bluuebunny/crossref_metadata_2025 huggingface dataset:
NIMG-67. REVISITING GLIOMATOSIS CEREBRI IN ADULT-TYPE DIFFUSE GLIOMAS: A COMPREHENSIVE ANALYSIS OF MANIFESTATION AND RISK FACTORS IN THE 2021 WHO CLASSIFICATION
Authors: Shin Ilah , Park Yae Won , Ahn Sung Soo , Chang Jong Hee
Published: November 2023

BACKGROUND

To investigate the incidence, clinicopathologic and imaging correlations and prognostic implications of gliomatosis cerebri (GC) pattern in adult-type diffuse glioma patients reflecting the 2021 WHO classification.

METHODS

Retrospective chart and imaging review was performed in 1,211 adult-type diffuse glioma patients at a single institution between 2005 and 2021. Clinicopathologic and imaging features were compared amongst adult-type diffuse glioma patients with and without GC based on the 2021 WHO classification. Clinical and imaging risk factors for predicting IDH mutation status within GC patients were investigated. Survival analysis was performed in entire patients and in subgroup of IDH-wildtype glioblastoma patients to evaluate whether GC is an independent prognostic factor.

RESULTS

Total 99 patients (8.9% of entire patients) showed GC. The distribution of tumor type was significantly different in patients with GC compared with those without GC (P = 0.017); IDH-wildtype glioblastoma being most common (77.8% vs. 68.9%), followed by IDH-mutant astrocytoma (16.2% vs 16.9%), and being less common in oligodendroglioma (6.1% vs 16.5%). Contrast enhancement, necrosis, cystic change, hemorrhage, and GC type 2 were independent risk factors for predicting IDH mutation status in GC patients. GC remained as an independent prognostic factor (HR=1.55, P=0.031) in IDH-wildtype glioblastoma patients on multivariable analyses.

CONCLUSIONS

The incidence of GC is 8.9% in adult-type diffuse gliomas, with IDH-wildtype glioblastoma showing significantly higher risk of GC. Preoperative imaging features may predict IDH mutation status in GC patients. GC is an independent prognostic factor in IDH-wildtype glioblastoma, and promote recognition of this pattern in clinical settings.
NIMG-73. RADIOGRAPHIC CHANGES AFTER RADIATION THERAPY IN SUBTOTALLY RESECTED ATYPICAL MENINGIOMAS
Authors: Yim Eunji , Pan Peter , Ivanidze Jana , Chiang Gloria , Pannullo Susan , Knisely Jonathan , Brandmaier Andrew , Fine Howard , Ramakrishna Rohan , Schwartz Theodore , Stieg Philip , Liechty Benjamin , Pisapia David , Magge Rajiv
Published: November 2023

BACKGROUND

Adjuvant radiotherapy (RT) has been shown to improve outcomes after subtotal resection (STR) of atypical meningioma, but little is known about how tumors evolve on magnetic resonance imaging (MRI) and whether this correlates with RT response.

METHODS

We retrospectively identified histologically confirmed cases of WHO Grade II atypical meningioma at a large academic institution from 2004-2020 who underwent subtotal resection and received radiation. Clinicodemographic, surgical, RT, and histopathologic data were collected, as well as clinical outcomes, with a median follow-up time of 69 months (IQR 61). Patients were stratified by external beam RT (EBRT) and stereotactic radiosurgery (SRS). Radiographic data included tumor location, cerebral edema, susceptibility changes, leptomeningeal enhancement, calcification, necrosis, and time to recurrence. Progression was defined by radiology report.

RESULTS

Of 31 patients with STR for newly diagnosed atypical meningioma, 65% (20 of 31) received RT and 11 of 20 (55%) received EBRT while nine received SRS. Of the convexity/parasagittal meningiomas (N=10), 40% demonstrated venous sinus invasion. 65% (N=13) of cases were treated upfront; the other seven progressed at a median 9.4 months before undergoing RT. Post-RT, 74% decreased in size (N=5) or stabilized (N=9); imaging was not available for one case. Recurrence after RT was observed in five cases. 11 cases developed restricted diffusion at median onset of 24.9 months; 7 developed adjacent leptomeningeal enhancement at median onset of 14.7 months, of which 86% (6 of 7) received SRS. Other patterns included adjacent nodular (N=3) and linear (N=2) enhancement, and adjacent confluent T2 signal (N=4); these could potentially represent radiation-related changes. Only one case developed calcification after 70 months.

CONCLUSIONS

Atypical meningiomas exhibit variable patterns of evolution after RT which may predict treatment outcomes. Multivariate analysis is planned to correlate radiographic changes with pathology characteristics, RT modality, and time to recurrence.
NIMG-75. POSTOPERATIVE68GA-DOTATATE/PET-CT IMAGING IS PROGNOSTIC FOR PROGRESSION-FREE SURVIVAL IN MENINGIOMA WHO GRADE 1: A PROSPECTIVE SINGLE-CENTER STUDY
Authors: Teske Nico , Biczok Annamaria , Quach Stefanie , Dekorsy Franziska J , Forbrig Robert , Bodensohn Raphael , Niyazi Maximilian , Tonn Joerg-Christian , Albert Nathalie , Schichor Christian , Ueberschaer Moritz
Published: November 2023

PURPOSE

Microsurgical tumor resection represents the first-line treatment for symptomatic meningiomas, and extent of resection has been shown to be of prognostic importance. Assessment of tumor remnants with somatostatin receptor PET proves to be superior to intraoperative estimation with Simpson grading or MRI. However, prognostic relevance of postoperative PET for progression-free survival in meningiomas remains unclear.

METHODS

We conducted a prospective study including patients with surgically removed meningioma WHO grade 1 and collected clinical and imaging data. All patients received postoperative MRI and 68Gallium-DOTATATE/PET, and were followed regularly with MRI surveillance scans for detection of tumor recurrence/progression.

RESULTS

We included 46 patients with 49 tumors. Mean age at diagnosis was 57.8 ± 1.7 years with a male-to-female ratio of 1:1.7. Local tumor progression occurred in 7/49 patients (14%) after a median follow-up of 52 months. Positive PET was associated with an increased risk for progression (*p = 0.015) and a lower progression-free survival (*p = 0.029) whereas MRI was not. 20/20 patients (100%) with negative PET findings remained recurrence-free. Location of recurrence/progression on MRI was adjacent to regions where postoperative PET indicated tumor remnants in all cases. Gross tumor volumes were higher on PET compared to MRI (*p = 0.032).

CONCLUSION

Our data show that 68Ga-DOTATATE/PET is highly sensitive in revealing tumor remnants in patients with meningioma WHO grade 1. Negative PET findings were associated with a higher progression-free survival, thus improving surveillance. In patients with tumor remnants, additional PET can optimize adjuvant radiotherapy target planning of surgically resected meningiomas.
NIMG-82. FET PET SENSITIVITY IN PRIMARY BRAIN TUMORS ACCORDING TO CNS WHO 2021: VALUE AND PITFALLS IN PRESURGICAL IMPLEMENTATION
Authors: Oster Christoph , Blau Tobias , Keyvani Kathy , Schmidt Teresa , Lazaridis Lazaros , Agkatsev Sarina , Sure Ulrich , Kleinschnitz Christoph , Scheffler Björn , Barbato Francesco , Herrmann Ken , Kebir Sied , Glas Martin
Published: November 2023

BACKGROUND

FET-PET imaging has emerged as a valuable tool in the characterization of unclear brain lesions that cannot be clearly diagnosed with conventional diagnostic methods. In 2021, the Central Nervous System (CNS) World Health Organization (WHO) introduced significant changes in the classification of CNS tumors. Notably, under the new classification, certain cases of WHO grade II glioma may be reclassified as glioblastoma. Unfortunately, a subset of grade II gliomas, as defined by the 2016 WHO classification, may exhibit no detectable FET uptake and lack contrast enhancement on MRI, which can lead to underestimation of such tumors and potentially result in inappropriate treatment decisions, including avoiding surgery. Question: In how many cases did the diagnostics consisting of MRI and FET PET underestimate the malignancy in unclear brain lesions?

METHODS

In this study, we conducted a retrospective analysis of all patients who underwent FET PET imaging of the brain between 2017 and June 2021, prior to their first surgical brain intervention. We obtained the results from presurgical MRI-scans and categorized the lesions based on whether they exhibited contrast media enhancement or not, in addition to the postsurgical molecular-histopathologic diagnosis. Our analysis focused on identifying the number of cases in which the diagnosis of malignancy was underestimated in the context of the WHO 2016 classification. To further investigate these cases, we conducted NGS and 850k-analysis to reclassify the results in accordance with the new CNS WHO 2021 classification.

RESULTS

After enrolling 85 patients, our study found that according to the 2016 classification, preoperative imaging underestimated malignancy in 14 cases (16%) where histopathological analysis revealed high-grade malignant brain tumors. In 32 cases (27%), the preoperative imaging correctly suggested a malignant tumor, which was later confirmed by histopathological analysis after surgery. The final results of this reclassification after the CNS-WHO-2021 will be presented at SNO-meeting.
NIMG-84. COMPARISON OF TRADITIONAL AND ADVANCED DEEP-LEARNING PATIENT SPECIFIC REGISTRATION METHODS IN PRESERVING TUMOR HABITAT IN GLIOBLASTOMA TUMORS: A PRELIMINARY STUDY
Authors: Safai Apoorva , Pineda Gustavo , Yadav Ipsa , Ismail Marwa , Tiwari Pallavi
Published: November 2023

PURPOSE

Registration of patient specific pre and post operative MRI scans in Glioblastoma patients is crucial to assess tumor progression, effect of radiation, and chemotherapy and survival prediction. However, in brain tumors longitudinal registration is challenging as tumor resection and mass effects causes tissue deformations and missing intensity correspondence across longitudinal scans. In this study, we compared effectiveness of traditional registration algorithms with state-of-the-art computationally extensive deep learning (DL) models in preserving intensity of tumor regions.

METHODS

Brain MRI scans (T1w,T1c,T2w,FLAIR) of 14 glioblastoma patients with first (tp1), intermediate (tp2) and follow up post operative scans (tp3) were obtained from two publicly available dataset (LUMIERE, XCURE). Preprocessing involved co-registration of all modalities within a timepoint, skull stripping, and uniformity in image dimensions, orientation, and origin across all timepoints. Tumor scans at tp2 and tp3 were registered to tp1 for all modalities independently, using different registration algorithms such as the traditional affine and symmetric diffeomorphic (SyN) registration with and without tumor masks using the ANTs software, along with the MICCAI-registration challenge winner DL algorithm (DIRAC). Intensity distribution of registered and unregistered scans for tp2 and tp3 were compared using the earth mover’s distance (EMD).

RESULTS

Traditional affine (tp2-0.074±0.06, tp3:0.058±0.051) and SyN (tp2-0.048±0.031 ,tp3:0.075±0.074) registration algorithm using T2w modality provided lower average EMD than DL method (tp2-0.624±0.69,tp3-0.595±0.67). Intensity distribution was better preserved for scans from intermediate timepoints compared to the post operative scans.

CONCLUSION

Our preliminary assessment suggests that traditional registration methods when implemented on pre-aligned MRI scans may better preserve image intensities in brain tumors scans compared to advanced and computationally expensive DL methods.
NIMG-86. ESTIMATING BRAIN TUMOR RELATED EDEMA FROM NON-INVASIVE IMAGING IN A CLINICALLY RELEVANT TIMELINE
Authors: Jackson Pamela , Ranjbar Sara , Singleton Kyle , Swanson Kristin
Published: November 2023

Image-based mathematical modeling is emerging as an advantageous tool to integrate into the care routine for patients with brain tumors. These tools can be used for a variety of tasks including predicting tumor growth, indicating spatial genomic alterations, and guiding radiation therapy. Often, the methods to produce these maps are developed in silos, using cohorts of retrospective patients and switching to a per patient map calculation can be an afterthought. Automatic pipelines are needed to enable creating mathematical maps in a clinically-relevant workflow. To this end, we developed a pipeline to create an image-based mathematical map of edema abundance based on T2-weighted (T2W) magnetic resonance imaging that includes preprocessing steps, abnormality and tissue type segmentation, brain extraction, map generation, and conversion between image file formats. The python-based pipeline was designed to take in T1-weighted (T1W), T1W with gadolinium contrast (T1Gd), T2W, and fluid attenuated inversion recovery (FLAIR) DICOM images for a single patient as input and convert them to NIfTIs for downstream steps. The pipeline preprocessed images by correcting bias field fluctuations using the N4 algorithm and normalizing intensities. The T1W, T1Gd, and FLAIR are then registered to the T2W. T1Gd and FLAIR are used in a deep learning algorithm to delineate the brain and abnormality including enhancing tumor, necrosis, and peripheral edema. Normal tissue segmentations are performed using the statistical parametric mapping (SPM) segmentation routine. The segmentations and preprocessed images are all utilized in the edema mathematical model. The resulting edema map is then reformatted as a DICOM and output for further use by clinicians and clinical imaging systems. Our future goals include integrating our pipeline in a clinical workstation and sending final maps to our hospital’s picture archiving and communication system (PACS) to allow for deployment of these models for patient care in a clinically efficient timeline.
NCMP-04. INITIAL REPORT OF BOSWELLIA SERRATA FOR MANAGEMENT OF CEREBRAL RADIATION NECROSIS AFTER STEREOTACTIC RADIOSURGERY FOR BRAIN METASTASES
Authors: Upadhyay Rituraj , Elguindy Ahmed Nader Mohammed , Sengupta Soma , Wang Kyle , Beyer Sasha , Thomas Evan , Raval Raju , Palmer Joshua
Published: November 2023

BACKGROUND

Radiation necrosis(RN) is a concerning late toxicity after radiation therapy(RT) for brain metastases(BM). Oral corticosteroids are the mainstay of management; however, not optimal given multiple side effects, particularly with the emergence of immunotherapy. Boswellia serrata(BS) is an over-the-counter supplement shown to reduce cerebral edema after brain RT. We evaluated the response rates with BS in a series of BM patients treated with stereotactic radiosurgery(SRS) who developed RN.

METHODS

We enrolled patients who developed RN after SRS for BM from 2020-2022 and were treated with BS 4.2-4.5g daily. Response was assessed using Response Assessment in Neuro-Oncology(RANO) criteria. Primary endpoint was ≥25% decrease in edema volume on T2-FLAIR MRI from baseline.

RESULTS

Fifty patients received BS for Grade 1-3 CTCAE v5.0 RN (G1=11, G2=36, and G3=3). Median time to RN was 10 months(m) after SRS and median follow-up was 6m. Best response was a complete response(CR) in 15% patients and partial response(PR) in 40% while 35% had stable disease(SD) and 10% had progression. At 3 , 6 , 9 and 12 months of follow-up respectively, 25%, 60%, 43% and 50% patients had any response(CR or PR). About 56% patients had symptomatic RN, of which 35.7% had improvement in symptoms with BS alone, while 64% required steroid use. Overall, median duration of response in patients with CR, PR or SD was 7.5m(range 2-31m). Salvage treatment for RN was steroids(33), surgery(4), Bevacizumab(5) or hyperbaric oxygen(1). No patient had ≥ CTCAE grade 3 toxicities, while 6% had grade 1-2 gastrointestinal intolerance. Overall, 39 patients remained on BS at last follow-up or death.

CONCLUSION

We observed > 50% response rates with use of BS in patients with grade 1-3 RN after SRS. Over 1/3rd patients with symptomatic RN were able to avoid long-term steroid use. Further prospective studies comparing Boswellia with placebo are warranted.
NCMP-06. INTRAMEDULLARY SPINAL CORD METASTASIS OF RENAL CELL CARCINOMA: A COMPARISON OF CASES AND TREATMENT OUTCOMES
Authors: Fukushima Corinna , Kuzel Timothy
Published: November 2023

Renal cell carcinoma (RCC) intramedullary spinal cord metastasis (ISCM) is a rare but frequently devastating diagnosis. Due to its rarity, there is currently no clear standard of care for RCC ISCM management. However, with improvements in metastatic RCC management and imaging for early detection of ISCM, we may see a rise in the incidence of RCC patients developing ISCM, for which treatment guidelines are lacking. Here, we aim to add to the growing body of RCC ISCM literature with two cases- one with ISCM managed with surgery, radiation, and immunotherapy, and the other managed with only radiation, immunotherapy, and chemotherapy. Case 1 is a 47 year old male with RCC and lung and bone metastases who underwent nephrectomy, lobectomy, high dose IL-2 therapy, and immunotherapy. He later developed worsening low back pain, and lower extremity paresthesias and weakness. Imaging revealed a thoracic metastasis for which he was started on steroids, underwent surgical resection followed by radiation therapy, and immunotherapy. Serial imaging and clinical exams have not found evidence of ISCM recurrence 5 years after initial resection. Case 2 is a 74 year old female with RCC with cerebellar metastasis who underwent nephrectomy, suboccipital craniotomy with mass resection, radiation therapy, and immunotherapy. 3 years later, she developed neck pain with right arm paresthesias, and imaging revealed cervical spine ISCM. Patient declined surgical intervention, and she was managed with steroids and radiation therapy, followed by chemotherapy. Approximately 5 years after initial ISCM findings, serial imaging and clinical exams have not revealed evidence of disease progression. These cases have several important differences including prior central nervous system metastases, ISCM location, and surgical intervention. However, both have not shown evidence of disease progression at similar time points. We hope these cases add to the small body of cases to move towards developing treatment guidelines.
NCMP-10. DEXAMETHASONE: A TOOL AND A PROBLEM IN GLIOBLASTOMA MANAGEMENT
Authors: Mohammad Amro , Jatana Sukhdeep , Sakalla Rawan , Davalan William , Khalaf Roy , Li Hongda , Aberra Rebecca , Carias Mathew , Al-saadi Tariq , Guevara-Moriones Natalia , Ruiz-Barrera Miguel Angel , Mindru Chritina , Diaz Roberto
Published: November 2023

Glioblastoma (GBM) patient morbidity and overall survival (OS) are associated with dexamethasone use. We studied dexamethasone-associated hyper-glycemia, leukocytosis, and accompanying complications in the peri-operative period. We also examined the effect of metformin treatment on GBM patient OS. We retrospectively studied 243 GBM patients admitted between 2014-2018. Patients with peri-operative glucose measurements and adequate follow-up to assess for complications were included. Metformin was used for treatment of diabetes or in the management of glucocorticoid-induced hyper-glycemia. Kaplan-Meier curves and log-rank p test were used for univariate analysis. Cox-proportional hazards model was used to generate adjusted hazard ratios for multivariate analysis. Dexamethasone dose was associated with hyper-glycemia and leukocytosis post-operatively. Poor glycemic control was associated with increased odds of 30-day any complication on univariate analysis and 30-day any complication and increased length of stay on multivariate analysis. We then demonstrated that metformin improved OS in methylated O6-methylguanine DNA methyltransferase gene (MGMT) promoter tumor patients (652 days, 95% CI 327 – 897, n = 25 vs 394 days, 95% CI 210 – 513, n = 63; P = .049). Non-diabetic MGMT-methylated tumor patients treated with metformin also had an improved median OS of 736 days, 95% CI 365 – 1036, n = 11 vs 395 days, 95% CI 210 – 513, n = 66; P = .01). Overall, peri-operative hyper-glycemia and higher average dexamethasone use are associated with poor glycemic control and leukocytosis. As a result, dexamethasone can increase the risk of peri-operative complications in GBM patients. Avoiding hyper-glycemia by limiting dexamethasone use or using Metformin may decrease the risk of complications and improve OS.
NCMP-14. HIGH GLIOMA PROLIFERATION SIGNIFICANTLY CORRELATES WITH LANGUAGE DEFICITS REGARDLESS OF MRI LESION SUBTYPE
Authors: Dada Abraham , Aabedi Alexander , Kaur Jasleen , Ahn EunSeon , Brang David , Hervey-Jumper Shawn
Published: November 2023

BACKGROUND

We recently demonstrated that glioma-infiltrated cortex engages in task-specific computations but may encode less information while doing so. The mechanistic underpinnings of glioma burden and its behavioral impact remains unknown

OBJECTIVE

To investigate the relationship between glioma burden and degree of cognitive impairments.

METHODS

One-hundred-eight patients with newly diagnosed WHO 2-4 diffuse glioma were recruited from a UCSF prospective registry. MRI FLAIR and T1 post-gadolinium tumor volume masks were demarcated. A “Composite” mask was generated from the sum of T1 post-gadolinium and FLAIR volume masks. Participants completed language assessments using the Quick Aphasia Battery (QAB). Glioma burden was determined by tumor-specific marker expression (IDH-1 mutation and P53) and glioma proliferation (Ki-67 and MIB-1 proliferation index). For each mask type, support-vector regression voxel-lesion symptom mapping (SVR-LSM) and linear regression analyses were performed to identify anatomic regions of interest (ROI) and glioma-specific factors associated with lower QAB scores.

RESULTS

SVR-LSM analysis by mask type revealed significant clusters in the left superior temporal gyrus: composite lesions within a cluster of 41,762 voxels and T1 post-gadolinium lesions within a cluster of 42,570 voxels. Both were independently predictive of lower QAB scores. SVR-LSM analysis of FLAIR masks failed to produce any significant clusters. Multivariate linear regression analysis of composite lesions was utilized to control for composite lesion voxel volume, glioma grade, and tumor-specific marker expression. The presence of voxels overlapping with the composite lesion ROI scores (beta = -0.13; p < 0.001), and, relative to a low glioma proliferation group (< 2%), glioma proliferation between 31-50% (beta = -2.00, p = 0.044) were independently and significantly predictive of lower QAB scores.

CONCLUSION

Relative to a low glioma proliferation group (< 2%), glioma proliferation between 31-50% significantly predicts language impairments, with a linear relationship between voxel overlap with ROI and impairment severity.
NCMP-21. AQP4-MOG DOUBLE-POSITIVE PARANEOPLASTIC NEUROLOGICAL SYNDROME IN A PATIENT WITH TRIPLE NEGATIVE BREAST CANCER
Authors: Siddiqui Amna , Ross Dylan , Jani Ronak , Prabhu Vikram , Lo Shelly , Wainwright Derek , Rouse Stasia , Refaat Tamer , Zhu Yirong , Thakkar Jigisha
Published: November 2023

We report a rare case of paraneoplastic neurological syndrome (PNS) with dual seropositivity of anti-aquaporin-4 (AQP-4) and myelin oligodendrocyte glycoprotein (MOG) antibodies in a 40 year-old lady with metastatic triple negative breast cancer with involvement of mediastinal, right axillary, and supraclavicular lymph nodes. She received multiple lines of anti-neoplastic treatment including immunotherapy with pembrolizumab as well as chemotherapy with taxol, carboplatin, gemcitabine, capecitabine, cytoxan, sacituzumab and nevalbine. Paraneoplastic meningoencephalomyelitis developed 2 years after diagnosis of breast cancer and 1 year after discontinuation of immunotherapy with pembrolizumab. She first developed longitudinally extending transverse myelitis extending from T11-12 that symptomatically improved with high dose intravenous methylprednisolone. One month later she developed left optic neuritis and meningoencephalitis with new enhancing lesions in the brain and spinal leptomeninges. Cerebrospinal fluid (CSF) analysis during both episodes revealed normal glucose, protein, elevated white blood cell count. Cytology was negative for malignancy. CSF was positive for neuromyelitis optica (NMO) IgG antibody APQ-4 and autoimmune myelopathy panel was positive for MOG antibody. Patient had significant clinical and radiographic improvement after completion of plasmapheresis and intravenous immunoglobulin. There was resolution of pleocytosis on repeat CSF testing. She did not have recurrence of paraneoplastic syndrome with maintenance rituximab every six months and daily low dose prednisone. She succumbed to progressive systemic metastatic disease 4.5 years after her breast cancer diagnosis. This case demonstrates that these antibodies can occur concurrently and cause clinical features like both neuromyelitis optica spectrum disorder (NMOSD) and myelin oligodendrocyte glycoprotein antibody disease (MOGAD) in a patient with a singular type of cancer. We highlight the importance of testing for paraneoplastic etiology in cancer patients with radiographic menigoencephalomyelitis or meningitis with atypical symptoms of meningeal carcinomatosis and/or CSF profile negative for leptomeningeal carcinomatosis.
NCMP-27. CHARACTERISTICS OF RADIATION-INDUCED BRAIN TUMORS AFTER CRANIAL IRRADIATION
Authors: Onishi Shumpei , Yamasaki Fumiyuki , Kinoshita Yasuyuki , Takayasu Takeshi , Yonezawa Ushio , Taguchi Akira , Ozono Iori , Khairunnisa Novita Ikbar , Horie Nobutaka
Published: November 2023

INTRODUCTION

The use of radiation therapy (RT) improves the outcome of cancer patients, while additionally increasing the risk of radiation-induced neoplasms in cancer survivors. The most common radiation-induced brain tumors (RIBT) are gliomas (RIG), meningiomas (RIM), and sarcomas (RIS). To investigate the characteristics of RIBT, we reviewed our case series and all relevant cases from the literature review.

METHODS

From our case series and literature review, we identified 920 RIBT patients who received cranial irradiation. We analyzed the age at irradiation for primary disease. The latency period from irradiation to the development of each RIBT and the median overall survival (OS) of RIBT were analyzed with Kaplan–Meier method.

RESULTS

Among RIBT patients, 23.2 % were irradiated less than 5 years, and 41.6% were irradiated in the first decade. The median age (interquartile range) at cranial irradiation were 9 (4.35 - 16) years in RIM patients, 9 (5 - 23) years in RIG patients, and 27 (13.7 - 40) years in RIS patients. The latency periods from irradiation to the development of RIM was significantly longer than RIG and RIS (RIM: 21 years, RIG: 9 years, RIS: 10 years; p < 0.0001). The median OS was significantly longer in patients with RIM than in those with RIG and RIS (RIM: not reached, RIG: 11 months, RIS 11 months; p < 0.0001).

CONCLUSIONS

RIG and RIS had a poor prognosis, while RIM had a relatively better prognosis. Because RIBTs develop more than a decade after cranial irradiation, long-term follow-up is crucial.
NCMP-29. THE NATURAL HISTORY OF NEUROLYMPHOMATOSIS
Authors: Xu Elizabeth , Ho Quan , Liu Ashley , Gautam Shiva , Wong Eric
Published: November 2023

Neurolymphomatosis is a rare lymphoid malignancy of the peripheral nervous system. Patients suffer from debilitating neurological deficits due to lymphoma invasion into one or more peripheral nerves and the natural history of this disease is still poorly understood. We performed a PubMed search and extracted data on year of publication, age, gender, type of lymphoma, nerves involved, imaging modalities, treatment received, and survival times. Kaplan-Meier statistics were used to determine outcome parameters over time and compare prognostic factors. Multiple comparisons were done to determine differences among groups. Our search identified 559 patients from 286 articles. Median age was 61 years (range 2-92) from 326 males, 222 females, and 11 with unspecified gender. Median overall survival (OS) was 12.0 months (range 10.0-15.0) for the entire cohort. Most frequent histologies were diffuse large B-cell lymphoma, unspecified T-cell lymphoma, and unspecified B-cell lymphoma, involving the brachial plexus, cranial nerves, and sciatic nerve. None had molecular profiling. There was a progressive lengthening of OS in successive decades, from 0.5 (95%CI 0.0-0.8) to 26.4 (95%CI 18.0-34.8) months from 1951-2022 (r2=0.9432, p< 0.001). Time from first treatment to progression also increased from 2.0 (95%CI N/A) to 28.3 (95%CI 23.1-33.5) months (r2=0.6923, p< 0.001). However, time from symptom onset to diagnosis remained unchanged during this period (r2=0.6206, p=0.277). Patients were most frequently treated with methotrexate-based chemotherapies (n=81), rituximab-based chemotherapies (n=176), and/or radiation (n=79). Only 12 received 3 or more regimens. Primary neurolymphomatosis had a better prognosis than secondary neurolymphomatosis, median OS 15.0 (95%CI 11.0-33.0) vs. 10.0 (95%CI 10.0-10.0) months (p=0.016). No OS difference was noted between B- and T-cell disease (p=0.4393), while patients with low-grade B-cell neurolymphomatosis did better than those with Burkitt's lymphoma (p≤0.001). Outcome for patients with neurolymphomatosis has improved over time. But timely diagnosis remains a major problem that needs improvement.
NCOG-02. MANAGEMENT OF PATIENTS WITH MELANOMA BRAIN METASTASES AND PRIOR SYSTEMIC TREATMENT EXPOSURE: A SINGLE-CENTER EXPERIENCE
Authors: Muniz Thiago P , Jablonska Paola A , Saibil Samuel D , Spreafico Anna , Hogg David , Shultz David , Butler Marcus O
Published: November 2023

Although systemic therapy (ST) for melanoma patients with untreated brain metastases (BrM) has activity, ST options for patients with melanoma BrM that develop on or after ST are limited. In this single-center retrospective study, we describe the characteristics and survival outcomes of patients aged > 18 years with metastatic melanoma and who developed BrM > 90 days after their first dose of checkpoint inhibitor (CPI) or targeted therapy (TT). Relevant clinical data and data on ST prior to and after BrM diagnosis were collected. MRIs were reviewed to assess intracranial progression-free survival (IC-PFS) as per RANO-BM criteria. The primary outcome was overall survival (OS). Cox regression model was applied to identify factors associated with OS. Between 2010 and 2019, we identified 103 patients with median age 56 (29-92) years, 69 (67%) of whom were male; 57 (55%) had BRAF V600-mutant tumors. Ninety-four (91%) had no known BrM at the time of first-line therapy. Median number of BrM was 3 (1-58), median number of prior ST was 1 (1-5), 75 (73%) had prior CPI exposure and 70 (68%) were on concurrent CPI or TT at the time of BrM diagnosis. Local treatments included SRS in 43 (42%) and WBRT in 46 (44%) patients. From BrM diagnosis, median IC-PFS and OS were 3.5 (95% CI 2.6-4.3) and 6.9 (95% CI 5.2-8.6) months, respectively. Adverse prognostic factors were: BRAF wild-type (HR 2.04, 95% CI 1.15-3.64), ECOG 2-4 (HR 3.55, 95% CI 1.93-6.53), and increasing number of prior ST (HR 1.62, 95% CI 1.23-2.13). CPI therapy that was continued, started, or restarted after BrM was associated with improved median OS compared to non-ICI therapy (9.9 vs. 3.5 months, HR 0.45, 95% CI 0.27-0.74). In conclusion, our data support development of CPI-based trials for patients who experience progression with BrM on or after modern ST.
NCOG-04. WHOLE-BRAIN STRUCTURAL CONNECTIVITY PREDICTS COGNITIVE DEFICITS IN PRETREATED PATIENTS WITH CNS WHO GRADE 3 OR 4 GLIOMAS
Authors: Friedrich Michel , Stoffels Gabriele , Filss Christian , Lohmann Philipp , Mottaghy Felix , Lucas Carolin Weiss , Ruge Maximilian , Shah Nadim , Caspers Svenja , Langen Karl-Josef , Fink Gereon , Galldiks Norbert , Kocher Martin
Published: November 2023

BACKGROUND

Glioma patients frequently suffer from cognitive dysfunction potentially caused by tumor invasion or treatment effects. We hypothesized that cognitive functioning in pretreated glioma patients critically depends on the maintained structural connectivity of multiple brain networks. PATIENTS AND

METHODS

The study included 121 pretreated glioma patients (median age, 52 years; median ECOG score, 1; CNS WHO grade, 3 or 4) who had biopsy or resection plus chemoradiation as first-line therapy. Cognitive performance was assessed by ten tests in five main cognitive domains after a median time of 14 months (range, 1-214 months) after treatment initiation. Hybrid amino acid PET/MRI using the tracer O-(2-[18F]fluoroethyl)-L-tyrosine, a network-based cortical parcellation, and advanced tractography tools were used to generate whole-brain fiber count-weighted connectivity matrices. These matrices were applied to a machine learning-based model to identify predictive fiber connections, essential cortical nodes, and the networks underlying cognitive performance in the evaluated domains.

RESULTS

Compared to healthy controls (n=121), the cognitive scores were significantly lower in nine cognitive tests. For each test, a subset of connections between nodes (median number, 254; range, 32-542) was identified whose fiber count sum was related to the actual scores in a linear model (median R2, 0.37; range, 0.16-0.44). Leave-one-out cross-validation confirmed the model's generalizability in 7 of 10 tests (median correlation coefficient for predicted vs. observed scores, 0.47; range, 0.39-0.57). Critically involved cortical regions (≥ 10 adjacent predictive edges) included predominantly left-sided cortical nodes of the visual, somatomotor, dorsal/ventral attention, and default mode networks. Highly critical nodes (≥ 15-20 edges) included the default-mode network’s left temporal and bilateral posterior cingulate cortex.

CONCLUSION

These results suggest that the cognitive performance of pretreated glioma patients is strongly related to structural connectivity between multiple brain networks and depends on the integrity of known network hubs also involved in other neurological disorders.
NCOG-09. EXAMINING THE CLINICAL UTILITY OF NEXT GENERATION SEQUENCING IN ADULT GLIOBLASTOMA – A SINGLE CENTER RETROSPECTIVE STUDY
Authors: Lomax Jerica , Taylor Thomas , Kong Xiao-Tang , Bota Daniela
Published: November 2023

BACKGROUND

Over the last 2 decades, the use of molecular targeted therapy has revolutionized the cancer treatment landscape and transformed the outcomes and disease prognoses of several cancer entities. The success of targeted therapy is closely tied to the advent of commercially available next generation sequencing (NGS) which has expanded our understanding and knowledge of the genetic drivers and mutations present in a variety of tumors. Unfortunately, in glioblastoma (GBM), the overall effectiveness of targeted therapies remains limited.1 Nonetheless, NGS for GBM patients has been integrated into the standard practice of many neuro-oncologists and is encouraged by the National Comprehensive Cancer Network (NCCN) Guidelines. The purpose of this retrospective chart review is to evaluate how NGS is incorporated into the management of glioblastoma at a single center academic institution.

METHODS

We identified 85 patients with pathologically confirmed GBM who had NGS done from January 2014-March 2021. The clinical records of all patients were reviewed to denote the frequency of clinical trial enrollment or off-label use of targeted therapy based on the results of NGS. We also denoted the time point in the disease course when NGS was ordered and the trend of frequency of ordering NGS from January 2014-December 2020.

RESULTS

28.2% (95% CI 19 to 39.1%) of patients received treatment based on the results of NGS. 8% were enrolled in a clinical trial and 20% were treated with off-label use of targeted therapy at the time of disease progression. There was no statistically significant survival difference seen between patients who had treatment done based on results of NGS compared to those who did not.

CONCLUSION

NGS results are used to guide treatment or clinical trial enrollment in a significant number of glioblastoma patients, however, the clinical efficacy of this treatment is yet to be determined.
NCOG-13. OUTCOMES OF CNS WHO GRADE 2 OLIGODENDROGLIOMA IN ADULT PATIENTS ; IMPACT OF ADJUVANT TREATMENT ON OVERALL RESULTS
Authors: Byeon Yukyeng , Song Sang Woo , Lee Chaejin
Published: November 2023

OBJECTIVE

Patients with oligodendroglioma (ODG), isocitrate dehydrogenase (IDH)-mutant, 1p19q codeleted, CNS WHO grade 2 have a median overall survival of longer than 10 years. Patients with ODG who are high-risk by RTOG criteria are offered radiation in combination with procarbazine, lomustine, and vincristine (PCV). The study aims to analyze the patient’s outcomes and assess the efficacy of postoperative treatment.

METHODS

A total of 138 adult patients who underwent resection for newly diagnosed grade 2 ODG between 1994 and 2021 were enrolled. Sixty-three (45.7%) were observed after gross total resection (GTR) or near total resection (NTR), 5 (0.4%) received adjuvant treatment after GTR or NTR, 37 (26.8%) were observed after subtotal resection (STR) or biopsy, and 33 (24.0%) received adjuvant treatment after STR or biopsy. The study analyzed progression-free survival (PFS), overall survival (OS), and risk variables.

RESULTS

Out of the 138 patients, 38 (27%) patients received adjuvant treatment, with 30 of them (78.9%) underwent radiotherapy only, 6 (15.8%) underwent radiochemotherapy, and 2 (5.3%) received chemotherapy alone. The median PFS was 6.8 years. Multivariate analysis revealed STR or biopsy (p=0.04), initial MR imaging enhancement (p=0.002), and postoperative observation(p=0.006) were associated with poorer PFS outcomes. The 5-year overall survival rate was 95.5% and the 10-year overall survival was 76.1%. Poor OS prognosis was associated with STR or biopsy (p=0.03), preoperative seizure (p=0.002), and initial MR imaging enhancement (p< 0.001).

CONCLUSIONS

: GTR and NTR is essential for achieving prolonged PFS and OS in ODG patients. Postoperative treatment is associated with favorable PFS outcomes but does not significantly affect OS. For an accurate diagnosis and appropriate treatment, the utmost care should be taken to avoid underestimating the results of the biopsy.
NCOG-17. RETROSPECTIVE REVIEW OF THE TREATMENT OF VENOUS THROMBOEMBOLISM IN GLIOBLASTOMA PATIENTS RECEIVING BEVACIZUMAB
Authors: Lomax Jerica , Kong Xiao-Tang
Published: November 2023

Patients with malignant gliomas have an inherently high risk of venous thromboembolism (VTE).1 Bevacizumab is an anti-vascular endothelial growth factor (VEGF) monoclonal antibody commonly used in the treatment of recurrent high-grade gliomas and is known to increase the risk of VTE as well as intracranial hemorrhage.2 Anticoagulation (AC) for VTE also increases the risk intracranial hemorrhage in patients with malignant brain tumors.3 This makes the treatment of VTE in patients with GBM receiving bevacizumab therapy particularly challenging. Here we completed a single center retrospective chart review of patients with glioblastoma (GBM) treated concurrently with anticoagulation for VTE and bevacizumab therapy. The purpose of this review was to assess the safety of concurrent use of these therapies and to note any statistically significant difference in the incidence of hemorrhagic complications between patients treated with anticoagulation at therapeutic versus prophylactic dosing. METHODS: 144 patients with a diagnosis of high-grade glioma and were treated at UCI from 1/1/2019 through 1/1/2022 were screened for the study. 13 GBM patients on treatment with bevacizumab and concurrent anticoagulation were included. Clinical records of these patients were reviewed for the incidence of intracranial hemorrhage or any clinically significant bleeding. RESULTS: The incidence of ICH in GBM patients on concurrent therapy with bevacizumab and anticoagulation was 7.7% and the incidence of other minor bleeding complications was 7.7%. ICH and other minor bleeding was exclusively seen in patients on therapeutic anticoagulant dosing. No bleeding complications were noted in patients on prophylactic AC dosing. CONCLUSION: There is a risk of ICH in patients receiving both bevacizumab and anticoagulation, however compared to historical data, the incidence reported in our study is less than the incidence of ICH in GBM patients on anticoagulation alone. Our study suggests that it is relatively safe to treat patients with these therapies simultaneously.
NCOG-18. CLINICAL CHARACTERISTICS OF H3.G34 DIFFUSE HEMISPHERIC GLIOMA: A CASE SERIES AND REVIEW OF THE LITERATURE
Authors: Williams Andrew , Neth Bryan , Campian Jian , Keating Gesina , Kizilbash Sani H , Lachance Daniel , Ruff Michael , Schwartz Jonathan , Uhm Joon , Atkinson John , Burns Terry , Daniels David , Nesvick Cody , Parney Ian , Breen William , Brown Paul , Mahajan Anita , Yan Elizabeth , Sener Ugur
Published: November 2023

BACKGROUND

Diffuse hemispheric glioma (DHG) is an uncommon infiltrating glioma, defined in the World Health Organization 2021 guidelines by a mutation in the H3F3A gene at codon 34 (H3.G34). DHG primarily affects children and young adults. Due to its rarity, there is paucity of knowledge regarding factors affecting overall survival (OS). We retrospectively assessed our institutional experience and present a review of the literature.

METHODS

We searched our institution’s pathology records to identify six cases of DHG. Demographic, clinical, diagnostic, treatment, and outcome data were recorded. We next searched for published articles regarding DHG cases via PubMed through May 2023. Four-hundred-fifty-four published individual cases of H3.G34 glioma were identified, 204 of which were hemispheric/supratentorial tumors with OS data reported, providing a combined 210 cases for analysis. 132 of these cases included MGMT promoter methylation status. 111 included extent of surgical resection. Kaplan-Meier and Cox regression survival analysis was performed.

RESULTS

Age range for DHG patients was 7-66 years (median 17). Median OS was 18.5 months (95% CI, 17-25). Greater extent of surgical resection and presence of MGMT promoter methylation were associated with better prognosis. Median OS for biopsy alone was 12 months (95% CI, 10-NA), subtotal resection was 16 months (95% CI, 14-23), and gross total resection was 27 months (95% CI 23-NA) – log-rank p< 0.001. OS for MGMT promoter unmethylated was 14 months (95% CI 12-22), MGMT promoter methylated was 24 months (95% CI, 17-30) – log-rank p=0.028. Contrary to previous reports, patient age was not found to impact prognosis (using 18, 21, and 40 years as cutoffs or as a continuous variable with Cox regression).

CONCLUSION

Extent of surgical resection and MGMT promoter methylation status are important prognostic factors for DHG. Unlike many primary brain tumors, survival does not appear to be significantly impacted by age.
NCOG-19. SEX DIFFERENCES IN GLIOBLASTOMA TREATMENT AND ADVERSE EVENTS
Authors: Dmukauskas Mantas , Cioffi Gino , Neff Corey , Price Mackenzie , Waite Kristin , Kruchko Carol , Ostrom Quinn , Barnholtz-Sloan Jill
Published: November 2023

Despite aggressive therapies, the survival rates for gliomas remain complex and accumulating evidence indicates that there are sex differences in patient response to treatment and the development of adverse events (AE). We utilized the Surveillance, Epidemiology and End. RESULTS: Program (SEER)-Medicare dataset to examine sex differences in treatment patterns and AEs in patients 65 years and older diagnosed with glioblastoma. We identified 15,693 individuals (≥65 years) diagnosed with glioblastoma between 2004-2017. Sex differences were assessed based upon their treatment modality: no-treatment (NT), standard treatment (ST) (surgery + radiation + chemotherapy), or other treatment (OT) (any other combination of surgery, radiation and/or chemotherapy). Multivariable models were used to determine the odds of males versus females experiencing an AE by treatment group. Sex differences in survival were assessed using Kaplan-Meier and Cox proportional hazards models. There was a significant sex difference in the type of treatment received by glioblastoma patients, with males receiving more ST than females (38% v 34%; p< 0.001). Females treated with ST had increased odds of rheumatoid arthritis or collagen vascular disease (OR=0.42; 95%CI, 0.30-0.59; p< 0.001). Females receiving OT had an increased odds of rheumatoid arthritis or collagen vascular disease (OR=0.59; 95%CI, 0.44-0.78; p< 0.001), and mood disorders (OR=0.81; 95%CI, 0.72-0.90; p< 0.001). Males receiving NT had an increased odds of blood disorders (OR=1.48; 95% CI, 1.12-1.96; p=0.006), cardiac events when treated with ST (OR=1.24; 95%CI, 1.10-1.41; p< 0.001), and respiratory events when treated with OT (OR=1.20; 95%CI, 1.07-1.35; p=0.002). Females with glioblastoma, receiving ST had an increased adjusted overall survival compared to males (p< 0.001), whereas no sex difference were observed in those receiving OT or NT. Our analysis demonstrates that there are sex differences in odds of AE and overall survival associated glioblastoma treatment, which could impact clinical treatment decisions.
NCOG-25. SINGLE-INSTITUTION SERIES OF SPINAL EPENDYMOMA IN CHILDREN WITH NF2-RELATED SCHWANNOMATOSIS
Authors: Burket Noah , Koenig Jenna , Tailor Jignesh
Published: November 2023

NF2-related schwannomatosis (NrS) is a tumor predisposition syndrome that results in the development of multiple central nervous system tumors, including spinal ependymomas (SP-EPN). SP-EPN are intramedullary tumors that are slow growing but can displace critical nerve pathways in the spinal cord, resulting in motor and sensory deficits that contribute to patient morbidity. Although surgery is the mainstay of treatment, it can also result in high morbidity, resection of sporadic SP-EPN is often curative; however, SP-EPN in NrS patients will often regrow, possibly resulting in additional surgeries and a lifetime of deficits, especially impacting pediatric patients. Yet, natural history and clinical outcomes for pediatric NrS patients with SP-EPN have not been well described. We reviewed 27 pediatric NrS patient cases from 1993 to 2023 at our institution and identified 10 patients with a diagnosis of SP-EPN, with a range of 1-3 tumors present at patient diagnosis. Demographic, clinical, pathologic, and radiologic data were collected from electronic medical records. Median age of diagnosis of NrS was 12 years old (range: 5-17) and median time from NrS diagnosis to SP-EPN diagnosis was 31 days (range: -1-1636). 14 out of 16 (87.5%) initial tumors were in the cervical spine region, with C2 being the most frequently involved level. Two patients underwent resection of their tumors, with both experiencing progression of their disease and one developing paraplegia. The most common symptoms from SP-EPN were pain (30%), motor deficits (30%), and sensory deficits (30%). 40% of the patients had progressive disease at last follow up, with median time from diagnosis to progression of 3.33 years. Median time from SP-EPN to last follow-up was 4.08 years. This series showcases the burden of SP-EPN in pediatric NrS patients, emphasizing the need for further exploration into clinical outcomes and novel treatments for this debilitating condition.