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Items 1 to 73 of about 73
1. Naini S, Etheridge KT, Adam SJ, Qualman SJ, Bentley RC, Counter CM, Linardic CM: Defining the cooperative genetic changes that temporally drive alveolar rhabdomyosarcoma. Cancer Res; 2008 Dec 1;68(23):9583-8
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  • [Title] Defining the cooperative genetic changes that temporally drive alveolar rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and adolescence.
  • Despite advances in therapy, patients with a histologic variant of RMS known as alveolar (aRMS) have a 5-year survival rate of <30%.

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  • (PMID = 19047133.001).
  • [ISSN] 1538-7445
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] ENG
  • [Grant] United States / NICHD NIH HHS / HD / HD043494-06; United States / NICHD NIH HHS / HD / 5K12-HD043494; United States / NCI NIH HHS / CA / CA094184-07; United States / NICHD NIH HHS / HD / K12 HD043494-05; United States / NCI NIH HHS / CA / R01-CA94184; United States / NCI NIH HHS / CA / R01 CA094184; United States / NICHD NIH HHS / HD / K12 HD043494; United States / NICHD NIH HHS / HD / K12 HD043494-06; United States / NCI NIH HHS / CA / R01 CA094184-07; United States / NICHD NIH HHS / HD / HD043494-05
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human
  • [Other-IDs] NLM/ NIHMS73714; NLM/ PMC2593800
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2. Cen L, Arnoczky KJ, Hsieh FC, Lin HJ, Qualman SJ, Yu S, Xiang H, Lin J: Phosphorylation profiles of protein kinases in alveolar and embryonal rhabdomyosarcoma. Mod Pathol; 2007 Sep;20(9):936-46
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  • [Title] Phosphorylation profiles of protein kinases in alveolar and embryonal rhabdomyosarcoma.
  • Rhabdomyosarcoma is the most common pediatric soft-tissue sarcoma, which includes two major subtypes, alveolar and embryonal rhabdomyosarcoma.
  • However, the oncogenic process of rhabdomyosarcoma involves multi-stages of signaling protein dysregulation characterized by prolonged activation of tyrosine and serine/threonine kinases.
  • To better understand this protein dysregulation, we evaluated the phosphorylation profiles of multiple tyrosine and serine/threonine kinases to identify whether these protein kinases are activated in rhabdomyosarcoma.
  • We applied immunohistochemistry with phospho-specific antibodies to examine phosphorylation levels of selected receptor and non-receptor tyrosine kinases, mammalian target of rapamycin (mTOR), p70S6K, and protein kinase C (PKC) isozymes on alveolar and embryonal rhabdomyosarcoma tissue microarray slides.
  • Our results showed that the phosphorylation levels of these kinases are elevated in some rhabdomyosarcoma tissues compared to normal tissues.
  • Phosphorylation levels of receptor and non-receptor tyrosine kinases are elevated between 26 and 68% in alveolar rhabdomyosarcoma and between 24 and 71% in embryonal rhabdomyosarcoma, respectively, compared to normal tissues.
  • In addition, phosphorylation levels of mTOR and its downstream targets, p70S6K, S6, and 4EBP1, are increased between 50 and 72% in both subtypes of rhabdomyosarcoma.
  • Further, phosphorylation levels of PKCalpha, PKCdelta, PKCtheta, and PKCzeta/lambda are upregulated between 57 and 69% in alveolar rhabdomyosarcoma and between 43 and 72% in embryonal rhabdomyosarcoma.
  • This is the first report to create a phosphorylation profile of tyrosine and serine/threonine kinases involved in the mTOR and PKC pathways of alveolar and embryonal rhabdomyosarcoma.
  • [MeSH-major] Protein-Serine-Threonine Kinases / analysis. Protein-Tyrosine Kinases / analysis. Rhabdomyosarcoma, Alveolar / enzymology. Rhabdomyosarcoma, Embryonal / enzymology

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  • (PMID = 17585318.001).
  • [ISSN] 0893-3952
  • [Journal-full-title] Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
  • [ISO-abbreviation] Mod. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Adaptor Proteins, Signal Transducing; 0 / EIF4EBP1 protein, human; 0 / Isoenzymes; 0 / Phosphoproteins; 0 / Ribosomal Protein S6; EC 2.7.- / Protein Kinases; EC 2.7.1.1 / MTOR protein, human; EC 2.7.1.1 / TOR Serine-Threonine Kinases; EC 2.7.10.1 / Protein-Tyrosine Kinases; EC 2.7.10.1 / Receptor Protein-Tyrosine Kinases; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 2.7.11.1 / Ribosomal Protein S6 Kinases, 70-kDa; EC 2.7.11.13 / Protein Kinase C
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3. Keller C, Capecchi MR: New genetic tactics to model alveolar rhabdomyosarcoma in the mouse. Cancer Res; 2005 Sep 1;65(17):7530-2
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  • [Title] New genetic tactics to model alveolar rhabdomyosarcoma in the mouse.
  • Using conditional knock-in and knock-out techniques, we designed a mouse model of the childhood muscle cancer alveolar rhabdomyosarcoma (ARMS) that is driven by the chromosomal translocation product, Pax3:Fkhr.
  • This model offers new insight into the roots of alveolar rhabdomyosarcoma and illustrates the utility of Cre-loxP technology for studying otherwise inaccessible cancers in the mouse.
  • [MeSH-major] DNA-Binding Proteins / genetics. Disease Models, Animal. Oncogene Proteins, Fusion / genetics. Rhabdomyosarcoma, Alveolar / genetics. Transcription Factors / genetics

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  • (PMID = 16140913.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / Forkhead Transcription Factors; 0 / Foxo1 protein, mouse; 0 / Oncogene Proteins, Fusion; 0 / Paired Box Transcription Factors; 0 / Transcription Factors; 138016-91-8 / Pax3 protein, mouse
  • [Number-of-references] 8
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4. Masola V, Maran C, Tassone E, Zin A, Rosolen A, Onisto M: Heparanase activity in alveolar and embryonal rhabdomyosarcoma: implications for tumor invasion. BMC Cancer; 2009;9:304
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  • [Title] Heparanase activity in alveolar and embryonal rhabdomyosarcoma: implications for tumor invasion.
  • BACKGROUND: Rhabdomyosarcoma (RMS) is a malignant soft tissue sarcoma of childhood including two major histological subtypes, alveolar (ARMS) and embryonal (ERMS) RMS.
  • [MeSH-major] Glucuronidase / metabolism. Neoplasm Invasiveness. Rhabdomyosarcoma, Alveolar / embryology. Rhabdomyosarcoma, Alveolar / enzymology

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  • (PMID = 19715595.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] EC 3.2.1.- / heparanase; EC 3.2.1.31 / Glucuronidase
  • [Other-IDs] NLM/ PMC2743710
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5. Jones AE, Albano EA, Lovell MA, Hunger SP: Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A. Pediatr Blood Cancer; 2010 Dec 1;55(6):1213-6
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  • [Title] Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A.
  • Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma, accounts for 3% of childhood malignancies.
  • We describe a previously unreported association of MEN-2A with metastatic alveolar RMS and review the literature on associated hereditary cancer predisposition syndromes and current therapeutic options.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 2a / pathology. Rhabdomyosarcoma, Alveolar / secondary

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  • (PMID = 20533522.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / T32 CA082086
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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6. Petricoin EF 3rd, Espina V, Araujo RP, Midura B, Yeung C, Wan X, Eichler GS, Johann DJ Jr, Qualman S, Tsokos M, Krishnan K, Helman LJ, Liotta LA: Phosphoprotein pathway mapping: Akt/mammalian target of rapamycin activation is negatively associated with childhood rhabdomyosarcoma survival. Cancer Res; 2007 Apr 1;67(7):3431-40
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  • [Title] Phosphoprotein pathway mapping: Akt/mammalian target of rapamycin activation is negatively associated with childhood rhabdomyosarcoma survival.
  • Despite advances in combination chemotherapy, the overall survival for childhood rhabdomyosarcoma remains approximately 60%.
  • Specimens (n = 59) were obtained from the Children's Oncology Group Intergroup Rhabdomyosarcoma Study (IRS) IV, D9502 and D9803, with 12-year follow-up.
  • CCI-779 suppressed phosphorylation of mTOR downstream proteins and greatly reduced the growth of two different rhabdomyosarcoma (RD embryonal P = 0.00008; Rh30 alveolar P = 0.0002) cell lines compared with controls.
  • [MeSH-major] Protein Kinases / metabolism. Proto-Oncogene Proteins c-akt / metabolism. Rhabdomyosarcoma / metabolism


7. Brown BJ, Oluwasola AO: Childhood rhabdomyosarcoma in Ibadan, Nigeria: 1984-2003. Ann Trop Paediatr; 2006 Dec;26(4):349-55
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  • [Title] Childhood rhabdomyosarcoma in Ibadan, Nigeria: 1984-2003.
  • BACKGROUND: Rhabdomyosarcoma is the most common soft tissue sarcoma in children under 15 years of age.
  • AIM: To describe the pattern of childhood rhabdomyosarcoma in Ibadan with respect to demography, morphology and tumour site.
  • All histologically confirmed cases of rhabdomyosarcoma in children under 15 years of age seen at the University College Hospital Ibadan between 1984 and 2003 were included.
  • Other subtypes were alveolar (13.2%), pleomorphic (4.4%) and rhabdomyosarcoma 'not otherwise specified' (20.9%).
  • CONCLUSION: The pattern of rhabdomyosarcoma in Nigeria is similar to that in the United States and Europe, except for the rarity of parameningeal sites and extremities.
  • There is a need for larger descriptive studies on childhood rhabdomyosarcoma in Africa.
  • [MeSH-major] Rhabdomyosarcoma / epidemiology. Soft Tissue Neoplasms / epidemiology
  • [MeSH-minor] Adolescent. Age Distribution. Child. Child, Preschool. Female. Head and Neck Neoplasms / epidemiology. Humans. Infant. Infant, Newborn. Male. Nigeria / epidemiology. Registries. Retrospective Studies. Rhabdomyosarcoma, Alveolar / epidemiology. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Embryonal / epidemiology. Rhabdomyosarcoma, Embryonal / pathology

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  • (PMID = 17132301.001).
  • [ISSN] 0272-4936
  • [Journal-full-title] Annals of tropical paediatrics
  • [ISO-abbreviation] Ann Trop Paediatr
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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8. Taniguchi E, Nishijo K, McCleish AT, Michalek JE, Grayson MH, Infante AJ, Abboud HE, Legallo RD, Qualman SJ, Rubin BP, Keller C: PDGFR-A is a therapeutic target in alveolar rhabdomyosarcoma. Oncogene; 2008 Nov 20;27(51):6550-60
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  • [Title] PDGFR-A is a therapeutic target in alveolar rhabdomyosarcoma.
  • Alveolar rhabdomyosarcoma is an aggressive skeletal muscle cancer of childhood.
  • Our initial studies of rhabdomyosarcoma gene expression for patients enrolled in a national clinical trial suggested that platelet-derived growth factor receptor A (PDGFR-A) may be a mediator of disease progression and metastasis.
  • Using our conditional mouse tumor models that authentically recapitulate the primary mutations and metastatic progression of alveolar rhabdomyosarcomas in humans, we found by immunoblotting and immunokinase assays that PDGFR-A and its downstream effectors, mitogen-activated protein kinase and Akt, were highly activated in both primary and metastatic tumors.
  • These results establish proof-of-principal for PDGFR-A as a therapeutic target in alveolar rhabdomyosarcoma.

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  • (PMID = 18679424.001).
  • [ISSN] 1476-5594
  • [Journal-full-title] Oncogene
  • [ISO-abbreviation] Oncogene
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P30CA54174; United States / NCI NIH HHS / CA / R01 CA133229; United States / NCI NIH HHS / CA / CA133229-01; United States / NCI NIH HHS / CA / P30 CA054174; United States / NCI NIH HHS / CA / R01 CA133229-01; United States / NCI NIH HHS / CA / R01CA133229
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Benzamides; 0 / Piperazines; 0 / Protein Kinase Inhibitors; 0 / Pyrimidines; 8A1O1M485B / Imatinib Mesylate; EC 2.7.10.1 / Receptor, Platelet-Derived Growth Factor alpha
  • [Other-IDs] NLM/ NIHMS161083; NLM/ PMC2813858
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9. Ben Arush MW, Bar Shalom R, Postovsky S, Militianu D, Haimi M, Zaidman I, Israel O: Assessing the use of FDG-PET in the detection of regional and metastatic nodes in alveolar rhabdomyosarcoma of extremities. J Pediatr Hematol Oncol; 2006 Jul;28(7):440-5
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  • [Title] Assessing the use of FDG-PET in the detection of regional and metastatic nodes in alveolar rhabdomyosarcoma of extremities.
  • Alveolar rhabdomyosarcoma (ARS) accounts for 20% to 30% of childhood rhabdomyosarcoma and is known to have a worse prognosis than embryonal rhabdomyosarcoma.
  • Metastatic disease is more frequent in patients with alveolar tumors and these children with metastatic disease fare poorly, with a 5-year survival between 20% and 30%.
  • [MeSH-major] Extremities / pathology. Fluorodeoxyglucose F18. Lymphoma, Non-Hodgkin / diagnosis. Positron-Emission Tomography / methods. Rhabdomyosarcoma, Alveolar / diagnosis. Soft Tissue Neoplasms / diagnosis

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  • (PMID = 16825990.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0Z5B2CJX4D / Fluorodeoxyglucose F18
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10. Rudnik-Schöneborn S, Anhuf D, Koscielniak E, Zerres K: Alveolar rhabdomyosarcoma in infantile spinal muscular atrophy: coincidence or predisposition? Neuromuscul Disord; 2005 Jan;15(1):45-7
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  • [Title] Alveolar rhabdomyosarcoma in infantile spinal muscular atrophy: coincidence or predisposition?
  • We report two unrelated patients with infantile spinal muscular atrophy (SMA) types II and IIIa who developed alveolar rhabdomyosarcoma (ARMS) at 15 and 19 years, respectively.
  • [MeSH-major] Disease Susceptibility. Rhabdomyosarcoma, Alveolar / etiology. Spinal Muscular Atrophies of Childhood / complications

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  • (PMID = 15639120.001).
  • [ISSN] 0960-8966
  • [Journal-full-title] Neuromuscular disorders : NMD
  • [ISO-abbreviation] Neuromuscul. Disord.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors; 0 / Transcription Factors
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11. Huh WW, Skapek SX: Childhood rhabdomyosarcoma: new insight on biology and treatment. Curr Oncol Rep; 2010 Nov;12(6):402-10
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  • [Title] Childhood rhabdomyosarcoma: new insight on biology and treatment.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood.
  • The two most common histologic variants are the embryonal and alveolar subtypes.
  • Recent studies have pointed to a possible mesenchymal stem cell as the progenitor for alveolar RMS.
  • [MeSH-major] Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / therapy. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / genetics. Rhabdomyosarcoma, Embryonal / therapy. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / genetics. Soft Tissue Neoplasms / therapy

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  • (PMID = 20820958.001).
  • [ISSN] 1534-6269
  • [Journal-full-title] Current oncology reports
  • [ISO-abbreviation] Curr Oncol Rep
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protein Kinase Inhibitors; EC 2.7.1.1 / TOR Serine-Threonine Kinases; W36ZG6FT64 / Sirolimus
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12. Ognjanovic S, Carozza SE, Chow EJ, Fox EE, Horel S, McLaughlin CC, Mueller BA, Puumala S, Reynolds P, Von Behren J, Spector L: Birth characteristics and the risk of childhood rhabdomyosarcoma based on histological subtype. Br J Cancer; 2010 Jan 5;102(1):227-31
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  • [Title] Birth characteristics and the risk of childhood rhabdomyosarcoma based on histological subtype.
  • BACKGROUND: Little is known about risk factors for childhood rhabdomyosarcoma (RMS) and the histology-specific details are rare.
  • METHODS: Case-control studies formed by linking cancer and birth registries of California, Minnesota, New York, Texas and Washington, which included 583 RMS cases (363 embryonal and 85 alveolar RMS) and 57 966 randomly selected control subjects, were analysed using logistic regression.
  • The associations of RMS (overall, and based on embryonal or alveolar histology) with birth weight across five 500 g categories (from 2000 to 4500 g) were examined using normal birth weight (2500-3999 g) as a reference.
  • CONCLUSIONS: These data suggest a positive association between accelerated in utero growth and embryonal RMS, but not alveolar RMS.
  • These results warrant cautious interpretation owing to the small number of alveolar RMS cases.
  • [MeSH-major] Rhabdomyosarcoma / epidemiology. Soft Tissue Neoplasms / epidemiology
  • [MeSH-minor] Adolescent. Adult. Age of Onset. Birth Order. Birth Weight. Child. Child, Preschool. Diseases in Twins / epidemiology. Embryonic Development. Female. Gestational Age. Humans. Infant. Infant, Newborn. Male. Maternal Age. Paternal Age. Rhabdomyosarcoma, Alveolar / embryology. Rhabdomyosarcoma, Alveolar / epidemiology. Rhabdomyosarcoma, Embryonal / embryology. Rhabdomyosarcoma, Embryonal / epidemiology. Risk Factors. Young Adult

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  • (PMID = 19997102.001).
  • [ISSN] 1532-1827
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2813761
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13. Ragsdale BD, Lee JP, Mines J: Alveolar rhabdomyosarcoma on the external ear: a case report. J Cutan Pathol; 2009 Feb;36(2):267-9
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  • [Title] Alveolar rhabdomyosarcoma on the external ear: a case report.
  • Alveolar rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood.
  • In fact, 'middle ear' is what is meant by 'ear' location in the many reports of pediatric RMS.
  • [MeSH-major] Ear Neoplasms / pathology. Rhabdomyosarcoma, Alveolar / pathology. Skin Neoplasms / pathology

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  • (PMID = 19208077.001).
  • [ISSN] 1600-0560
  • [Journal-full-title] Journal of cutaneous pathology
  • [ISO-abbreviation] J. Cutan. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Denmark
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14. Martinelli S, McDowell HP, Vigne SD, Kokai G, Uccini S, Tartaglia M, Dominici C: RAS signaling dysregulation in human embryonal Rhabdomyosarcoma. Genes Chromosomes Cancer; 2009 Nov;48(11):975-82
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  • [Title] RAS signaling dysregulation in human embryonal Rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS) is a common childhood solid tumor, resulting from dysregulation of the skeletal myogenesis program.
  • Two major histological subtypes occur in childhood RMS, embryonal and alveolar.
  • While chromosomal rearrangements account for the majority of alveolar tumors, the genetic defects underlying the pathogenesis of embryonal RMS remain largely undetermined.
  • [MeSH-major] Proto-Oncogene Proteins / genetics. Rhabdomyosarcoma, Embryonal / genetics. ras Proteins / genetics

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  • [Copyright] Copyright 2009 Wiley-Liss, Inc.
  • (PMID = 19681119.001).
  • [ISSN] 1098-2264
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Grant] Italy / Telethon / / TI/ GGP07115
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Proto-Oncogene Proteins; EC 2.7.1.- / MAP2K2 protein, human; EC 2.7.11.1 / BRAF protein, human; EC 2.7.11.1 / Proto-Oncogene Proteins B-raf; EC 2.7.12.2 / MAP Kinase Kinase 1; EC 2.7.12.2 / MAP Kinase Kinase 2; EC 2.7.12.2 / MAP2K1 protein, human; EC 3.1.3.48 / PTPN1 protein, human; EC 3.1.3.48 / Protein Tyrosine Phosphatase, Non-Receptor Type 1; EC 3.6.5.2 / ras Proteins
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15. Kazanowska B, Reich A, Stegmaier S, Békássy AN, Leuschner I, Chybicka A, Koscielniak E: Pax3-fkhr and pax7-fkhr fusion genes impact outcome of alveolar rhabdomyosarcoma in children. Fetal Pediatr Pathol; 2007 Jan-Feb;26(1):17-31
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  • [Title] Pax3-fkhr and pax7-fkhr fusion genes impact outcome of alveolar rhabdomyosarcoma in children.
  • Rhabdomyosarcoma is a highly malignant embryonic tumor of childhood.
  • [MeSH-major] Forkhead Transcription Factors / genetics. PAX7 Transcription Factor / genetics. Paired Box Transcription Factors / genetics. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics

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  • (PMID = 17613043.001).
  • [ISSN] 1551-3815
  • [Journal-full-title] Fetal and pediatric pathology
  • [ISO-abbreviation] Fetal Pediatr Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / PAX3 protein, human; 0 / PAX7 Transcription Factor; 0 / PAX7 protein, human; 0 / Paired Box Transcription Factors
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16. Nishijo K, Chen QR, Zhang L, McCleish AT, Rodriguez A, Cho MJ, Prajapati SI, Gelfond JA, Chisholm GB, Michalek JE, Aronow BJ, Barr FG, Randall RL, Ladanyi M, Qualman SJ, Rubin BP, LeGallo RD, Wang C, Khan J, Keller C: Credentialing a preclinical mouse model of alveolar rhabdomyosarcoma. Cancer Res; 2009 Apr 1;69(7):2902-11
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  • [Title] Credentialing a preclinical mouse model of alveolar rhabdomyosarcoma.
  • The highly aggressive muscle cancer alveolar rhabdomyosarcoma (ARMS) is one of the most common soft tissue sarcoma of childhood, yet the outcome for the unresectable and metastatic disease is dismal and unchanged for nearly three decades.
  • At a whole-genome level, a cross-species gene set enrichment analysis and metagene projection study showed that our mouse model is most similar to human ARMS when compared with other pediatric cancers.

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  • (PMID = 19339268.001).
  • [ISSN] 1538-7445
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / R01 CA074907-06; United States / NCI NIH HHS / CA / CA090438-06; United States / NCI NIH HHS / CA / CA064202-07; United States / NCI NIH HHS / CA / CA064202-13; United States / NCI NIH HHS / CA / R01 CA064202-08S1; United States / NCI NIH HHS / CA / P30CA54174; United States / NCI NIH HHS / CA / R01 CA064202-11; United States / NCI NIH HHS / CA / CA064202-08S1; United States / NCI NIH HHS / CA / R01 CA074907-09; United States / NCI NIH HHS / CA / K08 CA090438; United States / NCI NIH HHS / CA / CA074907-11; United States / NCI NIH HHS / CA / CA64202; United States / NCI NIH HHS / CA / R01 CA064202-06; United States / NCI NIH HHS / CA / R01 CA074907; United States / NCI NIH HHS / CA / CA064202-08; United States / NCI NIH HHS / CA / CA074907-07; United States / NCI NIH HHS / CA / R01 CA064202-14; United States / NCI NIH HHS / CA / R01 CA064202-13; United States / NCI NIH HHS / CA / R01 CA064202-07; United States / NCI NIH HHS / CA / R29 CA074907; United States / NCI NIH HHS / CA / CA074907-08; United States / NCI NIH HHS / CA / R01 CA064202-09; United States / NCI NIH HHS / CA / R01 CA064202; United States / NCI NIH HHS / CA / CA074907; United States / NCI NIH HHS / CA / CA074907-10A1; United States / NCI NIH HHS / CA / CA064202-11; United States / NCI NIH HHS / CA / CA074907-09; United States / NCI NIH HHS / CA / CA074907-06; United States / NCI NIH HHS / CA / R01 CA064202-08; United States / NCI NIH HHS / CA / R01 CA074907-11; United States / NCI NIH HHS / CA / CA064202-14; United States / NCI NIH HHS / CA / CA064202-10A1; United States / NCI NIH HHS / CA / R01 CA074907-07; United States / NCI NIH HHS / CA / P30 CA054174; United States / NCI NIH HHS / CA / CA064202-12; United States / NCI NIH HHS / CA / R01 CA074907-08; United States / NCI NIH HHS / CA / CA064202-06; United States / NCI NIH HHS / CA / R01 CA064202-12; United States / NCI NIH HHS / CA / K08 CA090438-06; United States / NCI NIH HHS / CA / R01 CA064202-10A1; United States / NCI NIH HHS / CA / CA064202-09; United States / NCI NIH HHS / CA / R01 CA074907-10A1
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cdkn2a protein, mouse; 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / Forkhead Transcription Factors; 0 / Foxo1 protein, mouse; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human; 0 / Paired Box Transcription Factors; 0 / Tumor Suppressor Protein p53; 138016-91-8 / Pax3 protein, mouse
  • [Other-IDs] NLM/ NIHMS96027; NLM/ PMC2789740
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17. De Pasquale MD, De Ioris MA, Serra A, Pessolano R, Donfrancesco A, De Sio L: Pancreatitis as an unusual presentation of rhabdomyosarcoma. Pediatr Blood Cancer; 2009 Jul;52(7):879-80
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  • [Title] Pancreatitis as an unusual presentation of rhabdomyosarcoma.
  • Acute pancreatitis is rarely associated with underlying childhood malignancies.
  • We report a 12-year-old male with acute pancreatitis as the presenting symptom of an alveolar metastatic rhabdomyosarcoma.
  • [MeSH-major] Pancreatitis / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis

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  • [Copyright] (c) 2009 Wiley-Liss, Inc.
  • (PMID = 19213073.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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18. Douglas JG, Arndt CA, Hawkins DS: Delayed radiotherapy following dose intensive chemotherapy for parameningeal rhabdomyosarcoma (PM-RMS) of childhood. Eur J Cancer; 2007 Apr;43(6):1045-50
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Delayed radiotherapy following dose intensive chemotherapy for parameningeal rhabdomyosarcoma (PM-RMS) of childhood.
  • PURPOSE: 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.
  • Twenty-three patients had embryonal histology with the remaining three alveolar.
  • [MeSH-major] Meningeal Neoplasms / drug therapy. Meningeal Neoplasms / radiotherapy. Rhabdomyosarcoma, Alveolar / drug therapy. Rhabdomyosarcoma, Alveolar / radiotherapy. Rhabdomyosarcoma, Embryonal / drug therapy. Rhabdomyosarcoma, Embryonal / radiotherapy

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  • (PMID = 17368885.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] England
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19. Punyko JA, Mertens AC, Baker KS, Ness KK, Robison LL, Gurney JG: Long-term survival probabilities for childhood rhabdomyosarcoma. A population-based evaluation. Cancer; 2005 Apr 1;103(7):1475-83
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Long-term survival probabilities for childhood rhabdomyosarcoma. A population-based evaluation.
  • BACKGROUND: Evidence from clinical trials has documented improvements in event-free survival from childhood rhabdomyosarcoma (RMS) since the 1970s; however, the survival experience of children enrolled on cancer clinical trials may not reflect the full range of patients treated in community settings.
  • Poor prognosis was associated with diagnosis during infancy (47%) and adolescence (48%); metastatic disease at the time of presentation (31%); alveolar histology (49%); and tumors of the extremities (50%), retroperitoneum (52%), and trunk (52%).
  • [MeSH-major] Rhabdomyosarcoma / mortality

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  • [Copyright] Copyright 2005 American Cancer Society.
  • (PMID = 15712283.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / U24 CA55727
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
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20. Ognjanovic S, Linabery AM, Charbonneau B, Ross JA: Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975-2005. Cancer; 2009 Sep 15;115(18):4218-26
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  • [Title] Trends in childhood rhabdomyosarcoma incidence and survival in the United States, 1975-2005.
  • BACKGROUND: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents aged<20 years; its etiology remains largely unknown.
  • It is believed that embryonal (ERMS) and alveolar rhabdomyosarcoma (ARMS), the most common subtypes, arise through distinct biologic mechanisms.

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  • [Copyright] Copyright (c) 2009 American Cancer Society.
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  • (PMID = 19536876.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / T32 CA099936-07; United States / NCI NIH HHS / CA / T32 CA099936-06S1; United States / NCI NIH HHS / CA / CA099936-07; United States / NCI NIH HHS / CA / T32 CA099936; United States / NCI NIH HHS / CA / CA099936-06S1
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS218300; NLM/ PMC2953716
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21. Taulli R, Scuoppo C, Bersani F, Accornero P, Forni PE, Miretti S, Grinza A, Allegra P, Schmitt-Ney M, Crepaldi T, Ponzetto C: Validation of met as a therapeutic target in alveolar and embryonal rhabdomyosarcoma. Cancer Res; 2006 May 1;66(9):4742-9
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  • [Title] Validation of met as a therapeutic target in alveolar and embryonal rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS) is a highly malignant soft-tissue tumor of childhood deriving from skeletal muscle cells.
  • RMS can be classified in two major histologic subtypes: embryonal (ERMS) and alveolar (ARMS), the latter being characterized by the PAX3/7-FKHR translocation.
  • [MeSH-major] Proto-Oncogene Proteins / antagonists & inhibitors. Proto-Oncogene Proteins / physiology. Receptors, Growth Factor / antagonists & inhibitors. Receptors, Growth Factor / physiology. Rhabdomyosarcoma, Alveolar / therapy. Rhabdomyosarcoma, Embryonal / therapy

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  • (PMID = 16651427.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors; 0 / Proto-Oncogene Proteins; 0 / RNA, Small Interfering; 0 / Receptors, Growth Factor; 67256-21-7 / Hepatocyte Growth Factor; EC 2.7.10.1 / MET protein, human; EC 2.7.10.1 / Proto-Oncogene Proteins c-met
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22. Herrmann D, Seitz G, Warmann SW, Bonin M, Fuchs J, Armeanu-Ebinger S: Cetuximab promotes immunotoxicity against rhabdomyosarcoma in vitro. J Immunother; 2010 Apr;33(3):279-86
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  • [Title] Cetuximab promotes immunotoxicity against rhabdomyosarcoma in vitro.
  • Multidrug resistance is a common problem in the treatment of childhood rhabdomyosarcoma (RMS).
  • Expression of EGFR and binding of its specific antibody Cetuximab to embryonal RMS cell lines RD and A-204 and alveolar RMS Rh30 were monitored by flow cytometry.
  • Gene expression analysis revealed a high expression of EGFR in all embryonal RMS compared with alveolar RMS.
  • [MeSH-major] Antibodies, Monoclonal / pharmacology. Antibody-Dependent Cell Cytotoxicity / drug effects. Rhabdomyosarcoma, Alveolar / immunology. Rhabdomyosarcoma, Embryonal / immunology

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  • (PMID = 20445348.001).
  • [ISSN] 1537-4513
  • [Journal-full-title] Journal of immunotherapy (Hagerstown, Md. : 1997)
  • [ISO-abbreviation] J. Immunother.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antineoplastic Agents; EC 2.7.10.1 / Receptor, Epidermal Growth Factor; PQX0D8J21J / Cetuximab
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23. Seitz G, Bonin M, Fuchs J, Poths S, Ruck P, Warmann SW, Armeanu-Ebinger S: Inhibition of glutathione-S-transferase as a treatment strategy for multidrug resistance in childhood rhabdomyosarcoma. Int J Oncol; 2010 Feb;36(2):491-500
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Inhibition of glutathione-S-transferase as a treatment strategy for multidrug resistance in childhood rhabdomyosarcoma.
  • Multidrug resistance (MDR) is a common problem in the treatment of childhood rhabdomyosarcoma (RMS).
  • The aim of this study was to investigate the role of glutathione-S-transferase (GST) as mechanism of MDR in childhood RMS and to analyze possible reversal strategies.
  • Female athymic mice underwent xenotransplantation with embryonal or alveolar RMS cells and were treated with vincristine.
  • Gene expression analysis using Affymetrix HU-Gene 1.0 arrays revealed 2314 differentially expressed genes between the groups in alveolar RMS and 1387 in embryonal RMS.
  • In order to analyze possible GST activity after chemotherapy with other commonly used drugs (doxorubicin, topotecan), cell culture experiments with alveolar and embryonal RMS cells were carried out.
  • We detected a novel mechanism for MDR in childhood RMS mediated via genes and proteins of the GST family.
  • The GST family represents a promising target for further treatment strategies in childhood RMS.
  • [MeSH-major] Drug Resistance, Multiple / genetics. Drug Resistance, Neoplasm / genetics. Glutathione Transferase / antagonists & inhibitors. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Embryonal / genetics

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  • (PMID = 20043085.001).
  • [ISSN] 1791-2423
  • [Journal-full-title] International journal of oncology
  • [ISO-abbreviation] Int. J. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Enzyme Inhibitors; 5J49Q6B70F / Vincristine; 7M7YKX2N15 / Topotecan; 80168379AG / Doxorubicin; EC 2.5.1.18 / Glutathione Transferase
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24. Seitz G, Warmann SW, Vokuhl CO, Heitmann H, Treuner C, Leuschner I, Fuchs J: Effects of standard chemotherapy on tumor growth and regulation of multidrug resistance genes and proteins in childhood rhabdomyosarcoma. Pediatr Surg Int; 2007 May;23(5):431-9
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  • [Title] Effects of standard chemotherapy on tumor growth and regulation of multidrug resistance genes and proteins in childhood rhabdomyosarcoma.
  • The prognosis of rhabdomyosarcoma (RMS) in advanced stages is still sobering.
  • The aim of this study was to investigate the development of multidrug resistance in cell lines and in xenografts of alveolar and embryonal RMS treated according to the German Soft Tissue Sarcoma Study (CWS).
  • Alveolar and embryonal RMS cell lines were treated with Vincristine, Topotecan, Carboplatin, Actinomycin D, or Ifosfamide.
  • Nude mice (NMRI nu/nu, n = 10 per group) underwent xenotransplantation of human embryonal or alveolar RMS.
  • 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.
  • Standard chemotherapy of alveolar rhabdomyosarcoma resulted in a significant reduction of tumor growth (P < 0.05) in all groups.
  • In embryonal rhabdomyosarcoma strongest effects were found after treatment with Ifosfamide, Vincristine and Carboplatin (P < 0.05).
  • RT-PCR revealed a MDR1-dependent mechanism in alveolar rhabdomyosarcoma.
  • In embryonal rhabdomyosarcoma, MDR1 occurred to a lower degree.
  • [MeSH-major] Drug Resistance, Neoplasm / drug effects. Gene Expression Regulation, Neoplastic / drug effects. Genes, MDR / genetics. Multidrug Resistance-Associated Proteins / drug effects. Rhabdomyosarcoma, Alveolar / drug therapy. Rhabdomyosarcoma, Embryonal / drug therapy. Soft Tissue Neoplasms / drug therapy

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  • (PMID = 17211591.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Multidrug Resistance-Associated Proteins; 0 / Neoplasm Proteins
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25. Linardic CM: PAX3-FOXO1 fusion gene in rhabdomyosarcoma. Cancer Lett; 2008 Oct 18;270(1):10-8
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  • [Title] PAX3-FOXO1 fusion gene in rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and adolescence.
  • The predominant histologic variants of this disease are termed embryonal (eRMS) and alveolar (aRMS), based on their appearance under light microscopy.

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  • (PMID = 18457914.001).
  • [ISSN] 1872-7980
  • [Journal-full-title] Cancer letters
  • [ISO-abbreviation] Cancer Lett.
  • [Language] ENG
  • [Grant] United States / NICHD NIH HHS / HD / HD043494-05; United States / NICHD NIH HHS / HD / K12 HD043494; United States / NICHD NIH HHS / HD / 5K12-HD043494; United States / NICHD NIH HHS / HD / K12 HD043494-05
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Ireland
  • [Chemical-registry-number] 0 / CXCL12 protein, human; 0 / Chemokine CXCL12; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FOXO1A fusion protein, human; 0 / Paired Box Transcription Factors; EC 2.7.10.1 / FLT1 protein, human; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-1
  • [Number-of-references] 59
  • [Other-IDs] NLM/ NIHMS72264; NLM/ PMC2575376
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26. Jha P, Frölich AM, McCarville B, Navarro OM, Babyn P, Goldsby R, Daldrup-Link H: Unusual association of alveolar rhabdomyosarcoma with pancreatic metastasis: emerging role of PET-CT in tumor staging. Pediatr Radiol; 2010 Aug;40(8):1380-6
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  • [Title] Unusual association of alveolar rhabdomyosarcoma with pancreatic metastasis: emerging role of PET-CT in tumor staging.
  • BACKGROUND: Pancreatic metastases in childhood cancer have been rarely reported in the radiology literature although ample evidence exists in pathology reports for its occurrence in patients with alveolar rhabdomyosarcomas (RMS).
  • OBJECTIVE: Assess the occurrence of pancreatic metastases in alveolar rhabdomyosarcomas, increase awareness of this association and reassess current staging protocols.
  • RESULTS: Pancreatic metastases occurred in eight patients with alveolar RMS.
  • Pancreatic metastases were not associated with certain primary tumor locations or presence of other metastases, mandating an evaluation of the pancreas in all cases of alveolar rhabdomyosarcomas.
  • CONCLUSION: Radiologists should be sensitized and actively evaluate the pancreas in patients with alveolar RMS.
  • [MeSH-major] Pancreatic Neoplasms / complications. Pancreatic Neoplasms / radiography. Positron-Emission Tomography. Rhabdomyosarcoma, Alveolar / complications. Rhabdomyosarcoma, Alveolar / radiography

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  • (PMID = 20180103.001).
  • [ISSN] 1432-1998
  • [Journal-full-title] Pediatric radiology
  • [ISO-abbreviation] Pediatr Radiol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Other-IDs] NLM/ PMC2895865
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27. Ohta H, Hashii Y, Yoneda A, Takizawa S, Kusuki S, Tokimasa S, Fukuzawa M, Tsuboi A, Murao A, Oka Y, Oji Y, Aozasa K, Nakatsuka S, Sugiyama H, Ozono K: WT1 (Wilms tumor 1) peptide immunotherapy for childhood rhabdomyosarcoma: a case report. Pediatr Hematol Oncol; 2009 Jan;26(1):74-83
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] WT1 (Wilms tumor 1) peptide immunotherapy for childhood rhabdomyosarcoma: a case report.
  • In this study, the authors used WT1 peptide vaccination to treat a 6-year-old girl with metastatic alveolar rhabdomyosarcoma.
  • WT1 peptide-based immunotherapy should be a promising option for high-risk rhabdomyosarcoma in childhood.
  • [MeSH-major] Immunotherapy / methods. Peptide Fragments / therapeutic use. Rhabdomyosarcoma, Alveolar / drug therapy. WT1 Proteins / therapeutic use

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  • (PMID = 19206012.001).
  • [ISSN] 1521-0669
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Peptide Fragments; 0 / WT1 Proteins
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28. Uba FA, Chirdan LB: Clinical characteristics and outcome of surgical treatment of childhood rhabdomyosarcoma: a 7-year experience. Afr J Paediatr Surg; 2008 Jan-Jun;5(1):19-23

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical characteristics and outcome of surgical treatment of childhood rhabdomyosarcoma: a 7-year experience.
  • BACKGROUND: The aim of this study was to describe the outcome and determine the prognostic factors of outcome of childhood rhabdomyosarcoma in a tertiary hospital in a developing country.
  • PATIENTS AND METHODS: This was a retrospective review of the clinical presentation, investigation, intervention, and treatment outcomes of children with rhabdomyosarcoma in our hospital over a 7-year period.
  • Two patients were Intergroup Rhabdomyosarcoma Study (IRS) group I, four group II, five group III, and seven group IV.
  • There were 12 patients with alveolar disease while six had embryonal type of rhabdomyosarcoma.
  • CONCLUSION: Mortality from rhabdomyosarcoma in our setting is still unacceptably high.

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  • (PMID = 19858658.001).
  • [ISSN] 0974-5998
  • [Journal-full-title] African journal of paediatric surgery : AJPS
  • [ISO-abbreviation] Afr J Paediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Nigeria
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29. Qualman S, Lynch J, Bridge J, Parham D, Teot L, Meyer W, Pappo A: Prevalence and clinical impact of anaplasia in childhood rhabdomyosarcoma : a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group. Cancer; 2008 Dec 1;113(11):3242-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Prevalence and clinical impact of anaplasia in childhood rhabdomyosarcoma : a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.
  • BACKGROUND: Anapalsia is rare in childhood rhabdomyosarcoma and has not been included in the International Classification of Rhabdomyosarcoma (ICR).
  • METHODS: The prevalence of anaplasia (focal or diffuse) was prospectively assessed in 546 eligible cases who were registered in an Intergroup Rhabdomyosarcoma Study Group (IRSG) or COG therapeutic trial from 1995 through 1998.
  • Regardless of its distribution (focal or diffuse), on univariate analysis the presence of anaplasia negatively influenced the failure-free survival rate (63% vs 77% at 5 years) and overall survival (68% vs 82% at 5 years) rates in patients with embryonal rhabdomyosarcoma.
  • Anaplasia did not affect outcome in patients with alveolar tumors.
  • CONCLUSIONS: The incidence of anaplasia in patients with rhabdomyosarcoma is higher than previously described and may be of prognostic significance in children with intermediate-risk embryonal rhabdomyosarcoma.

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  • [Copyright] (c) 2008 American Cancer Society
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  • (PMID = 18985676.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U10 CA098543-06; United States / NCI NIH HHS / CA / U10 CA098413-06; United States / NCI NIH HHS / CA / U10 CA098413; None / None / / U10 CA024507-25; United States / NCI NIH HHS / CA / U10 CA024507-26; United States / NCI NIH HHS / CA / U10 CA098543; None / None / / U10 CA098413-06; None / None / / U10 CA024507-26; United States / NCI NIH HHS / CA / U10 CA024507-25; None / None / / U10 CA098543-06; United States / NCI NIH HHS / CA / U10 CA024507
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS107192; NLM/ PMC2727712
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30. Seitz G, Warmann SW, Fuchs J, Heitmann H, Mahrt J, Busse AC, Ruck P, Hoffman RM, Wessels JT: Imaging of cell trafficking and metastases of paediatric rhabdomyosarcoma. Cell Prolif; 2008 Apr;41(2):365-74

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Imaging of cell trafficking and metastases of paediatric rhabdomyosarcoma.
  • OBJECTIVE: The aim of this study was to establish a preclinical mouse model to study metastases of paediatric rhabdomyosarcoma at the macroscopic and cellular levels, with different imaging methods.
  • EXPERIMENTAL DESIGN: The alveolar rhabdomyosarcoma cell line Rh30 was stably transfected with the red fluorescent protein (DsRed2) then was xenotransplanted (intravenous injection [n = 8], and footpad injection [n = 8]) into nude mice (NMRI nu/nu).
  • In a further series of animals (n = 8), in vivo cell trafficking of rhabdomyosarcoma cells using cellular imaging with an Olympus OV100 variable-magnification small-animal imaging system was used.
  • CONCLUSION: We established a model for visualization of experimental metastatic invasion and describe relevant tools for imaging childhood rhabdomyosarcoma metastases at the macroscopic and cellular levels.
  • Imaging of cell trafficking visualized the behaviour of tumour cells and development of metastases by accumulation and extravasation of rhabdomyosarcoma cells.
  • [MeSH-major] Image Interpretation, Computer-Assisted / methods. Imaging, Three-Dimensional / methods. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / secondary. X-Ray Intensifying Screens

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  • (PMID = 18336479.001).
  • [ISSN] 1365-2184
  • [Journal-full-title] Cell proliferation
  • [ISO-abbreviation] Cell Prolif.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Luminescent Proteins; 0 / red fluorescent protein
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31. Canner JA, Sobo M, Ball S, Hutzen B, DeAngelis S, Willis W, Studebaker AW, Ding K, Wang S, Yang D, Lin J: MI-63: a novel small-molecule inhibitor targets MDM2 and induces apoptosis in embryonal and alveolar rhabdomyosarcoma cells with wild-type p53. Br J Cancer; 2009 Sep 1;101(5):774-81
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MI-63: a novel small-molecule inhibitor targets MDM2 and induces apoptosis in embryonal and alveolar rhabdomyosarcoma cells with wild-type p53.
  • METHODS: Rhabdomyosarcoma (RMS), the most common childhood soft tissue sarcoma, expresses either wild-type or mutant p53 protein.
  • We examined the inhibitory effects of MI-63 in embryonal RMS (ERMS) and alveolar RMS (ARMS) cell lines expressing wild-type or mutated p53.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Apoptosis / drug effects. Indoles / pharmacology. Proto-Oncogene Proteins c-mdm2 / antagonists & inhibitors. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Embryonal / pathology. Spiro Compounds / pharmacology. Tumor Suppressor Protein p53 / metabolism

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  • (PMID = 19707204.001).
  • [ISSN] 1532-1827
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Imidazoles; 0 / Indoles; 0 / MI-63 compound; 0 / Piperazines; 0 / Spiro Compounds; 0 / Tumor Suppressor Protein p53; 0 / nutlin 3; 80168379AG / Doxorubicin; EC 3.4.22.- / Caspase 3; EC 6.3.2.19 / MDM2 protein, human; EC 6.3.2.19 / Proto-Oncogene Proteins c-mdm2
  • [Other-IDs] NLM/ PMC2736841
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32. Diomedi-Camassei F, McDowell HP, De Ioris MA, Uccini S, Altavista P, Raschellà G, Vitali R, Mannarino O, De Sio L, Cozzi DA, Donfrancesco A, Inserra A, Callea F, Dominici C: Clinical significance of CXC chemokine receptor-4 and c-Met in childhood rhabdomyosarcoma. Clin Cancer Res; 2008 Jul 1;14(13):4119-27
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical significance of CXC chemokine receptor-4 and c-Met in childhood rhabdomyosarcoma.
  • PURPOSE: The CXC chemokine receptor-4 (CXCR4)/stromal-derived factor-1 and c-Met/hepatocyte growth factor axes promote the metastatic potential of rhabdomyosarcoma cell lines in experimental models, but no data are available on their role in rhabdomyosarcoma tumors.
  • EXPERIMENTAL DESIGN: Forty patients with recently diagnosed rhabdomyosarcoma were retrospectively enrolled.
  • High CXCR4 expression correlated with alveolar histology (P = 0.006), unfavorable primary site (P = 0.009), advanced group (P < 0.001), marrow involvement (P = 0.007), and shorter overall survival and event-free survival (P < 0.001); high c-Met expression correlated with alveolar histology (P = 0.005), advanced group (P = 0.04), and marrow involvement (P = 0.02).
  • CONCLUSIONS: CXCR4 and c-Met are widely expressed in both rhabdomyosarcoma subtypes and, at higher levels, in isolated marrow-infiltrating tumor cells.
  • In rhabdomyosarcoma, CXCR4 and c-Met represent novel exploitable targets for disease-directed therapy.
  • [MeSH-major] Proto-Oncogene Proteins c-met / metabolism. Receptors, CXCR4 / metabolism. Rhabdomyosarcoma / metabolism

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  • (PMID = 18593989.001).
  • [ISSN] 1078-0432
  • [Journal-full-title] Clinical cancer research : an official journal of the American Association for Cancer Research
  • [ISO-abbreviation] Clin. Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / CXCR4 protein, human; 0 / Receptors, CXCR4; EC 2.7.10.1 / Proto-Oncogene Proteins c-met
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33. Das K, Mirani N, Hameed M, Pliner L, Aisner SC: Fine-needle aspiration cytology of alveolar rhabdomyosarcoma utilizing ThinPrep liquid-based sample and cytospin preparations: a case confirmed by FKHR break apart rearrangement by FISH probe. Diagn Cytopathol; 2006 Oct;34(10):704-6
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  • [Title] Fine-needle aspiration cytology of alveolar rhabdomyosarcoma utilizing ThinPrep liquid-based sample and cytospin preparations: a case confirmed by FKHR break apart rearrangement by FISH probe.
  • Rhabdomyosarcoma is the most common childhood sarcoma that occurs in the soft tissues of the head and neck, genitourinary system, and extremities.
  • Herein we present a case of metastatic alveolar rhabdomyosarcoma originating in the nasal sinus and recurring in the neck of an adult man 5 yr after his initial presentation.
  • The cytologic findings of alveolar rhabdomyosarcoma were confirmed utilizing a dual color break apart FISH probe which identified the FKHR translocation on the cytospin preparation prepared from a ThinPrep vial and processed as a cytospin preparation.
  • [MeSH-major] Forkhead Transcription Factors / genetics. Head and Neck Neoplasms / diagnosis. Head and Neck Neoplasms / genetics. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics. Translocation, Genetic / genetics

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  • (PMID = 16955479.001).
  • [ISSN] 8755-1039
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA, Neoplasm; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors
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34. Krsková L, Mrhalová M, Hilská I, Sumerauer D, Drahokoupilová E, Múdry P, Kodet R: Detection and clinical significance of bone marrow involvement in patients with rhabdomyosarcoma. Virchows Arch; 2010 May;456(5):463-72
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Detection and clinical significance of bone marrow involvement in patients with rhabdomyosarcoma.
  • Childhood rhabdomyosarcoma (RMS) has two major histological subtypes: alveolar (aRMS) and embryonal.
  • [MeSH-major] Biomarkers, Tumor / analysis. Bone Marrow / pathology. Forkhead Transcription Factors / analysis. PAX7 Transcription Factor / analysis. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma, Alveolar / pathology
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Infant. Male. MyoD Protein / genetics. Neoplasm Recurrence, Local / pathology. Paired Box Transcription Factors / analysis. Prognosis. Recombinant Fusion Proteins / analysis. Reverse Transcriptase Polymerase Chain Reaction / methods. Rhabdomyosarcoma, Embryonal / genetics. Rhabdomyosarcoma, Embryonal / pathology. Young Adult

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  • (PMID = 20405298.001).
  • [ISSN] 1432-2307
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / MyoD Protein; 0 / MyoD1 myogenic differentiation protein; 0 / PAX3 protein, human; 0 / PAX7 Transcription Factor; 0 / PAX7 protein, human; 0 / Paired Box Transcription Factors; 0 / Recombinant Fusion Proteins
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35. Pazzaglia L, Chiechi A, Conti A, Gamberi G, Magagnoli G, Novello C, Morandi L, Picci P, Mercuri M, Benassi MS: Genetic and molecular alterations in rhabdomyosarcoma: mRNA overexpression of MCL1 and MAP2K4 genes. Histol Histopathol; 2009 01;24(1):61-7
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  • [Title] Genetic and molecular alterations in rhabdomyosarcoma: mRNA overexpression of MCL1 and MAP2K4 genes.
  • Rhabdomyosarcoma, the most common soft tissue sarcoma in childhood, belongs to the small round cell tumor family and is classified according to its histopathological features as embryonal, alveolar and pleomorphic.
  • In this study we propose to explore genetic alterations involved in rhabdomyosarcoma tumorigenesis and assess the level of mRNA gene expression of controlling survival signalling pathways.
  • Our findings on rhabdomyosarcoma samples showed multiple copy number alterations in chromosome regions implicated in malignancy progression and indicated a strong expression of MAP2K4 and MCL1 genes, both involved in different biological functions of complicated signalling pathways.
  • [MeSH-major] MAP Kinase Kinase 4 / genetics. Proto-Oncogene Proteins c-bcl-2 / genetics. RNA, Messenger / analysis. Rhabdomyosarcoma / genetics. Soft Tissue Neoplasms / genetics

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  • (PMID = 19012245.001).
  • [ISSN] 1699-5848
  • [Journal-full-title] Histology and histopathology
  • [ISO-abbreviation] Histol. Histopathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Myeloid Cell Leukemia Sequence 1 Protein; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / RNA, Messenger; EC 2.7.12.2 / MAP Kinase Kinase 4; EC 2.7.12.2 / MAP2K4 protein, human
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36. Barlow JW, Wiley JC, Mous M, Narendran A, Gee MF, Goldberg M, Sexsmith E, Malkin D: Differentiation of rhabdomyosarcoma cell lines using retinoic acid. Pediatr Blood Cancer; 2006 Nov;47(6):773-84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Differentiation of rhabdomyosarcoma cell lines using retinoic acid.
  • BACKGROUND: Rhabdomyosarcoma (RMS) is the most frequent sporadic soft tissue sarcoma of childhood and adolescence.
  • Novel therapeutic approaches are necessary to improve on these outcomes particularly among the more aggressive alveolar RMS (ARMS) and late stages of disease, where 5-year survival is less than 20%.
  • [MeSH-major] Cell Differentiation / drug effects. Rhabdomyosarcoma / drug therapy. Tretinoin / pharmacology. Tumor Suppressor Protein p53 / genetics

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  • [Copyright] (c) 2005 Wiley-Liss, Inc.
  • (PMID = 16283617.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / TP53 protein, human; 0 / Troponin T; 0 / Tumor Suppressor Protein p53; 3K9958V90M / Ethanol; 5300-03-8 / alitretinoin; 5688UTC01R / Tretinoin
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37. Chen Y, Takita J, Mizuguchi M, Tanaka K, Ida K, Koh K, Igarashi T, Hanada R, Tanaka Y, Park MJ, Hayashi Y: Mutation and expression analyses of the MET and CDKN2A genes in rhabdomyosarcoma with emphasis on MET overexpression. Genes Chromosomes Cancer; 2007 Apr;46(4):348-58
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  • [Title] Mutation and expression analyses of the MET and CDKN2A genes in rhabdomyosarcoma with emphasis on MET overexpression.
  • Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood.
  • No mutations were detected in exons 14-21 of MET whereas a nonsense mutation at codon 80 of p16(INK4A) was identified in an alveolar RMS cell line.
  • [MeSH-major] Cyclin-Dependent Kinase Inhibitor p16 / genetics. DNA Mutational Analysis. Gene Expression Profiling. Proto-Oncogene Proteins / genetics. Receptors, Growth Factor / genetics. Rhabdomyosarcoma / genetics

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17243166.001).
  • [ISSN] 1045-2257
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Codon, Nonsense; 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / Proto-Oncogene Proteins; 0 / Receptors, Growth Factor; EC 2.7.10.1 / MET protein, human; EC 2.7.10.1 / Proto-Oncogene Proteins c-met
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38. Seshadri N, Wright P, Balan KK: Rhabdomyosarcoma with widespread bone marrow infiltration: beneficial management role of F-18 FDG PET. Clin Nucl Med; 2007 Oct;32(10):787-9

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rhabdomyosarcoma with widespread bone marrow infiltration: beneficial management role of F-18 FDG PET.
  • Alveolar rhabdomyosarcoma (RMS) accounts for 20% to 30% of childhood RMS and is associated with a prognosis worse than embryonal RMS.
  • [MeSH-major] Bone Marrow Neoplasms / radionuclide imaging. Bone Marrow Neoplasms / therapy. Fluorodeoxyglucose F18. Positron-Emission Tomography / methods. Rhabdomyosarcoma, Alveolar / radionuclide imaging. Rhabdomyosarcoma, Alveolar / therapy

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  • (PMID = 17885359.001).
  • [ISSN] 0363-9762
  • [Journal-full-title] Clinical nuclear medicine
  • [ISO-abbreviation] Clin Nucl Med
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Radiopharmaceuticals; 0Z5B2CJX4D / Fluorodeoxyglucose F18
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39. Linardic CM, Naini S, Herndon JE 2nd, Kesserwan C, Qualman SJ, Counter CM: The PAX3-FKHR fusion gene of rhabdomyosarcoma cooperates with loss of p16INK4A to promote bypass of cellular senescence. Cancer Res; 2007 Jul 15;67(14):6691-9
eagle-i research resources. PMID 17638879 (Special Collections) .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The PAX3-FKHR fusion gene of rhabdomyosarcoma cooperates with loss of p16INK4A to promote bypass of cellular senescence.
  • Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence.
  • Despite advances in therapy, patients with a histologic variant of rhabdomyosarcoma known as alveolar rhabdomyosarcoma (ARMS) have a 5-year survival of <30%.
  • This prompted us to explore the effect of expressing PAX3-FKHR in more relevant cells, specifically primary human skeletal muscle cells because these cells can be converted to a tumorigenic state that mimics rhabdomyosarcoma.
  • This association between PAX3-FKHR expression and p16(INK4A) loss was seen in human ARMS tumor tissue, as both human rhabdomyosarcoma cell lines and tissue microarrays showed a trend toward down-regulation of p16(INK4A) protein in alveolar subsets.
  • [MeSH-major] Cyclin-Dependent Kinase Inhibitor p16 / metabolism. Forkhead Transcription Factors / physiology. Gene Expression Regulation, Neoplastic. Lung Neoplasms / metabolism. Paired Box Transcription Factors / physiology. Rhabdomyosarcoma / metabolism

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  • (PMID = 17638879.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Grant] United States / NICHD NIH HHS / HD / K12 HD043494; United States / NICHD NIH HHS / HD / 5K12 HD 043494; United States / NCI NIH HHS / CA / R01 CA 94184
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / FOXO1 protein, human; 0 / Forkhead Box Protein O1; 0 / Forkhead Transcription Factors; 0 / PAX3 Transcription Factor; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors; 0 / Recombinant Fusion Proteins
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40. Morgenstern DA, Anderson J: MYCN deregulation as a potential target for novel therapies in rhabdomyosarcoma. Expert Rev Anticancer Ther; 2006 Feb;6(2):217-24

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] MYCN deregulation as a potential target for novel therapies in rhabdomyosarcoma.
  • Rhabdomyosarcoma is the most common soft-tissue sarcoma of childhood.
  • Although overall outcomes have improved considerably, the outlook for patients with high-risk disease, particularly the alveolar subtype, remains bleak and there is a clear need for new chemotherapeutic strategies.
  • The importance of aberrant expression of this oncogene is well established in neuroblastoma and recent data indicate that MYCN deregulation also occurs in up to a quarter of alveolar subtype cases.
  • [MeSH-major] Gene Expression Regulation, Neoplastic / drug effects. Nuclear Proteins / genetics. Oncogene Proteins / genetics. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / genetics

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  • (PMID = 16445374.001).
  • [ISSN] 1744-8328
  • [Journal-full-title] Expert review of anticancer therapy
  • [ISO-abbreviation] Expert Rev Anticancer Ther
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / MYCN protein, human; 0 / Nuclear Proteins; 0 / Nucleic Acids; 0 / Oligonucleotides, Antisense; 0 / Oncogene Proteins
  • [Number-of-references] 74
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41. Schaaf G, Hamdi M, Zwijnenburg D, Lakeman A, Geerts D, Versteeg R, Kool M: Silencing of SPRY1 triggers complete regression of rhabdomyosarcoma tumors carrying a mutated RAS gene. Cancer Res; 2010 Jan 15;70(2):762-71

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Silencing of SPRY1 triggers complete regression of rhabdomyosarcoma tumors carrying a mutated RAS gene.
  • Studies of RAS status in the human childhood tumor rhabdomyosarcoma (RMS) indicated mutations in approximately half of the tumors of the embryonal rhabdomyosarcoma subtype (ERMS) but not the alveolar subtype (ARMS).
  • Our findings argue that SPRY1 inhibition can offer a therapeutic strategy to treat childhood RMS and possibly other tumors carrying oncogenic RAS mutations.
  • [MeSH-major] Genes, ras. Membrane Proteins / genetics. Mutation. Phosphoproteins / genetics. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Embryonal / genetics

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  • (PMID = 20068162.001).
  • [ISSN] 1538-7445
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Membrane Proteins; 0 / Phosphoproteins; 0 / SPRY1 protein, human; EC 2.7.11.24 / Extracellular Signal-Regulated MAP Kinases; EC 3.6.5.2 / ras Proteins
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42. Seitz G, Pfeiffer M, Fuchs J, Warmann SW, Leuschner I, Vokuhl C, Lang P, Handgretinger R, Armeanu-Ebinger S: Establishment of a rhabdomyosarcoma xenograft model in human-adapted mice. Oncol Rep; 2010 Oct;24(4):1067-72

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Establishment of a rhabdomyosarcoma xenograft model in human-adapted mice.
  • The outcome of patients with advanced stage rhabdomyosarcoma (RMS) is still sobering.
  • The aim of this study was to develop a humanized mouse model of childhood RMS as a basis for the study of immunotherapeutic approaches.
  • Eight weeks after transplantation, the subcutaneous xenotransplantation of human alveolar and embryonal RMS cell lines was carried out.
  • The xenotransplantation of alveolar RMS resulting in subcutaneous tumor growth was feasible in 7 animals.
  • A histological work up showed either alveolar or embryonal RMS cells with central necrosis.
  • The establishment of subcutaneous tumor xenografts was more effective in the alveolar subtype.
  • This model offers a basic tool for further analyzing novel immunotherapeutic approaches in RMS, and could possibly be used in other solid pediatric tumors.
  • [MeSH-major] Disease Models, Animal. Rhabdomyosarcoma. Soft Tissue Neoplasms. Transplantation, Heterologous / methods

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  • (PMID = 20811690.001).
  • [ISSN] 1791-2431
  • [Journal-full-title] Oncology reports
  • [ISO-abbreviation] Oncol. Rep.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Greece
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43. Bersani F, Taulli R, Accornero P, Morotti A, Miretti S, Crepaldi T, Ponzetto C: Bortezomib-mediated proteasome inhibition as a potential strategy for the treatment of rhabdomyosarcoma. Eur J Cancer; 2008 Apr;44(6):876-84
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Bortezomib-mediated proteasome inhibition as a potential strategy for the treatment of rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma of childhood, divided into two major histological subtypes, embryonal (ERMS) and alveolar (ARMS).
  • To explore the possibility that the proteasome could be a target of therapeutic value in rhabdomyosarcoma, we treated several RMS cell lines with the proteasome inhibitor bortezomib (Velcade or PS-341) at a concentration of 13-26 nM.
  • [MeSH-major] Boronic Acids / therapeutic use. Protease Inhibitors / therapeutic use. Proteasome Inhibitors. Pyrazines / therapeutic use. Rhabdomyosarcoma / drug therapy

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  • (PMID = 18342500.001).
  • [ISSN] 0959-8049
  • [Journal-full-title] European journal of cancer (Oxford, England : 1990)
  • [ISO-abbreviation] Eur. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Angiogenesis Inhibitors; 0 / Boronic Acids; 0 / Protease Inhibitors; 0 / Proteasome Inhibitors; 0 / Pyrazines; 69G8BD63PP / Bortezomib
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44. Kuçi S, Rettinger E, Voss B, Weber G, Stais M, Kreyenberg H, Willasch A, Kuçi Z, Koscielniak E, Klöss S, von Laer D, Klingebiel T, Bader P: Efficient lysis of rhabdomyosarcoma cells by cytokine-induced killer cells: implications for adoptive immunotherapy after allogeneic stem cell transplantation. Haematologica; 2010 Sep;95(9):1579-86
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Efficient lysis of rhabdomyosarcoma cells by cytokine-induced killer cells: implications for adoptive immunotherapy after allogeneic stem cell transplantation.
  • BACKGROUND: Rhabdomyosarcoma is the most common soft tissue sarcoma in childhood and has a poor prognosis.
  • Here we assessed the capability of ex vivo expanded cytokine-induced killer cells to lyse both alveolar and embryonic rhabdomyosarcoma cell lines and investigated the mechanisms involved.
  • The phenotype and composition of these cells were determined by multiparameter flow cytometry, while their cytotoxic effect against rhabdomyosarcoma cells was evaluated by a europium release assay.
  • RESULTS: Cytokine-induced killer cells efficiently lysed cells from both rhabdomyosarcoma cell lines.
  • Antibody-mediated masking of either NKG2D molecule on cytokine-induced killer cells or its ligands on rhabdomyosarcoma cells (major histocompatibility antigen related chain A and B and UL16 binding protein 2) diminished this effect by 50%, suggesting a major role for the NKG2D molecule in rhabdomyosarcoma cell killing.
  • Accordingly, blocking TRAIL receptors on embryonic rhabdomyosarcoma cell lines significantly reduced the anti-tumor effect of cytokine-induced killer cells.
  • CONCLUSIONS: Our data suggest that cytokine-induced killer cells may be used as a novel adoptive immunotherapy for the treatment of patients with rhabdomyosarcoma after allogeneic stem cell transplantation.
  • [MeSH-major] Cytokine-Induced Killer Cells / immunology. Cytotoxicity, Immunologic. Rhabdomyosarcoma / immunology. Rhabdomyosarcoma / therapy

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  • (PMID = 20378565.001).
  • [ISSN] 1592-8721
  • [Journal-full-title] Haematologica
  • [ISO-abbreviation] Haematologica
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Italy
  • [Other-IDs] NLM/ PMC2930961
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45. Sultan I, Qaddoumi I, Yaser S, Rodriguez-Galindo C, Ferrari A: Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients. J Clin Oncol; 2009 Jul 10;27(20):3391-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Comparing adult and pediatric rhabdomyosarcoma in the surveillance, epidemiology and end results program, 1973 to 2005: an analysis of 2,600 patients.
  • PURPOSE: To compare clinical features and outcomes of adults and children reported to have rhabdomyosarcoma.
  • PATIENTS AND METHODS: We analyzed data from 1,071 adults (age > 19 years) and 1,529 children (age < or = 19 years) reported in the public-access Surveillance, Epidemiology and End Results database as having rhabdomyosarcoma, diagnosed from 1973 to 2005.
  • RESULTS: Adults with rhabdomyosarcoma had significantly worse outcome than children (5-year overall survival rates, 27% +/- 1.4% and 61% +/- 1.4%, respectively; P < .0001).
  • Tumors in adults were more likely to be at an unfavorable site (65% v 55%; P < .0001) and to have histologies that are unusual during childhood, particularly the pleomorphic subtype (19%) and not otherwise specified (43%).
  • However, alveolar subtype and unfavorable primary site lost significance when analysis was restricted to adults.
  • CONCLUSION: Adults reported to have rhabdomyosarcoma had worse survival than children with similar tumors.
  • Predictors of poor outcome in children were valid in adults except for alveolar histology and unfavorable tumor site.
  • [MeSH-major] Rhabdomyosarcoma / epidemiology. SEER Program / statistics & numerical data

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  • (PMID = 19398574.001).
  • [ISSN] 1527-7755
  • [Journal-full-title] Journal of clinical oncology : official journal of the American Society of Clinical Oncology
  • [ISO-abbreviation] J. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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46. Gattenlöhner S: [Rhabdomyosarcoma lysis by T cells expressing a human autoantibody based chimeric receptor targeting the fetal acetylcholine receptors]. Verh Dtsch Ges Pathol; 2006;90:264-76

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Rhabdomyosarcoma lysis by T cells expressing a human autoantibody based chimeric receptor targeting the fetal acetylcholine receptors].
  • Rhabdomyosarcomas (RMSs) are the most frequent malignant soft tissue tumors of childhood.
  • Additionally we established a duplex RT-PCR with simultaneous amplification of alpha and gamma subunit message of the fAChR and the quantification of both transcripts resulting in alpha/gammaAChR ratio > 1 was 100% sensitive in alveolar and embryonal rhabdomyosarcoma.
  • [MeSH-major] Autoantibodies / therapeutic use. Receptors, Cholinergic / immunology. Rhabdomyosarcoma / immunology. Rhabdomyosarcoma / pathology. T-Lymphocytes / immunology

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  • (PMID = 17867605.001).
  • [ISSN] 0070-4113
  • [Journal-full-title] Verhandlungen der Deutschen Gesellschaft für Pathologie
  • [ISO-abbreviation] Verh Dtsch Ges Pathol
  • [Language] ger
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Autoantibodies; 0 / Biomarkers; 0 / Receptors, Cholinergic
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47. Abd El-Aal HH, Habib EE, Mishrif MM: Rhabdomyosarcoma: the experience of the pediatric unit of Kasr El-Aini Center of Radiation Oncology and Nuclear Medicine (NEMROCK) (from January 1992 to January 2001). J Egypt Natl Canc Inst; 2006 Mar;18(1):51-60

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Rhabdomyosarcoma: the experience of the pediatric unit of Kasr El-Aini Center of Radiation Oncology and Nuclear Medicine (NEMROCK) (from January 1992 to January 2001).
  • Our present study is a retrospective analysis of the treatment results of new rhabdomyosarcoma pediatric patients who had attended the pediatric unit clinic of Kasr El-Aini Center of Radiation Oncology and Nuclear Medicine (NEMROCK) from January 1992 to January 2001).
  • PATIENTS AND METHODS: Fifty-five new cases of pediatric rhabdomyosarcoma attended the pediatric unit outpatient clinic of (NEMROCK) from the period of January 1992 until January 2001.
  • RESULTS: Fifty-five new cases of pediatric rhabdomyosarcoma attended the pediatric unit outpatient clinic of (NEMROCK) and were evaluated.
  • 87.3%) compared to the alveolar type (7/55, i.e. 12.7%).
  • CONCLUSION: Despite the advances in the therapy of rhabdomyosarcoma.
  • Nearly 30% of the pediatric cases with rhabdomyosarcoma experience progressive or relapsing disease, which has a fatal end.
  • These findings will form the basis of a multi-institutional risk adapted relapse protocol for childhood rhabdomyosarcoma patients.
  • [MeSH-major] Rhabdomyosarcoma / mortality. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / therapy

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  • (PMID = 17237856.001).
  • [ISSN] 1110-0362
  • [Journal-full-title] Journal of the Egyptian National Cancer Institute
  • [ISO-abbreviation] J Egypt Natl Canc Inst
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Egypt
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48. Strahm B, Durbin AD, Sexsmith E, Malkin D: The CXCR4-SDF1alpha axis is a critical mediator of rhabdomyosarcoma metastatic signaling induced by bone marrow stroma. Clin Exp Metastasis; 2008;25(1):1-10

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The CXCR4-SDF1alpha axis is a critical mediator of rhabdomyosarcoma metastatic signaling induced by bone marrow stroma.
  • Rhabdomyosarcoma (RMS) is the most common malignant soft-tissue tumor of childhood.
  • CXCR4 was expressed in RMS cell lines and primary tumors, with higher expression in alveolar subtype RMS.
  • [MeSH-major] Chemokine CXCL12 / metabolism. Neoplasm Invasiveness / physiopathology. Receptors, CXCR4 / metabolism. Rhabdomyosarcoma / pathology. Signal Transduction / physiology

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  • (PMID = 17768666.001).
  • [ISSN] 0262-0898
  • [Journal-full-title] Clinical & experimental metastasis
  • [ISO-abbreviation] Clin. Exp. Metastasis
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / CXCR4 protein, human; 0 / Chemokine CXCL12; 0 / Culture Media, Conditioned; 0 / Receptors, CXCR4
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49. Cen L, Hsieh FC, Lin HJ, Chen CS, Qualman SJ, Lin J: PDK-1/AKT pathway as a novel therapeutic target in rhabdomyosarcoma cells using OSU-03012 compound. Br J Cancer; 2007 Sep 17;97(6):785-91
The Lens. Cited by Patents in .

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] PDK-1/AKT pathway as a novel therapeutic target in rhabdomyosarcoma cells using OSU-03012 compound.
  • Rhabdomyosarcoma (RMS) is the most common paediatric soft-tissue sarcoma including two major subtypes, alveolar rhabdomyosarcoma (ARMS) and embryonal rhabdomyosarcoma (ERMS).
  • [MeSH-major] Antineoplastic Agents / pharmacology. Enzyme Inhibitors / pharmacology. Oncogene Protein v-akt / metabolism. Protein-Serine-Threonine Kinases / metabolism. Pyrazoles / pharmacology. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / metabolism. Sulfonamides / pharmacology

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  • (PMID = 17848913.001).
  • [ISSN] 0007-0920
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Chromones; 0 / Enzyme Inhibitors; 0 / Morpholines; 0 / OSU 03012; 0 / Pyrazoles; 0 / Sulfonamides; 154447-36-6 / 2-(4-morpholinyl)-8-phenyl-4H-1-benzopyran-4-one; EC 2.7.11.1 / Oncogene Protein v-akt; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 2.7.11.2 / pyruvate dehydrogenase (acetyl-transferring) kinase
  • [Other-IDs] NLM/ PMC2360380
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50. Blank LE, Koedooder K, van der Grient HN, Wolffs NA, van de Kar M, Merks JH, Pieters BR, Saeed P, Baldeschi L, Freling NJ, Koning CC: Brachytherapy as part of the multidisciplinary treatment of childhood rhabdomyosarcomas of the orbit. Int J Radiat Oncol Biol Phys; 2010 Aug 1;77(5):1463-9

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Brachytherapy as part of the multidisciplinary treatment of childhood rhabdomyosarcomas of the orbit.
  • INTRODUCTION: Rhabdomyosarcomas in the orbit form a major challenge in terms of cure without severe side effects in childhood cancer.
  • [MeSH-major] Brachytherapy / methods. Orbital Neoplasms / radiotherapy. Rhabdomyosarcoma, Alveolar / radiotherapy. Rhabdomyosarcoma, Embryonal / radiotherapy

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 19864080.001).
  • [ISSN] 1879-355X
  • [Journal-full-title] International journal of radiation oncology, biology, physics
  • [ISO-abbreviation] Int. J. Radiat. Oncol. Biol. Phys.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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51. Timmermann B, Schuck A, Niggli F, Weiss M, Lomax A, Goitein G: ["Spot-scanning" proton therapy for rhabdomyosarcomas of early childhood. First experiences at PSI]. Strahlenther Onkol; 2006 Nov;182(11):653-9

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  • [Title] ["Spot-scanning" proton therapy for rhabdomyosarcomas of early childhood. First experiences at PSI].
  • RESULTS: Nine children were included aged 0.9-3.8 years (embryonal RMS in six, and alveolar, undifferentiated or nonclassified in one each).
  • [MeSH-major] Protons / therapeutic use. Rhabdomyosarcoma / radiotherapy. Soft Tissue Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Age Factors. Child, Preschool. Combined Modality Therapy. Feasibility Studies. Female. Head and Neck Neoplasms / radiotherapy. Humans. Infant. Infant, Newborn. Male. Meningeal Neoplasms / radiotherapy. Orbital Neoplasms / radiotherapy. Prospective Studies. Prostatic Neoplasms / radiotherapy. Radiotherapy Planning, Computer-Assisted. Rhabdomyosarcoma, Alveolar / radiotherapy. Rhabdomyosarcoma, Embryonal / radiotherapy. Survival Analysis. Treatment Outcome

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  • (PMID = 17072523.001).
  • [ISSN] 0179-7158
  • [Journal-full-title] Strahlentherapie und Onkologie : Organ der Deutschen Röntgengesellschaft ... [et al]
  • [ISO-abbreviation] Strahlenther Onkol
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Evaluation Studies; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Protons
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52. Aitken L, Levin D, Blau A, Lewin MR: Acute hearing loss, dysarthria, dysphagia, and a rubbery intraoral mass in an 18-year-old woman. Pediatr Emerg Care; 2009 Aug;25(8):516-8
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  • Rhabdomyosarcoma is the most common soft tissue tumor of childhood, frequently presenting in the head and neck, genitourinary tract, or extremities.
  • We present a case of rhabdomyosarcoma in which an 18-year-old woman presented with abrupt onset unilateral hearing loss, tinnitus, dysarthria, dysphagia, and a new painless red bump on the palate.
  • With an alveolar subtype and older age, both predictors of poor prognosis, early recognition of disease of these symptoms is vital.
  • [MeSH-major] Deglutition Disorders / etiology. Dysarthria / etiology. Hearing Loss, Sensorineural / etiology. Palatal Neoplasms / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis. Tinnitus / etiology

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  • (PMID = 19687710.001).
  • [ISSN] 1535-1815
  • [Journal-full-title] Pediatric emergency care
  • [ISO-abbreviation] Pediatr Emerg Care
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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53. Miron I, Miron L, Dumitraş S, Aprodu G, Ciobanu A, Tansanu I: [Statistical study of the evolution over ten years of the clinical and therapeutic approach in childhood soft tissue sarcoma]. Rev Med Chir Soc Med Nat Iasi; 2007 Apr-Jun;111(2):358-62
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  • [Title] [Statistical study of the evolution over ten years of the clinical and therapeutic approach in childhood soft tissue sarcoma].
  • Rhabdomyosarcoma (RMS) constitutes 5.8% from the whole amount of pediatric solid tumors, taking the fourth place after CNS tumors, neuroblastoma, and Wilms tumors.
  • The other category of non-rhabdomyosarcoma tumors in children and teenagers represents 3% of the solid malignancies under 18 years old.
  • MATERIAL AND METHOD: The study was made between January 1995 and July 2005 in the Pediatric Department of Hematology and Oncology of the "Sf.
  • RESULTS: Positive diagnostic confirmation was established on pathological grounds in optic microscopy and immuno-histo-enzymology performed on bioptic samples in Department of Pediatric Surgery and analyzed in Pathology Laboratory of "Sf.
  • Based on histological examination 19 cases (32.75%) were of rhabdomyosarcoma type with following subtypes: alveolar--7 patients, embryonic-- 9 cases, fusiform - 2 cases, bothrioid--1 case), 8 cases were undifferentiated soft tissue sarcomas and one patient had a tumor of pleiomorphic type; 13 children (22.41%) had non-rhabdomyosarcoma soft tissue sarcomas: 6 fibrosarcomas, 2 synovial sarcomas, 1 leiomyosarcoma, 1 Kaposi sarcoma, 1 case of malignant peripheral nerve sheath tumor, 1 case of angioma tumor, one liposarcoma; 16 cases were included in soft tissue tumors of uncertain origin (fibromatosis--6 cases, fibrous histiocytoma--4 cases, hamartoma--cases, myoblastoma--1 case, fibro-xanthoma--1 case, hemangioendothelioma--1 case); 1 PPNET (Askin tumor).

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  • (PMID = 17983168.001).
  • [ISSN] 0048-7848
  • [Journal-full-title] Revista medico-chirurgicală̆ a Societă̆ţ̜ii de Medici ş̧i Naturaliş̧ti din Iaş̧i
  • [ISO-abbreviation] Rev Med Chir Soc Med Nat Iasi
  • [Language] rum
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Romania
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54. Seleye-Fubara D, Etebu EN: Juvenile rhabdomyosarcomas in Port Harcourt, Nigeria: A twelve year review. West Afr J Med; 2006 Jan-Mar;25(1):57-60

  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: Juvenile rhabdomyosarcoma (JRMS) though rare, is the most common soft tissue malignancy of childhood that exhibits bimodal age distribution pattern.
  • The most common histologic type is the embryonal rhabdomyosarcoma (71.5%).
  • Alveolar rhabdomyosarcoma accounted for (19%) and the sarcoma botryoides (9.5%).
  • If a tumor histologically shows as small round blue cells, rhabdomyosarcoma should be considered as a differential diagnosis.
  • [MeSH-major] Rhabdomyosarcoma / epidemiology
  • [MeSH-minor] Abdomen. Adolescent. Adult. Age Distribution. Child. Child, Preschool. Female. Head. Humans. Infant. Infant, Newborn. Lower Extremity. Male. Nigeria / epidemiology. Retrospective Studies. Rhabdomyosarcoma, Alveolar / epidemiology. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Embryonal / epidemiology. Rhabdomyosarcoma, Embryonal / pathology. Sarcoma / epidemiology. Sarcoma / pathology. Sex Distribution. Upper Extremity

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  • (PMID = 16722360.001).
  • [ISSN] 0189-160X
  • [Journal-full-title] West African journal of medicine
  • [ISO-abbreviation] West Afr J Med
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Nigeria
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55. Kurmasheva RT, Peterson CA, Parham DM, Chen B, McDonald RE, Cooney CA: Upstream CpG island methylation of the PAX3 gene in human rhabdomyosarcomas. Pediatr Blood Cancer; 2005 Apr;44(4):328-37
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • In contrast, much less is known about gene hypermethylation in childhood cancers, where methylation changes are not part of the aging process but likely represent developmental dysregulation.
  • PROCEDURES: We examined the methylation status of a PAX3 5'-CpG island in rhabdomyosarcoma subtypes and in normal fetal skeletal muscle.
  • PAX3 methylation was analyzed in 15 embryonal rhabdomyosarcomas, 12 alveolar rhabdomyosarcomas, and in six normal skeletal muscle samples, using semi-quantitative PCR analysis of DNA digested with methyl-sensitive restriction enzymes.
  • CONCLUSIONS: PAX3 CpG island methylation appears to distinguish embryonal subtype of rhabdomyosarcoma from alveolar, and methylation at certain sites within this CpG island is inversely correlated with PAX3 expression.
  • In addition to exemplifying developmental dysregulation, methylation of PAX3 has potential in the development of an epigenetic profile for the diagnosis of rhabdomyosarcoma.
  • [MeSH-major] CpG Islands. DNA Methylation. DNA-Binding Proteins / genetics. Gene Expression Regulation, Neoplastic / genetics. Rhabdomyosarcoma / genetics. Transcription Factors / genetics
  • [MeSH-minor] Child. Humans. Muscle Development / genetics. Muscle, Skeletal / chemistry. Muscle, Skeletal / embryology. Paired Box Transcription Factors. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Embryonal / genetics. Rhabdomyosarcoma, Embryonal / pathology

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  • (PMID = 15602708.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NIA NIH HHS / AG / AG20941
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / DNA-Binding Proteins; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors; 0 / Transcription Factors
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56. Smith MA, Morton CL, Phelps D, Girtman K, Neale G, Houghton PJ: SK-NEP-1 and Rh1 are Ewing family tumor lines. Pediatr Blood Cancer; 2008 Mar;50(3):703-6
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  • The utility of preclinical models of childhood cancers is contingent upon reliably classifying them with their corresponding clinical counterparts.
  • Rh1, which was previously categorized as an alveolar rhabdomyosarcoma cell line, also has a gene expression profile suggestive of Ewing sarcoma and expresses EWS-FLI1 fusion transcripts in which exon 7 of EWS is joined with exon 6 of FLI1.
  • These examples illustrate the importance of molecularly characterizing pediatric preclinical models used for testing new agents.
  • [MeSH-major] Cell Line, Tumor. Kidney Neoplasms / pathology. Rhabdomyosarcoma, Alveolar / pathology. Sarcoma, Ewing / pathology. Wilms Tumor / pathology

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  • [Copyright] (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17154184.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CM / N01CM42216
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / EWS-FLI fusion protein; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS; 0 / Transcription Factors
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57. Parham DM, Ellison DA: Rhabdomyosarcomas in adults and children: an update. Arch Pathol Lab Med; 2006 Oct;130(10):1454-65
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • CONTEXT: Rhabdomyosarcomas comprise a relatively common diagnostic entity among childhood cancers and a relatively rare one among adult tumors.
  • These lesions appear to be separate biologic entities as well as morphologic categories, with embryonal tumors having genetic lesions related to loss of heterozygosity and aberrant parental imprinting, alveolar tumors containing genetic fusions between PAX and forkhead genes, and pleomorphic tumors showing an accumulation of genetic lesions similar to other adult high-grade sarcomas.
  • OBJECTIVE: To present guidelines for diagnosis of rhabdomyosarcoma and recent finding concerning the biology and classification of these lesions.
  • CONCLUSIONS: Infants and young children tend to have embryonal rhabdomyosarcomas, adolescents and young adults tend to have alveolar rhabdomyosarcomas, and older adults tend to have pleomorphic rhabdomyosarcomas, although there is some overlap.
  • Newer rare entities, including spindle cell rhabdomyosarcoma and sclerosing rhabdomyosarcoma, have been described in children and adults.
  • [MeSH-major] Rhabdomyosarcoma / diagnosis

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  • (PMID = 17090187.001).
  • [ISSN] 1543-2165
  • [Journal-full-title] Archives of pathology & laboratory medicine
  • [ISO-abbreviation] Arch. Pathol. Lab. Med.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 152
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58. De Corti F, Dall'Igna P, Bisogno G, Casara D, Rossi CR, Foletto M, Alaggio R, Carli M, Cecchetto G: Sentinel node biopsy in pediatric soft tissue sarcomas of extremities. Pediatr Blood Cancer; 2009 Jan;52(1):51-4
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Sentinel node biopsy in pediatric soft tissue sarcomas of extremities.
  • However, only few reports describe this procedure for the evaluation of regional lymph nodes in childhood and adolescents.
  • The diagnosis was rhabdomyosarcoma in 5 and other soft tissue sarcomas in 12: Ewing/PNET sarcoma 6, epithelioid sarcoma 1, malignant peripheral-nerve-sheath tumor 1, undifferentiated sarcoma 1, myxoid liposarcoma 2, adult-type fibrosarcoma 1.
  • Nodes were positive for metastasis in two patients with alveolar rhabdomyosarcoma and undifferentiated sarcoma.
  • It could be an alternative to aggressive or random biopsies for extremity rhabdomyosarcoma and it can contribute to define those non-rhabdomyosarcoma soft tissue sarcomas that spread to regional nodes.
  • [MeSH-major] Extremities. Rhabdomyosarcoma / pathology

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  • (PMID = 18819127.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Evaluation Studies; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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59. Roumes H, Leloup L, Dargelos E, Brustis JJ, Daury L, Cottin P: Calpains: markers of tumor aggressiveness? Exp Cell Res; 2010 May 15;316(9):1587-99
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  • Rhabdomyosarcoma (RMS) are soft-tissue sarcoma commonly encountered in childhood.
  • [MeSH-major] Calcium-Binding Proteins / metabolism. Calpain / metabolism. Myoblasts / metabolism. Rhabdomyosarcoma, Alveolar / metabolism. Rhabdomyosarcoma, Alveolar / pathology

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20193680.001).
  • [ISSN] 1090-2422
  • [Journal-full-title] Experimental cell research
  • [ISO-abbreviation] Exp. Cell Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / Calcium-Binding Proteins; 0 / RNA, Messenger; 79079-11-1 / calpastatin; EC 3.4.22.- / Calpain; EC 3.4.22.- / m-calpain; EC 3.4.22.- / mu-calpain
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60. Tanko NM, Echejoh GO, Manasseh NA, Mandong MB, Uba AF: Paediatric solid tumours in Nigerian children: a changing pattern? Afr J Paediatr Surg; 2009 Jan-Jun;6(1):7-10
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  • BACKGROUND: Childhood cancer is fast becoming an important paediatric problem in Nigeria and several parts of Africa, with the progressive decline of infectious and nutritional diseases.
  • OBJECTIVE: To determine the relative frequencies of childhood solid malignant tumours in Jos, Central Nigeria and compare with reports of previous studies both locally and abroad.
  • The commonest malignant tumour diagnosed was rhabdomyosarcoma which accounted for 27 (31%), comprising of 15 (55.6%), 11 (40.7%) and 1 (3.7%) embryonal, alveolar and pleomorphic rhabdomyosarcomas, respectively.
  • CONCLUSION: Based on the result of our study, we conclude that the commonest solid malignancy of childhood in Jos, Nigeria is rhabdomyosarcoma.
  • [MeSH-major] Neoplasms / epidemiology. Neoplasms / pathology. Rhabdomyosarcoma / epidemiology. Rhabdomyosarcoma / pathology

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  • (PMID = 19661657.001).
  • [ISSN] 0974-5998
  • [Journal-full-title] African journal of paediatric surgery : AJPS
  • [ISO-abbreviation] Afr J Paediatr Surg
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Nigeria
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61. Caldas H, Holloway MP, Hall BM, Qualman SJ, Altura RA: Survivin-directed RNA interference cocktail is a potent suppressor of tumour growth in vivo. J Med Genet; 2006 Feb;43(2):119-28
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  • Our study aimed to identify rhabdomyosarcoma tumours that express Survivin, in order to test novel Survivin-targeted therapies in these tumours.
  • METHODS: Tumour microarray slides composed of 63 primary rhabdomyosarcoma tumours were stained with a polyclonal antibody to Survivin to identify tumours expressing Survivin.
  • Subcutaneous tumours were then established in NOD/SCID mice using RH30(red) cells, a red fluorescent clone of the RH30 human alveolar rhabdomyosarcoma cell line.
  • RESULTS: Over 80% of primary rhabdomyosarcoma tumours expressed Survivin.
  • Treatment of rhabdomyosarcoma xenografts showed greater than 70% reduction in growth when compared with control injected tumours at study completion (average tumour sizes: 1683 v 304 mm3, p<0.05).
  • CONCLUSIONS: Our findings support a role for Survivin in rhabdomyosarcoma biology and provide preliminary evidence for the therapeutic use of Survivin-targeted RNA interference for human tumours that express high levels of this molecule.
  • [MeSH-major] Microtubule-Associated Proteins / antagonists & inhibitors. Microtubule-Associated Proteins / genetics. Neoplasm Proteins / antagonists & inhibitors. Neoplasm Proteins / genetics. RNA Interference. Rhabdomyosarcoma / pathology

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  • (PMID = 15908567.001).
  • [ISSN] 1468-6244
  • [Journal-full-title] Journal of medical genetics
  • [ISO-abbreviation] J. Med. Genet.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / BIRC5 protein, human; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins; 0 / Neoplasm Proteins
  • [Other-IDs] NLM/ PMC2564625
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62. Dietz KN, Miller PJ, Hollenbach AD: Phosphorylation of serine 205 by the protein kinase CK2 persists on Pax3-FOXO1, but not Pax3, throughout early myogenic differentiation. Biochemistry; 2009 Dec 15;48(49):11786-95
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  • The myogenic transcription factor Pax3 plays an essential role in early skeletal muscle development and is a key component in alveolar rhabdomyosarcoma (ARMS), a childhood solid muscle tumor.

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  • (PMID = 19904978.001).
  • [ISSN] 1520-4995
  • [Journal-full-title] Biochemistry
  • [ISO-abbreviation] Biochemistry
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA138656-01A1; United States / NCI NIH HHS / CA / R01 CA138656-01A1; United States / NCI NIH HHS / CA / 1R01CA138656; United States / NCRR NIH HHS / RR / P20 RR020152; United States / NCRR NIH HHS / RR / RR020152-066702; United States / NCRR NIH HHS / RR / 1P20RR020152; United States / NCI NIH HHS / CA / R01 CA138656; United States / NCRR NIH HHS / RR / P20 RR020152-066702
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / PAX3-FOXO1A fusion protein, human; 0 / Paired Box Transcription Factors; 138016-91-8 / Pax3 protein, mouse; 452VLY9402 / Serine; EC 2.7.11.1 / Casein Kinase II
  • [Other-IDs] NLM/ NIHMS159458; NLM/ PMC2790557
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63. Mattke AC, Bailey EJ, Schuck A, Dantonello T, Leuschner I, Klingebiel T, Treuner J, Koscielniak E: Does the time-point of relapse influence outcome in pediatric rhabdomyosarcomas? Pediatr Blood Cancer; 2009 Jul;52(7):772-6
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  • [Title] Does the time-point of relapse influence outcome in pediatric rhabdomyosarcomas?
  • BACKGROUND: Childhood rhabdomyosarcoma (RMS), a soft tissue malignant tumor of skeletal muscle origin, accounts for approximately 3.5% of the cases of cancer among children 0-14 years and 2% of the cases among adolescents and young adults 15-19 years of age.
  • PROCEDURE: We evaluated survival (SUR) after first relapse depending on the time to relapse (TTR) in RMSs of childhood and adolescence.
  • Embryonal RMS showed four year SUR of 16%, 30%, and 46% (P < 0.001) whereas alveolar histology showed four year SUR of 8%, 6%, and 23% (P < 0.01) for early, intermediate, and late relapse respectively.
  • [MeSH-major] Neoplasm Recurrence, Local / mortality. Neoplasms, Muscle Tissue / mortality. Rhabdomyosarcoma / mortality


64. Hsieh MJ, Yao YL, Lai IL, Yang WM: Transcriptional repression activity of PAX3 is modulated by competition between corepressor KAP1 and heterochromatin protein 1. Biochem Biophys Res Commun; 2006 Oct 20;349(2):573-81
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  • Pax3 has been known to cause Waardenburg syndrome and pediatric alveolar rhabdomyosarcoma, but how Pax3 regulates transcription is not clear.

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  • (PMID = 16945326.001).
  • [ISSN] 0006-291X
  • [Journal-full-title] Biochemical and biophysical research communications
  • [ISO-abbreviation] Biochem. Biophys. Res. Commun.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Chromosomal Proteins, Non-Histone; 0 / DNA-Binding Proteins; 0 / Nuclear Proteins; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors; 0 / Repressor Proteins; 0 / TRIM28 protein, human; 0 / Transcription Factors; 0 / Trim28 protein, mouse; 107283-02-3 / heterochromatin-specific nonhistone chromosomal protein HP-1; 138016-91-8 / Pax3 protein, mouse
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65. Yasuda T, Perry KD, Nelson M, Bui MM, Nasir A, Goldschmidt R, Gnepp DR, Bridge JA: Alveolar rhabdomyosarcoma of the head and neck region in older adults: genetic characterization and a review of the literature. Hum Pathol; 2009 Mar;40(3):341-8
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  • [Title] Alveolar rhabdomyosarcoma of the head and neck region in older adults: genetic characterization and a review of the literature.
  • Alveolar rhabdomyosarcoma is remarkably rare in adults older than 45 years.
  • A valuable diagnostic aid and important prognostic parameter in alveolar rhabdomyosarcoma is the identification of PAX3-FOXO1 [t(2;13)(q35;q14)] or PAX7-FOXO1 [t(1;13)(p36;q14)] rearrangements.
  • The purpose of this study was to document the clinicopathologic, immunophenotypic, and genetic features of head/neck alveolar rhabdomyosarcoma in older adults.
  • Definitive alveolar rhabdomyosarcoma diagnoses were confirmed genetically.
  • This study illustrates the diagnosis of head/neck alveolar rhabdomyosarcoma in older adults is complicated by its rarity, lack of an alveolar pattern, and a potentially misleading immunoprofile (CD56 and synaptophysin immunoreactivity) if myogenic markers are not used.
  • Both PAX3- and PAX7-FOXO1 alveolar rhabdomyosarcomas were identified in these patients.
  • In children, PAX7-FOXO1 alveolar rhabdomyosarcoma is associated with a significantly longer event-free survival.
  • In contrast, adult alveolar rhabdomyosarcoma behaves more aggressively with a worse overall survival than pediatric alveolar rhabdomyosarcoma.

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  • (PMID = 18973919.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA036727-249009; United States / NCI NIH HHS / CA / P30 CA036727; United States / NCI NIH HHS / CA / P30 CA 36727; United States / NCI NIH HHS / CA / P30 CA036727-249009
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / PAX3 protein, human; 0 / PAX7 Transcription Factor; 0 / PAX7 protein, human; 0 / Paired Box Transcription Factors
  • [Other-IDs] NLM/ NIHMS96749; NLM/ PMC2753286
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66. Naithani R, Tyagi S, Choudhry VP: Secondary myelofibrosis in children. J Pediatr Hematol Oncol; 2008 Mar;30(3):196-8
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  • Myelofibrosis is a rare childhood myeloproliferative disorder.
  • [MeSH-major] Precursor Cell Lymphoblastic Leukemia-Lymphoma / complications. Primary Myelofibrosis / diagnosis. Rhabdomyosarcoma, Alveolar / complications

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  • (PMID = 18376280.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] X4W7ZR7023 / Methylprednisolone
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67. Martin LT, Glass M, Dosunmu E, Martin PT: Altered expression of natively glycosylated alpha dystroglycan in pediatric solid tumors. Hum Pathol; 2007 Nov;38(11):1657-68
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  • [Title] Altered expression of natively glycosylated alpha dystroglycan in pediatric solid tumors.
  • To date, however, there has been no study of the expression of dystroglycan in pediatric solid tumors.
  • Using a combination of immunostaining on tissue microarrays and immunoblotting of snap-frozen unfixed tissues, we demonstrate a significant reduction in native alpha dystroglycan expression in pediatric alveolar rhabdomyosarcoma (RMS), embryonal RMS, neuroblastoma (NBL), and medulloblastoma, whereas expression of beta dystroglycan, which is cotranslated with alpha dystroglycan, is largely unchanged.
  • Expression of natively glycosylated alpha dystroglycan was not altered in several other pediatric tumor types when compared with appropriate normal tissue controls.
  • These data provide the first evidence that alpha dystroglycan glycosylation and laminin binding to alpha dystroglycan are altered in certain pediatric solid tumors and suggest that aberrant dystroglycan glycosylation may contribute to tumor cell biology in patients with RMS, medulloblastoma, and NBL.

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  • (PMID = 17640712.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] ENG
  • [Grant] United States / NIAMS NIH HHS / AR / AR050202-02; United States / NIAMS NIH HHS / AR / R01 AR050202; United States / NIAMS NIH HHS / AR / R01 AR049722-01A2; United States / NIAMS NIH HHS / AR / AR 050202; United States / NIAMS NIH HHS / AR / AR049722-01A2; United States / NIAMS NIH HHS / AR / R01 AR050202-02; United States / NIAMS NIH HHS / AR / R01 AR049722
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Laminin; 146888-27-9 / Dystroglycans
  • [Other-IDs] NLM/ NIHMS190362; NLM/ PMC2850815
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68. Scuoppo C, Riess I, Schmitt-Ney M, Allegra P, Forni PE, Bersani F, Taulli R, Accornero P, Crepaldi T, Ponzetto C: The oncogenic transcription factor PAX3-FKHR can convert fibroblasts into contractile myotubes. Exp Cell Res; 2007 Jul 1;313(11):2308-17
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  • PAX3-FKHR, the product of a rearrangement of PAX3 with FKHR is the pathogenetic marker for alveolar rhabdomyosarcoma, an aggressive form of childhood cancer.
  • This work highlights the potential of PAX3-FKHR to functionally operate as a deregulated Pangene and may have implications with regard to the identity of the precursor cell giving rise to alveolar rhabdomyosarcoma.

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  • (PMID = 17490646.001).
  • [ISSN] 0014-4827
  • [Journal-full-title] Experimental cell research
  • [ISO-abbreviation] Exp. Cell Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / PAX3-FOXO1A fusion protein, human; 0 / Paired Box Transcription Factors
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69. Sakaguchi S, Garcia-Bournissen F, Kim R, Schwarz UI, Nathan PC, Ito S: Prolonged neutropenia after irinotecan-based chemotherapy in a child with polymorphisms of UGT1A1 and SLCO1B1. Arch Dis Child; 2009 Dec;94(12):981-2
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  • [MeSH-minor] Child. Female. Genetic Predisposition to Disease. Humans. Nasopharyngeal Neoplasms / drug therapy. Polymorphism, Single Nucleotide. Rhabdomyosarcoma, Alveolar / drug therapy

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  • (PMID = 19608554.001).
  • [ISSN] 1468-2044
  • [Journal-full-title] Archives of disease in childhood
  • [ISO-abbreviation] Arch. Dis. Child.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents, Phytogenic; 0 / Organic Anion Transporters; 0 / SLCO1B1 protein, human; 7673326042 / irinotecan; EC 2.4.1.- / UGT1A1 enzyme; EC 2.4.1.17 / Glucuronosyltransferase; XT3Z54Z28A / Camptothecin
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70. Santos ND, Habibi G, Wang M, Law JH, Andrews HN, Wei D, Triche T, Dedhar S, Dunn SE: Urokinase-type Plasminogen Activator (uPA) is Inhibited with QLT0267 a Small Molecule Targeting Integrin-linked Kinase (ILK). Transl Oncogenomics; 2007;2:85-97
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  • Whether uPA is also expressed in pediatric cancers is yet unknown.
  • We then questioned whether uPA was commonly expressed in childhood sarcomas and if QLT0267 might be effective in this setting.
  • In alveolar RMS (ARMS) cell lines, QLT0267 blocked cell signaling, uPA production, invasion and ultimately survival.

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  • (PMID = 23645983.001).
  • [Journal-full-title] Translational oncogenomics
  • [ISO-abbreviation] Transl Oncogenomics
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Other-IDs] NLM/ PMC3634623
  • [Keywords] NOTNLM ; breast cancer / integrin-linked kinase / recurrence / rhabdomyosarcoma / urokinase-type plasminogen activator
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71. Ebauer M, Wachtel M, Niggli FK, Schäfer BW: Comparative expression profiling identifies an in vivo target gene signature with TFAP2B as a mediator of the survival function of PAX3/FKHR. Oncogene; 2007 Nov 8;26(51):7267-81
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  • The chromosomal translocation t(2;13), characteristic for the aggressive childhood cancer alveolar rhabdomyosarcoma (aRMS), generates the chimeric transcription factor PAX3/FKHR with a well known oncogenic role.

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  • (PMID = 17525748.001).
  • [ISSN] 0950-9232
  • [Journal-full-title] Oncogene
  • [ISO-abbreviation] Oncogene
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors; 0 / TFAP2B protein, human; 0 / Transcription Factor AP-2
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72. Miller PJ, Hollenbach AD: The oncogenic fusion protein Pax3-FKHR has a greater post-translational stability relative to Pax3 during early myogenesis. Biochim Biophys Acta; 2007 Oct;1770(10):1450-8
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  • The childhood solid muscle tumor Alveolar Rhabdomyosarcoma (ARMS) is characterized by the t(2;13)(q35;q14) chromosomal translocation, which results in the fusion of two transcription factors important for myogenesis, Pax3 and FKHR (FOX01a).

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  • (PMID = 17698292.001).
  • [ISSN] 0006-3002
  • [Journal-full-title] Biochimica et biophysica acta
  • [ISO-abbreviation] Biochim. Biophys. Acta
  • [Language] ENG
  • [Grant] United States / NCRR NIH HHS / RR / RR020152-010001; United States / NCRR NIH HHS / RR / P20 RR020152-010001; United States / NCRR NIH HHS / RR / P20 RR020152; United States / NCRR NIH HHS / RR / 1-P20-RR020152-01; United States / NCRR NIH HHS / RR / RR020152-020001; United States / NCI NIH HHS / CA / R01 CA138656; United States / NCRR NIH HHS / RR / P20 RR020152-020001; United States / NCRR NIH HHS / RR / P20 RR020152-037526; United States / NCRR NIH HHS / RR / RR020152-037526
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human; 0 / Paired Box Transcription Factors; 138016-91-8 / Pax3 protein, mouse
  • [Other-IDs] NLM/ NIHMS31151; NLM/ PMC2043499
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73. Piersigilli F, Danhaive O, Auriti C: Blueberry muffin baby due to alveolar rhabdomyosarcoma with cutaneous metastasis. Arch Dis Child Fetal Neonatal Ed; 2010 Nov;95(6):F461
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Blueberry muffin baby due to alveolar rhabdomyosarcoma with cutaneous metastasis.
  • [MeSH-major] Rhabdomyosarcoma, Alveolar / secondary. Skin Neoplasms / secondary

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  • (PMID = 20547583.001).
  • [ISSN] 1468-2052
  • [Journal-full-title] Archives of disease in childhood. Fetal and neonatal edition
  • [ISO-abbreviation] Arch. Dis. Child. Fetal Neonatal Ed.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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