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Central Neurocytoma: a Review of Clinical Management and Histopathologic Features

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Central Neurocytoma: a Review of Clinical Management and Histopathologic Features Brain Tumor Res Treat 2016;4(2):49-57 / pISSN 2288-2405 / eISSN 2288-2413 REVIEW ARTICLE http://dx.doi.org/10.14791/btrt.2016.4.2.49 Central Neurocytoma: A Review of Clinical Management and Histopathologic Features Seung J. Lee1, Timothy T. Bui1, Cheng Hao Jacky Chen1, Carlito Lagman1, Lawrance K. Chung1, Sabrin Sidhu1, David J. Seo1, William H. Yong3, Todd L. Siegal4, Minsu Kim5, Isaac Yang1,2 1Department of Neurosurgery, University of California, Los Angeles, Los Angeles, CA, USA 2Jonsson Comprehensive Cancer Center, University of California, Los Angeles, Los Angeles, CA, USA 3Department of Pathology & Laboratory Medicine, University of California, Los Angeles, Los Angeles, CA, USA 4Department of Radiology, Division of Neuroradiology, Cooper University Hospital, Camden, NJ, USA 5Department of Neurosurgery, Yeungnam University College of Medicine, Daegu, Korea Central neurocytoma (CN) is a rare, benign brain tumor often located in the lateral ventricles. CN may Received September 1, 2016 cause obstructive hydrocephalus and manifest as signs of increased intracranial pressure. The goal of Revised September 21, 2016 treatment for CN is a gross total resection (GTR), which often yields excellent prognosis with a very high Accepted September 21, 2016 rate of tumor control and survival. Adjuvant radiosurgery and radiotherapy may be considered to im- Correspondence prove tumor control when GTR cannot be achieved. Chemotherapy is also not considered a primary Isaac Yang treatment, but has been used as a salvage therapy. The radiological features of CN are indistinguishable Department of Neurosurgery, from those of other brain tumors; therefore, many histological markers, such as synaptophysin, can be University of California, Los Angeles, very useful for diagnosing CNs. Furthermore, the MIB-1 Labeling Index seems to be correlated with the 300 Stein Plaza, Ste. 562, 5th Floor prognosis of CN. We also discuss oncogenes associated with these elusive tumors. Further studies Wasserman Bldg., Los Angeles, CA 90095-6901, USA may improve our ability to accurately diagnose CNs and to design the optimal treatment regimens for Tel: +1-310-267-2621 patients with CNs. Fax: +1-310-825-9385 E-mail: [email protected] Key Words Central neurocytoma; Histopathology; Management. INTRODUCTION EPIDEMIOLOGY Central neurocytoma (CN) was first described in the 1980’s CN remains relatively rare, comprising about 0.1–0.5% of by Hassoun et al. [1] who studied two patients with intraven- all brain tumors [5-8]. CNs are most prevalent among young tricular tumors using electron microscopy. CN is a benign tu- adults, and nearly 25% of all cases involve individuals in their mor of the central nervous system that is classified as a grade thirties [4,9]. The age of affected individuals ranges from 8 II tumor by the World Health Organization (WHO) [2,3]. A days old to 67 years old with an overall median age of 34 years combination of treatments, such as surgery with adjuvant ra- [6,10]. There is no correlation between gender and the inci- diation, can be considered for CN despite its good prognosis dence of CN [7,9,11,12]. Some studies have indicated higher [2-4]. Because of the tumor’s rarity and its elusive nature, only incidences of CNs in Korea, India, and Japan, which is possi- a limited number of studies, case reports, and reviews have bly attributed to genetic differences among racial groups that been published on CN. make certain individuals more prone to CNs than others [8,10,13-28]. The higher incidence in these Asian countries, make this tumor an important consideration when dealing with intraventricular tumors in these populations. This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. TUMOR LOCATION Copyright © 2016 The Korean Brain Tumor Society, The Korean Society for Neuro- Oncology, and The Korean Society for Pediatric Neuro-Oncology CNs are neurocytomas located within the ventricles. Most 49 A Review of Central Neurocytoma CNs are found in the anterior half of the lateral ventricle, al- tion of symptoms can vary from a few days to many years though some have reported to be found in the third and fourth [29,30,55,61]. The duration seems to be mostly related to tumor ventricles [29-31]. The tumor is also usually attached to the location, and does not seem to be correlated to the aggressive- septum pellucidum near the foramen of Monroe [32,33]. The ness of the tumor [4]. cellular origination of CN is unclear; however, various authors have suggested CN may develop from neuronal cells, neuronal RADIOLOGICAL FEATURES progenitor cells, neuronal stem cells, and multipotent precur- sor cells [11,21,29,34-36]. CN can appear as a dense mass in computerized tomogra- Neurocytomas can also occur extraventricularly, along the phy (CT) scans indicating calcifications, which occur in up to spinal cord or the brain parenchyma [10]. Extraventricular 50% of all cases, and present a patchy and coarse appearances neurocytomas (EVN) have been found in the cerebral hemi- (Fig. 1) [7,30,33]. The tumor can also be heterogeneous be- spheres, spinal cord, brainstem, thalamus, pons, amygdala, pi- cause of the hypodense areas related to cystic degeneration neal gland, retina, and cerebellum [5,10,37-50]. EVN is also [4,31]. In contrast-enhanced CT scans, CNs have mild to mod- classified as a grade II tumor [3]. However, EVN has a wider erate enhancements [32,62]. Furthermore, CNs appear slight- morphological spectrum and a higher degree of either focal or ly hypo-intense to iso-intense on T1-weighted magnetic reso- diffuse ganglionic differentiation than CN [6,10,38,51-53]. nance imaging (MRI) (Fig. 2), and iso-intense to hyper-intense on T2-weighted MRI (Fig. 3) [4,29,31,32,58]. In both T1- and CLINICAL MANIFESTATIONS T2-weighted MRI, hypointensity can indicate the presence of a hemorrhage, cyst, or calcification [31]. Typically, after a con- CN may increase the intracranial pressure by obstructing the trast agent is injected, moderate enhancement is seen on MRI interventricular foramen, which can lead to hydrocephalus for CN (Fig. 4) [29,58,63]. Unfortunately, there is no estab- [32,54]. Patients may also experience nausea, vomiting, head- lished criterion to distinguish between CN and other tumors ache, seizures, decreased consciousness, weakness, and memo- such as oligodendrogliomas on CT scans and MRI [4,11,64]. ry or vision problems [4,7,30,38,54-57]. In rare cases, intraven- tricular hemorrhage may also occur [58]. Patients with EVN SURGERY present with similar symptoms, in addition to weakness and numbness in the limbs [20,42,59,60]. These symptoms are typi- Surgical management with a gross-total resection (GTR) is cally present for approximately 3–6 months, although the dura- currently the gold standard treatment for CNs, which often has excellent prognosis and minimizes the chances of CN re- Fig. 1. Axial CT demonstrating a large hypodense central neuro- cytoma. Moderate, heterogenous hyperdensities are consistent Fig. 2. Axial T1-weighted MRI showing a large isointense CN in with calcifications. the lateral ventricles consistent with CN. CN, central neurocytoma. 50 Brain Tumor Res Treat 2016;4(2):49-57 SJ Lee et al. Fig. 3. Axial T2-weighted MRI showing a heterogenous, hyperin- Fig. 4. Axial T1-weighted MRI with contrast showing a large isoin- tense central neurocytoma. tense central neurocytoma with moderate enhancement. currence [65]. GTR is achieved in nearly 30–50% of all CN pa- Stereotactic radiosurgery tients. In an analysis of 310 patients with CN who underwent While FRT delivers multiple fractions of radiation in lower a GTR, there was a 99% five-year survival rate [14,54,65,66]. dosage, stereotactic radiosurgery (SRS) administers one higher In comparison, individuals who had surgery with only subto- dose (9 to 25 Gy) of radiation in 1-5 fractions. The first litera- tal resection (STR) had an 86% five-year survival rate. STR of ture on the use of SRS for CN was published by Schild et al. CN increases the rate of recurrence and decreases the rate of [54]. SRS has been suggested to be potentially favorable to survival [65]. A recent multi-center study found that in 71 pa- FRT. Although not statistically significant, Patel et al. [11] tients with CN, those with STR had a 3.8-fold higher risk of also reported that adjuvant SRS for patients with STRs dem- recurrence and adverse outcomes compared to patients with onstrated a 100% tumor control rate compared to an 87% tu- GTR [12]. For patients with STR, adjuvant radiotherapy was mor control rate for patients with adjuvant FRT [54]. administered in this study. Garcia et al. [70] also reported a higher tumor control rate of 93% with SRS versus 88% with FRT. The relative risk (RR) of RADIOTHERAPY SRS to FRT for recurrence was 0.57 less (95% CI: 0.21–1.57; log-rank p=0.85), and the RR for mortality was 0.23 less (95% Fractionated radiotherapy CI: 0.05–1.05; log-rank p=0.22), although statistically insignifi- Radiotherapy and radiosurgery are non-invasive adjuvant cant. Lower complication was noted for patients with SRS, al- treatments, but the toxicities from radiation are still being though distant tumor recurrence was slightly higher in patients weighed against the benefits of tumor control [65,67]. Because who received SRS than those who received FRT. SRS is suggest- CNs usually have excellent prognosis when GTR is achieved, ed to be at least as effective as FRT in achieving tumor control. radiation is not always indicated [25,54,55].
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