Site
No. of patients
%
Right
2
16.6 %
Left
5
41.6 %
Biventricular
4
33.3 %
Extending to third ventricle
1
8.3 %
Fig. 1
Tumor location
The transcortical approach was used for ten patients (83.3 %) and the transcallosal for two patients (16.6 %); two patients died early after total and subtotal removal, from sepsis and thalamic infarction, respectively, and they were excluded from the follow-up study. Six patients had a total removal (60 %), while subtotal resection was done for four patients (40 %).
Pathological confirmation aided by immunohistochemistry was diagnostic for central neurocytoma in all patients (100 %). An MIB LI of >2 % was detected in three patients (30 %; two total resections and one subtotal).
In the subtotal group adjuvant radiotherapy was given for three patients (30 %) early postoperatively, while one patient with small residual tumor received stereotactic radiosurgery (SRS) using a Gamma Knife (GK).
A V/P shunt was inserted for three patients (30 %; two early postoperatively, with subtotal removal, and one with recurrence after total removal and SRS).
A small recurrence was detected in two patients in the total resection group, at 12 and 18 months, respectively, in whom a GK was used to control the disease. One patient (10 %) in the subtotal group showed progression that required reoperation at 18 months after progression of the residual tumor and failure of RT.
At the time of the last follow-up, 24 months after the initial surgery, one patient had died (10 %) from hemorrhage during reoperation, while nine patients (90 %) had a favorable course. Local control was achieved in four patients (40 %; no evidence of disease), while five patients (50 %, with stable disease) were controlled with GK and RT, and three patients (30 %) had a V/P shunt. The rate of local control with total removal was (40 %), while the GK and RT control rate was 50 %; (Table 2).
Table 2
Summary of results and outcome
Total | (75 % to 80 % removal) | ||
---|---|---|---|
No. of patients | 6 (60 %) | 4 (40 % | |
MIB labeling index >2 | 2 (20 %) | 1 (10 %) | |
Residual | 0 | 4 (40 %) | |
Recurrence | 2 (20 %) (12 and 18 months) | 1 (10 %) | |
Radiotherapy (RT) | 0 | 3 (30 % | |
Gamma Knife (GK) | 0 | 1 (10 %) | |
Local control (24 months) | Free | 4 (40 %) | 0 |
GK | 2 (100 %) | 1 (100 %) | |
RT | 0 | 2 (66 %) | |
Reoperation | 0 | 1 (10 %) | |
Failure of control | 0 | 1 (10 %) | |
Ventriculoperitoneal (VP) shunt | 1 (10 %) | 2 (20 %) |
Case Illustration
Case (1) (Fig. 2)
Fig. 2
(a) Magnetic resonance imaging (MRI) showing left ventricular central neurocytoma (CN). (b) MRI showing total resection of the tumor during the follow-up period
Case (2) (Fig. 3)
Fig. 3
(a) Recurrence at 18 months after total resection; (b) local control with Gamma Knife radiosurgery and its effect after 6 months
Case (3) (Fig. 4)
Fig. 4
(a) MRI showing huge CN and hydrocephalus, (b) Computed tomography (CT) postoperatively, showing total removal of the tumor
Discussion
There is a lack of studies reporting on the outcome of treatment control of central neurocytomas. The management of neurocytomas has been guided by retrospective case reports, institutional case series, and meta-analysis of institutional experiences [6, 14, 25]. Surgery is the primary modality of initial intervention, with watchful waiting (surveillance) not documented as a common primary option [1, 19]. Despite the indolent nature of these tumors, most patients are symptomatic at presentation, with increased intracranial pressure due to mass effect or hydrocephalus, and thus they require intervention rather than surveillance. Clinically, the tumor causes signs of increased intracranial pressure, visual and mental disturbances, and occasionally, pyramidal or endocrine signs and symptoms. Rarely, neurocytomas may be discovered incidentally with no clinical symptoms.
In our study, the tumor was located predominantly in the left ventricle, in 41.6 % of cases, while it was biventricular in 33.3 % of cases. All the tumors were in the body of the ventricles attached to the septum, while Shin et al. [22] reported that 50 % of central neurocytomas had a typical location in the lateral ventricle around the foramen of Monro and 15 % had biventricular location.
The majority of neurocytomas are benign. In our study 30 % of cases showed an MIB LI of >2 % and were considered to be of an aggressive nature.
Brat et al. [2] and Soylemezoglu et al. [23] found that approximately 25 % of these rare CNS tumors were more aggressive, with an MIB-1 LI of >2 % or atypical histological features.