Tumor neuroepitelial disembrioplásico durante el embarazo. Reporte de un caso / Dysembryoplastic neuroepithelial tumor during pregnancy. A case report

Resumen ANTECEDENTES: el tumor neuroepitelial disembrioplásico es una neoplasia poco frecuente del sistema nervioso central que causa crisis convulsivas focales resistentes al tratamiento farmacológico en pacientes jóvenes; su manifestación durante el embarazo es excepcional. CASO CLÍNICO: paciente femenina de 33 años de edad, con antecedente de tres embarazos y que, durante el cuarto, en la semana 12, inició con crisis convulsivas. El diagnóstico, por resonancia magnética nuclear, fue de tumor en el lóbulo frontal izquierdo, razón por la que se vigiló estrechamente hasta el final del embarazo, que concluyó por parto, sin ninguna complicación. Debido a que en la segunda resonancia magnética se evidenció el crecimiento de la lesión, se decidió la resección quirúrgica. El diagnóstico histopatológico informó que se trató de un tumor neuroepitelial disembrioplásico. Aunque hubo una influencia del embarazo en el comportamiento y crecimiento de este tipo de tumor, no fue posible observar la expresión de receptores hormonales en las células del tumor y el tratamiento no se modificó por la gestación. CONCLUSIONES: la repercusión del embarazo en el tumor neuroepitelial disembrioplásico no se explica por la influencia de las hormonas; este tipo de tumor sigue siendo una neoplasia benigna en el contexto de una gestación, porque no ha mostrado complicaciones que pongan en riesgo la vida de la madre y su feto.

Abstract BACKGROUND: Dysembryoplastic neuroepithelial tumor is a less frequently primary central nervous system neoplasm that causes focal seizures resistant to pharmacological treatment in young patients and its presentation during pregnancy is very rare. CASE REPORT: We report here the case of a 33 years old woman who started with seizures at 12th week of her fourth gestation. She was diagnosed by cerebral magnetic resonance imaging with a left frontal lobe tumor and was observed carefully until the end of pregnancy solved by delivery without any complication. Second magnetic resonance was performed that evidenced enlargement of the injury, therefore resection was carried out and histopathological diagnosis was for dysembryoplastic neuroepithelial tumor. Although it was observed an influence of pregnancy on clinical behavior and growth of this kind of tumor we did not find expression of hormonal receptors in the cells of the lesion and treatment was not modified by gestation. CONCLUSIONS: So impact of pregnancy on dysembryoplastic neuroepithelial tumor is not explained by a hormonal influence and this kind of tumor stills being a benign neoplasm in the context of gestation, since it has shown no risk of maternal and fetal life threatening complications.

Tumor neuroepitelial disembrioplásico: presentación de caso y revisión de la literatura / Dysembryoplastic Neuroepithelial Tumor (DNT): Case Presentation and Literature Review

El tumor neuroepitelial disembrioplásico (DNT) fue descrito por primera vezpor Daumas-Duport, en 1988, e incorporado a la clasificación de tumores de laOrganización Mundial de la Salud, desde 1993, como parte del grupo de tumoresneurogliales. El artículo describe un caso típico, dada su presentación clínica, hallazgosimaginólógicos e histológicos, con adecuada evolución postoperatoria. Se haceuna revisión de la literatura del DNT, describiendo su epidemiología, presentaciónclínica, hallazgos imaginólógicos e histológicos, posibilidades de tratamiento actualy pronóstico...

Dysembryoplastic neuroepithelial tumors (DNT)were first described by Daumas-Duport in 1988and incorporated into the new World Health Organizationclassification of brain tumours as partof the group of glioneuronal tumours in 1993. Wedescribe a typical case due to its clinical presentation,image and hystologic findings, with goodpostoperative course. A review of the literatureof DNT is made, describing its epidemiology,clinical presentation, image and histological findings,current treatment options and prognosis...

Dysembryoplastic neuroepithelial tumor originally diagnosed as astrocytoma and oligodendroglioma / Tumor neuroepitelial disembrioplásico diagnosticado originalmente como astrocitoma ou oligodendroglioma

Dysembryoplastic neuroepithelial tumor (DNT), described in 1988 and introduced in the WHO classification in 1993, affects predominantly children or young adults causing intractable complex partial seizures. Since it is benign and treated with surgical resection, its recognition is important. It has similarities with low-grade gliomas and gangliogliomas, which may recur and become malignant. OBJECTIVES: To investigate whether DNT was previously diagnosed as astrocytoma, oligodendroglioma, or ganglioglioma and to determine its frequency in a series of low-grade glial/glio-neuronal tumors. METHODS: Clinical, radiological, and histological aspects of 58 tumors operated from 1978 to 2008, classified as astrocytomas (32, including 8 pilocytic), oligodendrogliomas (12), gangliogliomas (7), and DNT (7), were reviewed. RESULTS: Four new DNT, one operated before 1993, previously classified as astrocytoma (3) and oligodendroglioma (1), were identified. One DNT diagnosed in 2002 was classified once more as angiocentric glioma. Therefore, 10 DNT (17.2%) were identified. CONCLUSIONS: Clinical-radiological and histopathological correlations have contributed to diagnose the DNT.

O tumor neuroepitelial disembrioplásico (DNT), descrito em 1988 e incorporado na classificação da OMS em 1993, acomete predominantemente crianças ou adultos jovens, causando crises convulsivas parciais complexas farmacorresistentes. Como é benigno e tratável com ressecção cirúrgica, seu reconhecimento é importante. Tem semelhanças com gliomas de baixo grau e gangliogliomas, que podem recidivar e malignizar. OBJETIVOS: Investigar se o DNT foi originalmente diagnosticado como astrocitoma, oligodendroglioma ou ganglioglioma e determinar sua frequência numa série de neoplasias gliais/glioneuronais de baixo grau. MÉTODOS: Foram revistos aspectos clínicos, radiológicos e histológicos de 58 neoplasias operadas entre 1978 e 2008, classificadas como astrocitomas (32, sendo 8 pilocíticas), oligodendrogliomas (12), gangliogliomas (7) e DNT (7). RESULTADOS: Foram identificados quatro novos DNT, um operado antes de 1993, originalmente diagnosticado como astrocitoma (3) e oligodendroglioma (1). Um DNT diagnosticado em 2002 foi reclassificado como glioma angiocêntrico. Portanto, 10 DNT (17,2%) foram identificados. CONCLUSÕES: Correlações clínico-radiológicas e histopatológicas contribuíram para o diagnóstico do DNT.