Paeds Flashcards
What is the t(2;13) translation associated with
Alveolar rhabomyosarcoma
What is the t(11;22) translation associated with
Ewing’s sarcoma
What is a 17q gain associated with
What is the implication
Neuroblastoma
17q gain and N-Myc amplification are assoc with poor prognosis
Hyperdiploidy and triploidy are associated with better prognosis
What translocation is associated with alveolar rhabdomyosarcoma
t(2;13)
What translocation is associated with alveolar Ewings sarcoma
t(11;22)
What chromosomal gain is associated with neuroblastoma
17q gain
17q gain and N-Myc amplification are assoc with poor prognosis
Hyperdiploidy and triploidy are associated with better prognosis
From where do neuroblastomas arise
How do they present
Anywhere in the sympathetic nervous system (40% from adrenal)
Local pressure effect, systemic upset, paraneoplastic hypertension, diarrhoea (vasoactive intestinal peptide induced).
How are neuroblastomas risk staged
What does it take into account
International neuroblastoma pathology classification (INPC)
Age, n-myc copy number (poor prognostic marker), chromosomal abnormalities (17q), intra-spinal neuroblastoma >1/3 of spinal canal, pain, diarrhoea requiring NG/IVF, organ impairment
How are neuroblastomas treated
Systemic treatment - cyclophosphamide and vincristine
If high risk, add etoposide, carboplatin, doxorubicin
If very high risk, add cis-retinoic acid and high dose chemo
If intermediate or high risk, also add RT - 21Gy/14#
CTV = Pre-chemo tumour volume plus sites of residual disease +1-2cm
How is a Wilm’s tumour staged and when
International Society of paediatric oncology - post-chemo surgical evaluation, ie treat with neo-adjuvant chemo first.
What are the Wilm’s stages
Stage I - Localised to kidney
Stage II - Extends beyond the kidney but is completely resected
Stage III - Incomplete resection or nodal/peritoneal spread
Stage IV - Haematogenous spread or LN involvement beyond abdominopelvic region
Stage V - Bilateral renal tumours
How is a localised Wilm’s tumour treated
Biopsy, then 4wks neoadjuvant vincristine and dactinomycin
then surgery
then risk stratified post surgery
How is a metastatic (stage ≥4) Wilm’s tumour treated
Biopsy, then 4wks NA vincristine and dactinomycin, plus 2wks of doxorubicin
then surgery
then risk stratified post surgery
How are Wilm’s tumours classified post surgery
Low, intermediate and high risk, based on blasternal elements and presence of necrosis
What adjuvant treatment is given to a low risk Wilm’s tumour post-operatively
If stage 1 - no adjuvant treatment given
If stage ≥2 - vincristine and dactinomycin
What is given to an intermediate risk Wilm’s tumour postoperatively
Stage I & II - Post-op chemo: vincristine / dactinomycin
Stage III tumour - Post-op chemo: vincristine / dactinomycin AND flank RT
14.4Gy in 8# plus boost 10.8Gy/6# (25.2Gy/14# overall) to macroscopic residual disease
What is given to a high risk Wilm’s tumour post operatively
Stage I - Post-op chemo: vincristine/dactinomycin/doxorubicin
Stage II & III - post-op vincristine/dactinomycin/doxorubcin AND flank post-op RT 25.2Gy in 14#
Stage IV - as above plus RT to sites of metastatic disease
How is a bilateral Wilm’s tumour (stage 5) treated
Bilateral partial nephrectomy + chemo +/- RT
What are children with retinoblastoma at risk of in the future
Further tumours, specifically sarcoma
What is the treatment for retinoblastoma
Aim is to salvage the eye
Intra-vitreous or ophthalmic artery chemotherapy (melphalen)
Surgery (enucleation) if advanced
RT for salvage
What is the commonest liver tumour in patients <5
What marker is raised
What is the treatment
Hepatoblastoma, HCC is very rare.
AFP raised
Surgery and cisplatin +/- doxorubicin
No role for RT
How is ALL risk stratified
What investigation must be done
Low risk: 1 to <10 years old at dx, WCC <50 before treatment
High risk: all other patients
Bone marrow assessment
What is the treatment for ALL
Dexamethasone induction
Consolidation treatment - dex/vincristine/mercaptopurine/methotrexate (incl intrathecal) +/- asparaginase
What is the treatment for relapsed ALL
RT to sanctuary sites - CNS (whole brain RT - 24Gy/15#) and testis (24Gy/12# - orthovoltage or electrons)
If further relapse - stem cell transplant following total body irradiation (12Gy/6#)
When are protons indicated for rhabdomyosarcoma
How is it treated
PBT: Orbit, Parameningeal, H&N, Pelvis
Surgery followed by chemo dependent on risk
Low risk: vincristine and dactinomycin
Standard risk: vincristine, dactinomycin and ifosfamide with RT to the primary site unless complete excision at first surgery
High risk: vincristine, dactinomycin, ifosfamide, doxorubicin and RT to primary site
Where does medulloblastoma typically arise
How is it treated
Posterior fossa
Surgery with LP >14 days following
Adjuvant chemo - 4x vincristine/etoposide and cyclophosphamide/carboplatin alternating
CSA RT - 36Gy/20# with boost to posterior fossa to 55Gy
Avoid CSA in those <3yrs - opt for high dose chemo
How is an ependymoma treated
Surgery and adjuvant RT - 54Gy/30# or 59.4Gy/33#
If incomplete excision - vincristine, etoposide and cyclophosphamide +/- HD MTX
At what RT dose can azoospermia occur
2-3Gy, but recovers within 3yrs
Recovery less certain after 4-6yrs
>6Gy - high risk of sterility
TBI will sterilise
How is testosterone production affected by RT
Production declines >15Gy, falls to 0 at >24Gy
At what Rt dose does ovarian failure occur
Immediate ovarian failure occurs at 16.5Gy, but menopause brought forward at lower doses
What is the effect on the endometrium of RT
> 40Gy - loss of menstruation
20-30Gy - endometrial tissues hypertrophy and there is an increased risk of miscarriage
At what dose can hypothalamic and anterior pituitary function fail
> 24Gy
When is breast cancer monitoring indicated following RT as a child
NICE recommends annual mammogram to women who received >30Gy supra-diaphragmatic RT as a child
At what RT dose is sensineural deafness at risk
> 30-40Gy
