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**Supratentorial and spinal metastases from initial medulloblastoma possible. |
==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]]. |
*Mild to moderate nuclear pleomorphism. |
*High mitotic count. |
*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> |
==IHC== |
* [[MAP2]] usu. +ve |
* Synaptophysin +ve (weak to strong) |
* NSE +ve/-ve |
* NF +ve/-ve |
* Chromogranin +ve/-ve |
* GFAP +ve/-ve (mostly along blood vessels) |
* Vimentin +ve |
* Nestin +ve |
* [[INI1]] retained (no loss) |
Genetic grouping with IHC:<ref>{{Cite journal | last1 = Pietsch | first1 = T. | last2 = Haberler | first2 = C. | title = Update on the integrated histopathological and genetic classification of medulloblastoma - a practical diagnostic guideline. | journal = Clin Neuropathol | volume = 35 | issue = 6 | pages = 344-352 ... |
* WNT: beta-catein: nuclear +ve, YAP1+ve, OTX2+ve. |
* SHH: YAP1+ve, OTX2-ve, p75+ve. |
* Non-WNT/Non-SHH: * SHH: YAP1-ve, OTX2+ve, p75+ve. |
DDx: |
*[[Small round cell tumours]]. |
** [[AT/RT]]. (INI1 loss) |
** CNS-embryonal tumour (aka CNS-[[PNET]]). |
** ETMR (LIN28)-positive. |
** Ewing Sarcoma family members. |
** Small cell glioblastoma (Olig2 +ve) - esp. after RT and longer latency. <ref>{{Cite journal | last1 = Phi | first1 = JH. | last2 = Park | first2 = AK. | last3 = Lee | first3 = S. | last4 = Choi | first4 = SA. | last5 = Baek | first5 = IP. | last6 = Kim | first6 = P. | last7 = Kim | first7 = EH. | last8 = Park | fir... |
** Anaplastic ependymoma. |
==Images== |
<gallery> |
Image: Cerebellar medulloblastoma (1) in adult.JPG | Medulloblastoma. (WC/KGH) |
Image: Cerebellar medulloblastoma (2) in adult.JPG | Medulloblastoma. (WC/KGH) |
File:Medulloblastoma with rosettes.jpg | Homer-Wright rosettes in a medulloblastoma. (WC/jensflorian) |
File:Medulloblastoma stratum moleculare x40.jpg | Cerebellar infiltrative growth. (WC/jensflorian) |
File:Desmoplastic nodular medulloblastoma.jpg | Demsomplastic nodular medullloblastoma. (WC/jensflorian) |
File:Anaplastic medulloblastoma x200.jpg | Anaplastic large cell medulloblastoma. (WC/jensflorian) |
File:AFIP405820M-MEDULLOBLASTOMA.jpg | Medulloblastoma cells smear. (AFIP) |
File:Medulloblastoma .jpg | Medulloblastoma in the cerebellum. (AFIP) |
File:Desmoplastic_medulloblastoma_reticulin_stain_pale_island.jpg | Reticulin stain highlighting "pale islands" in a desmoplastic medulloblastoma (WC/jensflorian) |
File:Desmoplastic_medulloblastom_MIB1_proliferation.jpg | MIB-1 immunostaining in desmoplastic medulloblastoma (WC/jensflorian) |
</gallery> |
Case: |
<gallery> |
File:Medulloblastoma_SHH_type_adult.jpg | Classic Medulloblastoma of the SHH type. (WC/jensflorian) |
File:Medulloblastoma nuclear moulding HE stain.jpg | Epitheloid ribboning and nuclear moulding of tumor cells. (WC/jensflorian) |
File:Medulloblastoma_geographic_necrosis.jpg | Areas of geographic necrosis. (WC/jensflorian) |
File:Medulloblastoma_MAP2_IHC.jpg | Partial [[MAP2]] immunoreactivity. (WC/jensflorian) |
</gallery> |
www: |
*[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== |
===Histologically defined=== |
*Classic medulloblastoma (~85% of all medulloblastomas). |
*Variants of medulloblastoma (~15% of all medulloblastomas together): |
*#Anaplastic / Large cell variant. |
*#Desmoplastic/nodular medulloblastoma (DNMB). |
*#Medulloblastoma with extensive nodularity (MBEN). |
====Classic variant==== |
*Densely packed embryonal cells. |
*Mitosis / apoptosis present. |
*Nodules of neurocytic differentiation without internodular reticulin. |
*Rarely spongioblastic pattern (ribboning). |
====Anaplastic variant==== |
Features: |
*Larger cells. |
*Severe anaplasia. |
*Polygonal cells. |
====Desmoplastic/nodular variant==== |
Features: |
*Nodular, reticulin-free zones (pale islands). |
**Mitotic activity is reduced. |
*Densely packed internodal, reticulin-positive tumor areas. |
*Absence of neuroblastic rosettes. |
*Neuronal markers (NeuN, Synaptophysin) can be +ve. |
===Genetically defined=== |
*WNT (brainstem-related, usu. CTNNB1 mutations, less common: CSNK2B, AXIN2, APC, approx. 10%).<ref>{{Cite journal | last1 = Wefers | first1 = AK. | last2 = Warmuth-Metz | first2 = M. | last3 = Pöschl | first3 = J. | last4 = von Bueren | first4 = AO. | last5 = Monoranu | first5 = CM. | last6 = Seelos | first6 = K. | la... |
** Usu. classic MB in histology. |
** Caution: Some WNT-activated MB may show additional subclonal SHH-mutations.<ref>{{Cite journal | last1 = Iorgulescu | first1 = JB. | last2 = Van Ziffle | first2 = J. | last3 = Stevers | first3 = M. | last4 = Grenert | first4 = JP. | last5 = Bastian | first5 = BC. | last6 = Chavez | first6 = L. | last7 = Stichel | f... |
** Peak incidence: 7-14 years (15% adults). |
** Beta-catenin nuclear +ve. |
** Excellent prognosis. |
*SHH (PTCH1 & SUFU in infant, PTCH1, SMO, Gli2, MYCN in non-infant, approx 30%).<ref>{{Cite journal | last1 = Neumann | first1 = JE. | last2 = Swartling | first2 = FJ. | last3 = Schüller | first3 = U. | title = Medulloblastoma: experimental models and reality. | journal = Acta Neuropathol | volume = | issue = | page... |
**Origin in the cerebellar granule neuron cell precursors. |
**Infantile and adult cases are biologically different, esp p53 mutant tumors are associated with poor outcome.<ref>{{Cite journal | last1 = Kool | first1 = M. | last2 = Jones | first2 = DT. | last3 = Jäger | first3 = N. | last4 = Northcott | first4 = PA. | last5 = Pugh | first5 = TJ. | last6 = Hovestadt | first6 = V.... |
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