Primary Brain Tumours

Aetiology

Risk factors

  • Inherited syndromes with an increased risk of brain tumours include neurofibromatosis, von Hippel-Lindau disease, tuberous sclerosis and Li-Fraumeni syndrome
  • Ionising radiation
  • Immunosuppression

Pathophysiology

Aetiology of tumour headache

  • Raised ICP
  • Invasion/compression of dura, BVs, periosteum
  • Secondary to diplopia (CN III, IV, VI; INO)
  • Secondary to difficulty focusing
  • Extreme hypertension
  • Psychogenic (stress of loss of functional capacity)

Neurological signs correlate to tumour location

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Histological types

  • High-grade:
    • Gliomas and glioblastoma multiforme
    • Primary cerebral lymphomas
    • Medulloblastomas
  • Low-grade:
    • Meningiomas
    • Acoustic neuromas
    • Pituiary tumours
    • Pineal tumours
    • Craniopharyngiomas
    • Neurofibromas

Clinical presentation

  • Progressive neurological deficit
  • Usually motor weakness
  • Headache
    • May occur with or without raised ICP
    • Worse in the morning - wakes them up
    • Worse with coughing and leaning forward
    • May be associated with and made worse by vomiting
    • OR symptoms similar to tension HA/migrane
  • Seizures

Investigations

  • Diagnosis largely rests on brain imaging - e.g. CT scan and/or MRI scan (MRI more sensitive)
  • Other imaging may be used e.g. technetium brain scan is useful in the diagnosis of destructive skull vault (eg, metastases) and skull base lesions
  • Lesion biopsy

Management

Surgery

  • Tumours should be resected whenever possible (may not always be a viable option)
  • Surgery will also provide tissue for a formal diagnosis

Radiotherapy

  • External beam radiotherapy can be curative for many patients and also prolongs survival
  • Whole brain radiation is used with some tumours

Chemotherapy

  • The role of chemotherapy in brain tumours is not as marked as in other tumours - does provide modest benefit and is important in palliative care and as an adjunct to combined surgery and radiotherapy