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Childhood sarcoma

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https://www.readbyqxmd.com/read/29452297/lumbar-intraspinal-spindle-cell-rhabdomyosarcoma-as-a-rare-cause-of-spinal-stenosis-a-case-report
#1
Donel A Sequea, Maria E Reese
Rhabdomyosarcoma (RMS) represents the most common sarcoma in childhood, yet is extremely rare in adults with only a handful of cases reported. Here we present a case of intraspinal spindle cell RMS in an adult who presented as a typical case of spinal stenosis. To our knowledge, this is the first reported case of lumbar intraspinal spindle cell RMS in an adult patient. Furthermore, RMS phenotypically presents more aggressively in adults compared to children.
February 13, 2018: PM & R: the Journal of Injury, Function, and Rehabilitation
https://www.readbyqxmd.com/read/29434830/pediatric-sarcomas
#2
Junhua Cao, Qi An, Lei Wang
Sarcomas arise from primitive mesenchymal cells, which are classified, into two main groups: Bone and soft tissue sarcomas. We have searched all-important electronic databases including Google scholar and PubMed for the collection of latest literature pertaining to pediatric sarcomas. Latest literature confirmed that these tumors are relatively rare and represent only 1% of all malignancies but they have higher incidence in children. Pediatric sarcomas comprise about 13% of all pediatric malignancies and are ranked third in childhood cancers...
February 2018: Oncology Letters
https://www.readbyqxmd.com/read/29398803/therapy-related-aml-mds-following-treatment-for-childhood-cancer-experience-from-a-tertiary-care-centre-in-north-india
#3
Chintan Vyas, Sandeep Jain, Gauri Kapoor
Therapy-related acute myeloid leukemia/myelodysplastic syndrome (t-AML/MDS) is a devastating late effect of cancer treatment. There is limited data on incidence of t-AML/MDS from India. We retrospectively studied pediatric t AML/MDS at our institute between January 1996 and December 2015. Among 1285 children, 8 patients developed t-AML with a median age of 15.5 years. Overall incidence of t-AML/MDS was 0.62% [0.99% (4/402) in solid tumours and 0.45% (4/883) in leukemia/lymphoma, P = 0.26] with 6390 patient years of follow up...
January 2018: Indian Journal of Hematology & Blood Transfusion
https://www.readbyqxmd.com/read/29397557/targeting-histone-deacetylase-activity-to-arrest-cell-growth-and-promote-neural-differentiation-in-ewing-sarcoma
#4
Bárbara Kunzler Souza, Patrícia Luciana da Costa Lopez, Pâmela Rossi Menegotto, Igor Araujo Vieira, Nathalia Kersting, Ana Lúcia Abujamra, André T Brunetto, Algemir L Brunetto, Lauro Gregianin, Caroline Brunetto de Farias, Carol J Thiele, Rafael Roesler
There is an urgent need for advances in the treatment of Ewing sarcoma (EWS), an aggressive childhood tumor with possible neuroectodermal origin. Inhibition of histone deacetylases (HDAC) can revert aberrant epigenetic states and reduce growth in different experimental cancer types. Here, we investigated whether the potent HDAC inhibitor, sodium butyrate (NaB), has the ability to reprogram EWS cells towards a more differentiated state and affect their growth and survival. Exposure of two EWS cell lines to NaB resulted in rapid and potent inhibition of HDAC activity (1 h, IC50 1...
February 3, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29397238/hereditary-swi-snf-complex-deficiency-syndromes
#5
REVIEW
Abbas Agaimy, William D Foulkes
The SWItch Sucrose non-fermentable (SWI/SNF) complex is a highly conserved multi-subunit complex of proteins encoded by numerous genes mapped to different chromosomal regions. The complex regulates the process of chromatin remodelling and hence plays a central role in the epigenetic regulation of gene expression, cell proliferation and differentiation. During the last three decades, the SWI/SNF complex has been increasingly recognized as a central molecular event driving the initiation and/or progression of several benign and malignant neoplasms of different anatomic origin and having diverse histomorphological appearance...
January 31, 2018: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/29367756/pax3-foxo1-drives-mir-486-5p-and-represses-mir-221-contributing-to-pathogenesis-of-alveolar-rhabdomyosarcoma
#6
Jason A Hanna, Matthew R Garcia, Alicia Lardennois, Patrick J Leavey, Dino Maglic, Alexandre Fagnan, Jonathan C Go, Jordan Roach, Yong-Dong Wang, David Finkelstein, Mark E Hatley
Rhabdomyosarcoma is the most common soft-tissue sarcoma in childhood and histologically resembles developing skeletal muscle. Alveolar rhabdomyosarcoma (ARMS) is an aggressive subtype with a higher rate of metastasis and poorer prognosis. The majority of ARMS tumors (80%) harbor a PAX3-FOXO1 or less commonly a PAX7-FOXO1 fusion gene. The presence of either the PAX3-FOXO1 or PAX7-FOXO1 fusion gene foretells a poorer prognosis resulting in clinical re-classification as either fusion-positive (FP-RMS) or fusion-negative RMS (FN-RMS)...
January 25, 2018: Oncogene
https://www.readbyqxmd.com/read/29345507/ovarian-tissue-cryopreservation-in-young-females-through-the-oncofertility-consortium-s-national-physicians-cooperative
#7
Austin G Armstrong, Bruce F Kimler, Brigid M Smith, Teresa K Woodruff, Mary Ellen Pavone, Francesca E Duncan
AIM: To characterize the clinical indications of females (<15 years old) undergoing ovarian tissue cryopreservation (OTC) through the Oncofertility Consortium's National Physicians Cooperative (OC-NPC). PATIENTS & METHODS: The clinical indications of 114 females who underwent OTC were classified, and their incidence was compared with childhood cancer databases. RESULTS: Leukemias/myeloproliferative diseases/myelodysplastic diseases and hemoglobinopathies were the most prevalent oncologic and nononcologic indications for OTC, respectively...
January 18, 2018: Future Oncology
https://www.readbyqxmd.com/read/29343557/dicer1-and-associated-conditions-%C3%A2-identification-of-at-risk-individuals-and-recommended-surveillance-strategies
#8
Kris Ann P Schultz, Gretchen M Williams, Junne Kamihara, Douglas R Stewart, Anne K Harris, Andrew J Bauer, Joyce Turner, Rachana Shah, Katherine Schneider, Kami Wolfe Schneider, Ann Garrity Carr, Laura A Harney, Shari Baldinger, A Lindsay Frazier, Daniel Orbach, Dominik T Schneider, David Malkin, Louis P Dehner, Yoav H Messinger, Ashley Hill
Pathogenic germline DICER1 variants cause a hereditary cancer predisposition syndrome with a variety of manifestations. In addition to conferring increased cancer risks for pleuropulmonary blastoma (PPB) and ovarian sex cord-stromal tumors, particularly Sertoli-Leydig cell tumor, individuals with pathogenic germline DICER1 variants may also develop lung cysts, cystic nephroma, renal sarcoma and Wilms tumor, nodular hyperplasia of the thyroid, nasal chondromesenchymal hamartoma, ciliary body medulloepithelioma, genitourinary embryonal rhabdomyosarcoma and brain tumors including pineoblastoma and pituitary blastoma...
January 17, 2018: Clinical Cancer Research: An Official Journal of the American Association for Cancer Research
https://www.readbyqxmd.com/read/29333015/a-profile-of-pediatric-solid-tumors-a-single-institution-experience-in-kashmir
#9
Namita Sharma, Ayesha Ahmad, Gull M Bhat, Sheikh A Aziz, Mohammad Maqbool Lone, Nisar A Bhat
Aims: The purpose of this retroprospective study was to study the epidemiological characteristics and outcomes of children with solid tumors at our institution. Subjects and Methods: Three hundred and three pediatrics patients registered at Regional Cancer Centre (RCC), Sher-i-Kashmir Institute of Medical Sciences (SKIMS), Srinagar, Kashmir, between January 2008 and June 2014, were analyzed with regard to demographic status, presenting complaints, investigations, treatment, morbidity, and outcomes...
October 2017: Indian Journal of Medical and Paediatric Oncology
https://www.readbyqxmd.com/read/29280680/smarcb1-deficient-tumors-of-childhood-a-practical-guide
#10
Bruce R Pawel
The SMARCB1 gene ( INI1, BAF47) is a member of the SWItch/Sucrose Non-Fermentable (SWI/SNF) chromatin remodeling complex, involved in the epigenetic regulation of gene transcription. SMARCB1 acts as a tumor suppressor gene, and loss of function of both alleles gives rise to SMARCB1-deficient tumors. The prototypical SMARCB1-deficient tumor is the malignant rhabdoid tumor (MRT) which was first described in the kidney but also occurs in soft tissue, viscera, and the brain (where it is referred to as atypical teratoid rhabdoid tumor or AT/RT)...
January 1, 2017: Pediatric and Developmental Pathology
https://www.readbyqxmd.com/read/29226038/pencil-beam-scanning-proton-therapy-for-rhabdomyosarcoma-of-the-biliary-tract
#11
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
#12
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
#13
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
#14
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
#15
Manjulika Das
No abstract text is available yet for this article.
January 2018: Lancet Oncology
https://www.readbyqxmd.com/read/29198337/ovarian-sex-cord-stromal-tumours-and-small-cell-tumours-pathological-genetic-and-management-aspects
#16
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
#17
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
#18
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
#19
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
#20
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
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