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medulloblastoma children

Sara Bolin, Anna Borgenvik, Camilla U Persson, Anders Sundström, Jun Qi, James E Bradner, William A Weiss, Yoon-Jae Cho, Holger Weishaupt, Fredrik J Swartling
Medulloblastoma (MB) is the most common malignant brain tumor in children. MYC genes are frequently amplified and correlate with poor prognosis in MB. BET bromodomains recognize acetylated lysine residues and often promote and maintain MYC transcription. Certain cyclin-dependent kinases (CDKs) are further known to support MYC stabilization in tumor cells. In this report, MB cells were suppressed by combined targeting of MYC expression and MYC stabilization using BET bromodomain inhibition and CDK2 inhibition, respectively...
March 7, 2018: Oncogene
Sivan Gershanov, Helen Toledano, Shalom Michowiz, Orit Barinfeld, Albert Pinhasov, Nitza Goldenberg-Cohen, Mali Salmon-Divon
Purpose: Medulloblastoma (MB), the most common malignant brain tumor in children, is divided into four tumor subgroups: wingless-type (WNT), sonic hedgehog (SHH), Group 3, and Group 4. Ideally, clinical practice and treatment design should be subgroup specific. While WNT and SHH subgroups have well-defined biomarkers, distinguishing Group 3 from Group 4 is not straightforward. MicroRNAs (miRNAs), which regulate posttranscriptional gene expression, are involved in MB tumorigenesis. However, the miRNA-messenger RNA (mRNA) regulatory network in MB is far from being fully understood...
2018: Cancer Management and Research
Sreedharan Thankarajan Arunraj, Girish Kumar Parida, Nishikant Avinash Damle, Saurabh Arora, Sreenivasa Reddy, Dhritiman Chakraborty, Meghna Prabhu, Madhavi Tripathi, Chandrasekhar Bal
Medulloblastoma, also known as cerebellar primitive neuroectodermal tumor, is the most common brain tumor in children and arises in the posterior cranial fossa. We present the case of a patient with desmoplastic type of medulloblastoma, which showed recurrence more than once. When Ga-DOTANOC PET-CT was done, the lesions showed somatostatin receptor expression, opening another potential therapeutic option for this patient.
February 27, 2018: Clinical Nuclear Medicine
Laetitia Padovani, Frédérique Chapon, Nicolas André, Mohamed Boucekine, Anne Geoffray, Franck Bourdeau, Julien Masliah-Planchon, Line Claude, Aymeri Huchet, Anne Laprie, Stephane Supiot, Bernard Coche-Dequéant, Christine Kerr, Claire Alapetite, Julie Leseur, Tandat Nguyen, Sophie Chapet, Valerie Bernier, Pierre-Yves Bondiau, Georges Noel, Jean Louis Habrand, Stephanie Bolle, François Doz, Christelle Dufour, Xavier Muracciole, Christian Carrie
PURPOSE: To identify the incidence of patients with perihippocampal metastases to assess the risk of brain relapse when sparing the hippocampal area. Medulloblastoma (MB) represents 20% of pediatric brain tumors. For high-risk MB patients, the 3- to 5-year event-free survival rate has recently improved from 50% to >76%. Many survivors, however, experience neurocognitive side effects. Several retrospective studies of patients receiving whole brain irradiation (WBI) have suggested a relationship between the radiation dose to the hippocampus and neurocognitive decline...
March 15, 2018: International Journal of Radiation Oncology, Biology, Physics
Atosa Estekizadeh, Natalia Landázur, Jiri Bartek, Christian Beltoft Brøchner, Belghis Davoudi, Helle Broholm, Mohsen Karimi, Tomas J Ekström, Afsar Rahbar
Among all brain tumors diagnosed in children, medulloblastomas (MBs) are associated with a poor prognosis. The etiology of MB is not fully understood, yet the impact of epigenetic alterations of oncogenes has previously been established. During the past decade, the human cytomegalovirus (HCMV) has been detected in several types of cancer, including MB. Since DNA methylation occurs in the cell nucleus and this is considered a host defence response, we studied the impact of HCMV infection on DNA methyltransferase (DNMT‑1) in MB (D324) cells, human umbilical vein endothelial cells (HUVECs) as well as in MB tissue sections...
February 26, 2018: International Journal of Oncology
Adomas Bunevicius, Algimantas Matukevicius, Vytenis Deltuva, Inga Gudinaviciene, Darius Pranys, Arimantas Tamsauskas
OBJECTIVE: In vitro fertilization (IVF) is increasingly used for treatment of infertile couples worldwide. The association between IVF and cancer risk in offsprings is conflicting. We present a case of atypical teratoid/rhabdoid tumor (AT/RT) in a girl conceived by IVF and present results of systematic review of literature of primary intracranial neoplasms diagnosed in children conceived by IVF. METHODS: A systematic review of literature was conducted on April 12, 2017 to identify previously published reports of intracranial brain tumors in patients conceived after IVF...
February 13, 2018: World Neurosurgery
Xin He, Zhongping Chen, Yutong Dong, Dan Tong
RATIONALE: Central nervous system primitive neuroectodermal tumors (CNS PNETs) mostly occur in children and present as cerebellar medulloblastoma. A few cases of PNETs occur in the cerebral hemisphere. The presence of a PNET in ventricles is extremely rare. The prognosis of CNS PNET is extremely poor, and the 5-year survival rate does not exceed 35%. In the present study, we describe the first case of a PNET in the ventricles with good prognosis. PATIENT CONCERNS: The case of a 36-year-old man is reported, who presented with a progressively worsening headache for 2 months...
February 2018: Medicine (Baltimore)
Donna L Johnston, Daniel Keene, Douglas Strother, Maria Taneva, Lucie Lafay-Cousin, Chris Fryer, Katrin Scheinemann, Anne-Sophie Carret, Adam Fleming, Samina Afzal, Beverly Wilson, Lynette Bowes, Shayna Zelcer, Chris Mpofu, Mariana Silva, Valerie Larouche, Josee Brossard, Eric Bouffet
Medulloblastoma is the most common malignant brain tumor in children. Published survival rates for this tumor are ∼70%; however, there is limited published information on outcome after disease recurrence. This was an observational study which included all persons under the age of 18 years diagnosed with medulloblastoma from 1990 to 2009 inclusive in Canada. Data collected included date of diagnosis, age at diagnosis, sex, stage, pathology, treatment, recurrence, and current status. Survival rates were determined...
February 9, 2018: Journal of Pediatric Hematology/oncology
Tabitha Cooney, Paul G Fisher, Li Tao, Christina A Clarke, Sonia Partap
The objective of this study was to investigate racial/ethnic differences in survival for pediatric high-grade glioma (HGG) and medulloblastoma in the state of California. We obtained data from the California Cancer Registry on 552 high-grade glioma patients (110 brainstem, 442 non-brainstem) and 648 medulloblastoma patients ages 0-19 years from 1988 to 2012. Using multivariate Cox proportional hazards regression, we examined the impact of individual and neighborhood characteristics on survival. Socioeconomic quintile and insurance status differed significantly by race for both diagnoses...
February 7, 2018: Journal of Neuro-oncology
Michelle S Gentile, Beow Y Yeap, Harald Paganetti, Claire P Goebel, Dillon E Gaudet, Sara L Gallotto, Elizabeth A Weyman, Michael L Morgan, Shannon M MacDonald, Drosoula Giantsoudi, Judith Adams, Nancy J Tarbell, Hanne Kooy, Torunn I Yock
PURPOSE: Proton radiation therapy is commonly used in young children with brain tumors for its potential to reduce late effects. However, some proton series report higher rates of brainstem injury (0%-16%) than most photon series (2.2%-8.6%). We report the incidence of brainstem injury and a risk factor analysis in pediatric patients with posterior fossa primary tumors treated with proton radiation therapy at our institution. METHODS AND MATERIALS: The study included 216 consecutive patients treated between 2000 and 2015...
March 1, 2018: International Journal of Radiation Oncology, Biology, Physics
Arnold C Paulino, Anita Mahajan, Rong Ye, David R Grosshans, M Fatih Okcu, Jack Su, Mary Frances McAleer, Susan McGovern, Victor A Mangona, Murali Chintagumpala
PURPOSE: To compare ototoxicity rates between medulloblastoma patients treated with protons vs. photons. MATERIALS AND METHODS: The study included 84 children diagnosed with medulloblastoma treated with either passively scattered protons (n = 38) or photons (n = 46). Patients underwent maximal safe resection followed by craniospinal irradiation, posterior fossa and/or tumor bed boost and chemotherapy according to one of 3 multi-institutional trials. Median audiogram follow-up was 56 months for protons and 66 months for photons...
January 17, 2018: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
Ana S Guerreiro Stucklin, Vijay Ramaswamy, Craig Daniels, Michael D Taylor
PURPOSE OF REVIEW: Brain tumors are the most common solid tumors and leading cause of cancer-related death in children. The advent of large-scale genomics has resulted in a plethora of profiling studies that have mapped the genetic and epigenetic landscapes of pediatric brain tumors, ringing in a new era of precision diagnostics and targeted therapies. In this review, we highlight the most recent findings, focusing on studies published after 2015, and discuss how new evidence is changing the care of children with brain tumors...
February 2018: Current Opinion in Pediatrics
Tomoko Kurimoto, Akihide Kondo, Ikuko Ogino, Junya Fujimura, Atsushi Arakawa, Hajime Arai, Toshiaki Shimizu
Medulloblastoma is a highly malignant brain tumor that predominately affects children and requires multimodal treatment, including chemotherapy with alkylating agents. O6-methylguanine-DNA methyltransferase (MGMT) is a DNA repair enzyme that plays an important role in tumor resistance to alkylating agents. Recent studies demonstrated that MGMT promoter methylation suppresses the expression of MGMT and is associated with favorable outcomes of malignant glioma patients. However, the MGMT methylation status and its prognostic impact on medulloblastoma have not been fully elucidated to date...
December 2017: Molecular and Clinical Oncology
Teresa de Rojas, Francisco Bautista, Miguel Flores, Lucía Igual, Raquel Rubio, Eduardo Bardón, Lucía Navarro, Laura Murillo, Raquel Hladun, Adela Cañete, Miguel Garcia-Ariza, Carmen Garrido, Ana Fernández-Teijeiro, Eduardo Quiroga, Carlota Calvo, Anna Llort, Inmaculada de Prada, Luis Madero, Ofelia Cruz, Lucas Moreno
Non-medulloblastoma CNS embryonal tumors (former PNET/Pineoblastomas) are aggressive malignancies with poor outcome that have been historically treated with medulloblastoma protocols. The purpose of this study is to present a tumor-specific, real-world data cohort of patients with CNS-PNET/PB to analyze quality indicators that can be implemented to improve the outcome of these patients. Patients 0-21 years with CNS-PNET treated in eight large institutions were included. Baseline characteristics, treatment and outcome [progression-free and overall survival (PFS and OS respectively)] were analyzed...
December 16, 2017: Journal of Neuro-oncology
Meenu Gupta, Ravi Kant, Vipul Nautiyal, Jyoti Bisht, Shalindra Raghuvanshi, Manju Saini, B P Kalra, Mushtaq Ahmad
AIMS: To compare three-dimensional conformal radiation therapy (3DCRT) and intensity-modulated radiation therapy (IMRT) in posterior fossa (PF) boost in children with medulloblastoma (MB); dosimetrically evaluating and comparing both techniques with regard to target coverage and doses to organs at risk. SETTINGS AND DESIGN: Structured. SUBJECTS AND METHODS: Seven previously irradiated patients of MB were retrieved and re-planned with both 3DCRT and IMRT techniques...
October 2017: Journal of Cancer Research and Therapeutics
Fernando Carceller, Francisco Bautista, Irene Jiménez, Raquel Hladun-Álvaro, Cécile Giraud, Luca Bergamaschi, Madhumita Dandapani, Isabelle Aerts, François Doz, Didier Frappaz, Michela Casanova, Bruce Morland, Darren R Hargrave, Gilles Vassal, Andrew D J Pearson, Birgit Geoerger, Lucas Moreno, Lynley V Marshall
Central nervous system (CNS) tumors are a leading cause of death in pediatric oncology. New drugs are desperately needed to improve survival. We evaluated the outcome of children and adolescents with CNS tumors participating in phase I trials within the Innovative Therapies for Children with Cancer (ITCC) consortium. Patients with solid tumors aged < 18 years at enrollment in their first dose-finding trial between 2000 and 2014 at eight ITCC centers were included retrospectively. Survival was evaluated using univariate/multivariate analyses...
March 2018: Journal of Neuro-oncology
Michal Zapotocky, Daddy Mata-Mbemba, David Sumerauer, Petr Liby, Alvaro Lassaletta, Josef Zamecnik, Lenka Krskova, Martin Kyncl, Jan Stary, Suzanne Laughlin, Anthony Arnoldo, Cynthia Hawkins, Uri Tabori, Michael D Taylor, Eric Bouffet, Charles Raybaud, Vijay Ramaswamy
OBJECTIVE Metastatic dissemination is a major treatment challenge and cause of death in patients with medulloblastoma. However, the influence of molecular biology on the pattern of metastatic dissemination at diagnosis is not known. In this study, the authors sought to define the location, pattern, and imaging characteristics of medulloblastoma metastases across subgroups at diagnosis. METHODS A consecutive cohort of patients with metastatic medulloblastoma at The Hospital for Sick Children and the University Hospital Motol, who underwent up-front MRI of the craniospinal axis, was assembled and allocated to subgroups using NanoString limited gene-expression profiling...
February 2018: Journal of Neurosurgery. Pediatrics
Kadali Satya Vara Prasad, D Ravi, Vijay Pallikonda, Bhavana Venkata Satya Raman
Context: Brain tumor is one of the most devastating forms of human illness, especially when occurring in the posterior fossa and involving the brainstem. Tumors in the posterior fossa are considered some of the most critical brain lesions. This is primarily due to the limited space within the posterior fossa, as well as the potential involvement of the vital brainstem nuclei. Aims: The aim of this study is to analyze the incidence, clinical features, surgical outcome, complications, and prognosis in a series of 37 pediatric patients with posterior fossa tumors who underwent surgery between September 2012 and January 2015 from the Department of Neurosurgery, King George Hospital, Visakhapatnam (both prospective and retrospective study)...
July 2017: Journal of Pediatric Neurosciences
Supriya Gujjar Suresh, Arathi Srinivasan, Julius Xavier Scott, Santosh Mohan Rao, Balasubramaniam Chidambaram, Sanjay Chandrasekar
Context: Tumors of the central nervous system (CNS) constitute the second most common pediatric cancers. Unlike leukemia, management of CNS tumors requires a good multidisciplinary team. Higher rates of treatment abandonment are documented in view of complexity of the treatment with long duration, involving neurosurgery, radiation, chemotherapy, and high cost of treatment. Morbidity associated with CNS tumors may be significant in terms of physical deficits as well as neuropsychological and neuroendocrine sequelae...
July 2017: Journal of Pediatric Neurosciences
Soumen Khatua, Anne Song, Divyaswathi Citla Sridhar, Stephen C Mack
Medulloblastoma is the most common malignant brain tumor in children, currently treated uniformly based on histopathology and clinico-radiological risk stratification. This approach has been undermined by unpredictable relapses and therapeutic failures. Research endeavors over the last decade have clearly identified four molecular variants (WNT, SHH, Group 3, and Group 4) and their demographic, genomic, and epigenetic profile. Improved understanding of the signaling pathways and key oncogenic drivers in these subgroups, has shed light on the reasons for suboptimal clinical outcome and mechanisms of therapeutic resistance...
November 28, 2017: Current Neuropharmacology
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