Extraventricular Central Neurocytoma

Resumo

Introdução: Neurocitoma extraventricular (NE) é uma neoplasia de baixo grau que se origina do sistema nervoso central (SNC), sem envolver as estruturas ventriculares. Usualmente localizado no lobo frontal, seguido pelo temporal, parietal e occipital. Raramente pode se originar na região selar ou na medula espinhal. Nesse artigo descrevemos um caso operado no nosso serviço e realizamos uma breve revisão da literatura a respeito desta rara neoplasia. Relato de caso: Paciente feminina de 58 anos, admitida devido a quadro de cefaléia iniciado há 3 meses, com piora progressiva. Referia vertigem e quedas frequentes sobre seu lado esquerdo. Na admissão, apresentava-se em Glasgow 15, sem alterações no exame neurológico. Na ressonância magnética (RNM) de crânio, foi identificada uma lesão expansiva heterogênea frontal esquerda, sem realce ao contraste, com edema perilesional adjacente, exercendo efeito de massa e desvio de linha média. A hipótese inicial foi de glioma. Paciente foi submetida a microcirurgia para ressecção da lesão. Foi utilizada uma incisão de Soutar, seguida por uma craniotomia frontal esquerda e ressecção completa da lesão. O procedimento foi realizado sem intercorrências. Paciente recebeu alta hospitalar dois dias depois, apresentando hemiparesia direita completa e proporcionada grau IV+. O resultado da pesquisa imunohistoquímica confirmou o diagnóstico de neurocitoma extraventricular. A paciente foi submetida a radioterapia adjuvante e manteve seguimento ambulatorial sem sinais de remissão de doença. Discussão: NE é uma neoplasia rara, caracterizada como um tumor neuronal e glial. Geralmente consiste em uma lesão única, grande (>40mm), bem definida, com moderado edema perilesional e calcificações puntiformes. Se originaram na substância branca dos hemisférios cerebrais, envolvendo frequentemente o lobo frontal. Devido às suas características histológicas que se assemelham a outras lesões do SNC (especialmente oligodendrogliomas), o diagnóstico histológico pode ser desafiador. O NE é constituído por células pequenas, ovais e uniformes, com um citoplasma claro e halo perinuclear. Apresentam diferenciação neuronal sem atipias, pleomorfismos, proliferação ou necrose endotelial. Baixa taxa de mitose está presente, assim como positividade à sinaptofisina. O tratamento consiste na combinação entre ressecção cirúrgica, radioterapia e, em alguns casos, quimioterapia. Conclusão: NE deve sempre ser lembrado como um possível diagnóstico diferencial de lesões expansivas intracranianas. O objetivo deste artigo foi enfatizar o papel e importância da imunohistoquímica e dos marcadores neuronais no diagnóstico desta rara neoplasia.

Palavras-chave

Neuro-oncologia; Histopathologia; Neurocitoma

Abstract

Background: Extraventricular Neurocytoma (EVN) is a rare low grade neoplastic lesion that originates anywhere in the central nervous system, not involving the ventricular system; it usually presents itself in the frontal lobes, followed by temporal, parietal and occipital lobes, and, in rare cases, the sellar region or spinal cord. The following is a case report regarding an EVN located in the left frontal lobe, followed by a brief literature review. Case report: A 58 yo female, admitted with worsening of a chronic headache within the last 3 months, developing vertigo and frequent falls directed to her left side. During admission, she presented score 15 on the Glasgow Coma Scale (GCS), without any other change during the neurological examination. A brain Magnetic Resonance Imaging (MRI) was performed, showing a heterogeneous left frontal lesion, without contrast enhancement, with moderate to severe perilesional vasogenic edema, exerting mass effect and generating a marked midline shift. The initial diagnostic hypothesis was Glioma. A microsurgical resection of the lesion was performed, with a Soutar incision, followed by a left frontal rectangular craniotomy - the procedure was completed without any intraoperative complications. At hospital discharge, two days after surgery, the patient presented a contralateral complete and proportionate hemiparesis (grade IV+), maintaining the GCS of admission. The immunohistochemical results confirmed the diagnosis of extraventricular neurocytoma. The patient was submitted to adjuvant radiotherapy, and maintained outpatient follow-up without signs of remission. Discussion: EVN is an uncommon neoplastic lesion characterised as a neuronal and glial-mixed tumour. Because of its image and histological features, which may resemble other kinds of brain lesions (specially oligodendrogliomas), diagnostics difficulties may be present during histopathological analysis, which can include other non-related entities. It appears typically as a solitary, large (>40 mm), well-defined lesion, with moderate peritumoral edema and punctual calcifications. It usually originates in the white matter of the cerebral hemispheres, involving, most frequently, the frontal lobe. EVN is composed of small, round and uniform cells, with a clear cytoplasm and perinuclear halo and shows neuronal differentiation without atypical features, pleomorphism, proliferation or endothelial necrosis - low mitosis image counts are the norm, and a strong immune response to synaptophysin is present. Treatment for EVN is a combination of surgical resection, radiotherapy and, in some cases, chemotherapy. Conclusion: Regardless of its rarity and frequency, EVN should be remembered as a possible differential diagnosis for intracranial mass lesions. Therefore, this paper’s objective is to emphasise the role and importance of immunohistochemistry and neuronal biomarkers as means of diagnosis.

Keywords

Neuro-oncology; Histopathology; Neurocytoma

References

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Extraventricular Central Neurocytoma

Neurocitoma Central Extraventricular

Resumo

Palavras-chave

Abstract

Keywords

References

JBNC Brazilian Journal of Neurosurgery

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