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BMJ Case Reports Jan 2021This report presents the longest spanning intradural myxopapillary ependymoma consisting of 23 vertebral segments in the literature. An 11-year-old boy presented with... (Review)
Review
This report presents the longest spanning intradural myxopapillary ependymoma consisting of 23 vertebral segments in the literature. An 11-year-old boy presented with right arm pain, mid back pain and progressive paraparesis associated with urinary retention. On MRI, the patient was found to have an intradural lesion extending from C5 to S3. The patient underwent T7 and T8 laminectomies with an almost total resection except for a minimal residual adhering to the spinal cord. The patient with the largest spanning spinal cord ependymoma was managed satisfactorily without significant morbidity. A small laminectomy may be used in some occasions despite the tumour's extensive size because it may have a single point of attachment to the cord.
Topics: Cervical Vertebrae; Child; Ependymoma; Humans; Laminectomy; Lumbar Vertebrae; Magnetic Resonance Imaging; Male; Radiotherapy, Adjuvant; Sacrum; Spinal Cord Neoplasms; Thoracic Vertebrae; Tumor Burden
PubMed: 33509888
DOI: 10.1136/bcr-2020-239453 -
Neoplasia (New York, N.Y.) May 2023Pediatric intracranial ependymoma has seen a recent exponential expansion of biological findings, rapidly dividing the diagnosis into several subgroups, each with...
Pediatric intracranial ependymoma has seen a recent exponential expansion of biological findings, rapidly dividing the diagnosis into several subgroups, each with specific molecular and clinical characteristics. While such subdivision may complicate clinical conclusions from historical trials, this knowledge also provides an opportunity for interrogating the major clinical and biological questions preventing near-term translation into effective therapy for children with ependymoma. In this article, we briefly review some of the most critical clinical questions facing both patient management and the construct of future trials in childhood ependymoma, as well as explore some of the current barriers to efficient translation of preclinical discovery to the clinic.
Topics: Child; Humans; Brain Neoplasms; Ependymoma; Prognosis
PubMed: 36944298
DOI: 10.1016/j.neo.2023.100895 -
Neuro-oncology Oct 2022Myxopapillary ependymoma (MPE) is a heterogeneous disease regarding histopathology and outcome. The underlying molecular biology is poorly understood, and markers that...
BACKGROUND
Myxopapillary ependymoma (MPE) is a heterogeneous disease regarding histopathology and outcome. The underlying molecular biology is poorly understood, and markers that reliably predict the patients' clinical course are unknown.
METHODS
We assembled a cohort of 185 tumors classified as MPE based on DNA methylation. Methylation patterns, copy number profiles, and MGMT promoter methylation were analyzed for all tumors, 106 tumors were evaluated histomorphologically, and RNA sequencing was performed for 37 cases. Based on methylation profiling, we defined two subtypes MPE-A and MPE-B, and explored associations with epidemiological, clinical, pathological, and molecular characteristics of these tumors.
RESULTS
MPE-A occurred at a median age of 27 years and were enriched with tumors demonstrating papillary morphology and MGMT promoter hypermethylation. Half of these tumors could not be totally resected, and 85% relapsed within 10 years. Copy number alterations were more common in MPE-A. RNA sequencing revealed an enrichment for extracellular matrix and immune system-related signatures in MPE-A. MPE-B occurred at a median age of 45 years and included many tumors with a histological diagnosis of WHO grade II and tanycytic morphology. Patients within this subtype had a significantly better outcome with a relapse rate of 33% in 10 years (P = 3.4e-06).
CONCLUSIONS
We unraveled the morphological and clinical heterogeneity of MPE by identifying two molecularly distinct subtypes. These subtypes significantly differed in progression-free survival and will likely need different protocols for surveillance and treatment.
Topics: Adult; Cohort Studies; DNA Methylation; Ependymoma; Humans; Middle Aged; Recurrence; Spinal Cord Neoplasms
PubMed: 35380708
DOI: 10.1093/neuonc/noac088 -
Journal of Clinical Neuroscience :... Dec 2014Intracranial ependymomas are rare tumors in adults. Thus, factors affecting prognosis are poorly understood. We performed a study to investigate whether tumor location... (Review)
Review
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 3.38 cm, 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.
Topics: Adolescent; Adult; Aged; Aged, 80 and over; Brain Neoplasms; Disease-Free Survival; Ependymoma; Female; Humans; Male; Meta-Analysis as Topic; Middle Aged; Prognosis; Proportional Hazards Models; Supratentorial Neoplasms; Young Adult
PubMed: 25037313
DOI: 10.1016/j.jocn.2014.05.011 -
Spinal Cord Series and Cases Apr 2022Spinal myxopapillary ependymomas (SME) are rare WHO grade II neoplasms of the spinal cord. Despite their good prognosis, they have a high propensity for metastasis and... (Review)
Review
INTRODUCTION
Spinal myxopapillary ependymomas (SME) are rare WHO grade II neoplasms of the spinal cord. Despite their good prognosis, they have a high propensity for metastasis and recurrence, although the presentation of SME as multifocal is uncommon.
CASE PRESENTATION
Here we describe a rare case of a 34-year-old man who presented with painful bilateral radiculopathy with sexual dysfunction and altered sensation with defecation. The patient also reported worsening weakness of bilateral lower extremities when climbing stairs. Biopsy results revealed multifocal SME in the lumbar and sacral spine that was treated with staged surgical resection and post-operative focal radiation therapy.
DISCUSSION
We discuss and evaluate surgical resection and the role of postoperative radiotherapy for SME. We also review the literature surrounding multifocal SME presenting in adults.
Topics: Adult; Ependymoma; Humans; Magnetic Resonance Imaging; Male; Spinal Cord Neoplasms; Treatment Outcome
PubMed: 35459220
DOI: 10.1038/s41394-022-00513-x -
The Pan African Medical Journal 2021
Topics: Ependymoma; Humans; Magnetic Resonance Imaging; Musculoskeletal System; Spinal Cord Neoplasms
PubMed: 34539960
DOI: 10.11604/pamj.2021.39.164.29765 -
Acta Neuropathologica Jan 2017Multiple independent genomic profiling efforts have recently identified clinically and molecularly distinct subgroups of ependymoma arising from all three anatomic...
Multiple independent genomic profiling efforts have recently identified clinically and molecularly distinct subgroups of ependymoma arising from all three anatomic compartments of the central nervous system (supratentorial brain, posterior fossa, and spinal cord). These advances motivated a consensus meeting to discuss: (1) the utility of current histologic grading criteria, (2) the integration of molecular-based stratification schemes in future clinical trials for patients with ependymoma and (3) current therapy in the context of molecular subgroups. Discussion at the meeting generated a series of consensus statements and recommendations from the attendees, which comment on the prognostic evaluation and treatment decisions of patients with intracranial ependymoma (WHO Grade II/III) based on the knowledge of its molecular subgroups. The major consensus among attendees was reached that treatment decisions for ependymoma (outside of clinical trials) should not be based on grading (II vs III). Supratentorial and posterior fossa ependymomas are distinct diseases, although the impact on therapy is still evolving. Molecular subgrouping should be part of all clinical trials henceforth.
Topics: Animals; Brain Neoplasms; Consensus; Disease Management; Ependymoma; Humans; Neoplasm Staging
PubMed: 27858204
DOI: 10.1007/s00401-016-1643-0 -
CNS Oncology May 2013Pediatric intracranial ependymoma is a rare disease representing approximately 7% of brain tumors in children aged 15 years or younger. Due to the relative rarity of... (Review)
Review
Pediatric intracranial ependymoma is a rare disease representing approximately 7% of brain tumors in children aged 15 years or younger. Due to the relative rarity of these tumors, a clear standard therapy has been difficult to establish. The mainstay of treatment is surgical resection and the majority of data demonstrate improved outcome with gross total resection. The standard of care also includes postoperative radiation therapy for most patients with grade II and III tumors. Chemotherapy has been used in many capacities in this disease; however, its optimal role is yet to be defined. Current controversies such as treatment with surgery alone in completely resected tumors, use of chemotherapy for subtotally resected tumors and use of adjuvant postradiation chemotherapy are incorporated into the design of the current Children's Oncology Group clinical trial.
Topics: Brain; Brain Neoplasms; Child; Ependymoma; Humans; Neoplasm Recurrence, Local; Radiotherapy Planning, Computer-Assisted
PubMed: 25054465
DOI: 10.2217/cns.13.13 -
Trends in Molecular Medicine Nov 2009Cancer propagating cells (CPCs) within primary central nervous system (CNS) tumors (glioblastoma multiforme (GBM), medulloblastoma (MB) and ependymoma) might be integral... (Review)
Review
Cancer propagating cells (CPCs) within primary central nervous system (CNS) tumors (glioblastoma multiforme (GBM), medulloblastoma (MB) and ependymoma) might be integral to tumor development and perpetuation. These cells, also known as brain cancer propagating cells (BCPCs), have the ability to self-renew and proliferate. BCPCs can initiate new tumors in mice with high efficiency and these exhibit many features that are characteristic of patient's brain tumors. Accumulating evidence suggests that BCPCs might originate from the transformation of neural stem cells (NSCs) and their progenitors. Furthermore, recent studies have shown that NSC surface markers also define BCPCs. Ultimately, treatments that include specific targeting of BCPCs might potentially be more effective at treating the entire tumor mass, translating to improved patient survival and quality of life.
Topics: Animals; Brain Neoplasms; Ependymoma; Glioblastoma; Humans; Mice; Stem Cells
PubMed: 19889578
DOI: 10.1016/j.molmed.2009.09.003 -
Journal of Neurosurgical Sciences Feb 2018Ependymomas of the posterior fossa in adults are relatively rare, represent less than 1% of adult intracranial gliomas. Most of the cases are WHO grade II. Due to their... (Review)
Review
Ependymomas of the posterior fossa in adults are relatively rare, represent less than 1% of adult intracranial gliomas. Most of the cases are WHO grade II. Due to their rarity, there are no randomized studies regarding the best management of these tumors. It seems that surgical resection has a major role in their management. Unlike the scenario in children, these tumors can be cured by gross total resection without adjuvant therapy in the majority of cases. Hence, knowing the different surgical approaches, with their pros and cons, is important in order to offer the patient the best treatment. In this paper, we review the current literature on surgery for ependymomas of the posterior fossa in adults, and the advantages and risks of the principal surgical approaches, namely the telo-velar and the transvermian, are discussed.
Topics: Adult; Ependymoma; Female; Humans; Infratentorial Neoplasms; Male; Neurosurgical Procedures
PubMed: 28945053
DOI: 10.23736/S0390-5616.17.04190-X