Ganglioglioma

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Ganglioglioma is a epilepsy-associated glioneuronal tumour with benign course. Not to be confused with ganglioneuroma.

Ganglioglioma
Diagnosis in short
LM DDx piloid gliosis, pilocytic astrocytoma, DNT
Stains PAS-D +ve (eosinophilic granular bodies)
IHC GFAP +ve, Synapto +ve
Gross usually temporal +/-cystic
Site brain - usu. supratentorial
Syndromes associated with epilepsy
Prevalence rare - esp. in children
Prognosis good (WHO Grade I)

General

  • Gangliolioma: Grade I WHO mixed neuronal-glial tumour (ICD-O code: 9505/1).
  • Anaplastic ganglioglioma: Grade III (ICD-O: 9505/3)
  • Rare (approx. 0.5% of all CNS tumors).
  • Usu. temporal lobe.
  • Predominantly children (mean age: 9 years).
  • Recognized as a cause of epilepsy.[1]
  • Favourable prognosis (survival rates up to 97%)
    • Anaplastic ganglioglioma have a recurrence risk of 69%-100% and median OS: 27months[2][3]

Imaging

  • Well-defined, T2-hyperintense.
  • Strong CM enhancement.
  • May contain cysts.
  • Associated with temporal lobe.

Gross

  • Circumscribed lesion.
  • Usu. contrast enhancing.
  • Solid, but intracortical cysts may be present.
  • Little mass effect.

Microscopic

Microscopic

Features:

  • Dysplastic neurons.
    • Out of regular architecture / heterotopic location.
    • Cytomegaly
    • Abnormal clustering
    • Binucleated (very occassionally).
    • Perimembranous Nissl aggreation.
  • Atypical glia (ie neoplastic).
  • Eosinophilic granular bodies (more common than rosenthal fibers).
  • Dystrophic calcification.
  • Prominent capillary network.
  • Lymphocytic cuffing.
  • May contain some reticulin.
  • Glial component may resemble:
    • Fibrillary astrocytoma.
    • Oligodendroglioma.
    • Pilocytic astrocytoma.

Anaplastic ganglioglioma:

  • Brisk mitotic activity
  • Necrosis

IHC

  • Neurons:
    • MAP2 +ve
    • Synaptophysin +ve
      • Perikaryal surface immunoreactivity for synaptophysin.[4]
      • This is however also seen in reactive changes.[5]
    • Neurofilament +ve
    • Chromogranin +ve
  • Glia:
    • CD34+/-ve
  • BRAF V600E +ve (approx. 25%, mainly ganglion cells).
  • MAP2: usu. absent.
  • MIB-1 (low, but resembles proliferative tumor component).

Molecular

  • BRAF V600E-mutated(approx. 25%).[6]
    • BRAF V600E antibody stains especially neuronal cells.[7]
  • IDH1/2 wt.
  • No 1p/19q codeletion.
  • Usu. Chr. 7 gain.
  • Rare cases with KIAA1459-BRAF fusion.[8]
    • DDx: consider pilocytic astrocytoma with ganglioglioma component. [9]
  • CDKN2A deletions, ATRX loss and TERT promotor mutations in anaplastic ganglioglioma.[10]
    • DD: consider anaplastic astrocytoma with piloid features (similiar molecular profile).[11]
  • H3F3A wildtype.
    • DDx: consider G34-mutant glioblastoma/NET (rare cases may contain dysplastic ganglion cells).[12]
  • Rare cases with co-occurrence of K27M mutation.[13]

Images

  • Lymphocytic cuffing in ganglioglioma (WC/jensflorian)

  • Calcification in ganglioglioma (WC/jensflorian)

  • CD34 immunostain in ganglioglioma (WC/jensflorian)

  • Pleomorphic ganglion cells in ganglioglioma (WC/jensflorian)

Prognosis

  • Very good (10-year OS: 97%)
  • Primary treatment: surgery.
    • Seizure free outcome: 81%.
    • Incomplete resection as major factor for persisting epilepsia.[14]

DDx:

  • DNT.
  • Oligodendroglioma.
  • PXA.
  • Desmoplastic infantile astrocytoma and ganglioglioma.
  • Cortical tuber.
  • Trapped cortical neurons in diffuse astrocytoma.
  • Papillary glioneuronal tumor.

See also

References

  1. ↑ Im, SH.; Chung, CK.; Cho, BK.; Lee, SK. (Mar 2002). "Supratentorial ganglioglioma and epilepsy: postoperative seizure outcome.". J Neurooncol 57 (1): 59-66. PMID 12125968.
  2. ↑ Terrier, LM.; Bauchet, L.; Rigau, V.; Amelot, A.; Zouaoui, S.; Filipiak, I.; Caille, A.; Almairac, F. et al. (05 2017). "Natural course and prognosis of anaplastic gangliogliomas: a multicenter retrospective study of 43 cases from the French Brain Tumor Database.". Neuro Oncol 19 (5): 678-688. doi:10.1093/neuonc/now186. PMID 28453747.
  3. ↑ Zanello, M.; Pages, M.; Tauziède-Espariat, A.; Saffroy, R.; Puget, S.; Lacroix, L.; Dezamis, E.; Devaux, B. et al. (Oct 2016). "Clinical, Imaging, Histopathological and Molecular Characterization of Anaplastic Ganglioglioma.". J Neuropathol Exp Neurol 75 (10): 971-980. doi:10.1093/jnen/nlw074. PMID 27539475.
  4. ↑ "Central nervous system gangliogliomas. Part 2: Clinical outcome". J. Neurosurg. 79 (6): 867–73. December 1993. doi:10.3171/jns.1993.79.6.0867. PMID 8246055.
  5. ↑ "Synaptophysin staining in normal brain: importance for diagnosis of ganglioglioma". Am. J. Surg. Pathol. 22 (5): 550–6. May 1998. doi:10.1097/00000478-199805000-00005. PMID 9591724.
  6. ↑ Schindler, G.; Capper, D.; Meyer, J.; Janzarik, W.; Omran, H.; Herold-Mende, C.; Schmieder, K.; Wesseling, P. et al. (Mar 2011). "Analysis of BRAF V600E mutation in 1,320 nervous system tumors reveals high mutation frequencies in pleomorphic xanthoastrocytoma, ganglioglioma and extra-cerebellar pilocytic astrocytoma.". Acta Neuropathol 121 (3): 397-405. doi:10.1007/s00401-011-0802-6. PMID 21274720.
  7. ↑ Koelsche, C.; Wöhrer, A.; Jeibmann, A.; Schittenhelm, J.; Schindler, G.; Preusser, M.; Lasitschka, F.; von Deimling, A. et al. (Jun 2013). "Mutant BRAF V600E protein in ganglioglioma is predominantly expressed by neuronal tumor cells.". Acta Neuropathol 125 (6): 891-900. doi:10.1007/s00401-013-1100-2. PMID 23435618.
  8. ↑ Pekmezci, M.; Villanueva-Meyer, JE.; Goode, B.; Van Ziffle, J.; Onodera, C.; Grenert, JP.; Bastian, BC.; Chamyan, G. et al. (06 2018). "The genetic landscape of ganglioglioma.". Acta Neuropathol Commun 6 (1): 47. doi:10.1186/s40478-018-0551-z. PMID 29880043.
  9. ↑ Mesturoux, L.; Durand, K.; Pommepuy, I.; Robert, S.; Caire, F.; Labrousse, F. (Aug 2016). "Molecular Analysis of Tumor Cell Components in Pilocytic Astrocytomas, Gangliogliomas, and Oligodendrogliomas.". Appl Immunohistochem Mol Morphol 24 (7): 496-500. doi:10.1097/PAI.0000000000000288. PMID 27389560.
  10. ↑ Zanello, M.; Pages, M.; Tauziède-Espariat, A.; Saffroy, R.; Puget, S.; Lacroix, L.; Dezamis, E.; Devaux, B. et al. (Oct 2016). "Clinical, Imaging, Histopathological and Molecular Characterization of Anaplastic Ganglioglioma.". J Neuropathol Exp Neurol 75 (10): 971-980. doi:10.1093/jnen/nlw074. PMID 27539475.
  11. ↑ Reinhardt, A.; Stichel, D.; Schrimpf, D.; Sahm, F.; Korshunov, A.; Reuss, DE.; Koelsche, C.; Huang, K. et al. (Aug 2018). "Anaplastic astrocytoma with piloid features, a novel molecular class of IDH wildtype glioma with recurrent MAPK pathway, CDKN2A/B and ATRX alterations.". Acta Neuropathol 136 (2): 273-291. doi:10.1007/s00401-018-1837-8. PMID 29564591.
  12. ↑ Andreiuolo, F.; Lisner, T.; Zlocha, J.; Kramm, C.; Koch, A.; Bison, B.; Gareton, A.; Zanello, M. et al. (May 2019). "H3F3A-G34R mutant high grade neuroepithelial neoplasms with glial and dysplastic ganglion cell components.". Acta Neuropathol Commun 7 (1): 78. doi:10.1186/s40478-019-0731-5. PMID 31109382.
  13. ↑ Pagès, M.; Beccaria, K.; Boddaert, N.; Saffroy, R.; Besnard, A.; Castel, D.; Fina, F.; Barets, D. et al. (Dec 2016). "Co-occurrence of histone H3 K27M and BRAF V600E mutations in paediatric midline grade I ganglioglioma.". Brain Pathol. doi:10.1111/bpa.12473. PMID 27984673.
  14. ↑ Devaux, B.; Chassoux, F.; Landré, E.; Turak, B.; Laurent, A.; Zanello, M.; Mellerio, C.; Varlet, P. (Jun 2017). "Surgery for dysembryoplastic neuroepithelial tumors and gangliogliomas in eloquent areas. Functional results and seizure control.". Neurochirurgie 63 (3): 227-234. doi:10.1016/j.neuchi.2016.10.009. PMID 28506485.
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