Last Updated: March 27, 2020
- Well-marginated, usually benign intraventricular neuroepithelial tumor
- WHO grade II
- Higher grade atypical, anaplastic variant is rare
- Round cells with stippled nuclei are characteristic microscopic features
- Electron microscopy: synaptophysin-positive
- Affects all ages (commonly 3rd decade of life)
- No gender predilection
- Commonly presents with signs/symptoms of increased intracranial pressure secondary to obstructive hydrocephalus
- Headache, nausea, vomiting, and altered mental status
- Treatment: surgical resection; chemoradiation or radiosurgery for unresectable tumors
- Prognosis: recurrence uncommon following total resection; 5-year survival 90%
- Supratentorial, intraventricular mass with cystic or bubbly appearance
- Often located with lateral ventral frontal horn or body near the foramen of Monro
- Attachment to the septum pellucidum or lateral ventricular wall
- ± 3rd ventricular extension
- Frequently shows calcification
- Heterogeneous density intraventricular mass due to solid and cystic components
- ± Calcification
- Heterogeneous enhancement on contrast-enhanced CT
- T1WI: heterogeneous but predominantly isointense
- T2WI: usually hyperintense with cystic or bubbly appearance; ± flow voids
- FLAIR: heterogeneously hyperintense
- T2*/GRE/SWI: black signal blooming in foci of calcification
- T1WI+C: heterogeneous enhancement
- MRS: increased Cho, decreased NAA; glycine peak at 3.55
- MRI with contrast including coronal T2WI to better detect tumor attachment site
For more information, please see the corresponding chapter in Radiopaedia.
Contributor: Rachel Seltman, MD; Jacob A. Eitel, MD
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