135 Pediatric Supratentorial Ependymoma: Clinical, Radiographic and Molecular Analysis

Neurosurgery ◽  
2017 ◽  
Vol 64 (CN_suppl_1) ◽  
pp. 231-231
Author(s):  
Jock Lillard ◽  
Paul Klimo ◽  
Garrett Thomas Venable
Keyword(s):  
Overall Survival ◽  
Molecular Analysis ◽  
Progression Free Survival ◽  
Tumor Grade ◽  
Extent Of Resection ◽  
Gross Total Resection ◽  
Total Resection ◽  
Free Survival ◽  
Favorable Prognosis ◽  
Supratentorial Ependymoma

Abstract INTRODUCTION Recent molecular analyses support a behavioral and clinical distinction between supratentorial and infratentorial ependymomas, with supratentorial tumors, in general, having a more favorable prognosis. The goal of this study was todescribe our experience managing supratentorial ependymoma in children. METHODS A prospectively maintained neurooncology database was queried to identify cases ofsupratentorial ependymoma treated atSt Jude Children's Research Hospital (SJCRH) and LeBonheur from 1990 through December 31, 2014. Clinical, operative, and radiographic information were reviewed. Outcome measures, determined by review of clinic notes and subsequent imaging, included extent of resection, progression free survival (PFS), overall survival (OS), recurrence of disease and method of post-failure treatment, seizures, requirement of anti-seizure medications, hydrocephalus requiring shunt placement, and death.Detection ofthe C11or95-RELA fusion or rearrangement was performed using iFISH in those patients whose tumor tissue was still available. RESULTS >Seventy-four cases (42% male) of supratentorial ependymoma were identified. Median age at diagnosis was 6.8 years. In total, 59 (79.7%) of 74 patients underwent gross total resection (GTR). Overall survival was a median of 7.4 years. Fifteen patients died of disease. Progression free survival was a median of 3.6 years. Eighty percent of those children whose initial resection was subtotal developed recurrence compared to 50.8% in those who had a GTR. For those patients that recurred, all 42 received further treatment, including further resection, chemotherapy and radiation. Molecular analysis was available for 52 patients (70.3%). Age less than 3, gross total resection, and tumor grade were not associated with improved PFS or OS. Absence of C11-or95-RLEA fusion was associated with a significantly worse PFS, although OS was similar. CONCLUSION Supratentorial ependymoma often have a more favorable prognosis compared toinfratentorial variants. Like many pediatric tumors, supratentorial ependymomas can bestratified based on clinical, surgical and possibly molecular variables.

Pediatric Clival Chordoma: A Curable Disease that Conforms to Collins' Law

Neurosurgery ◽  
2017 ◽  
Vol 82 (5) ◽  
pp. 652-660 ◽  
Author(s):  
Marcio S Rassi ◽  
M Maher Hulou ◽  
Kaith Almefty ◽  
Wenya Linda Bi ◽  
Svetlana Pravdenkova ◽  
...  
Keyword(s):  
Overall Survival ◽  
Skull Base ◽  
Proton Beam ◽  
Statistical Significance ◽  
Progression Free Survival ◽  
Gross Total Resection ◽  
Total Resection ◽  
Free Survival ◽  
Proton Beam Radiotherapy ◽  
Skull Base Chordomas

Abstract BACKGROUND Skull base chordomas in children are extremely rare. Their course, management, and outcome have not been defined. OBJECTIVE To describe the preeminent clinical and radiological features in a series of pediatric patients with skull base chordomas and analyze the outcome of a cohort who underwent uniform treatment. We emphasize predictors of overall survival and progression-free survival, which aligns with Collins’ law for embryonal tumors. METHODS Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. We retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant proton-beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263). RESULTS Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression-free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival. CONCLUSION Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins’ law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure.

Ependymoma of the spinal cord in children and adolescents: a retrospective series from the HIT database

2010 ◽  
Vol 6 (2) ◽  
pp. 137-144 ◽  
Author(s):  
Martin Benesch ◽  
Daniela Weber-Mzell ◽  
Nicolas U. Gerber ◽  
Katja von Hoff ◽  
Frank Deinlein ◽  
...  
Keyword(s):  
Spinal Cord ◽  
Overall Survival ◽  
Adjuvant Treatment ◽  
Progression Free Survival ◽  
Gross Total Resection ◽  
Myxopapillary Ependymoma ◽  
Total Resection ◽  
Who Grade ◽  
Free Survival ◽  
Spinal Cord Ependymoma

Object Reports on spinal cord ependymoma in children are rare. The aim of this study was to evaluate the clinical spectrum, treatment, and outcome of children with primary ependymoma of the spinal cord who were registered in the database of the pediatric German brain tumor studies Hirntumor (HIT) '91 and HIT 2000. Methods Between 1991 and 2007, 29 patients (12 male and 17 female, median age at diagnosis 13.6 years) with primary spinal cord ependymoma (myxopapillary ependymoma WHO Grade I, II, and III tumors in 6, 17, and 6 patients, respectively) were identified. Four patients had neurofibromatosis Type 2. Results With a median follow-up of 4.2 years (range 0.48–15 years), 28 patients (96.6%) were alive. Seven patients (24.1%) developed progressive disease or relapse, 2 after gross-total resection (GTR) and 5 after incomplete resection or biopsy. One patient with anaplastic ependymoma (WHO Grade III) died 65 months after diagnosis of disease progression. Primary adjuvant treatment (radiotherapy, chemotherapy, or both) was used in 8 (50%) of 16 patients following GTR and in 9 (82%) of 11 patients who underwent less than a GTR. Three additional patients were treated adjuvantly following progression. Estimated progression-free survival and overall survival rates at 5 years were 72.3% (95% CI 50%–86%) and 100%, respectively. Progression-free survival at 5 years is 84.4% (95% CI 50%–96%) for patients following GTR compared with 57.1% (95% CI 25%–69%) for patients who achieved a less than GTR (p = 0.088, log-rank test). A high relapse incidence (4 of 6) was observed among patients with myxopapillary ependymoma. Conclusions Gross-total resection is the mainstay of treatment for patients with primary spinal cord ependymoma and may be achieved in about 50% of the patients using modern surgical techniques. Primary adjuvant treatment was commonly used in children with spinal cord ependymoma irrespective of the extent of resection or tumor grade. The impact of adjuvant treatment on progression-free and overall survival has to be investigated in a prospective trial.

Posterior fossa meningiomas: perioperative predictors of extent of resection, overall survival and progression-free survival

2019 ◽  
Vol 161 (5) ◽  
pp. 1003-1011 ◽  
Author(s):  
Marco V. Corniola ◽  
Jean-Michel Lemée ◽  
Michele Da Broi ◽  
Holger Joswig ◽  
Karl Schaller ◽  
...  
Keyword(s):  
Overall Survival ◽  
Posterior Fossa ◽  
Progression Free Survival ◽  
Extent Of Resection ◽  
Free Survival

scholarly journals Clinical Outcome After Microsurgical Resection of Central Neurocytoma: A Single-Centre Analysis of 15 Years

2021 ◽  
Vol 12 ◽  
Author(s):  
Dan Cao ◽  
Yong Chen ◽  
Zhengqian Guo ◽  
Yibo Ou ◽  
Jian Chen
Keyword(s):  
Overall Survival ◽  
Progression Free Survival ◽  
Extent Of Resection ◽  
Central Neurocytoma ◽  
Complication Rates ◽  
Long Term Outcome ◽  
Free Survival ◽  
Risk Of Death

Objective: This study aimed to explore the immediate postoperative and long-term outcomes of central neurocytoma (CN) based on 15 years of experience in our institution.Methods: This single-institution study collected data of 43 patients with CN who underwent surgery between 2005 and 2020. We reviewed data of clinical, immediate postoperative outcome, and long-term outcome of patients. More specifically, we divided complications into neurological and regional complications groups.Results: Among the 43 patients with CN who underwent surgery, the transcortical (72.1%) or transcallosal (25.6%) approach was used. There were 18 patients (41.9%) who complained about postoperative neurological complications, including motor weakness (25.6%), memory deficit (18.6%), aphasia (7.0%), and seizure (4.7%). In addition, 18 patients suffered postoperative regional complications such as hydrocephalus (2.3%), hematoma (34.9%), infection (4.7%), and subcutaneous hydrops (2.3%). Only one-quarter of patients had suffered permanent surgical complications. The majority of patients recovered from the deficit and could turn back to normal life. There were no significant differences in the clinical outcomes between transcortical and transcallosal approaches. At a median follow-up of 61.8 months, the 5-year overall survival and progression-free survival were 87.0 and 74.0%, respectively. A multivariate Cox model analysis showed that the extent of resection was not related to progression-free survival. However, the extent of resection was significantly associated with overall survival, and gross total resection decreased the risk of death.Conclusions: Patients with CN show favorable outcomes after surgery. The transcortical and transcallosal approaches have similar postoperative complication rates and long-term follow-up outcomes. In terms of long-term prognosis, maximal safety resection should be the first choice of CN.

scholarly journals Histological Aspects and Quantitative Assessment of Ki67 as Prognostic Factors in Breast Cancer Patients: Result from a Single-Center, Cross Sectional Study

Medicina ◽  
2020 ◽  
Vol 56 (11) ◽  
pp. 600
Author(s):  
Irina Niță ◽  
Cornelia Nițipir ◽  
Ștefania Andreea Toma ◽  
Alexandra Maria Limbău ◽  
Edvina Pîrvu ◽  
...  
Keyword(s):  
Breast Cancer ◽  
Overall Survival ◽  
Progression Free Survival ◽  
Tumor Grade ◽  
Cross Sectional Study ◽  
Histological Type ◽  
Breast Cancer Patients ◽  
Cross Sectional ◽  
Free Survival ◽  
Prognostic Assessment

Background and objectives: Our aim is to explore the relationship between the levels of protein encoded by Ki67 and the histopathological aspects regarding the overall survival and progression-free survival in a single university center. A secondary objective was to examine other factors that can influence these endpoints. New approaches to the prognostic assessment of breast cancer have come from molecular profiling studies. Ki67 is a nuclear protein associated with cell proliferation. Together with the histological type and tumor grade, it is used to appreciate the aggressiveness of the breast tumors. Materials and Methods: We conducted a retrospective single-institution study, at Elias University Emergency Hospital, Bucharest, Romania, in which we enrolled women with stage I to III breast cancer. The protocol was amended to include the immunohistochemistry determination of Ki67 and the histological aspects. The methodology consisted in using a Kaplan–Meier analysis for the entire sample and restricted mean survival time up to 36 months. Results: Both lower Ki67 and low tumor grade are associated with better prognosis in terms of overall survival (OS) and progression-free survival (PFS) for our patients’ cohort. In our group, the histological type did not impact the time to progression or survival. Conclusions: Both overall survival and progression-free survival may be influenced by the higher value of Ki67 and less differentiated tumors. Further studies are needed in order to establish if the histologic type may impact breast cancer prognostic, probably together with other histologic and molecular markers.

scholarly journals P14.116 Lack of correlation between incidental ipsilateral subventricular zone radiation dose and overall survival in glioblastoma patients

2019 ◽  
Vol 21 (Supplement_3) ◽  
pp. iii95-iii96
Author(s):  
G Hallaert ◽  
H Pinson ◽  
D Vanhauwaert ◽  
L Staelens ◽  
C Vandenbroecke ◽  
...  
Keyword(s):  
Overall Survival ◽  
Radiation Dose ◽  
Subventricular Zone ◽  
Cox Regression ◽  
Extent Of Resection ◽  
Gross Total Resection ◽  
Karnofsky Performance Score ◽  
Mgmt Methylation ◽  
Gene Methylation ◽  
Total Resection

Abstract BACKGROUND The role of the subventricular zone (SVZ) in glioblastoma (GBM) is controversial. The past decade, several retrospective studies were published concerning the potential correlation between incidental radiation of the SVZ and survival in GBM patients. Although these publications showed conflicting results, a large study claimed an overall survival (OS) benefit for GBM patients after gross total resection if the ipsilateral SVZ received a higher dose than 40 Gy. We investigated this finding in our own population of GBM patients. MATERIAL AND METHODS A multicenter retrospective study was conducted including all adult patients treated for histologically proven GBM from 2003–2014. All patients received 60 Gy radiation therapy after surgery and concomitant temozolomide. Exclusion criteria were: infratentorial GBM; presence of other neoplasm(s); known previous history of low grade glioma; incomplete radiotherapy data. Demographic data were collected from the patient charts. O6-methylguanin-DNA-methyltransferase-promotor-gene (MGMT) methylation was determined on stored tumor samples using semi quantitative methylation-specific polymerase chain reaction (qMSP). SVZs (ipsilateral, contralateral and bilateral) were contoured on radiotherapy treatment plans. Multivariate Cox regression analysis was used to study the correlation between incidental SVZ radiation dose and OS. Age (cut-off 65 years), Karnofsky Performance Score (KPS; cut-off 70), methylation of the MGMT-promotor gene and extent of resection (biopsy; subtotal resection, groos total resection) were used as covariates. Patients alive at time of database closure were censored for analysis. RESULTS 183 patients were eligible for analysis. Mean age at diagnosis was 62 years, with an average KPS of 70. In 34% of patients, gross total resection (GTR) was achieved, while in 28% only a biopsy was taken. MGMT-promoter gene methylation was present in 39% of cases. Median ipsilateral, contralateral and bilateral SVZ doses were 46.1 Gy, 25.35 Gy and 34.8 Gy resp. In multivariate Cox regression, all covariates (age, P = 0.011; KPS, P = 0.001; MGMT methylation, P = 0.000; extent of resection, P = 0.000) were significantly associated with OS. Mean OS was 23 months, but median OS 13 months. There was no correlation between incidental radiation dose of the ipsilateral SVZ and OS for 46 Gy or 40 Gy (hazard ratio 0.82 (0.6–1.1), P = 0.225 and 0.89 (0.63–1.23), P = 0.52 resp.) for the whole group nor for the subgroup of gross total resection. CONCLUSION In this group of GBM patients, age, KPS, extent of resection and methylation of the MGMT-promotor gene were significantly correlated with OS, but not incidental ipsilateral SVZ radiation dose. The previously published positive results may result from bias, possibly arising from lack of inclusion of MGMT-promotor gene methylation as an important independent prognostic factor.

Spinal ependymoma in adults: a multicenter investigation of surgical outcome and progression-free survival

2018 ◽  
Vol 28 (6) ◽  
pp. 654-662 ◽  
Author(s):  
Maria Wostrack ◽  
Florian Ringel ◽  
Sven O. Eicker ◽  
Max Jägersberg ◽  
Karl Schaller ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Progression Free Survival ◽  
Adult Patients ◽  
Gross Total Resection ◽  
Adjuvant Radiation ◽  
Total Resection ◽  
Ki 67 ◽  
Who Grade ◽  
Free Survival ◽  
Spinal Ependymoma

OBJECTIVESpinal ependymomas are rare glial neoplasms. Because their incidence is low, only a few larger studies have investigated this condition. There are no clear data concerning prognosis and therapy. The aim of the study was to describe the natural history, perioperative clinical course, and local tumor control of adult patients with spinal ependymomas who were surgically treated under modern treatment standards.METHODSThe authors performed a multicenter retrospective study. They identified 158 adult patients with spinal ependymomas who had received surgical treatment between January 2006 and June 2013. The authors analyzed the clinical and histological aspects of these cases to identify the predictive factors for postoperative morbidity, tumor resectability, and recurrence.RESULTSGross-total resection (GTR) was achieved in 80% of cases. At discharge, 37% of the patients showed a neurological decline. During follow-up the majority recovered, whereas 76% showed at least preoperative status. Permanent functional deterioration remained in 2% of the patients. Transient deficits were more frequent in patients with cervically located ependymomas (p = 0.004) and in older patients (p = 0.002). Permanent deficits were independently predicted only by older age (p = 0.026). Tumor progression was observed in 15 cases. The 5-year progression-free survival (PFS) rate was 80%, and GTR (p = 0.037), WHO grade II (p = 0.009), and low Ki-67 index (p = 0.005) were independent prognostic factors for PFS. Adjuvant radiation therapy was performed in 15 cases. No statistically relevant effects of radiation therapy were observed among patients with incompletely resected ependymomas (p = 0.079).CONCLUSIONSDue to its beneficial value for PFS, GTR is important in the treatment of spinal ependymoma. Gross-total resection is feasible in the majority of cases, with acceptable rates of permanent deficits. Also, Ki-67 appears to be an important prognostic factor and should be included in a grading scheme for spinal ependymomas.

scholarly journals STMO-6 Impact of the extent of resection on the survival of patients with lower grade gliomas using awake brain mapping

2021 ◽  
Vol 3 (Supplement_6) ◽  
pp. vi12-vi13
Author(s):  
Kazuya Motomura ◽  
Lushun Chalise ◽  
Fumiharu Ohka ◽  
Kosuke Aoki ◽  
Tomohide Nishikawa ◽  
...  
Keyword(s):  
Brain Mapping ◽  
Progression Free Survival ◽  
Extent Of Resection ◽  
Gross Total Resection ◽  
Lower Grade ◽  
Wild Type ◽  
Total Resection ◽  
Mutant Type ◽  
Who Grade ◽  
Supratotal Resection

Abstract Purpose: The aim of this study was to assess the effect of the extent of resection (EOR) of tumors on survival in a series of patients with lower-grade gliomas (LGGs) who underwent awake brain mapping. Methods: We retrospectively analyzed 126 patients with LGGs in the dominant and non-dominant hemisphere who underwent awake brain surgery at the same institution between December 2012 and May 2020. Results: The median progression-free survival (PFS) rate of patients with LGGs in the group with an EOR >100 %, including supratotal resection (n = 47; median survival [MS], not reached), was significantly higher than that in the group with an EOR <100% (n = 79; MS, 43.1 months; 95% CI: 37.8–48.4 months; p = 0.04). In patients with diffuse astrocytomas and anaplastic astrocytomas, the group with EOR >100 %, including supratotal resection (n = 25; MS, not reached), demonstrated a significantly better PFS rate than did the group with an EOR <100% (n = 45; MS, 35.8 months; 95% CI: 19.9–51.6 months; p = 0.03). Supratotal or gross total resection was correlated with better PFS in IDH-mutant type of diffuse astrocytomas and anaplastic astrocytomas (n = 19; MS, not reached vs. n = 35; MS, 40.6 months; 95% CI: 22.3–59.0 months; p = 0.02). By contrast, supratotal or gross total resection was not associated with longer PFS rates in patients with IDH-wild type of diffuse astrocytomas and anaplastic astrocytomas. Conclusions: It is noteworthy that supratotal or gross total resection significantly correlated with better PFS in IDH-mutant type of WHO grade II and III astrocytic tumors. In light of our finding that EOR did not correlate with PFS in patients with aggressive IDH-wild type of diffuse astrocytomas and anaplastic astrocytomas, we suggest treatments that are more intensive will be needed for the control of these tumors.

Sign in / Sign up

Export Citation Format

Share Document