Paediatric Oncology - Solid Tumours Flashcards
(46 cards)
information on retinoblastoma
common <5
RB1 inherited = screening
normally only affects one eye, treating both is difficult
99-100% survival
presentation for retinoblastoma
leukokoria (whiteness)
squint
inflammation/reddening of the eye
intraocular
within the eye
extraocular
spread to other tissues
treatment options for retinoblastoma
cyrotherapy
laser therapy
PBT = not localised in the retina
NO PHOTONS due to optic chiasm
eye removal [training to clean prosthesis, need to be changed over time]
brachytherapy [plaque inserted and left in place]
chemo: straight in eye (carbo or topotecan), systemic (doxorubicin)
post RT effects for retinoblastoma
bone formation changes (socket)
scarring
changes in eye lid and function
where is neuroblastomas common
adrenal gland and nerve tissue
where does neuroblastoma spread
lymphatics, liver bone, skin, BM (50%)
what is found in 20% of neuro pt
MYCN amplified
aggressive disease + poor prognosis (32% 5 year survival)
what is the range of neuroblastomas
spontaneous regression (50%) to poorly differentiated + met (50%)
what tests are carried out for neuroblastoma
urine sample: rule out infection
CT/MRI/US
biopsy
miBG (iodine- 131)
PET-CT
bone marrow biopsy (has it spread into the blood)
what are the symptoms for neuroblastoma
swollen, painful tummy, constipation, difficulty peeing
breathlessness + difficulty swallowing
weakness in legs + unsteady walking
numbness in lower body
fatigue
pale skin
loss of appetite
weight loss
bone pain, a limp
general irritability
blueish lumps on skin and bruising particularly around the eyes
irregular eye movements, jerky muscular movements
neck lump
risks of getting a neuroblastoma
age: less than 18 months has a low risk
stage
what do the cells look like
gene changes
what does the MYCN gene indicate
aggressive tumour
encourages tumour growth
treatment for high grade neuroblastoma
12-18 month treatment
induction chemo
surgery
high dose chemo
stem cell
RT
immunotherapy
staging for neuroblastoma
L1 = one place, NOT spread can be removed with surgery
L2 = one place, NOT spread, CANT be removed with surgery
M = spread to other parts of the body
Ms = spread to skin, liver or BM
staging will continuously happen as staging determines the trt
what is the RT for neuroblastoma
post op RT to tumour bed
21Gy in 14, 2.5 weeks
21.6Gy in 12, 2.5 weeks
what is the general diagnostic tests for these type of tumours
CT
MRU
US
biopsy: scar must be close to the tumour, which is included in the RT field
why is a biopsy done
only way to stage
the reason why the scar is in the field is due to the high risk of seeding
wilms tumour
nephroblastoma
5/100 have a birth defect
diagnosed mainly <4
only 10% have bi involvement
what is surgery based off for a wilms tumour
risk of rupture vs benefit (not advised < 6months)
what is the SIP UMBRELLA TRIAL for a wilms tumour
induction chemo to reduce surgical mortality ALMOST all children have 4 weeks of induction chemo
chemo (actinomycin)
nephrectomy (6 wks after)
pathological staging following removal
post op chemo (vincristine and actinomycin)
RT
what is the risk of a wilms tumour based off
pathology following removal
syndromes related to a wilms tumour
denys drash - affects kidneys + genetalia (born without it)
beckwith wiedmann (swelling and enlargement)