scholarly journals MPC-14 BRAF V600E MUTANT OLIGODENDROGLIOMA-LIKE TUMORS WITH CHROMOSOMAL INSTABILITY IN ADOLESCENTS AND YOUNG ADULTS

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii24-ii24
Author(s):  
Kohei Fukuoka ◽  
Uri Tabori ◽  
Cynthia Hawkins
Keyword(s):  
Young Adults ◽  
Idh1 Mutation ◽  
Adolescents And Young Adults ◽  
Braf V600e ◽  
Low Grade ◽  
Wild Type ◽  
Low Grade Gliomas ◽  
History Of ◽  
Mitotic Figures ◽  
Temporal Lobe Lesions

Abstract We performed genome-wide methylation analysis on 136 pediatric low grade gliomas, identifying a unique cluster consisting of oligodendroglioma-like BRAF V600E mutant tumors with Recurrent gain of Chromosome 7 and loss of Chromosome 10 (OLIVER). Hierarchical clustering and t-stochastic neighbor embedding analyses cluster them with previously described pediatric-type low grade gliomas, separate from adult gliomas. OLIVERS exhibit distinct clinical behavior as temporal lobe lesions in adolescents and young adults, prolonged history of seizures and all are alive with no recurrence (follow-up 3.2 to 13.2 years). Morphogically, all showed oligodendroglioma-like features, including round nuclei with perinuclear halos, a chicken-wire pattern of branching capillaries and microcalcification. None showed astrocytic features or characteristics suggestive of high-grade tumors including necrosis or mitotic figures. All tumors harbored multiple chromosomal copy number abnormalities (more than 10 chromosomes per OLIVER), but none showed 1p/19q co-deletion or IDH1 mutation. Interestingly, one tumor showed a TERT promoter mutation. Although the series is small, OLIVER may represent a new category of IDH wild-type low grade gliomas which may be confused with molecular GBM. Further, they highlight the heterogeneity of IDH wild-type gliomas and the relatively indolent behavior of pediatric-type gliomas.

Dynamic history of low-grade gliomas before and after temozolomide treatment

2007 ◽  
Vol 61 (5) ◽  
pp. 484-490 ◽  
Author(s):  
Damien Ricard ◽  
Gentian Kaloshi ◽  
Alexandra Amiel-Benouaich ◽  
Julie Lejeune ◽  
Yannick Marie ◽  
...  
Keyword(s):  
Low Grade ◽  
Low Grade Gliomas ◽  
Before And After ◽  
History Of

Molecular Alterations in Pediatric Low-Grade Gliomas That Led to Death

2021 ◽  
Author(s):  
Jared T Ahrendsen ◽  
Claire Sinai ◽  
David M Meredith ◽  
Seth W Malinowski ◽  
Tabitha M Cooney ◽  
...  
Keyword(s):  
Braf V600e ◽  
Molecular Characteristics ◽  
Low Grade ◽  
Diffuse Astrocytoma ◽  
Term Survival ◽  
Pten Loss ◽  
Low Grade Gliomas ◽  
Molecular Alterations ◽  
Idh1 R132h ◽  
Poor Outcomes

Abstract Pediatric low-grade gliomas (PLGGs) have excellent long-term survival, but death can occasionally occur. We reviewed all PLGG-related deaths between 1975 and 2019 at our institution: 48 patients were identified; clinical data and histology were reviewed; targeted exome sequencing was performed on available material. The median age at diagnosis was 5.2 years (0.4–23.4 years), at death was 13.0 years (1.9–43.2 years), and the overall survival was 7.2 years (0.0–33.3 years). Tumors were located throughout CNS, but predominantly in the diencephalon. Diagnoses included low-grade glioma, not otherwise specified (n = 25), pilocytic astrocytoma (n = 15), diffuse astrocytoma (n = 3), ganglioglioma (n = 3), and pilomyxoid astrocytoma (n = 2). Recurrence occurred in 42/48 cases, whereas progression occurred in 10. The cause of death was direct tumor involvement in 31/48 cases. Recurrent drivers included KIAA1549-BRAF (n = 13), BRAF(V600E) (n = 3), NF1 mutation (n = 3), EGFR mutation (n = 3), and FGFR1-TACC1 fusion (n = 2). Single cases were identified with IDH1(R132H), FGFR1(K656E), FGFR1 ITD, FGFR3 gain, PDGFRA amplification, and mismatch repair alteration. CDKN2A/B, CDKN2C, and PTEN loss was recurrent. Patients who received only chemotherapy had worse survival compared with patients who received radiation and chemotherapy. This study demonstrates that PLGG that led to death have diverse molecular characteristics. Location and co-occurring molecular alterations with malignant potential can predict poor outcomes.

scholarly journals The Role of Extent of Resection in IDH1 Wild-Type or Mutant Low-Grade Gliomas

Neurosurgery ◽  
2017 ◽  
Vol 82 (6) ◽  
pp. 808-814 ◽  
Author(s):  
Toral Patel ◽  
Evan D Bander ◽  
Rachael A Venn ◽  
Tiffany Powell ◽  
Gustav Young-Min Cederquist ◽  
...  
Keyword(s):  
Cox Regression ◽  
Extent Of Resection ◽  
World Health ◽  
Low Grade ◽  
Wild Type ◽  
Who Grade ◽  
Cox Regression Analysis ◽  
Low Grade Gliomas ◽  
Grade Ii ◽  
The Impact

Abstract BACKGROUND Maximizing extent of resection (EOR) improves outcomes in adults with World Health Organization (WHO) grade II low-grade gliomas (LGG). However, recent studies demonstrate that LGGs bearing a mutation in the isocitrate dehydrogenase 1 (IDH1) gene are a distinct molecular and clinical entity. It remains unclear whether maximizing EOR confers an equivalent clinical benefit in IDH mutated (mtIDH) and IDH wild-type (wtIDH) LGGs. OBJECTIVE To assess the impact of EOR on malignant progression-free survival (MPFS) and overall survival (OS) in mtIDH and wtIDH LGGs. METHODS We performed a retrospective review of 74 patients with WHO grade II gliomas and known IDH mutational status undergoing resection at a single institution. EOR was assessed with quantitative 3-dimensional volumetric analysis. The effect of predictor variables on MPFS and OS was analyzed with Cox regression models and the Kaplan–Meier method. RESULTS Fifty-two (70%) mtIDH patients and 22 (30%) wtIDH patients were included. Median preoperative tumor volume was 37.4 cm3; median EOR of 57.6% was achieved. Univariate Cox regression analysis confirmed EOR as a prognostic factor for the entire cohort. However, stratifying by IDH status demonstrates that greater EOR independently prolonged MPFS and OS for wtIDH patients (hazard ratio [HR] = 0.002 [95% confidence interval {CI} 0.000-0.074] and HR = 0.001 [95% CI 0.00-0.108], respectively), but not for mtIDH patients (HR = 0.84 [95% CI 0.17-4.13] and HR = 2.99 [95% CI 0.15-61.66], respectively). CONCLUSION Increasing EOR confers oncologic and survival benefits in IDH1 wtLGGs, but the impact on IDH1 mtLGGs requires further study.

scholarly journals 3D ultrasound–guided resection of low-grade gliomas: principles and clinical examples

2019 ◽  
Vol 47 (6) ◽  
pp. E9 ◽  
Author(s):  
Geirmund Unsgård ◽  
Frank Lindseth
Keyword(s):  
3D Ultrasound ◽  
High Accuracy ◽  
Low Grade ◽  
Ultrasound Guided ◽  
High Quality ◽  
Case Examples ◽  
Low Grade Gliomas ◽  
Mesial Temporal Lobe ◽  
Temporal Lobe Lesions

3D ultrasound (US) is a convenient tool for guiding the resection of low-grade gliomas, seemingly without deterioration in patients’ quality of life. This article offers an update of the intraoperative workflow and the general principles behind the 3D US acquisition of high-quality images.The authors also provide case examples illustrating the technique in two small mesial temporal lobe lesions and in one insular glioma. Due to the ease of acquiring new images for navigation, the operations can be guided by updated image volumes throughout the entire course of surgery. The high accuracy offered by 3D US systems, based on nearly real-time images, allows for precise and safe resections. This is especially useful when an operation is performed through very narrow transcortical corridors.

Anatomical location differences between mutated and wild-type isocitrate dehydrogenase 1 in low-grade gliomas

2017 ◽  
Vol 127 (10) ◽  
pp. 873-880 ◽  
Author(s):  
Jinhua Yu ◽  
Zhifeng Shi ◽  
Chunhong Ji ◽  
Yuxi Lian ◽  
Yuanyuan Wang ◽  
...  
Keyword(s):  
Isocitrate Dehydrogenase ◽  
Anatomical Location ◽  
Low Grade ◽  
Wild Type ◽  
Isocitrate Dehydrogenase 1 ◽  
Low Grade Gliomas

Noninvasive Prediction of IDH1 Mutation and ATRX Expression Loss in Low-Grade Gliomas Using Multiparametric MR Radiomic Features

2018 ◽  
Vol 49 (3) ◽  
pp. 808-817 ◽  
Author(s):  
Yan Ren ◽  
Xi Zhang ◽  
Wenting Rui ◽  
Haopeng Pang ◽  
Tianming Qiu ◽  
...  
Keyword(s):  
Idh1 Mutation ◽  
Low Grade ◽  
Low Grade Gliomas ◽  
Expression Loss

scholarly journals Therapeutic and Prognostic Implications of BRAF V600E in Pediatric Low-Grade Gliomas

2017 ◽  
Vol 35 (25) ◽  
pp. 2934-2941 ◽  
Author(s):  
Alvaro Lassaletta ◽  
Michal Zapotocky ◽  
Matthew Mistry ◽  
Vijay Ramaswamy ◽  
Marion Honnorat ◽  
...  
Keyword(s):  
Quantitative Imaging ◽  
Progression Free Survival ◽  
Extent Of Resection ◽  
Braf V600e ◽  
Low Grade ◽  
Low Grade Gliomas ◽  
Molecular Stratification ◽  
Treatment Data ◽  
Poor Outcomes ◽  
Independent Cohort

Purpose BRAF V600E is a potentially highly targetable mutation detected in a subset of pediatric low-grade gliomas (PLGGs). Its biologic and clinical effect within this diverse group of tumors remains unknown. Patients and Methods A combined clinical and genetic institutional study of patients with PLGGs with long-term follow-up was performed (N = 510). Clinical and treatment data of patients with BRAF V600E mutated PLGG (n = 99) were compared with a large international independent cohort of patients with BRAF V600E mutated-PLGG (n = 180). Results BRAF V600E mutation was detected in 69 of 405 patients (17%) with PLGG across a broad spectrum of histologies and sites, including midline locations, which are not often routinely biopsied in clinical practice. Patients with BRAF V600E PLGG exhibited poor outcomes after chemotherapy and radiation therapies that resulted in a 10-year progression-free survival of 27% (95% CI, 12.1% to 41.9%) and 60.2% (95% CI, 53.3% to 67.1%) for BRAF V600E and wild-type PLGG, respectively ( P < .001). Additional multivariable clinical and molecular stratification revealed that the extent of resection and CDKN2A deletion contributed independently to poor outcome in BRAF V600E PLGG. A similar independent role for CDKN2A and resection on outcome were observed in the independent cohort. Quantitative imaging analysis revealed progressive disease and a lack of response to conventional chemotherapy in most patients with BRAF V600E PLGG. Conclusion BRAF V600E PLGG constitutes a distinct entity with poor prognosis when treated with current adjuvant therapy.

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