Children & families · Conditions

Brainstem tumours, including DIPG and DMG

Tumours of the brainstem range from focal tumours, which often have a good outlook, to diffuse midline glioma (DIPG/DMG), which needs specialist, compassionate care.

What is it?

The brainstem connects the brain to the spinal cord and controls vital functions such as breathing, swallowing, eye movements and the nerves to the face. Brainstem tumours fall into two broad groups, which behave very differently:
• Focal brainstem tumours are well-defined tumours, often low-grade (slow-growing) gliomas, including tectal gliomas. Many grow very slowly and have a good long-term outlook.
• Diffuse intrinsic pontine glioma (DIPG) is a tumour that spreads through the pons, the middle part of the brainstem. Most DIPGs carry a specific genetic change and are now classified as diffuse midline glioma (DMG), H3 K27-altered, a high-grade tumour that can also occur in other midline structures such as the thalamus and spinal cord.

What symptoms can it cause?

Symptoms often include double vision or a squint, facial weakness, difficulty swallowing or speaking, unsteadiness and weakness of the arms or legs. DIPG/DMG symptoms typically develop over weeks. Focal tumours may cause symptoms more slowly, and tectal gliomas often cause hydrocephalus, with headaches and vomiting.

How is it assessed?

An MRI scan shows the tumour and often strongly suggests the type. Your child's case is discussed at the multidisciplinary tumour board.

The role of biopsy: DIPG can often be diagnosed from its typical MRI appearance. A biopsy, in which a small sample is taken through a small opening using stereotactic guidance, may be recommended when the appearance is not typical, and increasingly to confirm the diagnosis and identify molecular features that may open access to clinical trials or targeted treatments. In experienced hands brainstem biopsy is generally safe, but it carries a small risk, which is discussed with you. Dr Labuschagne has published on the use of intraoperative stimulation to help guide safe target sites for brainstem biopsy.

What are the treatment options?

Focal tumours may be observed with regular scans, removed surgically when this can be done safely (sometimes with the help of 5-ALA fluorescence and neuromonitoring), or treated with chemotherapy or radiotherapy. Hydrocephalus is often treated with ETV.

DIPG/DMG cannot be removed surgically because the tumour is spread through vital tissue. Radiotherapy is the standard treatment and usually improves symptoms for a period of time. Sadly, there is currently no cure for DIPG/DMG, and most children live for months to a year or two after diagnosis. Research is very active, and clinical trials of new treatments are available in some centres; the team will discuss whether a trial may be suitable and possible for your child. Palliative care teams support children and families from early on, alongside other treatment, to help with comfort, quality of life and the decisions ahead.

When might surgery be considered?

For focal tumours, surgery may be considered to remove the tumour, to obtain a diagnosis or to treat hydrocephalus. For DIPG/DMG, surgery is usually limited to biopsy and, when needed, treatment of hydrocephalus. Every decision is made with you, with time to ask questions.

What to expect from recovery and follow-up

Follow-up depends on the tumour type. Children with focal tumours have regular MRI scans and often do well over many years. Children with DIPG/DMG are cared for by the oncology and palliative care teams, with the neurosurgery team involved as needed, and families are supported throughout.

When to seek urgent help

If your child is very drowsy, difficult to wake, has a seizure, repeated vomiting or a severe headache, go to the nearest emergency unit or call 10177. If your child is under our care, also call the hospital switchboard and ask for Dr Labuschagne (Netcare Unitas Hospital 012 677 8000; Nelson Mandela Children's Hospital 010 133 0600). New difficulty swallowing or breathing needs urgent review.

Dr Labuschagne's publications on this topic

Consent forms to read in advance

If surgery is recommended, you are welcome to read the relevant form before your consultation. Dr Labuschagne will go through it and sign it with you at the consultation.

All consent forms

This page is general information for parents and caregivers. Your child's doctor will advise on what applies to your child.

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