Paediatric Oncology - Brain + CNS Flashcards
who is a child
a person under the age of 18
paediatric age
0-16
young person age
16-25
children + young adult age
0-25
peak age
0-4
how many cases every year
1,838
incidence increase since the early 1990s
12%
what is the five year survival rate
84%
risk factors
genetics [retinoblastoma RT1 NF2]
previous cancer trt
exposure to radiation
exposure to infection [EPV]
issues with development in the womb
medical conditions
what is NF1
skin pigmentation
multiple tumours along the spine
intellectual impairment
growth disorder
what effects are more prevalent
late
is there lower or greater tolerances
lower tolerances to RT - significant doses are needed + large field
combines RT with surgery/chemo as ++aggressive
what are the RT margins based off
diagnosis to prevent recurrence
whos involved in the care
SLT
surgeon
play therapists
play specialists
anathetises
neurologists
CNS
nurse specialist
physio
endocrinologist [for life]
what are the tests for a CNS cancer
MRI
CT
lumbar puncture
blood test
biopsy
tissue banking
why is tissue banking done
high risk of recurrence rates need to see if its from the original tumour genetics or a new primary
what are the tumour types
glial cells
embryonal cells
what are the glial cell types
astrocytoma
ependymoma
oligodendroglioma
what are the embryonal cell types
medulloblastoma
PNET [primitive neuro]
what category are they placed in
cat 1
how are they graded
using the WHO classification
high grade
fast growing
agressive
likely to infiltrate into brain tissue, damage the surrounding areas
symptoms
change in head circumference
seizures/fits
loss of balance/coordination
walking problems [dysmetria/ chorea]
abnormal eye movements
learning difficulties
sight issues [loss of vision or double vision]
behaviour changes
persistent vomiting
current headaches
abnormal head position: wry neck+ stiff head or neck
what is the most common type
astrocytoma
what is a focal astro
clear boundaries between tumour and brain tissue
what is a diffuse astro
filter out into the brain can’t distinguish between the tissue
what can surgery be used for
low grades
all tissue is removed
what is the trt for high astro
+/- targeted cancer therapy [bevauizamab]
what is the dose for an astro
54Gy in 30
when is chemo given over RT
for under 2 until they are grown enough for RT
what is the SIIOP II trial for ependymoma
chemo and extended RT
what is the trt for ependymoma
surgery and RT
if the ependymoma tumour is localised what can be given
PBT - 42 days [28 ideal]
what is the RT regime for ependymoma
phase 1: whole CNS
36Gy in 20 (>3)
25Gy in 20 (<3)
phase 2: tumour bed boost + boost to met sites
up to 59.4Gy in 33
(50.4Gy for spinal mets)
what do we need to know about HGG
biological markers
what is the regime for HGG dependent on
stage, age at time of RT
chemo for HGG
vincristine, cyclophosphamide, methotextrate
what treatment is given for HGG
surgery [as much as a complete resection as possible], chemo, RT
what are the different types of HGG
IDH wildetype, H3 wildetype, diffuse type,
e.g diffuse hemispheric glioma H3 G34 mutant
when should post-op RT for HGG be?
no later than 4-6 wks for post maximal safe resection
what is the dose for HGG
54-59.4Gy
can you give more than one fraction a day for a HGG
NO
slow cell growth, slow radiobiology
describe the GTV, CTV, PTV for the HGG
GTV: resection cavity and residual
CTV: GTV + 1.5-2cm margin
PTV: 3-5mm margin expansion
where are the craniopharyngioma’s found
in the pituitary
how do pt with craniopharyngioma present
personality changes
what is the trt for craniopharyngioma
surgery + RT [ideally protons]
what are the doses which can be given for craniopharyngioma
50.4Gy in 28
54Gy in 30
50Gy in 25
who will pt see if they have a craniopharyngioma
endocrinologist, might need HRT
give me information on choroid plexus carcinoma
highly vascular lesion
median age: 2 years
chemo often to delay RT
what is the chemo given for choroid plexus carcinoma
etoposide, carbo-platin, cyclophosphamide
where does a medulloblastoma originate from
post fossar cerebellum which are undeveloped brain cells which become malignant
where is a PNET found
anywhere else
how can you identify a medulloblastoma or PNET
biological mark up
what is the trt for a medulloblastoma
surgery, chemo and RT
very aggressive tumour eventhough it is radiosensitive
what is the trt for a standard risk patient with a medulloblastoma or PNET
standard chemo + RT
what is the trt for a low risk patient with a medulloblastoma or PNET
lower dose
what are the subgroups of medulloblastoma or PNET
group 3 (41%)
sonic hedgehog (41%)
group 4 (17%)
what are medulloblastoma/PNET split into two phases
radiosensitive but very aggressive might start to metastases
tend to be in the high risk category so boost prevents recurrence
high met spread (including to the spine)
what is the two phase doses for medulloblastoma/PNET
phase 1: 23.4Gy in 13 (whole CNS)
phase 2: bed boost 30.6Gy in 17
whole CNS due to high chance of met spread
what is intracranial germ cell
germ cells which are left behind with development issues in the womb
relapse tends to occur in ventricles
what is the trt for intracranial germ cell
surgery and chemo
what is mengioma
it is a tumour found in the meninges which uses complex field shapes when treating
what are the OARS
ovaries
kidneys
BS (<54Gy)
optic chiasm
lung
cochlea
parotid gland
pituitary gland
heart
testes
