alveolar rhabdomyosarcoma

Summary

Summary: A form of RHABDOMYOSARCOMA occurring mainly in adolescents and young adults, affecting muscles of the extremities, trunk, orbital region, etc. It is extremely malignant, metastasizing widely at an early stage. Few cures have been achieved and the prognosis is poor. "Alveolar" refers to its microscopic appearance simulating the cells of the respiratory alveolus. (Holland et al., Cancer Medicine, 3d ed, p2188)

Top Publications

  1. ncbi Fusion of a fork head domain gene to PAX3 in the solid tumour alveolar rhabdomyosarcoma
    N Galili
    Wistar Institute, Philadelphia, Pennsylvania 19104
    Nat Genet 5:230-5. 1993
  2. ncbi PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the children's oncology group
    Poul H B Sorensen
    Department of Pathology, Children s and Women s Hospital of British Columbia, Vancouver, British Columbia, Canada
    J Clin Oncol 20:2672-9. 2002
  3. ncbi Alveolar rhabdomyosarcoma: is the cell of origin a mesenchymal stem cell?
    Elizabeth Charytonowicz
    School of Exercise Biomedical and Health Science, Edith Cowan University, 100 Joondalup Drive, Joondalup, WA 6027, Australia
    Cancer Lett 279:126-36. 2009
  4. ncbi Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma
    F G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 36th Street and Hamilton Walk, Philadelphia, Pennsylvania, PA 19104 6082, USA
    Oncogene 20:5736-46. 2001
  5. pmc Credentialing a preclinical mouse model of alveolar rhabdomyosarcoma
    Koichi Nishijo
    Greehey Children s Cancer Research Institute, University of Texas Health Science Center, San Antonio, Texas, USA
    Cancer Res 69:2902-11. 2009
  6. pmc PDGFR-A is a therapeutic target in alveolar rhabdomyosarcoma
    E Taniguchi
    Greehey Children s Cancer Research Institute, Department of Cellular and Structural Biology, University of Texas Health Science Center, San Antonio, TX 78229 3900, USA
    Oncogene 27:6550-60. 2008
  7. pmc The hybrid PAX3-FKHR fusion protein of alveolar rhabdomyosarcoma transforms fibroblasts in culture
    S Scheidler
    Scripps Research Institute, La Jolla, CA 92037, USA
    Proc Natl Acad Sci U S A 93:9805-9. 1996
  8. ncbi Effects of PAX3-FKHR on malignant phenotypes in alveolar rhabdomyosarcoma
    Ken Kikuchi
    Department of Pediatrics, Kyoto Prefectural University of Medicine, 465 Kajii cho, Kawaramchi Hirokoji, Kamigyo ku, Kyoto 602 8566, Japan
    Biochem Biophys Res Commun 365:568-74. 2008
  9. pmc Genomic and clinical analyses of 2p24 and 12q13-q14 amplification in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Genes Chromosomes Cancer 48:661-72. 2009
  10. ncbi New genetic tactics to model alveolar rhabdomyosarcoma in the mouse
    Charles Keller
    Department of Cellular and Structural Biology, Children s Cancer Research Institute, The University of Texas Health Science Center, San Antonio, Texas, USA
    Cancer Res 65:7530-2. 2005

Detail Information

Publications201 found, 100 shown here

  1. ncbi Fusion of a fork head domain gene to PAX3 in the solid tumour alveolar rhabdomyosarcoma
    N Galili
    Wistar Institute, Philadelphia, Pennsylvania 19104
    Nat Genet 5:230-5. 1993
    ..and expression of the products associated with the t(2;13)(q35;q14) translocation associated with alveolar rhabdomyosarcoma. The chromosome 13 gene (FKHR) is identified as a member of the fork head domain family of transcription ..
  2. ncbi PAX3-FKHR and PAX7-FKHR gene fusions are prognostic indicators in alveolar rhabdomyosarcoma: a report from the children's oncology group
    Poul H B Sorensen
    Department of Pathology, Children s and Women s Hospital of British Columbia, Vancouver, British Columbia, Canada
    J Clin Oncol 20:2672-9. 2002
    b>Alveolar rhabdomyosarcoma (ARMS) is an aggressive soft tissue malignancy of children and adolescents. Most ARMS patients express PAX3-FKHR or PAX7-FKHR gene fusions resulting from t(2;13) or t(1;13) translocations, respectively...
  3. ncbi Alveolar rhabdomyosarcoma: is the cell of origin a mesenchymal stem cell?
    Elizabeth Charytonowicz
    School of Exercise Biomedical and Health Science, Edith Cowan University, 100 Joondalup Drive, Joondalup, WA 6027, Australia
    Cancer Lett 279:126-36. 2009
    b>Alveolar rhabdomyosarcoma (ARMS) is a pediatric sarcoma that typically occurs in older children predominantly arising in the trunk and extremities, and exhibits a worse prognosis than other types of rhabdomyosarcomas...
  4. ncbi Gene fusions involving PAX and FOX family members in alveolar rhabdomyosarcoma
    F G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 36th Street and Hamilton Walk, Philadelphia, Pennsylvania, PA 19104 6082, USA
    Oncogene 20:5736-46. 2001
    The chromosomal translocations t(2;13)(q35;q14) and t(1;13)(p36;q14) are characteristic of alveolar rhabdomyosarcoma, a pediatric soft tissue cancer related to the striated muscle lineage...
  5. pmc Credentialing a preclinical mouse model of alveolar rhabdomyosarcoma
    Koichi Nishijo
    Greehey Children s Cancer Research Institute, University of Texas Health Science Center, San Antonio, Texas, USA
    Cancer Res 69:2902-11. 2009
    The highly aggressive muscle cancer alveolar rhabdomyosarcoma (ARMS) is one of the most common soft tissue sarcoma of childhood, yet the outcome for the unresectable and metastatic disease is dismal and unchanged for nearly three decades...
  6. pmc PDGFR-A is a therapeutic target in alveolar rhabdomyosarcoma
    E Taniguchi
    Greehey Children s Cancer Research Institute, Department of Cellular and Structural Biology, University of Texas Health Science Center, San Antonio, TX 78229 3900, USA
    Oncogene 27:6550-60. 2008
    b>Alveolar rhabdomyosarcoma is an aggressive skeletal muscle cancer of childhood...
  7. pmc The hybrid PAX3-FKHR fusion protein of alveolar rhabdomyosarcoma transforms fibroblasts in culture
    S Scheidler
    Scripps Research Institute, La Jolla, CA 92037, USA
    Proc Natl Acad Sci U S A 93:9805-9. 1996
    Pediatric alveolar rhabdomyosarcoma is characterized by a chromosomal translocation that fuses parts of the PAX3 and FKHR genes...
  8. ncbi Effects of PAX3-FKHR on malignant phenotypes in alveolar rhabdomyosarcoma
    Ken Kikuchi
    Department of Pediatrics, Kyoto Prefectural University of Medicine, 465 Kajii cho, Kawaramchi Hirokoji, Kamigyo ku, Kyoto 602 8566, Japan
    Biochem Biophys Res Commun 365:568-74. 2008
    The malignancy of alveolar rhabdomyosarcoma (ARMS) has been linked to expression of the PAX3-FKHR chimeric gene...
  9. pmc Genomic and clinical analyses of 2p24 and 12q13-q14 amplification in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Genes Chromosomes Cancer 48:661-72. 2009
    b>Alveolar rhabdomyosarcoma (ARMS) is an aggressive pediatric cancer that is related to the skeletal muscle lineage and characterized by recurrent chromosomal translocations...
  10. ncbi New genetic tactics to model alveolar rhabdomyosarcoma in the mouse
    Charles Keller
    Department of Cellular and Structural Biology, Children s Cancer Research Institute, The University of Texas Health Science Center, San Antonio, Texas, USA
    Cancer Res 65:7530-2. 2005
    ..conditional knock-in and knock-out techniques, we designed a mouse model of the childhood muscle cancer alveolar rhabdomyosarcoma (ARMS) that is driven by the chromosomal translocation product, Pax3:Fkhr...
  11. ncbi Genomic gains and losses are similar in genetic and histologic subsets of rhabdomyosarcoma, whereas amplification predominates in embryonal with anaplasia and alveolar subtypes
    Julia A Bridge
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198, USA
    Genes Chromosomes Cancer 33:310-21. 2002
    ....
  12. pmc Pax3:Fkhr interferes with embryonic Pax3 and Pax7 function: implications for alveolar rhabdomyosarcoma cell of origin
    Charles Keller
    Division of Pediatric Hematology Oncology, Department of Pediatrics, University of Utah, Salt Lake City, Utah 84112, USA
    Genes Dev 18:2608-13. 2004
    ....
  13. pmc PAX3-FKHR sensitizes human alveolar rhabdomyosarcoma cells to camptothecin-mediated growth inhibition and apoptosis
    Fu Yue Zeng
    Department of Chemical Biology and Therapeutics, St Jude Children s Research Hospital, Memphis, TN 38105, USA
    Cancer Lett 284:157-64. 2009
    Patients with alveolar rhabdomyosarcoma (ARMS) have poorer response to conventional chemotherapy and lower survival rates than those with embryonal RMS (ERMS)...
  14. ncbi Neonatal alveolar rhabdomyosarcoma with skin and brain metastases
    C Rodriguez-Galindo
    Department of Hematology Oncology, St Jude Children s Research Hospital, Memphis, Tennessee 38105 2794, USA
    Cancer 92:1613-20. 2001
    ..However, because RMS rarely occurs during the neonatal period, little is known about neonatal RMS...
  15. ncbi TaqMan junction probes and the reverse transcriptase polymerase chain reaction: detection of alveolar rhabdomyosarcoma, synovial sarcoma, and desmoplastic small round cell tumor
    Thomas J Cummings
    Department of Pathology, Duke University Medical Center, Durham, North Carolina 27710, USA
    Ann Clin Lab Sci 32:219-24. 2002
    ..TaqMan probes, which hybridize to targets that overlap the junction of the chimeric gene fusions in alveolar rhabdomyosarcoma (ARMS), synovial sarcoma (SS), and desmoplastic small round cell tumor (DSRCT)...
  16. ncbi Primary cutaneous epidermotropic alveolar rhabdomyosarcoma with t(2;13) in an elderly woman: case report and review of the literature
    J Setterfield
    Department of Dermatopathology, St John s Institute of Dermatology, St Thomas Hospital, London, UK
    Am J Surg Pathol 26:938-44. 2002
    We report a case of a primary cutaneous alveolar rhabdomyosarcoma presenting on the lower limb of a 60-year old woman...
  17. ncbi Identification of target genes regulated by PAX3 and PAX3-FKHR in embryogenesis and alveolar rhabdomyosarcoma
    Thomas D Barber
    National Institute on Deafness and Other Communication Disorders, National Institutes of Health, 5 Research Court, Rockville, Maryland 20850, USA
    Genomics 79:278-84. 2002
    ....
  18. ncbi Gene expression profiling identifies potential relevant genes in alveolar rhabdomyosarcoma pathogenesis and discriminates PAX3-FKHR positive and negative tumors
    Cristiano De Pittà
    CRIBI Biotechnology Centre and Dipartimento di Biologia, Universita degli Studi di Padova, Padova, Italy
    Int J Cancer 118:2772-81. 2006
    We analyzed the expression signatures of 14 tumor biopsies from children affected by alveolar rhabdomyosarcoma (ARMS) to identify genes correlating to biological features of this tumor...
  19. pmc Examination of gene fusion status in archival samples of alveolar rhabdomyosarcoma entered on the Intergroup Rhabdomyosarcoma Study-III trial: a report from the Children's Oncology Group
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104 6100, USA
    J Mol Diagn 8:202-8. 2006
    b>Alveolar rhabdomyosarcoma (ARMS) is a soft tissue cancer in which chromosomal translocations generate PAX3-FKHR and PAX7-FKHR gene fusions...
  20. ncbi Fusion of PAX3 to a member of the forkhead family of transcription factors in human alveolar rhabdomyosarcoma
    D N Shapiro
    Department of Experimental Oncology, St Jude Children s Research Hospital, Memphis, Tennessee 38105
    Cancer Res 53:5108-12. 1993
    b>Alveolar rhabdomyosarcoma, a malignant tumor of skeletal muscle, is characterized by a chromosomal translocation, t(2;13)(q35;q14)...
  21. ncbi The alveolar rhabdomyosarcoma PAX3/FKHR fusion protein is a transcriptional activator
    J E Sublett
    Department of Experimental Oncology, St Jude Children s Research Hospital, Memphis, Tennessee 38101, USA
    Oncogene 11:545-52. 1995
    ..The PAX3/FKHR fusion gene, formed by a t(2;13)(q35;q14) in alveolar rhabdomyosarcoma, encodes a hybrid protein that contains both PAX3 DNA binding domains, the paired box and homeodomain, ..
  22. ncbi In vivo amplification of the PAX3-FKHR and PAX7-FKHR fusion genes in alveolar rhabdomyosarcoma
    F G Barr
    Department of Pathology, University of Pennsylvania School of Medicine, Philadelphia 19104, USA
    Hum Mol Genet 5:15-21. 1996
    In the pediatric cancer alveolar rhabdomyosarcoma, characteristic t(2;13)(q35;q14) or variant t(1;13)(p36;q14) chromosomal translocations generate PAX3-FKHR or PAX7-FKHR fusion genes...
  23. ncbi Correlation between histology and PAX/FKHR fusion status in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group
    David M Parham
    Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
    Am J Surg Pathol 31:895-901. 2007
    ..No features seemed to predict the presence of a particular fusion type. Our results suggest that histologic assessment of ARMS has limited correlation with PAX/FKHR fusion status...
  24. pmc Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975-2005
    Simona Ognjanovic
    Division of Pediatric Epidemiology and Clinical Research, Department of Pediatrics, University of Minnesota, Masonic Cancer Center, Minneapolis, Minnesota 55455, USA
    Cancer 115:4218-26. 2009
    ..It is believed that embryonal (ERMS) and alveolar rhabdomyosarcoma (ARMS), the most common subtypes, arise through distinct biologic mechanisms...
  25. ncbi Nascent pre-rRNA overexpression correlates with an adverse prognosis in alveolar rhabdomyosarcoma
    Daniel Williamson
    Molecular Cytogenetics, The Institute of Cancer Research, Sutton, Surrey SM2 5NG, UK
    Genes Chromosomes Cancer 45:839-45. 2006
    ..We conclude that measuring expression of pre-rRNA by real-time PCR is a useful prognostic marker in alveolar rhabdomyosarcoma. Furthermore, given that we have observed similar rDNA staining in all cancer types that we have studied ..
  26. ncbi IFN-beta restricts tumor growth and sensitizes alveolar rhabdomyosarcoma to ionizing radiation
    Thomas L Sims
    Department of Surgery, St Jude Children s Research Hospital, Memphis, Tennessee 38105, USA
    Mol Cancer Ther 9:761-71. 2010
    Ionizing radiation is an important component of multimodal therapy for alveolar rhabdomyosarcoma (ARMS). We sought to evaluate the ability of IFN-beta to enhance the activity of ionizing radiation...
  27. pmc Embryonic expression of the tumor-associated PAX3-FKHR fusion protein interferes with the developmental functions of Pax3
    M J Anderson
    Ludwig Institute for Cancer Research, University of California at San Diego, La Jolla, CA 92093 0660, USA
    Proc Natl Acad Sci U S A 98:1589-94. 2001
    ..These data suggest that the tumor-associated PAX3-FKHR fusion protein interferes with normal Pax3 developmental functions as a prelude to transformation...
  28. ncbi Poor outcome of two children with relapsed state stage IV alveolar rhabdomyosarcoma after allogeneic stem cell transplantation
    Regina Doelken
    Department of Pediatric Hematology and Oncology, University of Greifswald, Soldmannstrasse 15, 17487 Greifswald, Germany
    Pediatr Hematol Oncol 22:699-703. 2005
    ..stem cell transplantation (alloSCT) from their HLA-identical sibling after relapse of stage IV alveolar rhabdomyosarcoma. Both patients were transplanted in a non-remission status...
  29. ncbi Proliferative and apoptotic differences between alveolar rhabdomyosarcoma subtypes: a comparative study of tumors containing PAX3-FKHR or PAX7-FKHR gene fusions
    M H Collins
    Department of Pathology, Children s Hospital of Philadelphia, Philadelphia, PA, USA
    Med Pediatr Oncol 37:83-9. 2001
    ....
  30. pmc Treatment results for patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III and IV, 1984-1997: a report from the Children's Oncology Group
    R Beverly Raney
    Division of Pediatrics, The Children s Cancer Hospital, University of Texas M D Anderson Cancer Center, Houston, Texas 77030, USA
    Pediatr Blood Cancer 55:612-6. 2010
    ..survival (EFS), and overall survival (OS) rates in 71 patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma (ALV RMS) and their relation to radiation therapy (RT) on IRSG Protocols III and IV, 1984-1997.
  31. ncbi Fusion gene-negative alveolar rhabdomyosarcoma is clinically and molecularly indistinguishable from embryonal rhabdomyosarcoma
    Daniel Williamson
    INSERM Unité 830, Unité de Génétique Somatique, Institut Curie, Paris, France
    J Clin Oncol 28:2151-8. 2010
    To determine whether the clinical and molecular biologic characteristics of the alveolar rhabdomyosarcoma (ARMS) and embryonal rhabdomyosarcoma (ERMS) subtypes have relevance independent of the presence or absence of the PAX/FOXO1 fusion ..
  32. pmc Defining the cooperative genetic changes that temporally drive alveolar rhabdomyosarcoma
    Sarasija Naini
    Department of Pediatrics, Duke University Medical Center, Durham, North Carolina 27710, USA
    Cancer Res 68:9583-8. 2008
    ..These early steps, coupled with MycN amplification and telomere stabilization, then drive the cells to a fully tumorigenic state...
  33. ncbi Genetic heterogeneity in the alveolar rhabdomyosarcoma subset without typical gene fusions
    Frederic G Barr
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, Pennsylvania 19104, USA
    Cancer Res 62:4704-10. 2002
    Previous studies of the PAX3-FKHR and PAX7-FKHR gene fusions in alveolar rhabdomyosarcoma (ARMS) indicated that the corresponding fusiontranscripts are not detectable in 20% of ARMS cases...
  34. pmc Pax3-FKHR knock-in mice show developmental aberrations but do not develop tumors
    Irina Lagutina
    Department of Genetics Department of Infectious Diseases, St Jude Children s Research Hospital, Memphis, Tennessee 38105, USA
    Mol Cell Biol 22:7204-16. 2002
    b>Alveolar rhabdomyosarcoma is a pediatric disease specified by the recurrent chromosome translocations t(2;13) and t(1;13)...
  35. pmc Glycogen synthase kinase 3 regulates PAX3-FKHR-mediated cell proliferation in human alveolar rhabdomyosarcoma cells
    Fu Yue Zeng
    Department of Chemical Biology and Therapeutics, St Jude Children s Research Hospital, 262 Danny Thomas Place, Memphis, TN 38105, USA
    Biochem Biophys Res Commun 391:1049-55. 2010
    Patients with alveolar rhabdomyosarcoma (ARMS) have poorer response to conventional chemotherapy and lower survival rates than those with embryonal RMS (ERMS)...
  36. ncbi Altered expression and molecular abnormalities of cell-cycle-regulatory proteins in rhabdomyosarcoma
    Yukiko Takahashi
    Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
    Mod Pathol 17:660-9. 2004
    ..Alveolar type had a significantly higher both mitotic rate and E2F-1 labeling indices when compared with the embryonal type. The current study is the first report of the correlation of E2F-1 with alveolar rhabdomyosarcoma.
  37. ncbi Fusions involving PAX and FOX genes in the molecular pathogenesis of alveolar rhabdomyosarcoma: recent advances
    Gabriela E Mercado
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Curr Mol Med 7:47-61. 2007
    ....
  38. ncbi Multimodal genetic diagnosis of solid variant alveolar rhabdomyosarcoma
    Nuno Cerveira
    Department of Genetics, Portuguese Oncology Institute, Porto, Portugal
    Cancer Genet Cytogenet 163:138-43. 2005
    ..Furthermore, our findings and previous studies indicate that there are no apparent genetic differences between solid variant and typical ARMS...
  39. pmc Strong immunostaining for myogenin in rhabdomyosarcoma is significantly associated with tumors of the alveolar subclass
    P Dias
    Department of Cell Biology, Imgenex Corporation, San Diego, California, USA
    Am J Pathol 156:399-408. 2000
    ....
  40. ncbi A novel and consistent amplicon at 13q31 associated with alveolar rhabdomyosarcoma
    A T Gordon
    Molecular Cytogenetics Laboratory, Institute of Cancer Research, Sutton, Surrey, UK
    Genes Chromosomes Cancer 28:220-6. 2000
    ..Here, we substantially extended the number of alveolar rhabdomyosarcoma samples examined by comparative genomic hybridization analysis...
  41. ncbi Solid alveolar rhabdomyosarcoma originating from the urinary bladder in an adult. Diagnostic value of molecular genetics
    I Lambert
    Histopathology 44:508-10. 2004
  42. ncbi Pilot trial of tumor-specific peptide vaccination and continuous infusion interleukin-2 in patients with recurrent Ewing sarcoma and alveolar rhabdomyosarcoma: an inter-institute NIH study
    Ramzi Dagher
    Pediatric Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland 20892, USA
    Med Pediatr Oncol 38:158-64. 2002
    Patients with recurrent Ewing sarcoma and alveolar rhabdomyosarcoma have poor prognoses and limited therapeutic options...
  43. ncbi Identification of a PAX-FKHR gene expression signature that defines molecular classes and determines the prognosis of alveolar rhabdomyosarcomas
    Elai Davicioni
    Department of Pathology and Preventive Medicine, Keck School of Medicine, and Norris Comprehensive Cancer Center, University of Southern California 90027, USA
    Cancer Res 66:6936-46. 2006
    ..Our results show that PAX-FKHR dictate a specific expression signature that helps define the molecular phenotype of PAX-FKHR-positive ARMS tumors and, because it is linked with disease outcome in ARMS patients, determine tumor behavior...
  44. ncbi Alterations of RB1 gene in embryonal and alveolar rhabdomyosarcoma: special reference to utility of pRB immunoreactivity in differential diagnosis of rhabdomyosarcoma subtype
    Kenichi Kohashi
    Department of Anatomic Pathology, Pathological Sciences, Graduate School of Medical Sciences, Kyushu University, Maidashi 3 1 1, Higashi ku, Fukuoka, Japan
    J Cancer Res Clin Oncol 134:1097-103. 2008
    ..Therefore, there might be a correlation between the tumorigenesis of RMS and RB1 alteration...
  45. pmc MI-63: a novel small-molecule inhibitor targets MDM2 and induces apoptosis in embryonal and alveolar rhabdomyosarcoma cells with wild-type p53
    J A Canner
    Department of Pediatrics, Nationwide Children s Hospital, Columbus, OH, USA
    Br J Cancer 101:774-81. 2009
    ....
  46. ncbi Pax3-fkhr and pax7-fkhr fusion genes impact outcome of alveolar rhabdomyosarcoma in children
    Bernarda Kazanowska
    Department of Bone Marrow Transplantation, Pediatric Oncology, and Hematology, University of Medicine, Wroclaw, Poland
    Fetal Pediatr Pathol 26:17-31. 2007
    ..In addition, fusion gene analysis is a helpful tool in differential diagnosis of poorly differentiated soft tissue tumors...
  47. ncbi Hemophagocytic alveolar rhabdomyosarcoma
    Yutaka Saikawa
    Department of Pediatrics, Kanazawa University Graduate School of Medical Science, Kanazawa, Ishikawa, Japan
    J Clin Oncol 24:5783-4. 2006
  48. 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...
  49. ncbi Manifestation of alveolar rhabdomyosarcoma as primary cutaneous lesions in a neonate with Beckwith-Wiedemann syndrome
    Minoru Kuroiwa
    Department of Surgery, Gunma Children s Medical Center, Shibukawa, Gunma 377 8577, Japan
    J Pediatr Surg 44:e31-5. 2009
    We report a rare case of neonatal Beckwith-Wiedemann syndrome (BWS) associated with alveolar rhabdomyosarcoma (RMS). Alveolar RMS was diagnosed on the basis of excisional biopsy...
  50. pmc Detection of the PAX3-FKHR fusion gene in paediatric rhabdomyosarcoma: a reproducible predictor of outcome?
    J Anderson
    Section of Paediatric Oncology, Institute of Cancer Research, Sutton, Surrey, UK
    Br J Cancer 85:831-5. 2001
    ..With the difficulties in morphological diagnosis of alveolar rhabdomyosarcoma on increasingly used small needle biopsy specimens, these data suggest that molecular analysis for PAX3-..
  51. ncbi Expression and activity of vascular endothelial growth factor and metalloproteinases in alveolar and embryonal rhabdomyosarcoma cell lines
    Maurizio Onisto
    Department of Experimental Biomedical Sciences, University of Padova, I 35121 Padova, Italy
    Int J Oncol 27:791-8. 2005
    ..RMS) is a malignant tumour of skeletal muscle origin which includes two major histological subtypes: alveolar rhabdomyosarcoma (ARMS), the more aggressive, and embryonal rhabdomyosarcoma (ERMS)...
  52. ncbi Assessing the use of FDG-PET in the detection of regional and metastatic nodes in alveolar rhabdomyosarcoma of extremities
    Myriam Weyl Ben Arush
    Department of Pediatric Hematology Oncology, Meyer Children s Hospital, Haifa, Israel
    J Pediatr Hematol Oncol 28:440-5. 2006
    b>Alveolar rhabdomyosarcoma (ARS) accounts for 20% to 30% of childhood rhabdomyosarcoma and is known to have a worse prognosis than embryonal rhabdomyosarcoma...
  53. pmc The PAX3-FKHR fusion protein created by the t(2;13) translocation in alveolar rhabdomyosarcomas is a more potent transcriptional activator than PAX3
    W J Fredericks
    Wistar Institute, University of Pennsylvania School of Medicine, Philadelphia 19104
    Mol Cell Biol 15:1522-35. 1995
    ..The PAX3-FKHR protein may function as an oncogenic transcription factor by enhanced activation of normal PAX3 target genes...
  54. ncbi Unusual leptomeningeal dissemination in a child with extracranial metastatic alveolar rhabdomyosarcoma
    Myriam Weyl Ben Arush
    Department of Pediatric Hematology Oncology, Meyer Children s Hospital, Rambam Health Care Campus, Haifa, Israel m
    Pediatr Hematol Oncol 26:473-8. 2009
    The authors describe a 6-year-old boy diagnosed with alveolar rhabdomyosarcoma located in the thigh, with distal metastases to lungs, bones, and bone marrow...
  55. ncbi Pax3 inhibits myogenic differentiation of cultured myoblast cells
    J A Epstein
    Division of Cardiology, Brigham and Women s Hospital, Boston, Massachusetts, USA
    J Biol Chem 270:11719-22. 1995
    ..These results suggest that Pax3 may suppress the terminal differentiation of migrating limb myoblasts and that the PAX3-forkhead fusion may contribute to the phenotype of alveolar rhabdomyosarcoma by preventing terminal differentiation.
  56. ncbi Alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation: report of 3 cases
    M Adib Houreih
    Department of Histopathology, Christie Hospital NHS Foundation Trust, Manchester, United Kingdom
    Int J Surg Pathol 17:135-41. 2009
    The aim of this study is to report the clinicopathologic characteristics of 3 cases of alveolar rhabdomyosarcoma with neuroendocrine/neuronal differentiation...
  57. ncbi [Alveolar rhabdomyosarcoma of unknown origin mimicking acute leukemia at the initial presentation]
    Kan Yamaguchi
    Department of Pediatrics, Kyushu University Hospital, Kyushu University
    Rinsho Ketsueki 48:315-20. 2007
    ..polymerase chain reaction demonstrated PAX3/FKHR fusion transcripts, confirming the diagnosis of alveolar rhabdomyosarcoma. Radiological examination revealed only one enlarged lymph node being 1...
  58. pmc Detection of chromosomal regions showing differential gene expression in human skeletal muscle and in alveolar rhabdomyosarcoma
    Andrea Bisognin
    Department of Biology, University of Padua, 35131, Padova, Italy
    BMC Bioinformatics 5:68. 2004
    ....
  59. ncbi Congenital alveolar rhabdomyosarcoma: clinical and molecular distinction from alveolar rhabdomyosarcoma in older children
    R Grundy
    The Institute of Child Health, The University of Birmingham, Whittall Street, Birmingham B4 6NH, United Kingdom
    Cancer 91:606-12. 2001
    Congenital alveolar rhabdomyosarcoma (RMS) is extremely rare and invariably fatal with current therapy...
  60. ncbi Identification and epitope enhancement of a PAX-FKHR fusion protein breakpoint epitope in alveolar rhabdomyosarcoma cells created by a tumorigenic chromosomal translocation inducing CTL capable of lysing human tumors
    Leon T van den Broeke
    Vaccine Branch, Center for Cancer Research, National Cancer Institute NIH, Building 10, Rm 6B 04, 10 Center Drive, Bethesda, MD 20892, USA
    Cancer Res 66:1818-23. 2006
    ..epitope in the PAX-FKHR fusion protein created by the t(2;13) translocation present in 80% of cases of alveolar rhabdomyosarcoma, a highly aggressive pediatric soft-tissue sarcoma...
  61. pmc FOXO1a acts as a selective tumor suppressor in alveolar rhabdomyosarcoma
    Philippe R J Bois
    Department of Biochemistry, St Jude Children s Research Hospital, Memphis, TN 38105, USA
    J Cell Biol 170:903-12. 2005
    ..We conclude that FOXO1a is a potent and specific tumor suppressor in ARMS, suggesting that agents that restore or augment FOXO1a activity may be effective as ARMS therapeutics...
  62. ncbi Use of reverse transcriptase polymerase chain reaction for diagnosis and staging of alveolar rhabdomyosarcoma, Ewing sarcoma family of tumors, and desmoplastic small round cell tumor
    U H Athale
    Department of Hematology Oncology, St Jude Children s Research Hospital, Memphis, Tennessee 38105 2794, USA
    J Pediatr Hematol Oncol 23:99-104. 2001
    ..of morphology-based methods for diagnosis, staging, and detection of metastatic disease in pediatric alveolar rhabdomyosarcoma (ARMS), Ewing sarcoma family of tumors (ESFT), and desmoplastic small round cell tumors (DSRCT)...
  63. ncbi Use of a novel FISH assay on paraffin-embedded tissues as an adjunct to diagnosis of alveolar rhabdomyosarcoma
    Jun Nishio
    Department of Pathology and Microbiology, University of Nebraska Medical Center, 983135 Nebraska Medical Center, Omaha, 68198 3135, USA
    Lab Invest 86:547-56. 2006
    A valuable diagnostic adjunct and important prognostic parameter in alveolar rhabdomyosarcoma (ARMS) is the identification of translocations t(2;13)(q35;q14) and t(1;13)(p36;q14), and the associated PAX3-FKHR and PAX7-FKHR fusion ..
  64. ncbi Molecular cytogenetic analysis consistently identifies translocations involving chromosomes 1, 2 and 15 in five embryonal rhabdomyosarcoma cell lines and a PAX-FOXO1A fusion gene negative alveolar rhabdomyosarcoma cell line
    I Roberts
    Hutchison MRC Research Centre, Cambridge, UK
    Cytogenet Cell Genet 95:134-42. 2001
    ..Analysis of the translocation breakpoints may suggest mechanisms of ERMS tumourigenesis and may enable the development of novel approaches to the clinical management of this tumour...
  65. ncbi Chromosomal imbalances in pleomorphic rhabdomyosarcomas and identification of the alveolar rhabdomyosarcoma-associated PAX3-FOXO1A fusion gene in one case
    Anthony Gordon
    Section of Molecular Carcinogenesis, Molecular Cytogenetics Laboratory, Institute of Cancer Research, Sutton, UK SM2 5NG, Surrey, UK
    Cancer Genet Cytogenet 140:73-7. 2003
    ..In addition, one of the cases classified as PRMS showed evidence for the presence of a PAX3-FOXO1A fusion gene, which is characteristic of the alveolar subtype of RMS...
  66. ncbi Molecular biology of rhabdomyosarcoma
    S Gallego Melcon
    Servicio de Oncología y Hematología Pediátrica, Hospital Universitari Vall d Hebron, Barcelona, Spain
    Clin Transl Oncol 9:415-9. 2007
    ..In RMS, the advances in the knowledge of the biological characteristics of the tumour are slowly translated into the clinical management of children with this tumour...
  67. ncbi Vascular endothelial growth factor acts in an autocrine manner in rhabdomyosarcoma cell lines and can be inhibited with all-trans-retinoic acid
    Matthew F W Gee
    Division of Haematology Oncology, Department of Paediatrics, Hospital for Sick Children, University of Toronto, Toronto, Ontario, Canada
    Oncogene 24:8025-37. 2005
    ..These data suggest that autocrine VEGF signalling likely influences RMS growth and its inhibition may be an effective treatment for RMS...
  68. ncbi ["Spot-scanning" proton therapy for rhabdomyosarcomas of early childhood. First experiences at PSI]
    Beate Timmermann
    Abteilung Strahlenmedizin, Programm Protonen Therapie, Paul Scherrer Institut, 5232 Villigen PSI, Switzerland
    Strahlenther Onkol 182:653-9. 2006
    ..To evaluate the feasibility and acute toxicity of spot-scanning proton therapy under deep sedation in young children with rhabdomyosarcomas (RMS)...
  69. pmc Novel genes implicated in embryonal, alveolar, and pleomorphic rhabdomyosarcoma: a cytogenetic and molecular analysis of primary tumors
    Myriam Goldstein
    Genetic Institute, Tel Aviv Sourasky Medical Center, Tel Aviv, Israel
    Neoplasia 8:332-43. 2006
    ..Our data suggest the involvement of genes encoding cell adhesion, cytoskeletal signaling, and transcriptional and cell cycle components in RMS tumorigenesis...
  70. ncbi Mirk/Dyrk1b mediates cell survival in rhabdomyosarcomas
    Stephen E Mercer
    Department of Pathology, Upstate Medical University, State University of New York, Syracuse, New York 13210, USA
    Cancer Res 66:5143-50. 2006
    ..This survival function is maintained in rhabdomyosarcoma, where Mirk may be a novel therapeutic target...
  71. ncbi Upstream CpG island methylation of the PAX3 gene in human rhabdomyosarcomas
    Raushan T Kurmasheva
    Department of Biochemistry, University of Arkansas for Medical Sciences, Little Rock, Arkansas 72205, USA
    Pediatr Blood Cancer 44:328-37. 2005
    ..PAX3 is an important gene in muscle development and muscle-producing neoplasms such as rhabdomyosarcomas...
  72. ncbi Different pattern of matrix metalloproteinases expression in alveolar versus embryonal rhabdomyosarcoma
    Francesca Diomedi-Camassei
    Department of Pathology, Children s Hospital Bambino Gesu, Research Institute, Rome, Italy
    J Pediatr Surg 39:1673-9. 2004
    ..Data for rhabdomyosarcoma (RMS), the most frequent soft tissue sarcoma of childhood, are lacking. The aim of this study was to assess their expression in this tumor and to evaluate the correlation with clinicopathologic parameters...
  73. ncbi Effects of standard chemotherapy on tumor growth and regulation of multidrug resistance genes and proteins in childhood rhabdomyosarcoma
    Guido Seitz
    Department of Pediatric Surgery, University Children s Hospital, Hoppe Seyler Strasse 1, 72076 Tuebingen, Germany
    Pediatr Surg Int 23:431-9. 2007
    ..In the cell lines, an up-regulation of MDR-1 gene was found in alveolar rhabdomyosarcoma. In embryonal rhabdomyosarcoma, an up-regulation of LRP and MRP was found...
  74. ncbi Childhood rhabdomyosarcoma in Ibadan, Nigeria: 1984-2003
    B J Brown
    Department of Paediatrics, Faculty of Clinical Sciences, College of Medicine, University of Ibadan University College Hospital, Ibadan, Nigeria
    Ann Trop Paediatr 26:349-55. 2006
    ..Rhabdomyosarcoma is the most common soft tissue sarcoma in children under 15 years of age. There is, however, a paucity of reports on the pattern of its occurrence in Nigeria and other parts of Africa...
  75. pmc Altered expression of natively glycosylated alpha dystroglycan in pediatric solid tumors
    Laura T Martin
    Division of Pediatric Hematology Oncology, Department of Pediatrics, Ohio State University College of Medicine and Public Health, Columbus, OH 43205, USA
    Hum Pathol 38:1657-68. 2007
    ..tissues, we demonstrate a significant reduction in native alpha dystroglycan expression in pediatric alveolar rhabdomyosarcoma (RMS), embryonal RMS, neuroblastoma (NBL), and medulloblastoma, whereas expression of beta dystroglycan, ..
  76. pmc Methylation alterations of the MyoD1 upstream region are predictive of subclassification of human rhabdomyosarcomas
    B Chen
    Department of Pathology, University of Arkansas for Medical Sciences, and Arkansas Children s Hospital, Little Rock 72202, USA
    Am J Pathol 152:1071-9. 1998
    ..Our data suggest that the methylation status of the MyoD1 upstream CpG sites may be related to rhabdomyosarcoma tumorigenesis and may have valuable implications for its differential diagnosis...
  77. ncbi The challenging role of radiation therapy for very young children with rhabdomyosarcoma
    Dev R Puri
    Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York, NY 10021, USA
    Int J Radiat Oncol Biol Phys 65:1177-84. 2006
    ..To evaluate local control and toxicity for very young children treated with multimodality therapy for rhabdomyosarcoma (RMS)...
  78. ncbi Sinonasal rhabdomyosarcoma in children and young adults
    Atif Ali Ahmed
    Division of Anatomic Pathology, Children s National Medical Center, Washington, DC, USA
    Int J Surg Pathol 15:160-5. 2007
    ..A significant number of tumor cells in these cases had clear or vacuolated cytoplasm. Four alveolar rhabdomyosarcoma tumors were tested by RT-PCR; all showed PAX3/FKHR chromosomal translocation...
  79. ncbi Results of a prospective minimal disseminated disease study in human rhabdomyosarcoma using three different molecular markers
    Francesca Sartori
    Clinic for Pediatric Oncology, Azienda Ospedaliera University of Padova, Padova, Italy
    Cancer 106:1766-75. 2006
    ..ARMS is more aggressive and prone to distant tumor dissemination, whereas ERMS tends to expand and recur locally. Little information is available on bone marrow involvement by RMS...
  80. ncbi Plakoglobin is differentially expressed in alveolar and embryonal rhabdomyosarcoma and is regulated by DNA methylation and histone acetylation
    Tamara Gastaldi
    Clinica di Oncoematologia Pediatrica, Azienda Ospedaliera Universita di Padova, Via Giustiniani 1, 35128 Padova, Italy
    Carcinogenesis 27:1758-67. 2006
    ..These results demonstrate that plakoglobin is differentially expressed in ARMS and ERMS and that its expression depends on the methylation and acetylation status of the gene...
  81. ncbi Phosphorylation profiles of protein kinases in alveolar and embryonal rhabdomyosarcoma
    Ling Cen
    Department of Pediatrics, Center for Childhood Cancer, Columbus Children s Research Institute, The Ohio State University, Columbus, OH 43205, USA
    Mod Pathol 20:936-46. 2007
    ..Phosphorylation levels of receptor and non-receptor tyrosine kinases are elevated between 26 and 68% in alveolar rhabdomyosarcoma and between 24 and 71% in embryonal rhabdomyosarcoma, respectively, compared to normal tissues...
  82. ncbi Juvenile rhabdomyosarcomas in Port Harcourt, Nigeria: A twelve year review
    D Seleye-Fubara
    Department of Anatomical Pathology, University of Port Harcourt Teaching Hospital PMB 6173, Port Harcourt, Nigeria
    West Afr J Med 25:57-60. 2006
    ..Juvenile rhabdomyosarcoma (JRMS) though rare, is the most common soft tissue malignancy of childhood that exhibits bimodal age distribution pattern...
  83. ncbi Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcoma
    Ramapriya Ganti
    Department of Pathology and Laboratory Medicine, St Jude Children s Research Hospital, Memphis, TN 38105, USA
    Mod Pathol 19:1213-20. 2006
    ..Tissue microarray sections representing 66 rhabdomyosarcoma tumors (34 embryonal rhabdomyosarcoma, 32 alveolar rhabdomyosarcoma) were surveyed by immunohistochemistry using antibodies specific for EGFR and ErbB-2...
  84. ncbi Rhabdomyosarcoma: molecular diagnostics of patients classified by morphology and immunohistochemistry with emphasis on bone marrow and purged peripheral blood progenitor cells involvement
    L Krskova
    Department of Pathology and Molecular Medicine, 2nd Medical School, Charles University and Faculty Hospital in Motol, Prague, Czech Republic
    Virchows Arch 448:449-58. 2006
    ..We demonstrate that employing molecular diagnostics has an impact on staging, therapy monitoring and recognition of malignant cells at the tumor resection margins...
  85. ncbi Detection of FOXO1 (FKHR) gene break-apart by fluorescence in situ hybridization in formalin-fixed, paraffin-embedded alveolar rhabdomyosarcomas and its clinicopathologic correlation
    Shveta Mehra
    Department of Pathology, SUNY Upstate Medical University, Syracuse, NY, USA
    Diagn Mol Pathol 17:14-20. 2008
    ..As characteristic genetic alterations of ARMS can be identified in 40% of RMS-NOS cases in our study, the FISH assay would provide an additional useful tool in the diagnosis and prognosis of ARMS, and an alternative to RT-PCR...
  86. ncbi Delayed radiotherapy following dose intensive chemotherapy for parameningeal rhabdomyosarcoma (PM-RMS) of childhood
    James G Douglas
    Department of Radiation Oncology, University of Washington Affiliated Hospitals, Seattle, WA, United States
    Eur J Cancer 43:1045-50. 2007
    ..To evaluate the local control rates and survival rates of patients with Group III parameningeal rhabdomyosarcoma (PM-RMS) treated with a dose intensive chemotherapy regimen followed by irradiation...
  87. ncbi Ultrastructural characterization of poorly differentiated rhabdomyosarcomas
    L Lombardi
    Division of Experimental Oncology A, Istituto Nazionale Tumori, Milano, Italy
    Ultrastruct Pathol 17:669-80. 1993
    ..Oval cells may be used as markers of poorly differentiated rhabdomyosarcomas...
  88. ncbi Rhabdomyosarcoma: value of myogenin expression analysis and molecular testing in diagnosing the alveolar subtype: an analysis of 109 paraffin-embedded specimens
    Isabelle Hostein
    Department of Pathology, Institut Bergonie, Bordeaux, France
    Cancer 101:2817-24. 2004
    ..Two specific translocations have been found in ARMS, and fusion transcripts can be detected by reverse transcriptase-polymerase chain reaction (RT-PCR) analysis of paraffin-embedded tissue (PET)...
  89. ncbi Histology-specific expression of a DNA repair protein in pediatric rhabdomyosarcomas
    B Thomson
    Department of Pediatrics, Indiana University School of Medicine, Indianapolis, USA
    J Pediatr Hematol Oncol 23:234-9. 2001
    ..We examined the role of APE/ref1 in pediatric embryonal and alveolar rhabdomyosarcomas (ARMS)...
  90. ncbi p53 and mdm-2 expression in Rhabdomyosarcoma of childhood and adolescence: clinicopathologic study by the Kiel Pediatric Tumor Registry and the German Cooperative Soft Tissue Sarcoma Study
    Ivo Leuschner
    Department of Pathology, Kiel Paediatric Tumor Registry, Institute for Paediatric Pathology, University of Kiel, Michaelisstrasse 11, D 24105 Kiel, Germany
    Pediatr Dev Pathol 6:128-36. 2003
    ..In conclusion, we could not demonstrate that any of the investigated parameters had an influence on prognosis of RMS. p53 protein overexpression might be a crucial step in metastatic disease for patients with embryonal RMS...
  91. ncbi [Diagnosis of micrometastases of alveolar rhabdomyosarcoma]
    O P Blizniukov
    Arkh Patol 70:36-40. 2008
    ..Identification of micrometastases in alveolar rhabdomyosarcoma is important because the poor prognosis associated with this subgroup necessitates a modified ..
  92. ncbi Actin isoform pattern expression: a tool for the diagnosis and biological characterization of human rhabdomyosarcoma
    Sophie Clement
    Department of Pathology, University of Geneva, 1 rue Michel Servet, 1211 Geneva 4, Switzerland
    Virchows Arch 442:31-8. 2003
    ..They also suggest that the expression of alpha-skeletal actin is valuable in determining the subtype and possibly the state of differentiation of these tumors...
  93. ncbi ALK protein expression in rhabdomyosarcomas
    K Pillay
    Department of Pathology, Nelson R Mandela School of Medicine, University of Natal, Durban, South Africa
    Histopathology 41:461-7. 2002
    ..However, rhabdomyosarcomas (in particular, the alveolar subtype) have also been noted to show expression of this protein. This study set out to examine ALK expression in a large number of rhabdomyosarcomas...
  94. ncbi The expression of WT1 in the differentiation of rhabdomyosarcoma from other pediatric small round blue cell tumors
    D F Carpentieri
    Department of Pathology, The Children s Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
    Mod Pathol 15:1080-6. 2002
    ..A role for WT1 in the pathogenesis of rhabdomyosarcomas is raised. The limited sampling precludes any conclusions regarding the value of tissue or peripheral blood analysis for WT1 mRNA in patients with rhabdomyosarcoma...
  95. ncbi Up-front window trial of topotecan in previously untreated children and adolescents with metastatic rhabdomyosarcoma: an intergroup rhabdomyosarcoma study
    A S Pappo
    Intergroup Rhabdomyosarcoma Study Group representing the Children's Cancer Group, Arcadia, CA 91066-6012, USA
    J Clin Oncol 19:213-9. 2001
    ..CONCLUSION: The high response rate and acceptable toxicity profile of topotecan in children with advanced RMS support further evaluation of this agent in phase III trials. The superior responses in alveolar RMS are of interest...
  96. ncbi Value of image cytometry in the subclassification of rhabdomyosarcoma
    Ziva Pohar-Marinsek
    Department of Cytopathology, Institute of Oncology, Ljubljana, Slovenia
    Anal Quant Cytol Histol 24:212-20. 2002
    ..To test the discriminatory capability of nuclear features in the subclassification of rhabdomyosarcoma (RMS) and especially to differentiate embryonal from alveolar RMS...
  97. ncbi Diffuse myogenin expression by immunohistochemistry is an independent marker of poor survival in pediatric rhabdomyosarcoma: a tissue microarray study of 71 primary tumors including correlation with molecular phenotype
    Amy Heerema-McKenney
    Department of Pathology parallelBiochemistry, Stanford University School of Medicine, Stanford, CA 94305, USA
    Am J Surg Pathol 32:1513-22. 2008
    ..034) and OS (P=0.0069). In this retrospective analysis, diffuse immunohistochemical reactivity for myogenin in RMS correlates with decreased RFI and OS, independent of histologic subtype, translocation status, tumor site, or stage...
  98. ncbi Identification of a novel gene NCRMS on chromosome 12q21 with differential expression between rhabdomyosarcoma subtypes
    Agnes S Chan
    Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, Ontario, Canada M5G 1L5
    Oncogene 21:3029-37. 2002
    ..Known genes in its proximity include myogenic regulators Myf5 and Myf6, growth factor Igf1, and another potential differentially expressed gene (ATP2B1) in RMS isolated by RDA...
  99. ncbi Rhabdomyosarcoma of the oral and maxillofacial region in Jordanians: a retrospective analysis
    Taiseer Al-Khateeb
    Jordan University of Science and Technology Irbid, Jordan
    Oral Surg Oral Med Oral Pathol Oral Radiol Endod 93:580-5. 2002
    ..The objective was to study the clinicopathologic features of rhabdomyosarcoma (RMS) of the oral and maxillofacial region in Jordanians...
  100. ncbi DNA microarrays in pediatric cancer
    T J Triche
    Department of Pathology, Children's Hospital Los Angeles, California 90027, USA
    Cancer J 7:2-15. 2001
    ..Specifically, embryonal rhabdomyosarcoma could be distinguished from alveolar rhabdomyosarcoma, and even morphologically mixed embryonal and alveolar rhabdomyosarcoma showed similar gene expression ..
  101. ncbi Cytodifferentiation and clinical outcome after chemotherapy and radiation therapy for rhabdomyosarcoma (RMS)
    Lynn M Smith
    Department of Radiation Oncology, University of Utah Health Sciences Center, Salt Lake City, Utah, USA
    Med Pediatr Oncol 38:398-404. 2002
    ..Second-look surgery after therapy for rhabdomyosarcoma (RMS) may yield prognostic information regarding tumor responsiveness to treatment. Favorable outcome is suggested by tumor cells which have undergone maturation (cytodifferentiation)...

Research Grants27

  1. Pathogenic mechanisms of alveolar rhabdomyosarcoma
    MARGARET MARY CHOU; Fiscal Year: 2013
    ..wide panel of primary human tumors further revealed high expression specifically in a high percentage of alveolar rhabdomyosarcoma (ARMS) cases...
  2. 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 ..
  3. 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 ..
  4. Therapeutic Targets in Alveolar Rhabdomyosarcoma
    Charles Keller; Fiscal Year: 2012
    ..The childhood muscle cancer alveolar rhabdomyosarcoma is a classic example...
  5. 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)...
  6. 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...
  7. Elucidating the role of NF-kB signaling in rhabdomyocarcoma
    Charles Keller; Fiscal Year: 2013
    ..the embryonal form compromises the majority of cases and is more responsive to multi-modal therapy, alveolar rhabdomyosarcoma is a more primitive malignant neoplasm where prognosis for children with advanced disease remains dismal...
  8. Suv39H Targeted Therapy for Treatment of Childhood Rhabdomyosarcoma
    Asoke K Mal; Fiscal Year: 2012
    ..multimodality therapeutic treatment approaches, the outlook for patients with metastatic subtype, alveolar rhabdomyosarcoma (ARMS), remains unchanged...
  9. 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...
  10. MOLECULAR ANALYSIS OF PEDIATRIC CANCERS
    Giovanni Rovera; Fiscal Year: 1993
    ..Project 4 identifies and clones the t(2;13) translocation breakpoint characteristic of alveolar rhabdomyosarcoma because this translocation likely contributes to tumorigenicity...
  11. Molecular Targeting of Developmental Cancers in Children
    RICHARD O REILLY; Fiscal Year: 2009
    ..II 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 ..
  12. Conditional Mouse Model of Alveolar Rhabdomyosarcoma
    Charles Keller; Fiscal Year: 2006
    b>Alveolar rhabdomyosarcoma is an aggressive childhood muscle cancer associated with significant morbidity and mortality...
  13. 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...
  14. 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)...
  15. MODULATION OF RHABDOMYOSARCOMA PHENOTYPE
    PHILIP POTTER; Fiscal Year: 1999
    b>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...
  16. 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 ..
  17. Diagnostic and Prognostic Sarcoma Signatures
    Timothy Triche; Fiscal Year: 2009
    ..g., a molecular as opposed to morphologic definition of alveolar rhabdomyosarcoma and other sarcomas...
  18. REGULATION OF PAX3/PAX7 EXPRESSION IN RHABDOMYOSARCOMA
    FREDERIC BARR; Fiscal Year: 2001
    ..Studies of the pediatric cancer alveolar rhabdomyosarcoma (ARMS) have demonstrated that PAX3 and PAX7 play important roles in neoplastic develpment involving the ..
  19. 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...
  20. COG studies of gene amplification in rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2009
    ..abstract_text> ..
  21. Cancer Molecular Pathology Training Program
    FREDERIC BARR; Fiscal Year: 2006
    ....
  22. Studies of the t(2;13) of alveolar rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2007
    b>Alveolar rhabdomyosarcoma (ARMS) is an aggressive pediatric cancer of the striated muscle lineage that is characterized by 2;13 or 1;13 chromosomal translocations...
  23. Role of MN1-TEL and MN1 in Leukemogenesis
    Gerard C Grosveld; Fiscal Year: 2010
    ..These studies should also provide leads for the development of novel therapies for AML involving these transcription factors. ..
  24. DEK EFFECTS ON GROWTH OF HEMATOPOIETIC CELLS
    Gerard Grosveld; Fiscal Year: 2002
    ..The investigators state that these studies will provide valuable insights into the normal functions of DEK and generate important information on how DEK-CAN contributes to leukemogenesis. ..
  25. DYSTROPHIN REPLACEMENT IN MDX MICE
    Jeffrey Chamberlain; Fiscal Year: 2004
    ..These studies will provide new insights into both the structure of dystrophin and the mechanisms of dystrophic cell death and will help advance the development of gene therapy. ..
  26. Regulation of Differentiation Gene Expression by MyoD
    Asoke K Mal; Fiscal Year: 2010
    ..It is our expectation that completion of these AIMS will lead to novel therapies for muscle-related diseases. ..
  27. Muscular Dystrophy Cooperative Research Center
    Jeffrey Chamberlain; Fiscal Year: 2007
    ..Finally, Core C (T Bird) is a genetic counseling core for patients and families with muscular dystrophy that will also allow increased participation of patients in studies of a variety of types of muscular dystrophy. ..