Paeds

Question 1

What is the t(2;13) translation associated with

Answer 1

Alveolar rhabomyosarcoma

Question 2

What is the t(11;22) translation associated with

Answer 2

Ewing’s sarcoma

Question 3

What is a 17q gain associated with

What is the implication

Answer 3

Neuroblastoma

17q gain and N-Myc amplification are assoc with poor prognosis
Hyperdiploidy and triploidy are associated with better prognosis

Question 4

What translocation is associated with alveolar rhabdomyosarcoma

Answer 4

t(2;13)

Question 5

What translocation is associated with alveolar Ewings sarcoma

Answer 5

t(11;22)

Question 6

What chromosomal gain is associated with neuroblastoma

Answer 6

17q gain

17q gain and N-Myc amplification are assoc with poor prognosis
Hyperdiploidy and triploidy are associated with better prognosis

Question 7

From where do neuroblastomas arise
How do they present

Answer 7

Anywhere in the sympathetic nervous system (40% from adrenal)

Local pressure effect, systemic upset, paraneoplastic hypertension, diarrhoea (vasoactive intestinal peptide induced).

Question 8

How are neuroblastomas risk staged

What does it take into account

Answer 8

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

Question 9

How are neuroblastomas treated

Answer 9

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

Question 10

How is a Wilm’s tumour staged and when

Answer 10

International Society of paediatric oncology - post-chemo surgical evaluation, ie treat with neo-adjuvant chemo first.

Question 11

What are the Wilm’s stages

Answer 11

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

Question 12

How is a localised Wilm’s tumour treated

Answer 12

Biopsy, then 4wks neoadjuvant vincristine and dactinomycin
then surgery
then risk stratified post surgery

Question 13

How is a metastatic (stage ≥4) Wilm’s tumour treated

Answer 13

Biopsy, then 4wks NA vincristine and dactinomycin, plus 2wks of doxorubicin
then surgery
then risk stratified post surgery

Question 14

How are Wilm’s tumours classified post surgery

Answer 14

Low, intermediate and high risk, based on blasternal elements and presence of necrosis

Question 15

What adjuvant treatment is given to a low risk Wilm’s tumour post-operatively

Answer 15

If stage 1 - no adjuvant treatment given
If stage ≥2 - vincristine and dactinomycin

Question 16

What is given to an intermediate risk Wilm’s tumour postoperatively

Answer 16

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

Question 17

What is given to a high risk Wilm’s tumour post operatively

Answer 17

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

Question 18

How is a bilateral Wilm’s tumour (stage 5) treated

Answer 18

Bilateral partial nephrectomy + chemo +/- RT

Question 19

What are children with retinoblastoma at risk of in the future

Answer 19

Further tumours, specifically sarcoma

Question 20

What is the treatment for retinoblastoma

Answer 20

Aim is to salvage the eye
Intra-vitreous or ophthalmic artery chemotherapy (melphalen)
Surgery (enucleation) if advanced
RT for salvage

Question 21

What is the commonest liver tumour in patients <5
What marker is raised
What is the treatment

Answer 21

Hepatoblastoma, HCC is very rare.
AFP raised
Surgery and cisplatin +/- doxorubicin
No role for RT

Question 22

How is ALL risk stratified
What investigation must be done

Answer 22

Low risk: 1 to <10 years old at dx, WCC <50 before treatment
High risk: all other patients

Bone marrow assessment

Question 23

What is the treatment for ALL

Answer 23

Dexamethasone induction
Consolidation treatment - dex/vincristine/mercaptopurine/methotrexate (incl intrathecal) +/- asparaginase

Question 24

What is the treatment for relapsed ALL

Answer 24

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#)

Question 25

When are protons indicated for rhabdomyosarcoma How is it treated

Answer 25

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

Question 26

Where does medulloblastoma typically arise How is it treated

Answer 26

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

Question 27

How is an ependymoma treated

Answer 27

Surgery and adjuvant RT - 54Gy/30# or 59.4Gy/33# If incomplete excision - vincristine, etoposide and cyclophosphamide +/- HD MTX

Question 28

At what RT dose can azoospermia occur

Answer 28

2-3Gy, but recovers within 3yrs Recovery less certain after 4-6yrs >6Gy - high risk of sterility TBI will sterilise

Question 29

How is testosterone production affected by RT

Answer 29

Production declines >15Gy, falls to 0 at >24Gy

Question 30

At what Rt dose does ovarian failure occur

Answer 30

Immediate ovarian failure occurs at 16.5Gy, but menopause brought forward at lower doses

Question 31

What is the effect on the endometrium of RT

Answer 31

>40Gy - loss of menstruation 20-30Gy - endometrial tissues hypertrophy and there is an increased risk of miscarriage

Question 32

At what dose can hypothalamic and anterior pituitary function fail

Answer 32

>24Gy

Question 33

When is breast cancer monitoring indicated following RT as a child

Answer 33

NICE recommends annual mammogram to women who received >30Gy supra-diaphragmatic RT as a child

Question 34

At what RT dose is sensineural deafness at risk

Answer 34

>30-40Gy