rhabdomyosarcoma

Summary

Summary: A malignant solid tumor arising from mesenchymal tissues which normally differentiate to form striated muscle. It can occur in a wide variety of sites. It is divided into four distinct types: pleomorphic, predominantly in male adults; alveolar (RHABDOMYOSARCOMA, ALVEOLAR), mainly in adolescents and young adults; embryonal (RHABDOMYOSARCOMA, EMBRYONAL), predominantly in infants and children; and botryoidal, also in young children. It is one of the most frequently occurring soft tissue sarcomas and the most common in children under 15. (From Dorland, 27th ed; Holland et al., Cancer Medicine, 3d ed, p2186; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, pp1647-9)

Top Publications

  1. ncbi Development of rhabdomyosarcoma in HER-2/neu transgenic p53 mutant mice
    Patrizia Nanni
    Cancer Research Section, Department of Experimental Pathology, University of Bologna, I 40126 Bologna
    Cancer Res 63:2728-32. 2003
  2. pmc NF-kappaB-YY1-miR-29 regulatory circuitry in skeletal myogenesis and rhabdomyosarcoma
    Huating Wang
    Department of Molecular Virology, Human Cancer Genetics Program, The Ohio State University, Columbus, OH 43210, USA
    Cancer Cell 14:369-81. 2008
  3. pmc MicroRNA-1/206 targets c-Met and inhibits rhabdomyosarcoma development
    Dongsheng Yan
    School of Ophthalmology and Optometry, Eye Hospital, Wenzhou Medical College, Wenzhou, Zhejiang 325003, China
    J Biol Chem 284:29596-604. 2009
  4. pmc Rhabdomyosarcoma: review of the Children's Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and rationale for current COG studies
    Suman Malempati
    Department of Pediatrics, Oregon Health and Science University, Portland, Oregon 97239 3098, USA
    Pediatr Blood Cancer 59:5-10. 2012
  5. ncbi PAX3/FOXO1 fusion gene status is the key prognostic molecular marker in rhabdomyosarcoma and significantly improves current risk stratification
    Edoardo Missiaglia
    The Institute of Cancer Research, Sutton, Surrey, UK
    J Clin Oncol 30:1670-7. 2012
  6. ncbi Notch pathway inhibition significantly reduces rhabdomyosarcoma invasiveness and mobility in vitro
    Josep Roma
    Biomedical Research Unit, Hospital Universitari Vall d Hebron, Barcelona, Spain
    Clin Cancer Res 17:505-13. 2011
  7. ncbi CXCR4-SDF-1 signaling is active in rhabdomyosarcoma cells and regulates locomotion, chemotaxis, and adhesion
    Jolanta Libura
    Stem Cell Biology Program at the James Graham Brown Cancer Center, University of Louisville, KY 40202, USA
    Blood 100:2597-606. 2002
  8. pmc The muscle-specific microRNA miR-206 blocks human rhabdomyosarcoma growth in xenotransplanted mice by promoting myogenic differentiation
    Riccardo Taulli
    Department of Anatomy, Pharmacology and Forensic Medicine, and Center for Experimental Research and Medical Studies, University of Torino, Torino, Italy
    J Clin Invest 119:2366-78. 2009
  9. ncbi Molecular and cellular biology of rhabdomyosarcoma
    Carla De Giovanni
    Department of Experimental Pathology, Cancer Research Section, University of Bologna, Bologna, Italy
    Future Oncol 5:1449-75. 2009
  10. pmc Addiction to elevated insulin-like growth factor I receptor and initial modulation of the AKT pathway define the responsiveness of rhabdomyosarcoma to the targeting antibody
    Liang Cao
    Genetics Branch, Center for Cancer Research, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland 20892 4265, USA
    Cancer Res 68:8039-48. 2008

Detail Information

Publications305 found, 100 shown here

  1. ncbi Development of rhabdomyosarcoma in HER-2/neu transgenic p53 mutant mice
    Patrizia Nanni
    Cancer Research Section, Department of Experimental Pathology, University of Bologna, I 40126 Bologna
    Cancer Res 63:2728-32. 2003
    ..that activation of the HER-2/neu oncogene coupled with inactivation of the oncosuppressor gene p53 causes rhabdomyosarcoma in mice...
  2. pmc NF-kappaB-YY1-miR-29 regulatory circuitry in skeletal myogenesis and rhabdomyosarcoma
    Huating Wang
    Department of Molecular Virology, Human Cancer Genetics Program, The Ohio State University, Columbus, OH 43210, USA
    Cancer Cell 14:369-81. 2008
    ..Here we describe the regulation and function of miR-29 in myogenesis and rhabdomyosarcoma (RMS)...
  3. pmc MicroRNA-1/206 targets c-Met and inhibits rhabdomyosarcoma development
    Dongsheng Yan
    School of Ophthalmology and Optometry, Eye Hospital, Wenzhou Medical College, Wenzhou, Zhejiang 325003, China
    J Biol Chem 284:29596-604. 2009
    ..nature of miR-1/206 for skeletal muscles, we investigated the role of miR-1/206 in the development of rhabdomyosarcoma. Initially, we demonstrated that miR-1/206 expression was suppressed in rhabdomyosarcomas and found at very ..
  4. pmc Rhabdomyosarcoma: review of the Children's Oncology Group (COG) Soft-Tissue Sarcoma Committee experience and rationale for current COG studies
    Suman Malempati
    Department of Pediatrics, Oregon Health and Science University, Portland, Oregon 97239 3098, USA
    Pediatr Blood Cancer 59:5-10. 2012
    The prognosis for children and adolescents with rhabdomyosarcoma (RMS) has improved with refinements in multi-modal therapy...
  5. ncbi PAX3/FOXO1 fusion gene status is the key prognostic molecular marker in rhabdomyosarcoma and significantly improves current risk stratification
    Edoardo Missiaglia
    The Institute of Cancer Research, Sutton, Surrey, UK
    J Clin Oncol 30:1670-7. 2012
    To improve the risk stratification of patients with rhabdomyosarcoma (RMS) through the use of clinical and molecular biologic data.
  6. ncbi Notch pathway inhibition significantly reduces rhabdomyosarcoma invasiveness and mobility in vitro
    Josep Roma
    Biomedical Research Unit, Hospital Universitari Vall d Hebron, Barcelona, Spain
    Clin Cancer Res 17:505-13. 2011
    b>Rhabdomyosarcoma (RMS) is the most common type of soft tissue sarcoma in children and can be divided into two main subtypes: embryonal and alveolar RMS...
  7. ncbi CXCR4-SDF-1 signaling is active in rhabdomyosarcoma cells and regulates locomotion, chemotaxis, and adhesion
    Jolanta Libura
    Stem Cell Biology Program at the James Graham Brown Cancer Center, University of Louisville, KY 40202, USA
    Blood 100:2597-606. 2002
    ..various human tumor cell lines for expression of CXCR4 and found that it was highly expressed on several rhabdomyosarcoma (RMS) cell lines...
  8. pmc The muscle-specific microRNA miR-206 blocks human rhabdomyosarcoma growth in xenotransplanted mice by promoting myogenic differentiation
    Riccardo Taulli
    Department of Anatomy, Pharmacology and Forensic Medicine, and Center for Experimental Research and Medical Studies, University of Torino, Torino, Italy
    J Clin Invest 119:2366-78. 2009
    ..In this work, we examined the role of the muscle-specific miRNAs miR-1 and miR-206 in human rhabdomyosarcoma (RMS), a soft tissue sarcoma thought to arise from skeletal muscle progenitors...
  9. ncbi Molecular and cellular biology of rhabdomyosarcoma
    Carla De Giovanni
    Department of Experimental Pathology, Cancer Research Section, University of Bologna, Bologna, Italy
    Future Oncol 5:1449-75. 2009
    b>Rhabdomyosarcoma is a group of soft-tissue sarcomas that share features of skeletal myogenesis, but show extensive heterogeneity in histology, age and site of onset, and prognosis...
  10. pmc Addiction to elevated insulin-like growth factor I receptor and initial modulation of the AKT pathway define the responsiveness of rhabdomyosarcoma to the targeting antibody
    Liang Cao
    Genetics Branch, Center for Cancer Research, Center for Cancer Research, National Cancer Institute, Bethesda, Maryland 20892 4265, USA
    Cancer Res 68:8039-48. 2008
    ..Our results showed a large variation in IGF-IR levels in rhabdomyosarcoma tumor specimens that were comparable with those in rhabdomyosarcoma cell lines...
  11. pmc Macrophage migration inhibitory factor is secreted by rhabdomyosarcoma cells, modulates tumor metastasis by binding to CXCR4 and CXCR7 receptors and inhibits recruitment of cancer-associated fibroblasts
    Maciej Tarnowski
    Stem Cell Institute at James Graham Brown Cancer Center, University of Louisville, Louisville, Kentucky 40202, USA
    Mol Cancer Res 8:1328-43. 2010
    ..Here, we report that human rhabdomyosarcoma (RMS) cell lines secrete MIF and that this chemokine (a) induces phosphorylation of mitogen-activated protein ..
  12. ncbi Lysine-specific demethylase 1 (LSD1/KDM1A/AOF2/BHC110) is expressed and is an epigenetic drug target in chondrosarcoma, Ewing's sarcoma, osteosarcoma, and rhabdomyosarcoma
    Idriss M Bennani-Baiti
    Children s Cancer Research Institute, St Anna Kinderkrebsforschung, A 1090 Vienna, Austria
    Hum Pathol 43:1300-7. 2012
    ..microarray-coupled immunohistochemical analyses and confirmed lysine-specific demethylase 1 overexpression in rhabdomyosarcoma and synovial sarcoma...
  13. pmc A set of imprinted genes required for normal body growth also promotes growth of rhabdomyosarcoma cells
    Geoffrey Rezvani
    Program on Developmental Endocrinology and Genetics, Eunice Kennedy Shriver National Institute of Child Health and Human Development, National Institutes of Health, Bethesda, Maryland, USA
    Pediatr Res 71:32-8. 2012
    ..We hypothesized that the abnormal persistent rapid proliferation in embryonal cancers occurs in part because of abnormal persistent high expression of growth-promoting imprinted genes...
  14. pmc Response to chemotherapy and predictors of survival in adult rhabdomyosarcoma
    N F Esnaola
    Division of Surgical Oncology, Department of Surgery, Radiation Oncology and Pathology, Brigham and Women's Hospital, Dana Farber Cancer Institute, Harvard Medical School, Boston, Massachusetts 02115, USA
    Ann Surg 234:215-23. 2001
    OBJECTIVE: To assess outcome and identify predictors of survival of adults with rhabdomyosarcoma. SUMMARY BACKGROUND DATA: The literature on adult rhabdomyosarcoma is limited...
  15. ncbi Anaplastic lymphoma kinase aberrations in rhabdomyosarcoma: clinical and prognostic implications
    J Carlijn van Gaal
    Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    J Clin Oncol 30:308-15. 2012
    The aim of this study is to investigate anaplastic lymphoma kinase (ALK) protein expression and underlying genetic aberrations in rhabdomyosarcoma (RMS), with special attention to clinical and prognostic implications.
  16. pmc MEK/ERK inhibitor U0126 increases the radiosensitivity of rhabdomyosarcoma cells in vitro and in vivo by downregulating growth and DNA repair signals
    Francesco Marampon
    Francesco Marampon, Department of Experimental Medicine, Division of Radiotherapy and Radiobiology Laboratory, University of L Aquila Via Vetoio, Coppito 2, 67100, Italy L Aquila
    Mol Cancer Ther 10:159-68. 2011
    ..in vitro and in vivo growth and angiogenic signaling; and induces myogenic differentiation in the embryonal rhabdomyosarcoma (ERMS) cell lines (RD)...
  17. ncbi Prognostic factors after relapse in nonmetastatic rhabdomyosarcoma: a nomogram to better define patients who can be salvaged with further therapy
    Julia C Chisholm
    Department of Paediatric Oncology, Royal Marsden Hospital, Down s Rd, Sutton, SM2 5PT, United Kingdom
    J Clin Oncol 29:1319-25. 2011
    Previous studies suggest poor outcome in children with relapsed rhabdomyosarcoma (RMS). A better understanding is needed of which patients can be salvaged after first relapse.
  18. pmc A novel chemical screening strategy in zebrafish identifies common pathways in embryogenesis and rhabdomyosarcoma development
    Xiuning Le
    Stem Cell Program and Division of Hematology Oncology, Children s Hospital and Dana Farber Cancer Institute, Howard Hughes Medical Institute, Harvard Stem Cell Institute, Harvard Medical School, Boston, MA 02115, USA
    Development 140:2354-64. 2013
    ..A KRAS(G12D)-induced zebrafish embryonal rhabdomyosarcoma was then used to assess the therapeutic effects of the small molecules...
  19. pmc MyoD and E-protein heterodimers switch rhabdomyosarcoma cells from an arrested myoblast phase to a differentiated state
    Zhihong Yang
    Division of Human Biology, Fred Hutchinson Cancer Research Center, Seattle, Washington 98109, USA
    Genes Dev 23:694-707. 2009
    ..types, we have sought to determine the molecular mechanisms that prevent MyoD activity in human embryonal rhabdomyosarcoma cells...
  20. pmc Identification of FGFR4-activating mutations in human rhabdomyosarcomas that promote metastasis in xenotransplanted models
    James G Taylor
    Pulmonary and Vascular Medicine Branch, National Heart, Lung, and Blood Institute NHLBI, NIH, Bethesda, Maryland 20892 4605, USA
    J Clin Invest 119:3395-407. 2009
    b>Rhabdomyosarcoma (RMS) is a childhood cancer originating from skeletal muscle, and patient survival is poor in the presence of metastatic disease. Few determinants that regulate metastasis development have been identified...
  21. ncbi Patched target Igf2 is indispensable for the formation of medulloblastoma and rhabdomyosarcoma
    H Hahn
    Institute of Pathology, TUM Technical University of Munich GSF Research Center of Environment and Health, Ingolstadter Landstrasse 1, 85758 Neuherberg, Federal Republic of Germany
    J Biol Chem 275:28341-4. 2000
    b>Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children (Dagher, R., and Helman, L...
  22. ncbi Prognostic factors and clinical outcomes in children and adolescents with metastatic rhabdomyosarcoma--a report from the Intergroup Rhabdomyosarcoma Study IV
    John C Breneman
    Children s Hospital Medical Center, Division of Radiation Oncology, Cincinnati, OH 45219 0757, USA
    J Clin Oncol 21:78-84. 2003
    To identify risk factors associated with outcomes in children with metastatic rhabdomyosarcoma (RMS) treated on the fourth Intergroup Rhabdomyosarcoma Study (IRS-IV).
  23. pmc Malignant mesenchymal tumor with leiomyosarcoma, rhabdomyosarcoma, chondrosarcoma, and osteosarcoma differentiation: case report and literature review
    Yao Feng Li
    Department of Pathology, Tri Service General Hospital, Taipei, Taiwan, China
    Diagn Pathol 6:35. 2011
    A case of malignant mesenchymoma of the bladder containing leiomyosarcoma, rhabdomyosarcoma, chondrosarcoma, osteosarcoma, and myxomatous components is described...
  24. ncbi Genetic mapping of a Ptch1-associated rhabdomyosarcoma susceptibility locus on mouse chromosome 2
    Heidi Hahn
    Institute of Human Genetics, University of Gottingen, Heinrich Düker Weg 12, 37073 Gottingen, Federal Republic of Germany
    Genomics 84:853-8. 2004
    ..show genetic background-dependent susceptibility to the development of muscle tumors resembling human rhabdomyosarcoma (RMS); BALB/c (BALB) is a susceptible strain whereas C57BL/6 (B6) shows resistance...
  25. ncbi Differential mechanisms of acquired resistance to insulin-like growth factor-i receptor antibody therapy or to a small-molecule inhibitor, BMS-754807, in a human rhabdomyosarcoma model
    Fei Huang
    Bristol Myers Squibb Company, Princeton, NJ 08543, USA
    Cancer Res 70:7221-31. 2010
    ..In light of this problem, we developed two resistant models from the rhabdomyosarcoma cell line Rh41: Rh41-807R, with acquired resistance to BMS-754807, a small-molecule dual-kinase inhibitor ..
  26. ncbi Restoration of p53 pathway by nutlin-3 induces cell cycle arrest and apoptosis in human rhabdomyosarcoma cells
    Mitsuru Miyachi
    Department of Pediatrics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
    Clin Cancer Res 15:4077-84. 2009
    Seventy to eighty percent of rhabdomyosarcoma (RMS) tumors retain wild-type p53. The tumor suppressor p53 plays a central role in inducing cell cycle arrest or apoptosis in response to various stresses...
  27. pmc Efficient lysis of rhabdomyosarcoma cells by cytokine-induced killer cells: implications for adoptive immunotherapy after allogeneic stem cell transplantation
    Selim Kuci
    University Children s Hospital III, University Children s Hospital III, Department of Hematology Oncology Department of Hematology Oncology, Theodor Stern Kai 7
    Haematologica 95:1579-86. 2010
    b>Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and has a poor prognosis...
  28. ncbi Genomic imbalances in rhabdomyosarcoma cell lines affect expression of genes frequently altered in primary tumors: an approach to identify candidate genes involved in tumor development
    Edoardo Missiaglia
    Molecular Cytogenetics Team, The Institute of Cancer Research, Sutton, Surrey SM2 5NG, UK
    Genes Chromosomes Cancer 48:455-67. 2009
    ....
  29. ncbi Molecular pathogenesis of rhabdomyosarcoma
    Shujuan J Xia
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 36th Street and Hamilton Walk, Philadelphia, Pennsylvania 19104 6082, USA
    Cancer Biol Ther 1:97-104. 2002
    b>Rhabdomyosarcoma (RMS) is a family of soft tissue tumors that are associated with the skeletal muscle lineage and generally occur in the pediatric population...
  30. pmc Distinct roles for miR-1 and miR-133a in the proliferation and differentiation of rhabdomyosarcoma cells
    Prakash K Rao
    Whitehead Institute for Biomedical Research, 9 Cambridge Center, Cambridge, MA 02142, USA
    FASEB J 24:3427-37. 2010
    b>Rhabdomyosarcoma is the most common soft tissue sarcoma in the pediatric population...
  31. pmc Inhibition of Notch3 signalling induces rhabdomyosarcoma cell differentiation promoting p38 phosphorylation and p21(Cip1) expression and hampers tumour cell growth in vitro and in vivo
    L Raimondi
    Department of Oncohematology, Ospedale Pediatrico Bambino Gesu, IRCCS, Roma, Italy
    Cell Death Differ 19:871-81. 2012
    b>Rhabdomyosarcoma (RMS) is a paediatric soft-tissue sarcoma arising from skeletal muscle precursors coexpressing markers of proliferation and differentiation. Inducers of myogenic differentiation suppress RMS tumourigenic phenotype...
  32. ncbi Antitumor activity of sustained N-myc reduction in rhabdomyosarcomas and transcriptional block by antigene therapy
    Roberto Tonelli
    Department of Pediatric Hematology, University of Bologna, Bologna, Italy
    Clin Cancer Res 18:796-807. 2012
    ..Our aim was to determine the prevalence of N-Myc protein expression and the potential therapeutic effects of reducing expression in rhabdomyosarcomas, including use of an antigene strategy that inhibits transcription...
  33. ncbi Survival after relapse in children and adolescents with rhabdomyosarcoma: A report from the Intergroup Rhabdomyosarcoma Study Group
    A S Pappo
    Intergroup Rhabdomyosarcoma Study Group, Rochester, MN 55905 0001, USA
    J Clin Oncol 17:3487-93. 1999
    Despite advances in therapy, nearly 30% of children with rhabdomyosarcoma experience progressive or relapsed disease, which is often fatal.
  34. pmc Nonpeptidic urotensin-II receptor antagonists I: in vitro pharmacological characterization of SB-706375
    Stephen A Douglas
    CVU Department of Biology, Cardiovascular and Urogenital and Respiratory and Inflammation Centers of Excellence for Drug Discovery, GlaxoSmithKline, 709 Swedeland Road, UW2510 King of Prussia, Pa 19406 0939, USA
    Br J Pharmacol 145:620-35. 2005
    ..6. In summary, SB-706375 is a high-affinity, surmountable, reversible and selective nonpeptide UT receptor antagonist with cross-species activity that will assist in delineating the pathophysiological actions of U-II in mammals...
  35. ncbi TGF-beta autocrine loop regulates cell growth and myogenic differentiation in human rhabdomyosarcoma cells
    M Bouche
    Department of Histology and Medical Embryology, University of Rome La Sapienza, 00161, Rome, Italy
    FASEB J 14:1147-58. 2000
    ..factor beta (TGF) is a well-known inhibitor of myogenic differentiation as well as an autocrine product of rhabdomyosarcoma cells...
  36. ncbi Prognostic factors in metastatic rhabdomyosarcomas: results of a pooled analysis from United States and European cooperative groups
    Odile Oberlin
    Department of Paediatric Oncology, Institut Gustave Roussy, rue Camille Desmoulins, 94805 Villejuif Cedex, France
    J Clin Oncol 26:2384-9. 2008
    To identify risk factors associated with outcome in children with metastatic rhabdomyosarcoma in a large cohort of patients
  37. pmc Comparison of genome-wide binding of MyoD in normal human myogenic cells and rhabdomyosarcomas identifies regional and local suppression of promyogenic transcription factors
    Kyle L MacQuarrie
    Division of Human Biology, Fred Hutchinson Cancer Research Center, Seattle, Washington, USA
    Mol Cell Biol 33:773-84. 2013
    b>Rhabdomyosarcoma is a pediatric tumor of skeletal muscle that expresses the myogenic basic helix-loop-helix protein MyoD but fails to undergo terminal differentiation...
  38. ncbi Inhibition of connective tissue growth factor (CTGF/CCN2) expression decreases the survival and myogenic differentiation of human rhabdomyosarcoma cells
    Stefania Croci
    Cancer Research Section, Department of Experimental Pathology, University of Bologna, Bologna, Italy
    Cancer Res 64:1730-6. 2004
    ..of the CCN (Cyr61, CTGF, Nov) family of genes, emerged from a microarray screen of genes expressed by human rhabdomyosarcoma cells. Rhabdomyosarcoma is a soft tissue sarcoma of childhood deriving from skeletal muscle cells...
  39. ncbi Diffusion-weighted MR imaging in monitoring the effect of a vascular targeting agent on rhabdomyosarcoma in rats
    Harriet C Thoeny
    Department of Radiology, University Hospitals Leuven, Herestraat 49, B 3000 Leuven, Belgium
    Radiology 234:756-64. 2005
    ..To evaluate diffusion-weighted magnetic resonance (MR) imaging for monitoring tumor response in rats after administration of combretastatin A4 phosphate...
  40. ncbi Phosphorylation regulates transcriptional activity of PAX3/FKHR and reveals novel therapeutic possibilities
    Ralf Amstutz
    Department of Oncology and Division of Clinical Chemistry and Biochemistry, University Children s Hospital, University Hospital Zurich, Zurich, Switzerland
    Cancer Res 68:3767-76. 2008
    ..One example of this is alveolar rhabdomyosarcoma (aRMS), in which specific translocations lead to the formation of the chimeric transcription factor PAX3/FKHR...
  41. ncbi A caspase-8-independent component in TRAIL/Apo-2L-induced cell death in human rhabdomyosarcoma cells
    I Petak
    Division of Molecular Therapeutics, Department of Hematology Oncology, St Jude Children s Research Hospital, Memphis, Tennesse 38105, USA
    Cell Death Differ 10:729-39. 2003
    ..signaling downstream of receptor activation were examined in TRAIL - sensitive and -resistant models of human rhabdomyosarcoma (RMS)...
  42. ncbi Radiation-induced rhabdomyosarcoma of the brainstem in a patient with neurofibromatosis type 2
    Matthew L Carlson
    Department of Otorhinolaryngology, Mayo Clinic, Rochester, Minnesota 55905, USA
    J Neurosurg 112:81-7. 2010
    ..The authors present the first documented case of rhabdomyosarcoma following SRT for multiple NF2-associated schwannomas...
  43. pmc Drozitumab, a human antibody to death receptor 5, has potent antitumor activity against rhabdomyosarcoma with the expression of caspase-8 predictive of response
    Zhigang Kang
    Genetics Branch, Center for Cancer Research, National Cancer Institute, Bethesda, MD 20892, USA
    Clin Cancer Res 17:3181-92. 2011
    b>Rhabdomyosarcoma (RMS) is a common pediatric soft-tissue tumor. In this study, we evaluated the efficacy and selectivity of drozitumab, a death receptor DR5-targeted therapeutic antibody, in RMS preclinical models.
  44. ncbi Amplification of CDK4, MDM2, SAS and GLI genes in leiomyosarcoma, alveolar and embryonal rhabdomyosarcoma
    P Ragazzini
    Laboratory of Oncologic Research, Rizzoli Orthopaedic Institute, Bologna, Italy
    Histol Histopathol 19:401-11. 2004
    ..ERMS), to ascertain genomic alterations and possible differences within histologic subtypes of rhabdomyosarcoma (RMS)...
  45. pmc Disruption of imprinted genes at chromosome region 11p15.5 in paediatric rhabdomyosarcoma
    J Anderson
    Section of Paediatric Oncology, Institute of Cancer Research, Sutton, Surrey, UK
    Neoplasia 1:340-8. 1999
    ..Moreover, in tumours with p57(KIP2) expression, there was no evidence for inactivating mutations, suggesting that p57(KIP2) is not a tumour suppressor in rhabdomyosarcoma.
  46. ncbi Time-point and dosage of gene inactivation determine the tumor spectrum in conditional Ptch knockouts
    Arne Zibat
    Institute of Human Genetics, University of Goettingen, Germany
    Carcinogenesis 30:918-26. 2009
    ..in Patched (PTCH) have been associated with tumors characteristic both for children [medulloblastoma (MB) and rhabdomyosarcoma (RMS)] and for elderly [basal cell carcinoma (BCC)]...
  47. ncbi Two small molecule compounds, LLL12 and FLLL32, exhibit potent inhibitory activity on STAT3 in human rhabdomyosarcoma cells
    Chang Ching Wei
    Department of Pediatrics, China Medical University Hospital, Taichung, Taiwan, R O C
    Int J Oncol 38:279-85. 2011
    ....
  48. pmc Hedgehog pathway activity in pediatric embryonal rhabdomyosarcoma and undifferentiated sarcoma: a report from the Children's Oncology Group
    Joseph G Pressey
    Division of Oncology, Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Pediatr Blood Cancer 57:930-8. 2011
    ..The spectrum of tumors found in Gorlin Syndrome includes basal cell carcinoma, medulloblastoma, and rarely, rhabdomyosarcoma (RMS)...
  49. ncbi Association of ataxia telangiectasia mutated (ATM) gene mutation/deletion with rhabdomyosarcoma
    Peilin Zhang
    Department of Pathology, West Virginia University Health Sciences Center, Morgontown, West Virginia 26506 9203, USA
    Cancer Biol Ther 2:87-91. 2003
    b>Rhabdomyosarcoma is a common malignancy in children. There are two major types of rhabdomyosarcomas, the embryonal and the alveolar, differing in cytogenetic and morphologic features...
  50. ncbi Overexpression of insulin-like growth factor binding protein-6 inhibits rhabdomyosarcoma growth in vivo
    M A Gallicchio
    Department of Medicine, University of Melbourne, Austin and Repatriation Medical Centre (Austin Campus) Heidelberg, Victoria, Australia
    Int J Cancer 94:645-51. 2001
    b>Rhabdomyosarcoma is the most common soft-tissue sarcoma of childhood...
  51. ncbi Concomitant administration of vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide for high-risk sarcomas: the St. Jude Children's Research Hospital experience
    Fariba Navid
    Department of Hematology Oncology, St Jude Children s Research Hospital, Memphis, Tennessee 38105 2794, USA
    Cancer 106:1846-56. 2006
    ..Vincristine, doxorubicin, cyclophosphamide, ifosfamide, and etoposide are highly effective against pediatric sarcomas. The authors investigated the feasibility of administering these agents concomitantly within a defined period...
  52. pmc CCI-779 inhibits rhabdomyosarcoma xenograft growth by an antiangiogenic mechanism linked to the targeting of mTOR/Hif-1alpha/VEGF signaling
    Xiaolin Wan
    Molecular Oncology Section, Pediatric Oncology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892 1928, USA
    Neoplasia 8:394-401. 2006
    ..antitumor activity of CCI-779 is related to antiangiogenic effects in vivo in tumors of mice bearing human rhabdomyosarcoma (RMS) xenografts...
  53. ncbi High-resolution array CGH identifies common mechanisms that drive embryonal rhabdomyosarcoma pathogenesis
    Vera Paulson
    Department of Pediatrics, The University of Texas Southwestern Medical Center, Dallas, TX, USA
    Genes Chromosomes Cancer 50:397-408. 2011
    Pediatric rhabdomyosarcoma occurs as two biologically distinct histological variants, embryonal (ERMS) and alveolar (ARMS)...
  54. pmc PAX3-FOXO1 fusion gene in rhabdomyosarcoma
    Corinne M Linardic
    Department of Pediatrics and Pharmacology, Duke University Medical Center, Durham, NC 27710, USA
    Cancer Lett 270:10-8. 2008
    b>Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and adolescence...
  55. ncbi Deregulation of the hedgehog signalling pathway: a possible role for the PTCH and SUFU genes in human rhabdomyoma and rhabdomyosarcoma development
    Ulrica Tostar
    Karolinska Institute, Department of Bioscience at Novum, SE 14157 Huddinge, Sweden
    J Pathol 208:17-25. 2006
    ..loss of heterozygosity (LOH) in the PTCH region with two of these (one fetal rhabdomyoma and one embryonal rhabdomyosarcoma) also showing LOH in the SUFU region...
  56. ncbi Transcriptomic and proteomic analyses of rhabdomyosarcoma cells reveal differential cellular gene expression in response to enterovirus 71 infection
    Wai Fook Leong
    Human Genome Laboratory, Department of Microbiology, Yong Loo Lin School of Medicine, National University of Singapore, Kent Ridge, Singapore 117597
    Cell Microbiol 8:565-80. 2006
    ..An oligo-based microarray was employed to analyse mRNAs from rhabdomyosarcoma cells infected with the MS/7423/87 strain of enterovirus 71 (EV71) at 20 h post infection...
  57. pmc Regulation of expression of stromal-derived factor-1 receptors: CXCR4 and CXCR7 in human rhabdomyosarcomas
    Maciej Tarnowski
    Stem Cell Institute at James Graham Brown Cancer Center, University of Louisville, 500 South Floyd Street, Room 107, Louisville, KY 40202, USA
    Mol Cancer Res 8:1-14. 2010
    ..The upregulation of CXCR4 and downregulation of CXCR7 expression by PAX3-FKHR or hypoxia may give SDF-1 an advantage to better engage the CXCR4 receptor, thus increasing RMS motility...
  58. ncbi Rearrangement of the PAX3 paired box gene in the paediatric solid tumour alveolar rhabdomyosarcoma
    F G Barr
    Division of Human Genetics and Molecular Biology, Children s Hospital of Philadelphia, Pennsylvania
    Nat Genet 3:113-7. 1993
    ..chromosome 2 locus rearranged by the t(2;13)(q35;q14) translocation of the paediatric solid tumour alveolar rhabdomyosarcoma. The rearrangement breakpoints occur within an intron downstream of the paired box and homeodomain-encoding ..
  59. ncbi Subtype and prognostic classification of rhabdomyosarcoma by immunohistochemistry
    Marco Wachtel
    University Children s Hospital, Division of Oncology, Zurich, Switzerland
    J Clin Oncol 24:816-22. 2006
    b>Rhabdomyosarcoma (RMS) is classified into two main subgroups: the embryonal (ERMS) and the alveolar (ARMS) form. The majority of the ARMSs are associated with specific chromosomal translocations (pARMS)...
  60. ncbi Rhabdomyosarcomas in adults and children: an update
    David M Parham
    Department of Pathology, University of Arkansas for Medical Sciences and Arkansas Children s Hospital, Little Rock 72202, USA
    Arch Pathol Lab Med 130:1454-65. 2006
    ....
  61. pmc Tumour formation by single fibroblast growth factor receptor 3-positive rhabdomyosarcoma-initiating cells
    M Hirotsu
    Department of Orthopaedic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, 8 35 1 Sakuragaoka, Kagoshima 890 8520, Japan
    Br J Cancer 101:2030-7. 2009
    ..The objective of this study was to identify and characterise a subpopulation of human sarcoma-initiating cells...
  62. ncbi Global gene expression profiling of PAX-FKHR fusion-positive alveolar and PAX-FKHR fusion-negative embryonal rhabdomyosarcomas
    M Lae
    Department of Pathology, Memorial Sloan Kettering Cancer Center, New York, NY 10021, USA
    J Pathol 212:143-51. 2007
    ..The gene expression signature of ARMS provides a source of potential diagnostic markers, therapeutic targets, and PAX-FKHR downstream genes, and can be used to reliably distinguish these sarcomas from ERMS...
  63. ncbi Expression of receptor tyrosine kinases and apoptotic molecules in rhabdomyosarcoma: correlation with overall survival in 105 patients
    Paul M Armistead
    Department of Sarcoma Medical Oncology, The University of Texas M D Anderson Cancer Center, Houston, Texas 77030, USA
    Cancer 110:2293-303. 2007
    b>Rhabdomyosarcoma (RMS) is a rare mesenchymal tumor with few treatment options after the failure of first-line therapy...
  64. pmc Molecular classification of rhabdomyosarcoma--genotypic and phenotypic determinants of diagnosis: a report from the Children's Oncology Group
    Elai Davicioni
    Department of Pathology, Keck School of Medicine, University of Southern California, Los Angeles, California, USA
    Am J Pathol 174:550-64. 2009
    b>Rhabdomyosarcoma (RMS) in children occurs as two major histological subtypes, embryonal (ERMS) and alveolar (ARMS). ERMS is associated with an 11p15...
  65. pmc MicroRNA reexpression as differentiation therapy in cancer
    Prasun J Mishra
    Laboratory of Cancer Biology and Genetics, National Cancer Institute, NIH, Bethesda, Maryland 20892 4264, USA
    J Clin Invest 119:2119-23. 2009
    ..demonstrate that the miRNAs miR-1/miR-206, which are routinely lost in advanced, poorly differentiated rhabdomyosarcoma (RMS) but characteristically expressed in the mature skeletal muscle from which these tumors arise, restore ..
  66. ncbi Enhancement of radiation response in osteosarcoma and rhabdomyosarcoma cell lines by histone deacetylase inhibition
    Claudia Blattmann
    Department of Pediatric Oncology, University of Heidelberg, Heidelberg, Germany
    Int J Radiat Oncol Biol Phys 78:237-45. 2010
    ..We investigated the effect of pan-HDACIs such as suberoylanilide hydroxamic acid (SAHA) on radiation response in two osteosarcoma (OS) and two rhabdomyosarcoma (RMS) cell lines.
  67. pmc Classification and diagnostic prediction of cancers using gene expression profiling and artificial neural networks
    J Khan
    Cancer Genetics Branch, National Human Genome Research Institute, National Institutes of Health, Bethesda, Maryland, USA
    Nat Med 7:673-9. 2001
    ..This study demonstrates the potential applications of these methods for tumor diagnosis and the identification of candidate targets for therapy...
  68. ncbi Analysis of prognostic factors in patients with nonmetastatic rhabdomyosarcoma treated on intergroup rhabdomyosarcoma studies III and IV: the Children's Oncology Group
    Jane L Meza
    Department of Preventive and Societal Medicine, 984350 Nebraska Medical Center, Omaha, NE 68198 4350, USA
    J Clin Oncol 24:3844-51. 2006
    The outcome for localized rhabdomyosarcoma (RMS) or undifferentiated sarcoma (UDS) is affected by age, histology, primary anatomic site, extent of disease, and therapy.
  69. pmc Sclerosing rhabdomyosarcoma presenting in the masseter muscle: a case report
    Xu Yong Lin
    Department of Pathology, The First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang 110001, China
    Diagn Pathol 8:18. 2013
    Sclerosing rhabdomyosarcoma (SRMS) is exceedingly rare, and may cause a great diagnostic confusion. Histologically, it is characterized by abundant extracellular hyalinized matrix mimicking primitive chondroid or osteoid tissue...
  70. ncbi Mutations of the PTPN11 and RAS genes in rhabdomyosarcoma and pediatric hematological malignancies
    Yuyan Chen
    Department of Pediatrics, Graduate School of Medicine, University of Tokyo, Tokyo, Japan
    Genes Chromosomes Cancer 45:583-91. 2006
    ..risk of some malignancies, notably leukemia and probably some solid tumors including neuroblastoma (NB) and rhabdomyosarcoma (RMS), recent studies have reported that gain-of-function somatic mutations in PTPN11 occur in some ..
  71. ncbi Synthetic lethal interaction between PI3K/Akt/mTOR and Ras/MEK/ERK pathway inhibition in rhabdomyosarcoma
    Monika Katharina Guenther
    Institute for Experimental Cancer Research in Pediatrics, Goethe University Frankfurt, Frankfurt, Germany
    Cancer Lett 337:200-9. 2013
    b>Rhabdomyosarcoma (RMS) frequently exhibits concomitant activation of the PI3K/Akt/mTOR and the Ras/MEK/ERK pathways. Therefore, we investigated whether pharmacological cotargeting of these two key survival pathways suppresses RMS growth...
  72. ncbi Mechanisms of impaired differentiation in rhabdomyosarcoma
    Charles Keller
    Pediatric Cancer Biology Program, Papé Family Pediatric Research Institute, Department of Pediatrics, Oregon Health and Science University, Portland, OR, USA
    FEBS J 280:4323-34. 2013
    b>Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, with presumed skeletal muscle origins, because of its myogenic phenotype. RMS is composed of two main subtypes, embryonal RMS (eRMS) and alveolar RMS (aRMS)...
  73. ncbi MicroRNAs involved in skeletal muscle development and their roles in rhabdomyosarcoma pathogenesis
    Jan Novak
    Faculty of Medicine, Department of Physiology, Masaryk University, Brno, Czech Republic Faculty of Medicine, Department of Pathological Physiology, Masaryk University, Brno, Czech Republic
    Pediatr Blood Cancer 60:1739-46. 2013
    ..b>Rhabdomyosarcoma (RMS) represents the most common soft tissue tumor in the pediatric population...
  74. ncbi Outcomes and prognostic factors after recurrence in children and adolescents with nonmetastatic rhabdomyosarcoma
    Stefano Mazzoleni
    Division of Pediatrics, Maternity and Children s Department, Hospital of Camposampiero, Padua, Italy
    Cancer 104:183-90. 2005
    Although > 90% of children with nonmetastatic rhabdomyosarcoma (RMS) achieve complete remission with current treatment, up to one-third of them experience a recurrence...
  75. ncbi Leukemia inhibitory factor: a newly identified metastatic factor in rhabdomyosarcomas
    Marcin Wysoczynski
    James Graham Brown Cancer Center, University of Louisville, 580 South Preston Street, Louisville, KY 40202, USA
    Cancer Res 67:2131-40. 2007
    b>Rhabdomyosarcoma frequently infiltrates bone marrow and this process involves the stromal-derived factor-1 (SDF-1)-CXCR4 axis...
  76. ncbi Dedifferentiated liposarcomas with divergent myosarcomatous differentiation developed in the internal trunk: a study of 27 cases and comparison to conventional dedifferentiated liposarcomas and leiomyosarcomas
    Matthieu Bui Nguyen Binh
    Department of Pathology, Institut Bergonié and Université Victor Segalen, Bordeaux, France
    Am J Surg Pathol 31:1557-66. 2007
    ..In about 5% cases, the dedifferentiated component is an heterologous sarcoma such as leiomyosarcoma or rhabdomyosarcoma. We reviewed a series of 65 sarcomas with a myogenic differentiation developed in the internal trunk for ..
  77. pmc Distinct effects of ligand-induced PDGFRα and PDGFRβ signaling in the human rhabdomyosarcoma tumor cell and stroma cell compartments
    Monika Ehnman
    Department of Oncology Pathology, Karolinska Institutet, Stockholm, Sweden
    Cancer Res 73:2139-49. 2013
    ..growth factor receptors (PDGFR) α and β have been suggested as potential targets for treatment of rhabdomyosarcoma, the most common soft tissue sarcoma in children...
  78. ncbi Novel mdm2 splice variants identified in pediatric rhabdomyosarcoma tumors and cell lines
    F Bartel
    Department of Molecular Pharmacology, St Jude Children s Research Hospital, Memphis, TN 38105, USA
    Oncol Res 12:451-7. 2001
    ..This report describes expression of 11 different mdm2 splice variants in pediatric rhabdomyosarcoma (RMS) cell lines and tumors at a frequency of 75% and 82%, respectively...
  79. pmc High-dose chemotherapy followed by autologous stem cell transplantation for metastatic rhabdomyosarcoma--a systematic review
    Frank Peinemann
    Institute for Quality and Efficiency in Health Care IQWiG, Cologne, Germany
    PLoS ONE 6:e17127. 2011
    Patients with metastatic rhabdomyosarcoma (RMS) have a poor prognosis...
  80. pmc MicroRNAs in rhabdomyosarcoma: pathogenetic implications and translational potentiality
    Rossella Rota
    Department of Oncohematology, Ospedale Pediatrico Bambino Gesu, IRCCS, Roma, Italy
    Mol Cancer 10:120. 2011
    ..In recent years, microRNAs have emerged as prominent players in cancer as well. Rhabdomyosarcoma is a pediatric skeletal muscle-derived soft-tissue sarcoma that originates from myogenic precursors arrested ..
  81. ncbi In vivo evaluation of ixabepilone (BMS247550), a novel epothilone B derivative, against pediatric cancer models
    Jennifer K Peterson
    Department of Molecular Pharmacology, St Jude Children s Research Hospital, Memphis, Tennessee 38105, USA
    Clin Cancer Res 11:6950-8. 2005
    ..The purpose of the current study was to evaluate the antitumor activity of ixabepilone, an epothilone B derivative representing a new class of microtubule-stabilizing antimitotic agent in a wide variety of pediatric solid tumor models...
  82. pmc PAX3-FKHR induces morphological change and enhances cellular proliferation and invasion in rhabdomyosarcoma
    J Anderson
    Unit of Molecular Haematology, Institute of Child Health and Great Ormond Street Hospital for Children, London, United Kingdom
    Am J Pathol 159:1089-96. 2001
    Alveolar rhabdomyosarcoma (ARMS) is consistently associated with the characteristic translocations t(2;13)(q35;q14) and t(1;13)(p36;q14), which encode for the PAX3-FKHR and PAX7-FKHR fusion oncoproteins respectively...
  83. ncbi Duchenne muscular dystrophy-rhabdomyosarcoma, ichthyosis vulgaris/acute monoblastic leukemia: association of rare genetic disorders and childhood malignant diseases
    Zsuzsanna Jakab
    Department of Pediatrics, Medical and Health Science Center, University of Debrecen, Debrecen, Hungary
    Med Pediatr Oncol 39:66-8. 2002
  84. ncbi Cell-type-specific regulation of distinct sets of gene targets by Pax3 and Pax3/FKHR
    Salma Begum
    Department of Laboratory Medicine and Pathobiology, Faculty of Medicine, University of Toronto, Toronto, Ontario, Canada M5S 1A8
    Oncogene 24:1860-72. 2005
    ..BMP4 expression, for example, was repressed by both Pax3 and Pax3/FKHR in SaOS-2 cells, while in the rhabdomyosarcoma, RD, Pax3/FKHR, but not Pax3, induced BMP4 expression...
  85. pmc Dual blockade of the PI3K/AKT/mTOR (AZD8055) and RAS/MEK/ERK (AZD6244) pathways synergistically inhibits rhabdomyosarcoma cell growth in vitro and in vivo
    Jane Renshaw
    Authors Affiliations Divisions of Clinical Studies, Cancer Therapeutics, and Molecular Pathology, The Institute of Cancer Research, Sutton, Surrey Histopathology Department, The Royal Marsden Hospital NHS Foundation Trust, London, United Kingdom and Department of Neuropathology, University of Bonn, Bonn, Germany
    Clin Cancer Res 19:5940-51. 2013
    ..To provide rationale for using phosphoinositide 3-kinase (PI3K) and/or mitogen-activated protein kinase (MAPK) pathway inhibitors to treat rhabdomyosarcomas, a major cause of pediatric and adolescent cancer deaths...
  86. ncbi Relationship between MYCN copy number and expression in rhabdomyosarcomas and correlation with adverse prognosis in the alveolar subtype
    Daniel Williamson
    Molecular Cytogenetics, The Institute of Cancer Research, Sutton, Surrey, United Kingdom
    J Clin Oncol 23:880-8. 2005
    ..Increased copy number and overexpression of MYCN in the pediatric cancer rhabdomyosarcoma has been described in a number of small studies with conflicting conclusions about its association with ..
  87. ncbi p53 mutation and loss have different effects on tumourigenesis in a novel mouse model of pleomorphic rhabdomyosarcoma
    Brendan Doyle
    Beatson Institute for Cancer Research, Garscube Estate, Switchback Road, Glasgow, G61 1BD, UK
    J Pathol 222:129-37. 2010
    Pleomorphic rhabdomyosarcoma is the most common variant of this tumour in adults and has a very poor outcome. Two genes which are known to play a role in rhabdomyosarcoma development are KRas and p53...
  88. ncbi Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas
    F B Ruymann
    Children s Hospital, Columbus, Ohio, USA
    Cancer Invest 18:223-41. 2000
    Advances in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomas continue in the Intergroup Rhabdomyosarcoma Study Group (IRSG) and European cooperative groups...
  89. pmc Inhibition of phosphorylated c-Met in rhabdomyosarcoma cell lines by a small molecule inhibitor SU11274
    Jinxuan Hou
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha 68105, USA
    J Transl Med 9:64. 2011
    ..In this study, we investigated the role of c-Met in rhabdomyosarcoma (RMS) using its small molecule inhibitor SU11274, which has been hypothesized to be a potential therapeutic ..
  90. ncbi Normal and rearranged PAX3 expression in human rhabdomyosarcoma
    E Frascella
    Department of Pediatrics, University of Padova, Italy
    Cancer Genet Cytogenet 102:104-9. 1998
    ..with the FKHR gene it is involved in the t(2;13)(q35;q14), a specific translocation associated with alveolar rhabdomyosarcoma (ARMS). As a consequence of the rearrangement two chimeric transcripts originate: FKHR-PAX3 and PAX3-FKHR...
  91. ncbi Cannabinoid receptor 1 is a potential drug target for treatment of translocation-positive rhabdomyosarcoma
    Susanne Oesch
    Department of Oncology, University Children s Hospital, Zurich, Switzerland
    Mol Cancer Ther 8:1838-45. 2009
    ..profiling has revealed that the gene coding for cannabinoid receptor 1 (CB1) is highly up-regulated in rhabdomyosarcoma biopsies bearing the typical chromosomal translocations PAX3/FKHR or PAX7/FKHR...
  92. ncbi Role of GOLPH3 and GOLPH3L in the proliferation of human rhabdomyosarcoma
    Osamu Kunigou
    Department of Orthopaedic Surgery, Graduate School of Medical and Dental Sciences, Kagoshima University, Kagoshima 890 8520, Japan
    Oncol Rep 26:1337-42. 2011
    ..However, the mechanism through which GOLPH3 is involved in the pathogenesis of rhabdomyosarcoma remains unidentified...
  93. ncbi Cyclophosphamide dose intensification during induction therapy for intermediate-risk pediatric rhabdomyosarcoma is feasible but does not improve outcome: a report from the soft tissue sarcoma committee of the children's oncology group
    Sheri L Spunt
    St Jude Children s Research Hospital and University of Tennessee College of Medicine, Memphis, Tennessee 38105 2794, USA
    Clin Cancer Res 10:6072-9. 2004
    More than half of pediatric rhabdomyosarcoma cases have intermediate-risk features and suboptimal outcome (3-year failure-free survival estimates, 55 to 76%). Dose intensification of known active agents may improve outcome.
  94. ncbi Both hepatocyte growth factor (HGF) and stromal-derived factor-1 regulate the metastatic behavior of human rhabdomyosarcoma cells, but only HGF enhances their resistance to radiochemotherapy
    Kacper Jankowski
    Stem Cell Biology Program, James Graham Brown Cancer Center, University of Louisville, 529 South Jackson Street, Louisville, KY 40202, USA
    Cancer Res 63:7926-35. 2003
    ..Signaling from the c-MET receptor may also contribute to the resistance of RMS cells to conventional treatment modalities...
  95. ncbi Fractionated, three-dimensional, planning-assisted proton-radiation therapy for orbital rhabdomyosarcoma: a novel technique
    E B Hug
    Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA, USA
    Int J Radiat Oncol Biol Phys 47:979-84. 2000
    Most children with orbital rhabdomyosarcoma will survive their disease. However, conventional photon-radiation treatment, as part of multimodality therapy, results in varying degrees of long-term functional and cosmetic side effects...
  96. ncbi Hydrophobic statins induce autophagy and cell death in human rhabdomyosarcoma cells by depleting geranylgeranyl diphosphate
    Makoto Araki
    Department of Biochemistry, Meiji Pharmaceutical University, Kiyose, Tokyo 204 8588, Japan
    Eur J Pharmacol 674:95-103. 2012
    ..The results of our earlier work suggested that hydrophobic statins induce autophagy in cultured human rhabdomyosarcoma A204 cells...
  97. pmc Vincristine, actinomycin, and cyclophosphamide compared with vincristine, actinomycin, and cyclophosphamide alternating with vincristine, topotecan, and cyclophosphamide for intermediate-risk rhabdomyosarcoma: children's oncology group study D9803
    Carola A S Arndt
    Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, MN 55905, USA
    J Clin Oncol 27:5182-8. 2009
    The purpose of this study was to compare the outcome of patients with intermediate-risk rhabdomyosarcoma (RMS) treated with standard VAC (vincristine, dactinomycin, and cyclophosphamide) chemotherapy to that of patients treated with VAC ..
  98. pmc MicroRNA-206 expression levels correlate with clinical behaviour of rhabdomyosarcomas
    E Missiaglia
    Molecular Cytogenetics Team, The Institute of Cancer Research, Sutton, Surrey SM2 5NG, UK
    Br J Cancer 102:1769-77. 2010
    ..Our aim was to determine the effects of microRNAs (miRNA) that have been implicated in muscle development on the clinical behaviour of RMSs...
  99. pmc Advances in pediatric rhabdomyosarcoma characterization and disease model development
    D O'BRIEN
    The Center for Childhood Cancer, Columbus Children s Research Institute and The Department of Pediatrics, The Ohio State University, Columbus, Ohio, USA
    Histol Histopathol 27:13-22. 2012
    b>Rhabdomyosarcoma (RMS), a form of soft tissue sarcoma, is one of the most common pediatric malignancies...
  100. ncbi Antitumor effects of a combined 5-aza-2'deoxycytidine and valproic acid treatment on rhabdomyosarcoma and medulloblastoma in Ptch mutant mice
    Ines Ecke
    Institute of Human Genetics, University of Goettingen, Gottingen, Germany
    Cancer Res 69:887-95. 2009
    Patched (Ptch) heterozygous mice develop medulloblastoma (MB) and rhabdomyosarcoma (RMS) resembling the corresponding human tumors...
  101. ncbi Microvascular and tumor cell alterations during continuous hyperfractionated irradiation: an electron microscopic investigation on the rat R1H rhabdomyosarcoma
    D E Lorke
    Institute of Anatomy, Department of Neuroanatomy, University Hospital Eppendorf, Hamburg, Germany
    Int J Radiat Oncol Biol Phys 44:895-904. 1999
    ..The present study was undertaken to assess whether vasculo-connective changes are less pronounced after continuous hyperfractionated irradiation, implying better tumor oxygenation and improved radiosensitivity...

Research Grants63

  1. IRSG STUDIES OF ALVEOLAR RHABDOMYOSARCOMA GENE FUSIONS
    FREDERIC BARR; Fiscal Year: 2005
    DESCRIPTION: Alveolar rhabdomyosarcoma (ARMS) is an aggressive soft tissue tumor of the striated muscle lineage that occurs in children and young adults...
  2. The role of cMET in satellite cells during muscle regeneration
    MICAH TAUBE WEBSTER; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma that occurs in children...
  3. Regulation and function of Six1 in zebrafish muscle development and in rhabdomyos
    JENEAN O'BRIEN; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Rhabdomyosarcoma (RMS) accounts for ~8% of all pediatric cancers, where the overall 5-year survival rate for children diagnosed with metastatic RMS is less than 30%...
  4. Rapamycin-induced Selective Apoptosis in Malignant Cells
    Peter J Houghton; Fiscal Year: 2012
    ..However, the approach is not effective against IGF-2-driven rhabdomyosarcoma (RMS) xenografts...
  5. Cardiac Sparing Whole Lung IMRT in Children and Young Adults with Lung Metastases
    JOHN ANDREW KALAPURAKAL; Fiscal Year: 2012
    ..is considered the standard of care for most patients with lung metastases from Wilms tumor, Ewing Sarcoma and rhabdomyosarcoma. Several studies have shown increased cardiac complications after WLI and chemotherapy...
  6. Pathogenic mechanisms of alveolar rhabdomyosarcoma
    MARGARET MARY CHOU; Fiscal Year: 2013
    ..panel of primary human tumors further revealed high expression specifically in a high percentage of alveolar rhabdomyosarcoma (ARMS) cases...
  7. Transcriptional Coregulation in Pancreatic Adenocarcinoma Progression
    MARK JAKOB FERREIRA; Fiscal Year: 2013
    ..nuclear LMO4 expression in tumor cells correlates with increased metastasis in patients with breast cancer, rhabdomyosarcoma, or oral squamous cell carcinoma...
  8. TAK1/TRAF6 Signaling in Skeletal Muscle
    Ashok Kumar; Fiscal Year: 2013
    ..is the critical determinant of skeletal muscle-wasting in several chronic diseases and also development of rhabdomyosarcoma in children...
  9. Mechanism of regulation for the oncogenic Pax3-FOXO1 in Alveolar Rhabdomyosarcoma
    ANDREW DURRELL HOLLENBACH; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Alveolar Rhabdomyosarcoma (ARMS), an aggressive childhood solid muscle tumor with a poor prognosis, is frequently characterized by a t(2;13) chromosomal translocation resulting in the fusion of two ..
  10. Gene regulation in ARMS pathology;the role of FOXO1 in Pax3-FOXO1 DNA binding
    KELLY ELAINE JOHANSON; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Alveolar Rhabdomyosarcoma (ARMS) patients carrying the t (2;13) (q35;q14) chromosomal translocation have the lowest survival rate of all rhabdomyosarcomas;however, the reason for this fact is not ..
  11. Therapeutic Targets in Alveolar Rhabdomyosarcoma
    Charles Keller; Fiscal Year: 2012
    ..The childhood muscle cancer alveolar rhabdomyosarcoma is a classic example...
  12. PROTON RADIATION THERAPY RESEARCH
    Thomas F DeLaney; Fiscal Year: 2013
    ..to improve outcome for patients with non-small cell lung cancer, liver tumors, pediatric medulloblastoma and rhabdomyosarcoma, spine/skull base sarcomas, and paranasal sinus malignancies...
  13. Translation of Predictive Cancer Biomarkers into Clinical Practice
    TIMOTHY J contact TRICHE; Fiscal Year: 2010
    ..In the case of childhood rhabdomyosarcoma, the most common form of sarcoma in the young, this review is used to establish eligibility as well as ..
  14. Bone and Soft Tissue Tumor Etiology: Role and Function of TRE17/USP6
    MARGARET MARY CHOU; Fiscal Year: 2013
    ..TRE17 overexpression also arises selectively in other BSTTs, particularly the highly lethal alveolar rhabdomyosarcoma (ARMS)...
  15. Pharmacogenetics of Cyclophosphamide ADRs in
    Jeannine S McCune; Fiscal Year: 2012
    ..failure-free survival to vincristine/actinomycin/CY (VAC) in children with newly diagnosed intermediate risk rhabdomyosarcoma. This aim will be completed as a correlative study to a phase III Children's Oncology Group trial...
  16. Dissecting the Signaling Mechanisms of Smoothened
    STACEY KATHRYN OGDEN; Fiscal Year: 2013
    ..the most common cancer in humans, medulloblastoma, the most common malignant brain tumor in children, and rhabdomyosarcoma, where pathway activation correlates with poor prognosis...
  17. TARGETING OF GENES IN THE MAMMALIAN GENOME
    Mario R Capecchi; Fiscal Year: 2013
    ..We have successfully modeled alveolar rhabdomyosarcoma and synovial sarcoma and are in the process of modeling Ewing's sarcoma...
  18. DICER1 and the Pleuropulmonary Blastoma Family Cancer Syndrome
    Dana Ashley Hill; Fiscal Year: 2013
    ..This work will shed light on the role genetics plays in a new pediatric tumor syndrome and could ultimately lead to new strategies to the detection and treatment of PPB and related tumors such as rhabdomyosarcoma and Wilms tumor.
  19. Elucidating the role of NF-kB signaling in rhabdomyocarcoma
    Charles Keller; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Rhabdomyosarcoma is the most common soft-tissue sarcoma occurring in children and young adults. These tumors are unique among sarcomas in that they are thought to derive from skeletal muscle tissue...
  20. Identification of self-renewal programs in muscle stem cells
    DAVID MICHAEL LANGENAU; Fiscal Year: 2012
    ..to identify novel pathways that regulate stem cell self- renewal in both normal muscle and embryonal rhabdomyosarcoma (ERMS), a pediatric malignancy of muscle...
  21. Laser microdissection for molecular analysis of oral, dental and craniofacial dis
    Xiaofeng Zhou; Fiscal Year: 2010
    ..function in periodontal development and the periodontal ligament, 6) epithelial-connective tissue interactions and scar formation, 7) molecular mechanisms of serpins in oral cancer tumorgenesis, 8) molecular analysis of rhabdomyosarcoma.
  22. REGULATION OF CNS AND LIMB POLARITY BY SONIC HEDGEHOG
    Andrew P McMahon; Fiscal Year: 2011
    ..BCC) (the most common form of skin cancer), medulloblastoma (the most common brain tumor of children) and rhabdomyosarcoma (the most prevalent soft tissue cancer in children)...
  23. RGS12 as a MAPK Scaffold in Muscle Development and Disease
    EMILY ANNE OESTREICH; Fiscal Year: 2012
    ..We also hope to highlight the RGS12 scaffold as a point of therapeutic intervention in metastatic rhabdomyosarcoma, a satellite cell-derived cancer where H-Ras mutations and increased ERK activity have been described.
  24. Studies of Childhood Sarcomas
    Peter J Houghton; Fiscal Year: 2013
    ..survival rates are 30 percent or less in children with advanced or metastatic Ewing sarcoma, osteosarcoma or rhabdomyosarcoma and intensive chemo-radiotherapy has not significantly altered this outcome...
  25. Suv39H Targeted Therapy for Treatment of Childhood Rhabdomyosarcoma
    Asoke K Mal; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Rhabdomyosarcoma (RMS) is a childhood malignant tumor and is thought to arise due to the arrest of skeletal muscle differentiation (myogenesis) program. It accounts for 3...
  26. Molecular Modeling of Pediatric Skeletal Muscle Tumors
    Corinne Mary Linardic; Fiscal Year: 2013
    ABSTRACT Rhabdomyosarcoma (RMS) is a heterogeneous collection of cancers demonstrating varying degrees of skeletal muscle differentiation...
  27. Chemical Inhibitors of Rhabdomyosarcoma Self-Renawal
    DAVID MICHAEL LANGENAU; Fiscal Year: 2012
    ..Capitalizing on a unique zebrafish model of rhabdomyosarcoma, we have shown previously that zebrafish tumors are molecularly similar to human embryonal rhabdomyosaroma (..
  28. Genetics of Rhabdomyosarcoma
    DAVID MICHAEL LANGENAU; Fiscal Year: 2013
    ..The focus of this application is to uncover the mechanisms involved in cancer cell self-renewal in embryonal rhabdomyosarcoma (ERMS) - a devastating pediatric cancer of muscle...
  29. Novel Hedgehog Receptors As Therapeutic Targets In Pancreatic Cancer
    Marina Pasca di Magliano; Fiscal Year: 2013
    ..diseases and plays a pivotal role in the pathogenesis of several cancers, including basal cell carcinoma, rhabdomyosarcoma, and medulloblastoma...
  30. Ovarian Follicular Developmental and Hormone Action
    Joanne Richards; Fiscal Year: 2007
    ..the phosphorylation of PKB and the down-stream transcription factor Foxo1 or FKHR (forkhead homolog of rhabdomyosarcoma=forkhead)...
  31. FUSOGENIC MEMBRANE PROTEINS AS THERAPEUTIC TRANSGENES
    Evanthia Galanis; Fiscal Year: 2004
    ..tumor cell lines including A431 (epithelial carcinoma), C170 (colon cancer), HeLa (cervical cancer), TE671 (rhabdomyosarcoma), and the glioma cell lines U87 and U118...
  32. CELL CYCLE-DEPENDENT REGULATION OF MYOGENESIS
    STEPHEN SKAPEK; Fiscal Year: 1999
    ..e. in proliferating myoblasts, oncogenically transformed myoblasts, and rhabdomyosarcoma cells)...
  33. TREATMENT OF CHILDHOOD CANCER
    Martin Brecher; Fiscal Year: 2002
    ..studies for the treatment of advanced Hodgkin's disease, advanced small non- cleaved cell lymphoma, non-rhabdomyosarcoma soft tissue sarcomas, acute lymphoblastic leukemia in relapse, the National Wilms Tumor Study, brain tumors ..
  34. NOVEL TUMOR ANTIGEN FOR ANTIBODY TARGETING
    Nai Kong Cheung; Fiscal Year: 2001
    ..These tumors include rhabdomyosarcoma (RMS), osteosarcoma (OS), Ewing's/PNET (ES), desmoplastic small round cell tumor (DSRT), brain tumors and ..
  35. REGULATED TOXIN GENE THERAPY FOR RHABDOMYOSARCOMA
    TIMOTHY CRIPE; Fiscal Year: 2001
    ..This proposal seeks to devise a novel treatment strategy using alveolar rhabdomyosarcoma as a model system by exploiting genetic rearrangement which underly many cancers...
  36. ISOLATION OF TUMOR SUPPRESSOR GENES IN RHABDOMYOSARCOMA
    Mary Sugrue; Fiscal Year: 2000
    ..describes the application of a functional expression cloning system for the novel purpose of examining rhabdomyosarcoma for the presence of tumor suppressor gene(s)...
  37. MODULATION OF NUCLEOSIDE TRANSPORT IN CHEMOTHERAPY
    Judith Belt; Fiscal Year: 2000
    ..in vivo studies of the modulation of thymidylate synthase inhibitors by nucleoside transport inhibitors in rhabdomyosarcoma (Aims 1 and 2); and in vitro studies of the modulation of purine nucleoside analog (specifically, 2-..
  38. INTERGROUP RHABDOMYOSARCOMA STUDY
    Gregory Reaman; Fiscal Year: 2002
    The Intergroup Rhabdomyosarcoma Study (IRS) is designed to answer important therapeutic, clinical, and laboratory research questions about rhabdomyosarcoma...
  39. MOLECULAR ANALYSIS OF PEDIATRIC CANCERS
    Giovanni Rovera; Fiscal Year: 1993
    ..that will be studied include acute lymphoblastic leukemia, Wilms tumor, neuroblastoma, medulloblastoma, rhabdomyosarcoma and osteogenic sarcoma...
  40. CHILDREN'S CANCER STUDY GROUP
    WILLIAM NEWTON; Fiscal Year: 1980
    ..On an intergroup basis, there are active protocols for Rhabdomyosarcoma, Ewing's both non-metastatic and metastatic, Hodgkin's Disease and Wilms' tumor...
  41. SOUTHWEST ONCOLOGY GROUP (PEDIATRICS)
    JAN VAN EYS; Fiscal Year: 1980
    ..Several intergroup and national studies, ongoing and projected (National Wilms' Tumor Study, Intergroup Rhabdomyosarcoma Study, Intergroup Ewing's Sarcoma Study, Hepatoma Study, Hodgkin's Disease Study) are included...
  42. CHILDRENS CANCER GROUP
    Frederick Ruymann; Fiscal Year: 2002
    ..Dr. Frederick B. Ruymann has been the Principal Investigator (PI) and CCG representative to the Intergroup Rhabdomyosarcoma Study (IRS) since November 1982...
  43. Decidual Prolactin in Normal and Pathogenic Pregnancies
    Stuart Handwerger; Fiscal Year: 2007
    ..to be regulated during the decidualization process, including the transcription factors Ets-1, fork head in rhabdomyosarcoma (FKHR), Twist, and Id3...
  44. Molecular Targeting of Developmental Cancers in Children
    RICHARD O REILLY; Fiscal Year: 2009
    ..proposes to evaluate the function of the unique fusion genes that characterize Ewing's sarcoma, alveolar rhabdomyosarcoma and desmoplaslic small round cell tumor (DSRCT), to identify their targets and to characterize the ..
  45. BASIS OF PAX3-FKHR ONCOGENESIS IN RHABDOMYOSARCOMA
    Chiayeng Wang; Fiscal Year: 2001
    ..The long range goal of the proposed research is to understand the role of PAX3-FKHR in alveolar rhabdomyosarcoma (ARMS), a tumor of fetal skeletal origin that is most common in children...
  46. Conditional Mouse Model of Alveolar Rhabdomyosarcoma
    Charles Keller; Fiscal Year: 2006
    Alveolar rhabdomyosarcoma is an aggressive childhood muscle cancer associated with significant morbidity and mortality...
  47. THE ONCOGENICITY OF WINGED HELIX PROTEINS
    Peter Vogt; Fiscal Year: 2003
    ..in these proteins act in a dominant fashion to cause cancer in animals (avian sarcomas) and humans (alveolar rhabdomyosarcoma and acute lymphoblastic leukemia)...
  48. MODULATION OF RHABDOMYOSARCOMA PHENOTYPE
    PHILIP POTTER; Fiscal Year: 1999
    Alveolar rhabdomyosarcoma, an aggressive childhood tumor derived from skeletal muscle, exhibits a characteristic 2;13 chromosomal translocation that results in the aberrant expression of a novel fusion protein...
  49. IRSG LABORATORY CORRELATIVE STUDIES
    FREDERIC BARR; Fiscal Year: 1999
    Histopathologic classification of pediatric rhabdomyosarcoma (RMS) has established subtypes that are associated with distinct clinical characteristics...
  50. MOLECULAR MECHANISM OF MDM2 INHIBITION OF MYOGENESIS
    Mark Vossler; Fiscal Year: 2001
    ..b>Rhabdomyosarcoma is a common childhood malignancy of skeletal muscle that expresses MyoD yet fails to terminally differentiate...
  51. Role of Serine/threonine Phosphatases in Apoptosis
    Elizabeth Yang; Fiscal Year: 2006
    ..regulates apoptosis signaling by the forkhead transcription factors FKHR and FKHRL1, which are involved in rhabdomyosarcoma and leukemia translocations...
  52. AR-PTEN Antagonism in Prostate Cell Growth And Apoptosis
    Wenlong Bai; Fiscal Year: 2005
    ..Our further investigation showed that the AR and FKHR (fork head in rhabdomyosarcoma), a mammalian homologue of the C...
  53. Role of GLI in Tumor progression
    JOHN RUPPERT; Fiscal Year: 2006
    ..PTCH, a gatekeeper molecule important in basal cell carcinoma of the skin (BCC), medulloblastoma, and rhabdomyosarcoma. Loss-of-function and gain-of-function studies support a role for the GLI family of zinc finger transcription ..
  54. Diagnostic and Prognostic Sarcoma Signatures
    Timothy Triche; Fiscal Year: 2009
    ..g., a molecular as opposed to morphologic definition of alveolar rhabdomyosarcoma and other sarcomas...
  55. Childhood Cancer Gene Program Project Grant
    James Downing; Fiscal Year: 2005
    ..cell biological and mouse experiments to determine how Pax3-FKHR acts as an activated oncogene in alveolar rhabdomyosarcoma, and to identify the secondary genetic alterations that are required to cooperate with Pax3-FKHR to induce a ..
  56. The CXCR4-SDF-1 Axis in Metastatic Rhabdomyosarcoma
    Mariusz Ratajczak; Fiscal Year: 2009
    b>Rhabdomyosarcoma (RMS) is the most common sarcoma in children and very often infiltrates the bone marrow (BM)...
  57. SECOND MALIGNANCY IN RETINOBLASTOMA--ROLE FOR IMPRINTING
    Catherine Driscoll; Fiscal Year: 1990
    ..The recent observation tumor, and rhabdomyosarcoma, however, suggests that a phenomenon called genomic imprinting, may be involved in preferential inactivation ..
  58. Evaluation of Hedgehog Signaling in Myogenic Sarcomas
    John Lee; Fiscal Year: 2005
    ..One of these tumor types is rhabdomyosarcoma, an aggressive soft-tissue sarcoma histologically related to skeletal muscle...
  59. TREATMENT OF CHILDHOOD CANCER
    Arnold Freeman; Fiscal Year: 1980
    ..to enter patients on the National Wilms' Tumor Study, the Intergroup Ewing's Sarcoma Study, the Intergroup Rhabdomyosarcoma Study and the Intergroup Hodgkin's Disease Study...
  60. PEDIATRIC ONCOLOGY GROUP MEMBERSHIP
    Cindy Schwartz; Fiscal Year: 2002
    ..faculty are leaders of the POG commitments in ALL phenotyping, Neuropathology, Bone Tumors, Hodgkins disease, Rhabdomyosarcoma, Radiation Oncology, Bone Marrow Transplantation, Myeloid disease, Germ Cell Tumors, Late Effects of ..
  61. INTERGROUP RHABDOMYOSARCOMA STUDY STATISTICAL CENTER
    James Anderson; Fiscal Year: 2002
    This grant requests funding for a Statistical Center for the Intergroup Rhabdomyosarcoma Study Group (IRSG), a pediatric cooperative clinical trials group conducting ongoing research in the treatment of pediatric rhabdomyosarcoma...
  62. INTERGROUP RHABDOMYOSARCOMA STUDY
    Harold Maurer; Fiscal Year: 1980
    ..and for the conduct of special histo-pathology studies on tissue obtained from patients entered on Intergroup Rhabdomyosarcoma Study (IRS) I and II...
  63. Nuclear Targets of Akt Oncoproteins
    Masahiro Aoki; Fiscal Year: 2003
    ..First, the mechanism of Akt mediated regulation of FKHR (forkhead in rhabdomyosarcoma) will be examined...