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 Table of Contents  
ORIGINAL ARTICLE
Year : 2016  |  Volume : 53  |  Issue : 2  |  Page : 119-123

Rate of shunting and outcome after 2 years following excision of ventricular central neurocytoma


Department of Neurosurgery, Cairo University, Cairo, Egypt, presented-at This article has been presented as an oral presentation in the IV International Congress of International Society of Reconstructive Neurosurgery (ISRN) that was held in Rome, Egypt

Date of Submission15-Oct-2015
Date of Acceptance05-Nov-2015
Date of Web Publication2-Jun-2016

Correspondence Address:
Wessam S Soliman
MD, Department of Neurosurgery, Cairo University, Cairo, 11562
Egypt
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/1110-1083.183448

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  Abstract 

Background
Central neurocytoma (CN) is an intraventricular tumor that affects young adults. It has a favorable prognosis after adequate surgical intervention; however, an aggressive course may take place in some cases.
Objective
The aim of this study was to evaluate the rate of shunt insertion and outcome of control in CN excision.
Patients and methods
Ten patients were included in this study and followed up for 24 months. Data collected included age, sex, clinical presentation, early morbidity and mortality, and radiological findings (tumor location, features, residual, recurrence, and hydrocephalus). All patients underwent surgery for total or subtotal excision through a transcortical or transcallosal approach. An external ventricular drain was inserted and then removed and replaced by a shunt, if indicated. Histopathology and the MIB index were used to confirm diagnosis and guide the follow-up. Adjuvant radiotherapy or gamma knife radiosurgery was used for residual or recurrence.
Results
Patient ages ranged from 14 to 48 years and the mean age was 31 years. Two patients died early after total and subtotal excision from sepsis and thalamic infarction, respectively, and were excluded from the results. Six patients (60%) underwent total removal. Of them, two patients had a high MIB index and showed small recurrence at 12 and 18 months and received gamma knife. One case needed a shunt, and the other four cases were free. Subtotal excision was performed for the other four patients (40%). Early shunt was inserted for two cases. Radiosurgery was used to control the residual in one patient, whereas radiotherapy was used for three patients but failed in one patient who underwent surgery at 18-month follow-up.
Conclusion
CN may have favorable prognosis with a lower incidence of shunt insertion throughout its course compared with other intraventricular tumors if total removal is achieved.

Keywords: Central neurocytoma, gamma knife, shunt


How to cite this article:
Soliman WS. Rate of shunting and outcome after 2 years following excision of ventricular central neurocytoma. Egypt J Neurol Psychiatry Neurosurg 2016;53:119-23

How to cite this URL:
Soliman WS. Rate of shunting and outcome after 2 years following excision of ventricular central neurocytoma. Egypt J Neurol Psychiatry Neurosurg [serial online] 2016 [cited 2020 Jun 1];53:119-23. Available from: http://www.ejnpn.eg.net/text.asp?2016/53/2/119/183448


  Introduction Top


Neurocytomas are rare WHO grade II neuronal tumors, which were first identified as histologically distinct entities in 1982 [1],[2]. They likely have an incidence of less than 1%, given that a range of 0.1-0.5% has been reported [3],[4],[5],[6]. These tumors occur most commonly within the ventricles, with a generally favorable prognosis as a result of indolent growth [1],[7].

Immunohistochemistry is frequently used to help distinguish this tumor from other central nervous system (CNS) neoplasms. The MIB-1 proliferation index is commonly used in an attempt to predict biological behavior and may help direct adjuvant therapy. An MIB-1 labeling index greater than 2% often heralds poor prognosis and tumor recurrence [8],[9].

Owing to this rare incidence, the diagnosis and management of this neoplasm remain controversial [10].

Little is known about the management of patients with this tumor, because most literature reports are from the pathologists and contain little information on clinical management [10],[11]. Safe maximal resection is presently considered the ideal therapeutic option, with best long-term prognosis in terms of local control and survival. Moreover, an excellent prognosis can be expected if a gross total resection is achieved. Postoperative radiotherapy (RT) may be considered after subtotal resection. Otherwise, RT is an option for medically inoperable or recurrent disease [12].


  Aim Top


The aim of this study was to evaluate the rate of shunts and outcome of control after 2-year follow-up in central neurocytoma (CN) excision.


  Patients and methods Top


This retrospective study was conducted with IRB approval on 12 patients who presented with intraventricular central neurocytoma (CN) between January 2010 and January 2013 and were managed surgically and followed up for 24 months.

Data included age, sex, symptoms of presentation, and location of the tumor within the ventricles. All patients were managed surgically through a transcortical or transcallosal approach. If needed, an external ventricular drain was inserted and then removed or replaced by a shunt.

Histopathology and the proliferation MIB index were used to confirm the diagnosis and guide for further management. Computed tomography and MRI were used to detect the extent of tumor removal (total or subtotal) after surgery as well as during the follow-up period to assess recurrence or residual progression.

Adjuvant RT was given for all cases with residual tumors. For cases showing progression, gamma knife (GK) radiosurgery was an option. For recurrent or residual cases, V/P shunt insertion and reoperation was performed, if indicated.

Data abstracted from medical record included sex, symptoms at presentation, location of presentation, tumor location, extent of tumor resection based on gross total resection or subtotal resection, pathological details including features associated with atypical neurocytoma, date of tumor progression, and treatment at progression.

RT was considered an adjuvant therapy if it was given immediately postoperatively and was considered salvage therapy if the patient was initially observed and treated for clinical or radiographic progression postoperatively. The date of progression in computed tomography or MRI scans was detected.

Local control was defined as the absence of any tumor regrowth or progression on imaging and excluded the patient who died in the perioperative period. Survival was calculated from data of diagnosis, and local control was calculated from date of first surgery.


  Results Top


A total of 12 patients were included in this study; their ages ranged from 18 to 42 years (seven male and five female). The most common presentation was headache (83.3%), blurring of vision (50%), unsteadiness (16.6%), and seizures (8.3%).

Tumor location was confined to the body and/or extended to the frontal or occipital horns or the third ventricle. Tumor location is shown in [Table 1] and [Figure 1].
Figure 1: Tumor location.

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Table 1: Tumor location

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Transcortical approach was used for 10 cases (83.3%), whereas transcallosal approach was used for two cases (16.6%). Two patients died early after total and subtotal removal from sepsis and thalamic infarction, respectively, and were excluded from the follow-up study. Six patients underwent total removal (60%) (case 1 and case 3), whereas subtotal resection was performed for four patients (40%).

Pathological confirmation aided by immunohistochemistry was diagnostic for CN in all patients (100%). An MIB greater than 2% was detected in three patients (30%) (two total resection and one subtotal resection).

Adjuvant RT was given for three cases (30%) early postoperatively in the subtotal group. However, one patient with small residual received stereotactic radiosurgery (SRS) using GK.

A V/P shunt was inserted for three cases (30%) (two cases early postoperatively in subtotal removal and one case with recurrence after total removal and SRT).

Recurrence was detected in two patients in the totally resected group at 12 months (case 2) and 18 months, for which GK was used to control the disease. One case (10%) in the subtotal group showed progression, which required reoperation at 18 months after progression of the residual tumor and failure of RT; the patient died due to sepsis from ventriculitis and chest infection following surgery.

At last follow-up after 24 months, one patient died (10%) postoperatively from hemorrhage during the reoperation, whereas nine cases (90%) had a favorable course. Local control was achieved in four cases (40%) (no evidence of disease), whereas five cases (50%) (with stable disease) were managed with GK and RT and three patients (30%) had a V/P shunt. The rate of local control with total removal was 40%, whereas that with GK and RT was 50% [Table 2] and [Figure 2],[Figure 3] and [Figure 4].
Figure 2: (a) MRI showing left ventricular central neurocytoma (CN); (b) MRI showing total resection of the tumor during the follow-up period.

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Figure 3: (a) Recurrence after total resection at 18 months; (b) local control with gamma knife radiosurgery and its effect after 6 months.

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Figure 4: (a) MRI showing huge central neurocytoma (CN) and hydrocephalus; (b) postoperative computed tomography (CT) showing total removal of the tumor.

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Table 2: Summary of the results and outcome

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  Discussion Top


There is a lack of studies reporting about the outcome and local control in the treatment of CN. Management of neurocytomas has been guided by retrospective case reports, institutional case series, and meta-analysis of institutional experiences [13-15]. Surgery is the primary modality of initial intervention, with watchful waiting (surveillance) not documented as a common primary option [16],[17]. 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 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 accidentally with no clinical symptoms.

In our study, the tumor was located in the left ventricle predominantly in 41.6% of cases, whereas it was biventricular in 33.3% of cases. All tumors in the body of the ventricles were attached to the septum. In contrast, Shin et al. [18] reported that 50% of CN had a typical location in the lateral ventricle around the foramen of Monro and 15% of CN had biventricular location.

The majority of the neurocytomas are benign. In our study, 30% of cases showed an MIB index greater than 2% and were considered to be of aggressive nature.

Brat et al. [19] and Soylemezoglu et al. [20] found that ∼25% of these rare central nervous system tumors are more aggressive, with an MIB-1 labeling index greater than 2% or atypical histological features.

In a study of 14 cases by Mackenzie [21], clinical follow-up was available for 14 patients. The proliferation potential of CN is a useful predictor of clinical outcome, whereas histological atypia alone is not prognostically significant.

Therapeutic options in the treatment of CN are complete resection (CR), CR + RT, incomplete resection (IR), IR + RT, and SRS (GK). Several studies suggest that CR leads to a significantly better local control and survival compared with IR.

Rades et al. [22] reported that 25% of these CNS tumors are more aggressive, with an MIB greater than 2% or atypical histologic features with a local control rate at 3 years of 70% after CR.

In our study, total resection was achieved in six cases (60%), whereas subtotal resection was performed in four cases (40%). After 2-year follow-up, four of six patients (66.6%) were locally controlled without adjuvant therapy. However, two patients (33.3%) with an MIB greater than 2% showed control of recurrence after GK.

In this study, it was also found that, for patients with IR (subtotal) after 2 years of follow-up, RT was effective in two cases (66%) in controlling progression, whereas it failed in one patient and GK was effective in one case (100%). This is comparable to the results of other studies.

Rades et al. [12],[23] found that local control was significantly better after CR, CR+RT, and IR + RT than after IR, and that the overall survival rate was 99.2 and 86.1% after total and subtotal removal after 12-month follow-up.

They also found that RT is sufficient after IR (ITR), with a rate of local control of 83%.

The largest single institutional experience was reported by Leenstra et al. [24] in Mayo Clinic. It shows that 35% of their patients received adjuvant radiation as part of their initial management but that one-third of their patients recurred, and survival rates. They proposed patient selection on the basis of atypical neurocytomas for consideration of adjuvant therapy. Our results support the theory that atypical neurocytomas are at higher risk of recurrence.

In this study, SRS was very effective to control 100% of IR cases and 100% of recurrent cases after CR.

Rades et al. [23] commented on the value of postoperative SRS and RT in ITR of typical neurocytomas and found that local control was significantly better after ITR + RT (87%) and after ITR + SRS (100%) after 5 years of follow-up.

Rades et al. [23] found that in very indolent tumors, SRS may produce significant local control even though the tumor may eventually progress. With regard to the debate on adjuvant treatment, a 2002 analysis of published institution reports of 504 patients from 91 centers concluded that adjuvant radiation was beneficial after IR. The debate arises given that the extent of resection influences local control only but not survival, and the impact of symptomatic recurrences is not well established in the literature.

Kim et al. [25],[26] support the concept of GK (SRS) to be useful as a primary or secondary postoperative therapy for the treatment of CN.

In this study, three patients (30%) were shunted, two patients (20%) required V/P shunt early postoperatively after external ventricular drain was removed and in another patient a shunt was inserted due to recurrence near the foramen of Monro obstructing the cerebrospinal fluid (CSF) before GK. We do not have much data on the rate of shunt insertion in CN, but due to the pathological features of this tumor being located in the body of the ventricle near the foramen with a plane of cleavage from the ependyma, being soft, suckable, moderately vascular, attached to the septum which allows septostomy during surgery, all of the above factors may be attributed to the low incidence of developing hydrocephalus postoperatively with the ease of draining the ventricles at the level of the foramen with less blood.

In this study, the survival rate after 2 years was 90% with an overall favorable prognosis and local control rates, compared with the study by Leenstra et al. [24] and Rades et al. [22],[12],[10],[23].


  Conclusion Top


CR is much more effective for the treatment of CN compared with IR. After IR, postoperative adjuvant RT and radiosurgery significantly improve local control, with these types of tumors showing a low incidence of shunt insertion following total or even IR.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
    Tables

  [Table 1], [Table 2]


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