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https://www.readbyqxmd.com/read/29784668/centrosome-linker-induced-tetraploid-segregation-errors-link-rhabdoid-phenotypes-and-lethal-colorectal-cancers
#1
Andrea Remo, Erminia Manfrin, Pietro Parcesepe, Alberto Ferrarini, Hye Seung Han, Mickys Ugnius, Carmelo Laudanna, Michele Simbolo, Donatella Malanga, Duarte Mendes Oliveira, Elisabetta Baritono, Tommaso Colangelo, Lina Sabatino, Jacopo Giuliani, Enrico Molinari, Marianna Garonzi, Luciano Xumerle, Massimo Delledonne, Guido Giordano, Claudio Ghimenton, Fortunato Lonardo, Fulvio D'angelo, Federica Grillo, Luca Mastracci, Giuseppe Viglietto, Michele Ceccarelli, Vittorio Colantuoni, Aldo Scarpa, Massimo Pancione
Centrosome anomalies contribute to tumorigenesis but it remains unclear how they are generated in lethal cancer phenotypes. Here, it is demonstrated that human microsatellite instable (MSI) and BRAF(V600E) mutant colorectal cancers with a lethal rhabdoid phenotype are characterized by inactivation of centrosomal functions. A splice site mutation that causes an unbalanced dosage of rootletin (CROCC), a centrosomal-linker component required for centrosome cohesion and separation at the chromosome 1p36.13 locus, resulted in abnormally shaped centrosomes in rhabdoid cells from human colon tissues...
May 21, 2018: Molecular Cancer Research: MCR
https://www.readbyqxmd.com/read/29781567/localized-vaginal-uterine-rhabdomyosarcoma-results-of-a-pooled-analysis-from-four-international-cooperative-groups
#2
Veronique Minard-Colin, David Walterhouse, Gianni Bisogno, Helene Martelli, James Anderson, David A Rodeberg, Andrea Ferrari, Meriel Jenney, Suzanne Wolden, Gianluca De Salvo, Carola Arndt, Johannes H M Merks, Soledad Gallego, Dominique Schwob, Christine Haie-Meder, Christophe Bergeron, Michael C G Stevens, Odile Oberlin, Douglas Hawkins
BACKGROUND: Vaginal/uterine rhabdomyosarcoma (VU RMS) is one of the most favorable RMS sites. To determine the optimal therapy, the experience of four cooperative groups (Children's Oncology Group [COG], International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumor Group [MMT], Italian Cooperative Soft Tissue Sarcoma Group [ICG], and European pediatric Soft tissue sarcoma Study Group [EpSSG]) was analyzed. PROCEDURE: From 1981 to 2009, 237 patients were identified...
May 21, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29780794/pediatric-scurvy-when-contemporary-eating-habits-bring-back-the-past
#3
Alice Brambilla, Cristina Pizza, Donatella Lasagni, Lucia Lachina, Massimo Resti, Sandra Trapani
Vitamin C deficiency is anecdotal in developed countries, mainly associated with underling clinical morbidities as autism or neurological impairment. Chronic insufficient dietary supply is responsible for vascular fragility and impaired bone formation, resulting in gingival bleeding, petechial lesions, articular and bone pain or limb swelling. Children may present anorexia, irritability, failure to thrive, limping or refusal to walk. Accordingly, pediatric scurvy is frequently misdiagnosed with osteomyelitis, septic arthritis, bone and soft tissue tumor, leukemia, bleeding disorders, and rheumatologic conditions...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29778284/-clinical-and-epidemiological-profile-of-pediatric-uveitis-course-of-inflammatory-uveitis-treated-with-anti-tnf-alpha
#4
D Osswald, A C Rameau, C Speeg-Schatz, J Terzic, A Sauer
INTRODUCTION: Uveitis is the leading cause of acquired childhood blindness with a prevalence of 30 cases per 100,000 inhabitants. There are multiple causes ; nevertheless, there is no standardized etiological assessment. The goal of our study is to define an epidemiological and clinical profile of uveitis diagnosed in a university hospital and their course when treated with anti-tumor necrosis factor (TNF) α. PATIENTS AND METHODS: All cases of uveitis under 18 years old, from 1994 to 2016, were included...
May 16, 2018: Journal Français D'ophtalmologie
https://www.readbyqxmd.com/read/29777888/primary-intracranial-rhabdomyosarcoma-in-the-cerebellopontine-angle-resected-after-preoperative-embolization
#5
Keisuke Yoshida, Tomoru Miwa, Takenori Akiyama, Masaaki Nishimoto, Dai Kamamoto, Kazunari Yoshida
BACKGROUND: Primary intracranial rhabdomyosarcoma is a very rare malignant tumor. Due to moderately vascular and firm characteristics of the tumor, complete removal without complications is often difficult. Moreover, in pediatric patients, the volume of total blood is less than adults and minimal intraoperative hemorrhage is desirable. CASE DESCRIPTION: A 6-year-old boy presented with ataxia and was found to have a large cerebellopontine angle tumor and hydrocephalus...
May 16, 2018: World Neurosurgery
https://www.readbyqxmd.com/read/29776675/risk-factors-for-near-miss-events-and-safety-incidents-in-pediatric-radiation-therapy
#6
Nimrah Baig, Jiangxia Wang, Shereef Elnahal, Todd McNutt, Jean Wright, Theodore DeWeese, Stephanie Terezakis
BACKGROUND AND PURPOSE: Factors contributing to safety- or quality-related incidents (e.g. variances) in children are unknown. We identified clinical and RT treatment variables associated with risk for variances in a pediatric cohort. MATERIALS AND METHODS: Using our institution's incident learning system, 81 patients age ≤21 years old who experienced variances were compared to 191 pediatric patients without variances. Clinical and RT treatment variables were evaluated as potential predictors for variances using univariate and multivariate analyses...
May 15, 2018: Radiotherapy and Oncology: Journal of the European Society for Therapeutic Radiology and Oncology
https://www.readbyqxmd.com/read/29776650/signaling-pathway-deregulation-and-molecular-alterations-across-pediatric-medulloblastomas
#7
REVIEW
B Lhermitte, A F Blandin, A Coca, E Guerin, A Durand, N Entz-Werlé
Medulloblastomas (MBs) account for 15% of brain tumors in children under the age of 15. To date, the overall 5-year survival rate for all children is only around 60%. Recent advances in cancer genomics have led to a fundamental change in medulloblastoma classification and is evolving along with the genomic discoveries, allowing to regularly reclassify this disease. The previous molecular classification defined 4 groups (WNT-activated MB, SHH-activated MB and the groups 3 and 4 characterized partially by NMYC and MYC driven MBs)...
May 15, 2018: Neuro-Chirurgie
https://www.readbyqxmd.com/read/29774098/establishment-of-primary-cell-culture-and-an-intracranial-xenograft-model-of-pediatric-ependymoma-a-prospect-for-therapy-development-and-understanding-of-tumor-biology
#8
Lorena Favaro Pavon, Tatiana Tais Sibov, Silvia Regina Caminada de Toledo, Daniela Mara de Oliveira, Francisco Romero Cabral, Jean Gabriel de Souza, Pamela Boufleur, Luciana C Marti, Jackeline Moraes Malheiros, Edgar Ferreira da Cruz, Fernando F Paiva, Suzana M F Malheiros, Manoel A de Paiva Neto, Alberto Tannús, Sérgio Mascarenhas de Oliveira, Nasjla Saba Silva, Andrea Maria Cappellano, Antonio Sérgio Petrilli, Ana Marisa Chudzinski-Tavassi, Sérgio Cavalheiro
Background: Ependymoma (EPN), the third most common pediatric brain tumor, is a central nervous system (CNS) malignancy originating from the walls of the ventricular system. Surgical resection followed by radiation therapy has been the primary treatment for most pediatric intracranial EPNs. Despite numerous studies into the prognostic value of histological classification, the extent of surgical resection and adjuvant radiotherapy, there have been relatively few studies into the molecular and cellular biology of EPNs...
April 24, 2018: Oncotarget
https://www.readbyqxmd.com/read/29773567/balanced-steady-state-free-precession-sequence-ciss-fiesta-3d-driven-equilibrium-radiofrequency-reset-pulse-increases-the-diagnostic-yield-for-spinal-drop-metastases-in-children-with-brain-tumors
#9
K Buch, P Caruso, D Ebb, S Rincon
BACKGROUND AND PURPOSE: Identification of spinal drop metastases is important in the staging and management of pediatric patients with primary brain tumors. Our aim was to assess the diagnostic utility of the balanced steady-state free precession (bSSFP) sequence (CISS/FIESTA/3D driven equilibrium radiofrequency reset pulse) for the detection of spinal drop metastases in pediatric patients with primary intracranial tumors. MATERIALS AND METHODS: This was a retrospective study of 44 pediatric patients with primary intracranial tumors undergoing MR imaging spine evaluation for drop metastases before radiation treatment...
May 17, 2018: AJNR. American Journal of Neuroradiology
https://www.readbyqxmd.com/read/29770995/adolescent-and-young-adult-oncology-patients-in-france-heterogeneity-in-pathways-of-care
#10
Emmanuel Desandes, Laurence Brugières, Florence Molinié, Gautier Defossez, Patricia Delafosse, Karine Jehannin-Ligier, Michel Velten, Brigitte Trétarre, Brice Amadéo, Emilie Marrer, Anne-Sophie Woronoff, Olivier Ganry, Alain Monnereau, Tania d'Almeida, Xavier Troussard, Laetitia Daubisse-Marliac, Simona Bara, Anne-Valérie Guizard, Isabelle Baldi, Guy Launoy, Jacqueline Clavel, Brigitte Lacour
BACKGROUND: In order to evaluate at the population level the impact of the actions developed in France since 2004 to organize the care of adolescents and young adults (AYAs) with cancer, we conducted the present study to provide an unbiased view of the pathway of care of these patients. METHODS: Using a population-based registry, we conducted a review of all cases of cancer diagnosed during 2012 and 2013 in 15- to 24-year-old patients living in nineteen French administrative areas...
May 17, 2018: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/29770223/profound-hearing-loss-following-surgery-in-pediatric-patients-with-posterior-fossa-low-grade-glioma
#11
Yahya Ghazwani, Ibrahim Qaddoumi, Johnnie K Bass, Shengjie Wu, Jason Chiang, Frederick Boop, Amar Gajjar, Zsila Sadighi
Background: Hearing loss may occur in patients with posterior fossa low-grade glioma who undergo surgery. Methods: We retrospectively reviewed 217 patients with posterior fossa low-grade glioma, including 115 for whom results of hearing tests performed after surgery and before chemotherapy or radiation therapy were available. We explored the association of UHL with age at diagnosis, sex, race, tumor location, extent of resection, posterior fossa syndrome, ventriculoperitoneal shunt placement, and histology...
May 2018: Neuro-oncology Practice
https://www.readbyqxmd.com/read/29768295/requirement-for-head-magnetic-resonance-imaging-in-children-who-present-to-the-emergency-department-with-acute-nontraumatic-visual-disturbance
#12
Jeong-Yong Lee, Jung-Heon Kim, Hyung-Rae Cho, Jong-Seung Lee, Jeong-Min Ryu, Mi-Sun Yum, Tae-Sung Ko
OBJECTIVES: This study aimed to investigate the clinical features and head magnetic resonance imaging (MRI) findings in children who presented to the emergency department with acute nontraumatic visual disturbance and to study related clinical factors for discovering positive lesions on head MRI. METHODS: We performed a retrospective study of 1-month to 15-year-old children who underwent head MRI as an evaluation for acute nontraumatic visual disturbance as a chief complaint in our pediatric emergency department between March 2010 and March 2015...
May 15, 2018: Pediatric Emergency Care
https://www.readbyqxmd.com/read/29768074/cancer-treatment-and-end-of-life-care
#13
Sudha Sinha, Jaskirt Kaur Matharu, Jean Jacob, Gayatri Palat, Eva Brun, Thomas Wiebe, Mikael Segerlantz
AIM: To study to what extent tumor-specific treatment (chemo- or radiotherapy) was given during the last 30 days in life and to examine how many of the patients were referred to a specialized palliative care unit (PCU), at a low-resource governmental hospital in India. PATIENTS AND METHODS: Medical records of adult cancer patients deceased between April 1 and May 31 in 2016, and pediatric cancer patients deceased between April 1 and September 30 in 2016 were collected...
May 16, 2018: Journal of Palliative Medicine
https://www.readbyqxmd.com/read/29766555/hand-foot-skin-reaction-related-to-use-of-the-multikinase-inhibitor-sorafenib-and-hard-orthotics
#14
Ayan Kusari, Jenna Borok, Allison M Han, Alix Jessika Valderrama, Sheila Fallon Friedlander
Hand-foot-skin reaction is a distinct clinical condition arising in association with the use of multikinase inhibitors, including sorafenib. Because multikinase inhibitors are increasingly being used in children with cancer, recognition of this previously unfamiliar condition is of importance to pediatric dermatologists. We describe the diagnosis and successful treatment of a case of hand-foot-skin reaction in a child taking sorafenib for an unresectable desmoid tumor.
May 15, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29766548/dermal-non-neural-granular-cell-tumor-in-a-3-year-old-child
#15
Bilaal Kabir, Michele Ramien, Mofeedah Al Shammary, Joseph de Nanassy, Dina El Demellawy
Dermal non-neural granular cell tumors, also known as primitive polypoid granular cell tumors, are a rare group of distinct cutaneous non-neural granular cell tumors. Pediatric cases are rare, and to the best of our knowledge, we report the youngest patient with dermal non-neural granular cell tumors.
May 16, 2018: Pediatric Dermatology
https://www.readbyqxmd.com/read/29766265/surgery-for-subependymal-giant-cell-astrocytomas-in-children-with-tuberous-sclerosis-complex
#16
Martine Fohlen, Sarah Ferrand-Sorbets, Olivier Delalande, Georg Dorfmüller
OBJECTIVE: Subependymal giant cell astrocytomas (SEGAs) are low-grade intraventricular glial tumors that develop in 10-15% of patients with tuberous sclerosis complex; they often cause hydrocephalus and are potentially accessible to a surgical treatment. Our aim is to evaluate morbidity and results after surgery in symptomatic and asymptomatic patients. METHOD: We present a retrospective series of 18 pediatric patients operated on for SEGA between 2006 and 2016 at our institution...
May 15, 2018: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/29765823/the-natural-growth-rate-of-residual-juvenile-angiofibroma
#17
Nicholas R Rowan, Amanda L Stapleton, Molly E Heft-Neal, Paul A Gardner, Carl H Snyderman
Objectives  Examine the postoperative growth rate of residual juvenile angiofibroma (JA) in a large series of patients relative to pediatric growth parameters and other prognostic factors. Establish an algorithm for postoperative surveillance of patients with JA. Design  Retrospective case series. Setting  Tertiary referral academic center. Participants  Pediatric patients undergoing surgical resection of JA between September 2005 and June 2015. Main Outcome Measures  Postoperative recurrence and tumor growth rates...
June 2018: Journal of Neurological Surgery. Part B, Skull Base
https://www.readbyqxmd.com/read/29765083/adeno-associated-virus-vector-mediated-interleukin-10-induction-prevents-vascular-inflammation-in-a-murine-model-of-kawasaki-disease
#18
Jun Nakamura, Sachiko Watanabe, Hiroaki Kimura, Motoi Kobayashi, Tadayoshi Karasawa, Ryo Kamata, Fumitake Usui-Kawanishi, Ai Sadatomo, Hiroaki Mizukami, Noriko Nagi-Miura, Naohito Ohno, Tadashi Kasahara, Seiji Minota, Masafumi Takahashi
Kawasaki disease (KD), which is the leading cause of pediatric heart disease, is characterized by coronary vasculitis and subsequent aneurysm formation. Although intravenous immunoglobulin therapy is effective for reducing aneurysm formation, a certain number of patients are resistant to this therapy. Because interleukin-10 (IL-10) was identified as a negative regulator of cardiac inflammation in a murine model of KD induced by Candida albicans water-soluble fraction (CAWS), we investigated the effect of IL-10 supplementation in CAWS-induced vasculitis...
May 15, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29764855/magnetic-resonance-imaging-of-tumor-associated-macrophages-clinical-translation
#19
Maryam Aghighi, Ashok Joseph Theruvath, Anuj Pareek, Laura Pisani, Raphael Alford, Anne Monika Muehe, Tarsheen K Sethi, Samantha J Holdsworth, Florette K Hazard, Dita Gratzinger, Sandra Luna-Fineman, Ranjana H Advani, Sheri L Spunt, Heike E Daldrup-Link
PURPOSE: Tumor associated macrophages (TAM) in malignant tumors have been linked to tumor aggressiveness and represent a new target for cancer immunotherapy. As new TAM-targeted immunotherapies are entering clinical trials, it is important to detect and quantify TAM with non-invasive imaging techniques. The purpose of this study was to determine if ferumoxytol-enhanced MRI can detect TAM in lymphomas and bone sarcomas of pediatric patients and young adults. EXPERIMENTAL DESIGN: In a first-in-patient , IRB-approved prospective clinical trial, 25 pediatric and young adult patients with lymphoma or bone sarcoma underwent ferumoxytol-enhanced MRI...
May 15, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29764494/precision-medicine-becomes-reality-tumor-type-agnostic-therapy
#20
REVIEW
Li Yan, Wei Zhang
Precision medicine just witnessed two breakthroughs in oncology in 2017. Pembrolizumab (Keytruda), Merck's anti-programmed cell death-1 (PD-1) monoclonal antibody (mAb), received accelerated approval in May 2017 by the US Food and Drug Administration for the treatment of adult and pediatric patients with unresectable or metastatic solid tumors that have been identified as having microsatellite instability-high (MSI-H) or deficient DNA mismatch repair (dMMR). Shortly after, nivolumab (Opdivo), Bristol-Myers Squibb's anti-PD-1 mAb, gained an accelerated approval in August 2017 for adult and pediatric patients with MSI-H or dMMR metastatic colorectal cancer that has progressed after standard chemotherapy...
March 31, 2018: Cancer communications
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