A rare case of oligodendroglioma with gangliocytic differentiation in a 31-year-old male: importance of genetic testing for IDH1/2


We report a rare case of oligodendroglioma with gangliocytic differentiation. A 31-year-old male without a past medical history was admitted with a sudden seizure. On magnetic resonance imaging, an approximately 7-cm mass with necrosis was noted in the right frontal lobe. The patient underwent surgical resection. On microscopy, two morphologically distinct areas with oligodendroglioma- and ganglioglioma-like features were found. Immunohistochemistry showed an absence of CD34 expression, whereas isocitrate dehydrogenase 1 (IDH1) was positive in the glial component. Moreover, IDH1 was positive in the ganglion-like cells as well as in the glial component. Subsequent 1p/19q co-deletion was confirmed by fluorescence in situ hybridization. Finally, a diagnosis of oligodendroglioma with gangliocytic differentiation was made. IDH1/2 molecular test would be basic and essential diagnostic tool in central nervous system tumor of young patients.

This is a preview of subscription content, log in to check access.

Fig. 1
Fig. 2
Fig. 3


  1. 1.

    Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, World Health O, International Agency for Research on C (2016) WHO classification of tumours of the central nervous system

  2. 2.

    Perry A, Scheithauer BW, Macaulay RJB, Raffel C, Roth KA, Kros JM (2002) Oligodendrogliomas with neurocytic differentiation. A report of 4 cases with diagnostic and histogenetic implications. J Neuropathol Exp Neurol 61(11):947–955. https://doi.org/10.1093/jnen/61.11.947

    Article  PubMed  Google Scholar 

  3. 3.

    Perry A, Burton SS, Fuller GN, Robinson CA, Palmer CA, Resch L, Bigio EH, Gujrati M, Rosenblum MK (2010) Oligodendroglial neoplasms with ganglioglioma-like maturation: a diagnostic pitfall. Acta Neuropathol 120(2):237–252

    Article  Google Scholar 

  4. 4.

    Slegers RJ, Blumcke I (2020) Low-grade developmental and epilepsy associated brain tumors: a critical update 2020. Acta Neuropathol Commun 8(1):27. https://doi.org/10.1186/s40478-020-00904-x

    Article  PubMed  PubMed Central  Google Scholar 

  5. 5.

    Daumas-Duport C, Varlet P, Bacha S, Beuvon F, Cervera-Pierot P, Chodkiewicz JP (1999) Dysembryoplastic neuroepithelial tumors: nonspecific histological forms—a study of 40 cases. J Neurooncol 41(3):267–280. https://doi.org/10.1023/a:1006193018140

    Article  PubMed  CAS  Google Scholar 

  6. 6.

    Stone TJ, Keeley A, Virasami A, Harkness W, Tisdall M, Izquierdo Delgado E, Gutteridge A, Brooks T, Kristiansen M, Chalker J, Wilkhu L, Mifsud W, Apps J, Thom M, Hubank M, Forshew T, Cross JH, Hargrave D, Ham J, Jacques TS (2018) Comprehensive molecular characterisation of epilepsy-associated glioneuronal tumours. Acta Neuropathol 135(1):115–129. https://doi.org/10.1007/s00401-017-1773-z

    Article  PubMed  CAS  Google Scholar 

  7. 7.

    Blümcke I, Giencke K, Wardelmann E, Beyenburg S, Kral T, Sarioglu N, Pietsch T, Wolf HK, Schramm J, Elger CE, Wiestler OD (1999) The CD34 epitope is expressed in neoplastic and malformative lesions associated with chronic, focal epilepsies. Acta Neuropathol 97(5):481–490. https://doi.org/10.1007/s004010051017

    Article  PubMed  Google Scholar 

  8. 8.

    Hirose T, Nobusawa S, Nakazato Y, Sasaki A (2013) A case of oligodendroglioma with prominent neuronal differentiation. Hum Pathol 44(10):2353–2359. https://doi.org/10.1016/j.humpath.2013.02.013

    Article  PubMed  Google Scholar 

  9. 9.

    Tanaka Y, Nobusawa S, Yagi S, Ikota H, Yokoo H, Nakazato Y (2012) Anaplastic oligodendroglioma with ganglioglioma-like maturation. Brain Tumor Pathol 29(4):221–228. https://doi.org/10.1007/s10014-011-0078-5

    Article  PubMed  Google Scholar 

  10. 10.

    Horbinski C, Kofler J, Yeaney G, Camelo-Piragua S, Venneti S, Louis DN, Perry A, Murdoch G, Nikiforova M (2011) Isocitrate dehydrogenase 1 analysis differentiates gangliogliomas from infiltrative gliomas. Brain Pathol 21(5):564–574. https://doi.org/10.1111/j.1750-3639.2011.00480.x

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  11. 11.

    Fujisawa H, Marukawa K, Hasegawa M, Tohma Y, Hayashi Y, Uchiyama N, Tachibana O, Yamashita J (2002) Genetic differences between neurocytoma and dysembryoplastic neuroepithelial tumor and oligodendroglial tumors. J Neurosurg 97(6):1350–1355. https://doi.org/10.3171/jns.2002.97.6.1350

    Article  PubMed  Google Scholar 

  12. 12.

    Bielle F, Ducray F, Mokhtari K, Dehais C, Adle-Biassette H, Carpentier C, Chanut A, Polivka M, Poggioli S, Rosenberg S, Giry M, Marie Y, Duyckaerts C, Sanson M, Figarella-Branger D, Idbaih A (2017) Tumor cells with neuronal intermediate progenitor features define a subgroup of 1p/19q co-deleted anaplastic gliomas. Brain Pathol 27(5):567–579. https://doi.org/10.1111/bpa.12434

    Article  PubMed  CAS  Google Scholar 

  13. 13.

    Williams BP, Read J, Price J (1991) The generation of neurons and oligodendrocytes from a common precursor cell. Neuron 7(4):685–693. https://doi.org/10.1016/0896-6273(91)90381-9

    Article  PubMed  CAS  Google Scholar 

  14. 14.

    Price J, Williams B, Grove E (1992) The generation of cellular diversity in the cerebral cortex. Brain Pathol 2(1):23–29

    PubMed  CAS  Google Scholar 

  15. 15.

    Patt S, Labrakakis C, Bernstein M, Weydt P, Cervos-Navarro J, Nisch G, Kettenmann H (1996) Neuron-like physiological properties of cells from human oligodendroglial tumors. Neuroscience 71(2):601–611. https://doi.org/10.1016/0306-4522(95)00468-8

    Article  PubMed  CAS  Google Scholar 

  16. 16.

    Vyberg M, Ulhøi BP, Teglbjærg PS (2007) Neuronal features of oligodendrogliomas - An ultrastructural and immunohistochemical study. Histopathology 50(7):887–896. https://doi.org/10.1111/j.1365-2559.2007.02686.x

    Article  PubMed  CAS  Google Scholar 

  17. 17.

    Tenenbaum L, Teugels E, Dogusan Z, Avellana-Adalid V, Hooghe-Peters EL (1996) Plastic phenotype of human oligodendroglial tumour cells in vitro. Neuropathol Appl Neurobiol 22(4):302–310. https://doi.org/10.1111/j.1365-2990.1996.tb01108.x

    Article  PubMed  CAS  Google Scholar 

  18. 18.

    Lu QR, Park JK, Noll E, Chan JA, Alberta J, Yuk D, Alzamora MG, Louis DN, Stiles CD, Rowitch DH, Black PM (2001) Oligodendrocyte lineage genes (OLIG) as molecular markers for human glial brain tumors. Proc Natl Acad Sci U S A 98(19):10851–10856. https://doi.org/10.1073/pnas.181340798

    Article  PubMed  PubMed Central  CAS  Google Scholar 

  19. 19.

    Vajtai I, Arnold M, Vassella E (2005) Oligodendroglioma with neurocytic differentiation and characteristic loss of heterozygosity on chromosomes 1p and 19q. Acta Neuropathol 110(5):520–522. https://doi.org/10.1007/s00401-005-1091-8

    Article  PubMed  Google Scholar 

Download references

Author information



Corresponding author

Correspondence to Se Hoon Kim.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

The current case was presented as an oral presentation at the 19th International Congress of Neuropathology in 2018, Tokyo.

About this article

Verify currency and authenticity via CrossMark

Cite this article

Park, J.H., Cha, Y.J., Chang, J.H. et al. A rare case of oligodendroglioma with gangliocytic differentiation in a 31-year-old male: importance of genetic testing for IDH1/2. Brain Tumor Pathol 37, 95–99 (2020). https://doi.org/10.1007/s10014-020-00368-w

Download citation


  • Oligodendroglioma
  • Ganglioglioma
  • Immunohistochemistry
  • Fluorescence in situ hybridization
  • Neurocytic differentiation