scholarly journals Early Onset Epilepsy Caused by Low-Grade Epilepsy-Associated Tumors and Focal Meningeal Involvement

2020 ◽  
Vol 10 (10) ◽  
pp. 752
Author(s):  
Luca De Palma ◽  
Chiara Pepi ◽  
Alessandro De Benedictis ◽  
Nicola Pietrafusa ◽  
Angela Mastronuzzi ◽  
...  
Keyword(s):  
Surgical Outcome ◽  
Early Onset ◽  
Pediatric Patients ◽  
Seizure Outcome ◽  
Low Grade ◽  
Post Surgery ◽  
Meningeal Involvement ◽  
Patients With Epilepsy ◽  
Associated Tumors

Background: Low-grade epilepsy-associated neuroepithelial tumors (LEATs) are a frequent etiology in pediatric patients with epilepsy undergoing surgery. Objective: To identify differences in clinical and post-surgical follow-up between patients with focal meningeal involvement (MI) and those without MI within our cohort of pediatric patients with LEATs. Methods: We retrospectively reviewed all pediatric patients (<18 y) who underwent epilepsy surgery between 2011 and 2017 at our hospital. Cohort inclusion required histological diagnosis of LEATs and post-surgical follow-up of ≥2 y. We subsequently stratified patients according to presence of neuroradiological MI. Results: We identified 37 patients: five with MI and 32 without. Half of patients (19) were drug sensitive at surgery; similar between groups. The group with MI differed mainly for age of epilepsy-onset (0.6 vs. 7.0 y) but not for epilepsy duration (0.9 vs. 1.5 y). Post-surgery radiological follow-up (median 4.0 y; IQR 2.8–5.0 y) did not indicate disease progression. Seizure outcome was excellent in both groups, with 34 patients overall being both drug- and seizure-free. Conclusions: Our study identified a new subgroup of LEATs with focal MI and excellent post-surgical outcome. Moreover, this highlights the effectiveness of early surgery in pediatric LEATs.

Psychiatric and psychosocial morbidity 1 year after epilepsy surgery

2020 ◽  
pp. 1-8
Author(s):  
S. Patel ◽  
M. Clancy ◽  
H. Barry ◽  
N. Quigley ◽  
M. Clarke ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Surgical Treatment ◽  
Epilepsy Surgery ◽  
Refractory Epilepsy ◽  
Post Surgery ◽  
Axis I ◽  
Patients With Epilepsy ◽  
The Impact

Abstract Objectives: There is a high rate of psychiatric comorbidity in patients with epilepsy. However, the impact of surgical treatment of refractory epilepsy on psychopathology remains under investigation. We aimed to examine the impact of epilepsy surgery on psychopathology and quality of life at 1-year post-surgery in a population of patients with epilepsy refractory to medication. Methods: This study initially assessed 48 patients with refractory epilepsy using the Structured Clinical Interview for DSM-IV Axis I Disorders (SCID-I), the Hospital Anxiety and Depression Scale (HADS) and the Quality of Life in Epilepsy Inventory 89 (QOLIE-89) on admission to an Epilepsy Monitoring Unit (EMU) as part of their pre-surgical assessment. These patients were again assessed using the SCID-I, QOLIE-89 and HADS at 1-year follow-up post-surgery. Results: There was a significant reduction in psychopathology, particularly psychosis, following surgery at 1-year follow-up (p < 0.021). There were no new cases of de novo psychosis and surgery was also associated with a significant improvement in the quality of life scores (p < 0.001). Conclusions: This study demonstrates the impact of epilepsy surgery on psychopathology and quality of life in a patient population with refractory surgery. The presence of a psychiatric illness should not be a barrier to access surgical treatment.

Stereoelectroencephalography in Children and Adolescents With Difficult-to-Localize Refractory Focal Epilepsy

Neurosurgery ◽  
2014 ◽  
Vol 75 (3) ◽  
pp. 258-268 ◽  
Author(s):  
Jorge Gonzalez-Martinez ◽  
Jeffrey Mullin ◽  
Juan Bulacio ◽  
Ajay Gupta ◽  
Rei Enatsu ◽  
...  
Keyword(s):  
Children And Adolescents ◽  
Pediatric Patients ◽  
Focal Epilepsy ◽  
Early Experience ◽  
Morbidity Rate ◽  
Seizure Outcome ◽  
Seizure Freedom ◽  
Epileptogenic Zone ◽  
Eloquent Cortex

Abstract BACKGROUND: Although stereoelectroencephalography (SEEG) has been shown to be a valuable tool for preoperative decision making in focal epilepsy, there are few reports addressing the utility and safety of SEEG methodology applied to children and adolescents. OBJECTIVE: To present the results of our early experience using SEEG in pediatric patients with difficult-to-localize epilepsy who were not considered candidates for subdural grid evaluation. METHODS: Thirty children and adolescents with the diagnosis of medically refractory focal epilepsy (not considered ideal candidates for subdural grids and strip placement) underwent SEEG implantation. Demographics, electrophysiological localization of the hypothetical epileptogenic zone, complications, and seizure outcome after resections were analyzed. RESULTS: Eighteen patients (60%) underwent resections after SEEG implantations. In patients who did not undergo resections (12 patients), reasons included failure to localize the epileptogenic zone (4 patients); multifocal epileptogenic zone (4 patients); epileptogenic zone located in eloquent cortex, preventing resection (3 patients); and improvement in seizures after the implantation (1 patient). In patients who subsequently underwent resections, 10 patients (55.5%) were seizure free (Engel class I) and 5 patients (27.7%) experienced seizure improvement (Engel class II or III) at the end of the follow-up period (mean, 25.9 months; range, 12 to 47 months). The complication rate in SEEG implantations was 3%. CONCLUSION: The SEEG methodology is safe and should be considered in children/adolescents with difficult-to-localize epilepsy. When applied to highly complex and difficult-to-localize pediatric patients, SEEG may provide an additional opportunity for seizure freedom in association with a low morbidity rate.

scholarly journals Cortical Dyplasia: Complete Resection Correlates with Outcome…But, Complete Resection of What?

2009 ◽  
Vol 9 (4) ◽  
pp. 100-102 ◽  
Author(s):  
Theodore H. Schwartz
Keyword(s):  
Fdg Pet ◽  
Pediatric Patients ◽  
Focal Cortical Dysplasia ◽  
Cortical Dysplasia ◽  
Seizure Outcome ◽  
Type I ◽  
Type Ii ◽  
Presurgical Evaluation ◽  
Mri Scans

Incomplete Resection of Focal Cortical Dysplasia Is the Main Predictor of Poor Postsurgical Outcome. Krsek P, Maton B, Jayakar P, Dean P, Korman B, Rey G, Dunoyer C, Pacheco-Jacome E, Morrison G, Ragheb J, Vinters HV, Resnick T, Duchowny M. Neurology 2009;72(3):217–223. BACKGROUND: Focal cortical dysplasia (FCD) is recognized as the major cause of focal intractable epilepsy in childhood. Various factors influencing postsurgical seizure outcome in pediatric patients with FCD have been reported. OBJECTIVE: To analyze different variables in relation to seizure outcome in order to identify prognostic factors for selection of pediatric patients with FCD for epilepsy surgery. METHODS: A cohort of 149 patients with histologically confirmed mild malformations of cortical development or FCD with at least 2 years of postoperative follow-up was retrospectively studied; 113 subjects had at least 5 years of postoperative follow-up. Twenty-eight clinical, EEG, MRI, neuropsychological, surgical, and histopathologic parameters were evaluated. RESULTS: The only significant predictor of surgical success was completeness of surgical resection, defined as complete removal of the structural MRI lesion (if present) and the cortical region exhibiting prominent ictal and interictal abnormalities on intracranial EEG. Unfavorable surgical outcomes are mostly caused by overlap of dysplastic and eloquent cortical regions. There were nonsignificant trends toward better outcomes in patients with normal intelligence, after hemispherectomy and with FCD type II. Other factors such as age at seizure onset, duration of epilepsy, seizure frequency, associated pathologies including hippocampal sclerosis, extent of EEG and MRI abnormalities, as well as extent and localization of resections did not influence outcome. Twenty-five percent of patients changed Engel's class of seizure outcome after the second postoperative year. CONCLUSIONS: The ability to define and fully excise the entire region of dysplastic cortex is the most powerful variable influencing outcome in pediatric patients with focal cortical dysplasia. FDG-PET/MRI Coregistration Improves Detection of Cortical Dysplasia in Patients with Epilepsy. Salamon N, Kung J, Shaw SJ, Koo J, Koh S, Wu JY, Lerner JT, Sankar R, Shields WD, Engel J Jr, Fried I, Miyata H, Yong WH, Vinters HV, Mathern GW. Neurology 2008;71(20):1594–1601. OBJECTIVE: Patients with cortical dysplasia (CD) are difficult to treat because the MRI abnormality may be undetectable. This study determined whether fluorodeoxyglucose (FDG)-PET/MRI coregistration enhanced the recognition of CD in epilepsy surgery patients. METHODS: Patients from 2004–2007 in whom FDG-PET/MRI coregistration was a component of the presurgical evaluation were compared with patients from 2000–2003 without this technique. For the 2004–2007 cohort, neuroimaging and clinical variables were compared between patients with mild Palmini type I and severe Palmini type II CD. RESULTS: Compared with the 2000–2003 cohort, from 2004–2007 more CD patients were detected, most had type I CD, and fewer cases required intracranial electrodes. From 2004–2007, 85% of type I CD cases had normal non–University of California, Los Angeles (UCLA) MRI scans. UCLA MRI identified CD in 78% of patients, and 37% of type I CD cases had normal UCLA scans. EEG and neuroimaging findings were concordant in 52% of type I CD patients, compared with 89% of type II CD patients. FDG-PET scans were positive in 71% of CD cases, and type I CD patients had less hypometabolism compared with type II CD patients. Postoperative seizure freedom occurred in 82% of patients, without differences between type I and type II CD cases. CONCLUSIONS: Incorporating fluorodeoxyglucose-PET/MRI coregistration into the multimodality presurgical evaluation enhanced the noninvasive identification and successful surgical treatment of patients with cortical dysplasia (CD), especially for the 33% of patients with nonconcordant findings and those with normal MRI scans from mild type I CD.

scholarly journals Surgical and seizure outcome in children with DNETs who underwent epilepsy surgery

2015 ◽  
Vol 42 (S1) ◽  
pp. S22-S22
Author(s):  
AM Bueckert ◽  
J Pugh ◽  
T Snyder ◽  
M Wheatley ◽  
F Jacob ◽  
...  
Keyword(s):  
Surgical Resection ◽  
Epilepsy Surgery ◽  
Tumor Resection ◽  
Retrospective Chart Review ◽  
Benign Tumors ◽  
Seizure Outcome ◽  
Low Grade ◽  
Partial Seizures ◽  
Complex Partial Seizures

Background: Dysembryoblastic neuroepithelial tumors (DNETs) are benign tumors of the cerebral cortex that most commonly occur in children or young adults. Seizures are a frequent presenting feature, with an incidence of 80-100%, and are often an indication for surgical resection. Methods: We performed a retrospective chart review of children with DNETs who underwent epilepsy surgery between 1998 and 2014. Results: A total of 12 subjects were identified (6 males, 6 females), all of whom had seizures prior to surgical resection. Of these patients, 1 had infantile spasms, 2 had simple partial seizures and 10 had complex partial seizures. Tumors were located in the temporal (n=7), frontal (n=3) or parietal (n=2) cortex. These patients went on to have surgery on average 15 months after seizure onset, 3 had incomplete resections. At an average follow up of 6 years 4 months, all patients were class 1 on Engel’s Classification. All but one subject with rare non-disabling seizures were seizure free, with only 6 on medication. Follow up MR imaging revealed tumor recurrence in 1 subject. Conclusions: Despite differing seizure seminology and tumor location, surgical resection of these low-grade tumors resulted in excellent seizure outcome even in the setting of incomplete tumor resection.

Efficacy and safety of larotrectinib in adult and pediatric patients with tropomyosin receptor kinase (TRK) fusion-positive primary central nervous system tumors.

2021 ◽  
Vol 39 (15_suppl) ◽  
pp. 2002-2002
Author(s):  
Sébastien Perreault ◽  
Cornelis Martinus van Tilburg ◽  
Birgit Geoerger ◽  
Karsten Nysom ◽  
Ingrid Ora ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Disease Control ◽  
Pediatric Patients ◽  
Disease Control Rate ◽  
Cns Tumors ◽  
Low Grade ◽  
Gene Fusions ◽  
Receptor Kinase

2002 Background: Neurotrophic tyrosine receptor kinase ( NTRK) gene fusions are oncogenic drivers in various tumor types, including central nervous system (CNS) tumors. Larotrectinib is a first-in-class, highly selective TRK inhibitor approved for the treatment of adult and pediatric patients with TRK fusion cancer, with an objective response rate (ORR) of 78% across 175 adult and pediatric patients with various non-CNS cancers (McDermott et al, ESMO 2020). We report data on patients with TRK fusion-positive primary CNS tumors. Methods: Patients with primary CNS tumors harboring an NTRK gene fusion enrolled in two clinical trials (NCT02637687, NCT02576431) were identified. Larotrectinib was administered until disease progression, withdrawal, or unacceptable toxicity. Response was investigator assessed. Results: As of July 2020, 33 patients with TRK fusion-positive CNS tumors were identified: 19 high-grade gliomas (HGG), 8 low-grade gliomas (LGG), 2 glioneuronal tumors, 2 neuroepithelial tumors, 1 CNS neuroblastoma, and 1 small round blue cell tumor. The patients had gene fusions involving NTRK2 (n = 24; 73%), NTRK1 (n = 5; 15%), and NTRK3 (n = 4; 12%). Median age was 8.9 years (range 1.3–79.0); 26 patients were pediatric ( < 18 years). Patients were heavily pre-treated with 45% having 2 or more prior lines of systemic therapy. The ORR in all patients was 30% (95% CI 16–49): 3 complete responses (all in pediatric patients), 7 partial responses (2 pending confirmation), 20 stable disease (including 15 pts > 6 months), and 3 progressive disease. The ORR in patients with HGG and LGG were 26% (95% CI 9–51) and 38% (95% CI 9–76), respectively. In all patients, the 24-week disease control rate was 73% (95% CI 54–87). Twenty-three of 28 patients (82%) with measurable disease had tumor shrinkage. The median time to response was 1.9 months. Median duration of response (DoR) was not reached (95% CI 3.8–not estimable [NE]) at a median follow-up of 12.0 months. The 12-month DoR rate was 75% (95% CI 45–100). Median PFS was 18.3 months (95% CI 6.7–NE) at a median follow-up of 16.5 months. Median overall survival (OS) was not reached (95% CI 16.9–NE) at a median follow-up of 16.5 months, with a 12-month OS rate of 85% (95% CI 71–99). Duration of treatment ranged from 1.2 to 31.3+ months. Treatment-related adverse events (TRAE) were reported by 20 patients and were Grade 3–4 in 3 patients (9%). There were no treatment discontinuations due to TRAEs. Conclusions: In patients with TRK fusion-positive CNS tumors, larotrectinib demonstrated rapid and durable responses, high disease control rate, and a favorable safety profile. These results support testing for NTRK gene fusions in patients of all ages with CNS tumors. Clinical trial information: NCT02637687, NCT02576431.

Stereotactic Brachytherapy With Iodine-125 Seeds for the Treatment of Inoperable Low-Grade Gliomas in Children: Long-Term Outcome

2011 ◽  
Vol 29 (31) ◽  
pp. 4151-4159 ◽  
Author(s):  
Maximilian I. Ruge ◽  
Thorsten Simon ◽  
Bogdana Suchorska ◽  
Ralph Lehrke ◽  
Christina Hamisch ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Tumor Volume ◽  
Local Treatment ◽  
Low Grade ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Stereotactic Brachytherapy ◽  
Iodine 125

Purpose Resection is generally considered the gold standard for treatment of low-grade (WHO grades I and II) gliomas (LGGs) in childhood. However, approximately 30% to 50% of these tumors are inoperable because of their localization in highly eloquent brain areas. A few reports have suggested stereotactic brachytherapy (SBT) with implantation of iodine-125 (125I) seeds as a safe and effective local treatment alternative. This single-center study provides a summary of the long-term outcome after SBT in one of the largest reported patient series. Patients and Methods All pediatric patients treated with SBT (125I seeds; cumulative therapeutic dose 50-65 Gy within 9 months) by our group for LGG with follow-up of more than 6 months were included. Clinical and radiologic outcome, time to progression, and overall survival were evaluated. Prognostic factors (age, sex, Karnofsky performance score, tumor volume, and histology) for survival and disease progression were investigated. Results In all, 147 of 160 pediatric patients treated with SBT (from 1982 through 2009) were analyzed in detail. Procedure-related mortality was zero, and the 30-day morbidity was transient and low (5.4%). Survival rates at 5 and 10 years were 93%, and 82%, respectively, with no significant difference between WHO grades I and II tumors (median follow-up, 67.1 ± 57.7 months). Twenty-one (14.8%) of 147 patients presented with tumor relapse. The remaining 126 patients revealed complete response in 24.6%, partial response in 31.0%, and stable disease in 29.6%. Neurologic status improved (57.8%) or remained stable (23.0%). None of the evaluated factors had significant impact on the study's end points except tumor volume more than 15 mL, which caused significantly higher rates of tumor recurrence (P < .05). Conclusion We demonstrate that SBT represents a safe, minimally invasive, and highly effective local treatment option for pediatric patients with inoperable LGG WHO grades I and II.

Seizure-free and neuropsychological outcomes after temporal lobectomy with amygdalohippocampectomy in pediatric patients with hippocampal sclerosis

2012 ◽  
Vol 10 (2) ◽  
pp. 103-107 ◽  
Author(s):  
Sumeet Vadera ◽  
Varun R. Kshettry ◽  
Patricia Klaas ◽  
William Bingaman
Keyword(s):  
Pediatric Patients ◽  
Hippocampal Sclerosis ◽  
Pediatric Population ◽  
Cleveland Clinic ◽  
Intractable Epilepsy ◽  
Cortical Dysplasia ◽  
Temporal Lobectomy ◽  
Low Grade ◽  
Neuropsychological Outcomes

Object Temporal lobe epilepsy is an uncommon clinical syndrome in the pediatric population. The most common underlying pathologies include low-grade gliomas, cortical dysplasia, and, less commonly, hippocampal sclerosis (HS). There is a paucity of data on neuropsychological and seizure-free outcomes in these patients after temporal lobectomy. In this study, the authors reviewed their seizure-free and neuropsychological outcomes after temporal lobectomy for pediatric HS. Methods The authors retrospectively reviewed the medical records of pediatric patients with HS who underwent anterior temporal lobectomy and amygdalohippocampectomy between 1998 and 2011 at the Cleveland Clinic. Results of neuropsychological assessment before and after surgery and seizure-free outcome at last follow-up were obtained. Results Forty-five patients met the inclusion criteria. Thirty-four (76%) patients had pathology of HS alone and 10 (22%) had HS and cortical dysplasia. The mean duration of follow-up was 60.2 months. Eighty-four percent of patients had postoperative Engel Class I or II outcomes. Neuropsychological outcomes remained unchanged or minimally improved postoperatively. Conclusions Seizure-free outcomes in pediatric HS are similar to historical rates in adult HS. Neuropsychological assessments remain stable after temporal lobectomy. Standard temporal lobectomy should be considered in pediatric patients with medically intractable epilepsy secondary to HS.

Changes in the Maxillary Sinus Volume and the Surgical Outcome after the Canine Fossa Puncture Approach in Pediatric Patients with an Antrochoanal Polyp: Results of a Minimum 3-Year Follow-Up

2009 ◽  
Vol 23 (5) ◽  
pp. 531-534 ◽  
Author(s):  
Jae Yong Lee ◽  
Byoung Joon Baek ◽  
Dong Wook Kim ◽  
Jang Yul Byun ◽  
Seung Won Lee ◽  
...  
Keyword(s):  
Maxillary Sinus ◽  
Surgical Outcome ◽  
Pediatric Patients ◽  
Maxillary Antrum ◽  
Antrochoanal Polyp ◽  
Long Term Effects ◽  
Recurrence Rates ◽  
Maxillary Sinus Volume ◽  
Canine Fossa

Background An antrochoanal polyp (ACP) originates from the maxillary antrum and simple excision results in high recurrence rates. Canine fossa puncture (CFP) has been proposed as an alternative method of accessing the entire maxillary antrum. To investigate the long-term effects of the CFP approach on changes in the maxillary sinus volume in pediatric patients with an ACP, we compared the sinus volume on the operated and normal sides, using preoperative computed tomography (CT), and postoperative CT performed at least 3 years after the procedure. The surgical outcomes were also assessed using endoscopic and CT findings. Methods Seven patients met the inclusion criteria and the ACP was removed via the CFP approach. After a mean follow-up period of 43.9 months, we compared the changes in the maxillary sinus volume between the operated and normal sides, using the pre- and postoperative CT data. Results No contractures or decrease in the maxillary sinus volume on the diseased side were observed in any of the patients on postoperative CT, and the average volume of both maxillary sinuses was greater on postoperative CT compared with the preoperative scans. None of the patients showed evidence of recurrence on the endoscopic and CT examinations. Conclusion CFP did not affect the maxillary sinus volume in pediatric patients with an ACP and led to a successful surgical outcome in all of the patients. Based on these results, we recommend CFP as a safe, effective method for the treatment of ACP.

scholarly journals RONC-22. SECOND TUMORS IN PEDIATRIC PATIENTS TREATED WITH PROTON THERAPY TO THE CENTRAL NERVOUS SYSTEM

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii459-iii459
Author(s):  
Daniel J Indelicato ◽  
James Bates ◽  
Raymond Mailhot-Vega ◽  
Christopher Morris ◽  
Eric Sandler ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Proton Therapy ◽  
Cumulative Incidence ◽  
Pediatric Patients ◽  
Photon Radiation ◽  
Low Grade ◽  
High Grade ◽  
Second Tumors

Abstract BACKGROUND Previous institutional data suggests the 10-year cumulative incidence of second tumors is 3% in children treated with photon radiation for central nervous system (CNS) malignancy, with 90% of these tumors occurring in areas receiving ≤36 Gy. Comparative figures for children treated with proton therapy (PT) does not exist. METHODS 1056 consecutive pediatric patients with a median follow-up of 5.0 years were treated between 2006–2019 with double-scattered PT to a site within the craniospinal axis. 230 patients were ≤3 years old and 14 had neurofibromatosis. A second tumor was defined as any solid neoplasm with histologic features different from the original tumor that had arisen within the irradiated volume. RESULTS Five patients developed second tumors resulting in a 5- and 10-year cumulative incidence of 0.2% (95% CI: 0–1.2%) and 1.6% (95% CI: 0.6%-3.9%), respectively. Of those who developed second tumors, median age at radiation was 4.3 years old (range, 2.1 to 5.1 years old) and diagnoses consisted of medulloblastoma (n=2), ependymoma (n=2), and craniopharyngioma (n=1). The second tumors included high grade gliomas (n=3) and high grade sarcoma (n=1) that occurred in regions receiving at least 54 Gy. One patient with neurofibromatosis developed both a low-grade glioma and choroidal melanoma in craniospinal irradiation regions receiving 36 Gy. Four of five patients with second tumors are alive. CONCLUSION The reduction in moderate-to-low dose radiation exposure from proton therapy may be associated with a decreased incidence of second tumors in children treated for CNS neoplasms. More follow-up is needed to confirm these findings.

Spinal cord ependymomas and myxopapillary ependymomas in the first 2 decades of life: a clinicopathological and immunohistochemical characterization of 19 cases

2012 ◽  
Vol 9 (6) ◽  
pp. 646-653 ◽  
Author(s):  
James H. Stephen ◽  
Angela J. Sievert ◽  
Peter J. Madsen ◽  
Alexander R. Judkins ◽  
Adam C. Resnick ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Protein Expression ◽  
Pediatric Patients ◽  
Pathological Diagnosis ◽  
Gross Total Resection ◽  
Low Grade ◽  
Total Resection ◽  
Significant Difference ◽  
Grade Ii

Object Primary spinal cord ependymomas (EPNs) are rare in children, comprising classical WHO Grade II and III tumors and Grade I myxopapillary ependymomas (MEPNs). Despite their benign histology, recurrences and neural-axis dissemination have been reported in up to 33% MEPNs in the pediatric population. Treatment options beyond resection are limited, and little is known about their tumorigenesis. The purpose of this study was to explore the tumor biology and outcomes in a consecutive series of pediatric patients treated at a single institution. Methods The authors performed a retrospective clinicopathological review of 19 patients at a tertiary referral children's hospital for resection of a spinal cord ependymoma. The population included 8 patients with a pathological diagnosis of MEPN and 11 patients with a pathological diagnosis of spinal EPN (10 cases were Grade II and 1 case was Grade III). The upregulation of the following genes HOXB13, NEFL, PDGFRα, EGFR, EPHB3, AQP1, and JAGGED 1 was studied by immunohistochemistry from archived paraffin-embedded tumor samples of the entire cohort to compare the expression in MEPN versus EPN. Results Gross-total resection was achieved in 75% of patients presenting with MEPNs and in 100% of those with EPNs. The average follow-up period was 79 months for the MEPN subset and 53 months for Grade II/III EPNs. Overall survival for both subsets was 100%. However, event-free survival was only 50% for patients with MEPNs. Of note, in all cases involving MEPNs that recurred, the patients had undergone gross-total resection on initial surgery. In contrast, there were no tumor recurrences in patients with EPNs. Immunohistochemistry revealed no significant differences in protein expression between the two tumor types with the exception of EPHB3, which demonstrates a tendency to be positive in MEPNs (6 reactive tumors of 9) rather than in EPN (2 reactive tumors of 10). Conclusions The authors' experience shows that, following a gross-total resection, MEPNs are more likely to recur than their higher-grade counterpart, EPNs. This supports the recommendation for close long-term radiological follow-up of pediatric patients with MEPNs to monitor for recurrence, despite the tumor's low-grade histological feature. No significant difference in the protein expression of HOXB13, NEFL, PDGFRα, EGFR, EPHB3, AQP1, and JAGGED 1 was present in this selected cohort of pediatric patients.

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