CNS/PEDS Flashcards
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
GBM: Fields and dose (RTOG)
CTV 46Gy: T2 + 2cm
CTV 60Gy: T1 post / cavity + 2cm
3-5mm for PTV
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
GBM: max dose constraints for chiasm, brainstem, optic nerves, retina, and lenses
chiasm 55Gy
brainstem 60Gy
optic nerves 55Gy
retina 50Gy
lenses 7Gy
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
RANO criteria for pseudoprogression:
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
GBM: XRT toxicity
Pseudoprogression: 25%
Necrosis: 10%
GBM: Outcome
MS 17mo
24mo if MGMT methylated
GBM: temozolomide toxicity
nausea, constipation, low platelets, PCP (prophylaxis with bactrim)
General CNS simulation
supine, arms at sides
thermoplastic mask
fuse preop and postop MRI
Grade 3 Anaplastic gliomas with 1p19q codel: treatment
59.4Gy/33fxs to GTV + 2cm CTV margin
neoadjuvant PCV x4 cycles (procarbzine, CCNU/lomustine, vincristine)
also reasonable to do concurrent/adjvuant temozolomide
Grade 3 Anaplastic gliomas without codel: treatment
59.4Gy/33fxs to GTV + 2cm CTV margin
adjuvant temolozomide (no benefit yet with concurrent on CATNON)
also reasonable to do adjuvant PCV
Grade 2 glioma: treatment
54Gy/33fxs to GTV (FLAIR/T2) + 1.5cm
Adjuvant PCV x6 cycles
SATAN criteria
Size >6cm
Age >40
Tumor crossing midline
Astrocytoma
Neuro deficits
Ependymoma: indications for treatment
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
Spinal Ependymoma: fields and dose
- 4Gy/28fxs
- 5 cm margin superiorly and inferiorly, can include nerve roots radially
usually occurs at conus and filum terminale
Spinal Ependymoma: outcomes
10yr LC:
GTR + RT 90%
STR + RT: 70%
GTR 50%
STR 0%
Brain Ependymoma: field and dose
54Gy then off-cord boost to 59.4Gy
preop GTV + 1cm CTV + 0.3-0.5cm PTV
Brain Ependymoma: indications for CSI
Do CSI if CSF+ or MRI+
36Gy CSI the boost gross cord disease to 45Gy
Brain Ependymoma: Outcomes
10yr LC:
GTR + RT 100%
GTR 50%
STR + RT 30%
Pituitary tumor: treatment paradigm
transphenoidal surgery then medical management then radiation
stop medical management during radiation
for prolactinoma, medical management comes first
Pituitary tumor: medications for prolactinoma, ACTH, GH
prolactinoma: cabergoline or bromocriptine
ACTH: ketoconazole, mitotane
GH: octreotide, lanreotide, pegvisomat (IGF-1 blocker $$$$)
Pituitary tumor: indications for radiation
unresectable tumor
failure after surgery and/or medical management
TSH-secreting tumor (all TSH get post-op RT to 54Gy)
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
Primary CNS lymphoma: treatment paradigm
(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
Primary CNS lymphoma: fields and dose
23.4Gy WBRT then boost focal disease to 45Gy
WBRT should include C1-C2 and posterior eyes (or entire eye if involved)
grade 1-2 meningioma: fields and dose
GTV + 0.5-1cm CTV to 54Gy (50.4Gy if optic)
SRS 14-16Gy
grade 3 meningioma: fields and dose
GTV + 2cm CTV to 54 Gy then boost GTV + 1cm CTV to 60 Gy
diffuse intrinsic pontine glioma: fields and dose
54Gy to GTV + 0.5cm CTV
MS 10 months
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
criteria for standard risk medulloblastoma
>3yo
<1.5cm2 residual
M0
standard risk medulloblastoma: treatment paradigm
maximal safe resection then XRT with concurrent vincristine then adjuvant PCV
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
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.
standard risk medulloblastoma: adjuvant chemo
PCV (cisplatin, CCNU, vincristine) starting 6 weeks after RT
medulloblastoma: outcomes
standard risk 5yr EFS 80%
high risk 5yr EFS 60%
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
AT/RT: treatment paradigm
induction chemo with vincristine, cisplatin, cyclophosphamide, etoposide, methotrexate
36Gy CSI then boost primary to 54Gy
consolidation with thio/carbo/ASCT
2yr OS 50%
Intracranial germinoma (localized): fields and dose
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.
Intracranial germinoma (disseminated): fields and dose
24Gy CSI then boost gross disease to 45Gy (all using 1.5Gy fractions)
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)
Intracranial germinoma (disseminated): fields and dose
2-4 cycles of carbo/etoposide then 21 Gy CSI with boost to 30 Gy (if CR to chemo)
Intracranial germinoma and NSGCT: outcomes
germinoma 5yr PFS 90%
NSGCT 5yr PFS 60%
Intracranial Germinoma and NSGCT: incidence and tumor markers
66% germinoma / 33% NSGCT
15% of germinomas produce low level β-HCG
β-HCG is >100 or AFP >10 excludes germinoma
Intracranial germ cell tumor: workup
H&P (esp CNs, funduscopic exam)
MRI brain/spine
CBC, CMP, serum AFP/β-HCG, CSF AFP/β-HCG/cytology
Intracranial NSGCT: treatment paradigm
6 cycles alternating carbo/etop and ifos/etop q 3 wk
36Gy CSI then boost pre-chemo disease to 54Gy
Craniopharyngioma: treatment paradigm
max safe resection. Consider EBRT or intracystic chemo if subtotal resection or at recurrence
Craniopharyngioma: fields and dose
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
Craniopharyngioma: when to avoid RT
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)
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
VAD: components
vincristine, dactinomycin, doxorubicin
VDC/IE: components
vincristine, doxorubicin, cyclophosphamide, ifosfamide, etoposide
CAPETV: components
cyclophosphamide, doxorubicin, cisplatin, etoposide, topotecan, vincristine
Pediatrics: general follow up
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
Wilms: workup
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
Wilms: treatment paradigm
nephrectomy
adjuvant radiation at post-op day 14
VAD chemotherapy x25 weeks
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
Wilms: radiation doses
10.8 Gy to primary site
+10.8 Gy boost if gross residual to total 21.6 Gy
- 8 Gy to resected LNs
- 8 Gy to unresected LNs
- 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
- 8 Gy to flank for any stage rhabdoid (RTK) tumor or diffuse anaplasia Stage III
- 8 Gy for Stage I-II diffuse anaplasia
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.
Wilms: abdominal fields
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.
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
Wilms: criteria for stage III (radiation)
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)
Wilms: 10yr OS by stage with and without anaplasia
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%
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
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
Wilms: treatment of lung mets
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
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)
Wilms: doses for metastases to liver, brain, and bone
Liver: 19.8Gy to whole liver
Brain: 21.6Gy WBRT + 10.8Gy boost
Bone: 25.2Gy + 1cm margin
Neuroblastoma: INRG criteria for high risk disease
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
Neuroblastoma: INRG criteria for standard risk disease
stage MS<18 mos with no n-myc or 11q
Neuroblastoma: workup
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.
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)
Neuroblastoma: xrt doses
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
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)
Neuroblastoma: dose constraints
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%
Neuroblastoma: incidence
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
Neuroblastoma: xrt dose for cord compression
9Gy if <3yo
21.6Gy if >3yo
Neuroblastoma: 3yr OS for high risk
3yr OS 60%
Rhabdomyosarcoma: workup
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
Rhabdomyosarcoma: xrt doses
50.4 for gross residual
45 Gy for orbit
41.4 Gy to +nodes (resected)
36 Gy for microscopic margins
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
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)
Rhabdomyosarcoma: constraints
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
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
Rhabdomyosarcoma: stage/groups/favorable sites
add cards
Rhabdomyosarcoma: follow up
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
Rhabdomyosarcoma: 5yr OS for low, intermediat, high risk
low risk: 95%
intermediate risk: 65%
high risk: 45%
Rhabdomyosarcoma: subset 1 on IRS-VI
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
Rhabdomyosarcoma: subset 2 on IRS-VI
Embryonal, botyroid, spindle
Stage 1, Group III non-orbit
Stage 3, Group I-II
Rhabdomyosarcoma: criteria for intermediate risk
Embryonal, spindle, botyroid:
Group III, unfavorable (Stage 2-3, not stage 1)
Alveolar histology, no mets (Stage 1-3, Group I-III)
Rhabdomyosarcoma: intermediate risk chemo regimen
VAC/VI x 42 weeks
radiation at week 13
Rhabdomyosarcoma: criteria for high risk
Stage 4, Group IV >10yo embryonal
All alveolar Stage 4 Group IV
Rhabdomyosarcoma: high risk chemo regimen
VAC/VI x 48 weeks
radiation at week 20
Rhabdomyosarcoma: whole lung radiation dose
15 Gy/ 10 fx
12 Gy/ 8 fx for <7 yo
Boost tumor to 50.4 Gy total
Ewings: workup
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
Ewings: xrt doses
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
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
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
Ewings: treatment paradigm
VDC/IE x48 weeks
local therapy at week 12
Ewings: incidence of metastases
40% lung mets
20% bone mets
10% BM involvement
Ewings: 5yr OS
60% if localized
30% with lung/pleural mets
15% if bone/BM mets
Ewings: xrt for Askin tumor
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
Ewings: xrt dose for vertebral body
50.4 Gy
Ewings: xrt dose for involved lymph nodes
Resected: PTV1 to 50.4 Gy to that LN level
Unresected: PTV1 to 45 Gy then boost PTV2 10.8 Gy
Retinoblastoma: workup
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
Retinoblastoma: treatment paradigm
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
pediatric HL: criteria for intermediate risk disease
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)
pediatric HL: treatment paradigm
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
pediatric HL: criteria for low risk disease and treatment paradigm
IA or IIA
VAMP x4 cycles
May omit RT if CR after cycle 2 VAMP
Otherwise give 25 Gy RT
pediatric HL: criteria for high risk disease and treatment paradigm
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)
Trilateral retinoblastoma: xrt fields and dose
50.4 Gy to pineal gland
45 Gy to cranium
36 Gy to spine
pediatric T-cell ALL: risk stratification
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
pediatric T-cell ALL: criteria for intermediate and high risk disease
All CNS 2-3.
Also CNS 1 poor responders, age <1 or ≥10, or WBC >50K
pediatric T-cell ALL: treatment paradigm
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
