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CNS/PEDS Flashcards

1
Q

standard risk medulloblastoma: adjuvant chemo

A

PCV (cisplatin, CCNU, vincristine) starting 6 weeks after RT

8 cycles, q6wks

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2
Q

grade 1-2 meningioma: fields and dose

A

GTV + 0.5-1cm CTV to 54Gy (50.4Gy if optic)

SRS 14-16Gy

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3
Q

Wilms: criteria for stage III (radiation)

A

Stage III, BSSLURPP:

  • Biopsy
  • Spillage
  • Subtotal resection
  • Lymph nodes
  • Unresectable
  • Rupture
  • Peritoneal implants
  • Piecemeal resection
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4
Q

Ewings: 5yr OS

A

60% if localized

30% with lung/pleural mets

15% if bone/BM mets

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5
Q

Rhabdomyosarcoma: constraints

A

bladder <45 Gy

heart <30 Gy

liver <23.4 Gy

rectum <45 Gy

chiasm <54 Gy

small bowel <45 Gy

max cord point <45 Gy

50% of kidneys <24 Gy

lung V20<20 Gy

lacrimal gland/cornea <41.4 Gy

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6
Q

CAPETV: components

A

cyclophosphamide, doxorubicin, cisplatin, etoposide, topotecan, vincristine

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7
Q

standard risk medulloblastoma: treatment paradigm

A

maximal safe resection then XRT with concurrent vincristine then adjuvant PCV

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8
Q

Wilms: doses for metastases to liver, brain, and bone

A

Liver: 19.8Gy to whole liver

Brain: 21.6Gy WBRT + 10.8Gy boost

Bone: 25.2Gy + 1cm margin

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9
Q

Wilms: abdominal fields

A

sup: 1cm above diaphragm
inf: obturator foramen
lat: 1cm beyond abdominal wall

block femoral heads

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10
Q

Ewings: workup

A

H&P.

Labs: ESR, Alk phos

Imaging: Xray (onion skinning), MRI of primary

Biopsy (t11;22)

Staging: CT chest, Bone scan/PET, BM bx. For soft tissue ewings, eval nodes as per rhabdo

Surgery is preferred

After surgery, need a “cuff” of normal tissue: for bone, need margin of 2-5 cm. For natural barriers of fascia, periosteum, or intramuscular septa, need 2 mm. For fat, muscle, or medullary bone, 5 mm

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11
Q

Brain Ependymoma: field and dose

A

59.4Gy/33fxs

preop GTV + 1cm CTV + 0.3-0.5cm PTV

limit cord dose to 45Gy

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12
Q

Pediatrics: general follow up

A

Growth charts, Tanner staging

Screening for 2nd malignancy (thyroid, breast, etc. May be increased with IMRT)

Psychosocial (school liasons, community disability, psychology)

CNS

Endocrine: Deficiencies in GH, FSH/LH, ACTH, TSH. Hyperprolactinemia.

Obesity, central precocious puberty, low bone density

Reduced IQ

Hearing loss (leads to speech delay and reduced QOL and social function)

Risk of stroke, vascular malformations

See COG survivorship guidelines and long term follow-up guidelines

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13
Q

Neuroblastoma: INRG criteria for standard risk disease

A

stage MS<18 mos with no n-myc or 11q

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14
Q

Neuroblastoma: 3yr OS for high risk

A

3yr OS 60%

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15
Q

Rhabdomyosarcoma: criteria for intermediate risk

A

Embryonal, spindle, botyroid:

Group III, unfavorable (Stage 2-3, not stage 1)

Alveolar histology, no mets (Stage 1-3, Group I-III)

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16
Q

Craniopharyngioma: treatment paradigm

A

max safe resection. Consider EBRT or intracystic chemo if subtotal resection or at recurrence

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17
Q

Rhabdomyosarcoma: 5yr OS for low, intermediat, high risk

A

low risk: 95%

intermediate risk: 65%

high risk: 45%

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18
Q

Intracranial germ cell tumor: workup

A

H&P (esp CNs, funduscopic exam)

MRI brain/spine

CBC, CMP, serum AFP/β-HCG, CSF AFP/β-HCG/cytology

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19
Q

Intracranial germinoma (disseminated): fields and dose

A

2-4 cycles of carbo/etoposide then 21 Gy CSI with boost to 30 Gy (if CR to chemo)

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20
Q

AT/RT: treatment paradigm

A

induction chemo with vincristine, cisplatin, cyclophosphamide, etoposide, methotrexate

36Gy CSI then boost primary to 54Gy

consolidation with thio/carbo/ASCT

2yr OS 50%

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21
Q

VDC/IE: components

A

vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide

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22
Q

Wilms: treatment of lung mets

A

If no CR by week 6, try to biopsy or surgically remove nodule first, then do whole lung RT, especially if mass >1 cm AND/OR visible on CXR. If PR also add cis etoposide to chemo, otherwise continue VAD

12 Gy/8 fx in 1.5 Gy daily

(or 10.5 Gy at 1.5 Gy per fx for <1 yo)

Delayed WLI is at week 6 after chemo. Otherwise WLI is done with flank/abdomen RT by day 10-14 after surgery.

Treat flank/abdomen per standard. This portion always done after surgery.

If nodules still present after lung RT then these can be resected

May also resect lung nodules at week 6 if

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23
Q

Rhabdomyosarcoma: workup

A

H&P

Labs: CBC, LFTs, LDH

Imaging: CT/MRI of primary, CT C/A/P

Staging: bone scan, bone marrow biopsy. PET now used in protocols

Special workup:

Parameningeal: LP. MRI of spine/brain if LP is positive.

GU: cystoscopy. Consider EUA.

Extremity: SLN eval

Paratesticular: RPLND if age >10 or only if LN+ for age<10

Stage is based on pre-op studies

Group is based on surgical findings and guides RT dose

Risk stratification guides chemo

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24
Q

Ewings: xrt dose for involved lymph nodes

A

Resected: PTV1 to 50.4 Gy to that LN level

Unresected: PTV1 to 45 Gy then boost PTV2 10.8 Gy

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25
Wilms: treatment paradigm for stage V or solitary kidney
AREN0534: pre-op VAD--\>eval at week 6 and possible resect or biopsy, or continue chemo if CR--\>eval again at week 12--\> surgery or more chemo if CR
26
criteria for standard risk medulloblastoma
\>3yo \<1.5cm2 residual M0
27
Wilms: lung constraints
whole lung\<12 Gy lung if PTV occupies \>1/2 total lung volume, \<15 Gy Lung if PTV occupies \<1/2 total lung, \<18 Gy
28
Pituitary tumor: fields and dose
IMRT: tumor + 5mm CTV + 3-5mm PTV 54Gy for TSH 50.4Gy for all others SRS: treat GTV 16Gy for non-secreting 20Gy for secreting
29
Rhabdomyosarcoma: chemo regimens
Low risk subset 2: VAC or VAC/VI Low risk subset 1: VAC with low dose C for 22 weeks Int and high risk:VAC/VI VC given concurrent with XRT (actinomycin only right before XRT due to risk of radiation recall) (Tip: A in VAC is actinomycin in rhabdo, but adria in ewings and in VAD in Wilms)
30
Neuroblastoma: xrt doses
Primary: 21.6Gy to presurgery volume + 1.5cm CTV. If gross disease \>1cm then boost to 36Gy. Metastatic sites: If MIBG positive prior to transplant, give 21.6 Gy with no boost
31
Neuroblastoma: INRG criteria for high risk disease
any of the following: ## Footnote * N-myc amplification * Stage M \>18 mos * Stage MS \<18 mos with 11q abbertation
32
Wilms: workup
H&P, check for abdominal mass, poor appetite, nausea, hematuria. Imaging: abdominal US, CT/MRI, CXR or CT chest Surgery: radical nephrectomy (with lymph node sampling of renal hilar, para-aortic, and/or paracaval nodes. Palpate renal vein and ICV for extension) Histology and surgery determine chemo and RT. LOH determines only type of chemo Pulmonary lesions may be resected for diagnosis of metastatic disease, incomplete response to chemo, or recurrence
33
Common peds constraints: 50% heart, whole kidney, 50% kidney, whole liver, 50% liver, whole lung
50% heart \< 30.6 Gy whole kidney \< 14.4 Gy 50% kidney \< 19.8 Gy whole liver \< 23.4 Gy 50% liver \< 30.6 Gy whole lung age\<6 \< 12 Gy whole lung age\>6 \< 15 Gy
34
Wilms: indications for chemotherapy
indicated for all standard risk: Stage I-II FH with LOH Stage III FH without LOH Stages I-III with focal anaplasia Stage I with diffuse anaplasia Stage IV FH with CR of lung mets
35
GBM: temozolomide toxicity
nausea, constipation, low platelets, PCP (prophylaxis with bactrim)
36
Intracranial germinoma (disseminated): fields and dose
24Gy CSI then boost gross disease to 45Gy (all using 1.5Gy fractions)
37
VAD: components
vincristine, dactinomycin, doxorubicin
38
Ewings: xrt dose for vertebral body
50.4 Gy
39
grade 3 meningioma: fields and dose
GTV + 2cm CTV to 54 Gy then boost GTV + 1cm CTV to 60 Gy
40
Brain Ependymoma: indications for CSI
Do CSI if CSF+ or MRI+ 36Gy CSI the boost gross cord disease to 45Gy
41
Grade 3 Anaplastic gliomas with 1p19q codel: treatment
59.4Gy/33fxs to GTV + 2cm CTV margin concurrent/adjvuant temozolomide (adjuvant PCV is an option)
42
Ewings: sim
Wire scars. Use vac loc to stabilize extremity. Aquaplast fixation device is ideal (ORFIT). Clamshell for lower extremity site For girls keep ovaries \<8 Gy Consider ovarian transposition
43
Ewings: xrt doses
Definitive RT: ## Footnote * periacetabular lesions, vertebra, distal tibia, distal humerus, upper scapula, brain. * 45 Gy then boost to 55.8 Gy * Start at week 12 (cycle 5) Post-op: * 45 Gy for positive margins or \>10% viable cells (even if total resection) * 55.8 Gy for gross disease
44
Primary CNS lymphoma: fields and dose
in setting of chemo: * CR: 23.4Gy WBRT * PR: 30.6Gy WBRT, boost residual disese to 45Gy if no chemo: * 30.6Gy WBRT, boost groost disease to 45Gy
45
Rhabdomyosarcoma: intermediate risk chemo regimen
VAC/VI x 42 weeks radiation at week 13
46
Ewings: incidence of metastases
40% lung mets 20% bone mets 10% BM involvement
47
Rhabdomyosarcoma: high risk chemo regimen
VAC/VI x 48 weeks radiation at week 20
48
CSI: sim narrative
I would simulate the patient in the prone position. The superior border of the spine field would be located between C2-C5 and would be chosen to avoid divergence through the oral cavity. This would extend inferiorly to S2/S3 as seen on MRI with lateral borders 1-1.5 cm from the vertebral body. To match the cranial fields to the spine fields I would angle the collimator of the cranial fields and kick the couch toward the beam. (If the patient requires two spine fields) I would match at the posterior vertebral body, below L1, and add appropriate skin gap. At the junction of the cranial and spine fields I would match anterior to the cord (to create a cold match). I would feather the fields 1 cm every 9 Gy.
49
Neuroblastoma: xrt dose for cord compression
9Gy if \<3yo 21.6Gy if \>3yo
50
Ewings: xrt volumes
PTV1: 45 Gy to pre-chemo volume (i.e. volume at diagnosis) +1-2 cm margin (may reduce from pushing borders) PTV2: Boost to 55.8 Gy to pre-chemo bony GTV and post-chemo soft tissue GTV + 1 cm margin
51
Pituitary tumor: treatment paradigm
transphenoidal surgery then medical management then radiation stop medical management during radiation for prolactinoma, medical management comes first
52
Wilms: flank fields
treat sup/inf/lateral extent of preop tumor volume plus kidney with block edge at 1 cm, cover vertebral bodies in field completely (1 cm past edge. Can be tighter than 1 cm if close to opposite kidney). PA nodes: include if any LN+ (makes a large block from T11-L5. No strange shaped fields; no MLCs). Treat AP/PA. If needing to spare contralateral kidney, then use 3DCRT Boost gross residual disease. Contour then add 0.5 cm CTV and 0.5-1cm PTV. Use 3DCRT.
53
Rhabdomyosarcoma: whole lung radiation dose
15 Gy/ 10 fx 12 Gy/ 8 fx for \<7 yo Boost tumor to 50.4 Gy total
54
medulloblastoma: outcomes
standard risk 5yr EFS 80% high risk 5yr EFS 60%
55
Primary CNS lymphoma: workup
biopsy before steroids slit lamp, MRI brain/spine, PET/scan CBC, CMP, check HIV, lumbar puncture CR to high dose MTX: * high dose chemo with stem cell rescue * high dose cytarabine/etoposide * high dose MTX for one year * low dose WBRT PR to high dose MTX: * WBRT * high dose cytarabine/etoposide
56
Rhabdomyosarcoma: xrt doses
50.4 for gross residual 45 Gy for orbit 41.4 Gy to +nodes (resected) 36 Gy for microscopic margins
57
Intracranial NSGCT: treatment paradigm
6 cycles alternating carbo/etop and ifos/etop q 3 wk 36Gy CSI then boost pre-chemo disease to 54Gy
58
GBM: Fields and dose (RTOG)
CTV 46Gy: T2 + 2cm CTV 60Gy: T1 post / cavity + 2cm 3-5mm for PTV
59
Intracranial germinoma (localized): fields and dose
24Gy WVI 45Gy to gross disease (1.5Gy fractions) WVI: lateral/third/fourth ventricles, suprasellar/pineal cisternas, + NO extra CTV + 0.3cm PTV (consider pre-pontine cistern) Boost: pre-chemo GTV + 0.5cm CTV + 0.3cm PTV
60
Adult CNS Workup
H&P with neurologic assessment Consider dex (non PCNSL) and Keppra CBC, CMP, pituitary panel CT, MRI brain, stereotactic guided biopsy Baseline neurocognitive function testing, visual field testing, audiometry
61
Grade 3 Anaplastic gliomas without codel: treatment
59.4Gy/33fxs to GTV + 2cm CTV margin concurrent/adjuvant temozolomide adjuvant PCV also an option
62
Neuroblastoma: xrt fields
Primary: presurgery volume, then boost post surg/chemo volume if \>1cm residual disease. Can modify GTV based on collapsed tissue after surgery. Treat site to 21.6 Gy even if complete resection. CTV 1.5 cm. Boost: If gross disease \>1 cm is present after surgery, boost this to 36 Gy total. CTV 1 cm, PTV 0.5-1.0 cm Metastatic: 21.6 Gy to mets MIBG+ after chemo, no boost. 2 cm margin. Treat postchemo presurgical volume (i.e. MIBG volume. No RT for sites and metastatic sites that disappear with chemo. Do not treat if MIBG negative)
63
Spinal Ependymoma: fields and dose
45Gy/25 if along cord 50. 4Gy/28 if below cord 1. 5 cm margin superiorly and inferiorly, can include nerve roots radially usually occurs at conus and filum terminale
64
Intracranial germinoma (localized): treatment paradigm for induction chemo
2-4 cycles of carbo/etoposide then 21 Gy WVRT with boost to 30 Gy (if CR to chemo)
65
Craniopharyngioma: fields and dose
54Gy/30fxs post-op GTV + 1cm CTV to account for possible cyst expansion, weekly MRI SRS 12Gy can be done if small size and 1mm away from optics
66
Rhabdomyosarcoma: criteria for high risk
Stage 4, Group IV \>10yo embryonal All alveolar Stage 4 Group IV
67
pilocytic astrocytoma: treatment paradigm
Surgery then observation Carbo/vincristine at recurrence if age \< 10yrs 50.4Gy at recurrence if age \> 10yrs or failure after chemo
68
standard risk medulloblastoma: fields and dose
23.4Gy CSI then 54Gy IFRT boost to tumor bed + 1cm CTV if no concurrent vincristine then do 36Gy CSI
69
high risk medulloblastoma / supratentorial PNET: fields and dose
36Gy CSI then boost posterior fossa to 54Gy boost gross spine disease to 45Gy if above terminus of spinal cord or 50.4Gy if below terminus of spinal cord
70
Ewings: treatment paradigm
VDC/IE x48 weeks local therapy at week 12
71
Neuroblastoma: treatment paradigm for high risk disease
Initial resection/biopsy Induction CAPETV, then re-image with MIBG or bone scan Maximal surgical resection at cycle 4. (If later fibrosis may occur) MIBG or Bone scan after surgery Autologous SCT with BuMel RT to primary and metastatic sites (40 days after transplant) Isotretinoin for 6 mos Dinituximab (aka ch14.18)
72
Wilms: radiation doses
**Flank:** stage III: 10.8Gy gross residual: 10.8Gy + boost to 21.6Gy stage I-II anaplasia: 10.8Gy stage III diffuse anaplasia: 19.8Gy **Whole abdomen:** rupture/spillage: 10.5Gy gross disease: 10.5Gy + boost to 21Gy diffuse disease: 21Gy **Lymph nodes:** resected LNs: 10.8Gy unresected LNs: 19.8Gy 10.5 Gy /1.5 Gy daily to whole abdomen for pre op tumor rupture, peritoneal mets, "large" tumor spill, +10.5 Gy boost if gross residual 21 Gy if diffuse unresectable implants
73
GBM: RT options for elderly or poor KPS
age \> 70 or KPS \< 60 (per NCCN) 40.05Gy/15fxs (Roa) 34Gy/10fxs Test for MGMT to help guide therapy. If MGMT is not methylated, there is less benefit with TMZ
74
Wilms: whole lung fields
whole lung borders: top of clavicles to bottom of L1 (lung with 1 cm margins and simple blocking) If waiting until after chemo to possibly avoid, then WLI is matched to prior flank field if treating WL. Per AREN0533 overlap is acceptable. (Overlap only becomes problem if treating whole abdomen and overlapping contralateral kidney)
75
GBM: temozolomide dosing during/after RT
during RT: 75mg/m2 daily after RT: 150-200mg/m2 days 1-5 on q28day cycle for 6 months
76
Wilms: treatment paradigm
nephrectomy adjuvant radiation at post-op day 14 VAD chemotherapy x25 weeks
77
Wilms: treatment paradigm for anaplasia
nephrectomy RT for all Stage I-II anaplasia (focal or diffuse): 10.8 Gy Stage III focal anaplasia: 10.8 Gy Stage III diffuse anaplasia: 19.8 Gy VDCBE x 30 weeks
78
diffuse intrinsic pontine glioma: fields and dose
54Gy to GTV + 0.5cm CTV MS 10 months
79
Rhabdomyosarcoma: xrt volumes
prechemo/presurg volume (i.e. volume at diagnosis) GTV with 1.0 cm CTV, 0.5 cm PTV Can reduce volume from pushing borders after 36 Gy to 0.5 CTV +0.5 PTV
80
Rhabdomyosarcoma: treatment paradigm
Surgery --\> chemo/RT Time since chemo to begin RT: Low risk: week 13 Int risk: week 13 High risk (metastatic): week 20 or RT at end of chemo for extensive met sites base of skull or CNS: week 15 vaginal site: week 13
81
Grade 2 glioma: treatment paradigm
maximum safe resection Low risk (age\<40, GTR): observe High risk: 54Gy/30fxs to GTV (FLAIR/T2) + 1.5cm Adjuvant PCV x6 cycles (adjuvant +/- concurrent temozolomide is also an option per NCCN)
82
Neuroblastoma: workup
H&P, check for distended abdomen, fatigue, ataxia, opsoclonus myoclonus, diarrhea, hypertension. Labs: urine catecholamines (VMA, HVA), CBC, CMPs Imaging: CT or MRI of primary (arises from adrenal, calcifications, can cross midline), CXR Biopsy Functional: EKG, MUGA/ECHO, audiogram Staging: BM bx, I-123 MIBG scan, or bone scan if MIBG negative. PET optional Determine risk group using INRG staging. Only high risk is relevant for RT.
83
General CNS simulation
supine, arms at sides thermoplastic mask fuse preop and postop MRI

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