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DIPG2019-07-16T15:53:01+10:00

What is DIPG

Diffuse Intrinsic Pontine Glioma (DIPG) is a highly aggressive and difficult to treat brain tumour found at the base of the brain in an area called the Pons.

DIPG primarily affects children, with most diagnoses occurring between 5 and 7 years of age or younger. DIPG makes up 10-15% of all brain tumours in children, with about 150-300 new diagnoses per year in the United States. Unfortunately a child diagnosed with DIPG today faces the same prognosis as a child diagnosed 40 years ago. Unlike many other peadiatric cancers, there has been little progress in improving treatments and cure rates for DIPG over the last few decades. There is still no effective treatment and a very poor chance of survival. Only 10% of children with DIPG survive for 2 years following their diagnosis, and less than 1% survive for 5 years. The median survival time is 9 months from diagnosis.

Diffuse means that the tumour is not well-contained – it grows out into other tissue so that cancer cells mix with healthy cells. For this reason and due to its delicate position within the brainstem, it is impossible to surgically remove DIPG tumours safely. This makes DIPG very hard to treat.

Pontine indicates that the tumour is found in a part of the brainstem called the pons. The pons is a critical brain area that controls many of the body’s most vital functions such as breathing, sleeping, bladder control, and balance. Because these functions are vital to survival, the pressure from the growing tumuor is very dangerous.

Glioma is a general term for tumours originating from glial cells. Glial cells are found throughout the brain. They make up the white matter of the brain that surrounds and supports the neurons (neurons are cells that carry messages in the brain). Gliomas can form in different areas of the brain. DIPG occurs in glial cells in the pons.

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Symptoms of DIPG

The brainstem (pons) is responsible for controlling a number of critical life functions, including blood pressure, breathing, and a number of other vital functions. DIPG can cause significant neurological problems as pontine gliomas often affect the cranial nerves, so many of DIPG’s early symptoms appear in the facial muscles, often affecting the eye and eyelid movements. Unfortunately, the tumour grows so fast that symptoms often appear suddenly and get worse quickly.

The most common symptoms of DIPG are:

– Problems with eye movement. The most common one is difficulty looking to the side. Double vision is common because of the loss of alignment of the eyes. Other problems of eye movement include drooping eyelids and inability to close the eyes completely. Often the problems are seen in both eyes.

– Facial weakness, drooping on one side of the face.

– Sudden appearance of hearing problems, including deafness.

– Trouble chewingor swallowing, gagging while eating.

– Limb weakness, difficulty standing or walking, abnormal gaits, unbalanced limb movements.

– Headache.

– Nausea and vomiting from brain oedema (swelling) or hydrocephalus. Hydrocephalus is a condition in which the flow of cerebrospinal fluid around the brain is blocked, causing an increase in pressure inside the skull. About 10 percent of DIPG patients have hydrocephalus at the time of diagnosis.

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Diagnosing DIPG

Unlike tumours at other locations, a tumour in the brainstem is technically difficult to biopsy with the potential for devastating complications. Until recently they were not routinely biopsied for this reason. With newer technologies however, this practice is now becoming more standard and allows for a more informative diagnosis. The process involves drilling a small hole in the child’s skull and then passing a needle to collect a tissue sample. This tissue can then be sent for further testing. DIPG may also be diagnosed based on the patient’s symptoms and magnetic resonance imaging (MRI) studies. MRI scans produce detailed images of the brain and allow doctors to detect the presence of a tumour. Sometimes the patient is also given a contrast agent (a special dye) before the scan to help doctors better tell whether the abnormal tissue is a tumour and what type of tumour it is. The location in the pons, the way it infiltrates nearby tissue, and the lack of clear borders of the tumour all signify that a tumour is likely DIPG.

Treatment for DIPG

Treatments for DIPG are sorely limited, underpinned by a lack of understanding of the disease. These options include:

– Radiation therapy is the primary treatment for DIPG. Radiation therapy uses high-energy X-rays directed at the tumour to help shrink it and reduce pressure on the brain. This is typically performed as five daily sessions per week for a total of 30 to 33 sessions. Depending on the age of the child, they may require a general anaesthetic to undergo each of their radiation therapy sessions. This treatment can alleviate symptoms quickly and hold the cancer at bay for about six months on average. When the tumour comes back (progression), repeat radiation (re-irradiation) therapy may be an option but is generally less effective

– Chemotherapy has not been proven effective, at least in part because anti-cancer drugs cannot cross the blood-brain barrier and reach the tumour in sufficient amounts.

Surgical resection, although it is the treatment of choice for many other types of brain tumours, it is not considered a treatment option for children with DIPG. The tumour infiltrates so extensively into normal brain tissue, and the brainstem carries out such critical functions (like controlling breathing), that surgical removal of the tumour is impossible.

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We are a charitable organisation dedicated to improving treatment outcomes for children diagnosed with diffuse intrinsic pontine glioma (DIPG). DIPG is the deadliest form of childhood cancer with an average survival rate of just nine months following diagnosis.

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