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.

Lesiones oncológicas intracraneales de apariencia quística / Oncologic Intracranial Lesions with Cystic Appearance

Resumen La presencia de lesiones quísticas de etiología oncológica en el encéfalo es cada vez más frecuente. El rol del especialista en imágenes es describir sus características, morfología y comportamiento con el contraste endovenoso, para llegar a realizar un diagnóstico presuntivo y enumerar sus diagnósticos diferenciales, y así orientar al médico tratante. Haremos una revisión de las lesiones quísticas de etiología oncológica de presentación frecuente en el encéfalo.

Abstract The presence of an oncologic cystic formation in the brain is frequent. The imaging specialist's role is to describe its features, morphology, and image enhancement characteristics with endovenous contrast, to make a presumptive diagnosis, and enumerated differential diagnoses and thus orientate the practitioner. We will analyze the most common expansive cystic formations.

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

Neurocitoma central: presentación de un caso / Central Neurocytoma: A Case Report

El neurocitoma central es un tumor raro, de muy baja incidencia y de pronóstico favorable. Su presentación radiológica muestra similitudes con otros tumores cerebrales. Se describe el caso de un paciente de 36 años con diagnóstico incidental de neurocitoma central intraepitelial. Después de 13 años del diagnóstico el paciente refiere sintomatología clínica de cefalea y bradipsiquia. El paciente rechazó la cirugía. El diagnóstico y seguimiento es posible mediante imagen por resonancia magnética con espectroscopia.

Intraepithelial central neurocytoma is a rare tumor, with very low incidence and favorable prognosis. Its radiological presentation shows similarities with other brain tumors. The case of a 36-year-old patient with an incidental diagnosis of central intra-epithelial neurocytoma is described. After 13 years of diagnosis the patient refers clinical symptoms of headache and bradypsychia. The patient refused surgery. Diagnosis and follow-up is possible by means of magnetic resonance imaging with spectroscopy.

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.

Hypothalamic Extraventricular Neurocytoma (EVN) in a Pediatric Patient: A Case of EVN Treated with Subtotal Removal Followed by Adjuvant Radiotherapy

Extra ventricular neurocytoma (EVN) is a rare brain tumor with histologic features similar with a central neurocytoma, but located outside of the ventricular system. In this study, we present an unusual case of hypothalamic EVN in a 14-year-old patient. The patient underwent subtotal removal and had tumor relapse. The patient was then treated using intensity modulated radiation therapy, and the tumor remained stable for 24 months. This case report may be important in that this is the first pediatric case of EVN located in the hypothalamic region. EVN has similar radiologic features with pilocytic astrocytomas and therefore a hypothalamic EVN may be misdiagnosed as a hypothalamic glioma. Also, the pathologic-radiologic-clinical correlation of EVN located in the hypothalamic area may be different from that of EVNs originating from other usual sites.

Hypothalamic Extraventricular Neurocytoma (EVN) in a Pediatric Patient: A Case of EVN Treated with Subtotal Removal Followed by Adjuvant Radiotherapy

Extra ventricular neurocytoma (EVN) is a rare brain tumor with histologic features similar with a central neurocytoma, but located outside of the ventricular system. In this study, we present an unusual case of hypothalamic EVN in a 14-year-old patient. The patient underwent subtotal removal and had tumor relapse. The patient was then treated using intensity modulated radiation therapy, and the tumor remained stable for 24 months. This case report may be important in that this is the first pediatric case of EVN located in the hypothalamic region. EVN has similar radiologic features with pilocytic astrocytomas and therefore a hypothalamic EVN may be misdiagnosed as a hypothalamic glioma. Also, the pathologic-radiologic-clinical correlation of EVN located in the hypothalamic area may be different from that of EVNs originating from other usual sites.

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.

Treatment strategies for huge central neurocytomas / 华中科技大学学报（医学）（英德文版）

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

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 extraventricular atípico con siembra por trayecto de biopsia y diseminación leptomeníngea craneoespinal: reporte de primer caso y revisión de la literatura / Atipical extraventricular neurocytoma with needle tract seeding and craniospinal dissemination: case report and literature review

Objetivo: reportar el primer caso de EVN atípico con siembra neoplásica por trayecto de biopsia y diseminación craneoespinal. Descripción: paciente de 19 años con debilidad y parestesias en hemicuerpo derecho de dos meses de evolución evidenciando en resonancia magnética (IRM) tumor talámico izquierdo sin realce tras el contraste. Intervención: la biopsia esterotáctica revela un tumor redondo-celular compatible con oligodendroglioma. Se indica radioterapia y quimioterapia. Al tercer mes presenta hidrocefalia por progresión tumoral. Se coloca shunt de LCR. Al año se intensifica el síndrome talámico, presenta trastornos oculomotores y esfinterianos. IRM evidencia compromiso leptomeníngeo difuso con implantes nodulares espinales, en tronco encefálico y a nivel de abordaje estereotáctico frontal izquierdo compatible con siembra por trayecto de biopsia. Craneotomía centrada en trepanación previa permite resecar tejido neoplásico reinterpretado anatomopatológicamente como EVN atípico diseminado con Ki67>30%. Conclusión: los oligodendrogliomas representan el principal diagnóstico diferencial imagenológico y anatomopatológico de EVN. Se presume su origen en células precursoras biopotenciales de matriz germinal periventricular con capacidad de diferenciación glial y neuronal que explicaría su capacidad excepcional para la diseminación leptomeníngea. El Ki67 es el principal factor pronóstico evolutivo en neurocitomas. Aquellos con Ki67>2% (atípicos) requieren monitoreo estricto por mayor riesgo de recurrencia y diseminación.

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.

Emotion and cognitive function assessment of patients with central neurocytoma resection through transcortical frontal approach: a 5-year postoperative follow-up study / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Central neurocytoma accounts for 0.1% of primary brain tumor that often occurs in young adults. Surgery is the main treatment for central neurocytoma and the rate of 5-year survival reaches up to over 90%. This study aimed to assess the effect of transcortical frontal approach to surgical resection of central neurocytoma on emotion and cognitive function 5 years after surgery.</p><p><b>METHODS</b>Telephone following-up visits were used in this study. By means of neuropsychological testing, assayed emotion, memory and abstract thinking ability of 18 patients undergoing central neurocytoma resection by transcortical frontal approach for 5 years or more, with another 21 normal cases as control group were enrolled. The data were analyzed statistically by paired t test with SPSS11.5.</p><p><b>RESULTS</b>Patients whose central neurocytoma was removed by transcortical frontal approach were not affected on calculating ability 5 years after operation while ability of memory declined sharply (P = 0.000), the older, the more sharply (P = 0.036). Ability of abstract thinking was significantly reduced (P = 0.000), the older, the more significantly as well (P = 0.012); additionally, anxiety and depression occurred in patients rather more than those of control group (P = 0.000), especially cognitive impairment.</p><p><b>CONCLUSIONS</b>Transcortical frontal approach for surgical resection of central neurocytoma has certain long-term influence on patients' life quality, vulnerable to anxiety, depression and cognitive impairment, the severity of which was correlated to age. Therefore, improving surgical approach will be of value for better long-term life quality of patients.</p>

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.

Neurocitoma o tumor epitelial disembrioplásico / Neurocytoma or dysembryoplasia epithelial tumor

El neurocitoma o tumor epitelial disembrioplásico (TND) es un tumor infrecuente del sistema nervioso central (SNC) clasificado Grado I, según la OMS, y las series que aparecen en la literatura médica informan de pocos casos. Por considerarse una entidad poco frecuente y por haber asistido en un corto período a 2 pacientes adolescentes uno con recidiva de tumor cerebral que fue reintervenido y donde el diagnóstico patológico inicial se correspondía con un astrocitoma de bajo grado y la otra, llevada a sala de operaciones con el diagnóstico de astrocitoma de alto grado, y donde después de estudios de inmunohistoquímica el diagnóstico final fue de tumor neuroepitelial disembrioplásico extraventricular. Se indicó tratamiento con radiaciones ionizantes. Se decidió revisar la literatura y presentar estos 2 casos

The Neurocytoma or dysembryoplasia epithelial tumor (DET) is an infrequent tumor of the central nervous system (CNS) classified as Degree I according to the WHO and the series appearing in the medical literature report not much cases. It is considered a not much frequent entity and due to the care during a short period to 2 adolescent patients, one of them with relapse of cerebral tumor re-operated on and where the former pathologic diagnosis was in correspondence with a high degree astrocytoma and that after the immunohistochemistry studies the final diagnosis was of a extraventricular dysembryoplasia neuroepithelial tumor. The treatment prescribed was with ionizing radiations. Literature was reviewed and two cases were presented

Primary Culture of Central Neurocytoma: A Case Report

A seventeen-year-old female patient was admitted with sudden-onset of headache and vomiting. Brain magnetic resonance imaging demonstrated a heterogeneously enhancing tumour in the left lateral ventricle. The tumour was removed and confirmed as a central neurocytoma (CN). For the residual tumour in the left lateral ventricle, gamma knife stereotactic radiosurgery was done at fifteen months after the initial surgery. Tumour recurred in the 4th ventricle at 5 yr after initial surgery. The tumour was removed and proved as a CN. In vitro primary culture was done with both tumours obtained from the left lateral ventricle and the 4th ventricle, respectively. Nestin, a neuronal stem cell marker was expressed in reverse Transcriptase-Polymerase Chain Reaction of both tumors. Both tumours showed different morphology and phenotypes of neuron and glia depending on the culture condition. When cultured in insulin, transferrin selenium and fibronectin media with basic fibroblast growth factors, tumour cells showed neuronal morphology and phenotypes. When cultured in the Dulbeco's Modified Essential Media with 20% fetal bovine serum, tumors cells showed glial morphology and phenotypes. It is suggested that CN has the characteristics of neuronal stem cells and potential to differentiate into mature neuron and glial cells depending on the environmental cue.

Clinicopathological analysis of central and extraventricular neurocytoma: a report of 17 cases / 华中科技大学学报（医学）（英德文版）

Neurocytoma, a rare brain tumor, is characterized by a mass located mainly in cerebral ventricles. It is prone to be misdiagnosed as oligodendroglioma or ependymoma due to their similar histopathological features in clinical practice. This study aimed to examine the clinicopathological features and differential diagnosis of central and extraventricular neurocytoma. The clinical and histopathological data of 17 patients (male: female=7:10; age: 4-41 years; mean age: 27.4 years) with central or extraventricular neurocytoma were retrospectively analyzed. These patients showed typical radiological, histopathological and immunohistochemical features of neurocytoma. The tumor tissue was found to be composed of small uniform cells with round nuclei and clear cytoplasm resembling that of oligodendroglioma and ependymoma. Immunohistochemistry revealed the tumor tissues were positive for neuronal markers such as synaptophysin (SYN) and neuronal nuclear antigen (NeuN). It was concluded histopathological features of neurocytoma overlaps with some tumors in the central neural system. Immunopositivity for SYN and NeuN can help differentially diagnose neurocytoma.