Post on 27-Jan-2022
No this is untrue...and it has been proved by scientific studies
• Though the cause of primary brain tumor is still unknown in most of the cases,hereditary factors are implicated in 5%
• Various environmental risk factors play a vital role in evolving brain tumors
BRAIN TUMORSA condition which kills the patient much prior to his/her actual death!
• Each year approximately 1,90,000 people in USA and Canada will be diagnosed to have brain tumor
• In USA, the overall incidence of all primary brain tumors is around 14 per 1,00,000 people
• By that statistic, we in India should encounter at least 1 million brain tumors per year!
• Brain tumors including some benign tumors located in certain vital areas, lend to poor survival
• Brain tumors are the leading cause of solid tumor related deaths in children and young adults in 3rd & 4th decades of life
• There are 120 different varieties of brain tumors, making effective treatment complicated
• Symptoms of brain tumor vary from mild occasional headache to sudden onset of generalised seizures, or even sudden loss of consciousness (and impending death in a malignant brain swelling!)
• others may produce deficits pertaining to malfunction of structures the tumor involves, resulting in paralysis, dementia, imbalance and even blindness and deafness
What is Brain Tumor ?
It is an abnormal mass of tissue in which few cells multiply uncontrollably, apparently unregulated by the mechanisms which maintain 'milieu interior' of a normal cell
Oncogenesis: Does persistent use of cell phone cause Brain Tumor?
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Radiation: • Prior radiotherapy for pituitary tumors / hematologic malignancies have resulted in development of meningioma & intracranial sarcomas
• Radiation is also known to change the histology of gliomas
Genetic factors: • Activation of the proto-oncogene or inhibition of tumor suppressor genes
• Hereditary factor plays a role in origin of glioma
• Activation of oncogenes may be associated with production of growth factors like Epidermal Growth Factor (EGF), Nerve Growth Factors (NGF), Fibroblast Growth Factor (FGF), Tumor Angiogenesis Factor, Platelet Derived Growth Factors (PDGF) etc. which can stimulate the cell growth
e.g. location of NF1gene is on chromosome 17 location of NF2 gene is on chromosome 22 location of RB1 gene is on chromosome 13
Geographical variation: Germinomas, Pinealomas are more common in Japan Acoustic schwannomas are rare in African blacks
Hormonal factors: Meningiomas are more common in females and are associated with high count of estrogen & progesterone receptors
Trauma: Some meningiomas have been known to evolve at the site of old injuries
Infectious agents:
• No definite infectious agent has been identified to result in tumorogenesis
• However in animals few viruses like Polyoma JC virus,Simian virus-40, are known to cause gliomas
• Epstein Barr virus has been found to be association with Lymphomas
Estrogen receptors
Progesterone receptors
Adeno Virus
Chromosomal defects
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When to suspect a Brain Tumor?
Clinical presentation:
• Focal neurodeficit:
Mono/para/ hemiparesis
Focal sensory deficit
• Raised Intracranial Pressure (ICP) : Nausea, Vomiting, & Headache
“Classical early morning headache, relieved following vomiting”is a hallmark of raised Intra Cranial Pressure (ICP)
• Hydrocephalus:
Altered sensorium,
Quadriparesis,
Urinary incontinence,
Trunkal
& appendicular ataxia
VomitingHeadache
• Seizures: Focal seizures with secondary generalisation
Generalized tonic clonic
Temporal lobe seizures
Patients suffering from delayed onset seizures must be investigated thoroughlybefore offering them an antiepileptic drug
• Cranial nerve palsies:
Blindness
Diplopia, blurred vision
Hearing loss, vertigo, & tinnitus
Corneal ulceration, keratitis
Facial asymmetry, hemifacial spasm, drooling of saliva
Swallowing difficulty, hoarseness of voice
Corneal ulcer
Trigeminal Neuralgia Facial asymmetry Hearing loss vertigo
Convulsions
Hemisensory / Motor defecit Personality changes Ataxia
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Lay the personon the ground
Cushion theperson�s head
Loosen tightclothing
First Aid: Convulsions
Examination : Glasgow Coma Scale (GCS) Useful in case of unconscious patients
Visual Acuity & Fields:
Tubular vision Bitemporal hemianopia
Fundoscopy:
Normal Papilledema
Glasgow Coma Scale
Eye opening (E)
Motor response (M)
Verbal response (V)
ESpontaneous 4
To speech 3
To pain 2
Nil 1
MObeys 6
Localizes 5
Withdraws 4
Abnormal flexion 3
Extensor response 2
Nil 1
VOriented 5
Confused 4
Inappropriate words 3
Incomprehensible
sounds 2
Nil 1
Spontaneous = 4
Response to speech = 3
To pain = 2
Obeys = 6Localizes = 5 withdraws = 4
Abnormal flexion response = 3
Extensor response = 2
Open youreyes
Showme 2
fingers
Whatyear
is this?Yesterday
Mother
Scream,groan,moan
No response19722007
Nil (no response) = 1
Nil (no response) = 1
Confusedconversation = 4
Oriented = 5 Nil = 1
Extraocular movementsFacial sensationsPalatine movements
Corneal reflex Gag reflex
Tongue movementsFocal limb weaknessFocal sensory deficit
Babinski reflex
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Investigations:
Plain x-rays - Skull:
• Both AP & lateral views mandatory
• Detects distructive bony lesions viz. clival chordoma, chondrosarcoma & metastases
• Silver (copper) beaten appearance – chronic raised ICP
• Hyperostosis can be detected in the bone overlying the meningioma
CT scan:
Indications • Focal Neurodeficit • Altered Sensorium • Clinical Deterioration • GCS <13
• Detects mass lesion, associated brain shift, herniations and cerebral edema • Hydrocephalus • Hyperostosis • Calcified tumor • Contrast enhancement • Contrast cisternography- specialized study for delineation of an arachnoid cyst
MRI:
Indications • Inconclusive CT Scan • Intrinsic (Parenchymal) Brain Tumors • Posterior Fossa Mass Lesions • Suspected Spinal Metastases
• Better anatomical demonstration of tumor • Proven for posterior fossa lesions • Lesions smaller than 1 cm can be demonsrtrated • MR venogram- detects sinus infiltration by tumor • MR Angiogram shows vascularity of tumor
Neurocutaneous Markers:
Von Recklinghausen DiseaseAlso known as NF-1 / Neurofibromatosis
Associated other tumors such as Gliomas, Meningiomas etc.
Cafe-au-lait spots Diffuse Cutaneous Neurofibromatosis
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MR Spectroscopy:
• Helps in differentiating various mass lesions through biochemical analysis of selected tissue • Differentiates: Primary brain tumors from metastases Benign from malignant primary lesions Recurrence from radiation necrosis
Tumor Spectrum High grade Glioma Low grade Glioma
Diffuse Tensor Imaging & MR Tractography:
• Recent advancement in MR diffusion studies
• Three different varieties of tracts running in three different planes viz vertical, horizontal (sagittal), (coronal) and transverse are coded with three different colors Blue, Red and Green
• Involvement of these tracts by tumor can be demonstrated
Contrast T1 DTI T W2 DTI
MR Tractography
Image Guidance:
Pre operative feeding of data to image guiding machine can help Neurosurgeon to plan his trajectory for accurate and complete excision of tumor and preserving critical areas of the brain
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Amygdaloid nucleus
Arcuate libers
Uncinate fasciculus
Inferior occipitofrontal fasciculus
Superficial part of arcuate
Superior longitudinal fasciculus (arcuate)
Arcuate fibers
Superior occipitofronatal fasciculus
Corpus callosum
Cingulum
Longitudinal striaeMedial
Lateral
Arcuate fibers
Anterior limb ofinternal capsule
Caudate nucleus
Lentiform nucleus
Claustrum
Anteriorcommissure
sulcus centralis
commissural bundles
association bundles
projection bundles
Positron Emission Tomography (PET):
• Helps in detecting recurrence
• Conducted by administering 18 FDG or methionine which is selectively concentrated in highly metabolically active regions
Intraoperative MRI:
Ideal investigation for
Neurosurgeons to indicate the
extent of tumor excision while one
still operates on patient!
Pure Tone Audiometry
Electrophysiological studies:
Electroencephalography (EEG): Localization of seizure focus
Visual Evoked Potentials (VEP),
Brain Stem Evoked Potentials (BEP)
Classification of Nervous System Tumors
Neurosurgical Operation Suite
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Meningiomas:• Slow growing, extra-axial, benign tumors arising from arachnoid cap cells• Usually cured if completely removed• Complete excision includes tumor and 2cm of surrounding dura • Commonly located along falx, convexity, and sphenoid bone• Cause hyperostosis, erosion and even infiltration of adjacent bone• Psammoma bodies are hallmark• Rarely intraventricular : even more rarely it may metastasize
Plain X rays:
• Limited value
• Shows hyperostotic overlying bone & even calcified meningiomas
• Enlargement of vascular groove on the ipsilateral skull vault
CT Scan:
Plain : Homogenous, isodense extraxial Space occupying lesion (SOL)
Contrast : Brilliantly enhancing … lits like a bulb!
Plain Contrast
Bone Window 3D Reconstruction
Grade Meningioma
G I Meningothelial, Fibrous, Mixed, Angiomatous, Psammomatous, Myomatous, Microcystic, Lipomatous
G II Atypical, Chordoid, Clear cell
G III Rhabdoid, Anaplastic, Papillary
G IV Meningeal Sarcoma
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Meningioma Tumorattached to dura Dura
Midlineshift to rightside withsubfalcineherniation
Large tumourextending intothe brain, butis still sharplydemarcatedfrom the brainparenchyma
MRI : T W - Isointense Extra-axial lesions1
T W - Hypointense owing to hypercellularity, Vasogenic edema2
Contrast - Brilliant enhancement, Dural tail
MRA & MRV : Demonstrates vascularity and venous drainage
Conventional Angiography: Helpful in skull base meningiomas where
detection of feeders and their subsequent embolisation helps reduces tumor
vascularity saves time, blood loss and surgical stress during removal
Treatment:
Microsurgical excision :
• Direct excision of tumor • Relieves mass effect • Provides tissue for biopsy and molecular biological studies • Results can be devastating in inexperienced hands for any tumor especially those arising from Cerebellopontine (CP) Angle, Petroclival and Sphenoid wing
Radiotherapy/ Radiosurgery :
• Reserved only for recurrent tumors of high grade variety • Residues in critical locations like cavernous sinus, CP Angle etc.
EXPRESS NEWSLINEWEDNESDAY 8 AUGUST, 1996
TITANIC TUMORDr. Keki Turel Neurosurgeon, removed a massive 500 gm growth from a teenager's brain.
The opening andclosing of theskull took almosteight hours andthe actualoperation on thetumor anotherfour
Parasagittal Falcotentorial Sphenoid wing Convexity Foramen magnmum
THE DAILY Friday July 8, 1983
The world's largest brain tumor
weighing 550 gms
Mohamed Ali, after the operation
WORLD'S LARGEST
BRAIN TUMOR
REMOVED
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Acoustic Neuroma(AN) : (Vestibular Schwannoma)
• Though benign, it seemed a terrible and dreaded tumor once upon a time
• It was attributed to its location in the critical area….. CP Angle!
• Arises from Neurilemmal sheath, usually of the superior division of the vestibular nerve
• The epicentre is Obersteiner-Redlich Zone, a junction of central & peripheral myelin of vestibular nerve, located 8 – 12 mm distal to brain stem
• Bilateral AN in NF2
Symptoms:
Three most common symptoms:
• Hearing loss for high frequency (later for all) • Tinnitus • Dysequilibrium
Other less frequent symptoms (often with larger tumors): Headache, Facial numbness, Facial weakness, Diplopia, Nausea, Vomiting, & Altered taste sensations
Signs: Abnormal corneal reflex; Facial hypoesthesia, Facial paresis, Abnormal eye movements, Nystagmus, Babinski Sign, & Papilledema
Surgical treatment :
Treatment of choice
Though tumor removal is the goal, preservation of vital structures (Cranial Nerves, Vessels and Brain Stem) is paramount and hence tumor removal seems by product!
Post Op: CTPre Op: MR Pre Op: CT
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NF - 2
Pituitary adenomas :Arises primarily from anterior pituitary glandThe function of the anterior pituitary is to secret the various hormones
Classification:
Dependcing on size:
Macroadenoma 10 mm to 25 mmMicroadenoma < 10 mm Giant adenoma > 25 mm
Depend on Secretion of hormones:
Non secretory : Chromophobe tumors
• Constitute 10% of Intracranial tumors
Development of Pituitary glandFunctions of Pituitary gland
Secretory Islet Hormone Clinical Presentation
Acidophil Somatotroph Growth Hormone (GH) Gigantism / Acromegaly
Lactotroph Prolactin Amenorrhea, Galactorrhea
Thyrotroph TSH Secondary thyrotoxicosis
Basophil Corticotroph ACTH Cushing’s disease
Gonadotroph FSH/LH Adiposogenital Syndrome
Hypothalamus
anterior pituitary posterior pituitary
Infundibulum of brain
Roof of oral cavity
Third ventricle
Intermediated lobe
Central cavity
B
A
Residual lumen
Craniopharyngeal duct'Rathke's Pouch'
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Neurosecretory cells producereleasing and release-inhibitinghormones
These hormones aresecreted into a portal system
Each type of hypothalamichormone either stimulates orinhibits production and secretionof an anterior pituitary hormone
The anterior pituitary secretesits hormones into the bloodstream
Pituitaryportal system
Ovaries, Testes
aconadotropins(FSH & LH)
growthhormone (GH)
Bones, Tissues Mammaryclands
prolactin (PRL)
Adrenal cortex
adorenocorticotropin (ACTH)
Thyroid
thyroidstimulating
hormone (TSH)
Mammaryclands
oxytooin
oxytooin
Smooth musclein uterus
Kidney tubules
antiduretichormone (ADH)
When appropriate, ADH andoxytocin are secreted fromaxon endings into the blood stream
These hormones movedown axons to axon endines
Neurosecretorycells produceADH and oxytocin
• Prevalent in 3rd / 4th decades, equally affects both sexes
• Multiple Endocrine Neoplasia - 1 (Wermer syndrome)- congenital
disorder comprising pituitary, parathyroid, & pancreatic tumors
Clinical presentation:
Mass effect :
• Usually seen with nonsecretory adenomas
• Prolactinoma is probably the only secretory tumor that can grow to produce mass effect on:
Optic Chiasm : Bitemporal hemianopia (Noncongruous)
Pituitary gland : Varying degree of Hypopituitarism
• Hypothyroidism : cold intolerance, myxedema, coarse hair
• Hypoadrenalism : Orthostatic hypotension, easy fatiguability
• Hypogonadism : Amenorrhea (women) Loss of libido, infertility (men)
• Diabetes insipidus : Very rare, polyuria, polydipsia
• Hyperprolactinemia:
Cavernous sinus:
• Pressure on Cranial Nerves (Cns) : III (ptosis), V1, V2, (Facial pain, Keratitis, and Corneal ulceration), IV and VI (Diplopia)
• Occlusion of sinus : Proptosis, Chemosis
• Carotid encasement : Causes slight narrowing but virtually no occlusion
Functional tumors:
Prolactinomas : Most common secretory tumor
• Amenorrhea - Galactorrhea syndrome in females
• Impotence in males and infertility in both
Cushing's disease : Elevated ACTH
Ecchymosis Stria
Chiasmal Compression
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• F: M = 9:1
• Weight gain, centripetal fat distribution (Moon face, Buffalo hump), Supraclavicular pad of fat, dewlap tumor (episternal fat)
• Slender extremities
• Hypertension
• Ecchymoses, Purplish stria on flanks, breasts, and abdomen
• Hyperglycemia : Secondary Diabetes mellitus
• Atrophic, shiny, thin, and bruisable skin which shows poor wound healing
• Osteoporosis, muscle wasting
• Hirsutism, acne : Increase other hormonal secretions
• Depression, emotional liability, and dementia
Gigantism: • High GH secretion before epiphyseal fusion
• Tall person, long extremities
Acromegaly: • Over secretion of GH after age of epiphyseal fusion
• Skeletal overgrowth
• Increase hand, foot size, Prognathism (protruded mandible) spaced out teeth
• Thickened heelpad
• Frontal bossing
• Cardiomyopathy
• Macroglossia, High BP, Nerve entrapment syndrome (e.g. carpal tunnel syndrome) & oily skin
• Thick ear lobules, hypertrophied nasal tip
Acromegalic Big toes Thick & Coarse fingers
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Giant
Investigations:
Endocrinologic evaluation -
• GH(0-5 IU), Cortisol(4-7)(11-15), T (84-200ng/dl), T (5-14 g/dl), TSH(0.27-4.2 IU/ml), 3 4 M M
Prolactin(Male : 4.7-21.4ng/ml, Female : 6-30ng/ml), Estrogen(4.3-7.6 pg/ml), Testosterone(2.8-8ng/ml),
FSH(1.5-12.4mIU/ml), LH(1.7-8.6mIU/ml)
• Glucose tolerance test
• Specialized tests :
a. Low / high dose dexamethasone test
b. Cosyntropin stimulation test
c. Water deprivation test
Radiological investigations:
• Plain x-rays - shows Acromegaly
• Pituitary fossa enlargement,
silver / copper beaten appearance
CT Scan : Pituitary fossa enlargement,
Isodense tumor,
Enhancing brilliantly (macro) : less enhancing (micro)
MRI : T W- Hypointense, T W- Hyperintense1 2
Brilliantly contrast enhancing
Treatment:
Medical : Sr. Prolactin > 500, No mass effect
Prolactinomas: Dopamine agonists
Start 1.25mg / day HS, Bromocriptine: Doses can be raised upto 15mg / day
Microadenoma : Doses can be increased 3-4 wkly
Macroadenoma : Doses need to be increased every 3-4 day
Side effects : Nausea, Headache, Fatigue, Nightmare, and Orthostatic Hypotension
Long acting ergot alkaloid Pergolide: Yet not approved by FDA Dose - 0.05 mg HS
Selective D2 agonist Cabergoline: Long half life 60 - 100 hrs Hence couple of doses a week are usually sufficient Dose - 0.25 mg twice wkly Side effects: Headache, GI symptoms
Plain X-rays
MR: Giant pituitary tumor
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Acromegaly:
Bromocriptine Somatostatin analogue, Octreotide: 45 times more potent than Somatostatin Dose - 50 to 100 μg S.C. 8 hrly; Maximum upto 1500 μg / day Side effects : Diarrhoea, Steatorrhea, Flatulance, Nausea, and Abdominal discomfort
Cushing's disease:
Blocks adrenal steroid synthesis Ketoconazole: Dose - 200mg BD; Maximum upto1600mg / day Side effects : GI disturbances, Edema, Skin rash, and Hepototoxicity
Inhibits steroid synthesisAminoglutethimide: Dose - 125 to 250 mg BD maximum 1000 mg/ day Side effects : Sedation, Anorexia, Nausea, and Rash
Inhibits 11, 13 hydroxylase Metyrapone: Dose can vary from 750-6000mg / day Side effects : Hirsuitism, Nausea, Vomiting and Diarrhoea
Inhibits steroid synthesis Mitotane : Dose - 250 to 500mg HS, maximum upto 12 gm / day Side effects : Anorexia, Nausea, Lethargy, Dizziness, and Hypercholesterolemia, Surgical : Tumor removal is goal; preservation of pituitary gland and hormones is vital
In spite of best efforts, residual tumor may be left behind and this will need careful decision making
Options: Observation, Reoperation, Radiotherapy / Radiosurgery
Prolactinomas: • PRL level < 500mg/ml • PRL level >500, not controlled medically Primary Cushing's disease Acromegaly: Macroadenomas: • Prolactinoma - no acute regression • Non-Prolactin secreting- causing mass effect • Pituitary apoplexy
Surgical procedures:
Transsphenoidal Endomicroscopic excision: Gold standard, Minimal invasive technique No external scar, No brain retraction, Extra arachnoid approach Practically no complications, Short hospital stay (2-3days) if no complication occurs
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Transcranial Frontobasal / Pterional: Only for giant pituitary tumor with parasellar extension
Pituitary Apoplexy:
• Abrupt neurological deterioration • Headache, Visual disturbances, and impaired mental status • Usually sudden infarction, hemorrhage or necrosis of pituitary adenoma • Ophthalmoplegia, SAH, and assess hydrocephalus • Rapid decompression will improve neurological status • Hormonal supplementation can combat shock • Hydrocephalus would need shunting
Craniopharyngioma:
Controversy has surrounded craniopharyngiomas as if it has almost no other tumor affecting the brain!
• Tends to arise from anterior, superior aspect of pituitary fossa
• Constitutes 2 to 4% of all brain tumors; 50% occur in childhood
• Lined by stratified squamous epithelium
• All have solid & cystic components, and calcification to varied extent
• Though physical proportion of fluid in the cyst varies it contains cholesterol crystal & known as “Machine oil cyst”
• Calcification : Microscopic – 50% X-rays : 85% in childhood; 40% in adults
• Children usually present with Headache, Visual loss, Nausea, Vomiting, Diplopia, Mentation problem, & Endocrinal disturbance
• Adults present with Visual loss, Diplopia, Headache, & Vomiting
Investigations:
• Full endocrinologic evaluation
• CT scan: Shows hypodense lesion with calcification of the capsule, situated in peri third ventricular & around pituitary stalk in suprasellar cistern which shows ring enhancement
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Pituitary Apoplexy: CT & MR showing hemorrhage inside the pituitary gland
CT scan Contrast enhanced MRI
• MRI: Detailed information about extension of tumor in the crevices of suprasellar cistern
• But poor in detection of calcification
• Adhesion of capsule to vessels, nerves & pituitary stalk
Treatment:
Surgical: Craniotomy and total excision of lesion is Gold standard!
• One must be careful in preventing damage to pituitary stalk, hypothalamus, vessels in suprasellar cistern, & optic chiasm
Few patients with a large cyst may need emergency trans-sphenoidal decompression, and instillation of Bleomycin with an intent to retard tumor growth
Recurrence:
• Crucial problem faced by Neurosurgeons • Needs re-operation or radiotherapy (RT) • Role of RT is still controversial
Morbidity & mortality increases due to firm & dense adhesion with surrounding vital structures
Epidermoid & Dermoid :
• Developmental defects, known as pearly tumor / cholesteatoma
Sign & Symptoms of Hypopituitarism
Feature Epidermoid Dermoid
Frequency 0.5 to 1.5% 0.3%
Lining Stratified squamous Also includes hair follicles & epithelium sebaceous glands
Location Tend to be lateral Strictly midline
Anomalies Not exist 50% cases show some anomalies in the body
Contents Keratin, Cell debris, Same as epidermoid, additionally, hair & sebum & Cholesterol
Meningitis Aseptic, brief Recurrent, bacterial
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Decreased tone, poorbalance (does not run)
Constipation
Increased urination
Increased thirstand fluid intake
Visual impairment:blindness and crossedeyes (strabismus)
Thin, dry hair
Reducedgrowth
Sleeps 14-15hours per day
Height ofCraniopharyngiomaChild
Average height ofhealthy child
Investigations:
• CT scan: hypodense, nonenhancing lesion
• MRI:
T W- Hypointense1 T W- Hyperintense2 Diffusion- Hyperintense (Vital, as it differentiates it
from arachnoid cyst)
Treatment:
Microsurgical excision is the gold standard Endoscope facilitates in reaching deep areas of the skull base without brain retraction
Ultimate aim is total excision and preservation of vital structures
Though slow growing, recurrence is inevitable if not removed completely
Hemangioblastoma:
• Occurs exclusively in posterior fossa
• Comprise 1- 2.5% of intracranial tumors
• 85% occurs in cerebellum
• Sporadic : 4th decade, 3rd decade if associated with Von Hippel Lindau(VHL) disease
• Three types : Juvenile, Transitional and Clear cell
• Presentation: Symptoms of hydrocephalus and raised ICP
• Fundoscopy identifies retinal component
Retinal Angioma
Investigations:
• CT : Isodense (solid/solid-cum-cystic mass) & intense enhancement on contrast Cystic lesion with mural nodule is classical but cysts are small & may even be multiple
• MRI : Preferred, as this tumor is usually found in posterior fossa, and also identifies lesions in the spinal cord
• DSA : Spinal angiogram is helpful to rule out any other focus
• Blood investigations : polycythemia
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MR : enhancing Hemangioblastoma
Treatment:
Surgical treatment: Curative for sporadic variety
Cyst: Mural nodule removal is essential, else recurrence is inevitable
Solid tumors: Difficult to remove owing to vascularity
Enbloc removal, and never piecemeal
Multiple lesions: treat all lesions > 1 cm sizes: observe smaller ones
Radiation: May be useful in reducing tumor size & Vascularity, but generally not recommended
Glomus Jugulare: Known as chemodectoma / paragangliomas
Carotid & Glomus bodies Otoscopic view Chemodectoma
• Rarer tumors
• Arise from Glomus bodies in the area of jugular bulbs
• Usually tracks along vessels & have finger like extensions inside the jugular vein, which may embolise during tumor manipulation
• Highly vascular tumor as it is the only tissue in the body which enjoys higher blood flow than normal brain
• F:M = 6:1
• Symptoms: Hearing loss, Pulsatile tinnitus, Dizziness, & Earache
Signs: • Conductive (ear canal obstruction) or Sensorineural deafness (labyrinthine involvement)
• IX, X, XI & XII CN involvement
• VII CN paresis
• Otoscopy may show pulsatile, bluish red mass behind ear drum
• May secrete catecholamines: VMA / Metanephrines in urine
• Carcinoidlike symptoms: Bronchospasm, abdominal pain, cutaneous flushing, explosive diarrhoea, headache, and high BP
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Superior thyroid artery
Artery to carotid body
Carotid body
External carotid artery
Veins from carotid body
Carotid sinus nerve
Glassopharyngeal (IX) nerve
Vagus (X) nerve
Superior cervicalsympathetic trunk ganglion
Retromandibular andfacial veins
Lingual vein
Internal jugular vein
Internal carotid artery
Carotid sinus
CT : Assesses bony involvement (Temporal bone destruction)
MRI- Better soft tissue delineation, tumor detection & even its extensions
Angiogram- Patency of jugular vein and tumor blush
Axial Coronal
Axial Sagittal
Treatment:
• Preoperative embolisation facilitates complete surgical excision • Surgical excision: Ideal treatment • Requires team work (Neurosurgery & ENT) Approach : Suboccipital Translabyrinthine which needs facial nerve transposition • Radiotherapy: Is a poor second choice as its role is still controversial
Choroid Plexus tumors: • 1% of intracranial tumors • 70% of patients <2 years • In adults: Infratentorial (IV ventricular) • In children: Supratentorial (Lateral ventricular) • Varieties: Benign- Choroid plexus papilloma Malignant - Choroid plexus carcinoma (less common) • Symptoms: Raised ICP & Hydrocephalus
Type Description Intracranial extension
I Small within jugular bulb, middle ear & mastoid None
II Extends under IAC Possible
III Extends to Petrous apex Possible
IV Extends beyond Petrous apex into clivus or Possible infratemporal fossa
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Focal deficit: Hemiparesis, Sensory deficit, Cerebellar signs, and Cranial nerve palsies
Hydrocephalus:
CT Scan : Hypodense, brilliantly enhancing SOL
MRI : Contrast enhancing ventricular lesion
T W2 Contrast Angiography : Tumor blush
Treatment: Microsurgical total excision
GLIOMAS :
Possesses histologic characteristics of neuroglial tissue
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Juvenile Pilocytic:
• Young patients of second or third decade are affected
• A typical cystic lesion with enhancing mural nodule
• Common sites: Cerebellar hemispheres, followed by optic apparatus
• Prognosis is excellent if excised completely….. and is the only practically curable glioma!
CT Scan :
Hypodense mass in cerebellar hemisphere, & enhancing mural nodule
MRI : T W - Hypointense lesion & Isointense nodule1
T W - Hyperintense lesion & Isointense nodule2
Gadolinium - cyst wall shows no enhancement while mural nodule lits up!
Plain Contrast
Pre Op MR Post Op MR
Low grade gliomas (LGG): Glioma of Grade I & II (WHO)
Astrocytoma Oligodendroglioma Mixed Ependymoma Astrocytoma
Juvenile Pilocytic G II GII Subependymoma
Diffuse Fibrillary GII Myxopapillary
Protoplasmic
Pleomorphic Xanthoastrocytoma
Subependymal Giant cell
Gemistocytic
Dysplastic neuroectodermal(DNET)
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Brain Stem Glioma:
Pre Op MR Post Op MR
Treatment:
• Surgical excision is the best, time tested remedy
• No role of adjuvant treatments like Radiation & Chemotherapy (even for a small residue)
Diffuse Fibrillary GII:
• Most common variety of LGG
• Tumor cells synthesize fine & coarse neuroglial filbrils which occupy the matrix, hence tumor feels firm & rubbery
• They have shown dedifferentiation over period of time and convert into secondary Glioblastoma multiforme(GBM)
• A cure for this disease is impossible as periphery of tumor merges with normal brain parenchyma which is difficult to identify intraoperatively
CT Scan: Hypodense diffuse mass which may enhance sparingly
Plain Contrast
MRI: Post Gadolinium : Minimal or no enhancement
T W1Pre Op MRT W2
Post Op MR
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Treatment :
Conservative: Debatable, yet some clinicians do follow the protocol
Surgical: Ideal, Removes mass effect, and provides tissue for biopsy, which in turn helps in deciding further treatment
Chemotherapy: Temozolomide 150mg/m2/day for five days a month such 6 cycle course is found effective
Radiotherapy: Whole Brain Radiotherapy (WBRT)
Ganglioglioma :
• Rare childhood tumor
• Can affect cerebral hemispheres, spinal cord, brain stem, cerebellum, pineal region, thalamus, optic nerve and even peripheral nerves
• Two sub types
i) Ganglioneuroma: Rarer, benign, carries predominant neural component
ii) Ganglioglioma: Preponderance of glial cells
• Clinical features : Seizures or change in a preexisting seizure pattern or just medically uncontrolled epilepsy
Investigations:
• Plain x-rays: Show calcification
CT: Hypodense calcified lesions, moderate enhancement
MRI: High signal on T1 W; low signal on T2 W
• Calcification appears hypointense in both
Carries good prognosis unless astroglial component shows dedifferentiation
High Grade Gliomas (HGG)
• Most common primary brain tumor in adults, Comprises 50%
• M:F = 1.5:1
Intra Op
Grade Tumor
G III Anaplastic astrocytoma
Anaplastic oligodendroglioma
Mixed oligoastrocytoma
G IV Glioblastoma multiforme (GBM)
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Anaplastic Astrocytoma:
• Mean age of presentation is 5th decade
• Prognostically favourable than GBM
• Can dedifferentiate into GBM
GBM:
• Mean age is 6th decade
• Most common and most malignant primary brain tumor
• May be Primary or de novo Secondary: develops by dedifferentiation from LGG
• Central necrosis, neovascularization, and endothelial proliferation are unique
• Cystic lesion contains xanthochromic fluid
CT Scan: Hypodense lesion and perilesional edema Ring enhancement (G-IV) or diffuse inhomogeneous enhancement (G III)
MRI: Thin Crenated, ring enhancement Marked perilesional edema extending along fibre tracts
MR Spectroscopy : NAA, Choline:creatine ratio > 1.7, Lipid / lactate
Pre Op : GBM Post Op
Butterfly Glioma: Spreads across corpus callosum and hence involves both hemispheres
Grade Mean survival
I 8-10yrs
II 7-8yrs
III ~2yrs
IV <1 yr
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Treatment :
Surgery : Cytoreduction, mass effect, & provides tissue for Histopathology
Radiation: WBRT- 3000 rads
Chemotherapy : 2Temozolomide – 150mg / m /d PO x 5 days
For all Glima, aim is maximum cytoreductionwithout harming the patients
Total excision is far better than partial removal as it result in significant increase of edema and neurological deficit, making them unsuitable even for adjuvant therapy
Radical excision as resulted in significantly longer survivals, resumption of normal activities and good quality of life (QOL)
Oligodendroglioma :
• Presents frequently with seizures, carries much better prognosis
• Prevalent in frontal lobes (50%), followed by other lobes ( 40%)
• Histologic features : Fried egg cytoplasm, Chickenwire vasculature, and Calcification
• Clinical presentation : Seizures, headache, mental status changes, vertigo / nausea
Low grade gliomas presenting with seizures as prime symptom carry excellent prognosis
They are found to be much slow growing and post operative residue may respond to chemo / radiotherapy
However terrifying the tumor may look on scans, good clinical status of patient usually indicates relative benignity of tumor
Pre Op MRS Functional MR (fMRI)indicating speech area
Post Op
General pessimismand lack of efforts and attitude
result in their non radical excision by the Neurosurgeon
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Ependymoma:
• Arises from ependymal cells lining ventricles and central canal of spinal cord
• Constitutes 5% of intracranial Gliomas
• 70% of them occur in children, in the 4th ventricle
• Potential to spread along CSF pathways (10%)
• Associated with NF- Type 1 & chromosome 17p losses
• Perivascular Pseudorossettes are hallmark
• Clinical symptoms of raised ICP and or hydrocephalus
Supratentorial Infratentorial
CT: Hypodense, brilliantly enhancing intraventricular lesion
MRI : Brilliantly enhancing, Calcifications may be seen Banana sign- arises from floor of IV th ventricle Spinal screening is mandatory (pre-op and on follow up)
Primitive Neuroectodermal tumor (PNET):
• Unique group of tumors which include
Medulloblastoma, Retinoblastoma, Pinealoblastoma, Neuroblastoma, esthensioneuroblastoma, ependymoblastomas and polar spongioblastoma
• Tend to disseminate via CSF spontaneously or iatrogenically
Grade Ependymoma
G-I Subependymoma , Myxopapillary
G-II Papillary ependymoma, Cellular, Clear cell
G-III Anaplastic
G-IV Ependymoblastoma
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Medulloblastoma:
• 15-20% of intracranial tumors in children
• Most common malignant pediatric brain tumor
• M:F=2:1
• Arises from cerebellar vermis, hence presents as obstructive hydrocephalus
Clinical presentation:
Headache, Nausea, Vomiting, Trucal & or appendicular ataxia
CT scan: Hyperdense lesion on plain & patchy enhancement on contrast
T W1
Contrast
MRI: T2W image is valuable shows hypointense lesion which enhances brilliantly on contrast
Plain
Contrast Spine screening
Treatment:Microsurgical excision by midline suboccipital craniotomy
Chemotherapy:
CCNU / Procarbazine / Vincristine (PCV therapy)
Reserved for recurrence, poor risk patients, & babies < 3yrs old
Radiotherapy: 30-40 Gy to tumor bed while 10-15 Gy to spine
CNS Lymphoma:
• May be primary originating in the brain without systemic lesions
• Secondary tumor develops in the later stages of systemic lymphoma
• Generally periventricular location
• Suspected if homogeneously enhancing lesion (s), found in conjunction with uveitis
• Highly responsive to steroids (initially)……….. Ghost tumors
• 60% are 'B' cell non-Hodgkin's type
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Primary CNS lymphoma:
• 2% of whole brain tumors
• Gradual rising incidence due to silent epidemic of Acquired Immunodeficiency Syndrome (AIDS) and increasing number of transplant patients
• M:F= 1.5:1
• Average Age: 4th & 5th decade
• Locations: Frontal lobes, deep nuclei, & periventricular region
Predisposing Factors:
Collagen vascular disease: Immunosuppression:
Systemic lupus erythematosus (SLE) Transplant Patient
Sjogren`s syndrome Congenital immunodeficiency
Rheumatoid arthritis (RA) AIDS
Epstein Barr Virus: Lymphoproliferative disorder
Symptoms:
• 50% patients had non focal, nonspecific symptoms
• Mental status change, headache, vomiting, nausea, & seizures
• Focal Symptoms: Hemi motor / hemi sensory symptoms Partial seizures
Signs:
• Papilledema
• Hemi motor / sensory deficits
• Encephalopathy
• Aphasia
• Dementia
• Visual field deficit
CT Scan:
• Homogenously enhancing lesion (s) in the central gray matter or corpus callosum
• Pseudomeningioma Pattern: enhancing lesion which shows surrounding ependymal and meningeal enhancement have lack of calcification & tend to be multiple
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MRI:
Large vesicular atypical nuclei,increased mitotic activity
MRI : Nodular enhancement
Evaluation: Basically for grading and differentiating it from secondary Lymphoma
• Lymph node biopsy if enlarged • Bone marrow aspiration biopsy • Testicular Ultrasonography • CT Scan – Whole Body
Treatment:
Surgery: Decompression is not mandatory, and biopsy for Histopathology is usually sufficient
Chemotherapy :
Non AIDS: Systemic chemotherapy: CHOP- Cyclophosphamide, Doxorubicin, Oncovin (Vincristin), & Prednisolone
CHOD- Cyclophosphamide, Doxorubicin, Oncovin, & Dexamethasone
AIDS patients: Need antiretroviral therapy in addition to local chemotherapy
Local: Intrathecal Methotrexate is found effective
Disappear even on steroid administration, hence known as “Ghost tumors”
Toxicity: Nausea, Vomiting, Myelosuppression, Leukoencephalopathy, Mucositis, Hepato & Nephrotoxicity
Radiotherapy: Gold standard WBRT - 40-50 Gy is effective enough
Pineal tumors:
• Area of brain bounded Dorsally – Corpus callosum, Tela choroidea
Ventrally- Quadrigeminal plate, Tectum
Rostrally – Posterior aspect of III ventricle
Caudally- Cerebellar vermis
• Common in children • Variety of tumors can evolve in this area
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Germ cell tumors (GCT):
• Occur in midline in CNS • Males: Pineal region • Females : Suprasellar cisterns • Apart from benign Teratomas, all GCTs are malignant • Metastasise via CSF& may even spread systemically
Types:
Germinomas: • Malignant tumor of primitive germ cells • Testicular Seminomas • Dysgerminomas in ovary • Survival better with Germinomas
Non-germinomas • Embryonal carcinomas – AFP • Choriocarcinoma- βHCG • Teratoma – Rarely secretes
Clinical: • Symptoms of Hydrocephalus • Perinaud's syndrome owing to compression of tectal plate by developing tumorCTScan: Hypodense/ isodense lesion which enhances on contrast
Treatment: Hydrocephalus- Shunt / Extra ventricular drain (EVD) Microsurgical excision:- Infratentorial supracerebellar approach Suboccipital trans tentorial approach
Radiotherapy: Effective in treating these lesions
Suprasellar Germinoma
Contrast MR
Pineal Tumor
Tissue Tumor
Pineal gland Pineocytomas / Pineoblastomas
Glial cells Astrocytomas , Glial cysts, Oligodendrogliomas
Ependyma Ependymomas
Sympathetic nerves Chemodectomas
Germ cell rests Germ cell tumors
No Blood Brain Barrier Metastases
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Chordoma:
• Locally malignant tumor involves extremes of spine i.e. Clivus (35%) & Sacrococcygeal region (53%)
• Slow growing & radioresistant lesions
• Contain Physaliphorous cells which are rich in intracellular mucin
• Midline growth but can extend laterally to one or both sides
• To differentiate from Chondrosarcoma (laterally growing)
• Surgical treatment – en-bloc resection
Though histologically benign, they pursue a malignant course due to critical situation, difficulty of total removal, high recurrence rate and may even metastasise rarely
Cranial Chordoma: Any age, M:F = 1:1
Usual presentation is cranial nerve paresis
III : VI CNs are usually affected
CT Scan: Shows destructive, heterogenous enhancing clival lesion, which may infiltrate infratemporal, pterygopalatine fossa and even retro & parapharyngeal spaces
MRI: In addition to SOL, it demonstrates detail anatomical planes, dural involvement and intra dural extension
Treatment:
• Surgical en-bloc excision of tumor is the best treatment
• But total excision is practically impossible for fear of cranial nerve palsies & dural breach
• Usually needs multiple surgeries through various approaches
Radiation:
• Though response is guarded, high dose XRT (45 to 50 Gy) can be offered to clival region
• Cyber knife proclaims batter results than other forms of RT
• Major differential diagnosis is Chondro Sarcomg
• Eccentric / Paramedian situation differentiates them from central chordomas
CT scan: Chordoma
MR: Chordoma
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Colloid cyst:
• Known as Neuroepithelial cyst
• 1% of all intracranial tumors
• Age: 20-50 years
• Arise from diencehalic recess of the postvelar arch
• Comprises a fibrous epithelial lined wall filled with either mucoid or dense hyloid substance
• Classically occur in the 3rd ventricle, blocking foramina of Monro leading to obstructive hydrocephalus
Symptoms: Chronic headache, disturbed mentation, blurred vision, diplopia, nausea, vomiting, often Endocrine disturbances & rarely asymptomatic incidental detection
• But carries risk of sudden death due to central or tonsillar herniation
CT scan: Hypodense lesion showing patchy enhancement
MRI
Treatment: Surgical excision
• Microsurgical transcallosal excision • Endscopic drainage of cyst & excision • Streotactic aspiration of cyst – limited role due to thick viscous contents
Arachnoid cyst:
• Also known as Leptomeningeal cyst • Congenital lesion arises out of splitting of arachnoid membrane • CSF is secreted between two layers and is communicated with subarachnoid space by a small opening • Presentation : Early childhood • Sudden deterioration of clinical status is due to Bleeding / Rupture
Colloid cyst
Incidentally detected in head injury patient
Middle fossa cyst Suprasellar cyst causing Diffuse supra/ or hydrocephalus infratentorial cyst
Seizures Raised ICP Raised ICP
Headache Craniomegaly Craniomegaly
Hemiparesis Developmental delay, visual loss, Developmental delay precocious puberty, boble head doll syndrome
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Investigations:
CT Scan : Hypodense nonenhancing lesion, Expansion of overlying bone
Sylvian fissure cyst:
• Suprasellar, quadrigeminal plate- III ventricular compression (Perinaud syndrome)
• Posterior fossa midline cyst- IV ventricular compression
MRI: No contrast enhancement, Diffusion (DWI) : hyperintense
CT Cisternogram: Done after intrathecal injection of water soluble iodinated contrast or radiotracer
• If cyst is communicating, will take up contrast / radiotracer
Treatment: No treatment is needed if there is no mass effect
Surgical treatment:
• Simple drainage • Cystoperitonial shunt • Craniotomy and excision of cyst wall • Endoscopic fenestration
Supratentorial Arachnoid cyst: causing hydrocephalus
Infratentorial Arachnoid cyst: thinning of overlying bone
Type I Type II Type III
Small biconvex, Proximal and intermediate Involves entire Sylvian located at anterior temporal tip, segment of Sylvian fissure, fissure, marked midline no mass effect, completely open Insula shift, minimal communicates with gives rectangular shape, communication with subarachnoid space partial communication subarachnoid space
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Rathke's cleft cyst:
• Non neoplastic lesions
• Arise from remnants of Rathke's pouch
• Primarily intrasellar but may have suprasellar extension
• Presentation may range from mere incidental detection to visual disturbances or hypopituitarism
CT scan: Nonenhancing sellar / suprasellar lesion
MRI:
Treatment:
Incidentally detected cyst may just need regular monitoring and follow-up Surgical excision if neurological deterioration / endocrine disturbances occur
Metastatic Brain Tumors:
• Neoplasms that originate in tissues outside the brain and spread secondarily to involve the brain • Most common brain tumors, even overwhelming the primary brain tumors ! • They carry poor prognosis • 40% of cancer patients develop brain metastases over progress of disease • Incidence of metastases is rising, owing to longer survival of patient as a result of improved medical attention and exellent radioimaging • Primary tumor sources for metastases:
Adult Young / Child
Lung (60%) Osteogenic sarcoma
Breast (14%) Rhabdomyosarcoma
Kidney (2%) Ewing’s Sarcoma
Melanoma (4%)
GIT & other miscellaneous tumors (20%) Germ Cell Tumors
T W2 Contrast
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Mechanism of Spread :
• Most tumor cells reach brain by Hematogenous spread via arterial circulation
• Metastasis originates in the lung either from primary or from a metastasis to the lung
• Metastasis from prostatic malignancies via Batson's vertebral venous plexus
• Commonest site of seedling is grey white junction (due to sudden change in the caliber of vessels)
• Distribution of metastases roughly follows the relative weight of and blood flow to each area: Cerebrum (80%), Cerebellum (15%) & Brain stem (5%)
• 2/3rd to 3/4th of patients show multiple lesions
• Single brain metastasis: Single Brain Lesion Solitary brain metastasis: Single Brain Lesion that is the only site of metastasis
• Single : Colon, Breast, and Renal Cell Carcinoma (RCC) Multiple : Melanoma, Lung Cancer
Carcinoma Lung
Carcinoma Breast
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Treatment:
Surgical excision: Craniotomy & Maximum possible excision
Radiation: Rather sensitive, 45 to 48 Gy to tumor bed Spinal radiotherapy if associated Spinal Metastases
Radiosurgery: Only for Solitary Brain Metastasis
Chemotherapy: Little impact
Malignant Melanoma
RCC
Scalp & Skull Tumors:
Benign: Osteoma, Hemangioma, Dermoid and Epidermoid tumor, Chondroma & Aneurysmal bone cyst
Melignant : Metastases, Chondrosarcoma, Osteogenic sarcoma and Fibrosarcoma
Dermatofibrosarcoma Bony Tumor
Treatment:
Aim should be total Microsurgical excision Residual tumor needs radiotherapy or chemotherapy
Squamous cell carcinomaof scalp in a Syphilitic patient
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Modern Treatment
Surgical treatment: • Involves direct, complete excision of tumor which helps in cytoreduction & immediate elimination of mass effect • The best chance a Neurosurgeon can offer to the patients • Same is true for metastases and recurrences • Provides tumor tissue for Histopathological and molecular biological studies • Helps decide further plan of management and even prognosis of the patients
Chemotherapy: • Usually an adjuvant to Microsurgery, except in Lymphomas and GCTs where it can be utilized as primary modality
• Glidal wafers (Impregnated with CCNU) to be placed at tumor bed
Radiotherapy: • It has proved the test of time by improving survival of all brain tumors • Traditional method: Whole Brain Radiotherapy (WBRT) • Modern methods: Conformal (CRT), Stereotactic radiosurgery (SRS), Fractionated Stereotactic (SRT) or Stereotactic conformal (SCRT) • Conformal techniques selectively focusing on tumor, have minimised the doses of radiation to adjacent normal tissue
Immunotherapy: Monoclonal antibodies developed against the cell markers can be utilized to control tumor growth e.g. Anti EGFR, ANTI PDGFR drugs
Gene Therapy: Theoretical possibility of correction of these defects via retroviral vectors or plasmids should control these devastating conditions
Conclusions: • Improved Radio imaging has helped in early detection of Brain tumors
• Better Microsurgical techniques, Intra & postoperative image guidance and post-op ICU facilities have improved prognosis and survival
• Modern technology in delivering radiation has shown better control over tumor growth, without any significant impact on surrounding tissues
• Recently synthesized chemotherapeutic medications have shown promising results, with minimal or no side effects
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The technologic drive experienced in the field of medicine during the last few years has propelled neurosciences to the forefront of medical practice. Progress in neurosciences itself has been propelled by simultaneous advances in neuroradiology, neuroanaesthesia and intensive care management. The development of neurosurgery beginning with micro surgical techniques 3 decades ago and the use of computers, image guidance, stereotaxy, endoscopy and endovascular interventions have made neurosurgery minimally invasive and maximally safe and productive. The current scenario is full of excitement and future can only be mind boggling.
RSVP :Tel.: +91 22 2203 4104 � Hand Phone : +91 982 00 41039
email : kekiturel@rediffmail.com / neuturel@vsnl.com / ket@bom4.vsnl.net.inweb : www.drkekiturel.com
Clinic : 105, 1st Floor, New Wing, Bombay Hospital, 12 - Marine Lines, Mumbai - 400 020. INDIA
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- Dr. Keki E. TurelProfessor & Head,
Dept. of Neurosurgery,Bombay Hospital, Mumbai, INDIA