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CNS tumors
Dr. Waleed Dabbas
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Background
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Frequency
Children : -Posterior fossa tumors ( Infra-Tentorial).
-Astrocytoma, Ependymoma, and Medulloblastoma.
Adults : -The most common malignant brain tumor in adults is GBM.
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Incidence
• Overall incidence of primary brain tumor is 9.5 cases per 100,000 population in the United States.
• More than 50% are gliomas
• Brain tumors represent 30% of all childhood malignancies
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Epidemiology
• Gender
– Slight increased brain tumors in males
– Two peaks
• Smaller peak in pediatric age group
• Second peak in elderly population between 70-80 years of age
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Etiology and Risk Factors
• Several genetic disorders cause or increase risk for brain tumors.
– Neurofibromatosis
– Tuberous sclerosis
– Turcots syndrome
– Osler-Weber-Rendu syndrome
– Li-Fraumeni syndrome
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Symptoms and signs
• Seizures – 54% at presentation
• Headaches – 71%
• Mental change – 53%
• Hemiparesis – 43%
• Cranial nerve – 14%
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Brain tumor syndromes
• Seizure : Cortical lesion• Behavioral : Anterior Cranial fossa.• Weakness: internal capsule or primary motor cortex• Vision : optic nerve compression• Speech: Broca’s area.• Endocrine : prolactinoma , etc..• Torticollis : post. Cranial fossa tumor compressing the
XI nerve.• Anosmia : Foster-Kennedy Syndrome ( ant. Cranial
fossa tumor ) “ ipsilateral Optic nerve atrophy + Contralateral papilledema “
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Diagnosis
• Imaging studies available are MRI– On MRI the high grade of malignant gliomas
appear as contrast enhancing mass lesions
• CT scan– Contrast enhanced CT scan for use if MRI is
unavailable
• Nuclear medicine studies– Brain scan not used much now
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Classifications
By origin : Primary VS secondary
By location : supratentorial VS infratentorial
By cell type e.g. glioma,menengioma,oligodendroglioma>>>
By grade: 1,2,3,4
Benign VS malignant ?
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Meningioma
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Menengiomas
• Benign, Rarely malignanat
• Can grow at any part of the meninges, common sites: cerebral convexity, falx cerebri, parasagital.
• Age: 40-50 .
• More in Female “ progesterone receptors on meningiothelial cells” .
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Hemangioblastoma
• Benign
• Most common adult Primary Post. Cranial Fossa tumor
• Highly Vascularized
• Two forms : Cystic VS. Solid.
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Neuroepithelial TumorsGLIOMA
Type of Cells:
A. Astrocytic tumors
B. Oligodendroglial tumors
C. Ependymal tumors
D. Mixed gliomas
E. Choroid plexus tumors
Grade: Low VS. High
Location: SupraTentorial VS. InfraTent.
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Astrocytoma
Grade I & II:- Superficial & Infratentorial. ( Benign )
- Young ages(Juv. Cerebellar Astrocytoma)
Grade III & IV: Anaplastic Astrocytoma
Glioblastoma Multiforme
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Oligodendroglioma
• Microcalcification is common.
• Well differentiated VS Anaplastic “less common”
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Ependymoma
• Ependymal cells that line the ventricles.– less than 2% of brain
tumors.– Usually low grade
histology but have a high grade of recurrence
• Supratentorial→ 3rd
ventricle+malignantInfratentorial→ 4th
ventricle+Benign“morecommon”
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Choroid Plexus Papilloma* Rare *
• From the Choroid plexus → inc.CSF production
→Hydrocephalus.
• Supratentorial →Children ( Lateral Vent. )
Infratentorial → Adult ( Rare )
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Medulloblastoma
• PNET (30% of pediatric brain tumors )
• Midline in the vermis of the cerebellum & extend to the 4th
ventricle.
• Young, hydroceph., ataxia.
• Spread through CNS, bone.
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Pituitary tumors
Pituitary tumors have three features:
Local mass effect
Visual field disturbance (bitemporal hemianopia).
headache
Hormone deficiency: deficiency tend to first suppress LH and GH, followed in sequence by loss of TSH,ACTH and FSH
Hormone excess
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Craniopharyngioma
Derived from remnants of the structures that give rise to pituitary gland.
Mass effect (headache, visual field defect)
Hormonal deficiencies
Could be cystic or solid with or without calcification.
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Spinal tumors
1 Extradural :
Secondary deposits in the spine(commonest)
primary vertebral bony tumors(osteoclastoma,myeloma)
Lymphomas
2 Intradural extramedullary
Meningioma
Neurofibroma
3 Intramedullary (rare)
Glioma
Ependymoma
Others such as hemangioma
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Involvement of the CNS by Systemic Cancer• Brain metastases occur in 25-35% of all cancers.
– The peak incidence of brain metastases is bimodal ages 0-10 years and age 55-59 years.
– Location• 50% of brain metastases are supratentorial
– Lung cancer and breast cancers are most common metastatic tumors to the head.
– Melanoma has greatest potential for metastasis to the brain.
– Approximately 40% of all patients with systemic disease develop brain metastases.
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Prognostic Factors
• Include pathological grade, patient age and overall patient function at the time of diagnosis and duration of symptoms.
• Immediate survival with resection, radiotherapy and chemotherapy is three years for those with anaplastic astrocytoma and one year for those with glioblastoma multiform.
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Treatment
• Includes supportive and definitive treatment
– Supportive agents are anticonvulsant and corticosteroids
– Definitive therapy for intracranial tumor is surgery
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Treatment
• Surgical tools available include:
– Stereotactic frames• Definition provided three dimensional imaging system for accurate
targeting of brain lesions identifed on CT or MRI scan
– Image based guiding system• Definition frameless for image guided stereotactic system. Use
computer technology to co-register pre-operative imaging studies with intra-operative head position. These systems facilitate more complete tumor resection.
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Radiation Therapy
• Radiation plays a central role in treatment of brain tumors in adults.
– Whole brain versus partial brain radiation
– Dose to the response of low grade gliomas
– Other alternatives to conventional radiotherapy include hyperfractionated radiotherapy or use of radiosensitizers and boron neutron capture therapy
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Chemotherapy
• Chemotherapy regimens for gliomas include:
– Single agent BCN4 or combination chemotherapy of CCNU, procarbazine and vincristine