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
Astrocytoma whoII HE.jpg
Anaplastic Astrocytoma, WHO III diffuse gliomatosis cerebri
Anaplastic astrocytoma - very high mag.jpg
Glioblastoma, WHO IV diffuse small cell, epitheloid/rhabdoid, with PNET componet, with granular cell component, giant cell, gliosarcoma
Glioblastoma (1).jpg
Pilocytic astrocytoma, WHO I circumscribed pilomyxoid astrocytoma, anaplastic pilocytic astrocytoma
Rosenthal HE 40x.jpg
Pleomorphic xanthoastrocytoma, WHO II (PXA) circumscribed anaplastic PXA
PXA HE x20.jpg
Subependymal giant cell astrocytoma, WHO I (SEGA) circumscribed SEGA in tuberous sclerosis
SEGA HE.jpg

Common

Pilocytic astrocytoma

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

Diffuse astrocytoma

  • Grade II astrocytic tumors typically seen in adults.
  • Usually show progression to glioblastoma.

Anaplastic astrocytoma

  • Grade III astrocytic tumors typically seen in adults.
  • Lacks endothelial proliferations and necrosis of glioblastoma.

Glioblastoma

Uncommon

Subependymal giant cell astrocytoma

Pleomorphic xanthroastrocytoma (PXA)

  • Kids & young adults usually with good prognosis.
  • Large lipidized cells mimicking a malignant tumor

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]
  • MRI: May be or be not enhancing.
  • HIstologic spectrum ranges from minimal hypercellularity to full-blown glioblastoma.

Gliosarcoma

General

  • Considered to be a variant of glioblastoma by WHO.[6]
  • Rare ~ 200 cases reported in the literature.[6]
  • Definition: gliosarcoma = glioblastoma + sarcomatous component.[7]
  • Usual location (like glioblastoma): temporal lobe.

Microscopic

Features:

  • Glioblastoma.
  • Sarcomatous component (one of the following):[6][7]
    • Fibroblastic.
    • Cartilaginous.
    • Osseous.
    • Smooth muscle.
    • Striated muscle.
    • Adipocyte.

Images

www:

IHC

  • GFAP +ve -- astrocytic component.[8]
    • Spindle cell component -ve.[9]

Gliosarcoma with smooth muscle component (gliomyosarcoma):[10]

  • SMA +ve.
  • Factor VIII +ve.

Gliofibroma

  • Very rare indolent tumor in children [11]
  • 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. 6.0 6.1 6.2 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. //www.ncbi.nlm.nih.gov/pmc/articles/PMC2808523/.
  7. 7.0 7.1 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.
  8. 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.
  9. URL: http://path.upmc.edu/cases/case361.html. Accessed on: 15 January 2012.
  10. 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.
  11. 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.