How is chordoma treated?
Paediatric Chordoma Guide
National guidelines in the UK recommend that anyone with a bone cancer such as chordoma should be referred to a specialist centre for further assessment and treatment. Your child is likely to be referred to one of these centres – this may mean you and your child will have to travel some distance for treatment.
The centre at which your child will be treated will depend on the location of their chordoma.
The base of the skull and the bones of the neck (known as the cervical spine) are the most common locations of chordoma in children. Due to the proximity of these areas to the brain and spinal cord, these chordomas are generally treated in specialist neurosurgical units.
If a chordoma presents in the bones at the bottom of the spine (sacrum and coccyx regions, or tail bone) then the patient is generally treated in one of five specialist bone cancer centres in England, which are located in Birmingham, London, Newcastle, Oswestry and Oxford.
Patients in Wales and Northern Ireland can be treated in any of the UK specialist centres. Patients in Scotland will be treated in Glasgow, Edinburgh, Aberdeen, Dundee and Inverness.
If chordoma presents in the mobile spine – the middle section of the vertebral column – then it may be treated in a spinal or neurosurgical unit.
Words in bold are explained in a glossary.
Paediatric Chordoma Guide
Care provided by the MDT
Chordoma affects very important and complex parts of the body such as the brainstem, spinal cord and important nerves and arteries. Treatment is therefore complicated and requires very specialised care provided by multiple types of doctors. Your child’s care will be managed by a team of experts from a wide range of healthcare professions known as a multidisciplinary team (MDT).
The MDT may include:
- a paediatric oncologist (cancer specialist)
- a clinical oncologist (radiotherapy and chemotherapy specialist)
- a paediatric surgeon
- a pathologist
- a radiologist
- a nurse specialist
- a physiotherapist
- a pharmacist
- a dietitian
Treatment options
The treatment of chordoma usually involves a combination of surgery and radiotherapy, dependent on the age of your child. More rarely, chemotherapy may also be involved.
Due to the rarity of chordoma, treatment options are usually tailored to the child’s situation – no standardised treatment protocol exists. Chordoma in children can grow quickly, although this is not always the case. In order to try and prevent or prolong the time to relapse/recurrence, treatment of chordoma must be aggressive.
Your child’s doctor and members of the MDT will discuss the options with you in depth. Lean into their knowledge, expertise and support – they are there for you.
It might be recommended by the MDT that your child has surgery to remove as much of the chordoma as possible. Where surgery might not be possible because of the location of the chordoma, your specialists will recommend other treatment options.
A course of radiotherapy may be recommended by your MDT after surgery. The aim of radiotherapy is to kill off any cancer cells that have been left behind after surgery with the aim of lowering the risk of the chordoma coming back. Treatment of chordomas typically requires a high dose of radiation, but this may be lower if the tumour has been removed.
The decision to give radiotherapy will be based on a number of factors:
- your child’s age
- location of their chordoma
- type and size of their chordoma
- success of the surgery
Radiotherapy can cause side effects so your specialists will advise whether it’s the right choice for your child. If radiotherapy is used to treat tumours in the skull, side effects may include localised hair loss, headaches, blurry vision, mouth and throat irritation, changes to smell, taste and hearing, and difficulty swallowing. For tumours in the spine, radiotherapy may cause changes in bowel and bladder function. Doctors are looking at new ways of giving radiotherapy to reduce these side effects, while still treating the chordoma.
One of the newer ways of giving radiotherapy uses a different type of beam called a proton beam. Compared with conventional radiotherapy, proton beams deliver a higher dose of radiation straight to the cancer cells and less to the surrounding healthy cells. As proton beam therapy is more targeted than conventional radiotherapy, it can be used to treat areas close to critical parts of the body such as the spinal cord, with less damage to the nearby healthy tissue.
Proton beam therapy can now be accessed in the UK through the NHS. Proton beam therapy is currently available on the NHS at the Christie Hospital in Manchester. An additional NHS centre offering proton beam therapy is expected to open later in 2021 at University College Hospital London. The NHS will also cover the cost of proton beam therapy at approved centres in the USA and Europe, if this is the best option for your child.
The Bone Cancer Research Trust offers financial support to UK patients undergoing proton beam therapy, to cover travel costs not subsidised by the NHS. For further information, please see the Trust’s page on Financial Assistance Grants.
If your child has poorly differentiated or dedifferentiated chordoma, chemotherapy may be recommended along with, or in place of, surgery and radiotherapy.
Chordoma is generally a slow-growing cancer which means it usually does not respond to chemotherapy (which attacks rapidly dividing cells). However, there is some published evidence that chemotherapy may be effective in treating younger children with poorly differentiated and dedifferentiated chordoma using drugs used in the treatment of sarcoma, another type of bone cancer. These include the drugs doxorubicin, ifosfamide, etoposide, vincristine, and cyclophosphamide, which work in different ways to kill cancer cells or stop them replicating.
Chemotherapy will often be given intravenously (via the veins) but can also be given orally, depending on the type of drug. If given intravenously, your child may have a central line inserted to make it easier to access the blood system. A central line is a narrow tube inserted into a major blood vessel in the chest, which makes it easier to deliver chemotherapy. Central lines will be removed once treatment is finished.
Targeted therapies are drugs that specifically target cancer cells. By acting to block the growth and spread of cancer cells specifically, targeted therapies are more precise than conventional chemotherapy and cause less damage to healthy cells. Targeted therapies can work by:
- stopping the cancer cells from growing
- helping the immune system find and destroy cancer cells
- halting the development of the tumour’s blood supply
- causing cancer cell death
Research is ongoing to find targeted therapies that work for chordomas. So far, research has identified a number of genes specific to chordomas, that could potentially form the basis for targeted therapies. While targeted therapies are still in the early stages of development, the results so far are promising.
Some targeted drugs that are being investigated for chordoma include imatinib, erlotinib, sunitinib, afatinib. These drugs are all currently used to treat other types of cancers and studies are now looking at how well they work in chordoma. More information about targeted therapies for chordoma is available on the Bone Cancer Research Trust website.
As paediatric chordoma is rare, there usually aren’t many clinical trials of new drugs open to children. Your doctor will let you know if there is a clinical trial that may be suitable for your child. Please find details of trials available in the UK and USA below.
Trials in the UK
At the minute, there are no clinical trials open to children with paediatric chordoma in the UK.
Trials in the USA
As of July 2021, there are currently six clinical trials in the USA open to enrolment for children with chordoma. Four of these are investigating different treatments for chordoma. You can read more about the trials by clicking on the link below:
- Children and Adults With Chordoma
- Nivolumab With or Without Stereotactic Radiosurgery in Treating Patients With Recurrent, Advanced, or Metastatic Chordoma
- Genetic Clues to Chordoma Etiology: A Protocol to Identify Sporadic Chordoma Patients for Studies of Cancer-Susceptibility Genes
- Nivolumab and Relatlimab in Treating Participants With Advanced Chordoma
- Study of Nivolumab and Ipilimumab in Children and Young Adults With INI1-Negative Cancers
- Nivolumab (Opdivo®) Plus ABI-009 (Nab-rapamycin) for Advanced Sarcoma and Certain Cancers
We will keep this section updated with news of any clinical trials in chordoma open to children. You can also search for any new clinical trials in the UK and USA on the following pages.
In certain circumstances, your child may be eligible for treatments under a compassionate use programme, which allows the use of a treatment that has not been fully authorised when there is a clear medical need. A treatment can be offered under a compassionate use programme to people with serious conditions who don’t have suitable authorised treatments options and are unable to enter clinical trials.
The request for compassionate use of a treatment is usually made to the pharmaceutical company developing the drug/treatment in question and is made by the patient’s healthcare team.
