Reference Citation Analysis: Find an Article, Find a Category, Find a Journal, Find a Scholar

For: Adams H, Adams HHH, Jackson C, Rincon-Torroella J, Jallo GI, Quiñones-Hinojosa A. Evaluating extent of resection in pediatric glioblastoma: a multiple propensity score-adjusted population-based analysis. Childs Nerv Syst 2016;32:493-503. [PMID: 26767842 DOI: 10.1007/s00381-015-3006-x] [Citation(s) in RCA: 31] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [What about the content of this article? (0)] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2014] [Accepted: 12/23/2015] [Indexed: 01/17/2023]

For:	Adams H, Adams HHH, Jackson C, Rincon-Torroella J, Jallo GI, Quiñones-Hinojosa A. Evaluating extent of resection in pediatric glioblastoma: a multiple propensity score-adjusted population-based analysis. Childs Nerv Syst 2016;32:493-503. [PMID: 26767842 DOI: 10.1007/s00381-015-3006-x] [Citation(s) in RCA: 31] [Impact Index Per Article: 3.4] [Reference Citation Analysis] [What about the content of this article? (0)] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 10/14/2014] [Accepted: 12/23/2015] [Indexed: 01/17/2023]

Number

Cited by Other Article(s)

Baik SH, Baye F, McDonald CJ. Use of menopausal hormone therapy beyond age 65 years and its effects on women's health outcomes by types, routes, and doses. Menopause 2024;31:363-371. [PMID: 38595196 PMCID: PMC11465799 DOI: 10.1097/gme.0000000000002335] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/29/2023] [Revised: 01/02/2024] [Indexed: 04/11/2024]

Abstract

OBJECTIVES

The study aims to assess the use of menopausal hormone therapy beyond age 65 years and its health implications by types of estrogen/progestogen, routes of administration, and dose strengths.

METHODS

Using prescription drug and encounter records of 10 million senior Medicare women from 2007-2020 and Cox regression analyses adjusted for time-varying characteristics of the women, we examined the effects of different preparations of menopausal hormone therapy on all-cause mortality, five cancers, six cardiovascular diseases, and dementia.

RESULTS

Compared with never use or discontinuation of menopausal hormone therapy after age 65 years, the use of estrogen monotherapy beyond age 65 years was associated with significant risk reductions in mortality (19% or adjusted hazards ratio, 0.81; 95% CI, 0.79-0.82), breast cancer (16%), lung cancer (13%), colorectal cancer (12%), congestive heart failure (CHF) (5%), venous thromboembolism (3%), atrial fibrillation (4%), acute myocardial infarction (11%), and dementia (2%). For the use of estrogen and progestogen combo-therapy, both E+ progestin and E+ progesterone were associated with increased risk of breast cancer by 10%-19%, but such risk can be mitigated using low dose of transdermal or vaginal E+ progestin. Moreover, E+ progestin exhibited significant risk reductions in endometrial cancer (45% or adjusted hazards ratio, 0.55; 95% CI, 0.50-0.60), ovarian cancer (21%), ischemic heart disease (5%), CHF (5%), and venous thromboembolism (5%), whereas E+ progesterone exhibited risk reduction only in CHF (4%).

CONCLUSIONS

Among senior Medicare women, the implications of menopausal hormone therapy use beyond age 65 years vary by types, routes, and strengths. In general, risk reductions appear to be greater with low rather than medium or high doses, vaginal or transdermal rather than oral preparations, and with E2 rather than conjugated estrogen.

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I Kh Almadhoun MK, Hattab AAW. Pediatric Glioblastoma Multiforme: A Challenging Case of Rapid Growth and Clinical Deterioration in an 11-Year-Old Female Patient. Cureus 2023;15:e47697. [PMID: 38021881 PMCID: PMC10674094 DOI: 10.7759/cureus.47697] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Accepted: 10/26/2023] [Indexed: 12/01/2023] Open

Colopi A, Fuda S, Santi S, Onorato A, Cesarini V, Salvati M, Balistreri CR, Dolci S, Guida E. Impact of age and gender on glioblastoma onset, progression, and management. Mech Ageing Dev 2023;211:111801. [PMID: 36996926 DOI: 10.1016/j.mad.2023.111801] [Citation(s) in RCA: 21] [Impact Index Per Article: 10.5] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 01/19/2023] [Revised: 03/06/2023] [Accepted: 03/20/2023] [Indexed: 03/30/2023]

Hatoum R, Chen JS, Lavergne P, Shlobin NA, Wang A, Elkaim LM, Dodin P, Couturier CP, Ibrahim GM, Fallah A, Venne D, Perreault S, Wang AC, Jabado N, Dudley RWR, Weil AG. Extent of Tumor Resection and Survival in Pediatric Patients With High-Grade Gliomas: A Systematic Review and Meta-analysis. JAMA Netw Open 2022;5:e2226551. [PMID: 35972743 PMCID: PMC9382445 DOI: 10.1001/jamanetworkopen.2022.26551] [Citation(s) in RCA: 15] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Figures] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/01/2022] Open

Abstract

IMPORTANCE

Pediatric patients with high-grade gliomas have a poor prognosis. The association among the extent of resection, tumor location, and survival in these patients remains unclear.

OBJECTIVE

To ascertain whether gross total resection (GTR) in hemispheric, midline, or infratentorial pediatric high-grade gliomas (pHGGs) is independently associated with survival differences compared with subtotal resection (STR) and biopsy at 1 year and 2 years after tumor resection.

DATA SOURCES

PubMed, EBMR, Embase, and MEDLINE were systematically reviewed from inception to June 3, 2022, using the keywords high-grade glioma, pediatric, and surgery. No period or language restrictions were applied.

STUDY SELECTION

Randomized clinical trials and cohort studies of pHGGs that stratified patients by extent of resection and reported postoperative survival were included for study-level and individual patient data meta-analyses.

DATA EXTRACTION AND SYNTHESIS

Study characteristics and mortality rates were extracted from each article. Relative risk ratios (RRs) were pooled using random-effects models. Individual patient data were evaluated using multivariate mixed-effects Cox proportional hazards regression modeling. The PRISMA reporting guideline was followed, and the study was registered a priori.

MAIN OUTCOMES AND MEASURES

Hazard ratios (HRs) and RRs were extracted to indicate associations among extent of resection, 1-year and 2-year postoperative mortality, and overall survival.

RESULTS

A total of 37 studies with 1387 unique patients with pHGGs were included. In study-level meta-analysis, GTR had a lower mortality risk than STR at 1 year (RR, 0.69; 95% CI, 0.56-0.83; P < .001) and 2 years (RR, 0.74; 95% CI, 0.67-0.83; P < .001) after tumor resection. Subtotal resection was not associated with differential survival compared with biopsy at 1 year (RR, 0.82; 95% CI, 0.66-1.01; P = .07) but had decreased mortality risk at 2 years (RR, 0.89; 95% CI, 0.82-0.97; P = .01). The individual patient data meta-analysis of 27 articles included 427 patients (mean [SD] age at diagnosis, 9.3 [5.9] years), most of whom were boys (169 of 317 [53.3%]), had grade IV tumors (246 of 427 [57.7%]), and/or had tumors that were localized to either the cerebral hemispheres (133 of 349 [38.1%]) or midline structures (132 of 349 [37.8%]). In the multivariate Cox proportional hazards regression model, STR (HR, 1.91; 95% CI, 1.34-2.74; P < .001) and biopsy (HR, 2.10; 95% CI, 1.43-3.07; P < .001) had shortened overall survival compared with GTR but no survival differences between them (HR, 0.91; 95% CI, 0.67-1.24; P = .56). Gross total resection was associated with prolonged survival compared with STR for hemispheric (HR, 0.29; 95% CI, 0.15-0.54; P < .001) and infratentorial (HR, 0.44; 95% CI, 0.24-0.83; P = .01) tumors but not midline tumors (HR, 0.63; 95% CI, 0.34-1.19; P = .16).

CONCLUSIONS AND RELEVANCE

Results of this study show that, among patients with pHGG, GTR is independently associated with better overall survival compared with STR and biopsy, especially among patients with hemispheric and infratentorial tumors, and support the pursuit of maximal safe resection in the treatment of pHGGs.

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Affiliation(s)

Rami Hatoum University of Montréal School of Medicine, Montréal, Quebec, Canada
Jia-Shu Chen The Warren Alpert Medical School of Brown University, Providence, Rhode Island
Pascal Lavergne Department of Neurological Surgery, University of Washington, Seattle
Nathan A. Shlobin Department of Neurological Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois
Andrew Wang Department of Neurosurgery, David Geffen School of Medicine at UCLA (University of California, Los Angeles) College of Medicine, Charles R. Drew University of Medicine and Science, Los Angeles, California
Lior M. Elkaim Division of Neurology and Neurosurgery, McGill University, McGill University Health Center, Montreal, Quebec, Canada
Philippe Dodin Medical Library, Centre Hospitalier Universitaire (CHU) Sainte-Justine Children’s, Montréal, Quebec, Canada
Charles P. Couturier Department of Neurology and Neurosurgery, Montréal Neurological Institute–Hospital, Montréal, Quebec, Canada
George M. Ibrahim Division of Neurosurgery, The Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
Aria Fallah Department of Neurosurgery, David Geffen School of Medicine at UCLA (University of California, Los Angeles) Department of Pediatrics, David Geffen School of Medicine at UCLA
Dominic Venne Division of Neurosurgery, Ste Justine Hospital, University of Montréal, Montréal, Quebec, Canada
Sebastien Perreault Department of Neurology, CHU Sainte-Justine, Montréal, Quebec, Canada
Anthony C. Wang Department of Neurosurgery, David Geffen School of Medicine at UCLA (University of California, Los Angeles) Department of Pediatrics, David Geffen School of Medicine at UCLA
Nada Jabado Department of Human Genetics, McGill University, Montréal, Quebec, Canada Department of Pediatrics, McGill University and McGill University Health Centre Research Institute, Montréal, Quebec, Canada
Roy W. R. Dudley Neurosurgery Service, Department of Surgery, McGill University and McGill University Health Centre Research Institute, Montréal, Quebec, Canada
Alexander G. Weil Division of Neurosurgery, Ste Justine Hospital, University of Montréal, Montréal, Quebec, Canada Neurosurgery Service, Department of Surgery, University of Montreal Hospital Center, Montréal, Quebec, Canada Sainte-Justine University Hospital Research Center, Montréal, Quebec, Canada

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Molecular landscape of pediatric type IDH wildtype, H3 wildtype hemispheric glioblastomas. J Transl Med 2022;102:731-740. [PMID: 35332262 DOI: 10.1038/s41374-022-00769-9] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/02/2021] [Revised: 02/06/2022] [Accepted: 02/08/2022] [Indexed: 11/08/2022] Open

Abstract

The WHO (2021) Classification classified a group of pediatric-type high-grade gliomas as IDH wildtype, H3 wildtype but as of currently, they are characterized only by negative molecular features of IDH and H3. We recruited 35 cases of pediatric IDH wildtype and H3 wildtype hemispheric glioblastomas. We evaluated them with genome-wide methylation profiling, targeted sequencing, RNAseq, TERT promoter sequencing, and FISH. The median survival of the cohort was 27.6 months. With Capper et al.'s³⁶ methylation groups as a map, the cases were found to be epigenetically heterogeneous and were clustered in proximity or overlay of methylation groups PXA-like (n = 8), LGG-like (n = 10), GBM_MYCN (n = 9), GBM_midline (n = 5), and GBM_RTKIII (n = 3). Histology of the tumors in these groups was not different from regular glioblastomas. Methylation groups were not associated with OS. We were unable to identify groups specifically characterized by EGFR or PDGFRA amplification as proposed by other authors. EGFR, PDGFRA, and MYCN amplifications were not correlated with OS. 4/9 cases of the GBM_MYCN cluster did not show MYCN amplification; the group was also enriched for EGFR amplification (4/9 cases) and the two biomarkers overlapped in two cases. Overall, PDGFRA amplification was found in only four cases and they were not restricted to any groups. Cases in proximity to GBM_midline were all hemispheric and showed loss of H3K27me3 staining. Fusion genes ALK/NTRK/ROS1/MET characteristic of infantile glioblastomas were not identified in 17 cases successfully sequenced. BRAF V600E was only found in the PXA group but CDKN2A deletion could be found in other methylation groups. PXA-like cases did not show PXA histological features similar to findings by other authors. No case showed TERT promoter mutation. Mutations of mismatch repair (MMR) genes were poor prognosticators in single (p ≤ 0.001) but not in multivariate analyses (p = 0.229). MGMT had no survival significance in this cohort. Of the other common biomarkers, only TP53 and ATRX mutations were significant poor prognosticators and only TP53 mutation was significant after multivariate analyses (p = 0.024). We conclude that IDH wildtype, H3 wildtype pediatric hemispheric glioblastomas are molecularly heterogeneous and in routine practice, TP53, ATRX, and MMR status could profitably be screened for risk stratification in laboratories without ready access to methylation profiling.

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Outcomes of Lymphoma Among American Adolescent and Young Adult Patients Varied by Health Insurance-A SEER-based Study. J Pediatr Hematol Oncol 2022;44:e403-e412. [PMID: 34486562 DOI: 10.1097/mph.0000000000002314] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/07/2021] [Accepted: 08/06/2021] [Indexed: 11/26/2022]

Abstract

INTRODUCTION

Impacts of health insurance status on survival outcomes among adolescent and young adult (AYA, 15 to 39 years of age) patients with lymphoma in the United States are insufficiently known. This study aimed to clarify associations between health insurance status and overall survival (OS) estimates in this population.

MATERIALS AND METHODS

We examined 18 Surveillance, Epidemiology, and End Results registries in the United States and analyzed American AYA patients with lymphoma diagnosed during January 2007 and December 2016. Health insurance status was categorized, and Kaplan-Meier and multifactor Cox regressions were adopted using hazard ratio and 95% confidence interval. Probable baseline confounding was modulated by multiple propensity score.

RESULTS

A total of 21,149 patients were considered; ~28% were 18 to 25 years old, and 63.5% and 7.5% had private and no insurance, respectively. Private insurance rates increased in the 18 to 25 age group (60.1% to 6.1%, P<0.001) following the 2010 Patient Protection and Affordable Care Act (ACA), and lymphoma survival rates improved slightly 1 to 5 years postdiagnosis. Five-year OS rates decreased with age (93.9%, 90.4%, and 87.0% at 15 to 17, 18 to 25, and 26 to 39, respectively) and differed among insurance conditions (81.7%, 79.2%, 89.2%, and 92.0% for uninsured, Medicaid, insured, and insured/no specifics, respectively). Risk of death was significantly higher for those with Medicaid or no insurance than for those with private insurance in multiple propensity score-adjusted models (hazard ratio [95% confidence interval]=1.07 [1.03-1.12]), independent of stage at diagnosis.

CONCLUSIONS

No or insufficient insurance was linked to poor OS in our sample in exposure-outcome association analysis. Insurance coverage and health care availability may enhance disparate outcomes of AYAs with cancer. The ACA has improved insurance coverage and survival rates for out sample. Nevertheless, strategies are needed to identify causality and eliminate disparities.

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Owens MR, Nguyen S, Karsy M. Utility of Administrative Databases and Big Data on Understanding Glioma Treatment—A Systematic Review. INDIAN JOURNAL OF NEUROSURGERY 2022. [DOI: 10.1055/s-0042-1742333] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/19/2022] Open

Abstract Abstract Background Gliomas are a heterogeneous group of tumors where large multicenter clinical and genetic studies have become increasingly popular in their understanding. We reviewed and analyzed the findings from large databases in gliomas, seeking to understand clinically relevant information. Methods A systematic review was performed for gliomas studied using large administrative databases up to January 2020 (e.g., National Inpatient Sample [NIS], National Surgical Quality Improvement Program [NSQIP], and Surveillance, Epidemiology, and End Results Program [SEER], National Cancer Database [NCDB], and others). Results Out of 390 screened studies, 122 were analyzed. Studies included a wide range of gliomas including low- and high-grade gliomas. The SEER database (n = 83) was the most used database followed by NCDB (n = 28). The most common pathologies included glioblastoma multiforme (GBM) (n = 67), with the next category including mixes of grades II to IV glioma (n = 31). Common study themes involved evaluation of descriptive epidemiological trends, prognostic factors, comparison of different pathologies, and evaluation of outcome trends over time. Persistent health care disparities in patient outcomes were frequently seen depending on race, marital status, insurance status, hospital volume, and location, which did not change over time. Most studies showed improvement in survival because of advances in surgical and adjuvant treatments. Conclusions This study helps summarize the use of clinical administrative databases in gliomas research, informing on socioeconomic issues, surgical outcomes, and adjuvant treatments over time on a national level. Large databases allow for some study questions that would not be possible with single institution data; however, limitations remain in data curation, analysis, and reporting methods. Collapse

Al Shudifat A, Al Suqi H, Soub K, Al Nemrawi L, Abu Jaber M, Al Barbarawi M, Shewaikani N, El Adwan Y, Al Refaei A. AB Blood Group Confers Higher Risk for Primary Brain Tumors in Pediatrics. Risk Manag Healthc Policy 2021;14:4031-4035. [PMID: 34611451 PMCID: PMC8486269 DOI: 10.2147/rmhp.s322546] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/15/2021] [Accepted: 09/08/2021] [Indexed: 11/23/2022] Open

Jiao Y, Wang M, Liu X, Wang J, Wang Z, Luo W, Yu Y, Sun H. Clinical Features and Prognostic Factors of Pediatric Glioblastoma: Report of 38 Cases. World Neurosurg 2021;153:e105-e111. [PMID: 34129988 DOI: 10.1016/j.wneu.2021.06.033] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/05/2021] [Revised: 06/06/2021] [Accepted: 06/07/2021] [Indexed: 10/21/2022]

Abstract

OBJECTIVE

To better characterize children with glioblastoma, assess outcomes, and identify prognostic factors associated with overall survival and progression-free survival in a relatively large cohort from a single institution.

METHODS

For this retrospective review, 38 pediatric patients with a diagnosis of glioblastoma who were treated at The First Affiliated Hospital of Zhengzhou University between January 2015 and January 2020 were selected. Clinical and pathological characteristics, imaging, treatment, and survival variables were compared.

RESULTS

There were 24 boys and 14 girls with a median age of 11.5 years (range, 3-18 years). All patients underwent surgery, with gross total resection in 16 and subtotal resection in 22. Of patients, 18 received radiation combined with chemotherapy, 6 received radiation or chemotherapy alone, and 14 did not receive any adjuvant therapy. Contrast-enhanced magnetic resonance imaging of 21 patients showed rim enhancement, while heterogeneous enhancement was shown on imaging of the other 17 patients. Tumors were observed in hemispheric locations in 19 cases and in central locations in the others. Median overall survival was 10.5 months with a median progression-free survival of 6 months. Extent of resection, adjuvant therapy, and original site of tumor were identified as independent predictors for progression-free survival and overall survival on multivariate analysis. There were significant differences in prognosis among different enhancement characteristics; patients with rim-enhancing tumors had a better prognosis.

CONCLUSIONS

Pediatric glioblastoma carries a dismal prognosis. Maximum safe resection followed by adjuvant radiation with chemotherapy is considered standard treatment. Better outcomes are associated with hemispheric tumor locations and rim enhancement on magnetic resonance imaging.

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Sager O, Dincoglan F, Demiral S, Uysal B, Gamsiz H, Colak O, Ozcan F, Gundem E, Elcim Y, Dirican B, Beyzadeoglu M. Concise review of stereotactic irradiation for pediatric glial neoplasms: Current concepts and future directions. World J Methodol 2021;11:61-74. [PMID: 34026579 PMCID: PMC8127424 DOI: 10.5662/wjm.v11.i3.61] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/17/2020] [Revised: 04/07/2021] [Accepted: 04/14/2021] [Indexed: 02/06/2023] Open

Abstract

Brain tumors, which are among the most common solid tumors in childhood, remain a leading cause of cancer-related mortality in pediatric population. Gliomas, which may be broadly categorized as low grade glioma and high grade glioma, account for the majority of brain tumors in children. Expectant management, surgery, radiation therapy (RT), chemotherapy, targeted therapy or combinations of these modalities may be used for management of pediatric gliomas. Several patient, tumor and treatment-related characteristics including age, lesion size, grade, location, phenotypic and genotypic features, symptomatology, predicted outcomes and toxicity profile of available therapeutic options should be considered in decision making for optimal treatment. Management of pediatric gliomas poses a formidable challenge to the physicians due to concerns about treatment induced toxicity. Adverse effects of therapy may include neurological deficits, hemiparesis, dysphagia, ataxia, spasticity, endocrine sequelae, neurocognitive and communication impairment, deterioration in quality of life, adverse socioeconomic consequences, and secondary cancers. Nevertheless, improved understanding of molecular pathology and technological advancements may pave the way for progress in management of pediatric glial neoplasms. Multidisciplinary management with close collaboration of disciplines including pediatric oncology, surgery, and radiation oncology is warranted to achieve optimal therapeutic outcomes. In the context of RT, stereotactic irradiation is a viable treatment modality for several central nervous system disorders and brain tumors. Considering the importance of minimizing adverse effects of irradiation, radiosurgery has attracted great attention for clinical applications in both adults and children. Radiosurgical applications offer great potential for improving the toxicity profile of radiation delivery by focused and precise targeting of well-defined tumors under stereotactic immobilization and image guidance. Herein, we provide a concise review of stereotactic irradiation for pediatric glial neoplasms in light of the literature.

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High-Grade Gliomas in Children-A Multi-Institutional Polish Study. Cancers (Basel) 2021;13:cancers13092062. [PMID: 33923337 PMCID: PMC8123180 DOI: 10.3390/cancers13092062] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/26/2021] [Revised: 04/07/2021] [Accepted: 04/22/2021] [Indexed: 12/21/2022] Open

Abstract

Simple Summary

High-grade gliomas constitute less than 5% of pediatric brain tumors. Due to the rarity of such a diagnosis, the lack of consensus about the best therapeutic approach, and the difficulty in conducting prospective trials; a retrospective multi-institutional analysis, such as the one presented in this article, is needed. We carried out the survival analysis of children diagnosed and treated with high-grade gliomas in seven major polish institutions. The assessment of the outcome of 82 consecutive patients with grade III and grade IV tumors was performed and showed a 5-year overall survival of only 30%. The extent of resection, immediate temozolomide-based chemotherapy, and radical radiotherapy were found as factors positively influencing survival.

Abstract

Due to the rarity of high-grade gliomas (HGG) in children, data on this topic are scarce. The study aimed to investigate the long-term results of treatment of children with HGG and to identify factors related to better survival. We performed a retrospective analysis of patients treated for HGG who had the main tumor located outside the brainstem. The evaluation of factors that correlated with better survival was performed with the Cox proportional-hazard model. Survival was estimated with the Kaplan–Meier method. The study group consisted of 82 consecutive patients. All of them underwent surgery as primary treatment. Chemotherapy was applied in 93% of children with one third treated with temozolomide. After or during the systemic treatment, 79% of them received radiotherapy with a median dose of 54 Gy. Median follow-up was 122 months, and during that time, 59 patients died. One-, 2-, 5-, and 10-year overall survival was 78%, 48%, 30% and 17%, respectively. Patients with radical (R0) resection and temozolomide-based chemotherapy had better overall survival. Progression-free survival was better in patients after R0 resection and radical radiotherapy. The best outcome in HGG patients was observed in patients after R0 resection with immediate postoperative temozolomide-based chemotherapy and radical radiotherapy.

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Singla AK, Madan R, Gupta K, Goyal S, Kumar N, Sahoo SK, Uppal DK, Ahuja CK. Clinical behaviour and outcome in pediatric glioblastoma: current scenario. Radiat Oncol J 2021;39:72-77. [PMID: 33794576 PMCID: PMC8024182 DOI: 10.3857/roj.2020.00591] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/16/2020] [Accepted: 12/22/2020] [Indexed: 11/08/2022] Open

Alimohammadi E, Bagheri SR, Delfani N, Safari-Faramani R, Janatolmakan M. Pediatric Non–Brain Stem High-Grade Glioma: A Single-Center Experience. INDIAN JOURNAL OF NEUROSURGERY 2020. [DOI: 10.1055/s-0040-1712067] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 10/24/2022] Open

Coleman C, Stoller S, Grotzer M, Stucklin AG, Nazarian J, Mueller S. Pediatric hemispheric high-grade glioma: targeting the future. Cancer Metastasis Rev 2020;39:245-260. [PMID: 31989507 DOI: 10.1007/s10555-020-09850-5] [Citation(s) in RCA: 18] [Impact Index Per Article: 3.6] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/20/2022]

Ruiz-Garcia H, Huayllani MT, Incontri D, Whaley JJ, Marenco-Hillembrand L, Ebot J, Chaichana KL, Sheehan J, Quiñones-Hinojosa A, Trifiletti DM. Intraventricular choroid plexus tumors: clinical characteristics and impact of current management on survival. J Neurooncol 2020;149:283-292. [PMID: 32897467 DOI: 10.1007/s11060-020-03603-8] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/28/2020] [Accepted: 08/23/2020] [Indexed: 11/29/2022]

Abstract

INTRODUCTION

Choroid plexus tumors (CPTs) represent one of the most common intraventricular tumors. Although most are benign, they often reach considerable sizes before clinical manifestation, challenging their surgical management. We aim to describe the clinical characteristics and the impact of current management on the survival of patients harboring intraventricular CPT.

METHODS

The National Cancer Database (NCDB) was queried to identify biopsy-proven intraventricular CPT patients (2004-2015). Demographic and patterns of care were described, the log-rank method was used to independently analyze survival according to age, WHO grade and extent of resection (EOR). Multivariate analysis was performed to investigate the impact of prognostic factors on overall survival (OS).

RESULTS

A total of 439 CPT patients with known WHO grade were included. WHO grade I tumors were more frequent in adults, while WHO grade III tumors were more common in pediatric population. Most CPTs were benign, with a median tumor size of 3-4 cm. Mean tumor size in pediatric population was greater than in adult population (4.39 cm vs. 2.7 cm; p < 0.01). Frequency was similar between males and females (51.7% vs. 48.3%; p > 0.0.5). Five- and ten-year OS among all patients was 87% and 84%, respectively. EOR was not associated with survival for any WHO grade. On multivariable analysis, only patient age (p = 0.022), WHO grade (p = 0.003) and medical comorbidity scores (p = 0.002) were independently associated with OS after diagnosis.

CONCLUSION

Patients with CPTs present at different stages of life, with sizable tumor burden and distinct WHO grade prevalence. Considering their favorable survival, efforts to improve tumor control should be meticulously weighed against the long-term risk associated with surgery, radiation, and chemotherapy.

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Wang Q, Cheng J, Si Z, Liu W, Hui X, Li Q, Ju Y. Primary cerebellar glioblastomas in children: clinical presentation and management. Neurosurg Rev 2020;44:1747-1754. [PMID: 32845414 DOI: 10.1007/s10143-020-01373-5] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/10/2020] [Revised: 08/07/2020] [Accepted: 08/18/2020] [Indexed: 02/05/2023]

Malay S, Somasundaram E, Patil N, Buerki R, Sloan A, Barnholtz-Sloan JS. Treatment and surgical factors associated with longer-term glioblastoma survival: a National Cancer Database study. Neurooncol Adv 2020;2:1-10. [PMID: 32642726 PMCID: PMC7332237 DOI: 10.1093/noajnl/vdaa070] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/14/2022] Open

Primary bulbo-medullary glioblastoma in a child: case report. Childs Nerv Syst 2019;35:2417-2421. [PMID: 31667535 DOI: 10.1007/s00381-019-04396-6] [Citation(s) in RCA: 2] [Impact Index Per Article: 0.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/07/2019] [Accepted: 09/26/2019] [Indexed: 12/23/2022]

Uppar AM, Sugur H, Prabhuraj AR, Rao MB, Devi BI, Sampath S, Arivazhagan A, Santosh V. H3K27M, IDH1, and ATRX expression in pediatric GBM and their clinical and prognostic significance. Childs Nerv Syst 2019;35:1537-1545. [PMID: 31152217 DOI: 10.1007/s00381-019-04222-z] [Citation(s) in RCA: 4] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 04/14/2018] [Accepted: 05/23/2019] [Indexed: 01/21/2023]

Abstract

PURPOSE

Pediatric glioblastoma (pGBM) tumors have been identified as an entity distinct and different from the adult variety of GBM not only with respect to pathogenesis, genetics, and molecular alterations but also in clinical outcomes and overall survival. This study aims to evaluate the immunohistochemical profile of molecular markers in pediatric GBM and correlate them with clinical features and prognosis.

MATERIALS AND METHODS

We retrospectively analyzed 29 pGBMs (age range 3 to 18 years), operated at our institute between 2009 and 2014, and evaluated their clinical and histopathological features along with the immunohistochemical expression of clinically relevant molecular markers: H3K27M, p53, ATRX, and IDH1 (R132H), and correlated their expression with clinical features. We further assessed the prognostic value of these markers in our cohort of patients.

RESULTS

The median overall survival (OS) of the cohort was 6.00 ± 0.882 months. The mean overall survival was 7.571 ± 1.118 months which was lower than in most studies. Preoperative Karnofsky Performance Score (KPS), extent of surgical resection, and adjuvant radiotherapy were found to be the clinical factors strongly influencing median survival (p < 0.05). Loss of ATRX expression was predominantly noted in hemispheric tumors (84%), while p53 staining was maximum in thalamic tumors (8 out of 9 cases). H3K27M mutant protein expression was noted in 8/9 thalamic tumors and 5/7 tumors in the brain stem-cerebellar-peduncular region. Patients with tumors showing H3K27M immunopositivity had the worst prognosis with a mean OS of 5 months ± 0.832 months, as against patients with H3K27M-immunonegative tumors, which was 10.143 ± 1.866 months(p = 0.006). Other markers like p53, ATRX, and IDH1 did not influence the prognosis in this patient cohort. ATRX loss of expression was associated with a better OS, with a trend to significance, and such an association has not been reported earlier.

CONCLUSIONS

Ours is one among the few studies from India describing the clinical parameters and evaluating the key immunohistochemical markers in pGBM and deriving their prognostic significance. The study reiterates the poor prognostic significance of H3K27M immunopositivity.

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Boudaouara O, Charfi S, Bahri M, Daoud J, Boudawara MZ, Gouiaa N, Sellami Boudawara T. Pediatric high grade gliomas: Clinico-pathological profile, therapeutic approaches and factors affecting overall survival. Neurochirurgie 2019;65:63-68. [PMID: 30922839 DOI: 10.1016/j.neuchi.2019.03.001] [Citation(s) in RCA: 3] [Impact Index Per Article: 0.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/07/2018] [Revised: 01/19/2019] [Accepted: 03/09/2019] [Indexed: 11/18/2022]

Prognostic factors and survival in low grade gliomas of the spinal cord: A population-based analysis from 2006 to 2012. J Clin Neurosci 2019;61:14-21. [DOI: 10.1016/j.jocn.2018.11.025] [Citation(s) in RCA: 15] [Impact Index Per Article: 2.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/16/2018] [Accepted: 11/05/2018] [Indexed: 11/18/2022]

ReFaey K, Tripathi S, Yoon JW, Justice J, Kerezoudis P, Parney IF, Bendok BR, Chaichana KL, Quiñones-Hinojosa A. The reliability of YouTube videos in patients education for Glioblastoma Treatment. J Clin Neurosci 2018;55:1-4. [DOI: 10.1016/j.jocn.2018.07.001] [Citation(s) in RCA: 54] [Impact Index Per Article: 7.7] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/03/2018] [Accepted: 07/08/2018] [Indexed: 11/28/2022]

Blionas A, Giakoumettis D, Klonou A, Neromyliotis E, Karydakis P, Themistocleous MS. Paediatric gliomas: diagnosis, molecular biology and management. ANNALS OF TRANSLATIONAL MEDICINE 2018;6:251. [PMID: 30069453 PMCID: PMC6046297 DOI: 10.21037/atm.2018.05.11] [Citation(s) in RCA: 21] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Subscribe] [Scholar Register] [Received: 03/14/2018] [Accepted: 05/02/2018] [Indexed: 01/14/2023]

Abstract

Paediatric gliomas represent the most common brain tumour in children. Early diagnosis and treatment greatly improve survival. Histological grade is the most significant classification system affecting treatment planning and prognosis. Paediatric gliomas depend on pathways and genes responsible for mitotic activity and cell proliferation as well as angiogenesis (MAPK, VEGF, EFGR pathways). Symptoms such as focal neurologic deficit or seizures can facilitate diagnosis, but they are not always present and therefore diagnosis is occasionally delayed. Imaging has adequate diagnostic accuracy (surpassing 90%), and novel imaging techniques such as MR spectroscopy and PET increase only slightly this percentage. Low grade gliomas (LGG) can be approached conservatively but most authors suggest surgical excision. High grade gliomas (HGG) are always operated with exception of specific contradictions including butterfly or extensive dominant hemisphere gliomas. Surgical excision is universally followed by radiotherapy and chemotherapy, which slightly increase survival. Inoperable cases can be managed with or without radiosurgery depending on location and size, with adjunctive use of radiotherapy and chemotherapy. Surgical excision must be aggressive and gross total resection (GTR) should be attempted, if possible, since it can triple survival. Radiosurgery is effective on smaller tumours of <2 cm2. Surgical excision is always the treatment of choice, but glioma recurrences, and residual tumours in non-critical locations are candidates for radiosurgery especially if tumour volume is low. Management of recurrences includes surgery, radiosurgery and chemoradiotherapy and it should be individualized according to location and size. In combination with molecular targeted therapeutic schemes, glioma management will be immensely improved in the next years.

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Liu M, Thakkar JP, Garcia CR, Dolecek TA, Wagner LM, Dressler EVM, Villano JL. National cancer database analysis of outcomes in pediatric glioblastoma. Cancer Med 2018. [PMID: 29532996 PMCID: PMC5911617 DOI: 10.1002/cam4.1404] [Citation(s) in RCA: 22] [Impact Index Per Article: 3.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 02/06/2023] Open

Fallah A, Weil AG, Juraschka K, Ibrahim GM, Wang AC, Crevier L, Tseng CH, Kulkarni AV, Ragheb J, Bhatia S. The importance of extent of choroid plexus cauterization in addition to endoscopic third ventriculostomy for infantile hydrocephalus: a retrospective North American observational study using propensity score-adjusted analysis. J Neurosurg Pediatr 2017;20:503-510. [PMID: 28984539 DOI: 10.3171/2017.7.peds16379] [Citation(s) in RCA: 10] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 01/12/2023]

Abstract

OBJECTIVE Combined endoscopic third ventriculostomy (ETC) and choroid plexus cauterization (CPC)-ETV/CPC- is being investigated to increase the rate of shunt independence in infants with hydrocephalus. The degree of CPC necessary to achieve improved rates of shunt independence is currently unknown. METHODS Using data from a single-center, retrospective, observational cohort study involving patients who underwent ETV/CPC for treatment of infantile hydrocephalus, comparative statistical analyses were performed to detect a difference in need for subsequent CSF diversion procedure in patients undergoing partial CPC (describes unilateral CPC or bilateral CPC that only extended from the foramen of Monro [FM] to the atrium on one side) or subtotal CPC (describes CPC extending from the FM to the posterior temporal horn bilaterally) using a rigid neuroendoscope. Propensity scores for extent of CPC were calculated using age and etiology. Propensity scores were used to perform 1) case-matching comparisons and 2) Cox multivariable regression, adjusting for propensity score in the unmatched cohort. Cox multivariable regression adjusting for age and etiology, but not propensity score was also performed as a third statistical technique. RESULTS Eighty-four patients who underwent ETV/CPC had sufficient data to be included in the analysis. Subtotal CPC was performed in 58 patients (69%) and partial CPC in 26 (31%). The ETV/CPC success rates at 6 and 12 months, respectively, were 49% and 41% for patients undergoing subtotal CPC and 35% and 31% for those undergoing partial CPC. Cox multivariate regression in a 48-patient cohort case-matched by propensity score demonstrated no added effect of increased extent of CPC on ETV/CPC survival (HR 0.868, 95% CI 0.422-1.789, p = 0.702). Cox multivariate regression including all patients, with adjustment for propensity score, demonstrated no effect of extent of CPC on ETV/CPC survival (HR 0.845, 95% CI 0.462-1.548, p = 0.586). Cox multivariate regression including all patients, with adjustment for age and etiology, but not propensity score, demonstrated no effect of extent of CPC on ETV/CPC survival (HR 0.908, 95% CI 0.495-1.664, p = 0.755). CONCLUSIONS Using multiple comparative statistical analyses, no difference in need for subsequent CSF diversion procedure was detected between patients in this cohort who underwent partial versus subtotal CPC. Further investigation regarding whether there is truly no difference between partial versus subtotal extent of CPC in larger patient populations and whether further gain in CPC success can be achieved with complete CPC is warranted.

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Lucas JT, Cooper DA, Hwang S, Tinkle C, Li X, Li Y, Orr B, Merchant TE, Broniscer A. Prognostic Relevance of Treatment Failure Patterns in Pediatric High-Grade Glioma: Is There a Role for a Revised Failure Classification System? Int J Radiat Oncol Biol Phys 2017;99:450-458. [DOI: 10.1016/j.ijrobp.2017.04.039] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/17/2016] [Revised: 02/24/2017] [Accepted: 04/26/2017] [Indexed: 10/19/2022]

Bodeliwala S, Kumar V, Singh D. Neonatal Brain Tumors: A Review. J Neonatal Surg 2017;6:30. [PMID: 28770127 PMCID: PMC5538596 DOI: 10.21699/jns.v6i2.579] [Citation(s) in RCA: 5] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 02/13/2017] [Accepted: 03/15/2017] [Indexed: 11/24/2022] Open

Extent of surgical resection and adjuvant temozolomide improves survival in pediatric GBM: a single center experience. Childs Nerv Syst 2017;33:951-956. [PMID: 28424876 DOI: 10.1007/s00381-017-3381-6] [Citation(s) in RCA: 9] [Impact Index Per Article: 1.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 08/23/2016] [Accepted: 03/09/2017] [Indexed: 12/31/2022]

Abstract

BACKGROUND

Pediatric glioblastoma (pGBM) is an uncommon entity. The importance of concurrent and adjuvant temozolomide is not known in this subset of patients.

METHODS

We retrospectively analyzed our database between 2000 and 2015. All patients were treated with maximally safe surgical resection. This was followed by a uniform treatment schedule of post-operative radiation with concurrent daily temozolomide at 75 mg/m². Radiation dose was 60 Gy in 30 fractions planned by 3-dimensional conformal radiotherapy. Concurrent and adjuvant temozolomide was used in all patients treated after 2007. Four weeks later, adjuvant temozolomide was started at 150 mg/m², day 1 to 5 every 28 days and escalated to 200 mg/m² from the second cycle onwards if well tolerated. Log-rank test was used to compare survival distribution. The data was analyzed using SPSS (version 16).

RESULTS

Fifty-one patients were analyzed. Median age was 14 years (range: 5 to 21 years). Thirty-five males and 16 females were noted. Median symptom duration was 4 months. Twenty-eight patients underwent a gross total resection (GTR) while 17 underwent a subtotal resection; six patients underwent decompression. Thirty-three patients received concurrent chemotherapy while 27 received adjuvant chemotherapy. Median progression-free survival (PFS) was 15.1 months. One- and 3-year PFS was 54.4% and 3-year PFS was 24.6.7%. The median overall survival was 17.4 months. In univariate analysis survival was better for gross total resection (17.4 months vs. 11.5 months; p = 0.037), and significance maintained after multivariate analysis p = 0.026, HR 3.069, 95% CI 1.14-8.23. In univariate analysis, survival was better for patients receiving temozolomide but did not achieve significance. However, in multivariate analysis, use of temozolomide was associated with significantly improved survival p = 0.036, HR 3.315, 95% CI 1.07-10.19.

CONCLUSIONS

GTR improves survival significantly in pGBM. Adjuvant temozolomide may improve survival in pGBM.

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Chakroun RW, Zhang P, Lin R, Schiapparelli P, Quinones-Hinojosa A, Cui H. Nanotherapeutic systems for local treatment of brain tumors. WILEY INTERDISCIPLINARY REVIEWS-NANOMEDICINE AND NANOBIOTECHNOLOGY 2017;10. [PMID: 28544801 DOI: 10.1002/wnan.1479] [Citation(s) in RCA: 44] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Track Full Text] [Subscribe] [Scholar Register] [Received: 06/30/2016] [Revised: 04/14/2017] [Accepted: 04/18/2017] [Indexed: 12/31/2022]

Trifiletti DM, Alonso C, Grover S, Fadul CE, Sheehan JP, Showalter TN. Prognostic Implications of Extent of Resection in Glioblastoma: Analysis from a Large Database. World Neurosurg 2017;103:330-340. [PMID: 28427986 DOI: 10.1016/j.wneu.2017.04.035] [Citation(s) in RCA: 64] [Impact Index Per Article: 8.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 03/14/2017] [Revised: 04/04/2017] [Accepted: 04/06/2017] [Indexed: 10/19/2022]

Konar SK, Bir SC, Maiti TK, Nanda A. A systematic review of overall survival in pediatric primary glioblastoma multiforme of the spinal cord. J Neurosurg Pediatr 2017;19:239-248. [PMID: 27813458 DOI: 10.3171/2016.8.peds1631] [Citation(s) in RCA: 24] [Impact Index Per Article: 3.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Indexed: 12/23/2022]

Abstract

OBJECTIVE The incidence of primary spinal cord glioblastoma multiforme (GBM) in the pediatric age group is very rare. Only a few case series and case reports have been published in the literature; therefore, overall survival (OS) outcome and the as-yet poorly defined management options are not discussed in detail. The authors performed a cumulative survival analysis of all reported cases of pediatric spinal cord GBM to identify the predictive factors related to final survival outcome. METHODS A comprehensive search for relevant articles was performed on PubMed's electronic database MEDLINE for the period from 1950 to 2015 using the search words "malignant spinal cord tumor" and "spinal glioblastoma multiforme." This study was limited to patients younger than 18 years of age. Survival rates for children with various tumor locations and treatments were collected from the published articles and analyzed. RESULTS After an extensive literature search, 29 articles met the study inclusion criteria. From the detailed information in these articles, the authors found 53 children eligible for the survival analysis. The majority (45%) of the children were more than 12 years old. Thirty-four percent of the cases were between 7 and 12 years of age, and 21% were younger than 7 years. In the Kaplan-Meier survival analysis, children younger than 7 years of age had better survival (13 months) than the children older than 7 years (7-12 years: 10 months, > 12 years: 9 months; p = 0.01, log-rank test). Fifty-five percent of the children were female and 45% were male. A cervical tumor location (32%) was the most common, followed by thoracic (28.3%). Cervicothoracic (18.9%) and conus (18.8%) tumor locations shared the same percentage of cases. Cervical tumors had a worse outcome than tumors in other locations (p = 0.003, log-rank test). The most common presenting symptom was limb weakness (53%), followed by sensory disturbances (25%). Median OS was 10 months. The addition of adjuvant therapy (radiotherapy [RT] and/or chemotherapy [CT]) after surgery significantly improved OS (p = 0.01, log-rank test). Children who underwent gross-total resection and RT had better outcomes than those who underwent subtotal resection and RT (p = 0.04, log-rank test). Cerebrospinal fluid spread, hydrocephalus, brain metastasis, and spinal metastasis were not correlated with OS in primary spinal GBM. CONCLUSIONS Adjuvant therapy after surgery had a beneficial effect on overall outcome of spinal GBM in the pediatric age group. Gross-total resection followed by RT produced a better outcome than subtotal resection with RT. Further large-scale prospective study is required to establish the genetic and molecular factors related to OS in primary GBM of the spinal cord in pediatric patients.

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