scholarly journals Beyond IDH-Mutation: Emerging Molecular Diagnostic and Prognostic Features in Adult Diffuse Gliomas

Cancers ◽  
2020 ◽  
Vol 12 (7) ◽  
pp. 1817 ◽  
Author(s):  
Kanish Mirchia ◽  
Timothy E. Richardson
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Prognostic Factors ◽  
The United States ◽  
Molecular Diagnostic ◽  
Grading System ◽  
Diffuse Glioma ◽  
Prognostic Features ◽  
Molecular Features ◽  
Diffuse Gliomas

Diffuse gliomas are among the most common adult central nervous system tumors with an annual incidence of more than 16,000 cases in the United States. Until very recently, the diagnosis of these tumors was based solely on morphologic features, however, with the publication of the WHO Classification of Tumours of the Central Nervous System, revised 4th edition in 2016, certain molecular features are now included in the official diagnostic and grading system. One of the most significant of these changes has been the division of adult astrocytomas into IDH-wildtype and IDH-mutant categories in addition to histologic grade as part of the main-line diagnosis, although a great deal of heterogeneity in the clinical outcome still remains to be explained within these categories. Since then, numerous groups have been working to identify additional biomarkers and prognostic factors in diffuse gliomas to help further stratify these tumors in hopes of producing a more complete grading system, as well as understanding the underlying biology that results in differing outcomes. The field of neuro-oncology is currently in the midst of a “molecular revolution” in which increasing emphasis is being placed on genetic and epigenetic features driving current diagnostic, prognostic, and predictive considerations. In this review, we focus on recent advances in adult diffuse glioma biomarkers and prognostic factors and summarize the state of the field.

Glioblastoma multiforme metastasis outside the central nervous system: Three case reports and possible mechanisms of escape.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e13035-e13035
Author(s):  
Edwin Boelke ◽  
Christiane Matuschek ◽  
Lawrence E. Ginsberg ◽  
Sujit S. Prabhu ◽  
Wilfried Budach ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Brain Tumor ◽  
Malignant Tumors ◽  
Case Reports ◽  
The United States ◽  
Primary Brain Tumor ◽  
Diffuse Glioma ◽  
Average Survival ◽  
The Central Nervous System

e13035 Background: Primary brain and central nervous system (CNS) tumor incidence is approximately 19 per 100,000 individuals per year in the United States (US) compared with 7 per 100,000 individuals worldwide. The most common intra-axial tumor is gliomas, which account for 32% of all primary CNS tumors and 80% of all malignant tumors of the CNS.The most common diffuse glioma is grade 4 astrocytoma (glioblastoma, GBM), which makes up 54% of diffuse glial tumors. GBM is also the most aggressive brain tumor with poor prognosis.GBM metastases outside the CNS are rare, so therapeutic experience with these types of tumors is limited. Methods: Herein, we present 3 GBM patients with extra-CNS metastasis. Results: One patient developed GBM metastasis in the lung and pleura 5 years after his GBM diagnosis had been confirmed. Another patient who underwent resection of the primary GBM developed disease that extended through the sphenoid to involve the orbit and skull and subsequently invaded the parotid gland and neck nodes 1 year after diagnosis. A third patient developed GBM metastasis in the skull and L5 vertebra 2 years after her primary brain tumor had been resected. Conclusions: The exact mechanism of GBM metastasis outside the central nervous system is not well understood but likely involves the invasion of structures such as bone, lymphatics, and vasculature, especially veins. Above-average survival time and repeated surgical intervention may place GBM patients at higher risk for these unusual metastases.

New Classification for Central Nervous System Tumors: Implications for Diagnosis and Therapy

2017 ◽  
pp. 753-763
Author(s):  
Christine E. Fuller ◽  
David T. W. Jones ◽  
Mark W. Kieran
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Brain Tumor ◽  
Cns Tumors ◽  
World Health ◽  
Molecular Diagnostic ◽  
Diffuse Glioma ◽  
New Classification ◽  
The Impact

The 2016 World Health Organization Classification of Tumors of the Central Nervous System (WHO 2016) represents a noteworthy divergence from prior classification schemas. This new classification introduced the concept of “integrated diagnoses” based on a marriage of both phenotypic (microscopic) and genotypic parameters, with the intended goals of improving diagnostic accuracy and patient management. The result is a major restructuring in many of the brain tumor categories, with the codification of multiple new tumor entities and subgroups. It is therefore imperative that pathologists, clinicians, and neuro-oncology researchers alike rapidly become familiar with this new classification schema. Many of the diagnostic updates set forth in the WHO 2016 have impacted brain tumor types that commonly arise in the pediatric age group, particularly within the diffuse glioma, ependymoma, and embryonal tumor categories. This review gives a brief overview of (1) the WHO 2016 as it relates to pediatric central nervous system (CNS) tumors, with an emphasis on molecular diagnostic tools used in the clinical arena, (2) ongoing and developing approaches to the molecular and genomic classification of pediatric CNS tumors, and (3) the impact of this new classification schema on clinical trials in pediatric neuro-oncology.

scholarly journals Patients with Primary Central Nervous System Lymphoma Not Eligible for Clinical Trials: Prognostic Factors, Treatment and Outcome

Cancers ◽  
2021 ◽  
Vol 13 (12) ◽  
pp. 2934
Author(s):  
Sabine Seidel ◽  
Michelle Margold ◽  
Thomas Kowalski ◽  
Alexander Baraniskin ◽  
Roland Schroers ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Clinical Trials ◽  
Nervous System ◽  
Prognostic Factors ◽  
Central Nervous System Lymphoma ◽  
Initial Response ◽  
Early Response ◽  
Multicenter Trial ◽  
High Dose ◽  
Therapeutic Trials

Patients with primary central nervous system lymphoma (PCNSL) not fulfilling inclusion criteria for clinical trials represent an underreported population. Thirty-four consecutive PCNSL patients seen at our center between 2005 and 2019 with exclusion criteria for therapeutic trials were analyzed (non-study patients) and compared with patients from the G-PCNSL-SG-1 (German PCNSL Study Group 1) study (study patients), the largest prospective multicenter trial on PCNSL, comprising 551 patients. Median follow up was 68 months (range 1–141) in non-study patients and 51 months (1–105) in study patients. Twenty-seven/34 (79.4%) non-study patients received high dose methotrexate (HDMTX), while seven/34 (20.6%) with a glomerular filtration rate (GFR) < 50 mL/min did not. Median overall survival (OS) was six months (95% confidence interval [CI] 0–21 months) in those 34 non-study patients. The 27 non-study patients treated with HDMTX were compared with 526/551 G-PCNSL-SG-1 study patients who had received HDMTX as well. Median OS was 20 months (95% CI 0–45)/21 months (95% CI 18–25) in 27 non-study/526 study patients (p = 0.766). Favorable prognostic factors in non-study patients were young age, application of HDMTX and early response on magnet resonance imaging (MRI). If HDMTX-based chemotherapy can be applied, long-term disease control is possible even in patients not qualifying for clinical trials. Initial response on early MRI might be useful for decision on treatment continuation.

scholarly journals 55 Malignant primary brain and other central nervous system tumours diagnosed in the Canadian population from 2009 to 2013

2018 ◽  
Vol 45 (S3) ◽  
pp. S16-S17
Author(s):  
EV Walker ◽  
F Davis ◽  
Keyword(s):  
United States ◽  
Central Nervous System ◽  
Nervous System ◽  
Brain Tumour ◽  
Age Distribution ◽  
Paediatric Population ◽  
The United States ◽  
Incidence Rates ◽  
Canadian Population ◽  
Cns Tumours

The Canadian Brain Tumour Registry (CBTR) project was established in 2016 with the aim of enhancing infrastructure for surveillance and clinical research to improve health outcomes for brain tumour patients in Canada. We present a national surveillance report on malignant primary brain and central nervous system (CNS) tumours diagnosed in the Canadian population from 2009-2013. Patients were identified through the Canadian Cancer Registry (CCR); an administrative dataset that includes cancer incidence data from all provinces/territories in Canada. Cancer diagnoses are coded using the ICD-O3 system. Tumour types were classified by site and histology using The Central Brain Tumour Registry of the United States definitions. Incidence rates (IR) and 95% confidence intervals (CI) were calculated per 100,000 person-years and standardized to the 2011 census population age-distribution. Overall, 12,115 malignant brain and CNS tumours were diagnosed in the Canadian population from 2009-2013 (IR:8.43;95%CI:8.28,8.58). Of these, 6,845 were diagnosed in males (IR:9.72;95%CI:9.49,9.95) and 5,270 in females (IR:7.20;95%CI:7.00,7.39). The most common histology overall was glioblastoma (IR:4.06;95%CI:3.95,4.16). Among those aged 0-19 years, 1,130 malignant brain and CNS tumours were diagnosed from 2009-2013 (IR:3.36;95%CI:3.16,3.56). Of these, 625 were diagnosed in males (IR:3.32;95%CI:3.34,3.92) and 505 in females (IR:3.08;95%CI:2.81,3.36). The most common histology among the paediatric population was pilocytic astrocytoma (IR:0.73;95%CI:0.64,0.83). The presentation will include: IRs for other histologies, the geographic distribution of cases and a comparison between Canada and the United States.

scholarly journals CBTRUS Statistical Report: Primary brain and other central nervous system tumors diagnosed in the United States in 2010–2014

2017 ◽  
Vol 19 (suppl_5) ◽  
pp. v1-v88 ◽  
Author(s):  
Quinn T Ostrom ◽  
Haley Gittleman ◽  
Peter Liao ◽  
Toni Vecchione-Koval ◽  
Yingli Wolinsky ◽  
...  
Keyword(s):  
United States ◽  
Central Nervous System ◽  
Nervous System ◽  
The United States ◽  
Central Nervous System Tumors ◽  
Nervous System Tumors ◽  
Statistical Report

Primary Leptomeningeal Gliomatosis in Children and Adults: A Morphological and Molecular Comparative Study With Literature Review

Neurosurgery ◽  
2015 ◽  
Vol 78 (3) ◽  
pp. 343-352 ◽  
Author(s):  
Arnault Tauziede-Espariat ◽  
Andre Maues de Paula ◽  
Melanie Pages ◽  
Annie Laquerriere ◽  
Emilie Caietta ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Overall Survival ◽  
Cerebrospinal Fluid ◽  
Nervous System ◽  
Clinical Symptoms ◽  
Malignant Gliomas ◽  
Leptomeningeal Gliomatosis ◽  
Molecular Features ◽  
The Central Nervous System ◽  
The Mean

Abstract BACKGROUND: Primary leptomeningeal gliomatosis (PLG) is a poorly recognized tumor of the central nervous system. OBJECTIVE: To describe the histopathological, immunohistochemical, and molecular features of PLG. METHODS: Results of our multicentric retrospective study of 6 PLG cases (3 pediatric and 3 adult) were compared with literature data. RESULTS: The mean age was 54.7 years for adults and 8.7 years for children, with 3 males and 3 females. Clinical symptoms were nonspecific. Cerebrospinal fluid analyses showed a high protein level often associated with pleocytosis but without neoplastic cells. On neuroimaging, diffuse leptomeningeal enhancement and hydrocephalus were observed, except in 1 case. PLG was mostly misinterpreted as infectious or tumoral meningitis. The first biopsy was negative in 50% of cases. Histopathologically, PLG cases corresponded to 1 oligodendroglioma without 1p19q codeletion and 5 astrocytomas without expression of p53. No immunostaining for IDH1R132H and no mutations of IDH1/2 and H3F3A genes were found. Overall survival was highly variable (2-82 months) but seems to be increased in children treated with chemotherapy. CONCLUSION: This study shows the difficulties of PLG diagnosis. The challenge is to achieve an early biopsy to establish a diagnosis and to begin a treatment, but the prognosis remains poor. PLG seems to have a different molecular and immunohistochemical pattern compared with intraparenchymal malignant gliomas.

scholarly journals The Role of Biomaterials in Implantation for Central Nervous System Injury

2018 ◽  
Vol 27 (3) ◽  
pp. 407-422 ◽  
Author(s):  
Yu-Shuan Chen ◽  
Horng-Jyh Harn ◽  
Tzyy-Wen Chiou
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cell Transplantation ◽  
Chemical Properties ◽  
Host Responses ◽  
Cell Behaviors ◽  
Molecular Features ◽  
Biological Substance ◽  
Flexible Application

Permanent deficits that occur in memory, sensation, and cognition can result from central nervous system (CNS) trauma that causes dysfunction and/or unregulated CNS regeneration. Some therapeutic approaches are preferentially applied to the human body. Therefore, cell transplantation, one of the therapeutic strategies, may be used to benefit people. However, poor cell viability and low efficacy are the limitations to cell transplantation strategies. Biomaterials have been widely used in several fields (e.g., triggering cell differentiation, guiding cell migration, improving wound healing, and increasing tissue regeneration) by modulating their characteristics in chemistry, topography, and softness/stiffness for highly flexible application. We reviewed implanted biomaterials to investigate the roles and influences of physical/chemical properties on cell behaviors and applications. With their unique molecular features, biomaterials are delivered in several methods and mixed with transplanted cells, which assists in increasing postimplanted biological substance efficiency on cell survival, host responses, and functional recovery of animal models. Moreover, tracking the routes of these transplanted cells using biomaterials as labeling agents is crucial for addressing their location, distribution, activity, and viability. Here, we provide comprehensive comments and up-to-date research of the application of biomaterials.

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