"Ependymoma"

Ependymoma, Childhood Skip to main contentAn official website of the United States governmentEspañolMenuSearchHome Cancer Types Brain Cancer Health Professional Childhood Ependymoma Treatment (PDQ®)–Health Professional VersionPrintEmailChildhood Ependymoma Treatment (PDQ®)–Health Professional VersionGo to Patient VersionON THIS PAGEGeneral Information About Childhood EpendymomaMolecular /05/2022)About This PDQ SummaryGeneral Information About Childhood EpendymomaIn This SectionIncidenceAnatomyClinical FeaturesDiagnostic EvaluationPrognostic FactorsFollow-up After TreatmentPrimary brain tumors, including ependymomas, are a diverse group of diseases that together constitute the most common solid tumor of childhood. Immunohistochemical analysis, cytogenetic and molecular genetic

Strong nuclear expression of HOXB13 is a reliable surrogate marker for DNA methylome profiling to distinguish myxopapillary ependymoma from spinal ependymoma. Spinal ependymoma and myxopapillary ependymoma are the two most common spinal ependymal tumor types that feature distinct histological characteristics, genetic alterations and DNA methylation profiles. Their histological distinction may be difficult in individual cases and molecular diagnostic assessment, in particular DNA methylome profiling, may then be required to assign the correct diagnosis. Expression of the homeobox gene HOXB13 at the mRNA and protein levels has been reported as a frequent finding in myxopapillary ependymoma that may serve as a diagnostic marker for these tumors. Here, we evaluated the diagnostic role of HOXB13

Brain with coexistent acoustic schwannoma and ependymoma. This particular case is a world-first with no previous literature reports on patients presenting with both benign acoustic schwannoma and malignant ependymoma. A 60-year-old woman with unexplained right-sided hearing loss that had worsened progressively over 4 years, along with intermittent dizziness that had begun 3 years prior. Our preliminary diagnosis included: (1) Right acoustic neuroma; (2) Ependymoma of the fourth ventricle; and (3) Hydrocephalus. We employed the right sigmoid sinus posterior approach combined with the posterior median approach, beginning with removal of the fourth ventricle tumor and then proceeding to acoustic schwannomas resection through rotating operation positions. The case presented significant challenges

Convolutional neural network-based magnetic resonance image differentiation of filum terminale ependymomas from schwannomas. Preoperative diagnosis of filum terminale ependymomas (FTEs) versus schwannomas is difficult but essential for surgical planning and prognostic assessment. With the advancement of deep-learning approaches based on convolutional neural networks (CNNs), the aim of this study , with an accuracy of 0.87. CNN based MRI analysis has the potential to accurately differentiate ependymomas from schwannomas in the lumbar segment.

Outcome after treatment of pediatric supratentorial ependymoma: long-term follow-up of a single consecutive institutional series of 26 patients. Long-term outcome after surgical treatment of supratentorial ependymoma (STE) in children has not been extensively reported. We identified 26 children who underwent primary tumor resection of STE between 1953 and 2011, with at least 8 years follow-up . Ten patients (38%) had anaplastic and 16 had low grade ependymoma. Four of 15 children (26%) treated in the years 1953-1976 survived more than 5 years, but the observed 10-year survival was only 7%. One patient lived for 37 years, and second surgery for a local recurrent lesion disclosed a glioblastoma, possibly secondary to radiotherapy. In contrast, the observed 5-year survival rate for 11

Single-cell RNA sequencing of anaplastic ependymoma and H3K27M-mutant diffuse midline glioma. Anaplastic ependymoma and H3K27M-mutant diffuse midline glioma are two common subtypes of brain tumors with poor long-term prognosis. The present study analyzed and compared the differences in cell types between two tumors by single-cell RNA sequencing (scRNA-seq) technology. ScRNA-seq was performed to profile cells from cancer tissue from anaplastic ependymoma patient and H3K27M-mutant diffuse midline glioma patient. Cell clustering, marker gene identification, cell type annotation, copy number variation analysis and function analysis of differentially expressed genes were then performed. A total of 11,219 cells were obtained from anaplastic ependymoma and H3K27M mutant diffuse midline glioma

Development of chromosome 1q+ specific treatment for highest risk pediatric posterior fossa ependymoma. There are no effective treatment strategies for children with highest-risk posterior fossa ependymoma (PFA). Chromosome 1q gains (1q+) are present in approximately 25% of newly diagnosed PFA tumors and this number doubles at recurrence. Seventy percent of children with chromosome 1q+ PFA

Transcriptomic and epigenetic dissection of spinal ependymoma (SP-EPN) identifies clinically relevant subtypes enriched for tumors with and without NF2 mutation. Ependymomas encompass multiple clinically relevant tumor types based on localization and molecular profiles. Tumors of the methylation class "spinal ependymoma" (SP-EPN) represent the most common intramedullary neoplasms in children . Transcriptomic (n = 72), epigenetic (n = 225), genetic (n = 134), and clinical data (n = 112) were integrated for a detailed molecular overview on SP-EPN. Additionally, we mapped SP-EPN transcriptomes to developmental atlases of the developing and adult spinal cord to uncover potential developmental origins of these tumors. The integration of transcriptomic ependymoma data with single-cell atlases

Clinically relevant molecular hallmarks of PFA ependymomas display intratumoral heterogeneity and correlate with tumor morphology. Posterior fossa type A (PF-EPN-A, PFA) ependymoma are aggressive tumors that mainly affect children and have a poor prognosis. Histopathology shows significant intratumoral heterogeneity, ranging from loose tissue to often sharply demarcated, extremely cell-dense , or IGF2. Relapsing tumors displayed a higher proportion of cell-dense areas (p = 0.036), a change in PF-EPN-A methylation subtypes (13/32 patients), and novel chromosome 1q gains and 6q losses (12/32 cases) compared to corresponding primary tumors. Our data suggest that PF-EPN-A ependymomas habor a previously unrecognized intratumoral heterogeneity with clinical implications, which has to be accounted

Molecular characteristics and improved survival prediction in a cohort of 2023 ependymomas. The diagnosis of ependymoma has moved from a purely histopathological review with limited prognostic value to an integrated diagnosis, relying heavily on molecular information. However, as the integrated approach is still novel and some molecular ependymoma subtypes are quite rare, few studies have correlated integrated pathology and clinical outcome, often focusing on small series of single molecular types. We collected data from 2023 ependymomas as classified by DNA methylation profiling, consisting of 1736 previously published and 287 unpublished methylation profiles. Methylation data and clinical information were correlated, and an integrated model was developed to predict progression-free

PRO-QOL after gross total resection of spinal ependymoma: a retrospective study based on 3-year follow-up observations in a single center. Although many studies have reported clinical outcomes of spinal ependymoma (SE) patients after gross total resection (GTR), the data about the patient reported outcomes of the quality of life (PRO-QOL) was limited. This study investigated the recovery process

Toward Improved Diagnosis Accuracy and Treatment of Children, Adolescents, and Young Adults With Ependymoma: The International SIOP Ependymoma II Protocol. The clinical management of ependymoma in childhood and adolescence is complex and the clinicobiopathological correlates of outcome remain poorly understood. This international SIOP Ependymoma II (SIOP EPII) trial aims to improve the outcome of patients with ependymoma. SIOP EPII includes any patient <22 years at diagnosis with ependymoma, stratified by age, tumor location, and outcome of the initial surgery. Centralized pathology and imaging is required for diagnosis confirmation. SIOP EPII included three randomized studies according to age, postoperative residue, and suitability to receive radiotherapy. Patients ineligible for interventional

Multi-institutional characterization of outcomes for pediatric and young adult patients with high risk myxopapillary ependymoma after radiotherapy. Myxopapillary ependymoma (MPE) is a rare, typically slow-growing subtype of spinal ependymomas. There are no standard guidelines for radiotherapy and long-term outcomes after radiation, particularly patterns of relapse, for pediatric and young adult

Respective roles of surgery, chemotherapy and radiotherapy for recurrent pediatric and adolescent ependymoma: a national multicentric study. Half of the children and adolescents treated for intracranial ependymoma experience recurrences that are not managed in a standardized manner. Our study aimed at retrospectively evaluating recurrence treatments. We assessed overall survival (OS

Characterizing the tumor immune microenvironment of ependymomas using targeted gene expression profiles and RNA sequencing. Defining the tumor immune microenvironment (TIME) of patients using transcriptome analysis is gaining more popularity. Here, we examined and discussed the pros and cons of using RNA sequencing for fresh frozen samples and targeted gene expression immune profiles (NanoString ) for formalin-fixed, paraffin-embedded (FFPE) samples to characterize the TIME of ependymoma samples. Our results showed a stable expression of the 40 housekeeping genes throughout all samples. The Pearson correlation of the endogenous genes was high. To define the TIME, we first checked the expression of the PTPRC gene, known as CD45, and found it was above the detection limit in all samples by both

Conformal Radiation Therapy for Ependymoma Age 3 Years: 25 Year Experience. Adjuvant radiation therapy (RT) affects survival after surgery for young children (age <3 years) diagnosed with intracranial ependymoma. Conformal photon RT promised to spare normal tissue and was introduced more than 25 years ago to improve outcomes for these vulnerable patients. Long-term results for those first treated with conformal methods provide valuable information and serve as a comparison against newer methods. Between 1997 and 2018, 101 patients <3.1-years-old were treated with conformal and intensity modulated photon therapy after definitive surgery for intracranial ependymoma. The median age at RT was 2.1 years and the time from diagnosis to the start of RT was 10 weeks. The extent of resection

Clinical management and prognosis of spinal myxopapillary ependymoma: a single-institution cohort of 72 patients. Myxopapillary ependymoma (MPE) was classified as grade 2 tumor in the 2021 World Health Organization central nervous system classification because of its high recurrence probability. This study aimed to investigate predictive factors and management of tumor recurrence. Seventy-two

Mobile cauda equina ependymoma, case report and literature review. The aim of this study was to describe a very rare presentation of ependymoma that can derive from intraoperative complications for surgeons and increased morbidity for the patients. Multiple cases of mobile cauda equina tumors leading to inadequate approach have been reported in the literature, however, the reports of ependymoma the diagnostic of ependymoma. The preoperative image shows the migration of the tumor avoiding the wrong approach. The patient went to surgery for laminectomy, pedicular screw arthrodesis L1-S1, and tumor removal, obtaining a complete resection and confirmed histology diagnostic of ependymoma, unfortunately, the patient died five days after by a cardiac arrest. The recent literature reports only four cases

Challenges in treating childhood infratentorial ependymoma: a LMIC experience. Patients and physicians in low and middle-income countries (LMICs) face challenges due to limited expertise and suboptimal access to appropriate diagnostic and treatment modalities. We report our experience in treating posterior fossa ependymoma (PFE) at MAHAK, a charity organization in Iran. The radiation oncology department is the only one exclusively dedicated to childhood cancer in the whole country. Pediatric patients with PFE and referred to MAHAK between November 2008 and January 2016 were identified. Details on investigations and management done before referral were collected. Management at MAHAK and patients outcome were analyzed. Out of 80 patients diagnosed as having ependymoma, 54 with PFE were identified

Imaging features to distinguish posterior fossa ependymoma subgroups. Posterior fossa ependymoma group A (EPN_PFA) and group B (EPN_PFB) can be distinguished by their DNA methylation and give rise to different prognoses. We compared the MRI characteristics of EPN_PFA and EPN_PFB at presentation. Preoperative imaging of 68 patients with posterior fossa ependymoma from two centers was reviewed (p = 0.0008). All mainly cystic tumors were EPN_PFB (p = 0.002). The minimal and maximal relative ADC was slightly lower in EPN_PFA (p = 0.02 and p = 0.01, respectively). Morphological characteristics from imaging differ between posterior fossa ependymoma subtypes and may help to distinguish them preoperatively. This study provides a tool to differentiate between group A and group B ependymomas