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.

Central neurocytomas of uncommon locations: report of two cases / Localizações incomuns do neurocitoma central: relato de dois casos

We report two patients with central neurocytomas at an uncommon location in the brain. The first, a 58-year-old man presenting with signs and symptoms of increased intracranial pressure, had a tumor located at the pineal region. The second, a 21-year-old woman with tumor in the aqueductal region had worsening migraine-like headaches and diplopia. Both patients had obstructive hydrocephalus treated by neuroendoscopic third ventriculostomy and biopsy of the tumors. No additional treatment was done. We conclude that neurocytomas should be considered in the differential diagnosis of tumors located in the pineal and aqueductal regions.

Relatamos dois pacientes com neurocitoma central com localização incomum no sistema nervoso central. O primeiro, 58 anos, masculino, apresentava sinais e sintomas de hipertensão intracraniana, tinha um tumor na região da pineal. O segundo, feminino, 21 anos, tinha um tumor na região do aqueduto de Sylvius e apresentava cefaléia migranosa progressiva e diplopia. Ambos apresentavam hidrocefalia obstrutiva tratada com terceiroventriculostomia endoscópica e biópsia da lesão. Não foi feito tratamento adicional. Concluimos que os neurocitomas devem ser considerados no diagnóstico diferencial de tumores localizados na região da pineal e do aqueduto.

Central neurocytoma: a clinico-pathological study of eight cases.

Central neurocytomas are benign neuronal tumours generally found in the lateral or third ventricles. They are rare, comprising < 1% of all brain tumours. It is frequently confused with other tumours of the central nervous system particularly oligodendroglioma. The present study was done to analyse the histopathological features including immunohistochemical profile of these rare tumours. Eight cases were taken up for the study. Seven of the cases had an intraventricular location and one was located outside the ventricles. Increased intracranial pressure was the most common presenting symptom. Microscopically all tumours were composed of small uniform cells with perinuclear halos and regular round nuclei. The tumour in extraventricular location showed atypical features. Immunohistochemistry showed positivity for neuronal markers. The present series highlights the characteristic clinical and pathological findings of this rare brain tumour. Immunostaining for neuronal markers are essential for distinguishing them from other small round cell tumours of the brain.

Peritoneal dissemination from central neurocytoma: case report

OBJECTIVE: central neurocytoma is a low grade tumor of neuroglial origin and a relatively new histological entity. Only a few cases have been reported and its biological behavior is still uncertain. Some cases have shown an aggressive behavior (local recurrence, malignant dedifferentiation or CSF dissemination) and challenged the initial view of its relative benignity. A case of central neurocytoma with peritoneal dissemination is presented. CASE: a six years-old boy with recurrent neurocytoma of III ventricle and left thalamus showed fast growth of tumor rest and ascites three and a half years after subtotal removal of the lesion. Tumor cells were identified in the ascitic fluid and implanted in the peritoneum. Chemotherapy was initiated immediately after diagnosis of peritoneal dissemination (etoposide, carboplatin, doxorubicin and cyclophosphamide). The patient developed metabolic imbalance and respiratory failure due to rapid formation of ascitic fluid and died 3 days after the diagnosis of peritoneal dissemination was established. CONCLUSION: central neurocytoma is a low grade tumor with low values of the proliferative index in the majority of cases. In spite of that, some tumors may present a very aggressive behavior and extraneural dissemination. Evaluation of proliferative index may be a guideline parameter for planning adjuvant therapies after surgical treatment in selected cases. Extraneural dissemination may occur in some cases specially in patients with ventriculoperitoneal shunt

MR Imaging and Histopathologic Findings of A Case of Cerebral Ganglioneurocytoma

We report a case of ganglioneurocytoma manifesting as a complex partial seizure in a young adult male. MR images depicted a well-marginated cystic mass with a heterogeneous solid portion abutting the dura in the parietal lobe. The solid portion showed minimal heterogeneous enhancement, and pressure erosion of the overlying calvarium had occurred. Following gross total resection, the clinical outcome was satisfactory, with no further seizures, and during the five-year follow-up period, the tumor did not recur.

Neurocitomas do sistema nervoso central: análise clínico-patológica de três casos / Central nervous system neurocytomas: clinicopathological analysis of 3 cases

Os neurocitomas do sistema nervoso central säo tumores raros, na maioria intracranianos e caracterizados pela sua benignidade. Por muito tempo permaneceram insuficientemente conhecidos, em razäo da sua distinçäo clínica e radiológica difícil entre os tumores intraventriculares (ependimomas, papilomas do plexo coróide, oligodendrogliomas, astrocitomas sub-ependimßrios) e da semelhança histolóógica com os oligodendrogliomas. O diagnóstico passou a ser possível através da análise imuno-histoquínica específica e da microscopia eletrônica. Nós apresentamos três casos clínicos de neurocitomas intraventriculares confirmados pelo estudo imuno-histoquínico. A apresentaçäo clínica, os dados radiológicos, cirúrgicos, anatomopatológicos e o prognóstico seräo discutidos em comparaçäo aos dados recentes da literatura

Neurocitoma intraventricular: reporte de dos casos y análisis de posibilidades terapéuticas / Intraventricular neurocytoma: report ot two cases and therapeutic possibility analysis

El neurocitoma intraventricular o también llamado central es un tumor cerebral benigno, raro, recientemente descrito. Sus características más comunes incluyen: predominio de presentación en adultos jóvenes, localización a nivel del septum pellicidum y fórnix ventriculares, así como sintomatología frecuente de hipertensión intracraneana debida a hidrocefalia obstructiva. Histopatológicamente, este tumor se caracteriza por una población celular homogénea con diferenciación neuronal específica. Los hallazgos radiológicos más comunes son la presencia de calcificaciones, afección frecuente de los ventrículos laterales y III, volumen tumoral bien circunscrito, entre otros, lo cual ayuda a diferenciarlo de otros tumores intraventriculares del sistema nervioso central. El tratamiento habitual es quirúrgico y consiste en un abordaje anterior a través del cuerpo callos, generalmente con buenos resultados pronósticos. La radioterapia debe contemplarse en los casos de residual post-quirúrgico o en la recurrencia tumoral. Sin embargo, la radiosensibilidad del neurocitoma no está bien establecida y se requieren estudios posteriores para determinar la susceptibilidad de esta neoplasia a la irradiación

Intraventricular neurocytoma: a report of two cases.

Central neurocytomas are uncommon intracranial neoplasms. More than one hundred cases are documentated in the literature. In this report we describe the clinical and histopathological features in two patients with intraventricular neurocytoma. As the light microscopic features of neurocytoma resemble with that of an oligodendroglioma, it is essential to differentiate these two tumours, using either ultrastructural or immunohistochemical techniques.