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Pediatrics Flashcards

1
Q

Ddx for posterior fossa mass

A

Brainstem glioma

Medulloblastoma

Ependymoma

Astrocytoma (JPA)
ATRT

Hemangioblastoma

Mets

Lymphoma

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2
Q

DDx of suprasellar mass

A

Craniopharyngioma

Optic glioma

Pituitary adenoma

Germ cell tumor

Abcess

Meningioma

Ependymoma

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3
Q

Small round blue cell tumors

A
  • LEARN MR
    • Lymphoma
    • Ewings
    • ALL
    • RMS
    • Neuroblastoma
    • Medulloblastoma
    • Retinoblastoma
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4
Q

What is one way to avoid scoliosis

A

Don’t irradiate just part of vertebral body

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5
Q

Parinaud Syndrome

A

Impaired upward gaze

Pseudo-Argyll Robertson pupil (accomodates but does not react)

Convergence nystamus

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6
Q

What lesions associated with Parinaud syndrome

A

Pineal glad tumor or dorsal midbrain lesion

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7
Q

What is the most important management thing to state for pediatric patients?

A

Treat kid on protocol if available

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8
Q

For pediatric CNS workup, how to approach diagnosis of PF lesion

A

Do not biopsy as this risks herniation –> go for maximal safe resection

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9
Q

How to approach mgmt of PF mass if surgery not immediately available?

A

Shunt

ONLY if surgery not immediately available

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10
Q

Other workup of CNS lesions?

A

Neuro exam (CN, motor, sensory, cerebellar, Parinaud syndrome, fundoscopic exam)

CT and MRI of the brain and spine (through S4)

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11
Q

When should postop brain be done

A

Within 48 hours of surgery

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12
Q

When should LP be done if not prior to surgery

A

14 days post op

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13
Q

Treatment of HGG

A

Put on protocol

  • Maximally safe resection
    • If <3: chemo alone (vincristine, cyclophosphamide)
    • If >3: RT
      • If anaplastic oligo, consider adjuvant PCV
      • If GBM, consider concurrent and adjuvant TMZ
  • If unresectable: chemoRT
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14
Q

what is HGG RT dose

A

59.4 in 33 fractions

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15
Q

How does TMZ compare for kids vs adults

A

TMZ data not as good for kids

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16
Q

Contouring for pediatric HGG

A
  • CTV1 is contrast enhancing + T2 flair + 1.5 cm expansion –> 46 Gy
  • CTV2 is T1post + 1.5 cm –> 60 Gy
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17
Q

Where do pediatric ependymomas form

A

posterior fossa (60%)

Suprtentorial (30%)

Spinal cord (10%)

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18
Q

What is the treatment of anaplastic ependymoma

A
  • Maximal safe resection
  • Adjuvant RT to tumor bed alone
  • No chemo
  • Consider CSI if +LP or +MRI
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19
Q

Dose of RT for ependymoma

A
  • Tumor bed
    • 59.4 if age >3
    • 54 for children 1-3 years of age
  • Spinal cord
    • 36 Gy CSI for children > 3
    • Consider focal boosts to 45 Gy if gross disease
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20
Q

Most important prognostic factor for ependymoma

A

extent of resection

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21
Q

Contouring for ependymoma

A
  • GTV is surgical target and residual disease
  • CTV is GTV + 1 cm
  • PTV is CTV + 0.5 cm
  • Treat to 59.4 Gy
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22
Q

Typical age range of ATRT

A

<3 years old

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23
Q

Treatment of ATRT

A

Maximal safe resection

Chemotherapy (vincristine, MTX, cis, etop, cyclophosphamide x2 then carbo/thiotepa x3)

Adjuvant RTto 54 Gy

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24
Q

What staging/workup should occur for all pediatric brain pts

A

MRI brain, MRI spine (either preop or within 2 weeks)

LP (either preop or 14d postop)

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25
Q

Send kids with CNS tumors for what testing

A
  • Neurocog/IQ testing
  • Audiology
  • Genetics if syndromic tumor
  • Endocrine consult
  • Fundoscopic and eye exam with VF testing
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26
Q

Mgmt of LGG

A

Principally maximall safe resection

RT for recurrence

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27
Q

When should RT be used for pediatric LGG

A

If recurrent after resection

If bx or STR and patient asymptomatic

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28
Q

Dose of RT for LGG

A

54 Gy in 30

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29
Q

Contouring of LGG

A

GTV= FLAIR residual disease

CTV= GTV+0.5 cm

PTV=5 mm

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30
Q

Describe JPA

A

Large cystic mass of cerebellum, hypothalamus, brainstem, usually benign and slow growing

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31
Q

JPA is associated with what syndrome

A

NF1

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32
Q

What is path hallmark of JPA

A

rosenthal fibers

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33
Q

Treatment of JPA

A
  • Surgery is preferred
    • If GTR –> no RT
    • If STR –> can consider RT or obs
  • If unresectable
    • 3rd ventriculostomy for hydrocephalus
    • Chemo (vincristine/carbo) preferred
    • Consider RT if symptomatic, not responding, recurrent
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34
Q

Dose of RT for JPA

A

50.4 Gy in 28 fractions

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35
Q

Contouring for JPA

A

Get kid on trial!

GTV= residual T1post and T2 flair changes

CTV=GTV + 5mm

PTV = 3-5 mm

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36
Q

What is outcome for JPA

A

5 year OS > 90%

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37
Q

How to describe low grade ependymoma

A

Calcs and cysts are common as well as descent through the foramen magnum (tongue of tumor)

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38
Q

What is G1 ependymoma

A

subependymoma

myxopapillary

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39
Q

What is G2 ependymoma

A

classic ependymoma

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40
Q

what is path hallmark of low grade ependymoma

A

perivascular pseudorosettes

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41
Q

Most important prognostic factor for ependymoma

A

extent of resection

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42
Q

Mgmt of intracranial ependymoma

A
  • Maximal safe resection
    • STR - neoadjuvant chemo –> second look surgery
    • Grade I/GTR - maybe ok to observe (but high rate of recurrence)
    • Grade II/III GTR - adjuvant RT
    • Check for spine disease
      • If CSF+: 36
      • If gross diease: boost to 45
    • If <3 yo, chemo
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43
Q

Dose of RT for ependymoma

A

If GI/GII: 54 Gy

If GIII: 59.4 Gy

For CSI: 36 Gy boost gross disease to 45

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44
Q

Mgmt of spinal ependymoma

A
  • Gross total resection
    • Obs for GTR and maybe myxopapillary
    • Adjuvant RT for other situations
      • 45 Gy to 2 vert bodies above and below
      • Boost gross disease to 50.4 if cord, 59.4 if conus or cauda
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45
Q

Contouring for ependymoma

A

GTV = gross disease and tumor bed

CTV = GTV + 1.5

PTV = 3-5 mm

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46
Q

Most common location for brainstem glioma

A

pons (70%)

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47
Q

Most common form of brainstem glioma

A

DIPG - diffuse intrinsic pontine glioma

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48
Q

Mutation a/w brainstem glioma

A

H3 K27M

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49
Q

How to diagnose a DIPG

A

Typically a radiographic diagnosis but more recently a push to do biopsies to see if that can guide molecularly targeted therapies

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50
Q

What has been the only thing shown to improve OS for brainstem glioma

A

RT

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51
Q

Dose for DIPG

A

54 Gy in 30 fractions

Use photons as no advantage to protons

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52
Q

What chemo is used for DIPG

A

Nothing routine

There are trial testing various agents

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53
Q

Dose of reRT for DIPG

A

Consider additional 36-45 Gy

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54
Q

Median survival for DIPG

A

6-12 mos

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55
Q

What is the contouring for DIPG?

A

CTV = GTV + 1 cm

PTV = CTV+3 mm

Treat to 54 Gy

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56
Q

Workup for optic glioma

A
  • H&P
  • CT or MRI
  • Labs including HCG, AFP, endocrine labs
  • Ophthalmologic exam
  • Genetics referral
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57
Q

Should bx be performed of optic glioma

A

Only if diagnosis uncertain

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58
Q

Management of optic gliomas

A
  • Surgery - organ preservation if possible and maximally safe debulking
  • Chemo (carboplatin and vincristine)
  • RT - effective local control and prevents worsening of vision loss and deficits from POD
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59
Q

Which optic glioma patients should get chemo alone

A
  • Children <3
  • Children with NF1 due to 2nd malig risk
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60
Q

Dose of RT for optic glioma

A

54 Gy

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61
Q

Target for optic glioma

A

Optic nerve, chiasm, optic tracts

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62
Q

Median age for medulloblastoma

A

Median age 5-6

20% occur before 2 year

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63
Q

What are the path features which should be considered for medullo?

A

Anaplasia

Histologic subtype

Molecular group

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64
Q

Workup for medulloblastoma

A
  • MRI brain
  • MRI spine if possible
  • Labs - CBC, CMP, endocrine labs
  • Baseline neurocog testing
  • Audiology
  • Ophthalmology with VF testing

DO NOT DO UPFRONT CSF DUE TO RISK OF HYDRO

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65
Q

Management of medulloblastoma

A
  • Start steroids
  • Consider maximally safe resection (if not possible or unsafe, proceed to VP shunt placement)
  • Post op MRI in 48 hrs
  • CSF and MRI spine 10-14 days later
  • Determine if standard risk or high risk
    • RT with weekly vincristine
    • Possibly adjuvant chemo
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66
Q

When should medullo RT start after surgery

A

23-30 days post op

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67
Q

Total treatment duration for medulloblastoma

A

55 weeks

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68
Q

What is best prognosis molecular subtype of medulloblastoma

A

WNT, followed by SHH

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69
Q

What is poorest prognosis medulloblastoma subtype

A

Group 3 (frequently M+, Myc+)

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70
Q

How is medulloblastoma staged?

A

Chang staging, focusing on M

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71
Q

M0 medulloblastoma

A

No CSF or hematogeneous mets

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72
Q

M1 medulloblastoma

A

Microscopic disease in CSF

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73
Q

M2 medulloblastoma

A

Gross nodular cranial seeding beyond the primary site

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74
Q

M3 medulloblastoma

A

Gross nodular SPINAL seeding

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75
Q

M4 medulloblastoma

A

Mets outside CNS (bone)

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76
Q

What are the clinical categories of medulloblastoma

A

standard risk (2/3)

high risk (1/3)

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77
Q

How is average risk defined

A

GTR or < 1.5 cm2 residual

Age >3

M0

No anaplasia on path

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78
Q

Treatment approach for average risk medulloblastoma

A
  • CSI to 23.4 in 13 fractions of 1.8 Gy
  • Involved field boost to 54 Gy in 30 fractions
  • Weekly vincristine
  • Adjuvant chemotherapy with 6-8 cycles of cisplatin backbone chemotherapy
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79
Q

How to define high risk medulloblastoma

A
  • STR or >1.5 cm2 residual
  • Age <3 months
  • M+
  • Anaplastic histology
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80
Q

Treatment of high risk medulloblastoma

A
  • CSI to 36 Gy in 20 fractions
  • Boost to entire posterior fossa to total dose of 55.8 Gy in 31 fractions
  • Weekly vincristine
  • Other scenarios
    • Boost additional brain disease to 55.8
    • If focal spine disease, boost to 45 if above conus, 50.4 if below conus
    • If diffuse spinal disease 39.6 Gy
  • Consolidation with 6-8 cycles of cis based chemo
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81
Q

Dose for medullo diffuse spinal disease

A

39.6 (1.8.x 22)

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82
Q

5 year EFS for standard risk medullo

A

80%

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83
Q

5 year EFS for high risk medullo

A

65%

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84
Q

What is the youngest patient eligible for CSI?

A

age 3+

(catastrophic neurocognitive changes and patients can’t live independently if getting CSI before that age)

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85
Q

Strategy to try to bridge time to CSI for young children

A

Chemotherapy to help reserve RT for salvage

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86
Q

Posterior fossa syndrome

A
  • Child awakes from surgery fine
  • 1-4 days post op develops
    • Hypotonia
    • Ataxia
    • Mutism
    • Emotional lability
    • Irritability
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87
Q

How many children get PF syndrome

A

30%

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88
Q

What share of kids with posterior fossa syndrome get better?

A

20%

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89
Q

What is the treatment for posterior fossa syndrome

A

No specific treatment

Do not stop RT

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90
Q

Diseases which require CSI

A
  • Medulloblastoma
  • Pineoblastoma/ependymoblastoma
  • ATRT
  • M+ ependymoma
  • M+ germ cell (germinoma)
  • NGGCT
  • Supratentorial PNET
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91
Q

Contouring medullo tumor bed boost

A

GTV= tumor bed (T1post) + residual disease

CTV = GTV + 1.5 cm (respecting tentorium and bone)

PTV= 3-5 mm

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92
Q

What structure should be spared for medulloblastoma boost

A

cochlea

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93
Q

Cochlea constraint for kids

A

Dmax < 35

V30 < 50%

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94
Q

Pituitary constraint for kids

A

V35 < 50%

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95
Q

Most common locations for CNS germ cell tumors

A

Suprasellar

Pineal

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96
Q

What is the most common subtype of germ cell tumor?

A

germinoma (60%)

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97
Q

Serum marker profile of germinoma

A

mild elevation in hcg ok

normal AFP

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98
Q

DDx for a suprasellar or pineal mass

A

Germinoma or NGGCT

Pinealblastoma

Ependymoma

Craniopharyngioma

LGG

Hamartoma

Teratoma

LCH

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99
Q

Workup for germ cell tumor

A
  • H&P with evals from neuro, ophtho
  • MRI brain
  • Labs
    • CBC, CMP
    • AFP, bHCG, LDH (both serum/CSF)
    • TSH, GnRH, ADH, CRH
  • Biopsy if necessary
  • Referrals to audiology, neurocog testing, VF
  • Rule out testicular GCT - PEx and scrotal US
  • CSF and MRI spine 2 weeks post op (if not preop)
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100
Q

What is the surgical plan for germ cell tumors

A

If uncertain, bx is ok to confirm pure germinoma

Germinomas do not need resection

NGGCT will need resection

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101
Q

Treatment options for germinoma

A
  • NO SURGERY unless NGGCT
  • Combined modality therapy
    • Induction chemo of 2-4 cycles carbo-etop
      • If CR: whole ventricle to 21 Gy + boost to 9 Gy
      • If PR: second look surgery or WV
  • RT alone
    • Whole ventricle RT to 24 Gy
    • Boost to 45 Gy
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102
Q

RT approach if CR after induction chemo for Germinoma

A
  • Whole ventricle to 21 Gy in 1.5 Gy fractions (14 fractions)
  • Primary site boost to 9 Gy
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103
Q

RT approach if PR after induction chemo for Germinoma

A
  • Consider second look surgery to see if mature component
  • Whole ventricle to 24 Gy in 1.5 Gy fractions (16)
  • Primary site boost to 12 Gy (8)
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104
Q

RT approach if no induction chemo for Germinoma

A
  • No surgery
  • Whole ventricle to 24 Gy in 1.5 Gy fractions
  • Tumor boost to 45 Gy in 1.5 Gy fractions
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105
Q

What is a bifocal germ cell tumor

A

Tumor in suprasellar and pineal regions synchronously

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106
Q

Treatment approach for bifocal germ cell tumor

A

DO NOT NEED CSI

Whole ventricle to 24 Gy

Boost both sites to 45 Gy

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107
Q

Trilateral germ cell tumor

A

Retinoblastoma

Pineal

Suprasellar

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108
Q

5 year OS for germinoma

A

>90%

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109
Q

Whole ventricle contouring

A

Lateral ventricles, 3rd vent, 4th vent and pre-pontine cistern expanded by 1 cm for CTV

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110
Q

Contouring for tumor boost germ cell tumor

A

Fuse T1post and T2 MRI

contour pre-chemo disease and do 1.5 cm expansion

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111
Q

Markers for choriocarcinoma

A

Very high hcg

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112
Q

Markers for yolk sac tumor

A

elevated AFP

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113
Q

What is important prognostic consideration for NGGCT

A

extent of resection, though not always possible given anatomy

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114
Q

Treatment strategy for NGGCT

A
  • Maximally safe resection
  • Chemotherapy
    • carbo/etop alternating with ifos/etop for 4-6 cycles
  • Second look surgery if not in CR
  • RT
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115
Q

What is the RT approach for NGGCT

A

CSI + primary boost

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116
Q

RT strategy for NGGCT

A

CSI to 36 Gy (20 fractions)

Primary site boost to 54 Gy (tumor + 1 cm)

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117
Q

Location of prepontine cistern

A
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118
Q

Where does craniopharyngioma arise?

A

Rathke pouch

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119
Q

Radiographic hallmarks of craniopharyngioma

A

Distinct due to cysts and califications

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120
Q

What is the workup for craniopharyngioma

A
  • H&P with full neuro and visual exam
  • Labs: CBC, CMP, bHCG, AFP, LDH, endocrine panel
  • MRI of brain with sellar protocol
  • MRI spine if able and CSF if able prior to surgery, if not 10-14d postop
  • Neurocog testing, audiology, VF, IQ
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121
Q

Initial management of craniopharyngioma

A
  • Maximal safe resection via transsphenoidal approach
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122
Q

Next step if TSS for craniopharyngioma is GTR

A

Observation

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123
Q

Next step if TSS for craniopharyngioma is STR or bx

A

Radiation to dose of 54 Gy in 30 fractions of 1.8 Gy

UNLESS CHILD IS LESS THAN 3

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124
Q

What is recurrence of subtotally resected craniopharyngioma

A

50%

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125
Q

Contouring for craniopharyngioma

A
  • GTV = residual disease including cysts
  • CTV = GTV + 1 cm (respecting anatomic boundaries)
  • PTV = CTV + 3 mm with daily CBCT
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126
Q

What is control rate of GTR for craniopharyngioma

A

70-85% but associated with significant morbidity

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127
Q

Outcomes of STR + RT for craniopharyngioma

A

85-90%

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128
Q

What is a practical RT consideration for treating craniopharyngiomas with RT?

A

Cyst or tumor swelling during RT can threaten coverage and result in chiasmal compression

Thus should obtain WEEKLY MRIs to confirm target volumes and consider cyst fenestration if target gets too big

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129
Q

Late effects of RT to suprasellar region

A
  • DI (increased sodium and AMS)
  • Growth hormone dysfunction
  • Visual complications
  • Moya Moya syndrome with occlusion of carotid arteries
  • Hypothalamic dysfunction
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130
Q

Symptoms of hypothalamic dysfunction

A

Sleep disturbances

Weight gain

Memory impairment

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131
Q

Appearance of optic glioma

A
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132
Q

What percentage of RB patients are hereditary?

A

40% due to germline mutations in RB gene

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133
Q

What share of retinoblastomas are bilateral

A

20-30%

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134
Q

For germline RB cases, what is a management consideration

A

Try to avoid RT if possible given very high risk of secondary malignancy (osteosarcoma)

135
Q

What is the workup for retinoblastoma

A
  • Diagnosis is made by ophthalmologic exam and path confirmation is not required
  • Ocular US to determine depth
  • MRI of orbits and brain
  • BMBx, bone scan and LP if nerve involvement or extraocular disease
  • Genetics referral
136
Q

Treatment options for RB

A
  • For very large lesion- enucleation
  • For very small lesions (<3mm)
    • Cryotherapy
    • Photocoagulation
    • Laser
    • Brachytherapy
  • For medium lesions (>3 mm)
    • Carboplatin to delay RT –> local therapies
  • For large lesions or salvage: EBRT
137
Q

What is the dose used for plaque brachy for RB

A

40 Gy with I-125 dosed to apex of tumor

RT delivered over 2-3d

138
Q

EBRT approach for retinoblastoma

A
  • Refer for protons, if possible
  • 45 Gy in 25 fractions of 1.8 Gy
  • Cover entire retina and 8 mm of optic nerve
139
Q

Eye preservation if EBRT is used

A

95% if earlier stage

50% if more advanced stage

Visual acuity is excellent in most, poor if macula is involved

140
Q

Toxicity of retinoblastoma RT

A

Skin dermatitis

Dry eye

Corneal injuries

Cataracts

Retinopathy

Glaucoma

Secondary malignancies (osteosarcoma)

141
Q

Benefit of prone CSI over supine

A
  • Direct visualization of light fields for better spine field setups (better for therapists)
142
Q

Benefit of supine CSI over prone

A

More comfortable for kid

Better access to airway for anesthesiologist

More reproducible head tilt

143
Q

RT approach for CSI

A
  • If possible refer the patient to a proton facility for CSI
  • I would simulate the patient supine, with the neck hyperextended, the shoulders down using a 5 point mask and full body alpha cradles
  • I would scan from the vertex to the midfemurs using 3 mm slice thickness
144
Q

Approach for proton CSI

A
  • This is contouring based planning
  • For children who are post-pubertal, I would contour the whole brain and full thecal sac, including optic nerves
  • For children who are pre-pubertal, I would contour these structures plus the entire vetebral bodies to reduce risk of scoliosis
145
Q

Approach for 3D CSI

A
  • After simulation, I would first set the spinal field
  • The superior edge is the point where I would match with the WBRT field and is set as high as possible
  • but chosen to
    • Minimize divergence of the upper spinal field into the oral cavity and mandible.
    • Ensure no lateral entrance of the WBRT field into the shoulders
    • This is typically around C5-C6
  • Inferior edge is 2 cm below thecal sac (usually below S2)
  • Lateral edge is at least 1 cm lateral to the vertebral bodies and should be set to cover SI joints to include lower neuro-foramina
  • RX is prescribed to either:
    • Anterior edge of cord (if post-pubertal)
    • Anterior edge of vertebral body (if pre-pubertal)
146
Q

How to choose Rx depth point for 3D CSI

A

Take an average of depth at 2-3 spots in the field

147
Q

What to do if the patient is too long for single spine field?

A
  • Two options
    • Try extended distance with 110-115 cm SSD
    • Match a second spine field but requires gap an feathering
148
Q

Where are the spinal fields matched?

A

They are matched at anterior cord which leaves a 3-5 mm gap at the skin surface

149
Q

How to set WBRT fields for 3D CSI

A
  • Do RAO and LAO slight obliques (5 degrees) to avoid divergence into lenses
  • Set inferior border at C5/C6 match line
  • Rotate collimator to match the divergent beams of the spine field
  • Kick the couch towards the gantry to aboid divergence of WBRT into spine field
150
Q

What is the formula for collimation of the WBRT field?

A

arctan [(L/2) / SSD]

L=length of adjacent spinal field at surface

SSD = 100 if normal distance

151
Q

Typical angle of collimation for WBRT field

A

10-12 degrees

152
Q

Formula for couch kick of gantry for WBRT field

A

arctan (L/2)/SAD

L = length of cranial field

SAD = distance to the WBRT axis point

153
Q

Gap formula if using 2 fields for CSI

A
  • Calculate the contributions of the two spinal fields
  • Each is Depth from skin surface x (half distance of spinal field at depth / SAD)
154
Q

How to feather CSI fields

A

Consider feathering q9 Gy or weekly

Open cranial field 1 cm inferiorly and shift spine iso 1 cm inf

155
Q

Acute toxicity of CSI

A

Headaches

N/V

Fatigue

Cytopenias

156
Q

Subacute toxicities of CSI

A

Transient demyelination from damage to oligodendrocytes –> focal encephalopathy

Fatigue

Somnolence

Starts between 1-12 weeks, peaks at 8 weeks and done by 4 months

157
Q

Late toxicies of CSI

A

Radiation necrosis in ~5%

Cerebral atrophy

hemorrhagic vascular telangiectasias

Pituitary and hypothalamic dysfunction

Infertility, hearing loss

Cataracts

Heart and lung diesease

Chronic myelosuppression

158
Q

What is the second malignancy risk after CSI?

159
Q

IQ changes after CSI

A

If CSI given for kids < 7, all need special ed

Risks are greater with CSI dose, female gender and higher baseline IQ

160
Q

What long term surveillance do kids need after CSI

A

Auditory

Neurocognitive

Endocrine

Vision checks

161
Q

What are some genetic syndromes associated with Wilms tumor?

A

WAGR

Denys-Drash (mut WT1)

Beckwith-Widemann (mut WT2)

162
Q

What is the workup of suspected Wilms tumor

A
  • H&P
  • Labs: CBC, COMP, Coags, check urine catechols to rule out neuroblastoma
163
Q

General imaging for suspected Wilms tumor

A

Abdominal US

CT or MR abdomen

CT chest for mets

164
Q

Special imaging studies required for Wilms

A

If clear cell sarcoma of kidney –> PET and BMBx

If rhabdoid tumor of kidney –> brain MRI

165
Q

How to differentiate kids with Wilms from neuroblastoma

A
  • Kids are not generally sick with Wilms
  • WT typically doesn’t cross midline
  • Check urine catechols
166
Q

What is favorable histology wilms tumor?

A

Classic three cell types WITHOUT anaplasia

167
Q

What is unfavorable histology Wilms?

A
  • Anaplastic (4-5% of cases)
  • Clear cell sarcoma of kidney
  • Rhabdoid tumor of kidney
168
Q

What are positive prognostic factors for Wilms?

A

favorable histology

stage I/II

Age <2

Smaller tumor

169
Q

What are negative prognostic signs for Wilms?

A

unfavorable histology (anaplasia)

bilteral tumors

gain of 1q

LOH 1p or 16q

170
Q

General management of Wilms tumor

A

NO BIOPSY - risk tumor seeding with capsule rupture

If suspected WT–> go to surgery with radical nephrectomy + LN sampling

If bilateral or unresectable –> do biopsy and neoadjuvant chemo

171
Q

What is stage I Wilms?

A

Limited to kidney

R0 resection

Renal capsule intact

No prior biopsy

172
Q

What is stage II Wilms

A

R0 resection but with regional extension beyond the capsule or sinus

No biopsy

173
Q

What is stage III Wilms?

A

SLURP PIB

  • Subtotal resection (R1, R2, +margin)
  • Lymph nodes
  • Unresectable
  • Rupture or tumor spillage
  • Piecemeal resection
  • Preop chemo due to unresectability
  • Implants or peritoneal spread
  • Biopsy
  • Tumor in renal vein or IVC
174
Q

Stage IV Wilms

A

Distant metastases

LN+ outside of abdomen/pelvis

175
Q

Stage V Wilms

176
Q

What is chemo for stage I FH Wilms

A

If <2 years - none

If >2 years: 19 weeks of vincristine/actinomycin D

177
Q

What is chemo for stage I UH Wilms

A

19 weeks of vincristine + actinomycin D

178
Q

Chemo for stage II FH wilms?

A

Vincristine + Actinomycin D for 25 weeks

179
Q

Chemo for Stage III or IV FH Wilms

A

Vincristine + Actinomycin D + doxorubicin for 24 weeks

180
Q

Chemo for stage III/IV UH Wilms

A

24 weeks of CAVE

Cyclophosphamide

Actinomycin D

Vincristine

Etoposide

181
Q

When should chemo or RT be started for Wilms?

A

Ideally 10-14 days post op

182
Q

What are reasons to delay chemoRT for Wilms?

A

Diarrhea

Ileus

ANC < 1000

Hgb < 10.5

Rhabdoid starts week 6

183
Q

What is RT for stage I with FH?

184
Q

What is RT for stage II FH Wilms?

185
Q

What is RT for stage III FH?

A

Standard risk

Flank RT of 10.8 Gy (1.8 x 6)

186
Q

How to approach RT for focal anaplasia

A

Stage I-III are considered

  • Standard risk
  • Flank RT of 10.8 Gy (1.8 x 6)
187
Q

How to approach RT for diffuse anaplasia Wilms

A

Stage I and II are standard risk (1.8 x 6 = 10.8 to flank)

Stage III is high risk (1.8 x 11 = 19.8 to flank)

188
Q

How to approach clear cell Wilms?

A

stage I-III are considered standard risk (1.8 x 6 = 10.8 flank)

189
Q

How to approach RT for rhaboid tumor of kidney

A

Considered high risk (1.8 x 11 = 19.8 flank)

190
Q

What age group for Wilms is considered high risk?

A

>16

Should get 19.8 Gy to flank (1.8 x 11)

191
Q

How to approach if a patient has gross residual disease after Wilms

A

Deliver boost to 10.8 Gy (1.8 x 6)

192
Q

What is the indication for whole abdomen RT for Wilms?

A

SPAR

  • Spillage
  • Peritoneal seeding
  • Ascites with +cytology
  • Rupture
193
Q

Dose of whole abdomen RT for Wilms

A

10.5 Gy (1.5 x 7)

194
Q

What additional RT is needed after whole abdomen RT for Wilms?

A
  • If high risk boost flank with 9 Gy (1.5 x 6)
  • If any gross residual, boost additional 10.5 (1.5 x 7)
195
Q

What scenarios are high risk Wilms?

A
  • Stage III diffuse anaplasia
  • Stage I-III rhabdoid
  • Age > 16
196
Q

How to approach lung mets for Wilms?

A

If >1 year: Deliver WLI to 12 Gy in (1.5 x 8)

If < 1 year: Deliver WLI to 10.5 Gy (1.5 x 7)

If tumors are residual 2 weeks after WLI – boost with 7.5 Gy (1.5 x 5)

197
Q

How to approach liver mets for Wilms tumor?

A

If diffuse: Whole liver RT to total dose of 19.8 Gy with boost of additional 5.4-10.8 Gy

If focal: 19.8 Gy

198
Q

How to approach bone mets for Wilms

A
  1. 2 Gy if < 16
  2. 6 Gy if >16
199
Q

How to approach brain mets for Wilms

A

WBRT to 21.6

Boost gross mets to 30.6

200
Q

How to approach stage V Wilms

A

Preop chemo based on the more advanced tumor

If still unresectable can consider neoadjuvant RT 12-16 Gy

201
Q

How to determine if a Wilms patient has “real” lung mets

A

Visible on CXR or >1 cm on CT

202
Q

What should be given to patients getting whole lung RT

A

PCP prophylaxis

203
Q

Approach to whole lung RT

A

cardiac sparing IMRT

204
Q

What patients with lung mets should get WLI

A

If mets are persistent despite chemo

205
Q

How to deliver flank RT for wilms?

206
Q

Field borders for flank RT

A
  • Contour pre-op extent of disease, any residual disease
  • Contour intact contralateral kidney
  • Superior border is preop kidney + tumor + 1 cm
  • Inferior border is preop kidney + tumor + 1 cm
  • Medial border extends across midline to completely include vertebral body but no portion of contralateral kidney
207
Q

What is size of residual disease to get boost for Wilms?

A

>3 cm

Boost to additional 10.8 in 6 fractions

208
Q

When to hold flank RT for Wilms

A

ANC < 0.3

Platelets < 40

If delay 4-7 days, add one fx

If delay 7+ days add 2 extra fx

209
Q

Fields borders for whole abdomen RT

A

AP/PA to 10.5 Gy in 1.5 daily fractions

Superior: diaphragm

Inferior: bottom of obturator foramen

210
Q

Dose constraint to intact kidney for kids getting Wilms RT

A

D100 <14.4 Gy

50% kidney < 19.8 Gy

211
Q

What is low risk pediatric Hodgkin?

A

stage IA or IIA without bulk

212
Q

What is high risk pediatric Hodgkin

213
Q

What is intermediate risk pediatric Hodgkin

A

IA or IIA with bulk

IAE or IIAE

IB or IIB

IIIA

IVA

214
Q

What is the chemo regimen used for pediatric Hodgkin

A

ABVE-PC

Adriamycin

Bleomycin

Vincristine

Etoposide

Prednisone

Cyclophosphamide

215
Q

Treatment of intermediate risk Hodgkin disease

A
  • Give 2 cycles ABVE-PC
  • Assess with PET/CT after 2 cycles
    • Classify into one of two groups
      • Rapid early responders and those with rCR
      • Non-RER/CR
216
Q

How is RER defined for Hodgkin disease

A

>60% reduction on CT scan

217
Q

How should patients who are RER after 2 cycles ABVE be treated?

A

2 more cycles of ABVE-PC

If rCR after 4 cycles –> No ISRT

If not rCR after 4 cycles –> ISRT to 21 Gy

218
Q

How should patients nonRER or CR be managed after 2 cycles ABVE-PC

A

2 more cycles of ABVE-PC

2 cycles of DECA

ISRT

219
Q

Dose of ISRT for pediatric Hodgkin

A

21 Gy (1.5 x 14)

220
Q

How to approach high risk pediatric Hodgkin

A

ABVE-PC x 5

Deliver 21 Gy ISRT to sites of initial bulk or SER

221
Q

How is bulk defined for pediatric cHL

A

>6 cm or >1/3 length of widest area of mediastinum

222
Q

What is CNS1 for leukemia

A

Negative cytology

223
Q

What is CNS2 for leukemia

A

<5 WBC on cytology

224
Q

What is CNS3 for leukemia

A

>5 WBC on cytology

Gross CNS lesions or CN deficits

225
Q

What is role of RT for CNS1 disease

A

If T-ALL or lymphoblastic leukemia

Consider PCI of 12 Gy in 1.5 Gy fractions

226
Q

What is role of RT for CNS2 disease

A

If T-ALL or lymphoblastic leukemia

Consider 12 Gy in 1.5 Gy fractions

227
Q

What is role of RT for CNS3 disease

A

All histologies

WBRT to 18 Gy in 1.8 Gy fractions

228
Q

What share of Ewing sarcoma have mets at diagnosis

229
Q

What is the workup for Ewing sarcoma

A
  • H&P
  • Xray of primary site
  • CT/MRI of primary site
  • CT chest
  • PET to assess for mets
230
Q

What is the necessary pathology for Ewings?

A

BILATERAL bone marrow biopsy

Incisional biopsy of the primary site

231
Q

What is the translocation of Ewing

232
Q

General treatment paradigm for Ewing sarcoma

A
  • Induction chemo for weeks 1-12
  • Local therapy integrated at week 12
    • Surgery preferred if possible but don’t pursue unless R0 is anticipated
    • Definitive RT with chemotherapy
  • Adjuvant chemotherapy (22 weeks)
  • RT to mets
233
Q

What is the chemo for Ewing sarcoma

A

VAC-IE

Vincristine, ADRIAMYCIN, Cyclophosphamide

Alternating with

Ifosfamide, Etoposide

q2 weeks, dose dense

234
Q

What is OS rate for Ewing sarcoma - non metastatic extremities

235
Q

What is the 5 year OS rate for Ewing - pelvis

236
Q

What is 5 year OS rate for metastatic Ewing sarcoma

A

20% unless isolated

50% if lung only

237
Q

Unresectable areas for Ewing sarcoma

A

Spine, face, pelvis

238
Q

When is local therapy integrated for Ewing sarcoma

239
Q

What is the difference between surgery and CRT for local control for Ewing

A

OS is the same

LC with surgery probably better

Decreased risk of second malignancies

240
Q

Late effects of Ewing treatment

A

Fractures

Wound complications

Bone/soft tissue hypoplasia

Infertility

Lymphedema

Avascular necrosis

2nd cancers

241
Q

second cancer risk for Ewing sarcoma

A

6-9% at 20 years

242
Q

Defining volumes for Ewing sarcoma

A
  • GTV1 = prechemo bone and soft tissue extent of disease (fusing MRI)
  • CTV1 = GTV1 + 2 cm (reduce volume around joints and edge of bones)
  • GTV2 =
    • If definitive: pre-chemo bone disease and post chemo soft tissue disease
    • If post-op: residual bone, microscopic margin and soft tissue abnormalities
  • CTV2 = GTV2 + 2 cm
243
Q

Definitive CRT Ewing doses

A

PTV1 = 45 Gy in 25 fractions (1.8 x 25)

PTV2 = 55.8 (1.8 x 31)

If vertebral body 50.4 Gy

244
Q

When would neoadjuvant doses be used for Ewing

A

To help achieve R0 resection (but cannot convert unresectable to resectable)

245
Q

Downside of neoadjuvant RT for Ewing sarcom

A

Impairs bone healing/increases fracture risk

246
Q

Neoadjuvant RT dose for Ewing

A

Give 36 Gy in 20 fractions

If gross residual disease, boost with 19.8 Gy in 11 fractions

247
Q

Indications for adjuvant RT for Ewing

A
  • Positive or close margin
  • <90% necrosis of tumor on pathology
248
Q

What is a close margin for Ewing sarcoma

A

<5 mm for soft tissue

< 1 cm for bone

249
Q

Adjuvant RT doses for Ewing

A

If R2: treat like definitive (45 Gy to GTV1, 55.8 Gy to GTV2)

If >90% necrosis and R0 - no RT

If >90% necrosis and R1 - 50.4 to GTV2

If <90% necrosis and either R0 or R1: 50.4 to GTV1

250
Q

When should lung mets be treated for Ewing?

A

After completion of all chemo or during primary RT if concern for ovelap

251
Q

Approach to RT for lung mets for Ewing

A

Cardiac sparing whole lung IMRT (to bilateral lungs)

252
Q

Dose of whole lung RT for Ewing sarcoma

A

15 Gy in 10 fx (1.5 Gy per fraction)

253
Q

What about if there is residual disease?

A

After WLI, can either resect or boost these areas to total of 45 Gy

254
Q

What if there is a pleural effusion with +cytology from Ewing

A

Treat 15 Gy to pleural cavity

255
Q

How to approach other bone mets from Ewing

A

Treat all metastatic sites if feasible

Do SBRT total dose of 10 x 5

256
Q

Ewing vs. osteosarcoma imaging on xray

257
Q

Rhabdo reflects X% of childhood STS

258
Q

Epidemiology of Rhabdo

A

70% of kids are <10 years old

peak incidence is age 2-5

259
Q

What syndromes are associated with rhabdo

A

Li-Fraumeni

NF1

Beckwith-Weideman

But MOST ARE SPORADIC

260
Q

Initial workup for rhabdo

A
  • H&P, fiberoptic exam if H&N or parameningeal
  • CT/MRI of primary
  • CT chest
  • PET/CT
  • Perform biopsy of primary and bilateral bone marrow biopsies
261
Q

Additional rhabdo workup required for parameningeal location

A

MRI brain

MRI spine

CSF cytology

262
Q

Additional workup required for bladder/GU/Gyn rhabdo

A

EUA and cystoscopy

263
Q

Additional workup required for paratesticular rhabdo

A

Perform scrotal US

NO BIOPSY

Bilateral inguinal LND

Ipsilateral RP LND if age >10 and/or N+

264
Q

Additional workup required for extremity rhabdo

A

SLN biopsy or dissection

265
Q

Parameningeal sites

A

MNOP

  • Middle ear, Mastoid
  • Nasal cavity or nasopharynx
  • fOssa (pteryopalatine or infratemporal)
  • Paranasal sinus or parapharyngeal space
266
Q

General treatment paradigm for rhabdomyosarcoma

A
  • Maximal safe resection or decision for biopsy and organ preservation
    • LND for paratesticular age >10
  • Chemotherapy with VAC/VI for up to a year +/- mTOR inhibitor
  • Radiation with VC
  • Second look surgery if not fully resected upfront
  • More chemotherapy (high dose and possibly maintenance cyclophosphamide and vinorelbine)
267
Q

Which are the organs which should be considered for no surgery for rhabdo

A

Orbit

Vagina

Bladder

Biliary

268
Q

Chemo regimen for rhabdo

A

VAC-VI

Vincristine

ActinomycinD

Cyclophosphamide

Irinotecan

269
Q

Steps for figuring out appropriate RT plan for rhabdo

A
  1. Determine histology (favorable or unfavorable)
  2. Determine site (favorable or unfavorable)
  3. Determine the stage (clinical preop)
  4. Determine the group (surgical outcome)
  5. Determine the risk grouping (mix of stage and group)
  6. Determine the radiation dose
  7. Determine when to start RT
270
Q

What are the histologies of rhabdo

A

Favorable = embryonal, botryoid, spindle cell

Unfavorable = alveolar or undifferentiated

271
Q

Translocation hallmark of alveolar rhabdo

A

PAX/FOX01 fusion

272
Q

PAX/FOX01 fusion is what translocation

A

t(1;13) or t(2;13)

273
Q

What are the favorable sites for rhabdo

A

BONG

  • Biliary
  • Orbit
  • Non-parameningeal H&N
  • GU/GYN (non-bladder or prostate)
274
Q

Unfavorable sites for rhabdomyosarcoma

A

Extremity

Trunk

RP

Bladder or prostate

Parameningeal H&N

275
Q

How is stage determined for rhabdo

A

Clinically based on pre-op imaging

276
Q

What is S1 rhabdo

A

Favorable site, N0-1

277
Q

What is stage 2 rhabdo

A

Unfavorable site, <5 cm AND N0

278
Q

Stage 3 rhabdo

A

Unfavorable site, >5 cm OR N1

279
Q

Stage 4 rhabdo

280
Q

How is Group determined for rhabdo

A

Pathologically using surgical outcomes

281
Q

What is Group I rhabdo

282
Q

What is Group II rhabdo

A

R1 resection or N1

283
Q

What is Group III rhabdo

A

R2 resection

Unresectable

Bx only

284
Q

What is Group IV rhabdo

285
Q

What is low risk rhabdo group?

A
  • Embryonal histology UNLESS
    • Unfavorable site (extremity, trunk, RP, parameningeal, bladder, prostate)
    • Group III
286
Q

What is high risk rhabdo group?

A

Metastatic except for embryonal histology age < 10 (which is IR)

287
Q

What is intermediate risk rhabdo group?

A
  • Embryonal, stage 2-3 (unfavorable sites), Group 3 (unresectable)
  • Alveolar stage 1-3 (favorable or unfavorable site), Group 1-3 (resectable or unresectable)
  • Embryonal age <10, stage IV but resectable
288
Q

Outcome for low risk rhabdo

A

failure free survival 85%

289
Q

Intermediate risk rhabdo outcomes

A

FFS around 70%

290
Q

Outcomes for high risk rhabdo

A

OS is ~35%

291
Q

If rhabdo is resected Margin -, what is RT dose?

A

If embryonal: no RT

If alveolar: 36 Gy in 1.8 Gy fractions (20)

292
Q

If rhabdo is margin positive resection, what group and what RT dose?

A

Group 2

If embryonal - 36 Gy (1.8)

If alveolar - 36 Gy (1.8)

293
Q

If rhabdo is N+, what group and what RT dose

A

Group 2

If embryonal - 41.4 Gy (23)

If alveolar - 41.4 Gy (23)

Do 50.4 if nodes are not resected

294
Q

If rhabdo is N+ what should be treated

A

Primary and nodes even if CR to chemo

295
Q

What dose for Group 3 rhabdo

A

50.4 to 59.4 Gy in 28-33 fractions

296
Q

What rhabdo tumors should receive 59.4 Gy

A

Tumors > 5 cm

297
Q

What is RT dose to orbital rhabdo

A

Consider 45 Gy but can do 50.4 Gy if poor response to chemo

298
Q

How to approach paratesticular rhabdo

A

If there is GTR of the testicle and spermatic cord there is no tumor bed so no RT unless it is N+

If N+, do 41.4 dog leg

299
Q

How to approach RT for amputated rhabdo

A

If R0, no tumor bed and no RT

300
Q

What to do if lung mets

A

Do WLI, cardiac sparing IMRT at end of chemo

301
Q

How to approach rhabdo if CSF+

A

Consider CSI

302
Q

When to start RT for rhabdo for primary?

A

Start at week 12 for low and intermediate risk

Start at week 20 for high risk (primary and select metastatic sites)

303
Q

When to start RT if CSF+ or cord compression?

A

Day 0

Do not do upfront RT if CN palsy or BOS involvement

304
Q

When should metastatic sites be irradiated for rhabdo

A

Week 50

WLI

Spine/pelvic sites

(avoid marrow suppression during chemo)

Select sites ok to do week 20 with primary

305
Q

What is GTV for rhabdo

A

pre-chemo, pre-op disease but anatomically confined

306
Q

What is CTV for rhabdo?

A

GTV + 1 cm

Include LN region or chain if N+

307
Q

What is PTV for rhabdo

A

CTV + 5 mm

308
Q

If RT is delayed for rhabdo, how to change the Rx

A

If delayed 2-3w given 1 extra fraction

If delayed 3+ weeks, 2 extra fractions

309
Q

What is the origin cell for neuroblastoma?

A

Neural crest cells that fail to regress

310
Q

Most common location for neuroblastoma

A

adrenal gland (35%)

311
Q

What is epidemiology of Neuroblastoma

A

Most common non-CNS solid tumor of childhood

Most lethal pediatric solid tumor

312
Q

How to describe Neuroblastoma kids

A

They are SICK (in contrast to Wilms)

Their tumors are typically abdominal

Tumor can cross midline

313
Q

What are the symptoms of Neuroblastoma

A

Abdominal mass

Pain

Fever

HTN

Classic signs/symptoms

314
Q

Classic signs and symptoms of neuroblastoma

A
  • Raccoon eyes - periorbital ecchymosis from mets
  • Hutchinson syndrome - bone pain, refusal to walkm skull masses
  • Blueberry muffin sign - infants with bluish skin mets
  • Pepper syndrome - massive liver mets with respiratory compromise
  • Opsoclonus-myoclonus - dancing eyes
  • Cord compression - 10-15% of cases
315
Q

Imaging workup for neuroblastoma

A

Plain films

CT CAP

MRI of primary site

Bone and MIBG scans

316
Q

Path diagnosis of neuroblastoma

A
  • Biopsy of the primary site (unlike Wilms)
  • Bilateral bone marrow biopsies
317
Q

What labs should be checked for neuroblastoma

A

Urine catecholamines (produced 90% of time)

CMP
CBC

318
Q

What risk group is relevant for RT

A

HIGH RISK only

Low risk only if not responding to chemo

319
Q

How to define high risk Neuroblastoma

A
  • Myc-N amplication for all [Stage MS, M, locoregional]
  • Metastatic cases age > 18 months irrespective of Myc status
320
Q

How to define stage MS neuroblastoma

A

Localized tumor in patients <18mos with dissemination limited to skin, liver, bone marrow

321
Q

What is the typical treatment sequence for neuroblastoma

A
  • Induction chemo x 6 cycles with stem cell harvesting
  • Reimaging with MIBG
  • Radical surgery
  • Tandem consolidation with myeloablative HDT –> auto x2
  • Radiation therapy
  • Post-consolidation therapies (immunotherapy plus isotretinoin)
322
Q

When should RT be offered for neuroblastoma patients

A

28-42 days post transplant

323
Q

What are the RT doses for neuroblastoma

A

If resected: 21.6 Gy in 12 fractions

If residual disease: 36 Gy in 20 fractions

324
Q

Contouring for neuroblastoma

A
  • GTV1 is post-chemo, pre-surgical GTV
    • CTV1 = GTV1 + 1.5 cm margin (anatomically confined to shave off kidney and bone but NOT bowel)
    • PTV1= CTV1 + 5 mm
    • PTV1= 21.6 Gy in 12
      • GTV2 is residual MIBG active disease
    • CTV2 = GTV2+1cm (anatomically confined)
    • PTV2 = CTV2+ 5 mm
    • PTV2 = 36 Gy in 20 fx
325
Q

Which neuroblastoma metastatic sites should be irradiated

A

5 or fewer active residual mets on MIBG scan after chemo and surgery but preBMT

MIBG+ after chemo

Radiation should be given AFTER BMT

326
Q

Dose for stage 4S liver tumor

A

1.5 Gy x 3 (spare kidney)

327
Q

Dose for neuroblastoma cord compression

A

If <3 years old: 1.8 Gy x 5 (9 Gy)

If >3 years old: 1.8 x 12 (21.6 Gy)

328
Q

Neuroblastoma constraint to IPSI kidney

A

V19.8 < 50%

V14.4 < 100%

329
Q

Dose constraint for contralateral kidney

330
Q

Liver constraint for neuroblastoma

331
Q

Lung constraint for neuroblastoma

332
Q

Which histologies need bilateral BM BX?

A
  • Neuroblastoma
  • Rhabdomyosarcoma
  • Ewing sarcoma
333
Q

OS for high risk neuroblastoam

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