Young children <3yo

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Paediatric cranial tumours

Numbers

• Supratentorial tumours (1.5:1)

• High grade astrocytomas, ependymomas, medulloblastomas

• Other embryonal tumours eg. supratentorial PNET, ATRT rare

Types of tumours

Ependymoma

• Arise from ventricular ependymal lining

• Majority diagnosed <5yo

• ~60% posterior fossa

• More extrusion into CP angle and foramen magnum

• Ependymoma frequently expands into FM and CPA.

• Treatment by complete surgical resection.

 

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

Molecular features

• Infratentorial (CIMP)
• EPN-PFA:
• <3yo, CIMP +ve
• High recurrence, 33% PFS at 5yrs
• EPN-PFB:
• Adolescent to young adult, CIMP -ve
• 73% PFS, 100% OS at 5yrs

• Supratentorial (RELA)
• 70% extraventricular
• More heterogenous T1 and T2 signals
• Cyst / calcifications / haemorrhage
• EPN-RELA:
• 70% of all supratentorial EPN, median age 8
• C11orf95-RELA fusion, NF-kB pathway activation
• Worse outcome, 29% PFS 72% OS at 5 yrs
• EPN-YAP1:
• Relatively stable genome
• 66% PFS and 100% OS in 5yrs
• Currently no targeted therapy
• Arise from superior medullary velum

Treatment

• Better prognosis if complete resection

• Approaches
• Telovelar
• Transvermian
• Cerebellar mutism
• Intention tremor (dentate nucleus retraction)
• Limited lateral exposure
• Telovelar>transvermian approaches

• Propensity for leptomeningeal spread 5-22%

• LP, MRI spine to look for drop mets

• Cranio+/- spinal RT

• Limited role for chemotherapy

• Early postop MRI in 48 hrs +/- Second look surgery

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