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Extraventricular neurocytoma at the sellar region (EVNSR) is an exceedingly rare tumor. Given the paucity of specificity and its peculiar nature, our study aimed to characterize the clinical, imaging, and pathological features, including treatment and clinical outcomes of this tumor.Eight patients with pathologically confirmed EVNSR at Beijing Tiantan Hospital from 2012 to 2020 were retrospectively analyzed. Additionally, 7 cases from the prior detailed literature were also retrieved.Among the 8 patients from Beijing Tiantan Hospital, 2 males and 6 females with an average age of 45.3 (range, 8-61). Vision impairment and headache were the most common complaints at presentation. Preoperative diagnoses were pituitary adenoma (n = 6), meningioma (n = 1), and oligodendroglioma (n = 1). Treatment included subtotal tumor resection (n = 3), partial resection (n = 5), adjuvant therapy covered radiotherapy (n = 2), and gamma knife surgery (n = 3). The clinical outcomes of these 8 cases were stable (n = 5), survival after progression (n = 1), and death after recurrence (n = 2).EVNSRs are extremely rare tumors, and most have benign prognoses after appropriate treatment. Due to the unique location, total removal of the tumor is challenging; And adjuvant radiation therapy for eligible patients is recommended. Regular imaging review is also advised. Future studies with more patients are needed to elucidate the biological nature of EVNSRs.

Subependymal giant cell astrocytoma (SEGA) is a World Health Organization grade 1 neoplasm, which, due to its dubious morphologic features, may be misdiagnosed as a high-grade tumor at times. This tumor shows binary immunoexpression including both glial and neural markers, leading to a state of diagnostic quandary. Recent evidences have surmised the diagnostic utility of thyroid transcription factor 1 (TTF-1), spurring us to study the practicality of this marker in distinguishing SEGAs from its mimics.In this study, TTF-1 immunohistochemistry using clone 8G7G3/1 (1:50) was performed in 38 cases of SEGA, 30 cases of central neurocytoma, 10 cases each of intraventricular glioblastoma and ependymoma, and 5 cases of cortical tubers. Additionally, serine/threonine-protein kinase B-Raf (BRAFV600E) mutation, a common genetic alteration in pediatric low-grade-glial tumors with neuronal-differentiation, was analyzed using Ventana immunohistochemistry platform.TTF-1 immunopositivity was seen in all 38 cases (100%) of SEGAs, with 20 cases (52.6%) showing diffuse (50% of tumor area) expression while focal (50%) immunopositivity was seen in 18 cases (47.3%). None of the cases demonstrated serine/threonine-protein kinase B-Raf immunolabeling. Barring 2 cases of neurocytoma (6.6%), all other cases including ependymoma, glioblastoma, and cortical tubers were immunonegative for TTF-1.The congruous finding of TTF-1 expression in SEGA and cells of the developing neuroepithelium in the medial ganglionic eminence hint toward a primogenitor cell with neoplastic potential in the presence of impelling factors.

To investigate the efficacy and safety of adjuvant radiotherapy (RT) in patients with central neurocytoma (CN).The study included 68 patients with CN retrospectively, was further divided into surgery + RT group (31 patients) and surgery alone group (37 patients). Progression-free survival (PFS), overall survival (OS), and adverse reactions (AEs) were compared between the two groups.The median follow-up duration was 82.2 (interquartile range, 64.7-104.5) months. Patients in the surgery + RT group tended to have longer PFS than those in the surgery alone group (5-year PFS rate: 92.7% vs. 86.3%; P = 0.074). There was no significant difference in OS between the two groups (5-year OS rate: 96.8% vs. 94.3%; P = 0.639). Subgroup analysis revealed a significant improvement in PFS in patients receiving RT after surgery in patients who underwent subtotal resection (STR) (P = 0.045). In the overall population, univariate multivariate analysis revealed that gross total resection (GTR) (P = 0.002), tumor location in the unilateral ventricle (P = 0.008), and MIB-1 (Ki-67) labeling index (LI) 5% (P = 0.009) were favorable independent prognostic factors for PFS. Whereas tumor location in the unilateral ventricle (P = 0.043) was a favorable independent prognostic factor for OS. Moreover, RT patients experienced AEs (Grade 1-2, well-tolerated).Adjuvant RT in the treatment of CNs showed satisfactory safety, and postoperative RT could improve PFS in STR patients. Furthermore, GTR, tumor development in the unilateral ventricle, and MIB-1 LI 5% were found to be favorable factors affecting the prognosis of CNs.

Neurocytomas are rare low-grade brain tumors predominantly affecting young adults, but their cellular origin and molecular pathogenesis is largely unknown. We previously reported a sellar neurocytoma that secreted excess arginine vasopressin causing syndrome of inappropriate anti-diuretic hormone (SIADH). Whole exome sequencing in 21 neurocytoma tumor tissues identified somatic mutations in the plant homeodomain finger protein 14 (PHF14) in 3/21 (14%) tumors. Of these mutations, two were missense mutations and 4 caused splicing site losses, resulting in PHF14 dysfunction. Employing shRNA-mediated knockdown and CRISPR/Cas9-based knockout approaches, we demonstrated that loss of PHF14 increased proliferation and colony formation in five different human, mouse and rat mesenchymal and differentiated cell lines. Additionally, we demonstrated that PHF14 depletion resulted in upregulation of platelet derived growth factor receptor-alpha (PDGFRα) mRNA and protein in neuroblastoma SHSY-5Y cells and led to increased sensitivity to treatment with the PDGFR inhibitor Sunitinib. Furthermore, in a neurocytoma primary culture harboring splicing loss PHF14 mutations, overexpression of wild-type PHF14 and sunitinib treatment inhibited cell proliferation. Nude mice, inoculated with PHF14 knockout SHSY-5Y cells developed earlier and larger tumors than control cell-inoculated mice and Sunitinib administration caused greater tumor suppression in mice harboring PHF-14 knockout than control SHSY-5Y cells. Altogether our studies identified mutations of PHF14 in 14% of neurocytomas, demonstrate it can serve as an alternative pathway for certain cancerous behavior, and suggest a potential role for Sunitinib treatment in some patients with residual/recurrent neurocytoma.

Central neurocytomas (CNs) are extremely rare tumors that account for 0.1-0.5% of all intracranial neoplasms. Recently, Gamma Knife radiosurgery (GKRS) has become a treatment option in patients with CN. We aimed to evaluate the efficacy and safety of GKRS in 25 CN patients and review the results along with relevant literature. GKRS patient database was searched, and 25 patients who underwent GKRS for CN between 2009 and 2018, were evaluated retrospectively. The study cohort included 15 female and ten male patients with a median age of 32 years (range, 5-60). The most common presenting symptom was headache (88%). The neurological examination was unremarkable in all patients, except for one patient with decreased vision. Twenty patients (80%) had a history of surgical resection. Most of the tumors (92%) were located in the ventricles, and the median tumor volume was 4.8 cm(3) (range, 0.8-28.1). The median marginal dose was 14 Gy (range, 12-15) to a median isodose of 50% (range, 40-50). Following a median follow-up of 80 months (range, 36-138), local tumor control was achieved in 100% of patients. Distant recurrence was observed in one patient (4%). No adverse radiation effect was observed. Regarding non-specific post-GKRS symptoms, one patient experienced a prolonged headache, and one epileptic patient experienced a brief partial seizure. In our patient cohort, GKRS yielded favorable local tumor control (100%) during a median follow-up of 6.6 years. Our series demonstrates that GKRS is an effective and safe treatment option for patients with primary or residual CNs.

Central neurocytoma is a rare benign brain tumor that typically arises from the subependymal lining of the lateral ventricles in young adults and is generally associated with excellent survival following neurosurgical excision alone. This is a retrospective clinical audit of biopsy-proven neurocytoma registered between 2004 and 2019 at a single institution in India. All time-to event outcomes were analyzed using Kaplan-Meier method and compared with the log-rank test. Any p-value0.05 was considered statistically significant. A total of 66 patients with neurocytoma were included in the descriptive analysis. Median age of study cohort was 31 years with equitable gender ratio. Majority (83%) of tumors were intraventricular, lateral ventricle being the commonest location. Following maximal safe resection, patients were generally kept on close clinico-radiological surveillance. Most patients (80%) had typical World Health Organization (WHO) grade II neurocytoma with remaining 20% showing histological atypia and/or high-grade features. Outcome analysis was restricted to 35 patients with relevant treatment details and adequate follow-up information. Six patients experienced recurrent/progressive disease with 2 documented deaths. At a median follow up of 52 months, 5-year Kaplan-Meier estimates of progression-free survival and overall survival were 93.3% and 96.8% respectively. Three patients developed delayed recurrence (5-years after initial diagnosis) underscoring the importance of long-term follow-up. Atypical/high-grade histology was associated with inferior survival that may stand to benefit with upfront adjuvant radiotherapy. This represents the largest single-institution series of central neurocytoma and demonstrates excellent outcomes with adequate surgical resection alone, reserving radiotherapy for large residual tumor, recurrent disease, and/or atypical high-grade histology.

Purpose To highlight the clinical, neuroradiographic, neuropathologic, and molecular features of histologically identified neurocytoma in a pediatric cohort and highlight the evolving use methylation profiling in providing diagnostic clarity in difficult to diagnosis pediatric brain tumors. Methods Five consecutive children (ages 9–13, 2 girls 3 boys) were histologically diagnosed with neurocytoma at Rady Children’s Hospital San Diego from 2012 to 2018. Clinical and molecular features were analyzed with regards to treatment course and outcome. Results Presenting symptoms included seizures (n = 2), syncope (n = 1), headache (n = 2), visual disturbances (n = 2) and emesis (n = 2). Tumor location included intraventricular (n = 2), intraventricular with parenchymal spread (n = 1), and extraventricular (n = 2). Magnetic resonance imaging demonstrated reduced diffusivity (2/5), signal abnormality on susceptibility-weighted sequences (3/5), and varying degrees of contrast enhancement (4/5). All patients underwent surgical resection alone. Recurrence occurred in four children that were treated with surgery (4/4), adjuvant radiation (2/4), and chemoradiation (1/4). Neuropathologic features included positivity for GFAP (4/5), synaptophysin (4/5), NSE (2/2), NeuN (4/4), and variable Ki-67 ( Conclusion Neurocytomas are a rare clinical entity that warrants further investigation into molecular and pathologic prognosticating features. Methylation profiling may aid in differentiation of neurocytoma from other difficult to diagnose tumors who share similar histologic features.

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.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.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.

ObjectiveTo investigate the clinicopathological characteristics, molecular genetic characteristics and prognosis of extraventricular neurocytoma located in the sellar/suprasellar region.MethodsSeven archived tumor samples derived from 4 patients with neurocytoma in the sellar/suprasellar region were collected from the First Affiliated Hospital of Fujian Medical University and the Affiliated Hospital of Qingdao University and retrospectively analyzed for clinical manifestations, imaging features, and histopathological features. Neuronal and pituitary biomarkers and molecular features were detected in these tumor tissues by immunohistochemistry and FISH or Sanger sequencing. The related literature was reviewed.ResultsThree patients were female, while 1 was male, with an average age of 35.5 years (range: 27 to 45 years). The initial manifestations were mainly headache and blurred vision in both eyes. The first MRI examination showed marginally enhancing masses in the intrasellar or intra- to suprasellar region. The diagnosis of pituitary adenomas was based on imaging features. The levels of pituitary hormones were normal. Histologically, the tumor cells were arranged in a sheet-like, monotonous architecture and were uniform in size and shape with round to oval, exquisite and hyperchromatic nuclei, which densely packed close to one another and were separated only by a delicate neuropil background. There was no evident mitosis, necrosis or microvascular proliferation. The three cases of recurrent tumors were highly cellular and showed increased mitotic activity. Immunohistochemically, the tumor cells were positive for syn, CR, CgA, and vasopressin and were focally positive for NeuN, TTF-1, NF, CK8, vimentin, and S100 proteins. Other markers, including IDH1, BRAF VE1, Olig-2, and EMA, were negative. Pituitary transcription factors and anterior pituitary hormones were negative. Molecular genetic testing showed that the tumor cells lacked IDH gene mutations, LOH of 1p/19q, MYCN amplification, and EGFR alteration. With a median follow-up of 74.5 months (range 23 to 137 months), 3 patients relapsed at 11, 50, and 118 months after the initial surgery.ConclusionThe morphological features and immunophenotypes of neurocytoma in the sellar/suprasellar region are similar to those of classic central neurocytoma. The prognosis is relatively good. Gross-subtotal resection and atypical subtype may be related to tumor recurrence.

To determine the MR (magnetic resonance), pathologic, and clinical findings of extraventricular neurocytoma (EVN).Descriptive study.Department of Radiology, Affiliated Hospital of Jining Medical University, Jining, Shandong, China, from January 2020 to March 2022.The MR radiological and pathological data of 11 patients with EVNs proved by histopathology after surgery were analysed retrospectively. Above-mentioned features were studied.There were 5 men and 6 women, ages ranging from 16 to 56 years. Seven cases (63.6%) were located in the cerebral hemisphere, three cases (27.3%) in the cerebellar hemisphere, and one case in cervical cord. Ten cases (91.0%) were cystic-solid, and one case was predominantly solid with small cystic components. Six cases (54.5%) had mild peritumoural ooedema. The signal was isointense (8/11, 72.7%) or hypointense (3/11, 27.3%) on T1WI, and isointense (1/11, 9.1%) or hyperintense (10/11, 90.9%) on T2WI; all cases showed hyperintense on FLAIR and restricted diffusion on DWI. Haemorrhage was found in two cases (18.2%) and flow-void was found in one case (9.1%). All the tumours demonstrated contrast enhancement.An accurate diagnosis of EVN is difficult to be made preoperatively. It should be considered when a solid-cystic tumour with the solid part showing isointense on T1WI, hyperintense on FLAIR with mild to moderate enhancement especially restricted diffusion on DWI sequence in patients aged 20-30. When the radiologic manifestations are atypical, more aggressive treatment should be chosen.Neurocytoma, Extraventricular, Clinical, Imaging characteristics, MRI.

Central neurocytoma are rare primary brain tumors of the young and middle-aged adult, typically located in the lateral ventricles. Diagnosis has historically been difficult due to histomorphologic similarities to oligodendroglioma and ependymal tumors and remains a challenge even today. We present two cases of intraventricular central neurocytoma in which careful consideration of the clinical and radiological findings led to reevaluation of the preliminary histological interpretation, highlighting the importance of a meticulous differential diagnosis.

Background Retraction of white matter overlying a brain lesion can be difficult without causing significant trauma especially when using traditional methods of bladed retractors. These conventional retractors can produce regions of focal pressure resulting in contusions and areas of infarct. Methods In this article, we present a retrospective case series of six patients with deep-seated intraventricular and intra-axial tumors that were approached using a ViewSite Brain Access System (tubular retractor). The authors describe a unique method of creating a pathway using a dilated glove. We shall also review the relevant literature that reports this type of surgery. Cases included three cases with third ventricular colloid cysts, one case of a third ventricular arachnoid cyst, one case with a lateral ventricular neurocytoma, and a case with a deeply seated intra-axial metastatic tumor. Results Gross total resection was achieved in five cases with small residual in the central neurocytoma operation, with no documented neurological deficit in any case. One case had persistent memory problems and one case had continuing decline from the metastatic disease. Conclusion The introduction of tubular-shaped retractor systems has offered the advantage of reducing retraction pressures and distributing any remaining force in a more even and larger distributed area, thus reducing the risk of previous associated morbidity while also permitting great visualization of the target lesion.

Central neurocytoma (CN) is an uncommon brain tumor arising primarily in the lateral ventricular near the foramen of Monro and approximately accounting for 0.1-0.5% of all primary tumors of the central nervous system. We report a case of neurocytoma located in the fourth ventricle and extended into the third ventricle. The tumor is composed of uniform round cells with immunohistochemical features of neuronal differentiation. CN is probably a differential diagnosis with oligodendrogliomas and ependymoma. Morphology and immunohistochemical findings associated with clinical and radiological characteristics may support CN diagnosis.

Background Extraventricular neurocytoma (EVN) is a rare neurocytoma occurring in the brain parenchyma outside the ventricular system that shares similar biological behaviors and histopathologic characteristics with central neurocytoma. Reports of EVN localized in the brainstem and cerebellum are relatively uncommon. In addition, few cases with radiotherapy as the only treatment have been reported and their outcomes were unclear. Case Description We report a case of pathologically confirmed EVN of the brainstem and cerebellum in a 43-year-old male who presented with unprovoked nausea and dizziness. The patient received radiotherapy only and showed a favorable outcome during the 2-year follow-up period. Conclusions These results suggest that patients with EVN who are treated with radiotherapy without surgery may have a favorable prognosis.

Background Central neurocytomas (CNCs) are rare intraventricular lesions comprising 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. χ2 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 (χ2 = 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) (χ2 = 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.

Extraventricular neurocytoma (EVN) was firstly described in 1997. The current literature regarding imaging of EVN is limited to sporadic case reports and case series. EVN is still poorly considered in the differential diagnosis by neuroradiologists, thus diagnosis remains challenging. In this systematic review, we summarize and discuss computed tomography (CT) and magnetic resonance imaging (MRI) features of EVN cases described in the literature, in order to provide useful informations to neuroradiologists. To the best of our knowledge, this is the most extensive review about imaging of EVN. A systematic review of the literature about imaging of EVN cases was done. Only case reports or case series in which imaging (CT and/or MRI) features were deeply described were included in the revision. Eligibility of studies was assessed independently by two authors and any disagreements resolved by discussion. Our search strategy revealed 224 articles. After implementation of inclusion and exclusion criteria, 35 studies were considered, and a total of 79 cases of EVN were analyzed. EVN has not specific characteristics, with a large and variable imaging spectrum. Usually it appears as a large tumor, with diameters superior to 40 mm, frequently involving the frontal lobe. CT density and MRI signal intensity typically mirror the presence of cystic, solid, or calcified elements; contrast enhancement is visible in 87% of cases. Today, diagnosis of EVN with only imaging techniques is not univocal; neuroradiologists can only suspect this type of lesion, while the definitive diagnosis remains histological.

OBJECTIVECentral neurocytomas (CNs) are uncommon intraventricular tumors, and their rarity renders the risk-to-benefit profile of stereotactic radiosurgery (SRS) unknown. The aim of this multicenter, retrospective cohort study was to evaluate the outcomes of SRS for CNs and identify predictive factors.METHODSThe authors retrospectively analyzed a cohort of patients with CNs treated with SRS at 10 centers between 1994 and 2018. Tumor recurrences were classified as local or distant. Adverse radiation effects (AREs) and the need for a CSF shunt were also evaluated.RESULTSThe study cohort comprised 60 patients (median age 30 years), 92% of whom had undergone prior resection or biopsy and 8% received their diagnosis based on imaging alone. The median tumor volume and margin dose were 5.9 cm3 and 13 Gy, respectively. After a median clinical follow-up of 61 months, post-SRS tumor recurrence occurred in 8 patients (13%). The 5- and 10-year local tumor control rates were 93% and 87%, respectively. The 5- and 10-year progression-free survival rates were 89% and 80%, respectively. AREs were observed in 4 patients (7%), but only 1 was symptomatic (2%). Two patients underwent post-SRS tumor resection (3%). Prior radiotherapy was a predictor of distant tumor recurrence (p = 0.044). Larger tumor volume was associated with pre-SRS shunt surgery (p = 0.022).CONCLUSIONSTreatment of appropriately selected CNs with SRS achieves good tumor control rates with a reasonable complication profile. Distant tumor recurrence and dissemination were observed in a small proportion of patients, which underscores the importance of close post-SRS surveillance of CN patients. Patients with larger CNs are more likely to require shunt surgery before SRS.

entral neurocytoma is a neuroepithelial tumor described by Hassoun in 1982, predominantly located in the midline at the level of the septum pellucidum, or in the lateral ventricles wall1. They represent approximately 50% of intraventricular lesions in adults, and they are in total 0.25-0.5% of intracranial tumors.

Differential diagnosis between central neurocytoma and ependymoma is very important for making preoperative scheme. We explored the application of diffusion-weighted imaging (DWI) combined with apparent diffusion coefficient (ADC) in differential diagnosis between both. The data of preoperative MR plain and contrast-enhanced scan, DWI and ADC values of neoplastic solid parts from 18 cases with central neurocytoma and 19 cases with lateral ventricular ependymoma, were retrospectively analyzed. Mann-Whitney test was used for the comparison of ADC values between central neurocytoma and ependymoma. The application of ADC values in the differential diagnosis between central neurocytoma and ependymoma was evaluated by ROC curve. The lesions showed hyperintensity-dominant mixed signal intensity on DWI and mean ADC was (0.65 ± 0.13) × 10−3 mm2/s in the 18 cases with central neurocytoma. In the 19 cases with ependymoma, 13 had hyperintensity-dominant mixed signal intensity on DWI and 6 had hypointensity-dominant mixed signal intensity on DWI, and mean ADC was (1.20 ± 0.23) × 10−3 mm2/s. The mean ADC value was significantly higher in the 19 cases with ependymoma than in the 18 cases with central neurocytoma (P

According to the WHO classification, ependymal tumors are classified as subependymomas, myxopapillary ependymomas, classic ependymomas, anaplastic ependymomas, and RELA-fusion-positive ependymomas (RELA-EPN). Among classic ependymomas, the WHO defines rare histological variants, i.e., the clear cell, papillary, and tanycytic ependymoma. In parallel, global DNA methylation patterns distinguish nine molecular groups, some of which tightly overlap with histopathological subgroups. However, the match of the aforementioned histological variants to DNA methylation classes remains unclear. We analyzed histomorphology, clinical parameters, and global DNA methylation of tumors with the initial histological diagnoses of tanycytic (n = 12), clear cell (n = 14), or papillary ependymoma (n = 19). Forty percent of these tumors did not match to the epigenetic profile of ependymomas, using a previously published DNA methylation-based classifier for brain tumors. Instead, they were classified as low-grade glioma (n = 3), plexus tumor (n = 2), CNS high-grade neuroepithelial tumor with MN1 alteration (n = 2), papillary tumor of the pineal region (n = 2), neurocytoma (n = 1), or did not match to any known brain tumor methylation class (n = 8). Overall, integrated diagnosis had to be changed in 35.6% of cases as compared to the initial diagnosis. Among the tumors molecularly classified as ependymoma (27/45 cases), tanycytic ependymomas were mostly located in the spine (5/7 cases) and matched to spinal or myxopapillary ependymoma. 6/8 clear cell ependymomas were found supratentorially and fell into the methylation class of RELA-EPN. Papillary ependymomas with a positive ependymoma match (12/19 cases) showed either a 'papillary' (n = 5), a 'trabecular' (n = 1), or a 'pseudo-papillary' (n = 6) growth pattern. The papillary growth pattern was strongly associated with the methylation class B of posterior fossa ependymoma (PFB, 5/5 cases) and tumors displayed DNA methylation sites that were significantly different when compared to PFB ependymomas without papillary growth. Tumors with pseudo-papillary histology matched to the methylation class of myxopapillary ependymoma (4/6 cases), whereas the trabecular case was anatomically and molecularly a spinal ependymoma. Our results show that the diagnosis of histological ependymoma variants is challenging and epigenetic profiles may improve diagnostic accuracy of these cases. Whereas clear cell and papillary ependymomas display correlations between localization, histology, and methylation, tanycytic ependymoma does not represent a molecularly distinct subgroup.

Central neurocytomas are relatively rare well-differentiated neoplasms that arise from neuronal cells with the atypical variant having been noted to be more uncommon/aggressive. Herein, we present a case of an atypical neurocytoma confined to the third ventricle and in so doing review surgical management and outcomes associated with such tumors. We identify a number of cases of central neurocytomas within the third ventricle and clarify both clinical presentation and disease course. Central neurocytomas within the third ventricle are rare while atypical neurocytomas confined to the third ventricle are exceedingly rare. Review of the literature suggests gross total resection should be the preferred treatment modality for these tumors, atypical or not. When only subtotal resection can be achieved, postoperative radiation might be helpful to control tumor recurrence rates.

BACKGROUND To determine if histograms of ADC can be used to differentiate ventricular ependymomas, choroid plexus papillomas (CPPs), and central neurocytomas (CNCs). MATERIAL AND METHODS We retrospectively reviewed records from 185 patients from 1 January 2014 to 1 November 2018. We finally included a total of 60 patients: 36 (60.00%) had histologically confirmed ependymomas, 10 (16.67%) had CPPs, and 14 (23.33%) had CNCs, as determined by routine MRI scanning at 3.0T. The ADC histogram features were derived and then compared by Kruskal-Wallis test (they were not normally distributed). Bonferroni test was used to compare the 2 groups and then we determined the ROC. RESULTS Ependymomas had significantly higher mean, perc.01%, perc.10%, perc.50%, perc.90%, and perc.99% than CNCs. Ependymomas had significantly lower skewness than CNCs. Histogram metrics derived from mean, perc.01%, perc.10%, perc.50%, and perc.90% were significantly lower in the CNCs group than in the CPPs group. CPPs showed significantly lower skewness than CNCs. A threshold value of 86.50 for perc.50% to predict ependymomas from CNCs was estimated (AUC=0.97, sensitivity=97.20%, specificity=85.70%). Optimal diagnostic performance to predict CPPs from CNCs (AUC=0.96, sensitivity=100.00%, specificity=85.70%) was obtained when setting Perc.50%=84.00 as the threshold value. CONCLUSIONS The ADC histogram analysis may help to discriminate ependymomas, CPPs, and CNCs.

Central neurocytoma (CN) and extraventricular neurocytoma (EVN) are rare intracranial tumors. There is a paucity of studies reporting the population-based incidence of these tumors. We used the Central Brain Tumor Registry of the United States (CBTRUS), which contains the largest aggregation of population-based data on the incidence of primary central nervous system tumors in the United States to describe these tumors. The CBTRUS database, provided by CDC representing approximately 100% of the US population, was queried using the following search criteria: diagnosis years 2006–2014, ICD-0-3 histology codes (9506/0: central neurocytoma, benign; 9506/1: central neurocytoma, uncertain). Annual age-adjusted incidence rates are presented per 100,000 population. Incidence was estimated by age, gender, race, and ethnicity. The combined overall annual incidence rate of CN and EVN was 0.032 [0.030–0.034]. The incidence rates were 0.022 [0.021–0.024] and 0.009 [0.008–0.010] for CN and EVN, respectively. The most frequently documented locations for EVN were frontal lobe and cerebellum, followed by temporal lobe. Peak incidence was found in the 20–34 years range for both CN and EVN. The incidence rate was slightly lower in males compared to females for CN and identical for EVN. The overall incidence rate of CN and EVN combined was lower in Blacks 0.026 [0.021–0.032] and Hispanic Whites 0.020 [0.016–0.025] compared to Non-Hispanic Whites 0.035 [0.033–0.038]. CN and EVN are rare tumors with a peak incidence in the 20–34 years age group. This study represents the largest population-based epidemiological study on CN and EVN in the US.

Central neurocytomas are rare tumors, typically found in the third ventricle. We present the unusual case of a fourth ventricular central neurocytoma in a 70-year-old female presenting with imbalance and headaches, with an enhancing fourth ventricular mass on imaging. We examine the details of her clinical presentation, imaging characteristics, histopathologic analysis, and postoperative course. We performed a critical literature review of fourth ventricular central neurocytoma, identifying 6 previously reported cases. In contrast to our patient, the majority of these cases were in young males. The 2 females with fourth ventricular neurocytoma developed it at a much later age (mean 63.5 years) than their male counterparts (mean 22.8 years; P = 0.005). Despite being a rare cause for a fourth ventricular lesion, given the appropriate clinical context and radiographic findings, this should remain part of the differential diagnosis.

Objective To assess usefulness and limitations of flexible fiber carbon dioxide (CO2) laser in the microsurgical treatment of intraventricular tumors. Methods We reviewed a series of 9 patients treated with microsurgical resection of intraventricular tumors using a flexible fiber CO2 laser. The lesions involved the third ventricle (8) and the frontal horn of the right lateral ventricle (1). Histology revealed 6 craniopharyngiomas, 1 pituitary macroadenoma, 1 subependymoma, and 1 neurocytoma. In all cases, an interhemispheric transcallosal approach was performed. The laser was used during callosotomy, fornix column sectioning, tumor debulking, and to facilitate tumor dissection. We used a 5-tiered score system to assess laser's efficacy in each surgical step (approach, dissection, debulking): grade 1: laser was not at all helpful, grade 5: laser was extremely helpful. Limits of the instrument also are discussed. Results Gross total resection was achieved in 6 cases and subtotal resection in the remaining 3. Three patients had pulmonary complications treated without clinical sequelae. No laser-related complication was described. Mean utility score observed was 4.2 (range 3–5) during approach, 2.8 (range 2–4) during tumor dissection; and 3.3 (range 2–5) during tumor debulking. Main limits were low hemostatic effect and inefficiency versus calcified and highly vascularized tumors. Conclusions The CO2 laser proved to be a useful and safe tool that could be used for intraventricular pathology; its design is suitable for narrow surgical corridors like interhemispheric fissure and foramen of Monro; its main utility is the ability to create precise and relatively bloodless cut (callosotomy, tumor debulking); low hemostatic effect is its main limit.

Central neurocytomas are benign tumors accounting for0.5% of all intracranial tumors. They are usually intraventricular, the most common site being the lateral ventricles, and are associated with good prognosis if they are completely resected surgically.We report the case of a middle-aged woman who was evaluated for recent-onset headache and for whom magnetic resonance imaging of the brain was suggestive of a solid cystic intraventricular mass spanning all the ventricles. The patient underwent subtotal excision of the mass, with intraoperative neuromonitoring. The result of Histopathologic examination was suggestive of atypical neurocytoma. The patient had complete neurologic recovery in spite of immediate postoperative deficits.Gross total resection should be the goal in all patients with central neurocytoma, but it may be limited because of proximity of the tumor to eloquent areas, especially in large tumors. Intraoperative neuromonitoring plays a pivotal role in maximal safe resection of such large tumors. Tetraventricular atypical central neurocytoma is a very rare entity; and this case could be, to the best of our knowledge, only the second reported case.

Background: Cancers of the central nervous system (CNS) are considered to be the most notorious among all cancers. Brain tumors are a mixed group of neoplasms that originate from the intracranial tissues and the meninges with degrees of malignancy varying greatly from benign to aggressive. The WHO classification offers a crude histological grading system, in which each CNS tumor is classified as Grades I-IV according to its degree of malignancy. Primary neoplasms of CNS represent nearly two-thirds of all brain tumours. Methods: The study was carried out in the Department of Pathology, Sardar Patel Medical College and associated group of hospitals, Bikaner. It was a hospital based retrospective and prospective study for 5 years from 2015 to 2020, including brain biopsies / tissue following surgical resection, submitted at our department. Improper and autolysed specimens and cases without proper clinical history were excluded. Results: We included a total of 245 cases of CNS malignancies received at our department during the study period in our study. Of total 245 cases, as in Table no. 1, most common tumor noted was Astrocytoma with 37.55% cases, followed by Meningioma 18.78% cases, Pituitary adenoma 8.57% cases and Pilocytic Astrocytoma 7.76% cases, whereas only one case (0.41%) was recorded each for Subependymoma, Neurocytoma, Haemangioma, Haemangiopericytoma and Spindle cell tumor. The most common tumors were diffuse astrocytic and oligodendroglial tumors with total 41.63% (n=102) cases followed by Meningial tumors with total 18.78% (n=46) cases. Conclusions: The most frequent type of CNS tumours in this study was astrocytoma, followed by meningioma. This study may provide the representative prevalence of various types of CNS tumors. Thus, from the point of view of treatment of the different tumors of central nervous system mostly presenting with common clinical symptoms there is a direct need for a histopathological study in order to arrive at a correct diagnosis as for instance in the present study to know the type of tumor and their further typing and also its histological grade from the point of view of prognosis. Keywords: Brain Tumors, Astrocytoma, Meningioma, CNS Tumors

Cerebellar liponeurocytoma (cLNC), categorized as a World Health Organization grade II tumor, is a rare neoplasm characterized by advanced neuronal/neurocytic differentiation and focal lipid accumulation in neuroepithelial tumor cells. However, the expression and genetic profiling of cLNC have been poorly studied. A 44-year-old woman with a three-year history of cerebellar ataxia and numbness in lower extremities underwent radiological examination revealing multiple contrast-enhancing tumors at the floor of the fourth ventricle and in the lower vermis, and spinal dissemination. The high uptake of 11 C-methionine in positron emission tomography (Met-PET) supported the preoperative cLNC diagnosis. Subtotal removal of the tumor around the obex and inferior vermis was performed. Histologically, the tumor was composed of small, uniform cells with round nuclei in a sheet-like fashion. Tumor cells were diffusely reactive for the neuronal markers synaptophysin and neurofilament. Vacuolate cells with a displacement of nuclei suggested the accumulation of lipid, which was further supported by immunohistochemical staining of S-100. These findings confirmed the diagnosis of cLNC. Next-generation sequencing of tumoral DNA detected a splice site mutation in the ATRX gene. Further reports of cLNC cases with detailed expression and genetic profiles are essential for precise diagnosis and clarifying the oncogenic pathway in cLNC.

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.

Glioneuronal tumors are a group of rare neoplasms made up of neural and glial components in heterogenous proportions, generally exhibiting WHO grade I clinical behavior. These tumors affect infants, children and young adults, but are also described in adults and the elderly. They are strongly associated with seizures. Tumor subtypes described under the umbrella of glioneuronal tumors are actively evolving but to date comprise central, extraventricular and lipo- neurocytoma, desmoplastic infantile astrocytoma and ganglioglioma, diffuse leptomeningeal glioneuronal tumor, dysembryoplastic neuroepithelial tumor, papillary glioneuronal tumor, rosette-forming glioneuronal tumor of the fourth ventricle, rosetted glioneuronal tumor with neuropil-like islands, gangliocytoma, ganglioglioma, anaplastic ganglioglioma and paraganglioma. They vary in radiographic appearance, with some exhibiting large heterogenous solid/cystic masses. With large scale genetic and molecular analyses ongoing, classification continues to evolve. Seizure management and surgical resection represent the cornerstones of management, with the use of systemic agents and radiation lacking conclusive results. Optimal management requires multidisciplinary discussion including neuro-oncological and neuro-surgical expertise due to both the rarity of these tumors and the lack of evidence with data confined to small retrospective series and reviews.

Central neurocytoma is a rare benign brain tumor. These tumors may be giant and accompanied by compression of ventricular system and surrounding structures. Modern treatment of brain neurocytoma includes extended resection and restoration of normal CSF circulation. Surgical treatment does not often lead to total resection of these tumors. Redo resection was preferred in patients with tumor progression for a long time. In the last decade, various authors report stereotactic irradiation for continued tumor growth to ensure local growth control. This study was aimed at evaluation of postoperative outcomes in patients with brain neurocytomas, as well as treatment of tumor progression in long-term period.To analyze recurrence-free survival in patients with brain neurocytomas, risk factors of recurrence-free survival, effectiveness of various treatments for tumor progression and delayed complications.Long-term postoperative follow-up data of patients with brain neurocytomas are reported in the manuscript. We analyzed recurrence-free survival and risk factors of recurrence-free survival, treatment outcomes in patients with progression of brain neurocytomas, long-term complications and their prevention.Follow-up included 84 out of 115 patients with brain neurocytoma after surgical treatment in 2008-2017. Follow-up period ranged from 2 to 10 years (mean 6 years) after resection. Most patients had regression of neurological symptoms after surgery. Continued tumor growth within 12-96 months after surgery occurred in 26 (30.19%) out of 84 patients (19 cases after partial resection and 7 cases after total resection according to MRI data). Two-year recurrence-free survival was 94%, 5-year survival - 83%. Risk factors of continued tumor growth were resection quality and Ki-67 index. Redo resection was performed in 7 cases. Eleven patients underwent stereotactic irradiation for tumor progression. Indications for stereotactic irradiation of central neurocytoma are MR data on continued growth of lateral ventricle tumor without signs of ICH and CSF flow impairment. There were no cases of hemorrhage inside the residual tumor and CSF flow impairment in early postoperative period after redo resection. In all cases (Resection of central neurocytoma ensures long-term recurrence-free period. The main causes of tumor recurrence are partial resection and high proliferative activity (Ki-67 index over 5%). Redo resection is advisable for tumor progression followed by CSF flow impairment. In case of continued growth of neurocytoma without signs of intracranial hypertension, stereotactic irradiation with various fractionation modes ensures effective and safe control of tumor growth.Центральные нейроцитомы (ЦН) головного мозга являются редкими доброкачественными опухолями. Они могут достигать гигантских размеров, заполнять желудочковую систему и распространяться на окружающие структуры мозга. Современное лечение ЦН головного мозга включает максимально возможную резекцию опухоли и восстановление нормальной ликвородинамики, однако достаточно часто в ходе операции не удается удалить опухоль радикально. При прогрессии опухоли долгое время методом выбора считалось повторное удаление. В публикациях последнего десятилетия отмечено, что при продолженном росте ЦН чаще используются различные режимы стереотаксического облучения, благодаря чему удается достигнуть локального контроля роста опухоли. Данное исследование предпринято для оценки результатов катамнестического наблюдения пациентов с ЦН головного мозга после хирургического лечения, анализа возможных способов лечения и их эффективности при прогрессии опухоли в отдаленном периоде.Оценить безрецидивную выживаемость (БРВ) пациентов с ЦН головного мозга, провести анализ факторов, которые могут влиять на БРВ, определить эффективность различных методов лечения при прогрессии опухоли и развитии поздних осложнений.В работе представлены данные катамнеза 84 из 115 пациентов с ЦН головного мозга, оперированных в период с 2008 по 2017 г. Оценены БРВ и факторы, которые могут влиять на нее. Проанализированы результаты лечения пациентов с прогрессией ЦН головного мозга, осложнения, возникшие в отдаленном периоде, и методы их профилактики.Срок катамнеза после удаления опухоли составил от 2 до 10 лет (в среднем 6 лет). В большинстве случаев наблюдался регресс неврологической симптоматики, развившейся после операции. У 26 (30,19%) из 84 пациентов отмечен продолженный рост или рецидив опухоли в сроки 12—96 мес после операции: у 19 — после неполного удаления и у 7 — после тотального удаления по данным магнитно-резонансной томографии (МРТ); 2-летняя БРВ составила 94%, 5-летняя БРВ — 83%. При анализе факторов, влияющих на развитие продолженного роста опухоли, выявлено, что основное значение имеют радикальность удаления и индекс пролиферативной активности опухоли Ki-67, определяемый иммуногистохимическим методом. В 7 случаях проведено повторное удаление опухоли. Осложнений в виде кровоизлияния в остатки опухоли, нарушения ликвородинамики в раннем послеоперационном периоде не было. Стереотаксическое облучение по поводу прогрессии опухоли получили 11 пациентов. Показанием к облучению служило выявление по данным МРТ продолженного роста опухоли боковых желудочков без признаков внутричерепной гипертензии и нарушения ликвородинамики. Во всех случаях после стереотаксического облучения (средний срок наблюдения 2,5 года) отмечен удовлетворительный контроль опухолевого роста в виде уменьшения объема облученной опухоли (в 4 наблюдениях) и отсутствия роста опухоли (в 7 наблюдениях).Хирургическое удаление центральных нейроцитом позволяет добиваться длительного безрецидивного периода. Основные причины рецидивирования опухоли — ее неполное удаление и высокая пролиферативная активность (Ki-675%). При прогрессии опухоли, которая сопровождается нарушением ликвородинамики, целесообразно проводить повторное удаление центральной нейроцитомы. При отсутствии признаков повышенного внутричерепного давления применение стереотаксического облучения в различных режимах фракционирования, выбор которых зависит от размеров опухоли, позволяет эффективно и безопасно контролировать рост опухоли.

Rare/Uncommon brain tumors like ganglioglioma, neurocytoma, plasmacytoma, rosette forming glioneural tumors, etc. are difficult to manage due to absence of good evidence-based guidelines. The role of complete resection, adjuvant radiotherapy as well as recurrence patterns are not well described. This chapter will summarize salient features of such tumors along with evidence-based management guidelines.

Background Liponeurocytoma is an uncommon tumor of the central nervous system. It is very rare for this tumor to originate within the lateral ventricle. In the context of the rarity of this tumor entity, this review article aims to summarize the clinical, radiological, and pathological features of lateral ventricular liponeurocytoma to facilitate its diagnosis and management. Methods Here, we conduct a systematic literature review using the Pubmed, Scopus, and Cochrane Library database for all cases of lateral ventricular liponeurocytoma. A case illustration complements this review. Results The described cases from 1997 onward include 14 cases that have been published in full papers in the English literature. Six additional cases are reported in short English abstracts in full non-English papers, and one case was described in a central neurocytoma report. There is a definite male predominance of 70% (14 male) and a mean age of 37 years (range 24–62). Heterogenous enhancement and signals in magnetic resonant images (MRI) are the radiological characteristics. In all reported cases, the presence of lipocytes and fat vacuoles is considered the paramount histopathological feature. Total surgical resection was achieved in 80% (12 out of 15) of the cases. Only two cases (including ours) received radiation therapy. Recurrence was seen in two patients during follow-up that was treated by radiation therapy in one and surgery in the other. The proliferation index is mostly below 5% in all cases, with the Ki-67 range between Conclusions Lateral ventricular liponeurocytoma has been treated effectively by surgical resection in a limited number of cases. The decision for radiation therapy is based on a high proliferation index and tumor recurrence.

中枢/脑室外神经细胞瘤是WHO Ⅱ级伴神经元分化的中枢神经系统肿瘤。中枢神经细胞瘤发生于脑室内，脑室外神经细胞瘤可发生于整个中枢神经系统。中枢神经系统外神经细胞瘤非常罕见。该文报道1例27岁女性起源于卵巢成熟性畸胎瘤的神经细胞瘤。镜下肿瘤由一致的小圆细胞构成，位于纤丝背景内，并与衬覆室管膜样细胞的囊肿相邻。免疫表型：突触素、神经元特异性烯醇化酶阳性，神经元核抗原及神经胶原酸性蛋白阴性，Ki-67阳性指数1%。细胞遗传学分析未发现染色体1p及19q共缺失。中枢神经系统外神经细胞瘤非常罕见，大多起源于畸胎瘤，相较发生于中枢神经系统内的肿瘤，两者免疫表型存在轻度差异。.

Spinal neurocytoma (SN), although frequently reportedly as tumors of the central nervous system (CNS), are a distinct class of tumors, which can achieve a better prognosis following subtotal or gross total tumor resection. Nonetheless, even with the premise of successful treatment after tumor resection, poor prognosis after treatment due to the SN high proliferation index (typically known as atypical SN) have been reported. Over the past two decades, atypical SN was only reported in four pediatric cases, amidst the lingering controversy surrounding its postoperative adjuvant therapy. Thus, herein, we report a unique case of atypical SN with epidermal growth factor receptor (EGFR) amplification mutation in a 12-year-old boy. We, however, also highlighted the significance of radiotherapy and target therapy for patients with SN.

Central neurocytoma is a rare nervous tissue benign neoplasm. A subset of central neurocytoma has unfamiliar aggressive tendency: so-called atypical central neurocytoma (ACN). This retrospective study aims to analyze the prognostic factors and the impact of various therapy tools on atypical central neurocytoma.Twenty-two patients diagnosed with ACN between January 2009 and March 2018 were included. Data collected included the patient's age, gender, tumor location, presenting symptoms, and treatment received. Patients were followed up to detect recurrence and to assess survival.Median overall survival was 57 months, with a 5-year survival of 35%. Better survival was observed for patients35 years old (66 vs. 47 months; P = 0.061) and patients with gross total resection over subtotal resection or biopsy (76, 45, and 22 months, respectively; P0.0001). Patients with a tumor located in the posterior half of the lateral ventricle had better survival, with no statistical significance (P = 0.053). Multivariate analysis showed prognostic significance with the extent of resection (P = 0.000). Progression-free survival ranged from 6 to 82 months, with a median value of 38 months and showed a significant relation with subtotal resection compared with biopsy (P = 0.006). Recurrence was less in patients who received radiotherapy and was statistically significant (P = 0.007).Long-term survival is possible for patients with atypical central neurocytomas treated with surgery and postoperative radiation. Multivariate analysis confirmed that gross total resection was an independent prognostic factor for survival. Adjuvant radiotherapy reduces tumor recurrence, especially after incomplete surgery.

The aim of this article is to discuss the importance of staged surgeries when approaching atypical central neurocytoma in children. Also, we show the preoperative embolization of the lesion as a maneuver to reduce the intraoperative bleeding. Central neurocytomas represent less than 0.5% of all intracranial tumors, and atypical central neurocytomas usually have unfavorable outcome, with high recurrence rate. The intraventricular location is frequent, with a predilection for the lateral ventricles. When completely resected, these lesions have a good prognosis. We report a case of a 12-year old male patient that presented with a history of headache for about 6 months, which worsened for 1 week prior to admission. Magnetic resonance imaging (MRI) brain showed a massive lesion occupying both lateral ventricles. He underwent a microsurgical treatment of a highly vascularized lesion, but the perioperative bleeding required interruption of the surgery. Thus, a preoperative embolization was able to occlude most arterial feeders and allowed subtotal resection in a second surgery. The patient had complete neurological recovery despite immediate post-operative deficits, and the histopathology was suggestive of atypical neurocytoma. Two-stage surgery with preoperative adjuvant embolization is a feasible strategy for treatment of large central neurocytomas in children.

Aim We describe a case of mesial temporal extraventricular neurocytoma (mtEVN) in a 23-year-old male presenting with drug-resistant seizures and review the literature on this rare tumor. Methods A PubMed search was queried using the MeSH term “neurocytoma” and key search terms “extraventricular”, “temporal”, and “epilepsy”. Titles and abstracts were screened for temporal neurocytomas. References were reviewed to identify further studies. Results Twenty case reports were selected comparing the presentation, radiological, histopathological, and surgical outcomes of neocortex temporal EVNs (ntEVN) and mtEVNs. Conclusion Gross total resection of mtEVNs under intraoperative electrocorticography monitoring typically affords an excellent prognosis and successful seizure control., Highlights • Mesial temporal extraventricular neurocytomas may differ from neocortex temporal extraventricular neurocytomas. • Mesial temporal extraventricular neurocytomas can present with drug-resistant focal seizures responsive to epilepsy surgery. • Mesial temporal extraventricular neurocytomas are nonenhancing, noncalcified, and lack cysts. • Gross total resection of mesial temporal extraventricular neurocytomas affords an excellent prognosis.

To describe the far-anterior interhemispheric transcallosal approach for the treatment of a central neurocytoma at the roof of the lateral ventricle. In comparison to the view obtained during the usual anterior transcallosal approach, the far-anterior approach allowed for a higher view of the lateral ventricle to be obtained without further injury or retraction of the corpus callous. Two patients with central neurocytoma in the lateral ventricle were treated with the far-anterior interhemispheric transcallosal approach. Gross-total resections were achieved in both the patients without any postoperative neurological impairments by only 2-3 cm incisions of the corpus callosum. With the anterior transcallosal approach, which was usually used for the intraventricular tumors, the surgical view was relatively downward into the lateral ventricle and suitable for the resection of the tumors located at the base of the lateral ventricle or even in the third ventricle through the foramen of Monro. However, it was relatively difficult to reach the roof of the lateral ventricle using this approach. In contrast, the surgical corridor of the far-anterior transcallosal approach reaches upward to the roof of the lateral ventricle. The far-anterior transcallosal approach provides an alternative to reach the lesions, especially those located in the upper region of the lateral ventricle near important structures, such as the pyramidal tracts.

Hypothalamic tumors of neuronal derivation are rare. We describe the case of a 55-year-old woman with visual disturbances who was found by magnetic resonance imaging (MRI) to have a sellar and suprasellar tumor. She underwent subtotal surgical resection by a transsphe-noidal approach. By light microscopy the tumor displayed a uniform population of short spindle cells with round to oval nuclei, separated by an abundant fibrillary stroma containing axonal processes as shown by the Bodian stain. The neoplastic cells were immunoreactive for neuron-specific enolase (NSE), synaptophysin, and vasopressin, and nonimmunoreactive for glial fibrillary acidic protein (GFAP), vasoactive intestinal peptide (VIP), bombesin, chromogranin, neurofilament, cytokeratins (high and low molecular weight), vimentin, S100 protein, somatostatin, β-endorphin, galactosamine, growth hormone-releasing hormone (GRH), neurophysin, serotonin, adrenaline and noradrenaline, and corticotropin-releasing hormone (CRH). Ultrastructural features included an abundance of neurosecretory granules within neurites and perinuclear cytoplasm. Synapses and glial stroma were not demonstrable. The term hypothalamic neurocytoma delineates this neuronal tumor with distinctive histologic, immunohistochemical, and ultrastructural features. The identification of vasopressin within the tumor provides evidence of neuroendocrine function.

Central neurocytomas of the brain are rare benign tumors of the cerebral lateral ventricles. The main treatment for them is surgical resection. Resection provides a long-term relapse-free period, but surgical intervention is associated with a number of significant difficulties due to the location, size, and blood supply features of these tumors. The postoperative period is often accompanied by hemorrhagic complications, impaired cerebrospinal fluid circulation, and worsening of neurological symptoms.The study purpose was to evaluate the effectiveness of surgical treatment in neurocytoma patients, assess the risk of complications after neurocytoma resection, and develop techniques for their prevention.The paper presents surgical treatment outcomes in 115 patients with central neurocytomas for the period from 2008 to 2017. The choice of a surgical approach and the surgical features are described in detail, and the radicality is assessed with allowance for the location and size of tumors. The immediate clinical outcomes of treatment are evaluated, complications are analyzed, and methods for prevention and treatment of complications are described.Analysis of the data revealed that the tumor was resected totally in 41 (36%) patients, subtotally - in 37 (32%), and partially in 37 (32%). The most common and dangerous complications were as follows: CSF circulation occlusion with the development of hydrocephalus in 23 (20%) patients; hemorrhage into the residual tumor, which required immediate revision of the surgical wound in most cases, in 25 (22%) patients. In the early postoperative period after tumor resection, almost all patients developed deterioration of condition with transient worsening of cerebral and focal symptoms. The degree and duration of this worsening were different. In 50% of cases, the postoperative condition was characterized by reduced voluntary activity, drowsiness or jitteriness, and motor or speech anxiety.We consider resection of neurocytomas as the main treatment option, despite the potential complications and effects of the operation.Центральные нейроцитомы головного мозга относятся к редко встречающимся доброкачественным опухолям боковых желудочков головного мозга. Основным методом их лечения является хирургическая резекция. Удаление позволяет добиться длительного безрецидивного периода, однако хирургическое вмешательство сопряжено с рядом значительных трудностей, что определяется локализацией, размерами и особенностями кровоснабжения этих опухолей. Послеоперационный период часто осложняется развитием геморрагических осложнений, нарушением ликвороциркуляции и нарастанием неврологической симптоматики. Цель исследования - оценка эффективности хирургического лечения пациентов с нейроцитомами головного мозга; оценка риска развития осложнений после удаления нейроцитом головного мозга и разработка методов их профилактики. Материал и методы. В работе представлены результаты хирургического лечения 115 пациентов с центральными нейроцитомами головного мозга в период с 2008 по 2017 г. Детально описаны выбор хирургического доступа, особенности операций, проведена оценка радикальности с учетом локализации и размеров опухолей. Оценены ближайшие клинические результаты лечения, проанализированы осложнения и показаны методы их профилактики и лечения. Результаты. Анализ данных показал, что радикальное удаление выполнено у 41 (36%) пациента, субтотальная резекция - у 37 (32%) и частичное удаление - у 37 (32%). Самыми частыми и опасными осложнениями были нарушение ликвородинамики по окклюзионному типу с развитием гидроцефалии у 23 (20%) пациентов, кровоизлияния в остатки опухоли, в большинстве случаев явившиеся показанием к немедленной ревизии операционной раны, у 25 (22%) пациентов. После удаления опухоли в раннем послеоперационном периоде практически у всех больных наблюдалось ухудшение состояния с преходящим нарастанием общемозговой и очаговой симптоматики. Степень и продолжительность этого нарастания различались. В 50% случаев послеоперационное состояние характеризовалось снижением произвольной активности, сонливостью либо тревожным состоянием, двигательным или речевым беспокойством. Выводы. Мы предлагаем рассматривать удаление нейроцитом головного мозга как основной метод лечения, несмотря на возможные осложнения и последствия операции.

A rare case of extraventricular neurocytoma (EVN) arising from the VIIIth cranial nerve in a 34-year-old woman is reported. The patient had a 20-year history of hearing loss and facial palsy. Computed tomography showed a 3-cm enhancing lesion in the left cerebellopontine angle (CPA). At operation, the tumor was seen to originate from the cochlear and vestibular nerves. The tumor was subtotally resected. Histologically, the tumor consisted of uniform cells with oval to round nuclei and scant cytoplasm. Immunohistochemically, the tumor cells were positive for synaptophysin, but negative for glial fibrillary acid protein and S-100 protein. The Ki-67 labeling index was 0%. Twelve years after the operation, magnetic resonance imaging (MRI) showed tumor recurrence at the left CPA. The tumor was subtotally resected, and radiation therapy was given. Histologically, the tumor consisted of round cells with mild atypia and one mitosis/20 high-power fields (HPF). Immunohistochemically, tumor cells showed the same findings as the first operation sample, except for the Ki-67 labeling index (3%). Twelve years after the second operation, MRI showed a second tumor recurrence at the left CPA and surroundings of the brain stem. The tumor was subtotally resected. Histologically, the tumor consisted of anaplastic short spindle cells and five mitoses/10 HPF. The immunohistochemical findings were almost the same as the earlier operation samples. However, the Ki-67 labeling index was 20%. In addition, tumor cells from the third specimen were more strongly and more diffusely positive for GAB1 (growth factor receptor-bound protein 2-associated binding protein 1) compared to those of the earlier specimens. Electron microscopy showed the presence of numerous cell processes with a dense core and clear vesicles and microtubules. GAB1 immunostaining also indicated that malignant progression might be associated with the sonic hedgehog signaling pathways. To the best of our knowledge, this is the first report of an EVN arising from the VIIIth cranial nerve with malignant progression.

Objectives The list of tumors involving the pituitary gland has been expanded to include a variety of neuronal and paraneuronal tumors in the 2017 World Health Organization tumor classification of endocrine organs. All the entities included in this category are distinctly rare, with limited case reports in the literature. Methods We illustrate two extraordinary sellar tumors with neuronal differentiation: a sellar paraganglioma and a sellar neurocytoma, with thorough literature review and comparison of the clinicopathologic features of these entities. Results Both entities are exceptionally rare and tend to be misdiagnosed as pituitary adenoma preoperatively. Both entities demonstrate frequent clinical recurrence compared with pituitary adenoma, as well as the rare occurrence of metastatic disease. Conclusions In evaluating a sellar tumor with an uncommon morphology and neuroendocrine differentiation, an increased awareness of the unusual entities that may involve the sellar region and a judicious panel of immunohistochemical studies should improve the diagnosis.

The aim of studywas to describe a clinical case of central neurocytoma formation and course.Materials and methods.Patient C., 33 years old, admitted to the neurosurgical department of Regional Clinical Hospital with complaints of headache and sleep disturbance that troubled her for 2 months. No chronic diseases were detected. During the stay in the hospital, the patient was carried out a number of examinations: assessment of laboratory parameters in dynamics, magnetic resonance imaging of the brain, examination by specialists (neurologist, neuroophthalmologist).Results.Magnetic resonance imaging of the brain revealed a solid volume cystic formation of the left frontal lobe. Bone plastic trepanation and microsurgical removal of the intracerebral cystic tumor in the left frontal area were performed. Revealed neuroradiological and histological characteristics allowed differentiation of neurocytoma from other histological variants. According to magnetic resonance imaging no tumor growth was observed 5 months after surgery. A course of radiation therapy was conducted.Conclusion.This clinical case presented the peculiarities of the course, diagnosis, surgery and immunohistochemical analysis of patient with central neurocytoma.

Central neurocytoma (CN), first described in 1982 by Hassoun and colleagues, is a rare tumor accounting for 0.25% to 0.5% of all tumors of the central nervous system. The tumor is a neoplasm of neuroepithelial origin, with intermediate malignancy (WHO grade II), detectable with both computed tomography and magnetic resonance imaging. Complete excision of the tumor gives favorable long-term results, with infrequent recurrences and/or metastases. Only 3 previous cases in which CN presented with co-occurring psychotic symptoms were found in the PubMed database. This report presents the case of a 27-year-old patient with paranoid syndrome without neurological symptoms, in whom magnetic resonance imaging confirmed a large intracranial tumor located predominantly in the right lateral ventricle and third ventricle reaching down to the hypothalamus. Resection of the tumor (histopathologically a CN) resulted in complete remission of the psychotic symptoms. This case supports the need for neuroimaging in all patients with first-episode psychosis because of the possibility of neurologically silent brain tumors. Quick diagnosis in such cases is crucial for the selection of treatment methods and prognosis.

To evaluate the safety and efficacy of SRS treatment of central neurocytomas (CNCs), cavernous sinus hemangiomas (CSHs), and glomus tumors (GTs); and to compare upfront stereotactic radiosurgery (SRS) and adjuvant SRS in the treatment of these hypervascular tumors. This was a retrospective review of consecutive CNCs, CSHs, and GTs treated with SRS between 1993 and 2017. Tumor response was categorized based on volumetric analysis on magnetic resonance imaging: (1) tumor regression [> 10% decrease in tumor volume (TV)]; (2) stable tumor (≤ 10% change in TV); or (3) tumor progression (> 10% increase in TV). Sixty hypervascular tumors (CNC: 28; CSH: 16; GT: 16) underwent SRS between 1993 and 2017. Margin doses were 13 Gy, 12 Gy, and 14 Gy for CNCs, CSHs, and GTs, respectively. Tumor regression was observed in 54 tumors (90%) with initial SRS after a median follow-up of 82 months. Relative reductions in TVs for CNCs were 38%, 52%, and 73% at 12, 24, and 48 months, respectively. Relative reductions in TVs for CSHs were 51%, 68%, and 88% at 12, 24, and 48 months, respectively. Relative reductions in TVs for GTs were 22%, 31%, and 47% at 12, 24, and 48 months, respectively. Upfront SRS was performed in 26 patients (43%). No difference in relative TV reductions were found between upfront and adjuvant SRS. Adverse radiation effects were observed in five patients. No mortality was encountered. SRS for hypervascular tumors is associated with high rates of tumor regression with low risk of complications. No significant difference in rates of relative TV reduction were found between upfront and adjuvant SRS. Hence, upfront SRS may be considered in select patients.

Extraventricular neurocytoma (EVN) is an exceedingly rare tumor. In this study, we sought to characterize the imaging and pathological features of this uncommon tumor. Retrospective review of 18 patients (9 male; 9 female) with pathologically confirmed EVN treated at a single center between 2005 and 2017. All patients had a solitary lesion. Sixteen lesions were found in hemispheres. The greatest tumor diameter ranged from 2.6 to 8 cm. The lesions were generally solid with cystic components; the solid portion appeared isodense or hyperdense on CT, isointense to hypointense on T1WI, and slightly hyperintense on T2WI. Heterogeneous hyperintensity interspersed with isointense or hypointense areas suggestive of hemorrhage, calcification or vascular flow voids were seen on T2WI. Heterogeneous enhancement was noted in 17 lesions; no enhancement was observed in one lesion. Cystic components were observed in 13 lesions; 9 of these showed characteristic perilesional cysts (9/13). Mild to moderate peritumoral edema (15/18), calcification (4/16), intratumoral hemorrhage (11/18) and vascular flow voids (10/16) were observed in some lesions. Pathologically, tumor cells showed round nucleus and fine neuropil matrix. Foci of calcification in the solid portion of the tumor were seen in five cases. Microcystic changes were observed in almost all cases. Some lesions exhibited positive staining for synaptophysin (Syn) (15/16) and neuronal nuclei (NeuN) (7/8). MIB-1 was determined for 10 patients; seven of these had an MIB-1 ≥ 3. These six patients experienced recurrence; four of them relapsed twice. EVNs occur as single intracranial solid mass with cystic components (especially peripherally located cysts); solid portion exhibits slight hyperintensity or heterogeneous signal intensity. Mild to moderate peritumoral edema, calcification, intratumoral hemorrhage and vascular flow voids were characteristic features of extraventricular neurocytoma. Positive staining for synaptophysin and neuronal nuclei confirmed the diagnosis. A combination of atypical pathologic features and atypical radiologic features should be considered for prognostic assessment.

Central neurocytoma (CN) has long been regarded as a neuronal tumor based on the immunohistochemical expression of synaptophysin and the ultrastructural observation of neurosecretory granules, neurites, and synapses. Having diagnosed 11 CNs at our institution over the past thirty years, we set out to conduct an immunohistochemical study to assess the expression profile of neuronal markers across our cases. Markers of interest included synaptophysin, alpha-synuclein, chromogranin, neurofilament, and calretinin. Intriguingly, we observed a dichotomous expression profile between neurofilament and calretinin, suggesting the presence of histologic variants of CN based on the degree of neuronal maturation. We have further provided an overview of the clinico-pathologic heterogeneity within our series with respect to age of onset, overall outcome, and presence of anaplastic features. In highlighting the case of an infant with an incidental CN diagnosed at autopsy, we have discussed the role of a primitive neural cell of origin for driving tumor formation and accounting for our proposed differences in neuronal maturation within CN.

To increase our understanding of the imaging features of central neurocytoma (CN) and improve the preoperative MRI diagnosis accuracy. Preoperative MR images of 30 CNs and another 68 intraventricular non-CN tumours were analysed by one experienced neuroradiologist retrospectively to identify previously reported features and new features of CN. Six blinded radiologists independently reviewed all these MRI images, and scored all characteristic features on a five-point scale. Diagnostic value was assessed by the area under the receiver operating characteristic curve (AUC); sensitivity, specificity and accuracy were also calculated. In addition to the ‘scalloping’ sign, ‘broad-based attachment’ sign and ‘soap-bubble’ sign, three new MRI features of CN were identified, including the ‘peripheral cysts’ sign, ‘fluid-fluid level’ sign and the ‘gemstone’ sign. The scalloping sign showed the highest AUC value (0.82), followed by the peripheral cysts sign (0.75) and broad-based attachment sign (0.75). The scalloping sign exhibited the highest specificity (82%), followed by the fluid-fluid level sign (79%) and gemstone (78%) sign. The broad-based attachment sign (85%) was the most sensitive feature, followed by the soap-bubble sign (84%) and peripheral cysts sign (77%). There are six characteristic MRI features that help to improve the preoperative diagnostic accuracy of CN. • This study is the largest magnetic resonance imaging (MRI) cohort on central neurocytoma (CN). • Three new features helpful for the diagnosis of CN were reported. • Diagnostic value of six MRI features of CN was preliminarily determined.