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E Matyja, W Grajkowska, K Stępień, E Naganska
Pilocytic astrocytomas (PAs) are the most frequent primary astroglial tumours affecting children and adolescents. They occur sporadically or in association with a genetically determined syndrome - neurofibromatosis type 1. Classic PA usually manifests as a well-circumscribed, often cystic, slowly growing tumour, which corresponds to WHO grade I. The majority of pilocytic tumours arise along the neuraxis, predominantly in the cerebellum. They are associated with favourable long-term outcome or spontaneous regression, even after incomplete resection...
2016: Folia Neuropathologica
Aruna Nambirajan, Mehar C Sharma, Madhu Rajeshwari, Aanchal Kakkar, Vaishali Suri, Chitra Sarkar
Ependymomas are gliomas that recapitulate normal ependymal cells. The epithelial membrane antigen (EMA) shows "dot-like" and "ring-like" staining patterns, highlighting "microlumens" or intracytoplasmic rosettes, a pathognomonic ultrastructural feature. NHERF1/EBP50, an adaptor protein localized at the apical plasma membrane of human epithelia, has been found to localize to these microlumens. We aimed to analyze the staining patterns of EMA and EBP50 in ependymomas and other tumors, and thereby compare their diagnostic utility...
October 7, 2016: Applied Immunohistochemistry & Molecular Morphology: AIMM
Shilpa Rao, Palavalasa Sravya, Chitra Chandran, Jitender Saini, Sampath Somanna, Vani Santosh
Oligodendrogliomas are diffuse gliomas characterised by IDH mutation and 1p/19q co-deletion. Classical oligodendrocytes, minigemistocytes, gliofibrillary oligodendrocytes, granular cells, and mucocytes are morphologic cell types described in oligodendroglioma. Even though the occurrence of granular cells in oligodendroglioma is known, exact nature of these cells and their molecular characteristics remain undetermined. We describe a case of oligodendroglioma with granular cells, in which we have attempted to molecularly characterise the granular cells...
October 13, 2016: Brain Tumor Pathology
Tomokazu Aoki, Yoshiki Arakawa, Tetsuya Ueba, Masashi Oda, Namiko Nishida, Yukinori Akiyama, Tetsuya Tsukahara, Koichi Iwasaki, Nobuhiro Mikuni, Susumu Miyamoto
The objective of this phase I/II study was to examine the efficacy and toxicity profile of temozolomide (TMZ) plus nimustine (ACNU). Patients who had received a standard radiotherapy with one or two previous chemo-regimens were enrolled. In phase I, the maximum-tolerated dose (MTD) by TMZ (150 mg/m(2)/day) (Day 1-5) plus various doses of ACNU (30, 35, 40, 45 mg/m(2)/day) (Day 15) per 4 weeks was defined on a standard 3 + 3 design. In phase II, these therapeutic activity and safety of this regimen were evaluated...
October 11, 2016: Neurologia Medico-chirurgica
Veronica Villani, Roberta Merola, Antonello Vidiri, Alessandra Fabi, Mariantonia Carosi, Diana Giannarelli, Laura Marucci, Marta Maschio, Carmine M Carapella, Andrea Pace
PURPOSE: To explore the efficacy and toxicity of an extended schedule of temozolomide (50 mg/mq 1 week on/1 week off) in a population of newly diagnosed low-grade gliomas (LGG). METHODS: Primary endpoints were progression-free survival (PFS) at 12 and 24 months and response rate evaluated with Response Assessment in Neuro-Oncology Criteria. Secondary endpoints were clinical benefit (reduction of seizures frequency), reduction of steroid, and modifications of Karnofsky Performance Status...
September 22, 2016: Tumori
Andrew L Lin, Michael White, Michelle M Miller-Thomas, Robert S Fulton, Christina I Tsien, Keith M Rich, Robert E Schmidt, David D Tran, Sonika Dahiya
During the 6 month period following chemoradiotherapy, gliomas frequently develop new areas of contrast enhancement, which are due to treatment effect rather than tumor progression. We sought to characterize this phenomenon in oligodendrogliomas (OG) and mixed oligoastrocytomas (MOA). We reviewed the imaging findings from 143 patients with a WHO grade II or III OG or MOA for evidence of pseudoprogression (PsP) or early tumor progression. We characterized these cases for 1p/19q codeletions by FISH, IDH1 R132H mutation by immunohistochemistry, and TP53, ATRX, and EGFR mutations by next generation sequencing...
October 4, 2016: Journal of Neuro-oncology
Brigitta G Baumert, Monika E Hegi, Martin J van den Bent, Andreas von Deimling, Thierry Gorlia, Khê Hoang-Xuan, Alba A Brandes, Guy Kantor, Martin J B Taphoorn, Mohamed Ben Hassel, Christian Hartmann, Gail Ryan, David Capper, Johan M Kros, Sebastian Kurscheid, Wolfgang Wick, Roelien Enting, Michele Reni, Brian Thiessen, Frederic Dhermain, Jacoline E Bromberg, Loic Feuvret, Jaap C Reijneveld, Olivier Chinot, Johanna M M Gijtenbeek, John P Rossiter, Nicolas Dif, Carmen Balana, Jose Bravo-Marques, Paul M Clement, Christine Marosi, Tzahala Tzuk-Shina, Robert A Nordal, Jeremy Rees, Denis Lacombe, Warren P Mason, Roger Stupp
BACKGROUND: Outcome of low-grade glioma (WHO grade II) is highly variable, reflecting molecular heterogeneity of the disease. We compared two different, single-modality treatment strategies of standard radiotherapy versus primary temozolomide chemotherapy in patients with low-grade glioma, and assessed progression-free survival outcomes and identified predictive molecular factors. METHODS: For this randomised, open-label, phase 3 intergroup study (EORTC 22033-26033), undertaken in 78 clinical centres in 19 countries, we included patients aged 18 years or older who had a low-grade (WHO grade II) glioma (astrocytoma, oligoastrocytoma, or oligodendroglioma) with at least one high-risk feature (aged >40 years, progressive disease, tumour size >5 cm, tumour crossing the midline, or neurological symptoms), and without known HIV infection, chronic hepatitis B or C virus infection, or any condition that could interfere with oral drug administration...
September 26, 2016: Lancet Oncology
Jaap C Reijneveld, Martin J B Taphoorn, Corneel Coens, Jacoline E C Bromberg, Warren P Mason, Khê Hoang-Xuan, Gail Ryan, Mohamed Ben Hassel, Roelien H Enting, Alba A Brandes, Antje Wick, Olivier Chinot, Michele Reni, Guy Kantor, Brian Thiessen, Martin Klein, Eugenie Verger, Christian Borchers, Peter Hau, Michael Back, Anja Smits, Vassilis Golfinopoulos, Thierry Gorlia, Andrew Bottomley, Roger Stupp, Brigitta G Baumert
BACKGROUND: Temozolomide chemotherapy versus radiotherapy in patients with a high-risk low-grade glioma has been shown to have no significant effect on progression-free survival. If these treatments have a different effect on health-related quality of life (HRQOL), it might affect the choice of therapy. We postulated that temozolomide compromises HRQOL and global cognitive functioning to a lesser extent than does radiotherapy. METHODS: We did a prospective, phase 3, randomised controlled trial at 78 medical centres and large hospitals in 19 countries...
September 26, 2016: Lancet Oncology
Musteyde Yucebas, Sunde Yilmaz Susluer, Hasan Onur Caglar, Tugce Balci, Z Ozlem Dogan Sigva, Taner Akalin, Nezih Oktar, Tayfun Dalbasti, Cigir Biray Avci, Cumhur Gunduz
PURPOSE: The repressor element 1 (RE-1) silencing transcription factor (REST) is a transcription factor which represses the expression of neuronal differentiation-related genes including SYN1 gene. CoREST, encoded by RCOR1 gene, binds to the REST protein for remodeling of chromatin structure. Although there is a relation among REST, RCOR1, and SYN1 genes, the role of these genes in glioma tumors is still unclear. In this study, expressions of REST, RCOR1, and SYN1 genes were detected in primary cultures derived from tumor samples of diffuse astrocytoma (DA), anaplastic oligodendroglioma (AO), and glioblastoma multiforme (GBM) cases...
July 2016: Journal of B.U.ON.: Official Journal of the Balkan Union of Oncology
S Acharya, S M Perkins, J Campian, G Ansstas, M Chheda, C I Tsien, C G Robinson, J Huang
No abstract text is available yet for this article.
October 1, 2016: International Journal of Radiation Oncology, Biology, Physics
Payam Hacisalihoglu, Zafer Kucukodaci, Gokcen Gundogdu, Bilge Bilgic
AIM: The aim of our study is to observe the correlation between 1p/19q codeletion, IDH1 mutation and p53 protein overexpression and their prognostic value in Turkish anaplastic oligodendroglioma patients who were treated with adjuvant radiotherapy and temozolomide chemotherapy. MATERIAL AND METHODS: In our study we retrospectively evaluated 41 patients who were diagnosed as anaplastic oligodendroglioma. Thirty five patients received standard radiotherapy. Twenty six patients received standard temozolomide chemoterapy concurrent to radiotherapy...
August 23, 2016: Turkish Neurosurgery
Stuart A Grossman, Anna F Piotrowski
Novel diagnostic and therapeutic information has had a major impact on the care of patients with high-grade gliomas. These advances and discoveries are highlighted using case-based discussions to focus attention on the important diagnostic and treatment decisions that commonly arise during the care of patients with newly diagnosed glioblastoma, anaplastic astrocytoma, and anaplastic oligodendroglioma.
August 2016: Seminars in Neurology
Christos Yapijakis, Maria Adamopoulou, Konstantina Tasiouka, Costas Voumvourakis, George Stranjalis
BACKGROUND/AIM: A deeper understanding of the complex molecular pathology of brain malignancies is needed in order to develop more effective and targeted therapies of these highly lethal disorders. In an effort to further enlighten the molecular pathology of brain oncogenesis involving the her-2 (erbB-2/neu/ngl)/N-ras/nf1 pathway, we screened the genotypes of specimens from various types of brain tumors. MATERIALS AND METHODS: The studied specimens included 35 biopsies of four general categories: 13 neuroglial tumors (4 astrocytomas, 2 oligodendrogliomas, 7 glioblastomas multiforme), 14 meningiomas, 3 other nervous system tumors (2 schwannomas, 1 craniopharyngioma) and 5 metastatic tumors (such as lung carcinomas and chronic myelocytic leukemia)...
September 2016: Anticancer Research
Maria Podbielska, Zdzislaw M Szulc, Ewa Kurowska, Edward L Hogan, Jacek Bielawski, Alicja Bielawska, Narayan R Bhat
Th1 pro-inflammatory cytokines i.e., TNF-α and IFN-γ in combination are known to induce cell death in several cell types including oligodendrocytes but the mechanism of their synergistic cytotoxicity is unclear. Although ceramide (Cer) has been implicated in cytokine- and stress-induced cell death, its intracellular levels alone cannot explain cytokine synergy. We considered the possibility that Cer released as part of extracellular vesicles may contribute to cytokine-induced synergistic cell death. Using a human oligodendroglioma (HOG) cell line as a model, here we show that exosomes derived from TNF-α-treated donor cells, while being mildly toxic to fresh cultures (similar to individual cytokines), induce enhanced cell death when added to IFN-γ-primed target cultures in a fashion resembling the effect of cytokine combination...
September 13, 2016: Journal of Lipid Research
Johann-Martin Hempel, Sotirios Bisdas, Jens Schittenhelm, Cornelia Brendle, Benjamin Bender, Henk Wassmann, Marco Skardelly, Ghazaleh Tabatabai, Salvador Castaneda Vega, Ulrike Ernemann, Uwe Klose
The purpose of this study is to assess the diagnostic performance of diffusion kurtosis imaging (DKI) for in vivo molecular profiling of human glioma. Normalized mean kurtosis (MKn) and mean diffusivity (MDn) metrics from DKI were assessed in 50 patients with histopathologically confirmed glioma. The results were compared in regard to the WHO-based histological findings and molecular characteristics leading to integrated diagnosis (Haarlem Consensus): isocitrate-dehydrogenase (IDH1/2) mutation status, alpha-thalassemia/mental retardation syndrome X-linked (ATRX) expression, chromosome 1p/19q loss of heterozygosity (LOH), and O6-methylguanine DNA methyltransferase (MGMT) promoter methylation status...
September 7, 2016: Journal of Neuro-oncology
Liu Yang, Sayantanee Paul, Kenneth G Trieu, Lucas G Dent, Francesca Froldi, Marta Forés, Kaitlyn Webster, Kellee R Siegfried, Shu Kondo, Kieran Harvey, Louise Cheng, Gerardo Jiménez, Stanislav Y Shvartsman, Alexey Veraksa
The transcriptional repressor Capicua (Cic) controls tissue patterning and restricts organ growth, and has been recently implicated in several cancers. Cic has emerged as a primary sensor of signaling downstream of the receptor tyrosine kinase (RTK)/extracellular signal-regulated kinase (ERK) pathway, but how Cic activity is regulated in different cellular contexts remains poorly understood. We found that the kinase Minibrain (Mnb, ortholog of mammalian DYRK1A), acting through the adaptor protein Wings apart (Wap), physically interacts with and phosphorylates the Cic protein...
September 20, 2016: Proceedings of the National Academy of Sciences of the United States of America
Iris Völker, Iris Schwarze, Tina E Brezina, Saskia Köstlinger, Marion Hewicker-Trautwein
An 8-month-old, male African hedgehog clinically displayed a wobbly walk, anuria, inappetence and apathy, whereupon the suspected diagnosis wobbly hedgehog syndrome was made. After exacerbation, the hedgehog was euthanized. Histologically, a tumour mainly consisting of medium-sized, oval tumour cells and a smaller number of spindeloid cells was found in the cerebrum. The tumour contained neuropil islets and extracellular myxoid material. Immunohistochemically, the tumour cells expressed oligodendroglial (neurite outgrowth inhibitor, Nogo-A; oligodendrocyte transcription factor, Olig-2) and neuronal (neuron-specific enolase, NSE; microtubule-associated protein-2a, MAP-2a; synaptophysin) cell markers...
October 12, 2016: Tierärztliche Praxis. Ausgabe K, Kleintiere/Heimtiere
Emeline Tabouret, Anh Tuan Nguyen, Caroline Dehais, Catherine Carpentier, François Ducray, Ahmed Idbaih, Karima Mokhtari, Anne Jouvet, Emmanuelle Uro-Coste, Carole Colin, Olivier Chinot, Hugues Loiseau, Elisabeth Moyal, Claude-Alain Maurage, Marc Polivka, Emmanuèle Lechapt-Zalcman, Christine Desenclos, David Meyronet, Jean-Yves Delattre, Dominique Figarella-Branger
The new WHO classification of diffuse gliomas has been refined and now includes the 1p/19q codeletion, IDH1/2 mutation, and histone H3-K27M mutation. Our objective was to assess the prognostic value of the updated 2016 WHO classification in the French POLA cohort. All cases of high-grade oligodendroglial tumors sent for central pathological review and included into the French nationwide POLA cohort were reclassified according to the updated 4th WHO classification. In total, 1041 patients were included, with a median age at diagnosis of 50...
October 2016: Acta Neuropathologica
Yan-Xi Li, Zhifeng Shi, Abudumijiti Aibaidula, Hong Chen, Qisheng Tang, Kay Ka-Wai Li, Nellie Yuk-Fei Chung, Danny Tat-Ming Chan, Wai Sang Poon, Ying Mao, Jinsong Wu, Liangfu Zhou, Aden Ka-Yin Chan, Ho-Keung Ng
Although 1p/19q codeletion is the genetic hallmark defining oligodendrogliomas, approximately 30-40% of oligodendroglial tumors have intact 1p/19q in the literature and they demonstrate a worse prognosis. This group of 1p/19q intact oligodendroglial tumors is frequently suggested to be astrocytic in nature with TP53 and ATRX mutations but actually remains under-investigated. In the present study, we provided evidence that not all 1p/19q intact oligodendroglial tumors are astrocytic through histologic and molecular approaches...
August 18, 2016: Oncotarget
Franck Bielle, François Ducray, Karima Mokhtari, Caroline Dehais, Homa Adle-Biassette, Catherine Carpentier, Anaïs Chanut, Marc Polivka, Sylvie Poggioli, Shaï Rosenberg, Marine Giry, Yannick Marie, Charles Duyckaerts, Marc Sanson, Pola Network, Dominique Figarella-Branger, Ahmed Idbaih
The integrated diagnosis of anaplastic oligodendroglioma, IDH mutant and 1p/19q co-deleted, grade III (O3(id) ) is a histomolecular entity that WHO 2016 classification distinguished from other diffuse gliomas by specific molecular alterations. In contrast, its cell portrait is less well-known. The present study is focused on intertumor and intratumor, cell lineage-oriented, heterogeneity in O3(id) . Based on pathological, transcriptomic and immunophenotypic studies, a novel subgroup of newly diagnosed O3(id) overexpressing neuronal intermediate progenitor (NIP) genes was identified...
August 20, 2016: Brain Pathology
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