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Pediatric brain tumors

Maria Beran, Britta Okyere, Joshua Vova
Amantadine is commonly prescribed as a neurostimulant in patients with brain injuries. This is a case of a 14 year-old male with a history of brain tumor that developed corneal edema after initiation of amantadine, a rare but documented side effect of this medication. After discontinuation of amantadine, the corneal edema resolved within two months, but endothelial cells density remained low.
March 14, 2018: PM & R: the Journal of Injury, Function, and Rehabilitation
Martha Nowosielski, Patrick Y Wen
The identification of more effective therapies for brain tumors has been limited in part by the lack of reliable criteria for determining response and progression. Since its introduction in 1990, the MacDonald criteria have been used in neuro-oncology clinical trials to determine response, but they fail to address issues such as pseudoprogression, pseudoresponse, and nonenhancing tumor progression that have arisen with more recent therapies. The Response Assessment in Neuro-Oncology (RANO) working group, a multidisciplinary international group consisting of neuro-oncologists, medical oncologists, neuroradiologists, neurosurgeons, radiation oncologists, and neuropsychologists, was formed to improve response assessment and clinical trial endpoints in neuro-oncology...
February 2018: Seminars in Neurology
Istvan Bodi, Anastasios Giamouriadis, Naomi Sibtain, Ross Laxton, Andrew King, Francesco Vergani
Background: Primary CNS malignant rhabdoid tumors are very rare in adults and much less is known about their biological behavior than in children. Recently, two adult cases of SMARCB1 (also known as INI1)-deficient tumor with rhabdoid cells have been described, suggesting an emerging group of primary meningeal SMARCB1-deficient tumors. We have recently encountered a case of INI1-deficient tumor with similar histology and immunophenotype to the above cases, but with a superficial cerebral, yet apparent intra-axial origin...
2018: Surgical Neurology International
Michael Schwake, Andrei Nemes, Jana Dondrop, Juliane Schroeteler, Stephanie Schipmann, Volker Senner, Walter Stummer, Christian Ewelt
BACKGROUND: Light irradiation (635 nm) of cells containing protoporphyrin IX (PPIX) after 5- aminolevulinic acid (5-ALA) pretreatment causes cell death via different pathways including apoptosis and necrosis, as previously demonstrated for malignant glioma cells. OBJECTIVE: To elucidate whether various malignant pediatric brain tumors, which have been shown to accumulate PPIX, would also be susceptible to photodynamic therapy (PDT). METHODS: Medulloblastoma (DAOY, UW228), pNET (PFSK-1), and rhabdoid tumor (BT16) cell lines were incubated with 5-ALA in variable concentrations for 4 h...
March 12, 2018: Neurosurgery
Dario Presutti, Manuela Ceccarelli, Laura Micheli, Giuliana Papoff, Simonetta Santini, Simone Samperna, Cristiana Lalli, Lorena Zentilin, Giovina Ruberti, Felice Tirone
Medulloblastoma (MB), the tumor of the cerebellum, is the most frequent brain cancer in childhood and a major cause of pediatric mortality. Based on gene profiling, four MB subgroups have been identified, i.e., Wnt or Sonic Hedgehog (Shh) types, and subgroup 3 or 4. The Shh-type MB has been shown to arise from the cerebellar precursors of granule neurons (GCPs), where a hyperactivation of the Shh pathway leads to their neoplastic transformation. We have previously shown that the gene Tis21 (PC3/Btg2) inhibits the proliferation and promotes the differentiation and migration of GCPs...
2018: PloS One
Alexandra Garancher, Charles Y Lin, Morgane Morabito, Wilfrid Richer, Nathalie Rocques, Magalie Larcher, Laure Bihannic, Kyle Smith, Catherine Miquel, Sophie Leboucher, Nirmitha I Herath, Fanny Dupuy, Pascale Varlet, Christine Haberler, Christine Walczak, Nadine El Tayara, Andreas Volk, Stéphanie Puget, François Doz, Olivier Delattre, Sabine Druillennec, Olivier Ayrault, Robert J Wechsler-Reya, Alain Eychène, Franck Bourdeaut, Paul A Northcott, Celio Pouponnot
Cancer cells often express differentiation programs unrelated to their tissue of origin, although the contribution of these aberrant phenotypes to malignancy is poorly understood. An aggressive subgroup of medulloblastoma, a malignant pediatric brain tumor of the cerebellum, expresses a photoreceptor differentiation program normally expressed in the retina. We establish that two photoreceptor-specific transcription factors, NRL and CRX, are master regulators of this program and are required for tumor maintenance in this subgroup...
March 12, 2018: Cancer Cell
Wafik Zaky, Jonathan L Finlay
Choroid plexus (CP) carcinoma is a rare pediatric brain neoplasm. Recent studies have highlighted the potential of genome-wide methylation and gene expression profiling to provide additional layers of information to improve tumor risk-stratification. There is a lack of data regarding the best therapy, and approaches have been heterogeneous. Despite multidisciplinary treatment approaches, the outcome remains guarded and treatments have been based on case series and expert opinions. In this study, we discuss the recent wealth of data regarding CP carcinoma molecular biology and current management...
March 12, 2018: Pediatric Blood & Cancer
Fumihiko Nishimura, Young-Su Park, Yasushi Motoyama, Ichiro Nakagawa, Shuichi Yamada, Hiroyuki Nakase
BACKGROUND: Xanthomatous pituitary diseases rarely occur in childhood. We report a rare pediatric case of a xanthogranuloma that developed in the sellar region resulting in visual disturbance that was successfully treated by endoscopic endonasal surgery. CASE DESCRIPTIONS: A 13-year-old boy came to us with a headache and visual disturbance that had occurred 1 month prior. Clinical examination findings showed that he was alert with signs of bitemporal hemianopsia, an endocrinological examination showed partial hypopituitarism, and brain magnetic resonance imaging (MRI) revealed a cystic mass in the sellar turcica compressing the optic apparatus...
March 7, 2018: World Neurosurgery
José E Velázquez Vega, Daniel J Brat
Recent advances in molecular pathology have reshaped the practice of brain tumor diagnostics. The classification of gliomas has been restructured with the discovery of isocitrate dehydrogenase (IDH) 1/2 mutations in the vast majority of lower grade infiltrating gliomas and secondary glioblastomas (GBM), with IDH-mutant astrocytomas further characterized by TP53 and ATRX mutations. Whole-arm 1p/19q codeletion in conjunction with IDH mutations now define oligodendrogliomas, which are also enriched for CIC, FUBP1, PI3K, NOTCH1, and TERT-p mutations...
March 8, 2018: Advances in Anatomic Pathology
L Holmes, P Chavan, T Blake, K Dabney
BACKGROUND: While survival in overall pediatric malignancy has improved during recent decades, brain/central nervous system (CNS) tumors has not demonstrated comparable survival advantage. Incidence and mortality data in this malignancy continue to illustrate race and sex differences; however, there are few data in the pediatric setting. This study sought to characterize brain/CNS tumors by socio-demographic and assess racial and sex variances in both cumulative incidence and mortality...
March 7, 2018: Journal of Racial and Ethnic Health Disparities
Takashi Ishihara, Keiji Nogami, Yasufumi Takeshita, Satoshi Ochi, Midori Shima
Thrombosis and hemorrhage are serious complications in pediatric patients with solid tumors, and enhanced fibrinolysis associated with disseminated intravascular coagulation (DIC) is often observed. Fibrinolytic enzymes also play an important role in metastasis. Limited information is available, however, on the assessment of overall hemostatic function in children with malignant solid tumors. Methods We have investigated comprehensive hemostatic potential in these circumstances using simultaneous thrombin/plasmin generation assay (T/P-GA)...
March 5, 2018: Pediatrics International: Official Journal of the Japan Pediatric Society
Fatima Tensaouti, Anne Ducassou, Léonor Chaltiel, Annick Sevely, Stéphanie Bolle, Laetitia Padovani, Anais Jouin, Claire Alapetite, Stéphane Supiot, Aymeri Huchet, Valérie Bernier, Line Claude, Christine Kerr, Elisabeth Le Prisé, Anne-Isabelle Bertozzi-Salamon, Samuel Liceaga, Jean Albert Lotterie, Patrice Péran, Anne Laprie
BACKGROUND AND PURPOSE: Ependymoma is the third most common brain tumor in children. Radiation therapy (RT) is systematically administered after maximum surgical resection, utilizing recent advances in radiation delivery. Imaging can make a significant contribution to improving treatment outcome. This prompted us to look for significant preoperative and postoperative imaging markers for survival. MATERIAL AND METHODS: We undertook a national retrospective review of 121 patients who had undergone resection followed by RT...
February 27, 2018: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
Andrew H Zureick, Casey L Evans, Andrzej Niemierko, Julie A Grieco, Alexandra J Nichols, Barbara C Fullerton, Clayton B Hess, Claire P Goebel, Sara L Gallotto, Elizabeth A Weyman, Dillon E Gaudet, Jessica A Nartowicz, David H Ebb, Robin M Jones, Shannon M MacDonald, Nancy J Tarbell, Torunn I Yock, Margaret B Pulsifer
BACKGROUND: Radiotherapy (RT) in the pediatric brain tumor population causes late neurocognitive effects. In the current study, the authors investigated associations between clinical and dosimetric risk factors and memory outcomes in a cohort of patients treated with proton radiotherapy (PRT). METHODS: A total of 70 patients (median age at PRT, 12.1 years [range, 5.0-22.5 years]) who were treated with PRT were identified with baseline and follow-up evaluations of visual and verbal memory (Children's Memory Scale and the third edition of the Wechsler Memory Scale)...
March 2, 2018: Cancer
Hila Rosenfeld-Keidar, Rinat Eshel, Aviva Pinhasov, Menachem Bitan, Sabina Edelman, Marcela Broitman, Rina Dvir, Efraim Sadot, Dror Levin, Michal Manisterski, Sivan Berger-Achituv, Ronit Elhasid
Numerous adults' studies demonstrated that preaphaeresis CD34+ cells significantly correlate with the number of CD34+ cells collected by the aphaeresis procedure. Equivalent studies in children are scarce. We studied retrospectively 92 aphaeresis procedures performed following chemotherapy (44) or in steady state (48) in 60 pediatric patients (40 males, 20 females), median age of 7.5 years. Aphaeresis procedures were performed using a SPECTRA Optica (TERUMOBCT) continuous flow cell separator. CD34+ cell concentrations were assessed using flow cytometry...
March 2, 2018: Pediatric Transplantation
Ana María Granados, Camila Ospina, Stephania Paredes
The purpose of this paper is to report a rare case of a pediatric pineal gliosarcoma. Gliomas on the pineal region are uncommon, representing 0.4%-1% of all brain tumors. Furthermore, pediatric gliosarcomas are a very rare entity. We present a case of a 5-year-old girl, with a history of headache, vomiting, diplopia, and gait disturbances. A pineal tumor was found with pathology results consistent with a gliosarcoma. A total of 25 cases of pediatric gliosarcomas have been reported, none of them in pineal topography...
February 2018: Radiology Case Reports
Beat Bojaxhiu, Frank Ahlhelm, Marc Walser, Lorenzo Placidi, Ulrike Kliebsch, Lorentzos Mikroutsikos, Petra Morach, Alessandra Bolsi, Tony Lomax, Alessia Pica, Damien C Weber
PURPOSE: To assess the rate of radiation necrosis (RN) and white matter lesions (WMLs) in pediatric patients with primary brain tumors treated with pencil beam scanning (PBS) proton therapy (PT) with or without concomitant chemotherapy at the PSI. METHODS AND MATERIALS: Between 1999 and 2015, 171 pediatric patients (age <18 years) were treated with PT. Median age at diagnosis was 3.3 years (range, 0.3-17.0 years), and the median delivered dose was 54 Gy (relative biological effectiveness) (range, 40...
March 15, 2018: International Journal of Radiation Oncology, Biology, Physics
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
Maria Tsoli, Jie Liu, Laura Franshaw, Han Shen, Cecilia Cheng, MoonSun Jung, Swapna Joshi, Anahid Ehteda, Aaminah Khan, Angel Montero-Carcabosso, Pierre J Dilda, Philip Hogg, David S Ziegler
Diffuse Intrinsic Pontine Gliomas (DIPG) are the most devastating of all pediatric brain tumors. They mostly affect young children and, as there are no effective treatments, almost all patients with DIPG will die of their tumor within 12 months of diagnosis. A key feature of this devastating tumor is its intrinsic resistance to all clinically available therapies. It has been shown that glioma development is associated with metabolic reprogramming, redox state disruption and resistance to apoptotic pathways...
January 26, 2018: Oncotarget
Maria Koutourousiou, Juan C Fernandez-Miranda, Eric W Wang, Carl H Snyderman, Paul A Gardner
The proximity of craniopharyngiomas to vital neurovascular structures and their high recurrence rates make them one of the most challenging brain tumors to treat. Although surgery remains the first line of therapy and offers the best chance of radical resection and oncological cure, the high recurrence tendency of craniopharyngiomas, even after apparent total removal, often makes adjuvant treatment essential. The endoscopic endonasal approach (EEA) has been recently introduced as a treatment option for both pediatric and adult craniopharyngiomas, rapidly gaining wide acceptance over the traditional transcranial approaches...
February 23, 2018: Journal of Neurosurgical Sciences
Paul Gibson, Jason D Pole, Tanya Lazor, Donna Johnston, Carol Portwine, Mariana Silva, Sarah Alexander, Lillian Sung
Using a previously developed reliable and valid treatment-related mortality (TRM) definition, our objective was to describe the proportion of children newly diagnosed with cancer experiencing TRM and to identify risk factors for TRM in a population-based cohort. We included children with cancer <19 years diagnosed and treated in Ontario who were diagnosed between 2003 and 2012. Children with cancer were identified using data in a provincial registry. Cumulative incidence of TRM was calculated where progressive disease death was considered a competing event...
February 23, 2018: Cancer Medicine
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