Did you know that Pontine Glioma is an uncommon and extremely dangerous type of brain tumour that primarily affects children? Continue reading for more information.

Pontine glioma, or Diffuse intrinsic Pontine glioma (DIPG), begins in the pons, a part of the brainstem that controls vital processes such as breathing, swallowing, and balance. The pons is located near the base of the brain and links the cerebral hemispheres and spinal cord.

DIPGs are high-grade gliomas, meaning they are malignant and quickly spread. They are classified as “diffuse” because they spread throughout the brainstem and enter different areas, making surgery to remove them nearly impossible. The location and diffuse nature of diffuse pontine glioma make therapy extremely difficult.

There is no established risk factor for pontine glioma; the actual aetiology is still unknown. It is not linked to any easily avoidable environmental or lifestyle variables. Clinical signs of pontine gliomas, such as problems with balance, walking, and cranial nerve dysfunction, are frequently used to diagnose. 

Table Of Contents

1. What Are The Symptoms Of Pontine Glioma?
2. Treatment Of Pontine Glioma
3. DIPG In Adults 
4. Prognosis Of Pontine Glioma
5. Expert Advice 
6. FAQs
7. Reference Links 

What Are The Symptoms Of Pontine Glioma 

Children are typically affected by DIPG disease, a particular type of brain tumour. The tumour’s location in the brainstem, specifically in the pons, which regulates several crucial bodily systems, is frequently linked to the symptoms of DIPG. The following are some typical DIPG brain tumour symptoms:

1. Issues With Balance And Coordination 

Children with DIPG may stumble, have trouble walking and have coordination issues. They might also struggle to complete tasks that call on fine motor abilities.

2. Limb Weakness

DIPG can lead to muscle weakness, frequently affecting the arms and legs. Children may have trouble gripping objects, feel tired from exerting themselves, or struggle with tasks that call for strength.

3. Dysfunction Of The Cranial Nerves 

The cranial nerves are responsible for controlling swallowing, eye movements, and facial emotions. When these nerves are disrupted, symptoms include double vision, facial drooping, trouble swallowing or speaking, and issues with eye movement might result.

4. Speaking Modifications

Children with DIPG could encounter changes to their speaking patterns. They might speak with a slur, have trouble pronouncing words, or change the sound of their voice.

5. Breathing Difficulties 

Children with DIPG may have trouble breathing because the tumour affects the brainstem, which controls respiration. Breathlessness, fast breathing, or changes in respiratory rhythms can all be symptoms of this.

6. Headaches

Many brain tumours, including DIPG, frequently exhibit headaches as a symptom. Children may experience headaches that are ongoing or getting worse, frequently in conjunction with other symptoms.

7. Neurological And Behavioural Changes

DIPG can result in behavioural modifications such as impatience, mood swings, and personality changes. Children might also experience neurological symptoms like vertigo, convulsions, or shifts in awareness.

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5 Treatment Of Pontine Glioma

The prognosis for diffuse intrinsic pontine glioma (DIPG) currently has only a few treatment options available. It is not possible to remove the tumour surgically because of the brainstem’s location and the DIPG’s infiltrative nature. The blood-brain barrier makes it difficult to deliver medications to the tumour location efficiently. To manage DIPG and enhance the quality of life for affected children, numerous therapy modalities are employed. 

These consist of the following:

1. Radiation Therapy

The primary treatment method for DIPG tumours is radiation therapy. It seeks to lessen symptoms, decrease the tumour, and increase overall survival. Focused radiation is frequently administered to the tumour area over many weeks as part of the treatment. Although radiation therapy can temporarily reduce a tumour’s size, this rarely happens.

2. Chemotherapy

Several chemotherapeutic drugs have been used to treat DIPG, but their efficacy has been limited. They could be used as a palliative treatment or alongside radiation therapy. Clinical studies are continually being undertaken to evaluate new chemotherapeutic medicines and medication delivery strategies.

3. Therapies Targetting Molecular Abnormalities

Therapies targeting certain molecular abnormalities observed in DIPG tumours are being investigated by researchers. These treatments block the signalling pathways that contribute to tumour survival and proliferation. Targeted treatments for DIPG tumours are currently in the experimental stages, despite being hopeful, and are primarily accessible through clinical trials.

4. Immunotherapy

Immunotherapy employs the immune system’s ability to recognise and destroy cancerous cells in the patient’s body. In clinical trials for the treatment of DIPG, immunotherapeutic techniques such as immune checkpoint inhibitors and personalised vaccinations are being studied. However, the results are only beginning.

5. Supportive care

Giving the child supportive care to assist them to manage their symptoms and improve their quality of life is an important part of DIPG tumour treatment. Palliative care services, occupational therapy, physical therapy, speech therapy, and other therapies may be used to alleviate pain, breathing issues, and other symptoms.

Therapy possibilities are still developing because DIPG is a rare and complicated condition. Clinical studies and research projects are still being conducted to investigate novel treatments and enhance outcomes for kids with DIPG. 

The best way to fulfil the requirements of the affected child is for families to collaborate closely with a specialised medical team to explore the range of treatment choices, potential clinical trials, and supportive care strategies.

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DIPG In Adults 

Adults rarely acquire DIPG, which is primarily a childhood brain tumour. Children between the ages of 5 and 10 account for the vast majority of DIPG cases, and the frequency drops dramatically after adolescence.

Although DIPG typically affects children, a few sporadic cases of DIPG in adults have been reported. The clinical appearance, diagnosis, and treatment strategy for adult DIPG are comparable to those in juvenile patients.

However, because of the relatively small percentage of adult cases, there is less specific information available about the characteristics and prognoses of DIPG in adults compared to paediatric instances.

Adult DIPG, like childhood DIPG, frequently develops in the pons of the brainstem. It is aggressive and malignant since it is a high-grade glioma. Adult DIPG symptoms are similar to those seen in children and can include changes in speech, limb weakness, coordination difficulty, breathing concerns, and cranial nerve dysfunction.

Adults suspected of having DIPG should get a complete evaluation from a medical staff with expertise in neuro-oncology. They can accurately diagnose the condition, go over available treatments, and assist the patient in illness control.

Overall, even though adult DIPG is incredibly uncommon, those who are diagnosed with this disorder should speak with medical professionals skilled in treating brain tumours to identify the best course of action, depending on their unique situation.

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Prognosis Of Pontine Glioma

Unfortunately, diffuse intrinsic pontine glioma (DIPG) has an extremely bad prognosis. The pons, a section of the brainstem, is affected by the aggressive and seriously cancerous DIPG brain tumour. 

Complete surgical excision of the tumour is impossible due to its location and infiltrative characteristics. In general, DIPG prognosis is worse than other kinds of brain tumours.

Although individual situations vary, the average survival period for children with DIPG tends to be less than a year after diagnosis. Despite advances in treatment, overall survival rates have not altered significantly over time.

DIPG prognosis can be influenced by a number of factors, including the child’s age, specific tumour characteristics, therapy response, and the presence of specific genetic disorders. The general bad prognosis linked to this condition, however, is not greatly affected by these circumstances.

Effective treatment is significantly hampered by the aggressiveness of DIPG and its placement in the brainstem. The tumour is resistant to current treatment approaches because it has invaded important structures and because medicines only partially cross the blood-brain barrier.

Clinical studies and research are still being conducted to examine novel therapeutic modalities and enhance outcomes for kids with DIPG. However, DIPG continues to be a fatal illness with a typically dismal prognosis as of right now. To improve the quality of life and offer comfort to affected children and their families, supportive care and palliative measures are essential components of managing DIPG.

When To See A Doctor?

If you observe a change that is unusual for you, you should consult your doctor. Or if you have any of the probable cancer signs and symptoms. Even if you have concerns about the symptom, avoid putting off visiting them. If you do not make an appointment, your anxiety is unlikely to go away.

If you are having trouble thinking, speaking, or finding words. Personality or behaviour changes. Weakness, numbness, or lack of movement in one side or area of the body. If you have trouble keeping your balance or are dizzy, see your doctor right away.

The Final Say 

The fundamental goal of DIPG treatment should be to maintain proper nutrition in order to improve general health and well-being. Focus on eating a well-balanced diet rich in fruits, vegetables, whole grains, and lean proteins. Water intake must also be sufficient, hydration is key.

Work with a speech therapist if swallowing issues develop with the pontine glioma. Consider having easy-to-eat snacks and modest, regular meals. Get in touch with Bansal Hospital’s Medical Oncology and Radiation Oncology departments for more information.

FAQs 

1. How long does a kid with DIPG survive?

Individuals suffering from DIPG disease have an average overall survival of just under a year, generally between 8 and 11 months. 10% of patients live for at least two years following diagnosis. Only 2% of persons who are diagnosed live for at least 5 years.

2. Can be DIPG reversed?

DIPG develops so that it cannot be surgically removed (often called “resection”). The tumour margins are unclear, and the cancerous tissue invades healthy tissue. There is no way to avoid removing healthy brain tissue required for living. Thus, surgery cannot remove the tumour.

3. Children with DIPG put on weight?

Extreme appetite is one of the most acute side effects, so kids with DIPG frequently acquire much weight. Steroid use is also linked to personality changes like impatience, anxiety, and mood swings.

4. Is DIPG uncomfortable?

Headaches are brought on by it, especially in the morning. Pressure and pain are brought on by the tumour’s growth in the brain. Vomiting and nausea are two symptoms of elevated pressure inside the brain.

5. What is DIPG’s concluding phase?

Your child’s breathing and heartbeat will stop during the latter phases. If your child is hospitalized, the medical staff will confirm the death and take away equipment and medications. Some families want to assist in bathing the kid for the medical team.

About Bansal Hospital

Bansal Hospital is a multispeciality hospital and is one of the leading, reputable and reliable healthcare providers trusted by patients and their families across the region. It has all the major departments, including cardiology, neurology, oncology, orthopaedics, gastroenterology, urology, liver transplant, bone marrow transplantation, nephrology, gynaecology and more. The hospital is equipped with state-of-the-art facilities and technology and has a team of highly qualified and experienced doctors and medical staff who provide round-the-clock care to the patient.

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