Central neurocytoma

CNS tumor
Neuronal and mixed neuronal - glial tumors
Central neurocytoma

Author: Nat Pernick, M.D. (see Authors page)

Revised: 29 August 2017, last major update January 2006

Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.

PubMed Search: "Central neurocytoma" CNS

Table of Contents

Cite this page: Pernick, N. Central neurocytoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorcentralneurocytoma.html. Accessed September 22nd, 2017.

Definition / general

First described in 1982 (Acta Neuropathol 1982;56:151)
Grows from foramen of Monro or septum pellucidum into lateral or third ventricle; often calcified
Most common neoplasm of septum pellucidum in young adults but still < 1% of all CNS tumors
Often presents with increased intracranial pressure due to obstructive hydrocephalus
Similar tumors within CNS parenchyma are called extraventricular neurocytoma
Rare before age 10 years

Case reports

11 year old girl with headaches (University of Pittsburgh: Frontal Lobe Mass)
17 year old girl with ataxia and an intraventricular mass within lateral ventricle (Case of the Week #119)
19 year old woman with malignant tumor (Pathol Oncol Res 1999;5:155)
51 year old man with sudden death due to acute hemorrhage (Am J Forensic Med Pathol 1999;20:180)
Pigmented tumor due to lipofuscin and neuromelanin (Am J Surg Pathol 1999;23:1136)

Treatment

Good prognosis after complete surgical excision or gamma knife radiosurgery (Cancer 2007;110:2276) although up to 1/3 recur or progress (Int J Radiat Oncol Biol Phys 2007;67:1145)
Recurrence associated with incomplete excision or the presence of necrosis, mitotic activity / increased proliferation rates or microvascular proliferation (Cancer 2000;89:1111, Clin Neurol Neurosurg 2008;110:129)

Gross description

Well demarcated from surrounding tissue
Gray, friable, may be gritty due to microcalcifications

Gross images

Images hosted on other servers:

Ventricular mass

Microscopic (histologic) description

Well developed neuronal features - monotonous bland cells with modest cytoplasm, often empty appearing "halo" resembling oligodendroglioma, salt and pepper chromatin, embedded in eosinophilic fibrillar matrix with rare Homer Wright rosettes and ganglion cells
Usually no infiltrating margin, no / rare necrosis, no endothelial proliferation and no / rare mitotic figures

Microscopic (histologic) images

Images hosted on PathOut server:

Case of the week #119:

Various images

Images hosted on other servers:

Sheets of nonpleomorphic cells with modest cytoplasm and neurophil

Contributed by Dr. Eman Abdelzaher: central neurocytoma with ganglionic differentiation (ganglioneurocytoma)

Various images

Cytology description

Cellular; monotonous round cells with ill defined cytoplasm, oval nuclei, finely granular chromatin and micronucleoli
Background fibrillar matrix; variable histiocytic giant cells with hemosiderin (Acta Cytol 2004;48:194)

Positive stains

Synaptophysin, NeuN (strong, Histopathology 2006;48:438) and neuron specific enolase

Negative stains

GFAP (reactive astrocytes are GFAP+), Ki67 ( < 2%)

Electron microscopy description

Microtubules, 100 - 200 nm dense core vesicles, clear vesicles and neuritic type processes
Usually no intermediate filaments

Differential diagnosis

Clear cell ependymoma: negative for neuronal markers, strongly positive for GFAP, vimentin and EMA
Glioma: no / few halo cells, no / focal neuronal features and strongly GFAP+
Oligodendroglioma: no prominent neuronal features, negative or focal for neuronal markers