embryonal rhabdomyosarcoma

Summary

Summary: A form of RHABDOMYOSARCOMA arising primarily in the head and neck, especially the orbit, of children below the age of 10. The cells are smaller than those of other rhabdomyosarcomas and are of two basic cell types: spindle cells and round cells. This cancer is highly sensitive to chemotherapy and has a high cure rate with multi-modality therapy. (From Holland et al., Cancer Medicine, 3d ed, p2188)

Top Publications

  1. 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
  2. pmc Effects of RAS on the genesis of embryonal rhabdomyosarcoma
    David M Langenau
    Stem Cell Program and Division of Hematology, Children s Hospital Boston and Dana Farber Cancer Institute, Boston, Massachusetts 02115, USA
    Genes Dev 21:1382-95. 2007
  3. ncbi Lactoferrin inhibits enterovirus 71 infection of human embryonal rhabdomyosarcoma cells in vitro
    Tzou Yien Lin
    Department of Pediatrics, Chang Gung Children s Hospital, 5 Fu Hsin Street, Kweishan 333, Taoyuan, Taiwan
    J Infect Dis 186:1161-4. 2002
  4. doi 18 F-FDG avid lesion due to coexistent fibrous dysplasia in a child of embryonal rhabdomyosarcoma: source of false positive FDG-PET
    Sandip Basu
    Radiation Medicine Centre BARC, Tata Memorial Hospital Annexe, Jerbai Wadia Road, Parel, Mumbai 400 012, India
    J Cancer Res Ther 6:92-4. 2010
  5. 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
  6. 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
  7. ncbi Adult sclerosing rhabdomyosarcoma: cytogenetic link with embryonal rhabdomyosarcoma
    Romaric Croes
    Department of Morphology and Molecular Pathology, University Hospitals, K U Leuven, Leuven, Belgium
    Virchows Arch 446:64-7. 2005
  8. ncbi Novel genomic imbalances in embryonal rhabdomyosarcoma revealed by comparative genomic hybridization and fluorescence in situ hybridization: an intergroup rhabdomyosarcoma study
    J A Bridge
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198 3135, USA
    Genes Chromosomes Cancer 27:337-44. 2000
  9. doi Fusion of EWSR1 with the DUX4 facioscapulohumeral muscular dystrophy region resulting from t(4;22)(q35;q12) in a case of embryonal rhabdomyosarcoma
    Nicolas Sirvent
    Laboratory of Solid Tumor Genetics, Nice University Hospital, 06107 Nice, France
    Cancer Genet Cytogenet 195:12-8. 2009
  10. ncbi Myogenin and desmin immunohistochemistry in the assessment of post-chemotherapy genitourinary embryonal rhabdomyosarcoma: prognostic and management implications
    P Godbole
    Department of Paediatric Urology, Great Ormond Street Hospital for Children, London, United Kingdom
    J Urol 176:1751-4. 2006

Research Grants

  1. Cancer Biology in the Zebrafish
    LEONARD IRA ZON; Fiscal Year: 2010
  2. MyoD Activity in Rhabdomyosarcomas
    Zhihong Yang; Fiscal Year: 2007
  3. A MURINE MODEL OF SMITH-MAGENIS SYNDROME
    Cornelius Boerkoel; Fiscal Year: 2003
  4. Characterization of SMARCAL1:its expression/interactors
    Cornelius Boerkoel; Fiscal Year: 2003
  5. A Mouse Model for Schimke Immuno-osseous Dysplasia
    Cornelius Boerkoel; Fiscal Year: 2006
  6. COG studies of gene amplification in rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2009
  7. IRSG STUDIES OF ALVEOLAR RHABDOMYOSARCOMA GENE FUSIONS
    FREDERIC BARR; Fiscal Year: 2005
  8. CASE-CONTROL STUDY OF HODGKIN'S DISEASE IN CHILDREN
    Seymour Grufferman; Fiscal Year: 2003
  9. Studies of the t(2;13) of alveolar rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2007
  10. COG studies of gene amplification in rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2007

Detail Information

Publications190 found, 100 shown here

  1. 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)...
  2. pmc Effects of RAS on the genesis of embryonal rhabdomyosarcoma
    David M Langenau
    Stem Cell Program and Division of Hematology, Children s Hospital Boston and Dana Farber Cancer Institute, Boston, Massachusetts 02115, USA
    Genes Dev 21:1382-95. 2007
    b>Embryonal rhabdomyosarcoma (ERMS) is a devastating cancer with specific features of muscle differentiation that can result from mutational activation of RAS family members...
  3. ncbi Lactoferrin inhibits enterovirus 71 infection of human embryonal rhabdomyosarcoma cells in vitro
    Tzou Yien Lin
    Department of Pediatrics, Chang Gung Children s Hospital, 5 Fu Hsin Street, Kweishan 333, Taoyuan, Taiwan
    J Infect Dis 186:1161-4. 2002
    ..were assayed in vitro to assess their inhibiting capacity on the cytopathic effect of EV71 on human embryonal rhabdomyosarcoma (RD) cells...
  4. doi 18 F-FDG avid lesion due to coexistent fibrous dysplasia in a child of embryonal rhabdomyosarcoma: source of false positive FDG-PET
    Sandip Basu
    Radiation Medicine Centre BARC, Tata Memorial Hospital Annexe, Jerbai Wadia Road, Parel, Mumbai 400 012, India
    J Cancer Res Ther 6:92-4. 2010
    ..intensely avid 18 F-FDG uptake in fibrous dysplasia of right tibia in a 12-year-old child suffering from embryonal rhabdomyosarcoma (ERMS) that could have been easily mistaken to be a metastatic focus if not carefully correlated...
  5. 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
    ..is the most common pediatric soft-tissue sarcoma, which includes two major subtypes, alveolar and embryonal rhabdomyosarcoma. The mechanism of its oncogenesis is largely unknown...
  6. 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
    ..The authors of this report evaluated incidence and survival trends by RMS demographic subgroups to inform future etiologic hypotheses...
  7. ncbi Adult sclerosing rhabdomyosarcoma: cytogenetic link with embryonal rhabdomyosarcoma
    Romaric Croes
    Department of Morphology and Molecular Pathology, University Hospitals, K U Leuven, Leuven, Belgium
    Virchows Arch 446:64-7. 2005
    ..The chromosomal composition of malignant cells resembled the pattern of numerical changes frequently observed in embryonal rhabdomyosarcoma, suggesting a close relationship of an adult sclerosing rhabdomyosarcoma with this entity.
  8. ncbi Novel genomic imbalances in embryonal rhabdomyosarcoma revealed by comparative genomic hybridization and fluorescence in situ hybridization: an intergroup rhabdomyosarcoma study
    J A Bridge
    Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198 3135, USA
    Genes Chromosomes Cancer 27:337-44. 2000
    ..In summary, CGH and FISH analyses of 12 E-RMS specimens revealed novel genomic imbalances that may be useful in directing further molecular studies for the determination of E-RMS critically involved genes...
  9. doi Fusion of EWSR1 with the DUX4 facioscapulohumeral muscular dystrophy region resulting from t(4;22)(q35;q12) in a case of embryonal rhabdomyosarcoma
    Nicolas Sirvent
    Laboratory of Solid Tumor Genetics, Nice University Hospital, 06107 Nice, France
    Cancer Genet Cytogenet 195:12-8. 2009
    ..The involvement of the DUX4 region might represent the genetic hallmark of a novel subclass of small round cell tumors...
  10. ncbi Myogenin and desmin immunohistochemistry in the assessment of post-chemotherapy genitourinary embryonal rhabdomyosarcoma: prognostic and management implications
    P Godbole
    Department of Paediatric Urology, Great Ormond Street Hospital for Children, London, United Kingdom
    J Urol 176:1751-4. 2006
    Posttreatment genitourinary embryonal rhabdomyosarcoma often shows well differentiated rhabdomyoblasts, which are detectable on routine histological staining...
  11. doi Identification of PAX3-FKHR-regulated genes differentially expressed between alveolar and embryonal rhabdomyosarcoma: focus on MYCN as a biologically relevant target
    Gabriela E Mercado
    Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA
    Genes Chromosomes Cancer 47:510-20. 2008
    ..In conclusion, we identified a selected set of biologically relevant genes modulated by PAX3-FKHR, and demonstrated that PAX3-FKHR contributes to the expression of MYCN and in turn MYCN collaborates with PAX3-FKHR in tumorigenesis...
  12. ncbi Teratoma in an adolescent with malignant transformation into embryonal rhabdomyosarcoma: case report
    David Sumerauer
    Department of Pediatric Hematology and Oncology, Charles University, Second Medical School, University Hospital Motol, Prague, Czech Republic
    J Pediatr Hematol Oncol 28:688-92. 2006
    ..The somatic type tumors are occasionally found in nonseminomatous germ cell tumors in men. These malignancies are presumed to arise from malignant transformation (MT) of teratoma or by differentiation of totipotential germ cell...
  13. 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...
  14. doi MEK/ERK inhibitor U0126 affects in vitro and in vivo growth of embryonal rhabdomyosarcoma
    Francesco Marampon
    Department of Experimental Medicine, University of L Aquila, Via Vetoio Coppito II, 67100 L Aquila, Italy
    Mol Cancer Ther 8:543-51. 2009
    ..signal-regulated kinase (ERK) inhibition blocks the expression of the transformed phenotype in the embryonal rhabdomyosarcoma (ERMS) cell line (RD), thereby inducing myogenic differentiation in vitro...
  15. ncbi Aberrant PAX3 and PAX7 expression. A link to the metastatic potential of embryonal rhabdomyosarcoma and cutaneous malignant melanoma?
    J Blake
    School of Biomedical and Sports Science, Edith Cowan University, Joondalup, Western Australia, Australia
    Histol Histopathol 18:529-39. 2003
    ..In highly metastatic neoplasms arising from cells of neurogenic and myogenic lineages such as embryonal rhabdomyosarcoma and cutaneous malignant melanoma, markedly high expression levels of PAX3 and PAX7 support this ..
  16. doi Noonan syndrome, the SOS1 gene and embryonal rhabdomyosarcoma
    Marjolijn C J Jongmans
    Department of Human Genetics, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    Genes Chromosomes Cancer 49:635-41. 2010
    ..Sequence analysis of the SOS1 gene in 20 sporadic ERMS tumors failed to reveal any pathogenic mutations, implicating that SOS1 is not a major player in the development of this tumor outside the context of NS...
  17. ncbi Embryonal rhabdomyosarcoma of the larynx
    Oguz Dikbas
    Department of Medical Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey
    Otolaryngol Head Neck Surg 133:160-2. 2005
  18. ncbi Uniparental disomy at chromosome 11p15.5 followed by HRAS mutations in embryonal rhabdomyosarcoma: lessons from Costello syndrome
    Christian P Kratz
    Division of Pediatric Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, University of Freiburg, Mathildenstrasse 1, 79106 Freiburg, Germany
    Hum Mol Genet 16:374-9. 2007
    ..MIM 218040) is characterized by short stature, facial dysmorphism, cardiac defects and predisposition to embryonal rhabdomyosarcoma (CS/ERMS) and other neoplasias. CS is caused by germline mutations in the HRAS gene on chromosome 11p15...
  19. doi RAS signaling dysregulation in human embryonal Rhabdomyosarcoma
    Simone Martinelli
    Department of Hematology, Oncology and Molecular Medicine, Istituto Superiore di Sanita, Rome, Italy
    Genes Chromosomes Cancer 48:975-82. 2009
    ..Our findings provide evidence that dysregulation of RAS signaling is a major event contributing to embryonal RMS pathogenesis...
  20. 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...
  21. ncbi Botryoid-type of embryonal rhabdomyosarcoma of renal pelvis in an adult. A case report and review of the literature
    L Kren
    Department of Pathology, University Hospital Brno, Czech Republic
    Cesk Patol 39:31-5. 2003
    A case of botryoid-type embryonal rhabdomyosarcoma of the renal pelvis in a 49-year-old woman is reported. The tumor led to hydronephrosis...
  22. pmc Mice lacking dystrophin or alpha sarcoglycan spontaneously develop embryonal rhabdomyosarcoma with cancer-associated p53 mutations and alternatively spliced or mutant Mdm2 transcripts
    Karen Fernandez
    Division of Hematology Oncology, Nationwide Children s Hospital, Columbus, Ohio 43205, USA
    Am J Pathol 176:416-34. 2010
    ..mice or alpha sarcoglycan in Sgca(-/-) mice, results in the spontaneous development of muscle-derived embryonal rhabdomyosarcoma (RMS) after 1 year of age...
  23. ncbi Cell-specific toxicity of fibrates in human embryonal rhabdomyosarcoma cells
    Takayoshi Maiguma
    Department of Hospital Pharmacy, Faculty of Medicine, Kyushu University, 812 8582 Fukuoka, Japan
    Naunyn Schmiedebergs Arch Pharmacol 367:289-96. 2003
    The effects of a variety of fibrates on the cell viability were examined in human embryonal rhabdomyosarcoma cells (HRMSC)...
  24. doi 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
    ..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 gene.
  25. ncbi Embryonal rhabdomyosarcoma with a der(16)t(1;16) translocation
    Kayla M Kapels
    Department of Pathology and Microbiology, University of Nebraska Medical Center, 983135 Nebraska Medical Center, Omaha, NE 68198 3135, USA
    Cancer Genet Cytogenet 174:68-73. 2007
    b>Embryonal rhabdomyosarcoma (ERMS) is the most common subtype of RMS that predominantly involves the genitourinary tract and the head and neck regions in children younger than 10 years of age...
  26. pmc PAX7 expression in embryonal rhabdomyosarcoma suggests an origin in muscle satellite cells
    N Tiffin
    Section of Paediatric Oncology, Institute of Cancer Research, Belmont, Sutton, Surrey SM2 5NG, UK
    Br J Cancer 89:327-32. 2003
    ..This is corroborated by the detection of MET (c-met) expression, a marker for the myogenic satellite cell lineage, in all RMS samples expressing wild-type PAX7...
  27. ncbi Recognition of Li Fraumeni syndrome at diagnosis of a locally advanced extremity rhabdomyosarcoma
    T Trahair
    Centre for Children s Cancer and Blood Disorders, Sydney Children s Hospital, Randwick, New South Wales, Australia
    Pediatr Blood Cancer 48:345-8. 2007
    ....
  28. ncbi Hemihypertrophy and a poorly differentiated embryonal rhabdomyosarcoma of the pelvis
    D P Samuel
    Pediatric Branch, National Cancer Institute, National Institute of Health, Bethesda, MD 20895, USA
    Med Pediatr Oncol 32:38-43. 1999
    ..Loss of heterozygosity (LOH) of chromosome 11p15.5 is strongly associated with childhood embryonal tumors, particularly Wilms tumor, hepatoblastoma, and rhabdomyosarcoma...
  29. 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
    ..This hypothesis requires testing in a prospective study. Variant t(1;13)/PAX7-FKHR appears biologically different, occurring in younger patients with more localised disease...
  30. ncbi Insulin-like growth factor-I has different effects on myogenin induction and cell cycle progression in human alveolar and embryonal rhabdomyosarcoma cells
    Kunihiko Tsuchiya
    Department of Pediatrics, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Kamigyo ku, Kyoto 602 8566, Japan
    Int J Oncol 31:41-7. 2007
    ..These different responses to IGF-I help to explain immunohistochemical and clinical behavioral differences between alveolar and embryonal RMS...
  31. ncbi Embryonal rhabdomyosarcoma with uterine inversion: case report
    S B O Ojwang
    Department of Obstetrics and Gynaecology, College of Health Sciences, University of Nairobi P O Box 19676 00200, Nairobi, Kenya
    East Afr Med J 83:110-3. 2006
    Presented here is a 16-year-old nulliparous girl who had embryonal rhabdomyosarcoma causing uterine inversion. She had been referred with a one year history of continuous vaginal bleeding and vaginal swelling...
  32. ncbi Embryonal rhabdomyosarcoma passed per urethra
    M J A Perry
    Department of Urology, Frimley Park Hospital, Portsmouth Road, Surrey, United Kingdom
    J Urol 167:2167. 2002
  33. 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
    ..includes two major histological subtypes: alveolar rhabdomyosarcoma (ARMS), the more aggressive, and embryonal rhabdomyosarcoma (ERMS)...
  34. doi Different expression profiles of Y-box-binding protein-1 and multidrug resistance-associated proteins between alveolar and embryonal rhabdomyosarcoma
    Yoshinao Oda
    Department of Anatomic Pathology, Graduate School of Medical Sciences, Kyushu University, 3 1 1 Maidashi, Higashi ku, Fukuoka 812 8582, Japan
    Cancer Sci 99:726-32. 2008
    ..Apart from ERMS, frequent P-gp expression in ARMS may be independent from YB-1 regulation. However, YB-1 may be a candidate for a molecular target in rhabdomyosarcoma therapy, especially in ERMS...
  35. ncbi Validation of met as a therapeutic target in alveolar and embryonal rhabdomyosarcoma
    Riccardo Taulli
    Center for Experimental Research and Medical Studies CERMS, University of Turin, Turin, Italy
    Cancer Res 66:4742-9. 2006
    ....
  36. doi Embryonal rhabdomyosarcoma of the uterus associated with uterine inversion in an adolescent: a case report and published work review
    Benedito Borges da Silva
    Department of Gynecology, Federal University of Piaui, Teresina, Piaui, Brazil
    J Obstet Gynaecol Res 34:735-8. 2008
    ..Following biopsy of the lesion and confirmation of the diagnosis of embryonal rhabdomyosarcoma, the patient was submitted to total abdominal hysterectomy followed by chemotherapy and radiotherapy...
  37. 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 ..
  38. ncbi Expression of cyclin-dependent kinase inhibitor p27/Kip1 and AP-1 coactivator p38/Jab1 correlates with differentiation of embryonal rhabdomyosarcoma
    Rika Tsuchida
    Department of Pediatrics and Developmental Biology, Postgraduate Medical School, Tokyo Medical and Dental University, 1 5 45 Yushima, Bunkyo ku, Tokyo 113 8519, Japan
    Jpn J Cancer Res 93:1000-6. 2002
    ..Here we analyzed the expression of p27 and JAB1 by immunohistochemistry in embryonal rhabdomyosarcoma (E-RMS)...
  39. 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...
  40. ncbi An unusual localization of embryonal rhabdomyosarcoma in a neonate
    Askin Gura
    Division of Neonatology, Department of Pediatrics, Akdeniz University Faculty of Medicine, Antalya, Turkey
    Turk J Pediatr 49:82-4. 2007
    ..Herein, we report a neonate with embryonal RMS arising from the anterior chest wall musculature at birth...
  41. ncbi Primary embryonal rhabdomyosarcoma of prostate in adults: diagnosis and management
    G Nabi
    Department of Urology, All India Institute of Medical Sciences, New Delhi 29, India
    Int Urol Nephrol 34:531-4. 2002
    b>Embryonal rhabdomyosarcoma of the prostate is a common tumour in infants and children. It is rarely seen in adults. The combined modality has improved outcome in children, where as in adults prognosis is poor...
  42. ncbi Orbital rhabdomyosarcoma: relationship between DNA ploidy, p53, bcl-2, MDR-1 and Ki67 (MIB1) expression and clinical behavior
    Stefania Staibano
    Department of Biomorphological and Functional Sciences, Pathology Section, University of Naples Federico II, Italy
    Anticancer Res 24:249-57. 2004
    ..We investigated the role of DNA ploidy and immunohistochemical expression of p53, bcl-2, MDR-1 and Ki67 (MIB1) in the prognostic evaluation of orbital rhabdomyosarcomas...
  43. ncbi Results of treatment of fifty-six patients with localized retroperitoneal and pelvic rhabdomyosarcoma: a report from The Intergroup Rhabdomyosarcoma Study-IV, 1991-1997
    R Beverly Raney
    Department of Clinical Pediatrics, University of Texas M D Anderson Cancer Center, Houston, Texas 77030, USA
    Pediatr Blood Cancer 42:618-25. 2004
    ..We reviewed 56 IRS-IV patients with localized rhabdomyosarcoma [RMS] of the retroperitoneum/pelvis to assess outcome and prognostic factors, including the value of initially excising >or=50% of the tumor (debulking) before chemotherapy...
  44. 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...
  45. 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)...
  46. 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...
  47. ncbi [Study on microtubulin of human rhabdomyosarcoma]
    Lu Ye
    Department of Pathology, West China Hospital, Sichuan University, Chengdu 610041, China
    Sichuan Da Xue Xue Bao Yi Xue Ban 35:50-3. 2004
    ..To detect the expression of microtubules in human rhabdomyosarcoma...
  48. ncbi Paediatric rhabdomyosarcoma of the ear and temporal bone
    D V Durve
    Departments of Oncology and Otolaryngology, Great Ormond Street Hospital for Children NHS Trust, London, UK
    Clin Otolaryngol Allied Sci 29:32-7. 2004
    ..Discharge, hearing loss and aural polyp, although commonly because of chronic otitis media, should prompt urgent investigation and biopsy, particularly if associated with facial palsy, lymphadenopathy or an obvious mass...
  49. ncbi Is alveolar histotype a prognostic factor in paratesticular rhabdomyosarcoma? The experience of Italian and German Soft Tissue Sarcoma Cooperative Group
    Andrea Ferrari
    Pediatric Oncology Unit, Istituto Nazionale Tumori, Milano, Italy
    Pediatr Blood Cancer 42:134-8. 2004
    ..To ascertain whether alveolar histology retains its adverse prognostic role in the subset of paratesticular rhabdomyosarcoma (RMS) patients, generally characterized by a very good outcome...
  50. 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...
  51. ncbi Detection of bone marrow micrometastasis and microcirculating disease in rhabdomyosarcoma by a real-time RT-PCR assay
    Soledad Gallego
    Pediatric Oncology Unit, Hospital Universitari Vall d Hebron, Barcelona, Spain
    J Cancer Res Clin Oncol 132:356-62. 2006
    ..To assess if molecular detection of minimal disseminated disease by real-time reverse transcription and polymerase chain reaction (RT-PCR) could contribute to a better treatment stratification in patients with rhabdomyosarcoma (RMS)...
  52. 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...
  53. 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...
  54. 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)...
  55. ncbi [Orbital rhabdomyosarcoma: difficulties with European treatment protocol]
    N Olivier Pascual
    Hospital Universitario La Paz, Madrid, Spain
    Arch Soc Esp Oftalmol 80:331-8. 2005
    ..Review of the 12 cases of paediatric rhabdomiosarcoma and results of treatment in terms of mortality and morbidity comparing our results with other studies...
  56. ncbi Treatment strategies for perianal rhabdomyosarcoma: report of two cases
    Yoshio Watanabe
    Department of Surgery, Ogaki Municipal Hospital, Ogaki, Japan
    Surg Today 34:719-24. 2004
    ..She has since been tumor-free with preserved anal function. These case reports show the importance of cogitated treatments for preservation of anal function and optimal therapeutic outcome in patients with perianal RMS...
  57. 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...
  58. 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...
  59. 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
    ..showed 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...
  60. 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...
  61. 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...
  62. ncbi [Rhabdomysarcoma and combined treatment in oral and maxillofacial regions]
    Ge Feng
    Dept of Oral and Maxillofacial Surgery, West China College of Stomatology, Sichuan University, Chengdu 610041, China
    Hua Xi Kou Qiang Yi Xue Za Zhi 22:129-31. 2004
    ..To study the treatment outcome of rhabdomysarcoma(RMS)...
  63. ncbi O6-methylguanine-DNA methyltransferase activity and promoter methylation status in pediatric rhabdomyosarcoma
    Nicholas D Yeager
    Hematology Oncology, Children s Hospital, Columbus, Ohio 43205, USA
    J Pediatr Hematol Oncol 25:941-7. 2003
    ..To determine the activity of the DNA repair protein O6-methylguanine-DNA methyltransferase (MGMT) and MGMT promoter methylation status of pediatric rhabdomyosarcoma (RMS) and examine MGMT in RMS tumors from different prognostic groups...
  64. ncbi Primary and metastatic rhabdomyosarcoma in the breast: report of two pediatric cases
    F Binokay
    Department of Radiology, Faculty of Medicine, Cukurova University, Balcali Adana 01330, Turkey
    Eur J Radiol 48:282-4. 2003
    ..Primary one is alveolar, and the other embryonal subtype. Imaging findings with ultrasonography (US), computed tomography (CT) and magnetic resonance imaging (MRI) and a thorough review of literature are presented...
  65. 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...
  66. 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)...
  67. ncbi MYCN expression in human rhabdomyosarcoma cell lines and tumour samples
    Luisa Toffolatti
    Clinica di Oncoematologia Pediatrica, Azienda Ospedaliera Universita di Padova, Padova, Italy
    J Pathol 196:450-8. 2002
    ..MYCN expression and amplification were studied in five alveolar and five embryonal rhabdomyosarcoma cell lines and in 19 tumour biopsies...
  68. pmc Results of treatment of patients with superficial facial rhabdomyosarcomas on protocols of the Intergroup Rhabdomyosarcoma Study Group (IRSG), 1984-1997
    R Beverly Raney
    Division of Pediatrics and Children s Cancer Hospital, University of Texas M D Anderson Cancer Center, Houston, Texas, USA
    Pediatr Blood Cancer 50:958-64. 2008
    ..We analyzed the outcome of 47 patients with superficial facial rhabdomyosarcoma (RMS) treated on Intergroup Rhabdomyosarcoma Study Group (IRSG) Protocols-III, -IV-Pilot, and -IV...
  69. 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)...
  70. 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
    ....
  71. 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...
  72. ncbi Subcellular localization of the steroid receptor coactivators (SRCs) and MEF2 in muscle and rhabdomyosarcoma cells
    S L Chen
    University of Queensland Centre for Molecular and Cellular Biology Institute for Molecular Bioscience St. Lucia, 4072 Queensland, Australia
    Mol Endocrinol 15:783-96. 2001
    ..In contrast, RMS cells display aberrant transcription factor SRC localization and expression, which may underlie certain features of the RMS phenotype...
  73. doi Primary pulmonary rhabdomyosarcoma in a 5-month-old boy: a case report
    Henry L Chang
    Division of Pediatric Surgery, Department of Surgery, Department of Pediatrics, Massachusetts General Hospital, Boston, MA, USA
    J Pediatr Hematol Oncol 30:461-3. 2008
    ..This patient is the youngest reported to date with this entity. The prognostic significance of his young age and the morphologic findings of his tumor are unclear...
  74. 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
    ..identified in the 5' flanking region of the MyoD1 gene from the two major subtypes, ie, alveolar and embryonal rhabdomyosarcoma. The MyoD1 methylation patterns of 26 rhabdomyosarcomas were compared with that of normal skeletal ..
  75. 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 ..
  76. 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...
  77. doi 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...
  78. 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...
  79. 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
    ....
  80. 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...
  81. 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...
  82. ncbi The truth about alveolar rhabdomyosarcoma
    Dennis K Heffner
    Department of Endocrine and Otolaryngic Head and Neck Pathology, Armed Forces Institute of Pathology, Washington, DC 20307 6000, USA
    Ann Diagn Pathol 7:259-63. 2003
    ..This review discusses the important aspects of alveolar rhabdomyosarcoma and hopefully can be of some help in the proper diagnosis of this tumor...
  83. ncbi Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institution
    Andrea Ferrari
    Pediatric Oncology Unit, Istituto Nazionale Tumori, Milan, Italy
    Cancer 98:571-80. 2003
    ..Published series have reported definitively worse results for adults with RMS compared with children with RMS. This finding casts doubt on whether RMS is the same disease in adults as it is in children...
  84. pmc Myogenin and MyoD1 expression in paediatric rhabdomyosarcomas
    N J Sebire
    Department of Histopathology, Camelia Botnar Laboratories, Great Ormond Street Hospital, Great Ormond Street, London GU21 2FB, UK
    J Clin Pathol 56:412-6. 2003
    ..This review examines the use of the immunohistochemical markers, MyoD1 and myogenin, in the diagnosis of paediatric rhabdomyosarcoma, including its subtypes...
  85. 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
    ..In 2 cases, there was evidence of rhabdomyoblastic differentiation. Only one case was classified as embryonal rhabdomyosarcoma. A significant number of tumor cells in these cases had clear or vacuolated cytoplasm...
  86. ncbi Volumetric considerations in radiotherapy for pediatric parameningeal rhabdomyosarcomas
    Changhu Chen
    Department of Radiation Oncology, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA
    Int J Radiat Oncol Biol Phys 55:1294-9. 2003
    ..To assess the influence of radiation volume on outcome in pediatric parameningeal rhabdomyosarcomas (PM-RMSs)...
  87. 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...
  88. ncbi Possible benefits of high-dose chemotherapy as intensive consolidation in patients with high-risk rhabdomyosarcoma who achieve complete remission with conventional chemotherapy
    Hiroshi Matsubara
    Division of Pediatric Oncology, National Cancer Center Hospital, Tokyo, Japan
    Pediatr Hematol Oncol 20:201-10. 2003
    ..The overall survival rate at 5 years was 70% for 14 patients who were in CR at the time of SCT. This limited experience warrants the examination of SCT in a prospective study...
  89. 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...
  90. ncbi Cell division cycle control in embryonal and alveolar rhabdomyosarcomas
    A Moretti
    Department of Evolutive Age, University of Bari, Italy
    Eur J Cancer 38:2290-9. 2002
    ..Moreover, the data on butyrate-treated cell lines suggest that the two genes are potential novel therapeutic targets for the treatment of rhabdomyosarcomas...
  91. 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...
  92. 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...
  93. 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...
  94. doi Primary gallbladder sarcoma: a clinicopathologic study of 15 cases, heterogeneous sarcomas with poor outcome, except pediatric botryoid rhabdomyosarcoma
    Wael I Al-Daraji
    Department of Soft Tissue and Orthopaedic Pathology, Armed Forces Institute of Pathology, Washington, DC 20306 6000, USA
    Am J Surg Pathol 33:826-34. 2009
    ..Primary gallbladder sarcoma (PGBS) is rare, with only 39 documented cases, with the predominant type being leiomyosarcoma...
  95. ncbi Conservative treatment for lower gynecological tract malignancies in children and adolescents: the Institut Gustave-Roussy experience
    A P Gerbaulet
    Head of Brachytherapy Unit, Institut Gustave Roussy, Villejuif, France
    Int J Radiat Oncol Biol Phys 17:655-8. 1989
    ..Thirteen patients presented with clear cell adenocarcinoma, 14 patients with embryonal rhabdomyosarcoma, 6 patients with endodermal sinus tumor, 3 patients with sarcoma, and 1 patient with an undifferentiated ..
  96. ncbi Fine-needle aspiration (FNA) cytology diagnosis of small round cell tumors: value and limitations
    Dilip K Das
    Department of Pathology, Faculty of Medicine, Kuwait University, Safat, Kuwait
    Indian J Pathol Microbiol 47:309-18. 2004
    ..hepatoblastoma, blastema cells with tubular differentiation in nephroblastoma, tadpole shaped cells in embryonal rhabdomyosarcoma, extreme nuclear molding and perinuclear blue inclusion in small cell anaplastic carcinoma (SCAC), ..
  97. ncbi The evolution of the diagnosis and understanding of primitive and embryonic neoplasms in children: living through an epoch
    L P Dehner
    Lauren V Ackerman Laboratory of Surgical Pathology, Washington University Medical Center, Barnes Jewish and St Louis Children s Hospitals, Missouri, USA
    Mod Pathol 11:669-85. 1998
    ..Archetypes include classic neuroblastoma, Wilms' tumor, and embryonal rhabdomyosarcoma. This review traces the history of the principal types of dysontogenetic neoplasms and the primitive ..
  98. ncbi Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 cases
    Hugo Dominguez Malagon
    Department of Pathology, Instituto Nacional de Cancerologia, Mexico
    Am J Surg Pathol 31:1356-62. 2007
    ..The SC included embryonal rhabdomyosarcoma (29), angiosarcoma (6), leiomyosarcoma (4), undifferentiated sarcoma (3), myxoid liposarcoma (1), ..
  99. ncbi Smad4 and ERK2 stimulated by transforming growth factor beta1 in rhabdomyosarcoma
    Hua Guo
    Department of Pathology, Peking University First Hospital, Beijing 100034, China
    Chin Med J (Engl) 120:515-21. 2007
    ..other signal transduction pathways besides Smads, which are important to regulate the growth of human embryonal rhabdomyosarcoma (RMS) cells...
  100. 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
    ..The IRSG has reported improvement in the prognosis of both nonmetastatic and metastatic embryonal rhabdomyosarcoma as attributable to three, three-drug regimens that use cyclophosphamide at 2...
  101. ncbi PAX3/forkhead homolog in rhabdomyosarcoma oncoprotein activates glucose transporter 4 gene expression in vivo and in vitro
    Michal Armoni
    Institute of Endocrinology, Diabetes and Metabolism, Rambam Medical Center, Technion Israel Institute of Technology, Haifa 31096, Israel
    J Clin Endocrinol Metab 87:5312-24. 2002
    ..RH30 myoblasts, which both express the PAX3/FKHR chimeric protein, but not in either C2C12 or embryonal rhabdomyosarcoma-derived myoblasts. GLUT4 was functionally active in RH30 cells, because insulin induced a 1...

Research Grants64

  1. Cancer Biology in the Zebrafish
    LEONARD IRA ZON; Fiscal Year: 2010
    ..In a second model, a constitutive KRAS allele leads to embryonal rhabdomyosarcoma at 10 days of age. This is the fastest tumor model in any vertebrate...
  2. MyoD Activity in Rhabdomyosarcomas
    Zhihong Yang; Fiscal Year: 2007
    ..this research proposal is to investigate the molecular mechanisms that prevent differentiation in human embryonal rhabdomyosarcoma (RD) cells...
  3. A MURINE MODEL OF SMITH-MAGENIS SYNDROME
    Cornelius Boerkoel; Fiscal Year: 2003
    ..The Mentored Scientist Development Award would further facilitate this process. ..
  4. Characterization of SMARCAL1:its expression/interactors
    Cornelius Boerkoel; Fiscal Year: 2003
    ..abstract_text> ..
  5. A Mouse Model for Schimke Immuno-osseous Dysplasia
    Cornelius Boerkoel; Fiscal Year: 2006
    ..The funding provided by this R21 is critical for me, as a new investigator, to develop these murine models. ..
  6. COG studies of gene amplification in rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2009
    ..abstract_text> ..
  7. IRSG STUDIES OF ALVEOLAR RHABDOMYOSARCOMA GENE FUSIONS
    FREDERIC BARR; Fiscal Year: 2005
    ..These studies will provide a definitive analysis of the occurrence and clinical significance of these genetic alterations in ARMS, and will ultimately impact on the future design of clinical protocols for the treatment of ARMS patients. ..
  8. CASE-CONTROL STUDY OF HODGKIN'S DISEASE IN CHILDREN
    Seymour Grufferman; Fiscal Year: 2003
    ..The study will focus on Hispanic cases since they differ significantly from other cases in EBV-positivity of their tumors and other epidemiologic features. ..
  9. Studies of the t(2;13) of alveolar rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2007
    ..abstract_text> ..
  10. COG studies of gene amplification in rhabdomyosarcoma
    FREDERIC BARR; Fiscal Year: 2007
    ..abstract_text> ..
  11. Cancer Molecular Pathology Training Program
    FREDERIC BARR; Fiscal Year: 2006
    ....
  12. MOLECULAR CHARACTERIZATION OF TYPE XIX COLLAGEN
    JEANNE MYERS; Fiscal Year: 2005
    ..The experiments proposed here will significantly advance understanding of a collagen manifested in seemingly different roles in adult tissue BMZ, and in the myogenic pathway evidenced in RMS and muscle regeneration. ..
  13. A New Collagen Expressed Primarily in Brain Tissue
    JEANNE MYERS; Fiscal Year: 2002
    ..The highly unusual tissue representation of Collagen B presents a unique opportunity for future elucidation of its role in CNS development, and in neuropathological conditions that will become increasingly prevalent in an aging society. ..
  14. STUDIES OF THE T (2;13) OF ALVEOLAR RHABDOMYOSARCOMA
    FREDERIC BARR; Fiscal Year: 2002
    ....
  15. STRUCTURE, FUNCTION AND EXPRESSION OF TYPE XV COLLAGEN
    JEANNE MYERS; Fiscal Year: 2001
    ..The organization and structure of type XV collagen and the molecules with whom it interacts will elucidate their involvement in inherited diseases of connective tissue and other pathologic processes. ..
  16. REGULATION OF PAX3/PAX7 EXPRESSION IN RHABDOMYOSARCOMA
    FREDERIC BARR; Fiscal Year: 2001
    ..Studies of embryonal rhabdomyosarcoma (ERMS), a second pediatric cancer related to the myogenic lineage, have revealed differences in wild-..
  17. CASE-CONTROL STUDY OF HODGKIN'S DISEASE IN CHILDHOOD
    Seymour Grufferman; Fiscal Year: 1990
    ..An estimated total of 484 controls is expected. This study will fill several important gaps in knowledge of the epidemiology of HD and can provide important new insights into the possibly infectious etiology of human cancer...