Gunther Nussbaumer
A5088232005- Glioma Diagnosis and Treatment
- Neuroblastoma Research and Treatments
- Chromatin Remodeling and Cancer
- Vascular Malformations Diagnosis and Treatment
- Ubiquitin and proteasome pathways
- Pharmacological Effects of Medicinal Plants
- Cancer-related molecular mechanisms research
- Epigenetics and DNA Methylation
- Cancer-related Molecular Pathways
- DNA Repair Mechanisms
- RNA modifications and cancer
- Cancer Genomics and Diagnostics
- Protein Degradation and Inhibitors
- Radiomics and Machine Learning in Medical Imaging
- Adolescent and Pediatric Healthcare
- Ferroptosis and cancer prognosis
- Virus-based gene therapy research
- Testicular diseases and treatments
- Hedgehog Signaling Pathway Studies
- Tuberous Sclerosis Complex Research
- Meningioma and schwannoma management
- ATP Synthase and ATPases Research
- Genetic factors in colorectal cancer
- Pancreatic and Hepatic Oncology Research
- Cancer survivorship and care
Medical University of Graz
2020-2025
Abstract Diffuse intrinsic pontine gliomas (DIPG) are the most aggressive brain tumors in children with 5-year survival rates of only 2%. About 85% all DIPG characterized by a lysine-to-methionine substitution histone 3, which leads to global H3K27 hypomethylation accompanied hyperacetylation. Hyperacetylation favors action Bromodomain and Extra-Terminal (BET) protein BRD4, reprogramming enhancer landscape contributing activation super enhancer-driven oncogenes. The activity...
Abstract Pediatric high-grade glioma (pedHGG) can occur as first manifestation of cancer predisposition syndromes resulting from pathogenic germline variants in the DNA mismatch repair (MMR) genes MSH2 , MSH6 MLH1 and PMS2 . The aim this study was to establish a generalized screening for Lynch syndrome constitutional MMR deficiency (CMMRD) pedHGG patients, detection deficiencies (MMRD) may enable upfront therapeutic use checkpoint inhibitors identification variant carriers patients’...
Abstract Background The term gliomatosis cerebri (GC), a radiology-defined highly infiltrating diffuse glioma, has been abandoned since molecular GC-associated features could not be established. Methods We conducted multinational retrospective study of 104 children and adolescents with GC providing comprehensive clinical (epi-)genetic characterization. Results Median overall survival (OS) was 15.5 months (interquartile range, 10.9–27.7) 2-year rate 28%. Histopathological grading correlated...
As there is no standard of care treatment for recurrent/progressing pediatric high-grade gliomas (pHGG), we aimed to gain an overview different strategies.In a web-based questionnaire, members the SIOPE-BTG and GPOH were surveyed on therapeutic options in four case scenarios (children/adolescents with HGG).139 clinicians experience neuro-oncology from 22 European countries participated survey. Most respondents preferred further oncological three out cases chose palliative one marked...
Abstract Background This study aims at clarifying the impact of persistent residual lesions following first-line treatment for pediatric medulloblastoma. Methods Data on 84 patients with medulloblastoma and centrally reviewed magnetic resonance imaging (MRI) end therapy were analyzed. Results Twenty (23.8%) had in tumor bed (R+/M0), 51 (60.7%) distant (R0/M+) 13 (15.5%) both (R+/M+). Overall response to was minor or partial (≥ 25% reduction, [MR]/PR) 64 (76.2%) stable disease (SD) 20...
The WHO Classification of Tumors the Central Nervous System has undergone major restructuring. Molecularly defined diagnostic criteria were introduced in 2016 (revised 4th edition) and expanded 2021 (5th to incorporate further essential molecular parameters. We investigated potential differences between specialists perception these molecularly subtypes for pediatric high-grade gliomas (pedHGG).We designed a 22-question survey studying impact revised edition classification on pedHGG. Data...
Pediatric high-grade gliomas (pedHGG) comprise a very poor prognosis. Thus, parents of affected children are increasingly resorting to complementary and alternative medicine (CAM), among those Boswellia extracts. However, nothing is known about the therapeutic effectiveness their active substances, Boswellic acids (BA) in pedHGG. we aimed investigate if three main present plants, alpha-boswellic acid (α-BA), beta-boswellic (β-BA) 3-acetyl-11-keto-beta-boswellic (AKBA) hold any promising...
PurposeTo explore the perception of physical exercise programs for pediatric oncology patients among childhood cancer care professionals. We also aimed at comparing such perceptions between cultures. Healthcare professionals' endorsement may be essential initiating and promoting programs.MethodsAn anonymous survey was designed administered voluntarily to professionals (including oncologists, nurses, physiotherapists) in European, North-African Arab centers.ResultsFive-hundred-and...
Abstract BACKGROUND Pediatric central nervous system (CNS) tumors can occur as first manifestations of cancer predisposition syndromes (CPS) resulting from pathogenic variants in DNA mismatch repair (MMR) genes MLH1, MSH2, MSH6, and PMS2. Early detection MMR deficiencies (MMRD) may warrant the therapeutic use checkpoint inhibitors enable genetic consulting for patient’s families. METHODS We prospectively screened 128 pediatric high-grade gliomas (pedHGG) protein expression by...
Abstract BACKGROUND Choroid plexus carcinoma (CPC) is a rare pediatric brain tumor occuring predominantly in infancy. It commonly associated with the cancer predisposition Li-Fraumeni syndrome (LFS). Management of CPC patients LFS challenging and there no accepted standard treatment. This multinational retrospective analysis aimed to describe clinical characteristics outcome LFS-associated CPC. METHODS We identified 32 (male, n=24; median age at diagnosis 2.1 years [range, 0.3-13.2]) (CNS...
Abstract BACKGROUND Diffuse intrinsic pontine glioma (DIPG) is a childhood tumor with median survival of < 1 year. Survival-associated factors for this rare disease are poorly understood. METHODS Clinical, radiological, and histo-molecular data were abstracted from databases the IDIPGR SIOPE-DIPGR analyzed using descriptive statistics. Survival was estimated by Kaplan Meier method. Short term survivors (STS) defined as overall (OS) 2 years, long (LTS) had OS >2 years. We...
Abstract BACKGROUND Infant-type hemispheric gliomas (IHG) are epigenetically distinct pediatric high-grade characterized by fusions in receptor tyrosine kinase (RTK) genes. METHODS We performed a methodical literature search, including 30 publications (22 case reports), to identify patients who met the diagnostic criteria of IHG based on 2021 WHO Classification CNS Tumors. Individual patient data were obtained from published and/or via authors publications. Survival analysis was conducted...
Abstract BACKGROUND We aimed to assess frequency, patterns and potential prognostic impact of initial early dissemination in pediatric patients with diffuse intrinsic pontine glioma (DIPG) midline glioma, H3K27-altered (DMG). METHODS 199 (age, 0-18 years) DIPG/DMG positive CSF cytology and/or metastases on MRI at diagnosis (Types 1-3) were identified within the SIOPE International Registries. Data metastatic first progression (PD) (Type 4) analyzed from HIT-HGG-2007/2013 trials. RESULTS...
Abstract BACKGROUND Diffuse pediatric-type high-grade gliomas (pedHGG), H3-wildtype and IDH-wildtype, encompass three main methylome-based subclasses: pedHGG-MYCN, -RTK1A/B/C, -RTK2A/B. Since their first description in 2017, tumors of pedHGG-RTK2A/B have not been further characterized clinical significance is unknown. METHODS A yet published cases series on pedHGG with a gliomatosis cerebri (GC) growth pattern showed an increased incidence (n=18/40). We assembled cohort 14 additional...
Abstract Background Our aim is to investigate the association of treatment with survival in patients diffuse intrinsic pontine glioma (DIPG) by examining 6 historical paths. Methods We retrospectively analyzed data from 409 radiologically centrally reviewed DIPG, sourced German Society Pediatric Oncology and Hematology HIT-HGG trial database SIOPE-DIPG/DMG Registry. Survival outcomes were estimated using Kaplan–Meier method, univariable multivariable Cox proportional hazard models study...
Diffuse pediatric-type high-grade gliomas (pedHGG), H3- and IDH-wildtype, encompass three main DNA-methylation-based subtypes: pedHGG-MYCN, pedHGG-RTK1A/B/C, pedHGG-RTK2A/B. Since their first description in 2017 tumors of pedHGG-RTK2A/B have not been comprehensively characterized clinical correlates remain elusive. In a recent series pedHGG with Gliomatosis cerebri (GC) growth pattern, an increased incidence (n = 18) was observed. We added 14 epigenetically defined to this GC provided...
Abstract The aim of the HIT-HGG-2007 trial (ISRCTN19852453) was to demonstrate therapeutic non-inferiority temozolomide radiochemotherapy for pediatric patients (3-18 years) with high-grade gliomas (pedHGG) in comparison cisplatinum-based two previous clinical trials HIT-GBM-C/-D. Between 06/2009 and 12/2016, 456 were enrolled at 79 centers Germany, Austria, Switzerland (n=18 dropouts, remaining confirmatory analysis: n=438). 438 from HIT-GBM-C/-D served as historical control. All pedHGG...
Abstract Gliomatosis cerebri (GC), a radiologically defined highly infiltrating supratentorial glioma, is no longer considered distinct entity since the 2016 WHO classification for tumors of central nervous system (CNS). So far, neither prognostic factors, nor molecular GC-associated features have been established. We conducted multinational retrospective study 104 children and adolescents with GC providing comprehensive radiological, clinical (epi-)genetic characterization. Within median...
Abstract PURPOSE: Prognosis of pediatric high-grade gliomas (pedHGG) is dismal, and there no standard care treatment in case recurrence/progression. We aimed to gain an overview different strategies the setting recurrent/progressing pedHGG. METHODS: In a web-based questionnaire, members SIOPE-BTG GPOH were surveyed on therapeutic options four real-world scenarios (children/adolescents with HGG). RESULTS: One hundred thirty-nine clinicians experience neuro-oncology from 22 European countries...
Abstract BACKGROUND: Gliomatosis cerebri (GC), a radiologically defined diffusely infiltrating glioma, is no longer considered distinct entity since the 2016 WHO classification for tumors of CNS. Due to its rarity and dismal prognosis treatment recommendations in children remain ambiguous. Using central neuroradiological review, we performed multi-institutional, retrospective study GC providing comprehensive radiological, clinical, (epi)genetic characterization. RESULTS: We included 104...
Abstract BACKGROUND: As there is no standard of care treatment for recurrent/progressing paediatric high-grade gliomas (pHGG), we aimed to gain an overview different strategies. METHODS: In a web-based questionnaire, members the SIOPE-BTG and GPOH were surveyed on therapeutic options in four case scenarios (children/adolescents with HGG). RESULTS: Most respondents (139 clinicians from 22 European countries) preferred further oncological three out cases chose palliative one marked symptoms....