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Rare Tumors 2022Cortical ependymomas are currently not considered a subgroup of supratentorial ependymomas; however, there is a growing body of literature investigating the natural... (Review)
Review
Cortical ependymomas are currently not considered a subgroup of supratentorial ependymomas; however, there is a growing body of literature investigating the natural history of these lesions compared to supratentorial ependymomas. We performed a systematic literature review of cortical ependymomas with a focus on the natural history, clinical characteristics, and clinical outcomes of these lesions as compared to supratentorial ependymomas. Our search revealed 153 unique cases of cortical ependymomas. The mean age on presentation was 21.2 years. Males and females comprised 58.8% (90/153) and 41.2% (63/153) of cases, respectively. The most common presenting symptom was seizure activity occurring in 44.4% of the cohort (68/153). The recently recognized fusion was identified in 13.7% of the cohort (21/153) and 95.5% of cases (21/22) reporting molecular characterization. World Health Organization grades 2 and 3 were reported in 52.3% (79/151) and 47.7% (72/151) of cases, respectively. The frontal lobe was involved in the majority of cases (54.9%, 84/153). Gross total resection was achieved in 80.4% of cases (123/153). Tumor recurrence was identified in 27.7% of cases (39/141). Mean clinical follow-up was 41.3 months. Mean overall survival of patients who expired was 27.4 months whereas mean progression-free survival was 15.0 months. Comparatively, cortical ependymomas with - fusions and supratentorial ependymomas with fusions exhibited differing clinical outcomes. Further studies with larger sample sizes are necessary to investigate the significance of fusions on survival in cortical ependymomas and to determine whether cortical ependymomas with - fusions should be classified as a distinct entity.
PubMed: 35836750
DOI: 10.1177/20363613221112432 -
Journal of Clinical Neuroscience :... May 2019The objective was to determine the impact of surgical resection and adjuvant therapies on survival in intramedullary ependymoma and astrocytoma. Secondary goals were to... (Meta-Analysis)
Meta-Analysis
The objective was to determine the impact of surgical resection and adjuvant therapies on survival in intramedullary ependymoma and astrocytoma. Secondary goals were to determine predictors of survival in surgical patients including histological grading, age and gender. Searching of Medline, Embase and Clinicaltrials.gov databases were performed. Multivariate analyses were performed for overall survival (OS) and progression-free survival (PFS) through Monte Carlo methods and Maximum Likelihood Estimation. 57 articles detail results for 3022 patients. Meta-analysis revealed the following factors to have a statistically significant effect on OS. Patients undergoing gross-total resection (GTR) are 5.37 times more likely to survive than patients with lesser volumes of tumor resected (HR for OS 1.68, p < 0.01). High-grade tumors were associated with a 14 times risk of death over low-grade tumors (HR for OS 2.64, p < 0.01). Radiation increased the risk of mortality in low-grade tumors (HR for OS 5.20, p < 0.01), but decreased mortality in high-grade lesions (HR for OS 2.46, p < 0.01). Adult patients were more likely to die from disease compared with pediatric patients by a factor of 1.6 (HR for OS 0.47, p < 0.01). In PFS, radiotherapy was associated with a reduced time to recurrence (HR for PFS 1.90, p < 0.01). There was a male predominance of 58%. Gender did not influence survival. 79% of patients demonstrated stable or improved functional neurological outcomes six months post-operatively. Our data indicates GTR improves OS in all tumor grades. Radiation improves OS only in the presence of high-grade histology. Advancing age and high-grade histology are negative prognostic indicators.
Topics: Adolescent; Adult; Astrocytoma; Child; Child, Preschool; Combined Modality Therapy; Ependymoma; Female; Humans; Infant; Male; Neoplasm Grading; Neoplasm Recurrence, Local; Postoperative Period; Prognosis; Progression-Free Survival; Spinal Cord Neoplasms
PubMed: 30833131
DOI: 10.1016/j.jocn.2019.02.001 -
Neuro-oncology Practice Mar 2020Anaplastic ependymoma with extraneural metastases is associated with a poor clinical outcome. Metastatic spread to the parotid gland is a rare clinical entity that...
BACKGROUND
Anaplastic ependymoma with extraneural metastases is associated with a poor clinical outcome. Metastatic spread to the parotid gland is a rare clinical entity that requires multidisciplinary intervention. Herein, we present a systematic review of anaplastic ependymoma with extraneural metastases and report on a case with metastases to both parotid glands.
METHODS
Electronic databases were searched from their inception to February 2019. Inclusion criteria included reports of anaplastic ependymoma with extraneural metastasis. Studies were excluded if the tumor grade was not reported. A case illustration is provided.
RESULTS
The search yielded 15 cases of anaplastic ependymoma with extraneural metastases, including the present case. Mean age at diagnosis was 15 years. The initial tumor location was predominantly supratentorial (93.3%). All cases demonstrated leptomeningeal seeding before extraneural metastasis. Mean survival from initial diagnosis was 4.5 years. Metastasis to the parotid gland occurred in 2 cases, including the present case. We present a 17-year-old female patient who underwent gross total resection of a supratentorial, paraventricular anaplastic ependymoma followed by adjuvant external beam radiation therapy. The patient developed recurrent leptomeningeal seeding, treated with Gamma Knife radiosurgery over a 5-year period. She returned with a parotid mass and cervical lymphadenopathy and underwent parotidectomy and modified radical neck dissection. She continued to experience recurrences, including the left parotid gland, and was ultimately placed in hospice care.
CONCLUSIONS
Anaplastic ependymoma with extraneural metastasis is rare. A combination of repeated surgical resection, radiation therapy, and chemotherapy can be used to manage recurrent and metastatic disease, but outcomes remain poor.
PubMed: 32626590
DOI: 10.1093/nop/npz041 -
World Neurosurgery Sep 2019Spinal myxopapillary ependymoma (sMPE) is an uncommon primary spinal neoplasm infiltrating the spinal cord, conus medullaris (CM), and nerve roots. It is associated with...
BACKGROUND
Spinal myxopapillary ependymoma (sMPE) is an uncommon primary spinal neoplasm infiltrating the spinal cord, conus medullaris (CM), and nerve roots. It is associated with low resection and high recurrence rates. The purpose of this literature review is to evaluate the exact impact of the involvement of the CM and the role played by gross total resection (GTR) on overall survival (OS).
METHODS
The English literature was systematically investigated using MEDLINE, the NIH Library, PubMed, and Google Scholar search engines with relevant queries. Case series reporting details concerning OS, GTR, and CM involvement rate were included, with a differential statistical weight given by the number of patients enrolled. A final cohort of 1602 clinical records was analyzed according to the 3 selected end point variables.
RESULTS
The average age was 36.44 ± 3.41 years, and the CM was involved in 28.4% ± 28.2% of cases. The average GTR rate was 53.94% ± 22.20%. Five- and 10-year OS rates were respectively available in 1170 and 1167 cases, with an average 5- and 10-year OS rate of 94.99% ± 3.87% and 92.31% ± 5.73%. By means of analyses performed both on aggregated and disaggregated data a strong positive statistical connection between GTR and increased OS was demonstrated despite the real clinical advantage could range as low as around 1% of increased OS rate.
CONCLUSIONS
Given the indolent sMPE behavior, it is difficult to evaluate the exact impact of GTR and CM involvement on OS; however, GTR could be associated with a limited survival advantage, whereas CM involvement could be associated with a survival disadvantage.
Topics: Adult; Ependymoma; Female; Humans; Male; Spinal Cord; Spinal Cord Neoplasms
PubMed: 31152881
DOI: 10.1016/j.wneu.2019.05.206 -
Primary spinal anaplastic ependymoma: A single-institute retrospective cohort and systematic review.Frontiers in Oncology 2023Primary spinal anaplastic ependymoma (PSAE) is an extremely rare disease. We aim to report the largest PSAE cohort, evaluate the treatments, and investigate the...
OBJECTIVE
Primary spinal anaplastic ependymoma (PSAE) is an extremely rare disease. We aim to report the largest PSAE cohort, evaluate the treatments, and investigate the prognostic factors for progression-free survival (PFS).
METHODS
Clinical data collected from the authors' institute and literature articles were pooled and described. Survival analysis and multivariable Cox regression analysis were performed to evaluate therapies and investigate prognostic factors for PFS.
RESULTS
Our cohort included 22 females and 16 males, with a median age of 33 years. PSAE developed mostly on cervical and cervicothoracic levels. The median length measured 3 segments. Half of PSAE were intramedullary. Pain was the most common symptom. The median duration of symptoms was 6 months. Neurological statuses were improved in 76% following treatments, whereas clinical tumor progression occurred in 41.7%. The estimated median progression-free survival was 132 months, and the estimated median survival was 192 months. The median Ki-67 index was 15%. Patients aged less than or equal to 25 experienced worse neurological statuses and more repeated progression. Age less than or equal to 25 (HR 10.312, 95%CI 1.535-69.260, p=0.016), gross total resection (HR 0.116, 95%CI 0.020-0.688, p=0.018), and radiotherapy (HR 0.084, 95%CI 0.009-0.804, p=0.032) are three prognostic factors for tumor progression.
CONCLUSION
Tumor progression remains a big concern in the clinical course of PSAE. Being aged above 25, undergoing GTR, and accepting adjuvant radiotherapy put patients at lower risk for tumor progression. Younger patients might have worse neurological statuses compared with those aged over 25.
PubMed: 36824145
DOI: 10.3389/fonc.2023.1083085 -
Anticancer Research Oct 2016Historically, radiation oncologists have been cautious about re-irradiating brain tumors because of concerns about the risks of late central nervous system (CNS)... (Review)
Review
BACKGROUND
Historically, radiation oncologists have been cautious about re-irradiating brain tumors because of concerns about the risks of late central nervous system (CNS) toxicity, especially radionecrosis, that may occur several months to years following treatment. Today there are still limited prospective data addressing this approach.
MATERIALS AND METHODS
Systematic review of published trials reporting clinical results after re-irradiation of patients with different types of brain tumors was performed.
RESULTS
Data mainly related to glioblastoma, anaplastic glioma, medulloblastoma, ependymoma and meningioma have been published. Randomized studies are scarce. As in first-line scenarios, efficacy of radiotherapy is influenced by histology. Based on the reported outcomes, preliminary recommendations for dose/fractionation regimens can be given.
CONCLUSION
Re-irradiation of brain tumors is increasingly considered as our understanding of brain tolerance to radiation evolves and developments in radiation technology and imaging make highly accurate targeting of recurrent tumors possible. With developments in systemic therapy, further exploration of the role of re-irradiation on its own or in combination with novel agents is needed.
Topics: Animals; Brachytherapy; Brain Neoplasms; Central Nervous System; Combined Modality Therapy; Glioma; Humans; Meningioma; Neoplasm Recurrence, Local; Radiation Injuries; Re-Irradiation
PubMed: 27798857
DOI: 10.21873/anticanres.11067 -
Paediatric Drugs 2003Childhood intracranial ependymoma have a dismal prognosis, especially in young children and when a gross total resection cannot be performed. Even in the absence of a... (Review)
Review
Childhood intracranial ependymoma have a dismal prognosis, especially in young children and when a gross total resection cannot be performed. Even in the absence of a radiologically proven residuum, around two-thirds of these young children will have a recurrence. Adjuvant therapy is therefore necessary for most, if not all, patients. Despite some indication that benign ependymoma (WHO grade II) could show a better outcome, histology cannot be used at present to stratify treatment protocols.Craniospinal irradiation combined with posterior fossa boost has deleterious adverse effects on cognition. Consequently, pediatric oncology teams have, firstly, tried to use chemotherapy to delay or avoid irradiation, and secondly, progressively reduced irradiation fields to the tumor bed without altering the prognosis. Cisplatin, at a dose of 120 mg/m(2) (cumulated response rate of 34% [95% CI 19-54%]) is the only single agent that has reproducibly shown some efficacy in ependymoma. Despite some combinations showing efficacy in the adjuvant setting, childhood intracranial ependymomas can, in general, be considered as chemoresistant. The overexpression of the multidrug resistance-1 gene and the 06-methylguanine-DNA methyltransferase have been implicated as possible mechanisms for this phenomenon. As the use of chemotherapy with current agents is questionable, phase II studies with new agents and combinations are necessary. Since the main problem of this disease is local relapse, it may not be necessary to irradiate the whole posterior fossa. However, local control of the disease by irradiation has to be improved. In this respect, hyperfractionation or radiosensitizers may be valuable therapeutic options. The treatment of children with ependymoma is a challenge for all caregivers. There is no doubt that any possible improvement in the management of this rare tumor will only be the result of well designed cooperative trials.
Topics: Adolescent; Antineoplastic Agents; Brain Neoplasms; Chemotherapy, Adjuvant; Child; Child, Preschool; Drug Resistance, Neoplasm; Drug Therapy, Combination; Ependymoma; Humans; Infant; Infant, Newborn; Time Factors
PubMed: 12895136
DOI: 10.2165/00148581-200305080-00004 -
Journal of Neuro-oncology Sep 2019The purpose of this study was to determine outcomes in recurrent pediatric ependymoma. (Meta-Analysis)
Meta-Analysis
PURPOSE
The purpose of this study was to determine outcomes in recurrent pediatric ependymoma.
METHODS
We performed a systematic review of PubMed, Embase, Web of Science and the Cochrane Library for studies reporting on survival outcomes for pediatric patients with recurrent ependymoma. We then performed a meta-analysis of all eligible results. Survival outcomes were identified across location of recurrence, therapy at recurrence, and age at recurrence.
RESULTS
Eleven studies met final inclusion criteria. Pooled median progression free survival (PFS) from date of first recurrence was 6.7 months (95% confidence interval [95% CI] 4.7-8.8). Pooled median overall survival (OS) from date of first recurrence was 11.2 months (95% CI 6.4-16.0). Participants with supratentorial recurrences demonstrated a shorter OS of 8.3 months (95% CI 3.2-13.3) compared to 20.1 months (95% CI 8.4-31.7) for those with infratentorial recurrence. Patients who underwent surgery at recurrence had a median OS of 24.2 months (95% CI 14.2-34.1) compared to 29.2 months (95% CI 17.4-41.1) in those who received radiation compared to 19.3 months (95% CI 10.3-28.3) in those who received chemotherapy. Patients younger than age 3 years at time of recurrence demonstrated a median OS of 31.0 months (95% CI - 25.3-87.3) compared to 17.5 months (95% CI 9.9-25.2) for those that recurred beyond 3 years of age.
CONCLUSIONS
Our findings illustrate that children with recurrent ependymoma suffer from poor outcomes; however, these outcomes range widely depending on patient, tumor, and treatment characteristics. New therapies and treatment strategies are needed to improve outcomes in this group.
Topics: Child; Combined Modality Therapy; Ependymoma; Humans; Neoplasm Recurrence, Local; Survival Rate; Treatment Outcome
PubMed: 31502040
DOI: 10.1007/s11060-019-03255-3 -
Journal of Neurosurgery Mar 2022The tumor characteristics and surgical outcomes of intracranial subependymomas are poorly defined. In this study the authors aimed to provide a comprehensive review of... (Meta-Analysis)
Meta-Analysis Review
OBJECTIVE
The tumor characteristics and surgical outcomes of intracranial subependymomas are poorly defined. In this study the authors aimed to provide a comprehensive review of all clinical, pathological, radiological, and surgical aspects of this important neoplasm to inform future management strategies.
METHODS
A systematic review and meta-analysis of MEDLINE, EMBASE, Cochrane, and Google Scholar databases adherent to PRISMA guidelines was conducted.
RESULTS
Of the 1145 articles initially retrieved, 24 studies encompassing 890 cases were included. The authors identified 3 retrospective cohort studies and 21 case series, but no controlled trials. Mean age at presentation was 46.7 ± 18.1 years with a male predominance (70.2%). Common sites of tumor origin were the lateral ventricle (44.5%) and fourth ventricle (43.1%). Cumulative postoperative mortality and morbidity rates were 3.4% and 24.3% respectively. Meta-analysis revealed that male sex (HR 3.15, 95% CI 1.39-7.14, p = 0.006) was associated with poorer 5-year overall mortality rates. All-cause mortality rates were similar when performing subgroup meta-analyses for age (HR 0.50, 95% CI 0.03-7.36, p = 0.61), smaller subependymoma size (HR 1.51, 95% CI 0.78-2.92, p = 0.22), gross-total resection (HR 0.65, 95% CI 0.35-1.23, p = 0.18), and receipt of postoperative radiation therapy (HR 0.88, 95% CI 0.27-2.88, p = 0.84). Postoperative Karnofsky Performance Index scores improved by a mean difference of 1.62 ± 12.14 points (p = 0.42). The pooled overall 5-year survival rate was 89.2%, while the cumulative recurrence rate was 1.3% over a median follow-up ranging from 15.3 to 120.0 months. The pure subependymoma histopathological subtype was most prevalent (85.6%), followed by the mixed subependymoma-ependymoma tumor variant (13.7%).
CONCLUSIONS
Surgical extirpation without postoperative radiotherapy results in excellent postoperative survival and functional outcomes in the treatment of intracranial subependymomas. Aggressive tumor behavior should prompt histological reevaluation for a mixed subependymoma-ependymoma subtype. Further high-quality controlled trials are still required to investigate this rare tumor.
Topics: Female; Glioma, Subependymal; Humans; Lateral Ventricles; Male; Retrospective Studies; Survival Rate; Treatment Outcome
PubMed: 34416731
DOI: 10.3171/2021.2.JNS204052 -
Journal of Magnetic Resonance Imaging :... Jul 2022Medulloblastoma, ependymoma, and pilocytic astrocytoma are common pediatric posterior fossa tumors. These tumors show overlapping characteristics on conventional MRI... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Medulloblastoma, ependymoma, and pilocytic astrocytoma are common pediatric posterior fossa tumors. These tumors show overlapping characteristics on conventional MRI scans, making diagnosis difficult.
PURPOSE
To investigate whether apparent diffusion coefficient (ADC) values differ between tumor types and to identify optimum cut-off values to accurately classify the tumors using different performance metrics.
STUDY TYPE
Systematic review and meta-analysis.
SUBJECTS
Seven studies reporting ADC in pediatric posterior fossa tumors (115 medulloblastoma, 68 ependymoma, and 86 pilocytic astrocytoma) were included following PubMed and ScienceDirect searches.
SEQUENCE AND FIELD STRENGTH
Diffusion weighted imaging (DWI) was performed on 1.5 and 3 T across multiple institution and vendors.
ASSESSMENT
The combined mean and standard deviation of ADC were calculated for each tumor type using a random-effects model, and the effect size was calculated using Hedge's g.
STATISTICAL TESTS
Sensitivity/specificity, weighted classification accuracy, balanced classification accuracy. A P value < 0.05 was considered statistically significant, and a Hedge's g value of >1.2 was considered to represent a large difference.
RESULTS
The mean (± standard deviation) ADCs of medulloblastoma, ependymoma, and pilocytic astrocytoma were 0.76 ± 0.16, 1.10 ± 0.10, and 1.49 ± 0.16 mm /sec × 10 . To maximize sensitivity and specificity using the mean ADC, the cut-off was found to be 0.96 mm /sec × 10 for medulloblastoma and ependymoma and 1.26 mm /sec × 10 for ependymoma and pilocytic astrocytoma. The meta-analysis showed significantly different ADC distributions for the three posterior fossa tumors. The cut-off values changed markedly (up to 7%) based on the performance metric used and the prevalence of the tumor types.
DATA CONCLUSION
There were significant differences in ADC between tumor types. However, it should be noted that only summary statistics from each study were analyzed and there were differences in how regions of interest were defined between studies.
EVIDENCE LEVEL
1 TECHNICAL EFFICACY: Stage 3.
Topics: Astrocytoma; Cerebellar Neoplasms; Child; Diagnosis, Differential; Diffusion Magnetic Resonance Imaging; Ependymoma; Humans; Infratentorial Neoplasms; Medulloblastoma; Retrospective Studies
PubMed: 34842328
DOI: 10.1002/jmri.28007