Extraventricular Ependymoma Research Articles

Machine learning-based nomogram for distinguishing between supratentorial extraventricular ependymoma and supratentorial glioblastoma.

To develop a machine learning-based nomogram for distinguishing between supratentorial extraventricular ependymoma (STEE) and supratentorial glioblastoma (GBM). We conducted a retrospective analysis on MRI datasets obtained from 140 patients who were diagnosed with STEE (n=48) and GBM (n=92) from two institutions. Initially, we compared seven different machine learning algorithms to determine the most suitable signature (rad-score). Subsequently, univariate and multivariate logistic regression analyses were performed to identify significant clinical predictors that can differentiate between STEE and GBM. Finally, we developed a nomogram by visualizing the rad-score and clinical features for clinical evaluation. The TreeBagger (TB) outperformed the other six algorithms, yielding the best diagnostic efficacy in differentiating STEE from GBM, with area under the curve (AUC) values of 0.735 (95% CI: 0.625-0.845) and 0.796 (95% CI: 0.644-0.949) in the training set and test set. Furthermore, the nomogram incorporating both the rad-score and clinical variables demonstrated a robust predictive performance with an accuracy of 0.787 in the training set and 0.832 in the test set. The nomogram could serve as a valuable tool for non-invasively discriminating between STEE and GBM.

Ensemble learning-based pretreatment MRI radiomic model for distinguishing intracranial extraventricular ependymoma from glioblastoma multiforme.

This study aims to develop an ensemble learning (EL) method based on magnetic resonance (MR) radiomic features to preoperatively differentiate intracranial extraventricular ependymoma (IEE) from glioblastoma (GBM). This retrospective study enrolled patients with histopathologically confirmed IEE and GBM from June 2016 to June 2021. Radiomics features were extracted from T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI) sequence images, and classification models were constructed using EL methods and logistic regression (LR). The efficiency of the models was assessed using receiver operating characteristic (ROC) curves, calibration curves, and decision curve analysis. The combined EL model, based on clinical parameters and radiomic features from T1WI and T2WI images, demonstrated good discriminative ability, achieving an area under the receiver operating characteristics curve (AUC) of 0.96 (95% CI 0.94-0.98), a specificity of 0.84, an accuracy of 0.92, and a sensitivity of 0.95 in the training set, and an AUC of 0.89 (95% CI 0.83-0.94), a specificity of 0.83, an accuracy of 0.81, and a sensitivity of 0.74 in the validation set. The discriminative efficacy of the EL model was significantly higher than that of the LR model. Favorable calibration performance and clinical applicability for the EL model were observed. The EL model combining preoperative MR-based tumor radiomics and clinical data showed high accuracy and sensitivity in differentiating IEE from GBM preoperatively, which may potentially assist in clinical management of these brain tumors.

Nomogram incorporating preoperative MRI-VASARI features for differentiating intracranial extraventricular ependymoma from glioblastoma.

Intracranial extraventricular ependymoma (IEE) and glioblastoma (GBM) may have similar imaging findings but different prognosis. This study aimed to develop and validate a nomogram based on magnetic resonance imaging (MRI) Visually AcceSAble Rembrandt Images (VASARI) features for preoperatively differentiating IEE from GBM. The clinical data and the MRI-VASARI features of patients with confirmed IEE (n=114) and confirmed GBM (n=258) in a multicenter cohort were retrospectively analyzed. Predictive models for differentiating IEE from GBM were built using a multivariate logistic regression method. A nomogram was generated and the performance of the nomogram was assessed with respect to its calibration, discrimination, and clinical usefulness. The predictors identified in this study consisted of six VASARI features and four clinical features. Compared with the individual models, the combined model incorporating clinical and VASARI features had the highest area under the curve (AUC) value [training set: 0.99, 95% confidence interval (CI): 0.98-1.00; validation set: 0.97, 95% CI: 0.94-1.00] in comparison to the clinical model. The nomogram was well calibrated with significant clinical benefit according to the calibration curve and decision curve analyses. The nomogram combining clinical and MRI-VASARI characteristics was robust for differentiating IEE from GBM preoperatively and may potentially assist in diagnosis and treatment of brain tumors.

Supratentorial extra-ventricular ependymoma as a mass lesion in a child: report and literature review.

Supratentorial extra-ventricular ependymoma (SEE) are extremely rare in pediatric population and have varied presentation based on size, location, epicentre and compression on neurovascular structure. The authors report a 7-year-old girl presenting with seizure, who had a lobar SEE on MRI scan, successfully treated by microsurgical resection and adjuvant therapy.

Magnetic resonance imaging findings of intracranial extraventricular ependymoma: A retrospective multi-center cohort study of 114 cases.

Intracranial extraventricular ependymoma (IEE) is an ependymoma located in the brain parenchyma outside the ventricles. IEE has overlapping clinical and imaging characteristics with glioblastoma multiforme (GBM) but different treatment strategy and prognosis. Therefore, an accurate preoperative diagnosis is necessary for optimizing therapy for IEE. A retrospective multicenter cohort of IEE and GBM was identified. MR imaging characteristics assessed with the Visually Accessible Rembrandt Images (VASARI) feature set and clinicopathological findings were recorded. Independent predictors for IEE were identified using multivariate logistic regression, which was used to construct a diagnostic score for differentiating IEE from GBM. Compared to GBM, IEE tended to occur in younger patients. Multivariate logistic regression analysis identified seven independent predictors for IEE. Among them, 3 predictors including tumor necrosis rate (F7), age, and tumor-enhancing margin thickness (F11), demonstrated higher diagnostic performance with an Area Under Curve (AUC) of more than 70% in distinguishing IEE from GBM. The AUC was 0.85, 0.78, and 0.70, with sensitivity of 92.98%, 72.81%, and 96.49%, and specificity of 65.50%, 73.64%, and 43.41%, for F7, age, and F11, respectively. We identified specific MR imaging features such as tumor necrosis and thickness of enhancing tumor margins that could help to differentiate IEE from GBM. Our study results should be helpful to assist in diagnosis and clinical management of this rare brain tumor.

Extraventricular site indicates higher grade but better prognosis in adult supratentorial ependymomas: a 14-year single-center retrospective cohort.

Supratentorial extraventricular ependymoma (STEE) and supratentorial intraventricular ependymoma (STIE) are two subsets of supratentorial ependymoma (SE). These two subsets have similar gene features and only differ in original sites: STEE occurs in the brain parenchyma, and STIE is located in ventricles and surrounded by cerebral spinal fluid. The present study aims to depict the diversities of these two subsets and elucidate the potential effects of the anatomic site on the tumor with the same type, grade, and molecular features. Sixty-six consecutive adult SE patients from 2008 to 2021 were enrolled in our study. Clinical data, pathological features, and long-term outcomes were analyzed retrospectively. Results demonstrated that adult STEE presented with a higher proportion of WHO grade 3 (P = .028) and higher Ki-67 index (≥10%) (P = .019) compared to adult STIE. Survival analysis demonstrated that patients of grade 3 STEE exhibited a significantly longer overall survival (OS) than patients of grade 3 STIE (median OS, 24.4 months vs. 13.0 months; P = .004). Grade 2 (hazard ratio (HR) = 0.217; P < .001) and gross total resection (GTR) (HR = 0.156; P < .001) were identified as favorable prognostic factors for all adult SE. The STEE was also associated with a lesser hazard of death for patients of grade 3 on multivariate analysis (HR = 0.263; P = .047). These findings suggested that the extraventricular site was an indicator for higher grade and better prognosis in adult supratentorial ependymoma.

Differentiation between supratentorial pilocytic astrocytoma and extraventricular ependymoma using multiparametric MRI.

The differentiation of supratentorial pilocytic astrocytomas (STPAs) and supratentorial extraventricular ependymomas (STEEs) is clinically pivotal because of distinct therapeutic management and prognosis, which is sometimes challenging to both neuroradiologists and pathologists. To explore and compare the conventional and advanced magnetic resonance imaging (MRI) features between STPA and STEE. A total of 23 patients with STPAs and 23 patients with STEEs were reviewed in this study. All patients performed conventional MRI, susceptibility-weighted imaging (SWI), and diffusion-weighted imaging (DWI), and 34 patients (17 with STPAs and 17 with STEEs) examined dynamic susceptibility contrast-enhanced perfusion-weighted imaging (DSC-PWI) in addition. Clinical data, conventional MRI features, minimum relative apparent diffusion coefficient ratio (rADCmin), and maximum relative cerebral blood volume ratio (rCBVmax) were compared between the two groups and subgroups. The optimal cutoff values of rADCmin and rCBVmax with sensitivity and specificity were calculated. STPA manifested similar to STEE as a solid-cystic mass but more frequently presented with a marked enhancing deep nodule (P = 0.02), no peritumoral edema (P = 0.036), higher rADCmin value (2.0 ± 0.5 vs. 0.9 ± 0.2; P < 0.001), and lower rCBVmax value (2.1 ± 0.4 vs. 14.4 ± 5.5; P < 0.001). The cutoff value of >1.39 for rADCmin and ≤ 2.81 for rCBVmax produced a high sensitivity of 95.65% and 100.0%, respectively, and all produced a specificity of 100.0% in differentiating STPAs from STEEs. Multiparametric MRI techniques including conventional MRI, DWI, and DSC-PWI contribute to the differential diagnosis of STPA and STEE.

Developing a Radiomics Signature for Supratentorial Extra-Ventricular Ependymoma Using Multimodal MR Imaging.

Rationale and Objectives: To build a machine learning-based diagnostic model that can accurately distinguish adult supratentorial extraventricular ependymoma (STEE) from similarly appearing high-grade gliomas (HGG) using quantitative radiomic signatures from a multi-parametric MRI framework.Materials and Methods: We computed radiomic features on the preprocessed and segmented tumor masks from a pre-operative multimodal MRI dataset [contrast-enhanced T1 (T1ce), T2, fluid-attenuated inversion recovery (FLAIR), apparent diffusion coefficient (ADC)] from STEE (n = 15), HGG-Grade IV (HGG-G4) (n = 24), and HGG-Grade III (HGG-G3) (n = 36) patients, followed by an optimum two-stage feature selection and multiclass classification. Performance of multiple classifiers were evaluated on both unimodal and multimodal feature sets and most discriminative radiomic features involved in classification of STEE from HGG subtypes were obtained.Results: Multimodal features demonstrated higher classification performance over unimodal feature set in discriminating STEE and HGG subtypes with an accuracy of 68% on test data and above 80% on cross validation, along with an overall above 90% specificity. Among unimodal feature sets, those extracted from FLAIR demonstrated high classification performance in delineating all three tumor groups. Texture-based radiomic features particularly from FLAIR were most important in discriminating STEE from HGG-G4, whereas first-order features from T2 and ADC consistently ranked higher in differentiating multiple tumor groups.Conclusions: This study illustrates the utility of radiomics-based multimodal MRI framework in accurately discriminating similarly appearing adult STEE from HGG subtypes. Radiomic features from multiple MRI modalities could capture intricate and complementary information for a robust and highly accurate multiclass tumor classification.

Supratentorial Extraventricular Ependymomas: Imaging Features and the Added Value of Apparent Diffusion Coefficient.

To improve the understanding and the diagnosis of intracranial ependymal tumors. The clinical, radiological and prognostic features of 48 supratentorial extraventricular ependymomas and 74 intraventricular ependymomas were summarized and compared. Supratentorial extraventricular ependymomas, most often located in the frontal lobe (33.3%) and classified as grade III (75.0%), had relatively large eccentric cysts (3.07 ± 2.03 cm), significant enhancement (84.8%), low apparent diffusion coefficient (ADC) values, and associated with higher mortality (41.3%). The majority of intraventricular lesions occurred in the fourth ventricle (86.5%) and classified as grade II (78.4%), had relatively small and multiple cystic changes (1.04 ± 0.87 cm), slight or moderate enhancement (76.9%), high ADC values and associated with lower mortality (20.7%). There were few significant differences between grade II and grade III tumors in these 2 groups, respectively. Young age, high grade and low ADC values are worse prognostic indicators for patients with supratentorial extraventricular ependymomas, but not for those with intraventricular ependymomas. Conventional radiological features, combined with clinical manifestations and quantitative information provided by diffusion-weighted imaging, may not only enhance the diagnosis and assist in determining prognosis but also provide a better pathophysiological understanding of intracranial ependymal tumors.

The role of clinical factors and immunocheckpoint molecules in the prognosis of patients with supratentorial extraventricular ependymoma: a single-center retrospective study

PurposeSupratentorial extraventricular ependymoma (SEE) is a rare subset of ependymomas located in the supratentorial parenchyma, and little is known regarding its management and prognosis. Our study aimed to reveal the prognostic factors in patients with SEE and the roles of programmed death ligand-1 (PD-L1), programmed cell death protein 1 (PD-1), Ki-67, and neural cell adhesion molecule L1 (L1CAM) in predicting these patients’ outcomes.MethodsWe retrospectively studied the clinical features and prognostic factors in 48 patients with SEE admitted to our center from April 2008 to October 2018. Tissue slides were constructed from patient samples, and PD-L1, PD-1, Ki-67, and L1CAM expression levels were evaluated by immunohistochemistry.ResultsPatients with gross total resection (GTR) had better progression-free survival than patients with subtotal resection (STR). Moreover, the recurrence hazard ratios in patients with STR at 3, 5, and 10 years were 8.746, 6.866 and 3.962 times those of patients with GTR, respectively. PD-L1 positivity predicted worse progression-free survival, while the recurrence hazard ratios for patients with PD-L1 positivity at 3, 5, and 10 years were 10.445, 5.539, and 3.949 times those of patients with PD-L1 negativity, respectively. Multivariate analysis revealed that PD-L1 expression and GTR could independently predict outcomes in patients with SEE.ConclusionPD-L1 expression was an independent and more readily obtained predictor of outcomes, representing a simple and reliable biological prognostic factor for patients with SEE. Further studies are needed to explore PD-L1 inhibitor treatment for patients with ependymoma.Clinical trial registrationNo clinical trials were performed in the study.

World Health Organization grade III supratentorial extraventricular ependymomas in adults: Case series and review of treatment modalities

Context:Supratentorial ependymomas and their anaplastic variants are relatively uncommon central nervous system neoplasms that afflict both adults and children.Aims:Discuss the clinical and pathological features of patients with anaplastic ependymomas involving an extraventricular supratentorial location and review modalities and options of treatment for those rare tumors.Settings and Design:Whereas the treatment algorithm in the pediatric population is well established, however, treatment in the adult population is less defined. Treatment options are exposed through the author's cases and review of the literature.Subjects and Methods:In our case series of two adult patients with supratentorial ependymomas World Health Organization (WHO) Grade III (anaplastic variant), patients presented in both cases in the emergency room after having a generalized tonic–clonic seizure at home the first case, and mild hemiparesis the second case.Results:Patients underwent surgical treatment, and a gross total resection was achieved in both cases. The histopathological examination revealed a diagnosis of anaplastic ependymoma (WHO Grade III). Both patients had additional radiotherapy, and in the first case, adjuvant platinum-based chemotherapy was administered due to leptomeningeal gliomatosis.Conclusion:In our experience, gross total resection was achieved in all patients with supratentorial extraventricular ependymomas WHO Grade III with additional radiotherapy and platinum-based chemotherapy. Patients require initial close serial imaging follow-up. The role of chemotherapy is still uncertain but may be necessary in younger patients and in tumors that behave more like the pediatric ependymomas.

Ependymoma with Intraorbital Extracerebral Recurrence: Case Report

AbstractEpendymomas 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 to ∼ 2 to 6% of all gliomas. Although it corresponds only to ∼2 to 3% of all primary brain tumors, ependymoma is the fourth most common cerebral neoplasm in 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 follow-up 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.

RELA Fusion in Supratentorial Extraventricular Ependymomas: A Morphologic, Immunohistochemical, and Molecular Study of 43 Cases.

Supratentorial extraventricular ependymomas (STEEs) are relatively rare ependymomas, and their pathologic and genetic characteristics are still poorly understood. The aim of this study was to determine the histologic, immunohistochemical, and RELA fusion features, as well as to clarify in more detail the clinical courses of STEEs. Data from a total of 43 patients with STEEs was analyzed retrospectively. The status of RELA fusion was evaluated using fluorescence in situ hybridization. The expression levels of L1CAM, p65, cyclin D1, and p53 were assessed using immunohistochemistry. Progression-free survival and overall survival were calculated via Kaplan-Meier estimation using the log-rank test. Among all 43 STEEs, 65.1% (28/43) are positive for RELA fusion. Interestingly, almost half of the patients with RELA fusion-positive ependymomas are adults (13/28), and 89.3% (25/28) cases are anaplastic ependymomas, which suggests that RELA fusion testing is necessary in adults with STEEs. We investigated the immunohistochemical status of p65, L1CAM and CCND1 protein expression for their ability to predict RELA fusion status. RELA fusion-positive STEEs are frequently associated with expression of p65 (85.2%), L1CAM (85.2%), and CCND1 (81.5%). The accuracy of predicting RELA fusion status was much higher when the expression of p65 and L1CAM was combined, that is, when both were immunopositive. The status of RELA fusion, p53 overexpression, and extent of tumor resection are significantly associated with prognosis.

Clinical features and treatment analysis of intracranial extraventricular ependymomas

Objective To study the clinical features, imaging demonstration and treatment outcomes of intracranial extraventricular ependymomas. Methods A total of 17 patients with intracranial extraventricular ependymoma underwent surgical treatment at Department of Neurosurgery, Renmin Hospital of Wuhan University from March of 2010 to June of 2016 and were enrolled into this retrospective study. Pre-operative MR scan showed single lesion located at supratentorial region in all cases. Those lesions were located in occipital lobe (5 cases), occipitoparietal lobe (2 cases), temporal lobe (3 cases), frontal lobe (4 cases) and parietal lobe (3 cases). Among those, 7 were solid and 10 were cystic lesions. Surgical excision was performed for all lesions. Post-operative follow-up was performed for every patient through telephone and outpatient review. Results Of those cases, total resection was achieved in 15 cases, including 10 cases of WHO grade Ⅱ and 5 case of WHO grade Ⅲ, and partial resection in 2 (WHO grade Ⅲ). Intraoperative pathological diagnosis suggested that 10 cases were ependymoma (WHO gradeⅡ) and 7 cases were anaplastic ependymoma (WHO grade Ⅲ). All patients′ clinical symptoms were relieved following surgery. Four cases (grade Ⅲ), including 2 cases undergoing partial resection, received post-operative radiotherapy. The follow-up time of those patient were from 14 to 74 months, with a median of 33.2 months. During the follow-up, no case showed any signs of spinal cord metastasis. Recurrence was reported in 7 cases and 4 of them (grade Ⅲ) received post-operative radiotherapy. Among those, 1 case underwent the second tumor total resection and postoperative radiotherapy, 1 case died 3 months after the second tumor total resection, 2 cases of tumor partial resection rejected the second operation and died after 4 and 6 months, respectively, and the other 3 recurrent cases included 2 (grade Ⅱ) and 1 (grade Ⅲ) undergoing the second tumor total resection without postoperative radiotherapy and 1 (grade Ⅲ) that died of postoperative hemorrhage. Conclusions As intracranial extraventricular ependymoma has non-specific clinical and imaging features, accurate pre-operative diagnosis is difficult. Total tumor resection is the primary method of treatment, if it is possible. The choice of resection type and pathological grading could be predictive factors in postoperative evaluation. Key words: Ependymoma; Neurosurgical procedures; Treatment outcome; Intracranial extraventricular

Supratentorial Extraventricular Ependymoma: Retrospective Analysis of 15 Patients at a Single Institution

Supratentorial extraventricular ependymoma (STEE) is an extremely rare central nervous system (CNS) neoplasm, the clinical characteristics and optimal treatment of which remain unclear. We retrospectively analyzed the clinical characteristics and treatment outcomes of 15 patients with STEE. Fifteen patients with STEE were identified, and their clinical, radiologic, and surgical records were reviewed. The 15 patients included 6 males (40%) and 9 females (60%), with a median age of 15 years. Nine patients (60%) underwent gross total resection, 5 patients (33.3%) underwent subtotal resection, and 1 patient (6.7%) underwent biopsy. Eight patients received adjuvant radiotherapy, and 3 received adjuvant chemotherapy. The 5-year overall survival (OS) rates of patients with World Health Organization (WHO) grade II and grade III STEE were 100% and 30%, respectively, and the corresponding 2-year progression-free survival (PFS) rates were 77.8% and 20%. The differences in OS and PFS rates between WHO grades II and III STEE were statistically significant (P < 0.01 and 0.02, respectively). Anaplastic histology (WHO grade III) was a poor prognostic factor for PFS (P= 0.04). The recurrence pattern was local recurrence in all patients; all cases were treated primarily by redo surgery. Three patients with recurrent WHO grade II STEE were alive at more than 64, 52, and 44 months after redo surgery. STEE is an extremely rare CNS neoplasm. The histological grade of STEE is an important prognostic factor. Microsurgical resection might play a major role in both initial treatment and treatment of recurrent tumors.

Supratentorial extraventricular ependymomas: A retrospective study focused on long-term outcomes and prognostic factors

ObjectiveSupratentorial extraventricular ependymomas are relatively rare. Long-term outcomes and prognostic factor for this rare tumor have not been well established. The purpose of this study was to demonstrateprogression-freesurvival(PFS),overallsurvival(OS), and prognostic factors of such tumor. Patients and methodsFifty-five patients with supratentorial extraventricular ependymomas from our hospital were included in this study. Epidemiological characteristics, clinical features, treatment,long-term outcomes, and prognostic factors for PFS and OS were reviewed retrospectively. ResultsThe patients consisted of 30 males and 25 females with mean age of 30.0 ± 23.6 years (range, 1–74 years). Twenty-nine tumors were located in the right hemisphere, and 26 in the left side. The 2 most common tumor locations were the frontal (n = 19; 35%) and parietal lobe (n = 11; 20%). All patients underwent surgical resection. Gross-total resection (GTR) was achieved in 42 cases (76%) and subtotal resection (STR) was performed in 13 patients (24%). According to the WHO classification system, 38 tumors (69%) were Grade III (anaplastic ependymoma), and 17 (31%) were Grade II (ependymoma). Three-,5-, and 10 year PFS rates were 60%, 49%, and 36%, respectively. Three-,5-, and 10 year OS rates were 79%, 64%, and 49%, respectively. EOR and tumor grade were identified as prognostic factors for PFS and OS on univariate analysis, multivariate analysis, and Kaplan-Meierlog-rank testing. Subtotal resection (STR) predicted a worse PFS (HR = 4.808; 95%, 1.942–11.905; P = .001) and OS (HR = 5.650; 95%, 2.114–15.152; P = .001). WHO Grade III tumors also had worse PFS (HR = 3.922; 95%, 1.429–18.182; P = .012) and OS (HR = 6.329; 95%, 1.328–30.303; P = 0.021). For patients with tumor recurrence, reoperation was significant prognostic factors for OS (HR = 2.091; 95%, 0.939–4.654; p = .000). Age, sex, tumor side, and postoperativeradiotherapy were not prognostic factors for PFS and OS. ConclusionsMost supratentorial extraventricular ependymomas are WHO grade III tumors. STRandWHO Grade III pathology predicted worse PFS and OS. Gross-total resection remains the optimal treatment for patients with supratentorial extraventricular ependymoma. Reoperation should be considered first in cases of recurrence. The role of postoperative radiotherapy as an adjuvant treatment for supratentorial extraventricular ependymoma needs further investigation.

Clinical, radiological, and histological features and treatment outcomes of supratentorial extraventricular ependymoma: 14 cases from a single center.

OBJECTIVE Reports on supratentorial extraventricular ependymoma (STE) are relatively rare. The object of this study was to analyze the clinical, radiological, and histological features and treatment outcomes of 14 patients with STE. METHODS Overall, 227 patients with ependymoma underwent surgical treatment in the authors' department between January 2010 and June 2015; 14 of these patients had STE. Data on clinical presentation, radiological studies, histopathological findings, surgical strategies, and treatment outcomes in these 14 cases were retrospectively analyzed. RESULTS The patients consisted of 6 women and 8 men (sex ratio 0.75). Mean age at diagnosis was 24.5 ± 13.5 years (range 3-48 years). Tumors were predominantly located in the frontal and temporal lobes (5 and 4 cases, respectively). Typical radiological features were mild to moderate heterogeneous tumor enhancements on contrast-enhanced MRI. Other radiological features included well-circumscribed, "popcorn" enhancement and no distinct adjoining brain edema. Gross-total resection was achieved in 12 patients, while subtotal removal was performed in 2. Radiotherapy was administered in 7 patients after surgery. Seven tumors were classified as WHO Grade II and the other 7 were verified as WHO Grade III. The mean follow-up period was 22.6 months (range 8-39 months). There were 3 patients with recurrence, and 2 of these patients died. CONCLUSIONS Supratentorial extraventricular ependymoma has atypical clinical presentations, various radiological features, and heterogeneous histological forms; therefore, definitive diagnosis can be difficult. Anaplastic STE shows malignant biological behavior, a higher recurrence rate, and a relatively poor prognosis. Gross-total resection with or without postoperative radiotherapy is currently the optimal treatment for STE.

Advanced MR imaging of extraventricular supratentorial cortical ependymoma

Ependymomas arise from the ependymal cells of the ventricular system. Ependymomas are relatively uncommon nervous system tumors constituting 2% - 6% of all gliomas. They represent 4% of all CNS tumors in the adult. Extraventricular ependymomas are especially rare with pure cortical ependymomas uncommon. Ependymomas are the most common of the ependymal tumors, and are considered WHO grade II tumors. The location of ependymomas in adults tends to be different than the location of ependymomas in children. Total tumor resection has the best chance of long term survival. The extent of tumor removal continues to be the strongest factor influencing survival and recurrence. MRI of the brain is usually done every 3 months for the first two years following diagnosis to determine the effectiveness of treatment and to watch for possible recurrence. Supratentorial cortical ependymoma without attachment to the ventricular system is a rare tumor in the adult central nervous system. We present a case of a 25-year-old lady with a large supratentorial cortical ependymoma showing massive calcifications and central cyst formation manifested as headache and generalized seizures.

Paediatrics Supratentorial Extraventricular Ependymoma

Introduction: Majority of ependymomas are infratentorial and intraventricular. Supratentorial and purely cortical extraventricular ependymomas are extremely rare and only six cases were reported so far. Case report: We report a 12-year-old female with right parieto occipital ependymoma. She had complete excision of the lesion. Conclusion: Pure cortical, supratentorial extraventricular ependymoma in paediatrics age group is a rarity.

Prognosis by tumor location in adults with intracranial ependymomas

Intracranial ependymomas are rare tumors in adults. Thus, factors affecting prognosis are poorly understood. We performed a study to investigate whether tumor location is an important prognostic factor in adults who undergo surgery for intracranial ependymomas. PubMed was searched to identify studies that reported clinical outcomes in adult patients with intracranial ependymoma. Data were extracted for patient and tumor characteristics, extent of resection, progression-free survival (PFS), and overall survival (OS). Tumors were categorized as supratentorial or infratentorial and extraventricular or intraventricular. Presenting clinical features and tumor characteristics were tabulated. Kaplan–Meier and multivariate Cox regression survival analyses were performed to determine PFS and OS by tumor location. Extent of resection was also analyzed by tumor location. A total of 183 patients were included in the meta-analysis. Patients presented at a mean of 8.2months with a myriad of clinical features. The mean tumor size was 3.38cm, and 19.3% of tumors were cystic. Supratentorial tumors were most commonly located in the frontal and parietal lobes, and infratentorial tumors in the fourth ventricle. Supratentorial tumors demonstrated significantly poorer PFS (p<0.001) and OS (p=0.003) than infratentorial tumors, despite a higher rate of gross total resection (GTR) for the supratentorial tumors (72.6% versus 42.1%). Extraventricular ependymomas displayed significantly poorer PFS than intraventricular ependymomas (p=0.009). In summary, supratentorial ependymomas have significantly poorer PFS and OS than their infratentorial counterparts, despite being more conducive to GTR, suggesting increased clinical aggressiveness. Extraventricular location is also associated with significantly poorer PFS than intraventricular location.