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https://www.readbyqxmd.com/read/28527006/analysis-of-surgical-and-mri-factors-associated-with-cerebellar-mutism
#1
Anjali Sergeant, Michelle Masayo Kameda-Smith, Branavan Manoranjan, Brij Karmur, JoAnn Duckworth, Tina Petrelli, Katey Savage, Olufemi Ajani, Blake Yarascavitch, M Constantine Samaan, Katrin Scheinemann, Cheryl Alyman, Saleh Almenawer, Forough Farrokhyar, Adam J Fleming, Sheila Kumari Singh, Nina Stein
The surgical risk factors and neuro-imaging characteristics associated with cerebellar mutism (CM) remain unclear and require further investigation. Therefore, we aimed to examine surgical and MRI findings associated with CM in children following posterior fossa tumor resection. Using our data registry, we retrospectively collected data from pediatric patients who acquired CM and were matched based on age and pathology type with individuals who did not acquire CM after posterior fossa surgery. The strength of association between surgical and MRI variables and CM were examined using odds ratios (ORs) and corresponding 95% confidence intervals (CIs)...
May 19, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28516074/an-updated-nationwide-epidemiology-of-primary-brain-tumors-in-republic-of-korea-2013
#2
Yun-Sik Dho, Kyu-Won Jung, Johyun Ha, Youngbeom Seo, Chul-Kee Park, Young-Joo Won, Heon Yoo
BACKGROUND: This report aims to provide accurate nationwide epidemiologic data on primary brain and central nervous system (CNS) tumors in the Republic of Korea. We updated the data by analyzing primary brain and CNS tumors diagnosed in 2013 using the data from the national cancer incidence database. METHODS: Data on primary brain and CNS tumors diagnosed in 2013 were collected from the Korean Central Cancer Registry. Crude and age-standardized rates were calculated in terms of gender, age, and histological type...
April 2017: Brain Tumor Research and Treatment
https://www.readbyqxmd.com/read/28508928/embryonal-tumors-in-canadian-children-less-than-36-months-of-age-results-from-the-canadian-pediatric-brain-tumor-consortium-cpbtc
#3
E Story, D L Johnston, U Bartels, A S Carret, B Crooks, D D Eisenstat, C Fryer, L Lafay-Cousin, V Larouche, B Wilson, S Zelcer, M Silva, J Brossard, E Bouffet, D L Keene
Embryonal tumors are a heterogeneous group of central nervous system (CNS) tumors whose subgroups have varying incidence and outcome. Despite these differences, they are often grouped as a single entity for study purposes. To date, there are no Canadian multi-institutional studies examining the incidence and outcome of all embryonal subtypes. The current study is an observational study reviewing embryonal tumors in all patients less than 36 months of age diagnosed with a CNS tumor in Canada from 1990 to 2005...
May 16, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28504719/novel-myc-driven-medulloblastoma-models-from-multiple-embryonic-cerebellar-cells
#4
D Kawauchi, R J Ogg, L Liu, D J H Shih, D Finkelstein, B L Murphy, J E Rehg, A Korshunov, C Calabrese, F Zindy, T Phoenix, Y Kawaguchi, J Gronych, R J Gilbertson, P Lichter, A Gajjar, M Kool, P A Northcott, S M Pfister, M F Roussel
Group3 medulloblastoma (MBG3) that predominantly occur in young children are usually associated with MYC amplification and/or overexpression, frequent metastasis and a dismal prognosis. Physiologically relevant MBG3 models are currently lacking, making inferences related to their cellular origin thus far limited. Using in utero electroporation, we here report that MBG3 mouse models can be developed in situ from different multipotent embryonic cerebellar progenitor cells via conditional expression of Myc and loss of Trp53 function in several Cre driver mouse lines...
May 15, 2017: Oncogene
https://www.readbyqxmd.com/read/28498095/risk-factors-for-development-of-postoperative-cerebellar-mutism-syndrome-in-children-after-medulloblastoma-surgery
#5
San Y C V Pols, Marie Lise C van Veelen, Femke K Aarsen, Antonia Gonzalez Candel, Coriene E Catsman-Berrevoets
OBJECTIVE Postoperative cerebellar mutism syndrome (pCMS) occurs in 7%-50% of children after cerebellar tumor surgery. Typical features include a latent onset of 1-2 days after surgery, transient mutism, emotional lability, and a wide variety of motor and neurobehavioral abnormalities. Sequelae of this syndrome usually persist long term. The principal causal factor is bilateral surgical damage (regardless of tumor location) to any component of the proximal efferent cerebellar pathway, which leads to temporary dysfunction of cerebral cortical regions as a result of diaschisis...
May 12, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28469330/profile-of-primary-pediatric-brain-and-spinal-cord-tumors-from-north-india
#6
Nadia Shirazi, Meenu Gupta, Nowneet Kumar Bhat, Braham Prakash Kalra, Ranjit Kumar, Manju Saini
BACKGROUND AND OBJECTIVE: The study was carried out to find the profile of pediatric brain and spinal cord tumors during 2006-2015 in a tertiary referral center of North India. MATERIALS AND METHODS: It was a retrospective medical record-based observational study. All children <18 years of age with confirmed histopathological diagnosis of cancer were included in the study. RESULTS: Central nervous system (CNS) tumors constituted 5.6% of all pediatric solid malignancies in our hospital...
January 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/28445970/acid-ceramidase-is-a-novel-drug-target-for-pediatric-brain-tumors
#7
Ninh B Doan, Ha S Nguyen, Andrew Montoure, Mona M Al-Gizawiy, Wade M Mueller, Shekar Kurpad, Scott D Rand, Jennifer M Connelly, Christopher R Chitambar, Kathleen M Schmainda, Shama P Mirza
Pediatric brain tumors are the most common solid tumors in children and are also a leading culprit of cancer-related fatalities in children. Pediatric brain tumors remain hard to treat. In this study, we demonstrated that medulloblastoma, pediatric glioblastoma, and atypical teratoid rhabdoid tumors express significant levels of acid ceramidase, where levels are highest in the radioresistant tumors, suggesting that acid ceramidase may confer radioresistance. More importantly, we also showed that acid ceramidase inhibitors are highly effective at targeting these pediatric brain tumors with low IC50 values (4...
April 11, 2017: Oncotarget
https://www.readbyqxmd.com/read/28440450/comprehensive-analysis-of-a-microrna-expression-profile-in-pediatric-medulloblastoma
#8
Junqiang Dai, Qiao Li, Zhitong Bing, Yinian Zhang, Liang Niu, Hang Yin, Guoqiang Yuan, Yawen Pan
Medulloblastoma is the most common malignant brain tumor of the central nervous system among children. Medulloblastoma is an embryonal tumor, of which little is known about the pathogenesis. Several efforts have been made to understand the molecular aspects of its tumorigenic pathways; however, these are poorly understood. microRNA (miRNA), a type of non‑coding short RNA, has been proven to be associated with a number of physiological processes and pathological processes of serious diseases, including brain tumors...
June 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28435518/sparc-overexpression-alters-microrna-expression-profiles-involved-in-tumor-progression
#9
Bhavesh K Ahir, Nasya M Elias, Sajani S Lakka
Medulloblastoma is the most common malignant brain tumor in children. SPARC (secreted protein acidic and rich in cysteine), a multicellular non-structural glycoprotein is known to be involved in multiple processes in various cancers. Previously, we reported that SPARC expression significantly impairs medulloblastoma tumor growth in vitro and in vivo and also alters chemo sensitivity. MicroRNAs are a class of post-transcriptional gene regulators with critical functions in tumor progression. In addition, microRNA (miRNA) expression changes are also involved in chemo-resistance...
January 2017: Genes & Cancer
https://www.readbyqxmd.com/read/28432426/posterior-cranial-fossa-tumours-in-children-at-national-cancer-institute-sudan-a-single-institution-experience
#10
Moawia Mohammed Ali Elhassan, Haytham Hussein Mohammed Osman, Jeannette Parkes
BACKGROUND: Posterior cranial fossa tumours (PCF) comprise 54-70% of childhood brain tumours. The clinical profile and outcomes of these tumours differ in area of the world. The aim of this study is to describe clinical characteristics, treatment and outcome of posterior cranial fossa tumours in Sudan. MATERIAL AND METHODS: A retrospective study of children diagnosed with posterior cranial fossa tumours and treated between January 1998 and December 2015 at National Cancer Institute, Sudan...
April 21, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28426747/metabolic-analysis-of-radioresistant-medulloblastoma-stem-like-clones-and-potential-therapeutic-targets
#11
Lue Sun, Takashi Moritake, Kazuya Ito, Yoshitaka Matsumoto, Hironobu Yasui, Hidehiko Nakagawa, Aki Hirayama, Osamu Inanami, Koji Tsuboi
Medulloblastoma is a fatal brain tumor in children, primarily due to the presence of treatment-resistant medulloblastoma stem cells. The energy metabolic pathway is a potential target of cancer therapy because it is often different between cancer cells and normal cells. However, the metabolic properties of medulloblastoma stem cells, and whether specific metabolic pathways are essential for sustaining their stem cell-like phenotype and radioresistance, remain unclear. We have established radioresistant medulloblastoma stem-like clones (rMSLCs) by irradiation of the human medulloblastoma cell line ONS-76...
2017: PloS One
https://www.readbyqxmd.com/read/28416738/differential-expression-of-folate-receptor-1-in-medulloblastoma-and-the-correlation-with-clinicopathological-characters-and-target-therapeutic-potential
#12
Hailong Liu, Qianwen Sun, Mingshan Zhang, Zhihua Zhang, Xinyi Fan, Hongyu Yuan, Cheng Li, Yuduo Guo, Weihai Ning, Youliang Sun, Yongmei Song, Chunjiang Yu
Medulloblastoma is the most common malignant brain tumor in children. Folate receptor 1 (Folr1) was abundantly expressed in some epithelial malignancies. However the expression profile and the role of clinicopathological significance and therapeutic target potential in medulloblastoma still remain elusive. Currently we detected the expression of Folr1 in medulloblastoma and identified the diagnostic application by evaluating the clinical, pathological and neuroimaging values. Then we developed a target therapeutic compound with Folr1, which exhibited promising efficiency in treatment of medulloblastoma...
April 4, 2017: Oncotarget
https://www.readbyqxmd.com/read/28411324/determinants-of-social-competence-in-pediatric-brain-tumor-survivors-who-participated-in-an-intervention-study
#13
Maru Barrera, Eshetu G Atenafu, Fiona Schulte, Ute Bartels, Lillian Sung, Laura Janzen, Joanna Chung, Danielle Cataudella, Kelly Hancock, Amani Saleh, Douglas Strother, Dina McConnell, Andrea Downie, Juliette Hukin, Shayna Zelcer
PURPOSE: This prospective study describes disease/treatment, personal characteristics, and social/family contextual variables as risk and resilience factors that predict social competence in pediatric brain tumor survivors (PBTS). METHODS: Ninety-one PBTS (51% male, mean age 11.21 years, off-treatment, attending a regular classroom >50% of the time) participated. PBTS and their primary caregivers (proxy) completed the Social Skills Rating System (SSRS) to assess social competence at baseline, 2, and 8 months follow-up...
April 14, 2017: Supportive Care in Cancer: Official Journal of the Multinational Association of Supportive Care in Cancer
https://www.readbyqxmd.com/read/28405869/long-term-neuropsychological-follow-up-of-young-children-with-medulloblastoma-treated-with-sequential-high-dose-chemotherapy-and-irradiation-sparing-approach
#14
Taryn B Fay-McClymont, Danielle M Ploetz, Don Mabbott, Karin Walsh, Amy Smith, Susan N Chi, Elizabeth Wells, Jennifer Madden, Ashley Margol, Jonathan Finlay, Mark W Kieran, Douglas Strother, Girish Dhall, Roger J Packer, Nicholas K Foreman, E Bouffet, Lucie Lafay-Cousin
High-dose chemotherapy (HDC) strategies were developed in brain tumor protocols for young children to prevent neuropsychological (NP) impairments associated with radiotherapy. However, comprehensive NP evaluations of these children treated with such strategies remain limited. We examined the long-term neurocognitive outcomes of young children (<6 years) with medulloblastoma, treated similarly, with a HDC strategy "according to" the chemotherapy regimen of the protocol CCG 99703. This retrospective study included young children less than 6 years of age at diagnosis of medulloblastoma treated from 1998 to 2011 at 7 North American institutions...
April 12, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28401018/otx2-expression-contributes-to-proliferation-and-progression-in-myc-amplified-medulloblastoma
#15
REVIEW
Yining Lu, Collin M Labak, Neha Jain, Ian J Purvis, Maheedhara R Guda, Sarah E Bach, Andrew J Tsung, Swapna Asuthkar, Kiran K Velpula
Medulloblastoma is one of the most prevalent pediatric brain malignancies, accounting for approximately 20% of all primary CNS tumors in children under the age of 19. OTX2 is the member of a highly conserved family of bicoid-like homeodomain transcription factors responsible for the regulation of cerebellar development and of current investigational interest in the tumorigenesis of medulloblastoma. Recent studies have revealed that Group 3 and Group 4 medulloblastomas show marked overexpression of OTX2 with a concurrent amplification of the MYC and MYCN oncogenes, respectively, correlating with anaplasticity and unfavorable patient outcomes...
2017: American Journal of Cancer Research
https://www.readbyqxmd.com/read/28398210/out-of-field-doses-from-pediatric-craniospinal-irradiations-using-3d-crt-imrt-helical-tomotherapy-and-electron-based-therapy
#16
Marijke De Saint-Hubert, Dirk Verellen, Kenneth Poels, Wouter Crijns, Federica Magliona, Tom Depuydt, Filip Vanhavere, Lara Struelens
Medulloblastoma treatment involves irradiation of the entire central nervous system, i.e craniospinal irradiation (CSI). This is associated with significant exposure of large volumes of healthy tissue with a growing concern regarding treatment associated side effects. The current study compares out-of-field organ doses in children receiving CSI with three-dimensional-conformal radiotherapy (3D-CRT), intensity modulated radiotherapy (IMRT), helical tomotherapy (HT) and an electron-based technique, including as well radiation doses resulting from imaging performed during treatment...
April 11, 2017: Physics in Medicine and Biology
https://www.readbyqxmd.com/read/28376765/the-germline-variants-in-dna-repair-genes-in-pediatric-medulloblastoma-a-challenge-for-current-therapeutic-strategies
#17
Joanna Trubicka, Tomasz Żemojtel, Jochen Hecht, Katarzyna Falana, Dorota Piekutowska-Abramczuk, Rafał Płoski, Marta Perek-Polnik, Monika Drogosiewicz, Wiesława Grajkowska, Elżbieta Ciara, Elżbieta Moszczyńska, Bożenna Dembowska-Bagińska, Danuta Perek, Krystyna H Chrzanowska, Małgorzata Krajewska-Walasek, Maria Łastowska
BACKGROUND: The defects in DNA repair genes are potentially linked to development and response to therapy in medulloblastoma. Therefore the purpose of this study was to establish the spectrum and frequency of germline variants in selected DNA repair genes and their impact on response to chemotherapy in medulloblastoma patients. METHODS: The following genes were investigated in 102 paediatric patients: MSH2 and RAD50 using targeted gene panel sequencing and NBN variants (p...
April 4, 2017: BMC Cancer
https://www.readbyqxmd.com/read/28363815/proteomic-profiling-of-isogenic-primary-and-metastatic-medulloblastoma-cell-lines-reveals-differential-expression-of-key-metastatic-factors
#18
Shuo Gu, Kai Chen, Minzhi Yin, Zhixiang Wu, Yeming Wu
Medulloblastoma is the most common malignant brain tumor in children. Around 30% of medulloblastoma patients are diagnosed with metastasis, which often results in a poor prognosis. Unfortunately, molecular mechanisms of medulloblastoma metastasis remain largely unknown. In this study, we employed the recently developed deep proteome analysis approach to quantitatively profile the expression of >10,000 proteins from CHLA-01-MED and CHLA-01R-MED isogenic cell lines derived from the primary and metastatic tumor of the same patient diagnosed with a group IV medulloblastoma...
March 28, 2017: Journal of Proteomics
https://www.readbyqxmd.com/read/28333414/magnetic-resonance-imaging-to-diagnose-leptomeningeal-spread-of-medulloblastoma-in-children-decreasing-diagnostic-uncertainty-moving-in-new-directions
#19
Bruno P Soares, Andrea Poretti, Thierry A G M Huisman
No abstract text is available yet for this article.
March 23, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28329640/diagnostic-parameters-of-children-with-basal-cell-carcinoma-nevus-syndrome
#20
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
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