CNS Pathology case based MCQs-2

CASE : The spinal MRI of a 22-year old male with neurofibromatosis type-2 showed a hyperintense lesion. The lesion was resected and histopathologic section is shown in image.

Question 1: What is the diagnosis?

A. Medulloblastoma
B. Ependymoma
C. Retinoblastoma
D. Central neurocytoma

Question 2: Which of the following statements is true regarding this tumor?

A. They are predominantly supratentorial
B. Posterior fossa tumors in adults have a good prognosis
C. Spinal tumors have a bad prognosis
D. IDH 1 and 2 mutations are present in 100% cases.

Question 3: Identify the variant of the tumor shown in the image below.

A. Tancytic
B. Myxopapillary
C. Subependymoma
D. Anaplastic

Question 4: Which of the following statements is true regarding tancytic ependymoma?

A. GFAP negative
B. Abundant rosettes
C. Can be confused with pilocytic astrocytoma
D. WHO Grade 1

Question 5: Identify the FALSE statement regarding the variant of ependymoma shown below

Image credit- http://www.twitter.com/DrAldehyde

A. WHO Grade 1
B. Arises from filum terminale
C. Aggressive clinical course
D. Mucin stains positive

Question 6: TRUE statement regarding recent molecular classification (WHO CNS 2021 5th edition ) of ependymoma is

A. Spinal ependymomas with MYCN have a good prognosis
B. ZFTA Fusion- favorable prognosis
C. YAP Fusion- poor prognosis
D. Spinal ependymomas with NF 2 mutation- favorable prognosis

ANSWER- Question 1

Correct answer is B- Ependymoma

Ependymomas are characterized by perivascular rosettes and ependymal rosettes which have an empty central lumen

Types of rosettes
ANSWER- Question 2

CORRECT ANSWER IS B

🐱 Majority of of Ependymomas are infratentorial.

🐱Posterior fossa tumors in children have a poor prognosis (PF-A)- associated with loss of H3K27me, whereas Posterior fossa tumors in adults have a god prognosis (PF-B)- H3K27me is retained.

🐱 Spinal ependymomas have a good prognosis, whereas a small number of ependymomas associated with MYCN have a poor outcome.

🐱IDH 1 and 2 are associated with astroctyomas and oligodendroglomas but nor ependymomas.

ANSWER-Question 3

CORRECT ANSWER IS A

Diagnosis is TANCYTIC EPENDYMOMA, 🐱 Tancytes are cells with fibrillary processes which line ventricles along with ependymal cells.

Types of tancytes
ANSWER – Question 4

Correct They are GFAP positive 🐱Confused with pilocytic astrocytoma because of the prominent fibrillary projections 🐱It is a GRADE II Ependymoma 🐱 Ependymal rosettes and pseudorosettes are scanty in tanytic ependymoma, which makes diagnosis challenging.

ANSWER- Question 5

Correct answer is C
💀Diagnosis is myxopapillary ependymoma
💀It is a Grade II Tumor according to latest WHO
💀It is common in adults and arises from the filum terminale
💀It is clinically indolent ( nor aggressive)
💀Stains for Mucin is positive.

Mucin stains positive in myxopapillary ependymoma
ANSWER – Question 6

Correct answer is D

Spinal ependymomas with NF2 mutation has a good prognosis

Summary of latest molecular updates in ependymoma

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