This site is targeted at medical and radiology professionals, contains user contributed content, and material that may be confusing to a lay audience. Use of this site implies acceptance of our Terms of Use.

Central neurocytoma

Central neurocytomas are WHO Grade II neuroepithelial intraventricular tumours with typical imaging features. 

Epidemiology

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

Clinical presentation

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

Pathology

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

Markers

Purely neuronal origin is demonstrated positivity to neuronal markers such as:

  • synaptophysin
  • neuronal specific enolase
Variants

Ganglioneurocytoma: shows differentiation towards ganglion cells 6

Radiographic features

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. 

CT

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.

MRI
  • T1
    • isointense to grey matter 
    • heterogenous
  • T1 C+
    • mild-moderate heterogeneous enhancement
  • T2/FLAIR
    • typically iso to somewhat hyperintense 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
  • MR spectroscopy
    • may have a strong choline peak
    • glycine peak (3.55ppm) has also been reported 10
Angiography

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

Differential diagnosis

 

Updating… Please wait.
Loadinganimation

Alert_accept

Error Unable to process the form. Check for errors and try again.

Alert_accept Thank you for updating your details.