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What is DIPG?

DIPG, or Diffuse Intrinsic Pontine Glioma, is a form of brain tumor that originates in the brain stem, located just above the back of the neck and connected to the spine. The brain stem regulates crucial functions such as breathing, heart rate, and the nerves and muscles responsible for our abilities to see, hear, walk, talk, and eat. These tumors are classified as gliomas because they develop from supportive glial cells within the brain.

DIPG or brainstem gliomas account for approximately 10% to 20% of all childhood brain tumors. They tend to be more prevalent in children aged 5 to 10 years, although they can occur at any stage during childhood. While relatively rare, these tumors can also manifest in adults.

Diagnosing DIPG can be quite challenging, but there are measures you can take to enhance the quality of care for your child.

What are the causes of DIPG?

Although DIPG tumors may exhibit specific genetic alterations, referred to as mutations by medical professionals, it's important to understand that this condition is not inherited from parents. Additionally, there is no known connection between DIPG and environmental factors such as smoke, chemicals, or radiation exposure. Consequently, there are no preventive measures to reduce the risk of DIPG in children.

Medical experts speculate that DIPG may be associated with the rapid growth of a child's brain. Tumors tend to develop during periods when the brain undergoes significant changes. During these phases, there is an increased presence of a particular type of brain cell that may contribute to the growth of DIPG tumors.

What are the symptoms of DIPG?

In most cases, symptoms tend to manifest swiftly due to the rapid expansion of these tumors.

The primary symptoms associated with DIPG typically encompass the following:

  • Difficulties with balance and walking.

  • Visual impairments, such as double vision, eyelid drooping, involuntary eye movements, and blurred vision.

  • Challenges with chewing and swallowing.

  • Experiencing nausea and episodes of vomiting.

  • Occurrence of morning headaches or headaches that alleviate after the child vomits.

  • Facial weakness or drooping, typically affecting one side of the face.

How is DIPG diagnosed?

To ascertain a DIPG diagnosis, an initial step involves a thorough examination by a pediatrician. During this evaluation, the medical practitioner will review your child's medical history and assess their symptoms.

In the diagnostic process, various tests may be employed, including:

  • Imaging: Brain scans, such as CT scans, play a pivotal role in confirming the diagnosis of DIPG. Additionally, a magnetic resonance spectroscopy (MRS) test may be performed to analyze cellular chemicals. This may be combined with an MRI scan to identify indications that the tumor originates from glial cells. The use of a contrast dye before the MRI can enhance the tumor's visibility. Key factors, including the tumor's location in the pons, its spread into surrounding tissues, and indistinct borders, all contribute to the likelihood of a DIPG diagnosis.

  • Biopsy: In cases where children exhibit atypical symptoms or abnormal MRI findings, a biopsy may be required to validate a DIPG diagnosis. For DIPG, a pediatric neurosurgeon typically conducts a stereotactic biopsy. This procedure involves drilling a small hole in the child's skull, inserting a needle, and extracting a sample of the tumor tissue. Subsequently, a pathologist examines the tissue specimen for indications of DIPG.

What are the stages of DIPG?

DIPG is categorized within the Glioma staging system, which enables classification based on cell characteristics observed under a microscope. The four stages detailed below are graded from the mildest to the most severe.

  • Low Grade: Grade I or II corresponds to tumors with cells closely resembling normal cells.

  • High Grade: Grade III or IV represents the most aggressive and malignant tumors.

The primary challenge in dealing with DIPG is that a significant portion of these tumors cannot be categorized by grade due to the infeasibility of surgical procedures for biopsies or tumor removal. The tumors' location makes such surgeries unsafe. Consequently, when biopsies are attempted, they typically yield Grade III or Grade IV tumors, which are known for their highly aggressive behavior. In the majority of cases, DIPG diagnoses rely on the appearance of these tumors on MRI scans.

Are there treatment options for DIPG?

Treatment options for DIPG encompass surgical, radiological, and chemotherapeutic approaches.

Radiation therapy serves as the primary and central treatment for DIPG. In this procedure, high-energy X-rays are directed at the tumor over a series of sessions. Children with DIPG may necessitate up to 30 or more radiation sessions, typically administered five times a week.

Radiation therapy plays a crucial role in reducing the size of the tumor in most DIPG cases and may provide relief from associated symptoms. By diminishing the tumor's size, it alleviates pressure on the child's brain and may extend their life by several months. However, it's essential to note that the benefits of radiation therapy are typically temporary, as tumors frequently resume growth within a few months. In such cases, additional rounds of radiation therapy may be considered to prolong a child's life.

Chemotherapy complements radiation therapy and may involve the use of newer, experimental drugs and biologics. It's important to note that there are currently no established medications with a proven track record in treating DIPG. In certain novel treatment approaches, targeted cancer drugs are directly injected into the tumor to enhance treatment efficacy.

Surgical intervention, such as tumor removal, is typically not a viable option for DIPG. These tumors lack a well-defined, solid mass structure that would facilitate surgical extraction, and such procedures pose a significant risk of harming healthy brain tissue, including the potential for fatal outcomes.

What is the survival rate of DIPG?

Regrettably, the prognosis for DIPG is highly unfavorable, with a very limited survival rate. A mere 10% of children diagnosed with DIPG manage to live for two years, and fewer than 1% endure for five years.

It's crucial to understand that there is currently no cure for this form of tumor. Medical professionals focus on ensuring your child's comfort and alleviating symptoms, as available medications are unable to treat the disease itself but can provide relief from its associated symptoms.

How can I help?

Your donations to Mary's Magical Moment go to help fund a magical weekend for a child afflicted with DIPG.  Mary’s Magical Moment exists to bring joy into these children’s lives by giving the gift of Disney.  

In addition to giving the gift of DisneyWorld trips, they also aid and assist the children and families with travel to treatments, medical expenses, household expenses and care packages. They also assist in raising awareness to this condition by providing specific information when requested.

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