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https://www.readbyqxmd.com/read/29143922/pre-treatment-lymphopenia-and-indication-of-tumor-induced-systemic-immunosuppression-in-medulloblastoma
#1
Seema Patel, Shiyang Wang, Matija Snuderl, Matthias A Karajannis
The presence of tumor-induced systemic immune suppression, including lymphopenia, has been recognized in adult patients with glioblastoma for several decades, and pre-treatment neutrophil-to-lymphocyte count ratio (NLCR) is associated with inferior clinical outcome in patients with glioblastoma. Whether tumor-induced systemic immune suppression is also present in children with malignant brain tumors is not known. We performed a retrospective analysis of pretreatment neutrophil and lymphocyte counts in pediatric patients with medulloblastoma (MB) compared to a control group of children with posterior fossa pilocytic astrocytoma (PA)...
November 16, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/29070607/contribution-of-de-novo-and-mosaic-tp53-mutations-to-li-fraumeni-syndrome
#2
Mariette Renaux-Petel, Françoise Charbonnier, Jean-Christophe Théry, Pierre Fermey, Gwendoline Lienard, Jacqueline Bou, Sophie Coutant, Myriam Vezain, Edwige Kasper, Steeve Fourneaux, Sandrine Manase, Maud Blanluet, Bruno Leheup, Ludovic Mansuy, Jacqueline Champigneulle, Céline Chappé, Michel Longy, Nicolas Sévenet, Brigitte Bressac-de Paillerets, Léa Guerrini-Rousseau, Laurence Brugières, Olivier Caron, Jean-Christophe Sabourin, Isabelle Tournier, Stéphanie Baert-Desurmont, Thierry Frébourg, Gaëlle Bougeard
BACKGROUND: Development of tumours such as adrenocortical carcinomas (ACC), choroid plexus tumours (CPT) or female breast cancers before age 31 or multiple primary cancers belonging to the Li-Fraumeni (LFS) spectrum is, independently of the familial history, highly suggestive of a germline TP53 mutation. The aim of this study was to determine the contribution of de novo and mosaic mutations to LFS. METHODS AND RESULTS: Among 328 unrelated patients harbouring a germline TP53 mutation identified by Sanger sequencing and/or QMPSF, we could show that the mutations had occurred de novo in 40 cases, without detectable parental age effect...
October 25, 2017: Journal of Medical Genetics
https://www.readbyqxmd.com/read/29050204/irreversible-growth-plate-fusions-in-children-with-medulloblastoma-treated-with-a-targeted-hedgehog-pathway-inhibitor
#3
Giles W Robinson, Sue C Kaste, Wassim Chemaitilly, Daniel C Bowers, Stephen Laughton, Amy Smith, Nicholas G Gottardo, Sonia Partap, Anne Bendel, Karen D Wright, Brent A Orr, William C Warner, Arzu Onar-Thomas, Amar Gajjar
The permanent defects in bone growth observed in preclinical studies of hedgehog (Hh) pathway inhibitors were not substantiated in early phase clinical studies of vismodegib in children. Consequently, vismodegib advanced into pediatric trials for malignancies suspected of being driven by aberrant activation of the Hh pathway. In one multicenter phase II trial, vismodegib was added to the therapy regimen for newly diagnosed Hh pathway activated medulloblastoma. Herein, we report on 3 children (2 on trial and one off trial) treated with vismodegib who developed widespread growth plate fusions that persist long after cessation of therapy...
September 19, 2017: Oncotarget
https://www.readbyqxmd.com/read/29045264/patterns-of-failure-in-patients-with-adult-medulloblastoma-presenting-without-extraneural-metastasis
#4
Meriem Mokhtech, Christopher G Morris, Daniel J Indelicato, Michael S Rutenberg, Robert J Amdur
OBJECTIVES: The objective of this study is to evaluate long-term outcomes, specifically patterns of tumor recurrence, in patients with adult medulloblastoma treated with radiotherapy. METHODS: We recorded outcomes of 28 (median age, 25 y) patients 18 years old or above with M0 to M3 medulloblastoma treated between 1971 and 2012. Among them, 61% had standard-risk disease. All received craniospinal irradiation with a posterior fossa boost. Median dose to the craniospinal axis was 36 Gy...
October 17, 2017: American Journal of Clinical Oncology
https://www.readbyqxmd.com/read/29028423/2016-updates-to-the-who-brain-tumor-classification-system-what-the-radiologist-needs-to-know
#5
Derek R Johnson, Julie B Guerin, Caterina Giannini, Jonathan M Morris, Lawrence J Eckel, Timothy J Kaufmann
Radiologists play a key role in brain tumor diagnosis and management and must stay abreast of developments in the field to advance patient care and communicate with other health care providers. In 2016, the World Health Organization (WHO) released an update to its brain tumor classification system that included numerous significant changes. Several previously recognized brain tumor diagnoses, such as oligoastrocytoma, primitive neuroectodermal tumor, and gliomatosis cerebri, were redefined or eliminated altogether...
November 2017: Radiographics: a Review Publication of the Radiological Society of North America, Inc
https://www.readbyqxmd.com/read/29016818/posterior-fossa-syndrome-and-long-term-neuropsychological-outcomes-among-children-treated-for-medulloblastoma-on-a-multi-institutional-prospective-study
#6
Jane E Schreiber, Shawna L Palmer, Heather M Conklin, Donald J Mabbott, Michelle A Swain, Melanie J Bonner, Mary L Chapieski, Lu Huang, Hui Zhang, Amar Gajjar
Background: Patients treated for medulloblastoma who experience posterior fossa syndrome (PFS) demonstrate increased risk for neurocognitive impairment at one year post diagnosis. The aim of the study was to examine longitudinal trajectories of neuropsychological outcomes in patients who experienced PFS compared with patients who did not. Methods: Participants were 36 patients (22 males) who experienced PFS and 36 comparison patients (21 males) who were matched on age at diagnosis and treatment exposure but did not experience PFS...
July 19, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/28980418/medulloblastoma-in-children-and-adolescents-a-systematic-review-of-contemporary-phase-i-and-ii-clinical-trials-and-biology-update
#7
REVIEW
Francisco Bautista, Victoria Fioravantti, Teresa de Rojas, Fernando Carceller, Luis Madero, Alvaro Lassaletta, Lucas Moreno
Survival rates for patients with medulloblastoma have improved in the last decades but for those who relapse outcome is dismal and new approaches are needed. Emerging drugs have been tested in the last two decades within the context of phase I/II trials. In parallel, advances in genetic profiling have permitted to identify key molecular alterations for which new strategies are being developed. We performed a systematic review focused on the design and outcome of early-phase trials evaluating new agents in patients with relapsed medulloblastoma...
November 2017: Cancer Medicine
https://www.readbyqxmd.com/read/28974245/dose-escalation-study-of-intravenous-and-intra-arterial-n-acetylcysteine-for-the-prevention-of-oto-and-nephrotoxicity-of-cisplatin-with-a-contrast-induced-nephropathy-model-in-patients-with-renal-insufficiency
#8
Edit Dósa, Krisztina Heltai, Tamás Radovits, Gabriella Molnár, Judit Kapocsi, Béla Merkely, Rongwei Fu, Nancy D Doolittle, Gerda B Tóth, Zachary Urdang, Edward A Neuwelt
BACKGROUND: Cisplatin neuro-, oto-, and nephrotoxicity are major problems in children with malignant tumors, including medulloblastoma, negatively impacting educational achievement, socioemotional development, and overall quality of life. The blood-labyrinth barrier is somewhat permeable to cisplatin, and sensory hair cells and cochlear supporting cells are highly sensitive to this toxic drug. Several chemoprotective agents such as N-acetylcysteine (NAC) were utilized experimentally to avoid these potentially serious and life-long side effects, although no clinical phase I trial was performed before...
October 3, 2017: Fluids and Barriers of the CNS
https://www.readbyqxmd.com/read/28971956/tap73-is-a-marker-of-glutamine-addiction-in-medulloblastoma
#9
Maria Victoria Niklison-Chirou, Ida Erngren, Mikael Engskog, Jakob Haglöf, Daniel Picard, Marc Remke, Phelim Hugh Redmond McPolin, Matthew Selby, Daniel Williamson, Steven C Clifford, David Michod, Michalis Hadjiandreou, Torbjörn Arvidsson, Curt Pettersson, Gerry Melino, Silvia Marino
Medulloblastoma is the most common solid primary brain tumor in children. Remarkable advancements in the understanding of the genetic and epigenetic basis of these tumors have informed their recent molecular classification. However, the genotype/phenotype correlation of the subgroups remains largely uncharacterized. In particular, the metabolic phenotype is of great interest because of its druggability, which could lead to the development of novel and more tailored therapies for a subset of medulloblastoma...
September 1, 2017: Genes & Development
https://www.readbyqxmd.com/read/28960048/coexistence-of-radiation-induced-meningioma-and-moyamoya-syndrome-10-years-after-irradiation-against-medulloblastoma-a-case-report
#10
Ji Yeon Han, Jung Won Choi, Kyu Chang Wang, Ji Hoon Phi, Ji Yeoun Lee, Jong Hee Chae, Sung Hye Park, Jung Eun Cheon, Seung Ki Kim
Radiotherapy is one of the standard treatments for medulloblastoma. However, therapeutic central nervous system irradiation in children may carry delayed side effects, such as radiation-induced tumor and vasculopathy. Here, we report the first case of coexisting meningioma and moyamoya syndrome, presenting 10 years after radiotherapy for medulloblastoma. A 13-year-old boy presented with an enhancing mass at the cerebral falx on magnetic resonance imaging (MRI) after surgery, radiotherapy (30.6 Gy craniospinal axis, 19...
November 2017: Journal of Korean Medical Science
https://www.readbyqxmd.com/read/28947980/added-value-of-diffusion-weighted-imaging-in-pediatric-central-nervous-system-embryonal-tumors-surveillance
#11
Giovanni Morana, Cesar Augusto Alves, Domenico Tortora, Mariasavina Severino, Paolo Nozza, Armando Cama, Marcello Ravegnani, Gabriella D'Apolito, Alessandro Raso, Claudia Milanaccio, Claudia da Costa Leite, Maria Luisa Garrè, Andrea Rossi
Diffusion weighted imaging (DWI) has an established role in primary CNS embryonal tumor (ET) characterization; however, its diagnostic utility in detecting relapse has never been determined. We aimed to compare DWI and conventional MRI sensitivity in CNS ET recurrence detection, and to evaluate the DWI properties of contrast-enhancing radiation induced lesions (RIL). Fifty-six patients with CNS ET (25 with disease relapse, 6 with RIL and 25 with neither disease relapse nor RIL) were retrospectively evaluated with DWI, conventional MRI (including both T2/FLAIR and post-contrast images), or contrast-enhanced MR imaging (CE-MRI) alone...
September 1, 2017: Oncotarget
https://www.readbyqxmd.com/read/28941523/a-review-of-visual-and-oculomotor-outcomes-in-children-with-posterior-fossa-tumors
#12
Crandall E Peeler
Tumors of the posterior fossa represent the most common solid malignancy of childhood and can affect the visual system in several ways. This article outlines the relevant visual anatomy affected by these tumors and reviews the visual and oculomotor outcomes associated with the following 3 most common tumor types-medulloblastoma, juvenile pilocytic astrocytoma, and ependymoma. The available data suggest that the rate of permanent vision loss is low (5.9%-8.3%), with patients having juvenile pilocytic astrocytoma demonstrating the best outcomes...
May 2017: Seminars in Pediatric Neurology
https://www.readbyqxmd.com/read/28928843/expression-and-clinical-relevance-of-spopl-in-medulloblastoma
#13
Yuanjun Hu, Lixuan Yang, Maolei Zhang, Zhengsong Huang, Jiaping Lin, Nu Zhang
Medulloblastoma (MB) is a type of malignant brain tumor in children. Although knowledge of MB is increasing and the survival rate of patients with MB has improved in previous years, the long-term treatment-associated complications remain unfavorable. Early diagnosis and treatment is critical for patients with MB, but effective molecular markers for MB remain elusive. The Speckle-type POZ protein (SPOP) is a member of the MATH-BTB protein family and is a type of joint molecule for Cullin-3. SPOP inhibits tumor growth...
September 2017: Oncology Letters
https://www.readbyqxmd.com/read/28924528/near-fatal-gastrointestinal-hemorrhage-in-a-child-with-medulloblastoma-on-high-dose-dexamethasone
#14
Derek Yecies, Daniel Tawfik, Jennifer Damman, Chad Thorson, David S Hong, Gerald A Grant, Rachel Bensen, Mihaela Damian
A four-year-old female was admitted to a university-based children's hospital with a newly-diagnosed posterior fossa tumor. She was started on famotidine and high-dose dexamethasone and underwent gross total resection of a medulloblastoma. She was continued on dexamethasone and famotidine. She exhibited postoperative posterior fossa syndrome and was started on enteral feeds via the nasoduodenal tube. She had small gastrointestinal bleeds on postoperative days eight, 11, and 18, and was found to have a well-circumscribed posterior duodenal ulcer...
July 7, 2017: Curēus
https://www.readbyqxmd.com/read/28887665/impact-of-radiation-technique-radiation-fraction-dose-and-total-cisplatin-dose-on-hearing-retrospective-analysis-of-29%C3%A2-medulloblastoma-patients
#15
Sergiu Scobioala, Ross Parfitt, Peter Matulat, Christopher Kittel, Fatemeh Ebrahimi, Heidi Wolters, Antoinette Am Zehnhoff-Dinnesen, Hans Theodor Eich
PURPOSE: To analyze the incidence and degree of sensorineural hearing loss (SNHL) resulting from different radiation techniques, fractionation dose, mean cochlear radiation dose (Dmean), and total cisplatin dose. MATERIAL AND METHODS: In all, 29 children with medulloblastoma (58 ears) with subclinical pretreatment hearing thresholds participated. Radiotherapy (RT) and cisplatin had been applied sequentially according to the HIT MED Guidance. Audiological outcomes up to the latest follow-up (median 2...
November 2017: Strahlentherapie und Onkologie: Organ der Deutschen Röntgengesellschaft ... [et Al]
https://www.readbyqxmd.com/read/28877739/estrogen-and-soy-isoflavonoids-decrease-sensitivity-of-medulloblastoma-and-central-nervous-system-primitive-neuroectodermal-tumor-cells-to-chemotherapeutic-cytotoxicity
#16
Scott M Belcher, Caleb C Burton, Clifford J Cookman, Michelle Kirby, Gabriel L Miranda, Fatima O Saeed, Kathleen E Wray
BACKGROUND: Our previous studies demonstrated that growth and migration of medulloblastoma (MB), the most common malignant brain tumor in children, are stimulated by 17β-estradiol. The growth stimulating effects of estrogens are mediated through ERβ and insulin-like growth factor 1 signaling to inhibit caspase 3 activity and reduce tumor cell apoptosis. The objective of this study was to determine whether estrogens decreased sensitivity of MB cells to cytotoxic actions of chemotherapeutic drugs...
September 6, 2017: BMC Pharmacology & Toxicology
https://www.readbyqxmd.com/read/28867398/visual-and-ocular-motor-outcomes-in-children-with-posterior-fossa-tumors
#17
Crandall E Peeler, Jane C Edmond, Jeffrey Hollander, Jamie K Alexander, David Zurakowski, Nicole J Ullrich, Peter E Manley, Gena Heidary
PURPOSE: To describe the clinical characteristics and visual and ocular motor outcomes of a large cohort of pediatric patients treated for tumors of the posterior cranial fossa. METHODS: The medical records of all patients with posterior fossa tumors evaluated by the ophthalmology services at two large tertiary care academic hospitals between 2005 and 2011 were reviewed retrospectively. Data abstracted for each study patient included demographic information, presenting signs and symptoms, pathologic diagnosis, and results of the most recent ophthalmology examination...
August 31, 2017: Journal of AAPOS: the Official Publication of the American Association for Pediatric Ophthalmology and Strabismus
https://www.readbyqxmd.com/read/28844716/oncogenic-role-of-cytomegalovirus-in-medulloblastoma
#18
REVIEW
Alejandro M Hortal, Jeroen F Vermeulen, Wim Van Hecke, Niels Bovenschen
Medulloblastoma is the most common solid tumor among children. Current therapeutic strategies for this malignancy include surgical resection, radiation therapy and chemotherapy. However, these treatments are accompanied with serious side effects such as neurological complications and psychosocial problems, due to the severity of treatment on the developing nervous system. To solve this problem, novel therapeutic approaches are currently being investigated. One of them is targeting human cytomegalovirus in medulloblastoma cancer cells...
November 1, 2017: Cancer Letters
https://www.readbyqxmd.com/read/28808827/distinctive-localization-and-mri-features-correlate-of-molecular-subgroups-in-adult-medulloblastoma
#19
Fu Zhao, Chunde Li, Qiangyi Zhou, Peiran Qu, Bo Wang, Xin Wang, Shun Zhang, Xingchao Wang, Chi Zhao, Jing Zhang, Lin Luo, Lin Ai, Lei Xu, Pinan Liu
Medulloblastoma (MB) is recognized as comprising four molecular subgroups with distinct transcriptional profiles, clinical features, and outcomes. Previous studies demonstrate that pediatric MBs present with subgroup-specific MRI manifestations. We hypothesized that combination of anatomical localization and conventional features based on MR imaging can predict these subgroups in adult MBs. MR Imaging manifestations of 125 adult patients with MB were analyzed retrospectively based on pre-operative MRI scans...
August 14, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28798218/imaging-biomarkers-for-adult-medulloblastomas-genetic-entities-may-be-identified-by-their-mr-imaging-radiophenotype
#20
V C Keil, M Warmuth-Metz, C Reh, S J Enkirch, C Reinert, D Beier, D T W Jones, T Pietsch, H H Schild, E Hattingen, P Hau
BACKGROUND AND PURPOSE: The occurrence of medulloblastomas in adults is rare; nevertheless, these tumors can be subdivided into genetic and histologic entities each having distinct prognoses. This study aimed to identify MR imaging biomarkers to classify these entities and to uncover differences in MR imaging biomarkers identified in pediatric medulloblastomas. MATERIALS AND METHODS: Eligible preoperative MRIs from 28 patients (11 women; 22-53 years of age) of the Multicenter Pilot-study for the Therapy of Medulloblastoma of Adults (NOA-7) cohort were assessed by 3 experienced neuroradiologists...
August 10, 2017: AJNR. American Journal of Neuroradiology
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