Medulloblastoma pathophysiology
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2]
Overview
Pathogenesis
- Medulloblastoma arises from cerebellar stem cells, which are normally involved in the anatomical development of the cerebellum and other posterior fossa structures.[1]
- Medulloblastoma is usually located at the infratentorial region, where it forms a mass between the brain stem and the cerebellum in the vicinity of the fourth ventricle.[1]
- Medulloblastoma is an invasive and rapidly growing tumor.[1]
- Unlike most brain tumors, medulloblastoma may spread through the cerebrospinal fluid (CSF) and frequently metastasize to different organs of the body.[1]
- Medulloblastoma is often associated with gorlin syndrome and turcot syndrome.[1]
Genetics
- Development of medulloblastoma is the result of multiple genetic mutations.[1]
- Genes involved in the pathogenesis of medulloblastoma include:[1]
- CTNNB1 gene on chromosome 3
- PTCH1 gene located on chromosome 9
- MLL2 gene located on chromosome 12
- SMARCA4 gene located on chromosome 19
- DDX3X gene located on X chromosome
- CTDNEP1 gene located on chromosome 17
- KDM6A gene located on X chromosome
- TBR1 gene located on chromosome 2
Gross Pathology
- On gross pathology, a pink, solid, and well circumscribed mass is a characteristic finding of medulloblastoma.[1]
- The following image demonstrates the gross pathology observed in medulloblastoma:
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Image courtesy of Dr Frank Gaillard Radiopaedia(original file "here"). Creative Commons BY-SA-NC
Mircoscopic Pathology
- On microscopic histopathological analysis, round tumor cells, elevated mitotic rate, increased nucleus:cytoplasm ration, and presence of Homer-Wright rosettes are characteristic findings of medulloblastoma.
- Shown below is a series of microscopic images observed in medulloblastoma:
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Medulloblastoma smear[2]
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Medulloblastoma smear [2]
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Medulloblastoma smear demonstrating Homer-Wright rosettes[2]
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Medulloblastoma smear demonstrating cerebellar infiltrative growth[2]
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Anaplastic large cell medulloblastoma smear[2]
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Medulloblastoma smear[2]
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Desmoplastic medulloblastoma on MIB-1 immunostaining[2]
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Medulloblastoma smear demonstrating partial MAP2 immunoreactivity[2]
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 1.7 1.8 Medulloblastoma. Wikipedia(2015) https://en.wikipedia.org/wiki/Medulloblastoma Accessed on September 2015
- ↑ 2.00 2.01 2.02 2.03 2.04 2.05 2.06 2.07 2.08 2.09 2.10 2.11 Medulloblastoma. Librepathology(2015) http://librepathology.org/wiki/index.php/Medulloblastoma Accessed on September, 28th 2015