Adelheid Wöhrer

Washington University in St. Louis, San Luis, Missouri, United States

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Publications (84)306.33 Total impact

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    ABSTRACT: The Brain Tumor Epidemiology Consortium (BTEC) is an international consortium that has the mission of fostering the development of multi-center and inter-disciplinary collaborations aiming to improve understanding of the etiology, outcomes, and prevention of brain tumors. Mayo Clinic faculty, Robert Jenkins, MD, PhD and Brian Patrick O'Neill, MD, hosted the 16th annual BTEC meeting on June 2 - 4, 2015, in Rochester, MN, USA. The meeting included presentations that emphasized the impact of new tumor classifications, methodological practices of population studies, as well as intra- and inter-tumoral molecular complexities on patient outcomes. The 2016 meeting will be held in Barcelona, Spain in June.
    No preview · Article · Nov 2015 · Clinical neuropathology
  • Adelheid Woehrer · Johannes A Hainfellner
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    ABSTRACT: Several morphology- and polymerase chain reaction (PCR)-based methods for chromosome 1p 19q deletion status assessment are available. Important prerequisites for all molecular techniques concern tissue quality and selection of regions of interest. The most common methods for diagnostic 1p 19q assessment are fluorescence in situ hybridization and PCR-based microsatellite analysis. While the latter requires the use of autologous blood samples, more advanced techniques such as array comparative genomic hybridization, multiplex ligation-dependent probe amplification or real-time PCR are independent from autologous DNA samples. However, due to high technical demand and experience required their applicability as diagnostic tests remains to be shown. On the other hand, chromogenic in situ hybridization evolves as attractive alternative to FISH. Herein, the available test methods are reviewed and outlined, their advantages and drawbacks being discussed in detail.
    No preview · Article · Nov 2015
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    Preview · Article · Nov 2015 · Neuro-Oncology
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    ABSTRACT: O6-methylguanine-methyltransferase (MGMT) promoter methylation status has prognostic and, in the subpopulation of elderly patients, predictive value in newly diagnosed glioblastoma. Therefore, knowledge of the MGMT promoter methylation status is important for clinical decision-making. So far, MGMT testing has been limited by the lack of a robust test with sufficiently high analytical performance. Recently, one of several available pyrosequencing protocols has been shown to be an accurate and robust method for MGMT testing in an intra- and interlaboratory ring trial. However, some uncertainties remain with regard to methodological issues, cut-off definitions, and optimal use in the clinical setting. In this article, we highlight and discuss several of these open questions. The main unresolved issues are the definition of the most relevant CpG sites to analyze for clinical purposes and the determination of a cut-off value for dichotomization of quantitative MGMT pyrosequencing results into "MGMT methylated" and "MGMT unmethylated" patient subgroups as a basis for further treatment decisions.
    No preview · Article · Sep 2015 · Clinical neuropathology
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    ABSTRACT: OBJECT An important prognostic factor for the surgical outcome and recurrence of a pituitary adenoma is its invasiveness into parasellar tissue, particularly into the space of the cavernous sinus (CS). The aims of this study were to reevaluate the existing parasellar classifications using an endoscopic technique and to evaluate the clinical and radiological outcomes associated with each grade. METHODS The authors investigated 137 pituitary macroadenomas classified radiologically at least on one side as Grade 1 or higher (parasellar extension) and correlated the surgical findings using an endoscopic technique, with special reference to the invasiveness of the tumor into the CS. In each case, postoperative MRI was performed to evaluate the gross-total resection (GTR) rate and the rate of endocrinological remission (ER) in functioning adenomas. RESULTS The authors found a 16% rate of CS invasion during surgery for these macroadenomas. Adenomas radiologically classified as Grade 1 were found to be invasive in 1.5%, and the GTR/ER rate was 83%/88%. For Grade 2 adenomas, the rate of invasion was 9.9%, and the GTR/ER rate was 71%/60%. For Grade 3 adenomas, the rate of invasion was 37.9%, and the GTR/ER rate was 75%/33%. When the superior compartment of the CS (Grade 3A) was involved, the authors found a rate of invasion that was lower (p < 0.001) than that when the inferior compartment was involved (Grade 3B). The rate of invasion in Grade 3A adenomas was 26.5% with a GTR/ER rate of 85%/67%, whereas for Grade 3B adenomas, the rate of surgically observed invasion was 70.6% with a GTR/ER rate of 64%/0%. All of the Grade 4 adenomas were invasive, and the GTR/ER rate was 0%. A comparison of microscopic and endoscopic techniques revealed no difference in adenomas with Grade 1 or 4 parasellar extension. In Grade 2 adenomas, however, the CS was found by the endoscopic technique to be invaded in 9.9% and by microscopic evaluation to be invaded in 88% (p < 0.001); in Grade 3 adenomas, the difference was 37.9% versus 86%, respectively (p = 0.002). Grade 4 adenomas had a statistically significant lower rate of GTR than those of all the other grades. In case of ER only, Grade 1 adenomas had a statistically significant higher rate of remission than did Grade 3B and Grade 4 adenomas. CONCLUSIONS The proposed classification proved that with increasing grades, the likelihood of surgically observed invasion rises and the chance of GTR and ER decreases. The direct endoscopic view confirmed the low rate of invasion of Grade 1 adenomas but showed significantly lower rates of invasion in Grade 2 and 3 adenomas than those previously found using the microscopic technique. In cases in which the intracavernous internal carotid artery was encased (Grade 4), all the adenomas were invasive and the GTR/ER rate was 0%/0%. The authors suggest the addition of Grades 3A and 3B to distinguish the strikingly different outcomes of adenomas invading the superior CS compartments and those invading the inferior CS compartments.
    No preview · Article · Feb 2015 · Journal of Neurosurgery
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    ABSTRACT: OBJECT Surgery of suspected low-grade gliomas (LGGs) poses a special challenge for neurosurgeons due to their diffusely infiltrative growth and histopathological heterogeneity. Consequently, neuronavigation with multimodality imaging data, such as structural and metabolic data, fiber tracking, and 3D brain visualization, has been proposed to optimize surgery. However, currently no standardized protocol has been established for multimodality imaging data in modern glioma surgery. The aim of this study was therefore to define a specific protocol for multimodality imaging and navigation for suspected LGG. METHODS Fifty-one patients who underwent surgery for a diffusely infiltrating glioma with nonsignificant contrast enhancement on MRI and available multimodality imaging data were included. In the first 40 patients with glioma, the authors retrospectively reviewed the imaging data, including structural MRI (contrast-enhanced T1-weighted, T2-weighted, and FLAIR sequences), metabolic images derived from PET, or MR spectroscopy chemical shift imaging, fiber tracking, and 3D brain surface/vessel visualization, to define standardized image settings and specific indications for each imaging modality. The feasibility and surgical relevance of this new protocol was subsequently prospectively investigated during surgery with the assistance of an advanced electromagnetic navigation system in the remaining 11 patients. Furthermore, specific surgical outcome parameters, including the extent of resection, histological analysis of the metabolic hotspot, presence of a new postoperative neurological deficit, and intraoperative accuracy of 3D brain visualization models, were assessed in each of these patients. RESULTS After reviewing these first 40 cases of glioma, the authors defined a specific protocol with standardized image settings and specific indications that allows for optimal and simultaneous visualization of structural and metabolic data, fiber tracking, and 3D brain visualization. This new protocol was feasible and was estimated to be surgically relevant during navigation-guided surgery in all 11 patients. According to the authors' predefined surgical outcome parameters, they observed a complete resection in all resectable gliomas (n = 5) by using contour visualization with T2-weighted or FLAIR images. Additionally, tumor tissue derived from the metabolic hotspot showed the presence of malignant tissue in all WHO Grade III or IV gliomas (n = 5). Moreover, no permanent postoperative neurological deficits occurred in any of these patients, and fiber tracking and/or intraoperative monitoring were applied during surgery in the vast majority of cases (n = 10). Furthermore, the authors found a significant intraoperative topographical correlation of 3D brain surface and vessel models with gyral anatomy and superficial vessels. Finally, real-time navigation with multimodality imaging data using the advanced electromagnetic navigation system was found to be useful for precise guidance to surgical targets, such as the tumor margin or the metabolic hotspot. CONCLUSIONS In this study, the authors defined a specific protocol for multimodality imaging data in suspected LGGs, and they propose the application of this new protocol for advanced navigation-guided procedures optimally in conjunction with continuous electromagnetic instrument tracking to optimize glioma surgery.
    Preview · Article · Jan 2015 · Neurosurgical FOCUS
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    Adelheid Woehrer · Gabor G Kovacs

    Full-text · Article · Jan 2015 · Clinical neuropathology
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    ABSTRACT: The Brain Tumor Epidemiology Consortium (BTEC) is an open scientific forum, which fosters the development of multi-center, international and inter-disciplinary collaborations. BTEC aims to develop a better understanding of the etiology, outcomes, and prevention of brain tumors (http://epi.grants.cancer.gov/btec/). The 15th annual Brain Tumor Epidemiology Consortium Meeting, hosted by the Austrian Societies of Neuropathology and Neuro-oncology, was held on September 9 - 11, 2014 in Vienna, Austria. The meeting focused on the central role of brain tumor epidemiology within multidisciplinary neuro-oncology. Knowledge of disease incidence, outcomes, as well as risk factors is fundamental to all fields involved in research and treatment of patients with brain tumors; thus, epidemiology constitutes an important link between disciplines, indeed the very hub. This was reflected by the scientific program, which included various sessions linking brain tumor epidemiology with clinical neuro-oncology, tissue-based research, and cancer registration. Renowned experts from Europe and the United States contributed their personal perspectives stimulating further group discussions. Several concrete action plans evolved for the group to move forward until next year's meeting, which will be held at the Mayo Clinic at Rochester, MN, USA.
    Full-text · Article · Jan 2015 · Clinical neuropathology
  • Adelheid Woehrer · Luc Bauchet · Jill S Barnholtz-Sloan
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    ABSTRACT: Purpose of review: Glioblastoma is the most common malignant brain tumor in adults and carries a particularly poor prognosis. Since 2005, state-of-the-art therapy consists of maximal well tolerated surgical resection followed by combined radiotherapy and chemotherapy with temozolomide. Over the past decade, further advances have been achieved in various disciplines, most prominently including antiangiogenic treatment with bevacizumab. Still, whether these therapeutic innovations have translated to the general population remains unclear. Recent findings: Population-based outcome and pattern of care (POC) studies have recently documented the rapid dissemination of the treatment standard to community practice across countries. This has resulted in a modest but significant increase in survival at the population level. However, the increase was significantly less marked in elderly patients in whom undertreatment is a concern. Other serious concerns address diverging POC between academic versus nonacademic centers, patients with high-income versus low-income, and racial and marital status disparities. With regard to bevacizumab treatment, there is still insufficient evidence of a beneficial impact on population-based survival, so far. Summary: Despite the rapid incorporation of the current standard treatment in clinical practice and the thereby achieved modest survival gain at the population-level, prevailing POC needs to be reconsidered and standardized, especially for elderly glioblastoma patients who bear a large disease burden and carry the worst prognosis. Future POC studies are urgently needed and would benefit from the systematic inclusion of quality-of-life data and molecular tumor markers, so that this information could be captured in population-based cancer registries.
    No preview · Article · Dec 2014 · Current Opinion in Neurology
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    ABSTRACT: Primary central nervous system lymphoma (PCNSL) is a rare and malignant tumour type. Established treatment approaches include high-dose methotrexate (HD-MTX) -based chemotherapy and whole-brain radiotherapy (WBRT). WBRT is associated with significant neurotoxicity and autologous haematopoietic stem cell transplantation (ASCT) has been proposed as an alternative treatment – either in the 1st line setting after HD-MTX based chemotherapy or as salvage treatment for relapsed/refractory PCNSL. We here report our single centre experience with five PCNSL patients, who had achieved an objective response after a high-dose methotrexate based induction therapy and consecutively received a high-dose chemotherapy, consisting of carmustine and thiotepa, followed by ASCT. We also provide a literature review on ASCL for PCNSL. Our data, with three out of five patients in continuous complete remission and four out of five patients alive after a median follow-up time of 8 months, as well as previously published results, show that ASCT is a safe treatment option that is able to induce tumour remissions in patients with PCNSL. However, controlled trials are needed to compare the long-term efficacy and tolerability of ASCT with other treatment approaches and also to establish the optimal sequence of treatment regimens in PCNSL patients.This article is protected by copyright. All rights reserved.
    No preview · Article · Dec 2014 · European Journal Of Haematology
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    ABSTRACT: 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.
    Full-text · Article · Nov 2014 · Oncology
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    ABSTRACT: BACKGROUND: The outcome of patients with malignant high-grade glioma (HGG) has improved significantly during the last decade. In most patients the radiation field includes parts of the hypothalamus and/or the pituitary gland, which may cause a complex pattern of neuroendocrine dysfunction. The objective to this cross-sectional study was to evaluate the prevalence of secondary and tertiary hypothyroidism in patients following standard therapy of HGG. METHODS: Serum concentrations of pituitary and peripheral thyroid and sexual hormones were measured in 258 subjects (122 female, 136 male) with HGG. All patients had been treated with standard radio-chemotherapy (radiation up to 60 Gy, followed by adjuvant chemotherapy with temozolomide) up to ten years before testing. RESULTS: TSH was reduced in 22% and increased in 18% of all cases (n = 258). Circulating fT4 and fT3 levels were lowered in 5% and 20%, but increased in 10% and 3%, respectively. With regards to the sexual hormones, we observed an interesting hormonal profile in men (n = 123). Pituitary hormones LH, FSH and prolactin were increased in 64%, 62% and 47% of all men. However, circulating testosterone levels were decreased in 40% of men ≤ 50 years and 16% of men > 50 years. In contrast, LH and FSH concentrations were below the lower reference limit in 75% of women > 50 years, but only in 6% of women ≤ 50 years. Serum progesterone and estrogen levels were lowered in 39% and 36% of women ≤ 50 years and in 43% and 0% of women > 50 years. Of note, 78% of women ≤ 50 years and 78% of women > 50 years featured increased circulating testosterone levels. CONCLUSIONS: Standard treatment for HGG results in multiple changes of the pituitary-endocrine axis. Expecially the finding on increased testosterone concentrations in women is striking and warrants further investigation.
    No preview · Article · Nov 2014 · Neuro-Oncology
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    ABSTRACT: Background: Immune checkpoint inhibitors targeting programmed cell death 1 (PD1) or its ligand (PD-L1) showed activity in several cancer types. Methods: We performed immunohistochemistry for CD3, CD8, CD20, HLA-DR, phosphatase and tensin homolog (PTEN), PD-1, and PD-L1 and pyrosequencing for assessment of the O6-methylguanine-methyltransferase (MGMT) promoter methylation status in 135 glioblastoma specimens (117 initial resection, 18 first local recurrence). PD-L1 gene expression was analyzed in 446 cases from The Cancer Genome Atlas. Results: Diffuse/fibrillary PD-L1 expression of variable extent, with or without interspersed epithelioid tumor cells with membranous PD-L1 expression, was observed in 103 of 117 (88.0%) newly diagnosed and 13 of 18 (72.2%) recurrent glioblastoma specimens. Sparse-to-moderate density of tumor-infiltrating lymphocytes (TILs) was found in 85 of 117 (72.6%) specimens (CD3+ 78/117, 66.7%; CD8+ 52/117, 44.4%; CD20+ 27/117, 23.1%; PD1+ 34/117, 29.1%). PD1+ TIL density correlated positively with CD3+ (P < .001), CD8+ (P < .001), CD20+ TIL density (P < .001), and PTEN expression (P = .035). Enrichment of specimens with low PD-L1 gene expression levels was observed in the proneural and G-CIMP glioblastoma subtypes and in specimens with high PD-L1 gene expression in the mesenchymal subtype (P = 5.966e-10). No significant differences in PD-L1 expression or TIL density between initial and recurrent glioblastoma specimens or correlation of PD-L1 expression or TIL density with patient age or outcome were evident. Conclusion: TILs and PD-L1 expression are detectable in the majority of glioblastoma samples but are not related to outcome. Because the target is present, a clinical study with specific immune checkpoint inhibitors seems to be warranted in glioblastoma.
    Full-text · Article · Oct 2014 · Neuro-Oncology
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    ABSTRACT: Metastases to the central nervous system (CNS) are common in several cancer types. For most primary tumors that commonly metastasize to the CNS, molecular biomarker analyses are recommended in the clinical setting for selection of appropriate targeted therapies. Therapeutic efficacy of some of these agents has been documented in patients with brain metastases, and molecular testing of CNS metastases should be considered in the clinical setting. Here, we summarize the clinically relevant biomarker tests that should be considered in neurosurgical specimens based on the current recommendations of the European Society of Medical Oncology (ESMO) or the National Comprehensive Cancer Network (NCCN) for the most relevant primary tumor types: lung cancer (EGFR mutations, ALK rearrangement, BRAF mutations), breast cancer (HER2 amplification, steroid receptor overexpression), melanoma (BRAF mutations), and colorectal cancer (RAS mutations). Furthermore, we discuss emerging therapeutic targets including novel oncogenic alterations (ROS1 rearrangements, FGFR1 amplifications, CMET amplifications, and others) and molecular features of the tumor microenvironment (including immune-checkpoint molecules such as CTLA4 and PD-1/PD-L1). We also discuss the potential role of advanced biomarker tests such as next-generation sequencing and "liquid biopsies" for patients with CNS metastases.
    No preview · Article · Oct 2014 · Acta Neuropathologica
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    ABSTRACT: Objective To identify the prevalence of MRI features of Binswanger's disease (BD), specifically MRI with diffuse white matter lesions and scattered multiple lacunes (BD-MRI), and to describe neurological features and pathological outcomes of a community-based cohort study.Methods Of 697 participants (all 75 years old), 503 completed neurological examinations at baseline and were followed-up every 30 months thereafter with MRIs, the mini-mental state examination (MMSE) and the Unified Parkinson Disease Rating Scale-Motor Section (UPDRSM). Data from participants with BD-MRI were compared with those from participants with predominant white matter lesions (WML-MRI), scattered multiple lacunes (ML-MRI), or normal MRIs.ResultsFourteen BD-MRI patients (2.8%) were detected at baseline. The mean MMSE scores in the BD-MRI, WML-MRI, ML-MRI, and normal MRIs groups were 26.4, 28.2, 28.4, and 28.5, respectively, and the mean UPDRSM scores were 9.1, 1.3, 3.1, and 1.7, respectively. At the 30-month follow-up, mortality rates in the normal MRIs, WML-MRI and ML-MRI were 4%, 9.1%, and 22.2%, respectively, and follow-up MRIs were available for 80%, 82%, and 61% of the participants, respectively. In the BD-MRI, however, five patients were deceased, and only five follow-up individual MRIs were available (33.3%). Autopsies were performed on six of eight BD-MRI brains, and these brains fulfilled the pathological criteria for BD independent of Alzheimer disease pathology. All these six individuals also showed systemic atherosclerosis and renal arterio-arteriolosclerosis.InterpretationThe BD-MRI participants had poor prognoses and showed pure BD pathology with advanced systemic vascular disease. BD-MRI appears to be a predictor of vascular neurocognitive impairment.
    Full-text · Article · Oct 2014
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    ABSTRACT: The rapid increase in mobile phone use in young people has generated concern about possible health effects of exposure to radiofrequency (RF) and extremely low frequency (ELF) electromagnetic fields (EMF). MOBI-Kids, a multinational case-control study, investigates the potential effects of childhood and adolescent exposure to EMF from mobile communications technologies on brain tumor risk in 14 countries. The study, which aims to include approximately 1,000 brain tumor cases aged 10-24 years and two individually matched controls for each case, follows a common protocol and builds upon the methodological experience of the INTERPHONE study. The design and conduct of a study on EMF exposure and brain tumor risk in young people in a large number of countries is complex and poses methodological challenges. This manuscript discusses the design of MOBI-Kids and describes the challenges and approaches chosen to address them, including: (1) the choice of controls operated for suspected appendicitis, to reduce potential selection bias related to low response rates among population controls; (2) investigating a young study population spanning a relatively wide age range; (3) conducting a large, multinational epidemiological study, while adhering to increasingly stricter ethics requirements; (4) investigating a rare and potentially fatal disease; and (5) assessing exposure to EMF from communication technologies. Our experience in thus far developing and implementing the study protocol indicates that MOBI-Kids is feasible and will generate results that will contribute to the understanding of potential brain tumor risks associated with use of mobile phones and other wireless communications technologies among young people.
    Full-text · Article · Sep 2014 · Frontiers in Public Health
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    ABSTRACT: BACKGROUND: Glioblastoma constitutes the most common primary malignant brain tumor in adults. Its aggressive disease course is mediated by a hitherto poorly understood interplay of rapid tumor progression and therapy-related effects. Crucial factors include - amongst hypoxia, angiogenesis, and others - an immune response upfront and/or as a consequence to combined chemo-radiotherapy. Herein, we aim at a systematic morphological characterization of this immune response based on a homogenous series of paired pre- and posttherapeutic glioblastoma samples. PATIENTS AND METHODS: A total of 15 glioblastoma patients newly diagnosed from 2005-2010 at the Medical University of Vienna were included in the analysis. All patients underwent neursurgical resection followed by standard radio- and TMZ-chemotherapy. At time of tumor recurrence second surgery was performed in all cases. Information on clinical characteristics and last follow-up were available. The tumor-associated immune response was semiquantitatively assessed using immunohistochemistry for anti-HLA-DR, CD3, and CD8. In addition, tumor-associated eosinophilia was assessed on conventional HE stains. RESULTS: Median age at diagnosis was 54 ys (range 21-69 ys). Median time from first to second surgery was 12.0 mths (range 1-26 mths) and median overall survival was 46.0 mths of the total cohort. While tumor-infiltrating CD3+ and CD8+ T-lymphocytes did not show a significant difference between first and second surgery (p = 0.07 and p = 0.3, respectively), a significant increase of HLA-DR+ microglia/macrophages (p = 0.01) was observed at time of tumor recurrence in the majority of cases (10/15; 66.7%). No unequivocal differences in overall survival were observed for those patients with enhanced microglial/macrophage response at time of tumor recurrence (log rank test; p = 0.34). A single case showed massive infiltration of eosinophils at second surgery only. This case differed from others by young age at diagnosis (26 ys), positive IDH-1 mutational and MGMT methylation status, short time to second surgery (1 mth) and exceptional favorable overall survival with stable disease at 84 mths. No peripheral eosinophilia or prior history of atopic disease was noted. Further screening for anti-neuronal/glial antibodies on a tissue-based assay did not yield a positive result. CONCLUSION: Although the small sample size implies cautious interpretation, we observed a significant increase of tumor-infiltrating microglia/macrophages in glioblastoma samples obtained after first-line therapy, while no significant differences were observed for tumor-infiltrating CD3+ and CD8+ T-lymphocytes. Of note, a single patient showed prominent tumor-associated eosinophilia upon second surgery which was associated with long-term survival.
    Preview · Article · Sep 2014 · Neuro-Oncology
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    ABSTRACT: Low grade gliomas (LGG) comprise a spectrum of different tumor entities corresponding to WHO grade I and II, including pilocytic astrocytoma, diffuse glioma and glioneuronal tumors. LGG in children differ in histologic distribution, molecular profile, localisation in the CNS and outcome from LGGs in adults. We report on a series of patients treated at a single center over 20 years. PATIENTS: Between 1993 and 2012, 339 consecutive patients with a median age of 8 years (range 4 months - 18 years, p25 = 3.7 years, p75 = 13.4 years) were treated at the Medical University of Vienna. 19.5% of the patients were <3 years of age at diagnosis. Gender distribution was equal. Tumors were associated with syndromes in 84 of the patients (NF1: n = 64, TSC: n = 17, and other: n = 3). Localisation was supratentorial midline in 112, cerebral hemispheres in 105, posterior fossa in 86, ventricular system in 27 and spinal in 9 patients. 66 patients with a median age of 4.4 years had optic pathway gliomas. RESULTS: Gross total resection was performed in 109, subtotal resection in 49, partial resection in 57, and biopsy in 22 patients. 83 patients had no surgery (patients with NF1, TSC, tectal glioma or tumors confined to the optic nerves and chiasm), and in the remaining the degree of resection was not evaluable. 37 patients had 2 tumor surgeries, 14 three, 5 four and one had 5 tumor surgeries during their course of disease. Histology was pilocytic astrocytoma in 122, pilomyxoid astrocytoma in 10, diffuse astrocytoma in 42, ganglioglioma in 30, subependymal giant cell astrocytoma in 17, oligoastrocytoma in 11, and other rarer histologies in the remaining patients. Twelve patients (3.5%) died and 84 patients (24.8%) had at least one event. After a median follow-up of 107 months (14-300) the 1-year overall survival (OS) was 100%, the 10-year OS 96.1 ± 1.2%, and the 20-year OS 95.3 ± 1.4%. Event-free survival (EFS) after 1 year was 92.9 ± 1.4%, after 10 years 73.8 ± 2.6% and after 20 years 67.9 ± 3.4%. CONCLUSION: While long term survival of children and adolescents with low grade gliomas is better than of adults, recurrence and death may occur even in WHO grade I tumors and regular surveillance MRI is recommended.
    Preview · Article · Sep 2014 · Neuro-Oncology

  • No preview · Article · Sep 2014 · European geriatric medicine
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    ABSTRACT: Immunoglobulin G4- related disease (IgG4-RD) is a rare systemic fibro-inflammatory disorder (ORPHA284264). Although patients have been described more than 100 years ago, the systemic nature of this disease has been recognized in the 21st century only. Type 1 autoimmune pancreatitis is the most frequent manifestation of IgG4-RD. However, IgG4-RD can affect any organ such as salivary glands, orbits, retroperitoneum and many others. Recent research enabled a clear clinical and histopathological description of IgG4-RD. Typically, lymphoplasmacellular inflammation, storiform fibrosis and obliterative phlebitis are found in IgG4-RD biopsies and the tissue invading plasma cells largely produce IgG4. Elevated serum IgG4 levels are found in many but not all patients. Consequently, diagnostic criteria for IgG4-RD have been proposed recently. Treatment is largely based on clinical experience and retrospective case series. Glucocorticoids are the mainstay of therapy, although adjunctive immunosuppressive agents are used in relapsing patients. This review summarizes current knowledge on clinical manifestations, pathophysiology and treatment of IgG4-RD.
    Preview · Article · Jul 2014 · Orphanet Journal of Rare Diseases

Publication Stats

1k Citations
306.33 Total Impact Points

Institutions

  • 2015
    • Washington University in St. Louis
      • Division of Hematology and oncology
      San Luis, Missouri, United States
  • 2008-2015
    • Medical University of Vienna
      • Universitätsklinik für Neurochirurgie
      Wien, Vienna, Austria
  • 2014
    • French Institute of Health and Medical Research
      Lutetia Parisorum, Île-de-France, France
  • 2013-2014
    • IST Austria
      Klosterneuberg, Lower Austria, Austria