Difference between revisions of "Medulloblastoma"

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(→‎Subtypes: SHH subgroups)
(→‎Prognosis: molecular consensus)
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==Prognosis==
==Prognosis==
*Prognosis based on histology:<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><ref>{{Cite journal  | last1 = Rutkowski | first1 = S. | last2 = Bode | first2 = U. | last3 = Deinlein | first3 = F. | last4 = Ottensmeier | first4 = H. | last5 = Warmuth-Metz | first5 = M. | last6 = Soerensen | first6 = N. | last7 = Graf | first7 = N. | last8 = Emser | first8 = A. | last9 = Pietsch | first9 = T. | title = Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. | journal = N Engl J Med | volume = 352 | issue = 10 | pages = 978-86 | month = Mar | year = 2005 | doi = 10.1056/NEJMoa042176 | PMID = 15758008 }}
*Prognosis based on histology:<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><ref>{{Cite journal  | last1 = Rutkowski | first1 = S. | last2 = Bode | first2 = U. | last3 = Deinlein | first3 = F. | last4 = Ottensmeier | first4 = H. | last5 = Warmuth-Metz | first5 = M. | last6 = Soerensen | first6 = N. | last7 = Graf | first7 = N. | last8 = Emser | first8 = A. | last9 = Pietsch | first9 = T. | title = Treatment of early childhood medulloblastoma by postoperative chemotherapy alone. | journal = N Engl J Med | volume = 352 | issue = 10 | pages = 978-86 | month = Mar | year = 2005 | doi = 10.1056/NEJMoa042176 | PMID = 15758008 }}
</ref> DNMB & MBEN > classic > anaplastic variant, large cell variant.
</ref> DNMB & MBEN > classic > anaplastic & large cell variant.
*Prognosis based on genetics:<ref>{{Cite journal  | last1 = Ramaswamy | first1 = V. | last2 = Remke | first2 = M. | last3 = Bouffet | first3 = E. | last4 = Bailey | first4 = S. | last5 = Clifford | first5 = SC. | last6 = Doz | first6 = F. | last7 = Kool | first7 = M. | last8 = Dufour | first8 = C. | last9 = Vassal | first9 = G. | title = Risk stratification of childhood medulloblastoma in the molecular era: the current consensus. | journal = Acta Neuropathol | volume = 131 | issue = 6 | pages = 821-31 | month = Jun | year = 2016 | doi = 10.1007/s00401-016-1569-6 | PMID = 27040285 }}</ref> WNT > SHH (without Tp53 mut) > Group 4 > Group 3 > SHH (with Tp53 mut).


==See also==
==See also==

Revision as of 14:31, 21 September 2017

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

Histologically defined

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

Anaplastic variant

Features:

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

Genetically defined

  • WNT
  • SHH
    • infantile and adult cases are biologically different, esp p53 mutant tumors are associated with poor outcome.[5]
  • Group 3
  • Group 4

Prognosis

  • Prognosis based on histology:[6][7][8] DNMB & MBEN > classic > anaplastic & large cell variant.
  • Prognosis based on genetics:[9] WNT > SHH (without Tp53 mut) > Group 4 > Group 3 > SHH (with Tp53 mut).

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. ↑ Kool, M.; Jones, DT.; Jäger, N.; Northcott, PA.; Pugh, TJ.; Hovestadt, V.; Piro, RM.; Esparza, LA. et al. (Mar 2014). "Genome sequencing of SHH medulloblastoma predicts genotype-related response to smoothened inhibition.". Cancer Cell 25 (3): 393-405. doi:10.1016/j.ccr.2014.02.004. PMID 24651015.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ Rutkowski, S.; Bode, U.; Deinlein, F.; Ottensmeier, H.; Warmuth-Metz, M.; Soerensen, N.; Graf, N.; Emser, A. et al. (Mar 2005). "Treatment of early childhood medulloblastoma by postoperative chemotherapy alone.". N Engl J Med 352 (10): 978-86. doi:10.1056/NEJMoa042176. PMID 15758008.
  9. ↑ Ramaswamy, V.; Remke, M.; Bouffet, E.; Bailey, S.; Clifford, SC.; Doz, F.; Kool, M.; Dufour, C. et al. (Jun 2016). "Risk stratification of childhood medulloblastoma in the molecular era: the current consensus.". Acta Neuropathol 131 (6): 821-31. doi:10.1007/s00401-016-1569-6. PMID 27040285.
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