Mini Humans Flashcards
Peds brain tumors which ones do we not resect
Germinoma, NGGCT, Optic and brainstem Glioma
Only Peds CNS to biopsy
Germinoma
Only CNS peds tumor that doesnt go to 54 Gy
Germinoma
Carbo/etop x 4
M0: whole WVRT 21 Gy + boost to 30 Gy (36 PR)
M1: CSI 24 Gy -> boost to 45 Gy
Posterior Fossa Syndrome
PF syndrome (24-48 hrs after surgery):
-15% after craniotomy
SAME
- Swallowing dysfxn
- Ataxia (truncal)
- Mutism (cerebellar)
- Emotional lability
- can also have respiratory failure
- do NOT delay RT – improves over 1-6 months
Medulloblastoma imaging timing
Imaging:
MRI brain pre-op and 24-72 hr post-op
MRI total spine; then 10-14 days post (false positive if too early)
Medulloblastoma treatment algorithm
Imaging -> do not get CSF or biopsy -> straight to MSR-> CRT within 31 days post-op -> adj chemo (GTR achieved 90% of time)
Don’t forget post-op MRI within 48 hrs and spine MRI at day 10 followed by CSF
Medulloblastoma prognosis subtype
: WNT > group 4 > SHH > Group 3 bad
Medullo standard risk adjuvant treatment
Standard risk:
CSI 23.4 Gy + tumor bed boost (IF) to 54 Gy with concurrent vincristine (1.5 mg/m2 q week)
Adjuvant chemo
Medullo High risk adjuvant treatment
High risk: M+, STR, anaplasia
CSI 36 Gy + IF boost to 54 Gy with concurrent vincristine
Adjuvant chemo
Boosts:
Focal at/above cord: 45
Focal below cord: 50.4
Diffuse: CSI to 39.6
Adjuvant chemo: platinum, CCNU, vincristine x 8 cycles – NOT PCV (procarbazine)
Medullo High risk criteria
M+, STR, anaplasia
Medullo < 3 yo
Infant:
If <3 yo: max safe resection chemo alone (Head Start)
- Reserve RT for salvage or progression
- If you have to treat with RT, use IFRT 54 Gy (1 cm margin supratentorral, 1.5 cm margin infratentorial)
CSI script Medullo
1) CT based simulation - will fuse pre-op and post-op MRI
2) Prone immobilization with Aquaplast mask – also make mold in supine position – head extended to avoid divergence into the mandible, shoulders down – BBs on mask, shoulders, lumbosacral spine
-anesthesia if <11 years
3) Scan vertex to mid femur (2.5 mm slices thru brain, 5 mm elsewhere)
4) Contour globes, lenses, brain (including cribriform plate), cauda
5) Set spine field first
Sup: C7 (as low as possible without going through shoulders)
Inf: thecal sac (S2/S3)
Lat: 1 cm lat to vertebral body;
spade at sacrum to include SI joints
- use a single field if length < 40 cm
- if > 36 cm, can either:
a. Treat at 120 cm SSD
b. Use two fields
6) If using two fields, match anterior to cord (post edge of vertebral body) with skin gap equation
7) Next set-up the brain field. Parallel opposed laterals (or RAO/LAO to minimize divergence into C/L eye). Iso at mid plane (in same coronal plane as spine field).
8) Rotate collimator to match the divergence of the spine field
9) Kick the couch toward the gantry to match divergence of brain field
Note: theoretical underdose of cribiform
Brain – use RAO/LAO to avoid divergence of brain field into contralateral retina
10) Feather all junctions by increasing middle field and decreasing brain and inf spinal fields by 0.5 cm after every 9 Gy (1 week)
11) Image WBRT field daily, apply z shifts with table indexing for lower field isocenters
12) Image all fields weekly
13) Boost
IF boost
CTV = pre-op tumor + tumor bed + 1.5 cm (cropped at tentorium and bone)
Anatomically confined to the posterior fossa, trim inside tent/bones
PTV = CTV + 0.3-0.5 cm
Medullo what to check before treatment
Before RT, check that ANC > 1000 and plt > 100K.
If not, start with boost then do CSI after
Germ cell tumor
AFP or high B-HCG (>50)
indicates NGGCT
Embryonal – bHCG (> 50) + AFP (>10)
Yolk sac – AFP
Chorio - >> bHCG
Germ cell when to biopsy
Pure germinoma if markers elevated do not have to
Germinoma treatment M0
Chemo -> (biopsy if incomplete response) -> whole ventricle 21 Gy boost IF 30 (36 PR)
Germinoma treatment M1
M1:
Chemo -> 21 Gy CSI boost to 36 if CR
NGGCT treatment
Chemo x 6 -> restage
-chemo: alternating cis/etoposide and ifos/etop
If CR: RT
If < CR: max safe resection -> post-op RT
-RT: CSI 36 Gy + boost 54 Gy
Pineoblastoma
treat like high risk medullo:
CSI 36 Gy + IF boost to 54 Gy with concurrent vincristine
Adjuvant chemo
Boosts:
Focal at/above cord: 45
Focal below cord: 50.4
Diffuse: CSI to 39.6
Adjuvant chemo: cisplatinum, lomustine, vincristine x 8 cycles
Ependymoma workup
MRI brain/spine
-pre-op and 10-14 days post-op
LP 10-14 days post-op and after MRI spine
-contraindicated pre-op for posterior fossa mass for risk of herniation
Ependymoma
MRI spine/LP negative: treatment
MRI spine/LP negative:
Max safe resection (be aggressive!) -> post-op IFRT to 54)
-goal is GTR –chemo then re-resecting if needed to achieve GTR
Historically no role for chemo unless < 3 yrs s/p GTR to delay RT
Ependymoma
MRI spine/LP positive: treatment
MRI spine/LP positive:
Max safe resection -> post-op CSI to 36 Gy
-boost spine dz to 45, 50.4-59.4 if below cord
-boost intracranial primary to 59.4
Ependymoma start within
56 days
Ependymoma volumes and dose
GTV=tumor bed + residual + tissues originally involved w tumor
CTV=GTV + 5 mm
PTV=CTV + 5 mm
Dose: 59.4/1.8
Optic Glioma workup
Fundoscopic and CN exam
Visual field testing
No biopsy!!
Optic Glioma treatment
< 10 yo:
Observation w close surveillance
-Carbo/vincristine at radiographic or sx progression
-RT if progresses post-chemo
>10 yo:
RT to 54/1.8 Gy (confirm not 50.4)
craniopharyngioma presentation and workup
Arise from remnants of Rathke’s pouch
H&P – present w DI, growth failure, behavior issues, visual field defects
Labs – pituitary panel
Imaging – MRI brain – solid calcified nodule with cyst filled w crankcase oil
Biopsy – not necessary
Other:
Audiometry
Ophtho exam
Cranio treatment
Max safe resection
If GTR -> observe (20% recurrence)
if STR (70% recurrence) -> adjuvant RT to 54/1.8 Gy
Decompress cyst prior to RT
MRI q 1-2 wks to assess cyst growth requiring replanning. Daily CBCT can guide too.
EBRT
GTV (just post-op GTV) = tumor + cyst wall
(can use 0.5 cm CTV)
PTV = GTV + 0.5 cm
Dose: 54 Gy
DIPG
DIPG
Steroids and RT to 54 Gy
GTV=FLAIR + 1.5 cm
CTV=entire brainstem (midbrain to C1)
PTV=CTV+0.5 cm
LGG
GTR – obs
NF-1 indolent
<10 yo vincristine carbo to delay RT
RT-54Gy
HGG
High Grade Glioma: MGMT bad, H3.3 K27M bad, IDH better
Surgery, RT 54-60Gy
Rhabdo Favorable sites
• Favorable sites: BONG
Biliary
Orbit
Non parameningeal HN
GU (non bladder/prostate)
Rhabdo Unfavorable sites
Unfavorable sites:
• Parameningeal (MMNNOOPP):
Middle ear,
Mastoid region,
Nasal Cavity,
Nasopharynx,
Pterygopalatine fOssa,
Infratemporal fOssa,
Paranasal Sinus,
Parapharyngeal region
• Bladder/prostate
• Extremities
• Other: Trunk/retroperitoneum
RMS Staging
Simple RMS Staging
Stage=Site
1 Favorable (any size/N)
2 Unfavorable, small and N0
3 Unfavorable, large or N+ (or both)
4 Distant Mets
RMS Grouping
Simple RMS Grouping
Group = Surgical Group
Based on surgical status prior to chemo start.
I GTR, margins –
II GTR, + microscopic disease
III Gross residual
IV Mets
Rhabdo Risk group
Low – (all others): based on histology
FH, fav site, group 1-3 (no met)
FH, unfav site, group 1-2
Int –
unfav site & group 3(gross disease) or UH
High – mets
RMS overall treatment paradigm
Surgery* -> chemo -> RT -> chemo
RMS low risk paradigm
Low-risk: Surgery then
- VAC (vincristine/actinomycin D/cyclophos) wk 1-12
- RT at week 12 w chemo
- VA wk 13-24
RMS Int-risk paradigm
Int-risk (UH)
- VAC wk 1-42
-RT at week 12
** no actinomycin during RT **
RMS high risk
High-risk
- VAC + IE (ifos/etop) wk 1-20
- RT at week 20 to primary and mets
- VAC + IE wk 20-52 Gy
If compression etc can start with chemo
RT Doses by Group and Histology RMS
RT Doses by Group and Histology
Group. FH. UH.
1(neg marg) 0 36
+margin 36 36
node+ 41.4 41.4
3(gross) 50.4* 50.4*
(*45 Gy if orbit; **36 Gy if delayed primary excision)
FH: Embryonal
UH: Alveolar
Basically:
UH or + margin: 36 Gy
Node + 41.4
Gross: 50.4
RMS volumes
Pre-chemo, pre-surgical GTV based on the gross disease on MR.
CTV = GTV + 1 cm PTV = CTV + 5 mm
Lung mets:
15/1.5 Gy
Boost if gross disease to 50.4 Gy
RMS constraints
Optic nerve & chiasm < 46.8 Gy (per RMS protocol)
Contralat whole kidney < 14.4
Whole Liver < 23.4
Ewing’s Workup
H&P: w neuro and MSK exam
Labs: CBC, CMP, LDH, ESR
Imaging:
- MRI primary (T1 enhancing)
- PET with diagnostic CT
- CT chest
- Bone scan
Biopsy:
- Bx primary site – longitudinal incisional bx
- BM biopsy (25% have BM+!!!)
Ewing’s treatment
Chemo -> local therapy (surgery vs RT) -> chemo
1) VDC-IE q 2 wks x 6 cycles (wk 1-12)
2) local therapy at wk 14:
- surgery preferred for “expendable” bones
- otherwise RT + chemo (minus adriamycin)
45 Gy to pre-chemo volume (bone and soft tissue) + 2 cm
55.8 Gy boost to post-chemo soft tissue + pre-chemo bone + 2 cm
No elective nodes
-if vertebral body, do 45 then 5.4 to 50.4 total
Ewing dont forget
CLAM SHELL IF MALE!!!!!!!
OVARIAN transposition for women if RT to pelvis.
Osteosarcoma
Chemo -> surgery -> chemo
-Chemo: MAP (MTX, adria, cisplatin)
RT if:
- inoperable (70 Gy)
- R1/R2 resection (60 Gy)
Neuroblastoma workup
Labs: blood catecholamines Urine catecholamines (inc in 90%): VMA, HMV
- Imaging:
CT c/a/p - calcs
MIBG scan
Bone scan (if MIBG neg)
-Biopsy
Bilateral BM biopsy
Biopsy primary and involved nodes: do NOT biopsy primary until wilms ruled out
Homer-Wright pseudorosettes
Neuroblastoma risk cat
Low: Stage I:
- -unilateral GTR or R1
- -Stage II: STR, R2, ipsi LN
Int: all others
High Risk:
- -N-Myc
- -Mets >18mo
- -Stage III UH >18mo: cross midline, contralateral LN, unresectable
Neuroblastoma presentation
Young 2 and under, sick
pushes kidney out of the way,
urine catecholamines +, skeletal mets are common(MIBG scan)
MS is mets in <18months
calcifications in mass
Neural crest cells of the sympathetic ganglion
Neuroblastoma treatment
Low – surgery alone
-chemo for persistence or recurrence
Int
Max safe resection -> chemo
High
Make sure you have biopsy!
1) Induction CAPE x 5 months (cisplatin-based regimen)
2) Repeat MIBG
3) max safe resection
4) tandem transplant (ABMT x 2)
5) RT day 28 to primary and persistent MIBG+ sites
5) maintenance cis-retinoic acid x 6 months and immune tx x 5 months
Neuroblastoma dose
Primary site & mets: 21.6/1.8 Gy
Post-induction, pre-op volume with 2 cm margin (1.5 cm CTV, 0.5 cm PTV)
Cover entire vertebral body to prevent scoliosis.
4S liver palliation causing respiratory distress (Pepper syndrome) give 1.5 Gy x 3 fx
Symptomatic cord compression:
Try chemo first! RT if persistent
<3yo: 9/1.8 Gy
>3yo: 21.6/1.8
Neuroblastoma constraints
Liver V9 < 50% ; V18< 25%
Ipsilateral kidney < 14.4
Lung V15 < 30%
Wilms histology
FH: (90%) – no anaplasia or sarcomatous features
UH: (10%) anaplastic (focal or diffuse), clear cell, rhabdoid
Wilms workup
Labs – CBC, Chem7, LFTs, UA w catecholamines (to r/o NB)
Imaging -
Abd U/S
CT/MRI of primary
CT chest
For clear cell: add bone scan, MRI brain, BM bx
For rhabdoid: add MRI brain
DO NOT BIOPSY (unless bilateral or unresectable)
Wilms stage
I: completely resected, confined to kidney
II: completely resected, but beyond capsule, or vessel
III:
SLURPP-Bx
Spillage
LN+
Unresectable
Residual tumor
Positive margins
Peritoneal implants
Biopsy performed
IV: mets or LN+ outside abd/pelvis
V: bilateral (stage each side separately)
*pre-op chemo=Stage III*
Wilms overall treatment
Surgery - > Chemo - > +/- RT
Flank field
Flank Field: pre-op tumor +1.5cm anatomically modified, include vertebral bodies
Pediatric Hodgkins treatment
Low risk: AVPC x 3 c -> ISRT 21/1.5 Gy (AHOD 0431)
-adria, vincristine, pred, cyclo
Int risk: AVPC-BE x 4-6 c -> ISRT to 21/1.5 Gy
-adria, bleo, vincristine, etop, pred, cyclophos
High risk: ABPC-BE x 6-8 c -> ISRT 21/1.5
Ann Arbor staging
Low risk:
Favorable stage I-II (no B sx, non-bulky, no extranodal extension, 1-2 sites)
Int risk:
Unfav stage I-II
Stage IIIA (no B sx)
High risk:
Stage IIIB or IV
