Pediatrics Flashcards
Dose of RT for orbital embryonal rhabdo on ARST0331
45 Gy [Group 3]
Dose for group III disease on ARST0331
45 Gy orbit
50.4 all other sites
Dose for R1 rhabdo per ARST0331
41.4 Gy
Research question on COG ARST0331 rhabdo
reduction of cyclophosphamide dose to reduce sterility
When does RT start on ARST0331
week 13
What chemo is given on ARST0331
VAC - reduced C dose
ACNS0331 medullo trial question
- CSI dose (for young kids)
2. posterior fossa or involved field boost
ACNS0331 medullo Doses of RT used (age 3-7)
Age 3-7: 23.4 vs. 18 Gy CSI –> posterior fossa or IF boost to 54 Gy
ACNS0331 medullo Doses of RT used (age 8+)
23.4 CSI randomization to either PF or IF boost to 54 Gy
Chemo used on ACNS0331
Concurrent vincristine
then adjuvant vincristine/cisplatin/CCNU alternating with vincristine/cyclophosphamide
ACNS0331 medullo tumor bed boost contour
GTV + cavity plus 1.5 cm CTV margin off bone/tentorium
Predictor of anesthesia complications for kids
duration of procedure
Peak age for medulloblastoma
5-9 years old
How many medulloblastomas disseminated at diagnosis
1/3
What imaging is needed for medullo
brain and spine MRI
When should CSF cytology be obtained
10-14 days after surgery
What other tests should be obtained
- baseline neurocog eval
2. audiology eval
Chang stage M0
No metastasis
Chang stage M1
Microscopic tumor in CSF
Chang stage M2
Gross nodules intracranially beyond primary
Chang stage M3
Gross nodules in spinal subarachnoid space
Chang stage M4
mets outside Craniospinal axis
What is most favorable medullo subtype
Wnt (90% OS)
Wnt subtype associated with which syndrome
APC mutations - Turcot syndrome
SHH subtype prognosis
good for infants, intermediate for children
80% OSS
Worst prognosis medullo class
Group 3
Group 3 medullo has which mutation
Myc amplification
Group 4 prognosis
second to worst, above Group 3
What is standard risk medullo
M0
GTR or less than 1.5 cm of residual disease
Classic or desmoplastic histology
Treatment for standard risk medullo
CSI to 23.4
Posterior fossa or IF boost to 54
Chemo for medullo
weekly vincristine (controversial) then VCR - vincristine, cisplatin, CCNU
Prognosis for standard risk medullo
86% OS
Intermediate risk medullo definition
Standard risk plus diffuse anaplasia or large cell variant
Intermediate risk medullo
Treat with 36 Gy CSI like high risk
High risk medullo definition
M+ disease
STR with >1.5 cm gross residual
Treatment of high risk medullo
CSI to 36 Gy
PF/IF boost to 54-55.8 Gy
Concurrent vincristine
adjuvant cisplatin containing chemo
Prognosis of high risk medullo
EFS 60-70%
Total adjuvant chemo
9 cycles, 55 weeks
Results of ACNS0331 re dose
18 Gy inferior to 23.4 Gy
Results of ACNS0331 re field design
involved field boost to 54 Gy is ok
ACNS0331 risk of local failure
1-5%
Epidemiology of craniopharyngioma
Bimodal - infants/children and adults aged 55-65
Location for craniopharyngioma
remnant of Rathke’s pouch
Craniopharyngioma CT appearance
calcified suprasellar mass with cystic/solid components
Treatment of craniopharyngioma
GTR –> observation
Maximal safe resection –> RT (90% control)
CTV for craniopharyngioma
GTV + 1 cm
Dose for cranio
54 Gy is preferred since doses <54Gy associated with LF
Germ cell tumors are more common in males/females
Males
Where is most common intracranial GCT
pineal
Epidemiology of intracranial GCT
teens/puberty
Higher predominance in Japan/East Asia
Tumor marker profile for pure germinoma
Normal bHCG or slightly elevated
Normal AFP
Tumor marker profile for embryonal carcinoma
Elevated bHCG
Elevated AFP
Tumor marker profile for yolk sac tumor
Normal bHCG Elevated AFP (very high)
Tumor marker profile for choriocarcinoma
Very elevated bHCG
Normal AFP
Tumor marker profile for teratoma
Normal bHCG
Normal AFP
Role of biopsy for GCT
do unless markers are positive
Germinoma pathology
small round blue cells
Good prognosis GCT
pure germinoma, mature teratoma
Cure rate for pure germinomas
95%
Staging for GCT
M0 - CSF negative
M1 - CSF positive
Treatment for M0 pure germinoma
Radiation alone
21-24 Gy whole ventricle RT with boost to 30-36 Gy in 1.5 Gy fractions
Treatment for M1 pure germinoma
Two options
- RT alone: 24-30 Gy CSI –> IF boost to 45 Gy
- ChemoRT: carbo-etop x 2 –> 24 Gy CSI –> boost to 30-36 Gy
NGGCT treatment
Chemo first (4-6 cycle of carbo/etop then ifos/etop)
If CR with normal markers –> RT
If PR –> second look surgery
Dose of RT for NGGCT
CSI 36 Gy
IF boost to 54 Gy
Syndrome with risk factor of ependymoma
NF2
Where does spinal cord end in children
L3
Where does thecal sac end in children
S2
What is most important prognostic factor for ependymoma
extent of resection
Most common location for ependymoma
4th ventricle/PF
How do ependymomas spread?
along spinal axis
WHO I ependymoma
myxopapillary ependymoma or subependymoma
Treatment of WHO grade I
surgery alone
RT for STR or recurrence
WHO grade II ependymoma
ependymoma
WHO grade III ependymoma
anaplastic
WHO grade IV ependymoma
ependymoblastoma (now PNET)
Group A ependymomas
Most common, posterior fossa, more aggressive
H3K27
Group B ependymoma
more favorable, older children
Treatment approach for ependymoma
GTR followed by IFRT
RT approach for ependymoma
conformal RT with CTV margin of 1 cm
RT dose for ependymoma
59.4 in 33
54 if <18 months
Genetic syndrome associated with LGG
NF1
What is most common pediatric brain tumor
LGG
Most common location of LGG
infratentorial
Treatment of pediatric LGG
GTR if feasible: 10 year PFS 80-90%
Chemo if young to delay RT
CTV for LGG
GTV + 5mm
Recommended dose for HGG
54 Gy if GTR
60 Gy if STR
Dose for DIPG
54 Gy in 1.8 Gy fractions
What proportion of Rb is heritable
25%
Plaque options for Rb lesion
6-15 mm in size
<10 mm depth
Dose of plaque
40 Gy to apex
EBRT dose for locally advanced
45 Gy
Neuroblastoma is most common malignancy in children < X months
18
With neuroblastoma older children do better/worse
worse
What labs to check for neuroblastoma
VMA, HVA, epi, norepi in urine in 90% of cases
MIBG is an analogue of what hormone
norepi
MYCN amplification is associated with better/worse survival
worse
Stage I neuroblastoma
Localized, completely resected
Stage IIA neuroblastoma
Localized, incomplete resection, N0
Stage IIB neuroblastoma
Localized (R0/R1) + ipsi lymph node
Stage III neuroblastoma
Unresectable crossing midline +/- LN
Stage IV neuroblastoma
distant mets
Stage IV-S neuroblastoma
primary tumor with mets in skin, liver, bone in <1 year old –> good prognosis
Low risk treatment
surgery alone
High risk neuroblastoma treatment approach
Induction chemo Radical surgery Myeloablative consolidative therapy (x2) Local radiation Cis-retinoic acid or targeted therapies