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Medulloblastoma

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https://www.readbyqxmd.com/read/28329683/inactivation-of-ezh2-upregulates-gfi1-and-drives-aggressive-myc-driven-group-3-medulloblastoma
#1
BaoHan T Vo, Chunliang Li, Marc A Morgan, Ilan Theurillat, David Finkelstein, Shaela Wright, Judith Hyle, Stephanie M C Smith, Yiping Fan, Yong-Dong Wang, Gang Wu, Brent A Orr, Paul A Northcott, Ali Shilatifard, Charles J Sherr, Martine F Roussel
The most aggressive of four medulloblastoma (MB) subgroups are cMyc-driven group 3 (G3) tumors, some of which overexpress EZH2, the histone H3K27 mono-, di-, and trimethylase of polycomb-repressive complex 2. Ezh2 has a context-dependent role in different cancers as an oncogene or tumor suppressor and retards tumor progression in a mouse model of G3 MB. Engineered deletions of Ezh2 in G3 MBs by gene editing nucleases accelerated tumorigenesis, whereas Ezh2 re-expression reversed attendant histone modifications and slowed tumor progression...
March 21, 2017: Cell Reports
https://www.readbyqxmd.com/read/28329640/diagnostic-parameters-of-children-with-basal-cell-carcinoma-nevus-syndrome
#2
Fariha T Siddiqui
Basal Cell Carcinoma Nevus Syndrome (BCCNS) is a multi-system genetic disease characterized by the development of multiple basal cell carcinomas (BCCs), macrocephaly, medulloblastomas, jaw keratocysts, and coarse facial features, amongst other symptoms. The major and minor criteria for adults with BCCNS are often extrapolated for children, however, little is known about the disease presentation of children with BCCNS. Our study focused on bringing the pediatric presentation of BCCNS to light. To the best of our knowledge, we are the first to investigate BCCNS and its medical impact on children...
September 15, 2016: Dermatology Online Journal
https://www.readbyqxmd.com/read/28328116/brain-morphology-in-children-with-nevoid-basal-cell-carcinoma-syndrome
#3
Tadashi Shiohama, Katsunori Fujii, Toshiyuki Miyashita, Hiromi Mizuochi, Hideki Uchikawa, Naoki Shimojo
Brain morphology is tightly regulated by diverse signaling pathways. Hedgehog signaling is a candidate pathway considered responsible for regulating brain morphology. Nevoid basal cell carcinoma syndrome (NBCCS), caused by a PTCH1 mutation in the hedgehog signaling pathway, occasionally exhibits macrocephaly and medulloblastoma. Although cerebellar enlargement occurs in ptch1 heterozygous-deficient mice, its impact on human brain development remains unknown. We investigated the brain morphological characteristics of children with NBCCS...
April 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/28326824/osteopontin-its-potential-role-in-cancer-of-children-and-young-adults
#4
Gabrielle Karpinsky, Aleksandra Fatyga, Malgorzata Anna Krawczyk, Madeleine Chamera, Natalia Sande, Dagmara Szmyd, Ewa Izycka-Swieszewska, Ewa Bien
OBJECTIVE: Osteopontin (OPN) is aglyco-phosphoprotein, involved in tissue remodeling, inflammation and boneresorption. In various adult neoplasms OPN was shown to correlate with cancer progression, invasiveness and metastasis. AIM: to define the role of OPN in malignancies of children and young adults. MATERIAL AND METHODS: a structured PubMed and Google Scholar literature analysis based on reports published in English between I'1995 and XII'2015...
March 14, 2017: Biomarkers in Medicine
https://www.readbyqxmd.com/read/28325133/outcome-for-children-treated-for-medulloblastoma-and-supratentorial-primitive-neuroectodermal-tumor-cns-pnet-a-retrospective-analysis-spanning-40-years-of-treatment
#5
Einar Stensvold, Bård Kronen Krossnes, Tryggve Lundar, Bernt J Due-Tønnessen, Radek Frič, Paulina Due-Tønnessen, Anne Grete Bechensteen, Tor Åge Myklebust, Tom Børge Johannesen, Petter Brandal
BACKGROUND: Medulloblastoma (MB) and supratentorial primitive neuroectodermal tumor of the central nervous system (CNS-PNET) are among the most common pediatric brain tumors. The diagnosis, treatment, and outcome of MB/CNS-PNET patients treated during the last four decades at Oslo University Hospital (OUH) are described. MATERIAL AND METHODS: All patients younger than 20 years of age diagnosed and treated for MB/CNS-PNET at OUH between 1 January 1974 and 31 December 2013 were identified...
March 21, 2017: Acta Oncologica
https://www.readbyqxmd.com/read/28318880/highly-conformal-craniospinal-radiotherapy-techniques-can-underdose-the-cranial-clinical-target-volume-if-leptomeningeal-extension-through-skull-base-exit-foramina-is-not-contoured
#6
D J Noble, T Ajithkumar, J Lambert, I Gleeson, M V Williams, S J Jefferies
AIMS: Craniospinal irradiation (CSI) remains a crucial treatment for patients with medulloblastoma. There is uncertainty about how to manage meningeal surfaces and cerebrospinal fluid (CSF) that follows cranial nerves exiting skull base foramina. The purpose of this study was to assess plan quality and dose coverage of posterior cranial fossa foramina with both photon and proton therapy. MATERIALS AND METHODS: We analysed the radiotherapy plans of seven patients treated with CSI for medulloblastoma and primitive neuro-ectodermal tumours and three with ependymoma (total n = 10)...
March 15, 2017: Clinical Oncology: a Journal of the Royal College of Radiologists
https://www.readbyqxmd.com/read/28303061/polymorphisms-of-insulin-receptor-substrate-2-are-putative-biomarkers-for-pediatric-medulloblastoma-considering-the-genetic-susceptibility-and-pathological-diagnoses
#7
Wang Baocheng, Yang Zhao, Wei Meng, Yipeng Han, Jiajia Wang, Feili Liu, Shengying Qin, Jie Ma
Molecular profiling subgrouped medulloblastoma (MB) into four subtypes featured by distinct footprints. However, germline studies on genetic susceptibility in Chinese population have not been reported. To investigate the correlation of polymorphisms involved in the AKT signaling pathway with clinicopathological parameters in pediatric MB, and their contribution to the clinical outcome, we performed a case-controlled cohort consisting of 48 patients with pediatric MB and 190 healthy controls from Han population...
February 2017: Nagoya Journal of Medical Science
https://www.readbyqxmd.com/read/28302031/hif1a-is-overexpressed-in-medulloblastoma-and-its-inhibition-reduces-proliferation-and-increases-epas1-and-atg16l1-methylation
#8
Gustavo Alencastro Veiga Cruzeiro, Maristella Bergamo Dos Reis, Vanessa Silva Silveira, Régia Caroline Peixoto Lira, Carlos Gilberto Carlotti, Luciano Neder, Ricardo Santos Oliveira, José Andres Yunes, Silvia Regina Brandalise, Simone Aguiar, Agda Karina Eterovic, Luiz Gonzaga Tone, Carlos Alberto Scrideli, Elvis Terci Valera
BACKGROUND: Genetic and epigenetic modifications are closely related to tumor initiation and progression and can provide guidance for understanding tumor functioning, potentially leading to the discovery of new therapies. Studies have associated hypoxia-related genes to tumor progression and chemo/radioresistance in brain tumors. Information on the expression profile of hypoxia-related genes in pediatric medulloblastoma, although scarce, may reveal relevant information that could support treatment decisions...
March 15, 2017: Current Cancer Drug Targets
https://www.readbyqxmd.com/read/28298418/disrupting-the-cd47-sirp%C3%AE-anti-phagocytic-axis-by-a-humanized-anti-cd47-antibody-is-an-efficacious-treatment-for-malignant-pediatric-brain-tumors
#9
Sharareh Gholamin, Siddhartha S Mitra, Abdullah H Feroze, Jie Liu, Suzana A Kahn, Michael Zhang, Rogelio Esparza, Chase Richard, Vijay Ramaswamy, Marc Remke, Anne K Volkmer, Stephen Willingham, Anitha Ponnuswami, Aaron McCarty, Patricia Lovelace, Theresa A Storm, Simone Schubert, Gregor Hutter, Cyndhavi Narayanan, Pauline Chu, Eric H Raabe, Griffith Harsh, Michael D Taylor, Michelle Monje, Yoon-Jae Cho, Ravi Majeti, Jens P Volkmer, Paul G Fisher, Gerald Grant, Gary K Steinberg, Hannes Vogel, Michael Edwards, Irving L Weissman, Samuel H Cheshier
Morbidity and mortality associated with pediatric malignant primary brain tumors remain high in the absence of effective therapies. Macrophage-mediated phagocytosis of tumor cells via blockade of the anti-phagocytic CD47-SIRPα interaction using anti-CD47 antibodies has shown promise in preclinical xenografts of various human malignancies. We demonstrate the effect of a humanized anti-CD47 antibody, Hu5F9-G4, on five aggressive and etiologically distinct pediatric brain tumors: group 3 medulloblastoma (primary and metastatic), atypical teratoid rhabdoid tumor, primitive neuroectodermal tumor, pediatric glioblastoma, and diffuse intrinsic pontine glioma...
March 15, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28297703/molecular-basis-of-distant-medulloblastoma-recurrence
#10
Rafael Roesler, Caroline Brunetto de Farias, Gustavo R Isolan
No abstract text is available yet for this article.
March 16, 2017: Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28296680/primitive-neuroectodermal-tumors-of-the-female-genital-tract-a-morphologic-immunohistochemical-and-molecular-study-of-19-cases
#11
Sarah Chiang, Matija Snuderl, Sakiko Kojiro-Sanada, Ariadna Quer Pi-Sunyer, Dean Daya, Tohru Hayashi, Luisanna Bosincu, Fumihiro Ogawa, Andrew E Rosenberg, Lars-Christian Horn, Lu Wang, A John Iafrate, Esther Oliva
Primary primitive neuroectodermal tumor (PNET) of the female genital tract is rare, and its proper classification remains unclear. The clinical, histologic, and immunophenotypic features as well as EWSR1 rearrangement status of 19 gynecologic PNETs, including 10 ovarian, 8 uterine, and 1 vulvar tumors, are herein reported. Patient age ranged from 12 to 68 years, with a median age of 20 and 51 years among those with ovarian and uterine PNETs, respectively. Morphologic features of central nervous system (CNS) tumors were seen in 15 PNETs, including 9 medulloblastomas, 3 ependymomas, 2 medulloepitheliomas, and 1 glioblastoma, consistent with central PNET...
March 14, 2017: American Journal of Surgical Pathology
https://www.readbyqxmd.com/read/28293736/5ala-in-pediatric-brain-tumors-is-not-routinely-beneficial
#12
Jonathan Roth, Shlomi Constantini
PURPOSE: Over recent years, 5-aminoluvolinic acid (5ALA) has been increasingly used for resection guidance in adult high-grade gliomas. However, amongst pediatric patients, publication of intraoperative fluorescence has been limited, with inconsistent outcomes. We describe our experience and intraoperative finding amongst children with various brain tumors that were given 5ALA prior to tumor resection. METHODS: Since October 2014, data regarding intraoperative findings amongst children that received 5ALA prior to tumor resection were prospectively collected...
March 14, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28292958/molecular-mechanisms-and-therapeutic-targets-in-pediatric-brain-tumors
#13
REVIEW
Kun-Wei Liu, Kristian W Pajtler, Barbara C Worst, Stefan M Pfister, Robert J Wechsler-Reya
Brain tumors are among the leading causes of cancer-related deaths in children. Although surgery, aggressive radiation, and chemotherapy have improved outcomes, many patients still die of their disease. Moreover, those who survive often suffer devastating long-term side effects from the therapies. A greater understanding of the molecular underpinnings of these diseases will drive the development of new therapeutic approaches. Advances in genomics and epigenomics have provided unprecedented insight into the molecular diversity of these diseases and, in several cases, have revealed key genes and signaling pathways that drive tumor growth...
March 14, 2017: Science Signaling
https://www.readbyqxmd.com/read/28290395/indian-society-of-neuro-oncology-consensus-guidelines-for-the-contemporary-management-of-medulloblastoma
#14
Tejpal Gupta, Chitra Sarkar, Vedantam Rajshekhar, Sandip Chatterjee, Neelam Shirsat, Dattatreya Muzumdar, Sona Pungavkar, Girish Chinnaswamy, Rakesh Jalali
INTRODUCTION: The high success rate in the management medulloblastoma achieved in the western world is not exactly mirrored in developing countries including India. Socio-demographic differences, health-care disparity, and lack in uniformity of care with resultant widespread variations in the clinical practice are some of the reasons that may partly explain this difference in outcomes. Patients with medulloblastoma require a multi-disciplinary team approach involving but not limited to neuro-radiology, neurosurgery; neuropathology, molecular biology, radiation oncology, pediatric medical oncology and rehabilitative services for optimizing outcomes...
March 2017: Neurology India
https://www.readbyqxmd.com/read/28290003/demographics-patterns-of-care-and-survival-in-pediatric-medulloblastoma
#15
Emily V Dressler, Therese A Dolecek, Meng Liu, John L Villano
We evaluated the American College of Surgeon's National Cancer Data Base (NCDB) to describe current hospital-based epidemiologic frequency, survival, and patterns of care of pediatric medulloblastoma. We analyzed NCDB 1998-2011 data on medulloblastoma for children ages 0-19 years using logistic and poisson regression, Kaplan-Meier survival estimates, and Cox proportional hazards models. 3647 cases of medulloblastoma in those aged 0-19 years were identified. Chemotherapy was received by 79 and 74% received radiation, with 65% receiving both therapies...
March 13, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28283778/plk1-associated-micrornas-are-correlated-with-pediatric-medulloblastoma-prognosis
#16
Julia Alejandra Pezuk, María Sol Brassesco, Ricardo Santos de Oliveira, Hélio Rubens Machado, Luciano Neder, Carlos Alberto Scrideli, Luiz Gonzaga Tone
PURPOSE: Medulloblastoma (MB) is the most common malignant tumor of the central nervous system (CNS) in children. Despite its relative good survival rates, treatment can cause long time sequels and may impair patients' lifespan and quality, making the search for new treatment options still necessary. Polo like kinases (PLKs) constitute a five-member serine/threonine kinases family (PLK 1-5) that regulates different stages during cell cycle. Abnormal PLKs expression has been observed in several cancer types, including MB...
March 10, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28276480/selective-targeting-of-hdac1-2-elicits-anticancer-effects-through-gli1-acetylation-in-preclinical-models-of-shh-medulloblastoma
#17
Sonia Coni, Anna Barbara Mancuso, Laura Di Magno, Giulia Sdruscia, Simona Manni, Silvia Maria Serrao, Dante Rotili, Eleonora Spiombi, Francesca Bufalieri, Marialaura Petroni, Monika Kusio-Kobialka, Enrico De Smaele, Elisabetta Ferretti, Carlo Capalbo, Antonello Mai, Pawel Niewiadomski, Isabella Screpanti, Lucia Di Marcotullio, Gianluca Canettieri
SHH Medulloblastoma (SHH-MB) is a pediatric brain tumor characterized by an inappropriate activation of the developmental Hedgehog (Hh) signaling. SHH-MB patients treated with the FDA-approved vismodegib, an Hh inhibitor that targets the transmembrane activator Smoothened (Smo), have shown the rapid development of drug resistance and tumor relapse due to novel Smo mutations. Moreover, a subset of patients did not respond to vismodegib because mutations were localized downstream of Smo. Thus, targeting downstream Hh components is now considered a preferable approach...
March 9, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28270075/comparative-effects-of-polo-like-kinase-1-inhibitors-as-monotherapy-and-in-combination-with-current-treatments-for-medulloblastoma
#18
Julia Alejandra Pezuk, Maria Sol Brassesco, Priscila Maria Manzini Ramos, Carlos Alberto Scrideli, Luiz Gonzaga Tone
BACKGROUND: Medulloblastoma (MB) is one of most frequent malignant tumors that affects children. Despite relative good survival rates, long time sequels are still significant and represent a challenge for treatment of MB patients. Therefore, in an attempt to reduce treatment aftereffects new therapeutic targets are constantly being explored. Polo like kinase 1 (PLK1) is a master cell cycle regulator that has been reported increased in proliferative cells, while its depletion has been repeatedly proposed as an oncological therapeutic strategy...
February 13, 2017: Anti-cancer Agents in Medicinal Chemistry
https://www.readbyqxmd.com/read/28267079/medulloblastoma-with-extensive-nodularity-tailored-therapy-in-a-low-resource-setting
#19
Paula Aristizabal, Luke Burns, Rebeca Rivera-Gomez, Michael L Levy, William Roberts, John R Crawford
Treatment for medulloblastoma carries significant risks, particularly in resource-constrained settings. We report a case of a Mexican infant with desmoplastic/nodular medulloblastoma. Given the nature of her tumor, we developed a tailored regimen following subtotal resection to avoid both radiation therapy and the high-dose cisplatin therapy offered at most centers in the United States. The patient is in remission 4 years after the initial diagnosis. This case suggests an alternative treatment plan for this particular tumor variant that accommodates the limited resources of many centers around the world and avoids the risks associated with radiation therapy at a young age...
March 6, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28263211/extraneural-metastases-from-desmoplastic-medulloblastoma-masquerading-as-lymphoma
#20
Abhishek Mahajan, Peter Paul, Epari Sridhar, Venkatesh Rangarajan, Tejpal Gupta, Girish Chinnaswamy, Aliasgar Moiyadi, Rakesh Jalali
Medulloblastoma is a WHO grade IV neoplasm that is usually restricted to the central nervous system, with metastasis occurring along the neuraxis through the ventricular system and cerebrospinal fluid. Extraneural metastasis is rare, usually occurs in bones/lymph nodes, and carries poor prognosis. We report multimodal imaging in a 14-year-old boy with desmoplastic medulloblastoma having multiple extraneural nodal and skeletal metastases, which was misdiagnosed as lymphoma. This case highlights the importance of understanding tumor biology with active clinical and radiological surveillance that is useful in early detection of extraneural metastasis of this aggressive tumor that helps in advocating appropriate and timely treatment...
March 3, 2017: Clinical Nuclear Medicine
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