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CNS/PEDS Flashcards

1
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

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2
Q

GBM: Fields and dose (RTOG)

A

CTV 46Gy: T2 + 2cm

CTV 60Gy: T1 post / cavity + 2cm

3-5mm for PTV

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3
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

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4
Q

GBM: max dose constraints for chiasm, brainstem, optic nerves, retina, and lenses

A

chiasm 55Gy

brainstem 60Gy

optic nerves 55Gy

retina 50Gy

lenses 7Gy

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5
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

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6
Q

RANO criteria for pseudoprogression:

A

To confirm progression within 3 months, there must be progression outside of the 80% isodose line. Repeat another scan sometime after 12 weeks. If increase in lesion size >25% of sum of perpendicular diameters, the progression has occured

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7
Q

GBM: XRT toxicity

A

Pseudoprogression: 25%

Necrosis: 10%

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8
Q

GBM: Outcome

A

MS 17mo

24mo if MGMT methylated

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9
Q

GBM: temozolomide toxicity

A

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

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10
Q

General CNS simulation

A

supine, arms at sides

thermoplastic mask

fuse preop and postop MRI

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11
Q

Grade 3 Anaplastic gliomas with 1p19q codel: treatment

A

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

neoadjuvant PCV x4 cycles (procarbzine, CCNU/lomustine, vincristine)

also reasonable to do concurrent/adjvuant temozolomide

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12
Q

Grade 3 Anaplastic gliomas without codel: treatment

A

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

adjuvant temolozomide (no benefit yet with concurrent on CATNON)

also reasonable to do adjuvant PCV

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13
Q

Grade 2 glioma: treatment

A

54Gy/33fxs to GTV (FLAIR/T2) + 1.5cm

Adjuvant PCV x6 cycles

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14
Q

SATAN criteria

A

Size >6cm

Age >40

Tumor crossing midline

Astrocytoma

Neuro deficits

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15
Q

Ependymoma: indications for treatment

A

Treat if anaplastic or subtotal resection

Consider treatment if GTR and grade 1-2 myxopapillary

Okay to observe if GTR and grade 1-2 non-myxopapillary

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16
Q

Spinal Ependymoma: fields and dose

A
  1. 4Gy/28fxs
  2. 5 cm margin superiorly and inferiorly, can include nerve roots radially

usually occurs at conus and filum terminale

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17
Q

Spinal Ependymoma: outcomes

A

10yr LC:

GTR + RT 90%

STR + RT: 70%

GTR 50%

STR 0%

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18
Q

Brain Ependymoma: field and dose

A

54Gy then off-cord boost to 59.4Gy

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

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19
Q

Brain Ependymoma: indications for CSI

A

Do CSI if CSF+ or MRI+

36Gy CSI the boost gross cord disease to 45Gy

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20
Q

Brain Ependymoma: Outcomes

A

10yr LC:

GTR + RT 100%

GTR 50%

STR + RT 30%

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21
Q

Pituitary tumor: treatment paradigm

A

transphenoidal surgery then medical management then radiation

stop medical management during radiation

for prolactinoma, medical management comes first

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22
Q

Pituitary tumor: medications for prolactinoma, ACTH, GH

A

prolactinoma: cabergoline or bromocriptine

ACTH: ketoconazole, mitotane

GH: octreotide, lanreotide, pegvisomat (IGF-1 blocker $$$$)

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23
Q

Pituitary tumor: indications for radiation

A

unresectable tumor

failure after surgery and/or medical management

TSH-secreting tumor (all TSH get post-op RT to 54Gy)

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24
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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25
Q

Primary CNS lymphoma: treatment paradigm

A

(steroids after biopsy)

most do chemo alone and hold RT for persistent or recurrent disease

usually high dose MTX (8mg/m2) if planning on deferred XRT

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26
Q

Primary CNS lymphoma: fields and dose

A

23.4Gy WBRT then boost focal disease to 45Gy

WBRT should include C1-C2 and posterior eyes (or entire eye if involved)

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27
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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28
Q

grade 3 meningioma: fields and dose

A

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

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29
Q

diffuse intrinsic pontine glioma: fields and dose

A

54Gy to GTV + 0.5cm CTV

MS 10 months

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30
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

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31
Q

criteria for standard risk medulloblastoma

A

>3yo

<1.5cm2 residual

M0

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32
Q

standard risk medulloblastoma: treatment paradigm

A

maximal safe resection then XRT with concurrent vincristine then adjuvant PCV

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33
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

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34
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.

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35
Q

standard risk medulloblastoma: adjuvant chemo

A

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

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36
Q

medulloblastoma: outcomes

A

standard risk 5yr EFS 80%

high risk 5yr EFS 60%

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37
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

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38
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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39
Q

Intracranial germinoma (localized): fields and dose

A

24Gy to whole ventricle volume then boost gross disease to 45Gy (all using 1.5Gy fractions)

Whole ventricle: (lateral, third, fourth, suprasellar and pineal cisternas, plus pre-pontine cistern if 3rd ventriculostomy or large tumor) + NO extra CTV + 0.3-0.5 cm PTV

Boost: pre-chemo GTV + 0.5 cm CTV + 0.3-0.5 cm PTV.

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40
Q

Intracranial germinoma (disseminated): fields and dose

A

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

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41
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)

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42
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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43
Q

Intracranial germinoma and NSGCT: outcomes

A

germinoma 5yr PFS 90%

NSGCT 5yr PFS 60%

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44
Q

Intracranial Germinoma and NSGCT: incidence and tumor markers

A

66% germinoma / 33% NSGCT

15% of germinomas produce low level β-HCG

β-HCG is >100 or AFP >10 excludes germinoma

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45
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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46
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

47
Q

Craniopharyngioma: treatment paradigm

A

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

48
Q

Craniopharyngioma: fields and dose

A

54Gy/33fxs

post-op GTV + 1cm CTV to account for possible cyst expansion (occurs in 15-20%), do weekly imaging with MRI if possible to assess cyst expansion

SRS 12Gy can be done if small size and 1mm away from optics

49
Q

Craniopharyngioma: when to avoid RT

A

No adjuvant RT if gross total resection. Most hold off on RT if subtotal resection and age <5yr.

10yr local control is similar between adjuvant and salvage RT (~80% for both)

50
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

51
Q

VAD: components

A

vincristine, dactinomycin, doxorubicin

52
Q

VDC/IE: components

A

vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide

53
Q

CAPETV: components

A

cyclophosphamide, doxorubicin, cisplatin, etoposide, topotecan, vincristine

54
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

55
Q

Wilms: workup

A

H&P. Peak age 3-4. Palpable abdominal mass, low appetite, nausea, hematuria.

Imaging: abdominal US, CT/MRI, CXR or CT chest

Do not biopsy unless unresectable primary or bilateral Wilms. (Should be able to distininguish that this is not neuroblastoma, which requires biopsy, based on presentation, age, and imaging.)

Staging: CXR or CT chest. (No need for BM biopsy or bone scan)

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

56
Q

Wilms: treatment paradigm

A

nephrectomy

adjuvant radiation at post-op day 14

VAD chemotherapy x25 weeks

57
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

58
Q

Wilms: radiation doses

A

10.8 Gy to primary site

+10.8 Gy boost if gross residual to total 21.6 Gy

  1. 8 Gy to resected LNs
  2. 8 Gy to unresected LNs
  3. 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

  1. 8 Gy to flank for any stage rhabdoid (RTK) tumor or diffuse anaplasia Stage III
  2. 8 Gy for Stage I-II diffuse anaplasia
59
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.

60
Q

Wilms: abdominal fields

A

Whole abdomen: 1 cm above diaphragm down to bottom of obturator foramen. Lateral borders 1 cm beyond abdominal wall. Block femoral heads.

For ureteral extension, treat whole ureter. If IVC was invaded, treat that pre-op volume with 1 cm margin also.

61
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

62
Q

Wilms: criteria for stage III (radiation)

A

Stage III - Residual non-hematogenous tumor present following surgery, and confined to abdomen.

Any one of the following may occur:

*Lymph nodes in abdomen or pelvis (LNs in the thorax or outside abdomen/pelvis is Stage IV)

*Penetrated through peritoneal surface

*Peritoneal implants

*Incomplete resection, gross or microscopic (e.g., tumor cells are found at the margin of surgical resection on microscopic examination)

*Unresectable due to infiltration into vital structures

*Tumor spillage before or during surgery

*Preoperative chemotherapy (with or without a biopsy)

*Tumor removed in greater than one piece (e.g. tumor cells are found in a separately excised adrenal gland; a tumor thrombus within the renal vein is removed separately from the nephrectomy specimen).

*Extension within IVC into thoracic vena cava and heart (Stage III, rather than Stage IV even though outside abdomen)

63
Q

Wilms: 10yr OS by stage with and without anaplasia

A

10yr OS:

stage I 97%, anaplasia 90%

stage II 95%, anaplasia 50%

stage III 90%, anaplasia 50%

stage IV 80%, anaplasia 20%

stage V 80%, anaplasia 10%

64
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

65
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

66
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

67
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)

68
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

69
Q

Neuroblastoma: INRG criteria for high risk disease

A

any with N-myc amplification

All Stage M >18 mos (even if n-myc negative)

Stage MS <18 mos with 11q abbertation or n-myc

70
Q

Neuroblastoma: INRG criteria for standard risk disease

A

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

71
Q

Neuroblastoma: workup

A

H&P. Most within first year of life, and the rest occur mainly up to age 5. Distended abdomen, fatigue, rare ataxia, opsoclonus myoclonus, diarrhea, hypertension. Should be able to distininguish that this is not Wilms (which should not be biopsied) based on presentation, age, and imaging

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 (see left). Only high risk is relevant for RT.

72
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)

73
Q

Neuroblastoma: xrt doses

A

Primary: 21.6 Gy/ 12 fx in 1.8 Gy per fx, with boost to gross residual to total 36 Gy.

Metastatic sites: If MIBG positive prior to transplant, give 21.6 Gy with no boost

74
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)

75
Q

Neuroblastoma: dose constraints

A

Liver V30<15%

Liver mean <15 Gy

ipsilateral kidney V18<75%

ispsilateral kidney mean <18 Gy;contralateral kidney V18<25%

Total lung V20<30%

Lung ipsilateral V20<30%

Lung contralateral V20<10%

76
Q

Neuroblastoma: incidence

A

60% present with mets

25% present with n-myc

60% are abdominal (40% adrenal and 20% paraspinal)

5% sympathetic ganglia in neck

15% posterior mediastinum

5% in pelvis

77
Q

Neuroblastoma: xrt dose for cord compression

A

9Gy if <3yo

21.6Gy if >3yo

78
Q

Neuroblastoma: 3yr OS for high risk

A

3yr OS 60%

79
Q

Rhabdomyosarcoma: workup

A

H&P. Over in age <6, and also in ages up to 18.

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. If cytology is positive, get MRI of total spine/brain.

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

80
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

81
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

82
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)

83
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

84
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

85
Q

Rhabdomyosarcoma: stage/groups/favorable sites

A

add cards

86
Q

Rhabdomyosarcoma: follow up

A

H&P, CXR q 2 mo x 1 yr with imaging every other visit, then space out every 4 month visits in the 2nd and 3rd years, then annually

87
Q

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

A

low risk: 95%

intermediate risk: 65%

high risk: 45%

88
Q

Rhabdomyosarcoma: subset 1 on IRS-VI

A

Embryonal, botyroid, spindle

Stage 1-2, Group I-II;Stage 1, Group III orbit

i.e. favorable, resected with up to microscopic disease and/or nodes, OR orbit, OR stage II completely resected

89
Q

Rhabdomyosarcoma: subset 2 on IRS-VI

A

Embryonal, botyroid, spindle

Stage 1, Group III non-orbit

Stage 3, Group I-II

90
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)

91
Q

Rhabdomyosarcoma: intermediate risk chemo regimen

A

VAC/VI x 42 weeks

radiation at week 13

92
Q

Rhabdomyosarcoma: criteria for high risk

A

Stage 4, Group IV >10yo embryonal

All alveolar Stage 4 Group IV

93
Q

Rhabdomyosarcoma: high risk chemo regimen

A

VAC/VI x 48 weeks

radiation at week 20

94
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

95
Q

Ewings: workup

A

H&P. Peak ages 10-20

Labs: ESR, Alk phos

Imaging: Xray (onion skinning), MRI of primary (soft tissue component shrinks with chemo)

Biopsy, t11;22 (positive in 90%)

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

96
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
97
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

98
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

99
Q

Ewings: treatment paradigm

A

VDC/IE x48 weeks

local therapy at week 12

100
Q

Ewings: incidence of metastases

A

40% lung mets

20% bone mets

10% BM involvement

101
Q

Ewings: 5yr OS

A

60% if localized

30% with lung/pleural mets

15% if bone/BM mets

102
Q

Ewings: xrt for Askin tumor

A

First treat hemithorax to:

  • 15 Gy (age >6) for any lung tumor (mets, chest wall, pleural nodes, positive pleural effusion)
  • 12 Gy if ≤6 (uncommon)

Boosts:

Chest wall tumors with +cytology:

  • boost to ~50 Gy total
  • age >6, PTV1 30.6 Gy, PTV2 5.4
  • age ≤6, PTV1 32.4 Gy, PTV2 5.4
  • can shrink soft tissue lung component

Pleural nodules:

  • boost to ~50 Gy total
  • age >6, PTV1 21.6 Gy, PTV2 14.4 Gy
  • age ≤6, PTV1 23.4 Gy, PTV2 14.4 Gy
103
Q

Ewings: xrt dose for vertebral body

A

50.4 Gy

104
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

105
Q

Retinoblastoma: workup

A

H&P. Most all are diagnosed before age 3, and up to age 6. Leukocoria, family history of Rb.

No biopsy-this can seed tumor. Tumor easily recognizable by clinical features

Exam: fundoscopic exam (see white tumor with angiomatous dilation of blood vessels. Note size, diameter, thickness, seeding, distance from fovea, number of tumors), slit lamp test.

Imaging: US orbits, EUA orbits, CT/MRI orbits

Staging: MRI brain (trilateral retinoblastoma), bone scan or LP for metastatic disease (risk factors for mets include optic nerve, uveal, orbital, or choroidal invasion

Labs: genetic testing for RB1

106
Q

Retinoblastoma: treatment paradigm

A

RT is usually only used for salvage therapy.

Induction chemo first, consider RT if residual disease is present (rare). If no useful vision, do enucleation

EBRT dose is 45 Gy. May reduce dose to 36 Gy after chemo in Stage IVA disease if tumor is >5 mm

GTV = retina

CTV = globe, vitreous, 5 mm optic nerve. Do not include lens

PTV = none

107
Q

pediatric HL: criteria for intermediate risk disease

A

Stage IA-IIA with bulky disease or stage IB, IAE, IIB, IIAE, IIIA, IVA with or without bulky disease

Bulky= >6cm nodal aggregate or >1/3 ratio for mediastinum

(Anything not Stage IA, IIA, IIIB, or IVB)

108
Q

pediatric HL: treatment paradigm

A

ABVE-PC x4 (doxorubicin, bleomycin, vincristine, etoposide, prednisone, cyclophosphamide)

21 Gy in 1.5 Gy per fx

Consider avoiding in rapid early responders with CR (RER determined on CT at cycle 2 and CR determined on PET or CT at cycle 4)

Per AHOD Friedman trial, RT still required in all RERs without CR and all SERs!

RER=sum of perpenicular diameters of <60% of all volumes after cycle 2

109
Q

pediatric HL: criteria for low risk disease and treatment paradigm

A

IA or IIA

VAMP x4 cycles

May omit RT if CR after cycle 2 VAMP

Otherwise give 25 Gy RT

110
Q

pediatric HL: criteria for high risk disease and treatment paradigm

A

ABVE-PC x5 cycles

Treat all sites to 21 Gy (“standard”?)

Or “risk adapted RT”: RT to sites of initial bulk for RER, or for SER give RT to PET positive sites and sites >2.5 cm after cycle 2 (AHOD0831)

111
Q

Trilateral retinoblastoma: xrt fields and dose

A

50.4 Gy to pineal gland

45 Gy to cranium

36 Gy to spine

112
Q

pediatric T-cell ALL: risk stratification

A

CNS 1: CSF with no blasts, regardless of # WBCs

CNS 2: CSF with <5 WBCs and any blasts or ≥5 WBCs with negative;Steinherz Bleyer algorithm

CNS 3: CSF with ≥5 WBCs and blasts and/or clinical signs of CNS involved

113
Q

pediatric T-cell ALL: criteria for intermediate and high risk disease

A

All CNS 2-3.

Also CNS 1 poor responders, age <1 or ≥10, or WBC >50K

114
Q

pediatric T-cell ALL: treatment paradigm

A

Induction chemo with IT MTX first, then per AALL0434:

Int and high risk CNS1-2: 12 Gy in 1.5 Gy per fx

All CNS 3: 18 Gy in 1.8 Gy per fx

Low risk CNS 1-2: no RT (give prednisone)

residual testicular disease after chemo: 24 Gy

CN3 with overt cranial nerve involvement, symptoms: 24 Gy upfront before chemo, or 18 Gy

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