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Ganglioglioma and Gangliocytoma

Ganglioglioma and Gangliocytoma
  • Neuroepithelial tumors
    • Ganglioglioma: comprises a mixed population of mature neurons ("ganglio-") and glial cells (-"glioma"). Thus, immunochemistry will stain the neuronal component (synaptophysin, chromogranin, neurofilament, etc...) and the glial component (GFAP).
    • Gangliocytoma: purely comprises mature neurons.
    • Because of their neuronal origin, these tumors stain neuronal markers (synaptophysin, MAP-2, neurofilament, NeuN, etc...).
  • Classically present as a temporal lobe tumor in patients < 20 yo with a long-standing history of epilepsy.
    • Typically these are benign, slow-growing tumors, thus they can have long-standing symptoms before they are identified.
  • On radiographic imaging, they are classically cystic with a mural nodule (think: pilocytic astrocytoma) but can also be a circumscribed/solid variably enhancing mass that expands the gyrus and causes thinning of the overlying bone.
  • On histopathology, there is characteristically a fibrillary background with abnormal ganglion cells but other features are also common, including, notably: eosiniphilic granular bodies (commonly seen in pilocytic astrocytoma).
  • Ganglioglioma is different than dysembryoplastic neuroepithelial tumor (DNET) and desmoplastic infantile astrocytoma and ganglioglioma.
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.
  • Tonn, Jörg-Christian, Manfred Westphal, and J. T. Rutka. Oncology of CNS Tumors. Springer Science & Business Media, 2010.
  • Yachnis, Anthony T., and Marie L. Rivera-Zengotita. Neuropathology E-Book: A Volume in the High Yield Pathology Series. Elsevier Health Sciences, 2012.

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