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Craniopharyngioma
- 1. CRANIOPHARYNGIOMA Dr RE Anto Deptof Neurosurgery
- 2. • “The mostformidable of all intracranial tumours” • “The kaleidoscopic tumours, solid and cystic....” whose management is “one of the most baffling problems to the neurosurgeon” -Harvey Cushing (1936)
- 3. Craniopharyngioma -Benign intracranial tumour -Intimaterelationship with critical structures • Visual apparatus • Pituitary • Hypothalamus • Limbic system -Devestating clinical and psychosocial consequences
- 6. PATHOPHYSIOLOGY Two hypotheses • Embryogenic •Transformation of the squamous cell epithelium along the path of the craniopharyngeal duct • Metaplastic • Metaplasia of adenohypophyseal cells in pituitary stalk or gland “Rathke’s Pouch” Defect in Wnt signaling pathway reactivation • β-Catenin gene mutations effecting exon 3 suggesting nuclear β-Catenin accumulation
- 8. EPIDEMIOLOGY • 3% ofall intracranial tumours • >50% suprasellar tumours in childhood • Most common nonglial tumour of childhood • Bimodal distribution – Children (5-15 years) – Late adulthood (45-60 years) • Slight male predominance (55%)
- 9. PATHOLOGY Adamantinomatous (WHO Grade1) • More common variant • Epithelium of tooth primordia • Cysts in these tumours contain “motor oil” liquid • Associated with CTNNB1 mutation
- 11. Papillary (WHO Grade1) • Adults • Predominantly solid tumours • Cyst – fluid less oily and dark • Derived from buccal mucosa primordia • Associated with BRAF V600E mutation Mixed • 15% of craniopharyngiomas
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- 13. • Anterior pituitary deficiency(>85%) Paediatric • Visual dysfunction (>85%) Adult • Diabetes insipidus • HCP • Cognitive dys • Memory impx Both
- 14. Hypothalamic syndrome • Obesity •Sexual dysfunction • Sleep/Wake cycle • Pituitary failure • Temperature dysregulation • Behavioral (Depression/Aggression) • Social / Emotional withdrawal
- 15. NEUROIMAGING Various imaging modalities •Skull Xray/CT/MR sequences • 60-80%: calcifications in the suprasellar region • 75%: one or more cysts CT • Better at evaluating the calcifications • The skull base (enlargement of the sella turcica or erosion of the dorsum sella) MR • Better evaluation of the relationships of tumour to surrounding structures
- 16. MRI • Cysts areuniformly hyperintense on T2 • Heterogenous on T1 MR Angiography • Relationship between the tumour and the surrounding vessels and the circle of Willis MR Spectroscopy • Shows a dominant peak consistent with lactate or lipids
- 17. • 7 yearold boy with 2/12 hx of visual problems and headaches. CT scan
- 18. T1WI Sagittal T2WISagittal
- 19. T1WI C+ SagittalT1WI C+ Coronal
- 20. Perfusion MR MRSpectroscopy
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- 22. Role of surgery Maximalsafe resection • Transcranial vs Endoscopic endonasal • Gross total vs Subtotal Management of cysts • Drainage vs Ommaya reservoir Management of hydrocephalus • Shunt placement
- 23. Transcranial approaches • Subfrontal •Pterional • Orbitofrontal • Lamina terminalis • Transcallosal • Transcortical
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- 27. Intracystic therapy • Reservedfor tumours with one big dominant cyst • Delay time to defintive surgery or radiation • Requires surgery for placement and CT “leak test” • Radiation - P32 (one time) • 79.5-96% regression of cysts • Chemotherapy - Bleomycin (3 week course) • 50% of cyst regression • Chemotherapy - Interferon-α (4 week course) • 78% of cysts shrunk by 50% or more
- 28. • 2 xOmmaya reservoir insertions in 2020 at Polokwane Hospital
- 29. • A seriesof 17 children, intracystic bleomycin was well tolerated, with five complete remissions and a median progression-free interval of 1.8 years
- 30. • A seriesof 47 patients, given a radiation dose of 250 Gy, The overall response rate was 78% and the mean survival was 10 years. • The survival rate at 1, 3, 5, and 10 years after p32 therapy was 91%, 77%, 73%, and 52%, respectively.
- 31. Radiation LINAC • Linear Accelarator(external beam RT) • TruBeam • CyberKnife Stereotactic radiosurgery • GammaKnife Proton Beam • Uses charged particles rather than photons to deliver high doses of radiation to the target volume while limiting the "scatter" dose received by surrounding tissues
- 32. Radiation therapy • 54Gy in 30 fractions • 6 weeks of treatment • Adverse events • Cyst enlargement • HCP • Pituitary failure (20-60% at 5 years) • Cognitive decline • Total dose temporal lobes • Radiation necrosis
- 33. Evidence favouring radiation •Study of 122 patients, looked at those treated with GTR, STR and STR + RT, and the primary endpoints were PFS and OS.
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- 36. Timing of radiation •Controversial • Immediate post-op (adjunctive) vs recurrence (salvage) • Another series noted control rate did not depend on timing of RT – DI and Visual impx (delayed > immediate) • General recommendation is immediate adjunctive therapy – Cautious in children (delay) because of the greater sensitivity to carcinogenic and adverse neurocognitive effects of RT
- 37. SUMMARY Surgery • Maximum saferesection is 1st line Rx • Cyst may require Ommaya reservoir • HCP may require shunt Radiation • No proven difference between GK, CK, LINAC • SBT + RT = GTR with low morbidity • Radiation may be delayed until recurrence vs immediately post op. Chemotherapy • Intracystic interferon-α, bleomycin or p32 Medical therapy • Hormone replacement
- 38. FUTURE DIRECTION • Targetedmolecular therapies • Genetic studies – CTNNB1 (B-catennin) : Adamantinomatous – BRAF : Papillary • Inhibitors of BRAF Proto-oncogene – Dabrafenib/Vemurafanib • MEK inhibitors – Trametinib • Multicenter phase-2 clinical trials at the National Cancer Institute are currently underway evaluating BRAF/MEK inhibition in the treatment of craniopharyngioma
- 39. CONCLUSION • Regardless ofthe therapeutic strategies that are utilized, it is evident that craniopharyngiomas continue to present a distinct challenge that still needs to be overcome. • Quality of life is a key consideration in this disease, and long-term follow up, involving a multidisciplinary team, is a necessary element of care of these patients.