Brain, Central neurocytoma [LMP12069]

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Diagnostic Modality: 
Whole Slide Image
Disease Category: 
Neoplasia
Diagnosis: 
Central neurocytoma
Species: 
Human
Organ System: 
Central Nervous System
Gender: 
Female
Age: 
17 years
Diagnosis Code: 
ICD-10: C71.5 | ICD-O: 9506/1
Slide Contributor Information
Gao, A., Munoz, D.

Keywords

intraventricular, neuronal tumour

Clinical History

17-year-old girl with headache found to have a large tumour within the left lateral ventricle on neuroimaging.

Clinical Discussion

Central neurocytoma is an uncommon intraventricular neoplasm with neuronal differentiation, classified as WHO grade II. They generally occur in young adults, most commonly in the lateral or third ventricles. Microscopically, they are characterized by uniform round cells with salt-and-pepper chromatin. They may form neurocytomatous rosettes, composed of large anuclear fibrillary areas, or more rarely Homer Wright rosettes. Immunohistochemistry shows staining for neuronal markers, most reliably synaptophysin, NeuN, class III beta tubulin, and MAP2. However, chromogranin-A and neurofilament are usually negative. GFAP is also usually not expressed. Proliferation index is typically low (<3%). In rare cases, tumours may exhibit a high proliferation index or multiple anaplastic histological features (mitoses, microvascular proliferation, necrosis) and are termed anaplastic central neurocytoma. Prognosis is generally favourable although some tumours recur even after gross total resection.