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Rhabdomyosarcoma

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https://www.readbyqxmd.com/read/28326081/the-smac-mimetic-bv6-improves-nk-cell-mediated-killing-of-rhabdomyosarcoma-cells-by-simultaneously-targeting-tumor-and-effector-cells
#1
Kyra Fischer, Sara Tognarelli, Stefanie Roesler, Cathinka Boedicker, Ralf Schubert, Alexander Steinle, Thomas Klingebiel, Peter Bader, Simone Fulda, Evelyn Ullrich
Rhabdomyosarcoma (RMS), the most common cancer of connective tissues in pediatrics, is often resistant to conventional therapies. One underlying mechanism of this resistance is the overexpression of Inhibitor of Apoptosis (IAP) proteins, leading to a dysfunctional cell death program within tumor cells. Smac mimetics (SM) are small molecules that can reactivate the cell death program by antagonizing IAP proteins and thereby compensating their overexpression. Here, we report that SM sensitize two RMS cell lines (RD and RH30) toward natural killer (NK) cell-mediated killing on the one hand, and increase the cytotoxic potential of NK cells on the other...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28317309/infantile-fibrosarcoma-treated-with-postoperative-vincristine-and-dactinomycin
#2
Hiroki Yoshihara, Yuri Yoshimoto, Yosuke Hosoya, Daisuke Hasegawa, Takafumi Kawano, Akiko Sakoda, Hajime Okita, Atsushi Manabe
Infantile fibrosarcoma is a non-rhabdomyosarcoma soft-tissue sarcoma that occurs in infancy and which has a relatively good prognosis. A vincristine and dactinomycin (VA) regimen has been shown to be effective, although the duration of chemotherapy has not been well defined. We describe the case of a 4-month-old boy with a mass at the left dorsum of the foot who was diagnosed with infantile fibrosarcoma after resection of the tumor, the margin of which was macroscopically positive. VA treatment was carried out with careful monitoring of response and adverse effects...
March 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28314266/additive-interaction-of-cisplatin-and-histone-deacetylase-inhibitors-combined-treatment-in-rhabdomyosarcoma-cells-an-isobolographic-analysis
#3
Agata Jarząb, Jarogniew J Łuszczki, Małgorzata Guz, Ewelina Gumbarewicz, Krzysztof Polberg, Andrzej Stepulak
BACKGROUND/AIM: The aim of this study was to assess the anticancer effect and the type of pharmacologic drug-drug interaction of cisplatin (CDDP) and histone deacetylase inhibitors (HDIs) combined treatment on the rhabdomyosarcoma cell line. MATERIALS AND METHODS: The antiproliferative actions of cisplatin and suberoylanilide hydroxamic acid (SAHA, vorinostat), as well as valproic acid (VPA) alone and in combination, were evaluated using the tetrazolium dye-based MTT cell proliferation assay and isobolographic analysis...
March 2017: Anticancer Research
https://www.readbyqxmd.com/read/28306214/the-prognostic-value-of-early-radiographic-response-in-children-and-adolescents-with-embryonal-rhabdomyosarcoma-stage-iv-metastases-confined-to-the-lungs-a-report-from-the-cooperative-weichteilsarkom-studiengruppe-cws
#4
Monika Sparber-Sauer, Thekla von Kalle, Guido Seitz, Tobias Dantonello, Monika Scheer, Marc Münter, Joerg Fuchs, Ruth Ladenstein, Stefan S Bielack, Thomas Klingebiel, Ewa Koscielniak
BACKGROUND: Patients with metastatic rhabdomyosarcoma (RMS) have a poor prognosis apart from children with embryonal RMS whose metastases are confined to the lungs (PRME). The prognostic significance of response in patients with metastatic disease is still unknown and optimal treatment remains to be defined. METHODS: Patient-, tumor- and treatment-related factors of patients with PRME treated on multiple prospective trials of the Cooperative Weichteilsarkom Studiengruppe (CWS) (1981-2013) were analyzed with a focus on response to induction chemotherapy...
March 17, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28302659/clear-cell-adenocarcinoma-of-cervix-in-1-year-old-girl-without-in-utero-exposure-to-diethylstilbestrol-an-uncommon-tumour-at-an-uncommon-age-and-site
#5
Aditi Arora, Anurag Rastogi, Azfar Neyaz, Nuzhat Husain
Adenocarcinoma of cervix constitutes about 10-15% cases of carcinoma cervix. Clear cell variant is even a rarer variant of adenocarcinoma. It rarely occurs in the paediatric age group and a known risk factor is in utero exposure to diethylstilbestrol (DES). We report here a case of primary cervical tumour in a 1-year-old girl, which was initially suspected to be an embryonal rhabdomyosarcoma botryoides. Histopathology with immunohistochemical analysis revealed clear cell adenocarcinoma. There was no maternal history of DES intake during pregnancy...
March 16, 2017: BMJ Case Reports
https://www.readbyqxmd.com/read/28295798/rare-pattern-of-relapse-to-the-pancreas-and-bilateral-extraocular-muscles-in-paediatric-alveolar-rhabdomyosarcoma
#6
Anne L Ryan, Lakshmi Nagarajan, Angela J Alessandri, Nicholas G Gottardo, Rishi S Kotecha
No abstract text is available yet for this article.
March 13, 2017: Journal of Paediatrics and Child Health
https://www.readbyqxmd.com/read/28291660/degradation-of-endothelial-network-in-disordered-tumor-containing-cell-sheet
#7
Menglu Li, Masahiro Kino-Oka
Tumor angiogenesis is an important event in tumor malignancy; and the vasculature formed in tumor region is typically dysfunctional. Multiple factors are associated with tumor vessel abnormalities, but the precise mechanism has not been fully understood. In the present study, a tumor-containing cell sheet was prepared by mixing a small population of human embryonal rhabdomyosarcoma (RMS) cells (RDs) with human skeletal muscle myoblasts (HSMMs) to mimic muscle tissue invaded by RMS cells. Sheet fluidity and the extracellular matrix (ECM) meshwork of the tumor-containing cell sheet were found to be elevated and disordered, demonstrating the disruptive effect of tumor cells on sheet structure...
March 10, 2017: Journal of Bioscience and Bioengineering
https://www.readbyqxmd.com/read/28284478/wandering-pelvic-mass-rhabdomyosarcoma-of-the-meckel-diverticulum
#8
Sajid S Qureshi, Bharat Rekhi, Seema Kembhavi
No abstract text is available yet for this article.
March 8, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28273971/clinico-pathological-spectrum-of-ophthalmic-lesions-an-experience-in-tertiary-care-hospital-of-central-india
#9
Yashita Gupta, Renuka Gahine, Nighat Hussain, Mohd Jafar Memon
INTRODUCTION: Ophthalmic lesions show varied distribution on the basis of geographical location. Eye being a unique sensory organ needs to be studied both clinically and pathologically. AIM: This study was aimed to evaluate the histomorphological and clinico-pathlological spectrum of ophthalmic lesions at a tertiary care hospital of Central India. MATERIALS AND METHODS: We reviewed all the ophthalmic biopsies performed in the Department of Pathology of our institute between January 2008 and November 2014...
January 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28273136/dux4-induced-dsrna-and-myc-mrna-stabilization-activate-apoptotic-pathways-in-human-cell-models-of-facioscapulohumeral-dystrophy
#10
Sean C Shadle, Jun Wen Zhong, Amy E Campbell, Melissa L Conerly, Sujatha Jagannathan, Chao-Jen Wong, Timothy D Morello, Silvère M van der Maarel, Stephen J Tapscott
Facioscapulohumeral dystrophy (FSHD) is caused by the mis-expression of DUX4 in skeletal muscle cells. DUX4 is a transcription factor that activates genes normally associated with stem cell biology and its mis-expression in FSHD cells results in apoptosis. To identify genes and pathways necessary for DUX4-mediated apoptosis, we performed an siRNA screen in an RD rhabdomyosarcoma cell line with an inducible DUX4 transgene. Our screen identified components of the MYC-mediated apoptotic pathway and the double-stranded RNA (dsRNA) innate immune response pathway as mediators of DUX4-induced apoptosis...
March 2017: PLoS Genetics
https://www.readbyqxmd.com/read/28271871/soft-tissue-sarcomas-in-adolescents-and-young-adults-a-comparison-with-their-paediatric-and-adult-counterparts
#11
REVIEW
Winette T A van der Graaf, Daniel Orbach, Ian R Judson, Andrea Ferrari
Survival outcomes for adolescent and young adult patients with soft tissue sarcomas lag behind those of children diagnosed with histologically similar tumours. To help understand these differences in outcomes, we discuss the following issues with regard to the management of these patients with soft tissue sarcomas: delays in diagnosis, trial availability and participation, aspects of the organisation of care (with an emphasis on age-specific needs), national centralisation of sarcoma care, international consortia, and factors related to tumour biology...
March 2017: Lancet Oncology
https://www.readbyqxmd.com/read/28265395/orbital-rhabdomyosarcoma-and-traumatic-neuroma-following-enucleation-for-a-uveal-schwannoma-in-a-dog-a-case-report
#12
Jessica E McDonald, Amy M Knollinger, Leandro B Teixeira, Richard R Dubielzig
A 4-year-old, female spayed Siberian husky with history of a uveal schwannoma presented for orbital swelling 9 months after enucleation. A second, malignant tumor developed in the same orbit. Therefore, uveal schwannomas may warrant early surgical intervention in the dog.
March 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28262537/robot-assisted-retroperitoneal-lymph-node-dissection-ra-rplnd-in-the-adolescent-population
#13
A P Glaser, D K Bowen, B W Lindgren, J J Meeks
BACKGROUND: Robot-assisted retroperitoneal lymph node dissection (RA-RPLND) has built on success and techniques of laparoscopic RPLND, with the added benefits of robotic technology. This paper demonstrates use of the da Vinci Xi(®) system for RA-RPLND in two adolescent patients. METHODS: Case #1: A 17-year-old male presented with a left testicular mass and elevated alpha-fetoprotein (AFP) and human chorionic gonadotropin (HCG). Pathology revealed a mixed non-seminomatous germ cell tumor (60% embryonal, 35% yolk sac, 5% choriocarcinoma, + lymphovascular invasion)...
February 17, 2017: Journal of Pediatric Urology
https://www.readbyqxmd.com/read/28260453/first-report-on-stem-cell-transplant-from-iraq
#14
Abdul Majeed A Hammadi, Waleed A Azeez, Firas Hashim Jasim, Nadher Alshammary, Adel D Sewan, Khudhair Alrawaq, Ala Makki, Ahmed Mubarak, Amel Ameen, Andolina Marino, Ahmed Khudhair, Nedhal K Alrahhal
The Iraqi Bone Marrow Transplantation Center is located in the medical city complex of Bab Almuadham in Baghdad, Iraq. It was established on March 11, 2002, and performed its first mini-allotransplant for acute myeloid leukemia on January 24, 2003. Among 16 patients who received hematopoietic stem cell transplant between January 2003 and January 2010, one patient underwent allogeneic bone marrow transplant for acute myeloid leukemia and 15 patients received autologous bone marrow transplant for the following indications: 5 had multiple myeloma, 9 had lymphoma (8 with Hodgkin disease and 1 with non- Hodgkin lymphoma), and 1 had rhabdomyosarcoma...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28256213/update-on-molecular-findings-in-rhabdomyosarcoma
#15
Dina El Demellawy, Jean McGowan-Jordan, Joseph de Nanassy, Elizaveta Chernetsova, Ahmed Nasr
Rhabdomyosarcoma (RMS) is the most common malignant soft tissue tumour in children and adolescence. Histologically RMS resembles developing fetal striated skeletal muscle. RMS is stratified into different histological subtypes which appear to influence management plans and patients outcome. Importantly, molecular classification of RMS seems to more accurately capture the true biology and clinical course and prognosis of RMS to guide therapeutic decisions. The identification of PAX-FOXO1 fusion status in RMS is one of the most important updates in the risk stratification of RMS...
February 27, 2017: Pathology
https://www.readbyqxmd.com/read/28253504/genomic-characterization-of-a-metastatic-alveolar-rhabdomyosarcoma-case-using-fish-studies-and-cgh-snp-microarray-revealing-foxo1-pax7-rearrangement-with-mycn-and-mdm2-amplification-and-rb1-region-loss
#16
Arivarasan Karunamurthy, Lori Hoffner, Jie Hu, Peter Shaw, Sarangarajan Ranganathan, Svetlana A Yatsenko, Urvashi Surti
Rhabdomyosarcomas (RMS) are rare, heterogeneous, soft tissue sarcomas and a common type of childhood malignancy with a distinct histomorphology. At the molecular level, alveolar rhabdomyosarcoma (ARMS), a subtype of RMS, harbors a signature genetic makeup characterized by specific translocations. The type of translocation and associated genetic aberrations correlate with disease progression, hence we used multiple molecular modalities including high-resolution array comparative genomic hybridization to explore the oncogenic gene fusion and associated copy number variations in a case of metastatic ARMS...
March 3, 2017: Cytogenetic and Genome Research
https://www.readbyqxmd.com/read/28247225/update-from-the-4th-edition-of-the-world-health-organization-classification-of-head-and-neck-tumours-tumours-of-the-ear
#17
Lester D R Thompson
The 2017 fourth edition of the World Health Organization Classification of Tumours, specifically as it relates to the ear (Chap. 9), has several changes. Importantly, the number of entities has been significantly reduced by omitting tumors or lesions if they do not occur exclusively or predominantly at this site or if they are discussed in detail elsewhere in the book. These entities include: embryonal rhabdomyosarcoma, osteoma, exostosis, angiolymphoid hyperplasia with eosinophilia, Schneiderian papilloma, inverted papilloma, lipoma of the internal auditory canal, hemangioma, hematolymphoid tumors, and secondary tumors...
March 2017: Head and Neck Pathology
https://www.readbyqxmd.com/read/28243595/cns-metastases-from-bone-and-soft-tissue-sarcomas-in-children-adolescents-and-young-adults-are-they-really-so-rare
#18
Monika Bekiesinska-Figatowska, Agnieszka Duczkowska, Marek Duczkowski, Hanna Bragoszewska, Anna Romaniuk-Doroszewska, Beata Iwanowska, Sylwia Szkudlinska-Pawlak, Jaroslaw Madzik, Katarzyna Bilska, Anna Raciborska
Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas' metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas' CNS metastases. Methods. Patients with first diagnosis in 1999-2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI)/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28243259/hedgehog-pathway-inhibition-hampers-sphere-and-holoclone-formation-in-rhabdomyosarcoma
#19
Ana Almazán-Moga, Patricia Zarzosa, Isaac Vidal, Carla Molist, Irina Giralt, Natalia Navarro, Aroa Soriano, Miguel F Segura, Arantza Alfranca, Javier Garcia-Castro, José Sánchez de Toledo, Josep Roma, Soledad Gallego
Rhabdomyosarcoma (RMS) is the most common type of soft tissue sarcoma in children and can be divided into two main subtypes: embryonal (eRMS) and alveolar (aRMS). Among the cellular heterogeneity of tumors, the existence of a small fraction of cells called cancer stem cells (CSC), thought to be responsible for the onset and propagation of cancer, has been demonstrated in some neoplasia. Although the existence of CSC has been reported for eRMS, their existence in aRMS, the most malignant subtype, has not been demonstrated to date...
2017: Stem Cells International
https://www.readbyqxmd.com/read/28233470/pediatric-nonrhabdomyosarcoma-soft-tissue-sarcomas-arising-at-visceral-sites
#20
Andrea Ferrari, Chiara Magni, Luca Bergamaschi, Giovanni Cecchetto, Rita Alaggio, Giuseppe Maria Milano, Patrizia Bertolini, Eleonora Basso, Carla Manzitti, Martina Di Martino, Nauga Giurici, Fraia Melchionda, Valerio Cecinati, Stefano Chiaravalli, Maria Carmen Affinita, Angela Scagnellato, Michela Casanova, Gianni Bisogno
BACKGROUND: Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) may rarely occur in visceral tissues, and little is known about their clinical history. The present study retrospectively analyzed a group of patients prospectively registered in Italian pediatric protocols conducted between 1979 and 2004. METHODS: Inclusion criteria for the study were as follows: a pathological diagnosis of "adult-type NRSTS," arising at visceral sites (lung-pleurae, liver, kidney, and mesentery-bowel); age under 18 years; no previous treatment except for primary surgery; available clinical data; and written consent...
February 24, 2017: Pediatric Blood & Cancer
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