Central neurocytomas may derive from neuroglial precursor cells with the potentiality of dual differentiation because there is some evidence for both glial and neuronal differentiation in some tumors 1) 2) 3) 4) 5) 6) 7).


Central neurocytomas demonstrate neuronal differentiation and histologically appear similar to oligodendrogliomas. This has historically has resulted in many tumours erroneously categorised. They lack co-deletion of 1p19q which is characteristic of oligodendroglioma. The cells are typically uniform and round with a salt and pepper appearance.

The tumor is composed of small round cells with neuronal differentiation.

The initial description classified them as WHO grade I lesions, however this was upgraded in 1993 to WHO grade II as it was recognised that at least some of these tumours exhibited more aggressive behaviour 10.

Markers

Purely neuronal origin is demonstrated positivity to neuronal markers such as:

synaptophysin

neuronal specific enolase

Variants

Ganglioneurocytoma: shows differentiation towards ganglion cells.


Neumann et al. analyzed histomorphology, clinical parameters, and global DNA methylation of tumors with the initial histological diagnoses of tanycytic (n = 12), clear cell (n = 14), or papillary ependymoma (n = 19). Forty percent of these tumors did not match to the epigenetic profile of ependymomas, using a previously published DNA methylation-based classifier for brain tumors. Instead, they were classified as low-grade glioma (n = 3), plexus tumor (n = 2), CNS high-grade neuroepithelial tumor with MN1 alteration (n = 2), papillary tumor of the pineal region (n = 2), neurocytoma (n = 1), or did not match to any known brain tumor methylation class (n = 8) 8).


1)
Vasiljevic A, François P, Loundou A, et al.: Prog- nostic factors in central neurocytomas: a multi- center study of 71 cases. Am J Surg Pathol 36: 220–227, 2012
2)
Ligon KL, Alberta JA, Kho AT, et al.: The oligoden- droglial lineage marker OLIG2 is universally expressed in diffuse gliomas. J Neuropathol Exp Neurol 63: 499–509, 2004
3)
Soylemezoglu F, Onder S, Tezel GG, Berker M: Neuronal nuclear antigen (NeuN): a new tool in the diagnosis of central neurocytoma. Pathol Res Pract 199: 463–468, 2003
4)
Söylemezoglu F, Scheithauer BW, Esteve J, Kleihues P: Atypical central neurocytoma. J Neuropathol Exp Neurol 56: 551–556, 1997
5)
Tsuchida T, Matsumoto M, Shirayama Y, Imahori T, Kasai H, Kawamoto K: Neuronal and glial charac- teristics of central neurocytoma: electron micro- scopical analysis of two cases. Acta Neuropathol 91: 573–577, 1996
6)
von Deimling A, Janzer R, Kleihues P, Wiestler OD: Patterns of differentiation in central neurocytoma. An immunohistochemical study of eleven biopsies. Acta Neuropathol 79: 473–479, 1990
7)
von Deimling A, Kleihues P, Saremaslani P, et al.: Histogenesis and differentiation potential of central neurocytomas. Lab Invest 64: 585–591, 1991
8)
Neumann JE, Spohn M, Obrecht D, Mynarek M, Thomas C, Hasselblatt M, Dorostkar MM, Wefers AK, Frank S, Monoranu CM, Koch A, Witt H, Kool M, Pajtler KW, Rutkowski S, Glatzel M, Schüller U. Molecular characterization of histopathological ependymoma variants. Acta Neuropathol. 2019 Nov 2. doi: 10.1007/s00401-019-02090-0. [Epub ahead of print] PubMed PMID: 31679042.
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