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Subependymal Giant Cell Astrocytoma (SEGA)

Subependymal Giant Cell Astrocytoma (SEGA)

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Subependymal Giant Cell Astrocytoma (SEGA)
  • Subependymal giant cell astrocytomas are benign, astrocytic tumors (like pilocytic astrocytoma).
  • They are almost exclusively found in pediatric patients with tuberous sclerosis.
  • Like subependymomas and colloid cysts, they often present with pathologic obstructive hydrocephalus.
    • Because they position themselves at/near the foramen of Monro, the hydrocephalus can be deadly.
  • On MRI, they have heterogenous signal intensity, demonstrate calcifications, and have marked contrast enhancement, which distinguishes them from subependymomas, which typically display homogenous signal intensity and lack contrast enhancement.
  • Note that the "candle gutterings" are sub-ependymal nodules found in tuberous sclerosis: they are smooth, round projections into the ventricles that look like drippings of wax along a candle.
References
  • Adesina, Adekunle M., Tarik Tihan, Christine E. Fuller, and Tina Young Poussaint. Atlas of Pediatric Brain Tumors. Springer, 2016.
Gray, Frangoise, Charles Duyckaerts, and Umberto De Girolami. Escourolle and Poirier’s Manual of Basic Neuropathology. OUP USA, 2013.
  • Louis, David N., Arie Perry, Guido Reifenberger, Andreas von Deimling, Dominique Figarella-Branger, Webster K. Cavenee, Hiroko Ohgaki, Otmar D. Wiestler, Paul Kleihues, and David W. Ellison. “The 2016 World Health Organization Classification of Tumors of the Central Nervous System: A Summary.” Acta Neuropathologica 131, no. 6 (June 1, 2016): 803–20. https://doi.org/10.1007/s00401-016-1545-1.
  • Molavi, Diana Weedman. The Practice of Surgical Pathology: A Beginner’s Guide to the Diagnostic Process. Springer Science & Business Media, 2008.
  • Orkin, Stuart H., David E. Fisher, A. Thomas Look, Samuel Lux, David Ginsburg, and David G. Nathan. Oncology of Infancy and Childhood E-Book. Elsevier Health Sciences, 2009.
  • “December 27, 2010 e-Pearl of the Week: ‘Candle Guttering’ Sign in Tuberous Sclerosis | Neurology.” Accessed August 6, 2018. http://n.neurology.org/epearls/20101227.