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?

2

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

3

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

4

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

5

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

6

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

7

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

8

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

9

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

10

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

11

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'

12

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

13

• 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

14

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

15

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

16

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

17

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

18

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

19

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

20

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

21

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

22

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)

23

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

24

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

(For distribution amongst medical practitioners only)

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- Dr. Keki E. TurelProfessor & Head,

Dept. of Neurosurgery,Bombay Hospital, Mumbai, INDIA