ABSTRACT
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.
Subject(s)
Neurocytoma , Therapeutics , Neoplasms, Neuroepithelial , DiagnosisABSTRACT
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 Ìs prognosis directly
Subject(s)
Humans , Female , Neurocytoma , General Surgery , Brain Neoplasms , Central Nervous System , Intracranial Hypertension , HydrocephalusABSTRACT
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.
Subject(s)
Humans , Male , Adult , Neurosurgery , Central Nervous System Neoplasms/diagnosis , Neurocytoma/surgery , Neurocytoma/diagnosis , Hydrocephalus , Central Nervous System/pathologyABSTRACT
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
Subject(s)
Humans , Child , Adolescent , Central Nervous System Neoplasms/pathology , Neurocytoma/radiotherapy , Radiation, IonizingABSTRACT
El Neurocitoma Central (NC) es un tumor del SNC infrecuente, de estirpe neuronal, frecuentemente intraventricular, que generalmente afecta adultos jòvenes, tiene crecimiento lento y que al momento del diagnóstico tiene con frecuencia un volumen considerable. Su comportamiento es poco agresivo y una exéresis quirúrgica conservadora permite mejorar sustancialmente la calidad y espectativa vital. Presentamos aquí dos casos clínicos de pacientes con cuadros clínicos compatibles a los decritos en la literatura. Se realizaron estudios inmunohistoquímicos de las lesiones que confirman el diagnóstico.
Central Neurocytoma (CN) it's a rare Central Nervous System Tumor, derivated of the neuron, frequently intraventricular, it generally affects young adults, has a slow pattern of growth and at diagnosis is frequently voluminous. It's a less aggressive kind of tumor and a conservative surgery exeresis allows a better quality and expectative of life. We present two cases of patients with similar clinical presentation with the descriptions find in the literature and with histologyc and immunohistochemistry studies that confirms the diagnosis.