Difference between revisions of "Anaplastic astrocytoma"

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(to be continued)
(continued - molecular data is still missing)
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WHO 2016 categorization combines morphology and genetics into following groups:<ref>{{Cite journal  | last1 = Louis | first1 = DN. | last2 = Perry | first2 = A. | last3 = Reifenberger | first3 = G. | last4 = von Deimling | first4 = A. | last5 = Figarella-Branger | first5 = D. | last6 = Cavenee | first6 = WK. | last7 = Ohgaki | first7 = H. | last8 = Wiestler | first8 = OD. | last9 = Kleihues | first9 = P. | title = The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. | journal = Acta Neuropathol | volume = 131 | issue = 6 | pages = 803-20 | month = Jun | year = 2016 | doi = 10.1007/s00401-016-1545-1 | PMID = 27157931 }}</ref>
WHO 2016 categorization combines morphology and genetics into following groups:<ref>{{Cite journal  | last1 = Louis | first1 = DN. | last2 = Perry | first2 = A. | last3 = Reifenberger | first3 = G. | last4 = von Deimling | first4 = A. | last5 = Figarella-Branger | first5 = D. | last6 = Cavenee | first6 = WK. | last7 = Ohgaki | first7 = H. | last8 = Wiestler | first8 = OD. | last9 = Kleihues | first9 = P. | title = The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary. | journal = Acta Neuropathol | volume = 131 | issue = 6 | pages = 803-20 | month = Jun | year = 2016 | doi = 10.1007/s00401-016-1545-1 | PMID = 27157931 }}</ref>
*Anaplastic astrocytoma, IDH-mutant  (ICD-O: 9401/3).
*Anaplastic astrocytoma, IDH-mutant  (ICD-O: 9401/3).
*Anaplastic astrocytoma, IDH-wildtype (ICD-O: 9400/3).
*Anaplastic astrocytoma, IDH-wildtype (ICD-O: 9401/3).
*Anaplastic astrocytoma,NOS (ICD-O: 9400/3) - genetic data missing.
*Anaplastic astrocytoma,NOS (ICD-O: 9401/3) - genetic data missing.


==Radiology/Clinic==
==Radiology/Clinic==
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*The majority are contrast-enhanching, T2 bright.
*The majority are contrast-enhanching, T2 bright.


==Prognosis==
*Overall prognosis is rather poor (average survival 2-3 years).
*IDH-mutant tumors share a similiar prognosis to grade II IDH-mutant tumors.<ref>{{Cite journal  | last1 = Reuss | first1 = DE. | last2 = Mamatjan | first2 = Y. | last3 = Schrimpf | first3 = D. | last4 = Capper | first4 = D. | last5 = Hovestadt | first5 = V. | last6 = Kratz | first6 = A. | last7 = Sahm | first7 = F. | last8 = Koelsche | first8 = C. | last9 = Korshunov | first9 = A. | title = IDH mutant diffuse and anaplastic astrocytomas have similar age at presentation and little difference in survival: a grading problem for WHO. | journal = Acta Neuropathol | volume = 129 | issue = 6 | pages = 867-73 | month = Jun | year = 2015 | doi = 10.1007/s00401-015-1438-8 | PMID = 25962792 }}</ref>
*Anaplastic astrocytoma, IDH-wildtype perform worse than glioblastoma, IDH-mutant despite grading differences.<ref>{{Cite journal  | last1 = Hartmann | first1 = C. | last2 = Hentschel | first2 = B. | last3 = Wick | first3 = W. | last4 = Capper | first4 = D. | last5 = Felsberg | first5 = J. | last6 = Simon | first6 = M. | last7 = Westphal | first7 = M. | last8 = Schackert | first8 = G. | last9 = Meyermann | first9 = R. | title = Patients with IDH1 wild type anaplastic astrocytomas exhibit worse prognosis than IDH1-mutated glioblastomas, and IDH1 mutation status accounts for the unfavorable prognostic effect of higher age: implications for classification of gliomas. | journal = Acta Neuropathol | volume = 120 | issue = 6 | pages = 707-18 | month = Dec | year = 2010 | doi = 10.1007/s00401-010-0781-z | PMID = 21088844 }}</ref>
==Macroscopy==
==Macroscopy==
*No clear demarcation from white matter.
*No clear demarcation from white matter.
Line 21: Line 26:
*Softer consistency and opacity.  
*Softer consistency and opacity.  
*No  necrosis.
*No  necrosis.
==Histology==
Features: <ref name=AFIP2007>{{Ref AFIP2007|34}}</ref>
*Increased cellularity (compared to [[Diffuse Astrocytoma]]).
**Specimens with low cellularity but plenty mitoses are also considered anaplastic.
*Distinct nuclear atypia and  pleomorphism.
**May include multinucleated cells.
*Cytoplasm highly variable (even within the same tumour).
*Mitoses present (a single mitosis in a small specimen indicates a high-grade tumor).
*Microcystic spaces of the background (none to extensive).
*No necrosis, no vascular proliferations.
**Except radiation necrosis after pretreatment.
*Lymphocytic cuffing (mostly in gemistocytic type).
*Rosenthal fibers usu. absent.
<gallery>
File:Mitoses_astro_III.jpg | Marked mitotic activity in anaplastic astrocytoma (WC/jensflorian).
File:405551M-ANAPLASTIC_ASTROCYTOMA.jpg | Marked nuclear pleomorphism (AFIP).
</gallery>
==IHC==
*[[GFAP]]+ve.
*[[MAP2]]+ve (especially in cell processes).
*Vimentin+ve (often perinuclear).
*S-100+ve.
*MIB-1: usu. 5-10& (overlaps with grade II tumors).
*[[IDH-1]] (R132H)+ve in 60-70%.
**'Note:'' This antibody does not detect other rare IDH1/2 mutations.
*[[ATRX]] nuclear loss in 70%.
==DDx==
*[[Diffuse astrocytoma]] - absent or very low mitotic activity.
*Anaplastic [[Oligoastrocytoma]], NOS - esp. when genetic data on IDH and LOH 1p/19q are lacking.
*Anaplastic [[Oligodendroglioma]], when LOH 1p/19q is present.
*[[Glioblastoma]] - vascular proliferations and / or necrosis.





Revision as of 14:33, 19 May 2016

Anaplastic astrocytoma (AKA: high-grade astrocytoma) is a infiltrating neoplasm of the diffuse astrocytic and oligodendroglial tumor group occurring in the CNS white matter.

  • Most common grade III WHO glioma in adults (peaks between 40-50 years).
  • Approx 5% of all gliomas.[1]
  • Usually shows progression to glioblastoma sooner or later.

WHO 2016 categorization combines morphology and genetics into following groups:[2]

  • Anaplastic astrocytoma, IDH-mutant (ICD-O: 9401/3).
  • Anaplastic astrocytoma, IDH-wildtype (ICD-O: 9401/3).
  • Anaplastic astrocytoma,NOS (ICD-O: 9401/3) - genetic data missing.

Radiology/Clinic

  • Mass effect.
  • Seizures.
  • Neurologic decifit.
  • The majority are contrast-enhanching, T2 bright.

Prognosis

  • Overall prognosis is rather poor (average survival 2-3 years).
  • IDH-mutant tumors share a similiar prognosis to grade II IDH-mutant tumors.[3]
  • Anaplastic astrocytoma, IDH-wildtype perform worse than glioblastoma, IDH-mutant despite grading differences.[4]

Macroscopy

  • No clear demarcation from white matter.
  • Invaded structures may appear enlarged.
  • Softer consistency and opacity.
  • No necrosis.

Histology

Features: [5]

  • Increased cellularity (compared to Diffuse Astrocytoma).
    • Specimens with low cellularity but plenty mitoses are also considered anaplastic.
  • Distinct nuclear atypia and pleomorphism.
    • May include multinucleated cells.
  • Cytoplasm highly variable (even within the same tumour).
  • Mitoses present (a single mitosis in a small specimen indicates a high-grade tumor).
  • Microcystic spaces of the background (none to extensive).
  • No necrosis, no vascular proliferations.
    • Except radiation necrosis after pretreatment.
  • Lymphocytic cuffing (mostly in gemistocytic type).
  • Rosenthal fibers usu. absent.

IHC

  • GFAP+ve.
  • MAP2+ve (especially in cell processes).
  • Vimentin+ve (often perinuclear).
  • S-100+ve.
  • MIB-1: usu. 5-10& (overlaps with grade II tumors).
  • IDH-1 (R132H)+ve in 60-70%.
    • 'Note: This antibody does not detect other rare IDH1/2 mutations.
  • ATRX nuclear loss in 70%.

DDx


  1. redirect Neuropathology_tumours#Infiltrative_astrocytomas
  1. Ohgaki, H.; Kleihues, P. (Jun 2005). "Population-based studies on incidence, survival rates, and genetic alterations in astrocytic and oligodendroglial gliomas.". J Neuropathol Exp Neurol 64 (6): 479-89. PMID 15977639.
  2. Louis, DN.; Perry, A.; Reifenberger, G.; von Deimling, A.; Figarella-Branger, D.; Cavenee, WK.; Ohgaki, H.; Wiestler, OD. et al. (Jun 2016). "The 2016 World Health Organization Classification of Tumors of the Central Nervous System: a summary.". Acta Neuropathol 131 (6): 803-20. doi:10.1007/s00401-016-1545-1. PMID 27157931.
  3. Reuss, DE.; Mamatjan, Y.; Schrimpf, D.; Capper, D.; Hovestadt, V.; Kratz, A.; Sahm, F.; Koelsche, C. et al. (Jun 2015). "IDH mutant diffuse and anaplastic astrocytomas have similar age at presentation and little difference in survival: a grading problem for WHO.". Acta Neuropathol 129 (6): 867-73. doi:10.1007/s00401-015-1438-8. PMID 25962792.
  4. Hartmann, C.; Hentschel, B.; Wick, W.; Capper, D.; Felsberg, J.; Simon, M.; Westphal, M.; Schackert, G. et al. (Dec 2010). "Patients with IDH1 wild type anaplastic astrocytomas exhibit worse prognosis than IDH1-mutated glioblastomas, and IDH1 mutation status accounts for the unfavorable prognostic effect of higher age: implications for classification of gliomas.". Acta Neuropathol 120 (6): 707-18. doi:10.1007/s00401-010-0781-z. PMID 21088844.
  5. Burger, P.C.; Scheithauer, B.W. (2007). Tumors of the Central Nervous System (Afip Atlas of Tumor Pathology) (4th ed.). Washington: American Registry of Pathology. pp. 34. ISBN 1933477016.