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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 -
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 -
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 -
Asian Pacific Journal of Cancer... 2013Although primary malignant CNS tumors are registered in the national cancer registry (NCR) of Iran, there are no available data on the incidence of the primary malignant... (Review)
Review
BACKGROUND
Although primary malignant CNS tumors are registered in the national cancer registry (NCR) of Iran, there are no available data on the incidence of the primary malignant or benign CNS tumors and their common histopathologies in the country. This study analyzed the 10-year data of the Iranian NCR from March 21, 2000 to March 20, 2010, including a systematic review.
MATERIALS AND METHODS
The international and national scientific databases were searched using the search keywords CNS, tumor, malignancy, brain, spine, neoplasm and Iran.
RESULTS
Of the 1,086 primary results, 9 papers were selected and reviewed, along with analysis of 10-year NCR data. The results showed that primary malignant brain tumors have an overall incidence of 2.74 per 100,000 person-years. The analysis of the papers revealed a benign to malignant ratio of 1.07. The most common histopathologies are meningioma, astrocytoma, glioblastoma and ependymoma. These tumors are more common in men (M/F=1.48). Primary malignant spinal cord tumors constitute 7.1% of the primary malignant CNS tumors with incidence of 0.21/100,000.
CONCLUSIONS
This study shows that CNS tumors in Iran are in compliance with the pattern of CNS tumors in developing countries. The NCR must include benign lesions to understand the definitive epidemiology of primary CNS tumors in Iran.
Topics: Brain Neoplasms; Central Nervous System Neoplasms; Female; Humans; Incidence; Iran; Male
PubMed: 23886218
DOI: 10.7314/apjcp.2013.14.6.3979 -
Cancers Mar 2022Gliomas are primary tumors arising from supporting cells of the central nervous system (CNS), usually in the brain. The 2021 World Health Organization (WHO) classifies... (Review)
Review
THE ISSUE
Gliomas are primary tumors arising from supporting cells of the central nervous system (CNS), usually in the brain. The 2021 World Health Organization (WHO) classifies gliomas as adult-type diffuse gliomas or circumscribed astrocytic gliomas depending on their histology and molecular features. Spinal astrocytic gliomas are very rare, and nowadays no standard of therapy is available. Treatment options are limited: surgery is often not radical, and adjuvant therapies include mostly radiotherapy (RT) or systemic chemotherapy (CHT). There is lack of knowledge about the efficacy and safety of therapies and their multidisciplinary approaches.
THE AIM OF THE REVIEW
A systematic review of the literature from January 2000 to June 2021 was performed, including both clinical trials and observational studies on histological adult primary spinal cord astrocytomas (SCA), with a minimum follow-up of 6 months and reporting the overall survival, progression-free survival or clinical neurological outcome after any therapeutic approach (surgery, RT or CHT). What are the main findings? A total of 1197 citations were identified by the Medline search and additional records; based on our inclusion criteria, 18 studies were included with a total of 285 adult patients. We documented the lack of any clinical trial. What are the conclusions? The available literature data are limited to series/retrospective studies, including heterogeneous patients, i.e., astrocytoma as well as ependymoma or pediatric/adult age, with scanty data on the outcomes of interest. No clinical trials have been run. Due to the rarity of this disease, multicentric clinical trials with molecular investigations are mandatory to better manage such a rare disease.
PubMed: 35267601
DOI: 10.3390/cancers14051292 -
Cancers Nov 2022: Posterior fossa tumors (PFTs) are a morbid group of central nervous system tumors that most often present in childhood. While early diagnosis is critical to drive... (Review)
Review
: Posterior fossa tumors (PFTs) are a morbid group of central nervous system tumors that most often present in childhood. While early diagnosis is critical to drive appropriate treatment, definitive diagnosis is currently only achievable through invasive tissue collection and histopathological analyses. Machine learning has been investigated as an alternative means of diagnosis. In this systematic review and meta-analysis, we evaluated the primary literature to identify all machine learning algorithms developed to classify and diagnose pediatric PFTs using imaging or molecular data. : Of the 433 primary papers identified in PubMed, EMBASE, and Web of Science, 25 ultimately met the inclusion criteria. The included papers were extracted for algorithm architecture, study parameters, performance, strengths, and limitations. : The algorithms exhibited variable performance based on sample size, classifier(s) used, and individual tumor types being investigated. Ependymoma, medulloblastoma, and pilocytic astrocytoma were the most studied tumors with algorithm accuracies ranging from 37.5% to 94.5%. A minority of studies compared the developed algorithm to a trained neuroradiologist, with three imaging-based algorithms yielding superior performance. Common algorithm and study limitations included small sample sizes, uneven representation of individual tumor types, inconsistent performance reporting, and a lack of application in the clinical environment. : Artificial intelligence has the potential to improve the speed and accuracy of diagnosis in this field if the right algorithm is applied to the right scenario. Work is needed to standardize outcome reporting and facilitate additional trials to allow for clinical uptake.
PubMed: 36428701
DOI: 10.3390/cancers14225608 -
World Neurosurgery Jul 2023Staged surgery for skull base lesions has been utilized to facilitate maximal safe resection and optimize outcomes while minimizing morbidity and complications....
BACKGROUND
Staged surgery for skull base lesions has been utilized to facilitate maximal safe resection and optimize outcomes while minimizing morbidity and complications. Conversely, staged surgery for primary intraparenchymal neoplasms is less commonly performed and has not been reported as extensively within the literature. As such, we performed a systematic review to examine the unique surgical indications for staging, timing between stages, specific surgical approaches utilized, and postoperative complications of staged surgery for primary intra-axial neoplasms.
METHODS
A literature search was conducted in August 2021 using PubMed, Web of Science, and Cochrane databases using the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) recommendations. Titles and abstracts were evaluated independently by 2 authors, after which articles were selected for final analysis based on application of strict inclusion criteria during full text screen. Each included article was then qualitatively assessed and relevant variables-including operative approaches, timing, and outcomes-were extracted for synthesis.
RESULTS
Of 115 results, 7 articles were included for final analysis and consisted of 17 pediatric and 4 adult patients. Staged approaches were more commonly utilized in the pediatric patient population for resection of astrocytoma and glioma. Pediatric patients had a timing of surgeries ranging from 5-10 days between operations, compared with 18 days to 4 months in adult patients. Complications in pediatric patients were most commonly hemiparesis, hydrocephalus, cranial nerve VI and VII palsies, truncal ataxia, and cerebellar mutism, while complications in adult patients included language and abstract thinking deficits, respiratory failure, and motor weakness.
CONCLUSIONS
This study reports the first comprehensive review of staged surgical procedures for primary, intra-axial cranial neoplasms. There exists a large degree of heterogeneity in complications resulting from staged surgeries for intra-axial neoplasms, which are similar to complications associated with single-stage surgery for intraparenchymal lesions as well as multi-stage surgeries for skull base lesions.
Topics: Adult; Humans; Child; Skull Base; Glioma; Astrocytoma; Postoperative Complications
PubMed: 36924887
DOI: 10.1016/j.wneu.2023.03.046 -
PloS One 2019Risk of developing a malignancy when born premature is unknown. We hypothesised that risk of certain cancers might be increased in youth born preterm versus term. We... (Meta-Analysis)
Meta-Analysis
INTRODUCTION
Risk of developing a malignancy when born premature is unknown. We hypothesised that risk of certain cancers might be increased in youth born preterm versus term. We therefore performed a systematic review and meta-analysis to evaluate the incidence of malignancy in the context of preterm birth, according to various cancer types.
METHODS
The study was designed per MOOSE and PRISMA guidelines. Articles were identified through November 2015. Observational studies exploring the association between childhood malignancy and birth characteristics were included. Of the 1658 records identified, 109 full text articles were evaluated for eligibility. Random effects meta-analyses were conducted on 10/26 studies retained; 95% confidence intervals were computed and adjusted following sensitivity analysis. Publication bias was evaluated using funnel plots, Begg's and Egger's tests.
RESULTS
No differences in risk of primary central nervous system tumor [OR 1.05; 95% CI 0.93-1.17, 5 studies, 580 cases] and neuroblastoma [OR 1.09; 95% CI 0.90-1.32, 5 studies, 211 cases] were observed in individuals born <37 versus ≥37 weeks' gestation. Preterm birth was consistently associated with hepatoblastoma [ORs 3.12 (95% CI 2.32-4.20), 1.52 (95% CI 1.1-2.1), 1.82 (95% CI 1.01-3.26), and 2.65 (95% CI 1.98-3.55)], but not leukemia, astrocytoma, ependymoma, medulloblastoma, lymphoma, nephroblastoma, rhabdomyosarcoma, retinoblastoma or thyroid cancer.
CONCLUSIONS
Children born premature may be at increased risk for hepatoblastoma but there is no strong evidence of an increased risk of primary central nervous system tumours or neuroblastoma. There is insufficient evidence to conclude whether prematurity modulates the risk of other childhood cancers.
Topics: Central Nervous System Neoplasms; Child; Female; Gestational Age; Hepatoblastoma; Humans; Incidence; Infant, Newborn; Infant, Premature; Liver Neoplasms; Male; Neoplasms; Neuroblastoma; Observational Studies as Topic; Pregnancy; Premature Birth; Risk Factors; Young Adult
PubMed: 30608983
DOI: 10.1371/journal.pone.0210366 -
Global Spine Journal Jun 2021Systematic review.
STUDY DESIGN
Systematic review.
OBJECTIVE
To compare outcomes of complete versus incomplete resection in primary intramedullary spinal cord ependymoma.
METHODS
A comprehensive search of the MEDLINE, CENTRAL, and Embase databases was conducted by 2 independent investigators. Random-effect meta-analysis and meta-regression with seven covariates were performed to evaluate the reason for the heterogeneity among studies. We also used individual patient data in the integrative analysis to compare complete and incomplete resection based on 4 outcomes: progression-free survival (PFS), overall survival (OS), postoperative neurological improvement (PNI), and follow-up neurological improvement (FNI).
RESULTS
A total of 23 studies were identified, including 407 cases. Significant heterogeneity among included studies was observed in risk estimates (I for PFS, FNI, and PNI were 49.5%, 78.3%, and 87.2%, respectively). The mean follow-up time across cases was 48.6 ± 2.35 months. Cox proportional multivariable analysis revealed that the complete resection can prolong PFS (model, hazard ratio = 0.18, CI 0.05-0.54, = .004,) and improve the FNI (binary logistic regression, adjusted odds ratio = 16.5, CI 1.6-171, = .019). However, PNI and OS were similar in patients with incomplete resected spinal cord ependymoma compared with complete resection (binary logistic regression respectively and Cox multivariable analysis, > .5).
CONCLUSION
The data presented in this study showed that OS was not significantly affected by the degree of surgery. However, complete resection of intramedullary ependymomas provides the optimal outcomes with longer PFS and better long-term neurological outcomes than incomplete resection.
PubMed: 32783515
DOI: 10.1177/2192568220939523 -
Journal of Neuro-oncology Mar 2024Central nervous system (CNS) tumours account for around 25% of childhood neoplasms. With multi-modal therapy, 5-year survival is at around 75% in the UK. Conventional... (Review)
Review
BACKGROUND
Central nervous system (CNS) tumours account for around 25% of childhood neoplasms. With multi-modal therapy, 5-year survival is at around 75% in the UK. Conventional photon radiotherapy has made significant contributions to survival, but can be associated with long-term side effects. Proton beam radiotherapy (PBT) reduces the volume of irradiated tissue outside the tumour target volume which may potentially reduce toxicity. Our aim was to assess the effectiveness and safety of PBT and make recommendations for future research for this evolving treatment.
METHODS
A systematic review assessing the effects of PBT for treating CNS tumours in children/young adults was undertaken using methods recommended by Cochrane and reported using PRISMA guidelines. Any study design was included where clinical and toxicity outcomes were reported. Searches were to May 2021, with a narrative synthesis employed.
RESULTS
Thirty-one case series studies involving 1731 patients from 10 PBT centres were included. Eleven studies involved children with medulloblastoma / primitive neuroectodermal tumours (n = 712), five ependymoma (n = 398), four atypical teratoid/rhabdoid tumour (n = 72), six craniopharyngioma (n = 272), three low-grade gliomas (n = 233), one germ cell tumours (n = 22) and one pineoblastoma (n = 22). Clinical outcomes were the most frequently reported with overall survival values ranging from 100 to 28% depending on the tumour type. Endocrine outcomes were the most frequently reported toxicity outcomes with quality of life the least reported.
CONCLUSIONS
This review highlights areas of uncertainty in this research area. A well-defined, well-funded research agenda is needed to best maximise the potential of PBT.
SYSTEMATIC REVIEW REGISTRATION
PROSPERO-CRD42016036802.
Topics: Child; Humans; Young Adult; Proton Therapy; Quality of Life; Central Nervous System Neoplasms; Central Nervous System; Pituitary Neoplasms; Cerebellar Neoplasms
PubMed: 38294638
DOI: 10.1007/s11060-023-04510-4