Childhood and pre-adolescent 3-11yo

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Paediatric cranial tumours

Numbers

Types of tumours

Astrocytoma

• <3 yo and adolescence
• Usually supratentorial and high grade
• E.g. Anaplastic astrocytoma, GBM (esp if haemorrhage)

• Brainstem gliomas
• Up to 10-20% of all CNS paediatric tumours
• DIPG most common, present at 5-9 yo
• Average OS only 9-12 months
• NF1 better prognosis
• 80% H3K27M-mutant, diagnostic of DIPG
 

• Infratentorial pilocytic astrocytoma
• 10-20% of all paed tumours
• 4-10 yo, <20% in <3yo
• 80% WHO grade 1, 15% fibrillary astrocytoma, only 5% high grade
• MRI: large cyst with mural nodule, ~17-56% solid mass

• Treatment
• Surgical excision mainstay
• Reoperation for gross total resection if recurrence

• High grade gliomas
• 6.5% of all paediatric intracranial tumours
• Risk factors:
• Genetic syndromes: NF-1, Turcot syndrome, Li-Fraumeni
• Previous irradiation for haematological malignancies
• Differ in molecular characteristics and prognostic implications from adults
• High incidence 40.5% of TP53 mutation
• Less EGFR, PTEN mutation
• H3F3A histone gene mutation
• Very different genetic makeup of these tumour when compared to adults
• Treatment
• Aggressive maximal safe resection
• ChemoRT
• Future target therapies

Longterm complications

• ~74% children become 5 year survivors

• Problems related to associated syndromes:
• Secondary malignancy due to genomic instability

• Chemo
• Alkylating agents: fertility deficits 60% in men (sperm bank)
• Cisplatin: cochlear damage
• Etoposide: myeloplastic syndromes

• Radiotherapy
• Neurocognitive effects, especially if <5yo
• Endocrine, hypothalamic injury
• Increased stroke risk, moyamoya
• Secondary malignancies