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
Q

Wilms: treatment paradigm for stage V or solitary kidney

A

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

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26
Q

criteria for standard risk medulloblastoma

A

>3yo

<1.5cm2 residual

M0

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27
Q

Wilms: lung constraints

A

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

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28
Q

Pituitary tumor: fields and dose

A

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

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29
Q

Rhabdomyosarcoma: chemo regimens

A

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)

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30
Q

Neuroblastoma: xrt doses

A

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

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31
Q

Neuroblastoma: INRG criteria for high risk disease

A

any of the following:

  • N-myc amplification
  • Stage M >18 mos
  • Stage MS <18 mos with 11q abbertation
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32
Q

Wilms: workup

A

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

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33
Q

Common peds constraints: 50% heart, whole kidney, 50% kidney, whole liver, 50% liver, whole lung

A

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

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34
Q

Wilms: indications for chemotherapy

A

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
Q

GBM: temozolomide toxicity

A

nausea, constipation, low platelets, PCP (prophylaxis with bactrim)

36
Q

Intracranial germinoma (disseminated): fields and dose

A

24Gy CSI then boost gross disease to 45Gy (all using 1.5Gy fractions)

37
Q

VAD: components

A

vincristine, dactinomycin, doxorubicin

38
Q

Ewings: xrt dose for vertebral body

A

50.4 Gy

39
Q

grade 3 meningioma: fields and dose

A

GTV + 2cm CTV to 54 Gy then boost GTV + 1cm CTV to 60 Gy

40
Q

Brain Ependymoma: indications for CSI

A

Do CSI if CSF+ or MRI+

36Gy CSI the boost gross cord disease to 45Gy

41
Q

Grade 3 Anaplastic gliomas with 1p19q codel: treatment

A

59.4Gy/33fxs to GTV + 2cm CTV margin

concurrent/adjvuant temozolomide

(adjuvant PCV is an option)

42
Q

Ewings: sim

A

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
Q

Ewings: xrt doses

A

Definitive RT:

  • 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
Q

Primary CNS lymphoma: fields and dose

A

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
Q

Rhabdomyosarcoma: intermediate risk chemo regimen

A

VAC/VI x 42 weeks

radiation at week 13

46
Q

Ewings: incidence of metastases

A

40% lung mets

20% bone mets

10% BM involvement

47
Q

Rhabdomyosarcoma: high risk chemo regimen

A

VAC/VI x 48 weeks

radiation at week 20

48
Q

CSI: sim narrative

A

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
Q

Neuroblastoma: xrt dose for cord compression

A

9Gy if <3yo

21.6Gy if >3yo

50
Q

Ewings: xrt volumes

A

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
Q

Pituitary tumor: treatment paradigm

A

transphenoidal surgery then medical management then radiation

stop medical management during radiation

for prolactinoma, medical management comes first

52
Q

Wilms: flank fields

A

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
Q

Rhabdomyosarcoma: whole lung radiation dose

A

15 Gy/ 10 fx

12 Gy/ 8 fx for <7 yo

Boost tumor to 50.4 Gy total

54
Q

medulloblastoma: outcomes

A

standard risk 5yr EFS 80%

high risk 5yr EFS 60%

55
Q

Primary CNS lymphoma: workup

A

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
Q

Rhabdomyosarcoma: xrt doses

A

50.4 for gross residual

45 Gy for orbit

41.4 Gy to +nodes (resected)

36 Gy for microscopic margins

57
Q

Intracranial NSGCT: treatment paradigm

A

6 cycles alternating carbo/etop and ifos/etop q 3 wk

36Gy CSI then boost pre-chemo disease to 54Gy

58
Q

GBM: Fields and dose (RTOG)

A

CTV 46Gy: T2 + 2cm

CTV 60Gy: T1 post / cavity + 2cm

3-5mm for PTV

59
Q

Intracranial germinoma (localized): fields and dose

A

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
Q

Adult CNS Workup

A

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
Q

Grade 3 Anaplastic gliomas without codel: treatment

A

59.4Gy/33fxs to GTV + 2cm CTV margin

concurrent/adjuvant temozolomide

adjuvant PCV also an option

62
Q

Neuroblastoma: xrt fields

A

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
Q

Spinal Ependymoma: fields and dose

A

45Gy/25 if along cord

  1. 4Gy/28 if below cord
  2. 5 cm margin superiorly and inferiorly, can include nerve roots radially

usually occurs at conus and filum terminale

64
Q

Intracranial germinoma (localized): treatment paradigm for induction chemo

A

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

65
Q

Craniopharyngioma: fields and dose

A

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
Q

Rhabdomyosarcoma: criteria for high risk

A

Stage 4, Group IV >10yo embryonal

All alveolar Stage 4 Group IV

67
Q

pilocytic astrocytoma: treatment paradigm

A

Surgery then observation

Carbo/vincristine at recurrence if age < 10yrs

50.4Gy at recurrence if age > 10yrs or failure after chemo

68
Q

standard risk medulloblastoma: fields and dose

A

23.4Gy CSI then 54Gy IFRT boost to tumor bed + 1cm CTV

if no concurrent vincristine then do 36Gy CSI

69
Q

high risk medulloblastoma / supratentorial PNET: fields and dose

A

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
Q

Ewings: treatment paradigm

A

VDC/IE x48 weeks

local therapy at week 12

71
Q

Neuroblastoma: treatment paradigm for high risk disease

A

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
Q

Wilms: radiation doses

A

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
Q

GBM: RT options for elderly or poor KPS

A

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
Q

Wilms: whole lung fields

A

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
Q

GBM: temozolomide dosing during/after RT

A

during RT: 75mg/m2 daily

after RT: 150-200mg/m2 days 1-5 on q28day cycle for 6 months

76
Q

Wilms: treatment paradigm

A

nephrectomy

adjuvant radiation at post-op day 14

VAD chemotherapy x25 weeks

77
Q

Wilms: treatment paradigm for anaplasia

A

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
Q

diffuse intrinsic pontine glioma: fields and dose

A

54Gy to GTV + 0.5cm CTV

MS 10 months

79
Q

Rhabdomyosarcoma: xrt volumes

A

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
Q

Rhabdomyosarcoma: treatment paradigm

A

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
Q

Grade 2 glioma: treatment paradigm

A

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
Q

Neuroblastoma: workup

A

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
Q

General CNS simulation

A

supine, arms at sides

thermoplastic mask

fuse preop and postop MRI