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CNS > Ependymoma > Flashcards

Ependymoma Flashcards

1
Q

In children and adults, what % of brain tumors are ependymomas?

A

Children: 5% (3rd most common childhood CNS tumor)
Adults: 2%

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2
Q

What is the median age of Dx for ependymomas?

A

Bimodal peak distribution, with peaks at 5 yrs and 35 yrs

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3
Q

What % of ependymomas arise intracranially, and how does this differ in children vs. adults? What are the most common locations?

A

Children: 90% intracranial (10% cord). If intracranial, the posterior fossa is the most common site (60% infratentorial [floor of 4th ventricle], 40% supratentorial [lat ventricle]).

Adults: 75% arise in spinal canal. Of intracranial tumors, two-thirds are supratentorial and one-third are infratentorial.

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4
Q

What is the cell of origin for ependymomas?

A

Ependymomas arise from the ependymal cells lining the ventricles.

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5
Q

What % of primary spinal tumors is ependymoma?

A

∼20% (meningiomas comprise 33%, spinal nerve tumors 27%)

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6
Q

What genetic syndrome is associated with spinal cord ependymoma?

A

Spinal cord ependymoma is associated with NF-2.

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7
Q

What % of ependymoma pts present with CSF seeding? What features predispose to seeding?

A

5%–10%;

infratentorial location, high-grade tumors, and local failure predispose to CSF seeding.

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8
Q

What are the types of the WHO classification Grade 1 of ependymoma?

A

myxopapillary and subependymoma

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9
Q

What type of ependymoma is WHO classification grade 2 ?

A

Classic ependymoma

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10
Q

What are the WHO grade 3 types of ependymoma?

A

Anaplastic

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11
Q

What is the type of WHO grade 4 ependymoma?

A

Ependymoblastoma

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12
Q

Where do grade IV ependymomas generally arise?

A

Grade IV ependymomas usually arise in the supratentorium.

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13
Q

What is the classical pathologic feature of ependymomas?

A

Perivascular pseudorosettes with ependymal rosettes are a classical pathologic feature of ependymomas.

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14
Q

What defines malignant ependymomas on pathology?

A

Greater number of mitoses, cellular atypia, and more necrosis

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15
Q

Which histopathologic subtype is most commonly found in the lumbosacral spinal cord?

A

Myxopapillary ependymomas usually arise in the conus/filum region of the spinal cord.

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16
Q

What is the typical presentation of ependymomas?

A

Depends on location.

If infratentorial: CN deficits, ↑ ICP; if supratentorial: seizures, focal deficits

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17
Q

With what neurologic deficits are spinal cord ependymoma pts likely to present?

A

Sensory deficits (vs. cord astrocytomas, which present with pain/motor deficits)

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18
Q

What is the workup for ependymoma?

A

Ependymoma workup: H&P, basic labs,
-MRI Spine
- Post-op MRI B (within 24-48 hours)
- CSF post-op (10-14 days post surgery)

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19
Q

When is LP contraindicated?

A

LP is contraindicated with a posterior fossa tumor with surrounding mass effect.

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20
Q

When should spinal MRI (not MRI brain) or CSF cytology be obtained after resection?

A

2 wks (10–14 days) postop to avoid false+.

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21
Q

What is the Tx paradigm for ependymoma?

A

Traditional ependymoma Tx paradigm:
max safe resection with adj RT for children >18mo (adj chemo if <18mo)

22
Q

Under what circumstances should CSI be done for ependymomas?

A

CSI should be done if +CSF, +MRI neuroaxis, and ependymoblastoma histology.

For all others, local RT is sufficient

23
Q

What evidence supports the omission of CSI for anaplastic ependymomas after resection if there is no evidence of neuroaxial involvement?

A

Multiple retrospective reviews reveal the following: LR is the primary pattern of failure (>90%) regardless of field size; spinal seeding is uncommon without LR; and prophylaxis with CSI or WBRT does not affect survival when compared to local RT.

24
Q

What is the role of chemo in ependymoma? What is the response rate?

A

Traditionally, chemo is utilized for <3 yo to delay RT and for salvage (cisplatin, VP-16, temozolomide, nitrosoureas). The response rate typically is 5%–15%.

However, study from St. Jude’s Children’s Hospital (Merchant TE et al., Lancet Oncol 2009), which included many pts <3 yo (78%) treated with maximal safe resection and postoperative conformal RT to 59.4 Gy with 10-mm margin around postop bed, suggests that RT can be given safely and effectively for pts <3 yo. The 7-yr OS was 81%, EFS was 69%, and LC rate was 87.3% (cumulative LF rate is 16.3%). Therefore, young age should not preclude pts from receiving high-dose RT after surgery, except for infants <1 yo. Current protocols require postop RT in completely resected infratentorial ependymoma starting at age 18 mos.

If STR, chemo may be used to see if GTR is possible with 2nd-look surgery after 2 cycles of chemo.

25
Q

What is the single most important favorable prognostic factor in ependymoma?

A

Completeness of surgical resection (correlates closely with LC for ependymomas)

26
Q

What is the difference in 5-yr OS between GTR and STR for ependymomas?

A

75% vs. 35% (similar for low-grade vs. high-grade ependymomas)

27
Q

What ependymoma locations are most amenable to GTR? Least?

A

Spinal (GTR ∼100%) > supratentorial (80%) > infratentorial

28
Q

What is given to children <3 yo after STR for ependymoma?

A

2 cycles of chemo can be used as a bridge Tx to see if GTR can be achieved.
RT can be deferred with chemo until >18 mos, based on the St. Jude’s trial (Merchant TE et al., Lancet Oncol 2009) and SEER analysis (Koshy M et al., J Neurooncol 2011).

Both suggest that postop RT in children <3 yo improves survival.

29
Q

What types of chemo are typically used for ependymoma?

A

Cisplatin, cyclophosphamide, and etoposide are typical chemo agents for ependymoma.

30
Q

What is the dose and volume of RT to be used if no CSI is given for ependymomas?

A

Preop GTV + 1–2-cm margin to 54–59.4 Gy (54 Gy for children <18 mos and >18 mos with GTR)

31
Q

How is ependymoblastoma treated? What is the total dose to spine lesions vs. cranial lesions?

A

Treat like high-risk medulloblastoma/PNET:

CSI 36 Gy + vincristine +/– carboplatin, boost to cavity/gross Dz.

45–50.4 Gy if spine and 54–59.4 Gy if cranial → vincristine/Cytoxan/prednisolone 6 wks after RT.

32
Q

How is infratentorial ependymoma managed?

A

Max safe resection followed by involved field postop RT to a dose of 54–59.4 Gy.

33
Q

How is supratentorial ependymoma managed?

A

If not anaplastic (i.e., if grades I–II), observation after max GTR is acceptable.

34
Q

How is recurrent ependymoma managed?

A

If no prior RT: surgery → RT
If prior RT: surgery → stereotactic RT or chemo

35
Q

Which phase II study showed min neurocognitive decrement with conformal/small RT fields?

A

St. Jude’s study ACNS0121
(Merchant TE et al., JCO 2004): 88 pts, 33 pts with grade 3. 3-yr PFS was 74%. IQ testing was stable after 2 yrs.

36
Q

What is a major reason infratentorial lesions should get adj RT, regardless of histologic grade?

A

Difficulty with complete resection due to proximity to floor of 4th ventricle, or laterally protrusion through foramen of Luschka and involvement of CN nerves or CNS vessels → higher LR if infratentorial without RT

37
Q

Which studies showed a benefit with adj RT after GTR for posterior fossa ependymomas?

A

Rogers L et al.: 10-yr LC GTR/RT 100% vs. 50% GTR alone. Nonsignificant benefit in 10-yr OS GTR (67%), GTR/RT (83%). (J Neurosurg 2005)

Merchant TE et al.: 5.3-yr median follow-up update from the phase II study ACNS0121. All rcvd conformal RT to 59.4 Gy for NTR/all sites and grade, and for R0 infratentorial lesions of all histologies. Well-differentiated lesions after GTR were observed. Chemo for STR, then evaluated for surgery and RT. 7-yr OS was 81%, LC was 87.3%, and EFS was 69.1%. Median age 2.9 yrs, with 78% of the pts <3 yo. (Lancet Oncol 2009)

38
Q

When is RT used in spinal ependymomas?

A

When resection is incomplete or anaplastic histology (Kaiser data: Volpp PB et al., IJROBP 2007)

39
Q

What fields/doses are used for spinal ependymomas?

A

Primary tumour plus a 3-5cm margin to 45 Gy (boost if below cord to 50.4–59.4 Gy)

40
Q

What molecular profile is associated with poor outcomes in ependymoma?

A

Gain of chromosome 1q

41
Q

Do young children or young adults with ependymoma have a worse prognosis?

A

Children. Age <4 yrs is a poor prognostic factor.

42
Q

Which ependymoma lesions have a poorer prognosis: supratentorial or infratentorial?

A

Supratentorial (↑ high grade and more STR) (Mansur DB et al., IJROBP 2005)

43
Q

What are the 5- and 10-yr OS rates for pts with grades II–III ependymomas?

A

70% and 55%, respectively (Mansur DB et al., IJROBP 2005); no difference between grade II and grade III tumors (p = 0.71)

44
Q

What % of ependymoma pts eventually die of their Dz?

A

50% of ependymoma pts eventually die of their Dz.

45
Q

How long of a follow-up is required for pts with ependymoma?

A

At least 10 yrs, because late recurrences of >12 yrs after surgery can occur.

46
Q

What imaging is required during the follow-up for ependymoma pts?

A

Craniospinal MRI q3–4mos for yr 1, then q4–6mos for another yr, then q6–12mos.

47
Q

What is a commonly used dose constraint for the spinal cord?

A

45 Gy is the usual dose constraint for the spinal cord.

48
Q

What is a commonly used dose constraint for the chiasm?

A

50.4–54 Gy is the usual max point dose constraint for the chiasm.

49
Q

What is the macroscopic appearance of ependymoma?

A
  • Exophytic soft tan mass extending into 4th Ventricle
  • Discrete margin from surrounding brain/spinal cord
  • may fill entire 4th ventricle and exit through Foramen of Luschka or Magendie
50
Q

Microscopic features of ependymoma

A
  • Key histologic features:
  • Perivascular pseudorosettes and Ependymal rosettes
  • Moderately cellular
  • Salt and pepper chromatin
  • rare mitoses
  • well circumscribed and cellular neoplasm with sheet like growth pattern
51
Q

IHC profile of Ependymoma

A

GFAP+
EMA+
S100+
Vimentin +

52
Q

Describe a suitable dose and contour technique for ependymoma

A

Dose: 59.4Gy/ 33# (1.8Gy/#) (54Gy is <18mo and GTR)
CSI 36Gy if M+

GTV = residual and tumour bed
CTV = GTV +1cm clipped to anatomical boundaries
PTV = CTV +0.3-0.5cm

CNS (9 decks)

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