scholarly journals DIPG-17. BIOPSY-PROVEN DIFFUSE MIDLINE GLIOMA IN ADOLESCENTS AND YOUNG ADULTS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii290-iii290
Author(s):  
Shota Tanaka ◽  
Shunsaku Takayanagi ◽  
Yuki Shinya ◽  
Mariko Kawashima ◽  
Masahiro Shin ◽  
...  
Keyword(s):  
Young Adults ◽  
Idh1 Mutation ◽  
Adolescents And Young Adults ◽  
Molecular Profile ◽  
Diffuse Astrocytoma ◽  
Genetic Analyses ◽  
K27m Mutation ◽  
Histological Confirmation ◽  
Diffuse Midline Glioma ◽  
H3k27m Mutation

Abstract INTRODUCTION Diffuse midline glioma (DMG) mostly affects young children. The newly-introduced disease entity DMG, H3K27M-mutant uniformly portends poor prognosis, and therefore that in the pons is usually treated based upon radiological diagnosis without histological confirmation. DMG is rarer in adolescents and young adults (AYA), and remains poorly characterized. In this study, we sought to investigate the clinical, pathological, and molecular profiles of DMG in AYA generation. METHODS Patients of age between 16 and 39 undergoing biopsy at the University of Tokyo Hospital between 2003 and 2019 were included in the study. Clinical data and images were retrospectively reviewed. Genetic analyses were performed in cases with abundant tissues. RESULTS Ten patients included 8 brainstem and 2 thalamic DMG. The median age was 25 years (range, 19–38). Pathological diagnosis was DMG, H3K27M-mutant in 3 patients, glioblastoma, IDH-mutant in 1, anaplastic astrocytoma, IDH-wildtype in 4, diffuse astrocytoma, IDH-mutant in 1, and diffuse astrocytoma, IDH-wildtype in 1. Genetic analyses detected H3F3A-K27M mutation in 2, HIST1H3B-K27M mutation in 1, IDH1-R132H mutation in 1, and IDH1-R132S mutation in 1. With a median follow-up of 23 months (range, 2–61), only 3 patients died 29–61 months after diagnosis, and the remaining 7 patients survived for 2–59 months. Neither IDH1 mutation nor H3K27M mutation was associated with survival in this series. CONCLUSION Survival of AYA patients with DMG was seemingly variable with some long survivors. H3K27M mutation was present in a subset of patients. A further study is warranted to correlate molecular profile with clinical pictures including patient survival.

P06.01 Pediatric non-pontine diffuse midline glioma H3K27M mutant: a monoinstitutional experience

2021 ◽  
Vol 23 (Supplement_2) ◽  
pp. ii24-ii24
Author(s):  
E Schiavello ◽  
V Biassoni ◽  
L De Cecco ◽  
E Pecori ◽  
S Filippo ◽  
...  
Keyword(s):  
Liquid Biopsy ◽  
P53 Mutation ◽  
New Drugs ◽  
Molecular Profile ◽  
Histopathological Diagnosis ◽  
Diffuse Astrocytoma ◽  
Number Of Patients ◽  
Pathological Evaluation ◽  
Diffuse Midline Glioma ◽  
Astrocytoma Grade

Abstract BACKGROUND The new entity “diffuse-midline-glioma H3K27M-mutant”(DMG) was defined in the 2016 WHO classification. This tumor subtype, regardless of histological features, is associated with an aggressive clinical behavior and poor prognosis, sharing the same outcome as DIPG. MATERIAL AND METHODS We report our experience from 2016 to 2018 in treating patients with radiotherapy and concomitant Nimotuzumab/Vinorelbine, as already published for DIPG’s patients (DOI10.1007/s11060-014-1428-z). All patients’ parents provided written informed consent for treatment. RESULTS Patients were 9: 7/9 females, median age at diagnosis 13 years (range 1–26); 8 with localized disease, 1 with spinal fluid (CSF) dissemination. Five patients had thalamic disease (1 bithalamic), 2 ponto-cerebellar (one with 2 lesions), 1 pineal gland and one with disseminated CSF disease at diagnosis. 3/9 were biopsed in thalamus, the others had partial resection. Central neuropathological review with confirmed histopathological diagnosis of DMG mutated was necessary for inclusion. At the first pathological evaluation tumors had been classified according to the fourth edition of the WHO 2007 as glioblastoma (n=4), anaplastic astrocytoma grade III (n=1), diffuse astrocytoma grade II (n=1), and 1 glial tumor with oligodendroglial features; 2 patients already had DMG at the first pathological evaluation. Immunohistochemical analysis revealed p53 mutation in 3 patients. All 9 patients were evaluated on serial plasma samples with NGS targeted panel (Pan-Cancer) and 6/9 presented p53 mutation in at least one of the samples. 8/9 expressed MAP2K1 gain of function at liquid biopsy. The small number of patients did not allow correlations with the prognosis. Two patients completed the scheduled 2 years of treatment: one had a local relapse at 37 months after diagnosis, the other is alive without evidence of progression at 30 months. With a median follow up of 14.4 months, PFS and OS were 33% and 77% at 1 year; OS was 22% at 2 years, with 2 patients long survivors at 36 and 40 months after diagnosis. CONCLUSION The molecular and phenotypic pattern of DMG allows to include patients in clinical trials/registry shared with patients suffering from DIPG. Our knowledge is still limited about this entity; new insights into molecular profile should help us to study new drugs directed against the effects of the H3K27M mutations and to define new diagnostic/prognostic approach as liquid biopsy able to correlate treatments and prognosis

scholarly journals MPC-14 BRAF V600E MUTANT OLIGODENDROGLIOMA-LIKE TUMORS WITH CHROMOSOMAL INSTABILITY IN ADOLESCENTS AND YOUNG ADULTS

2019 ◽  
Vol 1 (Supplement_2) ◽  
pp. ii24-ii24
Author(s):  
Kohei Fukuoka ◽  
Uri Tabori ◽  
Cynthia Hawkins
Keyword(s):  
Young Adults ◽  
Idh1 Mutation ◽  
Adolescents And Young Adults ◽  
Braf V600e ◽  
Low Grade ◽  
Wild Type ◽  
Low Grade Gliomas ◽  
History Of ◽  
Mitotic Figures ◽  
Temporal Lobe Lesions

Abstract We performed genome-wide methylation analysis on 136 pediatric low grade gliomas, identifying a unique cluster consisting of oligodendroglioma-like BRAF V600E mutant tumors with Recurrent gain of Chromosome 7 and loss of Chromosome 10 (OLIVER). Hierarchical clustering and t-stochastic neighbor embedding analyses cluster them with previously described pediatric-type low grade gliomas, separate from adult gliomas. OLIVERS exhibit distinct clinical behavior as temporal lobe lesions in adolescents and young adults, prolonged history of seizures and all are alive with no recurrence (follow-up 3.2 to 13.2 years). Morphogically, all showed oligodendroglioma-like features, including round nuclei with perinuclear halos, a chicken-wire pattern of branching capillaries and microcalcification. None showed astrocytic features or characteristics suggestive of high-grade tumors including necrosis or mitotic figures. All tumors harbored multiple chromosomal copy number abnormalities (more than 10 chromosomes per OLIVER), but none showed 1p/19q co-deletion or IDH1 mutation. Interestingly, one tumor showed a TERT promoter mutation. Although the series is small, OLIVER may represent a new category of IDH wild-type low grade gliomas which may be confused with molecular GBM. Further, they highlight the heterogeneity of IDH wild-type gliomas and the relatively indolent behavior of pediatric-type gliomas.

The Relationship Between Narcissism and Personality Traits of the Five-Factor-Model in Adolescents and Young Adults

2018 ◽  
Vol 46 (6) ◽  
pp. 516-522 ◽  
Author(s):  
Marc Allroggen ◽  
Peter Rehmann ◽  
Eva Schürch ◽  
Carolyn C. Morf ◽  
Michael Kölch
Keyword(s):  
Young Adults ◽  
Personality Traits ◽  
Five Factor Model ◽  
Self Report ◽  
Adolescents And Young Adults ◽  
Personality Factors ◽  
Big Five Personality ◽  
Grandiose Narcissism ◽  
Vulnerable Narcissism ◽  
The Relationship

Abstract.Narcissism is seen as a multidimensional construct that consists of two manifestations: grandiose and vulnerable narcissism. In order to define these two manifestations, their relationship to personality factors has increasingly become of interest. However, so far no studies have considered the relationship between different phenotypes of narcissism and personality factors in adolescents. Method: In a cross-sectional study, we examine a group of adolescents (n = 98; average age 16.77 years; 23.5 % female) with regard to the relationship between Big Five personality factors and pathological narcissism using self-report instruments. This group is compared to a group of young adults (n = 38; average age 19.69 years; 25.6 % female). Results: Grandiose narcissism is primarily related to low Agreeableness and Extraversion, vulnerable narcissism to Neuroticism. We do not find differences between adolescents and young adults concerning the relationship between grandiose and vulnerable narcissism and personality traits. Discussion: Vulnerable and grandiose narcissism can be well differentiated in adolescents, and the pattern does not show substantial differences compared to young adults.

Epidemiological Trends in Attempted Suicide in Adolescents and Young Adults Between 1996 and 2004

Crisis ◽  
2009 ◽  
Vol 30 (3) ◽  
pp. 115-119 ◽  
Author(s):  
Stephanie De Munck ◽  
Gwendolyn Portzky ◽  
Kees Van Heeringen
Keyword(s):  
Young Adults ◽  
Attempted Suicide ◽  
Suicide Attempts ◽  
Epidemiological Studies ◽  
Adolescents And Young Adults ◽  
University Hospital ◽  
International Studies ◽  
Suicide Attempters ◽  
Increased Risk ◽  
Epidemiological Trends

Background: Notwithstanding the epidemiological studies indicating an increased risk of attempted suicide among adolescents and young adults, there is a scarcity of international studies that examine long-term epidemiological trends in rates and characteristics of this vulnerable group. Aims: This article describes the results of a 9-year monitoring study of suicide attempts in adolescents and young adults referred to the Accident and Emergency Department of the Gent University Hospital (Belgium). Methods: Between January 1996 and December 2004, trends, sociodemographic, and methodrelated characteristics of suicide attempts were assessed by a psychiatrist on data sheets. Results: Attempted suicide rates declined from 1996 to 2001 and then rose until 2004, but did not exceed previous rates. During the 9 years of monitoring, there was a preponderance of female suicide attempters, except for 1997. Rates of attempts and of fatal suicide were negatively correlated. Significantly more males than females deliberately injured themselves. Younger attempters, especially females, significantly more often poisoned themselves with analgesics. In nearly one in five attempts, alcohol was used in combination with other methods, and alcohol intake was more commonly observed in older suicide attempters. Nearly half of the adolescents were identified as repeaters. Conclusions: The results of this study warrant further monitoring of trends and characteristics of young suicide attempters.

Comparison of Three Questionnaires to Screen for Borderline Personality Disorder in Adolescents and Young Adults

2017 ◽  
Vol 33 (2) ◽  
pp. 123-128 ◽  
Author(s):  
Anne van Alebeek ◽  
Paul T. van der Heijden ◽  
Christel Hessels ◽  
Melissa S.Y. Thong ◽  
Marcel van Aken
Keyword(s):  
Young Adults ◽  
Borderline Personality Disorder ◽  
Personality Disorder ◽  
Borderline Personality ◽  
Adolescents And Young Adults ◽  
Patient Questionnaire ◽  
Statistical Manual ◽  
Axis Ii ◽  
Diagnostic And Statistical Manual ◽  
Dsm Iv

Abstract. One of the most common personality disorders among adolescents and young adults is the Borderline Personality Disorder (BPD). The objective of current study was to assess three questionnaires that can reliably screen for BPD in adolescents and young adults (N = 53): the McLean Screening Instrument for BPD (MSI-BPD; Zanarini et al., 2003 ), the Personality Diagnostic Questionnaire 4th edition – BPD scale (PDQ-4 BPD; Hyler, 1994 ), and the SCID-II Patient Questionnaire – BPD scale (SCID-II-PQ BPD). The nine criteria of BPD according to the Diagnostic and Statistical Manual of Mental Disorders-IV (DSM-IV; APA, 1994 ) were measured with the Structural Clinical Interview for DSM-IV Axis II disorders – BPD scale (SCID-II; First, Spitzer, Gibbon, Williams, & Benjamin, 1995 ). Correlations between the questionnaires and the SCID-II were calculated. In addition, the sensitivity and specificity of the questionnaires were tested. All instruments predicted the BPD diagnosis equally well.

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