Craniopharyngioma is thought to arise from ectodermally derived epithelial remnants of rathke’s pouch and there craniopharyngeal duct. Neoplastic transformation of cells derived from tooth primordia give rise to adamantinomatous craniopharnygioma, whereas such transformation in cells derived from buccal mucosa primodia give rise to papillary type

1. CRANIOPHARYNGIOMAS
SURESH BISHOKARMA,
MS, MCH Resident
Neurosurgery
NINAS

2. o Tumors of dysembryogenic origin
Craniopharyngioma, Epidermoid, Dermoid, Hamartoma,
Germ Cell tumor, Rathke’s cleft cyst
o Tumors originating from tissues of suprasellar structures
Gliomas of visual pathways and hypothalamus,
pituicytoma, granular cell tumor of neurohypophysis,
meningioma
o Tumors extending into the suprasellar space secondarily
Pituitary adenoma, Rathke’s cleft cyst
o Systemic tumors affecting the CNS
CNS metastasis, lymphoma, leukemia
Suprasellar Tumors

3. CRANIOPHARYNGIOMAS

4. *Craniopharyngioma is thought to arise from epithelial
remnants of rathke’s pouch.
*Tooth primordia adamantinomatous type
whereas
*Buccal mucosa primodia  papillary type.

5. By the 4th week of gestation, invagination of the stomodeum, lined by
epithelial cells, take place.
This upward migration is met by a downward movement of
neuroepithelium from the hypothalamus.
EMBRYOLOGY

6. *Embryonic rest to suprasellar or parasellar locations.
*This migration pathway from the primitive oral cavity is termed
the craniopharyngeal duct.

7. CRANIOPHARYNGEAL DUCT
1904: Erdheim: Craniopharyngioma: Incomplete involution of this pathway
Cranipharyngioma
Rathke’s cyst
Adenohypophysis

8. * Benign, extra-axial epithelial tumors.
* Aggressive clinical course – significant morbidity and shortened life
expectancy
CRANIOPHARYNGIOMAS

9. *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-chiasmatic tumors
Incidence

10. ERDHEIM EMBRYOGENIC THEORY:
1. Origin – remnant of craniopharyngeal duct or Rathke’s pouch.
1. Epithelial cell rests of the vanishing hypophyseal duct remain
adherent to the neuroepithelium before the pia is formed.
1. Arise from the antero-superior surface of the
adenohypophysis.
TUMOR DEVELOPMENT

11. PATHOPHYSIOLOGY

12. B catenin is a downstream component of the Wnt intracellular signaling pathway

13. PATHOLOGY AND SURGICAL
ANATOMY

14. MICROSCOPIC ANATOMY
ADAMANTINOMATOUS
CRANIPHARYNGIOMA
1. Complex epithelial
lesion resembling
enamel pulp of
developing teeth
2. Pallisading peripheral
layer of epithelium
3. Hydropic vacuolation
of inner layer
4. Loose stellate reticular
zone containing
nodules of plump
keratinocytes “wet
keratin”
5. Dystrophic calcification
6. Areas of cholesterol
deposits

15. PAPILLARY
CRANIPHARYNGIOMA
Papillary:
Composed of simple
squamous epithelium,
which rests on a villous
firovascular
stroma forming
papillae.
MICROSCOPIC ANATOMY

16. *Both variants:
* Brain parenchyma – Gliotic with profuse Rosenthal fibers

17. *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
Macroscopic anatomy

18. *Infradiaphragmatic tumor
*Supradiaphragmatic
*Topography depends on
* length of optic nerve
* position of the chiasm
Surgical Anatomy

19. *Grekhov: 4 groups according to the point of original growth
* Infrasellar
* Intrasellar (intrasellar and suprasellar)
* Pituitary stalk
* Infundibular
TUMOR DEVELOPMENT

20. Origin: Intrasellar
*Upward displacement of sellar diaphragm and arachnoid
*Grows below the chiasm, displaces it upward and compress the
floor of the third ventricle
INFRADIAPHRAGMATIC

21. * Origin:
1. Stalk or inside the infundibulum (most basal part of the floor of IIIrd ventricle)
2. Pituitary stalk → suprasellar extraventricular
Grows below the chiasm and the floor of the third ventricle)
* Extends anteriorly to the chiasm b/t optic nerves
* Retrochiasmatic if prefixed chiasm
3. Infundibulum → intraventricular and extraventricular (IEVC): Most common.
* Grows behind the chiasm in the region of its posterior angle
between the optic tracts.
Disrupts third ventricular floor at an early stage
* Partially inside and partially outside IIIrd ventricle
SUPRADIAPHRAGMATIC

22. *The structures of the third ventricular floor undergo atrophy of
different degrees.

23. * Most affected region of IIIrd ventricle – tuber (central) part:
Absent post infundibular eminence
*Compressesed mammillary bodies: common
*Infundibulum (median eminence) less frequently destroyed
PATHO-ANATOMY IN IVEC

24. * Commonly located exclusively behind
the chiasm
* Small part of tumor may extend below
the chiasm or b/t optic nerves.
IVEC

25. SUPRASELLAR EXTRAVENTRICULAR

26. *Rare subtype entirely within the ventricular
cavity
*Attached to a partially atrophied floor of IIIrd
ventricle
EXCLUSIVELY INTRAVENTRICULAR

27. ENDOCRIN
E
DEFICIENC
IES (90%)
IN
CHILDREN
VISUAL
DISTURBA
NCES
(96%) IN
ADULT
HYPOTHA
LAMIC
DISORDER
S –
OBESITY –
CHILDREN
MEMORY
IMPAIRME
NT:
50%
AMONG
>40YRS
MENTAL
DISORDER
S
HYDROCE
PHALUS
CLINICAL PRESENTATION
NINAS

28. ENDOCRINOPATHIES
NINAS
•Growth hormone deficiency : ~82%-
•Growth deceleration; Delayed puberty;.
•90% men – impotence; Decreased sexual drive
•Diabetes insipidus – presurgery – 8-35% cases
•Hyperprolactinemia- Amenorrhea – 40%

29. VISUAL DISTURBANCES
NINAS
Vision may also worsen after the surgery
• DEFECTS IN VISUAL FIELDS:
•Bitemporal hemianopia, homonymous hemianopia,
•concentric contractions of fields, central or paracentral scotoma
• DECREASED VISUAL ACUITY
•Compression not only by tumor but also by A1 or Acomm due to
displacement of chiasm towards those arteries.
•Compression of lower chiasmatic arteries may also be the cause.
• Long-standing intracranial hypertension causing atrophy of the optic disks
may lead to concentric narrowing of the visual fields.

30. MEMORY IMPAIRMENT
NINAS
•Lesion of the mammillary
bodies or
•Lesion in their connections
with:
•The hip pocampal
system,
•Fornix
•Mammillary-thalamic tract

31. Neurobehaviour abnormalities
NINAS
•Cognitive impairment
•Dementia, depression, hypersomnia, apathy
•Psychomotor retardation
•Flattening of affect

32. HYPOTHALAMIC DISTURBANCES
NINAS
•Growth failure (children):
•Hypogonadism
•(Adults)
•Central hyperphagia
•Disturbance of thirst
•Disturbed circadian rhythm

33. HYDROCEPHALUS
NINAS
•IEVCs:
•Hydrocephalus:
children (73%)
• Adult(49%).
•Extraventricular tumors: Unusual

34. THREE MAJOR CLINICAL SYNDROMES
BASED ON LOCATION
NINAS
•PRECHIASMAL/CHIASMAL:
•compression of optic apparatus:
•Optic atrophy (eg, progressive decline of visual acuity
and constriction of visual fields): bitemporal vision
loss
•RETROCHIASMAL:
•3rd ventricle obstruction
Hydrocephalus, with signs of increased intracranial
pressure (eg, papilledema and horizontal double
vision)
•INTRASELLAR:
•compression of pituitary stalk and hypothalamic region
•Endocrinopathy and headache

35. 1. Rathke Cleft Cyst
2. Suprasellar Arachnoid Cyst
3. Hypothalamic/Chiasmatic Astrocytoma
4. Pituitary Adenoma
5. Can mimic CP when cystic and hemorrhagic
• Epidermoid tumors
• Thrombosed Aneursym
• Germinoma or Mixed Germ Cell Tumor with Cystic Components
Differential Diagnosis

36. *Anatomical position
*Nature of tumor: monocystic with a solid component or
predominantly solid with a cystic component.
*Relation ship with surrounding structure
*Realtionship with intracranial vessels
*Hydrocephalus: IVEC
*Calcification: not unique feature of adamantinomatous but its
commonly seen
NEUROIMAGING

37. * 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
* Hydrocephalus
Radiographic Diagnosis

38. SUPRASELLAR EXTRAVENTRICULAR

39. IVEC
PREDOMINANTLY CYSTIC INTRAVENTRICULAR CRANIOPHARYNGIOMAS
WITH DIFFERENT CYSTS

40. RELATIONSHIP WITH THE OPTIC NERVES
INTRAVENTRICULAR AND EXTRAVENTRICULAR CRANIOPHARYNGIOMAS
(1), chiasm (2, open arrow), optic tracts (3), internal carotid (4), and anterior
communicating (arrows) arteries.

41. CALCIFICATION
Eggshell-like calcification of the capsule of intrasellar and suprasellar
craniopharyngioma

42. *MRI – heterogenous mass
*Solid part – Isointense on T1 + contrast
enhancement.
*Cyst wall: Hyperintense
*Cavity : high protein content: Hyperintense
*T2W: Mixed hypointensity or hyperintensity
*Edema along optic tract: Differentiating feature.
MRI CHARACTERISTIC

43. *Acomm – mostly in upper posterior surface of chiasm.
RELATION TO OPTIC NERVE AND ACOMM
SUPRASELLAR EXTRAVENTRICULAR CRANIOPHARYNGIOMAS
INTRAVENTRICULAR AND EXTRAVENTRICULAR CRANIOPHARYNGIOMAS NINAS

44. MRI: CRANIOPHARYNGIOMA
Relation to Sella and diaphragm – best in coronal view.

45. COMPUTED TOMOGRAPHY
CALCIFICATION
(45-57% overall: Adult: 40%: Children: 78-100%)

46. *There are no radiologic features that can absolutely discriminate
among the subtypes of craniopharyngioma.
*Favours to ACP
Lobulation, vessel encasement and calcification.
DIFFERENTIATING AMONG SUBTYPES

47. *2 intentions of Rx
*Remove totally whenever technically possible.
*Intentional incomplete removal and radiotherapy to lower
surgical morbidity
*Radical removal – only in patients where hypothalamus
NOT involved
TREATMENT

48. BASED ON DEGREE OF HYPOTHALAMIC
DISPLACEMENT
Puget et al
GRADING:
Grade 0 = None
Grade 1 =
Abutting/displacing
Grade 2 =
Involving/Infiltrating

49. * Two different management attitudes:
* Radical surgical excision
* Intentional incomplete (subtotal or partial) excision and
radiotherapy
MODALITIES FOR TREATMENT

50. *Transphenoidal : 90% of intrasellar and parasellar tumors
approached transphenoidally
*Use of pterional approach if above pituitary

51. *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
* Risk of post radiation malignant transformation: Rare
Radical Surgery vs Conservative Surgery

52. *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
ADJUVANT RADIOTHERAPY

53. *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

54. *Intracystic bleomycin in cystic craniopharyngiomas
* Cyst wall thickens and contracts, pulling away from brain
structures facilitating cyst removal.
* If Leak: highly toxic for neural structures
* Bleomycin – limited success.
CHEMOTHERAPY

55. • β emitter 32 P, Yttrium-90
▫ 200Gy to cyst wall
Treat residual or recurrent cyst formation
▫ Used in patients to delay definitive treatment
(ie. Surgery GTR or STR +EBRT) for young patients
Intracystic RT
Intracystic irradiation for craniopharyngiomas.Pituitary, 16:34-45; Halperin

56. *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
OTHER TECHNIQUES

57. *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
In general

58. *Accessibility does not mean resectability.
*Hypothalamic structure should be avoided.
SURGERY

59. *Intrasellar – transsphenoidal route
*Intra+suprasellar – transsphenoidal
*Giant/ dumbbell or multinodular shaped → extended transsphenoidal
→ allows removal of supradiaphragmatic also
INTRASELLAR AND INTRASELLAR + SUPRASELLAR
CRANIOPHARYNGIOMA

60. *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
TRANSPHENOIDAL

61. *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.
Suprasellar Extraventricular

62. *Orbitozygomatic and transpetrosal approach may be of great help.
*Bifrontal craniotomy for giant multicystic tumor.
*Approach preferably through non dominant 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.

63. *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.

64. *Removal of posterior pole does not pose problem as the basilar
artery and its branches and brainstem protected by liliequist’s
membrane.

65. *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 gliotic
tissue → represents capsule of anterior pole of tumor.
*Its opening between chiasm and AcomA → good exposure of anterior
and basal parts of intraventricular mass.
IVEC

66. * Majority can be removed by trans lamina terminalis approach
* Advantage: good access to the postero-basal expansion into the CP
angle
* Disadvantage: insufficient exposure of the supero-posterior 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.
IVEC

67. *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
IVEC

68. *Rare
*Push the third ventricle floor downward
*Extracerebral approach – jeopardize hypothalamic structures
*May be approached by opening the lamina terminalis or through
the foramina of Monro.
*Tumor adherence to lateral wall of third ventricle less pronounced
INTRAVENTRICULAR
CRANIOPHARYNGIOMAS

69. SURGICAL CORRIDORS TO A CRANIOPHARYNGIOMA

70. OPERATIVE
APPROACH
SUITABILITY ADVANTAGE OPERATIVE DETAIL DIFFICULTIES
1. Subfrontal/
Trans–Lamina
Terminalis
Approach
Midline
prechiasmatic tumor
that extends into the
anterior cranial
fossa floor
or superiorly into
the suprasellar
cistern and the third
ventricle
Midline orientation
Early access to both the
optic nerves and
internal carotid
arteries,
as well as the third
ventricle via the lamina
terminalis.
Exposure of the optic nerves,
optic chiasm, and carotid
arteries bilaterally is then
afforded.
Opening of the lamina
terminalis immediately
posterior to the chiasm then
allows access to tumors that
extend into the third ventricle
1. Bifrontal lobes
retractional postoperative
swelling.
2. Potential risk to one of
olfactory nerves.
3. Frontal sinus entry : will
require cranialization; risk
for infection, or CSF
leakage
4. Removal of an intrasellar
tumor can be difficult
when using a subfrontal
approach
2. Bifrontal
Basal
Interhemispheric
Approach
Large midline,
retrochiasmatic
tumors that may
have retrosellar
extension.
Wider visualization of
the optic pathway and
anterior circle of Willis
Once the olfactory tracts are
dissected from the brain surface,
the chiasmatic and
interhemispheric cisterns are
opened so that the optic chiasm,
bilateral A2 segments of the
anterior cerebral artery, and
anterior communicating vessels
are well visualized.
After opening the lamina
terminalis, the tumor is
internally
decompressed
1. More technically
challenging
2. Division of the bilateral
anterior bridging veins,
3. Frontal lobe retraction:
bilateral frontal lobe
injury.
CRANIOPHARYNGIOMA: APPROACH

71. OPERATIVE
APPROACH
SUITABILITY ADVANTAGE OPERATIVE
DETAIL
DIFFICULTIES
3. Transsphenoidal
and Extended
Transsphenoidal
Approaches
Smaller midline
tumors within
the sella or with
infradiaphragm
atic suprasellar
component.
Lack of need for brain
retraction and
Potentially better
visual outcomes
Sublabial, transseptal,
and direct approaches to
the sphenoid. Extended
Transsphenoidal
procedure, the
tuberculum sellae can
be removed, and
additionally, excision of
the planum sphenoidale
will provide improved
access to the suprasellar
region.
1. Lateral extension of the tumor can be
difficult to access.
2. Perforating vessels are at risk to injury
3. Direct control of intracranial neural
and vascular structures is difficult.
4. Suprasellar calcifiations are thought to
be a contraindication.
5. Difficult in poorly pneumatized sinus
6. Difficult in patients with nasal and
sinus pathology.
7. Reconstruction after tumor removal
can be complicated, and
8. High rates of CSF leakage
4. Pterional
Approach
Best be suited
for smaller
tumors confined
to the
suprasellar
space
It does allow access to
both prechiasmatic
and retrochiasmatic
lesions, along with
those above and below
the diaphragm
May be combined
with other approaches
to remove larger
lesions with signifiant
suprasellar
extension.
Frontal swing during
craniotomy
The dura is opened
and reflected anteriorly.
The sylvian fisure is
split, and frontal and
temporal retractors
applied.
1. Difficulties primarily relate to
visualization of the opposite
carotid artery and access to the third
ventricle.
2. Brain retraction may be necessary.
3. Large tumor: narrow to perforating
vessels.
CRANIOPHARYNGIOMA: APPROACH

72. OPERATIVE
APPROACH
SUITABILITY ADVANTAGE OPERATIVE
DETAIL
DIFFICULTIES
5. Orbitozygomatic
Approach
Essentially an
extension of the
pterional approach,
with removal of
the lateral orbital
bar and
zygoma
Tumors confined to
the suprasellar space.
Greater access
to tumors with
signifiant
suprasellar
extension.
The frontotemporal
craniotomy is extended
to involve the lateral
orbital bar and zygoma.
allow access to lesions
with more vertical
height.
1. Brain retraction may be necessary.
Interhemispheric-
Transcallosal
Approach
Large midline tumors
with suprasellar
extension into the
Third and potentially,
the lateral ventricle
can be accessed
Pure intraventricular
tumor
Great access to
significant
suprasellar
extension
Removal of
tumor from
upper pole
devoid of neural
structures
Exposure of the sagittal
sinus at approximately
two thirds of its length
anterior and one third of
its length posterior to
the coronal suture
Tumor may be resected
through an enlarged
foramen of Monro, or
the choroidal fisure can
be opened to allow
greater access to the
third ventricle.
1. Medial hemisphere Retraction.
2. Anteroinferior part of tumor may not
be exposed.
3. At risk: pericallosal, arteries and
fornix, as
4. well as the veins and floor of the third
ventricle.
CRANIOPHARYNGIOMA: APPROACH

73. OPERATIVE
APPROACH
SUITABILITY ADVANTAGE OPERATIVE
DETAIL
DIFFICULTIES
Transcortical-
Transventricular
Approach
Rarely used.
patient with
hydrocephalus and
craniopharyngioma
extending into the
third ventricle,
particularly through
the foramen of
Monro
less Corticectomy
performed through the
middle frontal gyrus.
The lateral ventricle is
entered.
The tumor is identified
and may be resected
from the ventricle and
followed through the
foramen of Monro
into the third ventricle.
1. Cortical injury: increased
postoperative seizures risk.
2. The size of the ventricle is important,
and hence in the absence of significant
hydrocephalus, this approach is
unfavorable.
3. Ipsilateral dissection of the wall of the
third ventricle is also diffiult
CRANIOPHARYNGIOMA: APPROACH

74. *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
1/5th on the fifth post operative day.
Perioperative Management and Management
of Complications.

75. *Hyperpyrexia, seizures, decreased level of consciousness,
DI, hypernatremia, hypokalemia.
*DI: Due to pituitary stalk disruption and damage of
osmoreceptors in anterior hypothalamus
COMPLICATIONS

76. *DI, SIADH, CSW – identified and appropriately corrected
*Bleeding in the tumor bed
*VP shunt if persistent hydrocephalus

77. *Dr. Suresh Bishokarma, MS, MCH Resident , Neurosurgery, NINAS

78. *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%
LONG TERM RESULTS

79. *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
*Some residual tumors may remain stable for years without
adjuvant therapy
RECURRENCES AND THEIR
MANAGEMENT

80. *Survival rate with CP are 80-91% at 5 years, and 83-96% at 10
years and 84% at 30 years (Youman’s 6th pp.1521)
* Karavitaki et al. found no difference in overall survival rate among patient
patient undergoing GTR (100%), Partial resection with radiotherapy (87%) and
partial resection alone (86%)
mortality

81. Favorable:
▫ Lack of calcifications (especial in adults)
▫ Extent of surgical resection
▫ Caucasian race
Unfavorable:
▫ Age younger than 5 years old ( Neonatal CP is highly fatal)
▫ Size > 5 cm
▫ Hydrocephalus: Need for CSF shunting
“ Tumor histological type, location , size and presence of
hydrocephalus don’t appear to be independent predictors of
survival.”
Prognostic Factors

82. TREATMENT OVERVIEW

83. *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
Cysts of Suprasellar region..

84. * 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
Clinical presentation..

85. *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
Rathke’s Cleft Cyst..

86. *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

87. *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

88. *Gliomas of the Chiasm and Hypothalamus

89. *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

90. 1. Tumors with predominant anterior growth
1. Tumors growing anteriorly and penetrating the third ventricle
1. Tumors with the main part occupying the third ventricle but
infiltrating the chiasm as well
1. Tumors of optic tract
1. Gliomas of the floor of the third ventricle
5 types of Nodular variant..

91. *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

92. * 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
*
Clinical Presentation..

93. *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
Management:

94. *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
Surgery..

95. *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
Surgical approach and extent of tumor
removal..

96. *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

97. *Shunt insertion after tumor resection may be necessary
*Preoperative CSF diversion may also be necessary in acute
setting
Management of hydrocephalus..

98. *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
Adjuvant therapy..

99. *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
Complications..

100. * 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
Outcome..

101. *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
Germinomas..

102. *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

103. * 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 Gy
radiation

104. *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
Hypothalamic hamartomas..

105. *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

106. *Pedunculated:
*
* approached via pterional or subtemporal route
* 50% patients cured after total removal
* subtotal removal – rarely leads to clinical or hormonal
cure

107. *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

108. *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
Pituicytomas (tumor of neurohypophysis)

109. * 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