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Childhood sarcoma

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https://www.readbyqxmd.com/read/29226038/pencil-beam-scanning-proton-therapy-for-rhabdomyosarcoma-of-the-biliary-tract
#1
Luke Pater, Brian Turpin, Anthony Mascia
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood with 250-350 cases diagnosed annually in the United States. Biliary tract rhabdomyosarcoma is rare, representing <1% of the RMS cases. Due to its location, resection is clinically challenging, and functional complications exist and persist from biliary obstruction. The anatomical location of this tumor presents both opportunities and challenges for pencil beam scanning proton therapy. Proton therapy offers a dosimetric and clinical advantage by sparing the healthy liver, stomach, contra-lateral kidney and bowel...
October 5, 2017: Curēus
https://www.readbyqxmd.com/read/29214757/clinical-and-cytogenetic-profiles-of-rhabdomyosarcoma-with-bone-marrow-involvement-in-korean-children-a-15-year-single-institution-experience
#2
Dong Hyun Lee, Chan Jeoung Park, Seongsoo Jang, Young Uk Cho, Jong Jin Seo, Ho Joon Im, Kyung Nam Koh, Kyung Ja Cho, Joon Seon Song, Eul Ju Seo
BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Alveolar RMS (ARMS) is characterized by FOXO1-related chromosomal translocations that result in a poorer clinical outcome compared with embryonal RMS (ERMS). Because the chromosomal features of RMS have not been comprehensively defined, we analyzed the clinical and laboratory data of childhood RMS patients and determined the clinical significance of chromosomal abnormalities in the bone marrow. METHODS: Fifty-one Korean patients with RMS <18 years of age treated between 2001 and 2015 were enrolled in this study...
March 2018: Annals of Laboratory Medicine
https://www.readbyqxmd.com/read/29207714/paediatric-peripheral-primitive-neuroectodermal-tumour-a-clinico-pathological-study-from-southern-india
#3
Rithika Rajendran, Leena Dennis Joseph, Thanka Johnson, Latha Magatha Sneha, Julius Xavier Scott, Satish Srinivasan
Introduction: Primitive Neuroectodermal Tumour (PNET)/Ewing Sarcomas (ES) are aggressive childhood malignancies with neuroectodermal differentiation. Aim: To study the clinical presentation, morphology, Immun-ohistochemistry (IHC), management and outcome of all the cases of paediatric pPNET/ES reported in our tertiary care centre over a period of six years. Materials and Methods: This was a retrospective study conducted at Sri Ramachandra Medical College and Research Institute, Chennai, India...
September 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/29202127/obesity-does-not-exacerbate-the-protumorigenic-systemic-environment-in-sarcoma-subjects
#4
Claire M Buchta, Shannon K Boi, Benjamin J Miller, Mohammed M Milhem, Lyse A Norian
Sarcomas are a rare but fatal tumor type that accounts for <1% of adult solid malignancies and ~15% of childhood malignancies. Although the use of immunotherapy is being actively investigated for other solid tumors, advances in immunotherapy for sarcoma patients are lacking. To better understand the systemic immune environment in sarcoma patients, we performed a detailed multiplex analysis of serum cytokines, chemokines, and protumorigenic factors from treatment-naive subjects with localized, high-grade sarcoma...
June 1, 2017: ImmunoHorizons
https://www.readbyqxmd.com/read/29198472/soft-tissue-sarcoma-risk-in-childhood-cancer-survivors
#5
Manjulika Das
No abstract text is available yet for this article.
November 30, 2017: Lancet Oncology
https://www.readbyqxmd.com/read/29198337/ovarian-sex-cord-stromal-tumours-and-small-cell-tumours-pathological-genetic-and-management-aspects
#6
REVIEW
Stergios Boussios, Michele Moschetta, George Zarkavelis, Alexandra Papadaki, Aristides Kefas, Konstantina Tatsi
Non-epithelial ovarian cancers (NEOC) constitute a group of uncommon malignancies and their treatment is still a challenging task. Collectively, these tumours account for about 10% of all ovarian cancers and occur in all age groups from childhood to old-age. They include malignancies of germ cell origin, sex cord-stromal cell origin, and a variety of extremely rare ovarian cancers, such as small-cell carcinomas and sarcomas. Each of these classifications encompasses multiple histologic subtypes. It is imperative that these rare tumours are managed with accurate diagnosis, staging, and treatment, to optimise the outcome...
December 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/29190181/identifying-opportunities-to-bridge-disparity-gaps-in-curing-childhood-cancer-in-malawi-malignancies-with-excellent-curative-potential-account-for-the-majority-of-diagnoses
#7
Nader Kim El-Mallawany, Peter Wasswa, Idah Mtete, Mercy Mutai, Christopher C Stanley, Mary Mtunda, Mary Chasela, Atupele Mpasa, Stella Wachepa, William Kamiyango, Jimmy Villiera, Peter N Kazembe, Parth S Mehta
The majority of African children with cancer die without access to resources. We describe efforts to build a public treatment program with curative intent for childhood cancer in Lilongwe, Malawi despite severe limitations in diagnostic and therapeutic resources. We retrospectively analyzed a cohort of childhood cancer patients at Kamuzu Central Hospital from 12/2011-6/2013. Consistently available chemotherapeutic agents were limited to cyclophosphamide, vincristine, doxorubicin, bleomycin, methotrexate, and prednisone...
November 30, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29183875/navigating-chronic-pain-how-taking-a-few-wrong-turns-can-miss-sinister-pathology
#8
Jennifer Turnbull, Richard Dodds, Andy Wainwright, Athimalaipet Vaidyanathan Ramanan, Kate Wheeler
Bone sarcomas are rare in childhood, and their presentation can often mimic more benign complaints or chronic musculoskeletal pain. Ewing sarcomas in particular are often diagnosed after a significant delay from the onset of symptoms. At a population level, a long diagnostic delay is not necessarily associated with worse survival , as tumours that display slow growth also tend to be less aggressive. For any specific individual however, a delayed diagnosis can result in a larger tumour that is more difficult to treat...
November 28, 2017: Archives of Disease in Childhood. Education and Practice Edition
https://www.readbyqxmd.com/read/29183026/late-effects-and-long-term-follow-up-after-cancer-in-childhood
#9
Thorsten Langer, Desiree Grabow, Diana Steinmann, Bernhard Wörmann, Gabriele Calaminus
Today, 80% of children and adolescents with cancer survive their disease. From the results of aftercare research arises the question: Are the survivors also healthy? Many late effects depend on the type of cancer and its treatment. Patients with brain tumors and with malignant sarcomas are very often affected by secondary diseases. Data from the USA report that around 2/3 of all patients still living 30 years after their cancer treatment in childhood suffer from late complications. Equivalent figures for Germany were previously unavailable...
2017: Oncology Research and Treatment
https://www.readbyqxmd.com/read/29178401/childhood-cancer-incidence-and-survival-in-japan-and-england-a-population-based-study-1993-2010
#10
Kayo Nakata, Yuri Ito, Winnie Magadi, Audrey Bonaventure, Charles A Stiller, Kota Katanoda, Tomohiro Matsuda, Isao Miyashiro, Kathy Pritchard-Jones, Bernard Rachet
This study aimed to compare cancer incidence and trends in survival for children diagnosed in Japan and England, using population-based cancer registry data. The analysis was based on 5,192 children with cancer (age 0-14 years) from six prefectural cancer registries in Japan and 21,295 children diagnosed in England during 1993-2010. Differences in incidence rates between the two countries were measured with Poisson regression models. Overall survival was estimated using the Kaplan-Meier method. Incidence rates for Hodgkin lymphoma, renal tumours, and Ewing sarcomas in England were more than twice as high as those in Japan...
November 24, 2017: Cancer Science
https://www.readbyqxmd.com/read/29165710/risk-of-soft-tissue-sarcoma-among-69-460-five-year-survivors-of-childhood-cancer-in-europe
#11
Chloe J Bright, Mike M Hawkins, David L Winter, Daniela Alessi, Rodrigue S Allodji, Francesca Bagnasco, Edit Bárdi, Andrea Bautz, Julianne Byrne, Elizabeth A M Feijen, Miranda M Fidler, Stanislaw Garwicz, Desiree Grabow, Thorgerdur Gudmundsdottir, Joyeeta Guha, Nadia Haddy, Momcilo Jankovic, Peter Kaatsch, Melanie Kaiser, Claudia E Kuehni, Helena Linge, Hilde Øfstaas, Cecile M Ronckers, Roderick Skinner, Jop C Teepen, Monica Terenziani, Giao Vu-Bezin, Finn Wesenberg, Thomas Wiebe, Carlotta Sacerdote, Zsuzsanna Jakab, Riccardo Haupt, Päivi Lähteenmäki, Lorna Zadravec Zaletel, Rahel Kuonen, Jeanette F Winther, Florent de Vathaire, Leontien C Kremer, Lars Hjorth, Raoul C Reulen
Background: Childhood cancer survivors are at risk of subsequent primary soft-tissue sarcomas (STS), but the risks of specific STS histological subtypes are unknown. We quantified the risk of STS histological subtypes after specific types of childhood cancer. Methods: We pooled data from 13 European cohorts, yielding a cohort of 69 460 five-year survivors of childhood cancer. Standardized incidence ratios (SIRs) and absolute excess risks (AERs) were calculated. Results: Overall, 301 STS developed compared with 19 expected (SIR = 15...
November 20, 2017: Journal of the National Cancer Institute
https://www.readbyqxmd.com/read/29099933/kaposi-s-sarcoma-associated-herpesvirus-in-a-rural-ugandan-cohort-1992-2008
#12
Robert Newton, Nazzarena Labo, Katie Wakeham, Wendell Miley, Gershim Asiki, W Thomas Johnston, Denise Whitby
Background: The prevalence and titres of antibodies against Kaposi's sarcoma associated herpesvirus (KSHV) in rural Africa are not completely understood, nor are their trends over time in populations in which HIV is also endemic. We examined prevalence, titres, temporal trends and determinants of anti KSHV antibodies in each of three time periods (1990-91, 1999-2000 and 2007-2008) within a long-standing, rural population-based cohort in southwestern Uganda. Methods: For each period, we measured antibodies to the K8...
November 1, 2017: Journal of Infectious Diseases
https://www.readbyqxmd.com/read/29093183/therapeutic-targeting-of-pgbd5-induced-dna-repair-dependency-in-pediatric-solid-tumors
#13
Anton G Henssen, Casie Reed, Eileen Jiang, Heathcliff Dorado Garcia, Jennifer von Stebut, Ian C MacArthur, Patrick Hundsdoerfer, Jun Hyun Kim, Elisa de Stanchina, Yasumichi Kuwahara, Hajime Hosoi, Neil J Ganem, Filemon Dela Cruz, Andrew L Kung, Johannes H Schulte, John H Petrini, Alex Kentsis
Despite intense efforts, the cure rates of childhood and adult solid tumors are not satisfactory. Resistance to intensive chemotherapy is common, and targets for molecular therapies are largely undefined. We have found that the majority of childhood solid tumors, including rhabdoid tumors, neuroblastoma, medulloblastoma, and Ewing sarcoma, express an active DNA transposase, PGBD5, that can promote site-specific genomic rearrangements in human cells. Using functional genetic approaches, we discovered that mouse and human cells deficient in nonhomologous end joining (NHEJ) DNA repair cannot tolerate the expression of PGBD5...
November 1, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/29081033/the-presence-of-alk-alterations-and-clinical-relevance-of-crizotinib-treatment-in-pediatric-solid-tumors
#14
Luca Felkai, Rita Bánusz, Ilona Kovalszky, Zoltán Sápi, Miklós Garami, Gergő Papp, Katalin Karászi, Edit Varga, Monika Csóka
Soft tissue sarcomas (STS) and neuroblastomas (NBL), are childhood malignancies still associated with poor prognoses despite the overall improvement in childhood tumor survival of the past decades. Anaplastic lymphoma kinase (ALK) inhibition is promising new strategy to improve the outcome of these pediatric tumors. Eighteen histologic samples of pediatric STS and 19 NBL patients were analyzed for ALK abnormalities using fluorescent in situ hybridization (FISH) with break-apart probes and immunohistochemistry (IHC)...
October 28, 2017: Pathology Oncology Research: POR
https://www.readbyqxmd.com/read/29078908/racial-and-ethnic-disparities-in-treatment-and-survival-of-pediatric-sarcoma
#15
Andrew J Jacobs, Erika B Lindholm, Carolyn Fein Levy, Jonathan D Fish, Richard D Glick
BACKGROUND: Childhood sarcomas are rare and require complex interdisciplinary care including surgery, chemotherapy, and radiation. The goal of this study was to determine if racial or ethnic disparities exist for pediatric sarcoma patients in the United States. METHODS: The United States' National Cancer Institute's Surveillance, Epidemiology, and End Results database was used to identify patients aged 0-21 diagnosed with primary sarcomas from 1973 to 2012. Patients were considered by race and ethnicity...
November 2017: Journal of Surgical Research
https://www.readbyqxmd.com/read/29076966/revisiting-tumor-patterns-and-penetrance-in-germline-tp53-mutation-carriers-temporal-phases-of-li-fraumeni-syndrome
#16
Amina Amadou, Maria I Waddington Achatz, Pierre Hainaut
PURPOSE OF REVIEW: Germline pathogenic TP53 mutation may predispose to multiple cancers but penetrance and cancer patterns remain incompletely documented. We have analyzed international agency for research on cancer TP53 database to reevaluate age and variant-dependent tumor patterns. RECENT FINDINGS: Genome-wide studies suggest that germline variants are more frequent than estimated prevalence of Li-Fraumeni syndrome (LFS), suggesting that many carriers of potentially pathogenic mutations may not develop the syndrome...
January 2018: Current Opinion in Oncology
https://www.readbyqxmd.com/read/29075871/-soft-tissue-tumors-epidemiology-classification-and-staging
#17
L Fenzl, M Mehrmann, K Kremp, G Schneider
Benign, intermediate and malignant soft tissue tumors can be differentiated histologically. Furthermore, the tumors can be subdivided according to their linear differentiation. In the new World Health Organization (WHO) classification of soft tissue tumors from 2013 changes have been made relating to the allocation of known entities, e. g. undifferentiated sarcomas have been formed into a new subgroup and are no longer assigned to the fibrohistiocytic tumors. The term malignant fibrous histiocytoma has been replaced by the undifferentiated sarcoma...
November 2017: Der Radiologe
https://www.readbyqxmd.com/read/29065267/the-prognostic-importance-of-tgf-%C3%AE-tgf-%C3%AE-receptor-and-fascin-in-childhood-solid-tumors
#18
Hikmet Gulsah Tanyildiz, Gulsah Kaygusuz, Emel Unal, Nurdan Tacyildiz, Handan Dincaslan, Gulsan Yavuz
Fascin plays a role in tumor metastasis under the influence of TGF-β, each potentiating the effect of the other. We retrospectively investigated whether there was a prognostic relationship between TGF-β and fascin, and disease stage, local recurrence, metastasis tendency, and response to treatment. Twelve neuroblastomas, 17 osteosarcomas, 14 Ewing's sarcomas, 15 rhabdomyosarcoma cases, and 8 rare solid tumors were included. Serum TGF-β levels were high at the time of diagnosis in all groups (p = .015) and decreased significantly during remission (p = ...
October 24, 2017: Pediatric Hematology and Oncology
https://www.readbyqxmd.com/read/29039119/clinical-implications-of-germline-mutations-in-breast-cancer-tp53
#19
REVIEW
Katherine Schon, Marc Tischkowitz
PURPOSE: This review describes the prevalence of germline TP53 mutations, the risk of breast cancer and other cancers in mutation carriers and management implications for women with breast cancer and unaffected women. METHODS: Literature review of English language papers available through PubMed. RESULTS: Women who carry germline mutations in the TP53 gene have a very high risk of breast cancer of up to 85% by age 60 years. Most of these breast cancers are early onset with a median age at diagnosis of 34 years...
October 16, 2017: Breast Cancer Research and Treatment
https://www.readbyqxmd.com/read/29029988/mandibular-embryonal-rhabdomyosarcoma-with-cartilaginous-metaplasia-report-of-a-case-and-review-of-literature
#20
Scott M Peters, Tim Kunkle, Michael A Perrino, Elizabeth M Philipone, Angela J Yoon
Rhabdomyosarcoma (RMS) is a malignant tumor of skeletal muscle origin and frequently involves the head and neck region. It represents the second most common pediatric soft tissue sarcoma and accounts for 3% of all childhood cancers. Here, we report a case of embryonal RMS presenting as a right-sided facial swelling in a 7-year-old boy. Histologically, the tumor consisted of classic embryonal rhabdosarcomatous areas with metaplastic cartilage, in both initial biopsy and final resection specimens. Cartilaginous metaplasia arising in the background of RMS is a rare finding, thus raising a diagnostic challenge...
September 6, 2017: Oral Surgery, Oral Medicine, Oral Pathology and Oral Radiology
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