Astrocytoma

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An astrocytoma is a neoplasm derived from an astrocyte. Astrocytomas are common. This article is a brief introduction them. An overview of CNS tumours is found in the CNS tumours article.

Overview

Name Type Variants / Patterns Image
Diffuse Astrocytoma, WHO II diffuse protoplasmatic, fibrillar, gemistocytic
 
Anaplastic Astrocytoma, WHO III diffuse gliomatosis cerebri
 
Glioblastoma, WHO IV diffuse small cell, epitheloid/rhabdoid, with PNET componet, with granular cell component, giant cell, gliosarcoma
 
Pilocytic astrocytoma, WHO I circumscribed pilomyxoid astrocytoma, anaplastic pilocytic astrocytoma
 
Pleomorphic xanthoastrocytoma, WHO II (PXA) circumscribed anaplastic PXA
 
Subependymal giant cell astrocytoma, WHO I (SEGA) circumscribed SEGA in tuberous sclerosis
 

Common

Pilocytic astrocytoma

  • Benign, cystic, infratentorial.
  • Classic childhood tumor, surgically resectable.
  • Variant: Pilomyxoid astrocytoma
Main article: Pilocytic astrocytoma

Diffuse astrocytoma

  • Grade II astrocytic tumors typically seen in adults.
  • Usually show progression to glioblastoma.
Main article: Diffuse astrocytoma

Anaplastic astrocytoma

  • Grade III astrocytic tumors typically seen in adults.
  • Lacks endothelial proliferations and necrosis of glioblastoma.
Main article: Anaplastic astrocytoma

Glioblastoma

Main article: Glioblastoma

Uncommon

Subependymal giant cell astrocytoma

Main article: Subependymal giant cell astrocytoma

Pleomorphic xanthroastrocytoma (PXA)

  • Kids & young adults usually with good prognosis.
  • Large lipidized cells mimicking a malignant tumor
Main article: Pleomorphic xanthoastrocytoma

Gliomatosis cerebri

  • Extensively diffusely growing astrocytic neoplasm.
    • Currently considered a rare pattern of diffuse glioma infiltration.
    • Introduced in 1938 as a post-mortem diagnosis.[1]
  • More than 3 lobes have to be involved, us. bilateral (radiology required).
  • biologic behaviour corresponds to WHO III (ICD-O: 9381/3)
  • Based on presence / absence of a solid component authors propose two types:[2]
    • GC type 1: classic diffuse growth, without IDH1/2 mutation.
    • GC type 2: with a solid portion, mostly IDH1 mutant.
  • Genetic studies indicate strong overlap with diffuse astrocytic gliomas, oligodendrogliomas and glioblastoma.
    • It is likely that suggests that in the upcoming WHO classification gliomatosis is no longer a separate glioma entity.[3]

Diffuse midline glioma, H3 K27M mutant

  • High-grade astrocytic neoplasm associated with midline structures (thalamus, brain stem, spinal cord).
  • Mostly in children and adolescents.
  • Includes diffuse intrinsic pontine gliomas (DPIG).
  • Newly defined entity since WHO 2016 classification.[4]
  • Distinct biological and clinical group with poor prognosis.[5]
    • EGFR amplification is usu. absent.[6]
    • Tumors usu. have unmethylated MGMT promotor.[7]
  • MRI: May be or be not enhancing.
  • Histologic spectrum ranges from minimal hypercellularity to full-blown glioblastoma.

Note: Cases may also appear outside midline structures and in adult patients.[8]

  • Nuclear H3F3A K27M immunostaining in a diffuse glioma of the midline. (WC/jensflorian)

Gliosarcoma

General

  • Considered to be a variant of glioblastoma by WHO.[9]
  • Rare ~ 200 cases reported in the literature.[9]
  • Definition: gliosarcoma = glioblastoma + sarcomatous component.[10]
  • Usual location (like glioblastoma): temporal lobe.

Microscopic

Features:

  • Glioblastoma.
  • Sarcomatous component (one of the following):[9][10]
    • Fibroblastic.
    • Cartilaginous.
    • Osseous.
    • Smooth muscle.
    • Striated muscle.
    • Adipocyte.

Images

  • Gliosarcoma - elastica van Giesson. (WC)

www:

IHC

  • GFAP +ve -- astrocytic component.[11]
    • Spindle cell component -ve.[12]

Gliosarcoma with smooth muscle component (gliomyosarcoma):[13]

  • SMA +ve.
  • Factor VIII +ve.

Gliofibroma

  • Very rare indolent tumor in children [14]
  • Usually not dura-based (DD: Desmoplastic infantile astrocytoma)
  • Glial tumor with non-neoplastic fibromatous component.


See also

References

  1. ↑ SAMUEL NEVIN - GLIOMATOSIS CEREBRI, DOI: http://dx.doi.org/10.1093/brain/61.2.170 170-191 First published online: 1 June 1938
  2. ↑ Seiz, M.; Tuettenberg, J.; Meyer, J.; Essig, M.; Schmieder, K.; Mawrin, C.; von Deimling, A.; Hartmann, C. (Aug 2010). "Detection of IDH1 mutations in gliomatosis cerebri, but only in tumors with additional solid component: evidence for molecular subtypes.". Acta Neuropathol 120 (2): 261-7. doi:10.1007/s00401-010-0701-2. PMID 20514489.
  3. ↑ Herrlinger, U.; Jones, DT.; Glas, M.; Hattingen, E.; Gramatzki, D.; Stuplich, M.; Felsberg, J.; Bähr, O. et al. (Oct 2015). "Gliomatosis cerebri: no evidence for a separate brain tumor entity.". Acta Neuropathol. doi:10.1007/s00401-015-1495-z. PMID 26493382.
  4. ↑ 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.
  5. ↑ Khuong-Quang, DA.; Buczkowicz, P.; Rakopoulos, P.; Liu, XY.; Fontebasso, AM.; Bouffet, E.; Bartels, U.; Albrecht, S. et al. (Sep 2012). "K27M mutation in histone H3.3 defines clinically and biologically distinct subgroups of pediatric diffuse intrinsic pontine gliomas.". Acta Neuropathol 124 (3): 439-47. doi:10.1007/s00401-012-0998-0. PMID 22661320.
  6. ↑ Meyronet, D.; Esteban-Mader, M.; Bonnet, C.; Joly, MO.; Uro-Coste, E.; Amiel-Benouaich, A.; Forest, F.; Rousselot-Denis, C. et al. (Aug 2017). "Characteristics of H3 K27M-mutant gliomas in adults.". Neuro Oncol 19 (8): 1127-1134. doi:10.1093/neuonc/now274. PMID 28201752.
  7. ↑ Banan, R.; Christians, A.; Bartels, S.; Lehmann, U.; Hartmann, C. (12 2017). "Absence of MGMT promoter methylation in diffuse midline glioma, H3 K27M-mutant.". Acta Neuropathol Commun 5 (1): 98. doi:10.1186/s40478-017-0500-2. PMID 29246238.
  8. ↑ Nakata, S.; Nobusawa, S.; Yamazaki, T.; Osawa, T.; Horiguchi, K.; Hashiba, Y.; Yaoita, H.; Matsumura, N. et al. (Jul 2017). "Histone H3 K27M mutations in adult cerebellar high-grade gliomas.". Brain Tumor Pathol 34 (3): 113-119. doi:10.1007/s10014-017-0288-6. PMID 28547652.
  9. ↑ 9.0 9.1 9.2 Han SJ, Yang I, Tihan T, Prados MD, Parsa AT (February 2010). "Primary gliosarcoma: key clinical and pathologic distinctions from glioblastoma with implications as a unique oncologic entity". J. Neurooncol. 96 (3): 313–20. doi:10.1007/s11060-009-9973-6. PMC 2808523. PMID 19618114. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2808523/.
  10. ↑ 10.0 10.1 Ayadi L, Charfi S, Khabir A, et al. (March 2010). "[Cerebral gliosarcoma: clinico-pathologic study of 8 cases]" (in French). Tunis Med 88 (3): 142–6. PMID 20415184.
  11. ↑ Horiguchi, H.; Hirose, T.; Kannuki, S.; Nagahiro, S.; Sano, T. (Aug 1998). "Gliosarcoma: an immunohistochemical, ultrastructural and fluorescence in situ hybridization study.". Pathol Int 48 (8): 595-602. PMID 9736406.
  12. ↑ URL: http://path.upmc.edu/cases/case361.html. Accessed on: 15 January 2012.
  13. ↑ Khanna, M.; Siraj, F.; Chopra, P.; Bhalla, S.; Roy, S.. "Gliosarcoma with prominent smooth muscle component (gliomyosarcoma): a report of 10 cases.". Indian J Pathol Microbiol 54 (1): 51-4. doi:10.4103/0377-4929.77324. PMID 21393877.
  14. ↑ Deb, P.; Sarkar, C.; Garg, A.; Singh, VP.; Kale, SS.; Sharma, MC. (Feb 2006). "Intracranial gliofibroma mimicking a meningioma: a case report and review of literature.". Clin Neurol Neurosurg 108 (2): 178-86. doi:10.1016/j.clineuro.2004.11.021. PMID 16412839.
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