This document discusses craniopharyngioma, a rare brain tumor that arises near the pituitary gland. It has a bimodal age distribution in children and adolescents. Surgical removal is the primary treatment but can be challenging due to the tumor's location near vital structures. The goals of surgery are complete removal while preserving neurological function. Radiation and chemotherapy may also be used for residual or recurrent tumors. Ongoing monitoring is important due to the risk of the tumor returning after initial treatment.

1. CRANIOPHARYNGIOMA
Cushing - “the kaleidoscopic tumors, solid and
cystic …” whose management is “one of the most
baffling problems to the neurosurgeon

2. EMBRYOLOGY

3. EPIDEMIOLOGY
• 3% of all intracranial neoplasms
• Bimodal distribution by age
• 6% to 9% of all pediatric brain tumors
• Most common nonglial tumor of childhood
• Incidence greatest at 6 to 10 years, followed
by 11 to 15 years
• Males > Females

4. PATHOLOGY
• From epithelial nests - Remnant of Rathke’s
pouch
• Extends from the sella turcica along the pituitary
stalk to the hypothalamus and the floor of the
third ventricle
• Craniopharyngiomas
– Adamantinomatous
– Papillary
– Mixed
– Craniopharyngioma and Rathke’s cleft cysts

5. ADAMANTINOMATOUS
• More common variant
• Layers
– Basal layer of small cells
– Intermediate layer of stellate
– Top layer of keratinized cells
• Keratin nodules
• These nodules may undergo mineralization
- metaplastic bone
• Cysts - oily liquid - Desquamated epithelium rich
in cholesterol, keratin, and occasionally calcium.

6. • Microscopic islets or “fingers” of tumor
embedded in densely gliotic parenchyma are
seen when tumor arises in the region of the
tuber cinereum, hypothalamus, and floor of
the third ventricle
• The gliotic reaction of Rosenthal fibers and
fibrillary astrocytes - Separates tumor from
the brain – Surgical plane
• Decreased risk of recurrence

7. PAPILLARY
• Adults , third ventricle
• Solid epithelium, without stellate zones, in a
papillary architecture - resembles metaplastic
respiratory epithelium
• Rarely undergo mineralization
• Cysts - Fluid is less oily and dark
• Due to absence of calcification and minimal
cyst formation, complete curative surgical
resection may be obtained

8. CLINICAL FEATURES
• Prodrome of 1 to 2 years
• Headache from raised ICP (MC)
• Visual symptoms
• Hypothalamic and endocrine dysfunction
– growth failure (short stature)
– delayed sexual maturation (20% to 50%)
– excessive weight gain
– Diabetes insipidus (DI)

9. Preoperative evaluation and
management
• Complete endocrinological evaluation to
uncover hypopituitarism particularly
Growth hormone
Cortisol
Thyroid hormone deficiencies.

10. • GH deficiency is the most common finding
(75%)
• Gonadotropin deficiency (60%)
• Thyroid or adrenal dysfunction (30%)
• DI (9% to 17%)

11. RADIOLOGY

12. X - ray
• Irregular speckled calcification seen just above
the sella turcica.
• The semicircular shell outlining the wall of
cystic lesion.
– Fine flaky calcium -fast growing tumours.
– Dense calcification -slow growing tumours.
• Mostly suprasellar.
• Calcification may be in cyst wall and/or solid
component.

14. CT
• Calcifications (90% in children)
• 20 changes in the skull base
– Enlargement of the sella turcica
– Erosion of the dorsum sella
• Noncalcified solid craniopharyngiomas Vs
Chiasmatic hypothalamic gliomas,
germinomas, and pituitary adenomas

16. MRI
• Hypo on T1,hyper on T2WI
• Multilobular
• Multicystic
• Enhances strongly/ heterogenously
• Often both cyst walls and solid
components enhance
• Completely solid (rare)

18. MRS
• Craniopharyngioma - Lactate or lipids and only
trace amount of other metabolites.
• Gliomas demonstrate - Choline, N-acetyl
aspartate, and creatine peaks with an
increased ratio of choline to NAA
• Pituitary adenomas - Choline peaks or no
metabolites at all

19. Samii and Tatagiba - Tumor extension
GRADE
I: Intrasellar or infradiaphragm
II: Occupying the cistern with/without an
intrasellar component
III: Lower half of the third ventricle
IV: Upper half of the third ventricle
V: Reaching the septum pellucidum or lateral
ventricles

20. • Postoperative scans - within 48 hours
• Residual tumor – Hoffman grading

21. THERAPUETIC GOALS
• Cure of disease with functional
preservation and restoration.

22. Surgical Approaches
• Ideal approach – Varies.
• Influenced by the tumour location with
respect to the sella, chiasm and third
ventricle.

24. Anterior Midline Approach
Trans-sphenoidal
• Grade I and II
• Decreased risk of visual injury
• Difficult in young children (non-
pneumatised sphenoid sinus)
• CSF leak

25. Anterior Midline Approach
Subfrontal
• Grade III and IV
• Pre-chiasmatic dissection of the tumour
• Potential violation of the frontal sinus
• Damage to the olfactory tract
• Technically more complicated (pre-fixed
chiasm)

26. Anterolateral Approach
Pterional
• Facilitating the resection of intrasellar,
suprasellar, pre-chiasmatic and
retrochiasmatic tumours.
• Restricted view of the contra lateral
opticocarotid triangle, the contralateral
retrocarotid space and the ipsilateral
hypothalamic wall.

27. Anterolateral Approach
Orbitozygomatic
• Expands on the pterional approach
• Significant suprasellar extension

28. Transpetrosal Approach
• Large retrochiasmatic tumors

29. Intraventricular Approaches
Transcallosal –transventricular
• Foramen of Monro is dilated by a tumour
projecting into the lateral ventricle
• Frontal lobe retraction injury

30. Intraventricular Approaches
Transcortical –transventricular
• Seizures
• Large ventricles and tumour extending to
the dorsal surface of the frontal lobe

31. Intraventricular Approaches
Trans lamina Terminalis
• Intraventricular tumors
• Pterional or a subfrontal approach to
access the lamina terminalis

32. Combined Approaches
• Subtemporal –transpetrosal –
– Primarily retrochiasmatic unilateral tumors extending to the
posterior fossa along the clivus.
• Pterional –transcallosal –
– Aid removal of adherent and calcified tumor within the third
ventricle.
• In transcallosal + pterional approach, intraventricular portions of
the tumour should be removed first, with the pterional approach
only being performed if basal portions of the tumor remain
inaccessible.

33. Radical surgery
• Possible in:
– Small or prechiasmatic
• Difficult in:
– Proximity and adherence of the lesion to the optic pathways and
adjacent neurovascular structures
– Reterochiasmatic
– Large
– Multicompartmental

34. Radical surgery
Advantage
• One treatment then only follow-up
Disadvantages
• Limited number of surgeons with adequate
expertise
• Difficult to assess true risks to individual
child
• Impaired quality of life
• Diabetes insipidus (95%)

35. Limited surgery
Goals
• Diagnosis
• Drain cysts
• Limit field of radiation
• Control hydrocephalus
• Improve vision
• Decompress chiasm

36. Limited surgery + radiation
Advantages
• Surgery can be performed with limited
experience
Disadvantages
• Decrease in IQ
• Cyst management (often multiple cyst
procedures)
• Complications of radiation
• Diabetes insipidus (5%)

37. Endoscopy
• Grade 1 and 2 tumors
– Transnasal
– Trans -sphenoidal
– Transethmoidal
– Transmaxillary
• Advantages :
– No brain retraction and the cosmetic deficit
– Less invasive
• Not appropriate
– When the lateral extent of the tumor passes more than 1cm beyond the lateral limits of
the exposure
– Epicentre of the tumour does not lie within the midline
• GTR rate 100%

38. Radiation Therapy
• Radiosensitive tumors
• STR alone- rec. rates of 55% to 85%.
• Postop radiotherapy in GRT- rec.rate to 15% to
20%
• The target for RT - tumor + margin of 5 to 10
mm
• If the cysts drained - target volume - based on
the decompressed volume

39. Radiation Therapy
• GTR not possible
– Conventional RT
– Intracavitary radiation
– Fractionated radiotherapy
– Stereotactic radiosurgery
• DOSE : 54 to 55.8 Gy (in 1.8-Gy daily fractions)

40. Stereotactic Radiosurgery
• Gamma Knife
• Best suited for
– Smaller tumors (<3 cm) with
– Predominant solid component
– Few mm away from the optic apparatus
– Small cystic or mixed tumors near the optic
apparatus
• Mean marginal dose - 12 Gy

41. Intracavitary brachytherapy
• Most effective - Primary monocystic tumors
with relatively thin walls

42. Aspiration
• Aspiration + Ommaya reservoir into the cyst -
never indicated as primary therapy in children
• To control cyst volume while waiting for the
therapeutic effect of intracavitary irradiation.

43. Intracavitary Irradiation
• Induces gradual, progressive shrinkage of the
cyst over the course of weeks to months.
• Minimum dose - 200 Gy
• The first radioisotope in literature -
Phosphorus 32 (32P), radiation range of 0.9
mm, t 1/2 14 days
• Yttrium 90 (90Y) t 1/2 - 3 days

44. • Cyst regression over several months
• Does not control solid tumor growth, nor does
it prevent the development of new cysts
• Can be used in combination with SRS for
tumors with larger cystic component and a
smaller residual solid component

45. Intracavitary Bleomycin
• At 1- to 2-day intervals depending on the cyst
volume, 1.5 to 10 mg of bleomycin
• Leakage of bleomycin into the subarachnoid
space or ventricles - ventriculitis, meningitis,
or vasospasm
• Involution of cyst - over several months

46. • Produces thick, tough cyst wall, resistant to
tearing - easier dissect
• Does not induce arachnoidal scarring or
damage the gliotic plane

47. Intracavitary Interferon
• Both systemic and intracavitary
• Cavalheiro and colleagues – 60 patients
– 78% - Short-term disease control
– 13% - New endocrine deficiencies
– 30% - Mild side effects
– 3 of the responders with tumor progression after
bleomycin but were salvaged
• No information on long-term outcome

48. RECURRENT CRANIOPHARYNGIOMAS
• Most common complication
• Presentation –
– most often visual deterioration
– Others - progressive headaches, hydrocephalus,
hormonal disturbances, and seizures
• Single factor closely associated - Extent of
resection at initial surgery
• Median time - 2 to 8 years

49. • Clinical factors for recurrence
– Larger tumors (>5 cm)
– Highly calcified tumors
– Tumors that extend beyond the suprasellar space
• Molecular factors – increased expression of
– Osteonectin
– Ki-67
– P53
– Epithelial cell adhesion molecule
– Pituitary transforming gene (PTTG-1)
RECURRENT CRANIOPHARYNGIOMAS

50. • Ectopic recurrence of craniopharyngioma –
adamantinomatous
• Contamination of tumor cells
• Surgical resection can be curative because of
preserved arachnoidal planes
• Reoperation, intracystic therapy, adjuvant
irradiation or re-irradiation, and SRS are all
potential options

51. • Reoperation for tumor resection should be
considered whenever possible both for
recurrences after GTR as well as after STR and
RT.
• Not candidates for reoperation
– Relatively asymptomatic
– Predominantly cystic tumor recurrence