CHORDOMAs are rare tumors that develop in the bones of the base of the skull or spine. It is a complex lesion that requires specialized care by a multidisciplinary team experienced with the management of chordoma.
Our team has some of the world’s largest experience in managing chordomas.
What is the origin of chordomas ?
Chordomas develop from remnant of the notochord. The notochord is an embryonic structure that gives the axis of development of the fetus and participates in the development of the spine.
The notochord disappears when the fetus is about 8 weeks old, but some cells of the notochord do not disappear and persist in the bones of the spine and in the base of the skull. The reason why some of these cells transform into chordoma is unknown but research is progressing.
The vast majority of chordomas occur at random and are not hereditary. There are certain genetic modifications in chordoma cells as is the case in all tumors. For example, more than 95% of individuals with chordoma have an abnormality in the DNA sequence of a gene called brachyury. This anomaly increases the risk of developing a chordoma but does not cause chordoma by itself.
Who is affected?
There are around 80 new cases in France each year. At one point, less than one in 100,000 people live with a chordoma.
Chordoma is most often diagnosed between 50 and 60 years, but it can occur at any age.
There is a slight male predominance with a ratio of about 2 men for 1 woman.
Familial formsare very rare and only a few families with chordoma have been identified.
Where are they located ?
About 35% of chordomas are located in the skull base, 20% are located in the bones of the mobile spine and about 45% are located in the sacrum.
They are usually located in the center of the base of the skull at the level of a bone called the clivus. They are therefore deep lesions, close to critical structures and are difficult to access and complex to treat. Cranial chordomas are sometimes called brain tumors because they can grow inside the cranial cavity towards the brain but they do not actually originate from brain cells.
They can also be located at the cervical spine and at the lower back in the lumbar spine. This is called the movable column. Chordoma at the level of the thoracic spine are much rarer.
Finally, they also occur in 45% of the cases in the sacrum.
One of their characteristic is their strong tendency to reccur locally. This is called local reccurence. In about 30% of patients, the tumor spreads to other parts of the body. If chordomas spread to other parts of the body (metastases), the most common places they spread to are lungs, liver, subcutaneous tissue, or lymph nodes. Metastases, when they occur, usually appear late in evolution, when the primary tumor recurs. They are rarely discovered at the time of initial diagnosis and are often fairly well tolerated.
What are the symptoms ?
Chordomas develop most often slowly and symptoms occur usually late in their evolution.
The symptoms are variable depending on the location of the chordoma. At the base of the skull, the most common symptoms are headaches and double vision. Other symptoms may occur such as visual loss, hearing loss, swallowing difficulties, hoarse voice, balance issues, motor weakness, etc.
Chordomas of the spine usually cause pain, tingling or weakness in a limb.
The chordomas of the sacrum are often discovered late and they cause pain and urinary disorders.
How does one diagnose it?
CT and MRI scan are the most important imaging studies to diagnose a chordoma. The specific appearance of MRI chordomas makes it most often possible to strongly suspect this diagnosis.
Special MRI sequences are needed to clarify the diagnosis on MRI. Being a rare tumor, the radiological diagnosis requires experienced radiologists.
Are there different types of chordoma?
Once removed and analyzed, different types of chordomas can be distinguished:
- The classic chordomes
- Low-grade chordomas more common in children and young adults
- Undifferentiated chordomas that are more aggressive.
What is the treatement ?
- Multidisciplinary approach
A multidisciplinary approach is crucial in the management of these lesions.
We collaborate for the management of these tumors with several teams including:
– ENT department of Lariboisière Hospital
– Neuroradiology Department of Lariboisière Hospital
– Orsay proton therapy center,
– The Curie Institute: Dr. Hamid Mammar, Dr. Valentin Calugaru
– Radiation therapy of La pitié Salpetrière Hospital: Dr. Loic Feuvret
– Gustave Roussy Institute: Dr. Stéphanie Bolle
A multidisciplinary meeting bringing together all the actors involved in the care of patients with chordoma, takes place every month at the neurosurgery department to discuss about the patients to be treated or treated.Tumor Board - Skull Base and Chordomas
- Is a biopsy necessary?
In the skull base and cervical spine, a biopsy is rarely necessary.
In the lumbar spine and sacrum, a biopsy can be performed to confirm the diagnosis. However, it is not necessary in all cases and must be performed under special conditions to prevent the spread of the tumor along the path of the biopsy.
- Surgical treatment
The surgery should be performed in centers experienced in the field of neurosurgery of the skull base and / or spinal surgery.
Skull base chordomas are complex lesions that require mastering a number of specific skull base approaches. Incomplete treatment in a center that does not have sufficient experience in this surgery may complicate a new surgery and compromise the possibilities of complete resection. Our team has an internationally recognized experience in the care of chordoma with a number of treated patients among the largest in the world.
The goal of surgery is to remove the entire tumor while preserving nerve structures in the immediate vicinity. This is not always possible, especially at in the skull base, because of the presence of critical structures around the tumor or running through the tumor. In this case, the goal is to reduce the size of the lesion as much as possible and to decompress critical structures in order to prepare the radiotherapy. Surgery in this region carries significant risk that requires a medical team (neurosurgeon, radiologist, ophthalmologist, anesthetists, ICY specialists) and paramedical specialized in the care of these patients.
Chordomas are tumors that have a strong tendency to recur locally. Surgical treatment must therefore be supplemented by high-dose radiotherapy. The most commonly used technique is proton therapy. It is carried out at the Proton Therapy Center of Orsay by specialized radiation :
– Dr. Hamid Mammar
– Dr. Valentin Calugaru
– Dr. Loic Feuvret
– Dr. Stéphanie Bolle
In case of recurrence of the tumor, a second surgery is most often possible and proposed.
After this surgery, in case of a new recurrence or when surgery is not possible, chemotherapy can be offered. Therapeutic clinical trials are underway to evaluate the efficacy of new treatments and may be offered.
The prognosis of each patient is unique and depends on many factors: age, type of chordoma, size at the time of diagnosis, localization, type of treatment possible, complete or not removal of the tumor ….