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Pediatrics > ROQ: Medulloblastoma, Ependymoma, Brainstem Glioma, Craniopharyngioma > Flashcards

ROQ: Medulloblastoma, Ependymoma, Brainstem Glioma, Craniopharyngioma Flashcards

1
Q

What is the most common location(s) for an ependymoma?

A

Most to least common:
- Intracranial: ~95%
– Posterior fossa (infratentorial): 2/3
– Supratentorial: 1/3
- Spinal Cord: ~5%

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2
Q

What is the most important prognostic factor for ependymomas?

A

Extent of resection

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3
Q

What is the rate of 7-yr EFS for GTR vs. STR for ependymomas?

A
  • GTR: 77%
  • STR: 34%
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4
Q

What is the rate of 7-yr OS for GTR vs. STR for ependymomas?

A
  • GTR: 88%
  • STR: 52%
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5
Q

What are the most common malignancies in children (0-19 yrs old)?

A
  • Leukemias
  • CNS (20-25%)
    – Highest death rate
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6
Q

Which molecular subtype and features of ependymomas has the worst prognosis?

A
  • Supratentorial ependymoma, ZFTA fusion-positive
    – Previously RELA-fusion positive
  • Gain of chromosome 1q
    – 5 yr EFS (47.4% vs. 82.8%, p=0.0013)
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7
Q

Which molecular subtype of ependymomas has the best prognosis?

A
  • Supratentorial ependymoma, YAP1-fusion positive.
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8
Q

What is the general tx paradigm for the tx of ependymomas?

A
  • Maximal Safe Resection: GTR a/w the greatest survival benefit
  • f/b IFRT
  • < 18 mos → CHT f/b 2nd look surgery if STR to delay RT in an infant
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9
Q

What is the peak incidence age range for a dx of medulloblastoma?

A

5-9 yrs

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10
Q

What is the typical location of Atypical Teratoid Rhabdoid Tumors (ATRT)?

A

Cerebellum: ~ 2/3 rd

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11
Q

What % of newly dx medulloblastoma pts will have CSF dissemination at dx?

A

33%

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12
Q

What locations within the cerebellum/posterior fossa are typical for medulloblastoma?

A
  • Vermis, typically
  • More lateralized in older pts
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13
Q

What are the general dose and contouring guidelines for ependymomas?

A
  • Per COG ACNS 0831
    – Fuse both T1 post-gad and T2 pre and post-op MRIs to the planning CT
    – GTV: Gross residual tumor and tumor bed based
    – CTV: GTV + 5 mm (NOTE that prior protocols used 1 cm)
    – Total Dose: 59.4 Gy / 33 fx
    — Unless age < 18 mos and GTR, then treated to 54 Gy
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14
Q

What is the open trial COG ACNS 0831 investigating?

A
  • Phase Ill study of:
    1. GTR of a differentiated, supratentorial ependymoma → Observation
    2. STR → induction chemo → 2nd-look surgery → RT → chemotherapy
    3. All others: RT f/b chemo vs. observation
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15
Q

What is the most common cerebellar malignant tumor in children?

A
  • Medulloblastoma
  • ~20% of all brain tumors
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16
Q

Is WNT subgroup of medulloblastoma fav. or unfav. and how does it affect tx recs?

A
  • Favorable
  • a/w Turcot Syndrome
  • Does not affect tx recs
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17
Q

What is the M staging for medulloblastoma?

A
  • M0
  • M1: Microscopic tumor cells in the CSF
  • M2: Gross nodular seeding in the intracranial subarachnoid space or ventricular system distant from the primary site
  • M3a - Gross nodular seeding in the spinal subarachnoid space with no evidence of
    intracranial seeding.
  • M3b - Gross nodular seeding in the spinal subarachnoid space plus intracranial seeding.
  • M4 - Extraneural metastasis.
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18
Q

What is the long-term disease control for a craniopharyngioma tx w/ STR f/b adj. RT?

A

80-100%

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19
Q

What is the rate of endocrinopathies for a craniopharyngioma tx w/ STR f/b adj. RT?

A

~20%

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20
Q

Are craniopharyngiomas benign?

A

Yes! They are benign, locally destructive lesions

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21
Q

What is the rate of endocrinopathies for a craniopharyngioma tx w/ GTR only?

A

~ 50%

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22
Q

What is the rate of diabetes insipidus for a craniopharyngioma tx w/ GTR only?

A

~ 80-90%

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23
Q

What is the rate of hypothalamic obesity for a craniopharyngioma tx w/ GTR only?

A

~ 50%

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24
Q

What is the buzzword for the fluid that drains out of a craniopharyngioma?

A

Crankcase Oil

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25
Q

What is the rate of recurrence of craniopharyngiomas s/p GTR alone vs. STR alone?

A
  • GTR: ~10% at 2 yrs, ~30% at 5 yrs
  • STR: >75% at 6 mos!
    – RT is always recommended (min dose 54-55.8 Gy)
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26
Q

What are the two main WHO sub-types for craniopharyngiomas?

A
  • Papillary
  • Adamantinomatous
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27
Q

What is an average risk medulloblastoma pediatric patient?

A
  • Avg. risk medulloblastoma
    – Incidence: 66%
    – ≥ 3 years old,
    – ≤1.5 cm2 residual
    – M0
    – Classic, or desmoplastic, or focal anaplastic histology
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28
Q

What are the different histologic variants of medulloblastoma?

A
  • Classic
  • Desmoplastic/nodular
  • Desmoplastic with extensive nodularity
  • Large cell
  • Anaplastic

– Desmoplastic variant has the best prognosis
- Anaplastic and large cells have the worst prognosis

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29
Q

What is the typical tx for an average-risk pediatric medulloblastoma patient?

A

Avg. risk medulloblastoma pt:
- CSI 23.4 Gy
- → IFRT to 30.6 Gy boost (54 Gy total) w/ conc. vincristine
- → PCV/Cyclov CHT (x9C q6 wks)

30
Q

What is the CSI dose for an average-risk pediatric medulloblastoma patient who cannot receive concurrent vincristine 2/2 renal issues?

A
  • 36 Gy
31
Q

What was the 5-yr EFS in the POG8631/CCG 923, which randomized medulloblastoma pts to 23.4 Gy vs. 36 Gy CSI. w/o CHT f/b posterior fossa boost to 54 Gy,

A
  • 23.4 Gy: 67%
  • 36 Gy: 52%
  • p =0.008
32
Q

How long does the tx of standard-risk medulloblastoma take?

A

~ 60 wks (~1 yr)

33
Q

How do you define the IFRT volume for avg. risk medulloblastoma pt s/p CSI?

A
  • Gross tumor + resection cavity
  • 1.5 cm expansion excluding bone and tentorium
34
Q

What are the presenting sx of medulloblastomas?

A
  • Sx 2/2 posterior fossa mass:
    – Raised ICP
    – Headache
    – Vomiting
    – Diplopia
    – Head tilt
    – Gait ataxia
    – Behavioral changes
35
Q

How often should you feather fields when treating w/ CSI?

A
  • q 9 Gy
  • Shift up and down by 0.5-1.0 cm to the field match points
36
Q

What is a high-risk pediatric medulloblastoma patient?

A
  • High-risk medulloblastoma
    – Incidence: 33%
    – < 3 yr old
    – STR > 1.5 cm2 residual
    – M+
    – Diffuse anaplastic histology
37
Q

What is the management of supratentorial neuroepithelial tumor (previously, PNET (primitive neuroectodermal tumors))?

A

Same as Medulloblastoma:
- CSI 36 Gy
- → PF + Met (>1 cm) to 19.8 Gy boost (55.8 Gy tota) w/ conc. vincristine
- → PCV CHT

38
Q

What is the typical tx for a high-risk pediatric medulloblastoma patient?

A
  • CSI 36 Gy
  • → PF + Met (>1 cm) to 19.8 Gy boost (54-55.8 Gy) w/ conc. vincristine
    – If discrete spots of disease within the spine, boost of 9 gy (45 Gy total)
    – If diffuse spine disease, a boost of 3.6 Gy (39.6 Gy)
  • → PCV CHT
39
Q

What are the sx of posterior fossa syndrome and when does it occur?

A
  • Occurs w/i 1-2 days of posterior fossa tumor resection
  • Incidence: ~ 22%
  • Sx: SAME
    – Swallowing difficulty
    – Ataxia
    – Mutism
    – Emotional lability
40
Q

What are the classic borders of the posterior fossa boost after CSI?

A
  • Borders:
    – Sup = Tentorium
    – Inf = C2-3 junction, through foramen magnum
    – Lat = Bony walls of the occiput and temporal bones
    – Ant = Posterior clinoids and anterior C1
    – Post = Cerebellar folia / Internal occipital protuberance
  • Editing:
    – The pituitary should be blocked unless involved
    – the ENTIRE brainstem SHOULD BE INCLUDED
    – PTV: CTV + 3-5 mm (should extend anterior to posterior clinoid)
41
Q

What were the purpose, pt population, and randomization of ACNS 0331?

A
  • Purpose
    – Role of reduced dose CSI and reduced boost field in double randomization in pediatric patients w/ average-risk medulloblastoma (≥ 3 years old, ≤ 1.5 cm2 residual, M0)
  • Pts
    – Aged 3-7
  • Double-Randomization
    – 23.4 Gy vs. 18 Gy CSI
    – Entire posterior fossa boost vs. involved surgical field boost
    – Weekly vincristine for all
42
Q

What were the results of ACNS 0331?

A
  • Median follow-up: 9.3 years
  • Surgical field boost vs. posterior fossa boost:
    – 5-yr OS: 85% vs 85% (NS)
    – 5-yr EFS: 82% vs 80% (NS)
    – 5-yr LR: 10% vs 8% (NS)
  • CSI: 18 Gy vs. 23.4 Gy
    – 5-yr OS 78% vs. 86% (p<0.05)
    – 5-yr EFS: 71% vs. 83% (p<0.05)
    – 5-yr isolated distant failure: 13% vs. 8% (NS)
43
Q

What is the age distribution for craniopharyngiomas?

A
  • Bi-modal distribution, w/ peaks around
    – 5-15 yrs
    – 50-75 yrs
44
Q

How often should you obtain MRIs for pts undergoing RT for craniopharyngiomas, and why?

A
  • Weekly MRIs to determine if there is a reaccumulation of the cyst.
  • CT scans, particularly cone-beam CT scans, are not sensitive enough to clearly define a reaccumulating cyst
45
Q

For pts receiving CSI for medulloblastoma, what radiation dose and age predict the least impact on IQ?

A
  • Low radiation dose
  • Older age (≥ 7 yrs)
46
Q

What is the initial tx of a newly-dx medulloblastoma?

A
  • Maximal safe resection
  • GTR results in a significantly improved prognosis
47
Q

For medulloblastomas, when should RT start after resection?

A

3-4 weeks

48
Q

What are the late-effect benefits of using protons vs. photons for CSI in children?

A
  • Lower risk of:
    – Cardiac damage
    – Lung damage
    – Renal damage
    – Cochlear damage (ototoxicity)
    – 2ndary neoplasms
    – Endocrinopathies
49
Q

How does using protons vs. photons for CSI in children affect adult height?

A
  • Adult height is comparable b/w protons and photons
    – Vertebral bodies are normally included in the proton CSI volume to prevent asymmetric growth in children
50
Q

Which embryonic remnant gives rise to cranipharyngiomas?

A

Rathke’s pouch
- An invagination from the roof of the oral cavity that develops into the anterior portion of the pituitary gland (adenohypophysis)
- The neurohypophysis (posterior pituitary gland) descends from the developing brain

51
Q

What is the optimal match level for cranial and spinal fields for CSI, especially when using photons?

A
  • As low as possible
    – ~C5-6
    – Reduces dose to the mouth
    – Limited by shoulders blocking lateral fields
52
Q

How can you distinguish atypical teratoid rhabdoid tumors (ATRT) from medulloblastomas?

A
  • IHC for INI-1
    – A tumor suppressor gene on Ch 22
    – Lost in ~90% of ATRT
    – Intact in Medulloblastomas
53
Q

What is the typical age of dx of ATRT, and what is the mainstay of tx?

A
  • ≤ 3 yrs
  • Surgical resection ± PORT
  • CHT under investigation
54
Q

In what order do pituitary hormones decrease after brain RT in children?

A
  • In order:
    – GH
    — Endocrinologists check IGF-1 levels to infer GH levels
    – FSH/LH
    – TSH / ACTH
  • ADH is unaffected by RT
    – it is adversely affected by surgery, which can lead to DI
55
Q

What is the workup for ependymoma and medulloblastoma?

A
  • Pre-op and post-op MR spine w/ contrast
  • LP w/ cytology
    – 6-16% incidence of neuraxis dissemination
    – Post-op LP 10-14 days post-op to allow for surgical debris to clear
    – Pre-op LP prior to surgical intervention, but only if there is no evidence of elevated intracranial pressure or hydrocephalus by imaging and exam
56
Q

What is the typical location of a medulloblastoma?

A
  • Cerebellar vermis
57
Q

Which CSF conduit is typically blocked by a medulloblastoma?

A

Cerebral Aqueduct (Aqueduct of Sylvius)

58
Q

When should the staging Spine MRI be obtained for medulloblastoma?

A
  • Pre-op
  • 10-14 days post-op
    – Avoid immediate leftover from surgery (blood products, etc) from meddling the results
59
Q

When should the post-op Brain MRI be obtained for Medulloblastoma?

A
  • < 48 hrs after surgery
  • To avoid confusing post-op changes, which start manifesting ≥ 48 hrs post-surgery from being mistaken fo residual tumor
60
Q

What are the different molecular/genetic classifications of medulloblastoma and their associated prognosis?

A
  • WNT: best prognosis
    – CTNNB1, stain for β catenin
  • SHH: intermediate prognosis
    – PTCH1 (Gorlin syndrome), SMO, SUFU, MYCN amplification
  • Group C/3: worst prognosis
    – MYC amplification and metastatic spread
  • Group D/4: intermediate prognosis
    – isochrome 17q

WNT: Winning
Group 3 > W

61
Q

Which medulloblastoma mutation is freq linked to the Gorlin syndrome?

A

SHH

62
Q

Which cells give rise to ependymomas?

A

Ependymal cells lining the ventricles

63
Q

Which cells give rise to meningiomas?

A

Arachnoid cap cells

64
Q

What is the reason for significant hematologic toxicity in medulloblastoma pts receiving CSI?

A

Irradiation of significant amounts of bone marrow

65
Q

How do you treat hematologic toxicity in medulloblastoma pts receiving CSI and boost?

A
  • Stop CSI, switch to boost
  • Resume CSI once leukopenias resolve
66
Q

Is vincristine a/w cytopenias?

A

NO!

That’s why you don’t have to stop it when a pt develops cytopenias while undergoing CSI.

67
Q

What is the prognosis of a child dx w/ DIPG?

A
  • Uniformly poor prognosis
  • Median survival after dx: 6-12 mos
68
Q

What is the general tx paradigm for pineoblastoma?

A
  • Similar to other high-risk CNS tumors
  • Maximal safe resection
  • CSI: 36 Gy
    –Gross disease above spinal cord terminus: Boost of 9 Gy (45 Gy)
    – Gross disease below spinal cord terminus: Boost of 18 Gy (54 Gy)
    – Diffuse spinal disease; Boost to 3.6 Gy (39.6 Gy)
  • Posterior fossa boost: 19.4 Gy
69
Q

What are the CT simulation and RT field considerations for CSI?

A
  • General:
    – Supine or prone
    – Deep sedation or general anesthesia for young children
    – Neck extended sufficiently to keep the mandible out of the exit beam of the spinal field
  • Brain Fields:
    – Parallel opposed fields
    – Extend to > 1 cm beyond the scalp
    – Collimator rotated to match the divergence of the spinal field
  • Spinal Field:
    – Single PA field.
    – Should cover 1-1.5 cm beyond the vertebral bodies laterally
  • Posterior fossa boost
    – Supine or prone
70
Q

What are the general targets for CSI?

A
  • Whole-brain CTV1:
    – Ant: Frontal lobe, cribriform plate anteriorly, superior orbital tissue but not posterior globe
    – Inf: > 0.5 cm below the base of the skull at the foramen magnum
  • Spine CTV1
    – Entire thecal sac using the spinal MRI to determine the inferior border, ~2 cm below the termination of the subdural space
  • Limited target volume boost CTV boost:
    – GTV using preop MRI
    – CTV boost is GTV + 1.5 cm margin
    – Limit to bone or tentorial interface
71
Q

What age cutoff is a/w the highest risk of endocrinopathies from RT?

A

≤ 10 yrs

Pediatrics (3 decks)

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