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Background: There is an urgent need to better understand the mechanisms underlying acute and long-term neurological symptoms after COVID-19. Neuropathological studies can contribute to a better understanding of some of these mechanisms. Methods: We conducted a detailed postmortem neuropathological analysis of 32 patients who died due to COVID-19 during 2020 and 2021 in Austria. Results: All cases showed diffuse white matter damage with a diffuse microglial activation of a variable severity, including one case of hemorrhagic leukoencephalopathy. Some cases revealed mild inflammatory changes, including olfactory neuritis (25%), nodular brainstem encephalitis (31%), and cranial nerve neuritis (6%), which were similar to those observed in non-COVID-19 severely ill patients. One previously immunosuppressed patient developed acute herpes simplex encephalitis. Acute vascular pathologies (acute infarcts 22%, vascular thrombosis 12%, diffuse hypoxic–ischemic brain damage 40%) and pre-existing small vessel diseases (34%) were frequent findings. Moreover, silent neurodegenerative pathologies in elderly persons were common (AD neuropathologic changes 32%, age-related neuronal and glial tau pathologies 22%, Lewy bodies 9%, argyrophilic grain disease 12.5%, TDP43 pathology 6%). Conclusions: Our results support some previous neuropathological findings of apparently multifactorial and most likely indirect brain damage in the context of SARS-CoV-2 infection rather than virus-specific damage, and they are in line with the recent experimental data on SARS-CoV-2-related diffuse white matter damage, microglial activation, and cytokine release. [ABSTRACT FROM AUTHOR]

Background: Corticosteroid therapy (CST) prior to biopsy may hinder histopathological diagnosis in primary central nervous system lymphoma (PCNSL). Therefore, preoperative CST in patients with suspected PCNSL should be avoided if clinically possible. The aim of this study was thus to analyze the difference in the rate of diagnostic surgeries in PCNSL patients with and without preoperative CST.Methods: A multicenter retrospective study including all immunocompetent patients diagnosed with PCNSL between 1/2004 and 9/2018 at four neurosurgical centers in Austria was conducted and the results were compared to literature.Results: A total of 143 patients were included in this study. All patients showed visible contrast enhancement on preoperative MRI. There was no statistically significant difference in the rate of diagnostic surgeries with and without preoperative CST with 97.1% (68/70) and 97.3% (71/73), respectively (p = 1.0). Tapering and pause of CST did not influence the diagnostic rate. Including our study, there are 788 PCNSL patients described in literature with an odds ratio for inconclusive surgeries after CST of 3.3 (CI 1.7-6.4).Conclusions: Preoperative CST should be avoided as it seems to diminish the diagnostic rate of biopsy in PCNSL patients. Yet, if CST has been administered preoperatively and there is still a contrast enhancing lesion to target for biopsy, surgeons should try to keep the diagnostic delay to a minimum as the likelihood for acquiring diagnostic tissue seems sufficiently high. [ABSTRACT FROM AUTHOR]

Background: For its numerous abilities including sedation, we have been using thalidomide (TH) as the ‘last therapeutic option' in patients with advanced gliomas. We noticed that a small subgroup, i.e. patients with secondary glioblastoma (GBM, whose GBM has evolved over several months or years from a less malignant glioma), survived for prolonged periods. Therefore, we retrospectively evaluated the outcomes of patients with secondary GBM treated with TH at our centre. Patients and Methods: Starting in the year 2000, we have studied 23 patients (13 females, 10 males, with a median age of 31.5 years) with secondary GBM who have received palliative treatment with TH 100 mg at bedtime. All patients had previously undergone radiotherapy and received at least 1 and up to 5 regimens of chemotherapy. Results: The median duration of TH administration was 4.0 months (range 0.8-32). The median duration of overall survival after the start of TH therapy was 18.3 months (range 0.8-57). Eleven patients with secondary GBM survived longer than 1 year. Symptomatic improvement was most prominent in the restoration of a normal sleep pattern. Conclusion: The palliative effects of TH, especially the normalization of a sleep pattern, were highly valued by patients and families. The prolongation of survival of patients with secondary GBM has not been reported previously. © 2014 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Background: This is the first study on trends in cancer incidence, survival and mortality for children and adolescents in Austria. The aim was to assess to what extent progress against childhood and adolescent cancer has been made in Austria since the 1990s and to complement the childhood and adolescent cancer trends for Central Europe.Methods: All malignant neoplasms and non-malignant tumours of the Central Nervous System (CNS) in patients aged less than 20 years and diagnosed between 1994 and 2011 (N=5425) were derived from the Austrian National Cancer Registry (ANCR). Incidence and mortality trends were evaluated by the average annual percentage change (AAPC). Observed survival rates were calculated based on follow-up until December 31st 2013.Results: Childhood cancer remained stable with 182 cases per million in 2011, but rose among girls by 1.4% (95% CI: .1, 3.6) annually due to an increase of non-malignant CNS tumours and Non-Hodgkin lymphoma. Adolescent cancer rose by 1.5% (95% CI: .4, 2.6) annually, from 182 cases per million in 1994-269 in 2011, especially leukaemia, CNS tumours (including non-malignant types) and epithelial tumours. Five-year survival improved by 5-7% reaching 86% for both groups (p<.05). Mortality declined by -2.4% (95% CI: -3.7, -1.2) and -2.0% (95% CI: -4.6, .5), respectively, especially for childhood leukaemia.Conclusion: Progress is demonstrated by improved survival and declined mortality most likely related to improved diagnostic techniques, more effective therapeutic regimes, supportive care and a central advisory function of experts in the Austrian paediatric oncology. [ABSTRACT FROM AUTHOR]

Background: Primary CNS lymphoma is a highly aggressive and rare type of extranodal non-Hodgkin lymphoma. Although, new therapeutic approaches have led to improved survival, the management of the disease poses a challenge, practice patterns vary across institutions and countries, and remain ill-defined for vulnerable patient subgroups. Material and Methods: Using information from the Austrian Brain Tumor Registry we followed a population-based cohort of 189 patients newly diagnosed from 2005 to 2010 through various lines of treatment until death or last follow-up (12-31-2016). Prognostic factors and treatment-related data were integrated in a comprehensive survival analysis including conditional survival estimates. Results: We find variable patterns of first-line treatment with increasing use of rituximab and high-dose methotrexate (HDMTX)-based poly-chemotherapy after 2007, paralleled by an increase in median overall survival restricted to patients aged below 70 years. In the entire cohort, 5-year overall survival was 24.4% while 5-year conditional survival increased with every year postdiagnosis. Conclusion: In conclusion, we show that the use of poly-chemotherapy and immunotherapy has disseminated to community practice to a fair extent and survival has increased over time at least in younger patients. Annually increasing conditional survival rates provide clinicians with an adequate and encouraging prognostic measure.

Background: Unlike malignant primary central nervous system (CNS) tumours outcome data on non-malignant CNS tumours are scarce. For patients diagnosed from 1996 to 2002 5-year relative survival of only 85.0% has been reported. We investigated this rate in a contemporary patient cohort to update information on survival., Methods: We followed a cohort of 3983 cases within the Austrian Brain Tumour Registry. All patients were newly diagnosed from 2005 to 2010 with a histologically confirmed non-malignant CNS tumour. Vital status, cause of death, and population life tables were obtained by 31 December 2011 to calculate relative survival., Results: Overall 5-year relative survival was 96.1% (95% CI 95.1-97.1%), being significantly lower in tumours of borderline (90.2%, 87.2-92.7%) than benign behaviour (97.4%, 96.3-98.3%). Benign tumour survival ranged from 86.8 for neurofibroma to 99.7% for Schwannoma; for borderline tumours survival rates varied from 83.2 for haemangiopericytoma to 98.4% for myxopapillary ependymoma. Cause of death was directly attributed to the CNS tumour in 39.6%, followed by other cancer (20.4%) and cardiovascular disease (15.8%)., Conclusion: The overall excess mortality in patients with non-malignant CNS tumours is 5.5%, indicating a significant improvement in survival over the last decade. Still, the remaining adverse impact on survival underpins the importance of systematic registration of these tumours.

Cancer registries provide incidence and mortality data on patients with cancer at the population level. Cancer registration is most often restricted to the group of malignant neoplasms, whereas information on benign and intermediate tumors is generally not available. Brain tumors however differ from other sites by 1) the large spectrum of different tumor types, and 2) the exclusive localization in proximity to eloquent areas with considerable neurological comorbidity and mortality irrespective of their biological behavior. In order to obtain a comprehensive overview of the brain tumor burden, specialized brain tumor registries, which provide information on all brain tumor types, have emerged in several countries. This thesis synopsis summarizes the Austrian experience on the establishment of such a specialized brain tumor registry: the Austrian Brain Tumor Registry (ABTR). The initial steps are reported - from consensus and commitment of the Austrian Society of Neuropathology, formation of an interdisciplinary team of experts, setup of the infrastructure including data confidentiality issues, to the sustained support of the Austrian neurooncology community and major cooperation with the Austrian National Cancer Registry. ABTR differs from other registries by its scientific setting and neuropathological background warranting strong expertise in brain tumor typing and tissue-based research. Thereby, ABTR constitutes also a virtual brain tumor biobank. By having achieved these steps, first investigational results demonstrate that ABTR provides valid and accurate population-based incidence and survival data for individual brain tumor types. The exact incidence of rare tumor entities is estimated, key diagnostic criteria of newly proposed tumor entities are refined, common standards for testing of molecular markers are advocated, and medical progress via real-life outcome analyses is assessed. The diverse scientific contributions highlight the enormous scientific potential of ABTR for continued work.

Background: Atypical teratoid/rhabdoid tumors are highly malignant embryonal central nervous system (CNS) tumors that were defined as an entity in 1996. As compared with other malignant CNS tumors, their biological behavior is particularly aggressive, but patients may benefit from an intensified treatment. Atypical teratoid/rhabdoid tumors display a complex histomorphology, which renders them prone to misdiagnosis. They occur predominantly in young children, with an estimated prevalence of 1% to 2% among all pediatric CNS tumors. However, population-based data on the incidence of these tumors are not yet available., Methods: A nation-wide survey of malignant high-grade CNS tumors (World Health Organization grade III/IV), diagnosed in children (aged birth to 14 years) from 1996 to 2006 was conducted by the Austrian Brain Tumor Registry. A central histopathology review was performed including the assessment of SMARCB1 (INI1) protein status., Results: A total of 311 newly diagnosed, malignant CNS tumors were included. Atypical teratoid/rhabdoid tumors constituted the sixth most common entity (6.1%), referring to an age-standardized incidence rate of 1.38 per 1,000,000 person-years in children. Peak incidence was found in the birth to 2 years age group, where they were as common as CNS primitive neuroectodermal tumors and medulloblastomas. A total of 47.4% of atypical teratoid/rhabdoid tumors were initially diagnosed, whereas 52.6% were retrospectively detected by the central review. The 5-year survival of atypical teratoid/rhabdoid tumor patients was 39.5%, with 66.7% in the correctly diagnosed group versus 15.0% in the not recognized group (P = .0469)., Conclusions: Clinicians and pathologists should be aware of the high incidence of atypical teratoid/rhabdoid tumors in young children to optimize diagnostic and therapeutic management of patients with these tumors., (Copyright © 2010 American Cancer Society.)