keyword
https://read.qxmd.com/read/38473359/frontline-and-relapsed-rhabdomyosarcoma-far-rms-clinical-trial-a-report-from-the-european-paediatric-soft-tissue-sarcoma-study-group-epssg
#1
JOURNAL ARTICLE
Julia Chisholm, Henry Mandeville, Madeleine Adams, Veronique Minard-Collin, Timothy Rogers, Anna Kelsey, Janet Shipley, Rick R van Rijn, Isabelle de Vries, Roelof van Ewijk, Bart de Keizer, Susanne A Gatz, Michela Casanova, Lisa Lyngsie Hjalgrim, Charlotte Firth, Keith Wheatley, Pamela Kearns, Wenyu Liu, Amanda Kirkham, Helen Rees, Gianni Bisogno, Ajla Wasti, Sara Wakeling, Delphine Heenen, Deborah A Tweddle, Johannes H M Merks, Meriel Jenney
The Frontline and Relapsed Rhabdomyosarcoma (FaR-RMS) clinical trial is an overarching, multinational study for children and adults with rhabdomyosarcoma (RMS). The trial, developed by the European Soft Tissue Sarcoma Study Group (EpSSG), incorporates multiple different research questions within a multistage design with a focus on (i) novel regimens for poor prognostic subgroups, (ii) optimal duration of maintenance chemotherapy, and (iii) optimal use of radiotherapy for local control and widespread metastatic disease...
February 29, 2024: Cancers
https://read.qxmd.com/read/38017132/the-dna-rna-helicase-dhx9-orchestrates-the-kdm2b-mediated-transcriptional-regulation-of-yap1-in-ewing-sarcoma
#2
JOURNAL ARTICLE
Lidia Chellini, Marzia Scarfò, Davide Bonvissuto, Claudio Sette, Maria Paola Paronetto
Ewing sarcomas (ES) are aggressive paediatric tumours of bone and soft tissues. Resistance to chemotherapy and high propensity to metastasize remain the main causes of treatment failure. Thus, identifying novel targets for alternative therapeutic approaches is urgently needed. DNA/RNA helicases are emerging as crucial regulators of many cellular processes often deregulated in cancer. Among them, DHX9 is up-regulated in ES and collaborates with EWS-FLI1 in ES transformation. We report that DHX9 silencing profoundly impacts on the oncogenic properties of ES cells...
November 28, 2023: Oncogene
https://read.qxmd.com/read/37972109/paediatric-cancer-burden-in-namibia-a-10-year-retrospective-analytical-cohort-study-of-patients-admitted-at-windhoek-central-hospital
#3
JOURNAL ARTICLE
Ndapewa Ottilie Kaholongo, Runyararo Mashingaidze-Mano
BACKGROUND: Childhood cancers are known to cause significant morbidity and mortality, and the incidence has been increasing exponentially in developing countries. Two studies performed in Namibia in 1988 and 2010 have shown changes in the pattern of paediatric cancers over the years. There is a constant need to have updated statistics on the changing trends in the frequency of different types of cancers to inform policy hence the reason for the current study. METHODS: An analytical retrospective cohort study was performed to analyse paediatric oncology cases that were admitted to the paediatric oncology unit (ward 8 west) at Windhoek Central Hospital (WCH) between 01 January 2011 and 31 December 2020...
2023: PloS One
https://read.qxmd.com/read/37846799/indeterminate-pulmonary-nodules-in-non-rhabdomyosarcoma-soft-tissue-sarcoma-a-study-of-the-european-paediatric-soft-tissue-sarcoma-study-group
#4
JOURNAL ARTICLE
Chiara Giraudo, Reineke Schoot, Liesbeth Cardoen, Roberto Stramare, Beatrice Coppadoro, Gianni Bisogno, Amine Bouhamama, Bernadette Brennan, Herve J Brisse, Daniel Orbach, Ana Coma, Pier Luigi Di Paolo, Cindy Fayard, Leigh McDonald, Salma Moalla, Carlo Morosi, Erika Pace, Vivian Tang, Max M van Noesel, Andrea Ferrari, Rick van Rijn
BACKGROUND: The aim of this study was to assess the clinical impact of indeterminate pulmonary nodules (no more than four pulmonary nodules of less than 5 mm or one nodule measuring between 5 and less than 10 mm by computed tomography [CT]) in children and adolescents with adult-type non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) at diagnosis. METHODS: Patients with NRSTS treated in 11 centers as part of the European paediatric Soft Tissue Sarcoma Study Group (EpSSG) were retrospectively assessed...
October 17, 2023: Cancer
https://read.qxmd.com/read/37470606/profile-of-193-pediatric-cancer-patients-managed-with-radiation-therapy-challenges-and-lessons-learned
#5
JOURNAL ARTICLE
Renu Madan, Shikha Goyal, Treshita Dey, Rakesh Kapoor, Nagarjun Ballari, Shiv Lal Soni, Bishan D Radotra, Deepak Bansal, Amita Trehan, Nitin James Peters, Ram Samujh
BACKGROUND: Series on radiotherapy (RT) practice in pediatric malignancies are limited in India as only a few centers practice pediatric RT, particularly under anesthesia. We aimed to study the clinical profile of pediatric cancer patients treated with RT and to analyze various challenges in pediatric RT under anesthesia. MATERIALS AND METHODS: The data were prospectively maintained in Microsoft Excel spreadsheets. Pediatric cancer patients aged 0-14 years, registered in the RT department between February 1, 2019 and July 30, 2021were analyzed...
2023: Journal of Cancer Research and Therapeutics
https://read.qxmd.com/read/37351338/optimal-delivery-of-follow-up-care-following-treatment-for-adults-treated-for-ewing-sarcoma
#6
REVIEW
Antonia Digklia, Ana Dolcan, Monika A Kucharczyk, Robin L Jones, Andrea Napolitano
Ewing sarcoma (ES) is a rare, highly malignant sarcoma. It usually presents in the second decade of life; however, patients can be diagnosed as early as newborns and as late as in their seventies. ES is most frequently found in the long bones of the extremities and the pelvis. In older patients, ES can also arise in the soft tissues. Currently, there is no standard schedule for surveillance of adult patients with ES after their initial treatment for localised disease, not only for the early detection of recurrence but also for long-term side effects...
2023: Cancer Management and Research
https://read.qxmd.com/read/37138963/outcome-and-prognostic-variables-in-childhood-rhabdomyosarcoma-rms-with-emphasis-on-impact-of-foxo1-fusions-in-non-metastatic-rms-experience-from-a-tertiary-cancer-centre-in-india
#7
JOURNAL ARTICLE
Subramaniam Ramanathan, Sneha Sisodiya, Omshree Shetty, Maya Prasad, Badira C Parambil, Sneha Shah, Mukta Ramadwar, Nehal Khanna, Siddhartha Laskar, Sajid Qureshi, Tushar Vora, Girish Chinnaswamy
While factors influencing outcomes of rhabdomyosarcoma (RMS) in developed countries have evolved from clinical characteristics to molecular profiles, similar data from developing countries are scarce. This is a single-centre analysis of outcomes in treated cases of RMS, with emphasis on prevalence, risk-migration and prognostic impact of Forkhead Box O1 (FOXO1) in non-metastatic RMS. All children with histopathologically proven RMS, treated between January 2013 and December 2018 were included. Intergroup Rhabdomyosarcoma Study-4 risk stratification was used, with treatment based on a multimodality-regimen with chemotherapy (Vincristine/Ifosfamide/Etoposide and Vincristine/Actinomycin-D/Cyclophosphamide) and appropriate local therapy...
2023: Ecancermedicalscience
https://read.qxmd.com/read/37084075/metastatic-adult-type-non-rhabdomyosarcoma-soft-tissue-sarcomas-in-children-and-adolescents-a-cohort-study-from-the-european-paediatric-soft-tissue-sarcoma-study-group
#8
JOURNAL ARTICLE
Andrea Ferrari, Daniel Orbach, Michela Casanova, Max M van Noesel, Pablo Berlanga, Bernadette Brennan, Nadege Corradini, Reineke A Schoot, Gema L Ramirez-Villar, Lisa Lyngsie Hjalgrim, Rita Alaggio, Gabriela Guillen Burrieza, Akmal Safwat, Alison L Cameron, Rick R van Rijn, Veronique Minard-Colin, Ilaria Zanetti, Gianni Bisogno, Julia C Chisholm, Johannes H M Merks
BACKGROUND: Limited data exist on the clinical behavior of pediatric non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with distant metastases at onset, and a clear standard of care has not yet been defined. METHODS: This cohort study reports on pediatric adult-type metastatic NRSTS enrolled in two concurrent prospective European studies, i.e., the randomized BERNIE study and the single-arm MTS 2008 study developed by the European paediatric Soft tissue sarcoma Study Group...
April 21, 2023: Cancer
https://read.qxmd.com/read/36910630/case-report-complete-and-durable-response-to-larotrectinib-trk-inhibitor-in-an-infant-diagnosed-with-angiosarcoma-harbouring-a-khdrbs1-ntrk3-fusion-gene
#9
Catherine Cervi, Zoltán Sápi, Gábor Bedics, Erik Zajta, Lajos Hegyi, Judit Pápay, Katalin Dezső, Edit Varga, Katalin Mudra, Csaba Bödör, Monika Csóka
Significant improvements in the survival rates of paediatric cancer have been achieved over the past decade owing to recent advances in therapeutic and diagnostic strategies. However, disease progression and relapse remain a major challenge for the clinical management of paediatric angiosarcoma. Comprehensive genomic profiling of these rare tumours using high-throughput sequencing technologies may improve patient stratification and identify actionable biomarkers for therapeutic intervention. Here, we describe the clinical, histopathological, immunohistochemical and molecular profile of a novel and precision medicine-informed case where a KHDRBS1-NTRK3 fusion determined by next-generation sequencing-based comprehensive genomic profiling led to complete and sustained remission (clinical and radiological response) in an otherwise incurable disease...
2023: Frontiers in Oncology
https://read.qxmd.com/read/36825373/-sentieri-epidemiological-study-of-residents-in-national-priority-contaminated-sites-sixth-report
#10
JOURNAL ARTICLE
Amerigo Zona, Lucia Fazzo, Marta Benedetti, Caterina Bruno, Simona Vecchi, Roberto Pasetto, Fabrizio Minichilli, Marco De Santis, Anna Maria Nannavecchia, Davide Di Fonzo, Paolo Contiero, Paolo Ricci, Lucia Bisceglia, Valerio Manno, Giada Minelli, Michele Santoro, Francesca Gorini, Carla Ancona, Salvatore Scondotto, Maria Elena Soggiu, Federica Scaini, Eleonora Beccaloni, Daniela Marsili, Marco Francesco Villa, Giovanni Maifredi, Michele Magoni, Ivano Iavarone
INTRODUCTION ADN OBJECTIVES: The Sixth Report presents the results of the "SENTIERI Project: implementation of the permanent epidemiological surveillance system of populations residing in Italian Sites of Remediation Interest", promoted and financed by the Italian Ministry of Health (Centre for Disease Control and Prevention - CCM Project 2018). The aim of this study is to update the mortality and hospitalization analyses concerning the 6,227,531 inhabitants (10.4% of the Italian population) residing in 46 contaminated sites (39 of national interest and 7 of regional interest)...
2023: Epidemiologia e Prevenzione
https://read.qxmd.com/read/36765685/targeting-the-hedgehog-pathway-in-rhabdomyosarcoma
#11
REVIEW
Patricia Zarzosa, Lia Garcia-Gilabert, Raquel Hladun, Gabriela Guillén, Gabriel Gallo-Oller, Guillem Pons, Julia Sansa-Girona, Miguel F Segura, Josep Sánchez de Toledo, Lucas Moreno, Soledad Gallego, Josep Roma
Aberrant activation of the Hedgehog (Hh) signalling pathway is known to play an oncogenic role in a wide range of cancers; in the particular case of rhabdomyosarcoma, this pathway has been demonstrated to be an important player for both oncogenesis and cancer progression. In this review, after a brief description of the pathway and the characteristics of its molecular components, we describe, in detail, the main activation mechanisms that have been found in cancer, including ligand-dependent, ligand-independent and non-canonical activation...
January 24, 2023: Cancers
https://read.qxmd.com/read/36765536/micrornas-in-the-pathogenesis-prognostication-and-prediction-of-treatment-resistance-in-soft-tissue-sarcomas
#12
REVIEW
Andrea York Tiang Teo, Vivian Yujing Lim, Valerie Shiwen Yang
Soft tissue sarcomas are highly aggressive malignant neoplasms of mesenchymal origin, accounting for less than 1% of adult cancers, but comprising over 20% of paediatric solid tumours. In locally advanced, unresectable, or metastatic disease, outcomes from even the first line of systemic treatment are invariably poor. MicroRNAs (miRNAs), which are short non-coding RNA molecules, target and modulate multiple dysregulated target genes and/or signalling pathways within cancer cells. Accordingly, miRNAs demonstrate great promise for their utility in diagnosing, prognosticating and improving treatment for soft tissue sarcomas...
January 18, 2023: Cancers
https://read.qxmd.com/read/36612064/using-evidence-based-medicine-to-support-clinical-decision-making-in-rms
#13
REVIEW
Robert S Phillips, Bas Vaarwerk, Jessica E Morgan
The foundations of evidence-based practice are the triad of patient values and preferences, healthcare professional experience, and best available evidence, used together to inform clinical decision-making. Within the field of rhabdomyosarcoma, collaborative groups such as the European Paediatric Soft Tissue Sarcoma Group (EpSSG) have worked to develop evidence to support this process. We have explored many of the key research developments within this review, including patient and public involvement, decision-making research, research into areas other than drug development, core outcome sets, reporting and dissemination of research, evidence synthesis, guideline development and clinical decision rules, research of research methodologies, and supporting research in RMS...
December 22, 2022: Cancers
https://read.qxmd.com/read/36599491/recurrence-of-primitive-germ-cell-tumour-20-years-on
#14
JOURNAL ARTICLE
Helen-Cara Younan, Georgina Keogh, Michael Seckl, Naveed Sarwar
We present a case of a woman in her 20s, with a prior history of paediatric sacrococcygeal germ cell tumour, presenting with a 6-month history of perianal pain. An MRI pelvis revealed a heterogeneous soft tissue mass causing destruction of the sacrococcygeal bone. A staging CT demonstrated metastatic deposits in the lungs and hypodense foci in the liver suspicious of metastatic disease. Her alpha-fetoprotein levels were raised and a CT-guided biopsy was in keeping with recurrent germ cell tumour. She was referred to a national centre for the treatment of germ cell tumours in adults and was rechallenged with cisplatin-based multiagent chemotherapy with a curative intent...
January 4, 2023: BMJ Case Reports
https://read.qxmd.com/read/36566574/lymph-node-metastases-in-paediatric-and-young-adult-patients-with-non-rhabdomyosarcoma-soft-tissue-sarcoma-nrsts-findings-from-children-s-oncology-group-cog-study-arst0332
#15
JOURNAL ARTICLE
Elysia Alvarez, Jiayi He, Sheri L Spunt, Andrea Hayes-Jordan, Simon C Kao, David M Parham, Lynn Million, Aaron R Weiss, Donald A Barkauskas
PURPOSE: The aim of this paper is to better define the clinical features and outcomes of young patients with non-rhabdomyosarcoma soft tissue sarcoma (NRSTS) with regional and distant lymph node (LN) metastases treated in a standardised fashion, we analysed LN involvement in COG study ARST0332, which evaluated a risk-based treatment strategy for young patients with all stages of NRSTS. PATIENTS AND METHODS: Patients <30 years old with newly diagnosed NRSTS and LN metastases enrolled on ARST0332 were studied...
November 25, 2022: European Journal of Cancer
https://read.qxmd.com/read/36440693/malignant-ectomesenchymoma-in-children-the-european-pediatric-soft-tissue-sarcoma-study-group-experience
#16
JOURNAL ARTICLE
Giuseppe Maria Milano, Daniel Orbach, Michela Casanova, Pablo Berlanga, Reineke A Schoot, Nadege Corradini, Bernadette Brennan, Gema L Ramirez-Villar, Lisa Lyngsie Hjalgrim, Max M van Noesel, Rita Alaggio, Andrea Ferrari
Malignant ectomesenchymoma (MEM) is an extremely rare soft tissue tumor typical of young children, currently included in the category of skeletal muscle malignancies and characterized by a neuroblastic component. This study describes a series of 10 patients prospectively registered in the European paediatric Soft tissue sarcoma Study Group (EpSSG) database Of the 10 cases, seven had an initial local diagnosis of rhabdomyosarcoma. All patients received chemotherapy according to rhabdomyosarcoma strategy, four had radiotherapy...
November 28, 2022: Pediatric Blood & Cancer
https://read.qxmd.com/read/36357104/intestinal-lmna-ntrk1-fused-spindle-cell-neoplasm-with-s100-and-cd34-coexpression-a-new-case
#17
JOURNAL ARTICLE
Shabina Rahim, Saif Sabah Alkhaldi, Khaledah Alasousi, Rola H Ali
Recurrent fusions involving neurotrophin tyrosine receptor kinase (NTRK) genes have been increasingly recognised in spindle cell tumours of somatic soft tissues due to the widespread use of RNA-based sequencing techniques. This heterogeneous group of neoplasms is included as an emerging entity in the current WHO Classification of Soft Tissue and Bone Tumors A subset of these tumours, associated with NTRK1 fusions, displays a distinctive phenotype in the form of monomorphic cytomorphology, patternless arrangement, perivascular and stromal hyalinisation, and CD34+/S100+/SOX10- immunoprofile...
November 10, 2022: BMJ Case Reports
https://read.qxmd.com/read/36176694/pediatric-non-rhabdomyosarcoma-soft-tissue-sarcomas-standard-of-care-and-treatment-recommendations-from-the-european-paediatric-soft-tissue-sarcoma-study-group-epssg
#18
REVIEW
Andrea Ferrari, Bernadette Brennan, Michela Casanova, Nadege Corradini, Pablo Berlanga, Reineke A Schoot, Gema L Ramirez-Villar, Akmal Safwat, Gabriela Guillen Burrieza, Patrizia Dall'Igna, Rita Alaggio, Lisa Lyngsie Hjalgrim, Susanne Andrea Gatz, Daniel Orbach, Max M van Noesel
This paper describes the standard of care for patients with non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) and the therapeutic recommendations developed by the European paediatric Soft tissue sarcoma Study Group (EpSSG). NRSTS form a very mixed group of mesenchymal extraskeletal malignancies. Their rarity, heterogeneity, and aggressiveness make the management of children and adolescents with these tumors complex and challenging. The overall cure rate for patients with NRSTS is around 70%, but survival depends on several prognostic variables, such as histotype and tumor grade, extent of disease and stage, tumor size, and tumor site...
2022: Cancer Management and Research
https://read.qxmd.com/read/36050131/long-term-follow-up-of-detaenial-sigmoid-neobladder-reconstruction-for-paediatric-patients-with-bladder-and-prostate-rhabdomyosarcoma-technique-and-results-from-a-single-high-volume-centre
#19
JOURNAL ARTICLE
Peng Xu, Chunxiao Chen, Binshen Chen, Enguang Bi, Wei Du, Ning Jiang, Zhe Liu, Hekui Lan, Manming Cao, Yazhen Liu, Jingwen Huang, Haiyan Shen, Cunrong Liu, Chunxiao Liu, Abai Xu
BACKGROUND: Rhabdomyosarcoma (RMS) is the most common paediatric soft-tissue sarcoma. Approximately 15-20% of RMS cases arise from the bladder and prostate (B/P). The optimal treatment strategy for B/P RMS remains unclear. OBJECTIVE: To retrospectively evaluate the applicability of our procedure performed to treat paediatric patients with B/P RMS. DESIGN, SETTING, AND PARTICIPANTS: This is a retrospective analysis from a single tertiary referral hospital...
November 2022: European Urology
https://read.qxmd.com/read/35839732/molecular-testing-of-rhabdomyosarcoma-in-clinical-trials-to-improve-risk-stratification-and-outcome-a-consensus-view-from-european-paediatric-soft-tissue-sarcoma-study-group-children-s-oncology-group-and-cooperative-weichteilsarkom-studiengruppe
#20
REVIEW
Simone Hettmer, Corinne M Linardic, Anna Kelsey, Erin R Rudzinski, Christian Vokuhl, Joanna Selfe, Olivia Ruhen, Jack F Shern, Javed Khan, Alexander R Kovach, Philip J Lupo, Susanne A Gatz, Beat W Schäfer, Samuel Volchenboum, Véronique Minard-Colin, Ewa Koscielniak, Douglas S Hawkins, Gianni Bisogno, Monika Sparber-Sauer, Rajkumar Venkatramani, Johannes H M Merks, Janet Shipley
Rhabdomyosarcomas (RMSs) are the most common soft tissue sarcomas in children/adolescents less than 18 years of age with an annual incidence of 1-2/million. Inter/intra-tumour heterogeneity raise challenges in clinical, pathological and biological research studies. Risk stratification in European and North American clinical trials previously relied on clinico-pathological features, but now, incorporates PAX3/7-FOXO1-fusion gene status in the place of alveolar histology. International working groups propose a coordinated approach through the INternational Soft Tissue SaRcoma ConsorTium to evaluate the specific genetic abnormalities and generate and integrate molecular and clinical data related to patients with RMS across different trial settings...
September 2022: European Journal of Cancer
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