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).

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.

Posterior fossa tumor in children

Primary brain tumors are the most common solid neoplasms of childhood, representing 20% of all pediatric tumors. The best current estimates place the incidence between 2.76 and 4.28/100,000 children per year. Compared with brain tumors in adults, a much higher percentage of pediatric brain tumors arise in the posterior fossa. Infratentorial tumors comprise as many as two thirds of all pediatric brain tumors in some large series. Tumor types that most often occur in the posterior fossa include medulloblastoma, ependymoma, cerebellar astrocytoma and brainstem glioma. All pediatric cases of posterior fossa tumor that were considered for surgery from 1981 to 2011 were selected and the demographic data including age, gender and tumor characteristics along with the location and pathological diagnosis were recorded. The surgical outcomes were assessed according to pathological diagnosis. Our series consisted of 84 patients [52 males, 32 females]. Cerebellar symptoms were the most common cause of presentation [80.9%] followed by headache [73.8%] and vomiting [38.1%]. The most common histology was medulloblastoma [42.8%] followed by cerebellar astrocytoma [28.6%], ependymoma [14.3%], brainstem glioma [7.2%] and miscellaneous pathologies [e.g., dermoid, and tuberculoma] [7.2%]. The diagnosis of brain tumors in the general pediatric population remains challenging. Most symptomatic children require several visits to a physician before the correct diagnosis is made. These patients are often misdiagnosed for gastrointestinal disorders. Greater understanding of the clinical presentation of these tumors and judicious use of modern neuroimaging techniques should lead to more efficacious therapies

spectrum of intradural spinal tumors

To assess the spectrum of clinical, radiological and histological features of patients with intradural spinal tumors. This descriptive study was carried out in Department of Neurosurgery Lady Reading Hospital Peshawar, from April 2003 to March 2009. Medical records of patients with spinal tumors were reviewed and patients operated for intradural spinal tumors were identified. A total of 312 patients, out of 525 cases of spinal tumors, with different intradural spinal tumors were considered in this study. Their clinical features, radiological reports, peroperative findings and histological reports were analyzed in different aspects. There were total of 312 patients with age range from 2 years to 74 years, with median age of 38 years. Out of these 187 were males and 125 were female, overall male to female ratio of 1.5: 1. Backache, leg weakness, parasthesia and poor sphincters were the main clinical features. MRI spine [274 cases] was the main diagnostic tool along with plain X-rays and X-ray myelography in limited cases [35 cases]. CT myelogram was done only in 3 cases. The common site of involvement was dorsal spine followed by lumber and cervical spines respectively in 185, 80 and 47 cases. Histological report was suggestive of Neurofibroma in 166 Meningioma in 96, Ependymoma in 20, Dermoid in 12, Astrocytoma in 7, Hemangioblastom and Tuberculoma in 3 cases each and Hydatid cyst in one case. Neurofibroma and meningioma constituted majority of cases belonging to intradural extramedulary group, while ependymoma and astrocytoma were common intramedullary tumors. Third and 5[th] decade of life was the common age group for both Intramedulary and extramedulary tumors. Intramedulary lesions were common in 3[rd] decade of life

Histological findings of posterior fossa tumors in children

To find out different histopathological types of posterior fossa tumors in children. This Descriptive study was carried out in Department of Neuro surgery Govt. Lady Reading Hospital Peshawar from June 2003 to May 2008, All patients with posterior fossa tumors under 14 years were included in the study. Patient with brainstem glioma, posterior fossa abscesses and pineal tumors were excluded. A total of 117 patients were included. There were 80 male and 37 female with sex ratio of 2.1:1 with age range from 6 months to 14 years with mean age of 8.9 years and SD + 5.4. Computerized Axial Tomography / Magnetic Resonance Imaging of the brain were done for establishing diagnosis. Different surgical procedures for tumor removal were performed and specimen was sent for histopathological study in all operative cases. Out of 117 patients Medulloblastoma was seen in 38 [32.7%], Ependymoma in 23 [19.65%] Astrocystoma in 25 [21.36%], Hemangioblastoma in 19 [16.23%] Tuberculoma in 4 [3.41%] Meningioma in 2 [1.7%] and dermoid and epidermoid cyst in 3 cases each [2.56%]. Medulloblastoma was seen commonly between 6-10 years in 22 [18.8%] patients. Astrocystoma between 6 -10 years in 12[10.25%] patients. Ependymoma is common below 5 years in 17[14.5%] patients. Hemangioblastoma between 6-10 years in 15 [12.82%] patients, tuberculoma in 3[2.56%] patients in 6 -10 years of age. Medulloblastoma and Pilocytic Astrocystoma and Hemangioblastoma are common tumors between 6-10 years of age and Ependymoma is common below 5 years. Tuberculoma is also tumor mimicking condition occurring in posterior fossa

Clinico-pathological study in large posterior fossa midline tumors.

This cross sectional analytic study was carried out among the admitted patients of the department of Neurosurgery, Bangabandhu Sheikh Mujib Medical University, Dhaka during the period of 1st July 2002 to 31st December 2004. The age ranged from 2.5 years to 70 years. The size of all posterior fossa tumors at presentation were more than 3 cm and the mean size of posterior fossa tumor was 4.38 cm. 62% of posterior fossa mid-line tumors were mixed density in NECT (non-contrast CT).Regarding enhancement characteristics, mild-moderate enhancement and marked heterogeneous enhancement was equally distributed 46% followed by marked homogeneous enhancement only 08%.. The calcification was present only in 07 (14%) patients and most of them were ependymoma. Histopathologically, medulloblastoma was the common variety (32%). The CT scan diagnostic modality sensitivity, accuracy and positive predictive value were 100%, 84.78% and 84.78% respectively but in MRI diagnostic modality 100%, 91.30% and 91.30% respectively.

Histological spectrum of ependymomas and correlation of p53 and Ki-67 expression with ependymoma grade and subtype.

BACKGROUND: Clinical and histological criteria for ependymoma prognosis are well recognized. Recently few studies have been done based on Immunohistochemistry for prognostication of these tumours. In this study we have correlated the histological spectrum with immmunoexpression of p53 and Ki67 in these tumors. AIMS: To know the incidence of ependymomas; study their morphological spectrum and to evaluate expression of P53 and Ki 67 in different morphological subtypes. MATERIAL AND METHOD: A retrospective study was preformed on 70 ependymomas received in a period between 1994 and 2001. Entire tissue received was processed for routine paraffin embedded H&E stained sections. Immunocytochemistry was performed using antibodies to GFAP, EMA, Pancytokeratin and synaptophysin, to differentiate papillary ependymoma from choroid plexus papilloma; clear cell ependymoma from oligodendroglioma and central neurocytoma; ependymoblastoma from other embryonal tumours. p53 and Ki-67 immunohistochemistry was performed to correlate their expression with various tumour grades and subtypes. RESULTS: There were 3 cases (4.2%) of Grade I ependymoma (2 cases of myxopapillary ependymoma and 1 case of subependymoma); 57 cases (81.5%) of ependymoma grade II (43 of these were of classical variety, 11 of clear cell ependymoma, 2 of papillary and 1 case of cellular ependymoma). There were 9 cases (12.8%) of anaplastic ependymoma (one of these was a clear cell ependymoma and 1 case (1.5%) of ependymoblastoma CONCLUSION: p53 and Ki67 indices can be used in routine diagnostic laboratories to supplement the tumor grade on histology and more studies with follow up should be performed to analyse the prognosis of different subtypes. The expression of Ki 67 and p53 was significantly higher in anaplastic ependymomas. 4 out of 11 cases of clear cell ependymomas showed higher Ki 67 indices as compared to classical grade II ependymomas, thus further highlighting the importance of differentiating the various subtypes.

Ependinomas: achados clínicos, epidemiologicos e anatomapatológicos de 22 casos / Ependymonas: Clinical, epidemiological and findings of 22 cases

Ependimomas são neoplasias compostas por células ependimárias neoplásicas, manifestando-se predominantemente em crianças e adultos jovens. Relatamos os achados clínicos, epidemiológicos e anatomopatológicos de 22 casos de ependimomas. Dos 22 casos analisados, 14 ocorreram em pacientes do sexo masculino e 8 em pacientes femininos. As idades variaram de 1 a 58 anos, com média de 24,63 anos. Quanto à localização, 10 casos acometeram medula, 7 ocorreram em hemisférios cerebrais, 2 intraventriculares e 1 no tronco cerebral. Os sinais e sintomas observados incluiram redução da força muscular (59,1 por cento dos pacientes), alterações da marcha (36,3 por cento), alterações da sensibilidade (36,3 por cento), hiperreflexia e sindrome de hipertensão endocraniana, com período de evolução variando de 15 dias a 8 anos, com média de 17,58 meses. Sete pacientes foram submetidos a ressecção cirúrgica total, 7 a exérese parcial, 4 a exérese parcial associada a radioterapia adjuvante, 3 a ressecção parcial seguida de quimioterapia e 1 a tumorectomia total associada a radioterapia. Evidenciou-se taxa de recidiva tumoral total de 18,2 por cento. Estes achados aproximam-se dos encontrados na literatura internacional, corroborando para a compreensão do comportamento biológico deste tumor.