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https://www.readbyqxmd.com/read/29230288/embryonal-tumor-with-multilayered-rosettes-c19mc-altered-report-of-an-extremely-rare-malignant-pediatric-central-nervous-system-neoplasm
#1
Muhammad Usman Tariq, Zubair Ahmad, Muhammad Khurram Minhas, Aisha Memon, Noreen Mushtaq, Cynthia Hawkins
The 2016 update of the WHO Classification of Tumours of the Central Nervous System has redefined a number of tumors. Embryonal tumor with multilayered rosettes, C19MC-altered is one such tumor entity which has been newly defined on the basis of a characteristic molecular alteration. We report, to our knowledge, the first case of this rare pediatric brain neoplasm in the Pakistani population. An 8-month-old girl was presented with vomiting and left-sided ptosis, and magnetic resonance imaging scan showed a cerebellar tumor...
2017: SAGE Open Medical Case Reports
https://www.readbyqxmd.com/read/29222305/biology-of-cns-lymphoma-and-the-potential-of-novel-agents
#2
REVIEW
James L Rubenstein
Primary and secondary CNS lymphomas are aggressive brain tumors that pose an immense challenge to define in terms of molecular pathogenesis, as well as to effectively treat. During the past 10 years improvements in survival have been achieved with the implementation of anti-CD20 immunotherapy and optimization of dose-intensive consolidation strategies. The applications of whole-exome sequencing, comparative genomic hybridization, transcriptional profiling, and examination of the tumor microenvironment, particularly in the context of clinical investigation, provide insights that create a roadmap for the development and implementation of novel targeted agents for this disease...
December 8, 2017: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/29221349/ab-thymoma-with-atypical-type-a-component-with-delayed-multiple-lung-and-brain-metastases
#3
Wieslawa Grajkowska, Ewa Matyja, Jacek Kunicki, Sylwia Szymanska, Alexander Marx, Cleo-Aron Weis, Renata Langfort, Malgorzata Szolkowska
An atypical type A thymoma is a newly added entity to the last World Health Organization (WHO) histological classification [2015] of uncertain prognosis. The conventional type A and AB thymomas are usually locally aggressive neoplasms that rarely metastasize with distant metastases to the central nervous system (CNS) occurring extremely exceptionally. We present a history of a woman with a mediastinal tumor originally considered to be a Masaoka-Koga stage II "mixed thymoma with well-differentiated thymic carcinoma component" according to the historic Müller-Hermelink nomenclature...
September 2017: Journal of Thoracic Disease
https://www.readbyqxmd.com/read/29216380/recent-advances-in-intradural-spinal-tumors
#4
Muhammad M Abd-El-Barr, Kevin T Huang, Ziev B Moses, J Bryan Iorgulescu, John H Chi
Intradural spinal tumors are rare tumors of the central nervous system. Due to the eloquence of the spinal cord and its tracts, compact architecture of the cord and nerves and the infiltrative nature of some of these tumors, surgical resection is difficult to achieve without causing neurological deficits. Likewise, chemotherapy and radiotherapy are utilized more cautiously in the treatment of intradural spinal tumors than their cranial counterparts. Targeted therapies aimed at the genetic alterations and molecular biology tailored to these tumors would be helpful, but are lacking...
December 5, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/29201475/a-rare-case-of-glioblastoma-multiforme-with-osseous-metastases
#5
Rubens Barros Costa, Ricardo Costa, Jason Kaplan, Marcelo Rocha Cruz, Hiral Shah, Maria Matsangou, Benedito Carneiro
Glioblastoma multiforme is the most common malignant primary central nervous system neoplasm in adults. It has a very aggressive natural history with a median overall survival estimated at 14.6 months despite multimodality treatment. Extracranial metastases are very rare with few case reports published to date. We report the case of a 65-year-old male who underwent maximal safe resection for a newly diagnosed brain mass after presentation with new neurologic symptoms. He then received standard postsurgical adjuvant treatment for glioblastoma...
2017: Case Reports in Oncological Medicine
https://www.readbyqxmd.com/read/29200117/common-histologically-benign-tumors-of-the-brain
#6
Roy E Strowd, Jaishri O Blakeley
PURPOSE OF REVIEW: As a group, benign tumors account for the majority of primary neoplasms affecting the central nervous system (CNS). This article reviews the epidemiology, clinical presentation, neuroimaging features, and management of the most common of these tumors: meningiomas, schwannomas, and pituitary adenomas. RECENT FINDINGS: Awareness of the most common nonmalignant tumors of the CNS and their management guidelines is important as many of these tumors are managed conservatively, with neurologists playing a primary role in both surveillance and symptom management...
December 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/29192869/predictors-of-clinical-outcome-in-pediatric-oligodendroglioma-meta-analysis-of-individual-patient-data-and-multiple-imputation
#7
Kevin Yuqi Wang, Emilian R Vankov, Doris Da May Lin
OBJECTIVE Oligodendroglioma is a rare primary CNS neoplasm in the pediatric population, and only a limited number of studies in the literature have characterized this entity. Existing studies are limited by small sample sizes and discrepant interstudy findings in identified prognostic factors. In the present study, the authors aimed to increase the statistical power in evaluating for potential prognostic factors of pediatric oligodendrogliomas and sought to reconcile the discrepant findings present among existing studies by performing an individual-patient-data (IPD) meta-analysis and using multiple imputation to address data not directly available from existing studies...
December 1, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/29187687/-symmetrical-lesions-of-the-cerebral-peduncles-and-internal-capsules-on-mri-in-a-patient-with-extranodal-nk-t-cell-lymphoma
#8
Yuko Takeuchi, Tomotsugu Kaga, Miho Ieda, Yumi Kojima, Jun Ochiai, Chiyuki Mabuchi
A 72-year-old man was admitted to our hospital because of subacute progressive consciousness disturbance. FLAIR images showed diffuse high intensity areas in the bilateral thalamus, basal ganglia, and white matter. Chest CT revealed a mass in the right lung. The patient was diagnosed with extranodal natural killer/T cell lymphoma (ENKL) according to the results of a biopsy of the right pulmonary mass. The FLAIR images showed diffuse white matter lesions, without a mass such as lymphomatosis cerebri. On diffusion-weighted images, symmetrical high-intensity lesions of the cerebral peduncle and the internal capsule suggested high-density lymphoma cells around neurons...
November 28, 2017: Rinshō Shinkeigaku, Clinical Neurology
https://www.readbyqxmd.com/read/29180935/atypical-bilateral-cerebellopontine-angle-medulloblastoma-differential-diagnosis-immunohistochemical-features-and-radiological-presentation
#9
Kelvin M Piña Batista, Sayoa Alvarez de Eulate-Beramendi, María Rico, Victor Rodrigo, Yordy E Batista Batista, Kenia Y Alvarez Reyes
Medulloblastoma (MB) is a highly aggressive soft tissue neoplasm, classified as a primitive neuroectodermal tumor. It is the most common posterior fossa tumor in children, but occurs rarely in adults. MB accounts for approximately 20% of all primary central nervous system (CNS) tumors of childhood, while its incidence is around 1% of adult brain tumors. Most often it occurs in the cerebellum. We report a case of multicentric MB involving the bilateral cerebellopontine angle (CPA) and right cerebellar hemisphere...
2017: Contemporary Oncology Współczesna Onkologia
https://www.readbyqxmd.com/read/29167820/engineering-chimeric-antigen-receptor-t-cells-to-treat-glioblastoma
#10
Bryan D Choi, Donald M O'Rourke, Marcela V Maus
Immunotherapy has emerged as a promising strategy for glioblastoma (GBM), a disease that remains universally fatal despite currently available standard-of-care. Adoptive T cell therapy has been shown to produce potent antitumor immunity while obviating the need for traditional antigen presentation and primary immune responses. Chimeric antigen receptors (CARs) are specialized molecules that can be expressed on the surface of T cells allowing for redirected cytotoxicity against tumor antigens of interest. To date, the application of CAR T cells for GBM has been relatively limited, in large part due to a dearth of well-described tumor specific antigens that are both homogenously and frequently expressed...
August 2017: Journal of Targeted Therapies in Cancer
https://www.readbyqxmd.com/read/29165710/risk-of-soft-tissue-sarcoma-among-69-460-five-year-survivors-of-childhood-cancer-in-europe
#11
Chloe J Bright, Mike M Hawkins, David L Winter, Daniela Alessi, Rodrigue S Allodji, Francesca Bagnasco, Edit Bárdi, Andrea Bautz, Julianne Byrne, Elizabeth A M Feijen, Miranda M Fidler, Stanislaw Garwicz, Desiree Grabow, Thorgerdur Gudmundsdottir, Joyeeta Guha, Nadia Haddy, Momcilo Jankovic, Peter Kaatsch, Melanie Kaiser, Claudia E Kuehni, Helena Linge, Hilde Øfstaas, Cecile M Ronckers, Roderick Skinner, Jop C Teepen, Monica Terenziani, Giao Vu-Bezin, Finn Wesenberg, Thomas Wiebe, Carlotta Sacerdote, Zsuzsanna Jakab, Riccardo Haupt, Päivi Lähteenmäki, Lorna Zadravec Zaletel, Rahel Kuonen, Jeanette F Winther, Florent de Vathaire, Leontien C Kremer, Lars Hjorth, Raoul C Reulen
Background: Childhood cancer survivors are at risk of subsequent primary soft-tissue sarcomas (STS), but the risks of specific STS histological subtypes are unknown. We quantified the risk of STS histological subtypes after specific types of childhood cancer. Methods: We pooled data from 13 European cohorts, yielding a cohort of 69 460 five-year survivors of childhood cancer. Standardized incidence ratios (SIRs) and absolute excess risks (AERs) were calculated. Results: Overall, 301 STS developed compared with 19 expected (SIR = 15...
November 20, 2017: Journal of the National Cancer Institute
https://www.readbyqxmd.com/read/29158570/expression-of-sry-related-hmg-box-transcription-factors-sox-2-and-9-in-craniopharyngioma-subtypes-and-surrounding-brain-tissue
#12
Vivian Thimsen, Nora John, Michael Buchfelder, Jörg Flitsch, Rudolf Fahlbusch, Harald Stefanits, Engelbert Knosp, Marco Losa, Rolf Buslei, Annett Hölsken
Stem cells have been discovered as key players in the genesis of different neoplasms including craniopharyngioma (CP), a rare tumour entity in the sellar region. Sox2 and Sox9 are well-known stem cell markers involved in pituitary development. In this study we analysed the expression of both transcription factors using immunohistochemistry in a large cohort of 64 adamantinomatous (aCP) and 9 papillary CP (pCP) and quantitative PCR in 26 aCP and 7 pCP. Whereas immunohistochemically Sox2+ cells were verifiable in only five aCP (7...
November 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29143062/-intracranial-cystic-lesions
#13
REVIEW
F Ahlhelm, K Shariat, S Götschi, S Ulmer
CLINICAL PROBLEM: Intracerebral cysts are common findings in imaging of the neurocranium and are not always clinically significant. The pathological spectrum of intracerebral cysts is, however, very broad and in addition to incidental findings includes developmental disorders, malformation tumors, primary and secondary neoplasms and infectious etiologies, such as cerebral abscess formation, cysticercosis or residuals after congenital cytomegalovirus infections. Intracerebral cystic defects may be caused by inflammatory central nervous system (CNS) diseases, such as multiple sclerosis as well as by mitochondriopathies, leukodystrophy, electrolyte disturbances or osmotic demyelination syndrome or brain infarctions, e...
November 15, 2017: Der Radiologe
https://www.readbyqxmd.com/read/29075565/assisting-the-neurologist-in-diagnosis-of-cns-malignancies-current-possibilities-and-limits-of-cerebrospinal-fluid-cytology-and-immunocytochemistry
#14
Jaroslava Dušková, Ondřej Sobek
OBJECTIVES: In tumorous impairment of CNS, cytological identification of the neoplastic cells in CSF frequently requires the use of ancillary techniques. Our methods are focused on identifying algorithms that increase the probability of identifying CSF malignant cells. MATERIALS AND METHODS: A total of 1.272 CSF samples from patients with tumorous infiltration of CNS of nonhematologic origin along with 721 samples from patients with hematologic malignancies were analyzed in a complex setting including cytological and immunocytochemical investigations...
October 2017: Brain and Behavior
https://www.readbyqxmd.com/read/29067721/the-perivascular-microenvironment-in-epstein-barr-virus-positive-primary-central-nervous-system-lymphoma-the-role-of-programmed-cell-death-1-and-programmed-cell-death-ligand-1
#15
Yasuo Sugita, Takuya Furuta, Koichi Ohshima, Satoru Komaki, Junko Miyoshi, Motohiro Morioka, Hideyuki Abe, Takanori Nozawa, Yukihiko Fujii, Hitoshi Takahashi, Akiyoshi Kakita
It has been shown that high expression of certain immune checkpoint molecules, including those of the programmed death protein 1/programmed death ligand 1 (PD-1/PD-L1) axis, can be utilized to regulate immunosuppression in the microenvironment of malignant neoplasms. For the purpose of clarifying the immune-escape mechanism of primary central nervous system lymphomas (PCNSLs), particularly in Epstein-Barr virus (EBV)-positive cases, markers for PD-1, PD-L1, tumor-associated macrophages (TAMs), and tumor-infiltrating lymphocytes (TILs) in 39 surgical specimens of PCNSLs (17 EBV-positive, 22 EBV-negative) were investigated by immunohistochemistry...
October 24, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/29055168/long-term-safety-of-growth-hormone-a-combined-registry-analysis
#16
Kirstine Stochholm, Wieland Kiess
OBJECTIVES: Preliminary data from the French cohort of the Safety and Appropriateness of Growth hormone treatments in Europe (SAGhE) study raised concerns regarding the safety of recombinant human GH, suggesting that GH may increase mortality and incidence of stroke in patients treated during childhood for GH deficiency or short stature. We evaluated published safety data, focusing on mortality, neoplasms, cerebrovascular events and diabetes across a number of large-scale pharmaceutical company GH registries...
October 21, 2017: Clinical Endocrinology
https://www.readbyqxmd.com/read/29049858/adolescents-and-young-adults-with-brain-tumors-in-the-context-of-molecular-advances-in-neuro-oncology
#17
REVIEW
Michal Zapotocky, Vijay Ramaswamy, Alvaro Lassaletta, Eric Bouffet
Adolescents and young adults (AYA) comprise a specific group of oncology patients with a distinct biological and epidemiological spectrum of central nervous system neoplasms. It has been well documented that they differ clinically, especially in relation to prognosis and chemotherapy tolerance; however, the underlying reasons for this are unclear. Recent advances in the genomics of both childhood and adult brain tumors have provided new explanations and insights into the previously described age-dependent heterogeneity...
October 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29049847/neurofibromatosis-type-1-and-optic-pathway-glioma-molecular-interplay-and-therapeutic-insights
#18
REVIEW
Soumen Khatua, David H Gutmann, Roger J Packer
Children with neurofibromatosis type 1 (NF1) are predisposed to develop central nervous system neoplasms, the most common of which are low-grade gliomas (LGGs). The absence of human NF1 associated LGG-derived cell lines, coupled with an inability to generate patient-derived xenograft models, represents barriers to profile molecularly targeted therapies for these tumors. Thus, genetically engineered mouse models have been identified to evaluate the interplay between Nf1-deficient tumor cells and nonneoplastic stromal cells to evaluate potential therapies for these neoplasms...
October 19, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29042334/primary-diffuse-large-b-cell-lymphoma-in-a-patient-with-rubinstein-taybi-syndrome-case-report-and-review-of-literature
#19
Christopher Sy, James Henry, Bhavani Kura, Andrew Brenner, Ramesh Grandhi
BACKGROUND: Rubinstein-Taybi syndrome (RSTS) is a rare, congenital syndrome that is known to be associated with neoplasms of various organ systems. Evaluation and treatment of such patients is challenging given the cognitive delay and heterogeneity of pathological presentations that define this syndrome. CASE DESCRIPTION: Presented here is a case of a patient with RSTS, diagnosed at birth, who presented with subtle symptoms of lethargy and a change in behavior. He was found to have a large (7...
October 14, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29036598/global-dna-methylation-analysis-reveals-mir-214-3p-contributes-to-cisplatin-resistance-in-pediatric-intracranial-nongerminomatous-malignant-germ-cell-tumors
#20
Tsung-Han Hsieh, Yun-Ru Liu, Ting-Yu Chang, Muh-Lii Liang, Hsin-Hung Chen, Hsei-Wei Wang, Yun Yen, Tai-Tong Wong
Background: Pediatric central nervous system germ cell tumors (CNSGCTs) are rare and heterogeneous neoplasms, and they can be divided into germinomas and nongerminomatous GCTs (NGGCTs). NGGCTs are further subdivided into mature teratomas and nongerminomatous malignant GCTs (NGMGCTs). Clinical outcomes suggest that NGMGCTs have poor prognosis and survival, and that they require more extensive radiotherapy and adjuvant chemotherapy. However, the mechanisms underlying this difference are still unclear...
October 3, 2017: Neuro-oncology
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