Pediatrics Flashcards
1
Q
Ddx for posterior fossa mass
A
Brainstem glioma
Medulloblastoma
Ependymoma
Astrocytoma (JPA)
ATRT
Hemangioblastoma
Mets
Lymphoma
2
Q
DDx of suprasellar mass
A
Craniopharyngioma
Optic glioma
Pituitary adenoma
Germ cell tumor
Abcess
Meningioma
Ependymoma
3
Q
Small round blue cell tumors
A
- LEARN MR
- Lymphoma
- Ewings
- ALL
- RMS
- Neuroblastoma
- Medulloblastoma
- Retinoblastoma
4
Q
What is one way to avoid scoliosis
A
Don’t irradiate just part of vertebral body
5
Q
Parinaud Syndrome
A
Impaired upward gaze
Pseudo-Argyll Robertson pupil (accomodates but does not react)
Convergence nystamus
6
Q
What lesions associated with Parinaud syndrome
A
Pineal glad tumor or dorsal midbrain lesion
7
Q
What is the most important management thing to state for pediatric patients?
A
Treat kid on protocol if available
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
9
Q
How to approach mgmt of PF mass if surgery not immediately available?
A
Shunt
ONLY if surgery not immediately available
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)
11
Q
When should postop brain be done
A
Within 48 hours of surgery
12
Q
When should LP be done if not prior to surgery
A
14 days post op
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
14
Q
what is HGG RT dose
A
59.4 in 33 fractions
15
Q
How does TMZ compare for kids vs adults
A
TMZ data not as good for kids
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
17
Q
Where do pediatric ependymomas form
A
posterior fossa (60%)
Suprtentorial (30%)
Spinal cord (10%)
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
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
20
Q
Most important prognostic factor for ependymoma
A
extent of resection
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
22
Q
Typical age range of ATRT
A
<3 years old
23
Q
Treatment of ATRT
A
Maximal safe resection
Chemotherapy (vincristine, MTX, cis, etop, cyclophosphamide x2 then carbo/thiotepa x3)
Adjuvant RTto 54 Gy
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)
25
Send kids with CNS tumors for what testing
* Neurocog/IQ testing
* Audiology
* Genetics if syndromic tumor
* Endocrine consult
* Fundoscopic and eye exam with VF testing
26
Mgmt of LGG
Principally maximall safe resection
RT for recurrence
27
When should RT be used for pediatric LGG
If recurrent after resection
If bx or STR and patient asymptomatic
28
Dose of RT for LGG
54 Gy in 30
29
Contouring of LGG
GTV= FLAIR residual disease
CTV= GTV+0.5 cm
PTV=5 mm
30
Describe JPA
Large cystic mass of cerebellum, hypothalamus, brainstem, usually benign and slow growing
![]()
31
JPA is associated with what syndrome
NF1
32
What is path hallmark of JPA
rosenthal fibers
33
Treatment of JPA
* 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
34
Dose of RT for JPA
50.4 Gy in 28 fractions
35
Contouring for JPA
Get kid on trial!
GTV= residual T1post and T2 flair changes
CTV=GTV + 5mm
PTV = 3-5 mm
36
What is outcome for JPA
5 year OS \> 90%
37
How to describe low grade ependymoma
Calcs and cysts are common as well as descent through the foramen magnum (tongue of tumor)
38
What is G1 ependymoma
subependymoma
myxopapillary
39
What is G2 ependymoma
classic ependymoma
40
what is path hallmark of low grade ependymoma
perivascular pseudorosettes
41
Most important prognostic factor for ependymoma
extent of resection
42
Mgmt of intracranial ependymoma
* 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
43
Dose of RT for ependymoma
If GI/GII: 54 Gy
If GIII: 59.4 Gy
For CSI: 36 Gy boost gross disease to 45
44
Mgmt of spinal ependymoma
* 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
45
Contouring for ependymoma
GTV = gross disease and tumor bed
CTV = GTV + 1.5
PTV = 3-5 mm
46
Most common location for brainstem glioma
pons (70%)
47
Most common form of brainstem glioma
DIPG - diffuse intrinsic pontine glioma
48
Mutation a/w brainstem glioma
H3 K27M
49
How to diagnose a DIPG
Typically a radiographic diagnosis but more recently a push to do biopsies to see if that can guide molecularly targeted therapies
50
What has been the only thing shown to improve OS for brainstem glioma
RT
51
Dose for DIPG
54 Gy in 30 fractions
Use photons as no advantage to protons
52
What chemo is used for DIPG
Nothing routine
There are trial testing various agents
53
Dose of reRT for DIPG
Consider additional 36-45 Gy
54
Median survival for DIPG
6-12 mos
55
What is the contouring for DIPG?
CTV = GTV + 1 cm
PTV = CTV+3 mm
Treat to 54 Gy
56
Workup for optic glioma
* H&P
* CT or MRI
* Labs including HCG, AFP, endocrine labs
* Ophthalmologic exam
* Genetics referral
57
Should bx be performed of optic glioma
Only if diagnosis uncertain
58
Management of optic gliomas
* 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
59
Which optic glioma patients should get chemo alone
* Children \<3
* Children with NF1 due to 2nd malig risk
60
Dose of RT for optic glioma
54 Gy
61
Target for optic glioma
Optic nerve, chiasm, optic tracts
62
Median age for medulloblastoma
Median age 5-6
20% occur before 2 year
63
What are the path features which should be considered for medullo?
Anaplasia
Histologic subtype
Molecular group
64
Workup for medulloblastoma
* 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
65
Management of medulloblastoma
* 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
66
When should medullo RT start after surgery
23-30 days post op
67
Total treatment duration for medulloblastoma
55 weeks
68
What is best prognosis molecular subtype of medulloblastoma
WNT, followed by SHH
69
What is poorest prognosis medulloblastoma subtype
Group 3 (frequently M+, Myc+)
70
How is medulloblastoma staged?
Chang staging, focusing on M
71
M0 medulloblastoma
No CSF or hematogeneous mets
72
M1 medulloblastoma
Microscopic disease in CSF
73
M2 medulloblastoma
Gross nodular **cranial** seeding beyond the primary site
74
M3 medulloblastoma
Gross nodular **_SPINAL_** seeding
75
M4 medulloblastoma
Mets outside CNS (bone)
76
What are the clinical categories of medulloblastoma
standard risk (2/3)
high risk (1/3)
77
How is average risk defined
GTR or \< 1.5 cm2 residual
Age \>3
M0
No anaplasia on path
78
Treatment approach for average risk medulloblastoma
* 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
79
How to define high risk medulloblastoma
* STR or \>1.5 cm2 residual
* Age \<3 months
* M+
* Anaplastic histology
80
Treatment of high risk medulloblastoma
* 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
81
Dose for medullo diffuse spinal disease
39.6 (1.8.x 22)
82
5 year EFS for standard risk medullo
80%
83
5 year EFS for high risk medullo
65%
84
What is the youngest patient eligible for CSI?
age 3+
(catastrophic neurocognitive changes and patients can't live independently if getting CSI before that age)
85
Strategy to try to bridge time to CSI for young children
Chemotherapy to help reserve RT for salvage
86
Posterior fossa syndrome
* Child awakes from surgery fine
* 1-4 days post op develops
* Hypotonia
* Ataxia
* Mutism
* Emotional lability
* Irritability
87
How many children get PF syndrome
30%
88
What share of kids with posterior fossa syndrome get better?
20%
89
What is the treatment for posterior fossa syndrome
No specific treatment
Do not stop RT
90
Diseases which require CSI
* Medulloblastoma
* Pineoblastoma/ependymoblastoma
* ATRT
* M+ ependymoma
* M+ germ cell (germinoma)
* NGGCT
* Supratentorial PNET
91
Contouring medullo tumor bed boost
GTV= tumor bed (T1post) + residual disease
CTV = GTV + 1.5 cm (respecting tentorium and bone)
PTV= 3-5 mm
92
What structure should be spared for medulloblastoma boost
cochlea
93
Cochlea constraint for kids
Dmax \< 35
V30 \< 50%
94
Pituitary constraint for kids
V35 \< 50%
95
Most common locations for CNS germ cell tumors
Suprasellar
Pineal
96
What is the most common subtype of germ cell tumor?
germinoma (60%)
97
Serum marker profile of germinoma
mild elevation in hcg ok
normal AFP
98
DDx for a suprasellar or pineal mass
Germinoma or NGGCT
Pinealblastoma
Ependymoma
Craniopharyngioma
LGG
Hamartoma
Teratoma
LCH
99
Workup for germ cell tumor
* 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)
100
What is the surgical plan for germ cell tumors
If uncertain, bx is ok to confirm pure germinoma
Germinomas do not need resection
NGGCT will need resection
101
Treatment options for germinoma
* 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
102
RT approach if CR after induction chemo for Germinoma
* Whole ventricle to 21 Gy in 1.5 Gy fractions (14 fractions)
* Primary site boost to 9 Gy
103
RT approach if PR after induction chemo for Germinoma
* 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)
104
RT approach if no induction chemo for Germinoma
* No surgery
* Whole ventricle to 24 Gy in 1.5 Gy fractions
* Tumor boost to 45 Gy in 1.5 Gy fractions
105
What is a bifocal germ cell tumor
Tumor in suprasellar and pineal regions synchronously
106
Treatment approach for bifocal germ cell tumor
DO NOT NEED CSI
Whole ventricle to 24 Gy
Boost both sites to 45 Gy
107
Trilateral germ cell tumor
Retinoblastoma
Pineal
Suprasellar
![]()
108
5 year OS for germinoma
\>90%
109
Whole ventricle contouring
![]()Lateral ventricles, 3rd vent, 4th vent and pre-pontine cistern expanded by 1 cm for CTV
![]()
110
Contouring for tumor boost germ cell tumor
Fuse T1post and T2 MRI
contour pre-chemo disease and do 1.5 cm expansion
111
Markers for choriocarcinoma
Very high hcg
112
Markers for yolk sac tumor
elevated AFP
113
What is important prognostic consideration for NGGCT
extent of resection, though not always possible given anatomy
114
Treatment strategy for NGGCT
* Maximally safe resection
* Chemotherapy
* carbo/etop alternating with ifos/etop for 4-6 cycles
* Second look surgery if not in CR
* RT
115
What is the RT approach for NGGCT
CSI + primary boost
116
RT strategy for NGGCT
CSI to 36 Gy (20 fractions)
Primary site boost to 54 Gy (tumor + 1 cm)
117
Location of prepontine cistern
![]()
118
Where does craniopharyngioma arise?
Rathke pouch
119
Radiographic hallmarks of craniopharyngioma
Distinct due to cysts and califications
![]()
120
What is the workup for craniopharyngioma
* 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
121
Initial management of craniopharyngioma
* Maximal safe resection via transsphenoidal approach
122
Next step if TSS for craniopharyngioma is GTR
Observation
123
Next step if TSS for craniopharyngioma is STR or bx
Radiation to dose of 54 Gy in 30 fractions of 1.8 Gy
## Footnote
**UNLESS CHILD IS LESS THAN 3**
124
What is recurrence of subtotally resected craniopharyngioma
50%
125
Contouring for craniopharyngioma
* GTV = residual disease including cysts
* CTV = GTV + 1 cm (respecting anatomic boundaries)
* PTV = CTV + 3 mm with daily CBCT
126
What is control rate of GTR for craniopharyngioma
70-85% but associated with significant morbidity
127
Outcomes of STR + RT for craniopharyngioma
85-90%
128
What is a practical RT consideration for treating craniopharyngiomas with RT?
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
129
Late effects of RT to suprasellar region
* DI (increased sodium and AMS)
* Growth hormone dysfunction
* Visual complications
* Moya Moya syndrome with occlusion of carotid arteries
* Hypothalamic dysfunction
130
Symptoms of hypothalamic dysfunction
Sleep disturbances
Weight gain
Memory impairment
131
Appearance of optic glioma
![]()
132
What percentage of RB patients are hereditary?
40% due to germline mutations in RB gene
133
What share of retinoblastomas are bilateral
20-30%
134
For germline RB cases, what is a management consideration
Try to avoid RT if possible given very high risk of secondary malignancy (osteosarcoma)
135
What is the workup for retinoblastoma
* 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
Treatment options for RB
* 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
What is the dose used for plaque brachy for RB
40 Gy with I-125 dosed to apex of tumor
RT delivered over 2-3d
![]()
138
EBRT approach for retinoblastoma
* Refer for protons, if possible
* 45 Gy in 25 fractions of 1.8 Gy
* Cover entire retina and 8 mm of optic nerve
139
Eye preservation if EBRT is used
95% if earlier stage
50% if more advanced stage
Visual acuity is excellent in most, poor if macula is involved
140
Toxicity of retinoblastoma RT
Skin dermatitis
Dry eye
Corneal injuries
Cataracts
Retinopathy
Glaucoma
Secondary malignancies (osteosarcoma)
141
Benefit of prone CSI over supine
* Direct visualization of light fields for better spine field setups (better for therapists)
142
Benefit of supine CSI over prone
More comfortable for kid
Better access to airway for anesthesiologist
More reproducible head tilt
143
RT approach for CSI
* 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
Approach for proton CSI
* 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
Approach for 3D CSI
* 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
How to choose Rx depth point for 3D CSI
Take an average of depth at 2-3 spots in the field
147
What to do if the patient is too long for single spine field?
* Two options
* Try extended distance with 110-115 cm SSD
* Match a second spine field but requires gap an feathering
148
Where are the spinal fields matched?
They are matched at anterior cord which leaves a 3-5 mm gap at the skin surface
![]()
149
How to set WBRT fields for 3D CSI
* 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
What is the formula for collimation of the WBRT field?
arctan [(L/2) / SSD]
L=length of adjacent spinal field at surface
SSD = 100 if normal distance
![]()
151
Typical angle of collimation for WBRT field
10-12 degrees
152
Formula for couch kick of gantry for WBRT field
arctan (L/2)/SAD
L = length of cranial field
SAD = distance to the WBRT axis point
![]()
153
Gap formula if using 2 fields for CSI
* Calculate the contributions of the two spinal fields
* Each is Depth from skin surface x (half distance of spinal field at depth / SAD)
* * ![]()
154
How to feather CSI fields
Consider feathering q9 Gy or weekly
Open cranial field 1 cm inferiorly and shift spine iso 1 cm inf
155
Acute toxicity of CSI
Headaches
N/V
Fatigue
Cytopenias
156
Subacute toxicities of CSI
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
Late toxicies of CSI
Radiation necrosis in ~5%
Cerebral atrophy
hemorrhagic vascular telangiectasias
Pituitary and hypothalamic dysfunction
Infertility, hearing loss
Cataracts
Heart and lung diesease
Chronic myelosuppression
158
What is the second malignancy risk after CSI?
10%+
159
IQ changes after CSI
If CSI given for kids \< 7, all need special ed
Risks are greater with CSI dose, female gender and higher baseline IQ
160
What long term surveillance do kids need after CSI
Auditory
Neurocognitive
Endocrine
Vision checks
161
What are some genetic syndromes associated with Wilms tumor?
WAGR
Denys-Drash (mut WT1)
Beckwith-Widemann (mut WT2)
162
What is the workup of suspected Wilms tumor
* H&P
* Labs: CBC, COMP, Coags, check urine catechols to rule out neuroblastoma
163
General imaging for suspected Wilms tumor
Abdominal US
CT or MR abdomen
CT chest for mets
164
Special imaging studies required for Wilms
If clear cell sarcoma of kidney --\> PET and BMBx
If rhabdoid tumor of kidney --\> brain MRI
165
How to differentiate kids with Wilms from neuroblastoma
* Kids are not generally sick with Wilms
* WT typically doesn't cross midline
* Check urine catechols
166
What is favorable histology wilms tumor?
Classic three cell types WITHOUT anaplasia
167
What is unfavorable histology Wilms?
* Anaplastic (4-5% of cases)
* Clear cell sarcoma of kidney
* Rhabdoid tumor of kidney
168
What are positive prognostic factors for Wilms?
favorable histology
stage I/II
Age \<2
Smaller tumor
169
What are negative prognostic signs for Wilms?
unfavorable histology (anaplasia)
bilteral tumors
gain of 1q
LOH 1p or 16q
170
General management of Wilms tumor
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
What is stage I Wilms?
Limited to kidney
R0 resection
Renal capsule intact
No prior biopsy
172
What is stage II Wilms
R0 resection but with regional extension beyond the capsule or sinus
No biopsy
173
What is stage III Wilms?
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
Stage IV Wilms
Distant metastases
LN+ outside of abdomen/pelvis
175
Stage V Wilms
Bilateral
176
What is chemo for stage I FH Wilms
If \<2 years - none
If \>2 years: 19 weeks of vincristine/actinomycin D
177
What is chemo for stage I UH Wilms
19 weeks of vincristine + actinomycin D
178
Chemo for stage II FH wilms?
Vincristine + Actinomycin D for 25 weeks
179
Chemo for Stage III or IV FH Wilms
Vincristine + Actinomycin D + doxorubicin for 24 weeks
180
Chemo for stage III/IV UH Wilms
24 weeks of CAVE
Cyclophosphamide
Actinomycin D
Vincristine
Etoposide
181
When should chemo or RT be started for Wilms?
Ideally 10-14 days post op
182
What are reasons to delay chemoRT for Wilms?
Diarrhea
Ileus
ANC \< 1000
Hgb \< 10.5
Rhabdoid starts week 6
183
What is RT for stage I with FH?
No RT
184
What is RT for stage II FH Wilms?
No RT
185
What is RT for stage III FH?
Standard risk
Flank RT of 10.8 Gy (1.8 x 6)
186
How to approach RT for focal anaplasia
Stage I-III are considered
* Standard risk
* Flank RT of 10.8 Gy (1.8 x 6)
187
How to approach RT for diffuse anaplasia Wilms
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
How to approach clear cell Wilms?
stage I-III are considered standard risk (1.8 x 6 = 10.8 flank)
189
How to approach RT for rhaboid tumor of kidney
Considered high risk (1.8 x 11 = 19.8 flank)
190
What age group for Wilms is considered high risk?
\>16
Should get 19.8 Gy to flank (1.8 x 11)
191
How to approach if a patient has gross residual disease after Wilms
Deliver boost to 10.8 Gy (1.8 x 6)
192
What is the indication for whole abdomen RT for Wilms?
SPAR
* Spillage
* Peritoneal seeding
* Ascites with +cytology
* Rupture
193
Dose of whole abdomen RT for Wilms
10.5 Gy (1.5 x 7)
194
What additional RT is needed after whole abdomen RT for Wilms?
* 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
What scenarios are high risk Wilms?
* Stage III diffuse anaplasia
* Stage I-III rhabdoid
* Age \> 16
196
How to approach lung mets for Wilms?
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
How to approach liver mets for Wilms tumor?
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
How to approach bone mets for Wilms
25. 2 Gy if \< 16
30. 6 Gy if \>16
199
How to approach brain mets for Wilms
WBRT to 21.6
Boost gross mets to 30.6
200
How to approach stage V Wilms
Preop chemo based on the more advanced tumor
If still unresectable can consider neoadjuvant RT 12-16 Gy
201
How to determine if a Wilms patient has "real" lung mets
Visible on CXR or \>1 cm on CT
202
What should be given to patients getting whole lung RT
PCP prophylaxis
203
Approach to whole lung RT
cardiac sparing IMRT
204
What patients with lung mets should get WLI
If mets are persistent despite chemo
205
How to deliver flank RT for wilms?
AP/PA
206
Field borders for flank RT
* 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
What is size of residual disease to get boost for Wilms?
\>3 cm
Boost to additional 10.8 in 6 fractions
208
When to hold flank RT for Wilms
ANC \< 0.3
Platelets \< 40
If delay 4-7 days, add one fx
If delay 7+ days add 2 extra fx
209
Fields borders for whole abdomen RT
AP/PA to 10.5 Gy in 1.5 daily fractions
Superior: diaphragm
Inferior: bottom of obturator foramen
![]()
210
Dose constraint to intact kidney for kids getting Wilms RT
D100 \<14.4 Gy
50% kidney \< 19.8 Gy
211
What is low risk pediatric Hodgkin?
stage IA or IIA without bulk
212
What is high risk pediatric Hodgkin
IIIB
IVB
213
What is intermediate risk pediatric Hodgkin
IA or IIA with bulk
IAE or IIAE
IB or IIB
IIIA
IVA
214
What is the chemo regimen used for pediatric Hodgkin
ABVE-PC
Adriamycin
Bleomycin
**_Vincristine_**
Etoposide
Prednisone
Cyclophosphamide
215
Treatment of intermediate risk Hodgkin disease
* 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
How is RER defined for Hodgkin disease
\>60% reduction on CT scan
217
How should patients who are RER after 2 cycles ABVE be treated?
2 more cycles of ABVE-PC
If rCR after 4 cycles --\> No ISRT
If not rCR after 4 cycles --\> ISRT to 21 Gy
218
How should patients nonRER or CR be managed after 2 cycles ABVE-PC
2 more cycles of ABVE-PC
2 cycles of DECA
ISRT
219
Dose of ISRT for pediatric Hodgkin
21 Gy (1.5 x 14)
220
How to approach high risk pediatric Hodgkin
ABVE-PC x 5
Deliver 21 Gy ISRT to sites of initial bulk or SER
221
How is bulk defined for pediatric cHL
\>6 cm or \>1/3 length of widest area of mediastinum
222
What is CNS1 for leukemia
Negative cytology
223
What is CNS2 for leukemia
\<5 WBC on cytology
224
What is CNS3 for leukemia
\>5 WBC on cytology
Gross CNS lesions or CN deficits
225
What is role of RT for CNS1 disease
If T-ALL or lymphoblastic leukemia
Consider PCI of 12 Gy in 1.5 Gy fractions
226
What is role of RT for CNS2 disease
If T-ALL or lymphoblastic leukemia
Consider 12 Gy in 1.5 Gy fractions
227
What is role of RT for CNS3 disease
All histologies
WBRT to 18 Gy in 1.8 Gy fractions
228
What share of Ewing sarcoma have mets at diagnosis
25%
229
What is the workup for Ewing sarcoma
* H&P
* Xray of primary site
* CT/MRI of primary site
* CT chest
* PET to assess for mets
230
What is the necessary pathology for Ewings?
BILATERAL bone marrow biopsy
Incisional biopsy of the primary site
231
What is the translocation of Ewing
t11;22
232
General treatment paradigm for Ewing sarcoma
* 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
What is the chemo for Ewing sarcoma
VAC-IE
Vincristine, ADRIAMYCIN, Cyclophosphamide
Alternating with
Ifosfamide, Etoposide
q2 weeks, dose dense
234
What is OS rate for Ewing sarcoma - non metastatic extremities
80%
235
What is the 5 year OS rate for Ewing - pelvis
60%
236
What is 5 year OS rate for metastatic Ewing sarcoma
20% unless isolated
50% if lung only
237
Unresectable areas for Ewing sarcoma
Spine, face, pelvis
238
When is local therapy integrated for Ewing sarcoma
week 12
239
What is the difference between surgery and CRT for local control for Ewing
OS is the same
LC with surgery probably better
Decreased risk of second malignancies
240
Late effects of Ewing treatment
Fractures
Wound complications
Bone/soft tissue hypoplasia
Infertility
Lymphedema
Avascular necrosis
2nd cancers
241
second cancer risk for Ewing sarcoma
6-9% at 20 years
242
Defining volumes for Ewing sarcoma
* 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
Definitive CRT Ewing doses
PTV1 = 45 Gy in 25 fractions (1.8 x 25)
PTV2 = 55.8 (1.8 x 31)
If vertebral body 50.4 Gy
244
When would neoadjuvant doses be used for Ewing
To help achieve R0 resection (but cannot convert unresectable to resectable)
245
Downside of neoadjuvant RT for Ewing sarcom
Impairs bone healing/increases fracture risk
246
Neoadjuvant RT dose for Ewing
Give 36 Gy in 20 fractions
If gross residual disease, boost with 19.8 Gy in 11 fractions
247
Indications for adjuvant RT for Ewing
* Positive or close margin
* \<90% necrosis of tumor on pathology
248
What is a close margin for Ewing sarcoma
\<5 mm for soft tissue
\< 1 cm for bone
249
Adjuvant RT doses for Ewing
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
When should lung mets be treated for Ewing?
After completion of all chemo or during primary RT if concern for ovelap
251
Approach to RT for lung mets for Ewing
Cardiac sparing whole lung IMRT (to bilateral lungs)
252
Dose of whole lung RT for Ewing sarcoma
15 Gy in 10 fx (1.5 Gy per fraction)
253
What about if there is residual disease?
After WLI, can either resect or boost these areas to total of 45 Gy
254
What if there is a pleural effusion with +cytology from Ewing
Treat 15 Gy to pleural cavity
255
How to approach other bone mets from Ewing
Treat all metastatic sites if feasible
Do SBRT total dose of 10 x 5
256
Ewing vs. osteosarcoma imaging on xray
![]()
257
Rhabdo reflects X% of childhood STS
\>50%
258
Epidemiology of Rhabdo
70% of kids are \<10 years old
peak incidence is age 2-5
259
What syndromes are associated with rhabdo
Li-Fraumeni
NF1
Beckwith-Weideman
But MOST ARE SPORADIC
260
Initial workup for rhabdo
* 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
Additional rhabdo workup required for parameningeal location
MRI brain
MRI spine
CSF cytology
262
Additional workup required for bladder/GU/Gyn rhabdo
EUA and cystoscopy
263
Additional workup required for paratesticular rhabdo
Perform scrotal US
NO BIOPSY
Bilateral inguinal LND
Ipsilateral RP LND if age \>10 and/or N+
264
Additional workup required for extremity rhabdo
SLN biopsy or dissection
265
Parameningeal sites
MNOP
* Middle ear, Mastoid
* Nasal cavity or nasopharynx
* fOssa (pteryopalatine or infratemporal)
* Paranasal sinus or parapharyngeal space
![]()
266
General treatment paradigm for rhabdomyosarcoma
* 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
Which are the organs which should be considered for no surgery for rhabdo
Orbit
Vagina
Bladder
Biliary
268
Chemo regimen for rhabdo
VAC-VI
Vincristine
ActinomycinD
Cyclophosphamide
Irinotecan
269
Steps for figuring out appropriate RT plan for rhabdo
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
What are the histologies of rhabdo
Favorable = embryonal, botryoid, spindle cell
Unfavorable = alveolar or undifferentiated
271
Translocation hallmark of alveolar rhabdo
PAX/FOX01 fusion
272
PAX/FOX01 fusion is what translocation
t(1;13) or t(2;13)
273
What are the favorable sites for rhabdo
BONG
* Biliary
* Orbit
* Non-parameningeal H&N
* GU/GYN (non-bladder or prostate)
274
Unfavorable sites for rhabdomyosarcoma
Extremity
Trunk
RP
Bladder or prostate
Parameningeal H&N
275
How is stage determined for rhabdo
Clinically based on pre-op imaging
276
What is S1 rhabdo
Favorable site, N0-1
277
What is stage 2 rhabdo
Unfavorable site, \<5 cm AND N0
278
Stage 3 rhabdo
Unfavorable site, \>5 cm OR N1
279
Stage 4 rhabdo
M1
280
How is Group determined for rhabdo
Pathologically using surgical outcomes
281
What is Group I rhabdo
R0
282
What is Group II rhabdo
R1 resection or N1
283
What is Group III rhabdo
R2 resection
Unresectable
Bx only
284
What is Group IV rhabdo
DM
285
What is low risk rhabdo group?
* Embryonal histology UNLESS
* Unfavorable site (extremity, trunk, RP, parameningeal, bladder, prostate)
* Group III
286
What is high risk rhabdo group?
Metastatic except for embryonal histology age \< 10 (which is IR)
287
What is intermediate risk rhabdo group?
* 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
Outcome for low risk rhabdo
failure free survival 85%
289
Intermediate risk rhabdo outcomes
FFS around 70%
290
Outcomes for high risk rhabdo
OS is ~35%
291
If rhabdo is resected Margin -, what is RT dose?
If embryonal: no RT
If alveolar: 36 Gy in 1.8 Gy fractions (20)
292
If rhabdo is margin positive resection, what group and what RT dose?
Group 2
If embryonal - 36 Gy (1.8)
If alveolar - 36 Gy (1.8)
293
If rhabdo is N+, what group and what RT dose
Group 2
If embryonal - 41.4 Gy (23)
If alveolar - 41.4 Gy (23)
Do 50.4 if nodes are not resected
294
If rhabdo is N+ what should be treated
Primary and nodes even if CR to chemo
295
What dose for Group 3 rhabdo
50.4 to 59.4 Gy in 28-33 fractions
296
What rhabdo tumors should receive 59.4 Gy
Tumors \> 5 cm
297
What is RT dose to orbital rhabdo
Consider 45 Gy but can do 50.4 Gy if poor response to chemo
298
How to approach paratesticular rhabdo
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
How to approach RT for amputated rhabdo
If R0, no tumor bed and no RT
300
What to do if lung mets
Do WLI, cardiac sparing IMRT at end of chemo
301
How to approach rhabdo if CSF+
Consider CSI
302
When to start RT for rhabdo for primary?
Start at week 12 for low and intermediate risk
Start at week 20 for high risk (primary and select metastatic sites)
303
When to start RT if CSF+ or cord compression?
Day 0
Do not do upfront RT if CN palsy or BOS involvement
304
When should metastatic sites be irradiated for rhabdo
Week 50
WLI
Spine/pelvic sites
(avoid marrow suppression during chemo)
Select sites ok to do week 20 with primary
305
What is GTV for rhabdo
pre-chemo, pre-op disease but anatomically confined
306
What is CTV for rhabdo?
GTV + 1 cm
Include LN region or chain if N+
307
What is PTV for rhabdo
CTV + 5 mm
308
If RT is delayed for rhabdo, how to change the Rx
If delayed 2-3w given 1 extra fraction
If delayed 3+ weeks, 2 extra fractions
309
What is the origin cell for neuroblastoma?
Neural crest cells that fail to regress
310
Most common location for neuroblastoma
adrenal gland (35%)
311
What is epidemiology of Neuroblastoma
Most common non-CNS solid tumor of childhood
Most lethal pediatric solid tumor
312
How to describe Neuroblastoma kids
They are SICK (in contrast to Wilms)
Their tumors are typically abdominal
Tumor can cross midline
313
What are the symptoms of Neuroblastoma
Abdominal mass
Pain
Fever
HTN
Classic signs/symptoms
314
Classic signs and symptoms of neuroblastoma
* 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
Imaging workup for neuroblastoma
Plain films
CT CAP
MRI of primary site
Bone and MIBG scans
316
Path diagnosis of neuroblastoma
* Biopsy of the primary site (unlike Wilms)
* Bilateral bone marrow biopsies
317
What labs should be checked for neuroblastoma
Urine catecholamines (produced 90% of time)
CMP
CBC
318
What risk group is relevant for RT
HIGH RISK only
Low risk only if not responding to chemo
319
How to define high risk Neuroblastoma
* Myc-N amplication for all [Stage MS, M, locoregional]
* Metastatic cases age \> 18 months irrespective of Myc status
320
How to define stage MS neuroblastoma
Localized tumor in patients \<18mos with dissemination limited to skin, liver, bone marrow
321
What is the typical treatment sequence for neuroblastoma
* 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
When should RT be offered for neuroblastoma patients
28-42 days post transplant
323
What are the RT doses for neuroblastoma
If resected: 21.6 Gy in 12 fractions
If residual disease: 36 Gy in 20 fractions
324
Contouring for neuroblastoma
* 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
Which neuroblastoma metastatic sites should be irradiated
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
Dose for stage 4S liver tumor
1.5 Gy x 3 (spare kidney)
327
Dose for neuroblastoma cord compression
If \<3 years old: 1.8 Gy x 5 (9 Gy)
If \>3 years old: 1.8 x 12 (21.6 Gy)
328
Neuroblastoma constraint to IPSI kidney
V19.8 \< 50%
V14.4 \< 100%
329
Dose constraint for contralateral kidney
V12 \< 20%
330
Liver constraint for neuroblastoma
V18 \< 25%
331
Lung constraint for neuroblastoma
V15 \< 33%
332
Which histologies need bilateral BM BX?
* Neuroblastoma
* Rhabdomyosarcoma
* Ewing sarcoma
333
OS for high risk neuroblastoam
70%
