Paeds · haematology-oncology-and-transfusion

Brain and spinal tumours

Also known as Paediatric central nervous system tumours · Childhood brain tumour · Posterior fossa tumour · Medulloblastoma · Diffuse intrinsic pontine glioma · DIPG · Diffuse midline glioma H3 K27-altered · Pilocytic astrocytoma · Spinal cord tumour

Fellowship guide to brain and spinal tumours in children. Covers the central nervous system as the second commonest site of childhood cancer and the leading cause of cancer death in children, the predominance of the posterior fossa in the young brain with medulloblastoma, cerebellar pilocytic astrocytoma, ependymoma and brainstem glioma, the supratentorial lesions of pilocytic astrocytoma and craniopharyngioma, the clinical red flags of raised intracranial pressure with early morning headache and vomiting and papilloedema, the triad of cranial nerve palsy, long tract signs and ataxia that declares the diffuse intrinsic pontine glioma, the urgent imaging pathway with magnetic resonance imaging of the brain and the whole neuraxis, the perioperative stabilisation with dexamethasone for vasogenic oedema and the management of hydrocephalus, the molecular classification that has reshaped medulloblastoma into WNT, SHH, Group 3 and Group 4 subgroups, the surgical, radiotherapy and chemotherapy principles, the devastating prognosis of the diffuse intrinsic pontine glioma and the curative potential of the pilocytic astrocytoma, and the long term late effects that shape survivorship.

high12 referencesUpdated 16 July 202636 min readVerification in progress

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Red flags

A child with progressive early morning headache and vomiting, worse on waking and relieved by vomiting, has raised intracranial pressure from a brain tumour until imaging proves otherwiseA child with the triad of a cranial nerve palsy, pyramidal long tract signs and cerebellar ataxia has a diffuse intrinsic pontine glioma until the magnetic resonance scan settles itA posterior fossa mass that compresses the fourth ventricle can obstruct the cerebrospinal fluid pathway and produce acute hydrocephalus and herniation within hoursA child with back pain, progressive limb weakness, a sensory level or new sphincter disturbance has spinal cord compression and needs emergency imaging and steroidsMedulloblastoma spreads through the cerebrospinal fluid, so craniospinal imaging and cerebrospinal fluid cytology are mandatory before the treatment plan is setCraniospinal irradiation is avoided under three years of age because of the devastating neurocognitive injury, and a chemotherapy-first strategy is used instead

Life stages

infanttoddlerpreschoolschool-ageadolescentyoung-adult-transition

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Recognises that brain tumours are the commonest solid tumour of childhood and the second commonest childhood malignancy after leukaemiaIdentifies the red flag pattern of progressive early morning headache and vomiting with papilloedema as raised intracranial pressure needing urgent imagingNames the common posterior fossa tumours of childhood: medulloblastoma, cerebellar pilocytic astrocytoma, ependymoma and brainstem gliomaExplains the molecular classification of medulloblastoma into WNT, SHH, Group 3 and Group 4 subgroups and how each drives prognosis and risk-adapted therapyDistinguishes the diffuse intrinsic pontine glioma, defined radiographically and by the H3 K27M mutation, from the resectable posterior fossa tumours, and explains why it is treated with focal radiotherapy aloneCoordinates the urgent diagnostic and multidisciplinary pathway from magnetic resonance imaging of the brain and whole neuraxis through surgery, radiotherapy and chemotherapy to the survivorship planProgressive early morning headache and vomiting with papilloedema is raised intracranial pressure from a tumour until imaging proves otherwiseThe triad of cranial nerve palsy, pyramidal long tract signs and cerebellar ataxia signals a brainstem tumour, classically the diffuse intrinsic pontine gliomaDexamethasone is used perioperatively to control vasogenic oedema, and craniospinal irradiation is avoided under three years of age because of neurocognitive harmCounsels the family of a child newly diagnosed with a diffuse intrinsic pontine glioma on the incurable nature of the disease, the role of palliative radiotherapy and the prognosisBuilds the multidisciplinary plan that runs paediatric oncology, neurosurgery, radiation oncology, endocrinology, neuropsychology and the family togetherSynthesises the long-case child with a posterior fossa tumour into a treatment and late-effects surveillance plan covering neurocognition, endocrinopathy and secondary malignancyLevel 1: Recognition of the red flag features of a brain tumour in a child and the urgent referral for imagingLevel 2: Application of the diagnostic pathway with magnetic resonance imaging of the brain and the whole neuraxis and the multidisciplinary assessmentLevel 3: Coordination of risk-adapted definitive therapy and long-term survivorship surveillance across oncology, endocrinology and neuropsychologyThe posterior fossa as the commonest site of paediatric brain tumour and the tumours that arise thereThe clinical features of raised intracranial pressure in the infant with the open fontanelle and in the older childThe molecular subgroups of medulloblastoma and their prognostic implicationsStructured neurological examination of the child with a suspected brain tumour for raised pressure, cranial nerves, cerebellar signs and long tractsCommunication of a new diagnosis of a brain tumour, including the incurable diffuse intrinsic pontine glioma, to a child and familyRecognition of the oncologic and surgical emergencies of hydrocephalus, herniation and spinal cord compressionBrain tumours as the commonest solid tumour of childhood and a leading cause of cancer death in childrenThe clinical presentation of paediatric brain tumours by location, posterior fossa, supratentorial, brainstem and spinalThe principles of multimodal therapy with surgery, radiotherapy and chemotherapy and the age-related considerationsRecognition of the red flag clinical features of a paediatric brain tumour that mandate urgent imaging and referralApplication of the initial stabilisation of a child with a brain tumour including dexamethasone and the management of hydrocephalusCoordination of the diagnostic and multidisciplinary pathway for a child with a brain or spinal tumourCanadian approach to the urgent evaluation and referral of a child with a suspected brain tumourThe molecular risk stratification of medulloblastoma and the contemporary risk-adapted therapyThe long-term late effects of cranial irradiation and the surveillance of the paediatric brain tumour survivor

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Target exams

RACP DWERACP DCEMRCPCH TheoryMRCPCH ClinicalABP General Pediatrics

Red flags

Life stages

infanttoddlerpreschoolschool-ageadolescentyoung-adult-transition

Care settings

outpatientwarded-acutepicuretrieval

Clinical exam formats

written-only

Board mappings

Overview & Definition

A child is brought in with three weeks of worsening headache on waking, vomiting that relieves the headache, and a new squint, and the question at the bedside is whether this is a benign tension headache or the first sign of a tumour inside a rigid box. Brain and spinal tumours are the commonest solid tumours of childhood and, after infancy, the leading cause of cancer death in children, and the single thing that separates the child who does well from the child who does not is how fast the clinician recognises the pattern of raised pressure and the focal deficit and reaches the scan. The central nervous system is a closed space, the tumour adds volume that the skull cannot expand in the older child, and the symptoms of raised intracranial pressure follow a logic that every paediatrician must own. [1][2]

A central nervous system tumour in a child is a mass of abnormal cells arising within the brain, the spinal cord, or their coverings, and it behaves very differently from its adult counterpart. The young brain is dominated by the posterior fossa, so that roughly half of all paediatric brain tumours sit below the tentorium, in the cerebellum, the fourth ventricle, or the brainstem, which is the reverse of the adult pattern. The tumours are also biologically distinct, with the embryonal tumours such as medulloblastoma, the grade one pilocytic astrocytoma, and the diffuse midline gliomas that have no adult equivalent. The molecular era has rewritten the classification, so that the diagnosis now pairs the histology with a defining molecular alteration that drives both the prognosis and the treatment. [2][3]

The first task at the bedside is not to name the tumour but to judge whether the child is in danger from raised pressure, from hydrocephalus, or from a focal deficit that threatens a critical pathway. The second task is to image the brain, and the third is to build the multidisciplinary plan that runs paediatric oncology, neurosurgery, radiation oncology, endocrinology, and neuropsychology together. The gravity of the topic is the reason it sits at the heart of the fellowship examination, because a candidate who can read the pattern, reach the scan, and chart a safe path through the surgery, the radiotherapy and the late effects is demonstrating exactly the reasoning the boards test. [1][12]

One-sentence answer for the exam

A child with a brain or spinal tumour presents most often with the features of raised intracranial pressure, progressive early morning headache and vomiting and papilloedema, or with a focal deficit such as ataxia, a cranial nerve palsy or a progressive paraparesis, the posterior fossa is the commonest site in children holding medulloblastoma, cerebellar pilocytic astrocytoma, ependymoma and the brainstem glioma, the diagnosis is made on magnetic resonance imaging of the brain and the whole neuraxis, the diffuse intrinsic pontine glioma carries the H3 K27M mutation and a near uniform fatal prognosis treated with focal radiotherapy alone, while medulloblastoma is risk-stratified by its molecular subgroup into WNT, SHH, Group 3 and Group 4 and treated with maximal safe resection, craniospinal irradiation and chemotherapy, and the long term survivor is watched for the neurocognitive, endocrine and second malignancy late effects of the therapy that cured the tumour.

[2][3][7][12]

Paediatrics Fellowship Pro

You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References12Show ledger

[1]Ostrom QT, Price M, Ryan K CBTRUS Statistical Report: Pediatric Brain Tumor Foundation Childhood and Adolescent Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2014-2018 Neuro Oncol, 2022.PMID 36066969
[2]Pollack IF, Agnihotri S, Broniscer A Childhood brain tumors: current management, biological insights, and future directions J Neurosurg Pediatr, 2019.PMID 30835699
[3]Northcott PA, Robinson GW, Kratz CP Medulloblastoma Nat Rev Dis Primers, 2019.PMID 30765705
[4]Taylor MD, Northcott PA, Korshunov A Molecular subgroups of medulloblastoma: the current consensus Acta Neuropathol, 2012.PMID 22134537
[5]Ramaswamy V, Remke M, Bouffet E Risk stratification of childhood medulloblastoma in the molecular era: the current consensus Acta Neuropathol, 2016.PMID 27040285
[6]Leary SES, Packer RJ, Li Y Efficacy of Carboplatin and Isotretinoin in Children With High-risk Medulloblastoma: A Randomized Clinical Trial From the Children's Oncology Group JAMA Oncol, 2021.PMID 34292305
[7]van den Bent M, Saratsis AM, Geurts M H3 K27M-altered glioma and diffuse intrinsic pontine glioma: Semi-systematic review of treatment landscape and future directions Neuro Oncol, 2024.PMID 38102230
[8]Bornhorst M, Frappaz D, Packer RJ Pilocytic astrocytomas Handb Clin Neurol, 2016.PMID 26948364
[9]Boop SH, Shimony N, Boop FA Review and update on pediatric ependymoma Childs Nerv Syst, 2023.PMID 37493720
[10]Gan HW, Morillon P, Albanese A National UK guidelines for the management of paediatric craniopharyngioma Lancet Diabetes Endocrinol, 2023.PMID 37549682
[11]Malbari F, Staggers KA, Minard CG Provider views on perioperative steroid use for patients with newly diagnosed pediatric brain tumors J Neurooncol, 2020.PMID 32026434
[12]Rey-Casserly C, Diver T Late effects of pediatric brain tumors Curr Opin Pediatr, 2019.PMID 31693589

Brain and spinal tumours

high12 referencesUpdated 16 July 202636 min readVerification in progress

Practise this topic

Overview & Definition

Paediatrics Fellowship Pro

You have read the opening of this topic. The complete unit — every section and its primary-source references — is part of the Paediatrics Fellowship fellowship atlas.

References12Show ledger

[1]Ostrom QT, Price M, Ryan K CBTRUS Statistical Report: Pediatric Brain Tumor Foundation Childhood and Adolescent Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2014-2018 Neuro Oncol, 2022.PMID 36066969
[2]Pollack IF, Agnihotri S, Broniscer A Childhood brain tumors: current management, biological insights, and future directions J Neurosurg Pediatr, 2019.PMID 30835699
[3]Northcott PA, Robinson GW, Kratz CP Medulloblastoma Nat Rev Dis Primers, 2019.PMID 30765705
[4]Taylor MD, Northcott PA, Korshunov A Molecular subgroups of medulloblastoma: the current consensus Acta Neuropathol, 2012.PMID 22134537
[5]Ramaswamy V, Remke M, Bouffet E Risk stratification of childhood medulloblastoma in the molecular era: the current consensus Acta Neuropathol, 2016.PMID 27040285
[6]Leary SES, Packer RJ, Li Y Efficacy of Carboplatin and Isotretinoin in Children With High-risk Medulloblastoma: A Randomized Clinical Trial From the Children's Oncology Group JAMA Oncol, 2021.PMID 34292305
[7]van den Bent M, Saratsis AM, Geurts M H3 K27M-altered glioma and diffuse intrinsic pontine glioma: Semi-systematic review of treatment landscape and future directions Neuro Oncol, 2024.PMID 38102230
[8]Bornhorst M, Frappaz D, Packer RJ Pilocytic astrocytomas Handb Clin Neurol, 2016.PMID 26948364
[9]Boop SH, Shimony N, Boop FA Review and update on pediatric ependymoma Childs Nerv Syst, 2023.PMID 37493720
[10]Gan HW, Morillon P, Albanese A National UK guidelines for the management of paediatric craniopharyngioma Lancet Diabetes Endocrinol, 2023.PMID 37549682
[11]Malbari F, Staggers KA, Minard CG Provider views on perioperative steroid use for patients with newly diagnosed pediatric brain tumors J Neurooncol, 2020.PMID 32026434
[12]Rey-Casserly C, Diver T Late effects of pediatric brain tumors Curr Opin Pediatr, 2019.PMID 31693589