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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 -
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 -
Medicine Jun 2019Most of the previous studies combined all types of intramedullary ependymomas without providing accurate pathological subtypes. In addition, it was very difficult to... (Meta-Analysis)
Meta-Analysis
BACKGROUND
Most of the previous studies combined all types of intramedullary ependymomas without providing accurate pathological subtypes. In addition, it was very difficult to evaluate the factors associated with postoperative outcomes of patients with different pathological subtypes of intramedullary Grade II ependymomas by traditional meta-analysis. This study evaluated the factors related with postoperative outcomes of patients with intramedullary Grade II ependymomas.
METHODS
Individual patient data analysis was performed using PubMed, Embase, and the Cochrane Central Register of Controlled Trials. The search included articles published up to April 2018 with no lower date limit on the search results. The topics were intramedullary Grade II ependymomas. Progression-free survival (PFS) and overall survival (OS) were analyzed by Kaplan-Meier survival analysis (log-rank test). The level of significance was set at P < .05.
RESULTS
A total of 21 studies with 70 patients were included in this article. PFS of patients who underwent total resection was much longer than the PFS of those who received subtotal resection (P < .001). Patients who received adjuvant therapy (P = .005) or radiotherapy and chemotherapy (P < .001) seemed to have shorter PFS than others; PFS of patients who had cerebrospinal fluid disease dissemination (P = .022) or scoliosis (P = .001) were significantly shorter than others. OS of cellular ependymoma patients was less than giant cell ependymoma patients (P < .001).
CONCLUSIONS
PFS of patients who received total resection was much longer than those who received subtotal resection. Patients treated with adjuvant therapy or radiotherapy and chemotherapy appeared to have shorter PFS than others; PFS of patients with cerebrospinal fluid disease dissemination or scoliosis were significantly shorter than others. Cellular ependymomas would have better OS than giant cell ependymoma. However, giant cell ependymoma patients might have the worst OS.
Topics: Adult; Ependymoma; Female; Humans; Male; Middle Aged; Postoperative Complications; Spinal Cord Neoplasms; Survival Analysis; Treatment Outcome
PubMed: 31232977
DOI: 10.1097/MD.0000000000016185 -
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 -
Journal of Medical Case Reports Dec 2014Ependymomas are rare glial tumors of the brain representing less than 5% of brain tumors. However, spinal cord ependymomas in adults account for over 60% of all... (Review)
Review
INTRODUCTION
Ependymomas are rare glial tumors of the brain representing less than 5% of brain tumors. However, spinal cord ependymomas in adults account for over 60% of all ependymomas including those arising from the filum terminale and only 40% are intracranial. Reports of the appearance of another neoplasia at a different location in patients with spinal ependymoma are scarce.
METHODS
We searched PubMed for studies related to spinal cord ependymomas published over the last 30 years (from January 1984) and retrieved 1197.
RESULTS
We identified only two studies that met our criteria and we found an incidence of 9% of secondary neoplasias after treatment for spinal ependymoma. The neoplasms were diagnosed from 2 months to 20 years after patients underwent surgery for intraspinal ependymoma. These included pancreatic cancer, prostate cancer, Hodgkin lymphoma, intracranial meningioma, mucin-producing pulmonary adenocarcinoma, gastric cancer and astrocytoma.
CONCLUSIONS
The genetic abnormalities affecting patients with spinal ependymomas may indicate a predisposition to the development of secondary cancers or a general failure of the repairing mechanism in their DNA. The unaffected survival rates in those individuals permit for a long period the accumulation of different mutations on the genome and thus the appearance of a second cancer. However, more studies are needed, particularly in young patients with high survival rates.
Topics: Ependymoma; Humans; Spinal Cord Neoplasms
PubMed: 25519213
DOI: 10.1186/1752-1947-8-438 -
PloS One 2014The aim of this study was to determine the suitability of magnetic resonance spectroscopy (MRS) for screening brain tumors, based on a systematic review and... (Meta-Analysis)
Meta-Analysis Review
OBJECT
The aim of this study was to determine the suitability of magnetic resonance spectroscopy (MRS) for screening brain tumors, based on a systematic review and meta-analysis of published data on the diagnostic performance of MRS.
METHODS
The PubMed and PHMC databases were systematically searched for relevant studies up to December 2013. The sensitivities and specificities of MRS in individual studies were calculated and the pooled diagnostic accuracies, with 95% confidence intervals (CI), were assessed under a fixed-effects model.
RESULTS
Twenty-four studies were included, comprising a total of 1013 participants. Overall, no heterogeneity of diagnostic effects was observed between studies. The pooled sensitivity and specificity of MRS were 80.05% (95% CI = 75.97%-83.59%) and 78.46% (95% CI: 73.40%-82.78%), respectively. The area under the summary receiver operating characteristic curve was 0.78. Stratified meta analysis showed higher sensitivity and specificity in child than adult. CSI had higher sensitivity and SV had higher specificity. Higher sensitivity and specificity were obtained in short TE value.
CONCLUSION
Although the qualities of the studies included in the meta-analysis were moderate, current evidence suggests that MRS may be a valuable adjunct to magnetic resonance imaging for diagnosing brain tumors, but requires selection of suitable technique and TE value.
Topics: Adult; Area Under Curve; Astrocytoma; Brain Neoplasms; Child; Ependymoma; Glioma; Humans; Magnetic Resonance Spectroscopy; Neuroectodermal Tumors; Sensitivity and Specificity
PubMed: 25393009
DOI: 10.1371/journal.pone.0112577 -
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 -
Pathology Oncology Research : POR Dec 2009Distinction between grade II ependymomas and anaplastic ependymomas based on histopathological examination solely is problematic and, therefore, the management of... (Meta-Analysis)
Meta-Analysis Review
Distinction between grade II ependymomas and anaplastic ependymomas based on histopathological examination solely is problematic and, therefore, the management of intracranial ependymomas remains controversial. The aim of this study was to conduct a systematic review (SR) and meta-analysis (MA) of data published on immunohistochemical prognostic markers (IPM) in intracranial ependymomas (IE), and to establish an evidence-based perspective on their clinical value. Following the extensive search based on a strictly defined group of key words, 30 studies reporting results on IPM in IE were identified. Due to a pronounced inter-study heterogeneity, only 14 publications fulfilled the criteria for inclusion into SR. From the total of 67 immunohistochemical markers, 18 were found to correlate with prognosis. However, owing to inadequate data publishing, MA could be performed only with data on proliferation marker MIB-1 (Ki-67) from 5 publications, including 337 patients: The pooled hazard ratio for overall survival was 3.16 (95% confidence interval = 1.96-5.09; p < 0.001) implicating that patients suffering from tumors with higher immunohistochemical expression of MIB-1 had a significantly worse outcome. Marked inter-study heterogeneity and incomplete data publishing in primary studies significantly limited extent of the SR, and the possibility of performing MA. Although the prognostic impact of MIB-1 immunoexpression in IE could be confirmed, there remains lack of further reliable IPM that could be used in routine diagnosis. We encourage to search for new, useful markers, as well as to standardize lab-techniques and data interpretation algorithms across laboratories in order to increase data compatibility.
Topics: Biomarkers, Tumor; Brain Neoplasms; Ependymoma; Humans; Ki-67 Antigen; Prognosis; Tumor Suppressor Protein p53
PubMed: 19301151
DOI: 10.1007/s12253-009-9160-2