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BACKGROUND: The management of lateral ventricle tumors requires a balance between maximizing safe resection and preserving neurological function. METHOD: The authors present a successful case of a left lateral ventricular central neurocytoma resection. The trans-superior frontal sulcus approach was employed, providing a safe corridor while minimizing damage to the surrounding neuroanatomy. The use of an endoscope further facilitated the procedure, enabling the confirmation of complete tumor removal and the preservation of deep venous drainage and periventricular structures. CONCLUSION: This case highlights the utility of the trans-sulcal approach and the benefits of endoscopic assistance in the management of lateral ventricle tumors.
Assuntos
Neoplasias do Ventrículo Cerebral , Neurocitoma , Humanos , Neurocitoma/cirurgia , Neurocitoma/patologia , Neurocitoma/diagnóstico por imagem , Neoplasias do Ventrículo Cerebral/cirurgia , Neoplasias do Ventrículo Cerebral/diagnóstico por imagem , Neoplasias do Ventrículo Cerebral/patologia , Ventrículos Laterais/cirurgia , Ventrículos Laterais/diagnóstico por imagem , Ventrículos Laterais/patologia , Procedimentos Neurocirúrgicos/métodos , Masculino , Adulto , Feminino , Resultado do TratamentoRESUMO
INTRODUCTION: Central Neurocytoma (CN) is a rare, WHO grade 2 brain tumor that predominantly affects young adults. Gross total resection (GTR) is often curative for CNs, but the optimal treatment paradigm including incorporation of RT, following subtotal resection (STR) and for scarcer pediatric cases has yet to be established. METHODS: Patients between 2001 and 2021 with a pathologic diagnosis of CN were reviewed. Demographic, treatment, and tumor characteristics were recorded. Recurrence free survival (RFS) and overall survival (OS) were calculated according to the Kaplan Meier-method. Post-RT tumor volumetric regression analysis was performed. RESULTS: Seventeen adults (≥ 18 years old) and 5 children (< 18 years old) met the criteria for data analysis (n = 22). With a median follow-up of 6.9 years, there was no tumor-related mortality. Patients who received STR and/or had atypical tumors (using a cut-off of Ki-67 > 4%) experienced decreased RFS compared to those who received GTR and/or were without atypical tumors. RFS at 5 years for typical CNs was 67% compared to 22% for atypical CNs. Every pediatric tumor was atypical and 3/5 recurred within 5 years. Salvage RT following tumor recurrence led to no further recurrences within the timeframe of continued follow-up; volumetric analysis for 3 recurrent tumors revealed an approximately 80% reduction in tumor size. CONCLUSION: We provide encouraging evidence that CNs treated with GTR or with RT after tumor recurrence demonstrate good long-term tumor control.
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Neoplasias Encefálicas , Neurocitoma , Humanos , Neurocitoma/patologia , Neurocitoma/terapia , Neurocitoma/mortalidade , Masculino , Feminino , Adolescente , Adulto , Neoplasias Encefálicas/terapia , Neoplasias Encefálicas/patologia , Neoplasias Encefálicas/mortalidade , Criança , Adulto Jovem , Seguimentos , Pessoa de Meia-Idade , Pré-Escolar , Recidiva Local de Neoplasia/patologia , Estudos Retrospectivos , Resultado do Tratamento , Taxa de SobrevidaRESUMO
We explored the genomic events underlying central neurocytoma (CN), a rare neoplasm of the central nervous system, via multiomics approaches, including whole-exome sequencing, bulk and single-nuclei RNA sequencing, and methylation sequencing. We identified FGFR3 hypomethylation leading to FGFR3 overexpression as a major event in the ontogeny of CN that affects crucial downstream events, such as aberrant PI3K-AKT activity and neuronal development pathways. Furthermore, we found similarities between CN and radial glial cells based on analyses of gene markers and CN tumor cells and postulate that CN tumorigenesis is due to dysregulation of radial glial cell differentiation into neurons. Our data demonstrate the potential role of FGFR3 as one of the leading drivers of tumorigenesis in CN.
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Metilação de DNA , Células Ependimogliais , Neurocitoma , Receptor Tipo 3 de Fator de Crescimento de Fibroblastos , Humanos , Receptor Tipo 3 de Fator de Crescimento de Fibroblastos/genética , Receptor Tipo 3 de Fator de Crescimento de Fibroblastos/metabolismo , Neurocitoma/genética , Neurocitoma/patologia , Neurocitoma/metabolismo , Células Ependimogliais/metabolismo , Células Ependimogliais/patologia , Regulação Neoplásica da Expressão GênicaRESUMO
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.
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Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Encefálicas/patologia , Aqueduto do Mesencéfalo/patologia , Neurocitoma/patologia , Glândula Pineal/patologia , Neoplasias Encefálicas/cirurgia , Neurocitoma/cirurgia , VentriculostomiaRESUMO
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
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Humanos , Masculino , Criança , Neoplasias do Ventrículo Cerebral/patologia , Neurocitoma/patologia , Neoplasias Peritoneais/secundário , Antineoplásicos/uso terapêutico , Evolução Fatal , Imageamento por Ressonância Magnética , Invasividade Neoplásica , Peritônio , Neoplasias Peritoneais/tratamento farmacológico , Neoplasias Peritoneais/cirurgia , Tomografia Computadorizada por Raios XRESUMO
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
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Humanos , Masculino , Feminino , Adolescente , Adulto , Neoplasias do Ventrículo Cerebral/diagnóstico , Neurocitoma/diagnóstico , Neoplasias do Ventrículo Cerebral/patologia , Neoplasias do Ventrículo Cerebral/cirurgia , Imuno-Histoquímica , Espectroscopia de Ressonância Magnética , Neurocitoma/patologia , Neurocitoma/cirurgia , Tomografia Computadorizada por Raios XRESUMO
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