first notice a dull, constant pain in the neck, back, or base of the skull. The pain often worsens slowly over months and may not improve with rest or simple pain medicine.Radicular pain (shooting nerve pain)
If the tumor presses on a spinal nerve root, pain can shoot down an arm or leg, sometimes described as electric or burning pain. This may be misdiagnosed as a slipped disc at first.Weakness in arms or legs
Pressure on the spinal cord or nerves can cause muscle weakness. Patients may drop objects, trip more often, or have trouble climbing stairs or lifting the arms.Numbness or tingling
Damage to sensory nerves can cause numbness, pins-and-needles, or reduced feeling in parts of the face, trunk, arms or legs, depending on the tumor level.Problems with walking and balance
When the spinal cord or brainstem is compressed, patients may have unsteady gait, difficulty walking in a straight line, or problems with coordination.Headache (especially in skull-base tumors)
Clival chordomas can cause chronic headaches due to pressure on the base of the skull or nearby structures. The pain may slowly worsen and does not follow a simple migraine pattern.Double vision or eye movement problems
Skull-base tumors can affect cranial nerves that move the eyes. Patients may notice double vision, difficulty looking to one side, or drooping eyelids, as seen in some reported cases of clival chordoma.Trouble swallowing or speaking
Tumors near the lower cranial nerves may cause difficulty swallowing, hoarseness, choking on food, or changes in voice. These symptoms point to brainstem or cranial nerve compression.Hearing changes or ringing in the ears
Some skull-base chordomas can press on hearing pathways, leading to hearing loss, ringing in the ears (tinnitus), or a sense of fullness in one ear.Visible or palpable mass near the sacrum
Large sacral tumors can cause a lump in the lower back or buttock region, sometimes noticed when sitting or lying down. The skin over the area may feel firm or tender.Bowel or bladder problems
Sacral chordomas may affect nerves that control bowel and bladder function. Patients can develop constipation, difficulty emptying the bladder, leakage of urine or stool, or loss of control (incontinence).Sexual dysfunction
Nerve involvement in the pelvis may cause erectile dysfunction, decreased genital sensation, or other sexual function problems in both men and women.Neck stiffness or back stiffness
Tumors in the cervical or thoracic spine can reduce normal spinal movement and cause stiffness or a feeling of “tightness” around the neck or chest.Fatigue and weight loss in advanced disease
In later stages, chronic pain, reduced activity, and general cancer effects can cause tiredness, loss of appetite, and unintended weight loss.Sleep disturbance and reduced quality of life
Ongoing pain and neurological symptoms often disrupt sleep and daily activities. Many patients experience emotional stress, anxiety, or low mood because of chronic symptoms and treatment burden.
Diagnostic tests
Diagnosis usually needs a combination of careful clinical examination, imaging, and tissue testing.
Physical examination tests
General physical and neurological examination
The doctor looks at overall health and checks muscle strength, reflexes, sensation, coordination, and mental status. This helps locate where the nervous system might be affected and guides which imaging tests are needed.Cranial nerve examination
For skull-base tumors, the doctor checks eye movements, facial sensation, facial muscles, hearing, swallowing, gag reflex, tongue movement, and shoulder shrug. Abnormal findings suggest pressure on specific cranial nerves near the clivus.Spine and sacral examination
The doctor examines the neck and back for tenderness, deformity, or a palpable mass and may perform a rectal or pelvic exam to feel the front of the sacrum. Pain or a lump in these areas increases suspicion for sacral chordoma.Gait and balance assessment
Walking tests, heel-toe walking, and Romberg test (standing with feet together and eyes closed) help reveal balance problems from spinal cord or brainstem compression and guide urgency of imaging.
Manual (hands-on) tests
Range-of-motion testing of neck or back
The clinician gently moves the neck or spine and asks the patient to bend or twist. Limited movement or pain at certain angles can suggest structural problems such as tumor, prompting further imaging.Straight leg raise (SLR) test
With the patient lying on their back, the doctor lifts each leg. Reproduction of radiating leg pain can indicate nerve root irritation, which may be caused by a tumor pressing on lumbosacral roots.Manual muscle strength testing
The doctor tests strength in key muscle groups of arms and legs by asking the patient to push or pull against resistance. Weakness in a pattern matching certain nerve roots or spinal cord levels supports the suspicion of a compressive lesion.Sensory and reflex testing with simple tools
Light touch, pin-prick, vibration, and temperature testing across the skin, plus tendon hammer reflex testing at knees, ankles, and elbows, help map out which nerves or spinal cord segments are affected.
Lab and pathological tests
Routine blood tests (CBC, chemistry panel)
Standard blood tests check general health, kidney and liver function, and readiness for surgery or radiotherapy. They are usually normal in chordoma but important to rule out other conditions and to plan treatment safely.Inflammatory markers (ESR, CRP)
Tests such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) may be used to help distinguish tumor from infection or inflammatory disease. In chordoma, they are often only mildly raised or even normal.CT-guided core needle biopsy
A key diagnostic step is taking a tissue sample. For many sacral and mobile-spine tumors, doctors use CT imaging to guide a special hollow needle into the mass and remove cores of tissue. The path of the needle is planned so that it can be removed later during surgery, to reduce the risk of tumor seeding.Open surgical biopsy (used selectively)
In some skull-base or complex cases, a small open operation is needed to obtain tissue safely. Surgeons try to design this biopsy so it does not interfere with later complete removal. Open biopsy is less favored for sacral and mobile-spine lesions because of higher seeding risk.Histopathology (microscopic examination)
Under the microscope, chordoma shows typical physaliphorous cells in a myxoid (mucous-like) stroma. The pathologist looks for these features and for any areas of chondroid, poorly differentiated, or dedifferentiated tumor to define the exact histologic type.Immunohistochemistry for brachyury and other markers
Tumor tissue is stained for special proteins. Nearly all notochordal tumors (BNCT and chordoma) show strong nuclear staining for brachyury. Chordoma also typically expresses cytokeratin, epithelial membrane antigen (EMA), and S100 protein. This pattern helps distinguish it from other bone tumors.Molecular and genetic testing (TBXT and other changes)
In some centers, additional tests look for TBXT gene duplication or other chromosomal changes. These tests can support the diagnosis, help with research, and may guide future targeted therapies.
Electrodiagnostic tests
Nerve conduction studies (NCS)
NCS measure how fast and how well electrical signals travel along nerves. They help assess whether limb symptoms, such as numbness or weakness, are due to nerve root compression from the tumor or from another nerve disease.Electromyography (EMG)
EMG uses small needles placed in muscles to record electrical activity at rest and during movement. Abnormal patterns can show irritation or damage of nerves leaving the spine, supporting imaging findings of a compressive lesion such as chordoma.
Imaging tests
Magnetic resonance imaging (MRI)
MRI is usually the most important imaging test. It shows the size, exact location, and extent of the tumor in bone, spinal canal, brainstem, and soft tissues. MRI helps with surgical planning and with follow-up after treatment.Computed tomography (CT) scan
CT uses X-rays to give detailed bone images. It shows bone destruction, calcification, and involvement of joints and helps plan the best surgical approach. CT is also used to guide biopsies.Plain X-ray of spine or skull base
Standard X-rays may show bone destruction or a mass in the sacrum, vertebrae, or clivus, but they are less detailed than CT or MRI. X-rays are often an early test that triggers more advanced imaging when abnormal.Positron emission tomography–CT (PET-CT) and bone scan
PET-CT and bone scintigraphy can help detect spread to other bones or distant sites and may be used in staging or follow-up. They are not always required but can provide whole-body information when needed.
Non-pharmacological treatments (therapies and others)
Multidisciplinary tumor board plan: A team (neurosurgery/orthopedic oncology, radiation oncology, medical oncology, radiology, pathology) reviews images and biopsy results together. This reduces mistakes and helps pick the safest plan for hard locations like skull base or sacrum. Purpose: best plan. Mechanism: shared expert decision-making.
Specialized imaging mapping (MRI/CT planning): High-detail scans show the exact tumor borders, nerve contact, and bone damage. This guides surgery and radiation fields. Purpose: precision. Mechanism: clearer boundary definition to avoid leaving tumor behind.
Wide/En-bloc surgical removal when possible: Removing the tumor in one piece with a margin can lower local recurrence in some sites. Purpose: strongest local control. Mechanism: fewer tumor cells left at the edges.
Skull-base endoscopic approaches (selected cases): For clival tumors, endoscopic routes may reduce brain retraction and speed recovery in some patients. Purpose: safer access. Mechanism: targeted corridor to the tumor with careful reconstruction.
Spine stabilization and reconstruction: If bone is removed, the spine may need hardware (rods/screws) or grafts. Purpose: prevent collapse and protect nerves. Mechanism: restores structural strength after tumor removal.
Proton beam radiation: Proton therapy can deliver high dose to tumor while reducing dose to nearby brainstem/spinal cord. Purpose: kill remaining cells and lower recurrence. Mechanism: particle beam dose “drop-off” helps spare normal tissue.
Carbon-ion radiation (where available): Carbon ions may be useful for radio-resistant tumors in select centers. Purpose: stronger local control options. Mechanism: higher biological effect per dose in some cancers.
IMRT (high-precision photon radiation): IMRT shapes radiation around complex tumors when particles are not available. Purpose: control tumor with less normal-tissue damage. Mechanism: many beam angles with intensity control.
Stereotactic radiosurgery / SBRT (selected cases): High-dose, very focused radiation may be used for small targets, residual disease, or recurrences depending on location and safety. Purpose: local control. Mechanism: steep dose gradients around the tumor.
Pre-radiation dental/ENT assessment (skull base cases): When radiation is near mouth/throat, dental and ENT care can reduce later complications. Purpose: prevent infections and tissue injury. Mechanism: fixing risks before radiation damage occurs.
Rehabilitation (physical therapy): Helps walking, balance, strength, and safe movement after spine/sacral surgery. Purpose: regain function. Mechanism: graded exercises retrain muscles and nerves.
Occupational therapy: Teaches safer ways to do daily tasks and may provide tools (chairs, braces, bathroom aids). Purpose: independence. Mechanism: energy saving + adaptive strategies.
Speech and swallowing therapy (skull base cases): If nerves controlling swallowing/speech are affected, therapy reduces choking risk and improves speech clarity. Purpose: safer eating and communication. Mechanism: muscle retraining and technique changes.
Pain procedures (nerve blocks / epidural injections, selected): Some patients benefit from interventional pain care. Purpose: reduce severe pain and improve sleep/mobility. Mechanism: interrupts pain signals and reduces local inflammation.
Psychological support (counseling/CBT): Cancer stress is real, especially with long treatments. Purpose: reduce anxiety and depression. Mechanism: coping skills training and support planning.
Palliative care early (not only “end of life”): Palliative teams help with pain, nausea, fatigue, sleep, and goals of care during any stage. Purpose: better quality of life. Mechanism: symptom-focused care alongside cancer therapy.
Nutrition counseling: Helps maintain weight and protein during recovery and radiation. Purpose: healing and strength. Mechanism: tailored meal planning and symptom-based adjustments.
Smoking cessation and alcohol moderation: These support wound healing and general health during cancer care. Purpose: fewer complications. Mechanism: improved circulation, immune function, and tissue repair.
Active surveillance schedule after treatment: Regular MRI/CT is used because local recurrence can happen years later. Purpose: catch recurrence early. Mechanism: planned imaging intervals to detect small regrowth.
Clinical trial participation (when available): Trials may test vaccines, targeted drugs, or new radiation methods for chordoma/notochordal tumors. Purpose: access new options. Mechanism: research treatments added to standard care under safety rules.
Drug treatments
Important: Many drugs below are not specifically FDA-approved for chordoma, but they are sometimes used for advanced sarcoma-type behavior, recurrence, or spread, based on tumor biology and specialist guidelines/experience. Always follow your cancer doctor’s plan.
Imatinib (Gleevec): A targeted drug that can help when the tumor uses certain growth signals (commonly discussed in chordoma care). Class: tyrosine-kinase inhibitor. Typical label dose concept: daily oral dosing (varies by indication). Purpose: slow tumor growth. Mechanism: blocks specific kinase signals. Key side effects: swelling, nausea, low blood counts, liver issues.
Pazopanib (Votrient): A targeted anti-angiogenic drug (reduces tumor blood-vessel signaling) used in soft-tissue sarcoma and sometimes considered in rare sarcoma settings. Class: multi-kinase inhibitor. Purpose: slow progression. Mechanism: blocks VEGF/other pathways. Key side effects: high blood pressure, diarrhea, liver toxicity risk.
Sorafenib (Nexavar): Another multi-kinase inhibitor sometimes used in hard-to-treat cancers; it may be considered in selected chordoma cases in specialist practice. Class: multi-kinase inhibitor. Purpose: disease control. Mechanism: blocks tumor growth and blood-vessel signals. Key side effects: hand-foot skin reaction, diarrhea, high blood pressure, liver injury risk.
Sunitinib (Sutent): Targeted therapy that blocks several growth signals and may be used off-label in selected rare tumors. Class: multi-kinase inhibitor. Purpose: slow tumor growth. Mechanism: inhibits VEGFR/PDGFR pathways. Key side effects: fatigue, high blood pressure, thyroid changes, liver toxicity risk.
Erlotinib (Tarceva): EGFR-pathway blocker sometimes tried in selected chordoma cases (based on pathway testing and expert choice). Class: EGFR tyrosine-kinase inhibitor. Purpose: slow growth. Mechanism: blocks EGFR signaling. Key side effects: rash, diarrhea, lung inflammation risk (rare).
Pembrolizumab (Keytruda): An immunotherapy drug that helps the immune system see cancer cells; used in many cancers and sometimes considered in rare tumors depending on biomarkers (like MSI-H/TMB-high) and specialist decision. Class: PD-1 inhibitor. Purpose: immune attack on tumor. Mechanism: removes “immune brakes.” Key side effects: immune inflammation (thyroid, lung, gut, liver).
Nivolumab (Opdivo): Another PD-1 pathway immunotherapy used in many cancers; may be considered in selected advanced cases with expert guidance. Class: immune checkpoint inhibitor. Purpose: immune control. Mechanism: PD-1 blockade. Key side effects: immune-related side effects similar to pembrolizumab.
Lenvatinib (Lenvima): Targeted anti-angiogenic drug; sometimes combined with immunotherapy in oncology. Class: multi-kinase inhibitor. Purpose: slow tumor growth. Mechanism: blocks VEGF/FGF and related signals. Key side effects: high blood pressure, diarrhea, fatigue, protein in urine.
Doxorubicin: A classic chemotherapy drug used in many sarcomas; may be used if the tumor acts like a high-grade sarcoma (for example, dedifferentiated chordoma). Class: anthracycline chemo. Purpose: shrink/slow tumor. Mechanism: damages DNA in fast-growing cells. Key side effects: low blood counts, nausea, heart toxicity risk.
Ifosfamide (IFEX): Often paired with other chemo drugs in sarcoma care when aggressive behavior is present. Class: alkylating agent. Purpose: tumor kill. Mechanism: DNA cross-linking. Key side effects: low blood counts, bladder irritation, confusion risk (rare).
Cisplatin (Platinol): Platinum chemotherapy used in several cancers and sometimes in sarcoma regimens depending on the plan. Class: platinum agent. Purpose: damage tumor DNA. Mechanism: DNA cross-links. Key side effects: kidney toxicity, hearing changes, nausea, nerve damage.
Carboplatin (Paraplatin): Another platinum drug, sometimes used when cisplatin is not suitable. Class: platinum agent. Purpose: tumor control. Mechanism: DNA damage. Key side effects: low blood counts, nausea, allergy reactions (possible).
Paclitaxel (Taxol): A chemotherapy that blocks cell division; used in many cancers and sometimes in combinations. Class: taxane chemo. Purpose: slow/stop division. Mechanism: stabilizes microtubules. Key side effects: nerve pain, low blood counts, allergy reactions.
Docetaxel (Taxotere): Another taxane often combined with gemcitabine in sarcoma protocols. Class: taxane chemo. Purpose: tumor control. Mechanism: blocks mitosis. Key side effects: low blood counts, fluid retention, mouth sores.
Gemcitabine (Gemzar): Often used in soft-tissue sarcoma combinations (like gemcitabine+docetaxel). Class: antimetabolite chemo. Purpose: slow growth. Mechanism: interferes with DNA building blocks. Key side effects: low blood counts, fatigue, liver enzyme changes.
Etoposide (Etopophos): A chemo that blocks DNA repair; sometimes used in aggressive cancer regimens. Class: topoisomerase inhibitor. Purpose: kill dividing cells. Mechanism: DNA strand break accumulation. Key side effects: low blood counts, hair loss, infection risk.
Cyclophosphamide: Used in several cancer regimens; sometimes chosen based on sarcoma-type treatment plans. Class: alkylating agent. Purpose: tumor kill. Mechanism: DNA cross-linking. Key side effects: low blood counts, bladder irritation, fertility risks.
Methotrexate: Used in some cancer protocols (dose and schedule depend heavily on the cancer plan). Class: antimetabolite. Purpose: slow cell growth. Mechanism: blocks folate pathway needed for DNA. Key side effects: mouth sores, liver toxicity, low blood counts (dose-dependent).
Vincristine (vincristine sulfate): A chemo that disrupts cell division; used in combination regimens. Class: vinca alkaloid. Purpose: stop cell division. Mechanism: blocks microtubule formation. Key side effects: nerve damage (tingling/weakness), constipation.
Decitabine (Dacogen): Mainly a blood-cancer drug, but sometimes discussed in research/complex cancer biology settings; it should only be used when a cancer specialist has a clear reason. Class: hypomethylating agent. Purpose: change abnormal gene “switching.” Mechanism: affects DNA methylation. Key side effects: low blood counts, infection risk.
Dietary molecular supplements (supportive only, not cures)
Important: Supplements can interact with cancer drugs and surgery/radiation. Always tell your doctor what you take.
Vitamin D: Helps bone health and muscle function, which matters when tumors affect bone and mobility. Dose: follow clinician advice; too much can be harmful. Function: supports calcium balance. Mechanism: hormone-like effects on calcium absorption and bone remodeling.
Omega-3 fatty acids (EPA/DHA): May support heart health and help with inflammation and appetite in some patients. Dose: varies; food sources are preferred when possible. Function: cell membrane support. Mechanism: changes inflammatory signaling molecules.
Zinc: Supports immune function and wound healing, but excess zinc can be harmful. Dose: do not exceed safe limits unless prescribed. Function: enzyme support. Mechanism: helps many immune and repair enzymes work.
Turmeric/Curcumin (careful with interactions): People use it for inflammation, but it can interact with medicines. Dose: varies widely; avoid high doses without guidance. Function: anti-inflammatory support. Mechanism: affects multiple inflammation pathways; evidence is mixed.
Calcium (only if you need it): Helpful if dietary intake is low, especially with low vitamin D or bone weakness. Dose: based on diet and labs. Function: bone strength. Mechanism: provides mineral for bone; works with vitamin D.
Magnesium: Can help if low due to poor intake or treatment effects (some therapies can alter minerals). Dose: follow clinician advice. Function: muscle/nerve support. Mechanism: cofactor for many enzymes and nerve signaling.
Vitamin B12: Useful only if you are deficient (which can worsen weakness and nerve symptoms). Dose: depends on lab results. Function: nerve and blood support. Mechanism: helps DNA and nerve myelin maintenance.
Iron (only if deficient): Helps anemia when iron is low; not everyone needs it. Dose: based on labs. Function: oxygen carrying capacity. Mechanism: supports hemoglobin production.
Probiotics (choose carefully): May help gut symptoms, but some cancer patients should avoid live bacteria products (immunosuppression). Dose: product-specific. Function: gut balance. Mechanism: may support microbiome and bowel function.
Protein/essential amino acid supplements (nutrition support): If eating is hard, protein shakes can help maintain weight and muscle. Dose: depends on body needs. Function: healing. Mechanism: provides building blocks for tissue repair and immune proteins.
Immunity booster / regenerative / stem cell support drugs (supportive care)
These medicines do not treat the tumor directly. They help the body recover from low blood counts or support transplantation plans when used for specific reasons.
Filgrastim (Neupogen): Dose (label concept): weight-based daily injections in certain settings. Function: raises neutrophils. Mechanism: G-CSF stimulates bone marrow to make white blood cells.
Pegfilgrastim (Neulasta): Dose (label concept): single injection per chemotherapy cycle in indicated settings. Function: lowers febrile neutropenia risk. Mechanism: long-acting G-CSF effect.
Epoetin alfa (Epogen/Procrit): Dose: individualized; used only when appropriate due to risks. Function: helps anemia in certain patients. Mechanism: stimulates red blood cell production. Key risk: can increase clot risk and has important warnings.
Darbepoetin alfa (Aranesp): Similar to epoetin, longer-acting. Function: anemia support in selected cases. Mechanism: stimulates erythropoiesis. Key risk: clot risk and important warnings; must be used carefully.
Sargramostim (Leukine): Function: supports recovery of certain white cells in specific medical situations. Mechanism: GM-CSF stimulates marrow cell growth. Use: only when your oncology team indicates it.
Plerixafor (Mozobil): Function: helps move stem cells into blood for collection in approved settings. Mechanism: blocks CXCR4 interaction to mobilize stem cells (used with G-CSF). Note: for transplant collection plans, not routine cancer care.
Surgeries (procedures and why they are done)
En-bloc tumor resection (wide removal): Done when surgeons can remove the tumor in one piece with a margin. Why: best chance of local control in some cases. Core idea: fewer cells left behind at the cut edge.
Skull-base tumor resection (open or endoscopic): Removes clival/skull-base tumors while protecting brainstem and cranial nerves. Why: reduce pressure and remove cancer. Core idea: careful corridor + reconstruction to prevent leaks/infection.
Sacrectomy (partial or total, selected cases): Removal of sacral bone tumor when feasible. Why: local control and pain relief. Core idea: remove tumor while planning for nerve function and stability.
Spinal decompression: Removes bone/tumor pressing on spinal cord or nerves. Why: protect movement, bowel/bladder, and reduce pain. Core idea: relieve pressure on nerve tissue.
Reconstruction/stabilization surgery: Hardware, grafts, or flaps may be needed after removal. Why: keep spine stable and help wounds heal. Core idea: rebuild support and cover sensitive areas.
Preventions (realistic and honest)
There is no guaranteed prevention for notochordal sarcoma/chordoma because it usually comes from leftover embryologic cells and is very rare. Prevention here means reducing complications and catching problems early.
Do not ignore new midline bone pain (deep spine/sacral pain that persists). Early evaluation can find tumors sooner.
Seek care early for new nerve symptoms (weakness, numbness, new bladder/bowel issues). These can mean pressure on nerves.
Use specialized centers for rare bone tumors when possible, because correct surgery and radiation planning matters.
Follow imaging follow-up faithfully after treatment, because recurrence can happen later.
Keep a “med list” and share it before surgery/radiation/chemo to prevent harmful interactions.
Stop smoking to support healing and reduce complications from surgery and radiation.
Maintain protein and hydration during treatment to support wound healing and recovery.
Prevent falls after spine/sacral surgery (home safety, assist devices) to protect reconstruction.
Protect skin in radiation areas (skin care plan from your radiation team) to reduce irritation and infection risk.
Vaccines and infection prevention when your immune system is low (hand hygiene, masks in crowded places when advised). This reduces dangerous infections during treatment.
When to see a doctor urgently
Go to urgent care/emergency (or call your oncology team fast) if you have new weakness, new trouble walking, loss of bladder/bowel control, severe uncontrolled pain, or fever during cancer treatment, because these can be signs of nerve compression or serious infection.
See a doctor soon (not waiting weeks) for persistent deep back/sacral pain, a growing lump, worsening numbness/tingling, or headaches/vision/swallowing changes when the tumor is near the skull base.
What to eat and what to avoid
Eat enough protein (eggs, fish, chicken, lentils, yogurt) to heal after surgery and maintain muscle. Avoid: skipping meals when possible.
Choose high-fiber foods (vegetables, oats) to reduce constipation, especially if pain medicines slow the bowel. Avoid: very low-fiber diets when constipated.
Use healthy fats (fish, nuts, olive oil). Omega-3 foods can be part of a balanced plan. Avoid: extreme “supplement megadoses” without approval.
Stay hydrated (water, soups). Hydration supports energy and kidney health, especially if chemo is used. Avoid: dehydration.
Vitamin D + calcium from food when possible (milk, fortified foods, fish). Avoid: high-dose vitamin D without monitoring.
Iron-rich foods if anemic (meat, beans, spinach with vitamin C foods). Avoid: iron pills unless labs show you need them.
Limit alcohol during treatment because it can worsen dehydration and interact with medicines.
Avoid smoking and tobacco because it slows wound healing and increases complications.
Be careful with turmeric/curcumin supplements because interactions are possible. Avoid: starting high-dose herbal products without your doctor.
Food safety if immune-suppressed (well-cooked foods, clean water). Avoid: raw or risky foods if your team says your counts are low.
FAQs
Is notochordal sarcoma the same as chordoma? Often, yes in everyday talk—many people mean chordoma (a notochord-origin sarcoma-family tumor). Some cases are more aggressive forms like dedifferentiated chordoma.
Where does it usually grow? Most are at the skull base, spine, or sacrum, along the body’s midline.
Is it fast or slow? Classic chordoma is often slow-growing but locally aggressive; dedifferentiated forms can grow faster.
What is the main treatment? Surgery when possible, often with radiation (like proton therapy) to control leftover cells or reduce recurrence.
Why is it hard to treat? It sits next to vital nerves and vessels, so doctors must balance “remove all tumor” with “protect function.”
Can radiation be used without surgery? Sometimes, if surgery is too risky or not possible; radiation can also be used after surgery.
Do medicines cure it? Medicines rarely cure by themselves; they are mainly used when disease returns, spreads, or cannot be fully removed, and choices are individualized.
Are targeted drugs used? They can be considered in selected cases (example: imatinib and other TKIs), especially under specialist guidance.
Is immunotherapy used? Sometimes, especially when biomarkers suggest it may help; it depends on tumor testing and expert decision.
Does it spread to other organs? It can metastasize in some patients, and the risk is higher in aggressive subtypes, so follow-up matters.
How long is follow-up needed? Often for many years, because recurrence can happen later.
Can diet cure it? No. Diet supports strength and healing, but it does not replace surgery/radiation/oncology treatment.
Are supplements always safe? No—some interact with cancer drugs. Always ask your oncology team first.
When is palliative care used? Any time symptoms need help (pain, fatigue, sleep), even while receiving active treatment.
What is the best next step after diagnosis? Get care at a center experienced with chordoma/rare sarcomas and ask for a clear plan covering surgery, radiation, and follow-up.
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.
Chordoma
