Central neurocytomas are typically seen in young patients (20 - 40 years of age), and accounts for less than 1% (0.25 - 0.5%) of intracranial tumours. There is no reported gender predilection 10.
Typically central neurocytomas present with symptoms of increased intracranial pressure, headaches being most frequent, or seizures (especially tumours with extra ventricular extension).
A relatively short clinical course, typically only a few months, is most common. Rarely central neurocytomas may be associated with sudden death secondary to acute ventricular obstruction 4. Also rare, is a sudden presentation due to intraventricular haemorrhage 7.
Initially described as recently as 1982, central neurocytomas demonstrate neuronal differentiation and histologically appear similar to oligodendrogliomas. This has historically has resulted in many tumours erroneously categorised. The cells are typically uniform and round with a salt and pepper appearance.
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.
Purely neuronal origin is demonstrated positivity to neuronal markers such as
- neuronal specific enolase
Ganglioneurocytoma : shows differentiation towards ganglion cells 6
The vast majority of central neurocytomas are located entirely within the ventricles. Typical locations include 4.
- lateral ventricles around foramen of Munro (most common): 50%
- both lateral and 3rd ventricles: 15%
- bilateral: 15%
- 3rd ventricle in isolation: 5%
Extra ventricular neurocytomas (or cerebral neurocytomas) are distinctly uncommon, and thought to be a separate entity due to the tendency to have prominent ganglionic or glial differentiation.
Central neurocytomas are usually hyperattenuating compared to white matter. Calcification seen in over half of cases, usually punctate in nature 4,10. Cystic regions are frequently present, especially in larger tumours. Contrast enhancement is usually mild to moderate. Accompanying ventricular dilatation often present.
- iso intense to grey matter
- mild-moderate heterogeneous enhancement
T2 / FLAIR
- typically iso to somewhat hyper intense compared to brain
- numerous cystic areas (bubbly appearance), many of which completely attenuate on FLAIR
- prominent flow voids may be seen 10
GE / SWI
- calcification is common, typically punctate
- haemorrhage (especially in larger tumours) is common
- uncommonly results in ventricular haemorrhage
- may have a strong choline peak
- glycine peak (3.55ppm) has also been reported 10
A tumour blush is frequently identified, with the mass supplied by choroidal vessels. No large feeding arteries are usually seen.
Treatment and prognosis
Complete surgical resection is usually curative (5 year survival 81%). When only incomplete resection possible or extraventricular extension is present then adjuvant radiotherapy (and sometimes chemotherapy) are added, although their benefit is not well established.
Cases of CSF dissemination have been reported, but are rare 10.
- more frequent in childhood
- more commonly in 4th ventricle
- supratentorial tumours (esp in children) often have a significant extraventricular (parenchymal) component 4
intra ventricular meningioma
- homogeneous contrast enhancement
- well circumscribed mass
- typically found in the 4th ventricle
- usually older individuals 8
- may have ependymoma components and look very similar 9
subependymal giant cell astrocytoma (SGCA)
- in patients with tuberous sclerosis
- vivid contrast enhancement
choroid plexus papilloma (CPP)
- mainly in children
- typically show intense contrast enhancement
intra ventricular metastasis
- older patients
- usually stronger contrast enhancement
- history of primary (e.g. RCC)
- this is especially difficult in cases where there is a parenchymal component as histologically the tumours are very similar
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Synonyms & Alternative Spellings
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|Central intraventricular neurocytoma||✗|