Histopathology 18: Neuro-oncology Flashcards
List 4 genetic syndromes responsible for primary CNS tumours ?
Neurofibromatosis 1 Neurofibromatosis 2 Tuberous Sclerosis 1 Tuberous Sclerosis 2 Turbot’s syndrome
Outline the WHO grading of CNS tumours ?
H
What type of tumours are the most common primary CNS tumours ?
Diffuse Gliomas
Give 2 examples of Glial tumours in adults ?
Astrocytoma
Oligodendrogliomas
What’s the most common glial tumour in children ?
Pilocytic astrocytoma
What are the typical histological features of Pilocytic Astrocytomas ?
Piloid (hairy) cells
Rosenthal fibres
Which tumours do astrocytomas turn into after about 5-7 years ?
Glioblastomas
It is a Grade 4 astrocytoma
What mutation is most commonly seen in astrocytomas ?
IDH2
Describe a typical feature of oligodendroglioma on histology ?
Fried eggs- Round cells with clear cytoplasm
Which genetic syndrome is associated with meningiomas ?
Neurofibromatosis 2
Where are medulloblastomas always found ?
The cerebellum
List some symptoms of medulloblastoma ?
ICP symptoms- morning headaches, nause and vomiting, blurry vision
Loss of balance and coordination
Abnormal eye movements
List 3 typical features of medulloblastoma on histology ?
Small blue round cells
Wright rosettes
Neuronal marker Synaptophysin positive
Where are metastatic brain tumours most commonly found ?
The grey-white junction (where grey and white matter meet)
A ventricular tumour presenting with hydrocephalus.
Most likely diagnosis ?
Ependymoma
A soft gelatinous, calcified tumour.
Most likely diagnosis ?
Oligodendroma
List 3 features of von hippel-Lindau syndrome ?
- Hemangioblastoma
- Renal cysts
- Phaeochromocytoma
List 5 features of Tuberous sclerosis
- Epilepsy
- Learning difficulties
- Skin: Ash leaf spots, angiofibromas, shagreen patches
- Neuro: Giant cell astrocytoma, cortical tubers
- Renal: Angiomyolipomas
- Cardio: Rhabdomyomas
List 3 features of Neurofibromatosis type 2
- Bilateral Schwannomas -Affect CNVIII causing hearing loss, tinitus
- Meningiomas
- Cataracts
List 5 features of Neurofibromatosis type 1
- Axillary freckling
- Cafe au lait spots
- Neurofibromas
- Lisch nodules
- Optic glioma
difference between extra-axial and intra-axial CNS tumours
extra-axial = coverings
tumours of bone, cranial soft tissue, meninges, nerves and metastatic deposits
intra-axial = parenchyma
glia, neurons, vessels, connective tissue
derived from other cell types (meets, lymphomas)
usual inheritance of CNS tumours
autosomal dominant
presentation of CNS tumours
supratentorial = focal neurological deficit, seizures, personality changes
subtentorial = cerebellar ataxia, long tract signs, cranial nerve palsy
what chemo is used in high grade gliomas
temozolamide
features of glial tumours
IDFFUSE INFILTRATION adults grades >2 astrocytomas or oligodendroglioma IDH1/2 mutation
CIRCUMSCRIBED GLIOMAS children grades 1-2 pliocytic astrocytomas MAPK mutation
features of pliocytic astrocytomas
grade 1 children NF 1 MRI - well circumscribed, cystic, enhanced lesion cerebellar piloid cells and rosenthal fibres BRAF mutation
features of astrocytomas
grade 1-4 cerebral hemispheres common sites malignant progression become glioblastomas IDH2 mutation
features of glioblastoma multiforme
grade 4
>50 yrs
microvascular proliferation and necrosis
wildtype IDH mutation
features of oligodendromas
grade 2-3
round cells with clear cytoplasm (fried egg)
IDH 1/2 mutations, co-deletion of 1p/19q
good prognosis
features of meningiomas
grade 1-3
can be multiple in NF2
focal symptoms
brain invasion
featres of medulloblastomas
grade 4 embryonal tumour cerebellum always 2nd most common brain tumour in children wright rosettes and synaptophysin
features of CNS mets
multiple
lung, breast, melanoma
poor prognosis
seen at grey-white junction
what can be used in future to classify CNS tumours
look at different patterns of DNA methylation
