Chordoma – Causes, Symptoms, Diagnosis, Treatment

Last updated: February 8, 2026Reviewed date: February 8, 2026Reading time: 9 min read
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A chordoma is a low-grade, slow-growing, but locally invasive and locally aggressive tumor. Chordomas belong to the sarcoma family of tumors. They arise from the remnants of the notochord and occur in the midline along the spinal axis from the clivus to the sacrum, anterior...

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বাংলা রোগী নোট এখনো যোগ করা হয়নি। পোস্ট এডিটরে “RX Bangla Patient Mode” বক্স থেকে সহজ বাংলা সারাংশ যোগ করুন।

এই তথ্য শিক্ষা ও সচেতনতার জন্য। এটি ডাক্তারি পরীক্ষা, রোগ নির্ণয় বা প্রেসক্রিপশনের বিকল্প নয়।

Article Summary

A chordoma is a low-grade, slow-growing, but locally invasive and locally aggressive tumor. Chordomas belong to the sarcoma family of tumors. They arise from the remnants of the notochord and occur in the midline along the spinal axis from the clivus to the sacrum, anterior to the spinal cord. The location distribution of chordomas is 50% sacral, 35% skull base, and 15% occur in the...

Key Takeaways

  • This article explains Causes of Chordoma in simple medical language.
  • This article explains Symptoms of Chordoma in simple medical language.
  • This article explains Diagnosis of Chordoma in simple medical language.
  • This article explains Treatment of Chordoma in simple medical language.
Educational health guideWritten for patient understanding and clinical awareness.
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  • Any symptom that feels urgent, unusual, or unsafe for the patient.
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2

See a doctor

Book a professional medical evaluation if symptoms persist, worsen, recur often, affect daily activities, or occur in a high-risk patient.

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Definition

A chordoma is a low-grade, slow-growing, but locally invasive and locally aggressive tumor. Chordomas belong to the sarcoma family of tumors. They arise from the remnants of the notochord and occur in the midline along the spinal axis from the clivus to the sacrum, anterior to the spinal cord. The location distribution of chordomas is 50% sacral, 35% skull base, and 15% occur in the vertebral bodies of the mobile spine (most commonly the C2 vertebrae followed by the lumbar then thoracic spine). Overall 5-year survival is approximately 50%, and treatment is en bloc surgical resection followed by high-dose conformal radiation therapy such as proton beam radiation.

Classification

Based upon location, a chondroma can be described as an enchondroma or ecchondroma.

  • enchondroma – tumor grows within the bone and expands it
  • ecchondroma – grows outward from the bone (rare)

Causes of Chordoma

Chordomas arise from the notochord. The notochord is the mesodermal structure in the embryo, which serves to help signal tissues for organization and differentiation. The notochord ultimately becomes the nucleus pulposus in humans as it regresses. Genes implicated in chordoma formation include the brachyury gene, mechanistic target of rapamycin (mTOR) signing pathway, phosphatase, and tensin homolog (PTEN) gene deficiency, INI-1 and platelet-derived growth factor receptor beta (PDGFR-beta) although no definitive genetic marker has as of yet been identified.  There are currently a few reported familial clusters of chordomas.

Symptoms of Chordoma

Chondromas grow slowly and can be present for a long time without any symptoms. They are often noticed during unrelated medical procedures.

Symptoms of a chondroma may include:

  • A mass or bump
  • pain in the head or upper neck. সহজ বাংলা: মাথাব্যথা।" data-rx-term="headache" data-rx-definition="Headache means pain in the head or upper neck. সহজ বাংলা: মাথাব্যথা।">Headache
  • Swelling
  • Vision changes

In rare cases, a chondroma can turn into cancer. When it does, it’s called chondrosarcoma. Symptoms of chondrosarcoma in the skull base include:

  • Headaches
  • Hearing problems
  • Swelling
  • Vision problems

Diagnosis of Chordoma

History and Physical

The patient history and physical findings are dependent on the specific location of the chordoma. Skull base and clival chordomas typically present with headaches and or cranial nerve dysfunctions, most often cranial nerve VI (abducens nerve), although the lower cranial nerves can also be affected. Rarely a clival chordoma will present with rhinorrhea due to a cerebrospinal fluid leak. Cervical chordomas typically present with non-specific neck, shoulder or arm pain, and occasionally dysphagia due to mass effect. Cervical chordomas can also invade cranially to cause lower cranial nerve dysfunction as well as compression of the spinal cord or exiting nerves causing weakness, numbness, balance trouble, or coordination problems. সহজ বাংলা: স্পাইনাল কর্ডের সমস্যা।" data-rx-term="myelopathy" data-rx-definition="Myelopathy means spinal cord dysfunction, often causing weakness, numbness, balance trouble, or coordination problems. সহজ বাংলা: স্পাইনাল কর্ডের সমস্যা।">myelopathy or radiculopathy, respectively. Thoracic and lumbar chordomas also present with non-specific localized pain and, also, maybe the cause of a pathologic fracture or radiculopathy or myelopathy. Sacral chordomas share a similar presentation as thoracic and lumbar chordomas with localized pain and possible radiculopathy as well as possible dysfunction of the bladder, bowel, or autonomic nervous system if the tumor involves the lumbosacral plexus.

Evaluation

The following tests and procedures may be used

  • Physical exam and history – An exam of the body to check general signs of health, including checking for signs of disease, such as high blood pressure or anything else that seems unusual. A history of the patient’s health habits and past illnesses and treatments will also be taken.
  • Twenty-four-hour urine test – A test in which urine is collected for 24 hours to measure the amounts of catecholamines in the urine. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher-than-normal amounts of certain catecholamines may be a sign of pheochromocytoma.
  • Blood catecholamine studies – A procedure in which a blood sample is checked to measure the amount of certain catecholamines released into the blood. Substances caused by the breakdown of these catecholamines are also measured. An unusual (higher than or lower than normal) amount of a substance can be a sign of disease in the organ or tissue that makes it. Higher-than-normal amounts of certain catecholamines may be a sign of pheochromocytoma.
  • CT scan (CAT scan) – A procedure that makes a series of detailed pictures of areas inside the body, such as the neck, chest, abdomen, and pelvis, taken from different angles. The pictures are made by a computer linked to an x-ray machine. A dye may be injected into a vein or swallowed to help the organs or tissues show up more clearly. This procedure is also called computed tomography, computerized tomography, or computerized axial tomography.
  • NMRI (Nuclear magnetic resonance imaging) – A procedure that uses a magnet, radio waves, and a computer to make a series of detailed pictures of areas inside the body such as the neck, chest, abdomen, and pelvis. This procedure is also called nuclear magnetic resonance imaging (NMRI).
  • MIBG scan – A procedure used to find neuroendocrine tumors, such as pheochromocytoma and paraganglioma. A very small amount of a substance called radioactive MIBG is injected into a vein and travels through the bloodstream. Neuroendocrine tumor cells take up the radioactive MIBG and are detected by a scanner. Scans may be taken over 1-3 days. An iodine solution may be given before or during the test to keep the thyroid gland from absorbing too much of the MIBG.
  • Scan – A type of radionuclide scan used to find certain tumors, including tumors that release catecholamine. A very small amount of radioactive (a hormone that attaches to certain tumors) is injected into a vein and travels through the bloodstream. The radioactive attaches to the tumor and a special camera that detects radioactivity is used to show where the tumors are in the body.
  • FDG-PET scan (fluorodeoxyglucose-positron emission tomography scan) – A procedure to find malignant tumor cells in the body. A small amount of FDG, a type of radioactive glucose (sugar), is injected into a vein. The PET scanner rotates around the body and makes a picture of where glucose is being used in the body. Malignant tumor cells show up brighter in the picture because they are more active and take up more glucose than normal cells do.
  • Immunohistochemistry – A diagnosis. সহজ বাংলা: রক্ত/প্রস্রাব/নমুনা পরীক্ষা।" data-rx-term="laboratory test" data-rx-definition="A laboratory test examines blood, urine, tissue, or other samples to help diagnosis. সহজ বাংলা: রক্ত/প্রস্রাব/নমুনা পরীক্ষা।">laboratory test that uses antibodies to check for certain antigens (markers) in a sample of a patient’s tissue. The antibodies are usually linked to an enzyme or a fluorescent dye. After the antibodies bind to a specific antigen in the tissue sample, the enzyme or dye is activated, and the antigen can then be seen under a microscope. This type of test looks for the enzyme SDH in a sample of the patient’s tissue. When SDH is not present, it is called SDH-deficient GIST. It is important to know whether the cancer is SDH-deficient in order to plan treatment.
  • Bone scan – A nuclear imaging method to evaluate any degenerative and/or arthritic changes in the joints, to detect bone diseases and tumors and to determine the cause of bone pain or infection, or irritation, often causing pain, swelling, heat, or redness. সহজ বাংলা: শরীরের প্রদাহ; ব্যথা, ফোলা বা লালভাব হতে পারে।" data-rx-term="inflammation" data-rx-definition="Inflammation is the body’s response to injury, infection, or irritation, often causing pain, swelling, heat, or redness. সহজ বাংলা: শরীরের প্রদাহ; ব্যথা, ফোলা বা লালভাব হতে পারে।">inflammation.
  • Biopsy – The removal of cells or tissues so they can be viewed under a microscope by a pathologist to check for signs of cancer.
    • Fine-needle aspiration – The removal of tissue using a thin needle.
    • Endoscopy – A procedure to look at organs and tissues inside the body to check for abnormal areas. An endoscope is inserted through an incision (cut) in the skin or opening in the body, such as the mouth or anus. An endoscope is a thin, tube-like instrument with a light and a lens for viewing. It may also have a tool to remove tissue or lymph node samples, which are checked under a microscope for signs of disease.

Evaluation of chordomas revolves around imaging and biopsy. A plan x-ray will demonstrate a locally destructive lytic ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।" data-rx-term="lesion" data-rx-definition="A lesion is an abnormal area of tissue such as a spot, wound, patch, lump, or ulcer. সহজ বাংলা: শরীরের অস্বাভাবিক দাগ, ক্ষত বা ফোলা অংশ।">lesion. Computed tomography (CT) imaging is better for demonstrating the destructive lytic chordoma. Occasionally the chordoma will have sclerosis at the margin. Chordomas are hypodense compared to bones on CT and may demonstrate irregular dystrophic calcification. Chordomas demonstrate moderate to significant enhancement in contrasted CT imaging. Magnetic resonance imaging (MRI) best delineates the extent of a chordoma. Chordomas have lower signal intensity on T1-weighted imaging and may show foci of hyperintensity, which represents intratumoral hemorrhage. T1-weighted imaging with gadolinium contrast demonstrates heterogeneous contrast enhancement of the tumor with a honeycomb appearance. On T2-weighed imaging, chordomas tend to be hyperintense. Gradient-echo MRI imaging can confirm intratumoral hemorrhage. Bone scans are sometimes obtained during the workup, and chordomas have normal to decreased uptake.

Many times a needle or open biopsy is performed to confirm the diagnosis of chordoma. Care needs to be taken when planning and performing the biopsy as the chordoma can seed along the biopsy tract. Thus the biopsy tract should be included in the future chordoma resection to decrease the risk of local recurrence.

Treatment of Chordoma

The treatment which provides the longest survival is complete en bloc resection of the tumor with clean margins. This can prove to be challenging either due to the location of the chordoma or reconstructive needs after resection. Intratumoral resection and piecemeal resection may also provide a similar benefit if complete chordoma resection can be achieved without local seeding. Local debulking is sometimes advocated if complete resection is not technically feasible.  Local debulking can alleviate symptoms secondary to mass effect as well as provide a smaller target volume for future radiation therapy.

Most physicians advocate for radiation therapy after any type of chordoma resection due to the high local recurrence rate. Chordomas are relatively radioresistant, necessitating high-dose radiation therapy. As chordomas are found near neuronal structures, highly-conformal radiotherapy is used, including proton beam radiation or radiosurgery. Conventional photon radiation is currently thought to be of no benefit to chordoma patients.

The slow-growing nature of chordomas makes them resistant to most current conventional chemotherapeutic agents. If a chordoma is treated with chemotherapy, then treatment typically occurs within a clinical trial.

Due to the high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. Metastatic chordoma should be on the differential if new lesions arise elsewhere in the body as up to 20% of chordomas can metastasize.

References

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What to tell the doctor

  • Write when the problem started and how it changed.
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  • What is the most likely cause of my symptoms?
  • Which danger signs mean I should go to hospital quickly?
  • Which tests are necessary now, and which can wait?
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  • Basic physical examination by a clinician
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  • Discuss physiotherapy, X-ray, or MRI only when clinically needed.

OTC medicine safety

  • For mild back pain, pain-relief medicine may be discussed with a doctor or pharmacist.
  • Avoid repeated painkiller use if you have kidney disease, stomach ulcer, uncontrolled blood pressure, or are taking blood thinners.

Avoid these mistakes

  • Do not start antibiotics without a proper medical decision.
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Get urgent help if

  • Back pain with leg weakness, numbness around private area, loss of urine/stool control, fever, cancer history, or major injury needs urgent care.
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Doctor to discuss: Orthopedic / spine specialist, physical medicine doctor, or qualified clinician
Tests to discuss with doctor
  • Neurological examination for leg power, sensation, reflexes, and straight leg raise
  • X-ray only if injury, deformity, long-lasting pain, or doctor suspects bone problem
  • MRI discussion if severe nerve symptoms, weakness, bladder/bowel problem, or persistent symptoms
Questions to ask
  • What is the most likely cause of my symptoms?
  • Which warning signs mean I should go to emergency care?
  • Which tests are really needed now?
  • Which medicines are safe for my age, pregnancy status, allergy, kidney/liver/stomach condition, and current medicines?
  • Is physiotherapy, posture correction, or activity modification needed?

Emergency warning signs such as chest pain, severe breathing difficulty, sudden weakness, confusion, severe dehydration, major injury, or loss of bladder/bowel control need urgent medical care. Do not wait for online information.

Safe pathway to proper treatment

Care roadmap for: Chordoma – Causes, Symptoms, Diagnosis, Treatment

Use this simple roadmap to understand the next safe steps. It is educational and does not replace examination by a doctor.

Go to emergency care if you notice:
  • Severe or rapidly worsening symptoms
  • Breathing difficulty, chest pain, fainting, confusion, severe weakness, major injury, or severe dehydration
Doctor / service to discuss: Qualified healthcare provider; specialist depends on symptoms and examination.
  1. Step 1

    Check danger signs first

    If danger signs are present, seek emergency care and do not wait for online information.

  2. Step 2

    Record the symptom story

    Write when symptoms started, severity, medicines already taken, allergies, pregnancy status, and test results.

  3. Step 3

    Visit a qualified clinician

    A doctor, nurse, or qualified healthcare provider can examine you and decide which tests or treatment are needed.

  4. Step 4

    Do only useful tests

    Do tests after clinical assessment. Avoid unnecessary tests, random antibiotics, or repeated medicines without diagnosis.

  5. Step 5

    Follow up and return early if worse

    If symptoms worsen, new warning signs appear, or treatment is not helping, return for review quickly.

Rural patient practical tips
  • Take a written symptom diary and all previous prescriptions/test reports.
  • Do not hide medicines already taken, even herbal or over-the-counter medicines.
  • Ask which warning signs mean urgent referral to hospital.

This roadmap is for education. A real diagnosis and treatment plan requires history, examination, and clinical judgment.

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Frequently Asked Questions

Is this article a replacement for a doctor?

No. It is educational content only. Patients should consult a qualified clinician for diagnosis and treatment.

When should I seek urgent care?

Seek urgent care for severe symptoms, rapidly worsening condition, breathing difficulty, severe pain, neurological changes, or any emergency warning sign.

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