Clinical efficacy and prognostic factors of postoperative adjuvant radiotherapy for pediatric ependymoma / 中华放射肿瘤学杂志

Objective:To investigate the clinic opathological features, treatment and prognosis of children newly diagnosed with ependymoma.Methods:Clinical data of 127 pediatric ependymoma (EPN) patients (0-16 years old) treated with tumor resection and postoperative radiotherapy at Xinhua Hospital Affiliated to Shanghai Jiao Tong University between 2001 and 2021 were retrospectively analyzed. Among them, 53 children were female and 74 were male. Local control (LR), event-free survival (EFS) and overall survival (OS) rates were analyzed by Kaplan-Meier method. The relationship between clinic opathological factors and clinical prognosis, and the effect of treatment on clinical prognosis of patients were analyzed by Cox proportional hazards model.Results:At a median follow-up time of 29 months (3-251 months), the 3-year OS and EFS rates were 89.5% and 71.5%, respectively. For patients undergoing incomplete resection followed by postoperative adjuvant radiotherapy, the 3-year LR, OS and EFS rates were 78.3%, 65.8% and 85.7%, respectively. A total of 43 children were aged <3 years old when diagnosed and 84 aged ≥3 years old. The interval time between surgery and radiotherapy in children aged <3 years old was 91 d, and 35.5 d in those aged ≥3 years old ( P<0.001). For patients <3 years old, the median EFS was 90 months when initiating radiotherapy within ≤70 d after surgery, compared to 43 months for those who initiated radiotherapy at >70 d after surgery ( P=0.053). According to fifth edition of the WHO classification of tumors of the central nervous system (WHO CNS5), 39 children were classified as posterior fossa ependymoma group A (PFA group). The OS and EFS rates in the PFA group were significantly less than those in other groups (3-year OS rate were 69.2% vs. 94.6%, P<0.001; 3-year EFS rate were 46.9% vs. 79.1%, P<0.001). In the PFA group, 12 patients received postoperative adjuvant chemotherapy, 14 did not receive chemotherapy, and whether chemotherapy was given was unknown in 13 cases. No significant differences were observed in OS and EFS between patients treated with and without chemotherapy ( P=0.260, P=0.730). Univariate Cox analysis showed that tumor location and WHO CNS5 molecular classification were significantly associated with EFS, and WHO CNS5 molecular classification was significantly correlated with OS. Multivariate Cox analysis showed that tumor location in the posterior fossa was an independent risk factor for EFS ( HR=2.72, 95% CI=1.1~6.71, P=0.03). Conclusions:Patients newly diagnosed with pediatric ependymoma can obtain favorable survival after surgery combined with postoperative adjuvant radiotherapy. Patients with residual tumors can achieve favorable LC and survival after postoperative adjuvant radiotherapy. Delaying of radiotherapy tends to lead to poor survival for patients aged <3 years old when diagnosed. Children in the PFA group obtain worse prognosis compared to their counterparts in other groups. The tumor location in the posterior fossa is an independent risk factor for pediatric ependymoma.

Extra-Axial supratentorial anaplastic ependymoma: Unusual location of an aggressive tumor, A case report

Introduction: Ependymomas are more common in the pediatric population, in whom they are commonly infratentorial. Extra axial location of a supratentorial ependymoma is extremely rare. Diagnosis: Radiologically these tumors are often misdiagnosed as meningioma or other extra axial lesions owing to their unusual location and lack of any pathognomonic features. Hence, histopathological examination becomes imperative for proper evaluation and an adequate diagnosis. Case: Herein we report a case of a supratentorial extra axial anaplastic ependymoma misdiagnosed as a metastatic tumor on radiological examination and mimicking meningioma intra operatively, located in the frontal and temporal region in a 20 year old man.

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.

Overview of recent advances in the classification of ependymomas in WHO CNS5 classification: Simplified approach to their integrated diagnosis

Ependymomas can arise along the entire neuraxis; however, they possess site-specific unique molecular alterations and a methylome pattern which is directly related with the prognostic outcomes. Since 2016, when the updated fourth edition of World Health Organization (WHO) classification of tumors of the central nervous system was published, it has been emphasized to classify ependymomas by anatomic site and molecular signatures associated genetic alterations so that classification of the disease reflects its underlying biology. In continuation, the fifth edition of the WHO classification of CNS tumors introduces major changes, including site-specific molecular profiles as the basis of classifying ependymomas. Furthermore, an integrated tier system of reporting is recommended for better clinical correlation and predicting outcomes. WHO grading can still be included in a specific tier, along with molecular markers.

Clinical characteristics and prognostic factors of intracranial ependymoma in children / 中华实用儿科临床杂志

Objective:To investigate the clinical features, diagnosis, treatment and prognostic factors of intracranial ependymoma in children.Methods:The clinical data of 32 children with intracranial ependymoma treated in the Department of Neurosurgery, Beijing Children′s Hospital of Capital Medical University from January 2012 to December 2020 were retrospectively analyzed.There were 23 males and 9 females.The median age of diagnosis was 4.5 years old (0.7-10.0 years old). Twelve cases (37.5%) were less than 3 years old and 20 cases (62.5%) were more than 3 years old.Tumors were supratentorial in 14 cases and infratentorial in 18 cases.All patients underwent tumor resection.Eighteen cases received postoperative radiotherapy, and 20 cases received chemotherapy.Four cases took neither radiotherapy nor chemotherapy. Kaplan- Meier method was used to establish the survival curve the intracranial ependymoma patients.Whether the difference of a single variable was significant was examined by the Log- rank test. Results:Of the 32 patients, 30 cases underwent gross total resection and 2 underwent subtotal resection.The mean follow-up time was 24 months (1-62 months). By the time of the last follow-up, 26 cases survived and 6 cases died.The 1-year and 3-year survival rates were 88% and 78%, respectively.Univariate analysis showed that the tumor location ( OR=1.5, 95% CI: 1.082-2.079, P=0.040) greatly affected the overall survival time in children with intracranial ependymoma, and chemotherapy ( OR=0.5, 95% CI: 0.125-1.999, P=0.034) had significant impact on the overall survival time of patients younger than 3 years old. Conclusions:Intracranial ependymoma is more prone to occur in the infratentorial region than the supratentorial region.The tumor location is an important factor affecting the survival of children with ependymoma.For children younger than 3 years old, postoperative chemotherapy can well improve the prognosis, but cannot change the progress of the disease development.

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

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.

Factores pronósticos en ependimomas intramedulares / Prognostic factors in intramedullary ependymomas

RESUMEN Objetivo: Investigar los factores de buen pronóstico, la sobrevida global (SG) y la sobrevida libre de progresión (SLP) en un grupo de personas operadas de ependimoma intramedular. Los factores están relacionados con la persona, el tumor, el tipo de resección quirúrgica y el tratamiento complementario. Materiales y métodos: Estudio retrospectivo, analítico y descriptivo de 40 pacientes operados de ependimoma intramedular entre los años 1985 y 2017 en el Instituto Nacional de Enfermedades Neoplásicas (INEN). Se usó el SPSS Windows 2017. Para calcular la sobrevida se empleó el producto de los límites de Kaplan Meier, y el test del logaritmo del rango para la comparación de curvas de sobrevida. La probabilidad (valor de p) para ser considerada significativa fue p < 0,05. Resultados: De los 40 casos, 33 pacientes fueron adultos y 7 niños; la relación hombre: mujer fue 1,4:1. El síntoma más frecuente fue déficit motor en 97,50 % de los pacientes. La media y mediana del tiempo de enfermedad fue de 24,90 y 19 meses, con un rango de 2 a 108 meses. Las localizaciones más frecuentes fueron la región cervical (47,50 %) y lumbar (25 %). La siringomielia se presentó en el 40,00 % de casos. Según el tamaño tumoral, el 60,00 % estuvo en los niveles 3 a 5; y el 32,50 % fue mayor de 5 niveles. El tipo de resección fue total en el 30 % de las cirugías; subtotal, en el 60 %; y parcial, en el 10 %. La patología correspondió a grado II en el 77,50 % de los casos; grado I, en el 15,00 %; y grado III, en el 7,50 %. El 82,50 % recibió radioterapia (RT) y tres pacientes, quimioterapia (QT). La supervivencia general fue de 240 meses. La edad, sexo, tamaño del tumor y la patología no fueron estadísticamente significativos en la sobrevida. Con la resección total, la sobrevida a 5 años fue de 100,00 %; a 10 años, 90,00 %; y a los 15 años fue también de 90,00 %, con diferencias estadísticamente significativas en relación con resección subtotal y parcial (p < 0,001). También existió una diferencia estadísticamente significativa en el grupo de pacientes que recibieron radioterapia (p < 0,002). La sobrevida libre de progresión fue de 168 meses. Hubo diferencias estadísticamente significativas en la sobrevida libre de progresión con resección quirúrgica total y radioterapia adyuvante y no existieron tales diferencias con las otras variables examinadas. El déficit motor leve a moderado mejoró el pronóstico de vida en el postoperatorio y durante su seguimiento. Conclusiones: Varios factores influyen en la sobrevida global y en la sobrevida libre de recurrencia: los más frecuentes son la resección total y la radioterapia; y el mejor estado de la función motriz en el preoperatorio, en menor proporción.

ABSTRACT Objective: To study the good prognostic factors, overall survival (OS) and progression-free survival (PFS) in a group of patients who underwent an intramedullary ependymoma surgery. Such factors are related to the patient, tumor, type of surgical resection and adjunctive treatment. Materials and methods: A retrospective, analytical and descriptive study conducted in 40 patients who underwent an intramedullary ependymoma surgery between 1985 and 2017 at the Instituto Nacional de Enfermedades Neoplásicas (INEN). The research used IBM SPSS Statistics for Windows 2017. The survival rate was estimated using the Kaplan-Meier product-limit model. The comparison of survival curves was determined by the log-rank test. A probability (p value) less than 0.05 was considered as statistically significant. Results: Out of the 40 patients, 33 were adults and seven were children. The male to female ratio was 1.4:1. The most frequent symptom was motor deficit, which occurred in 97.50 % of the patients. The mean and median of the disease period were 24.90 and 19 months, respectively, with a range of 2 to 108 months. The condition occurred most frequently in the cervical (47.50 %) and lumbar (25 %) areas. Forty percent (40 %) of the patients developed syringomyelia. Concerning their size, 60 % of the tumors covered three to five levels, and 32.50 % were larger than five levels. Thirty percent (30 %), 60 % and 10 % of the patients underwent a total, subtotal and partial resection, respectively. Seventy- seven point five zero percent (77.50 %) of the patients had grade II ependymoma, 15 % had grade I and 7.50 % had grade III. Thirty-three (33) patients (82.50 %) received radiotherapy (RTx) and three, chemotherapy (CTx). Overall survival accounted for 240 months. Age, sex, tumor size and disease were not statistically significant predictors of survival. Total resection showed survival rates of 100 %, 90 % and 90 % at 5, 10 and 15 years, respectively, and statistically significant differences in relation to partial and subtotal resections (p < 0.011). There were statistically significant differences in the group of patients who received radiotherapy (p < 0.002). Progression-free survival accounted for 168 months. There were statistically significant differences in the progression-free survival regarding total resection and adjuvant radiotherapy, but no differences in the other variables. A mild to moderate motor deficit improved life prognosis during postoperative and follow-up periods. Conclusions: Several factors affect overall survival and progression-free survival rates, with the most frequent being total resection and radiotherapy, and the least frequent a good preoperative motor function.

Establishment and Validation of A Prognostic Nomogram for Pediatric Ependymoma Based on SEER Database / 肿瘤防治研究

Objective To establish and validate a Nomogram for predicting the survival of patients with pediatric ependymoma based on SEER database. Methods We collected the clinicopathological data from 1975 to 2016 in the SEER database. Univariate and multivariate Cox proportional hazard regression models were used to identify potential predictors. A Nomogram was constructed to predict 5- and 10-year overall survival of patients with pediatric ependymoma. The consistency index (C-index), receiver operating characteristic curve and calibration curve were used to verify the discrimination and accuracy of the Nomogram. The decision curve analysis was performed to verify the clinical applicability of the Nomogram. Results A Nomogram model was established based on multivariate Cox proportional hazards model of training set. C-index values of the Nomogram were 0.713 (95%CI: 0.680-0.747) and 0.734 (95%CI: 0.681-0.787) in the training and validation sets, respectively. ROC curves also showed good discrimination in the training set. The calibration curves showed satisfactory consistency between Nomogram and ideal models. The decision curve analysis demonstrated the considerable clinical usefulness of the Nomogram. Conclusion The Nomogram model is constructed based on age at diagnosis, gender, race, primary tumor sites, tumor grade, surgery treatment and SEER registry to predict the survival of patients with pediatric ependymoma. It has good discrimination and accuracy degree, providing useful guidance to make more accurate and personalized survival prediction for patients in clinic.

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).

The value of MRI-T2WI texture analysis in the differentiation of atypical medulloblastoma and ependymoma of the fourth ventricle / 中国医师进修杂志

Objective To evaluate the value of MRI-T2WI texture analysis in the differentiation of atypical medulloblastoma and ependymoma of the fourth ventricle.Methods Preoperative MRI data of 36 cases of fourth ventricle tumor (19 cases of medulloblastoma and 17 cases of ependymoma) confirmed by the Central Theater General Hospital of the Chinese People's Liberation Army were retrospectively analyzed.Manually sketch areas of interest (ROI) were made using texture analysis software to get histogram parameters,including mean,median,standard deviation,skewness,kurtosis,maximum,minimum,heterogeneity,entropy,the 5th percentile (T2WI5th),the 10th percentile (T2WI10th),the 25th percentile (T2WI25th),the 50th percentile (T2WI50th),the 75th percentile(T2WI75th),the 95th percentile (T2WI95th).Independent sample t test or mann-whitney U test was used for statistical analysis of each parameter value between the two groups.Logistic regression analysis was used to perform multiparameter joint analysis on meaningful parameters.The area under the curve (AUC) was obtained by using the subject operating characteristic (ROC) curve to determine the threshold and diagnostic efficacy for distinguishing the two group.Results The mean,median,T2WI5th,T2W10th,T2WI25th,T2WI50th,T2WI75th values of the medulloblastoma group were smaller than those of the ependymoma group,and difference was statistically significant (P ＜ 0.05).The T2WI5th had the best diagnostic performance.When the critical value was 1 343,the diagnostic specificity was 75.0％ and the sensitivity was 92.9％.Logistic regression was used to predict the joint analysis of probability parameters.T2WI15th+T2WI10th had the best performance,and AUC was 0.928.When the critical value was 0.75,the diagnostic specificity was 75.0％ and the sensitivity was 100.0％.Conclusions MRI-T2WI texture analysis can provide quantitative information for preoperative diagnosis of atypical medulloblastoma and ependymoma and improve the accuracy of preoperative diagnosis,which has obvious clinical value.

The value of MRI-T2WI texture analysis in the differentiation of atypical medulloblastoma and ependymoma of the fourth ventricle / 中国医师进修杂志

Objective@#To evaluate the value of MRI-T2WI texture analysis in the differentiation of atypical medulloblastoma and ependymoma of the fourth ventricle.@*Methods@#Preoperative MRI data of 36 cases of fourth ventricle tumor (19 cases of medulloblastoma and 17 cases of ependymoma) confirmed by the Central Theater General Hospital of the Chinese People′s Liberation Army were retrospectively analyzed. Manually sketch areas of interest (ROI) were made using texture analysis software to get histogram parameters, including mean, median, standard deviation, skewness, kurtosis, maximum, minimum, heterogeneity, entropy, the 5th percentile (T2WI5th), the 10th percentile (T2WI10th), the 25th percentile (T2WI25th), the 50th percentile (T2WI50th), the 75th percentile(T2WI75th), the 95th percentile(T2WI95th). Independent sample t test or mann-whitney U test was used for statistical analysis of each parameter value between the two groups. Logistic regression analysis was used to perform multiparameter joint analysis on meaningful parameters. The area under the curve (AUC) was obtained by using the subject operating characteristic (ROC) curve to determine the threshold and diagnostic efficacy for distinguishing the two group.@*Results@#The mean, median, T2WI5th, T2WI10th, T2WI25th, T2WI50th, T2WI75th values of the medulloblastoma group were smaller than those of the ependymoma group, and difference was statistically significant (P<0.05). The T2WI5th had the best diagnostic performance. When the critical value was 1 343, the diagnostic specificity was 75.0% and the sensitivity was 92.9%. Logistic regression was used to predict the joint analysis of probability parameters. T2WI5th+T2WI10th had the best performance, and AUC was 0.928. When the critical value was 0.75, the diagnostic specificity was 75.0% and the sensitivity was 100.0%.@*Conclusions@#MRI-T2WI texture analysis can provide quantitative information for preoperative diagnosis of atypical medulloblastoma and ependymoma and improve the accuracy of preoperative diagnosis, which has obvious clinical value.

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

Primary extraneural ependymoma of the broad ligament: A rare entity

Extraneural broad ligament ependymoma is a rare entity. Herein, we present a case of unusually large broad ligament ependymoma in a 32-year-old female with pain and lump in the lower abdomen. Contrast-enhanced computed tomography abdomen revealed multiple heterogeneously enhancing pelvic masses with lobulated surface in bilateral adnexa along with multiple peritoneal nodules. Her relevant serum tumor markers were unremarkable. Core biopsy revealed tumor composed of elongated cells arranged predominantly in true and pseudoperivascular rosettes. The histopathological differentials included ependymoma, primitive neuroectodermal tumor, and teratoma with neural differentiation. Results of immunohistochemistry favored the diagnosis of ependymoma. Surgical exploration and optimal cytoreduction were done, and a final diagnosis of primary broad ligament ependymoma with peritoneal metastasis was made. The patient received six cycles of adjuvant chemotherapy and is doing well after 8-month follow-up. The present case highlights the diagnostic workup and management of a rare and an unusually large broad ligament ependymoma with peritoneal metastasis

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.

Supratentorial Ependymoma:Experience Of A Neurosurgeon

Background-Ependymoma account for 5 to 10 % of all brain tumors in young population however majority occur in infratentorial location and Supratentorial Ependymoma (STE) are extremely rare . Aim of this original article is to describe demographic , clinical features and surgical outcome of these rare tumors operated by Neurosurgeon DSM .Materials and Method-10 cases of STE were operated by a single Neurosurgeon DSM from May 2017 to January 2019 at a tertiary care hospital.Results-Gross total resection was performed in 8 cases and Near total resection was performed in 2 cases . Recurrence was seen in 3 cases during study period and 2 cases required resurgery Histopathology was anaplastic Ependymoma WHO grade 3 in all the cases.Conclusion-STE have a better prognosis as compared to infratentorial Ependymoma in view of good chance of gross total excision .

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.

RELA fusion-positive ependymoma: a clincopathologic analysis of eleven cases / 临床与实验病理学杂志

Purpose To investigate the clinicopathologic features of RELA fusion-positive ependymoma. Methods The clinical manifestations, radiological appearance, pathological and immunohistochemical findings and immunophenotype in 11 cases of RELA fusion-positive ependymoma were retrospectively analyzed, and the related literature was reviewed. Results Eleven cases of RELA fusion-positive ependymomas occured in the supratentorial region. There were 6 males and 5 females ranged in age from 3-56 years (median = 27). Imaging showed the occupying lesion in the supratentorial region. Histologically, a distinctive branching network of delicate capillaries and (true) ependymal rosettes/pseudorosettes were observed. Immunohistochemically, the tumor cells were diffusely positive for GFAP, L1 CAM and Cyclin D1, and EMA staining with dot-like cytoplasmic reactivity were observed. Most of the cases showed a positive for nestin, but all of the cases showed negative for Olig-2. Conclusion RELA fusion-positive ependymomas usually affect the supratentorial region of young people, which have unique immunophenotype and gene phenotype, and are characterized by poor prognosis. This type of ependymoma should be identified in the diagnosis and differential diagnosis.

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.