The challenges in treating embryonal tumors with multilayered rosettes (ETMR) and other infant brain tumors

2021 ◽  
Author(s):  
Pratiti Bandopadhayay ◽  
Susan N Chi
1992 ◽  
Vol 3 (4) ◽  
pp. 781-789 ◽  
Author(s):  
J. Russell Geyer
Keyword(s):  

2021 ◽  
Vol 10 (10) ◽  
pp. 2219
Author(s):  
Monika Prill ◽  
Agnieszka Karkucinska-Wieckowska ◽  
Magdalena Lebiedzinska-Arciszewska ◽  
Giampaolo Morciano ◽  
Agata Charzynska ◽  
...  

Numerous papers have reported altered expression patterns of Ras and/or ShcA proteins in different types of cancers. Their level can be potentially associated with oncogenic processes. We analyzed samples of pediatric brain tumors reflecting different groups such as choroid plexus tumors, diffuse astrocytic and oligodendroglial tumors, embryonal tumors, ependymal tumors, and other astrocytic tumors as well as tumor malignancy grade, in order to characterize the expression profile of Ras, TrkB, and three isoforms of ShcA, namely, p66Shc, p52Shc, and p46Shc proteins. The main aim of our study was to evaluate the potential correlation between the type of pediatric brain tumors, tumor malignancy grade, and the expression patterns of the investigated proteins.


2012 ◽  
Vol 43 (10) ◽  
pp. 1668-1676 ◽  
Author(s):  
Christopher Dunham ◽  
Shibu Pillai ◽  
Paul Steinbok

2018 ◽  
Vol 5 (4) ◽  
pp. 96 ◽  
Author(s):  
Anders Bailey ◽  
Amreena Suri ◽  
Pauline Chou ◽  
Tatiana Pundy ◽  
Samantha Gadd ◽  
...  

Neuroblastoma (NB) is the most common extracranial solid tumor in pediatrics, with rare occurrences of primary and metastatic tumors in the central nervous system (CNS). We previously reported the overexpression of the polo-like kinase 4 (PLK4) in embryonal brain tumors. PLK4 has also been found to be overexpressed in a variety of peripheral adult tumors and recently in peripheral NB. Here, we investigated PLK4 expression in NBs of the CNS (CNS-NB) and validated our findings by performing a multi-platform transcriptomic meta-analysis using publicly available data. We evaluated the PLK4 expression by quantitative real-time PCR (qRT-PCR) on the CNS-NB samples and compared the relative expression levels among other embryonal and non-embryonal brain tumors. The relative PLK4 expression levels of the NB samples were found to be significantly higher than the non-embryonal brain tumors (p-value < 0.0001 in both our samples and in public databases). Here, we expand upon our previous work that detected PLK4 overexpression in pediatric embryonal tumors to include CNS-NB. As we previously reported, inhibiting PLK4 in embryonal tumors led to decreased tumor cell proliferation, survival, invasion and migration in vitro and tumor growth in vivo, and therefore PLK4 may be a potential new therapeutic approach to CNS-NB.


2012 ◽  
Vol 84 (3) ◽  
pp. S643-S644
Author(s):  
J. Peacock ◽  
N.N. Laack ◽  
C. Nwachukwu ◽  
C.J. Beltran

2021 ◽  
Author(s):  
wei yang ◽  
Yingjie Cai ◽  
Jiashu Chen ◽  
Ping Yang ◽  
Zesheng Ying ◽  
...  

Abstract Objective Brain tumors have become the most common solid tumors in children. The epidemiology is poorly described in China. This retrospective study aimed to describe the epidemiologic characteristics, clinical presentation, and prognosis in national health center for children. Method From January 2015 to December 2020, 484 cases age 0-18 years old diagnosed with brain tumors and receiving neurosurgery treatment were enrolled into the database. Pathology was based on the World Health Organization 2012 nervous system tumor classification, and tumor behavior were classified on International Classification of Diseases for Oncology, third edition. A descriptive and comparative statistic was performed on clinical manifestation, symptom duration, sex, age, tumor location, tumor behavior, and survival time. Results Among the 484 brain tumors, the median age at diagnosis was 4.62 [2.19, 8.17] years old (4.07 [1.64, 7.13] for benign tumors and 5.36 [2.78, 8.84] for malignant tumors). The overall male to female ratios were 1.33:1, with 1.09:1 and 1.62:1 for benign and malignant tumors respectively. Nausea and vomiting, headache were the most frequent initial symptoms. The median symptoms duration was 4[2, 21] weeks. The three most frequent tumor type were embryonal tumors (22.45%), other astrocytic tumors (20.17%), and diffuse astrocytic tumors (11.02%). Supratentorial tumors comprise 57.38% of all brain tumors. And the most common tumor locations were cerebellum and forth ventricles (38.67%), sellar region (22.87%) and ventricles (10.60%). Male were more common among choroid plexus tumors (63.64%), embryonal tumors (61.11%), ependymal tumors (68.57%), and germ cell tumors (78.13%). Patients were followed for 1 to 82 months. The overall 5-year survival was 77.4%, with 90.0% for benign tumor and 65.3% for malignant tumors. log-rank test found significant different at p <0.001 level. Conclusion Brain tumors presented particularly sexual, age dependent, and regional dependent epidemiological characteristic. Our results were consistent with previous reports, and might reflect the real epidemiology status in China.


2019 ◽  
pp. 1-25 ◽  
Author(s):  
Fabio Girardi ◽  
Claudia Allemani ◽  
Michel P. Coleman

PURPOSE The histology of brain tumors determines treatment and predicts outcome. Population-based survival reflects the effectiveness of a health care system in managing cancer. No systematic review of worldwide variation and time trends in survival from brain tumors in children is currently available. PATIENTS AND METHODS We considered longitudinal, observational studies comprising children diagnosed with intracranial astrocytic or embryonal tumors. We searched six electronic databases from database inception to September 30, 2018, using complex search strategies. The outcome measure was 5-year survival, estimated through a time-to-event analysis. This study is registered with PROSPERO, number CRD42018111981. RESULTS Among 5,244 studies, we identified 47 eligible articles that provided 228 survival estimates. Only five studies were entirely or partially conducted in low-income or middle-income countries. Five-year survival from embryonal tumors increased from 37% in 1980 to approximately 60% in 2009. Although survival for medulloblastoma improved substantially (from 29% to 73% during 1959-2009), survival for primitive neuroectodermal tumors wavered over time (1973-2009) and between countries. Five-year survival from astrocytoma changed very little over the 27 years between 1982 and 2009 (from 78% to 89%). Interpretation of the literature was made difficult by the heterogeneity of study designs. CONCLUSION Survival has improved for embryonal tumors, but little change has been observed for astrocytic tumors. We found a striking gap in knowledge about survival from childhood brain tumor subtypes in middle-income and low-income countries, where half of these tumors are diagnosed. Larger studies are needed, including in under-represented countries and based on standardized data collection, to provide up-to-date survival estimates.


2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i30-i30
Author(s):  
Jessica Foster ◽  
Crystal Griffin ◽  
Allison Stern ◽  
Cameron Brimley ◽  
Samantha Buongervino ◽  
...  

Abstract Glypican 2 (GPC2) is a cell-surface oncoprotein initially identified in neuroblastoma, retinoblastoma, and medulloblastoma as an ideal target for immunotherapy (Cancer Cell, 2017). Here we evaluated GPC2 expression across the spectrum of pediatric brain tumors using RNA sequencing from specimens in the Children’s Brain Tumor Network (CBTN). High GPC2 expression, defined as &gt;10 FPKM, was found in 100% of embryonal tumors with multilayered rosettes (ETMRs) (n=6), 95% of medulloblastomas (n=122), 86% of other embryonal tumors (n=21), 50% of choroid plexus carcinomas (n=4), 42% of high grade gliomas (HGG) (n=117), and 37% of diffuse midline gliomas (DMG) (n=65). Within medulloblastoma subtypes, group 4 tumors had the highest expression, and within the HGG tumor cohort H3.3 G34 mutated gliomas had the highest GPC2 expression. High GPC2 protein expression was validated with medulloblastoma and HGG/DMG primary tumors and cell lines using IHC, Western blot, and flow cytometry. We next developed two potent CAR T cell constructs using the D3 specific scFv directed against GPC2 for testing in brain tumor models. GPC2-directed CAR T cells were tested in vitro against medulloblastoma and HGG cells lines, and in vivo using two patient-derived medulloblastoma xenograft models: Rcmb28 (group 3) and 7316-4509 (group 4). GPC2-directed mRNA CAR T cells induced significant GPC2-specific cell death in medulloblastoma and HGG cellular models with concomitant T cell degranulation compared to CD19-directed mRNA CAR T cells. In vivo, GPC2-directed mRNA CAR T cells delivered locoregionally induced significant tumor regression measured by bioluminescence after 4–6 intratumoral infusions of 4 x 106 CAR T cells (p&lt;0.0001 for Rcmb28, p&lt;0.05 for 7316-4509). No GPC2-directed CAR T cell related toxicity was observed. GPC2 is a highly differentially expressed cell surface protein on multiple malignant pediatric brain tumors that can be targeted safely with local delivery of mRNA CAR T cells.


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