Correspondencia entre los diagnósticos clínico, imagenológico e histopatológico en pacientes con tumores y seudotumores raquídeos intramedulares / Correspondence between the clinical, imaging and histopatologic diagnosis in patients with intramedullary spinal canal tumors and seudotumors

Introducción: Los tumores intramedulares son poco usuales y se generan dentro del tejido de la médula espinal, desplazando o invadiendo la materia blanca, los tractos y los cuerpos neuronales. Objetivo: Caracterizar a pacientes con tumores y seudotumores raquídeos intradurales intramedulares según variables clínicas, imagenológicas (resonancia magnética) e histopatológicas. Métodos: Se realizó un estudio observacional, descriptivo, longitudinal y prospectivo de 11 pacientes con diagnóstico clínico provisional de tumor o seudotumor raquídeo intramedular, confirmado por medio de la resonancia magnética, quienes fueron atendidos en el Departamento de Imagenología del Hospital General Docente Dr. Juan Bruno Zayas Alfonso de Santiago de Cuba de enero de 2014 a igual mes de 2017, procedentes de los hospitales generales de esta provincia y de Guantánamo. Resultados: En la investigación primaron las féminas (63,6 %), sobre todo en las edades de 40 a 59 años (45,4 %), así como las manifestaciones clínicas de debilidad muscular (100,0 %) y dolor en la columna vertebral (81,8 %). De acuerdo a la intensidad de las señales obtenidas en la resonancia magnética, resultaron más frecuentes las imágenes heterogéneas, predominantemente en la zona dorsal. Asimismo, sobresalió la mielopatía compresiva dorsal (27,3 %) como diagnóstico clínico de sospecha, mientras que 54,5 % de los diagnósticos imagenológicos fueron confirmados histopatológicamente, con un mayor porcentaje en el caso de los astrocitomas (36,4). Conclusiones: La resonancia magnética es el método imagenológico de elección para la identificación de estos tumores, dado que ofrece una aproximación al diagnóstico definitivo, el cual es corroborado mediante el estudio anatomopatológico.

Introduction: The intramedullary tumors are not very usual and they are generated inside the tissue of the spinal cord, displacing or invading the white matter, tracts and neuronal bodies. Objective: To characterize patients with intradural intramedullary spinal canal tumors and seudotumors according to clinical, imaging (magnetic resonance) and histopatologic variables. Methods: An observational, descriptive, longitudinal and prospective study of 11 patients with clinical provisional diagnosis of intramedullary spinal canal tumor or seudotumor was carried out, confirmed by means of magnetic resonance who were assisted in the Imaging Department of Dr. Juan Bruno Zayas Alfonso Teaching General Hospital in Santiago de Cuba from January, 2014 to the same month in 2017, coming from the general hospitals of this province and Guantánamo. Results: In the investigation there was a prevalence of females (63.6 %), mainly in the ages of 40 to 59 (45.4 %), as well as the clinical signs of muscular weakness (100.0 %) and pain in the spine (81.8 %). According to the intensity of signs obtained in the magnetic resonance, the heterogeneous images were more frequent, predominantly in the dorsal area. Also, dorsal compressive myelopathy was notable (27.3 %) as clinical diagnosis of suspicion, while 54.5 % of the imaging diagnosis was histopathologically confirmed, with a higher percentage in the case of astrocytomas (36.4). Conclusions: The magnetic resonance is the radiological method of election for the identification of these tumors, since it offers an approach to the definitive diagnosis, which is corroborated by means of the pathologic study.

Retrospective Analysis of Pathological Diagnosis of Central Nervous System Diseases in Tibet / 中国医学科学院学报

Objective To analyze the disease spectrum and clinicopathological characteristics of central nervous system(CNS)diseases diagnosed based on pathological findings in Tibet. Methods We collected the data of all the cases with CNS lesions in Tibet Autonomous Region People's Hospital from January 2013 to December 2020.The clinicopathological features were analyzed via light microscopy,immunohistochemical staining,and special staining. Results A total of 383 CNS cases confirmed by pathological diagnosis were enrolled in this study,with a male-to-female ratio of 188∶195 and an average age of(40.03±17.39)years(0-74 years).Among them,127(33.2%)cases had non-neoplastic diseases,with a male-to-female ratio of 82∶45 and an average age of(31.99±19.29)years;256(66.8%)cases had neoplastic diseases,with a male-to-female ratio of 106∶150 and an average age of(44.01±14.87)years.The main non-neoplastic diseases were nervous system infectious diseases,cerebral vascular diseases,meningocele,cerebral cyst,and brain trauma.Among the infectious diseases,brain abscess,granulomatous inflammation,cysticercosis,and hydatidosis were common.The main neoplastic diseases included meningioma,pituitary adenoma,neuroepithelial tumor,schwannoma,metastatic tumor,and hemangioblastoma.The meningioma cases consisted of 95.4%(103/108)cases of grade Ⅰ,3.7%(4/108)cases of grade Ⅱ,and only 1(1/108,0.9%)case of grade Ⅲ.Among the neuroepithelial tumor cases,the top three were glioblastoma,grade Ⅲ diffuse glioma,and ependymoma. Conclusions There are diverse CNS diseases confirmed by pathological diagnosis in Tibet,among which non-neoplastic diseases account for 1/3 of all the cases.Infectious and vascular diseases are the most common non-neoplastic diseases in Tibet,and tuberculosis and parasitic infections are relatively common.The types and proportion of brain tumors in Tibet are different from those in other regions of China,and meningioma is the most common in Tibet,with higher proportion than neuroepithelial tumor.

Abordaje interhemisférico contralateral transfalcino para resección de empendimoma supratentorial extraventricular: presentación de caso y análisis de literatura / Contralateral Transfalcine Interhemispheric Approach for Resection of Extraventricular Supratentorial Empendymoma: Case Presentation and Literature Analysis

Introducción: los ependimomas supratentoriales extraventriculares son una entidad sumamente infrecuente, solo 45 casos han sido reportados en la literatura. La mayoría de los ependimomas se localizan a nivel infratentorial e intraventricular, solo en un grupo pequeño de casos no presentan continuidad con el sistema ventricular. El objetivo de este trabajo es describir y presentar el caso de un tumor inusual, cuya importancia radica en la baja prevalencia de casos reportados en la literatura y en la particularidad del abordaje quirúrgico seleccionado. Descripción del caso: paciente de sexo masculino, de 16 años de edad, con diagnóstico de lesión ocupante de espacio sólida-quística, a nivel frontal izquierdo, entre el giro frontal superior y giro del cíngulo. Se optó por realizar un abordaje interhemisférico contralateral transfalcino, logrando la resección total de la lesión tumoral. Se obtuvo el diagnóstico histopatológico de ependimoma Grado II según clasificación de la OMS. Discusión: en base a la información analizada en los diferentes artículos, los hallazgos imagenológicos y anatomopatológicos del caso presentado coinciden con lo relatado en la literatura acerca de los ependimomas supratentoriales extraventricualres. Es imprescindible la utilización de técnicas de inmunohistoquímica para la correcta tipificación del tumor ya que las características del mismo son fácilmente confundibles con otras entidades y su correcta graduación tiene implicancias pronósticas y terapéuticas. Conclusión: los ependimomas supratentoriales extraventricualares son neoplasias sumamente inusuales. La resección quirúrgica es considerada el tratamiento de primera línea para mejorar el pronóstico y la sobrevida. El abordaje interhemisférico contralateral transfalcino nos permitió lograr la exéresis total de la lesión tumoral, favoreciéndonos un adecuado ángulo de trabajo y reduciendo así la transgresión del parénquima cerebral

ntroduction: extraventricular supratentorial ependymomas are an extremely rare entity, only 45 cases have been reported in the literature. Most ependymomas are located at the infratentorial and intraventricular level, only in a small group of cases don ́t present continuity with the ventricular system. The aim of this paper is to describe and to present the case of an unusual tumor, the importance lies in the low prevalence of cases reported in the literature and in the particularity of the selected surgical approach. Case description: a 16-year-old male patient with a diagnosis of a solid-cystic space-occupying lesion, at the left frontal level, between the superior frontal gyrus and the cingulate gyrus, measuring 40mm x 50mm x 60mm. A contralateral transfalcine interhemispheric approach was chosen, achieving total resection of the lesion. The histopathological diagnosis of Grade II ependymoma was obtained according to WHO. Discussion: based on the information analyzed in the different articles, the imaging and pathological findings of the case presented coincide with what is reported in the literature about supratentorial extraventricular ependymomas. The use of immunohistochemical techniques is essential for the correct typing of the tumor since its characteristics are easily confused with other entities and its correct graduation has prognostic and therapeutic implications Conclusion: extraventricular supratentorial ependymomas are extremely rare neoplasms. Surgical resection is considered the first-line treatment to improve prognosis and survival. The contralateral transfalcine interhemispheric approach allowed us to achieve a total resection of the lesion, favoring an adequate working angle and thus reducing the transgression of the brain parenchyma

Ependimomas intracraneales pediátricos: Si el objetivo es la curación, ¿cuál es el verdadero costo? / Pediatric Intracranial Ependymomas: If the Goal Is Cure, What Is the True Cost?

Introducción: Para caracterizar mejor la morbilidad neurológica después de una cirugía de resección máxima seguida de radioterapia en niños con ependimoma infratentorial, decidimos estudiar el estado neurológico prequirúrgico y compararlo con las evaluaciones postoperatorias a corto y largo plazo. Al mismo tiempo realizamos un estudio de sobrevida libre de progresión (SLP) tumoral para conocer qué factores tienen mayor impacto en el pronóstico de este tipo de lesiones. Métodos: Se realizó un estudio de cohorte retrospectivo donde se incluyeron todos los pacientes pediátricos con diagnóstico de ependimoma infratentorial. Se identificaron los distintos factores de riesgo y se observó cómo evolucionaron en el tiempo. Los pacientes se siguieron por un mínimo de 24 meses para el análisis de supervivencia. Resultados: Se analizaron 26 pacientes pediátricos con ependimomas de fosa posterior entre 2008-2019. Encontramos una diferencia estadísticamente significativa entre el FSS (Escala funcional neurológica) prequirúrgico y el FSS postoperatorio inmediato (p=0.03), sin embargo esta diferencia se pierde cuando comparamos el prequirúrgico con el FSS posterior al año (p=0.07).La exéresis total de la lesión tiene un efecto protector en la SLP tumoral (p=0.02), mientras que haber requerido más de 3 cirugías afecta negativamente la SLP tumoral (p=0.04), al igual que la localización lateralizada del tumor (p=0.04). Conclusión: La exéresis completa de los ependimomas de fosa posterior continúa siendo el factor pronóstico más importante para la sobrevida libre de progresión tumoral. El deterioro neurológico inmediato producido a causa del procedimiento quirúrgico parecería mejorar en la evaluación a largo plazo.

Introduction: To better characterize the neurological morbidity after maximal resection surgery followed by radiotherapy in children with infratentorial ependymoma, we decided to study the preoperative neurological status and compare it with short and long-term postoperative evaluations. At the same time, we conducted a tumor progression-free survival (PFS) study to find out which factors have the greatest impact on the prognosis of this type of injury. Methods: A retrospective cohort study was carried out in which all pediatric patients with a diagnosis of infratentorial ependymoma were included. The different risk factors were identified and it was observed how they evolved over time. Patients were followed for a minimum of 24 months for survival analysis. Results: 26 pediatric patients with posterior fossa ependymomas were analyzed between 2008-2019. We found a statistically significant difference between the presurgical FSS (Functional Status Scale) and the immediate postoperative FSS (p = 0.03), however this difference is lost when we compare the presurgical with the FSS after one year (p = 0.07).Total excision of the lesion has a protective effect on tumor PFS (p = 0.02), while having required more than 3 surgeries negatively affects tumor PFS (p = 0.04), as does the lateralized location of the tumor (p = 0.04). Conclusion: Complete excision of posterior fossa ependymomas continues to be the most important prognostic factor for tumor progression-free survival. The immediate neurological deterioration produced by the surgical procedure would appear to improve on the long-term evaluation

Manejo quirúrgico de ependimomas en médula cervical: A propósito de un caso clínico / Surgical management of ependymomas in the cervical spine: Apropos of a clinical case

Introducción: los ependimomas son tumores cerebrales que surgen de células ependimarias, células de soporte en el cerebro y la médula espinal. Representan entre el 2 y el 3% de todos los tumores cerebrales primarios. Son el cuarto tumor cerebral más común en los niños, donde el 90% de los mismos se localizan en la fosa posterior. En adultos, el 60% de estos tumores se encuentran en la médula espinal pudiendo presentarse a cualquier nivel de ésta; el caso a continuación reporta un ependimoma cervical. Objetivo: reportar un caso de ependimoma medular de ubicación poco frecuente, con resección total, sin recidiva porterior a 2 años de seguimiento.Descripción del caso: se reporta el caso de un paciente adulto, 44 años, masculino, con un síndrome medular cervical completo, progresivo, provocado por un ependimoma cervical. Intervención: se realizó laminectomía de dos espacios [C7 a T1], apertura dural, mielotomía posterior logrando exéresis total de la lesión con durorrafia a sello de agua y posterior cierre por planos sin complicaciones, con seguimiento de 2 años de sobrevida. Conclusiones: la resección total macroscópica de este tipo de tumores es muy importante para poder evitar residiva. El ependimoma cervical puede recidivar, sobre todo cuando existen residuales de la lesión. El manejo oportuno depende de gran manera de que los pacientes acudan tempranamente a valoración especializada; la resección guiada con monitorización neurofisiológica transoperatoria provee mayor oportunidad a los pacientes a no presentar secuelas permanentes y permite asimismo, mejores resultados de la rehabilitación neurológica postoperatoria

Introduction: ependymomas are brain tumors that arise from ependymal cells, supporting cells in the brain and spinal cord. They represent between 2 and 3% of all primary brain tumors. They are the fourth most common brain tumor in children, where 90% of them are located in the posterior fossa. In adults, 60% of these tumors are found in the spinal cord and can occur at any level of the spinal cord; the case below reports a cervical ependymoma. Objective: to report a case of medullary ependymoma of rare location, with total resection, without recurrence after 2 years of follow-up. Case description: a 44-year-old male adult patient with a progressive, complete cervical spinal cord syndrome, caused by a cervical ependymoma, is reported.Intervention: two-space laminectomy [C7 to T1], dural opening and posterior myelotomy were performed, achieving total excision of the lesion with water-seal durorrhaphy and subsequent closure by planes without complications, with a 2-year survival follow-up. Conclusions: macroscopic total resection of this type of tumor is very important to avoid residual. Cervical ependymoma can recur, especially when there is residual lesion. Timely management depends to a great extent on the patients attending early for a specialized evaluation; guided resection with intraoperative neurophysiological monitoring provides a greater opportunity for patients to not present permanent sequelae and also allows better results of postoperative neurological rehabilitation.

Lesiones oncológicas intracraneales de apariencia quística / Oncologic Intracranial Lesions with Cystic Appearance

Resumen La presencia de lesiones quísticas de etiología oncológica en el encéfalo es cada vez más frecuente. El rol del especialista en imágenes es describir sus características, morfología y comportamiento con el contraste endovenoso, para llegar a realizar un diagnóstico presuntivo y enumerar sus diagnósticos diferenciales, y así orientar al médico tratante. Haremos una revisión de las lesiones quísticas de etiología oncológica de presentación frecuente en el encéfalo.

Abstract The presence of an oncologic cystic formation in the brain is frequent. The imaging specialist's role is to describe its features, morphology, and image enhancement characteristics with endovenous contrast, to make a presumptive diagnosis, and enumerated differential diagnoses and thus orientate the practitioner. We will analyze the most common expansive cystic formations.

Ependimoma intracraneal: apuntes clínicos, moleculares y anatomopatológicos / Intracranial ependymoma: clinical, molecular and pathological notes

RESUMEN Los ependimomas surgen de las células ependimarias que revisten los ventrículos y los pasajes en el encéfalo y el centro de la médula espinal. Las células ependimarias producen líquido cefalorraquídeo. Se decidió la realización de una revisión acerca del ependimoma intracraneal teniendo en cuenta que no existe artículo nacional que trate este tema, siendo la mayoría de los trabajos consultados referentes a la misma variante histológica pero en localización espinal, cuyo objetivo es describir la características clínicas, moleculares y anatomopatológicas del ependimoma intracraneal. Se realizó la búsqueda de artículos en revistas de las bases de datos: PubMed, Scielo y EBSCO. La búsqueda se limitó a artículos con el texto completo, publicados fundamentalmente en los últimos cinco años. El ependimoma intracraneal es un tumor frecuente en la edad pediátrica, sus manifestaciones clínicas dependen de su localización, presenta una gran diversidad molecular y anatomoptológica (AU).

SUMMARY Ependymomas arise from ependymal cells that line the ventricles and passages in the brain and center of the spinal cord. Ependymal cells produce cerebrospinal fluid. It was decided to conduct a review about intracranial ependymoma taking into account that there is no national article dealing with this issue, with most of the works consulted referring to the same histological variant but in spinal location, whose objective is to describe the clinical characteristics, Molecular and pathological pathways of intracranial ependymoma. We searched articles in journals of the databases: PubMed, Scielo and EBSCO. The search was limited to articles with the full text, published mainly in the last five years. Intracranial ependymoma is a frequent tumor in the pediatric age, its clinical manifestations depend on its location, it has a great molecular and anatomoptological diversity (AU).

Caracterización de los pacientes con ependimoma intracraneal en el hospital pediátrico Juan Manuel Márquez. 2012-2017 / Characterization of patients with intracranial ependymoma in the pediatric hospital Juan Manuel Márquez. 2012-2017

RESUMEN Introducción: los ependimomas constituyen aproximadamente del 3-5 % de los tumores intracraneales y del 5-10 % de los tumores cerebrales, en la edad pediátrica. Objetivo: caracterizar los pacientes con ependimomas intracraneales intervenidos quirúrgicamente, en el Hospital Pediátrico ¨Juan Manuel Márquez. ¨ Materiales y método: estudio descriptivo, retrospectivo, a pacientes en edad pediátrica con diagnóstico histológico de ependimoma de localización intracraneal. En el período de enero 2012 a diciembre 2017. El universo quedó conformado por todos los pacientes en edad pediátrica operados con diagnóstico histológico de ependimoma intracraneal en el lugar y período antes mencionado (N=22). Resultados: la edad media fue 2,75 años con límites entre 1 y 17 y una desviación estándar de 3,65. Los pacientes del sexo masculino representaron el 63,64 %, la relación con el sexo femenino en los primeros 4 años fue de 1:1. En cuanto al cuadro clínico, se observó predominio de la hidrocefalia en el 72,73 % de los pacientes. Los ependimomas intracraneales de localización infratentorial, (63,64 %) predominaron. El 45,45 % de las lesiones estudiadas se correspondían con el subtipo histológico de ependimoma anaplásico. Conclusiones: la combinación de cirugía, radioterapia y quimioterapia se empleó en la mayoría de los casos. Predominó el abordaje directo de la lesión a través de craneotomía y exéresis adecuada a la localización del ependimoma, sin embargo, en la mayoría solo se logró resección entre el 50 y 90 %. En la mayoría de los pacientes la evolución luego del diagnóstico, evidenció una tendencia hacia la estabilidad (AU).

ABSTRACT Introduction: ependymoma are almost 3-5 % of the intracranial tumors and 5-10 % of the brain tumors in pediatric age. Objective: to characterize the patients with intracranial ependymoma who underwent surgery in the Pediatric Hospital ¨Juan Manuel Márquez.¨ Materials and method: retrospective, descriptive study of patients in pediatric age with histological diagnosis of ependymoma of intracranial location in the period January 2012-December 2017. The universe was formed by all patients of pediatric age who underwent surgery with histological diagnosis of intracranial ependymoma in the before-mentioned place and period (N=22). Results: the average age was 2.75 years with limits between 1 and 17 years old. Male patients represented 63.64 %; the relation with female sex during the first 4 years was 1:1. Regarding the clinical characteristics, hydrocephaly predominated in 72.73 % of patients. Intracranial ependymoma of infratentorial location (63.64 %) predominated. 45.45 % of the studied lesions corresponded to the histological subtype of anaplastic ependymoma. Conclusions: the combination of surgery, radiotherapy and chemotherapy was used in most of the cases. The direct approach of the lesion through craniotomy and a removal adequate to ependymoma location predominated. However, in most of them just the resection of 50-90 % was achieved. The evolution of most of patients after the diagnosis evidenced a tendency to the stability (AU).

Diagnóstico diferencial de las lesiones del IV ventrículo / Differential Diagnosis of Fourth Ventricular Lesions

La población pediátrica es, de lejos, la más afectada por las lesiones del cuarto (IV) ventrículo. La gran mayoría presentan un patrón radiológico similar; actualmente, con las secuencias de difusión, espectroscopia y mapas de ADC, se puede inferir la compatibilidad de los hallazgos visualizados con alguna de las patologías que se revisarán en el presente trabajo

The pediatric population is by far the most affected by lesions of the fourth (IV) ventricle. The vast majority present a similar radiological pattern, for which today, with the diffusion sequences, spectroscopy and ADC maps, it can be inferred that the visualized findings are more likely compatible with some of the pathologies that we will review later

Neurofibromatosis tipos 1 y 2 / Neurofibromatosis type 1 and 2

La neurofibromatosis (NF) comprende un grupo de enfermedades genéticas de herencia autosómica dominante, que se clasifican de la siguiente manera: neurofibromatosis tipo 1 (NF1), neurofibromatosis tipo 2 (NF2) y schwannomatosis (también conocida como neurofibromatosis tipo 3). Esta última es una enfermedad muy infrecuente, con una prevalencia aproximada de 1/126 000 personas, por lo que solo profundizaremos las dos primeras. La NF1, también conocida como la enfermedad de Von Recklinghausen, es la más frecuente de las tres y afecta principalmente la piel y el sistema nervioso periférico. Se caracteriza por la presencia de máculas "café con leche", pecas axilares o inguinales, nódulos de Lisch (hamartomas en el iris) y neurofibromas (tumores de la vaina de nervios periféricos). Otras manifestaciones menos frecuentes, aunque de mayor gravedad, incluyen gliomas del nervio óptico, meningiomas, neurofibromas malignos, escoliosis y displasia de la tibia. Su diagnóstico se suele realizar al nacimiento o durante los primeros años de vida, y se estima que un 50% de quienes la padecen presenta dificultades cognitivas. No hay datos concluyentes sobre la mortalidad en los pacientes con NF1, aunque se sabe que la expectativa de vida es menor que en la población general. La NF2 tiene una prevalencia considerablemente menor que la NF1 y su inicio es más tardío, afectando principalmente a adultos jóvenes. La presentación clínica típica se caracteriza por acúfenos, hipoacusia y ataxia en contexto de la presencia de schwannomas vestibulares bilaterales. Otros hallazgos menos frecuentes incluyen schwannomas de nervios periféricos, meningiomas, ependimomas o astrocitomas. La esperanza de vida es de unos 36 años, con una supervivencia media desde el momento del diagnóstico de 15 años. (AU)

Neurofibromatosis (NF) includes a group of genetic diseases with an autosomal-dominant inheritance pattern, and they are classified as follows: Neurofibromatosis type 1 (NF1), neurofibromatosis type 2 (NF2) and Schwannomatosis (also known as neurofibromatosis type 3). This last one is a very rare disease, with an approximate prevalence of 1/126000, so we will only deepen in the first two. NF1, also known as von Recklinghausen disease, is the most frequent, and mainly affects the skin and peripheral nervous system. Its typical manifestations are the presence of café-au-lait macules, axillary or inguinal freckles, Lisch nodules (hamartomas in the iris) and neurofibromas (peripheral nerve sheath tumors). Less frequent manifestations, although more serious, include optic nerve gliomas, meningiomas, malignant neurofibromas, scoliosis and tibial dysplasia. The diagnosis is usually made at birth or during the first years of life, and approximately 50% of patients present cognitive difficulties. There is no conclusive data on mortality in patients with NF1, although it is known that life expectancy is lower than in general population. NF2 has a considerably lower prevalence than NF1, and its onset is later in life, mainly affecting young adults. Its typical clinical presentation is characterized by tinnitus, hearing loss and ataxia in the context in the presence of bilateral vestibular schwannomas. Less frequent findings include peripheral nerve schwannomas, meningiomas, ependymomas or astrocytomas. Life expectancy is about 36 years old, with a median survival from the moment of diagnosis of 15 years. (AU)

Ependymoma with Intraorbital Extracerebral Recurrence: Case Report

Ependymomas are rare neuroepithelial tumors that originate from a type of glial cell called ependymal cell. In general, they correspond to 1.2 to 7.8% of all intracranial neoplasms, and to2 to 6%of all gliomas. Although it corresponds only to2 to 3%of all primary brain tumors, ependymoma is the fourthmost common cerebral neoplasmin children, especially in children younger than 3 years of age.1,2 In patients younger than 20 years of age, the majority (90%) of ependymomas are infratentorial,more precisely from the IV ventricle. In spite of this, in adults, medullary ependymomas are more frequent (60%). In this context, supratentorial and extraventricular ependymomas, as in the case reported in the present article, are infrequent in both adults and children.1,2 Both sexes are equally affected.3 Recurrence of intracranial ependymomas occurs in almost 50% of the cases, and the followup outcome is not favorable.4 In another perspective, the recurrence of extracerebral ependymomas is extremely rare, and even more unusual in the intraorbital site, as it occurred in the case in question.

Semi-quantitative evaluation of brain gliomas in adults: A focus on neuropathological characteristics

Introduction: Gliomas are neoplasms with high recurrence and mortality. Due to the difficulty to apply the World Health Organization (2016) classification, developing countries continue to use histological evaluation to diagnose and classify these neoplasms. Objective: To develop a semi-quantitative scale to numerically grade gliomas by its morphological characteristics. Method: A cohort of patients with gliomas was assessed and followed for 36 months. Tumor tissue sections were analyzed and graded, including aspects such as cell line, cellularity, nuclear pleomorphism, mitosis, endothelial hyperplasia, hypoxic changes, apoptotic bodies, necrosis, hemorrhage and proliferation index. Results: 58 cases were analyzed. Low-grade gliomas median score was 12 points (9 and 13.5 for percentiles 25 and 75, respectively), whereas for high-grade gliomas it was 17 points (16 and 20.5 for percentiles 25 and 75, respectively) (p < 0.0001). Thirty-six-month survival of patients with low (13/17) and high grade gliomas (6/41) was also significantly different (p < 0.0001). Conclusions: The semi-quantitative morphological scale allows an objective evaluation of gliomas, with an adequate correlation between the score, tumor grade and survival time.

Superficial siderosis of the central nervous system caused by myxopapillary ependymoma of conus medullaris and cauda equine: a case report and literature review / 北京大学学报(医学版)

Superficial siderosis of the central nervous system (SSCNS) is a rare disorder caused by hemosiderin deposits in the subpial layers of the brain and spinal cord due to prolonged or recurrent low-grade bleeding into the cerebrospinal fluid (CSF). Central nervous system tumor could be one of the sources of bleeding. Some problems exist at present regarding the diagnosis and treatment of SSCNS in China. On account of fewer cases, the insufficient awareness of the condition, and the lack of long-term follow-up data, enough attention has not been paid to etiological diagnosis. The speculative high rate of missed diagnoses of SSCNS indicates a great disparity in the treatment from the world's advanced level. Related data of clinical and basic research need to accumulate as soon as possible to promote the clinical diagnosis and treatment of the disease. The progressive neurological deficits are involved in the typical clinical manifestations of SSCNS with a triad of bilateral symmetrical sensorineural hearing loss, cerebellar ataxia and signs of corticospinal tract dysfunction. Nevertheless, there are few patients with the triad signs at the same time, which lead to a delayed diagnosis or misdiagnosis. Detection of this disease was commonly post-mortem until the advent of MRI with signal and location characteristics, which made diagnosis easier. Siderosis appears as a hypointense rim covering the surface of the cerebellum, the brain stem, the spinal cord, similar to a black pencil line, thin on SE-T2-weighted images, thick and conspicuous on GE-T2-weighted images or on susceptibility-weighted imaging (SWI). The only effective way of treating the disorder is to identify the source of bleeding and remove it. MR examination is useful for seeking a source of bleeding too. Therefore, once superficial siderosis is considered, lesions of the central nervous system must be searched using MRI of the brain and spine. We report here a 37-year-old male diagnosed of SSCNS with the classical clinical symptoms of cerebellar ataxia, sensorineural hearing loss and myelopathy. T2-weighed MRI showed characteristic marginal hypo-intensity around the central nervous system. Etiological explorations revealed a large conus medullaris / cauda equina ependymoma filling the lumbosacral spinal canal, a myxopapillary ependymoma (MPE) confirmed by surgical resection and histopathological examination. The related literature was reviewed to ascertain the mechanism of SSCNS secondary to MPE, and to discuss the pathogenesis, clinical features, diagnosis and treatment of SSCNS. This paper aims to improve the awareness of SSCNS and diagnostic level, and to lay stress on the etiological explorations that is beneficial to the development of exact treatment plan.

Ependymoma in Sellar Region:Report of One Case / 中国医学科学院学报

Ependymoma arises from the ependymal cells of the ventricles and the ependymal cell nest in the white matter of the extra-ventricles. It may be located inside or outside ventricle and can be anatomically classified into supra- and infratentorial ependymomas. Supratentorial ependymoma can be found both inside and outside ventricle but is rarely seen in the sellar regio. This article reports a pathologically confirmed ependymoma case featured by plastic growth,nodular calcification,and flow-void signal.

Fluorescence Guided Surgery with 5-Aminolevulinic Acid for Resection of Spinal Cord Ependymomas

STUDY DESIGN: A retrospective study. PURPOSE: We report our experience with 5-aminolevulinic acid (5-ALA)–assisted resection of spinal cord ependymomas in adults. OVERVIEW OF LITERATURE: Ependymoma is the most frequent primary spinal cord tumor in adults. Surgery is the treatment of choice in most cases. However, while complete resection is achieved in approximately 80% of cases, clinical improvement is achieved in 15% only. Five-ALA fluorescence–guided surgery seems to be useful for this tumor type. METHODS: We studied 14 patients undergoing 5-ALA fluorescence-guided surgery for spinal cord ependymomas in our service. The modified McCormick classification was used to determine clinical status and the degree of resection was assessed with magnetic resonance imaging. RESULTS: Of the 14 patients, the tumor showed an intense emission of fluorescence in 12 and the fluorescence was weak and nonuniform in two. Complete resection was achieved in 11 cases. According to the McCormick classification, 10 patients improved, two remained the same, and two deteriorated. CONCLUSIONS: Our results confirm that 5-ALA fluorescence-guided resection is useful in spinal cord ependymoma resection. Although the rate of complete resections is similar to that in published series without 5-ALA, clinical results are better when using 5-ALA with a lower percentage of clinical deterioration.

A Case of Recurrent Supratentorial Extraventricular Anaplastic Ependymoma in Adult

Supratentorial extraventricular anaplastic ependymoma (SEAE) in adults is a relatively rare intracranial tumor. Because of the very low prevalence, only a few cases have been reported. According to a recent study, SEAE is associated with a poor prognosis and there is no definite consensus on optimal treatment. We report a case of an adult SEAE patient who had no recurrence until seven years after a gross total resection (GTR) followed by conventional radiotherapy. A 42-year-old male had a persistent mild headache, left facial palsy, dysarthria, and left hemiparesis. Preoperative neuroimaging revealed an anaplastic astrocytoma or supratentorial ependymoma in the right frontal lobe. A GTR was performed, followed by adjuvant radiotherapy. Histologic and immunohistochemical results revealed anaplastic ependymoma. After seven years of initial therapy, a regular follow-up MRI showed a 3-cm-sized partially cystic mass in the same area as the initial tumor. The patient underwent a craniotomy, and a GTR was performed. Histopathologic examination revealed recurrence of the SEAE. External radiotherapy was performed. The patient has been stable without any disease progression or complications for 12 months since the surgery for recurrent SEAE.

Experience Profiling of Fluorescence-Guided Surgery I: Gliomas

BACKGROUND: Numerous studies reported a usefulness of 5-aminolevulinic acid (5-ALA) fluorescence-guided surgery (FGS) in high grade gliomas. However, fluorescence patterns and intensities are variable among gliomas. In this study, we report our extensive experience with FGS in various gliomas, focusing on epidemiological data of fluorescence patterns. METHODS: A total of 827 histologically proven glioma patients out of 900 brain tumor patients who had undergone FGS using 5-ALA during the period of 8.5 years between July 2010 and January 2019 were analyzed. Indications of FGS in glioma surgery are evidence for possible high-grade foci in putative gliomas in preoperative MRI. RESULTS: Among the 827 gliomas, the number of cases corresponding to 2016 World Health Organization (WHO) grade IV, III, II, and I are 528 (58.7%), 193 (21.4%), 87 (9.7%) and 19 (2.1%), respectively. In terms of fluorescence rate, grade IV gliomas showed positive fluorescence in 95.4% of cases including strong intensity in 85.6%. Grade III gliomas showed fluorescence in about half of cases (55.0%), but 45.0% of the cases showed no fluorescence at all. Anaplastic oligodendroglioma had a higher positive rate (63.9%) than anaplastic astrocytoma (46.2%). Both grade II and I gliomas still showed positive fluorescence in about one-fourth of cases (24.1% and 26.3% respectively). Among them ependymoma and pilocytic astrocytoma were fluorescence-prone tumors. CONCLUSION: This epidemiological data of 5-ALA fluorescence in various grades of glioma provides a basic reference to the clinical application of FGS with 5-ALA in glioma surgery.

Found at Old Age and Continuously Growing WHO Grade II Fourth Ventricle Ependymoma: A Case Report

A 74-year-old woman presented with a month-long nausea and vomiting, then she could not take a meal. She had found an asymptomatic 4th ventricular mass 6 year ago as a preoperative work-up for ovarian cancer. And during the yearly follow-up, the mass had grown continuously over 6 years, and caused symptoms in the seventh year. MRI revealed a large ovoid extra-axial mass in the fourth ventricle compressing adjacent medulla and cerebellum. Surgery achieved near total resection since the tumor tightly adhered to the brain stem of 4th ventricle floor. The histological diagnosis was ependymoma (WHO grade II). She transferred rehabilitation facility for mild gait disturbance, hoarseness and swallowing difficulty. Fourth ventricle ependymoma in the elderly is extremely rare and the growth rate has not been reported. Here, we present a rare care of 4th ventricle ependymoma found asymptomatic at elderly but continuously grow to cause local pressure symptoms.

Hallazgo de quiste ependimario (ependimoma) intramedular en el estudio de la vía piramidal y síndrome siringomiélico / Finding of intramedullary ependymal cyst (Ependymoma) in the study of pyramidal tract and syringomyelic syndrome

Introducción: Una paresia tiene que ser objeto de un detallado análisis para conocer su correcta etiología. La exploración física debe incluir una minuciosa valoración neurológica que oriente la localización de la lesión. Caso Clínico: Paciente con paresia de extremidad inferior derecha, hiperreflexia, clonus y disminución de sensibilidad térmica y algésica por debajo de la apófisis xifoides. Estos hallazgos señalan a nivel vertebral dorsal, D8, posible localización de una lesión que afecta a la vía piramidal motora y la vía sensitiva. La sospecha clínica se confirma mediante RMN que mostró la presencia de masa intramedular, posible ependimoma. Conclusiones: El conocimiento de la anatomía de los haces nerviosos que discurren por la médula espinal y la información nerviosa que proporcionan, contienen signos clínicos motores y sensitivos que permiten discernir qué haces nerviosos están lesionados. Este juicio clínico puede dirigir, de manera mucho más concreta, hacia el tipo de exploraciones y pruebas complementarias que pueden ser necesarias para concretar un diagnóstico y reducir el gasto sanitario, así como el tiempo de espera para un posible tratamiento quirúrgico(AU)

Introduction: A paresis must be the object of a detailed analysis to know its correct etiology. Physical examination should include a thorough neurological assessment to guide the location of the lesion. Clinical case: Patient with paresis of the lower right extremity, hyperreflexia, clonus, and decreased thermal and algic sensitivity below the xiphoid process is preseneted. These findings point to the dorsal vertebral level, D8, possible location of a lesion that affects the pyramidal motor pathway and the sensory pathway. Clinical suspicion is confirmed by MRI that showed the presence of intramedullary mass, possible ependymoma. Conclusions: The knowledge of the anatomy of the nerve bundles that run through the spinal cord, and the nerve information they provide contain clinical and sensory clinical signs, allowing to discern which nerve bundles are injured. This clinical judgment can lead, in a much more concrete way, to the type of explorations and complementary tests that may be necessary to diagnose and reduce health costs, as well as the waiting time for a possible surgical treatment(AU)

Ependimomas intracraneanos en el Instituto Nacional de Oncología y Radiobiología / Intracranial ependymomas at the National Institute of Oncology and Radiobiology

Introducción: los ependimomas representan del 2 al 3 por ciento de todos los tumores cerebrales primarios. Aproximadamente el 30 por ciento de los ependimomas pediátricos son diagnosticados en niños menores de tres años. La ubicación de los ependimomas difiere con la edad; en los adultos, el 60 por ciento se encuentra en la médula espinal, y en los niños, el 90 por ciento se encuentra en el cerebro, en su mayoría, en la fosa posterior. Objetivo: describir las características clínicas, tratamiento y supervivencia de los pacientes con diagnóstico de ependimomas tratados en el servicio de Oncopediatría en el Instituto Nacional de Oncología y Radiobiología. Métodos: se realizó un estudio descriptivo, longitudinal y retrospectivo desde el 1� de enero de 2006 al 31 de diciembre de 2016, según variables demográficas, clínicas y terapéuticas. Se identificaron los pacientes a partir de las bases de datos del registro hospitalario del Instituto Nacional de Oncología y Radiobiología. Se seleccionaron todos los pacientes con diagnóstico histológico de esta enfermedad. Resultados: se identificaron 20 pacientes, con ligero predominio del sexo masculino (45 por ciento), con una edad media de 5,3 años (rango entre 0 y 9 años). El tratamiento más utilizado fue la combinación de la cirugía más la radioterapia y la quimioterapia (45 por ciento), seguida de la cirugía más la radioterapia (30 por ciento). En estos momentos se cuenta con 47 por ciento de supervivencia. Conclusiones: los ependimomas son tumores infrecuentes. El tratamiento de elección es la resección total, acompañado de radioterapia y quimioterapia, dependiendo del tipo histológico y grado de resección(AU)

Introduction: ependymomas account for 2 to 3 percent of all primary brain tumors. About 30 percent of the pediatric ependymomas are diagnosed in children under three years of age. The location of ependymomas varies with age. In adults, 60 percent occur in the spinal cord, whereas in children they occur in the brain, mainly in the posterior cranial fossa. Objective: describe the clinical characteristics, treatment and survival of patients diagnosed with ependymoma cared for at the Oncopediatrics service of the National Institute of Oncology and Radiobiology. Methods: a retrospective longitudinal descriptive study was conducted from 1 January 2006 to 31 December 2016 based on demographic, clinical and therapeutic variables. The patients were identified using data obtained from databases from hospital records at the National Institute of Oncology and Radiobiology. All the patients with a histological diagnosis of the disease were selected. Results: twenty patients were selected, with a slight predominance of the male sex (45 percent) and a mean age of 5.3 years (range 0-9 years). The most common treatment was the combination of surgery plus radiotherapy and chemotherapy (45 percent), followed by surgery plus radiotherapy (30 percent). Current survival is 47 percent. Conclusions: ependymomas are infrequent tumors. The treatment of choice is total resection, accompanied by radiotherapy and chemotherapy, depending on the histological type and resection grade(AU)