Neurocitoma Central Atípico: reporte de un caso en Ecuador / Atypical Central Neurocytoma: report of a case in Ecuador

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.

Nuevos paradigmas en el diagnóstico y tratamiento de los tumores neuroepiteliales disembrioplásticos (DNT) / New paradigms in the diagnosis and treatment of dysembryoplastic neuroepithelial tumors (DNT)

Introducción: Los tumores neuroepiteliales disembrioplásticos (DNT) han sido considerados lesiones benignas y estables, sin embargo existen casos que han presentado evoluciones inusuales a lo largo de su seguimiento. Objetivo: Analizar las características clínicas, imagenológicas, quirúrgicas y hallazgos anatomopatológicos de un grupo de pacientes con diagnóstico original de DNT e identificar características que puedan prever una evolución atípica de los mismos. Métodos: Se analizaron retrospectivamente historias clínicas de 65 pacientes con diagnóstico confirmado de DNT desde 1985 a 2016. Se evaluó clínica, imágenes, cirugía, anatomía patológica y evolución a largo plazo haciendo hincapié en la recidiva tumoral y sus relaciones con los hallazgos de imágenes y clínica. Para el análisis estadístico se utilizó el Test X2 y en las muestras donde el valor esperado era menor a 5 se usó el Test de Fisher. Para comparar medias se utilizó el t-test. Resultados: La distribución etaria mostró un leve predominio masculino 1; 1,7, con una edad promedio de presentación de síntomas de 6,6 años, con una edad promedio a la cirugía de 9,7 años. La localización más frecuente fue el lóbulo frontal con el 45%, seguido por el lóbulo temporal con el 38% de los casos. Se realizó exéresis total solo en el 42% (27/65) de los pacientes. La patología mostró patrones típicos de DNT en el 90% de los casos. Las imágenes prequirúrgicas mostraron patrones típicos en un 65% (42/65) de los pacientes y atípicos en un 35% (23/65). El 46% (30/65) de los pacientes presentaron recidiva tumoral a lo largo del seguimiento. El tiempo promedio de la aparición de la recidiva fue de 4.5 años (6 meses a 13 años ). Los pacientes que recidivaron todos habían tenido exéresis parciales (30/30), entre los que no recidivaron el 77% (27/35) tenía exéresis completa y 23% (8/35) exéresis parcial. El síntoma más frecuente en las recidivas fue la aparición de crisis convulsivas en un 77%. El tiempo de seguimiento promedio fue de 9,2 años (±6,9 DS), el 85% de los pacientes están sin crisis convulsivas y el 55% (36/65) de estos están sin medicación. Conclusión: A pesar del comportamiento benigno en la mayoría de los DNT existen pacientes que pueden presentar recidiva. Estas recidivas no necesariamente implican transformación maligna a pesar de los cambios en las imágenes y la patología que pueden encontrarse. La exéresis completa de la lesión en la primera cirugía ha demostrado ser el factor pronóstico más importante. El seguimiento debe ser estricto, ya que se han visto recidivas posteriores a periodos libres de enfermedad prolongados. Es muy importante realizar un exhaustivo análisis de las imágenes pre y post quirúrgica para poder identificar características que nos permitan predecir comportamientos inusuales.

Introduction: Neuroepithelial dysembrioplastic tumors (DNT) have long been considered benign, stable lesions. However, some patients have unusual presentations and disease course. Objectives: To analyze clinical, imaging, surgical and anatomopathological findings in a cohort of patients with an original diagnosis of DNT, and identify characteristics that may predict an atypical disease course. Methods: We retrospectively analyzed the clinical records of 65 patients with a diagnosis of DNT confirmed from 1985 to 2017. Relationships between long-term disease evolution, emphasizing tumor recurrence, and baseline clinical, imaging, surgical and histopathological findings were assessed. For inter-group comparisons between patients with versus without tumor recurrence, categorical variables were analyzed using Pearson χ2 analysis or Fisher's Exact Test, as appropriate, while continuous variable means were compared via unpaired Student's t-tests. Results: Males out-numbered females by a ratio of 1.7 to 1. The average age at presentation with symptoms was 6.6 years, with an average age at surgery of 9.7 years. The frontal and temporal lobes were the most frequent tumor locations, accounting for 45 and 38% of cases, respectively. Gross total resection was achieved in 42% (27/65) of patients. Pathology was typical of DNT in 90% of cases, while pre-surgical images exhibited typical findings in 65%, atypical in 35%. Thirty patients (46%) experienced tumor recurrence over the course of follow-up. The average time to recurrence was 4.5 years (6 months to 13 years). All patients who relapsed had initially undergone partial excision (30/30). Among those whose tumor did not recur, 23% (8/35) had undergone partial excision. The most frequent presenting symptom with recurrences was seizures, which occurred in 77%. Overall follow-up of patients has been 9.2 years (±6.9 SD), amongst whom 85% are currently seizure-free, and 55% require no seizure medication Conclusions: Despite the benign behavior of most DNT, almost half of patients experience tumor recurrence. These recurrences do not necessarily involve malignant transformation, despite imaging and pathology changes that may be encountered. Extent of tumor resection (gross vs. partial) is the strongest predictor of tumor recurrence. Vigilant long-term monitoring is indicated, because recurrences may occur after prolonged disease-free periods.

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.

Dysembryoplastic Neuroepithelial Tumor: A case report

@#This reports a case of dysembryoplastic neuroepithelial tumor (DNET) in a 5-year old male presenting with visual hallucination and seizures. Diagnostic workup revealed a homogenous cystic tumor located in the right temporo-parietal area which was considered as a low grade glioma. The patient underwent endoscopic third ventriculostomy with complete tumor excision and biopsy, revealing NET. Findings were confirmed by immunohistochemical staining with glial fibrillary action protein (GFAP), Alcian blue and synaptophysin. DNET is a recently described intracranial tumor under the World Health Organization classification of central nervous system (CNS) tumors. It is a unique entity of neuroglial tumors with excellent prognosis. Its worldwide incidence among all neuroepithelial tumors is 1.2% in patients under 20 years and 0.2% among patients over 20 years. This is the first case of DNET in the country as well as in our institution. Key points on the clinical manifestation, approach to diagnosis, distinctive radiologic and histopathologic characteristics, and management are discussed.

Dysembryoplastic neuroepithelial tumor: A rare case report.

Dysembryoplastic neuroepithelial tumor (DNET) is a rare benign tumor generally seen in children and adolescents with intractable epilepsy. This tumor demonstrates typical histological features such as glial nodules and the so called glioneuronal element. The diagnosis of DNET cannot rely just upon imaging features but needs a multidisciplinary contribution from the clinical and diagnostic department involving the clinician, radiologist as well as the pathologist to reach a definite and conclusive diagnosis. However histopathology is the gold standard for the final diagnosis.

Dysembryoplastic Neuroepithelial Tumors

Dysembryoplastic neuroepithelial tumor (DNT) is a benign glioneuronal neoplasm that most commonly occurs in children and young adults and may present with medically intractable, chronic seizures. Radiologically, this tumor is characterized by a cortical topography and lack of mass effect or perilesional edema. Partial complex seizures are the most common presentation. Three histologic subtypes of DNTs have been described. Histologically, the recognition of a unique, specific glioneuronal element in brain tumor samples from patients with medically intractable, chronic epilepsy serves as a diagnostic feature for complex or simple DNT types. However, nonspecific DNT has diagnostic difficulty because its histology is indistinguishable from conventional gliomas and because a specific glioneuronal element and/or multinodularity are absent. This review will focus on the clinical, radiographic, histopathological, and immunohistochemical features as well as the molecular genetics of all three variants of DNTs. The histological and cytological differential diagnoses for this lesion, especially the nonspecific variant, will be discussed.

Magnetic resonance imaging diagnosis of dysembryoplastic neuroepithelial tumor in children / 中华实用儿科临床杂志

Objective To study magnetic resonance imaging(MRI) features of dysembryoplastic neuroepithelial tumor(DNT) and to improve accurate diagnosis of DNT.Methods The MRI appearance and clinical features of 10 patients with DNT confirmed by surgery and pathology were analyzed retrospectively.Results In 10 cases,9 tumors located in supratentorial hemisphere cortex,3 tumors located in the temporal lobe,5 in the frontal lobe,1 in the parietal lobe,and 2 of them encroached the adjacent white matter.In 9 tumors located in supratentorial hemisphere cortex,8 cases had decreased signal intensity on T1-weighted MR images,1 case iso-decreased mixed signal intensity on T1-weighted MR images,and 9 cases increased signal intensity on T2-weighted images,9 cases slightly increased signal intensity on fluid attenuated inversion recovery weighted images.The manifestation of tumors was cystic or cystic partially oriented and was seen separate section intratumoral in some cases.Three cases appeared as hyperintense ring sign and internal septation,2 cases appeared as a triangle in shape,3 cases appeared as gyms-like shape,and 1 case as round shape,similar to cyst.Nine tumors had no significant mass effect and peritumoral edema.Enhanced MR imaging showed only 1 case with slight and heterogeneous enhancement,the rest 6 cases showed non enhancement.One case located in cerebellar hemisphere,and appeared cystic-solid mass,the solid part had decreased signal intensity on T1-weighted MR images,and increased signal intensity on T2-weighted images,the cystic part had decreased signal intensity on T1-weighted MR images,and increased signal intensity on T2-weighted images.On enhanced MR imaging,the wall-node obviously contrast enhancement,cyst wall slightly contrast enhancement,cystic part non enhancement.The tumor had peritumoral edema and mass effect.Ten cases had no hemorrhage and calcification.Conclusion The MRI appearance of DNT is characteristic and is helpful for the preoperative diagnosis of DNT.

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.

Diffuse Ependymal Dysembryoplastic Neuroepithelial Tumor Causing Spinal Drop Metastases: A Case Report

Dysembryoplastic neuroepithelial tumors (DNETs) arise mostly in the supratentorial cerebral cortex. A very rare case of intraventricular DNET with diffuse ependymal involvement, which causes spinal drop metastasis, is presented.

Magnetoencephalography and awaking anesthesia in microsurgical resection of dysembryoplastic neuroepithelial tumor in function areas / 第二军医大学学报

Objective: To summarize our experience on application of magnetoencephalography (MEG) and awaking anesthesia in microsurgical treatment of dysembryoplastic neuroepithelial tumors in the functional areas. Methods: The range of the functional area was largely defined by MEG; intraoperative cortical electrical stimulation was used under awaking anesthesia to identify the border of function area and dysembryoplastic neuroepithelial tumors. Dysembryoplastic neuroepithelial tumors were resected under microscope in four patients and the outcomes of the patients were observed. Results: Satisfactory outcomes were obtained in the 4 patients and there were no injuries in the function area. No epileptic seizures or recurrence of dysembryoplastic neuroepithelial tumors were found during long term follow-up. Conclusion: Combined treatment using MEG and awaking anesthesia contributes to safe and effective surgical treatment of dysembroplastic neuroepithelial tumors in the function areas.

Sarcoma de Ewing extraesquelético que semeja abdomen agudo / Extra-skeletal Ewing's sarcoma resembling acute abdomen: case report

Introducción: El sarcoma de Ewing extraóseo es un tumor raro de origen neuroectodérmico que se ha presentado principalmente en las partes blandas de las extremidades y del tórax; histológicamente es similar al sarcoma de Ewing en el tejido óseo. Caso clínico: Paciente con abdomen agudo y leucocitosis, en quien por imagen (ultrasonido y tomografía axial computarizada) se diagnosticó enfermedad diverticular complicada del colon, por lo que fue intervenido quirúrgicamente, encontrando lesión localizada en el espacio de Retzius con extensión a la cavidad abdominal de 20 x 15 x 15 cm. El tratamiento consistió en extirpación del tumor, separándolo de las vísceras adyacentes y el paciente fue tratado con quimioterapia: epirrubicina, ciclofosfamida y vincristina por seis ciclos; la tomografía abdominal de control mostró actividad tumoral en el retroperitoneo, adyacente al colon ascendente y ciego, por lo que se requirió nuevo tratamiento resectivo. Conclusiones: En la revisión de la literatura no hay informes previos de sarcoma de Ewing extraóseo cuya presentación sea abdomen agudo. Por la rareza de esta lesión, en la literatura solo hay registro de casos o algunas series de casos, por lo que no fue posible llevar a cabo comparaciones; la extirpación de la lesión es el pilar del tratamiento. No se define el papel de la quimioterapia preoperatoria. La biopsia percutánea guiada por algún método de imagen es de utilidad para otorgar al paciente un tratamiento óptimo que le permita un mejor pronóstico.

BACKGROUND: Extraosseous Ewing's sarcoma is a rare tumor of neuroectodermal origin. It presents mainly in the soft tissue of the extremities and thorax. Histologically, it is similar to Ewing's sarcoma of the bone. CLINICAL CASE: We present the case of a male who arrived at the emergency room with acute abdomen, leucocytosis and imaging techniques (abdominal ultrasound and computed tomography) suggestive of complicated diverticular disease. He was treated with emergency surgery. Intraoperative findings were an unsuspected tumor (20 x 15 x 15 cm). Treatment consisted of extirpation of the tumor, separating it from the adjacent viscera and followed by chemotherapy based on epirubicin, cyclophosphamide and vincristine for six cycles. Because the control abdominal CT demonstrated tumor activity in the retroperitoneum adjacent to the ascending colon and cecum, further resection was decided upon. CONCLUSIONS: In a review of the literature, no previous reports of extraosseous Ewing's sarcoma were found presenting as acute abdomen. Due to the rarity of this tumor, only case reports or series have been found in the literature without randomized or comparative studies. Surgery was the cornerstone of treatment, without reports of preoperative chemotherapy. If the patient's condition permits, percutaneous needle biopsy is mandatory to obtain optimum treatment as well as to improve prognosis.

MRI Features of Dysembryoplastic Neuroepithelial Tumors / 实用放射学杂志

Objective To study MR imaging features of dysembryoplastic neuroepithelial tumors (DNET). Methods MRI ap-pearances of 10 patients with surgery and pathology proved DNET were analyzed retrospectively. All of 10 patients underwent routine and contrast-enhanced MRI. Results All lesions were located in the regions near the surface of brain,including frontal lobe (4 ca-ses) ,parietal lobe (4 cases),temporal lobe (1 case) and parietoinsular lobe (1 case). The lesions appeared as sector in 4 cases, re-versed triangle in 4 cases,irregularity in 2 cases. Homogeneous or heterogeneous long T_1 and long Tz signal intensity were seen on MR imaging. The lesion showed predominant cystic component and septa, no obvious mass effect and peritumoral edema could be found in all cases. Bone thinning of the adjacent calvaria could be seen in 6 cases. On post contrast MRI scan,the lesions had no obvi-ous enhancement in 6 cases ,while mild enhancement could be found in the intra-tumorous solid nodule or septations in 4 cases. Conclusion DNETs are of certain clinical manifestations and MRI features,it is helpful for preoperative diagnosis of DNET.

CT and MR Imaging Features of Dysembryoplastic Neuroepithelial Tumor / 实用放射学杂志

Objective To analyze CT and MR imaging features of dysembryoplastic neuroepithelial tumor(DNT),so that to improve its diagnostic accuracy.Methods CT and MRI fidnings of DNT in 10 cases proved surgically and pathologically were retrospectively reviewed.Results All lesions were located in the super-tentorial gray matter regions or involved the cererbral certex mainly.The lesions were round,lobular,triangular or irregular pseudocystic masses with definite borders mostly.The lesions were hypointense on T_1WI and hyperintense on T_2WI.On fluid attenuated inversion recovery weighted images,the hyperintense "ring sign" and septa of the lesions were seen.On diffuse-weighted images(DWI),the lesions were hypointense or slight hypointense.On MR spectroscopy,NAA of the lesions slightly decreased.There were no peripheral edema and mass effect with well-demarcated.7 lesions had no enhancement and 3 lesions had slight unhomogeneous enhancement after administration of contrast medium.The lesions in 2 cases on plain CT showed low attenuation and one had calcification inside the lesion.Conclusion DNTs are of typical neuroradiologic features,that may be helpful for the diagnosis of which preoperatively.

MRI Features of Dysembryoplastic Neuroepithelial Tumor / 实用放射学杂志

Objective To investigate MRI features of dysembryoplastic neuroepithelial tumor(DNT).Methods The MR images of ten cases with surgically and pathologically proved DNT were retrospectively reviewed.Results MR imaging showed cystic component with mural nodule in 6,mainly cystic component with septa in 3.The solid components of the tumors were long T_1 and long T_2 signal intensity,the cystic components were even longer T_1 and T_2 signal intensity which resembled the signal of cerebrospinal fluid.Mild enhancement could be found at the intra-tumoral solid nodule or septations after injection of constrast medium but the cystic component did not show any enhancement.No peri-tumoral edema and mass effect were found in most cases.Conclusion DNTs are of some special features on the MR images,the diagnosis can be exactly done before operation if MRI features in combination with clinical data of the patients.

Tumor disembrioplástico neuroepitelial y epilepsia focal de larga evolución: informe de caso y revisión en la literatura / Dysembrioplastic neuroephitelial tumor and long lasting focal epilepsy: case report an literature review

Se presenta el caso clínico de un paciente de 20 años con epilepsia focal sintomática de larga evolución con poco control de sus crisis por tratamiento médico irregular. Las imágenes de resonancia magnética evidenciaron lesión multiquística principalmente del lóbulo temporal izquierdo. Se tomó biopsia estereotáxica cerebral de la lesión; la histolopatología mostró un componente glioneural en una matriz mucinosa, y por tanto, se hizo el diagnóstico de tumor disembrioplástico neuroepitelial. El paciente logra control de las crisis con ácido valproico y carbamazepina, mejora su estado funcional, aunque persiste el déficit cognitivo previo. No se realizó resección quirúrgica por ser una lesión benigna muy extensa y por el adecuado control de las crisis con el tratamiento médico. Debe sugerirse siempre el diagnóstico de tumor disembrioplástico neuroepitelial, ante cualquier tumor glial, en un paciente con larga historia de crisis focales, que inicien antes de los 20 años, sin déficit neurológico y que la resonancia magnética cerebral muestre una lesión cortical microquística, localizada principalmente en lóbulo temporal.

The case of a 20 year old boy, with long-lasting focal symptomatic epilepsy and a poor control of his seizures, because of irregular adherence to the neurological treatment, is presented. Brain MRI showed a multi-cystic lesion, located mainly in the temporal lobe. A brain stereotaxic biopsy was performed, finding a specific neural gliosis in a mucin-like matrix. The diagnosis of dysembryoplastic neuroepithelial tumor was done. Patient had a good control of his seizures with valproic acid and carbamazepine. Patient improved his functional state; however his previous cognitive deficit remains unchanged. Surgical resection was not developed because the lesion is considered as very benign and because the control of his seizures. The diagnosis of dysembryoplastic neuroepithelial tumor should be kept in mind in a patient with a long history of focal seizures; mainly when seizures onset begin before 20 years old, without motor or sensory neurological deficit, and when the MRI shows a cortical multi-cystic lesion, without mass effect, located in the temporal lobe.

Surgical Treatment of Intractable Epilepsy with Benign Brain Lesion / 대한간질학회지

Epilepsy surgery can be a safe, effective treatment for individuals with intractable partial epilepsy. There is increasing evidence that brain abnormalities in focal epilepsy are not restricted to a single area. The longstanding debate around the relationship between structural lesions and the epileptic zone remains unresolved. Patients with DNT (dysembryoplastic neuroepithelial tumor), which is an essentially benign tumor, can be cured by epilepsy surgery-oriented approach. Cortical dysplasia is frequently associated with DNT and seems to contribute to epileptogenic activity of DNT. Surgical treatment should be aimed at removal of the associated cortical dysplasia as well as DNT itself for ideal treatment of the disease. Simple lesionectomy of cavernous angioma would relieve seizures significantly, but not always. The concept of epilepsy surgery needs to be recruited in the treatment of cavernous angioma with seizures because diffusion of hemosiderin into the surrounding brain tissue and formation of cortical scars can make epileptogenic areas. Cortical dysplasia is a highly epileptogenic lesion constituting an important cause of medically intractable epilepsy and surgery is a treatment of choice in a selected group of patients. Identification and complete resection of the lesion and ictal onset zone are necessary to achieve a good surgical results. Intractable epilepsy accompanied by benign brain lesions can be treated surgically using the entire armamentarium of presurgical investigations. Deliberate resective procedures aimed at complete removal of dysplastic tissue and epiletogenically active areas on and around the lesion ensure excellent seizure control without permanent neurologic deficit.

Dysembryoplastic neuroepithelial tumor : A case report.

We report on a classic case of dysembryoplastic neuroepithelial tumor (DNT) located in the right temporal lobe of a 12-years-old Thai boy presenting with visual, auditory and gustatory hallucinations for 4 years. DNT is an uncommon low-grade neuronal and mixed neuronal-glial tumor which has a characteristic clinical presentation, as well as characteristic radiological and histopathological finding. DNT has excellent prognosis and surgery is mainly curative treatment. Hence, it is important to recognize this entity in order to avoid unwarranted radio-or chemotherapy.

Dysembryoplastic Neuroepithelial Tumor in the Cerebellum: Case Report

Dysembryoplastic neuroepithelial tumor(DNT) is described by Daumas-Duport in 1988 and it was interpreted as low grade astrocytoma, oligodendroglioma, and mixed oligoastrocytoma previously. In recently revised World Health Organization classification of brain tumors, DNT is classified under 'neuronal and mixed neuronalglial tumor'. DNT is clinicopathologically unique tumor and commonly located in the temporal lobe associated with intractable complex partial seizure in young patients. We report a rare case of DNT located in the cerebellum.

Spinal Cord Tumors: An Analysis of 654 Cases(1973-1999)

OBJECTIVE: The spinal cord tumors(including vertebral tumors) are increasingly diagnosed and operated due to development of refined diagnostic and therapeutic tools. It is necessary to re-evaluate clinical features and surgical results of spinal cord tumors with increasing cases and developing treatment modalities. The authors reviewed the spinal cord tumor cases to evaluate their clinical characteristics. MATERIALS AND METHODS: The retrospective review of 654 cases of spinal cord tumors between 1973 and 1999 was done. The clinical features, pathological analysis and surgical results were analyzed and compared to the literature. The results of the study are analyzed with a more detailed consideration of each of major pathologies: neurogenic tumors, meningeal tumors, neuroepithelial tumors, and metastatic tumors. RESULTS AND CONCLUSION: The spinal cord tumor was most common in the 5th decade of age(145 cases, 22.1%) and 78 cases(11.9%) were found in children under 15 years of age. The ratio of male to female was 1.2:1. The pathologic diagnosis was neurogenic tumor in 266 cases(40.7%), neuroepithelial tumor in 131(20.0%), metastatic tumor in 118(18.0%), and meningeal tumor in 94(14.4%) in the order of frequency. The tumor was located most frequently in the thoracic area(36.5%) and in the intradural extramedullary space(38.1%). The most common initial presentation was pain(40.1%) and the mean duration for presentation to operation was 14.8 months. The total or gross total removal was possible in 404 cases(61.7%) and the surgical result on the postoperative one month was recovery or improvement in 424 cases(64.8%), stationary in 188(28.7%), progression in 42(6.4%). As a surgical complication, there was a spinal deformity(12 cases), wound infection(5 cases), aspiration pneumonia(5 cases) etc. Neurogenic tumors and menigiomas showed good surgical results, whereas neuroepithelial tumors(except ependymoma) and metastatic tumors showed relatively poor prognosis.