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https://www.readbyqxmd.com/read/29055168/long-term-safety-of-growth-hormone-a-combined-registry-analysis
#1
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
#2
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
#3
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
#4
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
#5
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
https://www.readbyqxmd.com/read/28989289/epidemiology-diagnosis-and-optimal-management-of-glioma-in-adolescents-and-young-adults
#6
REVIEW
Tejan P Diwanji, Alexander Engelman, James W Snider, Pranshu Mohindra
Neoplasms of the central nervous system (CNS) are the most frequently encountered solid tumors of childhood, but are less common in adolescents and young adults (AYA), aged 15-39 years. Gliomas account for 29%-35% of the CNS tumors in AYA, with approximately two-thirds being low-grade glioma (LGG) and the remaining being high-grade glioma (HGG). We review the epidemiology, work-up, and management of LGG and HGG, focusing on the particular issues faced by the AYA population relative to pediatric and adult populations...
2017: Adolescent Health, Medicine and Therapeutics
https://www.readbyqxmd.com/read/28968370/peripheral-nerve-hyperexcitability-syndromes
#7
Komal Sawlani, Bashar Katirji
PURPOSE OF REVIEW: This article provides a review of the clinical phenotypes and evaluation of peripheral nerve hyperexcitability syndromes. These rare diagnoses include cramp-fasciculation syndrome, Isaacs syndrome, and Morvan syndrome. Recent investigations have led to an understanding of the autoimmune underpinnings of these conditions and their specific associated antibodies. As the presentation of peripheral nerve hyperexcitability syndromes includes muscle stiffness, twitches, and spasms, which are also shared with certain central nervous system and myopathic conditions, the differential diagnosis of peripheral nerve hyperexcitability syndromes is reviewed...
October 2017: Continuum: Lifelong Learning in Neurology
https://www.readbyqxmd.com/read/28967307/introduction-surgical-management-of-skull-base-meningiomas
#8
Gabriel Zada, Mustafa K Başkaya, Mitesh V Shah
Meningiomas represent the most common primary intracranial neoplasm treated by neurosurgeons. Although multimodal treatment of meningiomas includes surgery, radiation-based treatments, and occasionally medical therapy, surgery remains the mainstay of treatment for most symptomatic meningiomas. Because of the intricate relationship of the dura mater and arachnoid mater with the central nervous system and cranial nerves, meningiomas can arise anywhere along the skull base or convexities, and occasionally even within the ventricular system, thereby mandating a catalog of surgical approaches that neurosurgeons may employ to individualize treatment for patients...
October 2017: Neurosurgical Focus
https://www.readbyqxmd.com/read/28966496/spectrum-of-meningioma-with-special-reference-to-prognostic-utility-of-er-pr-and-ki67-expression
#9
Madhumita Mukhopadhyay, Chhanda Das, Madhu Kumari, Ankita Sen, Bedabrata Mukhopadhyay, Biswanath Mukhopadhyay
BACKGROUND: Meningiomas are the most common primary central nervous system neoplasms originating from the arachnoid cap cells and constitute between 13% and 26% of all intracranial tumors. AIMS AND OBJECTIVES: The aim of the study was to analyze the age-, sex-, and site-wise distribution of different histological patterns of meningiomas seen in our center and to assess the status of estrogen receptor (ER), progesterone receptor (PR), and proliferation marker Ki-67 in various grades of meningioma...
October 2017: Journal of Laboratory Physicians
https://www.readbyqxmd.com/read/28954350/-clinical-characteristics-of-6-patients-with-blastic-plasmacytoid-dendritic-cell-neoplasm
#10
X Han, M Q Ouyang, Q Pei, M H Duan, X Y Jiang, D B Zhou
Objective: To explore the clinical characteristics, treatment, and prognosis of patients with blastic plasmacytoid dendritic cell neoplasm. Method: Clinical records of 6 patients diagnosed with blastic plasmacytoid dendritic cell neoplasm in our hospital from January 2008 to May 2016 were collected and retrospectively analyzed. Results: Six patients manifested with initial symptoms of skin lesions, other common symptoms included bone marrow involvement (5/6) , lymphadenectasis (4/6) , splenomegaly (4/6) , and hepatomegaly (3/6) ...
August 14, 2017: Zhonghua Xue Ye Xue za Zhi, Zhonghua Xueyexue Zazhi
https://www.readbyqxmd.com/read/28949028/cns-embryonal-tumours-who-2016-and-beyond
#11
Jessica C Pickles, Cynthia Hawkins, Torsten Pietsch, Thomas S Jacques
Embryonal tumours of the CNS present a significant clinical challenge. Many of these neoplasms affect young children, have a very high mortality and therapeutic strategies are often aggressive with poor long-term outcomes. There is a great need to accurately diagnose embryonal tumours, predict their outcome and adapt therapy to the individual patient's risk. For the first time in 2016, the WHO classification took into account molecular characteristics for the diagnosis of CNS tumours. This integration of histological features with genetic information has significantly changed the diagnostic work-up and reporting of tumours of the CNS...
September 26, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28945661/second-neoplasms-in-children-following-a-treatment-for-acute-leukemia-and-or-lymphoma-29-years-of-experience-in-a-single-institution-in-argentina
#12
María S Felice, Jorge G Rossi, Cristina N Alonso, Patricia Rubio, Marta S Gallego, María L Galluzzo, Fabiana Lubieniecki, Gladys Gutiérrez, Myriam R Guitter, Daniel H Alderete, Adriana B Rose, Walter D Cacciavillano, Viviana Herzovich, Elizabeth M Alfaro, Cristian G Sánchez La Rosa, Natalia Millán, Guillermo L Chantada, Carlos M Figueroa Turienzo, Pedro A Zubizarreta
INTRODUCTION: Childhood acute leukemias (AL) and lymphomas achieve good survival rates. However, second neoplasms (SN) are a devastating event. METHODS: From August 1987 to December 2016, 34 of 3321 (1%) patients with diagnosis of AL or lymphoma developed SN. SN were AL (n=16), CNS tumors (n=5), endocrinal tumors (n=3), lymphomas (n=2), schwannoma (n=2) assorted sarcomas (n=4), retinal melanoma (n=1), and Vanek tumor (n=1). Median latency was 51 (range, 10 to 110) months for hematological malignancies and 119 (range, 25 to 236) months for solid tumors (P=0...
September 22, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28945051/radiologic-characteristics-of-ependymomas-a-case-based-approach
#13
David Khatami, Ekkehard M Kasper, Rafeeque Bhadelia, Rafael Rojas
The purpose of this paper is to interoduce and review the characteristic imaging features of ependymal neoplasms through a comprehensive case-based approach. Illustrated cases highlight both common and uncommon manifestations of CNS-ependymomas. The combination of imaging features, location of the tumor, and patient's demographics often allow the suggestion of ependymoma as a lead entity in the differential diagnosis. However, significant overlap exists between the radiologic characteristics of ependymomas and those of other tumors commonly encountered in the same locations, which can pose a challenge for a definitive diagnosis based on imaging alone...
September 22, 2017: Journal of Neurosurgical Sciences
https://www.readbyqxmd.com/read/28944505/taxonomy-of-cns-tumours-a-series-of-three-short-reviews-on-the-who-2016-classification-and-beyond
#14
EDITORIAL
Pieter Wesseling, Thomas S Jacques
In May 2016, an update of the 4th edition of the World Health Organization (WHO) Classification of Tumours of the Central Nervous System (CNS) was officially released [1]. This revised 4th edition represents a paradigm shift as, for the first time, the definition of multiple CNS tumour entities is now partly based on particular genotypic characteristics. Additionally, compared to the 4th edition (published in 2007), some splitting and lumping of entities occurred because of new insights in the genetic underpinnings of CNS tumours and/or based on more recently published clinico-pathologic studies...
September 25, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28943417/giant-cell-ependymoma-of-the-lateral-ventricle-case-report-literature-review-and-analysis-of-prognostic-factors-and-genetic-profile
#15
Hirokazu Takami, Christopher Graffeo, Avital Perry, Aditya Raghunathan, Robert B Jenkins, Caterina Giannini, Terry C Burns
Giant cell ependymoma (GCE) is a rare primary CNS neoplasm. We report a case of GCE arising in the lateral ventricle. A 22-year-old female presented with generalized seizures. MRI demonstrated a diffuse, non-enhancing, multi-cystic mass centered in the atrium of the right lateral ventricle with extension throughout the frontal and temporal horns. An initial subtotal resection yielded the signature biphasic pattern of GCE. The dominant component contained pleomorphic, bizarre-appearing giant cells with low mitotic index, and a minor component comprised monomorphic, highly cellular, mitotically active cells that formed perivascular pseudorosettes...
September 21, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28936084/primary-central-nervous-system-fibrosarcoma
#16
V P Vinodh, Rahmat Harun, Pulivendhan Sellamuthu, Regunath Kandasamy
We report a rare case of a young female with primary brain fibrosarcoma, and to the best of our knowledge, we believe that only <50 cases have been reported or described worldwide so far. Fibrosarcoma is a malignant neoplasm, in which histologically the predominant cells are fibroblasts that divide excessively without cellular control and they can invade local tissues or metastasize. Primary central nervous system fibrosarcomas are very aggressive neoplasms and generally have a poor prognosis. This tumor is either from sarcomatous transformation of a meningioma or arises de novo within the brain parenchyma...
August 2017: Journal of Neurosciences in Rural Practice
https://www.readbyqxmd.com/read/28912286/association-of-developmental-venous-anomalies-with-demyelinating-lesions-in-patients-with-multiple-sclerosis
#17
D M Rogers, M E Peckham, L M Shah, R H Wiggins
We present 5 cases of demyelination in patients diagnosed with multiple sclerosis that are closely associated with a developmental venous anomaly. Although the presence of a central vein is a known phenomenon with multiple sclerosis plaques, demyelination occurring around developmental venous anomalies is an underreported phenomenon. Tumefactive demyelination can cause a diagnostic dilemma because of its overlapping imaging findings with central nervous system neoplasm. The relationship of a tumefactive plaque with a central vein can be diagnostically useful, and we suggest that if such a lesion is closely associated with a developmental venous anomaly, an inflammatory or demyelinating etiology should be a leading consideration...
September 14, 2017: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/28893601/seek-destroy-use-of-targeting-peptides-for-cancer-detection-and-drug-delivery
#18
Vadim Le Joncour, Pirjo Laakkonen
Accounting for 16 million new cases and 9 million deaths annually, cancer leaves a great number of patients helpless. It is a complex disease and still a major challenge for the scientific and medical communities. The efficacy of conventional chemotherapies is often poor and patients suffer from off-target effects. Each neoplasm exhibits molecular signatures - sometimes in a patient specific manner - that may completely differ from the organ of origin, may be expressed in markedly higher amounts and/or in different location compared to the normal tissue...
September 1, 2017: Bioorganic & Medicinal Chemistry
https://www.readbyqxmd.com/read/28890157/the-cumulative-burden-of-surviving-childhood-cancer-an-initial-report-from-the-st-jude-lifetime-cohort-study-sjlife
#19
Nickhill Bhakta, Qi Liu, Kirsten K Ness, Malek Baassiri, Hesham Eissa, Frederick Yeo, Wassim Chemaitilly, Matthew J Ehrhardt, Johnnie Bass, Michael W Bishop, Kyla Shelton, Lu Lu, Sujuan Huang, Zhenghong Li, Eric Caron, Jennifer Lanctot, Carrie Howell, Timothy Folse, Vijaya Joshi, Daniel M Green, Daniel A Mulrooney, Gregory T Armstrong, Kevin R Krull, Tara M Brinkman, Raja B Khan, Deo K Srivastava, Melissa M Hudson, Yutaka Yasui, Leslie L Robison
BACKGROUND: Survivors of childhood cancer develop early and severe chronic health conditions (CHCs). A quantitative landscape of morbidity of survivors, however, has not been described. We aimed to describe the cumulative burden of curative cancer therapy in a clinically assessed ageing population of long-term survivors of childhood cancer. METHODS: The St Jude Lifetime Cohort Study (SJLIFE) retrospectively collected data on CHCs in all patients treated for childhood cancer at the St Jude Children's Research Hospital who survived 10 years or longer from initial diagnosis and were 18 years or older as of June 30, 2015...
September 7, 2017: Lancet
https://www.readbyqxmd.com/read/28884048/expression-levels-of-%C3%AE-catenin-and-galectin-3-in-meningioma-and-their-effect-on-brain-invasion-and-recurrence-a-tissue-microarray-study
#20
Rehab Allah Ahmed, Abdelhadi M Shebl, Hany Onsy Habashy
OBJECTIVE: Meningiomas are neoplasms that arise from the meninges of the central nervous system (CNS). They constitute about 25.6% of CNS tumors diagnosed in Egypt. Some morphological variants of meningiomas display aggressive behavior, leading to brain-invasive growth pattern. Although meningiomas are usually treated by complete surgical excision, the risk of postoperative recurrence remains. Hence, additional biomarkers for predicting aggressive behavior must be discovered. This study aims to explore the clinical and biological relevance of the protein expression levels of β-catenin and galectine-3 in meningioma and to understand the pathobiology of this neoplasm...
August 2017: Cancer Biology & Medicine
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