scholarly journals Pilomyxoid astrocytoma: diagnosis, prognosis, and management

2005 ◽  
Vol 18 (6) ◽  
pp. 1-4 ◽  
Author(s):  
Ricardo J. Komotar ◽  
J Mocco ◽  
Jess E. Jones ◽  
Brad E. Zacharia ◽  
Tarik Tihan ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Pilocytic Astrocytoma ◽  
Pediatric Brain Tumor ◽  
Low Grade ◽  
Current Management ◽  
Pilomyxoid Astrocytoma ◽  
Histological Features ◽  
Unusual Variant ◽  
Prognostic Implications ◽  
Pediatric Brain

Pilomyxoid astrocytoma (PMA) is a recently defined pediatric brain tumor; PMAs were previously classified within the pilocytic astrocytoma (PA) category. Nevertheless, PMA has different histological features and has been shown to behave more aggressively than PA. These findings indicate that PMA may be a unique entity that is distinct from PA, or it may be an unusual variant. To increase awareness of PMA within the neurosurgical community, the authors review the diagnostic criteria, prognostic implications, and current management of this recently described pediatric low-grade astrocytoma.

scholarly journals PATH-21. TELOMERE LENGTH ANALYSIS OF CNS TUMORS IN THE PEDIATRIC BRAIN TUMOR ATLAS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii428-iii428
Author(s):  
Jo Lynn Rokita ◽  
Krutika Gaonkar ◽  
Heba Ijaz ◽  
Daniel Miller ◽  
Tasso Karras ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Gene Mutations ◽  
Pediatric Brain Tumor ◽  
High Grade Glioma ◽  
Pediatric Brain Tumors ◽  
Low Grade ◽  
Alternative Lengthening ◽  
Length Analysis ◽  
Telomere Lengthening ◽  
Pediatric Brain

Abstract Subsets of pediatric cancers, including high grade glioma (pHGG), have high rates of uniquely long telomeres, associated with ATRX gene mutations and alternative lengthening of telomeres (ALT). Ultimately, these cancers may benefit from a therapy stratification approach. In order to identify and further characterize pediatric brain tumors with telomere lengthening (TL), we determined the intratelomeric content in silico from paired WGS of 918 tumors from CBTTC Pediatric Brain Tumor Atlas (PBTA). The results were highly concordant with experimental assays to determine ALT in a subset of 45 pHGG tumors from the set. Overall, 13% of the PBTA cohort had telomere lengthening. We confirmed the highest rate of TL (37%) in the pHGG cohort (37/100 tumors; 30/82 patients). There was no statistical difference in age, gender or survival in subset analysis. As expected, the patient pHGG tumors with telomere lengthening were enriched for ATRX mutations (60%, q= 1.76e-3). However, 6 tumors without ATRX mutation also had normal protein expression, suggesting a different mechanism of inactivation or TL. The pHGG tumors with telomere lengthening had increased mutational burden (q=8.98e-3) and included all known pHGG cases (n=6) in the cohort with replication repair deficiencies. Of interest, the second highest rate of telomere lengthening was 9 subjects (24%) in the craniopharyngioma cohort. None of the craniopharyngioma tumors had ATRX mutations or low ATRX expression, and 55% of those with TL had CTNNB1 mutations. Finally, lower rates of telomere lengthening were found in medulloblastoma (10%), ependymoma (10%), low grade astrocytoma (8%) and ganglioglioma (7/47, 15%).

Exploiting Laboratory Insights to Improve Outcomes of Pediatric Central Nervous System Tumors

2016 ◽  
pp. e540-e546 ◽  
Author(s):  
Giles W. Robinson ◽  
Hendrik Witt ◽  
Adam Resnick
Keyword(s):  
Brain Tumor ◽  
Molecular Mechanisms ◽  
Therapeutic Potential ◽  
Pediatric Brain Tumor ◽  
Pediatric Brain Tumors ◽  
Low Grade ◽  
Short Period ◽  
Tumor Management ◽  
The Impact ◽  
Pediatric Brain

Over a relatively short period of time, owing to improvements in biotechnology, our ability to identify the molecular mechanisms within pediatric brain tumors has dramatically increased. These findings have reshaped the way that we describe these diseases and have provided insights into how to better treat these often devastating diseases. Although still far from reaching the full therapeutic potential these advancements hold, the impact of these findings is steadily taking hold of pediatric brain tumor management. In this article, we summarize the major discoveries within three common pediatric brain tumor categories; medulloblastoma, ependymoma, and low-grade glioma. We discuss the current impact of these findings on treatment and the direction these findings may take the field of pediatric neuro-oncology.

Proteomic Study of Pilocytic Astrocytoma Pediatric Brain Tumor Intracystic Fluid

2014 ◽  
Vol 13 (11) ◽  
pp. 4594-4606 ◽  
Author(s):  
Ilaria Inserra ◽  
Federica Iavarone ◽  
Claudia Martelli ◽  
Luca D’Angelo ◽  
Daniela Delfino ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Pilocytic Astrocytoma ◽  
Pediatric Brain Tumor ◽  
Proteomic Study ◽  
Pediatric Brain

A phase 1 study of AZD6244 in children with recurrent or refractory low-grade gliomas: A Pediatric Brain Tumor Consortium report.

2014 ◽  
Vol 32 (15_suppl) ◽  
pp. 10065-10065 ◽  
Author(s):  
Anuradha Banerjee ◽  
Regina Jakacki ◽  
Arzu Onar-Thomas ◽  
Shengjie Wu ◽  
Theodore Nicolaides ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Phase 1 ◽  
Pediatric Brain Tumor ◽  
Low Grade ◽  
Phase 1 Study ◽  
Low Grade Gliomas ◽  
Pediatric Brain

scholarly journals LGG-08. A PHASE II PROSPECTIVE STUDY OF SELUMETINIB IN CHILDREN WITH RECURRENT OR REFRACTORY LOW-GRADE GLIOMA (LGG): A PEDIATRIC BRAIN TUMOR CONSORTIUM (PBTC) STUDY

2017 ◽  
Vol 19 (suppl_4) ◽  
pp. iv34-iv35 ◽  
Author(s):  
Jason Fangusaro ◽  
Arzu Onar-Thomas ◽  
Tina Y Poussaint ◽  
Shengjie Wu ◽  
Azra H Ligon ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Prospective Study ◽  
Phase Ii ◽  
Pediatric Brain Tumor ◽  
Low Grade Glioma ◽  
Low Grade ◽  
Pediatric Brain

scholarly journals A phase I trial of the MEK inhibitor selumetinib (AZD6244) in pediatric patients with recurrent or refractory low-grade glioma: a Pediatric Brain Tumor Consortium (PBTC) study

2017 ◽  
Vol 19 (8) ◽  
pp. 1135-1144 ◽  
Author(s):  
Anuradha Banerjee ◽  
Regina I. Jakacki ◽  
Arzu Onar-Thomas ◽  
Shengjie Wu ◽  
Theodore Nicolaides ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Phase I ◽  
Phase I Trial ◽  
Pediatric Patients ◽  
Pediatric Brain Tumor ◽  
Mek Inhibitor ◽  
Low Grade Glioma ◽  
Low Grade ◽  
Pediatric Brain

scholarly journals Circular RNA expression profiles in pediatric ependymomas

2020 ◽  
Author(s):  
Ulvi Ahmadov ◽  
Meile M. Bendikas ◽  
Karoline K. Ebbesen ◽  
Astrid M. Sehested ◽  
Jorgen Kjems ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Brain Tumors ◽  
Pilocytic Astrocytoma ◽  
Expression Profiles ◽  
Pediatric Brain Tumor ◽  
Pediatric Brain Tumors ◽  
Disease Stage ◽  
Tumor Subtypes ◽  
Pediatric Brain ◽  
Control Samples

Pediatric brain tumors frequently develop in the cerebellum, where ependymoma, medulloblastoma and pilocytic astrocytoma are the most prevalent subtypes. These tumors are currently treated using non-specific therapies, in part because few somatically mutated driver genes are present, and the underlying pathobiology is poorly described. Circular RNAs (circRNAs) have recently emerged as a large class of primarily non-coding RNAs with important roles in tumorigenesis, but so far they have not been described in pediatric brain tumors. To advance our understanding of these tumors, we performed high-throughput sequencing of ribosomal RNA-depleted total RNA from 10 primary ependymoma and 3 control samples. CircRNA expression patterns were determined using two independent bioinformatics algorithms, and correlated to disease stage, outcome, age, and gender. We found a profound global downregulation of circRNAs in ependymoma relative to control samples. Many differentially expressed circRNAs were discovered and circSMARCA5 and circ-FBXW7, which are described as tumor suppressors in glioma and glioblastomas in adults, were among the most downregulated. Moreover, patients with a dismal outcome clustered separately from patients with a good prognosis in unsupervised hierarchical cluster analyses. Next, we performed NanoString nCounter experiments using a custom-designed panel including 66 selected circRNA targets and analyzed formalin-fixed paraffin-embedded (FFPE) samples from a larger cohort of ependymoma patients as well as patients diagnosed with medulloblastoma or pilocytic astrocytoma. These experiments were used to validate our findings and, in addition, indicated that circRNA expression profiles are different among distinct pediatric brain tumor subtypes. In particular, circRMST and a circRNA derived from the LRBA gene were specifically upregulated in ependymomas. In conclusion, circRNAs have profoundly different expression profiles in ependymomas relative to controls and other pediatric brain tumor subtypes.

scholarly journals Efficacy of bevacizumab plus irinotecan in children with recurrent low-grade gliomas—a Pediatric Brain Tumor Consortium study

2013 ◽  
Vol 16 (2) ◽  
pp. 310-317 ◽  
Author(s):  
Sridharan Gururangan ◽  
Jason Fangusaro ◽  
Tina Young Poussaint ◽  
Roger E. McLendon ◽  
Arzu Onar-Thomas ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Pediatric Brain Tumor ◽  
Low Grade ◽  
Low Grade Gliomas ◽  
Pediatric Brain

scholarly journals A phase II trial of selumetinib in children with recurrent optic pathway and hypothalamic low-grade glioma without NF1: a Pediatric Brain Tumor Consortium study

2021 ◽  
Author(s):  
Jason Fangusaro ◽  
Arzu Onar-Thomas ◽  
Tina Young Poussaint ◽  
Shengjie Wu ◽  
Azra H Ligon ◽  
...  
Keyword(s):  
Brain Tumor ◽  
Phase Ii ◽  
Phase Ii Trial ◽  
Visual Fields ◽  
Pediatric Brain Tumor ◽  
Progression Free Survival ◽  
Neurofibromatosis Type ◽  
Low Grade ◽  
Optic Pathway ◽  
Pediatric Brain

Abstract Background Pediatric low-grade gliomas (pLGGs) are the most common childhood brain tumor. Progression-free survival (PFS) is much lower than overall survival, emphasizing the need for alternative treatments. Sporadic (without neurofibromatosis type 1) optic pathway and hypothalamic gliomas (OPHGs) are often multiply recurrent and cause significant visual deficits. Recently, there has been a prioritization of functional outcomes. Methods We present results from children with recurrent/progressive OPHGs treated on a PBTC (Pediatric Brain Tumor Consortium) phase II trial evaluating efficacy of selumetinib (AZD6244, ARRY-142886) a MEK-1/2 inhibitor. Stratum 4 of PBTC-029 included patients with sporadic recurrent/progressive OPHGs treated with selumetinib at the recommended phase II dose (25mg/m2/dose BID) for a maximum of 26 courses. Results Twenty-five eligible and evaluable patients were enrolled with a median of 4 (1-11) previous therapies. Six of 25 (24%) had partial response, 14/25 (56%) had stable disease, and 5 (20%) had progressive disease while on treatment. The median treatment courses were 26 (2-26); 14/25 patients completed all 26 courses. Two-year PFS was 78 ± 8.5%. Nineteen of 25 patients were evaluable for visual acuity which improved in 4/19 patients (21%), was stable in 13/19 (68%), and worsened in 2/19 (11%). Five of 19 patients (26%) had improved visual fields and 14/19 (74%) were stable. The most common toxicities were grade 1/2 CPK elevation, anemia, diarrhea, headache, nausea/emesis, fatigue, AST and ALT increase, hypoalbuminemia, and rash. Conclusions Selumetinib was tolerable and led to responses and prolonged disease stability in children with recurrent/progressive OPHGs based upon radiographic response, PFS, and visual outcomes.

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