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https://www.readbyqxmd.com/read/28640700/cns-tumors-in-neurofibromatosis
#1
Jian Campian, David H Gutmann
Neurofibromatosis (NF) encompasses a group of distinct genetic disorders in which affected children and adults are prone to the development of benign and malignant tumors of the nervous system. The purpose of this review is to discuss the spectrum of CNS tumors arising in individuals with NF type 1 (NF1) and NF type 2 (NF2), their pathogenic etiologies, and the rational treatment options for people with these neoplasms. This article is a review of preclinical and clinical data focused on the treatment of the most common CNS tumors encountered in children and adults with NF1 and NF2...
June 22, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28633092/epilepsy-in-neurofibromatosis-type-1
#2
Anthony Pecoraro, Eric Arehart, William Gallentine, Rodney Radtke, Edward Smith, Carolyn Pizoli, Sujay Kansagra, Elie Abdelnour, Roger McLendon, Mohamad A Mikati
OBJECTIVES: To describe the characteristics of epilepsy in patients with Neurofibromatosis type 1 (NF1). METHODS: Analysis of a cohort of consecutive NF1 patients seen in our NF1 clinic during a three-year period. RESULTS: Of the 184 NF1 patients seen during that period, 26 had epilepsy and three had febrile seizures. Of the 26, 17 (65%) had localization-related epilepsy, seven of whom (41%) were drug resistant. Six (23%) had apparently primary generalized epilepsy (0/6 drug resistant), two (8%) Lennox-Gastaut syndrome, and one (4%) West syndrome (all three were drug-resistant)...
June 17, 2017: Epilepsy & Behavior: E&B
https://www.readbyqxmd.com/read/28631895/growth-hormone-excess-in-children-with-neurofibromatosis-type-1-and-optic-glioma
#3
Paola Cambiaso, Stefania Galassi, Melania Palmiero, Angela Mastronuzzi, Francesca Del Bufalo, Rossella Capolino, Antonella Cacchione, Paola S Buonuomo, Michaela V Gonfiantini, Andrea Bartuli, Marco Cappa, Marina Macchiaiolo
In children with neurofibromatosis type 1 (NF1) and optic pathways glioma (OPG), growth hormone (GH) excess has been rarely reported and mainly associated to central precocious puberty. The aim of our study is to evaluate the prevalence of GH excess, the association with central precocious puberty, the relation with tumor site and the evolution over time in a large cohort of children with NF1 and OPG. Sixty-four NF1 children with OPG were evaluated. Patients with stature and/or height velocity >2 SD for age were studied for GH secretion...
June 20, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28620004/cancer-and-central-nervous-system-tumor-surveillance-in-pediatric-neurofibromatosis-1
#4
REVIEW
D Gareth R Evans, Hector Salvador, Vivian Y Chang, Ayelet Erez, Stephan D Voss, Kami Wolfe Schneider, Hamish S Scott, Sharon E Plon, Uri Tabori
Although the neurofibromatoses consist of at least three autosomal dominantly inherited disorders, neurofibromatosis 1 (NF1), neurofibromatosis 2 (NF2), and schwannomatosis, NF1 represents a multisystem pleiotropic condition very different from the other two. NF1 is a genetic syndrome first manifesting in childhood; affecting multiple organs, childhood development, and neurocognitive status; and presenting the clinician with often complex management decisions that require a multidisciplinary approach. Molecular genetic testing (see article for detailed discussion) is recommended to confirm NF1, particularly in children fulfilling only pigmentary features of the diagnostic criteria...
June 15, 2017: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/28606795/endothelium-independent-primitive-myxoid-vascularization-creates-invertebrate-like-channels-to-maintain-blood-supply-in-optic-gliomas
#5
Matija Snuderl, Guoan Zhang, Pamela Wu, Tara S Jennings, Seema Shroff, Valerio Ortenzi, Rajan Jain, Benjamin Cohen, Jason J Reidy, Mitchell S Dushay, Jeffrey H Wisoff, David H Harter, Matthias A Karajannis, David Fenyo, Thomas A Neubert, David Zagzag
Optic gliomas are brain tumors characterized by slow growth, progressive loss of vision, and limited therapeutic options. Optic gliomas contain various amounts of myxoid matrix, which can represent most of the tumor mass. We sought to investigate biological function and protein structure of the myxoid matrix in optic gliomas to identify novel therapeutic targets. We reviewed histological features and clinical imaging properties, analyzed vasculature by immunohistochemistry and electron microscopy, and performed liquid chromatography-mass spectrometry on optic gliomas, which varied in the amount of myxoid matrix...
June 9, 2017: American Journal of Pathology
https://www.readbyqxmd.com/read/28593402/clinical-presentation-and-prognostic-indicators-in-100-adults-and-children-with-neurofibromatosis-1-associated-non-optic-pathway-brain-gliomas
#6
Susan Byrne, Steve Connor, Karine Lascelles, Ata Siddiqui, Darren Hargrave, Rosalie E Ferner
Type 1 Neurofibromatosis (NF1) is a common autosomal dominant condition, with a major impact on the nervous system, eye, bone, and skin, and a predisposition to malignancy. At present it is not possible to predict clinically or on imaging, whether a brain tumour will remain indolent or undergo high-grade change. There are no consensus guidelines on the follow-up of non-optic pathway glioma (non-OPG) tumours in NF1. One hundred patients from the National NF1 Service with generalised NF1 and a diagnosis of non-OPG glioma were followed up for a median time of 63 months after glioma detection...
June 7, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28587881/mr-imaging-findings-in-children-with-spasmus-nutans
#7
Meredith Bowen, Jason Peragallo, Stephen F Kralik, Andrea Poretti, Thierry A G M Huisman, Bruno P Soares
BACKGROUND: Spasmus Nutans (SN) is a rare pediatric ophthalmologic syndrome of nystagmus, head bobbing and abnormal head positioning. Historically, SN has been associated with underlying optic pathway gliomas (OPG); however, evidence of this is association is based primarily on a small number of isolated case reports. Prior retrospective analyses have found the rate of OPG to be less than 2%, but only intermittently utilized neuroimaging with CT, which has limited sensitivity for detection of small lesions in the optic pathway...
June 3, 2017: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/28587872/validation-of-an-automated-tractography-method-for-the-optic-radiations-as-a-biomarker-of-visual-acuity-in-neurofibromatosis-associated-optic-pathway-glioma
#8
REVIEW
Peter de Blank, Michael J Fisher, Haley Gittleman, Jill S Barnholtz-Sloan, Chaitra Badve, Jeffrey I Berman
INTRODUCTION: Fractional anisotropy (FA) of the optic radiations has been associated with vision deficit in multiple intrinsic brain pathologies including NF1 associated optic pathway glioma, but hand-drawn regions of interest used in previous tractography methods limit consistency of this potential biomarker. We created an automated method to identify white matter tracts in the optic radiations and compared this method to previously reported hand-drawn tractography. METHOD: Automated tractography of the optic radiation using probabilistic streamline fiber tracking between the lateral geniculate nucleus of the thalamus and the occipital cortex was compared to the hand-drawn method between regions of interest posterior to Meyer's loop and anterior to tract branching near the calcarine cortex...
June 3, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28577031/natural-history-of-optic-pathway-gliomas-in-a-cohort-of-unselected-patients-affected-by-neurofibromatosis-1
#9
Eva Trevisson, Matteo Cassina, Enrico Opocher, Virginia Vicenzi, Marta Lucchetta, Raffaele Parrozzani, Giacomo Miglionico, Rodica Mardari, Elisabetta Viscardi, Edoardo Midena, Maurizio Clementi
Optic pathway glioma (OPG) represents the most common central nervous system tumor in children with Neurofibromatosis type-1 (NF1). Although overall survival is usually good, no clear prognostic factors have been identified so far. We assessed the natural history of OPG in a cohort of unselected patients affected by NF1. We retrospectively evaluated 414 consecutive patients affected by NF1 and referred to our NF1 clinic before age 6. Average follow-up was 11.9 years: 52 out of 414 patients had OPG with a total cumulative incidence of 15...
June 2, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28575296/letter-intraoperative-near-infrared-optical-imaging-can-localize-gadolinium-enhancing-gliomas-during-surgery
#10
Marc Zanello, Alexandre Roux, Johan Pallud
No abstract text is available yet for this article.
June 1, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28558180/neural-tumors-of-the-orbit-what-is-new
#11
Mark P Ghassibi, Jan P Ulloa-Padilla, Sander R Dubovy
Primary neural tumors of the orbit account for approximately 10% of all orbital tumors. Different tumor entities include meningiomas, optic nerve gliomas, neurofibromas, schwannomas, malignant peripheral nerve sheath tumors, and granular cell tumors. This review summarizes current concepts regarding epidemiology, clinical presentation, diagnosis, pathology, immunohistochemistry, prognosis, and treatment for neural tumors of the orbit based on the available literature.
May 2017: Asia-Pacific Journal of Ophthalmology
https://www.readbyqxmd.com/read/28525381/the-cell-of-origin-dictates-the-temporal-course-of-neurofibromatosis-1-nf1-low-grade-glioma-formation
#12
Anne C Solga, Joseph A Toonen, Yuan Pan, Patrick J Cimino, Yu Ma, Guillaume A Castillon, Scott M Gianino, Mark H Ellisman, Da Yong Lee, David H Gutmann
Low-grade gliomas are one of the most common brain tumors in children, where they frequently form within the optic pathway (optic pathway gliomas; OPGs). Since many OPGs occur in the context of the Neurofibromatosis Type 1 (NF1) cancer predisposition syndrome, we have previously employed Nf1 genetically-engineered mouse (GEM) strains to study the pathogenesis of these low-grade glial neoplasms. In the light of the finding that human and mouse low-grade gliomas are composed of Olig2+ cells and that Olig2+ oligodendrocyte precursor cells (OPCs) give rise to murine high-grade gliomas, we sought to determine whether Olig2+ OPCs could be tumor-initiating cells for Nf1 optic glioma...
May 3, 2017: Oncotarget
https://www.readbyqxmd.com/read/28524459/prognostic-role-of-conventional-and-dynamic-contrast-enhanced-mri-in-optic-pathway-gliomas
#13
Nutchawan Jittapiromsak, Ping Hou, Ho-Ling Liu, Jia Sun, John M Slopis, T Linda Chi
BACKGROUND AND PURPOSE: The natural history of optic pathway glioma (OPG) is highly variable and unpredictable. We present a pilot study of the prognostic role of conventional and dynamic contrast-enhanced magnetic resonance imaging (DCE MRI) in the evaluation of OPG. METHODS: We retrospectively reviewed 17 patients with 20 pretreatment OPG lesions who underwent conventional and DCE MRI between January 2010 and December 2016. Conventional MRI was evaluated for enhancement pattern, cystic component, optic nerve tortuosity, optic nerve dural ectasia, and optic nerve perineural thickening...
May 19, 2017: Journal of Neuroimaging: Official Journal of the American Society of Neuroimaging
https://www.readbyqxmd.com/read/28524122/-a-plasminogen-regulation-system-in-brain-tumors
#14
O I Kit, E M Frantsiyants, L S Kozlova, E E Rostorguev, I V Balyazin-Parfenov, Yu A Pogorelova
INTRODUCTION: Tumor progression and neovascularization during malignant processes are believed to be associated with plasminogen activators and the PAI-1 inhibitor, but their role and interactions in various types of brain tumors have been studied insufficiently. AIM: To conduct a comparative study of plasminogen regulation in optic nerve sheath meningiomas, glioblastomas, and brain metastases of breast cancer, as well as in perifocal tissues surrounding the tumors...
2017: Zhurnal Voprosy Neĭrokhirurgii Imeni N. N. Burdenko
https://www.readbyqxmd.com/read/28506483/cortical-and-subcortical-functional-neuroanatomy-for-low-grade-glioma-surgery
#15
REVIEW
L Chenin, M Lefranc, S Velut, P Foulon, E Havet, J Peltier
Knowledge of the encephalon anatomy is crucial for neurosurgical practice, especially the main cortical functional structures and their connections. General organisation of the encephalon is presented with frontal, parietal, occipital, temporal, limbic and insular lobes and their Brodmann correspondence. Secondly, subcortical anatomy will be presented with main white matter fasciculi in three separated categories: association, commissural and projection fibers. Main association fibers are inferior occipitofrontal fasciculus, superior longitudinal fasciculus, arcuate fasciculus, inferior longitudinal fasciculus, uncinate fasciculus, and cingulum...
May 12, 2017: Neuro-Chirurgie
https://www.readbyqxmd.com/read/28484000/h2o2-responsive-liposomal-nanoprobe-for-photoacoustic-inflammation-imaging-and-tumor-theranostics-via-in-vivo-chromogenic-assay
#16
Qian Chen, Chao Liang, Xiaoqi Sun, Jiawen Chen, Zhijuan Yang, He Zhao, Liangzhu Feng, Zhuang Liu
Abnormal H2O2 levels are closely related to many diseases, including inflammation and cancers. Herein, we simultaneously load HRP and its substrate, 2,2'-azino-bis(3-ethylbenzothiazoline-6-sulfonic acid) (ABTS), into liposomal nanoparticles, obtaining a Lipo@HRP&ABTS optical nanoprobe for in vivo H2O2-responsive chromogenic assay with great specificity and sensitivity. In the presence of H2O2, colorless ABTS would be converted by HRP into the oxidized form with strong near-infrared (NIR) absorbance, enabling photoacoustic detection of H2O2 down to submicromolar concentrations...
May 23, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28477028/whole-tumor-rna-sequencing-and-deconvolution-reveal-a-clinically-prognostic-pten-pi3k-regulated-glioma-transcriptional-signature
#17
Yuan Pan, Erin C Bush, Joseph A Toonen, Yu Ma, Anne C Solga, Peter A Sims, David H Gutmann
The concept that solid tumors are maintained by a productive interplay between neoplastic and non-neoplastic elements has gained traction with the demonstration that stromal fibroblasts and immune system cells dictate cancer development and progression. While less studied, brain tumor (glioma) biology is likewise influenced by non-neoplastic immune system cells (macrophages and microglia) which interact with neoplastic glioma cells to create a unique physiological state (glioma ecosystem) distinct from that found in the normal tissue...
April 18, 2017: Oncotarget
https://www.readbyqxmd.com/read/28476644/autoantibody-mediated-diseases-of-the-cns-structure-dysfunction-and-therapy
#18
REVIEW
James Varley, Jennifer Taylor, Sarosh R Irani
The field of neuronal autoantibody associated diseases of the central nervous system has expanded dramatically in the last few years. The range of identified neuronal and glial antibody targets has led to the accurate classification of a number of syndromes which each associate with characteristic clinical features. These diseases are especially important due to their frequent response to immunotherapies. Antibodies against the N-methyl, d-aspartate receptor (NMDAR) and leucine-rich glioma inactivated 1 (LGI1) are the commonest autoantibodies known in patients with autoimmune forms of encephalitis...
May 3, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28445745/increased-tissue-stiffness-in-tumors-from-mice-with-neurofibromatosis-1-optic-glioma
#19
Christopher Walter, Lindsey Crawford, Melinda Lai, Joseph A Toonen, Yuan Pan, Shelly Sakiyama-Elbert, David H Gutmann, Amit Pathak
Children with neurofibromatosis type 1 (NF1) cancer predisposition syndrome are prone to the development of low-grade brain tumors (gliomas) within the optic pathway (optic gliomas). One of the key obstacles to developing successful therapeutic strategies for these tumors is the striking lack of information about the mechanical properties that characterize these tumors relative to non-neoplastic optic nerve tissue. To study the physical changes that may occur when an optic nerve glioma is present, we employed atomic force microscopy to measure the stiffness of healthy versus tumor-bearing optic nerve tissue...
April 25, 2017: Biophysical Journal
https://www.readbyqxmd.com/read/28439784/practical-calculation-method-to-estimate-the-absolute-boron-concentration-in-tissues-using-18-f-fbpa-pet
#20
Tadashi Watabe, Kohei Hanaoka, Sadahiro Naka, Yasukazu Kanai, Hayato Ikeda, Masanao Aoki, Eku Shimosegawa, Mitsunori Kirihata, Jun Hatazawa
PURPOSE: The purpose of this study was to establish a practical method to estimate the absolute boron concentrations in the tissues based on the standardized uptake values (SUVs) after administration of 4-borono-phenylalanine (BPA) using 4-borono-2-(18)F-fluoro-phenylalanine ((18)F-FBPA) PET. METHODS: Rat xenograft models of C6 glioma (n = 7, body weight 241 ± 28.0 g) were used for the study. PET was performed 60 min after intravenous injection of (18)F-FBPA (30...
April 24, 2017: Annals of Nuclear Medicine
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