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Chinese Clinical Oncology Apr 2024The role of adjuvant radiotherapy (RT) after gross total resection (GTR) of the World Health Organization (WHO) grade II ependymoma is controversial. Therefore, we aimed... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The role of adjuvant radiotherapy (RT) after gross total resection (GTR) of the World Health Organization (WHO) grade II ependymoma is controversial. Therefore, we aimed to compare the outcomes of adjuvant RT against observation after GTR of WHO grade II ependymoma. We also compared the outcomes of adjuvant RT against observation after subtotal resection (STR) of WHO grade II ependymoma and performed further subgroup analysis by age and tumor location.
METHODS
PubMed and Embase were systematically reviewed for studies published up till 25 November 2022. Studies that reported individual-participant data on patients who underwent surgery followed by adjuvant RT/observation for WHO grade II ependymoma were included. The exposure was whether adjuvant RT was administered, and the outcomes were recurrence and overall survival (OS). Subgroup analyses were performed by the extent of resection (GTR or STR), tumor location (supratentorial or infratentorial), and age at the first surgery (<18 or ≥18 years old).
RESULTS
Of the 4,647 studies screened, three studies reporting a total of 37 patients were included in the analysis. Of these 37 patients, 67.6% (25 patients) underwent GTR, and 51.4% (19 patients) underwent adjuvant RT. Adjuvant RT after GTR was not significantly associated with both recurrence (odds ratio =5.50; 95% confidence interval: 0.64-60.80; P=0.12) and OS (P=0.16). Adjuvant RT was also not significantly associated with both recurrence and OS when the cohort was analyzed as a whole and on subgroup analysis by age and tumor location. However, adjuvant RT was associated with significantly longer OS after STR (P=0.03) with the median OS being 6.33 years, as compared to 0.40 years for patients who underwent STR followed by observation.
CONCLUSIONS
Based on our meta-analysis of 37 patients, administration of adjuvant RT after GTR was not significantly associated with improvement in OS or recurrence in patients with WHO grade II ependymoma. However, due to the small number of patients included in the analysis, further prospective controlled studies are warranted.
Topics: Humans; Ependymoma; Radiotherapy, Adjuvant; Female; Male; Neoplasm Grading; World Health Organization
PubMed: 38644544
DOI: 10.21037/cco-23-136 -
Journal of Neurosurgery. Pediatrics Apr 2024More than one-third of pediatric patients who undergo resection of intradural spine lesions develop progressive postoperative deformity, with as many as half of these...
OBJECTIVE
More than one-third of pediatric patients who undergo resection of intradural spine lesions develop progressive postoperative deformity, with as many as half of these patients subsequently requiring surgical fusion. Intradural spinal procedures with simultaneous instrumented fusion in children, however, are infrequently performed. Moreover, the rationale for patient selection, outcomes, and safety of this single-stage surgery in children has not been systematically investigated. In this study, the authors review the practice of simultaneous intradural spinal resection and instrumented fusion in pediatric patients and provide two representative case examples from their institution.
METHODS
The authors searched the PubMed and Embase databases and performed a systematic review following the PRISMA protocol. Original articles of pediatric patients (age ≤ 18 years) who underwent intradural spine surgery, regardless of pathology, with concomitant instrumented fusion and reported outcomes were included. An institutional database of all spinal operations with instrumented fusion performed in patients aged ≤ 18 years over a 3-year period was screened to identify those who underwent intradural spine surgery with concomitant fusion.
RESULTS
Nine patients (median age 12 years) from 6 studies who underwent intradural lesion resection and concomitant fusion met inclusion criteria. Among all 11 patients included, primary rationales for concomitant fusion were extensive bone removal (i.e., corpectomy or total facetectomy, 73%), concerns for deformity in the setting of multilevel laminectomy/laminoplasty (18%), and severe baseline deformity (9%). The most represented pathology was neurenteric cyst (55%) followed by schwannoma (18%). Myxopapillary ependymoma, granular cell tumor, and pilocytic astrocytoma each were seen in 1 case. Seven patients (64%) underwent an anterior-approach corpectomy, tumor resection, and fusion, while the remaining 4 patients (36%) underwent a posterior approach. All patients with at least 1 year of follow-up cases achieved bony fusion. CSF leak and new-onset neurological deficit each occurred in 9% (1/11).
CONCLUSIONS
The rationales for performing single-stage intradural resection and fusion in pediatric patients in studies to date include the presence of severe baseline deformity, large extent of bone resection, and multilevel laminectomy/laminoplasty across cervicothoracic or thoracolumbar junctions. As current literature involving this cohort is limited, more data are needed to determine when concomitant fusion in intradural resections is appropriate in pediatric patients and whether its routine implementation is safe or beneficial.
PubMed: 38579345
DOI: 10.3171/2024.1.PEDS23444 -
Journal of Medical Case Reports Mar 2024Spinal cord tumors present a challenge in diagnosis and treatment due to their varied histopathological characteristics. While Ewing sarcoma is a rare malignant tumor...
BACKGROUND
Spinal cord tumors present a challenge in diagnosis and treatment due to their varied histopathological characteristics. While Ewing sarcoma is a rare malignant tumor typically originating from skeletal bone, cases of primary intradural extraskeletal Ewing sarcoma are exceptionally rare. The similarity of its presentation to other spinal tumors further complicates its identification and management.
CASE PRESENTATION
We report a case of a 58-year-old Palestinian male with intradural extraskeletal lumbar Ewing sarcoma. The patient initially presented with lower back pain and bilateral S1 radiculopathy, with more severe symptoms on the left side. Magnetic resonance imaging revealed a 7 cm oval-shaped mass with homogeneous contrast enhancement, obstructing the spinal canal from L3/L4 to L5/S1 levels. Initially, a myxopapillary ependymoma was suspected, but the patient's sensory and motor functions suddenly deteriorated during hospitalization. Repeat magnetic resonance imaging indicated heterogeneous contrast enhancement, indicating acute intratumoral hemorrhage. Consequently, the patient underwent emergent L3-L5 laminotomy, with successful gross total resection of the tumor. Histopathological and immunohistochemical analyses confirmed the diagnosis of intradural extraskeletal Ewing sarcoma. Adjuvant therapy was administered to minimize the risk of local recurrence or distant metastasis. A systematic review of relevant literature, along with retrospective analysis of medical records, operative reports, radiological studies, and histopathological findings of similar cases, was also conducted.
CONCLUSIONS
Intradural extraskeletal Ewing sarcoma is an infrequently encountered condition in adult patients, emphasizing the importance of considering it in the differential diagnosis of spinal tumors. Surgeons must possess a comprehensive understanding of this rare entity to ensure accurate staging and optimal management, particularly in the early stages when prompt intervention may improve prognosis.
Topics: Humans; Male; Middle Aged; Prognosis; Sarcoma, Ewing; Spinal Cord Neoplasms; Spinal Neoplasms
PubMed: 38459600
DOI: 10.1186/s13256-024-04384-8 -
Frontiers in Neurology 2023Anomalous origin of the middle meningeal artery (MMA) from the basilar artery is a rare congenital neurological variant that has been detected in both children and...
BACKGROUND
Anomalous origin of the middle meningeal artery (MMA) from the basilar artery is a rare congenital neurological variant that has been detected in both children and adults with diagnoses ranging from intracranial haemorrhage to ependymoma. This review aims to investigate the anatomical course of an anomalous basilar-middle meningeal artery and its clinical presentation.
METHODS
A systematic search was performed in PubMed using the keywords (middle meningeal artery) and (basilar artery). Ninety-four papers were identified, of which seven were included. One paper was further identified through cross-referencing.
RESULTS
The average age of presentation was 43 years with a male predominance (7/9). In most cases, the MMA arose between the superior cerebellar artery and the anterior inferior cerebellar artery (8/9) (versus 1 case between the anterior inferior cerebellar artery and the posterior inferior cerebellar artery). The anomaly mostly presented on the left side (6/11), but was bilateral in one case. Most of the cases showed a pontine artery branching from the basilar artery arising 5 mm to 10 mm proximal to the superior cerebellar artery, which would then assume the trajectory of the MMA. In three cases, the vessel increased in calibre near the trigeminal ganglion. Foramen spinosum absence in the anomalous side was noted in 3/6 of the patients.
CONCLUSION
To avoid unexpected complications during neurosurgical and neuroradiointerventional procedures, it is essential to have a clear understanding of the anomalous routes of the MMA. This is especially important when it proves to be the only available route for embolization.
PubMed: 38322796
DOI: 10.3389/fneur.2023.1301426 -
International Journal of Radiation... Jun 2024Reirradiation is increasingly used in children and adolescents/young adults (AYA) with recurrent primary central nervous system tumors. The Pediatric Normal Tissue... (Review)
Review
PURPOSE
Reirradiation is increasingly used in children and adolescents/young adults (AYA) with recurrent primary central nervous system tumors. The Pediatric Normal Tissue Effects in the Clinic (PENTEC) reirradiation task force aimed to quantify risks of brain and brain stem necrosis after reirradiation.
METHODS AND MATERIALS
A systematic literature search using the PubMed and Cochrane databases for peer-reviewed articles from 1975 to 2021 identified 92 studies on reirradiation for recurrent tumors in children/AYA. Seventeen studies representing 449 patients who reported brain and brain stem necrosis after reirradiation contained sufficient data for analysis. While all 17 studies described techniques and doses used for reirradiation, they lacked essential details on clinically significant dose-volume metrics necessary for dose-response modeling on late effects. We, therefore, estimated incidences of necrosis with an exact 95% CI and qualitatively described data. Results from multiple studies were pooled by taking the weighted average of the reported crude rates from individual studies.
RESULTS
Treated cancers included ependymoma (n = 279 patients; 7 studies), medulloblastoma (n = 98 patients; 6 studies), any CNS tumors (n = 62 patients; 3 studies), and supratentorial high-grade gliomas (n = 10 patients; 1 study). The median interval between initial and reirradiation was 2.3 years (range, 1.2-4.75 years). The median cumulative prescription dose in equivalent dose in 2-Gy fractions (EQD2; assuming α/β value = 2 Gy) was 103.8 Gy (range, 55.8-141.3 Gy). Among 449 reirradiated children/AYA, 22 (4.9%; 95% CI, 3.1%-7.3%) developed brain necrosis and 14 (3.1%; 95% CI, 1.7%-5.2%) developed brain stem necrosis with a weighted median follow-up of 1.6 years (range, 0.5-7.4 years). The median cumulative prescription EQD2 was 111.4 Gy (range, 55.8-141.3 Gy) for development of any necrosis, 107.7 Gy (range, 55.8-141.3 Gy) for brain necrosis, and 112.1 Gy (range, 100.2-117 Gy) for brain stem necrosis. The median latent period between reirradiation and the development of necrosis was 5.7 months (range, 4.3-24 months). Though there were more events among children/AYA undergoing hypofractionated versus conventionally fractionated reirradiation, the differences were not statistically significant (P = .46).
CONCLUSIONS
Existing reports suggest that in children/AYA with recurrent brain tumors, reirradiation with a total EQD2 of about 112 Gy is associated with an approximate 5% to 7% incidence of brain/brain stem necrosis after a median follow-up of 1.6 years (with the initial course of radiation therapy being given with conventional prescription doses of ≤2 Gy per fraction and the second course with variable fractionations). We recommend a uniform approach for reporting dosimetric endpoints to derive robust predictive models of late toxicities following reirradiation.
Topics: Humans; Re-Irradiation; Necrosis; Child; Neoplasm Recurrence, Local; Central Nervous System Neoplasms; Adolescent; Brain; Brain Stem; Ependymoma; Young Adult; Child, Preschool; Medulloblastoma; Radiation Injuries
PubMed: 38300187
DOI: 10.1016/j.ijrobp.2023.12.043 -
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 -
Neurosurgical Review Dec 2023Intradural spinal tumors present significant challenges due to involvement of critical motor and sensory tracts. Achieving maximal resection while preserving functional... (Meta-Analysis)
Meta-Analysis Review
Intradural spinal tumors present significant challenges due to involvement of critical motor and sensory tracts. Achieving maximal resection while preserving functional tissue is therefore crucial. Fluorescence-guided surgery aims to improve resection accuracy and is well studied for brain tumors, but its efficacy has not been fully assessed for spinal tumors. This meta-analysis aims to delineate the efficacy of fluorescence guidance in intradural spinal tumor resection. The authors performed a systematic review in four databases. We included studies that have utilized fluorescence agents, 5-aminolevulinic acid (5-ALA) or sodium fluorescein, for the resection of intradural spinal tumors. A meta-analysis was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement. A total of 12 studies involving 552 patients undergoing fluorescence-guided intradural spinal tumor resection were included. Meningiomas demonstrated a 98% fluorescence rate and were associated with a homogenous florescence pattern; however, astrocytomas had variable fluorescence rate with pooled proportion of 70%. There was no significant difference in gross total resection (GTR) rates between fluorescein and 5-ALA (94% vs 84%, p = .22). Pre-operative contrast enhancement was significantly associated with intraoperative fluorescence with fluorescein. Intramedullary tumors with positive intraoperative fluorescence were significantly associated with higher GTR rates (96% vs 73%, p = .03). Utilizing fluorescence guidance during intradural spinal tumor resection holds promise of improving intraoperative visualization for specific intradural spinal tumors. Meningiomas and ependymomas have the highest fluorescence rates especially with sodium fluorescein; on the other hand, astrocytomas have variable fluorescence rates with no superiority of either agent. Positive fluorescence of intramedullary tumors is associated with a higher degree of resection.
Topics: Humans; Spinal Neoplasms; Fluorescein; Fluorescence; Meningioma; Spinal Cord Neoplasms; Astrocytoma; Aminolevulinic Acid; Meningeal Neoplasms
PubMed: 38085385
DOI: 10.1007/s10143-023-02230-x -
Pediatric Radiology Sep 2023Radiomics is the process of converting radiological images into high-dimensional data that may be used to create machine learning models capable of predicting clinical...
BACKGROUND
Radiomics is the process of converting radiological images into high-dimensional data that may be used to create machine learning models capable of predicting clinical outcomes, such as disease progression, treatment response and survival. Pediatric central nervous system (CNS) tumors differ from adult CNS tumors in terms of their tissue morphology, molecular subtype and textural features. We set out to appraise the current impact of this technology in clinical pediatric neuro-oncology practice.
OBJECTIVES
The aims of the study were to assess radiomics' current impact and potential utility in pediatric neuro-oncology practice; to evaluate the accuracy of radiomics-based machine learning models and compare this to the current standard which is stereotactic brain biopsy; and finally, to identify the current limitations of radiomics applications in pediatric neuro-oncology.
MATERIALS AND METHODS
Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) standards, a systematic review of the literature was carried out with protocol number CRD42022372485 in the prospective register of systematic reviews (PROSPERO). We performed a systematic literature search via PubMed, Embase, Web of Science and Google Scholar. Studies involving CNS tumors, studies that utilized radiomics and studies involving pediatric patients (age<18 years) were included. Several parameters were collected including imaging modality, sample size, image segmentation technique, machine learning model used, tumor type, radiomics utility, model accuracy, radiomics quality score and reported limitations.
RESULTS
The study included a total of 17 articles that underwent full-text review, after excluding duplicates, conference abstracts and studies that did not meet the inclusion criteria. The most commonly used machine learning models were support vector machines (n=7) and random forests (n=6), with an area under the curve (AUC) range of 0.60-0.94. The included studies investigated several pediatric CNS tumors, with ependymoma and medulloblastoma being the most frequently studied. Radiomics was primarily used for lesion identification, molecular subtyping, survival prognostication and metastasis prediction in pediatric neuro-oncology. The low sample size of studies was a commonly reported limitation.
CONCLUSION
The current state of radiomics in pediatric neuro-oncology is promising, in terms of distinguishing between tumor types; however, its utility in response assessment requires further evaluation which, given the relatively low number of pediatric tumors, calls for multicenter collaboration.
Topics: Adult; Humans; Child; Adolescent; Area Under Curve; Biopsy; Disease Progression; Machine Learning; Radiology; Multicenter Studies as Topic
PubMed: 37195305
DOI: 10.1007/s00247-023-05679-6 -
Annales D'endocrinologie Aug 2023
Topics: Humans; Astrocytoma; Brain Neoplasms; Magnetic Resonance Imaging; Pituitary Gland; Multiple Endocrine Neoplasia Type 1; Male; Adolescent
PubMed: 37169283
DOI: 10.1016/j.ando.2023.04.004 -
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