Doctors Revision

Oncology

Central Nervous System (CNS) Tumours (Paediatric Overview)

Tumours arising within the brain or spinal cord. Most common solid tumours in childhood after leukaemias. Wide variety of tumour types (e.g., astrocytoma, medulloblastoma, ependymoma, craniopharyngioma) with different locations, behaviours, and prognoses.

Also known as: Brain Tumour (Child), Spinal Cord Tumour (Child)

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Referral and warning signs

When to refer

Immediate urgent referral to specialized Paediatric Neuro-oncology/Neurosurgery centre for ALL suspected cases.

Red flags

  • Signs of increased intracranial pressure (persistent headache, morning vomiting, papilloedema, altered consciousness)
  • New onset seizures
  • Progressive focal neurological deficit
  • Ataxia or significant gait disturbance
  • Cranial nerve palsies

Signs and symptoms

  • Symptoms often related to Increased Intracranial Pressure (ICP): Headache (often worse in morning, awakens child from sleep, worsened by coughing/straining), Nausea and vomiting (especially morning vomiting), Lethargy, drowsiness, altered mental status, Papilloedema (swelling of optic discs on fundoscopy). Infants: Increasing head circumference, bulging fontanelle, separated sutures, 'sun-setting' eyes.
  • Focal Neurological Deficits (depend on tumour location): Seizures (new onset), Motor weakness/hemiparesis, Ataxia (unsteady gait)/coordination problems (cerebellar tumours), Cranial nerve palsies (e.g., facial weakness, eye movement abnormalities, hearing loss), Visual disturbances (visual field defects, decreased acuity), Speech difficulties, Endocrine dysfunction (e.g., growth failure, diabetes insipidus - hypothalamic/pituitary region tumours).
  • Spinal Cord Tumour Symptoms: Back pain, weakness/sensory loss in limbs, bowel/bladder dysfunction.

Risk factors

  • Most cases sporadic.
  • Associated with certain genetic syndromes (e.g., Neurofibromatosis type 1 & 2, Tuberous Sclerosis, Li-Fraumeni syndrome, Von Hippel-Lindau).
  • Previous cranial irradiation.

Diagnosis

Clinical assessment

Suspect based on persistent/progressive neurological symptoms, signs of increased ICP, or new onset seizures. Requires thorough neurological examination.

Investigations

  • Neuroimaging: MRI Scan (Brain and/or Spine) with contrast is the investigation of choice. Defines location, size, characteristics of tumour, hydrocephalus, oedema.
  • CT Scan (Head): Often initial imaging in emergency setting (faster), good for detecting calcification/haemorrhage/hydrocephalus, but less detail than MRI.
  • Biopsy or Surgical Resection: Essential for definitive histological diagnosis and molecular characterization, which guides treatment and prognosis.
  • Lumbar Puncture with CSF analysis (Cytology, Tumour markers): Assess for leptomeningeal spread (dissemination) - perform cautiously if signs of high ICP.
  • Ophthalmological assessment (visual fields, acuity, fundoscopy for papilloedema).

Diagnostic criteria

Diagnosis based on neuroimaging findings suggesting tumour, confirmed by histopathology from biopsy/resection.

Management

Non-pharmacological management

  • Referral to specialized Paediatric Neuro-oncology/Neurosurgery centre.
  • Management is multidisciplinary (neurosurgery, neuro-oncology, radiation oncology, neurology, endocrinology, rehabilitation).
  • Surgery: Maximal safe resection is often the primary goal (depends on tumour type/location). May be curative for some low-grade tumours. Also provides tissue for diagnosis.
  • Radiotherapy: Crucial for many malignant CNS tumours (e.g., medulloblastoma, high-grade glioma, ependymoma). Dose/field depends on tumour type/age (often deferred/avoided in very young children due to neurocognitive effects).
  • Supportive care: Management of increased ICP (steroids - Dexamethasone, CSF shunting), seizure control (anticonvulsants), rehabilitation (physio, OT, speech).

Pharmacological treatment

  • Chemotherapy: Role varies significantly by tumour type. Used for medulloblastoma, ependymoma, germ cell tumours, some gliomas. May be used neoadjuvantly, adjuvantly, or for recurrent disease.
  • Corticosteroids (Dexamethasone): Reduce vasogenic oedema around tumour, alleviate symptoms of increased ICP.
  • Anticonvulsants: If seizures present.

Monitoring

Regular neuroimaging (MRI) to monitor treatment response and detect recurrence. Neurological assessments. Endocrine monitoring (if pituitary region affected). Neuropsychological testing (assess long-term cognitive effects). Long-term follow-up for late effects.

Complications

  • Neurological deficits (related to tumour or treatment)
  • Increased intracranial pressure / Hydrocephalus
  • Seizures
  • Endocrine dysfunction
  • Cognitive impairment / Learning difficulties (late effect)
  • Hearing loss (platinum chemo, radiation)
  • Secondary malignancies (radiation-induced)
  • Tumour recurrence / progression

Prevention

  • No specific prevention for most types.

Patient education

  • (To parents/guardians) Explain diagnosis of brain/spinal tumour.
  • Need for urgent specialist evaluation and multidisciplinary care.
  • Explain planned investigations (MRI, biopsy/surgery).
  • Discuss treatment plan (surgery, radiation, chemo) based on tumour type/stage.
  • Potential short-term and long-term side effects.
  • Importance of follow-up and rehabilitation.
  • Prognosis varies widely by tumour type.
  • Psychosocial support resources.

Uganda-specific notes

Diagnosis and management require advanced neuroimaging (MRI), neurosurgery, paediatric oncology expertise, radiotherapy, and intensive supportive care, primarily available at UCI/Mulago.

References

  • Uganda Clinical Guidelines (UCG) 2023

    Chapter 12, Section 12.3.1 (Listed as CNS Tumours)

  • Uganda Cancer Institute Treatment Guidelines / Paediatric Oncology Protocols

    (Specific protocols)

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