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FRCR2 > Paeds > Flashcards

Paeds Flashcards

1
Q

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

A

Alveolar rhabomyosarcoma

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2
Q

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

A

Ewing’s sarcoma

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3
Q

What is a 17q gain associated with

What is the implication

A

Neuroblastoma

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

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4
Q

What translocation is associated with alveolar rhabdomyosarcoma

A

t(2;13)

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5
Q

What translocation is associated with alveolar Ewings sarcoma

A

t(11;22)

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6
Q

What chromosomal gain is associated with neuroblastoma

A

17q gain

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

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7
Q

From where do neuroblastomas arise
How do they present

A

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

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

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8
Q

How are neuroblastomas risk staged

What does it take into account

A

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

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9
Q

How are neuroblastomas treated

A

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

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10
Q

How is a Wilm’s tumour staged and when

A

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

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11
Q

What are the Wilm’s stages

A

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

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12
Q

How is a localised Wilm’s tumour treated

A

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

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13
Q

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

A

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

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14
Q

How are Wilm’s tumours classified post surgery

A

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

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15
Q

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

A

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

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16
Q

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

A

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

17
Q

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

A

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

18
Q

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

A

Bilateral partial nephrectomy + chemo +/- RT

19
Q

What are children with retinoblastoma at risk of in the future

A

Further tumours, specifically sarcoma

20
Q

What is the treatment for retinoblastoma

A

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

21
Q

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

A

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

22
Q

How is ALL risk stratified
What investigation must be done

A

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

Bone marrow assessment

23
Q

What is the treatment for ALL

A

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

24
Q

What is the treatment for relapsed ALL

A

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#)

25
Q

When are protons indicated for rhabdomyosarcoma

How is it treated

A

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

26
Q

Where does medulloblastoma typically arise
How is it treated

A

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

27
Q

How is an ependymoma treated

A

Surgery and adjuvant RT - 54Gy/30# or 59.4Gy/33#

If incomplete excision - vincristine, etoposide and cyclophosphamide +/- HD MTX

28
Q

At what RT dose can azoospermia occur

A

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

29
Q

How is testosterone production affected by RT

A

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

30
Q

At what Rt dose does ovarian failure occur

A

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

31
Q

What is the effect on the endometrium of RT

A

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

32
Q

At what does can hypothalamic and anterior pituitary function fail

A

> 24Gy

33
Q

When is breast cancer monitoring indicated following RT as a child

A

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

34
Q

At what RT dose is sensineural deafness at risk

A

> 30-40Gy

FRCR2 (28 decks)

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