Chordoma is a slow-growing cancer that can affect the spine and skull base. It is most common in men and women over 30 years of age.
Symptoms vary depending on where the tumour is located within the spine. Pain is the most common symptom.
Surgery is the main treatment. Radiation therapy can be used before surgery to shrink the tumour, and after surgery to kill any cancer cells that have been left behind.
Chordoma is a rare type of bone cancer, or sarcoma. It happens most often in the bones of the skull or spine, but it can form anywhere in your body. The tumors grow slowly, but they may cause symptoms for years before doctors find them. They may also recur in the same place after treatment. In about 40 percent of cases, the cancer spreads to other parts of the body.
Doctors diagnose chordoma by taking a medical history and doing a physical exam. They will ask about any symptoms you have and how long they’ve been bothering you. Then they’ll order imaging tests, such as an X-ray or computed tomography (CT) scan. The most common symptoms are back pain and problems with the nerves of your legs and bladder. Your doctor may also ask about your family history of the disease and your past health problems.
You may be referred for a biopsy. This is a procedure that involves removing a small sample of tissue from the suspected tumor for testing. Specially trained doctors called pathologists examine the cells under a microscope to see if they are cancerous.
When a doctor suspects that you have a chordoma, they’ll often refer you to a specialist center for further tests and treatment. This is because these cancers are so rare and can be difficult to diagnose. A specialist center will have doctors who are experienced in diagnosing bone cancers, like chordoma.
Your healthcare team will also include radiologists to suggest the diagnosis through imaging tests, such as CT/CAT and MRI scanning, pathologists who make a diagnosis from a sample of tissue taken from the tumor (biopsy), surgeons to operate, and oncologists who treat you after surgery with radiation and drugs.
You may receive radiation before surgery to shrink the tumor and make it easier to remove. And you may be given radiation after surgery to destroy any remaining cancer cells and prevent them from growing. Some people also get chemotherapy as well. You might have other treatments, too, depending on where your tumor is and how serious it is.
A person with a suspected chordoma may be referred to a neurologist who specializes in brain and spinal tumors. The doctor will ask about your medical history and perform a general physical. This will include checking your alertness, coordination, eye movement and muscle strength. The doctor will also do a neurological exam to see how your nerves work.
X-rays and computed tomography (CT) scans may be done to locate the tumor. Magnetic resonance imaging (MRI) is usually used to diagnose a chordoma because it shows the location of the tumor and what is around it, such as bones, muscles and blood vessels. It can help doctors decide what type of surgery is needed to remove the tumor.
If a chordoma is in the skull or spine, doctors will likely need to do a biopsy to confirm the diagnosis and get information about what type of cancer it is. Because this is a rare disease, it is important to find a team of doctors who have experience treating these tumors.
The team of doctors will include experts in ear, nose and throat medicine (otolaryngology), cancer (oncology) and radiation therapy (radiation oncology). They will develop a treatment plan that is tailored to the needs of each patient in partnership with you.
In some cases, the team will recommend radiation before surgery to shrink a tumor and make it easier to remove. They will also use radiation after surgery to destroy any remaining cancer cells and prevent them from growing back. They may also use a new type of radiation called proton beam therapy.
After surgery, the team will do regular MRIs to check for recurrence of the tumor or other problems. It is very important to attend follow-up appointments so that doctors can treat any problems as soon as they appear.
A person with a chordoma in the sacrum or coccyx is likely to be treated at one of the national bone tumour referral centres, which are located at the Royal National Orthopaedic Hospital in Stanmore Middlesex which is part of London Sarcoma Service, the Nuffield Orthopaedic Centre Oxford, the Royal Orthopaedic Hospital in Birmingham and the Robert Jones and Angus Hunt Orthopaedic Hospital in Oswestry.
Unlike other bone tumors that grow quickly and can cause symptoms right away, chordomas are slow-growing and may grow for years before doctors discover them. This, combined with their non-specific symptom profile and their relative rarity, makes them difficult to diagnose. Once diagnosed, these tumors can be challenging to treat because they often grow in the skull base or spine and can compress important structures such as arteries, nerves or the spinal cord.
MRI and CT scans can show the presence of a chordoma and help doctors decide what treatment plan is best for you. To confirm a diagnosis, your doctor will take a small piece of tissue from the tumor for laboratory testing (biopsy).
A multidisciplinary team of experts at a specialized cancer center offers the best chance for survival and quality of life for patients with chordoma. This team should include surgeons, radiation oncologists and medical oncologists with expertise in this rare bone tumor.
Surgery is the mainstay of treatment for chordoma, and it provides the longest survival. However, the success of surgical management depends on the type and grade of the tumor. Conventional chordomas, those that start in the sacrum or bottom set of vertebrae in the spine, grow more slowly and tend to have a better prognosis than dedifferentiated tumors, which are cancerous but develop more quickly.
If possible, surgeons aim for a complete resection with clean margins. This is sometimes difficult, however, due to the location of the tumor or if the spine needs reconstruction after resection. If a complete resection isn’t feasible, surgeons will try to remove as much of the tumor as possible. This is known as “debulking” surgery.
Many people who undergo surgery for chordoma are also given radiotherapy to kill any remaining cancer cells. Radiation oncologists use a type of radiation called external beam therapy to deliver high-energy beams that destroy cancer cells from outside the body. This is also a common treatment for other types of bone cancer and for recurrent chordomas. When possible, this treatment is given in combination with surgery to improve the chances of a good outcome.
Chordoma is a very rare malignant bone tumor of the skull-base, axial skeleton and sacrum that presents as a clinical challenge despite surgical and chemoradiotherapeutic advances. Its low to intermediate histological grade and malignant behaviour, recurrence after surgery and local growth along the neurovascular anatomy lead to significant morbidity and mortality in a significant proportion of patients. The extent of surgical resection and the presence/absence of adequate margins are key prognostic factors. In addition, chordomas have a tendency to seed resection cavities and recur locally. Therefore, they should only be cared for by a highly experienced multidisciplinary surgical team at a specialized center.
Chordomas are presumed to derive from undifferentiated vestiges of the embryonic notochord, a structure that coordinates cell fate and development. While the exact mechanism of transformation from notochordal vestige to chordoma remains unclear, chromosomal and cell cycle aberrations have been reported. Platelet-derived growth factor (PDGF) receptor, epidermal growth factor (EGF) receptor and hepatocyte growth factor (HGF) receptor tyrosine kinases are overexpressed in chordomas and correlate with progression to malignancy. Brachyury expression is also associated with chordoma progression and relates to clinical outcome in skull-base chordomas [43].
The presenting features of skull-base chordomas vary according to their location. Skull-base chordomas may manifest with headaches, cranial neuropathies and endocrinopathies, while spinal chordomas can cause back pain, radiculopathy and/or sacral dysfunction (e.g. bowel/bladder dysfunction) [44].
On radiographs, skull-base chordomas usually appear as well-circumscribed expansile soft tissue masses with lytic bony destruction and sparing of intervertebral discs. On CT, they have a ring-like pattern of expansion around the periphery with myxoid and gelatinous areas within. Intratumoral hyperintensities are typically sequestrated bone fragments (except in chondroid chordomas), whereas calcification is not seen. On MRI, chordomas have predominant T2-weighted hyperintensities with hypointense septations and scattered calcifications. Unlike conventional radiation, which can have side effects such as lung cancer, proton therapy delivers high doses of radiation to the tumor without damaging the surrounding healthy tissues, making it an excellent treatment option for chordomas. In addition, it is possible to deliver the tumor-destroying radiation with more precision, thereby minimizing radiation-related side effects.
Chordoma is a slow-growing bone cancer that can affect the base of the skull or the spine. Almost 300 people in the US get this rare tumor each year. It develops from leftover cells from a bar that runs along the spine in the womb and disappears before birth.
These cells can grow and spread, and cause symptoms such as back ache or vision problems. Doctors treat chordoma by removing as much of the tumor as possible without harming the nerves, blood vessels and other tissue around it.
Chordomas are rare tumors that form from a group of cells called the notochord. This group of cells guides spinal cord formation as a baby develops in the womb. The notochord dissolves as the spinal bones grow, but in some cases pieces of it remain and can turn into cancerous (malignant) chordomas.
Most chordomas develop at the base of the skull or in the sacrum, the bottom set of vertebrae located in the lower part of the spine. They are less common than other bone sarcomas and occur in about 300 people each year in the United States. Most are found in men between the ages of 35 and 40.
In some cases, chordomas develop in areas near the brainstem or spinal cord and can cause symptoms that impact a person’s quality of life, including difficulty speaking, loss of balance or coordination, and a feeling of weakness in the legs. These symptoms typically get worse over time, and can become life-threatening.
A person with a suspected chordoma may undergo tests to confirm the diagnosis, including an MRI or CT scan. These tests are helpful for assessing the size of the tumor and where it is in the body. They also can reveal damage to other structures, such as blood vessels and nerves.
If the results of the MRI or CT scan suggest a possible chordoma, an interventional radiologist may perform a CT-guided core biopsy to obtain a tissue sample for testing. This procedure involves inserting a needle into the tumor, then injecting a solution that contains a dye. A pathologist can then determine if the tissue is chordoma.
Because chordomas are enmeshed in the delicate tissues, nerves and blood vessels of the spine, it can be difficult to remove them completely during surgery. A surgeon must take out enough of the tumor to prevent it from spreading, which is called metastasis. In 20-40% of spine chordomas and 10% of skull-base chordomas, this can occur. Chordomas that spread are often diagnosed late, when they are already large and have impacted vital brain and spinal cord structures.
Chordoma is a rare type of bone cancer that grows in the bones of the skull and spine. It usually forms in the place where the skull sits atop the spine (the skull base) or in the bottom of the spine (the sacrum).
Chordomomas grow slowly and often don’t cause symptoms until they have grown quite large. They can be difficult to diagnose because the tumors are often surrounded by important nerves, blood vessels and other structures. Symptoms vary depending on where the chordoma is located and how it affects the brain or spine.
Generally, the most common chordoma symptoms are pain in the area of the tumor. If the tumor is in the back, this may be felt as a painful lump or as pain that spreads into other parts of the body. If the chordoma is in the skull, it can cause headaches or problems with vision, if it presses on the nerves that control the muscles of the eyes and throat.
In some cases, the tumor can press on nerves that run down the spine, causing pain, weakness, and numbness in the arms or legs. In some people, the tumor can also push on the ureters and cause incontinence (inability to empty the bladder). If the chordoma is in the sacrum, it can affect the nerves that control bowel function.
X-rays and other imaging tests help doctors diagnose chordomas. They can sometimes be seen on X-rays as small round or oval areas in the bone. MRI scans can be used to look at the inside of the brain and spinal cord, and can also show the size and location of the chordoma.
Because they are slow-growing, it can take a long time for patients to get diagnosed with chordomas, especially if they see their doctor only for other health problems. This means that by the time a chordoma is discovered, it may have grown quite large and can be difficult to treat.
A patient’s doctor will ask about any symptoms they have and how they started. They will do a physical exam and order imaging tests. If they suspect that a chordoma is the cause of these symptoms, they will probably refer the patient to an orthopaedic surgeon who has experience treating these tumors.
A chordoma is a rare, slow-growing bone cancer that develops from pieces of the notochord—a collection of cells that helps guide spine formation while a baby is developing in the womb. It can occur anywhere along the spine, from the base of the skull to the tailbone (coccyx). Chordomas grow slowly, but they tend to recur and spread to other areas of the body. In up to 30 percent of patients, the tumors spread to the lungs.
A doctor will usually suspect a chordoma based on symptoms such as numbness, back pain, or headaches. They may also diagnose it with imaging tests, such as a CT scan or an MRI. These tests use X-rays and powerful magnets to make detailed pictures of the inside of your body. They can show the size and location of the tumor, as well as any damage caused by the tumor to surrounding tissues.
If your doctor thinks you have a brain or spinal chordoma, they’ll likely order a biopsy to confirm the diagnosis. A sample of tissue will be removed with a needle and sent to a lab for testing. Doctors can then compare the sample to other tissue samples to find out what type of tumor you have.
Depending on where the chordoma is located, your doctor might recommend different treatment options. For example, if the tumor is at the base of the skull, doctors might treat it with surgery and radiation. They might also prescribe medication to prevent the tumor from coming back.
Because of the risk that a chordoma might recur, your doctor will probably also recommend regular follow-up appointments. They will check your symptoms and take blood tests to see if the cancer has spread.
If the tumor is in your skull or spine, you might be able to have it removed with minimally invasive surgery. Your doctor might also suggest radiation therapy, which uses beams of radiation to destroy any remaining cancer cells. This can be done before or after surgery to lower your chances of the tumour returning. If your doctor suggests radiation, they may recommend a newer type of radiation treatment called proton beam therapy, which can deliver higher doses of radiation while protecting nearby healthy tissue.
Chordomas develop in leftover cells from a thin bar called the notochord, which helps form the early spine during fetal development. This bar disappears before birth, but in a small number of people, chordoma may form. It’s unclear what causes this to happen, but the tumor grows close to important areas like nerves and blood vessels and needs to be treated carefully.
The specific symptoms depend on where the chordoma starts in the spine. For example, if it begins at the base of the skull, you might experience headaches or double vision. If it is near the sacrum (the lower back bone) or coccyx (tailbone), you might experience pain in your legs or trouble controlling your bladder or bowels.
Like other cancers, it’s not always easy to diagnose a spinal chordoma. The aches and pains that often accompany the tumour are similar to those of many other conditions, so it can take a while for patients to see a doctor. MRI and CT scans help to identify the tumour, but a biopsy is usually necessary to confirm the diagnosis.
Once your doctors have confirmed the diagnosis, they’ll start planning your treatment. They’ll plan the best way to remove your spinal chordoma, taking into account any other parts of your body where the tumour might have spread. This is called staging and is an important part of your care.
A surgical team will use state-of-the-art techniques and instruments to access your spine. They’ll also remove as much of the tumour as possible without damaging your nerves or blood vessels. After surgery, your doctors will use radiation to kill any remaining cells.
Whether you’re treated in the private or NHS system, you’ll need to be seen by specialists who understand spinal chordomas. These experts include radiologists to suggest the initial diagnosis (through CT/CAT, MRI & PET Scanning), pathologists to make a diagnosis on a sample of tissue taken from the tumour, surgeons to operate and oncologists to treat you after surgery with radiation and drug therapies.
Chordoma is a low-grade notochordal tumor of the skull base, mobile spine and sacrum that behaves malignantly and confers poor prognosis despite surgical and chemoradiotherapeutic advances [1].
These tumors tend to encapsulate adjacent neurovascular anatomy, seed resection cavities, recur locally and demonstrate resistance to radiotherapy and chemotherapy.
Successful treatment of chordoma requires a multidisciplinary team consisting of specialists in neurosurgery, orthopedic oncology, urology and radiation oncology.
Chordomas are rare tumors of the bone. They grow close to the spine and vital structures of the central nervous system and are therefore challenging to treat. They are also located in locations that limit surgical accessibility, making clean resection difficult. Large case series have shown that a complete resection with negative microscopic margins is critical for positive outcomes.
Surgery is the treatment of choice for most chordomas. The multidisciplinary team should include a neurosurgeon, orthopedic surgeon, radiation oncologist and medical oncologist.
A complete neurological exam and a detailed history should be performed before surgery. Skull-base chordomas require additional endocrinological, ophthalmological and audiological evaluation. MRI and CT scans should be obtained to determine the extent of spinal involvement. If surgical implants or hardware limit the accuracy of MRI, myelo-CT may be used to assess the integrity of the spine. Angiography with balloon test occlusion may be useful in evaluating occluded vertebral arteries (ICA) and peridural space for surgical planning.
Depending on the location of the tumor, other treatments are often needed in addition to surgery to improve patient outcome. Cryosurgery, a procedure that uses extremely cold liquid to kill cancer cells, may be used in addition to curettage for some smaller, low-grade chordomas. Bone cement, a type of chemical that hardens when exposed to heat, can be used after curettage to fill in the hole left by the tumor and kill any remaining chordoma cells.
Radiation therapy can be used before surgery to shrink the tumor and make it easier to remove or after surgery to destroy any cancer cells that remain. Newer types of radiation, such as proton therapy, can provide higher doses with less damage to surrounding tissues.
Clinical trials are ongoing to evaluate the effectiveness of new treatment strategies for chordoma. Talk to your doctor about participating in a clinical trial. These studies look at new ways to prevent, find and treat diseases.
Chordomas are a type of bone tumor that affects the spine. They are difficult to treat because they grow very close to the spinal cord and brainstem, and they can also invade nerves in the area. Ideally, your neurologist will remove the chordoma as surgically as possible. This may include a wide or en bloc resection to ensure that all of the tumor is removed. In some cases, your doctor will recommend radiation therapy to kill any cancer cells that may remain after surgery. This can be done before surgery to shrink the tumor and make it easier to remove, or afterward to kill any remaining cancer cells. A newer type of radiation therapy, proton beam therapy, allows doctors to target specific areas of the body with high doses of radiation while minimizing damage to healthy tissue.
When it is impossible to remove a chordoma completely, your doctors will use radiation therapy to destroy any remaining cancer cells and prevent the tumor from growing back. They can also use this treatment to help relieve symptoms, such as pain or pressure on the spinal cord.
Because of the risk of recurrence, you will need to have follow-up tests after your treatment. These tests will look for signs that the cancer has spread to other parts of your body. These tests may include MRI and CT scans, blood work, and urine tests. You will also have a biopsy, which is when a sample of your tumor is taken and examined under a microscope to look for signs of cancer. The sample of your tumor may also be checked for a protein called brachyury.
In addition to standard treatments, there are clinical trials that test newer treatments for chordoma. These experimental therapies may be an option if you and your doctor feel they are safe for you. Your doctor will explain all of your options, including the pros and cons of each. You will need to weigh the risks against the benefits and decide what is right for you. If you decide to try a clinical trial, your doctor will refer you to a specialist in your condition, such as an ear, nose and throat surgeon (otolaryngologist) or an oncologist.
Chordoma is a type of bone cancer (sarcoma) that forms in bones of the skull base or spine. It most often forms where the brain sits atop the skull (skull base), or in the lower part of the spine (sacrum).
Because chordomas are slow-growing, they can be difficult to treat with conventional cytotoxic chemotherapy regimens. However, recent breakthroughs in genetics and targeted molecular therapy may offer new options for patients with recurrent chordoma. These advances, combined with improved surgical techniques aimed at maximum tumor removal and high-quality radiation treatment using protons or carbon ions at facilities experienced in treating this complex disease, have the potential to improve outcomes for patients with recurrent chordoma.
Chemotherapy can be used as a standalone therapy, or in combination with surgery and radiation. It works by killing cancer cells and stopping their growth. It can be given by mouth or through a vein (intravenous). It can also be delivered directly into the area where the chordoma is located, through a needle or a thin tube (catheter).
The chemotherapy drugs most commonly used to treat chordoma are azacitidine and dexamethasone. These medications are called mitosis inhibitors, and they work by blocking a pathway in the body that helps cancer cells grow and divide.
Patients with recurrent chordoma should be evaluated by a multidisciplinary team including at least a medical oncologist, surgeon, radiologist, pathologist and palliative care specialist with expertise in the management of musculoskeletal neoplasms. These specialists should be available to coordinate and review the patient’s status at each visit.
A recurrent chordoma should be treated with radiosurgery or targeted drug therapy in addition to surgery, if possible. Depending on the size and location of the tumor, it may be impossible to remove it completely. This is especially true of chordomas in the skull base, or when they are surrounded by important nerves and blood vessels.
If the chordoma is recurrent, tests will be done to find out whether it has spread within the spine or to other parts of the body. This process is called staging. The information gathered from these tests will help doctors decide what treatment is best for the patient.
When chordoma occurs in the skull or spine, symptoms vary depending on the location. They include pain or changes in sensation and function. For example, if it arises at the base of the skull, symptoms may include headache and neck pain, difficulty swallowing or double vision. If it occurs in the spine, it can cause back ache or numbness or weakness in the arms and legs. It also can impact bowel or bladder function. The tumors grow slowly, so it can take several years for them to cause symptoms.
The first step in diagnosing chordoma is to undergo a series of tests. These may include MRI, which uses magnetic fields to create images of the inside of the body. These images can show the presence of a tumor and help identify its location. MRI can also detect whether a tumor has spread to other parts of the body.
Another important diagnostic tool is a CT scan, which uses x-rays to produce images of the inside of the body. This type of scan can show the shape of the tumor and help doctors plan surgical removal. It can also reveal the extent of the spread of a tumor, which helps determine whether chemotherapy is an appropriate treatment option.
Once a diagnosis of chordoma has been made, the next step is surgery to remove the tumor. This is an important step because chordomas that are not removed can recur and cause more neurological problems. The goal of the operation is to remove as much of the tumor as possible. Generally, surgeons try to remove the tumor in one piece, which is known as an en bloc resection. When spinal chordomas are “broken” during surgery and not removed in a single operation, they are more likely to recur.
After surgery, patients often receive radiation therapy. This is a standard cancer treatment that uses high-energy beams to destroy the remaining cancer cells. Your doctor may also recommend other forms of radiosurgery, such as Gamma Knife.
Chordomas are very rare bone sarcomas that appear to originate from the notochord, a collection of cells that guides spine formation while a baby develops in the womb. The cancers can be sporadic or caused by familial genetic mutations in the T gene (6p27).
