ependymoma
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Introduction
corresponds to WHO grade II.
Etiology
- considered to arise from ependymal cells that line the ventricles & from ependymal rests
Epidemiology
- 2% of brain tumors
- ~10% of childhood brain tumors are ependymomas
- most ependymomas occur in children
- adults are generally 30-40 years of age[4]
Pathology
- most commonly occurs in the spinal canal, especially in the lumbosacral region, arising from the filum terminale
- in children may occur in 4th ventricle
- 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
- surgical excision
- post-operative radiation for residual tumor
- stereotaxic radiosurgery with gamma knife
Prognosis:
Comparative biology
- metformin slows tumor growth & even shrinks posterior fossa ependymomas & extends lifespan in some mice[6]
More general terms
More specific terms
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
- ↑ 4.0 4.1 4.2 4.3 Medical Knowledge Self Assessment Program (MKSAP) 16 American College of Physicians, Philadelphia 2012
- ↑ 5.0 5.1 Mack SC, Witt H, Piro RM, Epigenomic alterations define lethal CIMP-positive ependymomas of infancy. Nature. 2014 Feb 27;506(7489):445-50. PMID: https://www.ncbi.nlm.nih.gov/pubmed/24553142 Free PMC Article
- ↑ 6.0 6.1 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: https://www.ncbi.nlm.nih.gov/pubmed/34613815 https://www.science.org/doi/10.1126/scitranslmed.abc0497
Patient information
ependymoma patient information