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World Neurosurgery May 2023Intramedullary spinal cord ependymomas (IMSCEs) are rare tumors that mostly occur in adults. Management strategies and related outcomes are heterogeneously reported... (Review)
Review
BACKGROUND
Intramedullary spinal cord ependymomas (IMSCEs) are rare tumors that mostly occur in adults. Management strategies and related outcomes are heterogeneously reported across the literature, demanding a comprehensive analysis to standardize guidelines. We performed a systematic review of the literature on IMSCEs.
METHODS
A literature search was conducted using 6 databases from inception up to July 28, 2022. Studies with data on clinical characteristics, management strategies, and related outcomes in adult patients with histopathologically confirmed IMSCEs were pooled and analyzed.
RESULTS
The analysis included 69 studies comprising 457 patients (52.7% males). Mean age was 42.4 ± 7.4 years. Sensory deficit (58.0%) was the most prevalent symptom, followed by radicular pain (50.5%). Tumors mostly involved the cervical (64.4%) or thoracic (18.8%) spinal cord and were mostly World Health Organization grade II (80.5%) and classic subtype (72.4%). Gross total resection was performed in most cases (83.4%), with adjuvant radiotherapy delivered in 10.5% of cases. Progression-free survival ≥2 years was reported in 61.1% of cases, and tumor recurrence or progression was reported in only 7.0% of the patients. At last follow-up, 97.4% of patients were alive.
CONCLUSIONS
IMSCEs are uncommon tumors that frequently manifest with debilitating symptoms that require surgical treatment. When feasible, gross total resection may be pursued to improve the patient's functional status and prevent tumor progression, with adjuvant radiotherapy required only in some more aggressive grade III lesions. Future studies should investigate different growth patterns and prognoses based on different IMSCE subtypes.
Topics: Male; Adult; Humans; Middle Aged; Female; Treatment Outcome; Spinal Cord Neoplasms; Prognosis; Neurosurgical Procedures; Ependymoma; Retrospective Studies
PubMed: 36858296
DOI: 10.1016/j.wneu.2023.02.098 -
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 -
JCO Precision Oncology Dec 2022
Topics: Humans; Ependymoma; Repressor Proteins; Kruppel-Like Transcription Factors; RNA-Binding Protein EWS
PubMed: 36480780
DOI: 10.1200/PO.22.00312 -
Journal of Neurosurgery. Pediatrics Feb 2023The aim of this study was to summarize the prognosis of recurrent infratentorial ependymomas based on treatment and molecular characterization. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
The aim of this study was to summarize the prognosis of recurrent infratentorial ependymomas based on treatment and molecular characterization.
METHODS
Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, the authors searched the PubMed, Scopus, Embase, and Ovid databases for studies on recurrent infratentorial ependymomas in patients younger than 25 years of age. Exclusion criteria included case series of fewer than 5 patients and studies that did not provide time-dependent survival data.
RESULTS
The authors' database search yielded 482 unique articles, of which 18 were included in the final analysis. There were 479 recurrent infratentorial pediatric ependymomas reported; 53.4% were WHO grade II and 46.6% were WHO grade III tumors. The overall mortality for recurrent infratentorial pediatric ependymomas was 49.1% (226/460). The pooled mean survival was 30.2 months after recurrence (95% CI 22.4-38.0 months). Gross-total resection (GTR) was achieved in 243 (59.0%) patients at initial presentation. The mean survival postrecurrence for those who received initial GTR was 42.3 months (95% CI 35.7-47.6 months) versus 26.0 months (95% CI 9.6-44.6 months) for those who received subtotal resection (STR) (p = 0.032). There was no difference in the mean survival between patients who received GTR (49.3 months, 95% CI 32.3-66.3 months) versus those who received STR (41.4 months, 95% CI 11.6-71.2 months) for their recurrent tumor (p = 0.610). In the studies that included molecular classification data, there were 169 (83.3%) posterior fossa group A (PFA) tumors and 34 (16.7%) posterior fossa group B (PFB) tumors, with 28 tumors harboring a 1q gain. PFA tumors demonstrated worse mean postprogression patient survival (24.7 months, 95% CI 15.3-34.0 months) compared with PFB tumors (48.0 months, 95% CI 32.8-63.2 months) (p = 0.0073). The average postrecurrence survival for patients with 1q+ tumors was 14.7 months.
CONCLUSIONS
The overall mortality rate for recurrent infratentorial ependymomas was found to be 49.1%, with a pooled mean survival of 30.2 months in the included sample population. More than 80% of recurrent infratentorial ependymomas were of the PFA molecular subtype, and both PFA tumors and those with 1q gain demonstrated worse prognosis after recurrence.
Topics: Child; Humans; Neoplasm Recurrence, Local; Brain Neoplasms; Infratentorial Neoplasms; Prognosis; Ependymoma
PubMed: 36433871
DOI: 10.3171/2022.10.PEDS22154 -
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 Dec 2022Intramedullary spinal cord tumors (IMSCTs) are rare neoplasms and their management involves tumors resection in most cases. Regarding the surgical procedure, adequate... (Review)
Review
BACKGROUND
Intramedullary spinal cord tumors (IMSCTs) are rare neoplasms and their management involves tumors resection in most cases. Regarding the surgical procedure, adequate identification of tumor boundaries is paramount to achieve an extensive tumor resection. Fluorescence image-guided surgery (FIGS) has become an increasing popular intraoperative technique used in spine neuro-oncology surgery. However, evidence is lacking of their usefulness and their safety in spinal tumors. Therefore, the aim of the present study is to give an update of the existing literature and systematically review all studies that focus on the most-used fluorophores (5 aminolevulinic acid [5-ALA], sodium fluorescein, and indocyanine green [ICG]) in IMSCTs.
METHODS
Using PubMed and Scopus, we performed a systematic review in accordance with the PRISMA protocol and screened all original studies involving humans treated for neurosurgical conditions and studies evaluating FIGS application in IMSCTs.
RESULTS
After the screening phase, 27 articles were found to be relevant. The literature results were grouped according to the used fluorophores, resulting in 3 groups: 5-ALA (10 studies); fluorescein (5 studies), and ICG (12 studies).
CONCLUSIONS
In intramedullary tumor surgery, 5-ALA has shown its usefulness in identifying the tumor margins and in searching for residues because of its properties as a tumor-specific metabolic marker. Sodium fluorescein and ICG video angiography have shown promising application in ependymoma and hemangioblastomas surgery, respectively. However, the evolving role of fluorescent dyes in guiding surgical strategies in intramedullary spinal tumor has yet to be shown by randomized clinical trials.
Topics: Humans; Fluorescein; Feasibility Studies; Spinal Cord Neoplasms; Neurosurgical Procedures; Indocyanine Green; Aminolevulinic Acid; Fluorescent Dyes
PubMed: 36195181
DOI: 10.1016/j.wneu.2022.09.117 -
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 -
Frontiers in Oncology 2022Primary intracranial ependymomas (IE) are rare brain tumors rarely metastasizing outside the central nervous system. We systematically reviewed the literature on...
BACKGROUND
Primary intracranial ependymomas (IE) are rare brain tumors rarely metastasizing outside the central nervous system. We systematically reviewed the literature on extra-neural metastases from primary IEs.
METHODS
PubMed, Scopus, Web-of-Science, and Cochrane were searched following the PRISMA guidelines to include studies of extra-neural metastases from primary IEs. Clinical features, management strategies, and survival were analyzed.
RESULTS
We collected 48 patients from 43 studies. Median age was 13 years (range, 2-65). Primary IEs were frequently located in the parietal (22.9%) and frontal (16.7%) lobes, and mostly treated with resection (95.8%) and/or radiotherapy (62.5%). Most IEs were of grade-III (79.1%), and few of grade-I (6.3%) or grade-II (14.6%). 45 patients experienced intracranial recurrences, mostly treated with resection (86.7%), radiotherapy (60%), and/or chemotherapy (24.4%). Median time-interval from primary IEs was 28 months (range, 0-140). Most extra-neural metastases were diagnosed at imaging (37.5%) or autopsy (35.4%). Extra-neural metastases were multifocal in 38 patients (79.1%), mostly involving cervical or hilar lymph-nodes (66.7%), lung/pleura (47.9%), and/or scalp (29.1%). Surgical resection (31.3%), chemotherapy (31.3%) and locoregional radiotherapy (18.8%) were the most common treatments for extra-neural metastases, but 28 (58.3%) patients were not treated. At last follow-up, 37 patients died with median overall-survivals from primary IEs of 36 months (range, 1-239), and from extra-neural metastases of 3 months (range, 0.1-36). Overall-survival was significantly longer in patients with grade-I and II IEs (P=0.040).
CONCLUSION
Extra-neural metastases from primary IEs are rare, but mostly occur at later disease stages. Multidisciplinary management strategies should be intended mostly for palliation.
PubMed: 35574408
DOI: 10.3389/fonc.2022.831016 -
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 -
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