====== Central neurocytoma ======

Central [[neurocytoma]] (CN), is a [[intraventricular tumor]] of [[neuroepithelial]] origin with intermediate malignancy (WHO grade II) first described in [[1982]] by Hassoun and colleagues
((Hassoun J, Gambarelli D, Grisoli F, Pellet W, Salamon G, Pellissier JF, Toga
M. Central neurocytoma. An electron-microscopic study of two cases. Acta
Neuropathol. 1982;56(2):151-6. PubMed PMID: 7064664.
)).

Typically forms from the neuronal cells the [[septum pellucidum]].

===== Epidemiology =====

Central neurocytomas are typically seen in young patients (20-40 years of age) and account for less than 1% (0.25-0.5%) of [[intracranial tumor]]s. There is no reported gender predilection.

[[Central neurocytoma]]s (CNs) occurs typically in the [[lateral ventricle]], adjacent to the [[septum pellucidum]] and [[foramen of Monro]].
((Chandshah MI, Sadashiva N, Konar S, Devi BI. Central Neurocytoma Presenting with Bleed and Obstructive Hydrocephalus: A Rare Presentation. Asian J Neurosurg. 2019;14(3):919–921. doi:10.4103/ajns.AJNS_84_19)).

Isolated lesions in the third ventricle are uncommon
((Khoo J, Tollesson G. Endoscopic approach and stereotactic radiosurgery for a
posterior third ventricular Central Neurocytoma - case report and literature
review. Int J Surg Case Rep. 2020;68:119-123. doi: 10.1016/j.ijscr.2020.02.042.
Epub 2020 Feb 25. PubMed PMID: 32145562; PubMed Central PMCID: PMC7057151.
)).

===== Classification =====

Atypical central neurocytoma with extracranial metastases is a rare variant of benign central neurocytoma (CN).

===== Molecular Genetics =====

[[1p/19q co-deletion]] has not been reported in central neurocytoma, but it can be seen in [[extraventricular neurocytoma]].

===== Clinical features =====

The clinical presentation varies and many are [[incidental]]ly detected. 

Typically central neurocytomas present with symptoms of [[intracranial hypertension]], [[headache]]s being most frequent, or [[seizure]]s (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. Also rare, is a sudden presentation due to [[intraventricular hemorrhage]].

see [[Obstructive hydrocephalus from central neurocytoma]].

Only 3 previous cases in which CN presented with co-occurring psychotic symptoms were found in the PubMed database. A report presents the case of a 27-year-old patient with [[paranoid]] syndrome without neurological symptoms, in whom magnetic resonance imaging confirmed a large intracranial tumor located predominantly in the right lateral ventricle and third ventricle reaching down to the hypothalamus. Resection of the tumor (histopathologically a CN) resulted in complete remission of the psychotic symptoms. This case supports the need for neuroimaging in all patients with first-episode psychosis because of the possibility of neurologically silent brain tumors. Quick diagnosis in such cases is crucial for the selection of treatment methods and prognosis
((Karakula-Juchnowicz H, Morylowska-Topolska J, Juchnowicz D, Korzeniowska A,
Krukow P, Rola R. Paranoid Syndrome as the First Sign of Central Neurocytoma: A
Case Report. J Psychiatr Pract. 2018 Sep;24(5):359-363. doi:
10.1097/PRA.0000000000000332. PubMed PMID: 30427824.
)).

===== Complications =====

The incidence of [[spontaneous]] [[hemorrhage]] at presentation is rare. Chandshah et al. reported a case of the [[intraventricular]] lesion with bleed which was operated on an [[emergency]] basis as it caused [[obstructive hydrocephalus]] and the patient was in altered sensorium. The exact cause of hemorrhage in CNs is not known, and the previously discussed explanations are discussed
((Chandshah MI, Sadashiva N, Konar S, Devi BI. Central Neurocytoma Presenting with Bleed and Obstructive Hydrocephalus: A Rare Presentation. Asian J Neurosurg. 2019;14(3):919–921. doi:10.4103/ajns.AJNS_84_19)).

see [[Obstructive hydrocephalus from central neurocytoma]].

===== Diagnosis =====

Detectable with both [[computed tomography]] and [[magnetic resonance imaging]].
---- 
Fairly characteristic imaging features, appearing as heterogeneous masses of variable size and enhancement within the [[lateral ventricle]]. 

The vast majority of central neurocytomas are located entirely within the ventricles. Typical locations include:

[[lateral ventricle]]s around [[foramen of Monro]] (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.

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 

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 

==== Angiography ====


A tumor blush is frequently identified, with the mass supplied by choroidal vessels. No large feeding arteries are usually seen.

===== Pathology =====

[[Central Neurocytoma Pathology]].

===== Differential diagnosis =====

[[Central Neurocytoma Differential Diagnosis]].


===== Treatment =====

see [[Central neurocytoma treatment]].

===== Outcome =====
They generally have a good prognosis provided a complete resection can be achieved. 

Cases of CSF dissemination have been reported, but are rare.

===== Case series =====

see [[Central neurocytoma case series]].

===== Case reports =====

[[Central neurocytoma case reports]].

===== References =====