Difference between revisions of "Medulloblastoma"

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(→‎Images: images desmoplastic medulloblastoma)
(IHC + DDx)
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*Homer-Wright [[rosette]]s:
*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>
**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)


DDx:
DDx:
*[[Small round cell tumours]].
*[[Small round cell tumours]].
** [[AT/RT]]
** [[AT/RT]] (INI1 loss)
** [[PNET]]
** CNS-[[PNET]]
** ETMR (LIN28)-positive
** Ewing Sarcoma family members
** Small cell glioblastoma


===Images===
==Images==
<gallery>
<gallery>
Image: Cerebellar medulloblastoma (1) in adult.JPG | Medulloblastoma. (WC/KGH)
Image: Cerebellar medulloblastoma (1) in adult.JPG | Medulloblastoma. (WC/KGH)
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File:Medulloblastoma nuclear moulding HE stain.jpg | Epitheloid ribboning and nuclear moulding of tumor cells. (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_geographic_necrosis.jpg | Areas of geographic necrosis. (WC/jensflorian)
File:Medulloblastoma_MAP2_IHC.jpg | Partial MAP2 immunoreactivity. (WC/jensflorian)
File:Medulloblastoma_MAP2_IHC.jpg | Partial [[MAP2]] immunoreactivity. (WC/jensflorian)
</gallery>
</gallery>


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*[http://path.upmc.edu/cases/case685.html Medulloblastoma with rhabdomyoblastic differentiation - several images (upmc.edu)].
*[http://path.upmc.edu/cases/case685.html Medulloblastoma with rhabdomyoblastic differentiation - several images (upmc.edu)].


===Subtypes===
==Subtypes==
*Classic medulloblastoma (~85% of all medulloblastomas).
*Classic medulloblastoma (~85% of all medulloblastomas).
*Variants of medulloblastoma (~15% of all medulloblastomas together):
*Variants of medulloblastoma (~15% of all medulloblastomas together):

Revision as of 09:10, 30 September 2015

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

Gross

  • Location: cerebellum - key feature.
    • Morphologically identical supratentorial tumours are called primitive neuroectodermal tumour (PNET).
    • Supratentorial and spinal metastases from initial tumor possible.

Microscopic

Features:[3]

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)

DDx:

Images

  • Medulloblastoma. (WC/KGH)

  • Medulloblastoma. (WC/KGH)

  • Homer-Wright rosettes in a medulloblastoma. (WC/jensflorian)

  • Cerebellar infiltrative growth. (WC/jensflorian)

  • Demsomplastic nodular medullloblastoma. (WC/jensflorian)

  • Anaplastic large cell medulloblastoma. (WC/jensflorian)

  • Medulloblastoma cells smear. (AFIP)

  • Medulloblastoma in the cerebellum. (AFIP)

  • Reticulin stain highlighting "pale islands" in a desmoplastic medulloblastoma (WC/jensflorian)

  • MIB-1 immunostaining in desmoplastic medulloblastoma (WC/jensflorian)

Case:

  • Classic Medulloblastoma of the SHH type. (WC/jensflorian)

  • Epitheloid ribboning and nuclear moulding of tumor cells. (WC/jensflorian)

  • Areas of geographic necrosis. (WC/jensflorian)

  • Partial MAP2 immunoreactivity. (WC/jensflorian)

www:

Subtypes

  • Classic medulloblastoma (~85% of all medulloblastomas).
  • Variants of medulloblastoma (~15% of all medulloblastomas together):
    1. Anaplastic variant.
    2. Large cell variant.
    3. Desmoplastic/nodular medulloblastoma (DNMB).
    4. Medulloblastoma with extensive nodularity (MBEN).

Notes:

  • Prognosis:[5][6] DNMB & MBEN > classic > anaplastic variant, large cell variant.

Anaplastic variant

Features:

  • Larger cells.
  • Severe anaplasia.
  • Polygonal cells.

See also

References

  1. ↑ Online 'Mendelian Inheritance in Man' (OMIM) 601309
  2. ↑ Lefkowitch, Jay H. (2006). Anatomic Pathology Board Review (1st ed.). Saunders. pp. 424 Q34. ISBN 978-1416025887.
  3. ↑ URL: http://moon.ouhsc.edu/kfung/jty1/neurotest/Q93-Ans.htm. Accessed on: 26 October 2010.
  4. ↑ Wippold FJ, Perry A (March 2006). "Neuropathology for the neuroradiologist: rosettes and pseudorosettes". AJNR Am J Neuroradiol 27 (3): 488–92. PMID 16551982.
  5. ↑ 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.
  6. ↑ 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.
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