Paediatric Brain Tumours: Types, Causes, Symptoms, Diagnosis and Treatment

The spectrum of paediatric brain tumours encompasses a myriad of entities, each distinct in its histogenesis, molecular profile, and clinical course. Among the most prevalent types are:

1. Astrocytomas: Arising from astrocytes, the star-shaped glial cells that provide structural and metabolic support to neurons, astrocytomas can occur throughout the brain and spinal cord. They are further subclassified based on their location and grade, ranging from low-grade pilocytic astrocytomas to high-grade glioblastomas.
2. Medulloblastomas: Predominantly affecting the cerebellum, medulloblastomas are aggressive embryonal tumours arising from primitive neuroectodermal cells. They often disseminate through the cerebrospinal fluid, necessitating comprehensive treatment strategies.
3. Ependymomas: Originating from ependymal cells lining the ventricles of the brain and the central canal of the spinal cord, ependymomas exhibit diverse clinical behaviour based on their location and histological subtype.
4. Craniopharyngiomas: These rare tumours develop near the pituitary gland and hypothalamus, often presenting with endocrine dysfunction and visual disturbances due to their proximity to critical structures.
5. Gliomas: Representing a heterogeneous group of tumours arising from glial cells, gliomas encompass entities such as oligodendrogliomas, oligoastrocytomas, and diffuse intrinsic pontine gliomas (DIPGs), each posing unique diagnostic and therapeutic challenges.

The clinical presentation of paediatric brain tumours is diverse and often reflects the tumour's location, size, and rate of growth. Recognizing the subtle manifestations of these tumours is paramount for timely intervention. Common symptoms include:

• Headaches: Persistent headaches, especially upon awakening or exacerbated by changes in posture, may signal increased intracranial pressure due to tumour mass effect.
• Nausea and vomiting: These symptoms, particularly when occurring in the morning or unrelated to gastrointestinal disturbances, can indicate elevated intracranial pressure.
• Seizures: Seizures, either focal or generalised, may manifest as an early symptom, especially in tumours involving the cerebral cortex.
• Neurological deficits: Weakness, sensory disturbances, gait abnormalities, and cranial nerve palsies may arise depending on the tumour's location and its impact on adjacent neural structures.
• Cognitive and behavioural changes: Children may exhibit alterations in behaviour, personality, attention, and academic performance, reflecting the tumour's influence on cognitive function and emotional regulation.

The aetiology of paediatric brain tumours remains multifactorial and incompletely understood. While genetic predispositions and environmental exposures may contribute to tumour development, the precise interplay of these factors remains elusive. Key factors implicated in paediatric brain tumour pathogenesis include:

• Genetic predispositions: Hereditary cancer syndromes, such as neurofibromatosis type 1 and 2, Li-Fraumeni syndrome, tuberous sclerosis, and familial adenomatous polyposis, confer an increased risk of developing brain tumours.
• Environmental exposures: While rare, exposure to ionising radiation, certain chemicals, or environmental toxins during critical periods of neurodevelopment may influence tumour initiation or progression.
• Molecular aberrations: Advances in molecular profiling have unveiled intricate molecular signatures underlying paediatric brain tumours, offering insights into tumour biology and potential therapeutic targets.

While complete prevention may not be achievable due to the multifactorial nature of these tumours, there are steps individuals and healthcare providers can take to reduce the risk and detect tumours early:

1. Genetic Counseling and Testing: Genetic counselling can be invaluable for families with a history of genetic conditions associated with brain tumours. Genetic counsellors assess familial risk factors, offer genetic testing when appropriate, and provide information about potential preventive measures. Understanding genetic predispositions allows families to make informed decisions about their healthcare and may guide screening and surveillance efforts.
2. Minimising Environmental Exposures: Although rare, exposure to certain environmental toxins or radiation may contribute to the development of paediatric brain tumours. Parents and caregivers can take proactive steps to minimise environmental exposures by:
   • Limiting exposure to ionising radiation: Minimise unnecessary medical imaging procedures that involve radiation, especially in children. Healthcare providers should adhere to established guidelines for appropriate imaging techniques and doses, particularly in paediatric populations.
   • Avoiding exposure to carcinogens: Be mindful of potential environmental toxins and carcinogens in the home, workplace, and community. This includes avoiding exposure to tobacco smoke, industrial chemicals, and other known carcinogens whenever possible.
3. Promoting Healthy Lifestyle Habits: While lifestyle factors may not directly cause paediatric brain tumours, adopting healthy habits can support overall well-being and potentially reduce the risk of certain cancers. Encourage children to:
   • Maintain a balanced diet: Emphasise the importance of a diet rich in fruits, vegetables, whole grains, and lean proteins. Limit processed foods, sugary snacks, and high-fat foods.
   • Stay physically active: Regular physical activity not only supports physical health but also contributes to mental well-being. Encourage children to engage in age-appropriate physical activities and limit sedentary behaviours, such as excessive screen time.
   • Practice sun safety: Protect children from excessive sun exposure by applying sunscreen, wearing protective clothing, and seeking shade during peak sun hours. Sunburns during childhood may increase the risk of certain cancers later in life.
4. Early Detection and Screening: While proactive measures can help reduce the risk of paediatric brain tumours, early detection remains paramount for timely intervention and improved outcomes. Healthcare providers play a crucial role in identifying warning signs and implementing appropriate screening protocols. Parents and caregivers should:
   • Be vigilant for signs and symptoms: Educate yourself about the signs and symptoms of paediatric brain tumours, such as persistent headaches, nausea, vomiting, and changes in behaviour or vision. Promptly report any concerning symptoms to a healthcare provider for further evaluation.
   • Attend regular well-child visits: Routine paediatric check-ups provide opportunities for healthcare providers to monitor a child's growth and development and detect any abnormalities early. Be proactive about scheduling and attending recommended appointments, even in the absence of obvious symptoms.
   • Advocate for appropriate diagnostic testing: If a child exhibits concerning symptoms suggestive of a brain tumour, advocate for timely diagnostic testing, such as MRI or CT scans. Early detection allows for prompt intervention and may improve treatment outcomes.

Accurate diagnosis forms the cornerstone of paediatric brain tumour management, guiding treatment decisions and prognostication. The diagnostic workup typically involves a comprehensive evaluation encompassing:

• Medical history: Thorough assessment of the child's medical history, including symptoms, developmental milestones, and family history of genetic syndromes or malignancies, provides invaluable insights into the diagnostic process.
• Physical examination: A meticulous neurological examination may reveal focal deficits, altered mental status, or signs of increased intracranial pressure, aiding in localising the tumour and assessing its impact on neurological function.
• Neuroimaging studies: Magnetic resonance imaging (MRI) serves as the primary imaging modality for evaluating paediatric brain tumours, offering superior soft tissue contrast and multiplanar capabilities. Computed tomography (CT) may complement MRI in certain scenarios, providing rapid assessment of bony anatomy and acute complications.
• Biopsy: Tissue sampling via stereotactic biopsy or surgical resection is often necessary to establish a definitive histopathological diagnosis, characterise tumour subtype, and guide treatment planning.

The staging of paediatric brain tumours aims to stratify disease extent and guide treatment algorithms, facilitating prognostication and therapeutic decision-making. While traditional staging systems such as the TNM classification are less applicable to paediatric brain tumours, various classification schemes based on histology, anatomical location, and molecular characteristics are employed. Staging considerations encompass:

• Tumour size and location: The size and anatomical location of the tumour influence the feasibility of surgical resection, the risk of neurological sequelae, and the likelihood of adjuvant therapy.
• Tumour grade: Histological grading, ranging from low-grade (grade I-II) to high-grade (grade III-IV), reflects the tumour's proliferative potential, cellular atypia, and mitotic activity, correlating with clinical behaviour and prognosis.
• Metastatic spread: The presence of metastases, either within the central nervous system (CNS) or extracranially, significantly impacts treatment strategies and long-term outcomes, necessitating comprehensive staging evaluations.

The management of paediatric brain tumours demands a multimodal approach tailored to the tumour's histology, location, grade, and the child's overall health status. Treatment modalities encompass:

1. Surgery: Surgical intervention is often the primary treatment for paediatric brain tumours whenever feasible. The goal of surgery is to remove as much of the tumour as possible while preserving neurological function. Neurosurgeons may use advanced imaging techniques, such as intraoperative MRI or neuronavigation, to precisely locate and remove the tumour while minimising damage to surrounding healthy brain tissue. In some cases, if complete removal is not possible, surgeons may perform a biopsy to obtain tissue samples for further analysis to guide subsequent treatment decisions.
2. Radiation Therapy: Radiation therapy plays a crucial role in the treatment of paediatric brain tumours, particularly for tumours that are difficult to access surgically or for those with residual disease after surgery. External beam radiation therapy (EBRT) delivers high-energy X-rays or protons to the tumour site, targeting and destroying cancer cells while minimising damage to surrounding healthy tissue. Proton therapy, a type of EBRT, is particularly advantageous for paediatric patients as it can deliver radiation with greater precision, reducing the risk of long-term side effects, especially in developing brains. Radiation therapy may be administered alone or in combination with surgery and chemotherapy, depending on the tumour type and stage.
3. Chemotherapy: Chemotherapy involves the use of powerful medications to kill cancer cells or slow their growth. In the context of paediatric brain tumours, chemotherapy may be used as a primary treatment modality for certain tumour types, such as medulloblastomas or high-grade gliomas, or as an adjuvant therapy following surgery or radiation therapy. Chemotherapy drugs may be administered orally, intravenously, or directly into the cerebrospinal fluid (intrathecal chemotherapy) to target tumour cells within the central nervous system. Combination chemotherapy regimens are often employed to maximise effectiveness while minimising the risk of drug resistance and side effects.
4. Autologous Stem cell Transplant – High risk medulloblastoma, relapsed medulloblastoma, Atypical-Teratoid Rhaboid tumor need high dose chemotherapy to improve outcomes. High dose chemotherapy can damage bone marrow permanently so to overcome this problem patient’s bone marrow stem cells are taken and cryopreserved before giving high dose chemotherapy. Later on after giving high dose chemo these cells are transfused back into the patient and this is known as autologous stem cell transplant. 
5. Targeted Therapy: Targeted therapy is a relatively newer approach to treating paediatric brain tumours that focuses on targeting specific molecules or pathways involved in cancer growth and spread. Unlike traditional chemotherapy, which non-selectively targets rapidly dividing cells, targeted therapy drugs are designed to specifically inhibit or block the activity of proteins or signalling pathways that are dysregulated in cancer cells. For example, molecularly targeted agents such as bevacizumab (anti-VEGF) or BRAF inhibitors may be used to treat certain subtypes of paediatric brain tumours, such as high-grade gliomas or BRAF-mutant gliomas. Targeted therapy may be used as a standalone treatment or in combination with surgery, radiation therapy, or chemotherapy, depending on the tumour's molecular profile and responsiveness to treatment.
6. Immunotherapy: Immunotherapy is an emerging treatment modality for paediatric brain tumours that harnesses the body's immune system to recognize and attack cancer cells. Immune checkpoint inhibitors, adoptive T-cell therapy, and cancer vaccines are among the immunotherapy strategies being investigated for their potential efficacy in treating paediatric brain tumours. Clinical trials evaluating the safety and efficacy of immunotherapy agents in paediatric patients with brain tumours are ongoing, with the hope of providing more effective and less toxic treatment options in the future.

The journey of recovery and aftercare for children with paediatric brain tumours extends far beyond the completion of initial treatment. It encompasses a holistic approach to supporting the child's physical, emotional, and cognitive well-being, as well as providing ongoing monitoring and support for both the child and their family.

1. Rehabilitation Therapy: Rehabilitation therapy plays a crucial role in helping children regain lost skills and adapt to any physical, cognitive, or speech challenges resulting from the brain tumour or its treatment. This may include:
   1. Physical Therapy: Physical therapists work with children to improve strength, balance, coordination, and mobility. They may provide exercises, assistive devices, and strategies to help children achieve their maximum physical potential.
   2. Occupational Therapy: Occupational therapists focus on helping children develop or regain skills needed for everyday activities, such as self-care, school tasks, and play. They may provide interventions to improve fine motor skills, cognitive abilities, and independence.
   3. Speech Therapy: Speech-language pathologists help children overcome speech and language difficulties resulting from the brain tumour or its treatment. They may work on improving communication skills, swallowing function, and cognitive-linguistic abilities.
2. Educational Support: Children who have undergone treatment for paediatric brain tumours may face challenges in returning to school or keeping up with academic requirements. Educational support services can help ensure a smooth transition back to school and provide accommodations as needed. This may include:
   1. Individualised Education Plan (IEP): Children with brain tumours may qualify for an IEP, which outlines specific educational goals, accommodations, and support services tailored to their needs.
   2. School Reintegration Programs: Schools may offer reintegration programs to help children transition back to the classroom environment gradually. This may involve modified schedules, extra support from teachers or tutors, and accommodations for physical or cognitive challenges.
3. Monitoring and Follow-Up Care: Regular monitoring and follow-up care are essential for children who have undergone treatment for paediatric brain tumours. This includes:
   1. Imaging Studies: Periodic imaging studies, such as MRI or CT scans, may be performed to monitor for any signs of tumour recurrence or progression.
   2. Neurological Assessments: Neurological evaluations by healthcare providers can help monitor for any changes in neurological function, such as motor skills, sensory abilities, or cognitive function.
   3. Endocrine Monitoring: Children with brain tumours located near the pituitary gland or hypothalamus may require ongoing monitoring of hormone levels and endocrine function.
   4. Psychosocial Support: Regular check-ins with mental health professionals can provide emotional support for both the child and their family members as they navigate the challenges of recovery and adjustment.
4. Support Services: Families of children with paediatric brain tumours may benefit from a range of support services to help cope with the emotional, practical, and financial challenges of managing the condition. This may include:
   1. Support Groups: Support groups provide a valuable opportunity for children and families to connect with others who are facing similar challenges, share experiences, and offer mutual support.
   2. Counselling Services: Individual or family counselling can help children and their families process their emotions, cope with stress, and develop effective coping strategies.
   3. Respite Care: Respite care services offer temporary relief for caregivers, allowing them to take breaks and recharge while ensuring that their child's needs are still met.
   4. Financial Assistance: Managing the costs associated with paediatric brain tumour treatment can be overwhelming. Financial assistance programs and resources may be available to help families navigate insurance, medical expenses, and other financial challenges.

1. What are paediatric brain tumours?
   Paediatric brain tumours are abnormal growths of cells within the brain or spinal cord of children.
2. Are paediatric brain tumours common?
   While they are relatively rare compared to adult brain tumours, paediatric brain tumours are still a significant health concern.
3. What age group is most affected by paediatric brain tumours?
   Paediatric brain tumours can affect children of any age, but they are most commonly diagnosed in children between the ages of 0 and 14.
4. What are the common symptoms of paediatric brain tumours?
   Common symptoms include headaches, nausea, vomiting, seizures, changes in vision, weakness, changes in behaviour, and difficulty walking.
5. Are all paediatric brain tumours cancerous?
   No, paediatric brain tumours can be either benign (non-cancerous) or malignant (cancerous).
6. What causes paediatric brain tumours?
   The exact cause is often unknown, but factors such as genetic predisposition, exposure to radiation, and environmental factors may play a role.
7. Can paediatric brain tumours be prevented?
   Preventing paediatric brain tumours entirely may not be possible, but minimising radiation exposure and genetic counselling may help reduce the risk.
8. How are paediatric brain tumours diagnosed?
   Diagnosis typically involves a combination of medical history review, physical examination, and imaging tests such as MRI and CT scans.
9. What are the stages of paediatric brain tumours?
   Staging helps determine the extent of the disease and may include stages I through IV based on factors such as tumour size and spread.
10. What are the treatment options for paediatric brain tumours?
    Treatment options may include surgery, radiation therapy, chemotherapy, and targeted therapy, depending on the tumour type and other factors.
11. Is surgery always required for paediatric brain tumours?
    Surgery is often the first-line treatment whenever feasible, but not all tumours may be surgically accessible or appropriate for surgery.
12. How effective is radiation therapy for paediatric brain tumours?
    Radiation therapy can be highly effective in killing cancer cells or slowing their growth, but it may also cause side effects, especially in children.
13. What is chemotherapy's role in treating paediatric brain tumours?
    Chemotherapy may be used in combination with other treatments to kill cancer cells or shrink tumours, but it can also cause side effects.
14. Are there any alternative treatments for paediatric brain tumours?
    While some alternative therapies may be complementary to conventional treatments, it's essential to discuss them with healthcare providers to ensure safety and effectiveness.
15. How long does paediatric brain tumour treatment typically last?
    The duration of treatment varies depending on factors such as tumour type, stage, and response to treatment. It may last for several months to years.
16. What are the potential side effects of paediatric brain tumour treatment?
    Side effects may include fatigue, nausea, hair loss, cognitive changes, and long-term effects on growth and development.
17. Can paediatric brain tumours recur after treatment?
    Yes, paediatric brain tumours can recur even after successful treatment, which is why regular follow-up care is essential.
18. What are the long-term effects of paediatric brain tumour treatment?
    Long-term effects may include cognitive impairments, physical disabilities, hormonal imbalances, and increased risk of secondary cancers.
19. How can families cope with the emotional impact of paediatric brain tumours?
    Support groups, counselling, and other resources can help families cope with the emotional challenges of managing a paediatric brain tumour diagnosis.
20. Can children with paediatric brain tumours lead a normal life after treatment?
    While the effects of treatment and the tumour itself may present challenges, many children can lead fulfilling lives with appropriate support and care.
21. What is the survival rate for paediatric brain tumours?
    Survival rates vary depending on factors such as tumour type, stage, and response to treatment. Some children may achieve long-term remission, while others may face recurrence or complications.
22. How can I support a friend or family member whose child has been diagnosed with a paediatric brain tumour?
    Offering emotional support, practical assistance, and being a good listener can help families navigate the challenges of paediatric brain tumour diagnosis and treatment.
23. Are there any clinical trials available for paediatric brain tumours?
    Yes, there are ongoing clinical trials investigating new treatments and approaches for paediatric brain tumours. Healthcare providers can provide information about eligibility and participation.
24. Can paediatric brain tumours affect a child's development and learning abilities?
    Yes, depending on factors such as tumour location and treatment effects, paediatric brain tumours can impact a child's cognitive development and learning abilities.
25. Are there any support organisations for families of children with paediatric brain tumours?
    Yes, there are several support organisations and foundations dedicated to providing resources, support, and advocacy for families affected by paediatric brain tumours.
26. Can paediatric brain tumours cause permanent disability?
    In some cases, paediatric brain tumours and their treatment may result in permanent disabilities, but many children can still achieve significant functional improvement with appropriate support and rehabilitation.
27. What role does nutrition play in managing paediatric brain tumours?
    A balanced diet and adequate nutrition are essential for supporting overall health and well-being during paediatric brain tumour treatment and recovery.
28. Can paediatric brain tumours affect a child's emotional and social development?
    Yes, paediatric brain tumours and their treatment can impact a child's emotional and social development, requiring support and interventions to address these challenges.
29. Are there any complementary therapies that can help manage symptoms of paediatric brain tumours?
    Some complementary therapies, such as acupuncture, massage therapy, and mindfulness techniques, may help manage symptoms and improve quality of life, but it's essential to discuss them with healthcare providers.
30. Can paediatric brain tumours cause learning disabilities?
    Depending on factors such as tumour location and treatment effects, paediatric brain tumours can impact learning abilities and may result in learning disabilities that require additional support and accommodations.
31. Is there a genetic predisposition to paediatric brain tumours?
    Yes, some genetic conditions, such as neurofibromatosis, tuberous sclerosis, and Li-Fraumeni syndrome, can increase the risk of developing paediatric brain tumours.
32. Are there any lifestyle changes that can help reduce the risk of paediatric brain tumours?While the exact causes of paediatric brain tumours are often unknown, maintaining a healthy lifestyle, minimising exposure to environmental toxins, and seeking regular medical care may help reduce the risk.
33. How do paediatric brain tumours affect a child's schooling?
    Depending on treatment effects and associated disabilities, paediatric brain tumours may impact a child's ability to attend school regularly and may require accommodations or specialised educational services.
34. Can paediatric brain tumours cause behavioural changes?
    Yes, paediatric brain tumours and their treatment can cause behavioural changes such as mood swings, irritability, and changes in personality, which may require psychological support and interventions.
35. Can paediatric brain tumours affect a child's mobility?
    Yes, depending on factors such as tumour location and treatment effects, paediatric brain tumours can impact a child's mobility and may require physical therapy or assistive devices to maintain or improve function.
36. Are paediatric brain tumours hereditary?
    While most paediatric brain tumours are not hereditary, some genetic conditions associated with an increased risk of brain tumours can be passed down through families.
37. How do paediatric brain tumours affect family dynamics?
    Paediatric brain tumours can have a profound impact on family dynamics, requiring adjustments to routines, roles, and responsibilities to provide care and support for the affected child.
38. Can paediatric brain tumours cause speech and language difficulties?
    Yes, depending on factors such as tumour location and treatment effects, paediatric brain tumours can affect speech and language abilities and may require speech therapy or other interventions.
39. Can paediatric brain tumours cause hormonal imbalances?
    Yes, paediatric brain tumours located near hormone-producing glands can disrupt hormone production and may require hormonal replacement therapy or other treatments.
40. How do paediatric brain tumours affect siblings?
    Paediatric brain tumours can have a significant impact on siblings, who may experience feelings of fear, uncertainty, and guilt, requiring support and reassurance from parents and caregivers.
41. Can paediatric brain tumours affect a child's sleep patterns?
    Yes, paediatric brain tumours and their treatment can disrupt sleep patterns, causing difficulties falling asleep, staying asleep, or experiencing restful sleep, which may require management and support.
42. Can paediatric brain tumours affect a child's sensory processing?
    Yes, depending on factors such as tumour location and treatment effects, paediatric brain tumours can affect sensory processing, leading to sensory sensitivities or difficulties, which may require sensory integration therapy or other interventions.
43. How do paediatric brain tumours affect a child's self-esteem?
    Paediatric brain tumours and their treatment can impact a child's self-esteem due to changes in physical appearance, abilities, and social interactions, requiring support and encouragement to foster a positive self-image.
44. Can paediatric brain tumours affect a child's bladder and bowel function?
    Yes, depending on factors such as tumour location and treatment effects, paediatric brain tumours can affect bladder and bowel function, leading to urinary or faecal incontinence or other difficulties, which may require management and support.
45. Can paediatric brain tumours cause memory problems?
    Yes, paediatric brain tumours and their treatment can impact memory function, leading to difficulties with short-term or long-term memory, which may require cognitive rehabilitation or other interventions.
46. Can paediatric brain tumours affect a child's social skills?
    Yes, paediatric brain tumours and their treatment can impact social skills, leading to difficulties with communication, social interaction, and peer relationships, which may require social skills training or other interventions.
47. Can paediatric brain tumours affect a child's ability to regulate emotions?
    Yes, paediatric brain tumours and their treatment can impact emotional regulation, leading to difficulties with mood stability, impulse control, and coping strategies, which may require psychological support and interventions.
48. Can paediatric brain tumours affect a child's executive functioning?
    Yes, paediatric brain tumours and their treatment can impact executive functioning, leading to difficulties with planning, organisation, problem-solving, and decision-making, which may require cognitive rehabilitation or other interventions.
49. Can paediatric brain tumours affect a child's sense of identity?
    Yes, paediatric brain tumours and their treatment can impact a child's sense of identity due to changes in physical abilities, cognitive function, and social interactions, requiring support and validation to maintain a positive self-concept.
50. Can paediatric brain tumours affect a child's ability to participate in recreational activities?
    Yes, depending on factors such as tumour location and treatment effects, paediatric brain tumours can impact a child's ability to participate in recreational activities, which may require modifications or alternative options to accommodate abilities and interests.