craniopharyngiomas and suprasellar tumors by dr. shikher shrestha (fcps), ninas, nepal
TRANSCRIPT
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Craniopharyngiomas and Suprasellar Tumors
Shikher Shrestha
NINAS
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ANATOMY QUICK RUN THROUGH
CRASH COURSE!!!
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Suprasellar Tumors
1. Tumors of dysembryogenic origin
Craniopharyngioma, Epidermoid, Dermoid, Hamartoma,
Germ Cell tumor, Rathke’s cleft cyst
2. Tumors originating from tissues of suprasellar structures
Gliomas of visual pathways and hypothalamus, pituicytoma, granular cell tumor of neurohypophysis, meningioma
3. Tumors extending into the suprasellar space secondarily
Pituitary adenoma, Rathke’s cleft cyst
4. Systemic tumors affecting the CNS
CNS metastasis, lymphoma, leukemia
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Most common - Craniopharyngioma
Chiasmatic/hypothalamic glioma
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Craniopharyngiomas
Benign, extra-axial epithelial tumors
Aggressive clinical course – significant morbidity and shortened life expectancy
2 intentions of Rx
to remove totally whenever technically possible
intentional incomplete removal and radiotherapy to lower surgical morbidity
radical removal – only in patients where hypothalamus NOT involved
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Incidence..
1-4.6% of all intracranial tumors
13% of suprasellar tumors
0.5 – 2.5 new cases per million population per year
Papillary type – exclusively in adults 40-55 yrs
Adamantinomatous – bimodal age distribution with peaks in children aged 5-15 yrs and adult aged 40-55 yrs
Children – 2.5-13% of all tumors and 56% of sellar-chiasmatictumors
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Tumor Development..
Erdheim Embryogenic Theory:
Origin – remnant of craniopharyngeal duct or Rathke’s pouch
Epithelial cell rests of the vanishing hypohyseal duct remain
adherent to the neuroepithelium before the pia is formed
Arise from the anterosuperior surface of the adenohypophysis
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Grekhov: 4 groups according to the point of original growth
infrasellar
intrasellar (intrasellar and suprasellar)
pituitary stalk
infundibular
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Metaplastic theory:
Metaplasia of mature adenohypophysis cells nests of
squamous epithelium
Theory explains:
adamantinomatous type to embryonic remnants vs
papillary type (adult) - metaplasia
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Pathology and Surgical Anatomy..
Microscopic Anatomy:
Adamantinomatous
complex epithelial lesion resembling enamel pulp of developing teeth
Pallisading peripheral layer of epithelium
Hydropic vacuolation of inner layer
Loose stellate reticular zone containing nodules of plump keratinocytes “wet keratin”
Dystrophic calcification
Areas of cholesterol deposits
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Microscopic anatomy contd..
Papillary:
simple squamous epithelium
villous fibrovascular stroma forming papillae
Both variants:
Brain parenchyma – gliotic with profuse Rosenthal fibers
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Macroscopic anatomy..
Solid tumors with a variable, sometimes cystic or multicystic
component
Adamantinomatous – cholesterol rich, machine oil like, thick
brownish yellow-green fluid, crumbly debris
Occasional calcification
Papillary type – lacks cystic component
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Surgical Anatomy..
Infradiaphragmatic tumor – grows below the sellar diaphragm
Supradiaphragmatic
Topography depends on
length of optic nerve
position of the chiasm
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Infradiaphragmatic..
Intrasellar
Upward displacement of sellar diaphragm and arachnoid
Grows below the chiasm, compresses and displaces upward as
well as the floor of the third ventricle
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Supradiaphragmatic..
Point of orignial growth in the stalk or inside the infundibulum
(most basal part of the floor of IIIrd ventricle)
Pituitary stalk suprasellar extraventricular
extends anteriorly to the chiasm b/t optic nerves
retrochiasmatic if prefixed chiasm
Infundibulum intraventricular and extraventricular (IEVC)
grows between the optic tracts
disrupts third ventricular floor at an early stage
partially inside and partially outside IIIrd ventricle
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In IEVC
most affected region of IIIrd ventricle – tuber (central) part
compresses mammillary bodies
infundibulum (median eminence) less frequently destroyed
commonly located exclusively behind the chiasm
small part of tumor may extend below the chiasm or b/t optic
nerves
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Exclusively Intraventricular..
rare subtype entirely within the ventricular cavity
attached to a partially atrophied floor of IIIrd ventricle
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Clinical Presentation..
Most common S&S –
Endocrine deficiencies (90%) in children
Visual disturbances (96%) in adults
Hypothalamic disorders – obesity – children
Mental disorders
Increased ICP
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Visual Problems:
defects In visual fields
bitemporal hemianopia, homonymous hemianopia, concentric contractions of fields, central or paracentralscotoma
decreased visual acuity
Compression not only by tumor but also by A1 or AcomA due to displacement of chiasm towards those arteries
Compression of lower chiasmatic arteries may also be the cause
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Concentric narrowing – due to optic atrophy from long standing
hypertension
Endocrinopathies:
Growth deceleration; Delayed puberty; Decreased sexual drive
90% men – impotence; most women – amenorrhea
Diabetes insipidus – presurgery – 8-35% cases
Hyperprolactinemia – 40%
Obesity – 25% - due to growth hormone insufficiency
Cognitive impairment, personality changes
Memory disturbances – lesion in mammillary body
(mammillary thalamic tract)
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Radiographic Diagnosis..
Infradiaphragmatic (intrasellar, infrasellar, suprasellar)
enlarge the sella similar to pituitary adenoma
Supradiaphramatic – displace diaphragm and pituitary downward
Calcifications – 85% childhood/ 40% adult types
Egg shell calcification of the wall
Cyst may appear solid if they contain sufficient quantity of suspended calcium salts
Contrast enhancement of solid component and cyst wall
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MRI – heterogenous mass
solid part – isointense on T1 + contrast enhancement
Hyperintense ring enhancement of cyst wall
Hyperintense cyst cavity on T1 if high protein content
Relation to sella and diaphragm – best in coronal view
AcomA – mostly in upper posterior surface of chiasm
Presence or absence of hydrocephalus
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Modalities and Options for Treatment..
Two different management attitudes:
Radical surgical excision
Intentional incomplete (subtotal or partial) excision and
radiotherapy
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Radical Surgery vs Conservative Surgery
Radical surgery:
Considerably lower recurrence rate after complete tumor
removal
Worse outcome of second surgery
Conservative surgery:
lower incidence of endocrine deficit
hypothalamic insufficiency and major disability limited
higher rate of local tumor control by incorporation of
radiotherapy
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Adjuvant radiotherapy..
Recurrence free survival increased from 38% to 77% after
radiotherapy
No agreement concerning timing – early vs. no radiation for stable
residual tumor
Conventional radiation
Advanced: 3D conformal, intensity modulated and stereotactic
54 Gy in 30 fractions for tolerance of optic chiasm; if single
targeted dose then 8 Gy
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Instillation of solution of beta particle emitting radioactive
isotopes – yttrium90, gold198 and phosphorus32
AE:
damage to pituitary and hypothalamus and visual structures
moyomoya syndrome, cavernous malformation
secondary malignant astrocytoma
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Chemotherapy..
Intracystic bleomycin in cystic craniopharyngiomas
cyst wall thickens and contracts, pulling away from brain
structures facilitating cyst removal
highly toxic for neural structures if allowed to leak
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Other techniques..
VP shunt
EVD
Stereotactic technique for cyst evacuation, instillation of
radioisotopes or bleomycin into the tumor cyst
Ommaya reservoir – for repeatedly enlarging cyst enables
simple evacuation of cyst contents
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In general..
Radical removal in cases of extraventricular craniopharyngiomas
and contraindicated when the tumor is intraventricular
Imaging studies do not show whether the tumor is simply
compressing the hypothalamus or invading it
Final decision about the extent of safe removal cannot be made
before the operation
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Surgery..
Accessibility does not mean resectability
Hypothalamic structure should be avoided
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Intrasellar and Intrasellar + Suprasellar Cranio..
Intrasellar – transsphenoidal route
Intra+suprasellar – transsphenoidal
giant/ dumbbell or multinodular shaped extended transsphenoidal allows removal of supradiaphragmatic also
Unilateral paraseptal, sublabial approach
Large opening of sphenoid sinus and sellar floor
Neuronavigation guided
Tumor does not invade cavernous sinus as the pituitary adenomas do
Capsule adherent to dura separated by pulling into the sellar cavity and bluntly dissecting it
free
If eggshell calcification – caution while removal
Preservation of stalk may necessitate leaving behind a piece of capsule
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Suprasellar Extraventricular..
Superior displacement of chiasm
Removal of tumor through –
prechiasmatcic space between optic nerves
opticocarotid triangle
lateral to carotid
Unilateral subfrontal approach / pterional approach
Removal through lamina terminalis would jeopardize
hypothalamic structures of the floor of IIIrd ventricle
retrochiasmatic approach safe
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Orbitozygomatic and transpetrosal approach may be of great help
Bifrontal craniotomy for giant multicystic tumor
Approach preferably through nondominant side
Medial part of sylvian fissure may required to be opened
Prevent leakage of cyst content to the subarachnoid space by
cotton patty
Solid part of the tumor removed piecemeal
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Removal of calcified portion of tumor from the wall of the carotid may be dangerous
Extreme care to look for minute perforating vessels, the branches of the supraclinoid carotid and the PcomA supplying the visual pathways and the hypothalamus
Branches of A1 and AcomA in close contact with tumors growing in front of the chiasm
Part of capsule left behind if attached to these vessels
Capsule firmly adherent to the IIIrd ventricular floor or pia of hypothalamus – left behind
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Removal of posterior pole does not pose problem as the basilar
artery and its branches and brainstem protected by liliequist’s
membrane
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Intraventricular and Extraventricular
Craniopharyngiomas..
Atrophied hypothalamic structures within the remnant of the floor of the third ventricle displaced around the equator of the tumor
Both extracerebral and transventricular approach
Low position of chiasm and narrow opticocarotid triangle in such setting preclude extracerebral tumor exposure
Central lower part of lamina terminalis is often composed of gliotictissue represents capsule of anterior pole of tumor
Its opening between chiasm and AcomA good exposure of anterior and basal parts of intraventricular mass
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CUSA may be used to break the calcified basal part
Piecemeal removal of basal part pituitary stalk may be seen
below the chiasm after removal of basal part
Brainstem, basilar artery and displaced mammillary bodies can
thus be seen from the Liliequist’s membrane
Remnants of infundibulum and tuber may also be seen
If no plane of cleavage between gliotic tumor capsule and third
ventricular floor remnant, then tumor capsule left behind
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Majority can be removed by trans laminaterminalis approach
adv: good access to the postero-basal expansion into the CP angle
disadv: insufficient exposure of the superoposterior part of the third ventricle
Transcallosal approach for larger tumor
adv: removal of tumor from upper pole devoid of neural structures
limitations: anteroinferior part of tumor may not be exposed
Usually tumor only touches fornix without invading it
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Combination approach hence useful
Removal of tumor may be staged; one setting 2 craniotomies or 1
large craniotomy
Transfrontal transcortical is usually avoided for seizure risks
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Intraventricular Craniopharyngiomas..
Rare
Push the third ventricle floor downward
Extracerebral approach – jeopardize hypothalamic strucutures
May be approached by opening the lamina terminalis or through
the foramina of Monro
Tumor adherence to lateral wall of third ventricle less pronounced
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Perioperative Management and Management of
Complications..
Preoperative Pituitary insufficiency Perioperative hormonal replacement
Hydrocortisone 400mg on the day of surgery 100 mg before surgery, 100 mg during tumor removal and rest after the operation
If uneventful postop recovery – dose of hydrocortisone reduced to 1/5 th on the fifth post operative day
Complications: hyperpyrexia, seizures, decreased level of consciousness, DI, hypernatremia, hypokalemia
Due to pituitary stalk disruption and damage of osmoreceptors in anterior hypothalamus
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DI, SIADH, CSW – identified and appropriately corrected
Bleeding in the tumor bed
VP shunt if persistent hydrocephalus
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Long term results..
Outcome depends on the location of tumor
Overall surgical mortality <4%
In a series with >60% large or giant tumors, radical tumor removal
achieved in 90% with overall mortality of 9% and recurrence rate
of 7%
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Recurrences and their management..
Most important factor for recurrence extent of resection
Removal of recurrent tumor lower cure rate and higher
complication rate because of scarring and adherence
Radiation therapy as the primary Rx for recurrence
Some residual tumors may remain stable for years without
adjuvant therapy
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Morbidity rate..
Pituitary hormone deficiency
Hormonal replacement required in 85-100% of cases
GH deficiency 100%
Hypogonadism 65-80%
hypothyroidism 38.5-95%
hypocorticism 55.2-78.1%
new post surgical DI 56%
Obesity 58-62%
significant school problems 50%
postsurgical visual impairment 5-66%
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Cysts of Suprasellar region..
Epidermoid and Dermoid Cysts:
arise from the inclusion of ectodermally committed cells at the
time of closure of the neural groove
epidermoid – occurs in older age group and shows slow
progression
multilayered keratinized, squamous epithelium
that rests on an outer layer of collagen
contents – solid, flaky and keratinous
occasional degeneration of center with flakes
replaced by greasy brownish fluid containing cholesterol crystal
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Clinical presentation..
Similar to craniopharyngioma
Less commonly, aseptic meningitis due to leakage of cyst content
Both T1 and T2 – slightly more hyperintense compared to CSF containing arachnoid cyst
Dermoid cyst – signal intensity similar to lipoma
Rx – ideally complete removal
if dense adherence – should not attempt radical resection
incompletely resected – will slowly recur
caution – chemical meningitis during surgery –
cottonoids; voluminous NS irrigation, local steroids
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Rathke’s Cleft Cyst..
Microscopic cysts between anterior and posterior lobes of the mature pituitary gland
Remnants of cyst also persists above the level of sellar diaphragm in the stalk
Lining – columnar or cuboidal cells with apical cilia, stratified at places; keratinization NOT seen
Relatively common incidental finding
Symptoms – headache (common), compression of pituitary, optic chiasm and hypothalamus – related symptoms
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Recurrent episodes of Aseptic meningitis – cyst leakage into the
subarachnoid space
Mean age: 4th decade (2nd to 8th decade)
Females more commonly affected
MRI: single, uniloculated, round, sharply defined intra or
suprasellar mass typically lying anteriorly to the infundibular stalk
variable T1 and T2 intensity depending on cyst content
no contrast enhancement or calcifications
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Rx:
Transsphenoidal approach
partial resection of cyst wall and evacuation of contents
endoscope considered the best
radical resection endangers the stalk
Craniotomy for suprasellar lesion
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Gliomas of the Chiasm and Hypothalamus
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May start from optic chiasm to involve hypothalamus or vice versa
Impossible to determine clinically and radiologically, from where it
started
Referred to as a single disease – opticchiasmatic-hypothalamic
gliomas; optic pathway/hypothalamic gliomas
Konovalov et. Al: Nodular type and Diffuse type
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5 types of Nodular variant..
1. Tumors with predominant anterior growth
2. Tumors growing anteriorly and penetrating the third ventricle
3. Tumors with the main part occupying the third ventricle but
infiltrating the chiasm as well
4. Tumors of optic tract
5. Gliomas of the floor of the third ventricle
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90% in pediatric age group
Usual age: 2-4 yrs
Histology: 60% pilocytic and 40% fibrillary
8% anaplastic – group 5 nodular (3rd ventricle)
Tumors grow in the subependymal layer of lateral wall of the 3rd
ventricle break through it later grows inside 3rd ventricle
MRI – hypointense in T1 and hyperintense in T2 and FLAIR; large
tumors heterogenous with solid and cystic components
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Clinical Presentation..
Progressive loss of vision with slow development of optic atrophy
Typical for chiasmatic glioma: defects in temporal half of the visual field of the “better” eye with practical blindness in the other eye
DI, obesity and genital underdevelopment hypothalamic involvement
Diencephalic syndrome (25%) in children less than 3 yrs heralded by:
emaciation and loss of subcutaneous fat, which contrasts with normal height and normal muscle mass
normal appetite; child alert, vigorous, hyperactive, euphoric
nystagmoid eye movement
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Management:
Highly unpredictable natural course
May remain static or quiescent for many years; others might have
aggressive course increasing rapidly
Involution after partial removal has been documented
Association with NF 1 have been reported as better prognosis by
some and unfavorable prognosis by other authors
Some recommend conservative surgery or biopsy followed by
radio or chemotherapy while other stress more radical resection
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Surgery..
Main aim: histological diagnosis and restoration of CSF pathway
patency
Tendency towards more radical resection for better outcome and
lower recurrence rate
Indication:
nodular or exophytic growth without optic tract involvement or
visual failure
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Surgical approach and extent of tumor removal..
Various approaches to access suprasellar region:
subfrontal
pterional
subtemporal
anterior interhemispheric
transcallosal
combined transcallosal-subfrontal
transcallosal-pterional
Most common tumor in this location: pilocytic astrocytoma
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Tumor inside the third ventricle NOT intimately adherent to the
lateral wall is removed
Superoposterior part of tumor might not be reached by
translamina terminalis approach; for that it may be combined with
transcallosal approach
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Management of hydrocephalus..
Shunt insertion after tumor resection may be necessary
Preoperative CSF diversion may also be necessary in acute
setting
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Adjuvant therapy..
Radiotherapy and chemotherapy often recommended
Radiation therapy arrests tumor growth
Median dose 54 Gy for chiasmatic glioma and 55-60 Gy for hypothalamic tumors
Efficacy reported as low as 40%
Responds surprisingly well to chemotherapy – tumor shrinkage and stabilization or even disappearance in some instance
Radiotherapy postponed beyond 5 yrs to limit side effects
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Complications..
Acute hypothalamic insufficiency (most common)
Careful monitoring and correction of metabolic disorders
Bleeding into the tumor bed needs evacuation
Brain collapse and subdural effusion and blood collection
Tension pneumocephalus
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Outcome..
Radicality of surgery influences long term outcome
5 and 10 yrs PFS for radical, subtotal and partial surgery – 100%, 74% and 51% respectively
Visual function improvement 14%
Worsening of visual function 23%
DI 12%
Precocious puberty 18%
Hypopituitarism and memory disturbances
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Germinomas..
Accounts to 0.5% of all CNS tumors and 3% CNS tumors in children
Occurs in midline intracranial axis that traverses 3rd ventricle
Occasionally, synchronous pineal and suprasellar region tumor
Important to distinguish pure germinomas from admixture of other germ cell tumors or syncytiotrophoblastic giant cell tumors secreting hCG
Pure germinomas are non secreting type whereas nongerminomatous GCT secret AFP and bHCG
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Infiltrative lesions involving floor of 3rd ventricle, pituitary stalk,
pituitary gland and optic pathways
Clinical manifestations:
visual disturbances, DI, obesity, pituitary insufficiency
precocious puberty and hydrocephalus
MRI – T1 – hypo to isointense and T2 – iso to hyperintense
intense contrast enhancement
CSF borne metastasis along ventricular surfaces and
subarachnoid space
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Extremely radiosensitive
Biological biopsy:
in the past – single fractionated dose delivered to the tumor site radiographic response suggested tumor of germinal origin high dose focal radiotherapy initiated
craniospinal irradiation
Chemotherapy: 80% complete radiographic response
3 course of carboplatin-etoposide chemotherapy followed by 24 Gyradiation
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Hypothalamic hamartomas..
Non-neoplastic, non progressive congenital malformations
Composed of disordered neurons, glial cells, & myelinated tracts
Occurs in 2 general locations:
tuber cinerum – usually pedunculated
within 3rd ventricle – sessile
Pedunculated – more likely small (<2cm); causes precocious
puberty but no other neurological symptoms
gonadotropin-releasing factors positive
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Sessile: - more often large (2-5 cm)
associated with gelastic seizures
seizures are subsequently longer duration develops
secondary, generalized epileptic manifestation
cognitive deterioration and behavioral problems
2/3 rd children – developmental delays
½ - precocious puberty
MRI:
T1 – isointense to mildly hypointense
T2 – iso to hyperintense
no contrast enhancement
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Pedunculated:
approached via pterional or subtemporal route
50% patients cured after total removal
subtotal removal – rarely leads to clinical or hormonal cure
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Sessile:
transcallosal approach or trans lamina terminalis
endoscopic removal
complete removal controls or cures seizure, improves
behavior, school performance and quality of life
main difficulty in surgery is to differentiate hamartoma from
normal brain tissue
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Pituicytomas (tumor of neurohypophysis)
Originate in pituicytes (modified glial cells) of posterior pituitary
gland and infundibulum
Start to grow within or above sella
Historically, terms choristoma and infundibuloma also used
Benign (WHO grade I)
Extremely rare; occurs in adult; granular cell tumor (other tumor
of posterior pituitary) can rarely occur in children
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Clinical presentation:
visual field deficit
hypopituitarism
galactorrhea (stalk effect)
headache
DI (rare)
Firm and vascular nature without obvious dissection plane may
hamper gross total resection
Significant bleeding risk
MRI – isointense to T1 and T2 and significant contrast enhancement
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Thank you!!!