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https://www.readbyqxmd.com/read/28474160/-hereditary-tumor-syndromes-in-neuropathology
#1
REVIEW
C Mawrin
Neoplasms in the central (CNS) and peripheral nervous system (PNS) in hereditary tumor syndromes play an important role in the neuropathological diagnostics. The benign and malignant PNS and CNS tumors that occur in the frequent neurofibromatosis type 1 (NF1) and type 2 (NF2) often represent essential factors for the course of the disease in those affected. Furthermore, certain clinical constellations (e.g. bilateral schwannomas of the auditory nerve, schwannomas at a young age and multiple meningiomas) can be important indications for a previously undiagnosed hereditary tumor disease...
May 4, 2017: Der Pathologe
https://www.readbyqxmd.com/read/28464318/examination-of-phox2b-in-adult-neuroendocrine-neoplasms-reveals-relatively-frequent-expression-in-pheochromocytomas-and-paragangliomas
#2
John P Lee, Yin P Hung, Thomas M O'Dorisio, James R Howe, Jason L Hornick, Andrew M Bellizzi
AIMS: Paired-like homeobox 2b (PHOX2B) is a transcription factor with expression outside of the central nervous system restricted to neurons and chromaffin cells of the autonomic nervous system. Germline mutations cause congenital central hypoventilation syndrome and predispose to neuroblastoma and Hirschsprung disease. Among pediatric small round cell tumors, PHOX2B is neuroblastoma-specific. Two studies of adult autonomic nervous system tumors (n=62) produced conflicting results (all tumors stained in one; expression was restricted to 40% of paragangliomas in the other)...
May 2, 2017: Histopathology
https://www.readbyqxmd.com/read/28462555/neoplastic-fever-all-who-shiver-are-not-infected
#3
Yogesh B Sharma, Aparna Vishal More
We report the case of previously healthy 14 years old male who presented high grade fever and headache. There was a history of convulsion at age of 7 years, so MRI Brain was done. It was suggestive of a central nervous system neoplasm. Our patient had only two days of fever which is an unusual presentation of a neoplasm. The paper should be of interest to the clinicians as neoplastic fever as cause of acute febrile illness is considered as a remote possibility.
March 2017: Journal of the Association of Physicians of India
https://www.readbyqxmd.com/read/28454254/oculomotor-nerve-palsy-as-a-preceding-symptom-of-adult-sporadic-burkitt-lymphoma-a-case-report-and-review-of-the-literature
#4
Yun Liang, Luyin Ding, Xian Li, Weiqin Wang, Xiaohong Zhang
Adult sporadic Burkitt lymphoma (BL) is a rare aggressive neoplasm and represents ~1-5% of all lymphomas diagnosed in adults. The disease exhibits an aggressive clinical manifestation, which frequently involves the central nervous system (CNS) in the early stages, and is usually accompanied by abnormalities in cerebrospinal fluid (CSF) examinations and/or neuroradiography. The current study describes the rare case of a 29-year-old man who presented with oculomotor nerve palsy without palpated masses, B symptoms, peripheral blood anomaly and abnormalities of the nervous system...
March 2017: Oncology Letters
https://www.readbyqxmd.com/read/28453743/targeted-next-generation-sequencing-of-pediatric-neuro-oncology-patients-improves-diagnosis-identifies-pathogenic-germline-mutations-and-directs-targeted-therapy
#5
Cassie N Kline, Nancy M Joseph, James P Grenert, Jessica van Ziffle, Eric Talevich, Courtney Onodera, Mariam Aboian, Soonmee Cha, David R Raleigh, Steve Braunstein, Joseph Torkildson, David Samuel, Michelle Bloomer, Alejandra G de Alba Campomanes, Anuradha Banerjee, Nicholas Butowski, Corey Raffel, Tarik Tihan, Andrew W Bollen, Joanna J Phillips, W Michael Korn, Iwei Yeh, Boris C Bastian, Nalin Gupta, Sabine Mueller, Arie Perry, Theodore Nicolaides, David A Solomon
Background.: Molecular profiling is revolutionizing cancer diagnostics and leading to personalized therapeutic approaches. Herein we describe our clinical experience performing targeted sequencing for 31 pediatric neuro-oncology patients. Methods.: We sequenced 510 cancer-associated genes from tumor and peripheral blood to identify germline and somatic mutations, structural variants, and copy number changes. Results.: Genomic profiling was performed on 31 patients with tumors including 11 high-grade gliomas, 8 medulloblastomas, 6 low-grade gliomas, 1 embryonal tumor with multilayered rosettes, 1 pineoblastoma, 1 uveal ganglioneuroma, 1 choroid plexus carcinoma, 1 chordoma, and 1 high-grade neuroepithelial tumor...
May 1, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/28440987/-tumors-of-the-central-nervous-system
#6
Marco Antonio Alegría-Loyola, Javier Andrés Galnares-Olalde, Moisés Mercado
Central nervous system (CNS) tumors constitute a heterogeneous group of neoplasms that share a considerable morbidity and mortality rate. Recent advances in the underlying oncogenic mechanisms of these tumors have led to new classification systems, which, in turn, allow for a better diagnostic approach and therapeutic planning. Most of these neoplasms occur sporadically and several risk factors have been found to be associated with their development, such as exposure to ionizing radiation or electromagnetic fields and the concomitant presence of conditions like diabetes, hypertension and Parkinson's disease...
May 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28440461/the-role-of-mir-451-in-the-switching-between-proliferation-and-migration-in-malignant-glioma-cells-ampk-signaling-mtor-modulation-and-rac1-activation-required
#7
Kai Zhao, Leilei Wang, Tao Li, Meng Zhu, Chen Zhang, Lei Chen, Pengfei Zhao, Hua Zhou, Shengping Yu, Xuejun Yang
Glioblastoma multiforme (GBM), WHO grade IV astrocytoma, is the most common primary neoplasm of the central nervous system (CNS) and has the highest malignancy and mortality rates. The invasive nature of GBM complicates surgical resection and restricts chemotherapeutic access, contributing to poor patient prognosis. The migration of tumor cells is closely related to the tumor cell proliferation. The acquisition of migratory capability, in addition to intracellular factors, is proposed to be a crucial mechanism during the progression of invasion...
April 21, 2017: International Journal of Oncology
https://www.readbyqxmd.com/read/28426850/second-primary-malignant-neoplasms-and-survival-in-adolescent-and-young-adult-cancer-survivors
#8
Theresa H M Keegan, Archie Bleyer, Aaron S Rosenberg, Qian Li, Melanie Goldfarb
Importance: Although the increased incidence of second primary malignant neoplasms (SPMs) is a well-known late effect after cancer, few studies have compared survival after an SPM to survival of the same cancer occurring as first primary malignant neoplasm (PM) by age. Objective: To assess the survival impact of SPMs in adolescents and young adults (AYAs) (15-39 years) compared with that of pediatric (<15 years) and older adult (≥40 years) patients with the same SPMs...
April 20, 2017: JAMA Oncology
https://www.readbyqxmd.com/read/28422607/delusional-disorder-arising-from-a-cns-neoplasm
#9
John Stupinski, Jihye Kim, Dimitry Francois
Erotomania arising from a central nervous system (CNS) neoplasm has not been previously described. Here, we present the first known case, to our knowledge, of erotomania with associated persecutory delusions arising following diagnosis and treatment of a left frontal lobe brain tumor.
2017: Journal of Neuropsychiatry and Clinical Neurosciences
https://www.readbyqxmd.com/read/28413547/supratentorial-intermediate-grade-meningeal-melanocytoma-with-intratumoral-bleed-in-the-background-of-neurocutaneous-melanosis-report-of-an-unusual-case-and-review-of-literature
#10
Kuntal Das, Anup Nair, Sushila Jaiswal, Rabi Sahu, Arun Srivastava, Raj Kumar, Anant Mehrotra
Primary melanocytic tumours of the central nervous system (CNS) are rare. According to the WHO classification (2007), these tumours include diffuse leptomeningeal melanosis, melanomatosis, melanocytoma, and primary CNS melanoma. Meningeal melanocytoma, most commonly seen in the infratentorial compartment and cervical spinal cord, is a benign primary melanocytic neoplasm. Primary CNS melanoma, on the other hand, represents the malignant end of the spectrum. Intermediate grade melanocytoma is a rare histological subtype of primary meningeal tumours and is characterised by the clinicopathological features between the two extremes...
January 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28410997/international-incidence-of-childhood-cancer-2001-10-a-population-based-registry-study
#11
Eva Steliarova-Foucher, Murielle Colombet, Lynn A G Ries, Florencia Moreno, Anastasia Dolya, Freddie Bray, Peter Hesseling, Hee Young Shin, Charles A Stiller
BACKGROUND: Cancer is a major cause of death in children worldwide, and the recorded incidence tends to increase with time. Internationally comparable data on childhood cancer incidence in the past two decades are scarce. This study aimed to provide internationally comparable local data on the incidence of childhood cancer to promote research of causes and implementation of childhood cancer control. METHODS: This population-based registry study, devised by the International Agency for Research on Cancer in collaboration with the International Association of Cancer Registries, collected data on all malignancies and non-malignant neoplasms of the CNS diagnosed before age 20 years in populations covered by high-quality cancer registries with complete data for 2001-10...
April 11, 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28401439/primary-extracranial-meningioma-a-rare-location
#12
Inara Carneiro Costa Rege, Robson Rodrigues Garcia, Elismauro Francisco Mendonça
Meningiomas are benign extraaxial tumors of the central nervous system (CNS). Extracranial meningiomas are extremely rare (2%) and can develop as a direct extension from a primary intracranial meningioma or as true primary extracranial meningioma originating from ectopic arachnoid cells. Only eight cases of primary meningioma in the jaw have been reported to date. Extracranial meningiomas are frequently misdiagnosed, resulting in inappropriate clinical management. The aim of this article was to describe the case of a man with an asymptomatic swelling in the right retromolar area over a period of 2 months...
April 11, 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28401334/h3-idh-wild-type-pediatric-glioblastoma-is-comprised-of-molecularly-and-prognostically-distinct-subtypes-with-associated-oncogenic-drivers
#13
Andrey Korshunov, Daniel Schrimpf, Marina Ryzhova, Dominik Sturm, Lukas Chavez, Volker Hovestadt, Tanvi Sharma, Antje Habel, Anna Burford, Chris Jones, Olga Zheludkova, Ella Kumirova, Christof M Kramm, Andrey Golanov, David Capper, Andreas von Deimling, Stefan M Pfister, David T W Jones
Pediatric glioblastoma (pedGBM) is an extremely aggressive pediatric brain tumor, accounting for ~6% of all central nervous system neoplasms in children. Approximately half of pedGBM harbor recurrent somatic mutations in histone 3 variants or, infrequently, IDH1/2. The remaining subset of pedGBM is highly heterogeneous, and displays a variety of genomic and epigenetic features. In the current study, we aimed to further stratify an H3-/IDH-wild type (wt) pedGBM cohort assessed through genome-wide molecular profiling...
April 11, 2017: Acta Neuropathologica
https://www.readbyqxmd.com/read/28393977/-blastic-plasmacytoid-dendritic-cell-neoplasm-with-complete-clinical-remission-with-chemotherapy-and-central-nervous-system-relapse-report-of-one-case
#14
Loreto Contreras, Loreto Mercado, Carolina Delgado, Claudia Cabezas, Laksmi Starke, Mónica Romero, Fernando Ibieta, Mauricio Henríquez, Mauricio Chandia
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare, clinically aggressive hematologic malignancy that most commonly manifests as cutaneous lesions with or without bone marrow involvement and leukemic dissemination. The demonstration of tumor cells with the characteristic immunophenotype with expression of CD56, generally CD4 and dendritic cell antigens (CD123, cyTCL-1, HLA-DR), in the absence of myeloid or lymphoid lineage markers is required for the diagnosis. Responses to chemotherapy are initially satisfactory, with frequent systemic and central nervous system relapses...
January 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/28379644/radiotherapy-after-high-dose-chemotherapy-with-autologous-hematopoietic-cell-rescue-quality-assessment-of-head-start-iii
#15
Kenneth K Wong, Sean All, Jonathan Waxer, Arthur J Olch, Rajkumar Venkatramani, Girish Dhall, Tom Belle Davidson, Wafik Zaky, Jonathan L Finlay
BACKGROUND: The use of high-dose chemotherapy with autologous hematopoietic cell rescue (AuHCR) in Head Start III is a potentially curative approach for the management of young children with central nervous system neoplasms. We report the potential influence of quality and timing of radiation therapy on the survival of patients treated on the study. PROCEDURE: Between 2003 and 2009, 220 children with newly diagnosed central nervous system neoplasms were enrolled on the study...
April 5, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28379624/surgical-removal-of-a-choroid-plexus-oncocytoma-in-an-adult-cat
#16
B Cossic, G Silver, M Kent, E N Glass, D Agnew, S McDonough, A D Miller
An 11-year-old male castrated domestic shorthair cat presented with left central vestibular dysfunction. Magnetic resonance imaging of the brain revealed a large, extra-parenchymal, strongly contrast-enhancing mass at the level of the left cerebellopontine angle and compressing the cerebellum and brainstem. The mass was surgically excised via left rostral and sub-tentorial craniectomies and histopathology revealed an epithelial neoplasm composed of anastomosing cords of neoplastic cells that contained large amounts of finely granular hypereosinophilic cytoplasm and round nuclei...
April 5, 2017: Journal of Small Animal Practice
https://www.readbyqxmd.com/read/28362886/concomitant-primary-cns-lymphoma-and-fsh-pituitary-adenoma-arising-within-the-sella-entirely-coincidental
#17
Vin Shen Ban, Bedansh Roy Chaudhary, Kieren Allinson, Thomas Santarius, Ramez Wadie Kirollos
BACKGROUND AND IMPORTANCE: Collision tumors are the simultaneous occurrence of more than one type of neoplasm within an anatomic space. In the pituitary sella, collision tumors are exceedingly rare, and not much is known about their etiology and prognosis. CLINICAL PRESENTATION: A 74-year-old man presented with a concomitant primary pituitary lymphoma (diffuse large B-cell non-Hodgkin's lymphoma; DLBCL) and follicle-stimulating hormone (FSH)-adenoma diagnosed histologically after clinical features of apoplexy prompted urgent surgical decompression and resection...
January 1, 2017: Neurosurgery
https://www.readbyqxmd.com/read/28362182/pediatric-spinal-cord-astrocytomas-a-retrospective-study-of-348-patients-from-the-seer-database
#18
Andrew S Luksik, Tomas Garzon-Muvdi, Wuyang Yang, Judy Huang, George I Jallo
OBJECTIVE Intramedullary spinal cord tumors comprise 1%-10% of all childhood central nervous system neoplasms, with astrocytomas representing the most common subtype. Due to their rarity and poor prognosis, large population-based studies are needed to assess the epidemiology and survival risk factors associated with these tumors in the hope of improving outcome. The authors undertook this retrospective study to explore factors that may influence survival in pediatric patients with spinal cord astrocytomas. METHODS Utilizing the Surveillance, Epidemiology, and End Results (SEER) database, a prospective cancer registry, the authors retrospectively assessed survival in histologically confirmed, primary spinal cord astrocytomas in patients 21 years of age and younger...
March 31, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28355406/syndrome-of-inappropriate-antidiuretic-hormone-secretion-induced-by-the-phytotherapy-harpagophytum-procumbers-case-report
#19
Renata Reis Carvalho, Camila Dermínio Donadel, Arthur Fernandes Cortez, Vitor Ribeiro Gomes de Almeida Valviesse, Pedro Felipe de Almeida Vianna, Bruno Bordallo Corrêa
INTRODUCTION: The syndrome of inappropriate antidiuretic hormone secretion (SIADH) is the inability of antidiuretic hormone (ADH) suppression, compromising the mechanisms of water excretion and urinary concentration. It manifests as hyponatremia and its symptoms, especially neurological. There are many causes that trigger such disease, notably: central nervous system disorders, malignant neoplasm, drugs and others. CASE REPORT: A 65 years female hypertensive patient presented clinical and laboratory manifestations of hyponatremia due to SIADH...
March 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/28350475/occupational-radiation-exposure-and-deaths-from-malignant-intracranial-neoplasms-of-the-brain-and-cns-in-u-s-radiologic-technologists-1983-2012
#20
Cari M Kitahara, Martha S Linet, Stephen Balter, Donald L Miller, Preetha Rajaraman, Elizabeth K Cahoon, Raquel Velazquez-Kronen, Steven L Simon, Mark P Little, Michele M Doody, Bruce H Alexander, Dale L Preston
OBJECTIVE: Childhood exposure to acute, high-dose radiation has consistently been associated with risk of benign and malignant intracranial tumors of the brain and CNS, but data on risks of adulthood exposure to protracted, low-to-moderate doses of radiation are limited. In a large cohort of radiologic technologists, we quantified the association between protracted, low-to-moderate doses of radiation and malignant intracranial tumor mortality. MATERIALS AND METHODS: The study population included 83,655 female and 26,642 male U...
March 28, 2017: AJR. American Journal of Roentgenology
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