craniophryngeoma

2. Dr. Usman Haqqani
Resident, NSBW
Lady Reading Hospital Peshawar.

3. LEARNING OBJECTIVES
• Introduction
• Embriology and Anatomy
• Epidemiology
• Classification.
• Clinical perspectives.
• Differential diagnosis.
• Treatment options.
• Management of complications.
• Post-operative follow-up.
• Recent advances.
• Take home message.

4. Introduction
• Unique lesion
• Histologically benign but behave as
malignant.
• Slow growing but high recurrence.
• Treatment protocol can be modified
according to personal experience.

5. Embryology

6. Anatomy
•Pituitary gland lies in the base of the skull in a portion
of sphenoid bone called Sella Turcica.
•It consist of two lobe anterior lobe
(adenohypophysis),and posterior lobe
(neurohypophysis).
•The size of the gland which the anterior lobe consist
2/3rd but varies considerably.
•It measures 15x10x6 mml, wt upto 500-900mg.

7. Anatomy

9. Pituitary - Relation to sphenoid sinus

10. Pituitary - Sella tercica, sphenoid bone

11. Epidemiology
• Relatively rare tumors.
• Common in children.
• Bimodal distribution— first peak at 5-10
years and second peak at 55-65 years.
• Can become symptomatic at any age.
• Equal sex distribution.

12. Origin of Craniopharyngioma
• Rathke cleft, together with remnants of the
craniopharyngeal duct, can be the site of origin of
craniopharyngiomas..
• Products of metaplasia of the ant pituitary gland.
• Dual origin
.Childhood type from embryonic origin
.Adulthood type have metaplastic origin

13. Classification
Different classifications
1)Based on location – Retrochiasmatic
-- Subchiasmatic
-- Pre chiasmatic
-- Intrasellar

14. Cont..
2)CONSISTANCY
--Predominantly cystic
--Predominantly solid
-- Cystic with solid component.

15. Cont..
3)HISTOLOGY
--Childhood or adamantinomatous.
--Adult or papillary type.
4)CALCIFICATION
--Calcified (85% childhood)
--Non calcified (40%adult )

16. Sammi et al: vertical projection
Grade I- intra sellar/infra diaphragmatic
Grade II-Occupying cistern with/with out an intrasellar
component.
Grade III- Lower ½ of 3rd
ventricle
Grade IV- Upper ½ of 3rd
ventricle
Grade V- Reaching the septum pllucidum or lateral
ventricle

17. Pathology
Depends on the anatomic location it causes
different pathologies.
• Retrochiasmatic tumor displaces the pituitary
forward and chiasma upward and forward
• Subchiasmatic tumor displaces the stalk
backward and chiasma upward thus
stretching the optic nerve.

18. Cont..
• Prechismatic are cystic and attain
enormous size i.e. extending from the
sylvian fissure laterally to the prepontine
cistern posteriorly causing psychomotor
seizures and cranial neuropathies.
• Intrasellar tumor causes pituitary
dysfuction.

19. Contd…
• Hypothalamic function is mainly reserved
because it accommodate itself during
elevation of the floor of the third ventricle .
• Features of raised intracranial pressure
develop due obstruction.

20. MRI

21. Clevage line
Tumor capsule separated from the
surrounding structures by
• Normal layer of arachanoid ant, inf, and
laterally.
• Double layer of laliequist posteriorly.

22. Blood supply of the tumor
•Include
– ant communicating artery ,
– A1 segment of ant cerebral arteries
– post communicating artery
– meningohypophysial arteries
•Never from posterior cerebral and basilar artery.
•Important for safe removal of retrosellar tumor.

23. Blood supply of the tumor

24. Clinical features in children
• Raised ICP.
-Headach
-vomiting
-no neurological deficit
• Endocrinopathies.
1)common-short stature and delayed
puberty.
2)less common –diabetes insipidus

25. Contd…
• Hypothalamic syndrome.
1)common-central hyperphagia, obesity.
2)rare-sleep disturbance, disturbance in
temp and thirst regulation.

26. Contd…
• Visual symptoms.
• Neurobehavioral problems.
-abulia
-amnesia

27. C/F in Adults
• Visual problems in 80 percent.
• Neurobehavioral problems.
• Features of raised intracranial pressure-only
in one third of patients.
• Gonadal failure causing amenorhea in
women and loss of libedo in males.

28. Investigations
• X-ray. 66% of adults and 95% of children
have abnormal findings on x-ray.
• Findings are—errosion of ant clinoid and
dorsum sellae, expansion of the upper
sella and suprasellar calcification.
ballooned sella due to intrasellar extension
of craniopharyngioma.

29. X-ray.

30. Contd…
CT SCAN:
1. Less ambiguous than MRI in differentiating solid
from cystic—vital in classifying tumor into solid and
cystic—important in choosing the optimal therapy.
2. To define the anatomy of bones—help in
preparation for trasphenoidal approach.
3. Important in picking calcification especially in the
base of skull.

31. CT SCAN

32. CT SCAN

33. MRI
• Delineate the interface and relationship between
tumor and brain in multiplane displays.
• Enable the surgeon to select surgical approach for
surgical resection e.g.
1)sagital image – distortion of third ventricle
and hypothalamic structure.
2)coronal images– relation of lower pole of
tumor to sella, sphenoid ,cvaernous sinus –
frontobasal transphenoidal approach,

34. MRI

35. MRI (Coronal view)

36. MRI (Saggital)

37. Surgical specimen

38. Differential Diagnosis:
1. Tumours (dermoid/epidermoid, optic
glioma, meningioma, pituitary tumours).
2. Infectious/Inflammatory processes (T.B,
Syphilis).
3. Vascular malformation (hemangioma,
aneurysm).
4. Other congenital anomalies (arachnoid
cyst).

39. Management of Hydrocephalus
• 60% present with hydrocephalus
• Symptoms of high ICP are present
• CSF diversion procedure should be done.
• If not, then EVD at the time of tumour
resection.

40. Treatment options
Depends on the consistancy of tumor.
predominantly solid tumor.
Four treatment options.
1)Total resection.
2)Subtotal resection with
radiotheraphy.
3)Biopsy and radiation
4)Steriotactic radiosurgery.

41. Choice of Treatment
Depends on.
1)age of the patient.
2)anatomy of the tumor.
3)availability of stereotactic surgery.
4)experience of the surgeon.
5)extent and size of the tumor on
neuroimaging.

42. Contd…
• Predominantly cystic tumor.
1)Total resection.
2)Instillation of beta emitting radionuclide
into the cyst cavity.

43. Treatment of recurrent tumor
Different options depending on the :
• consistancy
• adherence to the surrounding
structures.
• radiated or not .
• resistance to treatments.

44. Contd…
1. Total excision in child, good medical
condition, regrowth of solid component of
predominantly cystic tumor.
2. Subtotal resection with radiation.
3. Radiation alone.
4. Intracavitery Yttrium 90 if cystic.
5. Intracavitery bleomycin if near to the
visual apparatus.

45. Surgical Approaches
Depends on;
1)Anatomic location
2)Direction of extension.

46. Approaches
Five approaches;
A- Subfrontal approach.
B- Pterional approach.
C- Transcallosal approach.
D- Subtemporal approach.
E- Transphenoidal approach.

47. Subfrontal Approach
Five steps:
1)Preparation;
• history and examination.
• perimetery.
• asessment for anesthesia.
• asessment of endocrine function.

48. Subfrontal Approach (contd)
2)Position.
Patient should be put in supine position with
the neck slightly extended.
3)Portal . -frontal hair line incision
-free bone flap/osteoplastic
-durotomy
-CSF suction.

49. Sub frontal Approach (contd)
4)Procedure:
• Inter-optic debulking
• Separation of the capsule from optic structures
• Rt. And Lt. lateral dissection
• Detaching upper pole of the tumor from the
hypothalamus.
• Intra seller dissection
• Closure

50. Subfrontal Approach (contd)
5. Post op care.
• Vitals monitoring.
• Intake/output record. (especially look
for DI).
• Monitoring of the conscious level.

51. Management of Complications
• Post-operative visual outcome depends upon
the pre-operative visual status.
• There is high likelihood of stalk injury, so DI is
then universal,
– 3 phases:
• Transient (resolves in 24 hrs)
• Temporary (24-96 hrs)
• Permanent (up to 3 yrs)

52. Management of Complications (contd)
• Dexamethasone used prophylactically to prevent
brain edema.
• Hypothyroidism: thyroxine
• FSH and LH deficiency is permanent.
– Estrogen, progesterone and testosterone
replacement.
• Growth Hormone: replacement usually started 6-9
months after surgery.
• Obesity: behavioral modification, exercises and
psychological counseling.

53. Post-operative Follow-up
• CT with Contrast: 24 hrs
• Next CT: 3-6 months
• Yearly CT/MRI is sufficient.
• Complete endocrine evaluation: 6 months
• Test VA : Twice yearly.

54. Recent Advances
• Endoscopic removal of the tumor.

55. Take Home Message
• Craniopharyngioma are rare tumor of childhood
constituting about 8-10% of all brain tumors.
• They are predominantly solid but almost always
have a cystic component. Almost all childhood
craniiopharyngioma have calcification.
• Children commonly present with hydrocephalus,
growth retardation and obesity. Adults present with
visual symptoms.
• Large cystic tumour can be treated with beta
emitting radionuclide.

56. Take Home Message
• Smaller solid tumor or recurrence can be
treated with stereotactic radio surgery.
• Total resection if possible is associated with
the best long-term results with 80-90% 10 yrs
disease free survival.
• Unilateral sub frontal resection is favored
approach.

57. THANK
YOU