Paediatric Oncology - Solid Tumours Flashcards
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)
stage 1 of a WT
only affects kidneys
4 weeks chemo + surgery
stage 2 of a WT
spread to nearby structures
4 weeks chemo + surgery + 27 weeks chemo (high risk = + RT or 7 extra weeks of chemo)
stage 3 of a WT
incomplete resection/lymph involvement/burst/lining of the abdomen
4 weeks chemo + surgery + 27 weeks chemo (high risk = + RT or 7 extra weeks of chemo)
stage 4 of a WT
distant spread (common = lungs)
stage 5 of a WT
tumour in both kidneys, each tumour is staged differently
what is treatment of a WT determined
how fit the pt is
why do we want to irradiate as less as possible
to minimise secondary cancer
what will the dose be for a pt with lung mets from their WT who dont respond well to chemo
whole lung RT
12Gy in 8 [intermediate]
15Gy in 10 [high]
what are the types of sarcoma
ewings sarcoma: leg, pelvis, arms, ribs
rhabomyosarcoma: H&N [70% survival]
oestosarcoma: common in TYA, 65% around the knee, 20% present with mets
sarcoma details
peak at TYA
previous RT
treated with alkylating agents
pagent disease
sarcoma symptoms
swelling/physical lump
broken bone
severe fatigue
pain worse at night
changes in mobility
sarcoma diagnosis
whole body MRI
PET-CT
bone scan (not enough as doesn’t show mass)
biopsy to confirm type
RT for rhabdomyosarcoma
50.4Gy in 24
RT for ewings sarcoma
surgery followed by RT
chemo due to high risk of mets
vincristine doxorubicin cyclophosphamide, ifosphomide etoposide
alternate two weekly for 14 cycles
pre op - 50.4Gy in 28
post op - 54Gy in 30
whole lung RT
<14 years: 15Gy in 10
>14 years 18Gy in 12
POP
RT for limbs
3DCRT is used NOT VMAT
2 phases to prevent lymphodema
difficult to treat complex shapes with CRT
field in field wedges
when can brachytherapy be used
sarcomas caught very early
vaginal vault and prostate rhabdomyosarcoma
doses for brachy
day 1: CT/dosim + 1st fraction
day 2: 2 fractions 6 hours apart
day 3: 2 fractions 6 hours apart
27.5Gy in 5 NO ENERGY
what are the challenges of brachy
cant walk
invasive
fertility issues
fit enough for GA
pain mediciation is needed
high risk of infection
other modalities used to treat sarcomas
gamma knife
cyber knife
tomotherapy: cant treat large fields
MRI linacs: must have an anaesthetic pack MRI compatible
treatment for hepatoblastoma
surgery + chemo
treatment for melanoma
mostly in TYA so drug trt
treatment for a pancreatic tumour
no guidance approx 30-40
