tbuckley/cabot-search huggingface dataset:
RADI-16. RANO CRITERIA AND VOLUMETRIC TUMOR MEASUREMENT PRODUCE SIGNIFICANTLY INCONGRUOUS RESULTS IN NON-RADIATED LOW GRADE ASTROCYTOMAS WHICH MAY LEAD TO MISINTERPRETATION OF THERAPY RESPONSE
Authors: Dianna Bardo, Jeffrey Miller, Patricia Cornejo, Marrit Thorkelson, Robyn Augystyn, Elizabeth Eden, Michael Etzl
Journal: Neuro-Oncology (2018)

Accurate tumor measurement and response to therapy continue to be a challenge due to tumor heterogeneity, variation of imaging techniques and contrast administration and inter-observer variability. Structural imaging (T1 with enhancement, T2, FLAIR) plus multimodal imaging (diffusion, perfusion, 1H-spectroscopy, PET) combined with clinical information and guidelines from the RANO and RAPNO working groups are very helpful. However, for unresectable, non-radiated low grade asstrocytomas with ill-defined borders accurate measurement is critical to assess response to therapy. We selected three patients with low-grade, mid-brain astrocytoma following biopsy or incomplete resection who received only chemotherapy. Three Pediatric Neuroradiologists measured the tumors at three time points in the longitudinal and two orthogonal short axes; RANO, linear measurements, and calculated tumor volume were recorded. Two experienced 3D-technologists used tumor segmentation software (IntelliSpace Portal, Philips, Cleveland, OH) to determine tumor volume. Percent change in tumor measurements over the three time points: Patient 1: RANO 0.41%, calculated volume 23.1%, segmented volume 48%. Patient 2: RANO 11.5%, calculated volume 14.6%, segmented volume 1.9%. Patient 3: RANO 1%, calculated volume 4.4%, segmented volume 8%. For our selected patients 3D measurements differed significantly and were incongruous with RANO measurements, indicating both minimal change and marked interval tumor growth in the same patient over time. Larger studies are required to confirm accuracy and feasibility of 3D volumetric analysis as a method to measure response to therapy in future low grade astrocytoma clinical trials.
NSRG-11. DIVERSION OF CSF WITH PROGRAMMABLE VENTRICULOPERITONEAL SHUNTS: ROLE WITH INTRAVENTRICULAR RADIOIMMUNOTHERAPY
Authors: Kim Kramer, Maria Donzelli, Milan Grkovski, Jeffrey Greenfield, Mark Souweidane, Neeta Pandit Taskar
Journal: Neuro-Oncology (2018)

The success of programmable ventriculoperitoneal shunts (pVP-shunts) in fully diverting therapeutics into cerebrospinal fluid (CSF) has not been reported. Patients with CNS tumors under consideration for intraventricular radioimmunotherapy were assessed with pre-treatment CSF flow studies. pVP-shunts were adjusted pre-administration, and readjusted to baseline approximately 4-5 hours post. 111-In-DTPA was injected into the pVP-shunts; nuclear images obtained immediately, 4-5 and 24 hours post-injection. Patients receiving radioimmunotherapy underwent the same procedure, with serial PET or SPECT imaging 2 to 48 hours using 124I/131I radiolabeled antibody. Imaging assessed CSF flow and adequacy of drug administration. 21 patients (ages 1 - 51 years) had medulloblastoma (N=9), choroid plexus carcinoma (N=2), rhabdoid tumor (N=2), ependymoma (N=1), metastatic neuroblastoma (N=4), retinoblastoma (N=2), or melanoma (N=1). On CSF flow studies, 5 patients (24%) had normal flow, 10 (48%) had delayed thecal sac ascent or descent. Of these, 4 had shunt revision or contralateral Ommaya placement; 3 then achieved normal flow. Six patients (29%) had adequate CSF flow down the thecal space with some activity in the peritoneum (6-23%). Similar drug localization was observed on nuclear scans after radioimmunotherapy, with PET and SPECT having greater sensitivity in detecting activity in the peritoneum. While pVP-shunts are effective intraventricular devices, normal CSF flow is seen in 24% of patients. Drug accumulation (up to 23%) in the peritoneum is still observed in 29% of patients. Shunt revision and/or contralateral Ommaya catheter placement may still be required to address drug delivery.
Enhanced activation of interleukin-10, heme oxygenase-1, and AKT in C5aR2-deficient mice is associated with protection from ischemia reperfusion injury–induced inflammation and fibrosis
Authors: Anja Thorenz, Katja Derlin, Christoph Schröder, Lisa Dressler, Vijith Vijayan, Pooja Pradhan, Stephan Immenschuh, Anne Jörns, Frank Echtermeyer, Christine Herzog, Rongjun Chen, Song Rong, Jan Hinrich Bräsen, Cees van Kooten, Torsten Kirsch, Christian Klemann, Martin Meier, Andreas Klos, Hermann Haller, Bennet Hensen, Faikah Gueler
Journal: Kidney International (2018)
Leveraging molecular quantitative trait loci to understand the genetic architecture of diseases and complex traits
Authors: Farhad Hormozdiari, Steven Gazal, Bryce van de Geijn, Hilary K. Finucane, Chelsea J.-T. Ju, Po-Ru Loh, Armin Schoech, Yakir Reshef, Xuanyao Liu, Luke O’Connor, Alexander Gusev, Eleazar Eskin, Alkes L. Price
Journal: Nature Genetics (2018)
LGG-12. ANGIOCENTRIC GLIOMA - EXPLORING A NOVEL THERAPEUTIC OPTION
Authors: Sayani Tewari, Rene McNall-Knapp
Journal: Neuro-Oncology (2018)

Angiocentric glioma (AG) is a slow-growing WHO grade I tumor classified as “other glioma” in 2016. Commonest location is supratentorial with a few reported elsewhere. Resection is curative. Treatment poses a challenge when resection is unattainable because of location. Per literature review 23 published articles described some form of therapy for AG. Only one article reported two cases receiving chemotherapy, vinblastine with bevacizumab in first, everolimus in second. The best response was stable disease. Almost all AGs have MYB rearrangement, often with its fusion partner QKI. On analysis of functional significance of MYB-QKI-driven transcriptional events with Connectivity Map, tyrosine kinase receptor c-KIT was highly upregulated. A 7 yo male presented with headache, nausea, vomiting and unsteady gait with CT head showing intraventricular mass and hydrocephalus. MRI brain showed tumor centered in third ventricle extending to thalami bilaterally. Only partial resection was feasible. Pathology review identified the tumor as AG with MYB rearrangement. Initially we observed without treatment, but 10 months later MRI showed slight tumor progression. We started standard LGG treatment with weekly carboplatin and vincristine. The tumor slowly grew and nine months into chemotherapy, alternative options were pursued. Based on effect of MYB-QKI rearrangement on c-kit, dasatinib was started. He is tolerating it well and is neurologically stable. Six months into treatment MRI shows a stable tumor. Due to the slow growth rate at baseline, he needs longer follow-up to reach a definitive conclusion about role of dasatinib in progressive AG and its potential as targeted therapy for AG.
QOL-20. THERAPY ASSESSMENT TOOL FOR LONG TERM SURVIVORS OF PAEDIATRIC CNS MALIGNANCIES
Authors: Louise Graham, Ailie Hodgson, Elizabeth Rowen, Sara Thompson, Fiona Court, Karen Heale, Jade Ryles, Sandra Barlow, Gail Halliday, Sarah Watts, Simon Bailey
Journal: Neuro-Oncology (2018)

Children and young people treated for central nervous system tumours are at risk of developing significant morbidities and complex late effects. NICE guidelines state the management of these morbidities should be provided by a multi-disciplinary team (MDT). Specialist paediatric oncology therapists have wide-ranging skills and knowledge and can holistically manage challenges these children and young people are faced with. Local therapy services can follow-up care but don’t have the specialist skills that the principle treatment centre can provide and are restricted by caseload pressures and resources. To develop an assessment tool to prioritise and categorise long-term problems affecting function and quality of life. To aid timely and appropriate referrals into local services to improve quality of survivorship. Discussion with the MDT to develop a suitable assessment tool and user guide which would identify therapeutic need. Assessment tool piloted and modified to ensure user friendliness and accurate interpretation of answers. Results collected and referral made if need identified. 46 patients were assessed in the first 12 months, 18 of which were identified to have therapeutic needs and referrals into local therapy services were made. Advice and education was provided regarding healthy life style. Encouragement and guidance offered to enable participation in activities of daily life. Assessment tool identifies non-medical late effects and results in appropriate and timely further management. Ongoing plan to develop a satellite education day for community services to provide guidance and support on best management for patients to optimise their quality of life.
Abstract 16386: Establishment of Enzyme-Linked Immunosorbent Assay for Oxidized High Density Lipoprotein and its Clinical Application for Cardiovascular Risk Assessment
Authors: Takeshi Okada, Tohru Ohama, Mizuki Sumida, Yuki Katayama, Kotaro Kanno, Hibiki Matsuda, Masami Sairyo, Yinghong Zhu, Ayami Saga, Takuya Kobayashi, Daisaku Masuda, Masahiro Koseki, Makoto Nishida, Norihiko Kayahara, Yasushi Sakata, Shizuya Yamashita
Journal: Circulation (2016)

Background:  Observational studies have shown that plasma HDL-C levels are negatively correlated with cardiovascular events. HDL particles have a variety of anti-atherogenic functions such as cholesterol efflux and its transport to the liver. However,  in vitro  studies also elucidated that HDL from coronary artery disease patients become dysfunctional or even proinflammatory. One possible mechanism for formation of dysfunctional HDL is the oxidation of HDL component. Therefore, the quantification of oxidized HDL (Ox-HDL) could be an useful and easy strategy to assess the quality or functionality of HDL.   Methods:  Using DLH3 antibody that can specifically recognize oxidized phosphatidylcholine, we have developed a sandwich enzyme-linked immunosorbent assay (ELISA) for Ox-HDL. We defined forced-oxidation of 1 mg/L HDL phospholipids (HDL-PL) as 1 U/L Ox-HDL. We analyzed the serum of 118 healthy normolipidemic controls and 173 dyslipidemic outpatients respectively.   Results:  Intra and inter-assay coefficients of variation for this ELISA were both less than 15%. In healthy controls, the serum Ox-HDL levels were 28.3 ± 4.9 U/L (mean ± SD). Ox-HDL levels were moderately correlated with HDL-PL levels (r=0.59), therefore we also evaluated Ox-HDL/HDL-PL ratio, which represents what proportion of phospholipids is oxidized in the HDL fraction. Ox-HDL/HDL-PL ratio in healthy controls was 0.232 ± 0.036. In dyslipidemic patients treated with drugs, the serum Ox-HDL levels were 27.2 ± 8.7 U/L, displayed quite a high variability ranging from 7.2 to 62.1 U/L. The serum Ox-HDL levels correlated positively with serum HDL-C levels and were extremely high (50.4 ± 13.3 U/L) in patients with hyperalphalipoproteinemia due to CETP deficiency. On the other hand, patients treated with probucol, which is a potent anti-oxidative anti-hyperlipidemic drug, showed significantly lower Ox-HDL (18.7 ± 6.6 vs 29.7 ± 7.7, p   Conclusion:  Our sandwich ELISA for Ox-HDL showed that Ox-HDL levels are very high in CETP-deficient patients, suggesting the presence of dysfunctional HDL. Probucol may prevent the formation of these Ox-HDLs.
LGG-30. EVALUATING PEDIATRIC SPINAL LOW-GRADE GLIOMAS-A 30-YEAR RETROSPECTIVE ANALYSIS
Authors: Steven Carey, Zsila Sadighi, Shengjie Wu, Jason Chiang, Yahya Ghazwani, Sahaja Acharya, Fredrick Boop, Amar Gajjar, Ibrahim Qaddoumi
Journal: Neuro-Oncology (2018)

Most pediatric spinal tumors are low-grade gliomas (LGGs). Characterization of these tumors has been difficult given their heterogeneity and rare incidence. Spinal tumors diagnosed in pediatric patients at our institution between 1984 and 2014 were reviewed retrospectively. Demographics, clinical presentation, pathology, imaging, management, and late effects were examined. Of the 730 patients with LGG, forty patients (5.5%) had spinal tumors, twenty-four (60%) of which were pilocytic astrocytomas. The most common initial presentations were pain (n=15), partial extremity paralysis (n=13), and ataxia (n=11), with the diagnosis frequently delayed by months and sometimes years (range: <1 week to 6 years). Seventy-five percent of tumors were at least partially resected, and more than half of those resected required adjuvant therapy with chemotherapy (n=10) or radiation (n=7). Nine other patients received a biopsy for histologic diagnosis and were treated with either chemotherapy (n=3) or radiation (n=5) or only followed (n=1). Tumor progression was noted in 35% of the patients, mostly locally; and 10 of 14 underwent salvage therapy (repeat surgery, radiation, or chemotherapy) without further progression. During the evaluation period (median follow-up: 7 years), two patients died, both secondary to tumor progression. Long-term sequelae of the disease/treatment were mostly residual neurologic deficits (paresthesia, paralysis), chemotherapy-induced hearing loss, and scoliosis. In conclusion, spinal LGG is a rare entity with significant long-term effects. Due to the tumors’ relatively slow growth and non-specific presentation, their diagnosis is frequently delayed, which could negatively affect the long-term sequelae in patients with these tumors.
High-Sensitivity Troponin and Heart Failure With Preserved Ejection Fraction—Balancing <i>P</i> Values With Prior Knowledge and Common Sense
Authors: John W. McEvoy
Journal: JAMA Cardiology (2018)

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The therapeutic effect of newly developed endoscopic irreversible electroporation ablative device in gastrointestinal tract: Application to live porcine esophagus, stomach and rectum
Authors: J. Lee, S. Choi, S. Kim, J. Lee, H. Choi, E. Kim, B. Keum, Y. Jeen, H. Chun, H. Lee, C. Kim
Journal: Annals of Oncology (2018)

Introduction: Irreversible electroporation (IRE) is known to remove undesired cells by affecting the cell membrane without thermally destructing connective tissues. Clinically IRE ablation is just performed recently on the target of pancreas and liver but not in gastrointestinal(GI) tract yet. We developed IRE ablative device that can be deployed through the channel of endoscopy and applied it to live porcine GI tract to study effectiveness and feasibility. Methods: Endoscopic IRE ablation was performed on the live porcine mucosa of esophagus, stomach and rectum with a voltage of 1HZ but in different amplitude and pulse number each. On the esophagus, ablation was done in 3 points. Range of amplitude was 1.0KVcm to 1.5KVcm and pulse number was 20 to 50. The stomach was ablated in 10 different points with an amplitude from 500V/cm to 2KV/cm and pulse from 20 to 50. In rectum, 5 points were ablated with amplitude of 1.0KV/cm to 2.0KV/cm and pulse was 10 to 20. The pigs were sacrificed next day. Ablated tissues were collected with surgical technique. Following fixation, tissues were stained with H&E. Results: Neither during the procedure nor in post-IRE ablation state, accompanied any complications in all porcine subjects. The novel endoscopic IRE device showed feasibility of effective ablation in live porcine GI tract. IRE ablative effect of electrical stimulus differs with variables such as amplitude and pulse number. Different range of mucosal and submucosal necrosis according to the degree of electrical stimuli without thermal damage to connective tissue such as submucosal vessel structure. Under certain circumstance of electrical stimulus, IRE ablative effect varies with each part of GI tract. When amplitude was 2000V/cm and pulse number was 50, necrosis was noted from mucosa, submucosa and even in muscle layer, prominent inflammation was noted. However, in case of esophagus, only squamous epithelial separation was noted with intact submucosa under the same electrical stimuli. Conclusion: Our endoscopic IRE ablation device performed different degree of decellularization in accordance with different electrical stimuli in each part of GI tract. Endoscopic IRE ablation could be promising technique in the treatment of GI tract disease.
Prognostic Implications of Single-Sample Confirmatory Testing for Undiagnosed Diabetes
Authors: Elizabeth Selvin, Dan Wang, Kunihiro Matsushita, Morgan E. Grams, Josef Coresh
Journal: Annals of Internal Medicine (2018)

Current clinical definitions of diabetes require repeated blood work to confirm elevated levels of glucose or hemoglobin A1c (HbA1c) to reduce the possibility of a false-positive diagnosis. Whether 2 different tests from a single blood sample provide adequate confirmation is uncertain.
HGG-40. HIGH GRADE GLIOMA CELL LINE COHORT AS AN EXAMPLE OF CHILDREN’S BRAIN TUMOR TISSUE CONSORTIUM TUMOR SPECIMEN PROCESSING PIPELINE
Authors: Mateusz Koptyra, Valerie Baubet, Lamiya Tauhid, Namrata Choudhari, Tiffany Smith, Kristina Cole, Heba Ijaz, Angela Waanders, Adam Resnick
Journal: Neuro-Oncology (2018)

The Children’s Brain Tumor Tissue Consortium (CBTTC) is a multi-institutional collaborative research program dedicated to accelerating pediatric brain tumor research. Tumor specimens collected by CBTTC undergo multidimensional tumor analysis including genomic, transcriptomic and proteomic data generation, providing freely available data for researchers worldwide. Moreover, CBTTC advances pediatric research efforts by generating cell lines and and patient-derived xenografts (PDX) providing these freely available materials for researchers worldwide. High-grade gliomas (HGG) in children is the most prevalent among brain tumors with a dismal prognosis. Preclinical testing has been limited due to lack of pediatric specific HGG in vitro and in vivo models. Here we present a cohort of pediatric HGG cell lines developed within the CBTTC. The cells were derived from 10 subjects from fresh or frozen tumor tissues and generated in different culture conditions. Results include the cells’ basic molecular characteristics and tumor formation in vivo, which presents valuable information for preclinical studies. We also report how our cell culture conditions affect the morphology, growth and genetic makeup of developed tumor cell lines. These results can intersect with tumor molecular profiling to provide a transparent view of cell line utility for cell biology and drug development studies. This project serves as an example of CBTTC’s pipeline for cell line development, and brings important insights for the processes of brain tumor specimen derived tool generation.
A20 and ABIN-1 team up against intestinal epithelial cell death
Authors: Ken Cadwell
Journal: The Journal of Experimental Medicine (2018)

A20 and its binding partner ABIN-1 are genetically linked to inflammatory diseases. In this issue of JEM, Kattah et al. (https://doi.org/10.1084/jem.20180198) demonstrate that simultaneous deletion in a mouse model leads to instantaneous cell death in the intestinal epithelium and mortality.
QOL-31. QUALITY OF LIFE (QOL) ACCORDING TO RISK GROUPS OF MEDULLOBLASTOMA SURVIVORS
Authors: Marta Perek-Polnik, Justyna Korzeniewska, Marta Grudzinska, Monika Drogosiewicz, Ewa Swieszkowska, Iwona Filipek, Pawel Kowalczyk, Marzanna Chojnacka, Danuta Perek, Bozenna Dembowska-Baginska
Journal: Neuro-Oncology (2018)

The aim of the study was to asses QoL in Medulloblastoma (MB) survivors according to risk groups. MATERIAL AND Between 2005-2015 132 patients (40 girls, 92 boys aged 2 9/12 – 17 6/12) were treated of MB. 44 patients were standard risk and 88 high risk. Patients in SR group received radiotherapy to CNS 25Gy whereas HR - 36Gy. Age at analysis 6 1/12 to 29. Analysis included 5 year EFS/OS and assessment of: weight/height in percentiles according to risk groups and age at diagnosis, hearing loss, hearing aids, hair loss and occurrence of posterior fossa syndrome(PFS). Psychosocial assessment, including IQ, education, work, physical and social activities, interpersonal relationships was performed and compared in risk groups. 5 years EFS was 83% in SR and 76,6% HR (p=0,112), OS 89,7% and 82,3%. There was no significant difference in hearing loss between risk groups, but there was greater use of hearing aids in the latter (p=0,003), hair loss was markedly worse in HR (p=0,022). SR patients and those older than 10 years at diagnosis were higher as compared to other survivors. PFS occurred more often in HR group (p=0,005). IQ, educational achievements, physical and social activity and interpersonal relationships were significantly better in SR group (p<0,01 for each) as well as in those without PFS (p<0.01 each). Qol depends on treatment intensity and PFS occurrence, although those factors are not independent. Final height of children who were older than 10 years at diagnosis was superior as compared to other survivors
CRAN-32. CASE BASED LEARNING: THREE CENTURIES OF LESSONS FROM TWO CRANIOPHARYNGEOMA PATIENTS
Authors: John R Apps, J Ciaran Hutchinson, Susan Shelmerdine, Alex Virasami, Eduard Winter, Thomas S Jacques, Juan-Pedro Martinez-Barbera, Owen Arthurs, Thomas Czech
Journal: Neuro-Oncology (2018)

Case-based learning is a core component of medical undergraduate curricula across the world; it has been used to impart knowledge of human physiology, symptomatology, pathology, and clinical therapy for millenia. We present two cases of craniopharyngeoma (CP) where separate studies at different time points between 1839 and 2017 have impacted on our understanding of the normal function of the pituitary and the hypothalamus as well as the classification of its pathology. Based on 2 specimens of CP stored in the Pathological-Anatomical Museum in Vienna, Austria, we retraced the clinical-pathological analyses of these cases starting in 1839 by Josef Engel under the auspices of Karl von Rokitansky, one of the fathers of modern pathology, reanalysed in detail by Jakob Erdheim in his 1904 paper recognizing these „hypophyseal duct tumors“ as a pathologic entity of its own. Ever since H.Cushing (improperly) renamed them craniopharyngeomas they remain one of the „most formidable intracranial tumors“ despite recent advances in understanding their molecular biology. Lastly in 2017 the two samples were reanalysed in London (UK) by advanced imaging (3T MR and high-resolution micro-CT) supplementing histological studies. The conclusions of the separate studies highlight how the interpretation of data is shaped by the technologies/methodologies and prevailing theories of the time.