
Contents
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4.1 Primary brain tumours 4.1 Primary brain tumours
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Introduction Introduction
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Classification and grading of CNS tumours Classification and grading of CNS tumours
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Benign or malignant? Benign or malignant?
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Incidence Incidence
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Aetiology Aetiology
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Genetic syndromes associated with brain tumour Genetic syndromes associated with brain tumour
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Other aetiological factors Other aetiological factors
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Referral pathways Referral pathways
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Key ‘NICE’ recommendations Key ‘NICE’ recommendations
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4.2 Clinicopathology of brain tumours 4.2 Clinicopathology of brain tumours
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Introduction Introduction
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WHO Classification System 2016 WHO Classification System 2016
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Astrocytic and oligodendroglial tumours Astrocytic and oligodendroglial tumours
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Other astrocytic tumours Other astrocytic tumours
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Ependymal tumours Ependymal tumours
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Other gliomas Other gliomas
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Choroid plexus tumours Choroid plexus tumours
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Neuronal and mixed neuronal-glial tumours Neuronal and mixed neuronal-glial tumours
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Pineal tumours Pineal tumours
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Embryonal tumours Embryonal tumours
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Tumours of cranial and paraspinal nerves Tumours of cranial and paraspinal nerves
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Meningiomas Meningiomas
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Mesenchymal (non-meningothelial) tumours Mesenchymal (non-meningothelial) tumours
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Lymphomas Lymphomas
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Germ cell tumours Germ cell tumours
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Tumours of the sellar region Tumours of the sellar region
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Metastatic tumours Metastatic tumours
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Clinical pathological behaviour Clinical pathological behaviour
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WHO grade I tumours WHO grade I tumours
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WHO grade II gliomas WHO grade II gliomas
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WHO grades III and IV glioma WHO grades III and IV glioma
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Central neurocytoma Central neurocytoma
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Medulloblastomas Medulloblastomas
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Mesenchymal tumours Mesenchymal tumours
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Primary CNS lymphomas Primary CNS lymphomas
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Germ cell tumours Germ cell tumours
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Molecular biology of brain tumours Molecular biology of brain tumours
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Glioblastoma Glioblastoma
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Oligodendrogliomas Oligodendrogliomas
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Medulloblastoma Medulloblastoma
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Ependymoma Ependymoma
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Meningioma Meningioma
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4.3 Presentation and general management of primary brain tumours 4.3 Presentation and general management of primary brain tumours
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Presentation Presentation
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Headache Headache
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Seizures Seizures
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Progressive neurological deficit Progressive neurological deficit
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Personality or cognitive change Personality or cognitive change
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General medical management General medical management
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Steroids Steroids
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Antiepileptic drugs (AEDs) Antiepileptic drugs (AEDs)
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Anticoagulants Anticoagulants
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Analgesics Analgesics
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Antidepressants Antidepressants
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Prognostic factors in brain tumours Prognostic factors in brain tumours
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Detailed pathology Detailed pathology
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Age Age
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Performance status (PS) Performance status (PS)
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Surgery Surgery
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4.4 Surgical management of primary brain tumours 4.4 Surgical management of primary brain tumours
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Preparation for surgery Preparation for surgery
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Surgical technique Surgical technique
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Biopsy Biopsy
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Surgical resection Surgical resection
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General principles General principles
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Image-guided stereotactic resection Image-guided stereotactic resection
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Neuronavigation Neuronavigation
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Cortical mapping ‘awake craniotomy’ Cortical mapping ‘awake craniotomy’
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Fluorescence-guided surgery Fluorescence-guided surgery
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Surgery and individual tumours Surgery and individual tumours
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WHO grade I tumours WHO grade I tumours
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WHO grade II gliomas WHO grade II gliomas
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WHO grade III and IV gliomas WHO grade III and IV gliomas
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Medulloblastoma Medulloblastoma
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Lymphoma Lymphoma
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Germ cell tumours Germ cell tumours
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Intraoperative, non-resective approaches Intraoperative, non-resective approaches
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Local chemotherapy Local chemotherapy
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Convection enhanced delivery Convection enhanced delivery
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4.5 Radiotherapy for primary brain tumours 4.5 Radiotherapy for primary brain tumours
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Introduction Introduction
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Indications for radiotherapy Indications for radiotherapy
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Radiotherapy process Radiotherapy process
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Immobilization and imaging Immobilization and imaging
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Volume delineation Volume delineation
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Radiotherapy planning Radiotherapy planning
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Dose prescription Dose prescription
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Timing of radiotherapy Timing of radiotherapy
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Radiotherapy delivery and patient assessment Radiotherapy delivery and patient assessment
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Palliative treatments Palliative treatments
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Stereotactic radiotherapy Stereotactic radiotherapy
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Individual tumour types Individual tumour types
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Normal tissue reactions to radiotherapy Normal tissue reactions to radiotherapy
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Sparing normal tissue Sparing normal tissue
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Radiation tolerance limits of normal tissues Radiation tolerance limits of normal tissues
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Acute effects Acute effects
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Early delayed (intermediate) effects Early delayed (intermediate) effects
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Delayed radiation damage Delayed radiation damage
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4.6 Chemotherapy and new agents for primary brain tumours 4.6 Chemotherapy and new agents for primary brain tumours
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Introduction Introduction
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Active agents Active agents
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Nitrosoureas Nitrosoureas
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Procarbazine Procarbazine
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Temozolomide Temozolomide
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Gliadel® Gliadel®
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Epipodophyllotoxins and platinum compounds Epipodophyllotoxins and platinum compounds
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Vinca alkaloids Vinca alkaloids
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Combination chemotherapy Combination chemotherapy
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Chemotherapy for individual tumours Chemotherapy for individual tumours
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Gliomas Gliomas
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WHO grade I WHO grade I
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WHO grade II WHO grade II
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WHO grades III and IV WHO grades III and IV
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Medulloblastoma Medulloblastoma
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Primary CNS lymphoma Primary CNS lymphoma
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Germ cell tumours Germ cell tumours
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Meningiomas Meningiomas
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New agents New agents
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Antiangiogenesis inhibitors Antiangiogenesis inhibitors
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Growth factor signalling pathways Growth factor signalling pathways
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4.7 Outcome and management of recurrence in primary brain tumours 4.7 Outcome and management of recurrence in primary brain tumours
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Outcome Outcome
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WHO grade I tumours WHO grade I tumours
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WHO grade II gliomas WHO grade II gliomas
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WHO grades III and IV glioma WHO grades III and IV glioma
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Anaplastic astrocytoma Anaplastic astrocytoma
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Anaplastic oligodendroglioma Anaplastic oligodendroglioma
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Glioblastoma Glioblastoma
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Medulloblastoma Medulloblastoma
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Meningioma Meningioma
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Primary CNS lymphoma Primary CNS lymphoma
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Treatment of relapse Treatment of relapse
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WHO grade 1 tumours WHO grade 1 tumours
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WHO grade II gliomas WHO grade II gliomas
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High-grade glioma High-grade glioma
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Medulloblastoma Medulloblastoma
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Meningioma Meningioma
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Primary CNS lymphoma Primary CNS lymphoma
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Palliative care input Palliative care input
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New approaches New approaches
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Vaccines Vaccines
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PARP inhibition PARP inhibition
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Gene therapy Gene therapy
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Other potential targets Other potential targets
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4.8 Summary of management for primary brain tumours 4.8 Summary of management for primary brain tumours
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WHO grade I tumours WHO grade I tumours
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WHO grade II gliomas WHO grade II gliomas
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WHO grades III and IV glioma WHO grades III and IV glioma
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Anaplastic astrocytoma Anaplastic astrocytoma
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Anaplastic oligodendroglioma Anaplastic oligodendroglioma
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Glioblastoma Glioblastoma
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Medulloblastoma Medulloblastoma
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Mesenchymal tumours Mesenchymal tumours
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Primary CNS lymphoma Primary CNS lymphoma
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Gangliocytoma (and variants) Gangliocytoma (and variants)
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Central neurocytoma Central neurocytoma
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Pineocytoma/pineoblastoma Pineocytoma/pineoblastoma
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Germ cell tumours Germ cell tumours
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Craniopharyngioma Craniopharyngioma
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Chordoma Chordoma
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4.9 Brain metastases overview 4.9 Brain metastases overview
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Introduction Introduction
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Single or solitary? Single or solitary?
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Incidence Incidence
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Presentation Presentation
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Clinicopathology Clinicopathology
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Histopathology Histopathology
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Natural history Natural history
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Evaluation of the patient with brain metastasis Evaluation of the patient with brain metastasis
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Brain imaging Brain imaging
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Clinical evaluation Clinical evaluation
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Laboratory and imaging investigations Laboratory and imaging investigations
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Diagnostic evaluation for brain metastasis from unknown primary Diagnostic evaluation for brain metastasis from unknown primary
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All cases All cases
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Selected cases Selected cases
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4.10 Treatment options for brain metastases 4.10 Treatment options for brain metastases
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Initial management of the patient with brain metastases Initial management of the patient with brain metastases
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Factors to consider in the management of the patients with brain metastases Factors to consider in the management of the patients with brain metastases
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Systemic treatment Systemic treatment
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Whole brain radiotherapy (WBRT) Whole brain radiotherapy (WBRT)
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Neurosurgery Neurosurgery
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Radiosurgery Radiosurgery
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Best supportive care Best supportive care
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Management of the patient with oligometastasis Management of the patient with oligometastasis
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Role of surgery Role of surgery
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Role of radiosurgery Role of radiosurgery
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Advantages of SRS compared to neurosurgery Advantages of SRS compared to neurosurgery
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Disadvantages compared to microsurgery Disadvantages compared to microsurgery
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Prophylactic cranial irradiation Prophylactic cranial irradiation
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Management of special cases of brain metastases Management of special cases of brain metastases
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Management of brain metastases after neurosurgery Management of brain metastases after neurosurgery
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Re-irradiation Re-irradiation
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4.11 Outcome and summary for brain metastases 4.11 Outcome and summary for brain metastases
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Outcome Outcome
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Multiple metastases Multiple metastases
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Solitary metastases Solitary metastases
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Summary of management Summary of management
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Patients with multiple brain metastases and unknown primary Patients with multiple brain metastases and unknown primary
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Patients with multiple brain metastases and known primary Patients with multiple brain metastases and known primary
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Single/oligo brain metastases Single/oligo brain metastases
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Patients with further intracranial relapse following treatment Patients with further intracranial relapse following treatment
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4.12 Spinal cord tumours overview 4.12 Spinal cord tumours overview
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Introduction Introduction
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Tumour sites Tumour sites
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Clinicopathology Clinicopathology
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Low-grade tumours Low-grade tumours
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Astrocytomas Astrocytomas
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Ependymomas Ependymomas
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Haemangioblastoma Haemangioblastoma
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Meningioma (WHO grade I) Meningioma (WHO grade I)
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Peripheral nerve sheath tumour (WHO grade I) Peripheral nerve sheath tumour (WHO grade I)
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Other low-grade lesions Other low-grade lesions
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High-grade tumours High-grade tumours
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Genetic associations Genetic associations
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Presentation Presentation
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Examination Examination
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Differential diagnosis Differential diagnosis
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Investigation Investigation
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4.13 Management of spinal cord tumours 4.13 Management of spinal cord tumours
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Management Management
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General management General management
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Surgery Surgery
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Radiotherapy Radiotherapy
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Chemotherapy Chemotherapy
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Management strategies Management strategies
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Low-grade astrocytoma Low-grade astrocytoma
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Ependymoma Ependymoma
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Meningioma Meningioma
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Haemangioblastoma Haemangioblastoma
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Nerve sheath tumours Nerve sheath tumours
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Other benign tumours Other benign tumours
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High-grade tumours High-grade tumours
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Prognostic factors Prognostic factors
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4.14 Outcome in spinal tumours 4.14 Outcome in spinal tumours
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Outcome in spinal tumours Outcome in spinal tumours
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Astrocytoma Astrocytoma
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Ependymoma Ependymoma
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Haemangioblastoma Haemangioblastoma
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Meningioma Meningioma
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Nerve sheath tumours Nerve sheath tumours
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Recurrence Recurrence
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Late sequelae of treatment Late sequelae of treatment
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4.15 Further reading and internet resources 4.15 Further reading and internet resources
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Further reading Further reading
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Internet resources Internet resources
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Cite
Abstract
Tumours of the central nervous system (CNS) are characterized by a remarkable diversity of pathological type and behaviour. Although rare, they affect people of all ages and hence have a disproportionate influence on the number of years of life lost to cancer. After many years of therapeutic stagnation, there have recently been some important improvements in molecular classification, treatment, and outcome. This chapter covers tumours of the CNS. Coverage of primary brain tumours includes: clinicopathology, presentation and general management, surgical management, radiotherapy, chemotherapy and new agents, and outcome/management of recurrence. The chapter also covers treatment options and outcomes for brain metastases and spinal cord tumours.
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