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Difference between revisions of "Medulloblastoma"
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'''Medulloblastoma''' is a [[malignant]] [[small round cell tumour]] that is found in the [[cerebellum]]. | |||
Morphologically identical supratentorial tumours are called ''[[primitive neuroectodermal tumour]]'' (PNET). | |||
==General== | |||
*Mostly paediatric population. | |||
*May be seen as a component of [[nevoid basal cell carcinoma syndrome]] (NBCCS). | |||
**Gene: ''patched'' (abbreviated ''PTCH1'').<ref name=omim601309>{{OMIM|601309}}</ref> | |||
*Commonly spread via cerebrospinal fluid (CSF).<ref>{{Ref APBR|424 Q34}}</ref> | |||
**May be detected in [[CSF cytopathology]] specimens. | |||
==Gross== | |||
*Location: cerebellum - '''key feature'''. | |||
**Morphologically identical supratentorial tumours are called ''[[primitive neuroectodermal tumour]]'' (PNET). | |||
==Microscopic== | |||
Features:<ref>URL: [http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm]. Accessed on: 26 October 2010.</ref> | |||
*[[Small round cell tumour]]. | |||
*Homer-Wright [[rosette]]s: | |||
**Rosette with a meshwork of fibers (neuropil) at the centre.<ref>{{cite journal |author=Wippold FJ, Perry A |title=Neuropathology for the neuroradiologist: rosettes and pseudorosettes |journal=AJNR Am J Neuroradiol |volume=27 |issue=3 |pages=488–92 |year=2006 |month=March |pmid=16551982 |doi= |url=}}</ref> | |||
DDx: | |||
*[[Small round cell tumours]]. | |||
===Images=== | |||
*[http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm Medulloblastoma (ouhsc.edu)]. | |||
*[http://path.upmc.edu/cases/case667.html Medulloblastoma - several images (upmc.edu)]. | |||
*[http://path.upmc.edu/cases/case685.html Medulloblastoma with rhabdomyoblastic differentiation - several images (upmc.edu)]. | |||
===Subtypes=== | |||
*Classic medulloblastoma (~85% of all medulloblastomas). | |||
*Variants of medulloblastoma (~15% of all medulloblastomas together): | |||
*#Anaplastic variant. | |||
*#Large cell variant. | |||
*#Desmoplastic/nodular medulloblastoma (DNMB). | |||
*#Medulloblastoma with extensive nodularity (MBEN). | |||
Notes: | |||
*Prognosis:<ref name=pmid18841049>{{cite journal |author=Gulino A, Arcella A, Giangaspero F |title=Pathological and molecular heterogeneity of medulloblastoma |journal=Curr Opin Oncol |volume=20 |issue=6 |pages=668–75 |year=2008 |month=November |pmid=18841049 |doi=10.1097/CCO.0b013e32831369f4 |url=}}</ref><ref name=pmid20940197>{{cite journal |author=Rutkowski S, von Hoff K, Emser A, ''et al.'' |title=Survival and Prognostic Factors of Early Childhood Medulloblastoma: An International Meta-Analysis |journal=J Clin Oncol |volume=28 |issue=33 |pages=4961–4968 |year=2010 |month=November |pmid=20940197 |doi=10.1200/JCO.2010.30.2299 |url=}}</ref> DNMB & MBEN > classic > anaplastic variant, large cell variant. | |||
====Anaplastic variant==== | |||
Features: | |||
*Larger cells. | |||
*Severe anaplasia. | |||
*Polygonal cells. | |||
==See also== | |||
*[[Neuropathology tumours]]. | |||
*[[Small round cell tumours]]. | |||
==References== | |||
{{Reflist|2}} | |||
[[Category:Neuropathology tumours]] | |||
[[Category:Diagnosis]] | [[Category:Diagnosis]] | ||