Ependymoma

Question 1

What is the appearance of an ependymoma compared to a medulloblastoma

What is

Answer 1

toothpasting out appearance vs. more solid pushing mass

Question 2

What is age of ependymoma

Answer 2

5-6 years and 35 years ( bimodal)

Question 3

Where do ependymomas occur

Answer 3

foramen of luschka and magnum ( arise from the floor of the 4th ventricle)

Question 4

WHat % will have CSF spread at diagnosis

Answer 4

5-10%

Question 4

What is the location of ependymomas

Answer 4

2/3 infratenrorial and 1/3 are supratentorial

Question 5

Where do ependymomas occur in kids vs adults

Answer 5

90% in kids are intracranial and 75% in adults are spinal

Question 6

Describe the workup for a ependymoma

Answer 6

History and physical exam
inlcuding neurolgical exam
MRI brain
MRI spine
Lumbar puncture with CSF cytology 10-14 days after post op and MRI

Question 7

What does the histolpathology of ependymomas look like

Answer 7

perivascular pseudoresettes- tumor cells around the blood vessels

Question 8

Describe ependymoma grading

Answer 8

grade 1: subpendymoma
grade 2: myxopapillary ependymoma, classic ependymoma
grade 3: anaplastic
grade 4: ependymoblastoma (now PNET)

Question 9

What are the molecular subtypes of ependymomas and their prognosis?

Answer 9

EPNA-B: good prognosis
YAP1 and RELA fusions and 1q gains are poor prognosis

Question 10

Decribe the treatmnt paradigm for ependymomas

Answer 10

max safe resection followed by RT
For kids under 18 months, chemo and second look surgerie are common to delay RT
Otherwise, chemo is not used

Question 11

Describe surgery

Answer 11

GTR is the most important factor in prognosis

Question 12

What are indications for RT

Answer 12

Infatentorial location
subtotal resection
Grade 3

Question 13

Decribe RT volumes

Answer 13

GTV1: preoperative disease
CTV 54 Gy: GTV1+ 1 cm, anatomically constrained to preop anatomy
Can extend through foramens magendie and luschka, jugurlar foramen, IAC, cavernous sinus
If there is gross nodular seeding of the posterior fossa, can cover entire posterior fossa with 54 Gy

GTV2: Postoperative residual disease (boost volume)
CTV59.4 Gy: GTV2+ tumor bed above cord + 5 mm

PTVs: CTV+ 3 mm

Question 14

What should be the max boost dose to GTV2 if the patient is 12-36 months?

Answer 14

54 Gy instead of 59.4 Gy

Question 15

What should be done from a RT perspective if the CSF is positive?

Answer 15

36 Gy CSI

Question 16

What are the indications for adjuvant RT for a spinal ependymoma

Answer 16

subtotal resection
Myxopapillary histology
/grade 2 ( this is controversial)
anaplastic histology

Question 17

Describe RT volumes

Answer 17

GTV1: preoperative disease
CTV1: GTV1+ 3-5 mm

GTV2: Postop residual disease+ tumor bed
CTV 50.4-59.4: GTV2+ 3-5mm
Can boost GTV2 if disease is below the cord