Consensus guidelines for the management of primary supra-tentorial intraventricular tumour for low- and middle-income countries.

Almost any primary or metastatic brain tumour can manifest in intraventricular (IV) locations. These tumours may either originate within the ventricular system or extend into the IV space through growth. Such neoplasms represent a broad spectrum, with supratentorial IV tumours forming a heterogeneous group.

Consensus guidelines for the management of intracranial ependymoma for low- and middle-income countries.

This paper presents comprehensive consensus guidelines for the management of intracranial ependymoma, neoplasms arising from ependymal cells in the central nervous system's ventricular system, in low- and middleincome countries (LMICs). Acknowledging the distinct epidemiological patterns of ependymomas, notably their higher incidence in paediatric patients, and variable survival rates, these guidelines emphasize tailored management approaches for different age groups. An expert panel, comprising specialists in neuro-oncology, convened to address gaps in diagnosis and management within LMICs, considering the varying clinical presentation based on tumour size and location.

Anaplastic cortical ependymoma in 10 months girl: A case report.

Introduction And Importance: Ependymomas arise from the ependymal cells that line the brain ventricles, and central canal. In children most of them are benign. However, cortical anaplastic ependymomas are very rare in pediatrics.

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.

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.

Prediction of intraoperative blood loss in pediatric posterior fossa tumors by neuroradiological evaluation: preliminary study.

Background: Hemorrhage management is crucial for surgical resection of pediatric posterior-fossa tumors (PPFTs). Tumor volume and vascularity on preoperative magnetic resonance imaging (MRI) can help predict and control intraoperative blood loss (IBL). The present study aimed to assess the correlation between MRI features and IBL in PPFTs.

Decoding pediatric spinal tumors: a single-center retrospective case series on etiology, presentation, therapeutic strategies, and outcomes.

Introduction: Spinal tumors (ST) often result in dire prognosis, carrying risks such as permanent paralysis, sensory loss, and sphincter dysfunction. Data on their incidence and etiology in pediatric populations are markedly scant. Our study investigates the etiology, clinical manifestation, treatment, and outcomes of pediatric ST.

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.

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.

Concurrent ependymal and ganglionic differentiation in a subset of supratentorial neuroepithelial tumors with EWSR1-PLAGL1 rearrangement.

Neuroepithelial tumors with fusion of PLAGL1 or amplification of PLAGL1/PLAGL2 have recently been described often with ependymoma-like or embryonal histology respectively. To further evaluate emerging entities with PLAG-family genetic alterations, the histologic, molecular, clinical, and imaging features are described for 8 clinical cases encountered at St. Jude (EWSR1-PLAGL1 fusion n = 6; PLAGL1 amplification n = 1; PLAGL2 amplification n = 1).

Dominant Malignant Clones Leverage Lineage Restricted Epigenomic Programs to Drive Ependymoma Development.

Unlabelled: ZFTA-RELA is the most recurrent genetic alteration seen in pediatric supratentorial ependymoma (EPN) and is sufficient to initiate tumors in mice. Despite ZFTA-RELA's potent oncogenic potential, gene fusions are observed exclusively in childhood EPN, with tumors located distinctly in the supratentorial region of the central nervous system (CNS). We hypothesized that specific chromatin modules accessible during brain development would render distinct cell lineage programs at direct risk of transformation by ZFTA-RELA.

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.

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.

The Role of Radiotherapy, Chemotherapy, and Targeted Therapies in Adult Intramedullary Spinal Cord Tumors.

Intramedullary primary spinal cord tumors are rare in adults and their classification has recently evolved. Their treatment most frequently relies on maximal safe surgical resection. Herein, we review, in light of the WHO 2021 classification of central nervous system tumors, the knowledge regarding the role of radiotherapy and systemic treatments in spinal ependymomas, spinal astrocytomas (pilocytic astrocytoma, diffuse astrocytoma, spinal glioblastoma IDH wildtype, diffuse midline glioma H3-K27M altered, and high-grade astrocytoma with piloid features), neuro-glial tumors (ganglioglioma and diffuse leptomeningeal glioneuronal tumor), and hemangioblastomas.

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.

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.

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.