Low-Grade Oncocytic Tumor of Kidney (CK7-Positive, CD117-Negative): Incidence in a Single Institutional Experience with Clinicopathological and Molecular Characteristics

2021 ◽  
Author(s):  
Oleksandr Kravtsov ◽  
Sounak Gupta ◽  
John C. Cheville ◽  
William R. Sukov ◽  
Ross Rowsey ◽  
...  
Keyword(s):  
Molecular Characteristics ◽  
Low Grade ◽  
Oncocytic Tumor ◽  
Institutional Experience

scholarly journals LGG-15. COMPREHENSIVE ANALYSIS OF MYB/MYB1-ALTERED GLIOMAS: A MULTI-INSTITUTIONAL EXPERIENCE OF 33 GLIOMAS

2021 ◽  
Vol 23 (Supplement_1) ◽  
pp. i34-i35
Author(s):  
Daniel Moreira ◽  
Susan Spiller ◽  
Thomas Bouldin ◽  
Alan Davidson ◽  
Nasjla Saba-Silva ◽  
...  
Keyword(s):  
Genetic Alterations ◽  
Myelogenous Leukemia ◽  
Molecular Characteristics ◽  
Low Grade ◽  
Cerebrospinal Fluid Diversion ◽  
Institutional Experience ◽  
Diffuse Gliomas ◽  
Acute Myelogenous ◽  
Single Disease Entity

Abstract Background Pediatric diffuse gliomas harbor recurrent genetic alterations, including those in MYB and MYBL1. Regardless of histopathologic classification, low-grade diffuse gliomas with MYB/MYBL1 alterations represent a single disease entity. Additional insight is needed to define optimal therapeutic strategies for these tumors. Methods We retrospectively reviewed gliomas with MYB or MYB1L alterations treated or referred for pathologic review at St. Jude Children’s Research Hospital (St. Jude). Tumor specimens were centrally reviewed. Molecular characterization and clinical data were collated from St. Jude and referring institutions. Results Thirty-three patients were identified. Two tumors had MYBL1 alterations, while 31 had MYB alterations. MYB-QKI fusion was the most common alteration. Eighteen (55%) were male. The median age at diagnosis was 5 years (range, 0–40 years). Most tumors were in the cerebral cortex (22/33), and the most common presentation was seizures (16/33). Three patients (9%) presented with hydrocephalus and required cerebrospinal fluid diversion. Two patients (6%) presented with metastatic disease. Gross-total resection was achieved in 15 patients (45%). Of the 7 patients receiving cytotoxic chemotherapy, no substantial response was observed. Of the 6 patients who received RT, one had disease progression. The median follow-up was 5.9 years. The 5-year event-free survival was 88.1%, while the 5-year overall survival was 96.3%. Two patients died, one of unclear cause and one of treatment-related acute myelogenous leukemia. Using log-rank tests, no difference in outcomes was observed based on molecular characteristics, degree of resection, metastatic status, or treatment modality. Conclusions Although tumors with MYB and MYBL1 alterations present with varying molecular and clinical features, they represent a group of tumors with favorable outcomes. Further characterization is required to identify the subgroup of tumors with a higher propensity for progression.

scholarly journals High-dose radiation associated with improved survival in IDH-wildtype low-grade glioma

2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Shuai Liu ◽  
Yanwei Liu ◽  
Guanzhang Li ◽  
Jin Feng ◽  
Li Chen ◽  
...  
Keyword(s):  
Overall Survival ◽  
Survival Analysis ◽  
Radiation Dose ◽  
Frontal Lobe ◽  
Low Grade Glioma ◽  
Molecular Characteristics ◽  
High Dose ◽  
Low Grade ◽  
Multivariate Survival ◽  
Dose Radiation

Abstract Background As molecular advances have deepened the knowledge on low-grade glioma (LGG), we investigated the effect of higher radiation dose on the survival of IDH-wildtype (IDHwt) LGG. Methods In the current study, 52 IDHwt LGG patients who received radiotherapy were enrolled from the Chinese Glioma Genome Atlas dataset. Radiation doses > 54 Gy were defined as high-dose, whereas doses ≤ 54 Gy were defined as low-dose. We performed univariate and multivariate survival analyses to examine the prognostic role of high-dose radiotherapy. Results In total, the radiation dose ranged from 48.6 Gy to 61.2 Gy, with a median of 55.8 Gy, and 31 patients were grouped into high-dose radiation. Univariate survival analysis indicated that high-dose radiotherapy (p = 0.015), tumors located in the frontal lobe (p = 0.009), and pathology of astrocytoma (p = 0.037) were significantly prognostic factors for overall survival. In multivariate survival analysis, high-dose radiotherapy (p = 0.028) and tumors located in the frontal lobe (p = 0.016) were independently associated with better overall survival. Conclusions In conclusion, high-dose radiotherapy independently improved the survival of IDHwt LGG. This can guide treatments for glioma with known molecular characteristics.

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 clinical and molecular characteristics in low grade gliomas

2017 ◽  
Vol 28 ◽  
pp. vi75-vi76
Author(s):  
A. Mura ◽  
E. Franceschi ◽  
S. Minichillo ◽  
A. Tosoni ◽  
A. Fioravanti ◽  
...  
Keyword(s):  
Molecular Characteristics ◽  
Low Grade ◽  
Low Grade Gliomas

scholarly journals Institutional Experience of High-Risk, Low-Grade Glioma in the Temozolomide Era

2016 ◽  
Vol 96 (2) ◽  
pp. E115
Author(s):  
C.M. Leyrer ◽  
E.S. Murphy ◽  
S.T. Chao ◽  
M.A. Vogelbaum ◽  
G.H.J. Stevens ◽  
...  
Keyword(s):  
High Risk ◽  
Low Grade Glioma ◽  
Low Grade ◽  
Institutional Experience

scholarly journals First-line temozolomide chemotherapy in progressive low-grade astrocytomas after radiotherapy: molecular characteristics in relation to response

2010 ◽  
Vol 13 (2) ◽  
pp. 235-241 ◽  
Author(s):  
W. Taal ◽  
H. J. Dubbink ◽  
C. B. L. Zonnenberg ◽  
B. A. Zonnenberg ◽  
T. J. Postma ◽  
...  
Keyword(s):  
Molecular Characteristics ◽  
Low Grade ◽  
First Line ◽  
Temozolomide Chemotherapy

Radiation dose response for supratentorial low-grade glioma—institutional experience and literature review

2003 ◽  
Vol 214 (1-2) ◽  
pp. 43-48 ◽  
Author(s):  
Simon S. Lo ◽  
Walter A. Hall ◽  
Kwan H. Cho ◽  
Jamie Orner ◽  
Chung K. Lee ◽  
...  
Keyword(s):  
Radiation Dose ◽  
Literature Review ◽  
Dose Response ◽  
Low Grade Glioma ◽  
Low Grade ◽  
Institutional Experience

scholarly journals Meningioma: not always a benign tumor. A review of advances in the treatment of meningiomas

CNS Oncology ◽  
2021 ◽  
pp. CNS72
Author(s):  
Ilaria Maggio ◽  
Enrico Franceschi ◽  
Alicia Tosoni ◽  
Vincenzo Di Nunno ◽  
Lidia Gatto ◽  
...  
Keyword(s):  
Benign Tumor ◽  
Treatment Strategies ◽  
Complete Resection ◽  
Molecular Characteristics ◽  
Low Grade ◽  
Intracranial Tumors ◽  
Review Of The Literature ◽  
Systemic Treatments ◽  
Grade Ii ◽  
Grade Iii

Meningiomas are the most common primary intracranial tumors. The majority of meningiomas are benign, but they can present different grades of dedifferentiation from grade I to grade III (anaplastic/malignant) that are associated with different outcomes. Radiological surveillance is a valid option for low-grade asymptomatic meningiomas. In other cases, the treatment is usually surgical, aimed at achieving a complete resection. The use of adjuvant radiotherapy is the gold standard for grade III, is debated for grade II and is not generally indicated for radically resected grade I meningiomas. The use of systemic treatments is not standardized. Here we report a review of the literature on the clinical, radiological and molecular characteristics of meningiomas, available treatment strategies and ongoing clinical trials.

OS02.6.A The TeloDIAG: How telomeric parameters can help in glioma rapid diagnosis and liquid biopsies approaches

2021 ◽  
Vol 23 (Supplement_2) ◽  
pp. ii5-ii6
Author(s):  
P Billard ◽  
C Guerriau ◽  
C Carpentier ◽  
F Juillard ◽  
N Grandin ◽  
...  
Keyword(s):  
Telomere Maintenance ◽  
Molecular Characteristics ◽  
Low Grade ◽  
New Classification ◽  
Liquid Biopsies ◽  
Efficient Tool ◽  
Isocitrate Dehydrogenase 1 ◽  
Mutational Status ◽  
Alternative Lengthening

Abstract BACKGROUND The integration of molecular markers into the WHO 2016 classification has clarified the complex diagnosis of gliomas. Among these biomarkers, the TERT promoter mutation and the loss of ATRX (ATRX loss) are mutually exclusive alterations associated with re-activation of telomerase or alternative lengthening of telomeres (ALT), respectively. Strangely, 25% of gliomas display neither or both these alterations, a situation referred to as abnormal telomere maintenance mechanism (aTMM). MATERIAL AND METHODS To investigate the TMM actually involved in gliomas, the C-circle (CC) assay was adapted to tumor (FFPE and frozen) samples. RESULTS We constructed a CC-based algorithm able to identify the TMM of 284 gliomas with either TERT or ATRX alteration, with a sensitivity of 100% and a specificity of 97.3%, and succeeded in deciphering the TMM involved in 122 aTMM gliomas. Additionally, the combination of the TMM, the mutational status of the Isocitrate dehydrogenase 1/2 (IDH) gene, and the histological grading was used as base for a new classification: TeloDIAG. Six subtypes are defined in this classification: tOD, tLGA, tGBM_IDHmt, tGBM, and tAIV, corresponding to oligodendroglioma, IDHmt low grade astrocytoma, IDHmt glioblastoma, and IDHwt glioblastoma, respectively, the last class gathers ALT+ IDHwt glioma. The TeloDIAG diagnosis is 99% concordant with the WHO classification for glioma displaying typical molecular characteristics (N=312). It modified the classification of 38% (N=156) discordant tumors, such as IDHwt Astrocytoma, aTMM tumors, or gliomas with unexpected TMM (e.g. TERTwt oligodendroglioma, ATRX loss GBM). Interestingly, 20% (N=69) of TERTwt, ATRXwt, or IDHwt GBM were actually tAIV, which is remarkable as tAIV-glioma patients’ survival tended to be longer (21.2 months) than tGBM patients’ survival (16.5 months). Importantly, CC in blood sampled from IDHmt astrocytoma patients was detected with a sensitivity of 56% and a specificity of 95% (N = 206). CONCLUSION In sum, the TeloDIAG is a new, simple, and efficient tool helping in glioma diagnosis and a promising option for liquid biopsy

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