Brainstem glioma: symptoms, diagnosis and treatment

A glioma is a tumour that develops from glial cells, the supporting cells of the nervous system. The NHS describes gliomas as a type of cancerous brain tumour that can be low grade (grade 2) or high grade (3 or 4), and explains that brain tumours are graded according to how fast they grow and how likely they are to grow back after treatment.

The Brain Tumour Charity explains that brainstem gliomas develop from glial cells within the brain stem, the lowest part of the brain at the back, which joins the brain to the spinal cord. It controls vital functions that we do not need to think about, including heartbeat and breathing. That is why this location makes treatment particularly difficult.

These tumours affect both children and adults but are more common in children. They are uncommon overall, and most of what families read relates to childhood tumours, so it is worth asking your specialist team which type applies in your case.

The Brain Tumour Charity says that childhood brainstem gliomas are often classified as either focal brainstem glioma or diffuse midline glioma. Focal tumours are low grade gliomas that form in an area of the brain stem outside the pons. Diffuse midline gliomas are diffuse, high grade tumours in the part of the brain stem called the pons, which are faster growing and tend to spread into the surrounding brain tissue.

The type is established by imaging and sometimes a biopsy, and it largely determines both the treatment options and the outlook. The two groups behave very differently, which is why the term brainstem glioma alone does not say much about what to expect.

The Brain Tumour Charity says the symptoms depend on the tumour location and which areas of the brain or nervous system are affected, so symptoms differ from one child to another. Common ones include the following.

• Headaches, which may be worse in the morning or after lying down because of a build-up of cerebrospinal fluid (hydrocephalus).

• Movement and coordination problems, such as difficulty walking, using the hands or unusual clumsiness.

• Facial nerve problems, such as drooping eyelids, a crooked smile or difficulty moving the eyes.

• Trouble chewing or swallowing.

• Slurred speech or difficulty speaking clearly.

• Nausea or vomiting, which may be more frequent in the morning or after physical activity.

The NHS advises seeing a GP if you have symptoms such as headaches, seizures, persistent sickness, drowsiness, mental or behavioural changes, progressive weakness on one side of the body or vision or speech problems, particularly if a headache feels different from your usual headaches or is getting worse. It adds that you may not have a brain tumour, but these symptoms should be checked.

If the GP cannot identify a more likely cause, the NHS says they may refer you to a neurologist for further assessment and tests such as a brain scan. For children, parents should say clearly if symptoms are getting worse or new problems with balance, swallowing or the eyes appear. Seizures, sudden severe symptoms or a rapidly worsening child need urgent assessment through NHS 111, emergency care or 999.

The Brain Tumour Charity says most brain tumours have no known cause, so there is no way to prevent them, but some brainstem gliomas can be caused by the genetic condition neurofibromatosis type 1 (NF1). People with NF1 usually have doctors who specialise in that condition, who work with the brain tumour team.

The NHS lists age, radiation exposure and family history or genetic conditions among the risk factors for brain tumours generally, naming tuberous sclerosis, neurofibromatosis types 1 and 2 and Turner syndrome. It describes radiation as accounting for a very small number of brain tumours.

The Brain Tumour Charity says surgeons can occasionally operate on focal tumours to remove some of the tumour, but usually surgery is not an option for brainstem gliomas because operating in such a delicate location could do more harm than good. Healthcare professionals therefore usually treat brainstem gliomas with chemotherapy and radiotherapy.

More generally, the NHS says brain tumour treatment depends on the type of tumour, where it is, how big it is and how far it has spread, how abnormal the cells are and overall health. Steroids can be prescribed to reduce swelling around a tumour, and anti-epileptic medicines and painkillers can help with seizures and headaches.

Treatment is planned by a multidisciplinary team that includes neurosurgeons, oncologists and specialist nurses, and for children it takes place in a children's cancer centre. Families can ask what the aim of treatment is, what side effects to expect and whether clinical trials are an option.

The Brain Tumour Charity says brainstem gliomas cover a wide range of tumours, so it is difficult to give an overall prognosis. It depends on the location and grade of the tumour as well as factors like genetics and overall health.

The charity says that for low-grade focal brainstem gliomas the prognosis is often hopeful, with many patients surviving for years, especially if surgery removes as much of the tumour as possible. For diffuse midline glioma (DIPG) it describes the prognosis as sadly not promising, with average life expectancy less than a year. These are averages and the care team is the right place to discuss what applies to an individual child or adult.

The NHS also notes that survival figures are difficult to predict because brain tumours are uncommon and there are many types. Hearing this can be very hard, and families are encouraged to use charity support services and to ask the team about palliative and supportive care alongside treatment.

On the NHS, a suspected brain tumour is investigated by imaging and referred to a specialist neurosurgical or paediatric neuro-oncology centre, where a multidisciplinary team agrees the plan. Treatment, rehabilitation and follow-up are provided free at the point of use.

Some families also seek a second opinion from a private specialist or an international centre. The underlying scans and the evidence base are the same, and the main differences are speed and choice of clinician. Ask any clinician which team will take overall responsibility and who to call if symptoms change.