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tanicytic ependymoma
corresponds to WHO grade II.
Etiology:
- considered to arise from ependymal cells that line the ventricles & from ependymal rests
Epidemiology:
1) 2% of brain tumors
2) ~10% of childhood brain tumors are ependymomas
3) most ependymomas occur in children
- adults are generally 30-40 years of age [4]
Pathology:
1) most commonly occurs in the spinal canal, especially in the lumbosacral region, arising from the filum terminale
2) in children may occur in 4th ventricle
3) metastasis may occur via CSF pathways
Microscopic Pathology:
- moderately cellular
- relatively uniform cells
- low mitotic activity
- perivascular pseudorosettes (nuclei free spaces around blood vessels)
- ependymal rosettes
Genetics:
- may be entirely epigenetic [5]
Radiology:
- CT or MRI scans reveal uniformly enhancing lesions in posteroir fossa or spinal cord well demarcated from adjacent neural tissue
- contrast enhancement
- calcification
- +/- hydrocephalis [4]
Management:
1) surgical excision
2) post-operative radiation for residual tumor
3) stereotaxic radiosurgery with gamma knife
Prognosis:
- 5 year survival is > 80%
- median survival is 15-20 years [4]
Comparative biology:
- metformin slows tumor growth & even shrinks posterior fossa ependymomas & extends lifespan in some mice [6]
Database Correlations
OMIM 131445
References
- Harrison's Principles of Internal Medicine, 14th ed.
Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 2398
- Harrison's Online, Chapter 370, McGraw-Hill, 2002
- WHO Classification Tumours of the Nervous System.
Kleihues & Cavenee eds. IARC Press 2000
- Medical Knowledge Self Assessment Program (MKSAP) 16
American College of Physicians, Philadelphia 2012
- Mack SC, Witt H, Piro RM,
Epigenomic alterations define lethal CIMP-positive ependymomas
of infancy.
Nature. 2014 Feb 27;506(7489):445-50.
PMID: 24553142 Free PMC Article
- Rapaport L
Common Diabetes Drug Shrinks Brain Tumors in Mice.
Medscape. November 03, 2021
https://www.medscape.com/viewarticle/962161
- Panwalkar P, Tamrazi B, Dang D et al
Targeting integrated epigenetic and metabolic pathways in lethal
childhood PFA ependymomas.
Sci Transl Med. 2021 Oct 13;13(615):eabf7860
PMID: 34613815
https://www.science.org/doi/10.1126/scitranslmed.abc0497