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Brain Pathology (Zurich, Switzerland) Jul 2022Ependymal neoplasms occur at all ages and encompass multiple tumor types and subtypes that develop in the supratentorial compartment, the posterior fossa, or the spinal... (Review)
Review
Ependymal neoplasms occur at all ages and encompass multiple tumor types and subtypes that develop in the supratentorial compartment, the posterior fossa, or the spinal cord. Clinically, ependymomas represent a very heterogeneous group of tumors from rather benign subependymomas to very aggressive and often deadly childhood ependymomas of the posterior fossa. Newly identified biological markers and classification schemes, e. g. based on global DNA methylation profiling, have led to the definition of 10 types of ependymal tumors and an improved prediction of patients' outcome by applying the new classification system. While the exact genetic basis for several ependymoma types still remains unclear, the knowledge about ependymoma driving events has significantly increased within the last decade and contributed to a classification based on molecular characteristics and localization rather than histological features alone. Convincing evidence is now pointing towards gene fusions involving ZFTA or YAP1 causing the development of supratentorial ependymomas. Also, H3, EZHIP, or TERT mutations have been detected in a fraction of infratentorial ependymal tumors. Finally, MYCN amplifications have recently been identified in spinal ependymomas, in addition to the previously known mutations in NF2. This review summarizes how recent findings regarding biology, molecular tumor typing, and clinical outcome have impacted the classification of ependymomas as suggested by the updated 2021 WHO CNS tumor classification system. We focus on changes compared to the previous classification of 2016 and discuss how a formal grading could evolve in the future and guide clinicians to treat ependymoma patients.
Topics: Brain Neoplasms; Child; Ependymoma; Humans; Infratentorial Neoplasms; Supratentorial Neoplasms; World Health Organization
PubMed: 35307892
DOI: 10.1111/bpa.13068 -
Journal of Clinical Oncology : Official... Apr 2019The Children's Oncology Group trial ACNS0121 estimated event-free survival (EFS) and overall survival for children with intracranial ependymoma treated with surgery,... (Clinical Trial)
Clinical Trial
Conformal Radiation Therapy for Pediatric Ependymoma, Chemotherapy for Incompletely Resected Ependymoma, and Observation for Completely Resected, Supratentorial Ependymoma.
PURPOSE
The Children's Oncology Group trial ACNS0121 estimated event-free survival (EFS) and overall survival for children with intracranial ependymoma treated with surgery, radiation therapy, and-selectively-with chemotherapy. Treatment was administered according to tumor location, histologic grade, and extent of resection. The impacts of histologic grade, focal copy number gain on chromosome 1q, and DNA methylation profiles were studied for those undergoing surgery and immediate postoperative conformal radiation therapy (CRT).
METHODS
ACNS0121 included 356 newly diagnosed patients (ages 1 to 21 years). Patients with classic supratentorial ependymoma were observed after gross total resection (GTR). Those undergoing subtotal resection received chemotherapy, second surgery, and CRT. The remaining patients received immediate postoperative CRT after near-total resection or GTR. CRT was administered with a 1.0-cm clinical target volume margin. The cumulative total dose was 59.4 Gy, except for patients who underwent GTR and were younger than age 18 months (who received 54 Gy). Patients were enrolled between October 2003 and September 2007 and were observed for 5 years. Supratentorial tumors were evaluated for fusion; infratentorial tumors, for chromosome 1q gain. Classification of posterior fossa groups A and B was made by methylation profiles.
RESULTS
The 5-year EFS rates were 61.4% (95% CI, 34.5% to 89.6%), 37.2% (95% CI, 24.8% to 49.6%), and 68.5% (95% CI, 62.8% to 74.2%) for observation, subtotal resection, and near-total resection/GTR groups given immediate postoperative CRT, respectively. The 5-year EFS rates differed significantly by tumor grade ( = .0044) but not by age, location, fusion status, or posterior fossa A/posterior fossa B grouping. EFS was higher for patients with infratentorial tumors without 1q gain than with 1q gain (82.8% [95% CI, 74.4% to 91.2%] 47.4% [95% CI, 26.0% to 68.8%]; = .0013).
CONCLUSION
The EFS for patients with ependymoma younger than 3 years of age who received immediate postoperative CRT and for older patients is similar. Irradiation should remain the mainstay of care for most subtypes.
Topics: Adolescent; Adult; Antineoplastic Combined Chemotherapy Protocols; Chemoradiotherapy; Child; Child, Preschool; Cytoreduction Surgical Procedures; Ependymoma; Female; Humans; Infant; Male; Progression-Free Survival; Radiotherapy, Conformal; Supratentorial Neoplasms; Transcription Factor RelA; Treatment Outcome; Young Adult
PubMed: 30811284
DOI: 10.1200/JCO.18.01765 -
No Shinkei Geka. Neurological Surgery Jan 2022Herein we discuss precision medicine for ependymoma. We reviewed the new molecular classifications of ependymoma, studies on the molecular mechanisms involved in... (Review)
Review
Herein we discuss precision medicine for ependymoma. We reviewed the new molecular classifications of ependymoma, studies on the molecular mechanisms involved in carcinogenesis and proliferation, and the various studies exploring new therapeutic strategies. Of the nine molecular classifications of ependymoma, supratentorial ependymomas with fusion, posterior fossa PFA group, and spinal ependymomas with amplification are treatment-resistant, and candidates for precision medicine. Precision medicine is considered to select a treatment method based on molecular biological information, but its application is thought to be difficult for ependymomas with few somatic mutations. Recent studies have shown that epigenetic mechanisms are involved in the development and growth of PFA ependymomas without recurrent somatic mutations. It has been found that forms fusion genes with various genes other than the typical fusion, and a common therapeutic target has been suggested for the genes downstream of it. Unfortunately, these findings have not yet been clinically applied to precision medicine for ependymoma, but newer discoveries are gradually accumulating. Further development of research is warranted.
Topics: Ependymoma; Humans; Precision Medicine; Supratentorial Neoplasms
PubMed: 35169089
DOI: 10.11477/mf.1436204534 -
Radiologie (Heidelberg, Germany) Aug 2023Pediatric brain tumors differ regarding location and histopathological features compared to those in adults. In children, 30% of pediatric brain tumors are... (Review)
Review
BACKGROUND
Pediatric brain tumors differ regarding location and histopathological features compared to those in adults. In children, 30% of pediatric brain tumors are supratentorial lesions. Low-grade astrocytomas, e.g. pilocystic astrocytoma or craniopharyngioma, are the most common tumors.
IMAGING MODALITIES
Magnetic resonance imaging (MRI) is the default imaging technique that is used to evaluate the findings. Ultrasound and cranial computed tomography (CCT) accompany the imaging, although CCT is mainly used in emergency situations.
TOPICS COVERED
The following article describes the most common pediatric supratentorial brain tumors with reference to imaging criteria as well as changes in the World Health Organization (WHO) classification.
Topics: Adult; Child; Humans; Brain Neoplasms; Brain; Supratentorial Neoplasms; Astrocytoma; Pituitary Neoplasms
PubMed: 37306748
DOI: 10.1007/s00117-023-01158-z -
The Neuroradiology Journal Feb 2015Intraventricular schwannoma in either infra or supratentorial location is an extremely rare tumor with less than 20 cases described in the literature to date. There is...
Intraventricular schwannoma in either infra or supratentorial location is an extremely rare tumor with less than 20 cases described in the literature to date. There is no consensus regarding the origin of this tumor. This paper describes an excised supratentorial schwannoma located on the wall of the left lateral ventricle. The relevant literature is reviewed. A 34-year-old man with no significant medical history presented with a recent episode of right focal motor seizures and weakness of the right lower extremity. Magnetic resonance imaging of the brain demonstrated a heterogeneous enhancing mass in the body of left lateral ventricle mass lesion with vasogenic edema in the adjacent brain parenchyma. The patient underwent a left frontoparietal parasagittal craniotomy with neuronavigational guidance to avoid damage to the primary motor cortex. The tumor originated from the ependymal layer and extended to the body of lateral ventricle. Complete surgical excision of the tumor was achieved. Intraventricular schwannomas are rare tumors amenable to complete surgical excision, having a good prognosis without the need for adjuvant therapy. The recognition of this benign and potentially curable neoplasm and its differentiation from other less favorable tumors is of obvious importance.
Topics: Adult; Humans; Lateral Ventricles; Magnetic Resonance Imaging; Male; Neurilemmoma; Supratentorial Neoplasms
PubMed: 25924172
DOI: 10.15274/NRJ-2014-10104 -
Neuroimaging Clinics of North America Feb 2017The breadth of tumors that can arise in the supratentorial brain in children is extensive. With the exception of those that result in seizures and the highly malignant... (Review)
Review
The breadth of tumors that can arise in the supratentorial brain in children is extensive. With the exception of those that result in seizures and the highly malignant histologies, supratentorial tumors may come to medical attention later compared with infratentorial tumors, as they are less commonly associated with ventricular obstruction. This article presents an overview of the neuroimaging characteristics of these entities, with particular attention to relevant features that may aid in narrowing the differential diagnosis, including correlation with demographics and clinical presentation.
Topics: Brain; Child; Diagnostic Imaging; Humans; Neuroimaging; Supratentorial Neoplasms
PubMed: 27889023
DOI: 10.1016/j.nic.2016.08.003 -
Journal of Neurosurgery Jun 2016OBJECT Meningioma is the most common benign intracranial tumor, and patients with supratentorial meningioma frequently suffer from seizures. The rates and predictors of... (Meta-Analysis)
Meta-Analysis Review
OBJECT Meningioma is the most common benign intracranial tumor, and patients with supratentorial meningioma frequently suffer from seizures. The rates and predictors of seizures in patients with meningioma have been significantly under-studied, even in comparison with other brain tumor types. Improved strategies for the prediction, treatment, and prevention of seizures in patients with meningioma is an important goal, because tumor-related epilepsy significantly impacts patient quality of life. METHODS The authors performed a systematic review of PubMed for manuscripts published between January 1980 and September 2014, examining rates of pre- and postoperative seizures in supratentorial meningioma, and evaluating potential predictors of seizures with separate meta-analyses. RESULTS The authors identified 39 observational case series for inclusion in the study, but no controlled trials. Preoperative seizures were observed in 29.2% of 4709 patients with supratentorial meningioma, and were significantly predicted by male sex (OR 1.74, 95% CI 1.30-2.34); an absence of headache (OR 1.77, 95% CI 1.04-3.25); peritumoral edema (OR 7.48, 95% CI 6.13-9.47); and non-skull base location (OR 1.77, 95% CI 1.04-3.25). After surgery, seizure freedom was achieved in 69.3% of 703 patients with preoperative epilepsy, and was more than twice as likely in those without peritumoral edema, although an insufficient number of studies were available for formal meta-analysis of this association. Of 1085 individuals without preoperative epilepsy who underwent resection, new postoperative seizures were seen in 12.3% of patients. No difference in the rate of new postoperative seizures was observed with or without perioperative prophylactic anticonvulsants. CONCLUSIONS Seizures are common in supratentorial meningioma, particularly in tumors associated with brain edema, and seizure freedom is a critical treatment goal. Favorable seizure control can be achieved with resection, but evidence does not support routine use of prophylactic anticonvulsants in patients without seizures. Limitations associated with systematic review and meta-analysis should be considered when interpreting these results.
Topics: Humans; Meningioma; Seizures; Supratentorial Neoplasms
PubMed: 26636386
DOI: 10.3171/2015.4.JNS142742 -
Cancer Discovery Sep 2021Molecular groups of supratentorial ependymomas comprise tumors with or -involving fusions and fusion-negative subependymoma. However, occasionally supratentorial...
Molecular groups of supratentorial ependymomas comprise tumors with or -involving fusions and fusion-negative subependymoma. However, occasionally supratentorial ependymomas cannot be readily assigned to any of these groups due to lack of detection of a typical fusion and/or ambiguous DNA methylation-based classification. An unbiased approach with a cohort of unprecedented size revealed distinct methylation clusters composed of tumors with ependymal but also various other histologic features containing alternative translocations that shared as a partner gene. Somatic overexpression of -associated fusion genes in the developing cerebral cortex is capable of inducing tumor formation , and cross-species comparative analyses identified as a key downstream regulator of tumorigenesis in all tumors. Targeting GLI2 with arsenic trioxide caused extended survival of tumor-bearing animals, indicating a potential therapeutic vulnerability in ZFTA fusion-positive tumors. SIGNIFICANCE: fusions are a hallmark feature of supratentorial ependymoma. We find that ZFTA acts as a partner for alternative transcriptional activators in oncogenic fusions of supratentorial tumors with various histologic characteristics. Establishing representative mouse models, we identify potential therapeutic targets shared by fusion-positive tumors, such as GLI2..
Topics: Animals; Cell Line, Tumor; DNA-Binding Proteins; Disease Models, Animal; Ependymoma; Genomics; Humans; Mice; Proteins; Supratentorial Neoplasms; Transcription Factors
PubMed: 33879448
DOI: 10.1158/2159-8290.CD-20-0963 -
Zhongguo Yi Xue Ke Xue Yuan Xue Bao.... Oct 2021Cerebral metastases are the most common intracranial tumors in adults,with an increasing incidence in recent years.Radiomics can quantitatively analyze and process... (Review)
Review
Cerebral metastases are the most common intracranial tumors in adults,with an increasing incidence in recent years.Radiomics can quantitatively analyze and process medical images to guide clinical practice.In recent years,CT and MRI-based radiomics has been gradually applied to the precise diagnosis and treatment of cerebral metastases,such as the precise detection and segmentation of tumors,the differential diagnosis with other cerebral tumors,the identification of primary tumors,the evaluation of treatment efficacy,and the prediction of prognosis.This article reviews the advances in radiomics of cerebral metastases.
Topics: Brain Neoplasms; Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Prognosis; Supratentorial Neoplasms
PubMed: 34728044
DOI: 10.3881/j.issn.1000-503X.13421 -
Neurosurgical Focus Aug 2023The objective of this review was to describe the immunological changes that take place in the dura mater in response to metastatic disease that seeds the CNS. The... (Review)
Review
OBJECTIVE
The objective of this review was to describe the immunological changes that take place in the dura mater in response to metastatic disease that seeds the CNS. The authors hypothesized that the dura's anatomy and resident immune cell population play a role in enabling metastasis to the brain and leptomeninges.
METHODS
An extensive literature search was conducted to identify evidence that supports the dura's participation in metastasis to the CNS. The authors' hypothesis was informed by a recent upsurge in studies that have investigated the dura's role in metastatic development, CNS infections, and autoimmunity. They reviewed this literature as well as the use of immunotherapy in treating brain metastases and how these therapies change the meningeal immune landscape to overcome and reverse tumor-promoting immunosuppression.
RESULTS
Evidence suggests that the unique architecture and immune cell profile of the dura, compared with other immune compartments within the CNS, facilitate entry of metastatic tumor cells into the brain. Once these tumor cells penetrate the dural barrier, they propagate an immunosuppressive tumor microenvironment. Therefore, immunotherapy may serve to overcome this immunosuppressive environment and liberate proinflammatory immune cells in an effort to combat metastatic disease.
CONCLUSIONS
Within the next few years, the authors expect the addition of several more scientific studies into the literature that further underscore the dura as a chief participant and neuroanatomical barrier in neuro-oncology.
Topics: Humans; Dura Mater; Brain Neoplasms; Brain; Supratentorial Neoplasms; Tumor Microenvironment
PubMed: 37527680
DOI: 10.3171/2023.5.FOCUS23229