Neurocitoma Central Atípico: reporte de un caso en Ecuador / Atypical Central Neurocytoma: report of a case in Ecuador

El Neurocitoma Central es un tumor benigno infrecuente del Sistema Nervioso Central que afecta principalmente a adultos jóvenes. Clínicamente se manifiesta con síntomas neurológicos debido a la hipertensión intracraneana. Para establecer el diagnóstico es necesario el uso de estudios de imagen, histopatología e inmunohistoquímica. El tratamiento de elección es la resección quirúrgica total, acompañada en casos selectos de radioterapia y quimioterapia. El pronóstico que generalmente es bueno depende del porcentaje de resección quirúrgica y de las características inmunohistoquímicas del tumor. El presente trabajo tiene como objetivo reportar un caso de un tumor neuroepitelial de baja incidencia y actualizar el manejo diagnóstico y terapéutico. Se presenta el caso de una paciente afrodescendiente de 22 años con un Neurocitoma Central atípico intraventricular bilateral (dominante izquierdo) a quien se le realizó una resección microquirúrgica subtotal del tumor y se colocó un catéter ventricular externo de seguridad y, como terapia coadyuvante, se le administró quimioterapia y radioterapia concurrente. En el seguimiento a los 6 meses luego de la intervención, la paciente presenta una evolución clínica y neurológica favorable.

Central Neurocytoma is a rare benign tumor of the Central Nervous System that mainly affects young adults. Clinically, it manifests with headache, nausea, vomiting, and visual disturbances due to intracranial hypertension. To establish the diagnosis, the use of imaging, histopathology, and immunohistochemistry is necessary. The treatment of choice is total surgical resection, accompanied in selected cases of radiotherapy and chemotherapy. The prognosis that generally is good, depends on the percentage of surgical resection and the characteristics of the tumor. The present work aims to report the case of a low incidence neuroepithelial tumor, such as atypical Central Neurocytoma, and to describe clearly and concisely the main characteristics of the tumor, as well as the diagnostic and therapeutic methods that currently are considered of choice according to international guidelines. We present the case of a 22-year-old patient with an atypical intraventricular Central Neurocytoma who underwent a subtotal microsurgical resection of the tumor and a safety external ventricular catheter placement and received concurrent chemotherapy and radiotherapy as adjunctive therapy. In the follow up 6 months after the intervention, the patient presented a favorable clinical and neurological evolution.

Neurocitoma central: a propósito de un caso / Central neurocytoma: about a case

Introducción: El neurocitoma central fue descripto por primera vez en 1982 por Hassoun et al. Se trata de una neoplasia rara, bien diferenciada del sistema nervioso central de origen neuroectodermico, ubicado más comúnmente a nivel del sistema ventricular, típicamente adyacente al foramen de Monro. Cursa generalmente con síntomas de hipertensión intracraneal secundaria a hidrocefalia no comunicante. Afecta generalmente a adultos jóvenes, con edad de presentación media de 29 años en las mayores series descriptas. Objetivos: Describir y presentar un caso de tumor cerebral específico, cuya importancia se da debido a su baja prevalencia y escasa casuística relatada en la literatura. Descripción del caso: En el presente artículo describimos un caso de una paciente de 35 años diagnosticada incidentalmente con una lesión ocupante de espacio a nivel del ventrículo lateral izquierdo redondeada, heterogénea, de bordes netos con dimensiones de 40x30x30 mm. La paciente fue intervenida quirúrgicamente para su resección. Se realizó abordaje interhemisférico transcalloso homolateral. Sin intercurrencias post-quirúrgicas fue dada de alta 4 días luego de la cirugía. El informe anatomo-patológico demostró tratarse de un Neurocitoma Central. Se comparó nuestro caso con lo descripto en la literatura. Conclusión: El neurocitoma central a pesar de no ser una patología prevalente, debe ser conocido en profundidad por los neurocirujanos, ya que su correcto manejo afecta directamente al pronóstico de los pacientes

Introduction: The central neurocytoma was first described in 1982 by Hassoun et al. It is a rare, well-differentiated neoplasm of the central nervous system of neuroectodermal origin, located most commonly at the level of the ventricular system, typically adjacent to the foramen of Monro. It usually presents with symptoms of intracranial hypertension secondary to non-communicating hydrocephalus. It generally affects young adults, with an average age of presentation of 29 years in the largest series described. Objetives: Describe and present one case of specific brain tumor, which is important due to its your low prevalence and scarce casuistic in the literature. Case presentation: In the present article, we describe a case of a female 35-year-old patient diagnosed incidentally with a heterogeneus rounded space-occupying lesion at the level of the left lateral ventricle, with net edges and dimensions of 40x30x30mm. The patient was surgically intervened for tumoral resection. We opteded to use a homolateral transcallosal interhemisferic approach. Without post-surgical complications, she was discharged 4 days after surgery. The anatomo-pathological report proved to be a Central Neurocytoma. We compared our case with the existing publications. Conclusion: Despite being an uncommon tumor, Central Neurocytoma must be well understood by every neurosurgeon, considering that its adequated management influences the patient ́s prognosis directly

Diagnostic value of magnetic resonance imaging and hydrogen ion magnetic resonance spectroscopy for cen-tral neurocytoma / 中国基层医药

Objective To analyze the clinical value of magnetic resonance imaging ( MRI) and hydrogen proton magnetic resonance spectroscopy (～1H-MRS) in the diagnosis of central neurocytoma (CNC).Methods From February 2010 to February 2018,the clinical data of 12 patients with CNC confirmed by surgery and pathology in Quzhou People＇s Hospital were retrospectively analyzed.All patients underwent MRI diagnosis before operation.Among them,9 patients received ～1H-MRS check.Results In this group of 12 patients,9 patients occurred in the 2/3 area of the lateral ventricle,2 cases occurred in the posterior third of the lateral ventricle,1 case occurred in the frontal lobe;11 cases were solid tumors,and 1 case was cystic tumors.MRI showed plaque calcification in the tumor, distortion of vascular shadow,T1WI was equal low signal,T2WI was contoured mixed signal;8 cases showed uneven light and moderate enhancement,3 cases showed uneven enhancement,1 case showed ring enhancement;8 cases of～1H-MRS showed a significant increase in choline peak (Cho),N-acetylaspartate (NAA) peak decreased,and Cho/NAA increased. Meanwhile, 1 case of glycine peak ( Gly) increased. Conclusion CNC has specific MRI performance,and plays an important role in preoperative diagnosis and differential diagnosis.Combined with ～1H-MRS examination,it can grasp the tumor biochemical index and metabolism from the molecular level and improve the accuracy of preoperative diagnosis.

Diagnostic value of magnetic resonance imaging and hydrogen ion magnetic resonance spectroscopy for central neurocytoma / 中国基层医药

Objective@#To analyze the clinical value of magnetic resonance imaging (MRI) and hydrogen proton magnetic resonance spectroscopy (~1H-MRS) in the diagnosis of central neurocytoma (CNC).@*Methods@#From February 2010 to February 2018, the clinical data of 12 patients with CNC confirmed by surgery and pathology in Quzhou People's Hospital were retrospectively analyzed.All patients underwent MRI diagnosis before operation.Among them, 9 patients received ~1H-MRS check.@*Results@#In this group of 12 patients, 9 patients occurred in the 2/3 area of the lateral ventricle, 2 cases occurred in the posterior third of the lateral ventricle, 1 case occurred in the frontal lobe; 11 cases were solid tumors, and 1 case was cystic tumors.MRI showed plaque calcification in the tumor, distortion of vascular shadow, T1WI was equal low signal, T2WI was contoured mixed signal; 8 cases showed uneven light and moderate enhancement, 3 cases showed uneven enhancement, 1 case showed ring enhancement; 8 cases of ~1H-MRS showed a significant increase in choline peak (Cho), N-acetylaspartate (NAA) peak decreased, and Cho/NAA increased.Meanwhile, 1 case of glycine peak (Gly) increased.@*Conclusion@#CNC has specific MRI performance, and plays an important role in preoperative diagnosis and differential diagnosis.Combined with ~1H-MRS examination, it can grasp the tumor biochemical index and metabolism from the molecular level and improve the accuracy of preoperative diagnosis.

The imaging manifestation and pathological analysis of intracerebroventricular central neurocytoma / 实用医学杂志

Objective To explore the radiological features of intracerebroventricular central neurocytoma and so as to improve the diagnosis of this disease. Methods Thirty patients with pathologically confirmed intra-cerebroventricular central neurocytoma in Nanfang Hospital in past 7 years were analyzed retrospectively for their clinical,radiological and pathological features combined with literatures review. Results Lesions of central neuro-cytoma located at the Monro's foramen areas,the anterior horns and bodies of lateral ventricles.Twenty eight of the patients underwent MRI(with 4 patients received MRS)and 11 patients underwent CT.All cases revealed unilateral or bilateral hydrocephalus of the lateral ventricles and ventriculomegaly.Plain CT revealed heterogeneous isodensity or slighly high density lesions with multiple cystic degeneration,necrosis and calcification while a few lesions(4 le-sions)showed hemorrhage.Heterogeneous isointense or mild hypointense were demonstrated on T1-weight MRI and heterogeneous isointense or slightly hyperintense on T2-weight MRI.Multiple cystic degeneration(always showed as distinctive "soap bubble sign"), necrosis and calcification could also be found within the tumor. The solid compo-nent of tumor revealed slightly high to high signal intensity on DWI while the cystic and necrosis components showed low signal intensity.Most of the tumors showed mild to moderate enhancement with vessels passing through on contrast scan. All cases with 1H MRS showed with obviously increased in Cho peak and decreased in Cr and NAA peaks.In immunohistochemical assay,most of the tumors revealed positive expressions of Neu-N,Synaptophy-sin, GFAP and hypo-level Ki-67. Conclusions The intracerebroventricular central neurocytoma has some typical imaging manifestations.Comprehensive analysis of CT and MRI can be conducive to preoperative diagnosis and dif-ferential diagnosis with other tumors in the lateral ventricle.

Central neurocytoma:MRI manifestation and clinicopathological findings / 实用放射学杂志

Objective To explore the clinical and MRI features of central neurocytoma(CNC).Methods The clinical and MRI data of the pathologically confirmed CNC were retrospectively collected and compared,all the patients had undergone pre-surgery MRI plain and contrast enhanced scans.Patients were divided according to the location of the tumor as intra-lateral ventricle and ex-lateral ventricle group.Results Forty patients were finally enrolled,all tumors were solitary,39 of which were located in the ventricular system,including 2 7 cases located in lateral ventricle and 1 2 in the lateral ventricle,2 in third ventricle,5 in both lateral and third ventricle and 5 involving the whole ventricles;and 1 located in ex-ventricle(left temporal lobe).The tumors showed iso-intense on T1WI and T2WI with varying degrees of cystic degeneration.Hemorrhage was shown in 5 cases,calcification in 2 cases,markedly hyper-vascular enhancements were shown in 36 cases with 26 cases presented visible enhanced vessel sign.The comparison analysis between intra-and ex-lateral ventricle groups revealed that,the patients age,tumor size and Ki-67 expression were higher in ex-lateral ventricle group(P<0.05);hypervascular enhancement (P<0.05),gender ratio,cystic/cystic-solid appearance and vessel sigh(P>0.05)were lower than those in intra-lateral ventricle group.Conclusion CNC is typically located in lateral ventricle,with cystic and necrotic degeneration areas,hypervascular enhancement with visible vessels in solid portion on enhanced MR images;The ex-lateral ventricle CNC is rare,it commonly presents a larger tumor with high Ki-67 expression,and moderate enhancement with less visible vessels on enhanced MRI.

MRI characteristic features for diagnosing central neurocytoma / 中国医学影像技术

Objective To investigate the diagnostic value of six MRI characteristic features for diagnosing central neurocytoma (CN).Methods MRI data of 30 cases of CN and 68 cases of non-CN located in lateral ventricles were retrospectively analyzed.Six characteristic MRI features,including scalloping sign,broad-based attachment sign,soapbubble sign,peripheral cyst sign,fluid-fluid level sign and gemstone sign were scored based on a five-point scale.ROC curve was used to assess the diagnostic value of each MRI sign.Results The scalloping sign showed the highest area under the curve (AUC) value (0.82) among all 6 signs (all P＜0.05),followed by broad-based attachment,soap-bubble andperipheral cyst signs (AUC 0.73-0.75),higher than that of fluid-fluid level sign and gemstone sign (all P＜0.05).The scalloping sign exhibited the highest specificity (84.56 ％),followed by fluid-fluid level (77.94 ％),gemstone (74.26 ％) and peripheral cyst (70.34％) sign.The soap-bubble sign (83.89％) was the most sensitive sign,followed by broad-based attachment sign (76.11％) and peripheral cyst sign (75.00％).Conclusion The scalloping sign is the most valuable indicator for CN among six characteristic MRI features.

Systematic Analysis of Clinical Outcomes Following Stereotactic Radiosurgery for Central Neurocytoma

Central neurocytoma (CN) typically presents as an intraventricular mass causing obstructive hydrocephalus. The first line of treatment is surgical resection with adjuvant conventional radiotherapy. Stereotactic radiosurgery (SRS) was proposed as an alternative therapy for CN because of its lower risk profile. The objective of this systematic analysis is to assess the efficacy of SRS for CN. A systematic analysis for CN treated with SRS was conducted in PubMed. Baseline patient characteristics and outcomes data were extracted. Heterogeneity and publication bias were also assessed. Univariate and multivariate linear regressions were used to test for correlations to the primary outcome: local control (LC). The estimated cumulative rate of LC was 92.2% (95% confidence interval: 86.5-95.7%, p<0.001). Mean follow-up time was 62.4 months (range 3-149 months). Heterogeneity and publication bias were insignificant. The univariate linear regression models for both mean tumor volume and mean dose were significantly correlated with improved LC (p<0.001). Our data suggests that SRS may be an effective and safe therapy for CN. However, the rarity of CN still limits the efficacy of a quantitative analysis. Future multi-institutional, randomized trials of CN patients should be considered to further elucidate this therapy.

MR imaging manifestations of extraventricular neurocytoma / 实用放射学杂志

Objective To analyze retrospectively the MR manifestations and differential diagnosis of extraventricular neurocytoma (EVN).Methods 7 patients with EVN confirmed by pathologic examination were performed non-enhanced and contrast-enhanced MR scan,and one of them was also performed DWI.Results 4 tumors were located in cerebra,2 tumors located in cerebellum,involving both cortex and white matter,and 1 tumor was located in the seller region.5 tumors were solid-cystic lesions and 2 tumors were solid lesions.5 lesions were well demarcated and 2 lesions were ill defined.Edema around the lesion was found in 2 cases.The solid part of tumors showed iso-or hyper intensity on T1WI,hypo-or hyper intensity on T2WI.Necrosis and hemorrhage occurred in some cases.The degree of enhancement in solid part ranged from mild enhancement to intense enhancement heterogeneously.The cystic part showed ring like enhancement with a thin wall.Conclusion The manifestations of EVN on MRI have some characteristics.If a solid-cystic lesion was well defined,involving both cortex and white matter,EVN should be considered.

Central Neurocytoma: A Review of Clinical Management and Histopathologic Features

Central neurocytoma (CN) is a rare, benign brain tumor often located in the lateral ventricles. CN may cause obstructive hydrocephalus and manifest as signs of increased intracranial pressure. The goal of treatment for CN is a gross total resection (GTR), which often yields excellent prognosis with a very high rate of tumor control and survival. Adjuvant radiosurgery and radiotherapy may be considered to improve tumor control when GTR cannot be achieved. Chemotherapy is also not considered a primary treatment, but has been used as a salvage therapy. The radiological features of CN are indistinguishable from those of other brain tumors; therefore, many histological markers, such as synaptophysin, can be very useful for diagnosing CNs. Furthermore, the MIB-1 Labeling Index seems to be correlated with the prognosis of CN. We also discuss oncogenes associated with these elusive tumors. Further studies may improve our ability to accurately diagnose CNs and to design the optimal treatment regimens for patients with CNs.

Role of adjuvant radiation in the management of central neurocytoma: Experience from a tertiary cancer care center of India.

BACKGROUND AND OBJECTIVE: Neurocytoma (NC) is a rare benign neuronal tumor. A complete excision remains curative for most of these tumors, but atypical histology and extra‑ventricular location often necessitates adjuvant therapy. We intended to explore the clinico‑pathological features and treatment outcome in patients of NC in our institute. MATERIALS AND METHODS: Medical records were reviewed and data collected on NC over a 6‑year period (2006‑2012) from the departmental archives. Disease free survival (DFS) was analyzed by Kaplan‑Meier method. RESULTS: A total of 18 patients met the study criteria. Fourteen patients had intra‑ventricular neurocytoma (IVNC), right lateral ventricle being the most common site of origin. Gross total resection and near total resection were achieved in eight cases each whereas tumor decompression and biopsy could be done in two cases. On post‑operative histopathological examination, eight patients were found to have atypical NC while 10 patients had typical NC. All patients underwent adjuvant radiation. The median dose of post‑operative radiation was 56 Gy. All patients were alive at their final follow‑up. One patient had both clinical and radiological evidence of local relapse. In the evaluable patients (n = 18), after a median follow‑up of 35 months the DFS rate at 2 years and 3 years are 100% and 83% respectively. CONCLUSION: Use of adjuvant radiation to a total dose of 56 Gy enhances the local control and achieves superior survival in patients of NC. Use of 3D conformal planning techniques may help us to achieve better therapeutic ratio in patients with NC.

Clinicopathological analysis of 12 cases with extraventricular neurocytoma / 临床与实验病理学杂志

Purpose To study the clinicopathological features of extraventricular neurocytoma. Methods 12 cases of extraventricular neurocytoma were retrospectively analyzed for their clinical information and histopathological features. Results The distribution of ex-traventricular neurocytoma was as follows: cerebral hemisphere (5 cases), right thalamus (2 cases), cerebellar vermis (2 cases), saddle area (2 cases), and spinal cord (1 case). The histological features were round uniform cells, round or oval nuclei, with peri-nuclear clearing commonly seen. Neuropil-like structure, ganglion cells or ganglion-like cells, perivascular rosettes, calcification, cap-illary wall thickening and hyaline degeneration and other pathological changes were also observed. All cases showed strong immunoreac-tivity for Syn. 7 cases exhibited Ki-67 labeling index of≤2%, and other 5 cases showed 3% ~8%. 3 cases were performed with fluo-rescence in situ hybridization ( FISH) to detect loss of chromosome 1p/19q, but no loss was detected. Postoperative follow-up for 4 months to 4 years showed one case had tumor recurrence and intracranial spread in postoperative 2 years. Conclusions Extraventricu-lar neurocytoma shares similar histological features with central neurocytoma, but often more complex. Ganglion cells or ganglion-like cells, calcification, capillary wall thickening and hyaline degeneration are more frequently seen, but neuropil-like structure is less seen. Extraventricular neurocytoma needs differential diagnoses from oligodendroglioma, ependymoma, dysembryoplastic neuroepitheli-al tumor and others. Immunohistochemistry and chromosome 1p/19q loss can help in the differential diagnosis. The treatment for ex-traventricular neurocytoma is grossly total resection, with post-operative radiotherapy for subtotal resection, atypical or recurrent cases.

Diagnosis of central neurocytoma by MRI:a report of 9 cases / 局解手术学杂志

Objective To study the clinical significance of MR imagings features in the diagnosis on central neurocytoma ( CNC ) . Methods From January 2010 to December 2012,9 patients with CNC were analyzed and examined by MRI before surgery,then received postoperative pathology examination. Results CNC were in the left lateral ventricle of 9 patients, closely linked with the Monro hole, of which 4 cases were in the first 2/3 of central ventricle of the left lateral ventricle,3 cases in the septum pellucidum and growth to bilateral ventricles,2 cases of infiltrating in the septum pellucidum and base side adhesion. For CNC,MRI signal was not uniform,solid part T1WI showed equal or slightly low signal,multiple cysts and signal cord like structure with the ventricular wall and septum pellucidum adhesion. By contrast-enhanced CT scan,there were the solid part heterogeneous obvious enhancement in 4 cases,moderate and slight uneven enhancement in 3 cases and 2 cases respectively. Uniform size,round or oval cells were showed by HE staining,and the synaptophysin was positive in 6 ca-ses by immunohistochemical staining,positive expression of glial fibrillary acidic protein in 3 cases. After operation,3 patients were lost to fol-low up,for 6 cases were followed up,survival of 2 cases in 3 years,4 cases in 2 years. Conclusion The results suggest that MRI display is located near the lateral ventricle central Monro hole before and lesions suggestive of CNC in young patients. For most CNC,synaptophysin has positive expression by immunohistochemical staining.

Magnetic resonance imaging diagnosis of central neurocytoma / 中国医师进修杂志

Objective To evaluate the characteristic appearance and diagnosis value of central neurocytoma on magnetic resonance imaging (MRI).Methods MRI data of 9 patients with central neurocytoma confirmed surgically and pathologically were retrospectively reviewed.Results All the lesions located in the lateral ventricle septum pellucidum,adjacent to Monro hole,in the anterior 1/3 to 2/3 of the lateral ventricle.Most of them were irregularity,sublobe and sharpness of border.They presented as inhomogeneous intense signal on MRI,with slight to equal enhancement on enhancement.DWI could be high or slightly high signal.Conclusion Central neurocytoma has the characteristic appearances on MRI,being helpful to the diagnosis.

Diagnosis and treatment of central neurocytoma: A report of 7 cases with review of the literature / 肿瘤

Objective: To analyze the imaging findings, diagnosis and treatment of central neurocytoma. Methods: The clinical information of 7 cases of central neurocytoma in Second Hospital of Hebei Medical University from January 2005 to March 2013 was retrospectively analyzed. The surgical experience was summarized, and the related literatures were reviewed. Results: Of 7 cases, 3 were females and 4 were males; 6 had tumor in anterior 2/3 of the ventricle, 5 originated from septum pellucidum around monro foramen, 1 originated from lateral ventricular wall. The MRI showed that the lesions were mostly hypointense and displayed mixed signals on T1WI images, mainly hypointense and slightly hyperintense signals on T2WI images; most of neurocytomas showed heterogeneous enhancement on dynamic contrast-enhanced MRI images. Six cases underwent gross total resection, 1 case underwent subtotal resection. Two cases received postoperative radiotherapy, 1 case received adjuvant chemotherapy. One case died postoperatively in hospital. Five cases were followed-up, and 1 case was lost to follow-up. The follow-up time was from 15 to 25 months. Four cases had satisfying prognosis, 1 case had hemiplegic paralysis, and no cases had recurrent tumor. Conclusion: Microneurosurgery is the best treatment for central neurocytoma, mastering the technique and key points of operation can achieve significant clinical effectiveness and improve the prognosis. Copyright © 2013 by TUMOR.

5-Aminolevulinic Acid Fluorescence Discriminates the Histological Grade of Extraventricular Neurocytoma

Extraventricular neurocytomas are rare brain tumors that have a diverse range of clinical characteristics. We describe two cases involving fluorescence-guided resection of extraventricular neurocytoma using 5-aminolevulinic acid (5-ALA) and evaluate the efficacy of the technique. We found that the tumor reactions to 5-ALA differed depending on the histologic grade. This finding shows that the 5-ALA fluorescence reaction may potentially be used as a biomarker of the clinical behavior of these tumors. To our knowledge, this is the first report in which fluorescence-guided resection was utilized for the resection of extraventricular neurocytomas.

Resección endoscópica guiada por estereotaxia de un neurocitoma pineal: reporte de un caso / Guided stereotactic endoscopic resection of a pineal neurocytoma: a case report

El neurocitoma debe considerarse en el diagnóstico diferencial de los tumores pineales. La éxeresis quirúrgica total es esencial, y se puede realizar a través de diferentes abordajes: trans-cerebeloso, transcallosal, transcortical-Transventricular. La cirugía con puerto cerebral (brain port) es un abordaje quirúrgico que combina la resección endoscópica guiada por estereotaxia delesiones expansivas intraventriculares. Este proceder es una técnica mínimamente invasiva y es capaz de lograr la resección tumoral con mínimo daño al tejido nervioso. Reportamos un caso con un neurocitoma pineal, una paciente femenina de 29 años, Que debutó con hipertensión intracraneal secundaria a hidrocefalia obstructiva. Se logró la exéresis quirúrgica mediante brain port sin complicaciones. Los marcadores tumorales previos a la cirugía fueron negativos. El índice MIB-1 fue menor a un 4 por ciento y durante un año de seguimiento no se ha demostrado recidiva tumoral. La cirugía brain-port es una técnica segura para el abordaje a tumores de región pineal.

Neurocytoma should be considered in the differential diagnosis of pineal tumours. Total surgical resection is essential, through different approaches: transcortical-transventricular, transcallosal and transcerebellar. Brain port surgery is a surgical approach that combines endoscopic resection via stereotactic guide in intraventricular mass. This procedure is a minimally invasive technique and it is able to achieve tumour resection with minimal nervous tissue damage. We report a case with a pineal neurocytoma, a 29 years old female patient with symptoms of intracranial hypertension secondary to obstructive hydrocephalus. Total removal was done with brain port surgery without complications. Tumoral markers (AFP, CEA, HCG-ß) performed previously to surgery were negative. MIB-1 index was less than 4 percent and patient’s follow-up during one year after surgery didn’t show recurrence. Brain port surgery is considered as a safe technique to approach pineal region tumours.

Neurocitoma central atípico: relato de caso / Atypical central neurocytoma: case report

Neurocitomas centrais são tumores do sistema nervoso central de comportamento usualmente benigno, que costumam ocorrer nos ventrículos laterais de indivíduos jovens. Apresentamos o caso de um paciente do sexo masculino, de 31 anos de idade, com uma lesão expansiva intraventricular ocasionando quadro de hidrocefalia e queixas visuais. O paciente foi submetido à ressecção completa da lesão. Apresentou hidrocefalia persistente no pós-operatório, necessitando de derivação ventrículo-peritoneal. A evolução foi favorável e o paciente recebeu alta para seguir acompanhamento ambulatorial. Exame anatomopatológico evidenciou neurocitoma central atípico. O objetivo deste relato é apresentar um caso clássico de uma neoplasia rara, realizando uma revisão da literatura e mostrando sua importância, dado o prognóstico favorável.

Central neurocytomas are central nervous system tumors. They are usually benign and tend to occur in the lateral ventricles of young adults. We report the case of a 31-year-old male patient with an intraventricular lesion, causing hydrocephalus and visual complaints. The patient was submitted to a complete surgical resection of the lesion. However, he persisted with hydrocephalus in the postoperative period and required a ventriculoperitoneal shunt. The clinical course was favorable and the patient was discharged to follow-up. Anatomic pathology test revealed an atypical central neurocytoma. The objective of this case report is to describe a classic presentation of a rare neoplasm, including a review of the literature, highlighting the importance of this diagnosis because of the favorable prognosis.

Atypical Extraventricular Neurocytoma

The authors report a case of atypical extraventricular neurocytoma (EVN) transformed from EVN which had been initially diagnosed as an oligodendroglioma 15 years ago. An 8-year-old boy underwent a surgical resection for a right frontal mass which was initially diagnosed as oligodendroglioma. When the tumor recurred 15 years later, a secondary operation was performed, followed by salvage gamma knife treatment. The recurrent tumor was diagnosed as an atypical EVN. The initial specimen was reviewed and immunohistochemistry revealed a strong positivity for synaptophysin. The diagnosis of the initial tumor was revised as an EVN. The patient maintained a stable disease state for 15 years after the first operation, and was followed up for one year without any complications or disease progression after the second operation. We diagnosed an atypical extraventricular neurocytoma transformed from EVN which had been initially diagnosed as an oligodendroglioma 15 years earlier. We emphasize that EVN should be included in the differential diagnosis of oligodendroglioma.