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1. Ren YX, Finckenstein FG, Abdueva DA, Shahbazian V, Chung B, Weinberg KI, Triche TJ, Shimada H, Anderson MJ: Mouse mesenchymal stem cells expressing PAX-FKHR form alveolar rhabdomyosarcomas by cooperating with secondary mutations. Cancer Res; 2008 Aug 15;68(16):6587-97
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  • [Title] Mouse mesenchymal stem cells expressing PAX-FKHR form alveolar rhabdomyosarcomas by cooperating with secondary mutations.
  • Alveolar rhabdomyosarcomas (ARMS) are highly malignant soft-tissue sarcomas that arise in children, adolescents, and young adults.
  • Despite exhibiting enhanced growth in vitro, the PAX-FKHR-expressing populations do not form colonies in soft agar or tumors in mice.
  • [MeSH-major] Antigens, Polyomavirus Transforming / genetics. Forkhead Transcription Factors / genetics. Genes, ras / physiology. Mesenchymal Stromal Cells / pathology. Mutation / genetics. Oncogene Proteins, Fusion / metabolism. PAX7 Transcription Factor / genetics. Paired Box Transcription Factors / genetics. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / pathology

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  • (PMID = 18701482.001).
  • [ISSN] 1538-7445
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antigens, Polyomavirus Transforming; 0 / Antigens, Viral, Tumor; 0 / Forkhead Transcription Factors; 0 / Foxo1 protein, mouse; 0 / Myogenin; 0 / Oncogene Proteins, Fusion; 0 / PAX7 Transcription Factor; 0 / Paired Box Transcription Factors; 0 / Pax7 protein, mouse; 0 / Tumor Suppressor Protein p53; 138016-91-8 / Pax3 protein, mouse
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2. Masola V, Maran C, Tassone E, Zin A, Rosolen A, Onisto M: Heparanase activity in alveolar and embryonal rhabdomyosarcoma: implications for tumor invasion. BMC Cancer; 2009;9:304
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  • [Title] Heparanase activity in alveolar and embryonal rhabdomyosarcoma: implications for tumor invasion.
  • BACKGROUND: Rhabdomyosarcoma (RMS) is a malignant soft tissue sarcoma of childhood including two major histological subtypes, alveolar (ARMS) and embryonal (ERMS) RMS.
  • [MeSH-major] Glucuronidase / metabolism. Neoplasm Invasiveness. Rhabdomyosarcoma, Alveolar / embryology. Rhabdomyosarcoma, Alveolar / enzymology

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  • (PMID = 19715595.001).
  • [ISSN] 1471-2407
  • [Journal-full-title] BMC cancer
  • [ISO-abbreviation] BMC Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] EC 3.2.1.- / heparanase; EC 3.2.1.31 / Glucuronidase
  • [Other-IDs] NLM/ PMC2743710
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3. Mentzel T, Kuhnen C: Spindle cell rhabdomyosarcoma in adults: clinicopathological and immunohistochemical analysis of seven new cases. Virchows Arch; 2006 Nov;449(5):554-60
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  • [Title] Spindle cell rhabdomyosarcoma in adults: clinicopathological and immunohistochemical analysis of seven new cases.
  • Rhabdomyosarcoma (RMS) is currently classified into embryonal RMS, including its botryoid and spindle cell variants, alveolar RMS, including a solid variant, and pleomorphic RMS.
  • In children and adolescents embryonal RMS occurs in a younger age group than alveolar RMS, and pleomorphic RMS is almost always seen in older adults.
  • All neoplasms arose in subcutaneous and deep soft tissues with dermal involvement in one case, and the size of the neoplasms ranged from 4 to 19 cm in largest diameter.
  • In summary, spindle cell rhabdomyosarcoma represents a rare neoplasm in adulthood characterized clinically by a rather poor prognosis, and shows a broad morphological spectrum including most likely the sclerosing, pseudovascular variant.
  • Immunohistochemically, tumour cells in RMS stain positively for CD 99 and WT1 as well, which is of importance in the differential diagnosis to other mesenchymal neoplasms, whereas fast myosin does not represent a reliable marker for RMS in adults.
  • [MeSH-major] Rhabdomyosarcoma / pathology. Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 17013628.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Antigens, CD; 0 / Antigens, CD99; 0 / CD99 protein, human; 0 / Cell Adhesion Molecules
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4. 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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  • Based on the data of the Surveillance, Epidemiology and End Results Section of the National Cancer Institute (SEER) program, soft tissue and bone sarcomas account for about 1% of all new malignancies diagnosed in the United States each year.
  • Anderson Cancer Center (MDACC) tumor registry was queried for all soft tissue sarcomas from 1990 through 2003, and all bone sarcomas from 1990 through 2002.
  • These sarcomas include rhabdomyosarcoma, synovial sarcoma, neurogenic sarcoma, epithelioid sarcomas, alveolar soft parts sarcoma, Ewing sarcoma, and osteosarcoma.
  • [MeSH-major] Bone Neoplasms / pathology. Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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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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5. 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.
  • Aim of the study was to evaluate clinical characteristics, treatment, and subsequent outcome, of patients with RMS metastasis in the breast, enrolled in four consecutive Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP) Soft Tissue Sarcoma Committee protocols during the last 20 years, in order to obtain information to establish a more adequate diagnostic and therapeutic approach.
  • RESULTS: From 1988 to 2008, among 189 patients with metastatic RMS, we identified 7 (3.7%) patients with RMS with breast involvement at diagnosis.
  • 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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6. 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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  • [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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7. Mercado GE, Xia SJ, Zhang C, Ahn EH, Gustafson DM, Laé M, Ladanyi M, Barr FG: Identification of PAX3-FKHR-regulated genes differentially expressed between alveolar and embryonal rhabdomyosarcoma: focus on MYCN as a biologically relevant target. Genes Chromosomes Cancer; 2008 Jun;47(6):510-20
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  • [Title] Identification of PAX3-FKHR-regulated genes differentially expressed between alveolar and embryonal rhabdomyosarcoma: focus on MYCN as a biologically relevant target.
  • Rhabdomyosarcoma is a family of myogenic soft tissue tumors subdivided into two main subtypes: alveolar (ARMS) and embryonal (ERMS).
  • [MeSH-major] Gene Expression Regulation, Neoplastic. Nuclear Proteins / physiology. Oncogene Proteins / physiology. Oncogene Proteins, Fusion / physiology. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Embryonal / genetics. Soft Tissue Neoplasms / genetics

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  • (PMID = 18335505.001).
  • [ISSN] 1098-2264
  • [Journal-full-title] Genes, chromosomes & cancer
  • [ISO-abbreviation] Genes Chromosomes Cancer
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA104896; United States / NCI NIH HHS / CA / CA106450; United States / NCI NIH HHS / CA / CA64202; United States / NCI NIH HHS / CA / CA87812
  • [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 / MYCN protein, human; 0 / Neoplasm Proteins; 0 / Nuclear Proteins; 0 / Oncogene Proteins; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human; 0 / Protein Synthesis Inhibitors; 0 / RNA, Messenger; 0 / RNA, Neoplasm; 0 / Recombinant Fusion Proteins; 98600C0908 / Cycloheximide
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8. Canner JA, Sobo M, Ball S, Hutzen B, DeAngelis S, Willis W, Studebaker AW, Ding K, Wang S, Yang D, Lin J: MI-63: a novel small-molecule inhibitor targets MDM2 and induces apoptosis in embryonal and alveolar rhabdomyosarcoma cells with wild-type p53. Br J Cancer; 2009 Sep 1;101(5):774-81
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  • [Title] MI-63: a novel small-molecule inhibitor targets MDM2 and induces apoptosis in embryonal and alveolar rhabdomyosarcoma cells with wild-type p53.
  • METHODS: Rhabdomyosarcoma (RMS), the most common childhood soft tissue sarcoma, expresses either wild-type or mutant p53 protein.
  • We examined the inhibitory effects of MI-63 in embryonal RMS (ERMS) and alveolar RMS (ARMS) cell lines expressing wild-type or mutated p53.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Apoptosis / drug effects. Indoles / pharmacology. Proto-Oncogene Proteins c-mdm2 / antagonists & inhibitors. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Embryonal / pathology. Spiro Compounds / pharmacology. Tumor Suppressor Protein p53 / metabolism

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  • (PMID = 19707204.001).
  • [ISSN] 1532-1827
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Imidazoles; 0 / Indoles; 0 / MI-63 compound; 0 / Piperazines; 0 / Spiro Compounds; 0 / Tumor Suppressor Protein p53; 0 / nutlin 3; 80168379AG / Doxorubicin; EC 3.4.22.- / Caspase 3; EC 6.3.2.19 / MDM2 protein, human; EC 6.3.2.19 / Proto-Oncogene Proteins c-mdm2
  • [Other-IDs] NLM/ PMC2736841
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9. 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.
  • PATIENTS AND TUMORS: Patients were 1-18 years at diagnosis (median, 6 years).

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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
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS263511; NLM/ PMC3128801
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10. Case AS, Kirby TO, Conner MG, Huh WK: A case report of rhabdomyosarcoma of the uterus associated with uterine inversion. Gynecol Oncol; 2005 Mar;96(3):850-3
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  • [Title] A case report of rhabdomyosarcoma of the uterus associated with uterine inversion.
  • BACKGROUND: Alveolar rhabdomyosarcoma is an uncommon malignant soft tissue tumor rarely found in the genital tract.
  • On exam, a large pedunculated mass protruding from the cervix was seen and biopsy of this mass revealed an alveolar rhabdomyosarcoma.
  • The patient was treated with adjuvant chemotherapy consisting of VAC (Vincristine, Actinomycin, and Cyclophosphamide) for a presumed cervical rhabdomyosarcoma.
  • Final pathology showed alveolar rhabdomyosarcoma of the uterus.
  • The patient then received additional postoperative VAC regimen for a total of 10 treatments and remains in good health with no evidence of disease 20 months from diagnosis.
  • CONCLUSION: This case report describes the only reported case of uterine inversion secondary to alveolar rhabdomyosarcoma of the uterus and discusses current therapeutic options for adults.
  • [MeSH-major] Rhabdomyosarcoma, Alveolar / complications. Uterine Inversion / etiology. Uterine Neoplasms / complications

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  • (PMID = 15721436.001).
  • [ISSN] 0090-8258
  • [Journal-full-title] Gynecologic oncology
  • [ISO-abbreviation] Gynecol. Oncol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 1CC1JFE158 / Dactinomycin; 5J49Q6B70F / Vincristine; 8N3DW7272P / Cyclophosphamide; VAC protocol
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11. Sinha R, Bandyopadhyay SN, Pathak K, Hati G: Rhabdomyosarcoma of nose and paranasal sinuses--a therapeutic challenge. J Indian Med Assoc; 2006 Sep;104(9):519-21
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  • [Title] Rhabdomyosarcoma of nose and paranasal sinuses--a therapeutic challenge.
  • Rhabdomyosarcoma is a highly malignant tumour of striated muscle and it is the most common soft tissue sarcoma in the paediatric age group.
  • Histologically embryonal rhabdomyosarcoma is the most common variant.
  • The present series deals with seven such cases of different histopathological types including the embryonal, botryoid, and alveolar varieties in the paediatric age group.
  • [MeSH-major] Nose Neoplasms / therapy. Paranasal Sinus Neoplasms / therapy. Rhabdomyosarcoma / therapy
  • [MeSH-minor] Biopsy. Child. Child, Preschool. Combined Modality Therapy. Diagnosis, Differential. Female. Follow-Up Studies. Humans. India / epidemiology. Male. Retrospective Studies. Survival Rate. Tomography, X-Ray Computed. Treatment Outcome

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  • (PMID = 17388011.001).
  • [ISSN] 0019-5847
  • [Journal-full-title] Journal of the Indian Medical Association
  • [ISO-abbreviation] J Indian Med Assoc
  • [Language] eng
  • [Publication-type] Journal Article; Multicenter Study
  • [Publication-country] India
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12. Pazzaglia L, Chiechi A, Conti A, Gamberi G, Magagnoli G, Novello C, Morandi L, Picci P, Mercuri M, Benassi MS: Genetic and molecular alterations in rhabdomyosarcoma: mRNA overexpression of MCL1 and MAP2K4 genes. Histol Histopathol; 2009 01;24(1):61-7
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  • [Title] Genetic and molecular alterations in rhabdomyosarcoma: mRNA overexpression of MCL1 and MAP2K4 genes.
  • Rhabdomyosarcoma, the most common soft tissue sarcoma in childhood, belongs to the small round cell tumor family and is classified according to its histopathological features as embryonal, alveolar and pleomorphic.
  • In this study we propose to explore genetic alterations involved in rhabdomyosarcoma tumorigenesis and assess the level of mRNA gene expression of controlling survival signalling pathways.
  • Our findings on rhabdomyosarcoma samples showed multiple copy number alterations in chromosome regions implicated in malignancy progression and indicated a strong expression of MAP2K4 and MCL1 genes, both involved in different biological functions of complicated signalling pathways.
  • [MeSH-major] MAP Kinase Kinase 4 / genetics. Proto-Oncogene Proteins c-bcl-2 / genetics. RNA, Messenger / analysis. Rhabdomyosarcoma / genetics. Soft Tissue Neoplasms / genetics

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  • (PMID = 19012245.001).
  • [ISSN] 1699-5848
  • [Journal-full-title] Histology and histopathology
  • [ISO-abbreviation] Histol. Histopathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Spain
  • [Chemical-registry-number] 0 / Myeloid Cell Leukemia Sequence 1 Protein; 0 / Proto-Oncogene Proteins c-bcl-2; 0 / RNA, Messenger; EC 2.7.12.2 / MAP Kinase Kinase 4; EC 2.7.12.2 / MAP2K4 protein, human
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13. Stejskalová E, Jarosová M, Malis J, Sumerauer D, Urbánková H, Krsková L, Pýcha K, Schovanec J, Balcárková J, Smelhaus V, Kodetová D, Starý J: [Clinical relevance of chromosomal aberrations in bone and soft tissue tumors in children and young adults]. Klin Onkol; 2009;22(2):58-66
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  • [Title] [Clinical relevance of chromosomal aberrations in bone and soft tissue tumors in children and young adults].
  • BACKGROUND: We present the results of a cytogenetic and molecular cytogenetic analysis of a series of patients with bone and soft tissue tumors.
  • PATIENTS ANDMETHODS: We analyzed a cohort of 26 patients with Ewing sarcoma/PNET, 15 patients with rhabdomyosarcoma, 5 with synovial sarcoma and one patient with an undifferentiated sarcoma using the cytogenetic and molecular cytogenetic techniques M-FISH and arrayCGH.
  • In the RMS patients we detected the t(1;13)(p36;q14) once and the t(2;13)(q35;q14) four times, both of them characteristic for the alveolar subtype with poor prognosis and numerical aberrations, characteristic for the embryonal subtype, in five patients.
  • CONCLUSION: Cytogenetic and molecular cytogenetic analysis is a valuable diagnostic tool in bone and soft tissue tumors, especially in less differentiated subtypes, and as such it should be an integral part of curative care.
  • [MeSH-major] Bone Neoplasms / genetics. Chromosome Aberrations. Soft Tissue Neoplasms / genetics

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  • (PMID = 19522375.001).
  • [ISSN] 0862-495X
  • [Journal-full-title] Klinická onkologie : casopis Ceské a Slovenské onkologické spolecnosti
  • [ISO-abbreviation] Klin Onkol
  • [Language] cze
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] Czech Republic
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14. 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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15. Cil T, Altintas A, Isikdogan A: Rhabdomyosarcoma presenting with destructive large lesion of the face. South Med J; 2008 Jan;101(1):104-5
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  • [Title] Rhabdomyosarcoma presenting with destructive large lesion of the face.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and adolescents.
  • Prognostic factors of RMS include tumor status, primary tumor site, localization in the organ or tissue of origin, patient age and histopathological type.
  • Alveolar histologic type is more aggressive than the other types and is seen in most patients with locally advanced and metastatic disease.
  • She was diagnosed with alveolar rhabdomyosarcoma on histopathological examination.
  • [MeSH-major] Facial Neoplasms / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis. Sarcoma / diagnosis

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  • (PMID = 18176304.001).
  • [ISSN] 1541-8243
  • [Journal-full-title] Southern medical journal
  • [ISO-abbreviation] South. Med. J.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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16. Javangula KC, O'Regan DJ: Emergency surgery for left atrial metastatic alveolar rhabdomyosarcoma manifesting as a right atrial mass. Ann Thorac Surg; 2008 Sep;86(3):1008-11
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  • [Title] Emergency surgery for left atrial metastatic alveolar rhabdomyosarcoma manifesting as a right atrial mass.
  • We report the case of a 43-year-old woman with a history of alveolar rhabdomyosarcoma of the forearm, scalp, and paraspinal region who developed acute shortness of breath owing to a tumor arising from the left atrium and extending through the coronary sinus.
  • Emergency resection of the atrial mass was performed, and histologic analysis confirmed the presence of metastatic rhabdomyosarcoma.
  • To our knowledge, this is the first reported case of emergency surgical resection of intracardiac metastatic alveolar rhabdomyosarcoma.
  • [MeSH-major] Heart Neoplasms / secondary. Heart Neoplasms / surgery. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Alveolar / secondary. Rhabdomyosarcoma, Alveolar / surgery. Soft Tissue Neoplasms / pathology

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  • (PMID = 18721606.001).
  • [ISSN] 1552-6259
  • [Journal-full-title] The Annals of thoracic surgery
  • [ISO-abbreviation] Ann. Thorac. Surg.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Netherlands
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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. Gautam U, Srinivasan R, Rajwanshi A, Bansal D, Marwaha RK, Vasishtha RK: Reverse transcriptase-polymerase chain reaction as an ancillary molecular technique in the diagnosis of small blue round cell tumors by fine-needle aspiration cytology. Am J Clin Pathol; 2010 Apr;133(4):633-45
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  • [Title] Reverse transcriptase-polymerase chain reaction as an ancillary molecular technique in the diagnosis of small blue round cell tumors by fine-needle aspiration cytology.
  • The PAX3/7-FKHR fusion transcript was detected in 2 of 4 cases of alveolar rhabdomyosarcoma and the EWS-WT1 transcript in both cases of DSRCT.
  • [MeSH-major] Biopsy, Fine-Needle / methods. Neuroblastoma / diagnosis. Neuroectodermal Tumors, Primitive, Peripheral / diagnosis. Reverse Transcriptase Polymerase Chain Reaction / methods. Rhabdomyosarcoma / diagnosis. Sarcoma, Ewing / diagnosis. Sarcoma, Small Cell / diagnosis. Soft Tissue Neoplasms / diagnosis

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  • (PMID = 20231617.001).
  • [ISSN] 1943-7722
  • [Journal-full-title] American journal of clinical pathology
  • [ISO-abbreviation] Am. J. Clin. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
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19. Davicioni E, Anderson MJ, Finckenstein FG, Lynch JC, Qualman SJ, Shimada H, Schofield DE, Buckley JD, Meyer WH, Sorensen PH, Triche TJ: Molecular classification of rhabdomyosarcoma--genotypic and phenotypic determinants of diagnosis: a report from the Children's Oncology Group. Am J Pathol; 2009 Feb;174(2):550-64
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  • [Title] Molecular classification of rhabdomyosarcoma--genotypic and phenotypic determinants of diagnosis: a report from the Children's Oncology Group.
  • Rhabdomyosarcoma (RMS) in children occurs as two major histological subtypes, embryonal (ERMS) and alveolar (ARMS).
  • ERMS is associated with an 11p15.5 loss of heterozygosity (LOH) and may be confused with nonmyogenic, non-RMS soft tissue sarcomas.
  • Here, we describe a genomic-based classification scheme that is derived from the combined gene expression profiling and LOH analysis of 160 cases of RMS and non-RMS soft tissue sarcomas that is at variance with conventional histopathological schemes.
  • These objectively derived molecular classes are based solely on genomic analysis at the time of diagnosis and are highly reproducible.

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  • (PMID = 19147825.001).
  • [ISSN] 1525-2191
  • [Journal-full-title] The American journal of pathology
  • [ISO-abbreviation] Am. J. Pathol.
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U01 CA088199; United States / NCI NIH HHS / CA / U01 CA114757; United States / NCI NIH HHS / CA / CA-88199; United States / NCI NIH HHS / CA / U01CA-114757
  • [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 / Biomarkers, Tumor; 0 / HMGA2 Protein; 0 / TFAP2B protein, human; 0 / Transcription Factor AP-2
  • [Other-IDs] NLM/ PMC2630563
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20. 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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21. Hosoi H, Kakazu N, Konishi E, Tsuchihashi Y, Hada S, Amaya E, Nakabayahi Y, Misawa-Furihata A, Tabata-Maruyama H, Iehara T, Sugimoto T, Yamane H, Yamasaki M, Shiwaku K, Yanagisawa A, Ono M, Tokiwa K, Iwai N, Hashiba M, Abe T, Sawada T: A novel PAX3 rearrangement in embryonal rhabdomyosarcoma. Cancer Genet Cytogenet; 2009 Mar;189(2):98-104
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  • [Title] A novel PAX3 rearrangement in embryonal rhabdomyosarcoma.
  • Rhabdomyosarcoma is the most common soft tissue tumor seen in children and young adults, and it can be classified into 2 major histological subtypes, alveolar and embryonal.
  • In the alveolar subtype, 2 recurrent chromosomal translocations, t(2;13)(q35;q14) and its variant t(1;13)(p36;q14), have been identified as the specific cytogenetic abnormalities.
  • In this study, we analyzed the complex chromosomal translocation in one case with embryonal rhabdomyosarcoma by means of spectral karyotyping (SKY) and identified a novel translocation involving chromosome band 2q35, which is the locus of PAX3 gene.
  • [MeSH-major] Mutation. Paired Box Transcription Factors / genetics. Rhabdomyosarcoma, Embryonal / genetics. Urogenital Neoplasms / genetics

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  • (PMID = 19215790.001).
  • [ISSN] 1873-4456
  • [Journal-full-title] Cancer genetics and cytogenetics
  • [ISO-abbreviation] Cancer Genet. Cytogenet.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / PAX3 protein, human; 0 / PAX3-FOXO1A fusion protein, human; 0 / Paired Box Transcription Factors; 0 / Recombinant Fusion Proteins
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22. Wurm J, Constantinidis J, Grabenbauer GG, Iro H: Rhabdomyosarcomas of the nose and paranasal sinuses: treatment results in 15 cases. Otolaryngol Head Neck Surg; 2005 Jul;133(1):42-50
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  • Treatment of these tumors usually is conducted according to standardized therapy protocols like the German Cooperative Soft Tissue Sarcoma Study (CWS) or the Intergroup Rhabdomyosarcoma Study (IRS).
  • Histologic subtypes encompassed 9 embryonal (e) and 6 alveolar (a) RMS.
  • [MeSH-major] Nose Neoplasms / therapy. Paranasal Sinus Neoplasms / therapy. Rhabdomyosarcoma / therapy

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  • (PMID = 16025051.001).
  • [ISSN] 0194-5998
  • [Journal-full-title] Otolaryngology--head and neck surgery : official journal of American Academy of Otolaryngology-Head and Neck Surgery
  • [ISO-abbreviation] Otolaryngol Head Neck Surg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
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23. Bui Nguyen Binh M, Collin F, Coindre JM: [Soft tissue sarcomas: update on molecular data]. Cancer Radiother; 2006 Feb-Mar;10(1-2):15-21
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  • [Title] [Soft tissue sarcomas: update on molecular data].
  • Soft tissue sarcomas are rare and may be a source of problems for diagnosis and treatment.
  • Detection of these disorders may help in diagnosis and in determining prognosis.
  • Detection of specific translocation is recommended in synovial sarcoma, alveolar rhabdomyosarcoma or PNET diagnosis because of therapeutic consequences; in case of rarer histologic type (low grade fibromyxoid sarcoma, clear cell sarcoma, infantile fibrosarcoma...), it may confirm the diagnosis.
  • In some cases, some translocations have a prognostic value (alveolar rhabdomyosarcoma) whereas it is discussed in others (synovial sarcoma).
  • The techniques used to detect these translocations are very sensitive so it may be used to detect microscopical metastasis (bone marrow metastasis of alveolar rhabdomyosarcoma for example).
  • Detection of MDM2 and CDK4 genes amplifications (FISH or quantitative PCR) may be sometimes useful in well differentiated and dedifferentiated liposarcomas diagnosis.
  • Mutation detection of KIT or PDGFRA may help in GIST diagnosis and type of mutation is predictive of response to treatment.
  • [MeSH-major] Sarcoma / genetics. Soft Tissue Neoplasms / genetics

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  • (PMID = 16442827.001).
  • [ISSN] 1278-3218
  • [Journal-full-title] Cancer radiothérapie : journal de la Société française de radiothérapie oncologique
  • [ISO-abbreviation] Cancer Radiother
  • [Language] fre
  • [Publication-type] English Abstract; Journal Article; Review
  • [Publication-country] France
  • [Number-of-references] 35
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24. Cen L, Hsieh FC, Lin HJ, Chen CS, Qualman SJ, Lin J: PDK-1/AKT pathway as a novel therapeutic target in rhabdomyosarcoma cells using OSU-03012 compound. Br J Cancer; 2007 Sep 17;97(6):785-91
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  • [Title] PDK-1/AKT pathway as a novel therapeutic target in rhabdomyosarcoma cells using OSU-03012 compound.
  • Rhabdomyosarcoma (RMS) is the most common paediatric soft-tissue sarcoma including two major subtypes, alveolar rhabdomyosarcoma (ARMS) and embryonal rhabdomyosarcoma (ERMS).
  • We examined phosphorylation levels of AKT using ARMS and ERMS tissue microarray and immunohistochemistry staining.
  • [MeSH-major] Antineoplastic Agents / pharmacology. Enzyme Inhibitors / pharmacology. Oncogene Protein v-akt / metabolism. Protein-Serine-Threonine Kinases / metabolism. Pyrazoles / pharmacology. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma / metabolism. Sulfonamides / pharmacology

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  • (PMID = 17848913.001).
  • [ISSN] 0007-0920
  • [Journal-full-title] British journal of cancer
  • [ISO-abbreviation] Br. J. Cancer
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / Antineoplastic Agents; 0 / Chromones; 0 / Enzyme Inhibitors; 0 / Morpholines; 0 / OSU 03012; 0 / Pyrazoles; 0 / Sulfonamides; 154447-36-6 / 2-(4-morpholinyl)-8-phenyl-4H-1-benzopyran-4-one; EC 2.7.11.1 / Oncogene Protein v-akt; EC 2.7.11.1 / Protein-Serine-Threonine Kinases; EC 2.7.11.2 / pyruvate dehydrogenase (acetyl-transferring) kinase
  • [Other-IDs] NLM/ PMC2360380
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25. Zibat A, Missiaglia E, Rosenberger A, Pritchard-Jones K, Shipley J, Hahn H, Fulda S: Activation of the hedgehog pathway confers a poor prognosis in embryonal and fusion gene-negative alveolar rhabdomyosarcoma. Oncogene; 2010 Dec 2;29(48):6323-30
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Activation of the hedgehog pathway confers a poor prognosis in embryonal and fusion gene-negative alveolar rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children and comprises two major histological subtypes: alveolar rhabdomyosarcoma (ARMS) and embryonal rhabdomyosarcoma (ERMS).
  • [MeSH-major] Hedgehog Proteins / physiology. Rhabdomyosarcoma, Alveolar / metabolism. Rhabdomyosarcoma, Embryonal / metabolism. Signal Transduction / physiology
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. Female. Gene Fusion. Humans. Infant. Kruppel-Like Transcription Factors / analysis. Male. Myogenic Regulatory Factor 5 / analysis. Nerve Tissue Proteins / analysis. Prognosis. Receptors, Cell Surface / analysis. Transcription Factors / analysis

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  • (PMID = 20818440.001).
  • [ISSN] 1476-5594
  • [Journal-full-title] Oncogene
  • [ISO-abbreviation] Oncogene
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / GLI1 protein, human; 0 / GLI3 protein, human; 0 / Hedgehog Proteins; 0 / Kruppel-Like Transcription Factors; 0 / MYF5 protein, human; 0 / Myogenic Regulatory Factor 5; 0 / Nerve Tissue Proteins; 0 / Receptors, Cell Surface; 0 / Transcription Factors; 0 / patched receptors
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26. Bolger JC, Walsh JC, Hughes RE, Eustace SJ, Harrington P: Alveolar rhabdomyosarcoma originating between the fourth and fifth metatarsal--case report and literature review. Foot Ankle Surg; 2010 Sep;16(3):e51-4
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  • [Title] Alveolar rhabdomyosarcoma originating between the fourth and fifth metatarsal--case report and literature review.
  • We report a case of alveolar rhabdomyosarcoma arising between the fourth and fifth metatarsal.
  • Plain radiographs and MRI showed a soft tissue mass displacing the fourth metatarsal.
  • Percutaneous biopsy revealed an alveolar rhabdomyosarcoma.
  • [MeSH-major] Metatarsus. Rhabdomyosarcoma, Alveolar / diagnosis
  • [MeSH-minor] Adolescent. Biopsy. Diagnosis, Differential. Follow-Up Studies. Humans. Magnetic Resonance Imaging. Male. Positron-Emission Tomography

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  • [Copyright] Copyright (c) 2010 European Foot and Ankle Society. Published by Elsevier Ltd. All rights reserved.
  • (PMID = 20654999.001).
  • [ISSN] 1460-9584
  • [Journal-full-title] Foot and ankle surgery : official journal of the European Society of Foot and Ankle Surgeons
  • [ISO-abbreviation] Foot Ankle Surg
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] France
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27. Breitfeld PP, Meyer WH: Rhabdomyosarcoma: new windows of opportunity. Oncologist; 2005 Aug;10(7):518-27
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  • [Title] Rhabdomyosarcoma: new windows of opportunity.
  • Rhabdomyosarcoma is a highly malignant, small blue cell tumor characterized by muscle differentiation.
  • Adequate biopsy to obtain sufficient tissue for accurate diagnosis and molecular characterization is critical.
  • Patients must be assessed for tumor extent; the Intergroup Rhabdomyosarcoma Study (IRS) clinical group and Staging system is universally applied in North America.
  • Local control relies on complete surgical excision when possible; those whose tumors are not completely excised and those with alveolar histology tumors require local irradiation to maximize local control.
  • [MeSH-major] Rhabdomyosarcoma. Soft Tissue Neoplasms

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  • (PMID = 16079319.001).
  • [ISSN] 1083-7159
  • [Journal-full-title] The oncologist
  • [ISO-abbreviation] Oncologist
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 99
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28. Barr FG, Smith LM, Lynch JC, Strzelecki D, Parham DM, Qualman SJ, Breitfeld PP: Examination of gene fusion status in archival samples of alveolar rhabdomyosarcoma entered on the Intergroup Rhabdomyosarcoma Study-III trial: a report from the Children's Oncology Group. J Mol Diagn; 2006 May;8(2):202-8
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  • [Title] Examination of gene fusion status in archival samples of alveolar rhabdomyosarcoma entered on the Intergroup Rhabdomyosarcoma Study-III trial: a report from the Children's Oncology Group.
  • Alveolar rhabdomyosarcoma (ARMS) is a soft tissue cancer in which chromosomal translocations generate PAX3-FKHR and PAX7-FKHR gene fusions.
  • We applied this approach to a convenience sample of 78 formalin-fixed, paraffin-embedded ARMS tumors from the Intergroup Rhabdomyosarcoma Study (IRS)-III clinical trial and obtained satisfactory results in 59 (76%) cases.

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  • (PMID = 16645206.001).
  • [ISSN] 1525-1578
  • [Journal-full-title] The Journal of molecular diagnostics : JMD
  • [ISO-abbreviation] J Mol Diagn
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / CA24507; United States / NCI NIH HHS / CA / CA89461; United States / NCI NIH HHS / CA / CA98543; United States / NCI NIH HHS / CA / U10 CA098413; United States / NCI NIH HHS / CA / U10 CA098543; United States / NCI NIH HHS / CA / CA98413; United States / NCI NIH HHS / CA / U10 CA024507; United States / NCI NIH HHS / CA / R01 CA089461
  • [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 / Neoplasm Proteins; 0 / PAX3 protein, human; 0 / PAX7 Transcription Factor; 0 / PAX7 protein, human; 0 / Paired Box Transcription Factors; 0 / RNA, Messenger
  • [Other-IDs] NLM/ PMC1867584
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29. 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.
  • This trend may have been attributable in part to shifts in diagnosis, because a significant negative trend in RMS, not otherwise specified was observed concurrently.

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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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30. Jain S, Xu R, Prieto VG, Lee P: Molecular classification of soft tissue sarcomas and its clinical applications. Int J Clin Exp Pathol; 2010;3(4):416-28
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  • [Title] Molecular classification of soft tissue sarcomas and its clinical applications.
  • Sarcomas are a heterogeneous group of tumors that are traditionally classified according to the morphology and type of tissue that they resemble, such as rhabdomyosarcoma, which resembles skeletal muscle.
  • These specific genetic alterations are an important adjunct to standard morphological and immunohistochemical diagnoses, and in some cases have a prognostic value, e. g., Ewing family tumors, synovial sarcoma, and alveolar rhabdomyosarcoma.
  • [MeSH-major] Sarcoma / classification. Sarcoma / genetics. Soft Tissue Neoplasms / classification. Soft Tissue Neoplasms / genetics

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  • (PMID = 20490332.001).
  • [ISSN] 1936-2625
  • [Journal-full-title] International journal of clinical and experimental pathology
  • [ISO-abbreviation] Int J Clin Exp Pathol
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 45
  • [Other-IDs] NLM/ PMC2872748
  • [Keywords] NOTNLM ; Sarcomas / molecular classification / molecular genetics / soft tissue tumors / specific genetic translocation
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31. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] [Statistical study of the evolution over ten years of the clinical and therapeutic approach in childhood soft tissue sarcoma].
  • Soft tissue sarcoma has a primitive mesenchymal origin and represents a heterogeneous group of malignant entities with a continuous rising frequency in the age range below 18.
  • 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.
  • We included in the study a well-known category of soft tissue tumors called of uncertain malignancy.
  • 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).
  • CONCLUSIONS: The continuously augmented incidence of soft tissue sarcoma in young ages and the advanced stages initial presentations of tumors require a much more serious and rhythmic survey at general practitioner level.
  • [MeSH-major] Sarcoma / pathology. Sarcoma / therapy. Soft Tissue Neoplasms / pathology. Soft Tissue Neoplasms / therapy

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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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32. Gattenlöhner S, Jörissen H, Huhn M, Vincent A, Beeson D, Tzartos S, Mamalaki A, Etschmann B, Muller-Hermelink HK, Koscielniak E, Barth S, Marx A: A human recombinant autoantibody-based immunotoxin specific for the fetal acetylcholine receptor inhibits rhabdomyosarcoma growth in vitro and in a murine transplantation model. J Biomed Biotechnol; 2010;2010:187621
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  • [Title] A human recombinant autoantibody-based immunotoxin specific for the fetal acetylcholine receptor inhibits rhabdomyosarcoma growth in vitro and in a murine transplantation model.
  • Rhabdomyosarcoma (RMS) is the most common malignant soft tissue tumor in children and is highly resistant to all forms of treatment currently available once metastasis or relapse has commenced.
  • While scFv35-ETA had no damaging effect on fAChR-negative control cell lines, it killed human embryonic and alveolar RMS cell lines in vitro and delayed RMS development in a murine transplantation model.
  • [MeSH-major] ADP Ribose Transferases / administration & dosage. Autoantibodies / immunology. Bacterial Toxins / administration & dosage. Exotoxins / administration & dosage. Immunotoxins / administration & dosage. Receptors, Nicotinic / immunology. Recombinant Fusion Proteins / administration & dosage. Rhabdomyosarcoma / drug therapy. Single-Chain Antibodies / immunology. Virulence Factors / administration & dosage

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  • (PMID = 20204062.001).
  • [ISSN] 1110-7251
  • [Journal-full-title] Journal of biomedicine & biotechnology
  • [ISO-abbreviation] J. Biomed. Biotechnol.
  • [Language] eng
  • [Grant] United Kingdom / Wellcome Trust / / 084655; United Kingdom / Medical Research Council / / G0701521
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Autoantibodies; 0 / Bacterial Toxins; 0 / CHRNG protein, human; 0 / Exotoxins; 0 / Immunotoxins; 0 / Receptors, Nicotinic; 0 / Recombinant Fusion Proteins; 0 / Single-Chain Antibodies; 0 / Virulence Factors; EC 2.4.2.- / ADP Ribose Transferases; EC 2.4.2.31 / toxA protein, Pseudomonas aeruginosa
  • [Other-IDs] NLM/ PMC2829619
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33. Huh WW, Skapek SX: Childhood rhabdomyosarcoma: new insight on biology and treatment. Curr Oncol Rep; 2010 Nov;12(6):402-10
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Childhood rhabdomyosarcoma: new insight on biology and treatment.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood.
  • The two most common histologic variants are the embryonal and alveolar subtypes.
  • Recent studies have pointed to a possible mesenchymal stem cell as the progenitor for alveolar RMS.
  • [MeSH-major] Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Alveolar / therapy. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / genetics. Rhabdomyosarcoma, Embryonal / therapy. Soft Tissue Neoplasms / diagnosis. Soft Tissue Neoplasms / genetics. Soft Tissue Neoplasms / therapy

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  • (PMID = 20820958.001).
  • [ISSN] 1534-6269
  • [Journal-full-title] Current oncology reports
  • [ISO-abbreviation] Curr Oncol Rep
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Protein Kinase Inhibitors; EC 2.7.1.1 / TOR Serine-Threonine Kinases; W36ZG6FT64 / Sirolimus
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34. Yuan XJ, Chan GC, Chan SK, Shek TW, Kwong DL, Wei WI, Ha SY, Chiang AK: Treatment outcome of rhabdomyosarcoma in Hong Kong Chinese children. Hong Kong Med J; 2008 Apr;14(2):116-23
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  • [Title] Treatment outcome of rhabdomyosarcoma in Hong Kong Chinese children.
  • OBJECTIVES: To review the treatment outcome of rhabdomyosarcoma in Hong Kong Chinese children.
  • PATIENTS: Consecutive cases of rhabdomyosarcoma diagnosed and treated by the Department of Paediatrics and Adolescent Medicine of Queen Mary Hospital between 1989 and 2005.
  • Each patient was staged and treated according to the Intergroup Rhabdomyosarcoma Study guidelines.
  • The median age at diagnosis was 6 (range, 0.5-17) years.
  • Primary sites of rhabdomyosarcoma included: the head and neck (n=8; 6 classified as cranial parameningeal), genitourinary (3), extremity (3), pelvis (3), and trunk (2).
  • Thirteen (68%) had embryonal and six (32%) had alveolar histology.
  • Two, 2, 9, and 6 were classified as belonging to Intergroup Rhabdomyosarcoma Study groups 1, 2, 3, and 4, respectively.
  • In non-metastatic cases (Intergroup Rhabdomyosarcoma Study groups 1-3), the 5-year overall survival rate was 66% (95% confidence interval, 39-93%) and in metastatic cases (group 4) it was 17% (0-46%).
  • CONCLUSIONS: Treatment outcome of rhabdomyosarcoma in this cohort of Chinese children was less favourable than that reported in international studies.

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  • (PMID = 18382018.001).
  • [ISSN] 1024-2708
  • [Journal-full-title] Hong Kong medical journal = Xianggang yi xue za zhi
  • [ISO-abbreviation] Hong Kong Med J
  • [Language] ENG
  • [Publication-type] Journal Article
  • [Publication-country] China
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35. 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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  • [Title] Sentinel node biopsy in pediatric soft tissue sarcomas of extremities.
  • Our experience with sentinel node biopsy in soft tissue sarcomas of extremities in children is reported.
  • 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.
  • No further lymph node metastases were recognized either at diagnosis or during the follow-up (6-78 months).
  • 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
  • [MeSH-minor] Adolescent. Child. Child, Preschool. Female. Humans. Male. Neoplasm Metastasis / pathology. Sentinel Lymph Node Biopsy. Soft Tissue Neoplasms / pathology. Young Adult

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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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36. Wang ZH, Shi HY, Wang ZB: [Metastatic alveolar soft tissue sarcoma of the central nervous system: a clinicopathological analysis of four cases]. Ai Zheng; 2009 Nov;28(11):1214-8
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  • [Title] [Metastatic alveolar soft tissue sarcoma of the central nervous system: a clinicopathological analysis of four cases].
  • BACKGROUND AND OBJECTIVE: Metastatic alveolar soft tissue sarcoma (ASTS) of the central nervous system is rare and is easy to be misdiagnosed as other primary tumors of central nervous system.
  • This study was to analyze the clinical and pathological features of four patients with ASTS of the central nervous system and to clarify their differential diagnosis as well as prognosis.
  • The tumor cells had clear or eosinophilic cytoplasm and prominent nucleoli, arranged in alveolar structures, which were surrounded by delicate blood sinuses.
  • CONCLUSION: ASTS of the central nervous system was mostly metastatic and should be differentiated from other CNS tumors such as meningioma, melonocytic tumor, rhabdomyosarcoma and paraganglioma.
  • [MeSH-major] Cranial Fossa, Posterior. Sarcoma, Alveolar Soft Part / pathology. Sarcoma, Alveolar Soft Part / secondary. Skull Base Neoplasms / pathology. Skull Base Neoplasms / secondary
  • [MeSH-minor] Actins / metabolism. Adult. Desmin / metabolism. Diagnosis, Differential. Female. Follow-Up Studies. Humans. Male. Meningeal Neoplasms / diagnosis. Meningioma / diagnosis. Neoplasm Recurrence, Local. Paraganglioma / diagnosis. Prognosis. Rhabdomyosarcoma / diagnosis. S100 Proteins / metabolism. Survival Rate

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  • (PMID = 19895745.001).
  • [Journal-full-title] Ai zheng = Aizheng = Chinese journal of cancer
  • [ISO-abbreviation] Ai Zheng
  • [Language] chi
  • [Publication-type] English Abstract; Journal Article
  • [Publication-country] China
  • [Chemical-registry-number] 0 / Actins; 0 / Desmin; 0 / S100 Proteins
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37. Timmermann B, Schuck A, Niggli F, Weiss M, Lomax A, Goitein G: ["Spot-scanning" proton therapy for rhabdomyosarcomas of early childhood. First experiences at PSI]. Strahlenther Onkol; 2006 Nov;182(11):653-9
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  • RESULTS: Nine children were included aged 0.9-3.8 years (embryonal RMS in six, and alveolar, undifferentiated or nonclassified in one each).
  • [MeSH-major] Protons / therapeutic use. Rhabdomyosarcoma / radiotherapy. Soft Tissue Neoplasms / radiotherapy
  • [MeSH-minor] Adolescent. Age Factors. Child, Preschool. Combined Modality Therapy. Feasibility Studies. Female. Head and Neck Neoplasms / radiotherapy. Humans. Infant. Infant, Newborn. Male. Meningeal Neoplasms / radiotherapy. Orbital Neoplasms / radiotherapy. Prospective Studies. Prostatic Neoplasms / radiotherapy. Radiotherapy Planning, Computer-Assisted. Rhabdomyosarcoma, Alveolar / radiotherapy. Rhabdomyosarcoma, Embryonal / radiotherapy. Survival Analysis. Treatment Outcome

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  • (PMID = 17072523.001).
  • [ISSN] 0179-7158
  • [Journal-full-title] Strahlentherapie und Onkologie : Organ der Deutschen Röntgengesellschaft ... [et al]
  • [ISO-abbreviation] Strahlenther Onkol
  • [Language] ger
  • [Publication-type] Comparative Study; English Abstract; Evaluation Studies; Journal Article
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Protons
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38. 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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39. Kuhnen C, Herter P, Leuschner I, Mentzel T, Druecke D, Jaworska M, Johnen G: Sclerosing pseudovascular rhabdomyosarcoma-immunohistochemical, ultrastructural, and genetic findings indicating a distinct subtype of rhabdomyosarcoma. Virchows Arch; 2006 Nov;449(5):572-8
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  • [Title] Sclerosing pseudovascular rhabdomyosarcoma-immunohistochemical, ultrastructural, and genetic findings indicating a distinct subtype of rhabdomyosarcoma.
  • Sclerosing (pseudovascular) rhabdomyosarcoma in adults has been described as a rare variant of rhabdomyosarcoma characterized by extensive hyaline fibrosis and pseudovascular growth patterns.
  • We describe another case of this rhabdomyosarcoma subtype including ultrastructural and genetic findings-the lesion presented in a 62-year-old male patient in the left lower leg.
  • The tumor was located within the deep soft tissue with maximum diameter of 11.8 cm and skin ulceration.
  • Reciprocal translocations t(1;13) and t(2;13)(q35;q14) which are characteristic of alveolar rhabdomyosarcoma could be excluded.
  • These findings, while showing a relation to other rhabdomyosarcoma subtypes, represent a relatively circumscribed genetic defect pattern in sclerosing (pseudovascular) rhabdomyosarcoma that is somewhat different from patterns described in most other rhabdomyosarcoma subtypes.
  • Further studies should concentrate on the identification of genes especially on chromosomal region 10q22 to elucidate more aspects in the pathogenesis of this rhabdomyosarcoma subtype.
  • [MeSH-major] Chromosome Aberrations. Rhabdomyosarcoma / pathology. Soft Tissue Neoplasms / pathology

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40. Odoi AT, Dassah ET, Darkey DE, Owusu-Afriyie O, Valkov AY: Advanced alveolar rhabdomyosarcoma of the uterus: a case report. Afr J Reprod Health; 2009 Mar;13(1):167-73
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  • [Title] Advanced alveolar rhabdomyosarcoma of the uterus: a case report.
  • Alveolar rhabdomyosarcoma is an uncommon malignant soft tissue tumour rarely found in the female genital tract and carries a very poor prognosis especially in adults.
  • The cervical, uterine and omental biopsies were all diagnosed alveolar rhabdomyosarcoma.
  • While the optimal management of this rare tumour is unknown, early recognition and diagnosis, and a prompt multimodality treatment approach of surgery, chemotherapy and radiotherapy offers the best chance of cure.
  • [MeSH-major] Omentum / pathology. Peritoneal Neoplasms / pathology. Rhabdomyosarcoma, Alveolar / pathology. Uterine Neoplasms / pathology

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  • (PMID = 20687274.001).
  • [ISSN] 1118-4841
  • [Journal-full-title] African journal of reproductive health
  • [ISO-abbreviation] Afr J Reprod Health
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Nigeria
  • [Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / PAX3-FKHR fusion protein, human
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41. 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
  • [MeSH-minor] Adolescent. Diagnostic Errors. Fatal Outcome. Humans. Magnetic Resonance Imaging. Male. Myoma / diagnosis. Positron-Emission Tomography

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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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42. Strahm B, Durbin AD, Sexsmith E, Malkin D: The CXCR4-SDF1alpha axis is a critical mediator of rhabdomyosarcoma metastatic signaling induced by bone marrow stroma. Clin Exp Metastasis; 2008;25(1):1-10
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  • [Title] The CXCR4-SDF1alpha axis is a critical mediator of rhabdomyosarcoma metastatic signaling induced by bone marrow stroma.
  • Rhabdomyosarcoma (RMS) is the most common malignant soft-tissue tumor of childhood.
  • CXCR4 was expressed in RMS cell lines and primary tumors, with higher expression in alveolar subtype RMS.
  • [MeSH-major] Chemokine CXCL12 / metabolism. Neoplasm Invasiveness / physiopathology. Receptors, CXCR4 / metabolism. Rhabdomyosarcoma / pathology. Signal Transduction / physiology

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  • (PMID = 17768666.001).
  • [ISSN] 0262-0898
  • [Journal-full-title] Clinical & experimental metastasis
  • [ISO-abbreviation] Clin. Exp. Metastasis
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / CXCR4 protein, human; 0 / Chemokine CXCL12; 0 / Culture Media, Conditioned; 0 / Receptors, CXCR4
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43. 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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44. 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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  • [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
  • [MeSH-minor] Biomarkers, Tumor / metabolism. Cell Line, Tumor. Fluorescent Antibody Technique, Direct. Forkhead Transcription Factors / genetics. Forkhead Transcription Factors / metabolism. Gene Expression Profiling. Gene Expression Regulation, Neoplastic / genetics. Humans. Paired Box Transcription Factors / genetics. Paired Box Transcription Factors / metabolism. RNA, Messenger / metabolism. RNA, Neoplasm / analysis. Tissue Array Analysis

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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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45. Aitken L, Levin D, Blau A, Lewin MR: Acute hearing loss, dysarthria, dysphagia, and a rubbery intraoral mass in an 18-year-old woman. Pediatr Emerg Care; 2009 Aug;25(8):516-8
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  • Rhabdomyosarcoma is the most common soft tissue tumor of childhood, frequently presenting in the head and neck, genitourinary tract, or extremities.
  • We present a case of rhabdomyosarcoma in which an 18-year-old woman presented with abrupt onset unilateral hearing loss, tinnitus, dysarthria, dysphagia, and a new painless red bump on the palate.
  • With an alveolar subtype and older age, both predictors of poor prognosis, early recognition of disease of these symptoms is vital.
  • [MeSH-major] Deglutition Disorders / etiology. Dysarthria / etiology. Hearing Loss, Sensorineural / etiology. Palatal Neoplasms / diagnosis. Rhabdomyosarcoma, Alveolar / diagnosis. Tinnitus / etiology

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  • (PMID = 19687710.001).
  • [ISSN] 1535-1815
  • [Journal-full-title] Pediatric emergency care
  • [ISO-abbreviation] Pediatr Emerg Care
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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46. Fatusi OA, Ajike SO, Olateju SO, Adebayo AT, Gbolahan OO, Ogunmuyiwa SA: Clinico-epidemiological analysis of orofacial rhabdomyosarcoma in a Nigerian population. Int J Oral Maxillofac Surg; 2009 Mar;38(3):256-60
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  • [Title] Clinico-epidemiological analysis of orofacial rhabdomyosarcoma in a Nigerian population.
  • Rhabdomyosarcoma (RMS) is a malignant soft tissue neoplasm, with varying degrees of striated muscle cell differentiation and a relative predilection for the head and neck region.
  • Embryonal and alveolar subtypes were mainly found in the maxilla.
  • [MeSH-major] Facial Neoplasms / epidemiology. Head and Neck Neoplasms / epidemiology. Mouth Neoplasms / epidemiology. Neoplasms, Multiple Primary / epidemiology. Rhabdomyosarcoma / epidemiology


47. Milanovic R, Vlajcic Z, Zic R, Stanec S, Rudman F, Stanec Z: Alveolar rhabdomyosarcoma of the hand in a 2 year-old child. J Hand Surg Eur Vol; 2007 Feb;32(1):109-10
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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 of the hand in a 2 year-old child.
  • [MeSH-major] Hand / surgery. Rhabdomyosarcoma, Alveolar / surgery. Soft Tissue Neoplasms / surgery

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  • (PMID = 17049698.001).
  • [ISSN] 1753-1934
  • [Journal-full-title] The Journal of hand surgery, European volume
  • [ISO-abbreviation] J Hand Surg Eur Vol
  • [Language] eng
  • [Publication-type] Case Reports; Letter
  • [Publication-country] Scotland
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48. 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.
  • PURPOSE: To describe the outcome of infants with a histologically confirmed diagnosis of malignant mesenchymal tumor (MMT) included in the International Society of Paediatric Oncology studies MMT 84 and MMT 89.
  • 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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49. Croes R, Debiec-Rychter M, Cokelaere K, De Vos R, Hagemeijer A, Sciot R: Adult sclerosing rhabdomyosarcoma: cytogenetic link with embryonal rhabdomyosarcoma. Virchows Arch; 2005 Jan;446(1):64-7
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  • [Title] Adult sclerosing rhabdomyosarcoma: cytogenetic link with embryonal rhabdomyosarcoma.
  • Rhabdomyosarcomas are classified into three well-defined categories: embryonal, alveolar and pleomorphic rhabdomyosarcoma.
  • Recently, seven cases of an unusual adult type of rhabdomyosarcoma with a prominent hyaline sclerosis have been described.
  • We report the hitherto unreported cytogenetic changes of an adult sclerosing rhabdomyosarcoma.
  • A 79-year-old woman underwent an amputation for a rapidly growing soft tissue mass in the anterior compartment of the right lower leg.
  • Using fluorescent in situ hybridization (FISH) analysis, the tumor cells were negative for FOXO1A-disrupting translocations specific for alveolar rhabdomyosarcoma.
  • The chromosomal composition of malignant cells resembled the pattern of numerical changes frequently observed in embryonal rhabdomyosarcoma, suggesting a close relationship of an adult sclerosing rhabdomyosarcoma with this entity.
  • [MeSH-major] Rhabdomyosarcoma / genetics. Rhabdomyosarcoma, Embryonal / genetics. Translocation, Genetic

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  • (PMID = 15660283.001).
  • [ISSN] 0945-6317
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Germany
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50. Moon HS, Kwon SW, Lee JH: A case of alveolar rhabdomyosarcoma of the ethmoid sinus invading the orbit in an adult. Korean J Ophthalmol; 2006 Mar;20(1):70-5
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  • [Title] A case of alveolar rhabdomyosarcoma of the ethmoid sinus invading the orbit in an adult.
  • PURPOSE: A case study and literature review of alveolar rhabdomyosarcoma (RMS) in an adult.
  • A computed tomography (CT) scan revealed a destructive soft-tissue mass in the left ethmoid sinus with invasion of the left orbit and compression of the medial rectus muscle.
  • Endoscopic intranasal biopsy revealed alveolar RMS.
  • RESULTS: Immunohistochemical testing was positive for desmin, S-100, and smooth muscle actin (SMA), supporting the diagnosis of RMS.
  • CONCLUSIONS: Although rarely found in adults, RMS should be considered in the differential diagnosis of orbital tumors.
  • Immunohistochemical analysis plays an important role in the definitive diagnosis of RMS.
  • [MeSH-major] Ethmoid Sinus. Orbital Neoplasms / pathology. Paranasal Sinus Neoplasms / pathology. Rhabdomyosarcoma, Alveolar / pathology
  • [MeSH-minor] Biopsy. Diagnosis, Differential. Humans. Magnetic Resonance Imaging. Male. Middle Aged. Neoplasm Invasiveness. Tomography, X-Ray Computed

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  • (PMID = 16768194.001).
  • [ISSN] 1011-8942
  • [Journal-full-title] Korean journal of ophthalmology : KJO
  • [ISO-abbreviation] Korean J Ophthalmol
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] Korea (South)
  • [Other-IDs] NLM/ PMC2908821
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51. Meng MV, Grossfeld GD, Sudilovsky D, Baehner FL: Fine needle aspiration cytology of adult perineal rhabdomyosarcoma: a case report. Acta Cytol; 2006 Jan-Feb;50(1):88-92
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  • [Title] Fine needle aspiration cytology of adult perineal rhabdomyosarcoma: a case report.
  • BACKGROUND: Adult perineal soft tissue sarcomas are rare.
  • Below we report a case in which the diagnosis was established preoperatively by fine needle aspiration (FNA).
  • The diagnosis of rhabdomyosarcoma was favored on FNA and was corroborated by immunohistochemical stains for desmin, myogenin and CD56.
  • Upon surgical resection, the diagnosis of alveolar rhabdomyosarcoma was confirmed histologically and immunophenotypically.
  • CONCLUSION: FNA is a useful tool in diagnosing soft tissue lessions of the perineum, including rare primary tumors, such as adult rhabdomyosarcoma.
  • [MeSH-major] Perineum / pathology. Rhabdomyosarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 16514847.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Desmin; 0 / Myogenin
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52. Fisher C: Soft tissue sarcomas with non-EWS translocations: molecular genetic features and pathologic and clinical correlations. Virchows Arch; 2010 Feb;456(2):153-66
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  • [Title] Soft tissue sarcomas with non-EWS translocations: molecular genetic features and pathologic and clinical correlations.
  • Many soft tissue sarcoma subtypes have consistent chromosomal translocations with novel fusion genes, which result in disordered cellular function.
  • These include synovial sarcoma, alveolar rhabdomyosarcoma, alveolar soft part sarcoma, dermatofibrosarcoma protuberans, low-grade fibromyxoid sarcoma, infantile fibrosarcoma and inflammatory myofibroblastic tumour.

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  • (PMID = 19396640.001).
  • [ISSN] 1432-2307
  • [Journal-full-title] Virchows Archiv : an international journal of pathology
  • [ISO-abbreviation] Virchows Arch.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] Germany
  • [Number-of-references] 196
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53. 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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  • 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.
  • 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


54. Kilpatrick SE, Bergman S, Pettenati MJ, Gulley ML: The usefulness of cytogenetic analysis in fine needle aspirates for the histologic subtyping of sarcomas. Mod Pathol; 2006 Jun;19(6):815-9
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  • Conventional cytogenetic analysis performed from open biopsy tissue samples may be a useful adjunct for the histologic subtyping of bone and soft tissue sarcomas.
  • We retrospectively reviewed 24 consecutive FNAB bone and soft tissue sarcoma specimens, procured from 1995 to 2003, in which aspirated material was obtained for cytogenetic analysis.
  • The t(2;13) was strongly suggested in one alveolar rhabdomyosarcoma.
  • For two of these cases (both of which were synovial sarcomas), cytogenetic analysis was necessary for definitive diagnosis.
  • Cytogenetic analysis was noncontributory (eg no growth) in 14 sarcoma cases, but excluding the case of atypical lipoma, this did not preclude the rendering of an accurate diagnosis.
  • Cytogenetic analysis can be performed on FNAB specimens from bone and soft tissue sarcomas and may be a useful diagnostic aid in difficult cases.
  • [MeSH-major] Bone Neoplasms / pathology. Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 16557276.001).
  • [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
  • [Publication-country] United States
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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. 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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  • No translocations associated with Ewing sarcoma, synovial sarcoma, or alveolar rhabdomyosarcoma were found.
  • [MeSH-major] Sarcoma / pathology. Soft Tissue Neoplasms / pathology

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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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57. 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.
  • [Title] Incidence and outcomes of extremity soft-tissue sarcomas in children.
  • The median age at diagnosis was 12 y, but most patients were ≥10 y of age (72%, n = 842).
  • Most tumors were non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) (79%, n = 879).
  • The most common tissue of origin was muscle (43%, n = 474).
  • Most rhabdomyosarcoma (RMS) (n = 220) were alveolar type (n = 140).
  • Multivariate analysis identified RMS (HR 2.20, P < 0.001), nerve and muscle (not synovial sarcoma) tissue of origin (HR 2.26, P = 0.002, and HR 1.59, P = 0.036), regional or distant disease (HR 1.65, P = 0.011, and HR 5.96, P < 0.001, respectively), and lack of surgical intervention (HR 2.20, P < 0.001) as independent predictors of poor outcome.
  • [MeSH-major] Extremities. Sarcoma / epidemiology. Soft Tissue Neoplasms / epidemiology
  • [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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58. Siegel HJ, Connor GS, Lee D, Lopez-Ben R, Kelly DR: Synchronous bifocal alveolar rhabdomyosarcoma: a case report. J Bone Joint Surg Br; 2006 Jul;88(7):955-8
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  • [Title] Synchronous bifocal alveolar rhabdomyosarcoma: a case report.
  • We report a case of bifocal rhabdomyosarcoma involving the hand and thigh in an 11-year-old female.
  • [MeSH-major] Rhabdomyosarcoma, Alveolar / pathology. Soft Tissue Neoplasms / pathology

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  • (PMID = 16799004.001).
  • [ISSN] 0301-620X
  • [Journal-full-title] The Journal of bone and joint surgery. British volume
  • [ISO-abbreviation] J Bone Joint Surg Br
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] England
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59. 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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60. 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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  • [Title] Current concepts and future perspectives in retroperitoneal soft-tissue sarcoma management.
  • Retroperitoneal soft-tissue sarcomas are complex, heterogeneous cancers requiring expert multidisciplinary care.
  • Pediatric subtypes mainly comprise extraskeletal Ewing sarcoma/pPNET and alveolar rhabdomyosarcoma.

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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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61. Ayati M, Izadpanahi MM, Nowroozi MR, Soroush Z, Jamshidian H, Rakhshani N: First case report of bilateral metastatic intratesticular rhabdomyosarcoma. Urology; 2010 Jun;75(6):1485-7
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  • [Title] First case report of bilateral metastatic intratesticular rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS) is the most common soft-tissue sarcoma in children.
  • We present a case of an 18-year-old boy with metastatic alveolar RMS to the testis and the first reported case of bilateral testicular involvement.
  • [MeSH-major] Lung Neoplasms / pathology. Lymph Nodes / pathology. Rhabdomyosarcoma / secondary. Rhabdomyosarcoma / therapy. Testicular Neoplasms / secondary. Testicular Neoplasms / therapy

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  • [Copyright] Copyright (c) 2010 Elsevier Inc. All rights reserved.
  • (PMID = 19819527.001).
  • [ISSN] 1527-9995
  • [Journal-full-title] Urology
  • [ISO-abbreviation] Urology
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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62. 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.
  • [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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63. Oda Y, Tsuneyoshi M: Recent advances in the molecular pathology of soft tissue sarcoma: implications for diagnosis, patient prognosis, and molecular target therapy in the future. Cancer Sci; 2009 Feb;100(2):200-8
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  • [Title] Recent advances in the molecular pathology of soft tissue sarcoma: implications for diagnosis, patient prognosis, and molecular target therapy in the future.
  • In the present paper, recent advances in the molecular pathology of soft tissue sarcomas (STS) and the implications for their prognostic value are reviewed, and the potential targets of molecular therapy are discussed.
  • In the former group,specific fusion transcripts, such as SS18–SSX, EWS–FLI1, and PAX3–FKHR, could be detected in synovial sarcoma, Ewing's sarcoma and primitive neuroectodermal tumor, and alveolar rhabdomyosarcoma,respectively.
  • [MeSH-major] Biomarkers, Tumor / metabolism. Sarcoma / diagnosis. Sarcoma / therapy


64. Tanas MR, Rubin BP, Tubbs RR, Billings SD, Downs-Kelly E, Goldblum JR: Utilization of fluorescence in situ hybridization in the diagnosis of 230 mesenchymal neoplasms: an institutional experience. Arch Pathol Lab Med; 2010 Dec;134(12):1797-803
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  • [Title] Utilization of fluorescence in situ hybridization in the diagnosis of 230 mesenchymal neoplasms: an institutional experience.
  • OBJECTIVE: To determine the utility of FISH in the diagnosis of mesenchymal neoplasms.
  • DESIGN: Two hundred thirty soft tissue cases analyzed by FISH were reviewed retrospectively.
  • EWSR1 FISH was very sensitive and specific in the differential diagnosis of melanocytic neoplasms (88% of clear cell sarcomas were positive; all melanomas were negative).
  • FOXO1A FISH was positive in ∼70% of cases of alveolar rhabdomyosarcoma.
  • CONCLUSION: FISH is a useful adjunct in the diagnosis of mesenchymal neoplasms.
  • [MeSH-major] In Situ Hybridization, Fluorescence / methods. Melanoma / diagnosis. Neoplasms, Adipose Tissue / diagnosis. Sarcoma / diagnosis. Soft Tissue Neoplasms / diagnosis


65. 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
  • [MeSH-minor] Adaptor Proteins, Signal Transducing / analysis. Adolescent. Child. Child, Preschool. Female. Humans. Immunohistochemistry. Infant. Infant, Newborn. Isoenzymes / analysis. Male. Neoplasm Staging. Phosphoproteins / analysis. Phosphorylation. Pilot Projects. Protein Kinase C / analysis. Protein Kinases / analysis. Receptor Protein-Tyrosine Kinases / analysis. Ribosomal Protein S6 / analysis. Ribosomal Protein S6 Kinases, 70-kDa / analysis. TOR Serine-Threonine Kinases. Tissue Array Analysis

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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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66. Linardic CM, Naini S, Herndon JE 2nd, Kesserwan C, Qualman SJ, Counter CM: The PAX3-FKHR fusion gene of rhabdomyosarcoma cooperates with loss of p16INK4A to promote bypass of cellular senescence. Cancer Res; 2007 Jul 15;67(14):6691-9
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  • [Title] The PAX3-FKHR fusion gene of rhabdomyosarcoma cooperates with loss of p16INK4A to promote bypass of cellular senescence.
  • Rhabdomyosarcoma is the most common soft tissue sarcoma of childhood and adolescence.
  • Despite advances in therapy, patients with a histologic variant of rhabdomyosarcoma known as alveolar rhabdomyosarcoma (ARMS) have a 5-year survival of <30%.
  • This prompted us to explore the effect of expressing PAX3-FKHR in more relevant cells, specifically primary human skeletal muscle cells because these cells can be converted to a tumorigenic state that mimics rhabdomyosarcoma.
  • This association between PAX3-FKHR expression and p16(INK4A) loss was seen in human ARMS tumor tissue, as both human rhabdomyosarcoma cell lines and tissue microarrays showed a trend toward down-regulation of p16(INK4A) protein in alveolar subsets.
  • [MeSH-major] Cyclin-Dependent Kinase Inhibitor p16 / metabolism. Forkhead Transcription Factors / physiology. Gene Expression Regulation, Neoplastic. Lung Neoplasms / metabolism. Paired Box Transcription Factors / physiology. Rhabdomyosarcoma / metabolism

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  • (PMID = 17638879.001).
  • [ISSN] 0008-5472
  • [Journal-full-title] Cancer research
  • [ISO-abbreviation] Cancer Res.
  • [Language] eng
  • [Grant] United States / NICHD NIH HHS / HD / K12 HD043494; United States / NICHD NIH HHS / HD / 5K12 HD 043494; United States / NCI NIH HHS / CA / R01 CA 94184
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cyclin-Dependent Kinase Inhibitor p16; 0 / FOXO1 protein, human; 0 / Forkhead Box Protein O1; 0 / Forkhead Transcription Factors; 0 / PAX3 Transcription Factor; 0 / PAX3 protein, human; 0 / Paired Box Transcription Factors; 0 / Recombinant Fusion Proteins
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67. Qualman S, Lynch J, Bridge J, Parham D, Teot L, Meyer W, Pappo A: Prevalence and clinical impact of anaplasia in childhood rhabdomyosarcoma : a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group. Cancer; 2008 Dec 1;113(11):3242-7
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  • [Title] Prevalence and clinical impact of anaplasia in childhood rhabdomyosarcoma : a report from the Soft Tissue Sarcoma Committee of the Children's Oncology Group.
  • BACKGROUND: Anapalsia is rare in childhood rhabdomyosarcoma and has not been included in the International Classification of Rhabdomyosarcoma (ICR).
  • A recent review of cases from the Soft Tissue Sarcoma Committee of the Children's Oncology Group (COG) suggests that anaplasia might be more common than previously reported and may impact clinical outcome.
  • METHODS: The prevalence of anaplasia (focal or diffuse) was prospectively assessed in 546 eligible cases who were registered in an Intergroup Rhabdomyosarcoma Study Group (IRSG) or COG therapeutic trial from 1995 through 1998.
  • Regardless of its distribution (focal or diffuse), on univariate analysis the presence of anaplasia negatively influenced the failure-free survival rate (63% vs 77% at 5 years) and overall survival (68% vs 82% at 5 years) rates in patients with embryonal rhabdomyosarcoma.
  • Anaplasia did not affect outcome in patients with alveolar tumors.
  • CONCLUSIONS: The incidence of anaplasia in patients with rhabdomyosarcoma is higher than previously described and may be of prognostic significance in children with intermediate-risk embryonal rhabdomyosarcoma.

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  • [Copyright] (c) 2008 American Cancer Society
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  • (PMID = 18985676.001).
  • [ISSN] 0008-543X
  • [Journal-full-title] Cancer
  • [ISO-abbreviation] Cancer
  • [Language] ENG
  • [Grant] United States / NCI NIH HHS / CA / U10 CA098543-06; United States / NCI NIH HHS / CA / U10 CA098413-06; United States / NCI NIH HHS / CA / U10 CA098413; None / None / / U10 CA024507-25; United States / NCI NIH HHS / CA / U10 CA024507-26; United States / NCI NIH HHS / CA / U10 CA098543; None / None / / U10 CA098413-06; None / None / / U10 CA024507-26; United States / NCI NIH HHS / CA / U10 CA024507-25; None / None / / U10 CA098543-06; United States / NCI NIH HHS / CA / U10 CA024507
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS107192; NLM/ PMC2727712
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68. Tanko NM, Echejoh GO, Manasseh NA, Mandong MB, Uba AF: Paediatric solid tumours in Nigerian children: a changing pattern? Afr J Paediatr Surg; 2009 Jan-Jun;6(1):7-10
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  • The commonest malignant tumour diagnosed was rhabdomyosarcoma which accounted for 27 (31%), comprising of 15 (55.6%), 11 (40.7%) and 1 (3.7%) embryonal, alveolar and pleomorphic rhabdomyosarcomas, respectively.
  • CONCLUSION: Based on the result of our study, we conclude that the commonest solid malignancy of childhood in Jos, Nigeria is rhabdomyosarcoma.
  • This has implications for diagnosis, management and prognosis of theses soft tissue sarcomas in our paediatric population.
  • [MeSH-major] Neoplasms / epidemiology. Neoplasms / pathology. Rhabdomyosarcoma / epidemiology. Rhabdomyosarcoma / pathology

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  • (PMID = 19661657.001).
  • [ISSN] 0974-5998
  • [Journal-full-title] African journal of paediatric surgery : AJPS
  • [ISO-abbreviation] Afr J Paediatr Surg
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article
  • [Publication-country] Nigeria
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69. 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).
  • In addition, immunohistochemical pRB LI may have the potential to be a useful ancillary tool in the differential diagnosis of RMS subtypes.
  • [MeSH-major] Retinoblastoma Protein / biosynthesis. Retinoblastoma Protein / genetics. Rhabdomyosarcoma, Alveolar / diagnosis. Rhabdomyosarcoma, Alveolar / genetics. Rhabdomyosarcoma, Embryonal / diagnosis. Rhabdomyosarcoma, Embryonal / genetics
  • [MeSH-minor] Base Sequence. Diagnosis, Differential. Gene Expression. Humans. Immunohistochemistry. Mutation. Reverse Transcriptase Polymerase Chain Reaction


70. Lott S, Lopez-Beltran A, Montironi R, MacLennan GT, Cheng L: Soft tissue tumors of the urinary bladder Part II: malignant neoplasms. Hum Pathol; 2007 Jul;38(7):963-77
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  • [Title] Soft tissue tumors of the urinary bladder Part II: malignant neoplasms.
  • The second half of this two-part review will describe rare nonurothelial malignant tumors of the urinary bladder including leiomyosarcoma, rhabdomyosarcoma, angiosarcoma, malignant fibrous histiocytoma (undifferentiated sarcoma), primitive neuroectodermal tumor, malignant peripheral nerve sheath tumor, hemangiopericytoma, and alveolar soft-parts sarcoma.
  • [MeSH-major] Soft Tissue Neoplasms / pathology. Urinary Bladder Neoplasms / pathology
  • [MeSH-minor] Diagnosis, Differential. Hemangiopericytoma / pathology. Histiocytoma, Malignant Fibrous / pathology. Humans. Immunohistochemistry. Nerve Sheath Neoplasms / pathology. Neuroectodermal Tumors, Primitive / pathology. Sarcoma / pathology

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  • (PMID = 17574946.001).
  • [ISSN] 0046-8177
  • [Journal-full-title] Human pathology
  • [ISO-abbreviation] Hum. Pathol.
  • [Language] eng
  • [Publication-type] Journal Article; Review
  • [Publication-country] United States
  • [Number-of-references] 60
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71. Skubitz KM, D'Adamo DR: Sarcoma. Mayo Clin Proc; 2007 Nov;82(11):1409-32
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  • They can be grouped into 2 general categories, soft tissue sarcoma and primary bone sarcoma, which have different staging and treatment approaches.
  • This review includes a discussion of both soft tissue sarcomas (malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, synovial sarcoma, dermatofibrosarcoma protuberans, angiosarcoma, Kaposi sarcoma, gastrointestinal stromal tumor, aggressive fibromatosis or desmoid tumor, rhabdomyosarcoma, and primary alveolar soft-part sarcoma) and primary bone sarcomas (osteosarcoma, Ewing sarcoma, giant cell tumor, and chondrosarcoma).
  • The approach to a patient with a sarcoma begins with a biopsy that obtains adequate tissue for diagnosis without interfering with subsequent optimal definitive surgery.
  • [MeSH-major] Bone Neoplasms / therapy. Sarcoma / therapy. Soft Tissue Neoplasms / therapy

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  • (PMID = 17976362.001).
  • [ISSN] 0025-6196
  • [Journal-full-title] Mayo Clinic proceedings
  • [ISO-abbreviation] Mayo Clin. Proc.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Antineoplastic Agents
  • [Number-of-references] 391
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72. Mercado GE, Barr FG: Fusions involving PAX and FOX genes in the molecular pathogenesis of alveolar rhabdomyosarcoma: recent advances. Curr Mol Med; 2007 Feb;7(1):47-61
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  • [Title] Fusions involving PAX and FOX genes in the molecular pathogenesis of alveolar rhabdomyosarcoma: recent advances.
  • Rhabdomyosarcoma is the most frequent soft tissue sarcoma in the pediatric population.
  • Two main histopathologic variants have been described, embryonal (ERMS) and alveolar (ARMS), which demonstrate clinical and genetic differences.
  • This review highlights recent advances in numerous areas of biomedical investigation that are providing new insights into the biology, molecular pathology, and translational science of ARMS: the identification of downstream targets of PAX3-FKHR and collaborating events in the process of tumorigenesis and metastasis; generation of animal models based on the gene fusion and collaborating events; development of new assays for diagnosis, prognosis, and detection of minimal disseminated disease; and exploration of immune recognition of this tumor and the fusion protein.
  • [MeSH-major] Oncogene Proteins, Fusion / genetics. Paired Box Transcription Factors / genetics. Rhabdomyosarcoma, Alveolar / pathology

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  • (PMID = 17311532.001).
  • [ISSN] 1566-5240
  • [Journal-full-title] Current molecular medicine
  • [ISO-abbreviation] Curr. Mol. Med.
  • [Language] eng
  • [Grant] United States / NCI NIH HHS / CA / CA64202; United States / NCI NIH HHS / CA / CA87812; United States / NCI NIH HHS / CA / CA89461
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Netherlands
  • [Chemical-registry-number] 0 / Oncogene Proteins, Fusion; 0 / PAX3-FOXO1A fusion protein, human; 0 / Paired Box Transcription Factors
  • [Number-of-references] 101
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73. Siddaraju N, Yaranal PJ, Mishra MM, Soundararaghavan J: Fine needle aspiration cytology in recurrent amelanotic melanoma: a case report. Acta Cytol; 2007 Sep-Oct;51(5):829-32
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  • Clinically, the lesion was interpreted as recurrent soft tissue sarcoma.
  • FNAC revealed malignant cells with highly varied morphology with plasmacytoid and pleomorphic malignant cells with occasional fibrocollagenous tissue strands showing adherent neoplastic cells.
  • A cytologic diagnosis of pleomorphic malignant tumor was suggested, and the original histologic slides were reviewed; they showed a striking alveolar pattern that vaguely resembled an alveolar rhabdomyosarcoma.
  • A final diagnosis of amelanotic melanoma was made.

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  • (PMID = 17910357.001).
  • [ISSN] 0001-5547
  • [Journal-full-title] Acta cytologica
  • [ISO-abbreviation] Acta Cytol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
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74. 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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75. Gee MF, Tsuchida R, Eichler-Jonsson C, Das B, Baruchel S, Malkin D: Vascular endothelial growth factor acts in an autocrine manner in rhabdomyosarcoma cell lines and can be inhibited with all-trans-retinoic acid. Oncogene; 2005 Dec 1;24(54):8025-37
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  • [Title] Vascular endothelial growth factor acts in an autocrine manner in rhabdomyosarcoma cell lines and can be inhibited with all-trans-retinoic acid.
  • Rhabdomyosarcoma (RMS) is the most common paediatric soft-tissue sarcoma, and is dependent on autocrine signalling for its growth.
  • The alveolar subtype of RMS is often characterized by the presence of a PAX3-FKHR translocation, and when introduced into non-RMS cells, the resultant fusion protein induces expression of VEGFR1.
  • [MeSH-major] Autocrine Communication / drug effects. Rhabdomyosarcoma, Alveolar / metabolism. Rhabdomyosarcoma, Embryonal / metabolism. Tretinoin / pharmacology. Vascular Endothelial Growth Factor A / pharmacology

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  • (PMID = 16116481.001).
  • [ISSN] 0950-9232
  • [Journal-full-title] Oncogene
  • [ISO-abbreviation] Oncogene
  • [Language] eng
  • [Publication-type] Comparative Study; Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] England
  • [Chemical-registry-number] 0 / RNA, Messenger; 0 / Recombinant Proteins; 0 / Vascular Endothelial Growth Factor A; 5688UTC01R / Tretinoin; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-1; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-2; EC 2.7.11.24 / Mitogen-Activated Protein Kinase 1; EC 2.7.11.24 / Mitogen-Activated Protein Kinase 3
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76. 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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77. 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.
  • [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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78. Stiff PJ, Agovi MA, Antman KH, Blaise D, Camitta BM, Cairo MS, Childs RW, Edwards JR, Gale RP, Hale GA, Lazarus HM, Arora M: High-dose chemotherapy with blood or bone marrow transplants for rhabdomyosarcoma. Biol Blood Marrow Transplant; 2010 Apr;16(4):525-32
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  • [Title] High-dose chemotherapy with blood or bone marrow transplants for rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS), the most common soft-tissue sarcoma in children, is cured with conventional therapy in 70%.
  • However, the 5-year survival for those who relapse is about 30%, and drops to about 15% for those with unfavorable histologies (alveolar/undifferentiated subtypes).
  • Overall, 73% of subjects were <20 years; 39% had cancer bulk >5 cm, 63% had metastasis at diagnosis, 55% had unfavorable histologies, 92% had cancer responsive to chemotherapy pretransplant, and 67% were in first remission.
  • Even when performed after relapse for alveolar/undifferentiated histologies, long-term survivals were seen seemingly better than results with conventional therapies.

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  • [Copyright] Copyright (c) 2010 American Society for Blood and Marrow Transplantation. Published by Elsevier Inc. All rights reserved.
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  • (PMID = 19961947.001).
  • [ISSN] 1523-6536
  • [Journal-full-title] Biology of blood and marrow transplantation : journal of the American Society for Blood and Marrow Transplantation
  • [ISO-abbreviation] Biol. Blood Marrow Transplant.
  • [Language] ENG
  • [Grant] United States / NHLBI NIH HHS / HL / 5U01 HL 069294; United States / NCI NIH HHS / CA / U24-CA 76518; United States / NCI NIH HHS / CA / U24 CA076518; United States / NCI NIH HHS / CA / U24 CA076518-12; United States / NHLBI NIH HHS / HL / U01 HL069294; United States / NCI NIH HHS / CA / CA076518-12
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S.
  • [Publication-country] United States
  • [Other-IDs] NLM/ NIHMS162698; NLM/ PMC2838953
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79. Koren J, Matecek L, Zamecnik M: Mitotically active deep juvenile xanthogranuloma. Ann Diagn Pathol; 2010 Feb;14(1):36-40
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  • A diagnosis of deep malignant melanoma or alveolar rhabdomyosarcoma was considered.
  • In the differential diagnosis, we considered especially an atypical diffuse giant cell tumor of tendon sheaths and joints (extra-articular pigmented villonodular synovitis) and some rare types of soft tissue leiomyosarcoma, such as epitheloid leiomyosarcoma and leiomyosarcoma with prominent osteoclast-like giant cells.
  • [MeSH-minor] Adult. Biomarkers. Biopsy, Fine-Needle. Diagnosis, Differential. Female. Giant Cell Tumors / pathology. Humans. Leiomyosarcoma / pathology. Melanoma / pathology. Mitosis. Rhabdomyosarcoma, Alveolar / pathology. Skin Neoplasms / pathology


80. Allen SD, Moskovic EC, Fisher C, Thomas JM: Adult rhabdomyosarcoma: cross-sectional imaging findings including histopathologic correlation. AJR Am J Roentgenol; 2007 Aug;189(2):371-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Adult rhabdomyosarcoma: cross-sectional imaging findings including histopathologic correlation.
  • The alveolar subtype was present in 13 patients, embryonal subtype in four patients, and pleomorphic subtype in nine patients.
  • Pleomorphic tumors were very high signal on T2-weighted/STIR imaging, and both pleomorphic and alveolar subtypes were extremely heterogeneous.
  • CONCLUSION: Although adult rhabdomyosarcomas have certain imaging appearances in common with other soft-tissue sarcomas, features at presentation such as tumor heterogeneity, site, regional lymphadenopathy, and pulmonary metastasis should make the radiologist consider this important diagnosis.
  • [MeSH-major] Rhabdomyosarcoma / diagnosis
  • [MeSH-minor] Adult. Contrast Media. Diagnosis, Differential. Female. Gadolinium DTPA. Humans. Magnetic Resonance Imaging / methods. Male. Middle Aged. Tomography, X-Ray Computed

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  • (PMID = 17646463.001).
  • [ISSN] 1546-3141
  • [Journal-full-title] AJR. American journal of roentgenology
  • [ISO-abbreviation] AJR Am J Roentgenol
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Contrast Media; K2I13DR72L / Gadolinium DTPA
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81. Jones AE, Albano EA, Lovell MA, Hunger SP: Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A. Pediatr Blood Cancer; 2010 Dec 1;55(6):1213-6
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Metastatic alveolar rhabdomyosarcoma in multiple endocrine neoplasia type 2A.
  • Rhabdomyosarcoma (RMS), the most common pediatric soft tissue sarcoma, accounts for 3% of childhood malignancies.
  • We describe a previously unreported association of MEN-2A with metastatic alveolar RMS and review the literature on associated hereditary cancer predisposition syndromes and current therapeutic options.
  • The diagnosis of RMS should prompt consideration of screening for familial genetic syndromes in certain patients.
  • [MeSH-major] Multiple Endocrine Neoplasia Type 2a / pathology. Rhabdomyosarcoma, Alveolar / secondary


82. Wolf SJ, Wakelin LP, He Z, Stewart BW, Catchpoole DR: In vitro assessment of novel transcription inhibitors and topoisomerase poisons in rhabdomyosarcoma cell lines. Cancer Chemother Pharmacol; 2009 Nov;64(6):1059-69
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] In vitro assessment of novel transcription inhibitors and topoisomerase poisons in rhabdomyosarcoma cell lines.
  • PURPOSE: Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children.
  • [MeSH-major] Drug Evaluation, Preclinical. Nucleic Acid Synthesis Inhibitors / pharmacology. Rhabdomyosarcoma / drug therapy. Rhabdomyosarcoma, Alveolar / drug therapy. Rhabdomyosarcoma, Embryonal / drug therapy. Topoisomerase Inhibitors

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  • (PMID = 19277661.001).
  • [ISSN] 1432-0843
  • [Journal-full-title] Cancer chemotherapy and pharmacology
  • [ISO-abbreviation] Cancer Chemother. Pharmacol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Germany
  • [Chemical-registry-number] 0 / Acridines; 0 / Aminoacridines; 0 / Antigens, Neoplasm; 0 / Antineoplastic Agents; 0 / DNA-Binding Proteins; 0 / Enzyme Inhibitors; 0 / Nucleic Acid Synthesis Inhibitors; 0 / Phenazines; 0 / Topoisomerase Inhibitors; EC 5.99.1.2 / DNA Topoisomerases, Type I; EC 5.99.1.3 / DNA Topoisomerases, Type II; EC 5.99.1.3 / DNA topoisomerase II alpha; EC 5.99.1.3 / DNA topoisomerase II beta
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83. Hulse N, Raja S, Kumar A, Paul AS: Rhabdomyosarcoma of the extremities in adults. Acta Orthop Belg; 2006 Apr;72(2):199-203
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  • [Title] Rhabdomyosarcoma of the extremities in adults.
  • Eight patients with an average age of 32.6 years (range: 21 to 75) who were treated for rhabdomyosarcomas on the extremities between 1991 and 2002 in a regional centre for the treatment of soft tissue sarcomas were studied retrospectively.
  • Histologically there were four alveolar, two pleomorphic, one embryonal and one anaplastic subtype.
  • Extremity rhabdomyosarcoma is a highly malignant tumour and our results are poor compared to the reported results in children.
  • [MeSH-major] Extremities. Rhabdomyosarcoma / therapy. Soft Tissue Neoplasms / therapy

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  • (PMID = 16768266.001).
  • [ISSN] 0001-6462
  • [Journal-full-title] Acta orthopaedica Belgica
  • [ISO-abbreviation] Acta Orthop Belg
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] Belgium
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84. Taulli R, Bersani F, Foglizzo V, Linari A, Vigna E, Ladanyi M, Tuschl T, Ponzetto C: The muscle-specific microRNA miR-206 blocks human rhabdomyosarcoma growth in xenotransplanted mice by promoting myogenic differentiation. J Clin Invest; 2009 Aug;119(8):2366-78
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  • [Title] The muscle-specific microRNA miR-206 blocks human rhabdomyosarcoma growth in xenotransplanted mice by promoting myogenic differentiation.
  • In this work, we examined the role of the muscle-specific miRNAs miR-1 and miR-206 in human rhabdomyosarcoma (RMS), a soft tissue sarcoma thought to arise from skeletal muscle progenitors.
  • We have shown that miR-1 was barely detectable in primary RMS of both the embryonal and alveolar subtypes and that both miR-1 and miR-206 failed to be induced in RMS cell lines upon serum deprivation.
  • We propose that tissue-specific miRNAs such as miR-1 and miR-206, given their ability to modulate hundreds of transcripts and to act as nontoxic differentiating agents, may override the genomic heterogeneity of solid tumors and ultimately hold greater therapeutic potential than single gene-directed drugs.
  • [MeSH-major] MicroRNAs / physiology. Muscle Development / physiology. Rhabdomyosarcoma / prevention & control


85. Püsküllüoglu M, Lukasiewicz E, Miekus K, Jarocha D, Majka M: Differential expression of Snail1 transcription factor and Snail1-related genes in alveolar and embryonal rhabdomyosarcoma subtypes. Folia Histochem Cytobiol; 2010 Dec;48(4):671-7
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] Differential expression of Snail1 transcription factor and Snail1-related genes in alveolar and embryonal rhabdomyosarcoma subtypes.
  • Rhabdomyosarcoma (RMS) represents the most common sarcoma of soft tissue among children.
  • Two main RMS subtypes are alveolar (ARMS) and embryonal (ERMS).
  • The major goal of this study was to find differentially expressed genes between RMS subtypes that could explain higher metastatic potential in ARMS and would be useful for the differential diagnosis.
  • Using RQ-PCR analysis we compared expression of Snail1 and Snail-related genes among 7 ARMS and 8 ERMS patients' samples obtained from the primary tumors and among 2 alveolar and 2 embryonal cell lines.
  • Our results show that Snail1 is highly expressed both in ARMS patients' samples and the alveolar cell lines.

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  • (PMID = 21478114.001).
  • [ISSN] 1897-5631
  • [Journal-full-title] Folia histochemica et cytobiologica
  • [ISO-abbreviation] Folia Histochem. Cytobiol.
  • [Language] ENG
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] Poland
  • [Chemical-registry-number] 0 / Transcription Factors; 0 / beta Catenin; 0 / snail family transcription factors; EC 3.4.24.24 / Matrix Metalloproteinase 2; EC 3.4.24.35 / Matrix Metalloproteinase 9
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86. Ventura-Holman T, Hahn H, Subauste JS, Maher JF: The Fem1a gene is downregulated in Rhabdomyosarcoma. Tumour Biol; 2005 Nov-Dec;26(6):294-9
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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [Title] The Fem1a gene is downregulated in Rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue neoplasm of children, and those metastatic at presentation have a poor prognosis.
  • We find that the human homolog, FEM1A, is downregulated in all of 8 different human RMS cell lines, including those derived from embryonal and alveolar RMS.
  • [MeSH-major] Cell Cycle Proteins / genetics. Down-Regulation / genetics. Gene Expression Regulation, Neoplastic / genetics. Rhabdomyosarcoma / genetics

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  • [Copyright] Copyright 2005 S. Karger AG, Basel.
  • (PMID = 16254458.001).
  • [ISSN] 1010-4283
  • [Journal-full-title] Tumour biology : the journal of the International Society for Oncodevelopmental Biology and Medicine
  • [ISO-abbreviation] Tumour Biol.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, U.S. Gov't, Non-P.H.S.
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Cell Cycle Proteins; 0 / FEM1A protein, human; 0 / Fem1a protein, mouse
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87. 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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  • [Source] The source of this record is MEDLINE®, a database of the U.S. National Library of Medicine.
  • [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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88. Brown BJ, Oluwasola AO: Childhood rhabdomyosarcoma in Ibadan, Nigeria: 1984-2003. Ann Trop Paediatr; 2006 Dec;26(4):349-55
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  • [Title] Childhood rhabdomyosarcoma in Ibadan, Nigeria: 1984-2003.
  • BACKGROUND: Rhabdomyosarcoma is the most common soft tissue sarcoma in children under 15 years of age.
  • AIM: To describe the pattern of childhood rhabdomyosarcoma in Ibadan with respect to demography, morphology and tumour site.
  • All histologically confirmed cases of rhabdomyosarcoma in children under 15 years of age seen at the University College Hospital Ibadan between 1984 and 2003 were included.
  • Other subtypes were alveolar (13.2%), pleomorphic (4.4%) and rhabdomyosarcoma 'not otherwise specified' (20.9%).
  • The majority (50.6%) of tumours were in the head and neck region and the common primary sites were soft tissue of the head, face (24.2%) and orbit (14.3%).
  • Other sites included soft tissue of the pelvis (11.0%), genito-urinary tract (9.9%) and abdomen (9.9%).
  • CONCLUSION: The pattern of rhabdomyosarcoma in Nigeria is similar to that in the United States and Europe, except for the rarity of parameningeal sites and extremities.
  • There is a need for larger descriptive studies on childhood rhabdomyosarcoma in Africa.
  • [MeSH-major] Rhabdomyosarcoma / epidemiology. Soft Tissue Neoplasms / epidemiology
  • [MeSH-minor] Adolescent. Age Distribution. Child. Child, Preschool. Female. Head and Neck Neoplasms / epidemiology. Humans. Infant. Infant, Newborn. Male. Nigeria / epidemiology. Registries. Retrospective Studies. Rhabdomyosarcoma, Alveolar / epidemiology. Rhabdomyosarcoma, Alveolar / pathology. Rhabdomyosarcoma, Embryonal / epidemiology. Rhabdomyosarcoma, Embryonal / pathology

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  • (PMID = 17132301.001).
  • [ISSN] 0272-4936
  • [Journal-full-title] Annals of tropical paediatrics
  • [ISO-abbreviation] Ann Trop Paediatr
  • [Language] eng
  • [Publication-type] Journal Article
  • [Publication-country] England
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89. 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%.
  • The value of FDG in patients with soft tissue sarcomas has been demonstrated in several series, but none specifically in ARS.
  • All the 3 patients we present had focally increased tracer uptake in nodal stations on a pretherapy PET performed at diagnosis.
  • Tissue confirmation available in 2 patients was negative in 1 patient and positive for metastatic nodal spread in the other.
  • [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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90. Cecchetto G, Alaggio R, Dall'Igna P, Bisogno G, Ferrari A, Gigante C, Casanova M, Sotti G, Zanetti I, Carli M: Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies. Cancer; 2005 Nov 1;104(9):2006-12
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  • [Title] Localized unresectable non-rhabdo soft tissue sarcomas of the extremities in pediatric age: results from the Italian studies.
  • BACKGROUND: Treatment of initially unresectable nonrhabdo soft tissue sarcomas (NRSTS) in pediatric age is debated, due to their different chemosensitivity.
  • Clinical TNM and surgical Intergroup Rhabdomyosarcoma Staging systems were adopted.
  • Nonchemosensitive (CTns) sarcomas, 31: fibrosarcoma, 11; malignant peripheral nerve sheet tumors, 10; liposarcoma, 2; hemangiopericitoma adult type, 2; epithelioid sarcoma, 2; and alveolar soft part sarcoma, leiomyosarcoma, clear cell sarcoma, and sarcoma NOS, each 1.
  • [MeSH-major] Extremities. Sarcoma / diagnosis

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  • [Copyright] (c) 2005 American Cancer Society.
  • (PMID = 16161038.001).
  • [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
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91. Linardic CM: PAX3-FOXO1 fusion gene in rhabdomyosarcoma. Cancer Lett; 2008 Oct 18;270(1):10-8
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  • [Title] PAX3-FOXO1 fusion gene in rhabdomyosarcoma.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood and adolescence.
  • The predominant histologic variants of this disease are termed embryonal (eRMS) and alveolar (aRMS), based on their appearance under light microscopy.

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  • (PMID = 18457914.001).
  • [ISSN] 1872-7980
  • [Journal-full-title] Cancer letters
  • [ISO-abbreviation] Cancer Lett.
  • [Language] ENG
  • [Grant] United States / NICHD NIH HHS / HD / HD043494-05; United States / NICHD NIH HHS / HD / K12 HD043494; United States / NICHD NIH HHS / HD / 5K12-HD043494; United States / NICHD NIH HHS / HD / K12 HD043494-05
  • [Publication-type] Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
  • [Publication-country] Ireland
  • [Chemical-registry-number] 0 / CXCL12 protein, human; 0 / Chemokine CXCL12; 0 / FOXO1 protein, human; 0 / Forkhead Transcription Factors; 0 / Oncogene Proteins, Fusion; 0 / PAX3-FOXO1A fusion protein, human; 0 / Paired Box Transcription Factors; EC 2.7.10.1 / FLT1 protein, human; EC 2.7.10.1 / Vascular Endothelial Growth Factor Receptor-1
  • [Number-of-references] 59
  • [Other-IDs] NLM/ NIHMS72264; NLM/ PMC2575376
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92. 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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93. Paner GP, McKenney JK, Epstein JI, Amin MB: Rhabdomyosarcoma of the urinary bladder in adults: predilection for alveolar morphology with anaplasia and significant morphologic overlap with small cell carcinoma. Am J Surg Pathol; 2008 Jul;32(7):1022-8
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  • [Title] Rhabdomyosarcoma of the urinary bladder in adults: predilection for alveolar morphology with anaplasia and significant morphologic overlap with small cell carcinoma.
  • Rhabdomyosarcoma (RMS) represents the most common malignant soft tissue tumor in children and adolescents with the urinary bladder representing a frequent site.
  • We report the clinicopathologic features of 5 bladder neoplasms with rhabdomyosarcomatous differentiation in adults and emphasize the differential diagnosis in the adult setting.
  • Three cases were of the alveolar subtype (1 admixed with embryonal histology) and 2 were RMS, not further classified.
  • In conclusion, (1) RMS of the urinary bladder in adults more commonly presents as a primitive round blue cell neoplasm that has significant morphologic and immunohistochemical overlap with small cell carcinoma of the bladder. (2) Although RMS in children generally have a botryoid embryonal histology with favorable outcome, bladder RMS in adults frequently demonstrates alveolar or unclassified histology, commonly with anaplasia, and have a uniformly aggressive clinical course.
  • [MeSH-major] Carcinoma, Small Cell / pathology. Rhabdomyosarcoma, Alveolar / pathology. Urinary Bladder Neoplasms / pathology
  • [MeSH-minor] Adult. Aged. Aged, 80 and over. Anaplasia. Biomarkers, Tumor / analysis. Cell Nucleus / pathology. Combined Modality Therapy. Desmin / analysis. Diagnosis, Differential. Fatal Outcome. Female. Humans. Male. Middle Aged. MyoD Protein / analysis. Myogenin / analysis. Synaptophysin / analysis

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  • (PMID = 18469707.001).
  • [ISSN] 1532-0979
  • [Journal-full-title] The American journal of surgical pathology
  • [ISO-abbreviation] Am. J. Surg. Pathol.
  • [Language] eng
  • [Publication-type] Case Reports; Journal Article
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Biomarkers, Tumor; 0 / Desmin; 0 / MyoD Protein; 0 / MyoD1 myogenic differentiation protein; 0 / Myogenin; 0 / Synaptophysin
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94. Athanasiou A, Vanel D, El Mesbahi O, Theodore C, Fizazi K: Non-germ cell tumours arising in germ cell tumours (teratoma with malignant transformation) in men: CT and MR findings. Eur J Radiol; 2009 Feb;69(2):230-5
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  • Sarcoma was identified in 10 out of 14 patients, with rhabdomyosarcoma ranking first (n=4), followed by osteosarcoma (n=2), fusiform cell sarcoma (n=1), undifferentiated sarcoma (n=1), neurosarcoma (n=1) and myxoid sarcoma (n=1).
  • The CT and MR imaging findings before treatment and after relapse were evaluated with emphasis on imaging features that could possibly imply the presence of malignant transformation (heterogeneously enhancing soft-tissue masses, ossified masses with calcified lymph nodes, diffuse epiploic thickening associated with ascites and peritoneal nodules, pulmonary alveolar infiltration with septal thickening).
  • [MeSH-major] Cell Transformation, Neoplastic / pathology. Magnetic Resonance Imaging. Teratoma / diagnosis. Tomography, X-Ray Computed


95. Ganti R, Skapek SX, Zhang J, Fuller CE, Wu J, Billups CA, Breitfeld PP, Dalton JD, Meyer WH, Khoury JD: Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcoma. Mod Pathol; 2006 Sep;19(9):1213-20
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  • [Title] Expression and genomic status of EGFR and ErbB-2 in alveolar and embryonal rhabdomyosarcoma.
  • EGFR and ErbB-2 expression has been observed in rhabdomyosarcoma cell lines but not analyzed systematically in rhabdomyosarcoma tumors.
  • Tissue microarray sections representing 66 rhabdomyosarcoma tumors (34 embryonal rhabdomyosarcoma, 32 alveolar rhabdomyosarcoma) were surveyed by immunohistochemistry using antibodies specific for EGFR and ErbB-2.
  • Expression of ErbB-2 was identified in 22/66 (33%) cases and tended to be more frequent in the alveolar subtype (13/32, 41%, vs 9/34, 26%, P=0.30).
  • Coexpression of EGFR and ErbB-2 was identified in eight tumors, of which six were embryonal rhabdomyosarcoma.
  • Furthermore, analysis of 11 additional rhabdomyosarcoma tumors (six alveolar; five embryonal) revealed no evidence of mutations in EGFR exons 18, 19, 20, and 21.
  • In summary, expression of EGFR and/or ErbB-2 is detected in a sizeable subset of rhabdomyosarcoma tumors without evidence of EGFR or ErbB-2 amplification or mutations in the EGFR tyrosine kinase domain.
  • [MeSH-major] Receptor, Epidermal Growth Factor / metabolism. Receptor, ErbB-2 / metabolism. Rhabdomyosarcoma, Alveolar / metabolism. Rhabdomyosarcoma, Embryonal / metabolism. Soft Tissue Neoplasms / metabolism
  • [MeSH-minor] Adolescent. Adult. Child. Child, Preschool. DNA Mutational Analysis. DNA, Neoplasm / analysis. Female. Gene Dosage. Humans. Immunoenzyme Techniques. In Situ Hybridization, Fluorescence. Infant. Male. Tissue Array Analysis


96. Roumes H, Leloup L, Dargelos E, Brustis JJ, Daury L, Cottin P: Calpains: markers of tumor aggressiveness? Exp Cell Res; 2010 May 15;316(9):1587-99
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  • Rhabdomyosarcoma (RMS) are soft-tissue sarcoma commonly encountered in childhood.
  • [MeSH-major] Calcium-Binding Proteins / metabolism. Calpain / metabolism. Myoblasts / metabolism. Rhabdomyosarcoma, Alveolar / metabolism. Rhabdomyosarcoma, Alveolar / pathology

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  • [Copyright] Copyright 2010 Elsevier Inc. All rights reserved.
  • (PMID = 20193680.001).
  • [ISSN] 1090-2422
  • [Journal-full-title] Experimental cell research
  • [ISO-abbreviation] Exp. Cell Res.
  • [Language] eng
  • [Publication-type] Journal Article; Research Support, Non-U.S. Gov't
  • [Publication-country] United States
  • [Chemical-registry-number] 0 / Actins; 0 / Calcium-Binding Proteins; 0 / RNA, Messenger; 79079-11-1 / calpastatin; EC 3.4.22.- / Calpain; EC 3.4.22.- / m-calpain; EC 3.4.22.- / mu-calpain
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97. Jelić-Puskarić B, Rajković-Molek K, Raić L, Batinić D, Konja J, Kardum-Skelin I: Rhabdomyosarcoma with bone marrow infiltration mimicking hematologic neoplasia. Coll Antropol; 2010 Jun;34(2):635-9
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  • [Title] Rhabdomyosarcoma with bone marrow infiltration mimicking hematologic neoplasia.
  • Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma