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Introduction: Central Neurocytoma (CN) is a rare, WHO grade 2 brain tumor that predominantly affects young adults. Gross total resection (GTR) is often curative for CNs, but the optimal treatment paradigm including incorporation of RT, following subtotal resection (STR) and for scarcer pediatric cases has yet to be established., Methods: Patients between 2001 and 2021 with a pathologic diagnosis of CN were reviewed. Demographic, treatment, and tumor characteristics were recorded. Recurrence free survival (RFS) and overall survival (OS) were calculated according to the Kaplan Meier-method. Post-RT tumor volumetric regression analysis was performed., Results: Seventeen adults (≥ 18 years old) and 5 children (< 18 years old) met the criteria for data analysis (n = 22). With a median follow-up of 6.9 years, there was no tumor-related mortality. Patients who received STR and/or had atypical tumors (using a cut-off of Ki-67 > 4%) experienced decreased RFS compared to those who received GTR and/or were without atypical tumors. RFS at 5 years for typical CNs was 67% compared to 22% for atypical CNs. Every pediatric tumor was atypical and 3/5 recurred within 5 years. Salvage RT following tumor recurrence led to no further recurrences within the timeframe of continued follow-up; volumetric analysis for 3 recurrent tumors revealed an approximately 80% reduction in tumor size., Conclusion: We provide encouraging evidence that CNs treated with GTR or with RT after tumor recurrence demonstrate good long-term tumor control., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Almost any primary or metastatic brain tumour can manifest in intraventricular (IV) locations. These tumours may either originate within the ventricular system or extend into the IV space through growth. Such neoplasms represent a broad spectrum, with supratentorial IV tumours forming a heterogeneous group. This group includes primary ependymal tumours, central neurocytomas, choroid plexus tumours, and notably, meningiomas, as well as a variety of non-neoplastic, benign, glial, and metastatic lesions that can secondarily invade the IV compartment. Often presenting with nonspecific symptoms, these tumours can lead to delayed medical attention. The diversity in potential diagnoses, combined with their deep and complex locations, poses significant management challenges. This paper aims to delineate optimal management strategies, underscoring the importance of multidisciplinary care, especially in settings with limited resources, to effectively navigate the complexities associated with treating intraventricular brain tumours.

Objective: The rarity of intracranial extraventricular neurocytomas (EVNs) has precluded accurate definition of its surgical characteristics to date. The authors present the first survival analysis of this unique entity that aims to clarify tumor characteristics, surgical outcomes, and efficacy of postoperative adjuvant therapy., Methods: A systematic review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. Searches of the MEDLINE, Embase, Scopus, Google Scholar, and Cochrane Database of Systematic Reviews databases were performed from inception to date. Primary outcomes were progression-free survival (PFS) and overall survival (OS). Prognostic variables were age, sex, tumor consistency, extent of resection, and receipt of postoperative adjuvant therapy. Survival data were analyzed using Kaplan-Meier survival curves and the log-rank test to compare dichotomized cohorts. Multivariate Cox regression models were constructed, interrogated with Schoenfeld residuals, and subsequently utilized to identify independent prognostic factors. Risk of bias was assessed with the Mayo Clinic instrument., Results: Five hundred fourteen articles were initially retrieved, which was distilled to 10 included articles consisting of 101 cases of intracranial EVNs. The 5-year OS rate was 90.4% (95% CI 81.8%-99.8%) and the PFS rate was 48.6% (95% CI 34.46%-68.8%). The median PFS was 60 months. Patients younger than 50 years of age experienced superior OS (p = 0.03) and PFS (p < 0.01). Gross-total resection (GTR) was superior to subtotal resection (STR) in reducing mortality (p < 0.01). Adjuvant therapy following either STR or GTR did not significantly improve survival., Conclusions: Intracranial EVNs are rare tumors that portend a poorer prognosis than central neurocytomas, despite both being WHO grade 2 tumors. Complete surgical extirpation is the cornerstone of management. There is no clearly established role for adjuvant postoperative therapy, but each case should be managed on an individual basis.

Background: Cerebellar liponeurocytoma is a rare entity with fewer than 100 reported cases and series in the available literature to date. Although the cerebellum remains the typical primary site, the entity has been shown to demonstrate increased aggressiveness and malignant progression with multiple recurrences., Case Description: We present a unique case in a 64-year-old gentleman of a cerebellar liponeurocytoma with multiple recurrences and progressive anaplasia. The tumor showed anaplastic features at first presentation and recurred in a more aggressive fashion in a short 2-year period despite surgical debulking and post-operative radiotherapy. It re-recurred within 6 months with subsequent re-debulking without further radiotherapy. At latest follow-up almost 3 years since surgical management of the patient's second recurrence, the patient remains well with minimal neurological impairment and no radiological signs of recurrence., Conclusion: Cerebellar liponeurocytoma may present with increasingly atypical histological features that may warrant more aggressive post-operative treatment to prevent disease recurrence and clinical deterioration. This may include a more aggressive surgical resection margin and consideration of adjuvant radiotherapy in all cases.

Rationale: Central neurocytoma occurring outside the ventricles is known as extraventricular neurocytoma (EVN). EVN is rare and its magnetic resonance imaging (MRI) findings vary greatly and overlap with the imaging features of other intracerebral primary tumors., Patient Concerns: A 21-year-old woman with an intrauterine pregnancy of 18+2 weeks complained of dizziness and headache for 3 months., Diagnosis: A 8.6 cm × 5.8 cm × 3.7 cm space-occupying lesion was seen in the right frontal lobe on MRI, with mixed long signals on T1-weighted imaging and mixed slightly long signals on T2-weighted imaging, slightly hyperintense on T2-weighted imaging fluid attenuated inversion recovery images, and a large-scope long T1-weighted imaging and long T2-weighted imaging cystic component at the center of the lesion. A thick fence-like enhancement of the solid component at the periphery of the lesion was observed after injecting a contrast medium, while the internal cystic component was not enhanced. The MRI diagnosis was of glioma. The lesion was pathologically confirmed as an atypical central neurocytoma of the right frontal lobe., Interventions: Resection of the lesion and postoperative radiotherapy., Outcomes: The patient was lost to follow-up., Lessons: EVN can be considered as a differential diagnosis for lesions occurring in the cerebral hemispheres of young patients with cystic degeneration, thick fence-like enhancement, and peritumoral edema on MRI., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2021 the Author(s). Published by Wolters Kluwer Health, Inc.)

Extraventricular neurocytomas (EVNs) are rare neuroepithelial neoplasms of the central nervous system that were first described in 1997. Most studies in patients with EVNs have incorporated mixed age groups. The tumor's clinical behavior specifically in children has not been explored in depth, while a detailed statistical analysis has never been performed in this age group. Hence, we performed a systematic review to address possible prognostic factors and the appropriate management in children with EVNs. Relevant studies were identified by searching the MEDLINE and SCOPUS databases. We included studies concerning patients 18 years of age or younger who were histologically diagnosed with EVNs. A total of 52 studies with 79 patients were included. The mean age of the patients was ~ 10 years with a male predilection (~ 2:1). Most of these tumors were located in the frontal (49%) lobe. We observed that gross total resection of the tumor was significantly lower in cases of atypical EVNs (p < 0.05). Additionally, atypical EVNs were associated with worse overall survival compared to typical EVNs (p = 0.05). Children 4 years of age or under had a worst outcome (p = 0.001). The patient's sex and the extent of the tumor's resection did not appear to affect the prognosis in a statistically significant manner. Contrary to the results of previous studies, the use of adjuvant radiotherapy or chemotherapy for the treatment of EVNs was not associated with better outcomes in the pediatric population. Thus, a less aggressive management of children with EVNs compared to the adult population is suggested., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Central neurocytomas represent 0.25-0.5% of all intracranial tumors in adults. Leptomeningeal spread is uncommon, and the exact incidence of meningeal spread is unknown due to sparse literature. We present the clinical course and management outcome of a case of atypical central neurocytoma with leptomeningeal spread., Case Presentation: A young gentleman, who initially presented with memory loss, was found to have a right intra-axial periventricular mass on imaging. He underwent subtotal resection, and operative histopathology suggested a periventricular atypical neurocytoma. In view of subtotal resection, adjuvant focal radiation therapy was recommended, but he developed headache and blurring of vision 10 days postoperatively. Contrast enhanced craniospinal magnetic resonance imaging (MRI) showed residual primary tumor as well as diffuse leptomeningeal spread. Cerebrospinal fluid cytology also showed malignant cells. After tumor board discussion, craniospinal axis irradiation was advised and delivered. He remained disease-free for 10 months after radiation therapy, but then developed local and spinal recurrence, and offered salvage chemotherapy. His general condition deteriorated following chemotherapy with disease progression, and he was subsequently advised best supportive care., Conclusion: Leptomeningeal dissemination in atypical neurocytomas portends an aggressive course and adverse prognosis; management decisions may need tailoring as per individual presentation.

Background: Central neurocytomas (CNCs) are rare intraventricular lesions comprising <1% of primary brain tumors. Their surgical and adjuvant management is unclear., Objective: Our goal was to update Rades et al.'s 2006 systematic review to assess the outcome differences among 3 fundamental therapies for CNC: gross total resection with and without radiation therapy (RT) versus maximal safe resection with adjuvant RT., Methods: Articles indexed on PubMed and Google Scholar and published between January 1, 2006 and December 31, 2019 were selected using the PRISMA criteria. Studies were excluded if they had fewer than 3 cases, did not categorize extent of resection, or were duplicate studies, technical reports, case reports, or studies without follow-up. Complication rates, recurrence rates, overall survival and progression-free survival were extracted where possible. χ 2 proportionality tests were used for comparison (P values >0.05 suggested significance)., Results: On aggregation, 615 patients from 13 studies including ours were assessed. Although overall survival was not significantly different (χ 2  = 1.56; P = 0.46), the recurrence rate differed significantly between GTR + RT (6.9%, 92.11 months), GTR-RT (23.9%, 96.8 months), and MSR + RT (16.8%, 85 months) (χ 2  = 10.94; P = 0.004). Pooled complication rates for GTR and MSR + RT were 31.2% and 24% (P = 0.049), respectively., Conclusions: RT remains an important adjuvant treatment that can improve patient survival in the presence of MSR to levels comparable to those of GTR or GTR + RT. Where total resection carries too much risk, MSR + RT can be considered as the next best alternative for tumor control., (Copyright © 2020. Published by Elsevier Inc.)

Background: Brain aneurysms (BAs) are the most common intracranial vascular condition, with an overall incidence of 1%-2%. Among the common causes of their initial formation and growth, the role of radiation therapy (RT) has been reported in some studies. The aim of the present study is to report the most relevant features of BA related to a previous cranial RT., Methods: Data deriving from 1 patient treated for RT-induced BA in our institution were added to reports of another 66 BAs retrieved from the literature. The following parameters were evaluated: age, sex, location, primary lesion, clinical presentation, dosage/amount of radiation delivered, type of treatment for the BA, dimension, morphology, chemotherapy, comorbidities, risk factors, and number of BAs., Results: The most commonly involved vessel was the internal carotid artery (34%). In general, the anterior circulation showed higher vulnerability compared with the posterior circulation and middle cerebral artery (56.7%). The average latency between RT and the first imaging showing the BA was 9.01 ± 6.85 years. Vessels coursing in the posterior cranial fossa showed a significant univariate association with lower X-ray dosages (P = 0.014) compared with the other locations. No statistically significant correlation between the continuous variables age, latency of BA appearance, RT delivered dose, and dimension of the BA was shown., Conclusions: The apparent higher fragility of the vascular structures of the posterior cranial fossa was statistically outlined, and the X-ray dosage, the primary condition target of the RT, the age of the patients, and no statistically significant correlation were outlined. Biological factors could play a significant role., (Copyright © 2019. Published by Elsevier Inc.)

A 13-year-old female arrived at the Emergency Department with a two-week history of headache, and bilateral papilloedema on examination. The initial study with CT and MRI showed a large multicystic left frontal mass with calcification surrounded by peripheral oedema, subacute intralesional bleeding and association of multiple large vessels. She was initially operated on in another centre where a subacute haematoma was found, evacuating to multiple vessels and arteriolised veins. Despite the earlier neuroimaging findings, arteriovenous malformation (AVM) was suspected, so she was referred to our centre for further treatment. We performed angiography, MR angiography and MRI with advanced sequences, diagnosing a highly vascularised intra-axial tumour which was embolised. The patient was then definitively operated on, with the resulting finding of extraventricular neurocytoma (EVN). EVN are extremely rare lesions, not previously described in the literature as hypervascularised lesions, which in our case required prior angiography and embolisation for proper diagnosis and adequate management., (Copyright © 2018 Sociedad Española de Neurocirugía. Publicado por Elsevier España, S.L.U. All rights reserved.)

Introduction: Central neurocytoma (CN) is a very rare neuronal neoplasm. The clinical implications of the potential prognostic factors for these lesions, including tumor atypia, have therefore not been clarified., Methods: Forty CN patients were enrolled and reclassified as typical or atypical in accordance with an MIB-1 labeling index (LI) of above and below 2%., Results: We classified our retrospective study cohort as 21 (52.5%) typical and 19 (47.5%) atypical CN cases. No significant differences were found in terms of sex, mean age, mean tumor size or tumor location between these groups. Recurrences occurred in 2 (9.5%) typical and 6 (33.3%) atypical cases. The typical CN 2-,3- and 5-year PFS rates were 100%, 100%, 92.3%, and those for the atypical group were 93.8%, 78.1%, 65.1%, respectively (p = 0.02). The PFS rates did not statistically differ by treatment modality (gross total resection alone, subtotal resection (STR) alone and STR plus radiation therapy (RT) or radiosurgery (RS)) either in the whole cohort (p = 0.75) or in the typical CN and atypical CN subgroups (p = 0.45 and 0.98, respectively). An atypical histology was the only prognostic indicator of recurrence by univariate analysis (hazard ratio: 5.40, p = 0.04)., Conclusions: An atypical lesion (MIB-LI > 2%) is an important prognostic indicator in CN. The clinical implications of the extent of resection for CN patients are still debatable. The use of STR plus RT or RS may be a viable treatment strategy for CN but different therapeutic and follow-up approaches for atypical CN will be needed.

Background: Cerebellar liponeurocytoma (cLNC) is a rare benign glioneuronal tumor with only ~60 cases reported since its first description in 1978. They have occurred largely as sporadic cases; however, familial cases with possible autosomal dominant inheritance have also been reported. Surgical excision has been considered the main modality of treatment, even for recurrent lesions. Uncertainties exist regarding the natural history, long-term outcomes, and optimal postoperative management and follow-up protocols for cLNC in general and multifocal cLNC in particular. Multifocality is exceedingly rare, with only 4 cases reported previously., Case Description: We present a case of multifocal cLNC in a young woman who had presented with progressive cerebellar symptoms of 5 months' duration. Her diagnostic neuroimaging evaluations revealed 2 asymmetric mass lesions (1 on each cerebellar hemisphere). We excised the larger lesion to relieve the mass effect and decided to monitor the smaller lesion radiologically. However, she developed a new lesion during the follow-up period and required adjuvant radiotherapy., Conclusion: Surgical excision forms the cornerstone of management of cLNCs. However, it is necessary to vigilantly monitor these patients owing to the high recurrence rates of cLNC despite the innocuous histologic features., (Copyright © 2018. Published by Elsevier Inc.)

Purpose: Central neurocytomas (CNs) are rare World Health Organization grade II tumors managed with surgery and radiation therapy. We report our experience in managing CN with proton beam therapy (PBT) when radiation therapy was used., Methods and Materials: We identified 61 patients with pathologically diagnosed CN treated at our institution between 1996 and 2016, of which 24 met inclusion criteria. Patient, tumor, and treatment characteristics are reported in context of progression-free survival and treatment-related adverse events., Results: Of 24 patients identified, median age at diagnosis was 21 years (range, 14-60). Median maximal tumor diameter was 4.5 cm (range, 1.4-6.8). Eighteen (75%) patients underwent upfront surgery alone. Sixteen (67%) patients received adjuvant or salvage PBT at a median dose of 54 Gy (relative biological effectiveness). Median follow-up was 56 months. Median progression-free survival (PFS) was 61 months. Eleven patients had disease progression with median time to progression of 22 months. Of the 5 patients with gross total resection, 4 experienced local recurrence and had MIB-1 >4% (range, 4.5-30). There was improved PFS with addition of PBT to definitive surgery (log-rank, P = .06); there was no disease progression to date. In patients who experienced disease recurrence/progression, MIB-1 <4% was associated with improved PFS (log-rank, P = .007). All patients tolerated PBT well with toxicities typical for cranial irradiation and with no grade 3+ toxicities., Conclusion: In our cohort, CN with elevated MIB-1 index were at increased risk for disease progression. However, adjuvant radiation therapy appears to effectively prevent failure. PBT toxicities appear to be comparable to if not less than published photon experiences., (Copyright © 2018 American Society for Radiation Oncology. Published by Elsevier Inc. All rights reserved.)

Purpose: To investigate clinical characteristics and patterns of care among patients with central neurocytomas in a large cohort of patients., Methods: The National Cancer Database (NCDB) was queried to identify patients with biopsy confirmed neurocytoma from 2004 to 2015. Patterns of care were described and univariable and multivariable models were performed to investigate the impact of prognostic factors on overall survival., Results: Among 223,404 patients with brain tumors in the NCDB, 868 patients were diagnosed with biopsy-proven neurocytoma and analyzed (0.4% or approximately 75 patients annually). Median age at diagnosis was 31 years and median tumor size was 4-5 cm. Diagnosis was similar between male (49.5%) and female (50.5%). Regarding location, 622 (72%) tumors were intraventricular, 168 (19%) were extra-ventricular, and 78 (9%) overlapping or unspecified. Five-year overall survival among all patients was 89%. On multivariable analysis tumor location, extent of resection, and use of radiation, were not predictive for improved survival (each p > 0.05); however, patient age (p < 0.001), WHO grade (p < 0.001), and medical comorbidity scores (p = 0.002) were independently associated with overall survival., Conclusion: Patients with central neurocytoma often present as young adults with sizable tumor burden and are well managed with surgery alone. Considering their favorable survival, efforts to improve tumor control should be carefully weighed against the long-term risks associated with adjuvant therapy like radiation., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Competing Interests: There are no conflicts of interest

Introduction:: Extraventricular neurocytoma is a rare neuronal tumor arising outside the ventricles. However, because of its rarity, its optimum treatment remains undefined., Materials and Methods: We intended to perform an individual patient data analysis to examine the patterns of care and prognostic factors involved in the treatment of extraventricular neurocytomas. PubMed, SCOPUS, and Google Scholar were searched with the following MeSH terms: "Neurocytoma, Extra ventricular neurocytoma, Spinal neurocytoma AND treatment, Survival" to find all possible publications pertaining to EVN., Results: From 108 publications, we retrieved 201 patients of extraventricular neurocytoma. Their median age was 30 years (range: 0.6-78 years). Sixty seven patients were in the pediatric (age ≤20 years) age group. There was a bimodal age distribution. Surgical details were available for 132 cases, and 51.5% underwent a gross total resection whereas 41.7% underwent a subtotal resection. Adjuvant radiation was used in 40% cases. For the entire cohort, the median progression free survival was 77 months (53.3-100.7). However, we could not find an impact of any of the prognostic factors on survival., Conclusion: An extraventricular neurocytoma is a very rare disease with varied presentations and different sites of origin. Gross total resection remains the standard of care. Adjuvant radiation may be used for salvage. However, radiation therapy after subtotal resection of an atypical neurocytoma may be administered., Competing Interests: There are no conflicts of interest

Studies comparing intraventricular oligodendroglioma (IVO) and central neurocytoma (CN) in terms of their clinical, radiological and pathological features are scarce. We, therefore, investigated the similarities and differences between these types of tumors to get a better understanding of how they may be more properly diagnosed and treated. The clinical manifestations, CT/MRI findings, pathological characteristics and clinical outcomes of 8 cases of IVOs and 12 cases of CNs were analyzed retrospectively. Both IVO and CN occurred most commonly in young adults and manifested with symptoms of increased intracranial pressure secondary to obstructive hydrocephalus. However, they were radiologically different in location (p = 0.007), diffusion-weighted imaging (p = 0.001), "scalloping" appearance (p = 0.006), flow void sign (p = 0.006) and ventricular wall invasion (p = 0.000). Histologically, significant differences in mitotic count (p = 0.008) and parenchymal infiltration (p = 0.01) were noted. Immunohistochemically, significant differences in the expression of Olig2 (p = 0.000), Syn (p = 0.01) and NeuN (p = 0.000) were observed. In addition, MIB-1 labeling index (p = 0.035) and case fatality rate (p = 0.021) of IVO were much higher than those of CN, while survival rate of IVO was much lower than that of CN (p = 0.028). IVO and CN are similar in onset age and clinical manifestations, but have different imaging and pathological features. Patients with IVOs may have a relatively poorer prognosis compared to those with CNs.

Purpose: To summarize the clinical and radiologic features of pediatric basal ganglia region tumors (PBGRT) in correlation with their histopathologic findings to reduce inappropriate surgery and identify tumors that can benefit from maximal safe resection., Methods: The records of 35 children with PBGRT treated in our hospital from December 2011 to December 2015 were analyzed retrospectively. The clinical and radiologic features of these tumors were summarized and correlated with their histopathologic diagnosis., Results: Our series included 15 astrocytomas and 11 germ cell tumors (GCTs). Basal ganglia astrocytomas were characterized by various clinical presentations and an ill-circumscribed mass with the involvement of surrounding structures on neuroimaging and mostly occurred in the first decade of life (n = 10; 66.7%). Basal ganglia GCT mostly occurred in the second decade of life (n = 8; 72.7%) with hemiparesis as the most common symptom (n = 9; 81.8%). The tumors were located predominantly in the caput of caudate nucleus (n = 8; 72.7%) with hemiatrophy as the typical sign (n = 8; 72.7%). Occasionally, other tumors also could occur in this region, including primitive neuroectodermal tumor (n = 1), atypical teratoid/rhabdoid tumor (n = 1), anaplastic ependymoma (n = 1), lymphoma (n = 1), extraventricular neurocytoma (n = 1), gangliogliomas (n = 2), oligodendroglioma (n = 1), and dysembryoplastic neuroepithelial tumor (n = 1)., Conclusions: Astrocytoma and GCT are the most common PBGRTs. Low-grade astrocytomas could benefit from maximal surgical resection, whereas GCTs merit neoadjuvant chemoradiation therapy followed by second-look surgery. We advocate routine testing of tumor markers and analysis of their clinical and radiologic features to optimize the therapeutic strategy., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Central neurocytomas are well-differentiated tumors of neuronal origin. These are relatively uncommon in the pediatric population. Anaplastic features reflected by brisk mitotic activity, microvascular proliferation, necrosis, and MIB-1 labeling index >2% or 3% have been proposed to indicate aggressive behavior. Because of its rarity, there is paucity of data regarding the histologic spectrum and outcome of central neurocytomas in children. With this short series, we describe our observations of the clinicopathologic characteristics and outcome of this tumor in children over a 5-year period.

We report a childhood case of thalamic atypical extraventricular neurocytoma that progressed to highly anaplastic ganglioglioma after 8 years of dormancy after subtotal resection and chemotherapy. The neurocytoma displayed immunoreactivity only for synaptophysin, β-catenin, S100, and CD56. The ganglioglioma acquired strong immunoreactivity for chromogranin, glial fibrillary acidic protein, neuron-specific enolase, and p53 and showed a very high proliferation rate approaching 50% in some areas. Tumor transformation was associated with overexpression of components of the sonic hedgehog and Wnt developmental signaling pathways, which are known to regulate tumor-initiating cells in malignant brain neoplasms., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background: In this report, we present the results of using upfront Gamma Knife surgery (GKS) in the management of giant central neurocytoma (CNC) (volume >50 mL) without the initial removal of the tumor mass., Case Descriptions: Two patients underwent GKS for histologically proven CNC. Clinical and imaging studies were performed to evaluate the response to treatment. GKS involved delivery doses of 12 or 13 Gy to the tumor margin at the isodose line of 50%. Tumor response to GKS appeared as early as 4-6 months after GKS, at which point a dramatic reduction in volume was observed. No adverse effects of radiation or new neurologic deficits were observed in either of the cases. In case 1, we observed a reduction in tumor volume from 69 to 20 mL at 6 months and a further reduction to 10.3 mL at 86 months. In case 2, we observed a reduction in tumor volume from 62 to 31 mL at 4 months with a further reduction to 22.5 mL at 30 months. The female patient (case 1) showed mild weakness in the right lower limb after the minimal surgical removal of tumor using the cortical approach. No additional neurologic deficits were observed after GKS. The young male patient (case 2) presented a complete recovery without any signs of headache at 3 months after GKS., Conclusions: Based on this initial experience, it appears that GKS is an effective treatment for CNC and may be used for upfront management in cases of indolent clinical symptoms, even when the tumor is very large., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Background & Aims: This study is designed for the clinical characteristics and prognostic factors of central neurocytoma (CN)., Methods: CN patients from 2004 to 2012 were enrolled from the Surveillance Epidemiology and End Results (SEER) data. Clinical characteristics including age, sex, race, tumor size, tumor number, surgery, and radiation therapy were summarized. Univariate and multivariate analysis were performed to explore the prognostic factors of CN., Results: CN tended to be borderline malignant and single lesion. Compared with other brain tumor (NCN), Patients with CN (CNs) were more likely to be female, young, and non-white race. Surgery was the primary treatment of CN. Univariate and Multivariate analysis indicated tumor number and surgery were both independent prognostic factors of CN (P < 0.05). Unifocal CNs had a lower mortality risk than multifocal ones (HR 0.167, 95% CI 0.052-0.537), surgery significantly reduced the death risk of CNs (HR 0.284, 95% CI 0.088-0.921)., Conclusions: CN tend to be borderline malignant, single lesion, operated on. Most CNs are female and younger. single lesion and surgery are the independent positive prognostic factors of CN.

Background: Central neurocytomas (CN) are rare pediatric CNS tumors most often with a benign clinical course. Occasionally, these tumors occur outside the ventricles and are called extraventricular neurocytomas (EVN). We present a retrospective institutional analysis of children with neurocytoma with prolonged follow-up., Procedure: Twelve patients were diagnosed with neurocytoma at our institution between 1993 and 2004., Results: Six patients were male and the median age at diagnosis was 12 years (1.5 to 16 y). Seven patients had CN and 5 had EVN. Presenting symptoms included headaches (67%), vomiting (50%), nausea (33%), seizures (33%), and mental status changes (25%). Obstructive hydrocephalus was present at diagnosis in 42% of the cases. Younger age and seizures were more common in patients with EVN. Gross total resection (GTR) was achieved in 42% (5/12) of the patients. Patients with GTR received no adjuvant therapy upfront; 1 patient subsequently had recurrence with leptomeningeal disease. Patients with subtotal resection received additional treatment: 1 underwent reoperation (GTR), 2 patients received focal radiation, 2 patients received adjuvant chemotherapy, and 2 patients received craniospinal irradiation followed by chemotherapy. The 20-year overall survival for this cohort was 83% with event free survival of 56%. Overall survival for CNs was 100%, versus 40% for EVN. Event free survival for CNs was 57% and 53% for the EVNs. An MIB-1 fraction >2% was associated with worse prognosis., Conclusions: Neurocytomas are rare brain tumors in children usually cured with GTR. Adjuvant focal radiation therapy and/or chemotherapy may improve disease control in cases with subtotal resection, but case-by-case analysis should be done. EVNs might be associated with worse outcome due to a higher proliferative index.

Central neurocytomas are uncommon intraventricular neoplasms whose optimal management remains controversial due to their rarity. We assessed outcomes for a historical cohort of neurocytoma patients and evaluated effects of tumor atypia, size, resection extent, and adjuvant radiotherapy. Progression-free survival (PFS) was measured by Kaplan-Meier and Cox proportional hazards methods. A total of 28 patients (15 males, 13 females) were treated between 1995 and 2014, with a median age at diagnosis of 26 years (range 5-61). Median follow-up was 62.2 months and 3 patients were lost to follow-up postoperatively. Thirteen patients experienced recurrent/progressive disease and 2-year PFS was 75% (95% CI 53-88%). Two-year PFS was 48% for MIB-1 labeling >4% versus 90% for ≤4% (HR 5.4, CI 2.2-27.8, p = 0.0026). Nine patients (32%) had gross total resections (GTR) and 19 (68%) had subtotal resections (STR). PFS for >80% resection was 83 versus 67% for ≤80% resection (HR 0.67, CI 0.23-2.0, p = 0.47). Three STR patients (16%) received adjuvant radiation which significantly improved overall PFS (p = 0.049). Estimated 5-year PFS was 67% for STR with radiotherapy versus 53% for STR without radiotherapy. Salvage therapy regimens were diverse and resulted in stable disease for 54% of patients and additional progression for 38 %. Two patients with neuropathology-confirmed atypical neurocytomas died at 4.3 and 113.4 months after initial surgery. For central neurocytomas, MIB-1 labeling index >4% is predictive of poorer outcome and our data suggest that adjuvant radiotherapy after STR may improve PFS. Most patients requiring salvage therapy will be stabilized and multiple modalities can be effectively utilized.

Central neurocytomas (CNs), initially asymptomatic, sometimes become huge before detection. We described and analyzed the clinical, radiological, operational and outcome data of 13 cases of huge intraventricular CNs, and discussed the treatment strategies in this study. All huge CNs (n=13) in our study were located in bilateral lateral ventricle with diameter ≥5.0 cm and had a broad-based attachment to at least one side of the ventricle wall. All patients received craniotomy to remove the tumor through transcallosal or transcortical approach and CNs were of typical histologic and immunohistochemical features. Adjuvant therapies including conventional radiation therapy (RT) or gamma knife radiosurgery (GKRS) were also performed postoperatively. Transcallosal and transcortical approaches were used in 8 and 5 patients, respectively. Two patients died within one month after operation and 3 patients with gross total resection (GTR) were additionally given a decompressive craniectomy (DC) and/or ventriculoperitoneal shunt (VPS) as the salvage therapy. Six patients received GTR(+RT) and 7 patients received subtotal resection (STR)(+GKRS). Eight patients suffered serious complications such as hydrocephalus, paralysis and seizure after operation, and patients who underwent GTR showed worse functional outcome [less Karnofsky performance scale (KPS) scores] than those having STR(+GKRS) during the follow-up period. The clinical outcome of huge CNs seemed not to be favorable as that described in previous reports. Surgical resection for huge CNs should be meticulously considered to guarantee the maximum safety. Better results were achieved in STR(+GKRS) compared with GTR(+RT) for huge CNs, suggesting that STR(+GKRS) may be a better treatment choice. The recurrent or residual tumor can be treated with GKRS effectively.

Similar histology and clinical behavior of both intraventricular central neurocytomas (CNs) and extraventricular neurocytomas (EVNs) may argue against the idea that EVNs were the distinct entity to distinguish from CNs in the 2007 World Health Organization classification. To explore respective characteristics and compare similarities and differences in CNs and EVNs, relevant clinical, radiological, operative and pathological data of 49 patients (35 CNs and 14 EVNs) in the Department of Neurosurgery at our hospital from 2005 to 2012 was reviewed and some comparisons between CNs and EVNs were conducted. The factors affecting posttreatment recurrence of CNs and EVNs were assessed by Cox regression analysis. In comparison, CNs showed a more typical clinical manifestation, and radiological and histopathological features, while EVNs demonstrated more malignant biological behavior, with higher MIB-1 index (p = 0.006), higher rate of atypia (p = 0.042), higher recurrence rate (p = 0.028), and shorter time to recurrence (p = 0.049). Subtotal resection was associated with higher rates of recurrence in both CNs (hazard ratio [HR] 6.16, p = 0.046) and EVNs (HR 5.26, p = 0.045), and atypia was also associated with a higher recurrence rate in CNs (HR 5.03, p = 0.042). CNs were thus easier to diagnose than EVNs, with typical clinical, radiological, and histopathological features, while the latter were more likely to show malignant biological behavior associated with atypia and recurrence. Total surgical resection is the optimal treatment choice for both CNs and EVNs, and patients with either CN or EVN with typical and/or totally resected lesions showed favorable clinical outcomes.

We report a case of an intraventricular tumor with features of atypical central neurocytoma with a sarcomatous component in a 44-year-old woman who presented with headaches and vomiting. Magnetic resonance imaging revealed a 3.4-cm lobulated enhancing mass in the occipital horn of the left lateral ventricle, and the patient subsequently underwent a left occipital-parietal craniotomy for debulking. The tumor contained 2 cell populations: round cells with perinuclear halos in a fibrillary background, and spindle cells with oval nuclei arranged in interlacing fascicles with focal necrosis. The round cells had diffuse synaptophysin immunopositivity, while the spindle cells were diffusely immunopositive for vimentin and had intercellular reticulin. The mitotic activity (8 mitotic figures per 10 high-power fields) and the high Ki-67 proliferation index (15.0%) were consistent with atypical central neurocytoma with a sarcomatous component. Although different histologic variants have been described, this is the first reported case, to our knowledge, of central neurocytoma with spindle cell sarcomatous features.

Central neurocytoma is a rare primary central nervous system tumour of young adults with good prognosis. Typical and atypical forms are described according to various histologic and histopathologic parameters. Central neurocytoma develops in the periventricular areas and is revealed by increased intracranial pressure. The tumour exhibits typical characteristics on CT scan and MRI and a characteristic peak of glycine on spectroscopy-MRI. The main treatment is total resection, which is achievable only in half of the cases. External beam therapy improves local control of partially resected and/or atypical central neurocytoma. Many studies show that stereotactic radiotherapy can be used in the therapeutic management as exclusive treatment, in postoperatives residues and in case of distant recurrence. Chemotherapy is the last line of treatment in refractory forms, especially in the forms with extracranial and/or neuromeningeal spread and in recurrent forms after treatment with surgery and/or radiotherapy., (Copyright © 2014 Société française de radiothérapie oncologique (SFRO). Published by Elsevier SAS. All rights reserved.)

Aims and Objective: Extra ventricular neurocytoms (EVN) are a rare parenchymal brain tumour distinct from central neurocytomas. This review attempts to analyze a series of seven cases of EVN from a single institute against the background of available literature., Method: Retrospective analysis of the clinical manifestation, pathological features, imaging findings, microsurgical treatment effectiveness and progression free survival of seven cases of EVN operated between 2000-2012., Results: The study group included five females and two males in the age group 7-65 years (mean 30.71 yrs). The distribution of the lesion was as follows Lobar 4, Cerebellum 1, Pineal 1, spinal 1. The duration of clinical symptoms ranged from three months to seven years and the presentation was site and size specific. Cystic Changes (71 %), perilesional oedema (57 %) and heterogeneous contrast enhancement (85.7 %), and calcification (42 %) were a few distinct imaging characteristics. Increased perfusion correlated with atypical histology in two patients. Surgical resection remains the main management strategy for longer outcome and patients with STR + RT and GTR had comparable follow-up. Extremes of age and atypical histological features were adverse prognostic factors., Conclusion: EVN are rare tumors with either glial or neuronal differentiation or a potential for atypical changes. Both, GTR and STR combined with RT, offer reasonably good outcome with overall comparable survival. The available literature of EVN is limited, larger series with long term follow-ups are needed to dictate the management protocol. The role of adjuvant therapy is still debatable and needs further validation.

Central neurocytomas are rare benign tumors of the central nervous system that are typically located in the lateral ventricles. Since they were first reported in the early 1980s, many advancements have been made in terms of their diagnosis and treatment. Despite the progress made, the origin of these rare tumors and effective newer treatment strategies remain elusive. Central neurocytomas represent 0.1-0.5% of all primary brain tumors. Since they are typically intraventricular, these tumors tend to present clinically with hydrocephalus. CT scanning and MRI are useful in localizing these tumors; however, due to their numerous ambiguous features, the ultimate diagnosis relies on immunohistochemistry and electron microscopy studies of sampled tissue. Currently, surgical removal with a gross-total resection of these tumors is the treatment of choice. Various radiotherapy techniques, including both conventional radiotherapy and stereotactic radiosurgery, have been shown to be useful in cases of residual tumor after sub-total resection and tumor recurrence. The benign nature of these tumors tends to offer a favorable outcome for most patients; however, recurrence rates are relatively high and tumors with high-grade features or extraventricular location tend to have a less favorable prognosis. We present a comprehensive review of these rare tumors, including their epidemiology, clinical presentation, radiological presentation, histopathological findings, and options for intervention including surgery, radiation therapy, stereotactic radiosurgery, and chemotherapy., (Copyright © 2013 Elsevier Ltd. All rights reserved.)

To describe the clinical and imaging characteristics of patients with central neurocytoma (CN), we reviewed data on 27 patients who had histologically confirmed CN and were treated in our institution between 1999 and 2010. Neuro-imaging findings on CT scan (n=18) and MRI (n=25) were retrospectively evaluated. There were 15 males and 12 females with a mean age of 29 years (range, 11-46 years). The most frequent presentations included headache (n=21) and vomiting (n=6). Tumor sites included bilateral lateral ventricles (n=10), right lateral ventricle (n=7), left lateral ventricle (n=7) and fourth ventricle (n=3). On MRI, the T1-weighted signal was hypointense in 12 patients and isointense in 13, and the T2-weighted signal was isointense in 8 patients and hyperintense in 15. CT scans/MRI revealed a cystic component in 18 patients. Tumors showed a mild to marked enhancement in 26 patients. Flow voids from tumor vessels on MRI were present in 14 patients, and calcification was noted in six of 18 patients with CT scans. All lateral ventricle tumors were resected through a transcortical or transcallosal approach. Gross total resection was achieved in 19 patients, near total in two and subtotal in six. One patient died of cerebral infarction in the perioperative period. At the last follow up, there were three known clinical recurrences in this series. However, no recurrence was noted in 17 patients who underwent gross total resection with no adjuvant therapy., (Copyright © 2012 Elsevier Ltd. All rights reserved.)

Background: A thorough investigation of the long-term outcomes of central neurocytoma (CN) after different treatments is required to establish optimal management strategies., Objective: We retrospectively reviewed the long-term clinical outcomes of patients with CN according to various treatments and suggest treatment strategies based on 30 years of experience in a single institution., Methods: Fifty-eight consecutive patients with CN were treated at our institution between 1982 and 2008. Patient demographics, overall survival, local control rates according to multimodal treatments, and functional outcomes were evaluated. The mean clinical and radiological follow-up periods were 119 months (range, 18-304 months) and 98 months (range, 13-245 months), respectively., Results: The initial treatment modality was classified into 4 subgroups: operation only (34 patients), operation followed by radiation therapy (7 patients) or radiosurgery (7 patients), and radiosurgery alone (10 patients). The actuarial overall survival was 91% at 5 years and 88% at 10 years. The actuarial overall survival and local tumor control rate did not differ significantly according to the various treatments and the initial extent of the surgical resection. However, functional outcomes, such as the postoperative seizure outcome at the last follow-up, differed according to the surgical approach., Conclusion: The long-term clinical outcomes of CN after multimodal treatment seem to be excellent. Our study suggests that treatment strategies for CN should focus on the patient's quality of life, as well as on tumor control, because of the benign nature of CN.

Background: Neurocytoma represents a rare tumor of the central nervous system usually slowly growing and generally with good prognosis after surgical resection with or without adjuvant radiotherapy., Case: A 25-year- old woman presented with sudden fainting. During the initial workup, brain CT was completed with finding of tumor inside the third ventricle spreading into both lateral ventricles. The patient underwent partial surgical resection followed by radical gross resection, no adjuvant radiotherapy was indicated during the initial treatment and the patient was followed up with regular brain MRIs and clinical examinations. Thirty six months after the initial resection, there was progression on MRI and radiotherapy was recommended. At this moment, patient is 12 months after radiotherapy with stable disease on MRI and with good stable performance status., Conclusion: One of the greatest problems in the management of neurocytoma is the timing of adjuvant radiotherapy. From published data, it is clear that adjuvant radiotherapy increases local control; however, this has to be considered carefully against the possible risks from late side effects of radiotherapy considering long-time survival of the patients.

Central neurocytomas (CN) are rare intraventricular tumors with prominent neuronal differentiation. CN commonly arise in the lateral ventricles of young adults who predominantly present with raised intracranial pressure. Few studies have described the clinical, pathological, and radiological features of these tumors, and those that have are typically single case reports. Herein, we report ten patients with CN with variable clinical and pathological features and discuss the management of these tumors. Nine tumors occupied the lateral ventricle and only one was located in the sellar region. On MRI, all 10 tumors showed heterogeneous hypo-or iso-intensity on T1-weighted and hyperintensity on T2-weighted MRI. Contrast enhancement varied greatly from very slight to intense. All patients were surgically treated by macroscopic total or subtotal removal. Postoperative radiotherapy was given to six patients (four of whom had undergone subtotal resection and two of whom had undergone total resection). The surgical and histopathological data of these patients were reviewed and analyzed. No recurrences were noted although we were unable to contact two patients for follow-up. A brief review of the literature concerning differential diagnosis and therapeutic aspects of these tumors is also presented., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Object: The literature, at present, provides limited information about extraventricular neurocytomas (EVNs) and is almost exclusively composed of case reports or small case series. Treatment for EVNs has largely been guided by results from central neurocytoma outcome studies. The authors present an analysis of all reported intracranial EVN cases to establish if tumor histopathological features can substratify EVN into groups with differing prognosis and help guide treatment decisions., Methods: The authors identified studies reporting histology, treatment modality, and outcomes for patients with intracranial EVN. The rates of recurrence and survival for patients were compared using Kaplan-Meier analysis. Atypical tumors, defined by MIB-1 labeling index exceeding 3% or atypical histological features, were compared with typical tumors, and patients 50 years of age or older were compared with those younger than 50 years of age., Results: Eighty-five patients met the inclusion criteria, and 27% of them had an atypical histology. Typical EVNs had a better prognosis than atypical EVNs after primary treatment, with a 5-year recurrence rate of 36% compared with 68% (p < 0.001), and a 5-year mortality rate of 4% compared with 44%, respectively (p < 0.001). Age younger 50 years was associated with a better prognosis than age equal to or greater than 50 years, with a 5-year recurrence rate of 33% and 74%, respectively (p < 0.001), and a 5-year mortality rate of 4% and 52%, respectively (p < 0.001). Multivariate analysis demonstrated that atypical EVNs carried significantly increased risk for recurrence (hazard ratio [HR] 4.91, p < 0.001) and death (HR 22.91, p < 0.01). Gross-total resection was superior to subtotal resection (STR) alone in tumor control rates for typical EVNs (95% and 68%, p < 0.05), and there was a trend for adjuvant external-beam radiotherapy to benefit STR. There was suggestion of similar trends in patients with atypical EVNs., Conclusions: There are at least 2 distinct histological subtypes of EVN, with different prognostic significances. Atypia or MIB-1 labeling index greater than 3% is a significant predictor of poor prognosis for EVNs. Complete resection or more aggressive attempts at providing adjuvant therapy following STR appear to improve the prognosis for patients with EVNs. Although the authors' results are informative, there are limitations to their analysis. Given the relatively modest total number of cases reported, as well as the nature of the disaggregated analysis, the authors were not able to use formal meta-analytical methods to limit the impact of between center heterogeneity. Additionally, they were not able to control for individual differences in data analysis and presentation across the different studies included in their analysis.

There is a lack of studies reporting on outcomes of control and treatment toxicities for neurocytomas. A 25-year retrospective review of a tertiary center's experience with neurocytomas was completed to report on these outcomes. All cerebral neurocytoma cases (19 patients; median age, 31 years; range, 18-62 years; 18 intraventricular and 1 extraventricular) treated between 1984 and 2009 were analyzed, including central pathology and radiology reviews. Median follow-up was 104.5 months (range, 0.75-261.7 months). Primary treatment was surgery alone (n = 18 patients), followed by surgery and adjuvant radiotherapy (n = 1). The crude local control rate after surgery was 68% for all cases (cerebral neurocytomas) and 74% for central neurocytomas. Salvage therapies included further surgery (n = 4), radiation (n = 3), and chemotherapy (n = 1). Ten-year Kaplan-Meier overall and relapse-free survival rates were 82% and 62% and 81% and 57%, respectively, for all cases and for central neurocytomas only. The median overall survival and relapse-free survival were 104.5 and 79.3 months, respectively, for all cases and for central neurocytomas. Ten patients had grade 3/4 toxicity, and 1 patient had a grade 5 perioperative hemorrhage that resulted in death 23 days after surgery. Late grade 3/4 toxicities occurred in 9 patients. Three patients had permanent grade 2 motor or cognitive deficits. We provide the first report outlining toxicities and survival outcomes in a series of 19 patients. Our experience suggests that initial surgery provides durable local control rates in two-thirds of patients, with low risk for significant permanent deficits. Salvage therapy with surgery and/or radiation provides durable local control in tumors that recur after surgery.

(18)F-FDOPA is an amino acid analogue used to evaluate presynaptic dopaminergic activity, which has aroused great interest in neuro-oncology. We have evaluated five (18)F-FDOPA PET studies of patients referred for study of parkinsonian syndrome. Two subjects had previously treated high-grade brain tumors, one nonspecific brain injury, and 2 subjects presented unexpected tumoral lesions. For all lesions SUVmax, time to SUVmax and tumor-to-normal grey matter SUVmax rate (T/N) were calculated, and 90 minutes (18)F-FDOPA kinetics were analyzed. Tumor lesions corresponded to three malignant neurocytomas, one meningioma, one pineocytoma and one intrasinusal hemangioma. Both malignant and benign tumors exhibited high uptake of (18)F-FDOPA well above the normal cortex. However, the analysis of the curve uptake displayed characteristic patterns that facilitate the characterization of tumor lesions. A dual phase maximum uptake was observed, with an early 10 minutes uptake in malignant lesions, and a late 60 to 90 minutes uptake in benign or low grade lesions., (Copyright © 2010 Elsevier España, S.L. y SEMNIM. All rights reserved.)

Two new cases of extraventricular neurocytoma are reported. The first concerns an 18-year-old female with a left frontal lobe lesion and the second occurred in a 54-year-old female in the thoracic spinal cord. The first patient is free of disease after 18 months of follow up. The second patient is stable after 16 months of follow up. Both were operated - total excision in Case 1 and partial resection in Case 2. The latter underwent adjuvant radiotherapy due to atypical histological features. Cases of extraventricular neurocytoma are reviewed. Differences and similarities between extraventricular and central neurocytoma are outlined.

Benign central neurocytoma (CN) is a rare neuronal tumour of the central nervous system recognised since the early eighties. More than 300 articles have been published in the literature, mostly comprising of case reports and short series from individual specialties. These tumours, though normally benign, are more often likely to recur after surgery than previously thought. A multi-modality team involvement, therefore, has become increasingly necessary for their optimum management. In this article, the authors from various neurosciences sub-specialties, with a specific interest and experience in managing CN, review the epidemiology, clinical features, pathological findings, radiological characteristics and surgical treatment, with an emphasis on the latest developments in their histology, molecular biology and adjuvant treatment modalities for recurrent or residual disease.

The spinal cord is a very rare location for extra-ventricular neurocytomas. Composed of small round cells with neuronal differentiation, neurocytoma has a particularly favourable prognosis. Long disease-free intervals have been observed in patients who received sub-total removal and radiotherapy. A careful intra-operative frozen section examination is helpful in avoiding excessive surgical tissue disturbance and to identify the best treatment option. To date, eight cases of neurocytoma of the spinal cord have been described. We report three more patients; one with very long survival, and review the published literature for this condition.

Neurocytomas are typically located within the supratentorial ventricular system. Extraventricular neurocytoma is a rare brain tumour that poses diagnostic difficulty. We report a case of cerebral extraventricular neurocytoma. A 44-year-old man presented with a 6-month history of tonic-clonic seizures. Magnetic resonance imaging demonstrated a left frontoparietal mass lesion. The patient underwent surgical therapy, and a radical excision was performed. The histological appearance and immuno-histochemical findings of the tumour are consistent with an extraventricular neurocytoma with anaplastic features. An excellent prognosis can be expected if a gross total resection is achieved. Postoperatively, radiotherapy and chemotherapy may be used as adjuvant therapies.

Melanotic or melanocytic medulloblastoma is a rare variant of medulloblastoma, especially when the tumor shows advanced neuronal differentiation. We report a case of this tumor, which developed in the cerebellar vermis in an 8-year-old girl. Initial biopsy specimens were identified as classical medulloblastoma with a high MIB1 index. Surgical removal of the tumor was performed after chemo-radiotherapy, and black pigments were noticed on the tumor surface. Histologically, the tumor was composed of classical medulloblastoma with the presence of pigmented epithelial cells forming tubules and clusters. Immunohistochemically, the pigmented tumor cells were positive for S100 protein, HMB45, and MART1, indicating that the pigments were derived from melanosomes, and these features were compatible with melanocytic medulloblastoma. Interestingly, some of the non-pigmented or amelanotic tumor cells were also positive for HMB45 and S100 protein. Although the tumor showed an unusual cell combination, it was distinguished from atypical teratoid/rhabdoid tumor (AT/RT) by nuclear expression of INI1/BAF45 protein. The tumor also possessed ganglion-like cells within the neuropil matrix, which resembled small mature ganglion cells, and was consequently designated as ganglioneurocytoma. The melanotic medulloblastoma and part of the ganglioneurocytomatous area were fused with each other. Hence, the present case provides new information indicating that melanocytic medulloblastoma differs from AT/RT, and that it can exhibit advanced neuronal differentiation. In addition, reduction of the tumor MIB1 index was observed after chemo-radiotherapy.

Background: Central neurocytoma (CN) represents a rare, relatively recently described primary central nervous system tumor. It ranks among intraventricular tumors due to its predominant location within the lateral brain ventricles. CN occurs mostly in young adults around the 3rd decade of life; almost a fifth of the cases are children under 18 years of age., Objectives: The authors present three cases of patients with histopathologically confirmed CN, emphasizing diagnostic imaging issues. A review of the literature concerning differential diagnosis and clinical and therapeutic aspects is also presented., Conclusion: Literature reports of CN comprise most likely case reports, small cohorts of patients, and meta-analytic studies due to the generally low incidence of this tumor. In the current paper, the authors summarize up-to-date knowledge of this rare disease on the background of their own observations. CN should be included in the list for differential diagnostics of intraventricular brain tumors, especially those located in lateral ventricles.

Background: Reviewing the literature on central neurocytoma revealed that functional outcomes traditionally have been assessed by KPS. However, KPS is not sufficiently sensitive to assess functional outcomes compared with the Barthel Index and the Functional Independence Measure (the FIM instrument). Discussion of other functional assessments and various aspects of central neurocytomas has never been documented clearly., Methods: Nine case series of primary central neurocytomas received total or STR at our hospital. The clinical features, neuroradiologic findings, histopathologic features, and functional outcomes were listed and analyzed. Moreover, the functional outcomes were assessed using KPS, Barthel Index, and FIM instrument. The follow-up period ranged from 8 to 33 months after surgery., Results: Patients with increased GFAP positivity or MIB-1 index greater than 3% did not have the worst scores in functional assessments. The functional outcomes of the cases presented here, including KPS, Barthel Index, and FIM instrument, were listed in Table 4. Most patients achieved good functional outcomes. The average KPS, Barthel Index, and FIM instrument were 86.25 +/- 14.08 (range, 60-100), 86.88 +/- 17.31 (range, 50-100), and 111 +/- 17.02 (range, 78-126), respectively., Conclusion: Most of the patients in this investigation achieved good ability to independently perform daily activity. Whether the high score of functional outcome was correlated with benign tumor course or 5-year survival rate remains uncertain. We recommend regular follow-up for detecting tumor growth or recurrence, although central neurocytomas are always benign.

Purpose: Central neurocytoma is a rare intraventricular brain tumor that affects young adults and presents with increased intracranial pressure secondary to obstructive hydrocephalus. Typically, it has a favorable prognosis after adequate surgical intervention, but in some cases the clinical course is more aggressive. In this report, we describe the diagnosis and treatment of central neurocytoma in a series of patients at our institution., Patients and Methods: Our series of nine patients (M:F=2:7, mean age, 28.2 years) with ventricular tumors showed typical radiological, histologic and immunohistochemical features of central neurocytoma. Most patients received craniotomy with removal of the tumor through transcallosal or transcortical approach. The surgical and histopathologic data of these patients were reviewed and analyzed., Results: The prognosis is generally favorable. Although most patients were alive and well at the last follow-up, two developed recurrence. Typical histologic features of recurrent neurocytoma include high proliferative activity (MIB-1 labeling index: 2.0-6.8%), prominent vascular proliferation and remarkable synaptophysin expression. Two patients (non-recurrent) died during follow-up due to sepsis or central failure. The MIB-1 labeling indices were as high as 2.2-5.4% for these two patients., Conclusion: Although central neurocytoma is generally a benign neoplasm, some variant forms of recurrence are also present. Complete resection provides favorable long-term prognosis in most cases. Recurrent tumors are often local and the patients seem to recover well after a second resection followed by radiotherapy. Histologic features such as tumor proliferation (MIB-1 labeling index), vascular proliferation, and synaptophysin expression are often prominent in the recurrent tumor. We recommend that these histologic features be considered for tumor recurrence during treatment and follow-up of these patients.

Purpose: To examine the outcomes of patients with histologically confirmed central neurocytomas., Methods and Materials: The data from 45 patients with central neurocytomas diagnosed between 1971 and 2003 were retrospectively evaluated. Various combinations of surgery, radiotherapy (RT), and chemotherapy had been used for treatment., Results: The median follow-up was 10.0 years. The 10-year overall survival and local control rate was 83% and 60%, respectively. Patients whose tumor had a mitotic index of <3 (per 10 high-power fields) experienced a 10-year survival and local control rate of 89% and 74%, respectively, compared with 57% (p = 0.040) and 46% (p = 0.14) for patients with a tumor mitotic index of > or =3. The 10-year survival and local control rate was 90% and 74% for patients with typical tumors compared with 63% (p = 0.055) and 46% (p = 0.41) for those with atypical tumors. A comparison of gross total resection with subtotal resection showed no significant difference in survival or local control. Postoperative RT improved local control at 10 years (75% with RT vs. 51% without RT, p = 0.045); however, this did not translate into a survival benefit. No 1p19q deletions were found in the 19 tumors tested., Conclusion: Although the overall prognosis is quite favorable, one-third of patients experienced tumor recurrence or progression at 10 years, regardless of the extent of the initial resection. Postoperative RT significantly improved local control but not survival, most likely because of the effectiveness of salvage RT. For incompletely resected atypical tumors and/or those with a high mitotic index, consideration should be given to adjuvant RT because of the more aggressive nature.

We report an unusual case of extraventricular (cerebral) neurocytoma with a long, 25-year history, and which appeared to transform to neuroblastoma. In 1978, an 18-year-old woman was treated for right frontal oligodendroglioma. Eighteen years later (in 1996), recurrence of tumor in the fourth ventricle was noted and was treated with gamma-knife radiotherapy. The tumor shrunk transiently, but 7 years later (in 2004), MRI study demonstrated a second recurrence and ventricular dissemination. Partial removal was performed, and histological examination revealed that tumor cells had round or oval nuclei with halos. Frequent mitoses and vascular proliferation were observed. The MIB-1 LI was 80%. Despite postoperative whole-brain radiotherapy to a total dose of 30 Gy, the tumor progressed, and she died at 4 months after the second surgery.

Objective and Importance: Central neurocytoma is recognized as a indolent intraventricular tumor arising from the ependyma around the foramen of Monro and anterior part of the lateral ventricles, and well demarcated from the brain parenchyma. Surgical removal can be curative without postoperative therapy. However, malignant central neurocytoma refractory to even aggressive treatment is known., Clinical Presentation: We report two cases of extraventricular central neurocytomas with significant vascular proliferation, mitoses, and MIB-1 labeling index of more than 10%., Intervention: Subtotal removal for the one patient and open biopsy for other followed by radiotherapy with chemotherapy were performed. However, the disease progressed and dissemination occurred. Both patients subsequently died 23 and 18 month after the histological diagnosis was established., Conclusion: Extraventricular central neurocytoma may present with frequent vascular proliferation and high MIB-1 labeling index. Even if they lack malignant histological findings like frequent mitosis and/or necrosis, the prognosis for such patients is very poor.