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1. 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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2. 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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3. Durbin AD, Somers GR, Forrester M, Pienkowska M, Hannigan GE, Malkin D: JNK1 determines the oncogenic or tumor-suppressive activity of the integrin-linked kinase in human rhabdomyosarcoma. J Clin Invest; 2009 Jun;119(6):1558-70
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  • [Title] JNK1 determines the oncogenic or tumor-suppressive activity of the integrin-linked kinase in human rhabdomyosarcoma.
  • Here, we demonstrated that ILK functions as an oncogene in the highly aggressive pediatric sarcoma alveolar rhabdomyosarcoma (ARMS) and as a tumor suppressor in the related embryonal rhabdomyosarcoma (ERMS).
  • Ectopic expression of the fusion gene characteristic of ARMS (paired box 3-forkhead homolog in rhabdomyosarcoma [PAX3-FKHR]) in ERMS cells was sufficient to convert them to an ARMS signaling phenotype and render ILK activity oncogenic.
  • These findings indicate what we believe to be a novel effector pathway regulated by ILK, provide a mechanism for interconversion of oncogenic and tumor-suppressor functions of a single regulatory protein based on the genetic background of the tumor cells, and suggest a rationale for tailored therapy of rhabdomyosarcoma based on the different activities of ILK.
  • [MeSH-major] Mitogen-Activated Protein Kinase 8 / metabolism. Oncogene Proteins / metabolism. Protein-Serine-Threonine Kinases / metabolism. Rhabdomyosarcoma / enzymology. Tumor Suppressor Proteins / metabolism

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  • (PMID = 19478459.001).
  • [ISSN] 1558-8238
  • [Journal-full-title] The Journal of clinical investigation
  • [ISO-abbreviation] J. Clin. Invest.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Oncogene Proteins; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human; 0 / RNA, Small Interfering; 0 / Tumor Suppressor Proteins; EC 2.7.1.- / integrin-linked kinase; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 2.7.11.24 / Mitogen-Activated Protein Kinase 8
  • [Other-IDs] NLM/ PMC2689127
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4. Tanyous GH: Rhabdomyosarcoma of the nasal vestibule in a child. Sultan Qaboos Univ Med J; 2006 Dec;6(2):87-9
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  • [Title] Rhabdomyosarcoma of the nasal vestibule in a child.
  • A case of rhabdomyosarcoma in a 2 year old girl without a pre-existing predisposing factor visited the ENT Department of Sultan Qaboos University Hospital (SQUH).
  • The histopathology report was alveolar rhabdomyosarcoma.

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  • (PMID = 21748141.001).
  • [ISSN] 2075-051X
  • [Journal-full-title] Sultan Qaboos University medical journal
  • [ISO-abbreviation] Sultan Qaboos Univ Med J
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Oman
  • [Other-IDs] NLM/ PMC3074915
  • [Keywords] NOTNLM ; Case Report / Nasal rhabdomyosarcoma / Oman / pediatric age
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5. Rao PK, Missiaglia E, Shields L, Hyde G, Yuan B, Shepherd CJ, Shipley J, Lodish HF: Distinct roles for miR-1 and miR-133a in the proliferation and differentiation of rhabdomyosarcoma cells. FASEB J; 2010 Sep;24(9):3427-37
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  • [Title] Distinct roles for miR-1 and miR-133a in the proliferation and differentiation of rhabdomyosarcoma cells.
  • Rhabdomyosarcoma is the most common soft tissue sarcoma in the pediatric population.
  • Levels of miR-1 and miR-133a are drastically reduced in representative cell lines from each major rhabdomyosarcoma subtype (embryonal and alveolar).
  • Introduction of miR-1 and miR-133a into an embryonal rhabdomyosarcoma-derived cell line is cytostatic, thereby suggesting a tumor suppressor-like role for these myogenic miRNAs.
  • More important, these results point to the promise of enhancing rhabdomyosarcoma therapy using miRNAs as agents that mediate cytostasis and promote muscle differentiation.

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  • (PMID = 20466878.001).
  • [ISSN] 1530-6860
  • [Journal-full-title] FASEB journal : official publication of the Federation of American Societies for Experimental Biology
  • [ISO-abbreviation] FASEB J.
  • [Language] ENG
  • [Grant] United States / NIDDK NIH HHS / DK / R01 DK068348; United Kingdom / Cancer Research UK / / C5066/A9541
  • [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 / MicroRNAs
  • [Other-IDs] NLM/ PMC3231107
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6. 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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7. 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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8. 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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9. Kinsey M, Smith R, Lessnick SL: NR0B1 is required for the oncogenic phenotype mediated by EWS/FLI in Ewing's sarcoma. Mol Cancer Res; 2006 Nov;4(11):851-9
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  • A number of solid tumors, such as alveolar rhabdomyosarcoma, synovial sarcoma, and myxoid liposarcoma, are associated with recurrent translocation events that encode fusion proteins.
  • Ewing's sarcoma is a pediatric tumor that serves as a prototype for this tumor class.

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  • (PMID = 17114343.001).
  • [ISSN] 1541-7786
  • [Journal-full-title] Molecular cancer research : MCR
  • [ISO-abbreviation] Mol. Cancer Res.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA096755-05; United States / NCI NIH HHS / CA / K08 CA096755; United States / NCI NIH HHS / CA / K08 CA096755-05; United States / NCI NIH HHS / CA / K08 CA96755
  • [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 / DAX-1 Orphan Nuclear Receptor; 0 / DNA-Binding Proteins; 0 / EWS-FLI fusion protein; 0 / NR0B1 protein, human; 0 / Nr0b1 protein, mouse; 0 / Oncogene Proteins, Fusion; 0 / Proto-Oncogene Protein c-fli-1; 0 / RNA-Binding Protein EWS; 0 / Receptors, Retinoic Acid; 0 / Repressor Proteins; 0 / Transcription Factors
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10. Huh WW, Anderson JR, Rodeberg D, Teot L, Yock T, Raney RB: Orbital sarcoma with metastases at diagnosis: a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group. Pediatr Blood Cancer; 2010 Jul 1;54(7):1045-7
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  • Three patients had embryonal rhabdomyosarcoma (RMS), and two patients each had alveolar RMS or unclassified sarcoma.

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  • [Copyright] Copyright 2010 Wiley-Liss, Inc.
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  • (PMID = 20162686.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U10 CA98413; United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / U10 CA098543-08; None / None / / U10 CA098543-08; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / U10 CA098413-08; None / None / / U10 CA098413-08; United States / NCI NIH HHS / CA / U10 CA98543
  • [Publication-type] Case Reports; Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; Q20Q21Q62J / Cisplatin
  • [Other-IDs] NLM/ NIHMS248174; NLM/ PMC3059586
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11. Cheung MC, Zhuge Y, Yang R, Ogilvie MP, Koniaris LG, Rodríguez MM, Sola JE: Incidence and outcomes of extremity soft-tissue sarcomas in children. J Surg Res; 2010 Oct;163(2):282-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • BACKGROUND: We studied the outcomes of pediatric extremity tumors on a population scale.
  • Most tumors were non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) (79%, n = 879).
  • Most rhabdomyosarcoma (RMS) (n = 220) were alveolar type (n = 140).
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Humans. Incidence. Infant. Infant, Newborn. Male. Multivariate Analysis. Rhabdomyosarcoma / epidemiology. Rhabdomyosarcoma / mortality. SEER Program

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  • [Copyright] Copyright © 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20638678.001).
  • [ISSN] 1095-8673
  • [Journal-full-title] The Journal of surgical research
  • [ISO-abbreviation] J. Surg. Res.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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12. Singh S, Vinson C, Gurley CM, Nolen GT, Beggs ML, Nagarajan R, Wagner EF, Parham DM, Peterson CA: Impaired Wnt signaling in embryonal rhabdomyosarcoma cells from p53/c-fos double mutant mice. Am J Pathol; 2010 Oct;177(4):2055-66
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  • [Title] Impaired Wnt signaling in embryonal rhabdomyosarcoma cells from p53/c-fos double mutant mice.
  • Rhabdomyosarcoma is a primitive neoplasm with a poorly understood etiology that exhibits features of fetal skeletal muscle.
  • It represents the most frequent malignant soft tissue sarcoma affecting the pediatric population and is often treated very aggressively.
  • Embryonal rhabdomyosarcoma (ERMS) and alveolar rhabdomyosarcoma constitute the two major subtypes and exhibit different molecular features.
  • Identification of the Wnt2 gene and its overexpression in ERMS cells was confirmed in human rhabdomyosarcoma cell lines and prompted further analysis of the Wnt signaling pathway.

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  • (PMID = 20829439.001).
  • [ISSN] 1525-2191
  • [Journal-full-title] The American journal of pathology
  • [ISO-abbreviation] Am. J. Pathol.
  • [Language] ENG
  • [Grant] United States / NIA NIH HHS / AG / R01 AG020941; United States / NIA NIH HHS / AG / AG20941
  • [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 / RNA, Messenger; 0 / Transcription Factor AP-1; 0 / Tumor Suppressor Protein p53; 0 / Wnt Proteins; 0 / beta Catenin; EC 1.13.12.- / Luciferases
  • [Other-IDs] NLM/ PMC2947299
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13. McDonald MW, Esiashvili N, George BA, Katzenstein HM, Olson TA, Rapkin LB, Marcus RB Jr: Intensity-modulated radiotherapy with use of cone-down boost for pediatric head-and-neck rhabdomyosarcoma. Int J Radiat Oncol Biol Phys; 2008 Nov 1;72(3):884-91
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Intensity-modulated radiotherapy with use of cone-down boost for pediatric head-and-neck rhabdomyosarcoma.
  • PURPOSE: To report our initial experience using intensity-modulated radiotherapy (IMRT) with a cone-down boost for pediatric head-and-neck rhabdomyosarcoma (RMS).
  • CONCLUSIONS: Our preliminary follow-up of pediatric head-and-neck RMS patients treated with IMRT revealed excellent local control.
  • [MeSH-major] Head and Neck Neoplasms / radiotherapy. Radiotherapy, Intensity-Modulated / methods. Rhabdomyosarcoma, Alveolar / radiotherapy

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  • (PMID = 18455321.001).
  • [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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14. 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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15. Barr FG, Duan F, Smith LM, Gustafson D, Pitts M, Hammond S, Gastier-Foster JM: Genomic and clinical analyses of 2p24 and 12q13-q14 amplification in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group. Genes Chromosomes Cancer; 2009 Aug;48(8):661-72
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  • [Title] Genomic and clinical analyses of 2p24 and 12q13-q14 amplification in alveolar rhabdomyosarcoma: a report from the Children's Oncology Group.
  • Alveolar rhabdomyosarcoma (ARMS) is an aggressive pediatric cancer that is related to the skeletal muscle lineage and characterized by recurrent chromosomal translocations.

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  • (PMID = 19422036.001).
  • [ISSN] 1098-2264
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA81659; United States / NCI NIH HHS / CA / CA24507; United States / NCI NIH HHS / CA / R01 CA104896; United States / NCI NIH HHS / CA / CA104896; United States / NCI NIH HHS / CA / U01 CA054021; United States / NCI NIH HHS / CA / CA081659-02; United States / NCI NIH HHS / CA / U10 CA081659-02; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / R01 CA104896-04; United States / NCI NIH HHS / CA / U10 CA024507; United States / NCI NIH HHS / CA / U10 CA98543; United States / NCI NIH HHS / CA / CA054021
  • [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 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / MYCN protein, human; 0 / Nuclear Proteins; 0 / Oncogene Proteins; 0 / PAX3 protein, human; 0 / PAX7 Transcription Factor; 0 / PAX7 protein, human; 0 / Paired Box Transcription Factors; EC 2.7.11.22 / CDK4 protein, human; EC 2.7.11.22 / Cyclin-Dependent Kinase 4; EC 3.6.1.- / DDX1 protein, human; EC 3.6.4.13 / DEAD-box RNA Helicases
  • [Other-IDs] NLM/ NIHMS138256; NLM/ PMC2739400
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16. 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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17. Goldstein M, Meller I, Orr-Urtreger A: FGFR1 over-expression in primary rhabdomyosarcoma tumors is associated with hypomethylation of a 5' CpG island and abnormal expression of the AKT1, NOG, and BMP4 genes. Genes Chromosomes Cancer; 2007 Nov;46(11):1028-38
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  • [Title] FGFR1 over-expression in primary rhabdomyosarcoma tumors is associated with hypomethylation of a 5' CpG island and abnormal expression of the AKT1, NOG, and BMP4 genes.
  • Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma likely results from abnormal proliferation and differentiation during skeletal myogenesis.
  • Multiple genetic alterations are associated with the three RMS histopathological subtypes, embryonal, alveolar, and pleomorphic adult variant.
  • [MeSH-major] Bone Morphogenetic Proteins / genetics. Carrier Proteins / genetics. CpG Islands. DNA Methylation. Proto-Oncogene Proteins c-akt / genetics. Receptor, Fibroblast Growth Factor, Type 1 / genetics. Rhabdomyosarcoma / genetics

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  • [Copyright] Copyright (c) 2007 Wiley-Liss, Inc.
  • (PMID = 17696196.001).
  • [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 / BMP4 protein, human; 0 / Bone Morphogenetic Protein 4; 0 / Bone Morphogenetic Proteins; 0 / Carrier Proteins; 0 / DNA Primers; 148294-77-3 / noggin protein; EC 2.7.10.1 / FGFR1 protein, human; EC 2.7.10.1 / Receptor, Fibroblast Growth Factor, Type 1; EC 2.7.11.1 / AKT1 protein, human; EC 2.7.11.1 / Proto-Oncogene Proteins c-akt
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18. Antillon F, Castellanos M, Valverde P, Luna-Fineman S, Garrido C, Serrato T, Rodriguez-Galindo C, Casanova M, Ferrari A: Treating Pediatric soft tissue sarcomas in a country with limited resources: the experience of the Unidad Nacional de Oncologia Pediatrica in Guatemala. Pediatr Blood Cancer; 2008 Dec;51(6):760-4
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  • [Title] Treating Pediatric soft tissue sarcomas in a country with limited resources: the experience of the Unidad Nacional de Oncologia Pediatrica in Guatemala.
  • PATIENTS AND METHODS: We reviewed the clinical data, treatment and outcome of 80 patients, 47 cases of rhabdomyosarcoma (RMS) and 33 of non-rhabdomyosarcoma soft tissue sarcoma (NRSTS), treated between January 2000 and October 2007.
  • [MeSH-major] Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Developing Countries. Rhabdomyosarcoma, Alveolar / drug therapy. Rhabdomyosarcoma, Embryonal / drug therapy. Soft Tissue Neoplasms / drug therapy

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  • (PMID = 18680154.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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19. Suita S, Noguchi S, Takamatsu H, Mizote H, Nagasaki A, Inomata Y, Hara T, Okamura J, Miyazaki S, Kawakami K, Eguchi H, Tsuneyoshi M, Committee for Pediatric Solid Malignant Tumors in the Kyushu Area: Clinical characteristics and the prognosis of rhabdomyosarcoma - a report from the Study Group for Pediatric Solid Malignant Tumors in the Kyushu Area, Japan. Eur J Pediatr Surg; 2005 Dec;15(6):409-13
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Clinical characteristics and the prognosis of rhabdomyosarcoma - a report from the Study Group for Pediatric Solid Malignant Tumors in the Kyushu Area, Japan.
  • AIM: There have been no nationwide group studies for patients with rhabdomyosarcoma in Japan.
  • PATIENTS AND METHODS: From 1982 to 1996, 79 rhabdomyosarcomas were registered by the Study Group for Pediatric Solid Malignant Tumors in the Kyushu Area.
  • The staging was done according to the Intergroup Rhabdomyosarcoma Study (IRS) Clinical Grouping Classification.
  • 3) histology: 35.8 % for the embryonal type, 36.8 % for the alveolar type.
  • To improve outcomes, a new nationwide group study for rhabdomyosarcoma, which we belong to, has just started in Japan.
  • [MeSH-major] Head and Neck Neoplasms / mortality. Rhabdomyosarcoma / mortality

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  • (PMID = 16418958.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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20. 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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21. 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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22. Sartori F, Alaggio R, Zanazzo G, Garaventa A, Di Cataldo A, Carli M, Rosolen A, AIEOP Comitato Strategico de Studio-Sarcomi: Results of a prospective minimal disseminated disease study in human rhabdomyosarcoma using three different molecular markers. Cancer; 2006 Apr 15;106(8):1766-75
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Results of a prospective minimal disseminated disease study in human rhabdomyosarcoma using three different molecular markers.
  • BACKGROUND: Rhabdomyosarcoma (RMS) has 2 major histologic subtypes: alveolar (ARMS) and embryonal (ERMS).
  • METHODS: We determined the sensitivity and specificity of MyoD1, myogenin, and PAX-FKHR transcripts as RMS markers and used them to study prospectively by reverse-transcriptase polymerase chain reaction (RT-PCR) a series of consecutive unselected RMS patients enrolled in the Italian Association of Pediatric Hematology and Oncology national trial.
  • [MeSH-major] Rhabdomyosarcoma / diagnosis. Rhabdomyosarcoma / secondary
  • [MeSH-minor] Biomarkers, Tumor / analysis. Bone Marrow Neoplasms / diagnosis. Bone Marrow Neoplasms / secondary. Child. Forkhead Transcription Factors / analysis. Humans. Immunohistochemistry. MyoD Protein / analysis. Myogenin / analysis. Paired Box Transcription Factors / analysis. Reverse Transcriptase Polymerase Chain Reaction. Rhabdomyosarcoma, Alveolar / chemistry. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / secondary. Rhabdomyosarcoma, Embryonal / chemistry. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / secondary. Sensitivity and Specificity

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  • [Copyright] 2006 American Cancer Society
  • (PMID = 16544315.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] eng
  • [Publication-type] Journal Article; 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 / MyoD Protein; 0 / MyoD1 myogenic differentiation protein; 0 / Myogenin; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors
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23. Rodeberg DA, Erskine C, Celis E: In vitro induction of immune responses to shared tumor-associated antigens in rhabdomyosarcoma. J Pediatr Surg; 2007 Aug;42(8):1396-402
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  • [Title] In vitro induction of immune responses to shared tumor-associated antigens in rhabdomyosarcoma.
  • PURPOSE: Currently, novel therapies to improve survival of patients with rhabdomyosarcoma (RMS) are being investigated.
  • METHODS: Rhabdomyosarcoma tumor lysate-pulsed human dendritic cells were used to stimulate HTL precursors (naive CD4+ T-cells) in vitro.
  • The HTLs were induced using lysate from a single alveolar RMS tumor cell line (RMS13).
  • The clones generated recognized all of the alveolar RMS cell lines (RMS13, Rh18, Rh28, Rh30, and Rh41), prostate cancer cell lines (LNCAP and LAPC4), melanoma cell lines (Mel 624 and G361), and breast cancer cell line (SKBR3).
  • [MeSH-major] Antigens, Neoplasm / immunology. CD4-Positive T-Lymphocytes / immunology. Cancer Vaccines / immunology. Dendritic Cells / immunology. Rhabdomyosarcoma / immunology

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  • (PMID = 17706503.001).
  • [ISSN] 1531-5037
  • [Journal-full-title] Journal of pediatric surgery
  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P50CA91956; United States / NCI NIH HHS / CA / R01CA80782
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Neoplasm; 0 / Cancer Vaccines
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24. Myers AL, Williams RF, Ng CY, Hartwich JE, Davidoff AM: Bevacizumab-induced tumor vessel remodeling in rhabdomyosarcoma xenografts increases the effectiveness of adjuvant ionizing radiation. J Pediatr Surg; 2010 Jun;45(6):1080-5
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  • [Title] Bevacizumab-induced tumor vessel remodeling in rhabdomyosarcoma xenografts increases the effectiveness of adjuvant ionizing radiation.
  • We tested this hypothesis using bevacizumab, an anti-VEGF antibody, in rhabdomyosarcoma (RMS) xenografts.
  • METHODS: Mice bearing orthotopic alveolar RMS xenografts were treated with a single dose of bevacizumab, IR, or a combination of the two on different schedules.

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
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  • [ISO-abbreviation] J. Pediatr. Surg.
  • [Language] ENG
  • [Grant] United States / PHS HHS / / 21766; United States / NCI NIH HHS / CA / CA23099; United States / NCI NIH HHS / CA / P30 CA021765-29; United States / NCI NIH HHS / CA / P01 CA023099-290014; None / None / / P30 CA021765-29; United States / NCI NIH HHS / CA / CA023099-290014; United States / NCI NIH HHS / CA / P01 CA023099; United States / NCI NIH HHS / CA / P30 CA021765
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / Antibodies, Monoclonal; 0 / Antibodies, Monoclonal, Humanized; 0 / Antigens, CD34; 0 / Vascular Endothelial Growth Factor A; 2S9ZZM9Q9V / Bevacizumab
  • [Other-IDs] NLM/ NIHMS182905; NLM/ PMC2904306
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25. Lazaridou MN, Nabili S, Lavy T: Orbital rhabdomyosarcoma masquerading as a mucocele. J Pediatr Ophthalmol Strabismus; 2008 Sep-Oct;45(5):306-8
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  • [Title] Orbital rhabdomyosarcoma masquerading as a mucocele.
  • The authors describe a case of orbital rhabdomyosarcoma masquerading as a lacrimal mucocele in a newborn infant.
  • Rhabdomyosarcoma is one of the few life-threatening diseases that an ophthalmologist may be the first to diagnose.
  • [MeSH-major] Lacrimal Apparatus Diseases / diagnosis. Mucocele / diagnosis. Orbital Neoplasms / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis

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  • (PMID = 18825904.001).
  • [ISSN] 0191-3913
  • [Journal-full-title] Journal of pediatric ophthalmology and strabismus
  • [ISO-abbreviation] J Pediatr Ophthalmol Strabismus
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor
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26. Kayton ML, Meyers P, Wexler LH, Gerald WL, LaQuaglia MP: Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults. J Pediatr Surg; 2006 Jan;41(1):187-93
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  • [Title] Clinical presentation, treatment, and outcome of alveolar soft part sarcoma in children, adolescents, and young adults.
  • PURPOSE: Alveolar soft part sarcoma is a rare soft tissue neoplasm that can affect children and adolescents.
  • METHODS: After institutional review board approval, we examined the records of all patients younger than 25 years old who received treatment at our institution for alveolar soft part sarcoma in the past 30 years.
  • Younger patients tended to have Intergroup Rhabdomyosarcoma Study group I disease.
  • CONCLUSIONS: Achievement of complete microscopic resection is critical in localized alveolar soft part sarcoma, but incomplete excision and misdiagnosis are often encountered.


27. Cecchetto G, Carretto E, Bisogno G, Dall'Igna P, Ferrari A, Scarzello G, Donfrancesco A, Alaggio R, Indolfi P, Carli M: Complete second look operation and radiotherapy in locally advanced non-alveolar rhabdomyosarcoma in children: A report from the AIEOP soft tissue sarcoma committee. Pediatr Blood Cancer; 2008 Nov;51(5):593-7
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  • [Title] Complete second look operation and radiotherapy in locally advanced non-alveolar rhabdomyosarcoma in children: A report from the AIEOP soft tissue sarcoma committee.
  • BACKGROUND: To evaluate the effect of radiotherapy (RT) in association with complete second look operation, histologically confirmed, on outcome of patients with IRS Gr.III non-alveolar RMS.
  • Although the limited number of patients does not allow statistically significant conclusions, our experience suggests that RT may have a positive influence on local control for completely resected non-alveolar RMS.
  • [MeSH-major] Rhabdomyosarcoma / radiotherapy. Rhabdomyosarcoma / surgery. Second-Look Surgery

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18668515.001).
  • [ISSN] 1545-5017
  • [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
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28. Gallego S, Llort A, Roma J, Sabado C, Gros L, de Toledo JS: Detection of bone marrow micrometastasis and microcirculating disease in rhabdomyosarcoma by a real-time RT-PCR assay. J Cancer Res Clin Oncol; 2006 Jun;132(6):356-62
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  • [Title] Detection of bone marrow micrometastasis and microcirculating disease in rhabdomyosarcoma by a real-time RT-PCR assay.
  • PURPOSE: To assess if molecular detection of minimal disseminated disease by real-time reverse transcription and polymerase chain reaction (RT-PCR) could contribute to a better treatment stratification in patients with rhabdomyosarcoma (RMS).
  • METHODS: Relative quantification of the tumor-mRNA present in serial samples of bone marrow (BM) and peripheral blood (PB) from 16 patients with RMS (7 alveolar and 9 embryonal) was performed by a real-time RT-PCR assay.
  • Expression of MyoD1 and acetylcholine receptor (AChR) was analyzed in all samples, along with PAX3/7-FKHR in samples from alveolar tumors.
  • [MeSH-major] Bone Marrow Neoplasms / diagnosis. Bone Marrow Neoplasms / secondary. Neoplastic Cells, Circulating. Reverse Transcriptase Polymerase Chain Reaction / methods. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Embryonal / diagnosis

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  • [Cites] Bone Marrow Transplant. 1999 Sep;24(5):527-33 [10482938.001]
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  • (PMID = 16435141.001).
  • [ISSN] 0171-5216
  • [Journal-full-title] Journal of cancer research and clinical oncology
  • [ISO-abbreviation] J. Cancer Res. Clin. Oncol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / MyoD Protein; 0 / MyoD1 myogenic differentiation protein; 0 / Oncogene Proteins, Fusion; 0 / RNA, Messenger; 0 / Receptors, Nicotinic
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29. Sullivan LM, Atkins KA, LeGallo RD: PAX immunoreactivity identifies alveolar rhabdomyosarcoma. Am J Surg Pathol; 2009 May;33(5):775-80
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  • [Title] PAX immunoreactivity identifies alveolar rhabdomyosarcoma.
  • As such, we sought to assess it as a diagnostic marker in the evaluation of pediatric small round blue cell tumors.
  • Tumors selected for evaluation included embryonal rhabdomyosarcoma (55 cases), alveolar rhabdomyosarcoma (ARMS) (51 cases), neuroblastoma (22 cases), Wilms tumor (18 cases), Ewing Family of Tumors (11 cases), lymphoblastic lymphoma (8 cases), hepatoblastoma (6 cases), and granulocytic sarcoma (3 cases) as either cores in a tissue microarray or whole mount sections.
  • Of the rhabdomyosarcoma cases, 34 of 51 (67%) ARMS were immunoreactive whereas none of the 55 embryonal rhabdomyosarcoma cases stained.
  • [MeSH-major] B-Cell-Specific Activator Protein / analysis. Rhabdomyosarcoma, Alveolar / chemistry
  • [MeSH-minor] Adolescent. Bone Neoplasms / chemistry. Child. Child, Preschool. Gene Expression Regulation, Neoplastic. Hepatoblastoma / chemistry. Humans. Immunohistochemistry. Infant. Infant, Newborn. Kidney Neoplasms / chemistry. Liver Neoplasms / chemistry. Neuroblastoma / chemistry. Precursor Cell Lymphoblastic Leukemia-Lymphoma / metabolism. Predictive Value of Tests. Retrospective Studies. Rhabdomyosarcoma, Embryonal / chemistry. Sarcoma, Ewing / chemistry. Sarcoma, Myeloid / metabolism. Tissue Array Analysis. Translocation, Genetic. Wilms Tumor / chemistry

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  • (PMID = 19145202.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / B-Cell-Specific Activator Protein; 0 / PAX5 protein, human
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30. Seifert G, Seeger K, Reindl T, Voelker T, Henze G: A metastatic alveolar rhabdomyosarcoma of the hand. Pediatr Blood Cancer; 2006 Jan;46(1):115-6
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  • [Title] A metastatic alveolar rhabdomyosarcoma of the hand.
  • [MeSH-major] Bone Neoplasms / secondary. Fingers. Rhabdomyosarcoma, Alveolar / secondary. Soft Tissue Neoplasms / pathology

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  • (PMID = 16200631.001).
  • [ISSN] 1545-5009
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] United States
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31. 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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32. Morton CL, Houghton PJ, Kolb EA, Gorlick R, Reynolds CP, Kang MH, Maris JM, Keir ST, Wu J, Smith MA: Initial testing of the replication competent Seneca Valley virus (NTX-010) by the pediatric preclinical testing program. Pediatr Blood Cancer; 2010 Aug;55(2):295-303
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  • [Title] Initial testing of the replication competent Seneca Valley virus (NTX-010) by the pediatric preclinical testing program.
  • RESULTS: In vitro NTX-010 demonstrated a marked cytotoxic effect in a subset of the cell lines from the neuroblastoma, Ewing sarcoma, and rhabdomyosarcoma panels.
  • In vivo the most consistent activity was observed for the rhabdomyosarcoma and the neuroblastoma panels, with all four of the alveolar rhabdomyosarcoma xenografts and four of five neuroblastoma xenografts achieving CR or maintained CR.
  • [MeSH-minor] Animals. Cell Line, Tumor. Disease-Free Survival. Humans. Mice. Neuroblastoma / pathology. Neuroblastoma / therapy. RNA Viruses. Rhabdomyosarcoma / pathology. Rhabdomyosarcoma / therapy. Sarcoma, Ewing / pathology. Sarcoma, Ewing / therapy. Treatment Outcome. Tumor Burden. Xenograft Model Antitumor Assays

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  • [Copyright] (c) 2010 Wiley-Liss, Inc.
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  • (PMID = 20582972.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / P50 CA108786; United States / NCI NIH HHS / CA / CA108786; United States / NCI NIH HHS / CA / CA21765; United States / NCI NIH HHS / CM / N01 CM042216; United States / NCI NIH HHS / CA / P30 CA021765; United States / NCI NIH HHS / CM / N01-CM-42216; United States / NCI NIH HHS / CA / N01CM42216
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS218652; NLM/ PMC3003870
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33. Hanke CA, Roessler J, Stegmaier S, Koscielniak E, Niemeyer CM, Kontny U: Alveolar rhabdomyosarcoma mimicking lymphoma with bone marrow involvement. Eur J Pediatr; 2007 May;166(5):505-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Alveolar rhabdomyosarcoma mimicking lymphoma with bone marrow involvement.
  • [MeSH-major] Bone Marrow Diseases / pathology. Rhabdomyosarcoma, Alveolar / pathology

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  • (PMID = 17008999.001).
  • [ISSN] 0340-6199
  • [Journal-full-title] European journal of pediatrics
  • [ISO-abbreviation] Eur. J. Pediatr.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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34. 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
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  • [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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35. Okamura K, Yamamoto H, Ishimaru Y, Takayasu H, Otani Y, Yamagishi J, Takahashi A, Kuwano H, Nagashima K, Ikeda H: Clinical characteristics and surgical treatment of perianal and perineal rhabdomyosarcoma: analysis of Japanese patients and comparison with IRSG reports. Pediatr Surg Int; 2006 Feb;22(2):129-34
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  • [Title] Clinical characteristics and surgical treatment of perianal and perineal rhabdomyosarcoma: analysis of Japanese patients and comparison with IRSG reports.
  • Twenty-nine patients, 26 patients identified in the Japanese literature and three of our own, were analyzed and the results were compared with the data reported from the Intergroup Rhabdomyosarcoma Study Group (IRSG).
  • Alveolar histology was diagnosed in 18 patients.
  • In patients more than 10 years of age, the female predominance was more prominent and the incidences of advanced clinical groups/stages and alveolar histology were significantly higher than those in patients younger than 10 years of age.
  • [MeSH-major] Anus Neoplasms / surgery. Perineum. Rhabdomyosarcoma / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 16308704.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Comparative Study; Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 36
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36. Missiaglia E, Selfe J, Hamdi M, Williamson D, Schaaf G, Fang C, Koster J, Summersgill B, Messahel B, Versteeg R, Pritchard-Jones K, Kool M, Shipley J: Genomic imbalances in rhabdomyosarcoma cell lines affect expression of genes frequently altered in primary tumors: an approach to identify candidate genes involved in tumor development. Genes Chromosomes Cancer; 2009 Jun;48(6):455-67
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  • [Title] Genomic imbalances in rhabdomyosarcoma cell lines affect expression of genes frequently altered in primary tumors: an approach to identify candidate genes involved in tumor development.
  • Rhabdomyosarcomas (RMS) are the most common pediatric soft tissue sarcomas.
  • They resemble developing skeletal muscle and are histologically divided into two main subtypes; alveolar and embryonal RMS.
  • Characteristic genomic aberrations, including the PAX3- and PAX7-FOXO1 fusion genes in alveolar cases, have led to increased understanding of their molecular biology.
  • Copy number and expression of FGFR1 was validated in additional primary material and found amplified in 6 out of 196 cases and overexpressed relative to skeletal muscle and myoblasts, with significantly higher expression levels in the embryonal compared with alveolar subtypes.
  • [MeSH-major] Allelic Imbalance. Gene Dosage. Gene Expression Regulation, Neoplastic. Neoplasm Proteins / genetics. Rhabdomyosarcoma / genetics

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  • (PMID = 19235922.001).
  • [ISSN] 1098-2264
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Grant] United Kingdom / Cancer Research UK / /
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Neoplasm Proteins; EC 2.7.10.1 / FGFR1 protein, human; EC 2.7.10.1 / Receptor, Fibroblast Growth Factor, Type 1
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37. 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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38. Casanova M, Meazza C, Favini F, Fiore M, Morosi C, Ferrari A: Rhabdomyosarcoma of the extremities: a focus on tumors arising in the hand and foot. Pediatr Hematol Oncol; 2009 Jul-Aug;26(5):321-31
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  • [Title] Rhabdomyosarcoma of the extremities: a focus on tumors arising in the hand and foot.
  • METHODS: The authors reviewed the clinical data on 60 patients <21 years old with limb RMS treated at the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, in Italy, over a 30-year period.
  • The alveolar subtype was identified in 62% of cases.
  • [MeSH-major] Foot / pathology. Hand / pathology. Rhabdomyosarcoma / mortality. Rhabdomyosarcoma / therapy

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  • (PMID = 19579078.001).
  • [ISSN] 1521-0669
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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39. Williamson D, Lu YJ, Gordon T, Sciot R, Kelsey A, Fisher C, Poremba C, Anderson J, Pritchard-Jones K, Shipley J: Relationship between MYCN copy number and expression in rhabdomyosarcomas and correlation with adverse prognosis in the alveolar subtype. J Clin Oncol; 2005 Feb 1;23(4):880-8
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  • [Title] Relationship between MYCN copy number and expression in rhabdomyosarcomas and correlation with adverse prognosis in the alveolar subtype.
  • Increased copy number and overexpression of MYCN in the pediatric cancer rhabdomyosarcoma has been described in a number of small studies with conflicting conclusions about its association with clinicopathologic characteristics.
  • PATIENTS AND METHODS: Using quantitative polymerase chain reaction, we measured MYCN copy number and expression levels in rhabdomyosarcoma samples from 113 and 92 individuals with a confirmed diagnosis of rhabdomyosarcoma, respectively.
  • RESULTS: Increased copy number of MYCN was found to be a feature of both the embryonal and alveolar subtypes.
  • The copy number and expression levels were significantly greater in the alveolar subtype, although the range of expression in both subtypes spanned several orders of magnitude.
  • MYCN copy number showed a significant correlation with expression in the alveolar subtype; this relationship between copy number and expression could be modeled as a logarithmic function.
  • It is notable that relatively high expression frequently occurred in embryonal rhabdomyosarcoma without high copy number and that low expression was found in some cases with high copy number.
  • In patients with alveolar rhabdomyosarcoma, overexpression (greater than median) or gain of genomic copies of MYCN were significantly associated with adverse outcome.
  • CONCLUSION: MYCN deregulation is a feature of rhabdomyosarcoma tumorigenesis, defines groups of patients with a poor prognosis, and is a potential target for novel therapies.
  • [MeSH-major] Gene Dosage. Genes, myc. Rhabdomyosarcoma / genetics

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  • (PMID = 15681534.001).
  • [ISSN] 0732-183X
  • [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] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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40. Ganesan P, Thulkar S, Rajan A, Bakhshi S: Solid variant of alveolar rhabdomyosarcoma mimicking non-Hodgkin lymphoma: case report and review of literature. J Pediatr Hematol Oncol; 2008 Oct;30(10):772-4
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  • [Title] Solid variant of alveolar rhabdomyosarcoma mimicking non-Hodgkin lymphoma: case report and review of literature.
  • Alveolar rhabdomyosarcoma is a high-grade neoplasm, which forms about 30% of rhabdomyosarcomas.
  • A histopathologic diagnosis of solid variant of alveolar rhabdomyosarcoma was made.
  • The pediatric and adolescent cases of this rare tumor reported in English language literature are reviewed.
  • [MeSH-major] Lymphoma, Non-Hodgkin / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis


41. Goldstein M, Meller I, Issakov J, Orr-Urtreger A: Novel genes implicated in embryonal, alveolar, and pleomorphic rhabdomyosarcoma: a cytogenetic and molecular analysis of primary tumors. Neoplasia; 2006 May;8(5):332-43
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  • [Title] Novel genes implicated in embryonal, alveolar, and pleomorphic rhabdomyosarcoma: a cytogenetic and molecular analysis of primary tumors.
  • Rhabdomyosarcoma, the most common pediatric soft tissue sarcoma, likely results from deregulation of the skeletal myogenesis program.
  • Using a combined approach of spectral karyotyping, array-based comparative genomic hybridization (CGH), and expression analysis, we examined 10 primary RMS tumors, including embryonal, alveolar, and the rare adult pleomorphic variant, to explore the involvement of different genes and genetic pathways in RMS tumorigenesis.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Genetic Predisposition to Disease. Neoplasms / genetics. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / metabolism. Rhabdomyosarcoma, Embryonal / embryology. Rhabdomyosarcoma, Embryonal / genetics


42. 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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43. van den Broeke LT, Pendleton CD, Mackall C, Helman LJ, Berzofsky JA: Identification and epitope enhancement of a PAX-FKHR fusion protein breakpoint epitope in alveolar rhabdomyosarcoma cells created by a tumorigenic chromosomal translocation inducing CTL capable of lysing human tumors. Cancer Res; 2006 Feb 1;66(3):1818-23
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  • [Title] Identification and epitope enhancement of a PAX-FKHR fusion protein breakpoint epitope in alveolar rhabdomyosarcoma cells created by a tumorigenic chromosomal translocation inducing CTL capable of lysing human tumors.
  • Here, we identify such a fusion protein breakpoint epitope in the PAX-FKHR fusion protein created by the t(2;13) translocation present in 80% of cases of alveolar rhabdomyosarcoma, a highly aggressive pediatric soft-tissue sarcoma.
  • These human peptide-specific CTL lyse human HLA-B7+ rhabdomyosarcoma tumor cells.
  • This epitope-enhanced peptide may serve as a candidate cancer vaccine for HLA-B7+ patients with alveolar rhabdomyosarcoma.
  • [MeSH-major] Epitopes / immunology. Forkhead Transcription Factors / immunology. Immunotherapy, Adoptive / methods. Oncogene Proteins, Fusion / immunology. Paired Box Transcription Factors / immunology. Rhabdomyosarcoma, Alveolar / immunology. T-Lymphocytes, Cytotoxic / immunology

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  • (PMID = 16452243.001).
  • [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 / Epitopes; 0 / Forkhead Transcription Factors; 0 / HLA-B7 Antigen; 0 / Oncogene Proteins, Fusion; 0 / Paired Box Transcription Factors
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44. Mackall CL, Rhee EH, Read EJ, Khuu HM, Leitman SF, Bernstein D, Tesso M, Long LM, Grindler D, Merino M, Kopp W, Tsokos M, Berzofsky JA, Helman LJ: A pilot study of consolidative immunotherapy in patients with high-risk pediatric sarcomas. Clin Cancer Res; 2008 Aug 1;14(15):4850-8
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  • [Title] A pilot study of consolidative immunotherapy in patients with high-risk pediatric sarcomas.
  • PURPOSE: Patients with metastatic or recurrent Ewing's sarcoma family of tumors and alveolar rhabdomyosarcoma have <25% 5-year survival in most studies.
  • EXPERIMENTAL DESIGN: Fifty-two patients with translocation positive, recurrent, or metastatic Ewing's sarcoma family of tumors or alveolar rhabdomyosarcoma underwent prechemotherapy cell harvest via apheresis for potential receipt of immunotherapy.


45. Wang Z, Velagaleti GV, Eltorky MA, Tang WW, Hawkins HK, Jones EA, Northup J, Panova N, Qiu S: Cytogenetic and molecular studies of an unusual case of multiple primary alveolar rhabdomyosarcomas: low-level chromosomal instability and reciprocal translocation t(6;11). Exp Mol Pathol; 2007 Feb;82(1):58-62
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  • [Title] Cytogenetic and molecular studies of an unusual case of multiple primary alveolar rhabdomyosarcomas: low-level chromosomal instability and reciprocal translocation t(6;11).
  • Cytogenetic and molecular studies have shown that approximately 80% of cases of alveolar rhabdomyosarcoma (ARMS) have consistent chromosomal translocation of either t(2;13) or t(1;13), resulting in either PAX3-FKHR or PAX7-FKHR gene fusions.
  • The findings suggest that cytogenetic abnormalities of chromosome 6 may be associated with the development of early onset multiple ARMS in a subgroup of pediatric patients as seen in this case.
  • [MeSH-major] Chromosomes, Human, Pair 6 / genetics. Neoplasms, Multiple Primary / genetics. Neoplasms, Multiple Primary / pathology. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / pathology

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  • (PMID = 17097083.001).
  • [ISSN] 0014-4800
  • [Journal-full-title] Experimental and molecular pathology
  • [ISO-abbreviation] Exp. Mol. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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46. Ehlers JP, Penne RB, Eagle RC Jr, Carrasco JR: Alveolar rhabdomyosarcoma presenting as an acute orbital mass in the medial rectus muscle. Ophthal Plast Reconstr Surg; 2007 Mar-Apr;23(2):149-51
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  • [Title] Alveolar rhabdomyosarcoma presenting as an acute orbital mass in the medial rectus muscle.
  • Rhabdomyosarcoma is the most common pediatric primary neoplasm in the orbit, often presenting with rapid proptosis and orbital symptoms.
  • We describe a 15-year-old girl who presented with an acute mass in her medial rectus muscle that was subsequently diagnosed as widely disseminated alveolar rhabdomyosarcoma.
  • To our knowledge, this represents the first reported case in which an enlarged extraocular muscle was the initial manifestation of disseminated alveolar rhabdomyosarcoma.
  • [MeSH-major] Oculomotor Muscles / pathology. Orbital Neoplasms / pathology. Rhabdomyosarcoma, Alveolar / pathology

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  • (PMID = 17413634.001).
  • [ISSN] 0740-9303
  • [Journal-full-title] Ophthalmic plastic and reconstructive surgery
  • [ISO-abbreviation] Ophthal Plast Reconstr Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Neoplasm Proteins; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors
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47. Leventhal DD, Spiegel J, Keane W: Laryngeal alveolar rhabdomyosarcoma involving the true vocal fold in an adult: Case report. Ear Nose Throat J; 2010 Dec;89(12):E8
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  • [Title] Laryngeal alveolar rhabdomyosarcoma involving the true vocal fold in an adult: Case report.
  • Rhabdomyosarcoma of the larynx is extremely rare in adults, as only 17 well-documented cases have been previously reported in the English-language literature.
  • Of these, only 2 cases (both male) involved the alveolar subtype of rhabdomyosarcoma, and neither involved the true vocal folds.
  • We report a case of alveolar rhabdomyosarcoma of the true vocal fold in 54-year-old woman.
  • Management of head and neck rhabdomyosarcoma has evolved from radical surgery to less morbid procedures supplemented with radiation and chemotherapy.
  • Nevertheless, the success of treatment in the pediatric population supports its use in adults.
  • [MeSH-major] Laryngeal Neoplasms / pathology. Laryngeal Neoplasms / therapy. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Alveolar / therapy. Vocal Cords / pathology

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  • (PMID = 21174270.001).
  • [ISSN] 1942-7522
  • [Journal-full-title] Ear, nose, & throat journal
  • [ISO-abbreviation] Ear Nose Throat J
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] United States
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48. 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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  • 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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49. 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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50. Bouron-Dal Soglio D, Rougemont AL, Absi R, Giroux LM, Sanchez R, Barrette S, Fournet JC: Beta-catenin mutation does not seem to have an effect on the tumorigenesis of pediatric rhabdomyosarcomas. Pediatr Dev Pathol; 2009 Sep-Oct;12(5):371-3
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  • [Title] Beta-catenin mutation does not seem to have an effect on the tumorigenesis of pediatric rhabdomyosarcomas.
  • Involvement of the Wnt signal transduction pathway has been shown in different pediatric embryonal tumors, such as hepatoblastoma, nephroblastoma, pancreatoblastoma, and medulloblastoma.
  • There are few data available on the status of beta-catenin in rhabdomyosarcoma (RMS), another pediatric embryonal tumor.
  • The aims of this study were 1st to verify the status of the exon 3 of CTNNB1 and 2nd to assess the usefulness of beta-catenin immunostaining in a small series of 8 embryonal RMS, 3 alveolar RMS, and 1 sclerosing RMS (SRMS).
  • We conclude that there is no evidence of beta-catenin mutation in the genesis of rhabdomyosarcoma and that beta-catenin does not represent a useful immunomarker to help distinguish between embryonal RMS and alveolar RMS.
  • [MeSH-major] Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Embryonal / genetics. Rhabdomyosarcoma, Embryonal / pathology. beta Catenin / genetics

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  • (PMID = 19222307.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / beta Catenin
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51. Alaggio R, Bisogno G, Rosato A, Ninfo V, Coffin CM: Undifferentiated sarcoma: does it exist? A clinicopathologic study of 7 pediatric cases and review of literature. Hum Pathol; 2009 Nov;40(11):1600-10
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  • [Title] Undifferentiated sarcoma: does it exist? A clinicopathologic study of 7 pediatric cases and review of literature.
  • No translocations associated with Ewing sarcoma, synovial sarcoma, or alveolar rhabdomyosarcoma were found.

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  • (PMID = 19647855.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / BIRC5 protein, human; 0 / Biomarkers, Tumor; 0 / Inhibitor of Apoptosis Proteins; 0 / Microtubule-Associated Proteins
  • [Number-of-references] 35
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52. Verkarre V, Galmiche-Rolland L, Sarnacki S, Jaubert F: Rhabdomyosarcoma an ubiquitous pediatric tumour. Arkh Patol; 2008 May-Jun;70(3):50-3
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  • [Title] Rhabdomyosarcoma an ubiquitous pediatric tumour.
  • Rhabdomyosarcoma is the most common soft tissue tumor in children.
  • It occurs everywhere and its prognosis depends on the location and its histological type--embryonic or alveolar.
  • The new immunohistochemical markers desmin and myogenin in combination with molecular biological detection of specific translocations in alveolar rhabdomyosarcoma improved diagnostic capacities.
  • [MeSH-major] Rhabdomyosarcoma

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  • (PMID = 18727437.001).
  • [ISSN] 0004-1955
  • [Journal-full-title] Arkhiv patologii
  • [ISO-abbreviation] Arkh. Patol.
  • [Language] eng
  • [Publication-type] Lectures
  • [Publication-country] Russia (Federation)
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53. 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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54. Davicioni E, Anderson JR, Buckley JD, Meyer WH, Triche TJ: Gene expression profiling for survival prediction in pediatric rhabdomyosarcomas: a report from the children's oncology group. J Clin Oncol; 2010 Mar 1;28(7):1240-6
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  • [Title] Gene expression profiling for survival prediction in pediatric rhabdomyosarcomas: a report from the children's oncology group.
  • PURPOSE: We investigated whether tumors from diagnostic biopsies of primary rhabdomyosarcoma (RMS) contain relevant prognostic information in the form of gene expression signatures that can be used to model and predict outcome of patients.
  • However, it was correlated with a risk classification used by the Children's Oncology Group and the biologic subsets of alveolar histology tumors.

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  • (PMID = 20124188.001).
  • [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
  • [Grant] United States / NCI NIH HHS / CA / U01 CA114757; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / U01-CA-114757; United States / NCI NIH HHS / CA / U10CA98543
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors
  • [Other-IDs] NLM/ PMC3040045
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55. Arush MW, Kollender Y, Issakov J, Shalom RB, Arieh YB, Malkin L, Postovsky S: Unusual leptomeningeal dissemination in a child with extracranial metastatic alveolar rhabdomyosarcoma. Pediatr Hematol Oncol; 2009 Sep;26(6):473-8
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  • [Title] Unusual leptomeningeal dissemination in a child with extracranial metastatic alveolar rhabdomyosarcoma.
  • The authors describe a 6-year-old boy diagnosed with alveolar rhabdomyosarcoma located in the thigh, with distal metastases to lungs, bones, and bone marrow.
  • This case demonstrates the rapidity with which leptomeningeal spread of extracranial metastatic alveolar rhabdomyosarcoma can occur and underscores the importance of diagnostic lumbar puncture and brain radiological investigations at diagnosis, even when the tumors are not in the parameningeal location.
  • [MeSH-major] Bone Marrow Neoplasms / secondary. Brain Neoplasms / secondary. Lung Neoplasms / secondary. Meningeal Neoplasms / secondary. Rhabdomyosarcoma, Alveolar / secondary. Soft Tissue Neoplasms / pathology


56. Kuroiwa M, Sakamoto J, Shimada A, Suzuki N, Hirato J, Park MJ, Sotomatsu M, Hayashi Y: Manifestation of alveolar rhabdomyosarcoma as primary cutaneous lesions in a neonate with Beckwith-Wiedemann syndrome. J Pediatr Surg; 2009 Mar;44(3):e31-5
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  • [Title] Manifestation of alveolar rhabdomyosarcoma as primary cutaneous lesions in a neonate with Beckwith-Wiedemann syndrome.
  • We report a rare case of neonatal Beckwith-Wiedemann syndrome (BWS) associated with alveolar rhabdomyosarcoma (RMS).
  • Alveolar RMS was diagnosed on the basis of excisional biopsy.
  • Thus, neonatal alveolar RMS with BWS may result from an alternate molecular pathway.
  • [MeSH-major] Beckwith-Wiedemann Syndrome / complications. Rhabdomyosarcoma, Alveolar / complications. Rhabdomyosarcoma, Alveolar / diagnosis. Skin Neoplasms / complications


57. 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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58. Paulino AC, Pappo A: Alveolar rhabdomyosarcoma of the extremity and nodal metastasis: Is the in-transit lymphatic system at risk? Pediatr Blood Cancer; 2009 Dec 15;53(7):1332-3
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  • [Title] Alveolar rhabdomyosarcoma of the extremity and nodal metastasis: Is the in-transit lymphatic system at risk?
  • Alveolar rhabdomyosarcoma (RMS) of the extremity is not infrequently associated with regional node metastasis.
  • In this report we describe two patients with alveolar RMS of the lower extremity with inguinal metastasis at presentation.
  • The in-transit lymphatics can be a site of failure in children with alveolar RMS of the extremity and nodal involvement.
  • [MeSH-major] Lymphatic Metastasis / physiopathology. Rhabdomyosarcoma, Alveolar / secondary. Soft Tissue Neoplasms / pathology

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  • [Copyright] (c) 2009 Wiley-Liss, Inc.
  • (PMID = 19711439.001).
  • [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
  • [Chemical-registry-number] 0 / Benzenesulfonates; 0 / Phenylurea Compounds; 0 / Pyridines; 1CC1JFE158 / Dactinomycin; 24R60NVC41 / cositecan; 25X51I8RD4 / Niacinamide; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 7673326042 / irinotecan; 7GR28W0FJI / Dacarbazine; 85622-93-1 / temozolomide; 8N3DW7272P / Cyclophosphamide; 9ZOQ3TZI87 / sorafenib; UM20QQM95Y / Ifosfamide; XT3Z54Z28A / Camptothecin
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59. Charytonowicz E, Cordon-Cardo C, Matushansky I, Ziman M: Alveolar rhabdomyosarcoma: is the cell of origin a mesenchymal stem cell? Cancer Lett; 2009 Jul 8;279(2):126-36
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  • [Title] Alveolar rhabdomyosarcoma: is the cell of origin a mesenchymal stem cell?
  • Alveolar rhabdomyosarcoma (ARMS) is a pediatric sarcoma that typically occurs in older children predominantly arising in the trunk and extremities, and exhibits a worse prognosis than other types of rhabdomyosarcomas.
  • [MeSH-major] Mesenchymal Stromal Cells / pathology. Rhabdomyosarcoma, Alveolar / metabolism. Rhabdomyosarcoma, Alveolar / pathology

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  • (PMID = 19008039.001).
  • [ISSN] 1872-7980
  • [Journal-full-title] Cancer letters
  • [ISO-abbreviation] Cancer Lett.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Ireland
  • [Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / Paired Box Transcription Factors
  • [Number-of-references] 100
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60. Nikas I, Theofanopoulou M, Lampropoulou P, Pourtsidis A, Hadjigeorgi C, Kosmidis H: Optic pathway glioma associated with orbital rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia in a child with neurofibromatosis-1. Pediatr Radiol; 2006 Nov;36(11):1200-3
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  • [Title] Optic pathway glioma associated with orbital rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia in a child with neurofibromatosis-1.
  • We present a 2(1/2)-year-old boy with NF-1 who demonstrated coexisting optic pathway glioma with involvement of the chiasm and optic nerve, orbital alveolar rhabdomyosarcoma and bilateral optic nerve sheath dural ectasia.
  • [MeSH-major] Dilatation, Pathologic / diagnostic imaging. Glioma / diagnostic imaging. Neurofibromatoses / diagnostic imaging. Optic Nerve Neoplasms / diagnostic imaging. Orbital Neoplasms / diagnostic imaging. Rhabdomyosarcoma / diagnostic imaging


61. 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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62. Morris CL, Mukundan S Jr, Heimann A, Cummings TJ, Chesnutt DA: Stage IV primitive-appearing sinus and orbital rhabdomyosarcoma presenting in a 68-year-old female previously treated for breast cancer. Orbit; 2008;27(1):73-7
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  • [Title] Stage IV primitive-appearing sinus and orbital rhabdomyosarcoma presenting in a 68-year-old female previously treated for breast cancer.
  • A 68-year-old female who had undergone treatment several years previously for breast cancer presented with diplopia and unilateral proptosis and exposure keratopathy related to biopsy-proven rhabdomyosarcoma of the sinus and orbit.
  • Histopathologic examination showed primitive-appearing rhabdomyosarcoma with some features suggestive of the alveolar subtype.
  • Orbital or sinus rhabdomyosarcoma is seen almost exclusively in the pediatric population, but may very rarely occur in adults.
  • There are several genetic mutations that appear to play a role in both rhabdomyosarcoma and certain breast tumors.
  • In our patient with atypical demographics for rhabdomyosarcoma, the previous neoplasm and treatment thereof may have predisposed to the development of this rare tumor.
  • [MeSH-major] Maxillary Sinus Neoplasms / pathology. Orbital Neoplasms / pathology. Rhabdomyosarcoma / pathology

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  • (PMID = 18307153.001).
  • [ISSN] 1744-5108
  • [Journal-full-title] Orbit (Amsterdam, Netherlands)
  • [ISO-abbreviation] Orbit
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 7GR28W0FJI / Dacarbazine; 80168379AG / Doxorubicin; NR7O1405Q9 / Mesna; UM20QQM95Y / Ifosfamide; MAID protocol
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63. Heerema-McKenney A, Wijnaendts LC, Pulliam JF, Lopez-Terrada D, McKenney JK, Zhu S, Montgomery K, Mitchell J, Marinelli RJ, Hart AA, van de Rijn M, Linn SC: Diffuse myogenin expression by immunohistochemistry is an independent marker of poor survival in pediatric rhabdomyosarcoma: a tissue microarray study of 71 primary tumors including correlation with molecular phenotype. Am J Surg Pathol; 2008 Oct;32(10):1513-22
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  • [Title] Diffuse myogenin expression by immunohistochemistry is an independent marker of poor survival in pediatric rhabdomyosarcoma: a tissue microarray study of 71 primary tumors including correlation with molecular phenotype.
  • The pathologic classification of rhabdomyosarcoma (RMS) into embryonal or alveolar subtype is an important prognostic factor guiding the therapeutic protocol chosen for an individual patient.
  • The aim of this study is to identify immunohistochemical markers of potential prognostic significance in pediatric RMS and to correlate their expression with PAX-3/FKHR and PAX-7/FKHR fusion status.
  • A single tissue microarray containing 71 paraffin-embedded pediatric RMSs was immunostained with antibodies against p53, bcl-2, Ki-67, CD44, myogenin, and MyoD1.
  • After adjustment for Intergroup Rhabdomyosarcoma Study-TNM stage, tumor site, age, tumor histology, and translocation status by multivariable analysis, only myogenin retained an independent association with RFI (P=0.034) and OS (P=0.0069).
  • [MeSH-major] Biomarkers, Tumor / analysis. Immunohistochemistry. Myogenin / analysis. Rhabdomyosarcoma, Alveolar / chemistry. Rhabdomyosarcoma, Embryonal / chemistry. Tissue Array Analysis

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  • (PMID = 18708938.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, CD44; 0 / Biomarkers, Tumor; 0 / CD44 protein, human; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Ki-67 Antigen; 0 / MYOG protein, human; 0 / MyoD Protein; 0 / MyoD1 myogenic differentiation protein; 0 / Myogenin; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human; 0 / PAX7 Transcription Factor; 0 / PAX7 protein, human; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / TP53 protein, human; 0 / Tumor Suppressor Protein p53
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64. Bouron-Dal Soglio D, Rougemont AL, Absi R, Barrette S, Montpetit A, Fetni R, Fournet JC: SNP genotyping of a sclerosing rhabdomyosarcoma: reveals highly aneuploid profile and a specific MDM2/HMGA2 amplification. Hum Pathol; 2009 Sep;40(9):1347-52
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  • [Title] SNP genotyping of a sclerosing rhabdomyosarcoma: reveals highly aneuploid profile and a specific MDM2/HMGA2 amplification.
  • Since the first description of sclerosing rhabdomyosarcoma in 2000, 19 pediatric cases have been reported in the literature.
  • However, it is debated whether sclerosing rhabdomyosarcoma represents a specific rhabdomyosarcoma entity or a variant of embryonal or alveolar rhabdomyosarcoma.
  • To date, 6 sclerosing rhabdomyosarcoma karyotypes and 1 sclerosing rhabdomyosarcoma comparative genomic hybridization profile have been reported.
  • We present the first whole-genome tumoral genotyping of a sclerosing rhabdomyosarcoma by high-density single nucleotide polymorphism array.
  • Amplification of the 12q13-q15 region containing SAS, GLI, CDK4, and MDM2 has been observed in rhabdomyosarcoma.
  • Further studies are needed to assess if this anomaly is a specific marker of sclerosing rhabdomyosarcoma.
  • [MeSH-major] Aneuploidy. HMGA2 Protein / genetics. Polymorphism, Single Nucleotide. Proto-Oncogene Proteins c-mdm2 / genetics. Rhabdomyosarcoma / genetics

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  • (PMID = 19454362.001).
  • [ISSN] 1532-8392
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / HMGA2 Protein; EC 6.3.2.19 / MDM2 protein, human; EC 6.3.2.19 / Proto-Oncogene Proteins c-mdm2
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65. Raney RB, Anderson JR, Brown KL, Huh WW, Maurer HM, Meyer WH, Parham DM, Rodeberg DA, Wolden SL, Donaldson SS, Soft-Tissue Sarcoma Committee of the Children's Oncology Group Arcadia California USA: Treatment results for patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III and IV, 1984-1997: a report from the Children's Oncology Group. Pediatr Blood Cancer; 2010 Oct;55(4):612-6
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  • [Title] Treatment results for patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma on Intergroup Rhabdomyosarcoma Study Group (IRSG) protocols III and IV, 1984-1997: a report from the Children's Oncology Group.
  • PURPOSE: To assess local control, event-free survival (EFS), and overall survival (OS) rates in 71 patients with localized, completely resected (Group I) alveolar rhabdomyosarcoma (ALV RMS) and their relation to radiation therapy (RT) on IRSG Protocols III and IV, 1984-1997.

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  • [Copyright] Copyright 2010 Wiley-Liss, Inc.
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  • (PMID = 20806360.001).
  • [ISSN] 1545-5017
  • [Journal-full-title] Pediatric blood & cancer
  • [ISO-abbreviation] Pediatr Blood Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA-98543; United States / NCI NIH HHS / CA / U10 CA072989; United States / NCI NIH HHS / CA / U10 CA029511; United States / NCI NIH HHS / CA / CA-24507; United States / NCI NIH HHS / CA / CA-72989; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / CA-29511; United States / NCI NIH HHS / CA / U10 CA024507
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  • [Other-IDs] NLM/ NIHMS263511; NLM/ PMC3128801
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66. 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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67. 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
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  • [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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68. 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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69. 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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  • [Journal-full-title] Pediatric radiology
  • [ISO-abbreviation] Pediatr Radiol
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  • [Publication-type] Journal Article
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70. Zaidi TS, Mushtaq I, Sebire N: Alveolar rhabdomyosarcoma of the bladder in a child. Pediatr Surg Int; 2006 May;22(5):474-6
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  • [Title] Alveolar rhabdomyosarcoma of the bladder in a child.
  • [MeSH-major] Rhabdomyosarcoma, Alveolar / surgery. Urinary Bladder Neoplasms / surgery

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  • [Cites] Histopathology. 2004 May;44(5):508-10 [15140004.001]
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  • (PMID = 16518598.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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71. Ferguson SE, Gerald W, Barakat RR, Chi DS, Soslow RA: Clinicopathologic features of rhabdomyosarcoma of gynecologic origin in adults. Am J Surg Pathol; 2007 Mar;31(3):382-9
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  • [Title] Clinicopathologic features of rhabdomyosarcoma of gynecologic origin in adults.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue tumor found in children.
  • Of the remaining 4 tumors, 2 were of alveolar (vulva) and 2 of pleomorphic (uterus, 1; fallopian tube, 1) histologic subtype.
  • Adult RMS of gynecologic origin presents with locoregional disease and most are morphologically similar to pediatric RMS; however, adult RMS behaves more aggressively, with worse overall survival.
  • [MeSH-major] Genital Neoplasms, Female / pathology. Rhabdomyosarcoma / pathology

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  • [CommentIn] Am J Surg Pathol. 2008 Jan;32(1):174 [18162788.001]
  • (PMID = 17325479.001).
  • [ISSN] 0147-5185
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
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72. Lisboa S, Cerveira N, Vieira J, Torres L, Ferreira AM, Afonso M, Norton L, Henrique R, Teixeira MR: Genetic diagnosis of alveolar rhabdomyosarcoma in the bone marrow of a patient without evidence of primary tumor. Pediatr Blood Cancer; 2008 Oct;51(4):554-7
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  • [Title] Genetic diagnosis of alveolar rhabdomyosarcoma in the bone marrow of a patient without evidence of primary tumor.
  • Alveolar rhabdomyosarcoma (ARMS) is characterized by two pathognomonic translocations, both involving the FOXO1 gene.
  • [MeSH-major] Bone Marrow Neoplasms / diagnosis. Bone Marrow Neoplasms / genetics. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics

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  • [Copyright] (c) 2008 Wiley-Liss, Inc.
  • (PMID = 18561177.001).
  • [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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73. 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


74. Kohashi K, Oda Y, Yamamoto H, Tamiya S, Takahira T, Takahashi Y, Tajiri T, Taguchi T, Suita S, Tsuneyoshi M: Alterations of RB1 gene in embryonal and alveolar rhabdomyosarcoma: special reference to utility of pRB immunoreactivity in differential diagnosis of rhabdomyosarcoma subtype. J Cancer Res Clin Oncol; 2008 Oct;134(10):1097-103
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  • [Title] Alterations of RB1 gene in embryonal and alveolar rhabdomyosarcoma: special reference to utility of pRB immunoreactivity in differential diagnosis of rhabdomyosarcoma subtype.
  • PURPOSE: Rhabdomyosarcoma (RMS), which is the most common pediatric soft tissue sarcoma, is classified into two major histologic subtypes, embryonal RMS (ERMS) and alveolar RMS (ARMS).
  • [MeSH-major] Retinoblastoma Protein / biosynthesis. Retinoblastoma Protein / genetics. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / genetics


75. Blizniukov OP, Petrovichev NN, Perevoshchikov AG, Poliakov VG: [Diagnosis of micrometastases of alveolar rhabdomyosarcoma]. Arkh Patol; 2008 Mar-Apr;70(2):36-40
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  • [Title] [Diagnosis of micrometastases of alveolar rhabdomyosarcoma].
  • Alveolar rhabgomyosarcoma is a highly malignant, small blue cell pediatric soft tissue tumor.
  • Identification of micrometastases in alveolar rhabdomyosarcoma is important because the poor prognosis associated with this subgroup necessitates a modified therapeutic regimen.
  • Since the obtained lymph node specimen can be very small; rhabdomyosarcoma cells are not easily detected using conventional histological methods.
  • To assess the value of myogenin staining in the diagnosis of micrometastases in alveolar rhabdomyosarcoma, the authors examined 36 lymph nodes from children bearing this tumor.
  • The PAX3/7-FKHR gene fusion that resulted from chromosomal translocation in alveolar rhabdomyosarcoma provided potential molecular diagnostic markers.
  • Thirty-six lymph nodes were examined and of them 17 lymph nodes had PAX3/7-FKHR fusion transcripts of alveolar rhadomyosarcoma cells.
  • The study demonstrates that molecular RT-PCR detection of micrometastases is the most sensitive method for diagnosing alveolar rhabdomyosarcoma.
  • [MeSH-major] Biomarkers, Tumor / biosynthesis. Eye Proteins / biosynthesis. Forkhead Transcription Factors / biosynthesis. Homeodomain Proteins / biosynthesis. Oncogene Proteins, Fusion / biosynthesis. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / metabolism. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / metabolism. Transcription Factors / biosynthesis

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  • (PMID = 18540440.001).
  • [ISSN] 0004-1955
  • [Journal-full-title] Arkhiv patologii
  • [ISO-abbreviation] Arkh. Patol.
  • [Language] rus
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Russia (Federation)
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Eye Proteins; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Homeodomain Proteins; 0 / Oncogene Proteins, Fusion; 0 / RAX protein, human; 0 / RNA, Neoplasm; 0 / Transcription Factors
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76. D'Angelo P, Carli M, Ferrari A, Manzitti C, Mura R, Miglionico L, Di Cataldo A, Grigoli A, Cecchetto G, Bisogno G, AIEOP Soft Tissue Sarcoma Committee: Breast metastases in children and adolescents with rhabdomyosarcoma: Experience of the Italian Soft Tissue Sarcoma Committee. Pediatr Blood Cancer; 2010 Dec 15;55(7):1306-9
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  • [Title] Breast metastases in children and adolescents with rhabdomyosarcoma: Experience of the Italian Soft Tissue Sarcoma Committee.
  • BACKGROUND: Breast metastasis from rhabdomyosarcoma (RMS) is an uncommon event but may be problematic in treatment decision-making.
  • All patients were females, aged 13-17 years with alveolar histology and multiple metastasis sites (2-5).
  • CONCLUSIONS: Our data suggest that investigations of the mammary region should be part of the usual diagnostic workup in adolescent girls with alveolar RMS, especially if the primary tumor arises in the extremities.
  • [MeSH-major] Breast Neoplasms / secondary. Muscle Neoplasms / pathology. Rhabdomyosarcoma / secondary

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  • [Copyright] Copyright © 2010 Wiley-Liss, Inc.
  • (PMID = 20730885.001).
  • [ISSN] 1545-5017
  • [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
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77. Barroca H: Fine needle biopsy and genetics, two allied weapons in the diagnosis, prognosis, and target therapeutics of solid pediatric tumors. Diagn Cytopathol; 2008 Sep;36(9):678-84
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  • [Title] Fine needle biopsy and genetics, two allied weapons in the diagnosis, prognosis, and target therapeutics of solid pediatric tumors.
  • The recognition that genetic defects identify some pediatric solid tumors and may represent prognostic markers has provided cytologists with an extra tool for dealing with such tumors.
  • Using some entities as archetypes, we discuss the importance of the association of fine needle biopsy and genetics, in the diagnosis, prognosis, and therapy selection of solid pediatric tumors.
  • Immunocytochemistry is important to differentiate neuroblastoma, PNET/Ewing sarcoma, alveolar rhabdomyosarcoma, lymphoma, and desmoplastic small round cell tumor.
  • Cytopathologists should be aware of the genetic alterations characterizing pediatric tumors in order to collect extra material to perform cytogenetics, FISH, PCR, and Southern blotting, to achieve the correct identification of such genetic changes.

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  • (PMID = 18677757.001).
  • [ISSN] 1097-0339
  • [Journal-full-title] Diagnostic cytopathology
  • [ISO-abbreviation] Diagn. Cytopathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 29
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78. Khalil BA, Baillie CT, Kenny SE, Lamont GL, Turnock RR, Pizer BL, van Saene HF, Losty PD: Surgical strategies in the management of ecthyma gangrenosum in paediatric oncology patients. Pediatr Surg Int; 2008 Jul;24(7):793-7
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  • Eight had acute lymphoblastic leukaemia, one child had acute myeloid leukaemia and another had rhabdomyosarcoma.
  • [MeSH-major] Debridement / methods. Ecthyma / surgery. Practice Guidelines as Topic. Precursor Cell Lymphoblastic Leukemia-Lymphoma / complications. Rhabdomyosarcoma, Alveolar / complications. Soft Tissue Neoplasms / complications

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  • (PMID = 18427811.001).
  • [ISSN] 0179-0358
  • [Journal-full-title] Pediatric surgery international
  • [ISO-abbreviation] Pediatr. Surg. Int.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Germany
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79. Thomas DM, O'Sullivan B, Gronchi A: Current concepts and future perspectives in retroperitoneal soft-tissue sarcoma management. Expert Rev Anticancer Ther; 2009 Aug;9(8):1145-57
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  • Pediatric subtypes mainly comprise extraskeletal Ewing sarcoma/pPNET and alveolar rhabdomyosarcoma.
  • Chemotherapy has a limited role in the adjuvant setting for most forms of retroperitoneal sarcoma (excluding pediatric subtypes), but has an increasing role in advanced disease.

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  • (PMID = 19671034.001).
  • [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
  • [Number-of-references] 93
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80. Casanova M, Meazza C, Gronchi A, Fiore M, Zaffignani E, Podda M, Collini P, Gandola L, Ferrari A: Soft-tissue sarcomas of the extremities in patients of pediatric age. J Child Orthop; 2007 Sep;1(3):195-203
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  • [Title] Soft-tissue sarcomas of the extremities in patients of pediatric age.
  • Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006.
  • METHODS: The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma.
  • DISCUSSION: While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results.

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  • (PMID = 19308495.001).
  • [ISSN] 1863-2521
  • [Journal-full-title] Journal of children's orthopaedics
  • [ISO-abbreviation] J Child Orthop
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Other-IDs] NLM/ PMC2656726
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81. Kumar S, Chougle A, Meadows TH: Rhabdomyosarcoma presenting as a calf pyomyositis. J Pediatr Orthop B; 2005 May;14(3):226-7
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  • [Title] Rhabdomyosarcoma presenting as a calf pyomyositis.
  • [MeSH-major] Leg. Myositis / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis

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  • (PMID = 15812297.001).
  • [ISSN] 1060-152X
  • [Journal-full-title] Journal of pediatric orthopedics. Part B
  • [ISO-abbreviation] J Pediatr Orthop B
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Immunoglobulin M; EC 1.1.1.27 / L-Lactate Dehydrogenase
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82. Makawita S, Ho M, Durbin AD, Thorner PS, Malkin D, Somers GR: Expression of insulin-like growth factor pathway proteins in rhabdomyosarcoma: IGF-2 expression is associated with translocation-negative tumors. Pediatr Dev Pathol; 2009 Mar-Apr;12(2):127-35
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Expression of insulin-like growth factor pathway proteins in rhabdomyosarcoma: IGF-2 expression is associated with translocation-negative tumors.
  • Recent studies have shown a significant involvement of insulin-like growth factor (IGF) signaling components in the pathogenesis of rhabdomyosarcoma (RMS).
  • The present study utilized immunohistochemistry to determine the expression patterns of IGF1, IGF2, IGF binding protein 2 (IGFBP2), IGF receptor 1 (IGF1R), and IGF receptor 2 (IGF2R) in 24 embryonal RMS (ERMS) and 8 alveolar RMS (ARMS).
  • [MeSH-major] Insulin-Like Growth Factor II / metabolism. Rhabdomyosarcoma / metabolism. Soft Tissue Neoplasms / metabolism. Translocation, Genetic / genetics

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  • (PMID = 18788888.001).
  • [ISSN] 1093-5266
  • [Journal-full-title] Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
  • [ISO-abbreviation] Pediatr. Dev. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Forkhead Transcription Factors; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 67763-97-7 / Insulin-Like Growth Factor II
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83. Machleder DJ, Banik R, Rosenberg RB, Parikh SR: An unusual case of rhabdomyosarcoma presenting as orbital apex syndrome. Int J Pediatr Otorhinolaryngol; 2005 Feb;69(2):249-54
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  • [Title] An unusual case of rhabdomyosarcoma presenting as orbital apex syndrome.
  • PRECIS: A 12-year-old female presented with symptoms and signs of orbital apex syndrome (OAS), secondary to stage IV alveolar rhabdomyosarcoma (RMS) originating in the sphenoid and ethmoid sinuses.
  • OBJECTIVE: To present a case of alveolar rhabdomyosarcoma, unusual in its presentation as orbital apex syndrome and also its origin from the sphenoid and ethmoid sinuses.
  • Emergent biopsy was interpreted as alveolar rhabdomyosarcoma; subsequent metastatic work-up revealed bone marrow metastases.
  • The patient was diagnosed with stage IV alveolar rhabdomyosarcoma and immediately started on combination orbital radiation therapy (RT) and systemic chemotherapy.
  • CONCLUSION: Alveolar rhabdomyosarcoma of paranasal origin, specifically from the sphenoid and ethmoid sinuses, should be included in the differential diagnosis for orbital apex syndrome in children.
  • [MeSH-major] Orbital Neoplasms / diagnosis. Orbital Neoplasms / secondary. Paranasal Sinus Neoplasms / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis

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  • (PMID = 15656960.001).
  • [ISSN] 0165-5876
  • [Journal-full-title] International journal of pediatric otorhinolaryngology
  • [ISO-abbreviation] Int. J. Pediatr. Otorhinolaryngol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Review
  • [Publication-country] Ireland
  • [Number-of-references] 22
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84. Bois PR, Izeradjene K, Houghton PJ, Cleveland JL, Houghton JA, Grosveld GC: FOXO1a acts as a selective tumor suppressor in alveolar rhabdomyosarcoma. J Cell Biol; 2005 Sep 12;170(6):903-12
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] FOXO1a acts as a selective tumor suppressor in alveolar rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS), the most common pediatric soft-tissue sarcoma, has two major histological subtypes: embryonal RMS (ERMS), which has a favorable prognosis, and alveolar RMS (ARMS), which has a poor outcome.

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  • (PMID = 16157701.001).
  • [ISSN] 0021-9525
  • [Journal-full-title] The Journal of cell biology
  • [ISO-abbreviation] J. Cell Biol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / P01 CA071907; United States / NCI NIH HHS / CA / R01 CA087952; United States / NCI NIH HHS / CA / CA 71907; United States / NCI NIH HHS / CA / P01 CA023099; United States / NCI NIH HHS / CA / P30 CA021765; United States / NCI NIH HHS / CA / CA 96696; United States / NCI NIH HHS / CA / CA 87952; United States / NCI NIH HHS / CA / CA 23099; United States / NCI NIH HHS / CA / R01 CA096696; United States / NCI NIH HHS / CA / CA 21765
  • [Publication-type] Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.; Retracted Publication
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / FOXO1 protein, human; 0 / Fluorescent Dyes; 0 / Forkhead Transcription Factors; 0 / Indoles; 0 / Recombinant Fusion Proteins; 47165-04-8 / DAPI; EC 1.13.12.- / Luciferases; EC 3.4.22.- / CASP3 protein, human; EC 3.4.22.- / Casp3 protein, mouse; EC 3.4.22.- / Caspase 3; EC 3.4.22.- / Caspases
  • [Other-IDs] NLM/ PMC2171446
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85. 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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86. Galindo RL, Allport JA, Olson EN: A Drosophila model of the rhabdomyosarcoma initiator PAX7-FKHR. Proc Natl Acad Sci U S A; 2006 Sep 5;103(36):13439-44
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  • [Title] A Drosophila model of the rhabdomyosarcoma initiator PAX7-FKHR.
  • Alveolar rhabdomyosarcoma (ARMS) is an aggressive myogenic-type tumor and a gain-of-function disease, caused by misexpression of the PAX3-FKHR or PAX7-FKHR fusion oncoprotein from structurally rearranged chromosomes.
  • PAX3-FKHR misexpressed in terminally differentiating mouse myofibers can cause rhabdomyosarcoma at a low frequency, suggesting that skeletal muscle is an ARMS tissue of origin.
  • [MeSH-major] Drosophila / genetics. Forkhead Transcription Factors / genetics. Oncogene Proteins, Fusion. PAX7 Transcription Factor / genetics. Rhabdomyosarcoma, Alveolar / genetics

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  • (PMID = 16938866.001).
  • [ISSN] 0027-8424
  • [Journal-full-title] Proceedings of the National Academy of Sciences of the United States of America
  • [ISO-abbreviation] Proc. Natl. Acad. Sci. U.S.A.
  • [Language] eng
  • [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 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / PAX7 Transcription Factor; 0 / PAX7 protein, human
  • [Other-IDs] NLM/ PMC1569182
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87. Kayton ML: Pulmonary metastasectomy in pediatric patients. Thorac Surg Clin; 2006 May;16(2):167-83, vi
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  • [Title] Pulmonary metastasectomy in pediatric patients.
  • This article describes the historical development of pediatric pulmonary metastasectomy but demonstrates that progress has been slow in understanding its proper applications.
  • Because many pediatric metastatic tumors are rare, surgeons have grouped together patients of different histologies for the generation and analysis of case series.
  • By examining tumor types individually, however, it is seen that certain histologies (adrenocortical carcinoma, alveolar soft part sarcoma, osteosarcoma) mandate surgical metastasectomy for patient survival.
  • Other pediatric tumors (Wilms tumor, Ewing's sarcoma) are radiation sensitive, and the application of metastasectomy is controversial.
  • In the case of still other types of tumor (neuroblastoma, differentiated thyroid cancer, rhabdomyosarcoma), metastasectomy is seldom performed except in highly unusual situations.
  • Techniques for minimally invasive biopsy and for muscle-sparing thoracotomy are described for pediatric patients.

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  • (PMID = 16805206.001).
  • [ISSN] 1547-4127
  • [Journal-full-title] Thoracic surgery clinics
  • [ISO-abbreviation] Thorac Surg Clin
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 91
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88. Naithani R, Kumar R, Mahapatra M, Agrawal N, Saxena R, Sharma S: Pelvic alveolar rhabdomyosarcoma with bone marrow involvement misdiagnosed as acute myeloid leukemia. Pediatr Hematol Oncol; 2007 Mar;24(2):153-5
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  • [Title] Pelvic alveolar rhabdomyosarcoma with bone marrow involvement misdiagnosed as acute myeloid leukemia.
  • [MeSH-major] Bone Marrow / pathology. Leukemia, Myeloid / diagnosis. Pelvic Neoplasms / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis

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  • (PMID = 17454783.001).
  • [ISSN] 1521-0669
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] England
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89. 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


90. 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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91. Häcker FM, von Schweinitz D, Gambazzi F: The relevance of surgical therapy for bilateral and/or multiple pulmonary metastases in children. Eur J Pediatr Surg; 2007 Apr;17(2):84-9
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  • The primary malignancies were osteosarcoma (n = 4), hepatoblastoma (n = 3), malignant peripheral nerve sheath tumor (n = 1), adrenocortical carcinoma (n = 1) and alveolar rhabdomyosarcoma (n = 1).

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  • (PMID = 17503299.001).
  • [ISSN] 0939-7248
  • [Journal-full-title] European journal of pediatric surgery : official journal of Austrian Association of Pediatric Surgery ... [et al] = Zeitschrift für Kinderchirurgie
  • [ISO-abbreviation] Eur J Pediatr Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
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92. Orbach D, Rey A, Oberlin O, Sanchez de Toledo J, Terrier-Lacombe MJ, van Unnik A, Quintana E, Stevens MC: Soft tissue sarcoma or malignant mesenchymal tumors in the first year of life: experience of the International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumor Committee. J Clin Oncol; 2005 Jul 1;23(19):4363-71
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  • [Title] Soft tissue sarcoma or malignant mesenchymal tumors in the first year of life: experience of the International Society of Pediatric Oncology (SIOP) Malignant Mesenchymal Tumor Committee.
  • Sixty-four patients had rhabdomyosarcoma (RMS), 26 had undifferentiated sarcoma, and 12 had other histology.
  • Infants with alveolar subtype had a poorer survival than those with non-RMS MMT or nonalveolar RMS (5-year OS, 37% v 75% or 82%, respectively; P = .002).
  • CONCLUSION: OS was satisfactory even when local treatment was not aggressive, although the prognosis was poor for infants with alveolar RMS or metastatic tumors.
  • [MeSH-minor] Age Factors. Antineoplastic Combined Chemotherapy Protocols / therapeutic use. Child. Child, Preschool. Cyclophosphamide / therapeutic use. Dactinomycin / therapeutic use. Doxorubicin / administration & dosage. Epirubicin / therapeutic use. Etoposide / administration & dosage. Humans. Ifosfamide / therapeutic use. Infant. Infant, Newborn. Neoplasm Metastasis. Neoplasm Recurrence, Local. Rhabdomyosarcoma / drug therapy. Survival Analysis. Teniposide / administration & dosage. Vincristine / therapeutic use

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  • (PMID = 15994146.001).
  • [ISSN] 0732-183X
  • [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] Clinical Trial; Comparative Study; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 3Z8479ZZ5X / Epirubicin; 5J49Q6B70F / Vincristine; 6PLQ3CP4P3 / Etoposide; 80168379AG / Doxorubicin; 8N3DW7272P / Cyclophosphamide; 957E6438QA / Teniposide; UM20QQM95Y / Ifosfamide; CEV protocol; IVA protocol
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93. Herzog CE: Overview of sarcomas in the adolescent and young adult population. J Pediatr Hematol Oncol; 2005 Apr;27(4):215-8
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  • The SEER program collects data regarding age but only limited data on histology, while most series reported in the literature include either adults or pediatric patients, but rarely both.
  • These sarcomas include rhabdomyosarcoma, synovial sarcoma, neurogenic sarcoma, epithelioid sarcomas, alveolar soft parts sarcoma, Ewing sarcoma, and osteosarcoma.

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  • (PMID = 15838394.001).
  • [ISSN] 1077-4114
  • [Journal-full-title] Journal of pediatric hematology/oncology
  • [ISO-abbreviation] J. Pediatr. Hematol. Oncol.
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 19
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94. Passmore LM, Myers P, Gilbert-Barness E: Pathology teach and tell: solid variant alveolar rhabdomyosarcoma of the orbit. Fetal Pediatr Pathol; 2006 Jan-Feb;25(1):51-7
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  • [Title] Pathology teach and tell: solid variant alveolar rhabdomyosarcoma of the orbit.
  • [MeSH-major] Orbital Neoplasms / diagnosis. Orbital Neoplasms / pathology. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / pathology

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  • (PMID = 16754488.001).
  • [ISSN] 1551-3815
  • [Journal-full-title] Fetal and pediatric pathology
  • [ISO-abbreviation] Fetal Pediatr Pathol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Myogenin
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95. Doelken R, Weigel S, Schueler F, Doelken G, Beck JF: Poor outcome of two children with relapsed state stage IV alveolar rhabdomyosarcoma after allogeneic stem cell transplantation. Pediatr Hematol Oncol; 2005 Dec;22(8):699-703
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  • [Title] Poor outcome of two children with relapsed state stage IV alveolar rhabdomyosarcoma after allogeneic stem cell transplantation.
  • The authors report on 2 boys, 11(1/2) and 13 years old, who received allogeneic stem cell transplantation (alloSCT) from their HLA-identical sibling after relapse of stage IV alveolar rhabdomyosarcoma.
  • The authors conclude that an alloSCT derived graft versus tumor effect might not be effective enough to overcome alveolar rhabdomyosarcoma when transplantation is carried out in a nonremission status.
  • [MeSH-major] Hematopoietic Stem Cell Transplantation / adverse effects. Rhabdomyosarcoma, Alveolar / therapy

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  • (PMID = 16251176.001).
  • [ISSN] 0888-0018
  • [Journal-full-title] Pediatric hematology and oncology
  • [ISO-abbreviation] Pediatr Hematol Oncol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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96. Chan WM, Liu DT, Pang CP, Lam DS, To KF, Choi PC, Choy KW, Wong CY, Chan DD: Pediatric malignancies. Case 1. Hypermethylation in orbital alveolar rhabdomyosarcoma. J Clin Oncol; 2005 Jul 20;23(21):4790-1
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Pediatric malignancies. Case 1. Hypermethylation in orbital alveolar rhabdomyosarcoma.
  • [MeSH-major] Orbital Neoplasms / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis

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  • (PMID = 16034055.001).
  • [ISSN] 0732-183X
  • [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] Case Reports; Journal Article
  • [Publication-country] United States
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97. Ferrari A, Miceli R, Meazza C, Casanova M, Favini F, Morosi C, Trecate G, Marchianò A, Luksch R, Cefalo G, Terenziani M, Spreafico F, Polastri D, Podda M, Catania S, Schiavello E, Giannatempo P, Gandola L, Massimino M, Mariani L: Comparison of the prognostic value of assessing tumor diameter versus tumor volume at diagnosis or in response to initial chemotherapy in rhabdomyosarcoma. J Clin Oncol; 2010 Mar 10;28(8):1322-8
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  • [Title] Comparison of the prognostic value of assessing tumor diameter versus tumor volume at diagnosis or in response to initial chemotherapy in rhabdomyosarcoma.
  • PURPOSE: In this study on a series of 205 patients with rhabdomyosarcoma, we investigated whether the prognostic effect of tumor size, at diagnosis or in terms of tumor response after induction chemotherapy, differed when tumor diameter or tumor volume were considered.
  • RESULTS: Initial tumor size was significantly larger in male or older patients and in T2 or alveolar tumors, but was not associated with the achievement of complete surgical resection.
  • CONCLUSION: In our analysis, initial tumor size and tumor response were significant prognostic factors in rhabdomyosarcoma, regardless of whether tumor diameter or volume was considered.
  • [MeSH-major] Decision Support Techniques. Imaging, Three-Dimensional. Rhabdomyosarcoma / pathology


98. 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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  • [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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99. Hagan KA, Bienvenue JM, Moskaluk CA, Landers JP: Microchip-based solid-phase purification of RNA from biological samples. Anal Chem; 2008 Nov 15;80(22):8453-60
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  • RNA was purified using the microchip-based method from neat semen, a mock semen stain, and cultured cells from a common pediatric cancer, alveolar rhabdomyosarcoma.

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  • (PMID = 18855414.001).
  • [ISSN] 1520-6882
  • [Journal-full-title] Analytical chemistry
  • [ISO-abbreviation] Anal. Chem.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / RNA, Messenger; 63231-63-0 / RNA; 7631-86-9 / Silicon Dioxide
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100. Tan GC, Shiran MS, Hayati AR, Sharifah NA, Nuru AS, Rohaizak M: Alveolar rhabdomyosarcoma of the left hand with bilateral breast metastases in an adolescent female. J Chin Med Assoc; 2008 Dec;71(12):639-42
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  • [Title] Alveolar rhabdomyosarcoma of the left hand with bilateral breast metastases in an adolescent female.
  • Rhabdomyosarcoma is a common extramammary malignancy in pediatric age groups, but it rarely metastasizes to the breast.
  • We report a case of primary alveolar rhabdomyosarcoma of the upper extremities in a 17-year-old adolescent female who presented with bilateral lower limb weakness and bilateral breast lumps.
  • [MeSH-major] Breast Neoplasms / secondary. Hand / pathology. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Alveolar / secondary

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  • (PMID = 19114329.001).
  • [ISSN] 1726-4901
  • [Journal-full-title] Journal of the Chinese Medical Association : JCMA
  • [ISO-abbreviation] J Chin Med Assoc
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] China (Republic : 1949- )
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