Neuro-oncology Flashcards
Genetic Syndromes
Neuroocctaneous Disorders
- Neurofibromatosis I/II
- Neurofibromin is the tumor supressor protein encoded by the NF1 gene on chromosome 17 (17q11.2)
- 1:4000
- Neuro fibromas
- axillary or inguinal freckling
- Cafe-au-lait spots
- skeletal abnormalities
- Lisch nodules
- optic nerve glioma
- gliomas and pheochromocytomas
- bilateral vestibular schwannomas
Epidemiology
18% brain in pediatric Adult-way less common 63% benign. 37% malignant 20-40% of all other cancers later devlop to a brain in mestases not cell phones genetic syndromes longetivity bad luck
Tuberous sclerosis
Epilepsy
brain tumours (astrocytomas, coritcal tumours, sub-edimal nodules)
benign tumours
surgery-hydrocephalus, seizures
medicine treatment-everlimus-mTOR inhibitor
Pediatric Brain Tumours-Epidemiology
20% of pediatric tumours most common solid tumours in children second only to leukomia incidence: 3-4/100000 about 200 new cases dianosied each year in caanada. 25% increase from 1973 to 1991. Age important Neonate-supratentorial less than 2 years-supratentorial 2-12 year infratentorial over 12 years-supratentorial
Neonate Tumours
Poor apgar scores, failure to thrive, intracranial hemorrhage, seizures (uncommon), large head circumference and hydrocephalus
Infant Tumours
Non specific, macrocephaly, diencephalic syndrome (thin skin, motor hyperactvity, abnormal alter, FTT, jittery)
Toddler
Headaches, nausea, particularly in morning emesis, gait abnormality, visual findings.
10-15% have a headache disorder so hard to tell if tumour or not
Adolscent and Adult
Localizing symptoms, ocular manifestations and visual changes, precocoous puberty, seizures (15%)
General Management
Surgery the goal is complete ressection
Radiotherapy-growth retardation, endocrine dysfunction, progressive decrease in IG, risk of secodnary tumour development, avoid in children less than 3
Chemotherapy-used to delay XRT
Medulloblastoma-General
20% of CNS tumours, 30% of p-fossa.
hydrocephalus-25% require a shunt after surgery
Gross total resection: flush with floor of 4th ventricle, cerebellar mutism-10-15% can last up to one year.
Chang Classification: Standard Rsik-Age greater than 3, residual tumour less than 1.5 cm3 and no dissemination (MRI and CSF cytology).High risk is less than three with residual tumour or a metastases.
Medullablastoma-4 types
- WNT-6q loss and CTNNB1 mutations. classic medulloblastomas. rarely ahve large cell/anaplastic features. primarily observed in older children, adolscents and adults. less common but very good outcomes. Good one to have
- SHH: PTCH1;PTCH2. 9q deletions. desmoplasic/nodular histology. Bimodal age distribution-children less than three and older adolscence and adults. Relatively favourable outcomes, but negatively affected by other molecular genetic changes. Good response to chemo
3) Group C: MYC amplification. Classic or large cell/anaplastic histology. frequenctly metastic at time of diagnosis. occur through childhood and more commonly in male (2:1). Variable prognosis: Follows Changs Classification. Not responsive to anything other than ressectoin?
4) Group D: CPK6 and MYCN amplification
Either classic or large cell anaplstic histology. Metastisias at presentation is common. Occurs throughput childgood and into adulthoof. prognosis is better than group 3 but not as good at WNT. Recurrence not depedent on extent of surgery
Pilocytic Atrocytoma
20% of pediatric CNS tumours, 30% of p-fossa tumours
70% oresent in children with mostly lss than 7 year olds
Gross total ressection IS VERY IMPORTANT.
XRT is not recommended unless recurrence or unresectale tumour
90% long term survival with GTR
Management of residual-observation, XRT or SRS for growth/recurrence. Cists
Brainstem Glioma
20% of pediatric CNS tumours, 30% of p-fossa tumours.
4 distinct subtypes:
1. Diffuse (pontine) 60-80%
2. Focal (tectal) increase recognition with MRI. Tectal glioma, enhancement or size (surgery)
3. Dorsally Exophytic-20%. Remove exophytic portion without entering brainstem.
4. Cervicomedulalry-radical excision early, prior to neuro deterioration.
XRT for diffuse tumours.
Ependymoma
10% of p-fossa tumours in children. 30% in children under 3.
5 year survival-about 50% of the time Improving with more aggressive treatment.
Factors: extent of resection, age, craniospinal dissemination, histopathology, location (spinal>supratentorial>indratentorial)
Have to take it all out. NOT SENTIVE TO ANYTHING AND AGGRESSIVE
Chemo is not effective-tumours releative slow gorwing but relentless.
PF-A-more comon in males, younger, higher grade and more invansive and chemo resistant, more likely to recur and higher mortality. Easier to get but more aggressive
PF-B-less invasive of the floor of the brainsteam, easier to resect. IF get all youre good to go but hard to get it all due to the placement.
Gross total ressection in still important for overall survival in PF-A. extent of ressection and even radiation does not impact overall survival for PF-B.
Suprasellar Tumours
Craniopharyngioma, geminoma, astrocytoma, Pi
