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]] | ||
Revision as of 03:45, 17 February 2014
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).[1]
- Commonly spread via cerebrospinal fluid (CSF).[2]
- May be detected in CSF cytopathology specimens.
Gross
- Location: cerebellum - key feature.
- Morphologically identical supratentorial tumours are called primitive neuroectodermal tumour (PNET).
Microscopic
Features:[3]
- Small round cell tumour.
- Homer-Wright rosettes:
- Rosette with a meshwork of fibers (neuropil) at the centre.[4]
DDx:
Images
- Medulloblastoma (ouhsc.edu).
- Medulloblastoma - several images (upmc.edu).
- 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:
Anaplastic variant
Features:
- Larger cells.
- Severe anaplasia.
- Polygonal cells.
See also
References
- ↑ Online 'Mendelian Inheritance in Man' (OMIM) 601309
- ↑ Lefkowitch, Jay H. (2006). Anatomic Pathology Board Review (1st ed.). Saunders. pp. 424 Q34. ISBN 978-1416025887.
- ↑ URL: http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm. Accessed on: 26 October 2010.
- ↑ Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
- ↑ Gulino A, Arcella A, Giangaspero F (November 2008). "Pathological and molecular heterogeneity of medulloblastoma". Curr Opin Oncol 20 (6): 668–75. doi:10.1097/CCO.0b013e32831369f4. PMID 18841049.
- ↑ Rutkowski S, von Hoff K, Emser A, et al. (November 2010). "Survival and Prognostic Factors of Early Childhood Medulloblastoma: An International Meta-Analysis". J Clin Oncol 28 (33): 4961–4968. doi:10.1200/JCO.2010.30.2299. PMID 20940197.