CNS tumor
Neuronal and mixed neuronal-glial tumors
Central neurocytoma
Author: Nat Pernick, M.D.
Topic Completed: 1 January 2006
Revised: 2 January 2019, last major update January 2006
Copyright: (c) 2002-2017, PathologyOutlines.com, Inc.
PubMed Search: "Central neurocytoma" CNS
Table of Contents
Definition / general | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Positive stains | Negative stains | Electron microscopy description | Differential diagnosisCite this page: Pernick N. Central neurocytoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/cnstumorcentralneurocytoma.html. Accessed July 13th, 2020.
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
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
