Pediatrics Flashcards
1
Q
Dose of RT for orbital embryonal rhabdo on ARST0331
A
45 Gy [Group 3]
2
Q
Dose for group III disease on ARST0331
A
45 Gy orbit
50.4 all other sites
3
Q
Dose for R1 rhabdo per ARST0331
A
41.4 Gy
4
Q
Research question on COG ARST0331 rhabdo
A
reduction of cyclophosphamide dose to reduce sterility
5
Q
When does RT start on ARST0331
A
week 13
6
Q
What chemo is given on ARST0331
A
VAC - reduced C dose
7
Q
ACNS0331 medullo trial question
A
- CSI dose (for young kids)
2. posterior fossa or involved field boost
8
Q
ACNS0331 medullo Doses of RT used (age 3-7)
A
Age 3-7: 23.4 vs. 18 Gy CSI –> posterior fossa or IF boost to 54 Gy
9
Q
ACNS0331 medullo Doses of RT used (age 8+)
A
23.4 CSI randomization to either PF or IF boost to 54 Gy
10
Q
Chemo used on ACNS0331
A
Concurrent vincristine
then adjuvant vincristine/cisplatin/CCNU alternating with vincristine/cyclophosphamide
11
Q
ACNS0331 medullo tumor bed boost contour
A
GTV + cavity plus 1.5 cm CTV margin off bone/tentorium
12
Q
Predictor of anesthesia complications for kids
A
duration of procedure
13
Q
Peak age for medulloblastoma
A
5-9 years old
14
Q
How many medulloblastomas disseminated at diagnosis
A
1/3
15
Q
What imaging is needed for medullo
A
brain and spine MRI
16
Q
When should CSF cytology be obtained
A
10-14 days after surgery
17
Q
What other tests should be obtained
A
- baseline neurocog eval
2. audiology eval
18
Q
Chang stage M0
A
No metastasis
19
Q
Chang stage M1
A
Microscopic tumor in CSF
20
Q
Chang stage M2
A
Gross nodules intracranially beyond primary
21
Q
Chang stage M3
A
Gross nodules in spinal subarachnoid space
22
Q
Chang stage M4
A
mets outside Craniospinal axis
23
Q
What is most favorable medullo subtype
A
Wnt (90% OS)
24
Q
Wnt subtype associated with which syndrome
A
APC mutations - Turcot syndrome
25
SHH subtype prognosis
good for infants, intermediate for children
| 80% OSS
26
Worst prognosis medullo class
Group 3
27
Group 3 medullo has which mutation
Myc amplification
28
Group 4 prognosis
second to worst, above Group 3
29
What is standard risk medullo
M0
GTR or less than 1.5 cm of residual disease
Classic or desmoplastic histology
30
Treatment for standard risk medullo
CSI to 23.4
| Posterior fossa or IF boost to 54
31
Chemo for medullo
```
weekly vincristine (controversial)
then VCR - vincristine, cisplatin, CCNU
```
32
Prognosis for standard risk medullo
86% OS
33
Intermediate risk medullo definition
Standard risk plus diffuse anaplasia or large cell variant
34
Intermediate risk medullo
Treat with 36 Gy CSI like high risk
35
High risk medullo definition
M+ disease
| STR with >1.5 cm gross residual
36
Treatment of high risk medullo
CSI to 36 Gy
PF/IF boost to 54-55.8 Gy
Concurrent vincristine
adjuvant cisplatin containing chemo
37
Prognosis of high risk medullo
EFS 60-70%
38
Total adjuvant chemo
9 cycles, 55 weeks
39
Results of ACNS0331 re dose
18 Gy inferior to 23.4 Gy
40
Results of ACNS0331 re field design
involved field boost to 54 Gy is ok
41
ACNS0331 risk of local failure
1-5%
42
Epidemiology of craniopharyngioma
Bimodal - infants/children and adults aged 55-65
43
Location for craniopharyngioma
remnant of Rathke's pouch
44
Craniopharyngioma CT appearance
calcified suprasellar mass with cystic/solid components
45
Treatment of craniopharyngioma
GTR --> observation
| Maximal safe resection --> RT (90% control)
46
CTV for craniopharyngioma
GTV + 1 cm
47
Dose for cranio
54 Gy is preferred since doses <54Gy associated with LF
48
Germ cell tumors are more common in males/females
Males
49
Where is most common intracranial GCT
pineal
50
Epidemiology of intracranial GCT
teens/puberty
| Higher predominance in Japan/East Asia
51
Tumor marker profile for pure germinoma
Normal bHCG or slightly elevated
| Normal AFP
52
Tumor marker profile for embryonal carcinoma
Elevated bHCG
| Elevated AFP
53
Tumor marker profile for yolk sac tumor
```
Normal bHCG
Elevated AFP (very high)
```
54
Tumor marker profile for choriocarcinoma
Very elevated bHCG
| Normal AFP
55
Tumor marker profile for teratoma
Normal bHCG
| Normal AFP
56
Role of biopsy for GCT
do unless markers are positive
57
Germinoma pathology
small round blue cells
58
Good prognosis GCT
pure germinoma, mature teratoma
59
Cure rate for pure germinomas
95%
60
Staging for GCT
M0 - CSF negative
| M1 - CSF positive
61
Treatment for M0 pure germinoma
Radiation alone
| 21-24 Gy whole ventricle RT with boost to 30-36 Gy in 1.5 Gy fractions
62
Treatment for M1 pure germinoma
Two options
1. RT alone: 24-30 Gy CSI --> IF boost to 45 Gy
2. ChemoRT: carbo-etop x 2 --> 24 Gy CSI --> boost to 30-36 Gy
63
NGGCT treatment
Chemo first (4-6 cycle of carbo/etop then ifos/etop)
If CR with normal markers --> RT
If PR --> second look surgery
64
Dose of RT for NGGCT
CSI 36 Gy
| IF boost to 54 Gy
65
Syndrome with risk factor of ependymoma
NF2
66
Where does spinal cord end in children
L3
67
Where does thecal sac end in children
S2
68
What is most important prognostic factor for ependymoma
extent of resection
69
Most common location for ependymoma
4th ventricle/PF
70
How do ependymomas spread?
along spinal axis
71
WHO I ependymoma
myxopapillary ependymoma or subependymoma
72
Treatment of WHO grade I
surgery alone
| RT for STR or recurrence
73
WHO grade II ependymoma
ependymoma
74
WHO grade III ependymoma
anaplastic
75
WHO grade IV ependymoma
ependymoblastoma (now PNET)
76
Group A ependymomas
Most common, posterior fossa, more aggressive
| H3K27
77
Group B ependymoma
more favorable, older children
78
Treatment approach for ependymoma
GTR followed by IFRT
79
RT approach for ependymoma
conformal RT with CTV margin of 1 cm
80
RT dose for ependymoma
59.4 in 33
| 54 if <18 months
81
Genetic syndrome associated with LGG
NF1
82
What is most common pediatric brain tumor
LGG
83
Most common location of LGG
infratentorial
84
Treatment of pediatric LGG
GTR if feasible: 10 year PFS 80-90%
| Chemo if young to delay RT
85
CTV for LGG
GTV + 5mm
86
Recommended dose for HGG
54 Gy if GTR
| 60 Gy if STR
87
Dose for DIPG
54 Gy in 1.8 Gy fractions
88
What proportion of Rb is heritable
25%
89
Plaque options for Rb lesion
6-15 mm in size
| <10 mm depth
90
Dose of plaque
40 Gy to apex
91
EBRT dose for locally advanced
45 Gy
92
Neuroblastoma is most common malignancy in children < X months
18
93
With neuroblastoma older children do better/worse
worse
94
What labs to check for neuroblastoma
VMA, HVA, epi, norepi in urine in 90% of cases
95
MIBG is an analogue of what hormone
norepi
96
MYCN amplification is associated with better/worse survival
worse
97
Stage I neuroblastoma
Localized, completely resected
98
Stage IIA neuroblastoma
Localized, incomplete resection, N0
99
Stage IIB neuroblastoma
Localized (R0/R1) + ipsi lymph node
100
Stage III neuroblastoma
Unresectable crossing midline +/- LN
101
Stage IV neuroblastoma
distant mets
102
Stage IV-S neuroblastoma
primary tumor with mets in skin, liver, bone in <1 year old --> good prognosis
103
Low risk treatment
surgery alone
104
High risk neuroblastoma treatment approach
```
Induction chemo
Radical surgery
Myeloablative consolidative therapy (x2)
Local radiation
Cis-retinoic acid or targeted therapies
```
105
What is the volume used for radiation for neuroblastoma
Post induction chemo
| Pre surgery
106
Dose to preop primary
21.6 (boost to 36 if residual)
107
Dose to mets
21.6
108
Dose for 4S with massive hepatomegaly
4.5 / 3 fx
109
Dose for cord compression in young child
9 / 5
110
How does cis retinoid acid work
causes differentiation
111
Syndromes that predispose to Wilms tumor
Beckwidth-Wiedemann
WAGR
Denys-Drash
112
What does WAGR stand for
Wilms, Aniridia, ambiguous genitalia, MR
113
What is mutation associated with WAGR
WT1
114
When should biopsy be done for Wilms
Never, unless unresectable or bilateral disease
115
Stage I Wilms
limited to kidney
completely excised
intact renal capsule
uninvolved vessels
116
Stage II Wilms
Completely resected but tumor extends regionally (capsule, soft tissue, blood vessel outside parenchyma)
117
Stage III Wilms
```
BSLURPP
Biopsy
STR/+margin
LN (abdominal)
Unresectable
Rupture/spillage
Piecemeal resection
Perionteal implant
```
118
Stage IV Wilms
hematogenous mets
| LN mets outside abdominopelvic region
119
Stage V Wilms
bilateral renal involvement
120
Approach for high risk Wilms tumor
1. Preop chemo
2. Surgery
3. Postop RT
121
Chemo for Wilms
Stage I/II: Vincristine + actinomycin-D
| Stage III/IV adriamycin 3+ agents
122
Dose for whole lung RT
12/6
123
When should whole lung be used
lung mets on CXR (not if only visible on CT)
124
Dose for flank RT
10.8 in 6 fractions
| Boost to 19.6
125
Ideal start date of RT for Wilms
9-14 days after surgery
126
Dose for rhabdoid tumor or stage III with diffuse anaplasia
19.8
127
Favorable rhabdo sites
1. non-parameningeal HN
2. orbit
3. GU non-bladder/prostate
4. biliary tract
128
Unfavorable rhabdo sites
1. parameningeal
2. bladder/prostate
3. extremities
4. trunk/RP
129
Which subtype associated with FOX01 fusion
alveolar
130
FOX01 is what translocation
t(2;13)
131
Wilms RT approach for flank
Preop tumor bed + 1cm
Do not split vertebral bodies to prevent scoliosis
Cross midline 1 cm beyond vert body to include bilateral PA if tumor is N+
132
Stage 1 rhabdo
favorable sites (any size, any N)
133
Stage 2 rhabdo
<5 cm unfavorable site, N0
134
Stage 3 rhabdo
>5cm unfavorable site, N+
135
Stage 4 rhabdo
mets
136
Group 1 rhabdo
completely excised
137
Group 2 rhabdo
microscopic margin or LN+
138
Group 3 rhabdo
gross residual or unresected
139
Group 4 rhabdo
mets
140
Low risk rhabdo
stage 1 rhabdo embryonal
| stage 2 or 3 embryonal group 1/2
141
High risk rhabdo
mets other than age <10 embryonal
142
Approach to treat low risk rhabdo
VAC x 12 weeks --> surgery and/or RT --> more chemo
143
Approach to treat high risk rhabdo
RT at week 12 or 20, treat mets after completion of chemo
144
When to start RT for low/intermediate risk
week 12
145
Rhabdo chemo backbone
VAC
Vincristine
Actinomycin
Cyclophosphamide
146
Dose for favorable sites, group I
36 Gy
147
Dose for unfavorable, group 2, N0
36 Gy
148
Dose for unfavorable, group 2, N+
41.4 Gy
149
Dose for Group 3 orbital
45 Gy
150
Dose for Group 3, gross disease < 5cm
50.4 Gy
151
Dose for Group 3, gross disease > 5cm
59.4 Gy
152
Dose for whole lung RT
15 Gy in 10
153
Approach for paratesticular rhabdo
If >10 --> RPLND
| If <10 --> thin slice CT to eval for nodes
154
Peak age for Ewing
15
155
Epidemiology
more common in whites
| M:F 1.5:1
156
Ewing sarcoma usual presents where in long bones
diaphysis (central)
157
Osteosarcoma usually presents where in long bones
metaphysis
158
Chemo for Ewing
```
VAC-IE
Vincristine
Adriamycin
Cyclophosphamide
Ifos
Etoposide
```
159
When is RT given for Ewing
12 weeks
160
RT dose for gross disease Ewing
55.8
161
RT dose for microscopic Ewing
50.4
162
What to treat for Ewing
45 Gy to pre-chemo + 2 cm
| CD to prechemo bone volume, postchemo soft tissue volume
163
Low risk Hodgkin stages
IA
| IIA
164
Treatment of low risk Hodgkin
ABVE-PC x 4
21 Gy ISRT for PR
No RT for CR
165
Intermediate risk Hodgkin stages
IIA (bulk), IIIA, IVA (w or wo bulk)
166
Treatment of intermediate risk Hodgkin
```
ABVE-PC x 2
Assessment of Response
ABVE-PC x2
If RER and CR: no RT
If PR: 21 Gy
If SER: escalate to DECA --> 21 Gy
```
167
Definition of RER
60% reduction in CT PPD
168
High risk Hodgkin
IIIB or IVB
169
Role of RT for high risk Hodgkin
ISRT to initial PR or sites of bulk
170
Risk factor for cardiac complications in 5 year Ewing sarcoma survivors
chest RT
| NOT anthracycline dose
171
Any alveolar rhabdo histology is considered what risk
intermediate
| unless mets --> high risk
172
Intermediate risk peds Hodgkin includes what stages
```
IA with bulk
IIA with bulk
I-IIAE with or without bulk
I-IIB with or without bulk
IIIA with or without bulk
IVA with or without bulk
```
173
What endpoints were improved in Hodgkin pts with RER
EFS
| OS
174
Standard Wilm's fields for postop flank RT
Preop tumor bed + 1 cm
175
RT dose for rhabdoid tumor of kidney or stage III with anaplasia
19.8 Gy
176
what is 4S neuroblastoma
localized primary with only skin, liver or bone marrow involvement in infant < 12 months
177
Rate of secondary malignancies in 35 year followup for Ewing survivors
14%
178
OS of osteosarcoma with surgery alone
20%
179
OS of osteosarcoma with surgery + chemo
70% at 5 years
180
ATRT treatment
surgery
if children < 3: 50.4 to primary site alone
if children > 3: add 36 Gy CSI
181
Parinaud symptoms
Impaired upward gaze
psuedo Argyll-Robertson pupil
vertical nystagmus
182
psuedo Argyll-Robertson pupil
impaired pupil reaction to light but not accomodation
183
When do kids with T-ALL get testicular RT
remains positive after chemo
184
Dose of testicular RT
24 Gy / 12
185
CNS 1 leukemia
CSF negative
186
CNS 2 leukemia
WBC count <5 and blasts on cytology
187
CNS 3 leukemia
WBC >5 and blasts on cystology or clinical signs of CNS leukemia
188
Ewing translocation
t(11;22) EWS-FLI1
189
What stages of favorable histology Wilms require RT
stage III and IV
190
Chemo used for Wilms
Vincristine + actinomycinD
191
Radiation dose for anaplastic ependymoma
59.4 (54 Gy then 5.4 Gy boost)
| If younger than 18 months: ok to do 54 only
192
Most common malignant brain tumor of childhood
medulloblastoma
193
Peak incidence of medulloblastoma
5-9
194
10 year cumulative incidence of second malignancies in medullo patients treated with CSI, PF boost, chemo
4%
195
Median age of presentation for neuroblastoma
22 months
196
Most common extracranial solid tumor in children
neuroblastoma
197
Ewing sarcoma dose for unresectable
45 Gy to pre-chemo volume + 2cm
| CD to 55.8 Gy to prechemo bone and postchemo soft tissue
198
When does posterior fossa syndrome occur
1-2 days after resection of midline PF tumor
199
Symptoms of PF syndrome
mutism
difficulty swallowing
emotional lability
ataxia
200
How should treatment change for medullo kids with PF syndrome?
It shouldn't
201
5 year OS for average risk medullo per ACNS 0331
85%
202
Local recurrence per ACNS 0331
2-4% (no difference if IF boost)
203
Which lesions of suspected NGGCT do not require bx?
bifocal pineal and suprasellar tumors
| pineal with DI
204
What did NGGCT study conclude about whole ventricle vs. CSI
More relapses, not recommended
| Standard remains 36 Gy CSI --> boost to 54
205
What stage of kidney rhabdoid tumor should get RT
I-III (19.8 Gy)
206
Doses for Wilms tumors with anaplasia
Stage III with diffuse anaplasia --> 19.8
Stage I-III with focal anaplasia --> 10.8
Stage I-II with diffuse anaplasia --> 10.8
Stage I-III clear cell sarcoma of kidney --> 10.8
207
RT dose for residual Wilms disease measuring > 3cm
10. 5 whole abdomen
| 10. 5 boost
208
Peak incidence of Wilms
3-4 years for unilateral
209
Denys-Drash syndrome etiology
WT1 gene on chromosome 11
210
Denys-Drash symptoms
Wilms
pseudohermaphroditism
masangial renal sclerosis
211
Beckwith-Widemann symptoms
```
macroglossia
macrosomia
midline abdominal wall defects
ear creases
neonatal hypoglycemia
hemihypertrophy
```
212
Prognosis of favorable histology WIlms
>90% unless stage IV (80%)
213
Chemo for Ewing
```
VAC-IE
Vincristina
Adriamycin
Cytoxan
Ifos
Etop
```
214
Local failure risk after RT for Ewing
overall 10-20% but site dependent
| pelvis is 20-30%
215
Preferred treatment for low grade osteosarcoma
wide surgical excision
216
Preferred mgmt of high grade osteosarcoma
neoadjuvant chemo --> surgical resection --> adjuvant RT if +margins or significant necrosis
217
What chemo is used for osteosarcoma
MAP - methotrexate, adriamycin, cisplatin
218
Study of 36 Gy vs. 23.4 Gy for standard risk medullo without chemo concluded?
Higher relapse rate in 23.4 Gy arm
219
Typical IHC markers for rhabdo
Myogenin
| MyoD1
220
Tumors positive for S100 staining
melanoma
schwannoma
MPNST
221
Rate of incidentally detected pulm nodules in otherwise healthy children
40%
222
What dose of HPA RT can cause altered puberty timing?
18-24 Gy
223
What is threshold of developing gonadotropin insufficency?
>30 Gy
224
Mutation associated with embryonal rhabdo
DICER1
225
Surgery for Wilms
radical nephroureterectomy with ipsilateral LN sampling
226
Proportion of Ewing sarcoma patients metastatic at diagnosis
20-25%
227
Regimen for medullo if patient cannot get chemo
36 Gy CSI
| PF boost to 55.8
228
Locations of bifocal germinoma
Pineal gland
| Suprasella
229
Locations of trilateral retinoblastoma
Bilateral retina
| Pineal
230
RT doses used for trilateral RB
36 Gy spine
45 Gy cranium
50.4 to pineal
231
Is hereditary RB dominant or recessive?
dominant
232
Ewing sarcoma radiographic finding
onion skinning
233
Osteosarcoma radiographic finding
sunburst pattern near metaphysis
234
Where should optimal CSI match line be
as low as possible to avoid PA exit through mouth
| usually C5-6
235
How is spinal disease boosted for high risk medullo
total of 45 Gy if above the terminus
| 50.4 Gy if below terminus
236
Role for RT in GTR of LGG
none
237
Most common cancer of teenagers
Hodgkin lymphoma
238
Osteosarcoma epidemiology
More common in Males/Blacks
| Bimodal - teens and 65+
239
Treatment approach for pineoblastoma
Similar to high risk medullo
36 Gy CSI with conucrrent vincristine
IF boost to 55.8
Adjuvant cis based chemo
240
How to manage bifocal germinoma
Platinum chemo followed by full ventricle RT
| 24 Gy WVRT followed by boost to 45
241
Dose of RT for neuroblastoma cord compression
Under age 3: 9 Gy (1.8 x 5)
| Over age 3: 21.6 Gy
242
What is whole lung dose for Wilms
12 Gy
243
Which Wilms patients should get whole lung
unfavorable histology and lung mets visible on CXR (anaplasia)
if FAVORABLE histology --> chemo
244
For Wilms lung lesions - if visible on CT only they should be > X mm in order to get WLI
10 (1 cm)
245
Which low risk T-ALL patients need cranial RT
CNS 3
246
Dose of RT for CNS 3 disease
18 Gy in 10 fx
247
When do CNS 1 and 2 get PCI
intermediate/high risk
248
Dose of PCI
12 Gy in 1.5 Gy fx
249
Tyoical PCI field
brain and 1/2 globes and optic nerves
| inferior border is c2/c3
250
Treatment of intracranial NGGCT
36 Gy CSI
| Boost to 54 (1.8 Gy per fx)
251
Treatment of intracranial germinoma
24 Gy whole ventricle
| Boost to 45 (1.5 Gy per fx)
252
Which hormone usually declines first after irradiating pituitary
GH
Followed by FSH/LH
TSH/ACTH later
253
Median age of ependymoma
3-5 years old
254
Median age of medullo
5-9 years old
255
Inferior border of CSI
bottom of thecal sac
256
What stage is a wilms which was biopsied but is otherwise intact
stage III
257
Like what tumors are supratentorial neuroepithelial tumors managed?
High risk medulloblastoma
| 36 Gy CSI + 19.8 Gy boost to PF
258
What is current COG protocol testing for high risk medullo
Efficacy of carboplatin with RT and isotretinoin as pro-apoptotic agent
259
Current chemo regimen for rhabdo
VAC
Vincristine
ACTINOMYCIN
Cyclophosphamide
260
Dose for orbital rhabdo
45 Gy if CR to VAC chemo
| 50.4 Gy if PR to chemo
261
What stages of Wilms with focal anaplasia should get RT
I-IV
262
Mutation associated with ATRT
INI-1
| SMARCB1 gene family
