tbuckley/cabot-search huggingface dataset:
EPID-12. PRIMARY PAEDIATRIC BRAIN TUMORS IN SINGAPORE: A 17-YEAR SOUTHEAST-ASIAN PERSPECTIVE ON EPIDEMIOLOGY AND OUTCOMES
Authors: Jiani Sherry Liu, Sharon Yin Yee Low, Wan Teow Seow, David Low, May Lian Leong, Tseng Tsai Yeo, Ning Chou, Miriam Kimpo, Vincent Nga
Journal: Neuro-Oncology (2018)

Epidemiological studies comparing primary pediatric brain tumors (PBTs) worldwide with various sub-regions in Asia have reported varying disease patterns, likely secondary to genetic susceptibilities and environmental insults. We describe, for the first time, PBT epidemiology in Southeast-Asian, a population distinct from other Asian sub-populations. A retrospective review of 480 patients, diagnosed over a 17-year period in Singapore public pediatric hospitals, was performed to determine: tumor histopathology, location, age distribution and survival rates. Medulloblastoma (19.6%) was the most common subtype, followed by pilocytic astrocytoma (13.5%) and germ cell tumors (9.8%). 42% of PBTs was treated via surgery alone, whereas 53% with surgery/biopsy+adjuvant treatment. The overall survival for all PBTs is 93% at 1-year (1YSR) and 77% at 5-years (5YSR). The 1YSR of medulloblastoma was 73% and 5YSR 71%. The median length of survival is 1-year for those who died of disease, while those who survived>3 years all went into remission, highlighting two extremes in prognosis of medulloblastoma here. Meanwhile, OS and PFS varied for medulloblastoma patients diagnosed at <3-years-old, compared to those aged≥4-years-old. Other than limited use of radiotherapy in infants, this might reflect different frequencies of genetic mutations that predispose infants in Singapore to develop more aggressive medulloblastoma subtypes, given that this age-group more frequently had recurrences/metastasis. This is despite their symptomatic interval till diagnosis being similar to the group that went into remission. There is hence, a need to investigate and elucidate high-risk genetic loci in this population to identify novel molecular targets in biologic therapy, especially for infants.
NURS-18. DEVELOPMENT OF A MULTIDISCIPLINARY CARE SERVICE FOR ADOLESCENT AND YOUNG ADULT ONCOLOGY PATIENTS AT AN URBAN PEDIATRIC ACADEMIC CENTER
Authors: Kasey Rangan, Laura Bava, Chelsea Bailan, Sherry Carcich, Sarah Green, Mary Beth Vingelen, Jacqueline O’Connell, Stefanie Thomas
Journal: Neuro-Oncology (2018)

The adolescent /young adult (AYA) population has distinct medical and developmental needs requiring specialized care different than those of children and adults. Expert recommendations identify fertility preservation and behavioral health services as two areas of critical need. However, research indicates a lack of systematic and consistent delivery of services. Therefore, developing a comprehensive understanding of AYA care needs, particularly AYAs from minority and underserved backgrounds, remains important. Our primary goal was to develop an AYA service to improve clinical practices informed by current recommendations. Our secondary goal was to identify the unmet needs for this population at our institution. We focused on programmatic considerations for service development, implementation, and research in culturally and economically diverse population. Our multidisciplinary service consists of a pediatric oncologist with AYA training, a pediatric psychologist, and embedded nurse practitioners/nurse care managers AYA champions. We systematically identified approximately 100 newly diagnosed and relapsed AYAs (age 13-21 years) over the past year and evaluated and improved delivery of AYA fertility preservation and behavioral health screening. We created a patient registry to capture clinical, demographic, and service provision and utilization information. Data collected will be used to improve our understanding of population needs, barriers to care, including fertility preservation, and service development expected to lead to improved health outcomes. Analysis of the registry data will assist with developing educational material and support for patients, families, and providers.
IMMU-15. HIGH-RESOLUTION ANALYSIS OF THE T-CELL RECEPTOR REPERTOIRE AFTER ADOPTIVE CELLULAR THERAPY IN PEDIATRIC PATIENTS WITH CENTRAL PNETs (Re-MATCH TRIAL)
Authors: Oleg Yegorov, Yanina Yegorova, Anjelika Dechkovskaia, Sridharan Gururangan, Duane Mitchell
Journal: Neuro-Oncology (2018)

Adoptive cellular therapy (ACT) using transfer of tumor-specific lymphocytes has emerged as a potent strategy for treatment of advanced and refractory malignancies. We have employed deep TCR repertoire sequencing approach to explore dynamics of T-cell immunity in the nine patients with recurrent medulloblastoma and PNETs undergoing adoptive cellular therapy (Re-MATCH protocol, FDA IND BB-14058). Total RNA was isolated from patient PBMC samples collected prior to adoptive cellular therapy and weekly for one month and then monthly following immunotherapy treatment. cDNA was generated with addition of a common adapter at 5’ end of cDNA using RACE technology. Raw sequencing data were preprocessed using MiXCR software. Further TCR repertoire analysis was performed using tcR R-package and VDJtools software. We observed remarkable dynamic changes in the TCR repertoire in peripheral blood following ACT in all patients. The blood samples collected after adoptive cellular therapy revealed that certain T-cells were clonally expanded after ACT, with an increasing number of “hyper-expanded” TCR clones (comprising >1% of all TCR beta or alpha sequences). Hyper-expanded TCR clones and increased TCR diversity following ACT was associated with radiographic response to ACT and prolonged progression-free and overall survival. Limited TCR expansion and diversity following ACT was observed in patients with short overall and progression-free survival. These findings support further study of the use of TCR sequencing to monitor responses to adoptive cellular therapy and suggest that TCR clonal expansion and increasing TCR diversity following treatment may be associated with positive clinical responses.
High-Sensitivity Troponin and Heart Failure With Preserved Ejection Fraction—Balancing <i>P</i> Values With Prior Knowledge and Common Sense—Reply
Authors: Rudolf A. de Boer, Matthew Nayor, Jennifer E. Ho
Journal: JAMA Cardiology (2018)

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NFM-10. TARGETING NuRD AND KAT7 FOR EPIGENOME MODIFICATION IN NEUROFIBROMATOSIS1 ASSOCIATED MALIGNANT PERIPHERAL NERVE SHEATH TUMORS
Authors: Chandra Mayanil, Mariam Khan, Vineet Mohanty, Raj Kumar, Guifa Xi, Shunsuke Ichi, Bal Ram Singh, Barbara Mania-Farnell, Tadanori Tomita
Journal: Neuro-Oncology (2018)

Neurofibromatosis type 1 (NF1) is common, occurring in 1/3000 live births, and results in skin pigmentation and the growth of tumors along nerves in the skin, brain, and other parts of the body. Loss of polycomb repressive complex 2 (PRC2) has been implicated in the tumorigenesis of Schwann cells (SCs) in malignant peripheral nerve sheath tumors (MPNSTs). In the absence of PRC2, the H3K27me repressive marks are absent and thus the SCs can re-enter the cell cycle and form tumors. In the absence of PRC2, nucleosome remodeling deacetylase (NuRD) chromatin silencing complex, by itself cannot takes over its function. To compensate for the loss of PRC2 complex, there must be a decrease in activating histone marks, H3K27Ac and H3K4me3, by a reciprocal increase in HDAC2 expression and the decrease in H3K4 methylation. Using a systems biology approach as a part of the combination therapy, we have identified a novel naturally occurring multi-target cocktail in the methanolic extracts from the hydrophilic fraction of Ocimum Sanctum leaves (OSHP-1) as potential treatment option for MPNSTs. Here we show that OSHP-1 (i) increased the expression of HDAC2 (a component of NuRD), and decreased the expression of lysine acetyl transferase 7 (KAT7); (ii) altered FRα signaling and disrupted the DNA replication complex comprising of FRα-NuRD-PCNA-CAF-1; (iii) reversed the expression of certain actively transcribed genes PAX2, FOXN4, IGF2 and TLX1 in NF1-MPNST cells; (iv) increased the expression of a Schwann cell differentiation marker NCAM-140 KD. Thus, NuRD and KAT7 in NF1-MPNST can be potential therapeutic targets.
MBCL-31. A WHOLE CHROMOSOME ABERRATION PHENOTYPE IN NON-WNT/NON-SHH TUMORS PREDICTS OUTCOME WITHIN STANDARD-RISK MEDULLOBLASTOMAS FROM THE HIT-SIOP-PNET4 CLINICAL TRIAL
Authors: Tobias Goschzik, Edward C Schwalbe, Debbie Hicks, Dominique Figarella-Branger, Francois Doz, Stefan Rutkowski, Göran Gustafsson, Birgitta Lannering, Torsten Pietsch, Steven C Clifford
Journal: Neuro-Oncology (2018)

Most children with medulloblastoma are clinically ‘standard-risk’ (SR-MB; 50-60% of patients; 75-85% 5-year PFS), defined by absence of favorable (WNT subgroup) or high-risk (e.g. metastatic disease, large-cell/anaplastic histology, MYC amplification) features. The identification of novel prognostic biomarkers within SR-MB is essential to improve risk-adapted therapies, increase survival and reduce treatment-related late-effects. We comprehensively analyzed the pan-European HIT-SIOP-PNET4 clinical trial (2001-2006; 4-21 years at diagnosis), encompassing central clinical/radiological review, and systematic analysis of molecular pathological features (methylation subgroup, copy number aberration and mutational status) in 136 patients. Independent prognostic markers/models for non-WNT/non-SHH patients (n=91) were identified by multivariate analyses, and validated in a representative independent cohort (n=70). SR-MB subgroup distribution differed from disease-wide estimates; WNT (n=28; 21%) and Group 4 (n=76; 56%) were enriched. Favorable-risk WNT disease was confirmed in patients <16.0 years. All SHH events (4/17) occurred in TP53mut and/or chr17p loss tumors. In non-WNT/non-SHH, a novel whole chromosomal aberration phenotype characterized by chr7 gain, chr8 and/or chr11 loss, predicted an excellent prognosis in both the HIT-SIOP-PNET4 and validation cohort (100/98% 5-year PFS). This cytogenetic phenotype was highly associated with novel methylation subgroups (MBGrp4-LowRisk; Group 3/4 sub-subgroups VI, VII). Its incorporation into survival models out-performed current risk-stratification schemes (AUC 0.765 vs 0.562). Data from HIT-SIOP-PNET4 provide a blueprint for the redistribution of clinically-defined SR-MB into biomarker-defined favorable-risk (incorporating WNT <16years, SHH/TP53wt, and the clinical application of novel non-WNT/non-SHH subgroups; 51% of patients, 100% 5-year PFS) and high-risk groups (49%; 63% PFS), and a basis for future biomarker-driven risk-adapted clinical trials.
A Mismatch Repair–Deficient Hepatoid Adenocarcinoma of the Lung Responding to Anti–PD-L1 Durvalumab Therapy Despite no PD-L1 Expression
Authors: Victor Basse, Ulrike Schick, Paul Guéguen, Cédric Le Maréchal, Isabelle Quintin-Roué, Renaud Descourt, Hélène Simon, Arnaud Uguen, Gilles Quéré
Journal: Journal of Thoracic Oncology (2018)
CRAN-33. ENDOSCOPIC CHALLENGE FOR SELLAR AND PARASELLAR TUMORS: FROM ABOVE OR BELOW
Authors: Mohamed M Arnaout, Diego Mazzatenta, Khaled Aziz
Journal: Neuro-Oncology (2018)

Sellar and parasellar tumors are generally remain a neurosurgical challenge due their complexity and the lack of a standardized approach. Several approaches to the sellar and parasellar regions are popular among neurosurgeons; of which supraorbital keyhole and the transsphenoidal approaches. PATIENTS AND We prospectively studied 54 patients with sellar and parasellar tumors who were undergoing 28 from above by endoscope-assisted supraorbital keyhole approach in one hospital and 26 from below by the endoscopic endonasal approach in another hospital by another surgical team. All patients data were collected and analyzed. The histopathology in this study was limited to meningioma and craniopharyngioma. Although the gross total tumor removal was slightly higher in the supraorbital approach in both groups of meningioma and craniopharyngioma (77.2% Versus 72.7%) and (83.3% Versus 75.0%) respectively, near total was slightly much more in the endonasal group than supraorbital (22.7% Versus 18.1%) and (25.0% Versus 22.7%) for meningioma and craniopharyngioma respectively. Although There was no significant difference regard vision improvement between endonasal and supraorbital in both craniopharyngioma (75.0% Versus 83.3%) and meningioma (27.2% Versus 50.0%). However, percentage of improvement was higher in supraorbital. Although these approaches typically yield an excellent cosmetic result and rapid patient recovery relative to traditional larger craniotomies, they are technically demanding requiring low-profile instrumentation. In conclusion; we believe that after learning and practicing both and other approaches then, Adapt your knowledge to your patient not the patient for your knowledge.
LGG-55. MALIGNANT TRANSFORMATION, SECOND MALIGNANT NEOPLASMS, AND SURVIVAL IN 50 YEARS OF FOLLOW-UP OF CHILDHOOD LOW-GRADE GLIOMA: A SINGLE-INSTITUTE EXPERIENCE
Authors: Sebastiaan Somers, Cees Lucas, Eleonora Aronica, Antoinette Schouten-van Meeteren
Journal: Neuro-Oncology (2018)

Pediatric low-grade glioma (LGG) contain a heterogeneous group of central nervous system tumors treated with different strategies. This study investigates incidence and risk factor for malignant transformation (MT) and second malignant neoplasms (SMN) as well as survival rates in LGG. A retrospective cohort study regarding LGG diagnosed below 19 years old from our university hospital between 1950-2011 analysed MT and SMN in the Dutch registry of histo&cytopathology. Clinical characteristics, treatment and follow-up were analysed with OS, EFS and PFS as secondary outcome. Logistic regression analysis and the Cox proportional hazards model were used to identify risk factors for MT, SMN, OS, EFS and PFS. During follow-up of 232 children with pediatric LGG 10 patients experienced MT of tumor and 9 patients developed a SMN. Children with a DNET, oligodendroglioma and fibrillary astrocytoma were at risk for MT and children with NF-1 were at risk for SMN. Death occurred in 71/232 patients with 10-year OS 72,4 ± 3,2 % and PFS % for all subgroups. Factors associated with OS were: tumor histology and grading, tumor location and radiotherapy. MT and SMN were not associated with overall survival. Children with LGG have a good long-term survival, but age at diagnosis, tumor location and type of therapy influence survival, thus MT and the occurrence of SMN become more relevant. Tumor histology was a predictor for MT and NF-1 status was a predictor for SMN. Current follow-up of 10 years is sufficient to detect the majority of progressions and MTs.
Prevalence of ALK gene alterations among the spectrum of plexiform spitzoid lesions
Authors: Deborah Saraggi, Roberto Salmaso, Carolina Zamuner, Giada Munari, Cristiano Lanza, Mauro Salvatore Alaibac, Franco Bassetto, Massimo Rugge, Maria Cristina Montesco, Lorenzo Cerroni, Matteo Fassan
Journal: Journal of the American Academy of Dermatology (2018)
Impact of revision surgery timing on overall survival in incidentally detected gall bladder cancer: An experience from tertiary care centre of Northern India
Authors: A. Mishra, M. Gowda, S. Bhoriwal, S. Deo, N. Shukla, A. Sharma, S. Pathy, S. Kumar
Journal: Annals of Oncology (2018)

Introduction: Gall bladder cancer is very common in South East Asia. It is an aggressive malignancy. Currently half of the patients of GBC are being detected as incidental. Most of these incidentally detected gall bladder cancer found to be early staged. As per current literature, there is no consensus on ideal time of revision or completion surgery if required. The timing of surgery may play crucial role in outcome of these early staged gall bladder cancer. Methods: Retrospective analysis of prospectively maintained computerized data-base of GBC patients was carried out at Institute Rotary Cancer Hospital, All India Institute of Medical Sciences, New Delhi, India. We analyzed the patient's details pertaining to clinical presentation, preoperative therapy, operative procedure, histopathological examination, postoperative complications, adjuvant treatment, and outcomes. Results: Total of 135 GBC patients underwent gall bladder surgery in between January 2010 to December 2016. Out of these 134 patients, 74 (54.81%) were incidentally detected gall bladder cancer. 48.7% incidentally detected GBC patients found to be Stage I (AJCC 8th) while 43.24%, 8% restaged as Stage II and III respectively. During intra-operative assessment, 52 (70.27%) patients were found operable and underwent Completion Radical cholecystectomy. In rest 22 (29.73%) patients the procedure was abandoned in view of metastatic or inoperable disease. For analysis, these 52 patients were divided into two groups. Group A patients (n = 37) underwent Completion radical cholecystectomy within 4 months of primary surgery while Group B patients (n = 15) underwent Completion radical cholecystectomy after 4 months. In Group A, 46.1% & 40.8% patients were in TNM stage I and stage II respectively compare to 37.5 & 44.6%, each in stage I & II respectively in Group B. DFS in Group A & Group B patient were 28.7 months and 19.4 months respectively (p <.05). Conclusion: The outcome of completion radical cholecystectomy is inversely proportional to the interval between primary and definitive surgery even in similarly staged patients.
GERM-23. INTRACRANIAL GROWING TERATOMA SYNDROME (IGTS): AN INTERNATIONAL RETROSPECTIVE STUDY
Authors: George Michaiel, Douglas Strother, Nicholas Gottardo, Ute Bartels, Hallie Coltin, David D Eisenstat, Juliette Hukin, Donna L Johnston, Beverly Wilson, Shayna Zelcer, Jordan R Hansford, Olivia Wells, Mohamed S AbdelBaki, Mohammad H Abu-Arja, Kristina A Cole, Girish Dhall, Paul G Fisher, Lindsey Hoffman, Sarah E S Leary, Emily E Owens Pickle, Natasha P Smiley, Amy Smith, Anna Vinitsky, Nicholas A Vitanza, Avery Wright, Kee K Yeo, Lionel M L Chow, Maria Kirby, Santosh Valvi, Magimairajan I Vanan, Grace Wong, David Ziegler, Eric Bouffet, Lucie Lafay-Cousin
Journal: Neuro-Oncology (2018)

IGTS is a rare phenomenon of paradoxical germ cell tumor (GCT) growth during or following treatment despite normalization of tumor markers. We sought to evaluate the frequency, clinical characteristics and outcome of IGTS in patients in 21 North-American and Australian institutions. Patients with IGTS diagnosed from 2000-2017 were retrospectively evaluated. Out of 739 GCT diagnoses, IGTS was identified in 33 patients (4.5%). IGTS occurred in 9/191 (4.7%) mixed-malignant GCTs, 4/22 (18.2%) immature teratomas (ITs), 3/472 (0.6%) germinomas/germinomas with mature teratoma, and in 17 secreting non-biopsied tumours. Median age at GCT diagnosis was 10.9 years (range 1.8-19.4). Male gender (84%) and pineal location (88%) predominated. Of 27 patients with elevated markers, median serum AFP and ßHCG were 70 ng/mL (range 9.2-932) and 44 IU/L (range 4.2-493), respectively. IGTS occurred at a median time of 2 months (range 0.5-32) from diagnosis, during chemotherapy in 85%, radiation in 3%, and after treatment completion in 12%. Surgical resection was attempted in all, leading to gross total resection in 76%. Most patients (79%) resumed GCT chemotherapy/radiation after surgery. At a median follow-up of 5.3 years (range 0.3-12), all but 2 patients are alive (1 succumbed to progressive disease, 1 to malignant transformation of GCT). IGTS occurred in less than 5% of patients with GCT and most commonly after initiation of chemotherapy. IGTS was more common in patients with IT-only on biopsy than with mixed-malignant GCT. Surgical resection is a principal treatment modality. Survival outcomes for patients who developed IGTS are favourable.
CRAN-16. IMPORTANCE OF SURGICAL INTERVENTION IN RECOVERY OF VISUAL FUNCTION IN A TEENAGER WITH AN ACIDOPHILIC STEM CELL ADENOMA
Authors: Courtney Johnson, John Jane, Priya Vaidyanathan, Young Kwok, Steven Stasheff, Roger Packer, Eugene Hwang
Journal: Neuro-Oncology (2018)

mutation and recapitulation of her germline APC frameshift variant.Whole transcriptome analysis of tumor RNA revealed overexpression of multiple Wnt
Influence of vitamin D (Vit D) on mammographic density (MD) and insulin like growth factor 1 (IGF1): Results of CALGB (Alliance) 70806.
Authors: Marie Wood, Drew K. Seisler, Meng-Kang Hsieh, Despina Kontos, Abiy Berhie Ambaye, Huong T. Le-Petross, Sin-Ho Jung, Heshan Liu, Patricia J. Zekan, Lucien Cardinal, Jayne Charlamb, Lili X. Wang, Gary Walter Unzeitig, Judy Ellen Garber, James Roger Marshall
Journal: Journal of Clinical Oncology (2018)

1549 Background: Vitamin D is safe and has breast cancer prevention properties. CALGB 70806 was a randomized phase II trial evaluating the effect of vit D on several breast cancer biomarkers (including MD and serum IGF1). Methods: Premenopausal women were assigned to receive either 2000IU of Vit D or placebo for 12 months, stratified by baseline (BL) vit D level (sufficient vs insufficient). Eligible women were premenopausal, age < 55, with at least 25% dense breast tissue. Biomarker specimens were collected at baseline and 12 months. MD was determined using the Breast Imaging Reporting and Data System (BIRADS), semi-automated and automated methods. Serum IGF1 was determined by ELISA. Biomarkers were compared between arms using Wilcoxon and t-tests. Results: 300 women were recruited from 41 institutions across the US between 1/11 -12/13. The mean age was 42.6 years with 14% Hispanic, 12% African American, 74% European. 62% of participants were vitamin D deficient at enrollment and 49% of women had MD between 25-50% with only 12% over 50% dense. 216 (72%) of participants completed treatment, 8 withdrew due to side effects, and 76 for other reasons (28% withdrawal rate). A significant increase in Vit D was seen with treatment with 99% and 72% of experimental and control subjects having sufficient levels at 12 months(P < 0.0001)). MD decreased 2.2% over 1 year for the entire cohort, with no significant difference between arms (Table 1). Similarly, no significant change in IGF1 levels was seen with Vit D. Conclusions: Vit D supplementation resulted in a significant increase in serum Vit D (from a mean of 35.5 to 49.7 ng/mL, p = < 0.0001). However, no significant change in MD was observed with treatment; potentially due to small change in MD seen at 1 year, the low percentage of high MD or that Vit D works by another mechanism. Further study with longer Vit D exposure is warranted. Support: UG1CA189823, U24CA196171. Clinical trial information: NCT01224678.Biomarker changes over 12 months. Biomarker Placebo Vit D P-value 25(OH)D (ng/mL) -0.9 (12.5) +14.3 (12.9) < 0.0001 IGF1 (ng/mL) -0.1 (0.6) -0.1 (0.6) 0.3699 MD -2.4% (7.9%) -1.9% (9.8%) 0.7048