
Subtitle: A case-based ependymoma quiz: a spinal tumour in NF2, perivascular pseudorosettes, tanycytic and myxopapillary patterns, IHC and WHO CNS5 molecular groups, with 10 MCQs and explanations.
Author: PathologyMCQ Editorial Board
Category: Neuropathology
Last Updated: October 2026
Read Time: 6 minutes
At-a-Glance
- Hallmarks: perivascular pseudorosettes and true ependymal rosettes; GFAP positive with dot-like EMA.
- Patterns: clear cell, papillary and tanycytic are histological patterns in WHO CNS5, not separate types.
- Myxopapillary ependymoma (filum terminale) is now CNS WHO grade 2.
- Molecular groups: ZFTA and YAP1 (supratentorial), PFA and PFB (posterior fossa), spinal and MYCN-amplified spinal.
- Spinal ependymomas are linked to NF2 and generally do well.
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The Case
A 22-year-old man with neurofibromatosis type 2 has a hyperintense intramedullary lesion on spinal MRI. The lesion is resected. Look at the images below, then answer the 10 MCQs.
Case Images



Test Yourself: 10 MCQs
Ependymoma case: 10 MCQs
Rosettes, patterns, myxopapillary and molecular groups, with instant explanations.
More practice: the CNS tumours MCQ bank โ
Answer Images
Open these after the quiz.



Quick Review
| Type | Key features | Grade / behaviour |
|---|---|---|
| Supratentorial, ZFTA fusion-positive | Children and adults; L1CAM and p65 positive | Grade 2 or 3; worse |
| Supratentorial, YAP1 fusion-positive | Infants; mostly boys | Grade 2 or 3; better |
| Posterior fossa, PFA | Infants; H3 K27me3 loss, EZHIP | Grade 2 or 3; worse |
| Posterior fossa, PFB | Adolescents and adults; H3 K27me3 retained | Grade 2 or 3; better |
| Spinal ependymoma | Adults; NF2 association | Grade 2 or 3; good |
| Spinal, MYCN-amplified | Young adults; early dissemination | Aggressive |
| Myxopapillary ependymoma | Filum terminale; myxoid perivascular cores | Grade 2 |
| Subependymoma | Fourth and lateral ventricles; clustered nuclei in fibrillary matrix | Grade 1 |
Full detail: ependymoma molecular types โ ยท WHO CNS5 updates โ
Exam Pearls
- Perivascular pseudorosettes + GFAP + dot-like EMA = ependymoma.
- Tanycytic pattern mimics pilocytic astrocytoma and schwannoma.
- Myxopapillary ependymoma is grade 2 in WHO CNS5.
- PFA (H3 K27me3 loss) and MYCN-amplified spinal ependymomas behave worst.
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References
- WHO Classification of Tumours Editorial Board. Central Nervous System Tumours. 5th ed. IARC; 2021.
- Pajtler KW, et al. Molecular classification of ependymal tumors across all CNS compartments, histopathological grades, and age groups. Cancer Cell. 2015;27:728โ43.
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