DIPG (diffuse intrinsic pontine glioma) in children — coordinated specialist care for your family

DIPG stands for diffuse intrinsic pontine glioma. It is a tumour that grows within the pons — the middle section of the brainstem — and is called 'diffuse' because its cells are spread throughout the normal brainstem tissue rather than forming a single compact mass. This diffuse growth pattern is the key reason DIPG cannot be surgically removed. DIPG is most commonly diagnosed in school-age children between five and ten years old. The tumour carries a specific genetic change called the H3 K27M mutation in most cases, which was formally recognised when the World Health Organisation reclassified it as 'diffuse midline glioma, H3 K27M-altered' in 2021. International research into this mutation is active, and early clinical trials are exploring new treatment directions.

The symptoms of DIPG usually appear and progress over a few weeks to months rather than suddenly. The most common early signs include a new squint or double vision — caused by pressure on the sixth cranial nerve, which controls eye movement — one-sided facial weakness, and difficulty with walking, balance, or coordination. Some children develop weakness in an arm or leg on one side of the body. Swallowing problems or a change in the voice can appear as the tumour affects the lower brainstem. Morning headaches that ease later in the day, sometimes with vomiting, may occur if pressure builds inside the skull. Because many of these signs can resemble other, far more common conditions, parents often wait some weeks before a medical review. If your child develops a new squint alongside any other neurological change, please see a doctor promptly — a brain MRI is usually the first investigation.

DIPG is diagnosed primarily by MRI of the brain and brainstem, which shows the characteristic appearance of a diffuse tumour expanding within the pons. The MRI images — together with the child's age, symptom history, and clinical examination findings — are enough to make a diagnosis of DIPG in the majority of cases; a surgical biopsy is not always required and carries significant risk given the brainstem location. When a biopsy is considered — usually to access tissue for a clinical trial — it is done by an experienced neurosurgical team using stereotactic guidance. Blood and cerebrospinal fluid are not routinely diagnostic for DIPG, though liquid biopsy techniques are an active area of research. The diagnosis is always confirmed by a multidisciplinary team that includes paediatric oncology, neurosurgery, radiation oncology, and neuroradiology.

The current standard of care for DIPG is focal radiation therapy delivered to the brainstem over approximately six weeks. Radiation does not cure DIPG, but it reliably improves neurological symptoms and provides a period of stability in most children. Conventional chemotherapy has not been shown to add benefit over radiation alone based on decades of clinical trials. Corticosteroids are often used alongside radiation to reduce inflammation and swelling in the brainstem, which helps symptoms in the short term. For eligible families, participation in a clinical trial — which may test targeted agents, immunotherapy approaches, or convection-enhanced delivery of drugs directly into the brainstem — is actively encouraged by international paediatric oncology guidelines. Every DIPG case at CION is reviewed by our full multidisciplinary tumor board so that no option goes unexamined.

DIPG carries a very difficult prognosis, and we believe every parent deserves honest, compassionate information rather than vague reassurances. The disease is not curable with current standard treatments, and most children experience tumour progression within months of completing radiation. Prognosis for an individual child depends on several factors including age, the extent of the tumour, the speed of symptom onset, and the degree of response to radiation. A minority of children with DIPG do have a longer course, and international research is producing new insights every year. Our team will walk through what the current evidence means specifically for your child, in clear language, with as much time as you need. We also connect families with supportive care — nutritional support, psychological care for the whole family, pain and symptom management, and, where relevant, palliative care planning — from the point of diagnosis.

Clinical trials are worth discussing for almost every family dealing with DIPG, because standard treatment has not changed the prognosis significantly over several decades and trials represent the front edge of what science is testing. Current international trials are exploring approaches including ONC201 (targeting the H3 K27M mutation), immunotherapy, CAR-T cell therapy, convection-enhanced drug delivery, and combinations of targeted agents with radiation. Eligibility depends on your child's specific tumour characteristics, age, and overall condition. Our team will explain which trials may be open and relevant, help you understand what participation involves, and support you in seeking a second opinion or referral if a trial at another centre seems appropriate. You deserve full information — not a rushed decision.