The information on this website is not intended for diagnosis or treatment. Please consult your physician for health concerns.

+90 850 321 50 00

Pediatric High-Grade Glioma (pHGG): Modern Care

Aggressive childhood brain tumors with molecular-driven treatment

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Onkoloji department. Book Appointment →

What is Pediatric High-Grade Glioma (pHGG): Modern Care?

Pediatric HGG includes diffuse midline glioma H3K27M-mutant, diffuse hemispheric glioma H3 G34-mutant and infant-type hemispheric glioma with characteristic molecular alterations.

Diffuse intrinsic pontine glioma (DIPG) is a subset of diffuse midline glioma with brainstem location and historically poor prognosis.

Diagnosis requires neuroimaging with MRI and molecular testing through biopsy where feasible, with H3K27M, H3 G34, BRAF, NTRK and ATRX testing.

WHO 2021 classification incorporates molecular alterations as defining features of pediatric HGG entities.

Prognosis varies by molecular subtype with H3K27M-mutant DIPG having median survival of about 12 months and other entities showing variable outcomes.

Symptoms

Headache often worse in morning, increasing in frequency and severity.
Nausea and vomiting from increased intracranial pressure.
Focal neurologic deficits depending on tumor location including weakness, sensory changes or speech difficulties.
Cranial nerve palsies particularly facial palsy and double vision in DIPG.
Ataxia, gait disturbance, swallowing difficulties and behavioral changes.

Risk Factors

Most pediatric HGG occurs sporadically without identifiable predisposing factors.
Cancer predisposition syndromes including Li-Fraumeni, neurofibromatosis type 1, constitutional mismatch repair deficiency and Lynch syndrome.
Prior cranial radiation increases secondary glioma risk.
Specific molecular alterations including H3K27M and H3 G34 mutations are central tumorigenic events.
Age and tumor location influence biology with infant gliomas often having distinct fusion-driven biology.

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

  • Persistent or worsening headaches, vomiting, vision changes or new neurologic deficits in children require prompt evaluation.
  • Symptoms of increased intracranial pressure including altered consciousness or papilledema are emergencies.
  • New seizures, weakness, swallowing problems or behavioral changes warrant urgent neurologic assessment.
  • Worsening symptoms during therapy or after treatment completion may indicate progression and need restaging.
  • Long-term survivorship care addresses neurocognitive, endocrine, audiologic and vascular complications.

Treatment Methods

01
Maximal safe resection when feasible is associated with improved outcomes in non-DIPG entities.
02
Radiation therapy with focal or craniospinal approaches based on disease distribution and patient age.
03
Standard chemotherapy with temozolomide and other agents has limited efficacy compared to adult HGG.
04
Molecularly targeted therapies are tailored to specific alterations including BRAF and MEK inhibitors for BRAF-altered tumors and NTRK inhibitors for NTRK-fusion tumors.
05
Clinical trials investigating immunotherapy, ONC201, panobinostat and convection-enhanced delivery offer hope; multidisciplinary pediatric neuro-oncology, palliative care, neurocognitive support and family-centered care are essential throughout the disease course.

Which Department to Visit?

You can visit our Onkoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

Learn About Onkoloji Department

Let us help you

You can make an appointment with our specialists or contact us for your concerns.

Book AppointmentContact Us

Related Health Topics

Other articles from the same department you may want to explore.

Anaemia

Dahiliye (İç Hastalıkları)

Anaemia is a low haemoglobin level that reduces oxygen delivery, causing fatigue, pallor, and shortness of breath. It is not a disease itself but a sign of many underlying conditions. Most cases are correctable with appropriate diagnosis and treatment.

Iron Deficiency Anaemia

Dahiliye (İç Hastalıkları)

Iron deficiency anaemia develops when dietary intake, absorption, or losses create an iron shortfall, most often affecting women and children. Identifying the underlying cause is the core of management, alongside iron replacement.

Vitamin B12 Deficiency

Dahiliye (İç Hastalıkları)

Vitamin B12 deficiency can cause megaloblastic anaemia, neurological symptoms, and cognitive impairment. Early treatment with intramuscular or oral B12 largely prevents irreversible complications.

Hypertension (High Blood Pressure) Management

Dahiliye (İç Hastalıkları)

Hypertension is often called the silent killer because it progresses symptom-free for years and can damage the heart, brain, kidneys, and eyes. Regular monitoring, lifestyle change, and evidence-based drug therapy dramatically reduce cardiovascular risk.

Chronic Kidney Disease

Dahiliye (İç Hastalıkları)

Chronic kidney disease is one of the most common complications of chronic conditions such as diabetes and hypertension, and can be silent in its early stages.

Hepatitis B (HBV)

Dahiliye (İç Hastalıkları)

Hepatitis B is a DNA virus infection causing acute and chronic hepatitis with risk of cirrhosis and hepatocellular carcinoma; diagnosis integrates HBsAg, HBeAg, anti-HBc, and HBV DNA with management based on disease phase using nucleos(t)ide analogues (entecavir, tenofovir) and universal infant vaccination.

Hepatitis C (HCV)

Dahiliye (İç Hastalıkları)

Hepatitis C is an RNA virus causing chronic hepatitis that may progress to cirrhosis and hepatocellular carcinoma; modern direct-acting antiviral (DAA) pangenotypic regimens (sofosbuvir/velpatasvir, glecaprevir/pibrentasvir) achieve sustained virologic response over 95% in 8–12 weeks with universal adult screening and cure for nearly all patients.

Fatty Liver Disease

Dahiliye (İç Hastalıkları)

Non-alcoholic fatty liver disease (NAFLD) is closely related to obesity and metabolic syndrome and is largely reversible with early treatment.

Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.