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https://www.readbyqxmd.com/read/28208865/adult-neuroblastoma-case-report-and-literature-review
#1
Vishwapriya Mahadev Godkhindi, Maheboob M Basade, Kamran Khan, Kiran Thorat
Neuroblastoma is the most common solid malignant neoplasm in the paediatric age group; accounting for 7% of all childhood malignancies, but neuroblastoma in adult, is a rare occurrence, finding mention in aeons of medical literature with an overall incidence of 1 in 10 million adults/year. We report the case of a 24-year-old male patient presenting with the complaints of progressive abdominal distention of 2 months duration. Multiple Detector Computed Tomography (MD-CT) of abdomen revealed a huge enhancing mass in the retroperitoneum abutting the left kidney, and a subsequent CT-guided biopsy and immunohistochemistry confirmed the diagnosis of ganglioneuroblastoma...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28205365/paratesticular-rhabdomyosarcoma-in-children-and-adolescents-outcome-and-patterns-of-relapse-when-utilizing-a-nonsurgical-strategy-for-lymph-node-staging-report-from-the-international-society-of-paediatric-oncology-siop-malignant-mesenchymal-tumour-89-and-95
#2
Timothy Rogers, Veronique Minard-Colin, Nathalie Cozic, Meriel Jenney, Johannes H M Merks, Soledad Gallego, Christine Devalck, Mark N Gaze, Anna Kelsey, Odile Oberlin, Mike Stevens, Richard D Spicer, Christophe Bergeron, Helene Martelli
PURPOSE: To report the results from International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumors studies (MMT 89 and 95) of males with nonmetastatic paratesticular rhabdomyosarcoma. METHODS: From 1989 to 2003, 159 patients were included. Radical inguinal orchidectomy was recommended, but retroperitoneal lymph node (LN) assessment was based on imaging alone. The treatment was stratified by stage (SIOP tumor-node-metastasis staging system) and histology...
February 16, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28187790/retinoic-acid-and-tgf-%C3%AE-signalling-cooperate-to-overcome-mycn-induced-retinoid-resistance
#3
David J Duffy, Aleksandar Krstic, Melinda Halasz, Thomas Schwarzl, Anja Konietzny, Kristiina Iljin, Desmond G Higgins, Walter Kolch
BACKGROUND: Retinoid therapy is widely employed in clinical oncology to differentiate malignant cells into their more benign counterparts. However, certain high-risk cohorts, such as patients with MYCN-amplified neuroblastoma, are innately resistant to retinoid therapy. Therefore, we employed a precision medicine approach to globally profile the retinoid signalling response and to determine how an excess of cellular MYCN antagonises these signalling events to prevent differentiation and confer resistance...
February 10, 2017: Genome Medicine
https://www.readbyqxmd.com/read/28182813/second-malignant-neoplasms-in-childhood-cancer-survivors-treated-in-a-tertiary-paediatric-oncology-centre
#4
Jia Wei Lim, Frances Sh Yeap, Yiong Huak Chan, Allen Ej Yeoh, Thuan Chong Quah, Poh Lin Tan
Introduction: One of the most feared complications of childhood cancer treatment is second malignant neoplasms (SMNs). This study evaluates the incidence, risk factors and outcomes of SMNs in a tertiary paediatric oncology centre in Singapore. Materials and Methods: A retrospective review was conducted on patients diagnosed with childhood cancer under age 21 and treated at the National University Hospital, Singapore, from January 1990 to 15 April 2012. Case records of patients with SMNs were reviewed. Results: We identified 1124 cases of childhood cancers with a median follow-up of 3...
January 2017: Annals of the Academy of Medicine, Singapore
https://www.readbyqxmd.com/read/28164435/childhood-neoplasms-presenting-at-autopsy-a-20-year-experience
#5
Victoria A Bryant, John Booth, Liina Palm, Michael Ashworth, Thomas S Jacques, Neil J Sebire
OBJECTIVE: The aims of the review are to establish the number of undiagnosed neoplasms presenting at autopsy in a single centre and to determine the incidence and most common causes of sudden unexpected death due to neoplasia in infancy and childhood (SUDNIC). DESIGN: Retrospective observational study of paediatric autopsies performed on behalf of Her Majesty's Coroner over a 20-year period (1996-2015; n = 2,432). Neoplasms first diagnosed at autopsy were identified from an established database and cases meeting the criteria for sudden unexpected death were further categorised...
February 6, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28135773/epidemiological-study-of-paediatric-germ-cell-tumours-revealed-the-incidence-and-distribution-that-was-expected-but-a-low-mortality-rate
#6
Madeline Evers, Catherine Rechnitzer, Niels Graem, Peder Skov Wehner, Henrik Schroeder, Steen Rosthoej, Christiane Hammershaimb Mosbech, Christina Engel Hoei-Hansen, Astrid Sehested, Taryn D Treger, Jesper Brok
AIM: Germ cell tumours (GCTs) are a rare heterogeneous tumour group derived from primordial germ cells, which can be benign or malignant and occur in the gonads or extragonadally. This study mapped the paediatric GCTs in Denmark from 1984-2013 to study the incidence and outcome. METHODS: We identified paediatric GCTs from the Danish Childhood Cancer and National Pathology Registries and reviewed the case records for patient characteristics, tumour characteristics and clinical outcome...
January 30, 2017: Acta Paediatrica
https://www.readbyqxmd.com/read/28127386/the-surgical-pathology-laboratory-in-mwanza-tanzania-a-survey-on-the-reproducibility-of-diagnoses-after-the-first-years-of-autonomous-activity
#7
R Tumino, P F Rambau, F Callea, L Leoncini, R Monaco, J Kahima, V Stracca Pansa, L Viberti, D Amadori, P Giovenali, K A Mteta
BACKGROUND: In 2000, an Italian non-governmental organisation (NGO) began a 9-year project to establish a surgical pathology laboratory at the Bugando Medical Centre (BMC) in Mwanza, Tanzania, a country with a low Human Development Index (HDI), and as of 2009, the laboratory was operating autonomously. The present survey aims to evaluate the reproducibility of histological and cytological diagnoses assigned in the laboratory's early years of autonomous activity. We selected a random sample of 196 histological and cytological diagnoses issued in 2010-2011 at the BMC surgical pathology laboratory...
2017: Infectious Agents and Cancer
https://www.readbyqxmd.com/read/28110411/development-of-the-siope-dipg-network-registry-and-imaging-repository-a-collaborative-effort-to-optimize-research-into-a-rare-and-lethal-disease
#8
Sophie E M Veldhuijzen van Zanten, Joshua Baugh, Brooklyn Chaney, Dennis De Jongh, Esther Sanchez Aliaga, Frederik Barkhof, Johan Noltes, Ruben De Wolf, Jet Van Dijk, Antonio Cannarozzo, Carin M Damen-Korbijn, Jan A Lieverst, Niclas Colditz, Marion Hoffmann, Monika Warmuth-Metz, Brigitte Bison, David T W Jones, Dominik Sturm, Gerrit H Gielen, Chris Jones, Esther Hulleman, Raphael Calmon, David Castel, Pascale Varlet, Géraldine Giraud, Irene Slavc, Stefaan Van Gool, Sandra Jacobs, Filip Jadrijevic-Cvrlje, David Sumerauer, Karsten Nysom, Virve Pentikainen, Sanna-Maria Kivivuori, Pierre Leblond, Natasha Entz-Werle, Andre O von Bueren, Antonis Kattamis, Darren R Hargrave, Péter Hauser, Miklos Garami, Halldora K Thorarinsdottir, Jane Pears, Lorenza Gandola, Giedre Rutkauskiene, Geert O Janssens, Ingrid K Torsvik, Marta Perek-Polnik, Maria J Gil-da-Costa, Olga Zheludkova, Liudmila Shats, Ladislav Deak, Lidija Kitanovski, Ofelia Cruz, Andres Morales La Madrid, Stefan Holm, Nicolas Gerber, Rejin Kebudi, Richard Grundy, Enrique Lopez-Aguilar, Marta Zapata-Tarres, John Emmerik, Tim Hayden, Simon Bailey, Veronica Biassoni, Maura Massimino, Jacques Grill, William P Vandertop, Gertjan J L Kaspers, Maryam Fouladi, Christof M Kramm, Dannis G van Vuurden
Diffuse intrinsic pontine glioma (DIPG) is a rare and deadly childhood malignancy. After 40 years of mostly single-center, often non-randomized trials with variable patient inclusions, there has been no improvement in survival. It is therefore time for international collaboration in DIPG research, to provide new hope for children, parents and medical professionals fighting DIPG. In a first step towards collaboration, in 2011, a network of biologists and clinicians working in the field of DIPG was established within the European Society for Paediatric Oncology (SIOPE) Brain Tumour Group: the SIOPE DIPG Network...
January 21, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28078429/aims-and-tasks-in-parental-caregiving-for-children-receiving-palliative-care-at-home-a-qualitative-study
#9
Lisa M Verberne, Marijke C Kars, Antoinette Y N Schouten-van Meeteren, Diederik K Bosman, Derk A Colenbrander, Martha A Grootenhuis, Johannes J M van Delden
: In paediatric palliative care (PPC), parents are confronted with increasing caregiving demands. More children are cared for at home, and the need for PPC of children is lengthened due to technical and medical improvements. Therefore, a clear understanding of the content of parental caregiving in PPC becomes increasingly important. The objective is to gain insight into parental caregiving based on the lived experience of parents with a child with a life-limiting disease. An interpretative qualitative study using thematic analysis was performed...
January 11, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28070671/place-of-death-of-children-with-complex-chronic-conditions-cross-national-study-of-11-countries
#10
Cecilia Håkanson, Joakim Öhlén, Ulrika Kreicbergs, Marylou Cardenas-Turanzas, Donna M Wilson, Martin Loucka, Sandra Frache, Lucia Giovannetti, Wayne Naylor, YongJoo Rhee, Miguel Ruiz Ramos, Joan Teno, Kim Beernaert, Luc Deliens, Dirk Houttekier, Joachim Cohen
: Cross-national understanding of place of death is crucial for health service systems for their provision of efficient and equal access to paediatric palliative care. The objectives of this population-level study were to examine where children with complex chronic conditions (CCC) die and to investigate associations between places of death and sex, cause of death and country. The study used death certificate data of all deceased 1- to 17-year-old children (n = 40,624) who died in 2008, in 11 European and non-European countries...
January 9, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28069156/effectiveness-of-a-relaxation-intervention-progressive-muscle-relaxation-and-guided-imagery-techniques-to-reduce-anxiety-and-improve-mood-of-parents-of-hospitalized-children-with-malignancies-a-randomized-controlled-trial-in-republic-of-cyprus-and-greece
#11
Theologia Tsitsi, Andreas Charalambous, Evridiki Papastavrou, Vasilios Raftopoulos
PURPOSE: To explore the effect of Progressive Muscle Relaxation (PMR) and Guided Imagery (GI),in reducing anxiety levels among parents of children diagnosed with any type of malignancy receiving active treatment at a Paediatric Oncology Unit in Republic of Cyprus and in Greece. METHOD: A randomized non-blinded control trial was conducted between April 2012 to October 2013, at two public paediatric hospitals. Fifty four eligible parents of children hospitalized with a malignancy were randomly assigned to the intervention (PMR and GI) (n = 29) and a control group (n = 25)...
February 2017: European Journal of Oncology Nursing: the Official Journal of European Oncology Nursing Society
https://www.readbyqxmd.com/read/28061374/catecholamines-profiles-at-diagnosis-increased-diagnostic-sensitivity-and-correlation-with-biological-and-clinical-features-in-neuroblastoma-patients
#12
Iedan R N Verly, André B P van Kuilenburg, Nico G G M Abeling, Susan M I Goorden, Marta Fiocco, Frédéric M Vaz, Max M van Noesel, C Michel Zwaan, GertJan L Kaspers, Johannes H M Merks, Huib N Caron, Godelieve A M Tytgat
INTRODUCTION: Neuroblastoma (NBL) accounts for 10% of the paediatric malignancies and is responsible for 15% of the paediatric cancer-related deaths. Vanillylmandelic acid (VMA) and homovanillic acid (HVA) are most commonly analysed in urine of NBL patients. However, their diagnostic sensitivity is suboptimal (82%). Therefore, we performed in-depth analysis of the diagnostic sensitivity of a panel of urinary catecholamine metabolites. PATIENTS AND METHODS: Retrospective study of a panel of 8 urinary catecholamine metabolites (VMA, HVA, 3-methoxytyramine [3MT], dopamine, epinephrine, metanephrine, norepinephrine and normetanephrine [NMN]) from 301 NBL patients at diagnosis...
February 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28054101/management-of-bilateral-wilms-tumours
#13
REVIEW
Alastair J W Millar, Sharon Cox, Alan Davidson
Wilms tumour is named after Max Wilms. It is an embryonal tumour derived from the metanephros. It is the commonest childhood renal tumour and the third commonest paediatric malignancy. Synchronous bilateral Wilms tumours (BWT) represent 4-7% of all Wilms tumours (WT) and present at a younger age than unilateral Wilms tumours. At least 10% of synchronous BWTs have unfavourable histology, and up to 22% are associated with genitourinary abnormalities, aniridia, WAGR syndrome, Denys-Drash Syndrome, hemihypertrophy, or one of the other overgrowth syndromes...
January 4, 2017: Pediatric Surgery International
https://www.readbyqxmd.com/read/28050470/neurofibromatosis-type-1-presenting-with-ophthalmic-features-a-case-series
#14
Gunjan Jain, Vaibhav Kumar Jain, Indra Kumar Sharma, Reena Sharma, Neeraj Saraswat
Neurofibromatosis type 1 (NF-1) is an autosomal dominant disorder involving multiple systems and affects approximately 1 out of 3000 persons. Ocular manifestations are lisch nodules, plexiform neurofibroma, optic pathway gliomas. The proper diagnosis of NF-1 is a crucial task for a clinician due to the various clinical manifestations including vision and life threatening malignancies in few patients, which may arise in the different phases of life. The authors report three cases of NF-1, presenting with ophthalmic symptoms in teenager boys...
November 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28035744/impact-of-fusion-gene-status-versus-histology-on-risk-stratification-for-rhabdomyosarcoma-retrospective-analyses-of-patients-on-uk-trials
#15
Joanna Selfe, David Olmos, Reem Al-Saadi, Khin Thway, Julia Chisholm, Anna Kelsey, Janet Shipley
BACKGROUND: Long-term toxicities from current treatments are a major issue in paediatric cancer. Previous studies, including our own, have shown prognostic value for the presence of PAX3/7-FOXO1 fusion genes in rhabdomyosarcoma (RMS). It is proposed to introduce PAX3/7-FOXO1 positivity as a component of risk stratification, rather than alveolar histology, in future clinical trials. PROCEDURE: To assess the potential impact of this reclassification, we have determined the changes to risk category assignment of 210 histologically reviewed patients treated in the UK from previous malignant mesenchymal tumour clinical trials for non-metastatic RMS based on identification of PAX3/7-FOXO1 by fluorescence in situ hybridisation and/or reverse transcription PCR...
December 30, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28028390/spectrum-of-childhood-and-adolescent-ovarian-tumors-in-india-25-years-experience-at-a-single-institution
#16
Ruchi Rathore, Sonal Sharma, Deepshikha Arora
BACKGROUND: Ovarian tumour in children and adolescent girls form an uncommon but important part of gynaecological malignancies. They account for 1% of all the childhood malignancies and 8% of all abdominal tumours in children. Since the ovarian cysts are thought to arise from mature follicles, these tumours were considered to be infrequent in the paediatric population. AIM: The rarity of this condition prompted us to conduct this study and share our experience on the incidence and clinicopathological features of different ovarian tumours in girls up to 20 years of age observed in last 25 years at a single tertiary care hospital...
December 15, 2016: Open Access Macedonian Journal of Medical Sciences
https://www.readbyqxmd.com/read/28011926/evaluation-of-age-dependent-treatment-strategies-for-children-and-young-adults-with-pineoblastoma-analysis-of-pooled-european-society-for-paediatric-oncology-siop-e-and-us-head-start-data
#17
Martin Mynarek, Barry Pizer, Christelle Dufour, Dannis van Vuurden, Miklos Garami, Maura Massimino, Jason Fangusaro, Tom Davidson, Maria Joao Gil-da-Costa, Jaroslav Sterba, Martin Benesch, Nicolas Gerber, B Ole Juhnke, Robert Kwiecien, Torsten Pietsch, Marcel Kool, Steve Clifford, David W Ellison, Felice Giangaspero, Pieter Wesseling, Floyd Gilles, Nicholas Gottardo, Jonathan L Finlay, Stefan Rutkowski, Katja von Hoff
BACKGROUND: Pineoblastoma is a rare pineal region brain tumor. Treatment strategies have reflected those for other malignant embryonal brain tumors. PATIENTS AND METHODS: Original prospective treatment and outcome data from international trial groups were pooled. Cox regression models were developed considering treatment elements as time-dependent covariates. RESULTS: Data on 135 patients with pineoblastoma aged 0.01-20.7 (median 4.9) years were analyzed...
December 23, 2016: Neuro-oncology
https://www.readbyqxmd.com/read/27969569/biology-and-treatment-of-renal-tumours-in-childhood
#18
REVIEW
Jesper Brok, Taryn D Treger, Saskia L Gooskens, Marry M van den Heuvel-Eibrink, Kathy Pritchard-Jones
In Europe, almost 1000 children are diagnosed with a malignant renal tumour each year. The vast majority of cases are nephroblastoma, also known as Wilms' tumour (WT). Most children are treated according to Société Internationale d'Oncologie Pédiatrique Renal Tumour Study Group (SIOP-RTSG) protocols with pre-operative chemotherapy, surgery, and post-operative treatment dependent on stage and histology. Overall survival approaches 90%, but a subgroup of WT, with high-risk histology and/or relapsed disease, still have a much poorer prognosis...
October 28, 2016: European Journal of Cancer
https://www.readbyqxmd.com/read/27965825/aminoaciduria-in-the-prediction-of-ifosfamide-induced-tubulopathy-after-childhood-cancer-a-feasibility-study
#19
Jessica E Morgan, Karl McKeever, Kay S Tyerman, Michael Henderson, Susan Picton, Robert S Phillips
BACKGROUND: Ifosfamide, an alkylating agent used widely in the treatment of childhood malignancy, can cause many side effects including a proximal tubulopathy. Studies suggest that aminoaciduria is seen most commonly of all the biochemical abnormalities of ifosfamide-induced tubulopathy. A recent systematic review has found a paucity of data regarding the value of early markers indicating clinically significant tubulopathy. We undertook a pilot study to determine the feasibility of examining whether patients can be risk-stratified on the basis of aminoaciduria for the development of future significant ifosfamide-induced tubulopathy, to allow the evolution of appropriate follow-up strategies...
2016: Pilot and Feasibility Studies
https://www.readbyqxmd.com/read/27960236/radiation-associated-neoplasia-clinical-pathological-and-genomic-correlates
#20
REVIEW
Lynette M Sholl, Justine A Barletta, Jason L Hornick
Ionizing radiation is an established risk factor for the development of benign and malignant tumours. The epidemiology of radiation-associated neoplasia has been studied over the decades in diverse populations, including Japanese atomic bomb survivors, exposed communities following the Chernobyl nuclear power plant disaster, and paediatric and adult populations receiving therapeutic irradiation. Radiation has been associated with an increased risk of neoplasia throughout the human body, with some sites showing a markedly increased relative risk of developing tumours (thyroid; soft tissues), depending on patient age and the context of exposure...
January 2017: Histopathology
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