bluuebunny/crossref_metadata_2025_split huggingface dataset:
NCMP-25. TUMOR-RELATED EPILEPSY IN PATIENTS WITH BRAIN TUMORS (PBT): AN ACADEMIC INSTITUTION EXPERIENCE
Authors: Cabrera Nynoshka Bassatt , Nasany Ruham Alshiekh , Zhao Kehan , Elwan Sherif , Rusnak Rebecca , Rasekhi Roozbeh Tarighati , Izadyar Shahram 
Published: November 2023

Abstract

BACKGROUND
Brain tumors are the second most common cause of intractable focal epilepsy. Seizures can be the presenting symptom of PBT or develop later in the disease course, impacting morbidity and quality of life. Despite the high prevalence of PBT-related epilepsy, there is a scarcity of literature to help better identify its unique features and characterizations that can guide medical management and reach a standardized approach for it. We present a retrospective review of a cohort of PBT-related epilepsy cases at a single academic institution.

METHODS
Records of patients with PBT seen at Upstate Medical University between 2012 and 2022 were reviewed. Inclusion criteria were: 18 years of age and older at the time of diagnosis and having at least one seizure that thought to be related to a PBT. Patients with history of previous epilepsy or psychogenic non-epileptic seizures were excluded.

RESULTS
Of the 1103 reviewed records, 293 patients (26.56%) met the inclusion criteria. Mean age at tumor diagnosis was 57.7 years +/- 16.6, and mean follow-up after tumor diagnosis was 17.9 months. Seizures were the initial presentation of the brain tumor in 166 (56.66%) patients. The most common tumor was grade 4 glioma in 45% of patients. Anti-seizure medications were started in 92.1%, and the most commonly used medication was levetiracetam in 75%. Number of patients who underwent total resection of the tumor was 123 (42.4%). On the last follow-up visit, 56% of patients remained seizure-free.

CONCLUSION
Patients with PBT often develop epilepsy that, at times, can be intractable. More than half of our patients experienced a seizure as the presenting symptom. Even though more than 90% were started on anti-seizure medications, only 56% remained seizure-free on follow-ups. Since seizures dramatically affect the quality of life, managing seizures in this patient population warrants further studies.
NCOG-05. TREATMENT STRATEGY AND SURVIVAL ANALYSIS OF PATIENTS WITH PRIMARY ADULT-TYPE DIFFUSE GLIOMA IN THE MOLECULAR ERA: A PROSPECTIVE COHORT STUDY FROM THE CGGA NETWORK
Authors: Zhang Kenan , Li Guanzhang , Fang Shengyu , Zhang Wei , Wang Anxin , Jiang Tao 
Published: November 2023

Abstract

IMPORTANCE
The fifth edition of the WHO Classification of Tumors of the Central Nervous System (CNS) marks the entry of the diagnosis and treatment of diffuse glioma into the molecular era. The clinical implications of this landmark change in primary adult-type diffuse glioma need to be revealed.

OBJECTIVE
To reveal the clinical prognostic factors of adult diffuse glioma under the WHO CNS 5 classification.

DESIGN, SETTING, AND PARTICIPANTS
In this clinical-based prospective observational registration study, 3,308 glioma patients from 3 major neurosurgical centers were enrolled between Mar. 2005 to Jun. 2021. After excluding patients who did not meet the inclusion criteria, 1,099 primary adult-type diffuse glioma patients were eligible for inclusion in this study. Student t-test, Chi-squared test, and ANOVA were used for differential analysis. Kaplan-Meier curve and univariate and multivariate Cox regression analyses were used for survival analysis.

MAIN OUTCOMES AND MEASURES
Totally, 1,099 patients diagnosed with primary adult-type diffuse glioma were included in this study. The male-to-female ratio is about 1.4-1.5 to 1. The mean age at diagnosis for astrocytoma, IDH-mutant, oligodendroglioma, IDH-mutant and 1p/19q-codeleted and glioblastoma, IDH-wildtype were 39.0, 40.0, and 53.0 years, respectively. In astrocytoma, preoperative epilepsy, WHO grade, MGMT promoter methylation status, the extent of resection, and postoperative comprehensive treatment were independently associated with overall survival. For oligodendroglioma, gender, WHO grade, and extent of resection were associated with overall survival. As for glioblastoma patients, gender, age at diagnosis, preoperative KPS, preoperative epilepsy, the extent of resection, and postoperative comprehensive treatment were prognostically correlated.

CONCLUSIONS AND RELEVANCE
Patients in distinct molecular subtypes showed different epidemiological features and were associated with different clinical prognostic factors.
NCOG-08. BEVACIZUMAB ALONE VERSUS BEVACIZUMAB PLUS IRINOTECAN IN PATIENTS WITH RECURRENT GLIOBLASTOMA: A NATIONWIDE POPULATION-BASED STUDY
Authors: Lee Yeonhu , Roh Tae Hoon , Kim Se-Hyuk 
Published: November 2023

Abstract

INTRODUCTION
There is no established treatment for recurrent glioblastoma. For the treatment of recurrent glioblastoma, the antiangiogenic antibody, bevacizumab, is used either alone or in combination with Irinotecan. This study aimed to compare the survival of patients receiving bevacizumab monotherapy (BEV) and those receiving bevacizumab plus irinotecan combination therapy (B+I) in recurrent glioblastoma (GBM), using a nationwide population-based dataset.

METHODS
Patients matching the ICD code C71.x were screened from the national database. From January 2008 to November 2021, 846 patients who had undergone surgery or biopsy and received concurrent chemoradiotherapy with Temozolomide were included. Of these, 450 received BEV, while 396 received B+I. The patients' demographic characteristics, inpatient stay duration, prescription frequency, and survival outcomes were compared between the two groups.

RESULTS
The BEV group was older and had a higher percentage of females compared to the B+I group. The survival analysis revealed no significant difference in survival times between the two groups in the univariate and multivariate analysis. Younger age, female gender, and having surgery rather than biopsy were associated with better prognosis.

CONCLUSION
This study found no significant difference in overall survival between bevacizumab monotherapy and bevacizumab plus irinotecan combination therapy in patients with recurrent GBM. Considering the additional potential toxicity associated with irinotecan, bevacizumab monotherapy may be a suitable treatment option.
NCOG-11. PREOPERATIVE NANO SCORE AS A PREDICTOR FOR SURVIVAL IN GLIOBLASTOMA
Authors: Mireles Eduard Mendoza , Blakstad Hanne Kristin , Skaga Erlend , Helseth Eirik , Rønning Pål , Vik-Mo Einar 
Published: November 2023

Abstract

BACKGROUND
Age, molecular features, extent of resection, and adjuvant treatment are well established prognostic factors for patients with glioblastoma. Neurological Assessment in Neuro-Oncology (NANO) Scale was designed as a tool to provide an objective clinician-reported outcome of neurologic and complements the radiological evaluation of the Response Assessment in Neuro-Oncology (RANO) criteria. Despite the generally detrimental prognosis associated with severe new neurological deficits after surgery, a comprehensive understanding of the impact of pre- and postoperative neurological status remains limited. This study explores the potential of the NANO scale to predict survival when applied immediately before and after surgery.

METHODS
This retrospective study examined adult patients in South-Eastern Norway who underwent resection for IDH wildtype glioblastoma between January 2020 and December 2021. We used Kaplan Meier curve calculate median overall survival and chi-square and log rank test were used to compare groups.

RESULTS
The study included 187 patients, with a median overall survival was 14.9 months. Patients with a preoperative NANO-score of 0-2 had a median survival of 17.4 months, compared to 11.3 months for patients scoring 3 or more points (p&lt; 0.001). Neither Postoperative NANO scores nor changes in NANO scores in pre- to postoperative score were found to be significant predictors for survival.

CONCLUSIONS
This study indicates that a low preoperative NANO were predictive for a longer median survival, whereas postoperative NANO scores didn’t show the same impact. Surprisingly, an increase in the postoperative NANO score, indicating new, or worsening of the neurological deficits, didn′t correlate with a shorter median survival.
NCOG-12. SURVIVAL OF THE ELDERLY: OUTCOMES OF VERY OLD GLIOBLASTOMA PATIENTS – RESULTS FROM AN AUSTRIAN MULTICENTER STUDY
Authors: Demetz Matthias , Hecker Constantin , Krigers Aleksandrs , Machegger Lukas , Kerschbaumer Johannes , Pöppe Johannes , Geiger Philipp , Spinello Antonio , Griessenauer Christoph J , Thomé Claudius , Schwartz Christoph , Freyschlag Christian 
Published: November 2023

Abstract

INTRODUCTION
Glioblastoma (GBM) outcomes in very old patients remain understudied. Limited data exist on outcomes and treatment options for this specific population, necessitating further research. Materials and

METHODS
We retrospectively analyzed all elderly GBM patients ( &gt;65 years), who underwent treatment at two Austrian academic centers from 2007 to 2021. We conducted a comparative analysis between patients aged 65-79 years (old cohort) and a cohort of patients aged over 80 years (very old cohort).

RESULTS
387 patients were included, 43 of them over 80 years of age at the time of surgery. Median preoperative Karnofsky Performance Score (KPS) amounted to 80, median preoperative tumor volume was 26.44cm3. Among elderly patients 283 (75%) underwent resection and 61 (25%) only biopsy. 66.5% of the elderly cohort underwent adjuvant treatment following the EORTC protocol. 88 patients (25.6%) experienced preoperative seizures. Among the very old cohort 29 patients (67%) underwent resection and 14 (33%) biopsy only. 42.5% received adjuvant treatment, 5 patients (11.6%) presented with preoperative epilepsy. Mean PFS was 8.9 months in old patients and 8.4 months in very old patients. Kaplan Meier Analysis revealed a mean OS of 12.8 vs 10.7 months respectively. Elderly patients showed significantly better preoperative KPS (p=0.033) as well as higher preoperative tumor volumes (p=0.039). Old patients showed significantly higher incidences of both preoperative (p=0.043) and postoperative (p=0.032) epilepsy. Furthermore, old patients underwent significantly more often resection (p=0.020) and adjuvant therapy (p=0.009) compared to very old patients. However, survival analysis showed no statistic differences regarding PFS and OS between the two groups (p &gt;0.05).

CONCLUSION
Despite preexisting unfavorable prognostic features, we could not find any significant differences between old and very old GBM patients. Hence, the treatment of glioblastoma in very old patients should adopt a more inclusive approach considering the patient's overall health status and comorbidities.
NCOG-15. COMPARATIVE ANALYSIS OF GLIOMA INDUCED IMPAIRMENT MODELS
Authors: Kaur Jasleen , Aabedi Alexander , Brandes Kirsti , Hervey-Jumper Shawn 
Published: November 2023

Abstract
Speech is produced by the coordination of neural networks involved in language processing and motor control of vocal outputs, both of which can be disrupted by diffuse gliomas. Natural language is the instinctual written or spoken language profile that an individual has developed throughout his or her lifetime. Recent psycholinguistic analyses suggest that patterns of natural language may be an accurate measure for assessing clinical and behavioral conditions. One important feature of natural language is speech onset. Speech delays are common complaints among patients with glioma. Here, we assess the relationship between speech onset and histologic-molecular features of glioma. Speech onset was defined as acoustic measure of the interval of time between the release of the oral constriction in a stop consonant and the onset of subsequent voicing. Audio samples and video analysis of oral kinetics were analyzed from patients with newly diagnosed low- and high-grade glioma. Features were extracted by manual segmentation and aligned for each trial with high-density electrode arrays to record task specific responses from gliomas projecting to the cortical surface. A total of 1,120 stimuli (672 = picture naming (PN) and 448 auditory naming (AN) trials were presented. As expected, patients with glioblastoma had higher picture and auditory naming error rates (p &lt; 0.001, Mann-Whitney U test) compared with diffuse low-grade gliomas. Assuming speech errors produce slower response times, we next measured only correct behavioral responses which demonstrated 7% slower speech initiation responses (p &lt; 0.0001, Mann-Whitney U test) when comparing patients with glioblastoma and diffuse low-grade gliomas. Slowed speech responses did not require glioma infiltration into canonical cortical speech areas of the lateral prefrontal cortex and superior temporal gyrus. Finally, we identified electrophysiological biomarkers of speech errors. These results deepen our understanding of speech processing impairments, which are not based purely on coding errors.
NCOG-21. TESTING AND VALIDATION OF A DIFFUSE GLIOMA COGNITIVE RESERVE MODEL
Authors: Brandes Kirsti , Kaur Jasleen , Hervey-Jumper Shawn 
Published: November 2023

Abstract
Behavioral and cognitive symptom severity varies widely across patients with glioma because of unknown mechanisms. The concept of cognitive reserve (CR) recognizes that there are individual differences in each person’s brain reserve capacity and this theory accounts for the common disconnection between the degree of brain injury and its symptom manifestations. CR is a fixed variable throughout an individual’s life and works by recruitment of dormant yet redundant neural networks, and by efficient information modulation. New strategies to treat cognitive impairments have been encumbered by a lack of models that accurately reflect human disease. Here, we measure the Cognitive Reserve Index (CRI) in a registry of 450 patients with WHO grade 2-4 glioma. Transient behavioral impairments were induced by anesthetic medications propofol, dexmedetomidine, and remifentanil during standard of clinical care. We administered and analyzed 48 picture naming (PN) and 32 auditory stimulus naming (AN) trials per patient both before and after drug administration with impairments defined as a decline in task performance by more than one standard deviation from the mean. We discovered that the CRI in patients with glioma follows a normal distribution, like the general US population. Next, we found a significant decline in task performance in a subgroup of patients in both PN and AN. Electrode arrays in vivo to record human local field potentials from glioma infiltrated cortex demonstrated electrophysiological biomarkers of cognitive decline. Further elucidation and validation of CR measures in patients may significantly influence the delivery and timing of cancer directed therapies.
NCOG-29. THE NEURO-ONCOLOGY MAGNITUDE OF CLINICAL BENEFIT SCALE (NEURO-MCBS) AS A COMPREHENSIVE AND CLINICALLY RELEVANT ASSESSMENT TOOL TO DETERMINE CLINICAL BENEFIT FROM TARGETED THERAPIES IN CNS TUMORS
Authors: Kurz Sylvia C , Renovanz Mirjam , Rieger Johannes , Bombach Paula , Grosse Lucia , Rieger David , Hucker Svenja , Hille Hanni , Hippler Melina , Oener Oeznur , Ruhm Kristina , Beha Janina , Malek Holly S , Moeller Yvonne , Bitzer Michael , Malek Nisar , Tabatabai Ghazaleh 
Published: November 2023

Abstract

BACKGROUND
Once first-line therapy options fail, there are no effective therapy options for most neuro-oncological conditions, including glioblastoma, gliomas, and brain metastases, thereby representing a high unmet clinical need. Novel targeted therapy options are often explored in small phase-I studies or are given as off-label therapies. Assessment of treatment efficacy in this setting is challenging and should incorporate outcome measures such as progression-free survival (PFS) or objective response rate (ORR) as well as clinical parameters. To address this, we developed the Neuro-Oncology Magnitude of Clinical Benefit Scale (Neuro-MCBS) as a comprehensive clinical tool to assess treatment efficacy in patients with CNS tumors who receive off-label targeted therapies outside of clinical trials. We prospectively evaluated the feasibility and clinical utility of the Neuro MCBS in a neuro-oncology patient cohort enrolled in MTB@ZPM.

METHODS
MTB@ZPM (NCT03503149) is a longitudinal observational study that provides comprehensive molecular profiling, consensus-guided and ranked targeted therapy recommendations and collection of prospective clinical outcome data. The Neuro-MCBS (grade 1 + 2=clinical benefit, grade 3=minimal benefit, grade 0=no benefit) was applied and correlated with PFS, the PFS2/PFS1 ratio, performance status (KPS, ECOG), and imaging parameters.

RESULTS
From February 1st, 2022 until January 31st, 2023, 70 patients were enrolled. While only a minority of n=17/36 (47%) glioblastoma and n=2/8 (25%) IDH-mutant astrocytoma patients benefited from targeted therapies, n=7/8 (88%) meningioma, n=9/10 (90%) brain metastases, and n=5/8 (63%) other tumor patients achieved a Neuro-MCBS of 1 or 2, i.e. derived clinical benefit from the molecularly based therapy.

CONCLUSIONS
The Neuro-MCBS is a comprehensive and clinically relevant assessment tool that incorporates clinical, imaging, and survival outcome data to determine the degree of clinical benefit from targeted therapies in patients with primary and secondary CNS tumors. Its incorporation into clinical practice provides an objective outcome measure of clinical utility vs. futility of targeted therapies.
NCOG-35. WEIGHING BENEFITS OF IMPROVED LONG TERM SURVIVAL AGAINST THE EFFECTS OF POST-RADIOTHERAPY NEUROCOGNITIVE DECLINE: ROLE OF RADIOTHERAPY FOR TREATMENT OF PEDIATRIC PRIMARY CNS NEOPLASMS
Authors: Brown Nolan , Kuo Cathleen , Nguyen Oanh , Gendreau Julian 
Published: November 2023

Abstract

OBJECTIVE
To comprehensively review and weigh benefits of radiotherapy for pediatric primary central nervous system (CNS) tumors, which lengthens survival in the setting of potentially debilitating morbidity, including post-radiotherapy neurocognitive decline.

METHODS
In this review, the authors examined all the published literature analyzing results of clinical trials, case series, and technical notes on patients undergoing radiotherapy for the treatment of tumors of the central nervous system, with an emphasis on neurocognitive decline and survival outcomes.

RESULTS
Radiotherapy is an essential component of the treatment regimen for pediatric medulloblastoma. A recent trial demonstrated association between contemporary treatment protocols and significant post-radiotherapy neurotoxicity. A new protocol for WNT 2-driven medulloblastoma has been proposed and is undergoing testing: 18 Gy in 10 fractions plus a tumor-bed boost of 36 Gy in 20 fractions for a total primary site dose of 54 Gy in 30 fractions without concurrent chemotherapy followed by the standard six cycles of adjuvant systemic chemotherapy. Maximal surgical resection remains the mainstay of therapy for low grade glioma, as radiation therapy is reserved for subsequent recurrence or progression. Management paradigms are similar for high grade gliomas and ependymomas. Overall, radiotherapy allows for greater lifetime survival but also lengthens the morbidity from late effects, especially neurocognitive decline. This is largely due to the unintended consequence of radiation- induced damage to the normal neural tissue caught in the beam trajectory. Advancements in photon-based radiotherapy confined the volume of the brain that was irradiated to fit the tumor margins more closely; however, the advent of PBRT further allows for greater sparing of normal neuronal tissue, by maximizing energy deposition at the tumor location while minimizing energy deposition both proximal and distal to the tumor target.

CONCLUSION
Future research is needed to develop interventions for those already suffering from the late effects of post-radiotherapy neurocognitive decline.
NCOG-38. UNDERSTANDING GLIOMA IN LI-FRAUMENI SYNDROME: HIGHLIGHTING THE VALUE OF SCREENING AND GENE SEQUENCING
Authors: Padilla Hannah , Carabenciov Ivan , Uhm Joon , Neth Bryan 
Published: November 2023

Abstract
Li-Fraumeni Syndrome (LFS) is a rare autosomal dominant disorder characterized by a germline mutation in the TP53 gene that confers an increased risk of developing cancers, including brain tumors. There is continued need to understand how LFS diagnosis impacts clinical management and outcomes of gliomas. We report the clinical course of two patients from our Neuro-Oncology. Patient 1 is a male who was diagnosed with a low-grade astrocytoma, IDH1 mutant at age 20 status post gross total resection. Given radiographic concern for tumor progression, he underwent re-resection 8 years later. Pathology showed gliosis with TP53-mutant cells. Next-generation sequencing (NGS) was positive a germline TP53 mutation, consistent with LFS. He is currently in surveillance 12 years after diagnosis without need for radiation or chemotherapy. Patient 2 is a female who was diagnosed with glioblastoma, IDH wildtype, MGMT promoter unmethylated at age 40. She was asymptomatic at the time of diagnosis as the tumor was found during routine screening. She underwent gross total resection, radiation with concurrent and adjuvant temozolomide. She is now 5 years from last treatment without recurrence. These cases illustrate the clinical spectrum of glioma in LFS. Both patients have had prolonged periods of survival without progression, even accounting for respective tumor type. Patient 1 was only diagnosed with LFS after radiographic concerns for progression. Using NGS to diagnosis LFS was clinically important, as previous reports have shown greater propensity for malignant transformation of low-grade glioma in LFS. Patient 2 was diagnosed with glioblastoma during screening and has had no recurrence. This highlights the value of early diagnosis and treatment of gliomas in patients with LFS, even if asymptomatic at discovery. Further research is needed to understand the clinical spectrum of glioma in LFS and to optimize the care of this unique patient population.