Novel case of ependymoma-like tumor with mesenchymal differentiation harboring ZFTA::RELA fusion in an adult.

High-grade supratentorial tumors harboring ZFTA::NCOA1/2 fusion in infants presenting with mixed histology of embryonal-appearing components resembling ependymoma and mesenchymal sarcomatous components have recently been reported as ependymoma-like tumors with mesenchymal differentiation (ELTMDs). In contrast, we describe herein a pathologically similar case with a novel ZFTA::RELA fusion in an adult. A frontal lobe lesion was resected from a 30-year-old woman and displayed mixed components on pathological examination, showing ependymoma-like and sarcomatous parts. The absence of perivascular pseudorosettes was inconsistent with a diagnosis of ependymoma. Fluorescence in situ hybridization analysis confirmed ZFTA::RELA fusion. The DKFZ methylation classifier (v12.8) did not categorize this case among established methylation classes. In addition, t-distributed stochastic neighbor embedding analysis using DNA methylation data revealed that the present case was distant from ependymomas but close to two previously reported cases of ELTMD involving ZFTA::NCOA1/2 fusion. Taken together, we concluded that this tumor should be considered under the entity of ELTMD. This represents the first description of an adult patient with ELTMD harboring ZFTA::RELA fusion analyzed by DNA methylation profiling, supporting the establishment of ELTMD as a possible new tumor type.

Ventricular system-unrelated cerebellar ependymoma: A case report.

BACKGROUND: An ependymoma is a glial tumor that usually occurs in or near the ventricle, close to the ependyma. It rarely occurs exclusively in the brain parenchyma without being associated with the ventricle. CASE SUMMARY: Here, we report a rare case of a cerebellar ependymoma completely located in the brain parenchyma. A previously healthy 32-year-old female with a 1-month history of dizziness was admitted to our hospital. During hospitalization, magnetic resonance imaging of the brain revealed a space-occupying lesion measuring 57 mm × 41 mm × 51 mm in the right cerebellar hemisphere and inferior cerebellar vermis. The patient underwent surgical resection for the right cerebellar mass. Postoperative pathological examination revealed an ependymoma. At 1 year follow-up, the patient was doing well and showed no recurrence. CONCLUSION: We conducted a literature review and summarized three theories regarding ependymomas located exclusively in the brain parenchyma, which are key to the diagnosis of intraparenchymal cerebellar ependymomas. Surgery and postoperative radiotherapy are the primary treatment options for ependymomas.

Giant metastatic mixed-type spinal ependymoma presenting with hydrocephalus.

Ependymomas are rare nervous system tumors that can arise anywhere in the neuraxis. While having a high propensity for leptomeningeal dissemination, retrograde dissemination (from the spine to the CNS) remains infrequent. We describe the case of a 31-year-old female who presented with hydrocephalus secondary to an intracranial leptomeningeal metastasis of a giant spinal ependymoma with mixed (classic and myxopapillary) histopathologic features, successfully treated with surgical resection and radiotherapy of the entire neuraxis. This case highlights the importance of including ependymomas in the differential diagnosis for lesions in atypical extra-axial locations, of systematically obtaining imaging of the entire neuraxis when suspecting it, and of considering retrograde dissemination when both intracranial and spinal lesions are present.

Lack of classical astroblastoma features in pediatric MN1::BEND2-fused brain tumors.

Three distinct MN1::BEND2 fusion-positive tumors in pediatric patients. (A) Clinical course for each patient was variable in part due to differences in initial diagnosis. Each patient responded favorably to gross total resection and is stable at last follow-up. (B) Histologic diversity, lack of prominent classical astroblastoma features, and variable immunoexpression of key markers makes microscopic diagnosis challenging.

Collision tumor comprised of chronic lymphocytic leukemia and myxopapillary ependymoma.

Background: Collision tumors involving the co-occurrence of two morphologically and genomically distinct neoplasms in the same anatomical site are exceptionally rare in the central nervous system (CNS). Case Description: We report a unique case of a CNS collision tumor comprising chronic lymphocytic leukemia and myxopapillary ependymoma in a 77-year-old male with acute neurological decline. Presumed to represent leukemic infiltration, urgent laminectomy was pursued for tissue diagnosis and spinal cord decompression, revealing the unexpected ependymal component. Conclusion: This case highlights the diagnostic and therapeutic challenges inherent to managing collision CNS tumors, particularly when one neoplasm is hematological.

Identification and validation of miRNA-target genes network in pediatric brain tumors.

Alterations in miRNA levels have been observed in various types of cancer, impacting numerous cellular processes and increasing their potential usefulness in combination therapies also in brain tumors. Recent advances in understanding the genetics and epigenetics of brain tumours point to new aberrations and associations, making it essential to continually update knowledge and classification. Here we conducted molecular analysis of 123 samples of childhood brain tumors (pilocytic astrocytoma, medulloblastoma, ependymoma), focusing on identification of genes that could potentially be regulated by crucial representatives of OncomiR-1: miR-17-5p and miR-20a-5p. On the basis of microarray gene expression analysis and qRTPCR profiling, we selected six (WEE1, CCND1, VEGFA, PTPRO, TP53INP1, BCL2L11) the most promising target genes for further experiments. The WEE1, CCND1, PTPRO, TP53INP1 genes showed increased expression levels in all tested entities with the lowest increase in the pilocytic astrocytoma compared to the ependymoma and medulloblastoma. The obtained results indicate a correlation between gene expression and the WHO grade and subtype. Furthermore, our analysis showed that the integration between genomic and epigenetic pathways should now point the way to further molecular research.

Microsurgery resection of giant cervicothoracic spinal ependymoma: Two-dimensional operative video.

Background: Ependymomas, rare glial brain tumors, account for <5% of all brain tumors. Interestingly, over 60% of ependymomas occur in the spinal cord of adults, including those originating from the filum terminale, while the rest are found within the brain. The World Health Organization (WHO) categorizes ependymomas into three grades: subependymomas and myxopapillary ependymomas ([MEPNs]; WHO grade I), classic ependymomas (WHO grade II), and anaplastic ependymomas (WHO grade III). Spinal ependymomas generally exhibit a more favorable prognosis compared to their intracranial counterparts and are primarily treated through gross total resection, which is considered the most effective surgical approach. As such, they are recognized as a distinct clinical entity that demands tailored management strategies. MEPNs, which constitute 13% of ependymomas, typically occur in the cauda equina and sometimes extend into the conus medullaris. Most other spinal ependymomas are of the classic type and predominantly arise in the cervical and thoracic regions of the spine. The mean age at diagnosis is 45 years of age. While prognosis varies based on molecular subtypes, complete resection is associated with improved survival. Case Description: Here, we demonstrate the technical nuances to safely achieve gross total resection of a giant spinal ependymoma in a 29-year-old female with a medical history notable for sept-optic dysplasia, and panhypopituitarism. The patient presented with progressive neck pain, upper and lower extremity weakness, and numbness for 1 year. On physical examination, she demonstrated mild weakness in her left arm. The preoperative magnetic resonance imaging revealed a cervicothoracic intramedullary mass extending from C4 to T2 with an associated syrinx at C4. Under intraoperative neural monitoring (somatosensory evoked potentials, motor-evoked potentials, and epidural direct wave recordings), the patient underwent a C4 - T2 laminectomy. In addition, spinal ultrasonography helped differentiate solid tumor mass from syrinx formation, thus guiding the focus and extent of the decompression . Conclusion: Gross total resection was achieved; at 18 postoperative months, the patient had mild residual motor deficit. The pathological evaluation revealed a WHO grade II ependymoma. Subsequent sequential enhanced MR studies at 3, 6, and 12 months confirmed no tumor recurrence.

The medical and functional burden of surviving childhood ependymoma: A population-based study in Ontario, Canada.

BACKGROUND: Few studies have characterized the burden of late effects among childhood ependymoma survivors. To address this gap, we examined these sequelae using real-world health services data in a population-based ependymoma survivor cohort. METHODS: All individuals younger than 18 years diagnosed with an ependymoma in Ontario, Canada between 1987 and 2015 who had survived at least 5 years from their latest pediatric cancer event (index date) were matched 1:5 with population controls. Following linkage with provincial health services data, the cumulative incidences of multiple medical and functional outcomes between survivors and controls were compared. RESULTS: Among 96 survivors, 77.1% had been irradiated and 9.4% had received cisplatin. At 10 years post-index, survivors were at significantly higher risk of all-cause mortality (7.1%, 95% confidence interval [CI]: 1.0-13.3 vs. 0.3%, 95% CI: 0.0-1.0; p = .0002), non-obstetric hospitalization (45.1%, 95% CI: 32.6-56.7 vs. 10.6%, 95% CI: 7.6-14.1; p < .0001), stroke (6.5%, 95% CI: 2.3-13.7 vs. 0%; p < .0001), severe hearing loss requiring an amplification device (7.5%, 95% CI: 2.7-15.7 vs. 0%; p < .0001), receiving homecare service (27.6%, 95% CI: 18.5-37.5 vs. 7.7%, 95% CI: 5.3-10.7; p < .0001), and submitting a disability support prescription claim (24.0%, 95% CI: 14.8-34.3 vs. 5.4%, 95% CI: 3.5-7.8; p < .0001) compared to controls. CONCLUSIONS: Pediatric ependymoma survivors are highly vulnerable to severe late sequelae, including death, stroke, severe hearing loss, and disability. Urgent efforts are needed to improve risk-stratification approaches that mitigate exposure to toxic therapies for children with lower risk disease. Interventions to prevent or decrease the risk of developing late sequelae are critical to optimizing survivor long-term health.

Myxopapillary Ependymoma Metastasis Mimicking Pulmonary Embolism: An Illustrative Case.

Myxopapillary ependymomas (MPEs) are rare spinal cord tumors with low rates of metastasis outside of the neuraxis. Gross total resection of MPEs can significantly improve progression-free survival; however, adjunctive treatment remains unstandardized. A 29-year-old female with a history of spina bifida occulta surgical correction and lower back pain presented with dyspnea and tachycardia. A large pulmonary artery mass was discovered consistent with pulmonary thromboembolism. It was subsequently determined to be an intravascular metastasis secondary to sacral MPE. Standardization of MPE treatment and clinical suspicion of spinal neoplasm in the setting of chronic back pain with undetermined origin are of value.

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.

Treatment of Extraneural Metastases of Myxopapillary Ependymomas With Dose-Dense Temozolomide and Lapatinib.

Myxopapillary ependymomas (MPEs) are rare tumors of the central nervous system, and outcomes are generally worse with recurrent disease. These tumors can rarely metastasize outside the neuraxis. We present a case of a 35-year-old female with a history of MPEs who developed extraneural metastases 11 years after her initial gross total resection. Sites of metastases included multiple bilateral intrapulmonary and pleural-based masses with pleural effusion and a pelvic mass. The patient was treated with dose-dense TMZ and lapatinib and had a mixed radiographic response after 12 cycles of treatment. This is the first known case of extraneural metastases of MPEs to demonstrate a radiographic response to dose-dense TMZ and lapatinib. This case presentation discusses the need to establish optimal treatment of extraneural ependymal metastases, duration of treatment, and strategy for the management of recurrent diseases.

Histopathological Spectrum of Gliomas and Its Immunohistochemical Correlation in a Tertiary Care Setup.

Introduction Central nervous system (CNS) tumors pose significant diagnostic challenges due to their varied morphological and differentiating characteristics. Modern advancements in immunohistochemistry (IHC) and molecular pathology have greatly enhanced prognostication, screening, and therapeutic management. Gliomas, a type of tumor originating from glial cells in the CNS, can develop from astrocytes, oligodendrocytes, or ependymal cells. According to the 2021 update, the classification of diffuse gliomas is primarily based on the presence or absence of isocitrate dehydrogenase (IDH1/2) mutations. IDH-wildtype gliomas (glioblastomas) have a significantly poorer prognosis compared to IDH-mutant gliomas (astrocytomas and oligodendrogliomas). Gliomas are highly infiltrative and resistant to treatment, making them largely incurable regardless of their grade and prognosis. Objective This study aimed to determine the histopathological diversity of gliomas and its correlation with protein expressions of IDH, ATRX gene (α-thalassemia/mental retardation syndrome X-linked), Ki-67, and p53 mutations (tumor suppressor gene-53), according to the 2021 World Health Organization (WHO) Classification of CNS Tumors, Fifth Edition. Methods This descriptive cross-sectional study was carried out in the Department of Pathology at a tertiary care center, focusing on various types of gliomas received over a two-year period. A total of 54 specimens of gliomas received from the Department of Neurosurgery were subjected to histopathological examination. Sections were stained using hematoxylin and eosin (H&E), and IHC was performed using four markers (IDH, ATRX, p53, Ki-67) in each case. Results were analyzed according to the 2021 WHO Classification of CNS Tumors, Fifth Edition. Results The majority of individuals were between the age group of 40 and 60 years, showing a male predominance (65%). The most common site was the frontal lobe. Glioblastoma constituted the largest proportion (46.2%) of the total cases, followed by astrocytoma (20.3%), oligodendroglioma (18.5%), pilocytic astrocytoma (7.4%), and ependymoma (7.4%). All 11 cases of astrocytoma exhibited IDH mutation and ATRX loss, with p53 positive in the majority of cases. Strong nuclear p53 immunohistochemical positivity in >10% of tumor nuclei correlates with TP53 mutations. Among 25 cases of glioblastoma, IDH was negative, ATRX was retained in all cases, and 11 cases were positive for p53 mutation. For oligodendroglioma, out of 10 cases, IDH mutation was positive, and ATRX was retained in all cases. p53 mutation was not seen in any case. All cases of pilocytic astrocytoma were negative for IDH and p53 mutations, with ATRX retained in all cases. In all cases of ependymoma, IDH and p53 mutations were negative, and ATRX was retained in all cases. Glioblastomas exhibited the highest Ki-67 expression. Conclusion The 2021 WHO Classification of CNS Tumors, Fifth Edition, was updated, building on previously established concepts and continuing to evolve. The final diagnosis of gliomas relies on a comprehensive combination of clinical evaluation, neuroimaging, pathological examination, and molecular analysis. Nonetheless, histopathological examination, along with IHC, remains the cornerstone of diagnosis.

Transcranial motor-evoked potentials disappear with pons transection.

Motor-evoked potential (MEP) monitoring by transcranial electrical stimulation (TES) is important for intraoperative motor function assessment in neurosurgery; however, false-negative results sometimes occur, and these findings should be interpreted with caution. Herein, we report an interesting MEP change resulting from a pons transection. The patient was a boy aged 5 years and 2 months. He underwent multiple craniotomies for cerebellar anaplastic ependymoma and was already paralyzed in the right upper and lower limbs. Therefore, we decided to remove the recurrent lesion from the left anterior pons. MEPs were recorded on both the right and left sides after the start of surgery but disappeared 1 h 30 min after the start of surgery in the TES on the operative side, even when the stimulation intensity was increased. The contralateral TES consistently recorded stable MEPs throughout the surgery. The tumor was completely resected on imaging. Immediately postoperatively, the patient experienced flaccid paralysis on the right side of the body, which recovered to preoperative levels over time. A transcranial MEP cannot be derived if the corticospinal tract is transected at the pons. Transcranial MEP findings may accurately reflect the corticospinal tract function if the injury is caudal to the pons.

Distinct relapse pattern across molecular ependymoma types.

BACKGROUND: Ependymoma (EPN) is not a uniform disease but represents different disease types with biological and clinical heterogeneity. However, the pattern of when and where different types of EPN relapse is not yet comprehensively described. METHODS: We assembled 269 relapsed intracranial EPN from pediatric (n=233) and adult (n=36) patients from European and Northern American cohorts and correlated DNA methylation patterns and copy-number alterations with clinical information. RESULTS: The cohort comprised the following molecular EPN types: PF-EPN-A (n=177), ST-EPN-ZFTA (n=45), PF-EPN-B (n=31), PF-EPN-SE (n=12), and ST-EPN-YAP (n=4). First relapses of PF-EPN-B (PF: posterior-fossa) and PF-EPN-SE (SE: subependymoma) occurred later than of PF-EPN-A, ST-EPN-YAP (ST: supratentorial), or ST-EPN-ZFTA (median time to relapse: 4.3 and 6.0 years vs. 1.9/1.0/2.4 years; p<0.01). Metastatic or combined recurrences in PF-EPN-B and -A more often involved the spinal cord than in ST-EPN-ZFTA (72.7% and 40.0 vs. 12.5%; p<0.01). No distant relapses were observed in ST-EPN-YAP (n=4) or PF-EPN-SE (n=12). Post-relapse survival (PRS) was poor for PF-EPN-A and ST-EPN-ZFTA (5-year PRS: 44.5±4.4/47.8±9.1%), whereas PF-EPN-B and PF-EPN-SE displayed a 5-year PRS of 89.5±7.1/90.0±9.5% (p=0.03). However, 10-year PRS for PF-EPN-B dropped to 45.8±17.3%. Neither between radiation field and relapse pattern nor between radiation field and spinal involvement at relapse an impact was identified. Notably, all patients with relapsed ST-EPN-YAP did not receive upfront radiotherapy, but were successfully salvaged using irradiation at relapse. CONCLUSIONS: Relapse patterns of specific EPN types are different. Future clinical trials, treatment adaptions, duration of surveillance and diagnostics should be planned incorporating entity-specific relapse information.

Survival Factors in 1580 Adults with Spinal Ependymoma: Insights from a Multicenter Oncology Database.

BACKGROUND: Using a multi-institutional oncology database, we investigate the survival rates and the impacts of demographic, clinical, and management characteristics on overall survival among adult patients diagnosed with spinal ependymoma. METHODS: Utilizing the SEER registry, patients with histologically or radiologically confirmed ependymomas were included. Factors impacting overall survival were analyzed using Kaplan-Meier survival curves and log-rank statistical analyses. RESULTS: A total of 1,580 patients were included. Their mean ± standard deviation age was 46.68 ± 15.96 years, and 51.1% were women. Gross total resection (GTR) was achieved in 66.4% of patients. The 5- and 10-year survival rates were 96.7% and 95.4%, respectively. A multivariable backward Cox regression showed that age ≥65 years was a significant predictor for mortality (hazard ratio [HR]: 3.93; 95% confidence interval [CI]: 2.21-7.00; P < 0.001). Likewise, tumor grade 3 (HR: 6.36; 95% CI: 1.95-20.76; P = 0.002), tumor grade 4 (HR: 7.74; 95% CI: 3.97-15.11; P < 0.001), presence of extra-neural metastasis (HR: 13.81; 95% CI: 3.67-51.96; P < 0.001), and receiving radiotherapy (HR: 2.50; 95% CI: 1.50-4.19; P < 0.001) were significant risk factors for mortality, while GTR was significantly associated with improved overall survival compared with subtotal resection or nonsurgical management (HR: 0.42; 95% CI: 0.25-0.73; P = 0.002). There were no significant effects for gender, race, marital status, income, residential area, chemotherapy, tumor size, and the presence of other benign or malignant tumors on the survival hazards (P > 0.05 for each). CONCLUSION: Early diagnosis and surgical management of spinal ependymomas, such as GTR, were associated with remarkable survival benefits. Old age, high-grade spinal ependymoma, and extra-neural metastasis were associated with worse overall survival, whereas radiotherapy's role remains unclear.

Radiotherapy dosing in intracranial ependymoma using the national cancer database.

PURPOSE: To determine the dose-dependent effect of adjuvant radiotherapy on survival for pediatric intracranial ependymomas and explore patient and disease characteristics that experience survival benefit from higher doses. METHODS: Data was accessed from the National Cancer Database. Inclusion criteria was comprised of a diagnosis of non-metastatic intracranial ependymoma, World Health Organization (WHO) grade 2 or 3, surgical resection, adjuvant radiotherapy between 4500-6300 cGy, and non-missing survivorship data. Crude and adjusted Cox proportional hazard ratios (HRs) were calculated to estimate the associations of patient, tumor, and treatment characteristics with overall survival (OS). Kaplan-Meier (KM) estimations were used to visualize survival curves for dosing for the general cohort and by subgroups (age, resection extent, and grade). RESULTS: Of the 1154 patients who met inclusion criteria, 405 received ≤ 5400 cGy and 749 received > 5400 cGy. We found no difference in OS crude (0.95, 95% CI 0.72-1.06) or adjusted (0.88, 95% CI 0.46-1.69) HR for those receiving ≤ 5400 cGy. KM curves showed no difference in OS for dosing for the general cohort based on age, surgical extent, and grade. However, there was better OS in those with WHO grade 2 tumors compared to grade 3 regardless of dose received. CONCLUSIONS: There was no difference in OS between patients who received ≤ 5400 cGy compared to > 5400 cGy. We found improved OS in those with grade 2 tumors compared to grade 3, however there was no difference in OS based on dose received by tumor grade, age, or resection extent. Limitations in data available prevent exploring other outcomes or toxicity.

Posterior Fossa Ependymoma in a Child with Extensive Chondro-Osseous Metaplasia Occurring at Cerebellopontine Angle and Masquerading as a Vestibular Schwannoma: An Exceptionally Rare Clinicopathological Manifestation with Review of Literature.

Ependymoma occurring at the cerebellopontine (CP) angle is an extremely uncommon sight and poses diagnostic and management dilemmas to neurosurgeons, radiologists, and neuropathologists alike. Moreover, the presence of extensive chondro-osseous metaplastic elements in ependymomas is an exceptionally infrequent histopathological manifestation. However, due to the seldom-seen nature of this histomorphological feature, there is no definite consensus regarding its etiopathogenesis and clinical consequences, and there is an extreme scarcity of literature elucidating its clinicopathological spectrum and prognostic significance. Herein, we illustrate an intriguing clinical tale of a 7-year-old male child with posterior fossa ependymoma, central nervous system (CNS) World Health Organization (WHO) grade 3, arising at the right CP angle and masquerading as a vestibular schwannoma, which in itself is a rare presentation, and additionally, exhibiting extensive chondro-osseous metaplasia, which is a very uncommon histomorphological observation. To the best of the authors' knowledge and after a comprehensive literature search, the coexistence of these two rare observations has merely been described once in international literature. This case sheds light on and highlights the importance of keeping ependymoma as a possible differential while coming across CP angle space-occupying lesions. They should be diligently distinguished from schwannomas and other masqueraders that typically occur at this site, as they have diverse management and follow-up protocols, with varying prognostic outcomes for the patients. Moreover, this case also unravels and details the clinicopathological characteristics of a scarcely described feature of chondro-osseous metaplasia in ependymomas.

Supra-tentorial Ependymomas with ZFTA Fusion, YAP1 Fusion, and Astroblastomas, MN1-altered: Characteristic Imaging Features.

PURPOSE: Supratentorial (ST) ependymoma subgroups are defined by two different fusions with different prognoses. Astroblastomas, MN1-altered, have ependymal-like histopathologic features and represent a differential diagnosis in children. We hypothesized that ZFTA-fused ependymoma and YAP1-fused ependymoma on the one hand, and astroblastoma, MN1-altered, on the other hand, show different MRI characteristics. METHODS: We retrospectively analyzed the preoperative imaging of 45 patients with ST ependymoma or astroblastoma between January 2000 and September 2020, blinded to histomolecular grouping. Several characteristics, such as location, tumor volume, calcifications, solid/cystic component, and signal enhancement or diffusion were evaluated. We compared imaging characteristics according to their molecular subtype (ZFTA-fused, YAP1-fused, and astroblastoma, MN1-altered). RESULTS: Thirty-nine patients were classified as having an ependymoma, 35 with a ZFTA fusion and four with a YAP1 fusion, and six as having an astroblastoma, MN1-altered. YAP1-fused ependymomas were more likely to involve at least 3 lobes than ZFTA-fused ependymomas. Astroblastomas were located in the frontal lobe in 100% of the tumors versus 49% of the ependymomas. Cerebral blood flow by arterial spin labeling was higher in astroblastomas than in ependymomas. There were no differences in the other characteristics between the molecular groups. All the tumors showed common features: intra-axial extra-ventricular tumors, very frequent contrast enhancement (39/43, 91%), a cystic/necrotic component (41/45, 91%), restricted diffusion (32/36, 89%), calcifications (15/18, 83%), and peri-tumoral edema (38/44, 86%). CONCLUSION: The distinction between ST ependymoma subtypes and astroblastomas can be guided by several imaging features. These tumors share common imaging features that may help to differentiate ST ependymomas and astroblastomas from other pediatric ST tumors.

Intracranial ependymomas in pediatric patients: patterns of care, disparities, and survival outcomes from the National Cancer Database.

OBJECTIVE: This study aimed to extract and analyze comprehensive data from the National Cancer Database (NCDB) to gain insights into the epidemiological prevalence, treatment patterns, and survival outcomes associated with intracranial ependymomas in pediatric patients. METHODS: The authors examined data extracted from the NCDB spanning the years 2010 to 2017, with a specific emphasis on intracranial ependymomas in individuals aged 0-21 years. The study used logistic and Poisson regression, along with Kaplan-Meier survival estimates and Cox proportional hazards models, for analysis. RESULTS: Among 908 included pediatric patients, 495 (54.5%) were male, and 702 (80.6%) were White. Kaplan-Meier analysis determined overall survival (OS) rates of 97.1% (95% CI 96%-98.2%) at 1 year postdiagnosis, 89% (95% CI 86.9%-91.1%) at 3 years, 82.9% (95% CI 80.3%-85.7%) at 5 years, and 74.5% (95% CI 69.8%-79.4%) at 10 years. Grade 3 tumors predicted a more than fourfold higher mortality hazard (p < 0.001; reference = grade 2). Infratentorial localization was also associated with a 1.7-fold increase in mortality hazard (p = 0.002; reference = supratentorial). Larger maximum tumor size (> 5 cm) correlated with a lower mortality hazard (HR 0.64, p = 0.011; reference ≤ 5 cm). The vast majority of patients (85.9%, n = 780) underwent resection. Uninsured patients had over fourfold higher prolonged length of stay (LOS) odds than those privately insured (OR 4.645, p = 0.007). Radiotherapy was received by 76.1% of patients, and the highest rates of radiotherapy occurred among children aged 5-12 years (p < 0.001). Only 25.6% received chemotherapy at any point during their treatment. Peak chemotherapy use emerged within ages 0-4 years, reaching 33.6% in this age group. Kaplan-Meier analysis indicated chemotherapy was associated with significantly worse OS (p = 0.041). CONCLUSIONS: This comprehensive analysis of the NCDB provides valuable insights into the epidemiology, treatment patterns, and survival outcomes of intracranial ependymomas in pediatric patients. Higher tumor grade, infratentorial localization, and chemotherapy use was associated with worse OS, while larger tumor size correlated with lower mortality hazard. Disparities in care were identified, with uninsured patients experiencing prolonged LOS. These findings underscore the need for tailored treatment strategies based on patient and tumor characteristics and highlight the importance of addressing socioeconomic barriers to optimize outcomes for children with ependymomas.

Imaging of supratentorial ependymomas with radio-pathological correlation.

Aim: Supratentorial ependymoma (STE) is a rare tumor with distinct genetic alterations, whose imaging features have been scarcely studied. This study aims to review the computed tomography (CT) and magnetic resonance imaging (MRI) features of a cohort of histopathologically proven STE to identify the distinguishing features of STE, and look for specific signs of zinc finger translocation associated (ZFTA) fused STEs. Methods: Ethical clearance was obtained from the institutional ethics committee. The magnetic resonance (MR) images, CT images when available, clinical details, and pathological reports of 25 patients from a single institute with histopathologically proven STE were retrospectively reviewed. Imaging features, demographic details, pathological and molecular features, and type of surgical resection were described and tabulated. Relevant associations with imaging features were computed and tabulated. Results: The study showed that STEs are common in the pediatric population with no sex predilection. The periventricular location was the most common. A significant association between periventricular location and the presence of a cystic component (P value = 0.023) and the presence of the periwinkle sign/stellate sign (P value = 0.045) was found. Common features of ZFTA fused STEs included periventricular or intraventricular location, cystic component, necrosis, and the periwinkle sign. A significant association was found between ZFTA fusion and cystic component (P value = 0.048). Conclusions: This study attempts to identify the imaging features of STEs and their associations with molecular pathology and surgical outcome, and the distinguishing features of ZFTA fused STEs.