CTNI-10. ADVANCES IN MANAGEMENT OF LOW-GRADE GLIOMAS

2021 ◽  
Vol 23 (Supplement_6) ◽  
pp. vi61-vi61
Author(s):  
Ruchi Raval ◽  
Aadi Pandya ◽  
Jaspreet Behl ◽  
Sumul Raval
Keyword(s):  
Treatment Plan ◽  
Treatment Options ◽  
Expression Patterns ◽  
Molecular Genetic ◽  
Tumor Resection ◽  
Low Grade ◽  
Low Grade Gliomas ◽  
Wait And See ◽  
Microsurgical Resection ◽  
High Doses

Abstract BACKGROUND Low-grade gliomas (LGGs) are common benign brain tumors that arise from glial cells of the brain. The complex and variable backgrounds of LGGs cause difficulties in assigning therapies. In this report, we compile findings relating to LGG genetics and treatment options in order to create a coherent summary for referential use. METHODS We compiled 70 articles from the past ten years that cover a broad range of topics pertaining to LGGs, including molecular, genetic, epigenetic, morphological, and other diagnostic factors, as well as prognosis and treatment options. RESULTS In order to provide proper treatment, the molecular basis and histology of the tumor must be addressed. The 1p/19q co-deletion indicator has been considered the gold standard of glioma diagnosis, but oft-sighted mutations in the IDH1 and IDH2 genes have given rise to three subgroups of 1p/19q co-deleted tumors, each associated with specific patterns of nervous cells. Other molecular markers and microRNA expression patterns have been studied for possible diagnostic and prognostic methods. Microsurgical resection is the singular treatment with highest overall survival (OS) and quality of life (QOL). Total gross resection is optimal, with patients having a 5-year OS of 100% with 90% tumor resection. Low doses of radiation are as effective as high doses and are better tolerated (with less cognitive deficits). Chemotherapy, specifically temozolomide, offers a favorable toxicity profile and QOL; additionally, seizure reduction is an early and consistent prognostic marker for survival after treatment with temozolomide. CONCLUSIONS The “wait and see” approach for treatment is no longer the standard for LGGs. Immediate treatment after diagnosis is recommended. Gross total resection (if achievable) is the most favorable treatment, with the highest OS and QOL. The use of temozolomide and radiotherapy is recommended. Histological background and genetic markers are vital for determining a treatment plan.

scholarly journals Low-grade gliomas with the V600E mutation in the BRAF gene in children: clinical features and treatment options

2020 ◽  
Vol 19 (4) ◽  
pp. 58-65
Author(s):  
L. I. Papusha ◽  
E. F. Valiakhmetova ◽  
A. E. Druy ◽  
L. A. Yasko ◽  
K. A. Voronin ◽  
...  
Keyword(s):  
Targeted Therapy ◽  
Treatment Options ◽  
Molecular Genetic ◽  
Braf Inhibitor ◽  
Complete Response ◽  
Braf V600e Mutation ◽  
Braf V600e ◽  
Molecular Characteristics ◽  
Low Grade ◽  
Low Grade Gliomas

The main pathogenetic mechanism of the development of pediatric low grade gliomas (pLGGs) is genetic aberrations in BRAF gene. This study is supported by the Independent Ethics Committee and approved by the Academic Council of the Dmitry Rogachev National Medical Research Center of Pediatric Hematology, Oncology, and Immunology. We analyzed the clinical and molecular characteristics of 69 patients with LGGs. Molecular genetic testing for BRAF V600E mutation was performed by allele-specific real-time PCR and Sanger sequencing. BRAF V600E mutation was detected in 15 (21.7%) patients with LGG. The majority of BRAF-mutated cases of LGGs had the midline location: OPG – 7, subcortical ganglia – 1, brainstem – 2. The 2-year PFS was much worse in patients with BRAF V600E compared to patients without this mutation – 30% and 66.2%, respectively. The median time to progression for patients with BRAF V600E mutation was 9.5 months compared to 3.1 years for patients without indicated substitution. 5 patients with BRAF V600E-mutated LGGs who experienced progression after the conventional treatment, received targeted therapy (BRAF-inhibitor-3, BRAF + MEK inhibitors – 2) with good response (complete response – 2, partial response – 3). BRAF V600E mutation contributes to poor outcome in patients with LGGs Targeted therapy could be effective in this cohort of patients.

scholarly journals SURG-31. INCIDENTAL LOW GRADE GLIOMAS: “WAIT AND SEE” OR “SURGERY”

2017 ◽  
Vol 19 (suppl_6) ◽  
pp. vi241-vi241 ◽  
Author(s):  
Jaejoon Lim ◽  
Youngjoon Park ◽  
Min Jun Kim ◽  
Juwon Ahn ◽  
Kyung gi Cho
Keyword(s):  
Low Grade ◽  
Low Grade Gliomas ◽  
Wait And See

Neurosurgical tools to extend tumor resection in hemispheric low-grade gliomas: conventional and contrast enhanced ultrasonography

2016 ◽  
Vol 32 (10) ◽  
pp. 1907-1914 ◽  
Author(s):  
Luca Mattei ◽  
Francesco Prada ◽  
Federico Giuseppe Legnani ◽  
Alessandro Perin ◽  
Alessandro Olivi ◽  
...  
Keyword(s):  
Tumor Resection ◽  
Low Grade ◽  
Low Grade Gliomas ◽  
Contrast Enhanced ◽  
Contrast Enhanced Ultrasonography

scholarly journals Brainstem Low-Grade Gliomas in Children—Excellent Outcomes With Multimodality Therapy

2016 ◽  
Vol 32 (2) ◽  
pp. 194-203 ◽  
Author(s):  
Santhosh A. Upadhyaya ◽  
Carl Koschmann ◽  
Karin Muraszko ◽  
Sriram Venneti ◽  
Hugh J. Garton ◽  
...  
Keyword(s):  
Progressive Disease ◽  
Survival Rates ◽  
Tumor Resection ◽  
Progression Free Survival ◽  
Low Grade ◽  
University Of Michigan ◽  
Single Institution ◽  
Free Survival ◽  
Low Grade Gliomas ◽  
The University

Safe maximal surgical resection is the initial treatment of choice for pediatric brainstem low-grade gliomas. Optimal therapy for incompletely resected tumors or that progress after surgery is uncertain. We reviewed the clinical characteristics, therapy, and outcomes of all children with nontectal brainstem low-grade gliomas treated at the University of Michigan between 1993 and 2013. Median age at diagnosis was 6 years; histology was confirmed in 23 of 25 tumors, 64% were pilocytic astrocytoma. Nineteen patients underwent initial tumor resection; 14/19 received no upfront adjuvant therapy. Eight patients in the study had progressive disease; 5 initially resected tumors received chemotherapy at tumor relapse, all with partial or complete radiographic responses. Ten-year progression-free survival is 71% and overall survival, 100%. This single-institution retrospective study demonstrates excellent survival rates for children with brainstem low-grade gliomas. The efficacy of the well-tolerated chemotherapy in this series supports its role in the treatment of unresectable or progressive brainstem low-grade gliomas.

scholarly journals Molecular features assisting in diagnosis, surgery, and treatment decision making in low-grade gliomas

2015 ◽  
Vol 38 (3) ◽  
pp. E2 ◽  
Author(s):  
Ricky Chen ◽  
Vijay M. Ravindra ◽  
Adam L. Cohen ◽  
Randy L. Jensen ◽  
Karen L. Salzman ◽  
...  
Keyword(s):  
Imaging Techniques ◽  
Molecular Genetic ◽  
Treatment Decision ◽  
Resonance Spectroscopy ◽  
Low Grade ◽  
Isocitrate Dehydrogenase 1 ◽  
Genetic Event ◽  
Surgical Interventions ◽  
Low Grade Gliomas ◽  
Molecular Features

The preferred management of suspected low-grade gliomas (LGGs) has been disputed, and the implications of molecular changes for medical and surgical management of LGGs are important to consider. Current strategies that make use of molecular markers and imaging techniques and therapeutic considerations offer additional options for management of LGGs. Mutations in the isocitrate dehydrogenase 1 and 2 (IDH1 and IDH2) genes suggest a role for this abnormal metabolic pathway in the pathogenesis and progression of these primary brain tumors. Use of magnetic resonance spectroscopy can provide preoperative detection of IDH-mutated gliomas and affect surgical planning. In addition, IDH1 and IDH2 mutation status may have an effect on surgical resectability of gliomas. The IDH-mutated tumors exhibit better prognosis throughout every grade of glioma, and mutation may be an early genetic event, preceding lineage-specific secondary and tertiary alterations that transform LGGs into secondary glioblastomas. The O6-methylguanine-DNAmethyltransferase (MGMT) promoter methylation and 1p19q codeletion status can predict sensitivity to chemotherapy and radiation in low- and intermediate-grade gliomas. Thus, these recent advances, which have led to a better understanding of how molecular, genetic, and epigenetic alterations influence the pathogenicity of the different histological grades of gliomas, can lead to better prognostication and may lead to specific targeted surgical interventions and medical therapies.

Low-grade gliomas of the cerebral hemispheres in children: an analysis of 71 cases

1995 ◽  
Vol 82 (4) ◽  
pp. 536-547 ◽  
Author(s):  
Ian F. Pollack ◽  
Diana Claassen ◽  
Qasim Al-Shboul ◽  
Janine E. Janosky ◽  
Melvin Deutsch
Keyword(s):  
Overall Survival ◽  
Univariate Analysis ◽  
Tumor Resection ◽  
Progression Free Survival ◽  
Low Grade ◽  
Free Survival ◽  
Content Type ◽  
Low Grade Gliomas ◽  
Fine Print

✓ Low-grade gliomas constitute the largest group of cerebral hemispheric tumors in the pediatric population. Although complete tumor resection is generally the goal in the management of these lesions, this can prove difficult to achieve because tumor margins may blend into the surrounding brain. This raises several important questions on the long-term behavior of the residual tumor and the role of adjuvant therapy in the management of these lesions. To examine these issues, the authors reviewed their experience in 71 children with low-grade cerebral hemispheric gliomas who were treated at their institution between 1956 and 1991 and assessed the relationship between clinical, radiographic, pathological, and treatment-related factors and outcome. Only seven patients in the series died, one from perioperative complications, five from progressive disease, and one (a child with neurofibromatosis) from a second neoplasm. For the 70 patients who survived the perioperative period, overall actuarial survivals at 5, 10, and 20 years were 95%, 93%, and 85%, respectively; progression-free status was maintained in 88%, 79%, and 76%, respectively. On univariate analysis, the factor that was most strongly associated with both overall and progression-free survival was the extent of tumor resection (p = 0.013 and p = 0.015, respectively). A relationship between extent of resection and progression-free survival was present both in patients with pilocytic astrocytomas (p = 0.041) and those with nonpilocytic tumors (p = 0.037). Histopathological diagnosis was also associated with overall survival on univariate analysis; poorer results were seen in the patients with nonpilocytic astrocytoma compared to those with other low-grade gliomas, such as pilocytic astrocytoma, mixed glioma, and oligodendroglioma (p = 0.021). The use of radiotherapy was not associated with a significant improvement in overall survival (p = 0.6). All three patients who ultimately developed histologically confirmed anaplastic changes in the vicinity of the original tumor had received prior radiotherapy, 20, 46, and 137 months, respectively, before the detection of malignant progression. In addition, children who received radiotherapy had a significantly higher incidence of late cognitive and endocrine dysfunction than the nonirradiated patients (p < 0.01 and 0.05, respectively). The authors conclude that children with low-grade gliomas of the cerebral hemispheres have an excellent overall prognosis. Complete tumor resection provides the best opportunity for long-term progression-free survival. However, even with incomplete tumor excision, long-term progression-free survival is common. The findings in this study do not support the routine use of postoperative radiotherapy after an initial incomplete tumor resection: although irradiation appears to increase the likelihood of long-term progression-free survival, overall survival is not improved significantly, and long-term morbidity may be increased.

scholarly journals Pharmacoresistant seizures and IDH mutation in low grade gliomas

2021 ◽  
Author(s):  
Carlos Eduardo Correia ◽  
Yoshie Umemura ◽  
Jessica R Flynn ◽  
Anne S Reiner ◽  
Edward K Avila
Keyword(s):  
Tumor Resection ◽  
Incidence Rates ◽  
P Value ◽  
Low Grade ◽  
Idh Mutation ◽  
Wild Type ◽  
Mutation Status ◽  
Low Grade Gliomas ◽  
Idh1 R132h ◽  
Idh Mutations

Abstract Purpose Many low-grade gliomas (LGG) harbor isocitrate dehydrogenase (IDH) mutations. Although IDH mutation is known to be epileptogenic, the rate of refractory seizures in LGG with IDH mutation vs wild-type had not been previously compared. We therefore compared seizure pharmacoresistance in IDH-mutated and wild-type LGGs. Methods Single-institution retrospective study of patients with histologic proven LGG, known IDH mutation status, seizures, and ≥ 2 neurology clinic encounters. Seizure history was followed until histological high-grade transformation or death. Seizures requiring ≥ 2 changes in anti-epileptic drugs were considered pharmacoresistant. Incidence rates of pharmacoresistant seizures were estimated using competing risks methodology. Results Of 135 patients, 25 patients (19%) had LGGs classified as IDH wild-type. Of those with IDH mutation, 104 (94.5%) were IDH1 R132H; only six were IDH2 R172K. 120 patients (89%) had tumor resection and 14 (10%) had biopsy. Initial post-surgical management included observation (64%), concurrent chemoradiation (23%), chemotherapy alone (9%), and radiotherapy alone (4%). Seizures became pharmacoresistant in 24 IDH-mutated patients (22%) and in 3 IDH wild-type patients (12%). The 4-year cumulative incidence of intractable seizures was 17.6% (95% CI: 10.6%-25.9%) in IDH-mutated and 11% (95% CI: 1.3%-32.6%) in IDH wild-type LGG (Gray’s P-value= 0.26). Conclusions 22% of the IDH-mutated patients developed pharmacoresistant seizures, compared to 12% of the IDH wild-type tumors.The likelihood of developing pharmacoresistant seizures in patients with LGG-related epilepsy is independent to IDH mutation status, however, IDH-mutated tumors were approximately twice as likely to experience LGG-related pharmacoresistant seizures.

scholarly journals Management of low-grade gliomas

2021 ◽  
Author(s):  
Milla Giancristofaro Dutra ◽  
Bernardo Valle Zanetti ◽  
Ana Luiza Badini Tubenchlak ◽  
Bárbara Gomes Muffato ◽  
Leonardo Moreira Dutra ◽  
...  
Keyword(s):  
Large Scale ◽  
Randomized Clinical Trials ◽  
Malignant Tumors ◽  
Tumor Resection ◽  
Progression Free Survival ◽  
Functional Results ◽  
Therapeutic Modality ◽  
Low Grade ◽  
Low Grade Gliomas ◽  
The Central Nervous System

Background: Gliomas are the most aggressive and prevalent primary malignant tumors of the central nervous system. For better mapping, they are subclassified into degrees in proportion to their malignancy. Although low-grade patients have a better prognosis, they are extremely heterogeneous. Since the high variability in the outcomes of the condition, it is essential to investigate the current therapeutic strategies available. Objective: Analyze the management of low-grade gliomas. Methods: In April 2021, a literature review was conducted on MEDLINE using the descriptors: “Glioma”, “Low Grade”; “Treatment”; as well as their variations obtained in MeSH. Controlled and randomized clinical trials carried out on humans in the last five years were included. Results: 63 articles were found and 10 of them were analyzed in this review. The research has shown that total tumor resection is the therapeutic modality that causes the greatest drop in the mortality rates. Furthermore, the greater the extraction, the greater the progression-free survival. In this way, for greater safety of large-scale surgeries, several intraoperative techniques have been developed. An example is the waking approach, which presents favorable long-term functional results and low failure rates. However, the isolated surgery is often not sufficiently curative. Therefore, it is necessary to complement radiotherapy and chemotherapy with temozolomide, associated with a 5 to 10 year survival rate when combined. Conclusions: Studies have shown that total resection of the tumor is the best way to manage low-grade gliomas, but it is often combined with temozolamide chemotherapy and radiotherapy for a better prognosis.

Neurosurgical tools to extend tumor resection in pediatric hemispheric low-grade gliomas: iMRI

2016 ◽  
Vol 32 (10) ◽  
pp. 1915-1922 ◽  
Author(s):  
Mario Giordano ◽  
Cinta Arraez ◽  
Amir Samii ◽  
Madjid Samii ◽  
Concezio Di Rocco
Keyword(s):  
Tumor Resection ◽  
Low Grade ◽  
Low Grade Gliomas
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