Subependymal giant cell astrocytoma

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Subependymal giant cell astrocytoma
Diagnosis in short

Subependymal giant cell astrocytoma H&E stain.

Synonyms SEGA
LM DDx ganglioglioma, pleomorphic xanthoastrocytoma, glioblastoma
IHC GFAP +ve
Site brain - usu. wall of ventricles

Prevalence rare - esp. in young adults
Prognosis good (WHO Grade I)


Subependymal giant cell astrocytoma, abbreviated SEGA, is a low-grade astrocytoma associated with tuberous sclerosis complex.

General

Gross/radiology

  • Well-demarcated.
  • Often projecting into a ventricle.
  • May be calcified
  • Circumscribed tumour.

Microscopic

Features:[2][3]

  • Giant cells with nuclear atypia ("bizarre cells", "ganglioid cells").
  • Glassy eosinophilic cytoplasm.
  • Elongated cells in a fibrillary background.
  • Abundant mast cells.[4]
  • Lymphocytic infiltrates.
  • Endothelial proliferations and/or necrosis are not a sign of malignancy.

Images

www:

IHC

Features:[3][5]

  • GFAP +ve. (50%)
  • Vimentin +ve. (100%)
  • S100 +ve. (100%)
  • Neurofilament +/-ve (ganglionic component).
  • Synaptophysin +/-ve (ganglionic component)..
  • TTF-1 (7 out of 7).[6]
  • MIB-1 usu. low (1-5%).

See also

References

  1. Grajkowska, W.; Kotulska, K.; Jurkiewicz, E.; Roszkowski, M.; Daszkiewicz, P.; Jóźwiak, S.; Matyja, E. (2011). "Subependymal giant cell astrocytomas with atypical histological features mimicking malignant gliomas.". Folia Neuropathol 49 (1): 39-46. PMID 21455842.
  2. 2.0 2.1 URL: http://path.upmc.edu/cases/case179.html. Accessed on: 29 July 2011.
  3. 3.0 3.1 Taraszewska, A.; Kroh, H.; Majchrowski, A. (1997). "Subependymal giant cell astrocytoma: clinical, histologic and immunohistochemical characteristic of 3 cases.". Folia Neuropathol 35 (3): 181-6. PMID 9595853.
  4. 4.0 4.1 URL: http://path.upmc.edu/cases/case179/micro.html. Accessed on: 8 January 2012.
  5. Hirose, T.; Scheithauer, BW.; Lopes, MB.; Gerber, HA.; Altermatt, HJ.; Hukee, MJ.; VandenBerg, SR.; Charlesworth, JC. (1995). "Tuber and subependymal giant cell astrocytoma associated with tuberous sclerosis: an immunohistochemical, ultrastructural, and immunoelectron and microscopic study.". Acta Neuropathol 90 (4): 387-99. PMID 8546029.
  6. Hewer, E.; Vajtai, I.. "Consistent nuclear expression of thyroid transcription factor 1 in subependymal giant cell astrocytomas suggests lineage-restricted histogenesis.". Clin Neuropathol 34 (3): 128-31. doi:10.5414/NP300818. PMID 25669749.