Genomes and Genes
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)
Publications173 found, 100 shown here
- ["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)...
- Fusion gene-negative alveolar rhabdomyosarcoma is clinically and molecularly indistinguishable from embryonal rhabdomyosarcomaDaniel 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.
- PAX7 expression in embryonal rhabdomyosarcoma suggests an origin in muscle satellite cellsN 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...
- Mice lacking dystrophin or alpha sarcoglycan spontaneously develop embryonal rhabdomyosarcoma with cancer-associated p53 mutations and alternatively spliced or mutant Mdm2 transcriptsKaren 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...
- Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcomaRamapriya 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 ..
- MEK/ERK inhibitor U0126 affects in vitro and in vivo growth of embryonal rhabdomyosarcomaFrancesco 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...
- Effects of RAS on the genesis of embryonal rhabdomyosarcomaDavid 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. 2007b>Embryonal rhabdomyosarcoma (ERMS) is a devastating cancer with specific features of muscle differentiation that can result from mutational activation of RAS family members...
- 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 ..
- Uniparental disomy at chromosome 11p15.5 followed by HRAS mutations in embryonal rhabdomyosarcoma: lessons from Costello syndromeChristian 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...
- Embryonal rhabdomyosarcoma of the larynxOguz Dikbas
Department of Medical Oncology, Hacettepe University Faculty of Medicine, Ankara, Turkey
Otolaryngol Head Neck Surg 133:160-2. 2005
- Cell-specific toxicity of fibrates in human embryonal rhabdomyosarcoma cellsTakayoshi Maiguma
Department of Hospital Pharmacy, Faculty of Medicine, Kyushu University, 812-8582 Fukuoka, Japan
Naunyn Schmiedebergs Arch Pharmacol 367:289-96. 2003The effects of a variety of fibrates on the cell viability were examined in human embryonal rhabdomyosarcoma cells (HRMSC)...
- Novel genomic imbalances in embryonal rhabdomyosarcoma revealed by comparative genomic hybridization and fluorescence in situ hybridization: an intergroup rhabdomyosarcoma studyJ 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...
- RAS signaling dysregulation in human embryonal RhabdomyosarcomaSimone 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...
- Noonan syndrome, the SOS1 gene and embryonal rhabdomyosarcomaMarjolijn 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...
- Phosphorylation profiles of protein kinases in alveolar and embryonal rhabdomyosarcomaLing 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...
- Botryoid-type of embryonal rhabdomyosarcoma of renal pelvis in an adult. A case report and review of the literatureL Kren
Department of Pathology, University Hospital Brno, Czech Republic
Cesk Patol 39:31-5. 2003A case of botryoid-type embryonal rhabdomyosarcoma of the renal pelvis in a 49-year-old woman is reported. The tumor led to hydronephrosis...
- Embryonal rhabdomyosarcoma with a der(16)t(1;16) translocationKayla 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. 2007b>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...
- Embryonal rhabdomyosarcoma of the uterus associated with uterine inversion in an adolescent: a case report and published work reviewBenedito 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...
- Altered expression and molecular abnormalities of cell-cycle-regulatory proteins in rhabdomyosarcomaYukiko 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...
- Expression of cyclin-dependent kinase inhibitor p27/Kip1 and AP-1 coactivator p38/Jab1 correlates with differentiation of embryonal rhabdomyosarcomaRika 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)...
- 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...
- Adult sclerosing rhabdomyosarcoma: cytogenetic link with embryonal rhabdomyosarcomaRomaric 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.
- Embryonal rhabdomyosarcoma with uterine inversion: case reportS 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. 2006Presented 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...
- Embryonal rhabdomyosarcoma passed per urethraM J A Perry
Department of Urology, Frimley Park Hospital, Portsmouth Road, Surrey, United Kingdom
J Urol 167:2167. 2002
- Validation of met as a therapeutic target in alveolar and embryonal rhabdomyosarcomaRiccardo Taulli
Center for Experimental Research and Medical Studies (CERMS, University of Turin, Turin, Italy
Cancer Res 66:4742-9. 2006....
- Different expression profiles of Y-box-binding protein-1 and multidrug resistance-associated proteins between alveolar and embryonal rhabdomyosarcomaYoshinao 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...
- Insulin-like growth factor-I has different effects on myogenin induction and cell cycle progression in human alveolar and embryonal rhabdomyosarcoma cellsKunihiko 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...
- Expression and activity of vascular endothelial growth factor and metalloproteinases in alveolar and embryonal rhabdomyosarcoma cell linesMaurizio 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)...
- An unusual localization of embryonal rhabdomyosarcoma in a neonateAskin 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...
- Primary embryonal rhabdomyosarcoma of prostate in adults: diagnosis and managementG Nabi
Department of Urology, All India Institute of Medical Sciences, New Delhi 29, India
Int Urol Nephrol 34:531-4. 2002b>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...
- Molecular biology of rhabdomyosarcomaS 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...
- Treatment strategies for perianal rhabdomyosarcoma: report of two casesYoshio 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...
- Paediatric rhabdomyosarcoma of the ear and temporal boneD 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...
- O6-methylguanine-DNA methyltransferase activity and promoter methylation status in pediatric rhabdomyosarcomaNicholas 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...
- [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..The decrease and hypogenesis of microtubules in human rhabdomyosarcoma have relationship with tumor's histological grade, malignant degree, metastasis, and recurrence...
- Results of treatment of fifty-six patients with localized retroperitoneal and pelvic rhabdomyosarcoma: a report from The Intergroup Rhabdomyosarcoma Study-IV, 1991-1997R 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...
- Orbital rhabdomyosarcoma: relationship between DNA ploidy, p53, bcl-2, MDR-1 and Ki67 (MIB1) expression and clinical behaviorStefania 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...
- Delayed radiotherapy following dose intensive chemotherapy for parameningeal rhabdomyosarcoma (PM-RMS) of childhoodJames 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...
- Results of treatment of patients with superficial facial rhabdomyosarcomas on protocols of the Intergroup Rhabdomyosarcoma Study Group (IRSG), 1984-1997R 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...
- Is alveolar histotype a prognostic factor in paratesticular rhabdomyosarcoma? The experience of Italian and German Soft Tissue Sarcoma Cooperative GroupAndrea 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...
- Rhabdomyosarcoma: value of myogenin expression analysis and molecular testing in diagnosing the alveolar subtype: an analysis of 109 paraffin-embedded specimensIsabelle Hostein
Department of Pathology, Institut Bergoni, Bordeaux, France
Cancer 101:2817-24. 2004..Although RT-PCR analysis appears not to possess diagnostic value in tumors with < 50% tumor cell immunopositivity, it is strongly recommended for the diagnosis of tumors containing > 50% myogenin-positive cells...
- Childhood rhabdomyosarcoma in Ibadan, Nigeria: 1984-2003B 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..There is a need for larger descriptive studies on childhood rhabdomyosarcoma in Africa...
- Novel genes implicated in embryonal, alveolar, and pleomorphic rhabdomyosarcoma: a cytogenetic and molecular analysis of primary tumorsMyriam 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...
- Juvenile rhabdomyosarcomas in Port Harcourt, Nigeria: A twelve year reviewD 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..8%) in this study. The most common histologic type is the embryonal rhabdomyosarcoma (71.5%). Alveolar rhabdomyosarcoma accounted for (19%) and the sarcoma botryoides (9.5%)...
- Sinonasal rhabdomyosarcoma in children and young adultsAtif 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...
- Mirk/Dyrk1b mediates cell survival in rhabdomyosarcomasStephen 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...
- The challenging role of radiation therapy for very young children with rhabdomyosarcomaDev 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..Local control may be maximized when EBRT begins <or=18 weeks after initiation of CMT, but further study is warranted. Longer follow-up is required to determine the full extent of late effects...
- Rhabdomyosarcoma: molecular diagnostics of patients classified by morphology and immunohistochemistry with emphasis on bone marrow and purged peripheral blood progenitor cells involvementL 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...
- Deregulation of the hedgehog signalling pathway: a possible role for the PTCH and SUFU genes in human rhabdomyoma and rhabdomyosarcoma developmentUlrica 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...
- Detection of bone marrow micrometastasis and microcirculating disease in rhabdomyosarcoma by a real-time RT-PCR assaySoledad 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)...
- [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...
- Altered expression of natively glycosylated alpha dystroglycan in pediatric solid tumorsLaura 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....
- Upstream CpG island methylation of the PAX3 gene in human rhabdomyosarcomasRaushan 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...
- Different pattern of matrix metalloproteinases expression in alveolar versus embryonal rhabdomyosarcomaFrancesca Diomedi-Camassei
Department of Pathology, Children's Hospital Bambino Gesu, Research Institute, Rome, Italy
J Pediatr Surg 39:1673-9. 2004..Further characterization of the expression of MMPs and consequent utilization of their inhibitors in aggressive alveolar RMSs might lead to the development of novel anticancer therapies...
- Effects of standard chemotherapy on tumor growth and regulation of multidrug resistance genes and proteins in childhood rhabdomyosarcomaGuido 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...
- Primary and metastatic rhabdomyosarcoma in the breast: report of two pediatric casesF 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...
- Ultrastructural characterization of poorly differentiated rhabdomyosarcomasL 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...
- Value of image cytometry in the subclassification of rhabdomyosarcomaZiva 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...
- Identification of a novel gene NCRMS on chromosome 12q21 with differential expression between rhabdomyosarcoma subtypesAgnes 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...
- Rhabdomyosarcoma of the oral and maxillofacial region in Jordanians: a retrospective analysisTaiseer 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...
- 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 phenotypeAmy 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...
- 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)...
- MYCN expression in human rhabdomyosarcoma cell lines and tumour samplesLuisa Toffolatti
Clinica di Oncoematologia Pediatrica, , 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...
- Results of a prospective minimal disseminated disease study in human rhabdomyosarcoma using three different molecular markersFrancesca Sartori
Clinic for Pediatric Oncology, Azienda Ospedaliera-University of Padova, Padova, Italy
Cancer 106:1766-75. 2006..Our study suggests that bone marrow involvement is more common in ARMS compared with ERMS, and that MDD can be often cleared by the initial chemotherapy cycles...
- Histology-specific expression of a DNA repair protein in pediatric rhabdomyosarcomasB 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)...
- Genomic gains and losses are similar in genetic and histologic subsets of rhabdomyosarcoma, whereas amplification predominates in embryonal with anaplasia and alveolar subtypesJulia A Bridge
Department of Pathology and Microbiology, University of Nebraska Medical Center, Omaha, NE 68198, USA
Genes Chromosomes Cancer 33:310-21. 2002....
- Subcellular localization of the steroid receptor coactivators (SRCs) and MEF2 in muscle and rhabdomyosarcoma cellsS 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...
- DNA microarrays in pediatric cancerT 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 ..
- Up-front window trial of topotecan in previously untreated children and adolescents with metastatic rhabdomyosarcoma: an intergroup rhabdomyosarcoma studyA 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...
- 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 StudyIvo 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...
- Methylation alterations of the MyoD1 upstream region are predictive of subclassification of human rhabdomyosarcomasB 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 ..
- Primary pulmonary rhabdomyosarcoma in a 5-month-old boy: a case reportHenry 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...
- The expression of WT1 in the differentiation of rhabdomyosarcoma from other pediatric small round blue cell tumorsD 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...
- The truth about alveolar rhabdomyosarcomaDennis 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...
- Rhabdomyosarcoma in adults. A retrospective analysis of 171 patients treated at a single institutionAndrea 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...
- [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..The survival rates demonstrated great differences depending on the different clinical stages and therapy. CONCLUSION: The combined therapy including radiotherapy after surgical treatment may increase 5-year survival rate...
- Myogenin and MyoD1 expression in paediatric rhabdomyosarcomasN 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...
- Identification of PAX3-FKHR-regulated genes differentially expressed between alveolar and embryonal rhabdomyosarcoma: focus on MYCN as a biologically relevant targetGabriela 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...
- Volumetric considerations in radiotherapy for pediatric parameningeal rhabdomyosarcomasChanghu 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)...
- Possible benefits of high-dose chemotherapy as intensive consolidation in patients with high-risk rhabdomyosarcoma who achieve complete remission with conventional chemotherapyHiroshi 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...
- Actin isoform pattern expression: a tool for the diagnosis and biological characterization of human rhabdomyosarcomaSophie 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...
- Cell division cycle control in embryonal and alveolar rhabdomyosarcomasA 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...
- ALK protein expression in rhabdomyosarcomasK Pillay
Department of Pathology, Nelson R. Mandela School of Medicine, University of Natal, Durban, South Africa
Histopathology 41:461-7. 2002..However, ALK expression is not restricted to this subtype. An extension of this study is to determine if this over-expression is as a result of the t(2;5) translocation...
- Primary gallbladder sarcoma: a clinicopathologic study of 15 cases, heterogeneous sarcomas with poor outcome, except pediatric botryoid rhabdomyosarcomaWael 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...
- Conservative treatment for lower gynecological tract malignancies in children and adolescents: the Institut Gustave-Roussy experienceA 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 ..
- Fine-needle aspiration (FNA) cytology diagnosis of small round cell tumors: value and limitationsDilip 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), ..
- The evolution of the diagnosis and understanding of primitive and embryonic neoplasms in children: living through an epochL 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 ..
- Smad4 and ERK2 stimulated by transforming growth factor beta1 in rhabdomyosarcomaHua 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...
- Germ cell tumors with sarcomatous components: a clinicopathologic and immunohistochemical study of 46 casesHugo 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), ..
- PAX3/forkhead homolog in rhabdomyosarcoma oncoprotein activates glucose transporter 4 gene expression in vivo and in vitroMichal 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...
- Progress in the diagnosis and treatment of rhabdomyosarcoma and related soft tissue sarcomasF 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...
- Primary cerebral rhabdomyosarcoma presenting as haemorrhagic strokeH P Grebe
Department of Neurology, Hospital São Sebastião, Santa Maria da Feira, Portugal
Zentralbl Neurochir 69:93-5. 2008..Histological analysis proved it to be an embryonal rhabdomyosarcoma. The patient's neurological deficits gradually improved...
- Vaginal neoplasms in infancy: the combined role of chemotherapy and conservative surgical resectionC R McHenry
Division of Pediatric Surgery, Children s Memorial Hospital, Chicago, IL 60614
J Pediatr Surg 23:842-5. 1988..Seven had malignant tumors, two with endodermal sinus lesions and five with embryonal rhabdomyosarcoma. Early in this series, surgical therapy for embryonal rhabdomyosarcoma consisted of either anterior or ..
- Primary sarcoma of the breastManoj Pandey
Division of Surgical Oncology, Regional Cancer Centre, Medical College, Trivandrum, Kerala, India
J Surg Oncol 87:121-5. 2004..Primary sarcoma occurring in breast is rare and comprises 0.5-1% of all breast neoplasm. Majority of the series include both stromal and cystosarcoma phyllodes, only a few hundred cases of sarcomas other then cystosarcoma are reported...
- 18 F-FDG avid lesion due to coexistent fibrous dysplasia in a child of embryonal rhabdomyosarcoma: source of false positive FDG-PETSandip 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...
- Impaired Wnt signaling in embryonal rhabdomyosarcoma cells from p53/c-fos double mutant miceShalini Singh
Department of Geriatrics, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA
Am J Pathol 177:2055-66. 2010..b>Embryonal rhabdomyosarcoma (ERMS) and alveolar rhabdomyosarcoma constitute the two major subtypes and exhibit different molecular ..
- Correlation between histology and PAX/FKHR fusion status in alveolar rhabdomyosarcoma: a report from the Children's Oncology GroupDavid M Parham
Department of Pathology, University of Arkansas for Medical Sciences, Little Rock, AR, USA
Am J Surg Pathol 31:895-901. 2007..necrosis, presence and amount of rhabdomyoblastic differentiation, and the presence of foci resembling embryonal rhabdomyosarcoma. We analyzed the results using a simple chi formula...
- Activation of the hedgehog pathway confers a poor prognosis in embryonal and fusion gene-negative alveolar rhabdomyosarcomaA Zibat
Institute of Human Genetics, University of Goettingen, Goettingen, Germany
Oncogene 29:6323-30. 2010..sarcoma in children and comprises two major histological subtypes: alveolar rhabdomyosarcoma (ARMS) and embryonal rhabdomyosarcoma (ERMS)...
- Uncoupling of growth inhibition and differentiation in dexamethasone-treated human rhabdomyosarcoma cellsC De Giovanni
Istituto di Cancerologia, University of Bologna, Italy
Br J Cancer 67:674-9. 1993..RD/18 clone cells (derived from the embryonal rhabdomyosarcoma cell line RD) treated with 100 nM dexamethasone showed an almost complete block of differentiation: ..
- Ovarian small cell tumors: an electron microscopic reviewG R Dickersin
Department of Pathology, Harvard Medical School, Massachusetts General Hospital, Boston 02114, USA
Ultrastruct Pathol 22:199-226. 1998..The ultrastructural features of the metastatic tumors, such as embryonal rhabdomyosarcoma, neuroblastoma, and melanoma, are identical to those of their respective primary tumors, are well known, ..
- JNK1 determines the oncogenic or tumor-suppressive activity of the integrin-linked kinase in human rhabdomyosarcomaAdam D Durbin
Department of Medical Biophysics, University of Toronto, Toronto, Ontario, Canada
J Clin Invest 119:1558-70. 2009..aggressive pediatric sarcoma alveolar rhabdomyosarcoma (ARMS) and as a tumor suppressor in the related embryonal rhabdomyosarcoma (ERMS)...
- Cancer Biology in the ZebrafishLEONARD 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...
- MyoD Activity in RhabdomyosarcomasZhihong Yang; Fiscal Year: 2007..this research proposal is to investigate the molecular mechanisms that prevent differentiation in human embryonal rhabdomyosarcoma (RD) cells...
- A New Collagen Expressed Primarily in Brain TissueJEANNE 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. ..
- STUDIES OF THE T (2;13) OF ALVEOLAR RHABDOMYOSARCOMAFREDERIC BARR; Fiscal Year: 2002....
- COG studies of gene amplification in rhabdomyosarcomaFREDERIC BARR; Fiscal Year: 2009..abstract_text> ..
- IRSG STUDIES OF ALVEOLAR RHABDOMYOSARCOMA GENE FUSIONSFREDERIC 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. ..
- CASE-CONTROL STUDY OF HODGKIN'S DISEASE IN CHILDRENSeymour 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. ..
- Studies of the t(2;13) of alveolar rhabdomyosarcomaFREDERIC BARR; Fiscal Year: 2007..abstract_text> ..
- COG studies of gene amplification in rhabdomyosarcomaFREDERIC BARR; Fiscal Year: 2007..abstract_text> ..
- Cancer Molecular Pathology Training ProgramFREDERIC BARR; Fiscal Year: 2006....
- MOLECULAR CHARACTERIZATION OF TYPE XIX COLLAGENJEANNE 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. ..
- STRUCTURE, FUNCTION AND EXPRESSION OF TYPE XV COLLAGENJEANNE 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. ..
- REGULATION OF PAX3/PAX7 EXPRESSION IN RHABDOMYOSARCOMAFREDERIC BARR; Fiscal Year: 2001..Studies of embryonal rhabdomyosarcoma (ERMS), a second pediatric cancer related to the myogenic lineage, have revealed differences in wild-..
- CASE-CONTROL STUDY OF HODGKIN'S DISEASE IN CHILDHOODSeymour 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...