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World Neurosurgery Feb 2022Hemangioblastomas (HBs) are well-vascularized, benign central nervous system tumors and the third most common primary spinal cord tumor after astrocytoma/ependymoma,... (Review)
Review
BACKGROUND
Hemangioblastomas (HBs) are well-vascularized, benign central nervous system tumors and the third most common primary spinal cord tumor after astrocytoma/ependymoma, occurring sporadically or as a part of autosomal dominant von Hippel-Lindau disease, in which tumors are often multiple and prone to relapse. Spinal HBs are commonly located in the cervical cord and associated with a syrinx formation. Owing to location and growth trends, they may cause significant neurological deficit, impairing quality of life. We conducted a systematic review to understand better clinical insights into spinal HB in adults and compare spinal HB versus posterior cranial fossa HB.
METHODS
Following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines for conducting systematic reviews, we reviewed the English-language literature on adult spinal HB in the MEDLINE/PubMed database over the last 40 years.
RESULTS
We reviewed 237 articles on adult spinal HB and analyzed national and continental distribution, clinical symptoms, tumor location and presence of syringomyelia, treatment strategies and postoperative complications, histology and immunochemistry, and treatment outcomes. We compared individual characteristics in sporadic and von Hippel-Lindau disease spinal HBs. Finally, we compared features of posterior cranial fossa and spinal HBs.
CONCLUSIONS
Spinal cord HBs most commonly have a dorsal intramedullary location. Total surgical tumor resection is the first treatment option; preoperative embolization may be performed to reduce intraoperative bleeding and surgical time. HBs located in the spine have decreased mortality and rate of infection, but increased rates of cardiopulmonary complications compared with HBs in the posterior cranial fossa.
Topics: Adult; Hemangioblastoma; Humans; Neoplasm Recurrence, Local; Quality of Life; Spinal Cord Neoplasms; Syringomyelia; von Hippel-Lindau Disease
PubMed: 34687932
DOI: 10.1016/j.wneu.2021.10.105 -
World Neurosurgery Jan 2022Artificial intelligence (AI) has facilitated the analysis of medical imaging given increased computational capacity and medical data availability in recent years....
OBJECTIVE
Artificial intelligence (AI) has facilitated the analysis of medical imaging given increased computational capacity and medical data availability in recent years. Although many applications for AI in the imaging of brain tumors have been proposed, their potential clinical impact remains to be explored. A systematic review was performed to examine the role of AI in the analysis of pediatric brain tumor imaging.
METHODS
PubMed, Embase, and Scopus were searched for relevant articles up to January 27, 2021.
RESULTS
Literature search identified 298 records, of which 22 studies were included. The most commonly studied tumors were posterior fossa tumors including brainstem glioma, ependymoma, medulloblastoma, and pilocytic astrocytoma (15, 68%). Tumor diagnosis was the most frequently performed task (14, 64%), followed by tumor segmentation (3, 14%) and tumor detection (3, 14%). Of the 6 studies comparing AI to clinical experts, 5 demonstrated superiority of AI for tumor diagnosis. Other tasks including tumor segmentation, attenuation correction of positron emission tomography scans, image registration for patient positioning, and dose calculation for radiotherapy were performed with high accuracy comparable with clinical experts. No studies described use of the AI tool in routine clinical practice.
CONCLUSIONS
AI methods for analysis of pediatric brain tumor imaging have increased exponentially in recent years. However, adoption of these methods in clinical practice requires further characterization of validity and utility. Implementation of these methods may streamline clinical workflows by improving diagnostic accuracy and automating basic imaging analysis tasks.
Topics: Artificial Intelligence; Brain Neoplasms; Child; Humans; Neuroimaging
PubMed: 34648981
DOI: 10.1016/j.wneu.2021.10.068 -
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 -
Neurosurgical Review Feb 2022Cauda equina paragangliomas are rare benign extra-adrenal neuroendocrine tumours arising from the neural crest cells associated with autonomic ganglia. These tumours are... (Review)
Review
Cauda equina paragangliomas are rare benign extra-adrenal neuroendocrine tumours arising from the neural crest cells associated with autonomic ganglia. These tumours are often mistaken preoperatively for ependymomas or schwannomas. Patients present with axial or radicular pain with or without neurological deficits. Recurrence, secretory features and length of follow-up are controversial. We conducted a retrospective cohort study of paraganglioma through searching a prospectively maintained histopathology database. Patient demographics, presentation, surgery, complications, recurrence, follow-up and outcome between 2004 and 2016 were studied. The primary aim was to collate and describe the current evidence base for recurrence and secretory features of the tumour. The secondary objective was to report outcome and follow-up strategy. A scoping review was performed in accordance with the PRISMA-ScR Checklist. Ten patients were diagnosed (M:F 7:3) with a mean age of 53.6 ± 5.1 (range 34-71 years). MRI scans revealed intradural lumbar enhancing lesions. All patients had complete microsurgical excisions without adjuvant therapy with no recurrence with a mean follow-up of 5.1 ± 1.4 years. Tumours were attached to the filum terminale. Electron microscopic images demonstrated abundant neurosecretory granules with no evidence of catecholamine production. A total of 620 articles were screened and 65 papers (including ours) combining 121 patients (mean age 48.8 and M:F 71:50) were included. The mean follow-up was 3.48 ± 0.46 (range 0.15-23 years). Back pain was the most common symptom (94%). Cure following surgery was achieved in 93% of the patients whilst 7% had recurrence. Total resection likely results in cure without the need for adjuvant therapy or prolonged follow-up. However, in certain situations, the length of follow-up should be determined by the treating surgeon.
Topics: Adult; Aged; Cauda Equina; Ependymoma; Humans; Magnetic Resonance Imaging; Middle Aged; Paraganglioma; Peripheral Nervous System Neoplasms; Retrospective Studies
PubMed: 34021421
DOI: 10.1007/s10143-021-01565-7 -
Journal of Clinical Neuroscience :... Apr 2021Neurofibromatosis type 2 (NF2) is a rare, hereditary tumor syndrome, often requiring repeated surgeries for multiple lesions with significant cumulative morbidity. As...
Neurofibromatosis type 2 (NF2) is a rare, hereditary tumor syndrome, often requiring repeated surgeries for multiple lesions with significant cumulative morbidity. As such, non-operative management should be considered when possible for this patient population. The aim of this study is to provide a systematic review of the literature regarding this treatment strategy. A descriptive case of a patient in whom bevacizumab treatments enabled over 15 years of surgical postponement for a symptomatic spinal cord ependymoma is also provided. Evidence suggests that bevacizumab is a reasonable surgery-deferring option for cystic lesions, and it may be especially useful in NF2 patients to reduce cumulative morbidity.
Topics: Adult; Antineoplastic Agents, Immunological; Bevacizumab; Conservative Treatment; Ependymoma; Female; Humans; Neurofibromatosis 2; Spinal Cord Neoplasms
PubMed: 33775351
DOI: 10.1016/j.jocn.2021.01.010 -
World Neurosurgery Jan 2021The survival outcomes of clear cell ependymomas are poorly understood. This study clarifies the role of surgery and adjuvant therapy when this morphologically distinct... (Meta-Analysis)
Meta-Analysis
BACKGROUND
The survival outcomes of clear cell ependymomas are poorly understood. This study clarifies the role of surgery and adjuvant therapy when this morphologically distinct tumor is encountered.
METHODS
A systematic search for studies relating to clear cell ependymomas was conducted. Primary outcomes were progression-free survival and overall survival. Prognostic variables were age, sex, tumor consistency, extent of resection, and postoperative adjuvant therapy. Kaplan-Meier survival curves were generated and compared by the log-rank test. Multivariate Cox regression models were constructed, interrogated with Schoenfeld residuals, and used to identify independent prognostic factors.
RESULTS
Of the 384 articles retrieved, 8 articles comprising 77 cases of clear cell ependymoma were included. Five-year overall survival and progression-free survival were 58.1% (95% confidence interval [CI], 46.3%-72.9%) and 46.3% (95% CI, 34.2%-62.8%), respectively. Kaplan-Meier analysis with the log-rank test showed that gross total resection was superior to subtotal resection in prolonging survival (P = 0.047) and delayed time to recurrence (P < 0.01). Multivariate analysis confirmed gross total resection as an independent protective factor against relapse (odds ratio, 0.39; 95% CI, 0.17-0.89; P = 0.03). Age <50 years predicted longer overall survival (odds ratio, 0.16; 95% CI, 0.05-0.49; P < 0.01). Postoperative adjuvant therapy after gross total resection did not affect overall survival (P = 0.98) or progression-free survival (P = 0.93). Adjuvant therapy after subtotal resection favored improved overall survival (P = 0.052).
CONCLUSIONS
Clear cell ependymomas are particularly aggressive in those aged >50 years. Gross total resection remains the cornerstone of management. Postoperative adjuvant therapy is likely to be of survival benefit only after subtotal resection.
Topics: Brain Neoplasms; Chemoradiotherapy, Adjuvant; Combined Modality Therapy; Ependymoma; Humans; Kaplan-Meier Estimate; Neurosurgical Procedures; Progression-Free Survival; Survival Analysis; Treatment Outcome
PubMed: 32949795
DOI: 10.1016/j.wneu.2020.09.044 -
World Neurosurgery Jan 2021The rare clinical entity of primary posterior pituitary tumors (PPTs) includes pituicytomas, granular cell tumors, spine cell oncocytomas, and sellar ependymomas. The...
Primary Tumors of the Posterior Pituitary Gland: A Systematic Review of the Literature in Light of the New 2017 World Health Organization Classification of Pituitary Tumors.
OBJECTIVE
The rare clinical entity of primary posterior pituitary tumors (PPTs) includes pituicytomas, granular cell tumors, spine cell oncocytomas, and sellar ependymomas. The recent World Health Organization classification of PPTs based on thyroid transcription factor 1 positivity has led to more investigations into the epidemiology, clinical presentation, nature history, histologic features, and operative characteristics of these tumors. The aim of this review is to summarize the characteristics of primary PPTs.
METHODS
Our summary involved an in-depth review of the literature on PPTs. Our systematic review was carried out using the PubMed database and PRISMA guidelines.
RESULTS
An initial search identified 282 publications. After strict application of the inclusion criteria, we found 16 articles for case series of patients with primary PPT (N > 5), which were included in our table for literature review. An additional 10 articles were review articles on PPTs published in the last 20 years and were used as resource for our systematic review. An extensive analysis was then performed to extract relevant clinical data with respect to the clinical radiologic histopathologic profile of primary PPTs and their treatment outcome.
CONCLUSIONS
Primary PPTs are a rare group of pituicyte-derived low-grade nonneuroendocrine neoplasms that arise from the sellar region. The nondescript radiographic findings and subtle endocrine abnormalities also veil their accurate diagnostic prediction. As shown through the narrative as well as the literature review, there is still a lot to be understood about PPTs. A prospective multicenter registry of these rare tumors would benefit both the neurosurgical as well as the endocrinologic knowledge base.
Topics: Humans; Pituitary Gland, Posterior; Pituitary Neoplasms; World Health Organization
PubMed: 32916355
DOI: 10.1016/j.wneu.2020.09.023 -
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 -
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 -
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