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General
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• 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

Radiologic images
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Intraventricular tumor

Case reports
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• 11 year old girl with headaches (University of Pittsburgh Case 74)
• 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)
• 32 year old woman with ventricular tumor (University of Oklahoma Case 307-1)
• 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
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• 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
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• Well demarcated from surrounding tissue
• Gray, friable, may be gritty due to microcalcifications

Gross images
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Ventricular mass

Micro description
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• 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

Micro images
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Sheets of non-pleomorphic cells with modest cytoplasm and neurophil

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

Various images

Case of the week #119:

     

Various images

   

Various images

Cytology description
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• 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)

Cytology images
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Monotonous round tumor cells with minimal cytoplasm
and fine chromatin, with reactive astrocytes and fibrillar background

Positive stains
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• Synaptophysin, Neu-N (strong, Histopathology 2006;48:438) and neuron specific enolase

Negative stains
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• GFAP (reactive astrocytes are GFAP+), Ki-67 (< 2%)

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

Differential diagnosis
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• 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

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