Difference between revisions of "Anaplastic astrocytoma"

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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.
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*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%.
==Molecular==
*TERT promotor mutations in 20-25%<ref>{{Cite journal  | last1 = Lee | first1 = Y. | last2 = Koh | first2 = J. | last3 = Kim | first3 = SI. | last4 = Won | first4 = JK. | last5 = Park | first5 = CK. | last6 = Choi | first6 = SH. | last7 = Park | first7 = SH. | title = The frequency and prognostic effect of TERT promoter mutation in diffuse gliomas. | journal = Acta Neuropathol Commun | volume = 5 | issue = 1 | pages = 62 | month = Aug | year = 2017 | doi = 10.1186/s40478-017-0465-1 | PMID = 28851427 }}</ref><ref>{{Cite journal  | last1 = Koelsche | first1 = C. | last2 = Sahm | first2 = F. | last3 = Capper | first3 = D. | last4 = Reuss | first4 = D. | last5 = Sturm | first5 = D. | last6 = Jones | first6 = DT. | last7 = Kool | first7 = M. | last8 = Northcott | first8 = PA. | last9 = Wiestler | first9 = B. | title = Distribution of TERT promoter mutations in pediatric and adult tumors of the nervous system. | journal = Acta Neuropathol | volume = 126 | issue = 6 | pages = 907-15 | month = Dec | year = 2013 | doi = 10.1007/s00401-013-1195-5 | PMID = 24154961 }}</ref>
*Approximately 80 % of IDH wildtype astrocytomas in fact represent underdiagnosed GBM.<ref>{{Cite journal  | last1 = Reuss | first1 = DE. | last2 = Kratz | first2 = A. | last3 = Sahm | first3 = F. | last4 = Capper | first4 = D. | last5 = Schrimpf | first5 = D. | last6 = Koelsche | first6 = C. | last7 = Hovestadt | first7 = V. | last8 = Bewerunge-Hudler | first8 = M. | last9 = Jones | first9 = DT. | title = Adult IDH wild type astrocytomas biologically and clinically resolve into other tumor entities. | journal = Acta Neuropathol | volume = 130 | issue = 3 | pages = 407-17 | month = Sep | year = 2015 | doi = 10.1007/s00401-015-1454-8 | PMID = 26087904 }}</ref>
==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.




Latest revision as of 09:24, 19 March 2018

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.

  • Marked mitotic activity in anaplastic astrocytoma (WC/jensflorian).

  • Marked nuclear pleomorphism (AFIP).

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%.


Molecular

  • TERT promotor mutations in 20-25%[6][7]
  • Approximately 80 % of IDH wildtype astrocytomas in fact represent underdiagnosed GBM.[8]

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.
  6. ↑ Lee, Y.; Koh, J.; Kim, SI.; Won, JK.; Park, CK.; Choi, SH.; Park, SH. (Aug 2017). "The frequency and prognostic effect of TERT promoter mutation in diffuse gliomas.". Acta Neuropathol Commun 5 (1): 62. doi:10.1186/s40478-017-0465-1. PMID 28851427.
  7. ↑ Koelsche, C.; Sahm, F.; Capper, D.; Reuss, D.; Sturm, D.; Jones, DT.; Kool, M.; Northcott, PA. et al. (Dec 2013). "Distribution of TERT promoter mutations in pediatric and adult tumors of the nervous system.". Acta Neuropathol 126 (6): 907-15. doi:10.1007/s00401-013-1195-5. PMID 24154961.
  8. ↑ Reuss, DE.; Kratz, A.; Sahm, F.; Capper, D.; Schrimpf, D.; Koelsche, C.; Hovestadt, V.; Bewerunge-Hudler, M. et al. (Sep 2015). "Adult IDH wild type astrocytomas biologically and clinically resolve into other tumor entities.". Acta Neuropathol 130 (3): 407-17. doi:10.1007/s00401-015-1454-8. PMID 26087904.
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