rhabdomyosarcoma

Summary

Summary: A malignant solid tumor arising from mesenchymal tissues which normally differentiate to form striated muscle. It can occur in a wide variety of sites. It is divided into four distinct types: pleomorphic, predominantly in male adults; alveolar (RHABDOMYOSARCOMA, ALVEOLAR), mainly in adolescents and young adults; embryonal (RHABDOMYOSARCOMA, EMBRYONAL), predominantly in infants and children; and botryoidal, also in young children. It is one of the most frequently occurring soft tissue sarcomas and the most common in children under 15. (From Dorland, 27th ed; Holland et al., Cancer Medicine, 3d ed, p2186; DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, pp1647-9)

Top Publications

  1. Pressey J, Anderson J, Crossman D, Lynch J, Barr F. Hedgehog pathway activity in pediatric embryonal rhabdomyosarcoma and undifferentiated sarcoma: a report from the Children's Oncology Group. Pediatr Blood Cancer. 2011;57:930-8 pubmed publisher
    ..The spectrum of tumors found in Gorlin Syndrome includes basal cell carcinoma, medulloblastoma, and rarely, rhabdomyosarcoma (RMS)...
  2. Chisholm J, Marandet J, Rey A, Scopinaro M, de Toledo J, Merks J, et al. Prognostic factors after relapse in nonmetastatic rhabdomyosarcoma: a nomogram to better define patients who can be salvaged with further therapy. J Clin Oncol. 2011;29:1319-25 pubmed publisher
    Previous studies suggest poor outcome in children with relapsed rhabdomyosarcoma (RMS). A better understanding is needed of which patients can be salvaged after first relapse...
  3. Rosenberg A, Skapek S, Hawkins D. The inconvenience of convenience cohorts: rhabdomyosarcoma and the PAX-FOXO1 biomarker. Cancer Epidemiol Biomarkers Prev. 2012;21:1012-8 pubmed publisher
    ..the prognostic significance of chromosomal translocations between the PAX3 or PAX7 and the FOXO1 genes in rhabdomyosarcoma, the most common pediatric sarcoma...
  4. Gupta A, Anderson J, Pappo A, Spunt S, Dasgupta R, Indelicato D, et al. Patterns of chemotherapy-induced toxicities in younger children and adolescents with rhabdomyosarcoma: a report from the Children's Oncology Group Soft Tissue Sarcoma Committee. Cancer. 2012;118:1130-7 pubmed publisher
    Patients aged >10 years with rhabdomyosarcoma have an inferior outcome compared with patients ages 1 to 9 years, which may be explained by toxicities (adverse events [AEs]) that result in chemotherapy dose reductions...
  5. Paulson V, Chandler G, Rakheja D, Galindo R, Wilson K, Amatruda J, et al. High-resolution array CGH identifies common mechanisms that drive embryonal rhabdomyosarcoma pathogenesis. Genes Chromosomes Cancer. 2011;50:397-408 pubmed publisher
    Pediatric rhabdomyosarcoma occurs as two biologically distinct histological variants, embryonal (ERMS) and alveolar (ARMS)...
  6. Lee J, Lim S, Rha S, Roh J, Cho Y, Shin K, et al. Prognostic implications of anaplastic lymphoma kinase gene aberrations in rhabdomyosarcoma; an immunohistochemical and fluorescence in situ hybridisation study. J Clin Pathol. 2014;67:33-9 pubmed publisher
    ..investigated the diagnostic and prognostic usefulness of anaplastic lymphoma kinase (ALK) expression in Asian rhabdomyosarcoma (RMS) patients. A total of 38 RMS tissue samples were collected over a 14-year period (1998-2012)...
  7. Kojima Y, Hashimoto K, Ando M, Yonemori K, Hirakawa A, Kodaira M, et al. Clinical outcomes of adult and childhood rhabdomyosarcoma treated with vincristine, d-actinomycin, and cyclophosphamide chemotherapy. J Cancer Res Clin Oncol. 2012;138:1249-57 pubmed publisher
    Outcomes in adult patients with rhabdomyosarcoma are poor, with a 5-year survival rate of approximately 30 %...
  8. Masia A, Almazán Moga A, Velasco P, Reventos J, Toran N, Sanchez de Toledo J, et al. Notch-mediated induction of N-cadherin and ?9-integrin confers higher invasive phenotype on rhabdomyosarcoma cells. Br J Cancer. 2012;107:1374-83 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is the commonest type of soft-tissue sarcoma in children. Patients with metastatic RMS continue to have very poor prognosis...
  9. Hafez M, Hegazy M, Abd Elwahab K, Arafa M, Abdou I, Refky B. Metastatic rhabdomyosarcoma of the thyroid gland, a case report. Head Neck Oncol. 2012;4:27 pubmed
    ..We report a case of 22-years-old woman with right leg rhabdomyosarcoma metastatic to the thyroid gland.

More Information

Publications81

  1. Yu Y, Zeng P, Xiong J, Liu Z, Berger S, Merlino G. Epigenetic drugs can stimulate metastasis through enhanced expression of the pro-metastatic Ezrin gene. PLoS ONE. 2010;5:e12710 pubmed publisher
    ..Here, we report that highly metastatic rhabdomyosarcoma (RMS) cells with high levels of Ezrin have elevated acetyl-H3-K9 and tri-methyl-H3-K4 as well as reduced DNA ..
  2. Roma J, Masià A, Reventos J, Sánchez de Toledo J, Gallego S. Notch pathway inhibition significantly reduces rhabdomyosarcoma invasiveness and mobility in vitro. Clin Cancer Res. 2011;17:505-13 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is the most common type of soft tissue sarcoma in children and can be divided into two main subtypes: embryonal and alveolar RMS...
  3. Walters Z, Villarejo Balcells B, Olmos D, Buist T, Missiaglia E, Allen R, et al. JARID2 is a direct target of the PAX3-FOXO1 fusion protein and inhibits myogenic differentiation of rhabdomyosarcoma cells. Oncogene. 2014;33:1148-57 pubmed publisher
    ..JARID2 and other components of PRC2 may represent novel therapeutic targets for treating RMS patients. ..
  4. Bennani Baiti I, Machado I, Llombart Bosch A, Kovar H. Lysine-specific demethylase 1 (LSD1/KDM1A/AOF2/BHC110) is expressed and is an epigenetic drug target in chondrosarcoma, Ewing's sarcoma, osteosarcoma, and rhabdomyosarcoma. Hum Pathol. 2012;43:1300-7 pubmed publisher
    ..microarray-coupled immunohistochemical analyses and confirmed lysine-specific demethylase 1 overexpression in rhabdomyosarcoma and synovial sarcoma...
  5. Pressey J, Haas M, Pressey C, Kelly V, Parker J, Gillespie G, et al. CD133 marks a myogenically primitive subpopulation in rhabdomyosarcoma cell lines that are relatively chemoresistant but sensitive to mutant HSV. Pediatr Blood Cancer. 2013;60:45-52 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is characterized by features of skeletal muscle and is comprised of two major histological subtypes, embryonal (E-RMS), and alveolar (A-RMS)...
  6. Le X, Pugach E, Hettmer S, Storer N, Liu J, Wills A, et al. A novel chemical screening strategy in zebrafish identifies common pathways in embryogenesis and rhabdomyosarcoma development. Development. 2013;140:2354-64 pubmed publisher
    ..A KRAS(G12D)-induced zebrafish embryonal rhabdomyosarcoma was then used to assess the therapeutic effects of the small molecules...
  7. Meazza C, Casanova M, Zaffignani E, Clerici C, Favini F, Vasquez R, et al. An adolescent with rhabdomyosarcoma during pregnancy. Tumori. 2008;94:431-3 pubmed
    We report the case of a 15-year-old girl with a large gluteal and perineal rhabdomyosarcoma diagnosed at 24 weeks of pregnancy, whose management posed a great clinical dilemma for us...
  8. Rezvani G, Lui J, Barnes K, Baron J. A set of imprinted genes required for normal body growth also promotes growth of rhabdomyosarcoma cells. Pediatr Res. 2012;71:32-8 pubmed publisher
    ..showed elevated expression of MEST, PLAGL1, PEG3, DLK1, and IGF2 in various embryonal cancers, especially rhabdomyosarcoma, as compared to nonembryonal cancers and normal tissues...
  9. Zhu B, Zhang M, Byrum S, Tackett A, Davie J. TBX2 blocks myogenesis and promotes proliferation in rhabdomyosarcoma cells. Int J Cancer. 2014;135:785-97 pubmed publisher
    ..These results show that deregulated TBX2 serves as an oncogene in RMS, suggesting that TBX2 may serve as a new diagnostic marker or therapeutic target for RMS tumors. ..
  10. Rota R, Ciarapica R, Giordano A, Miele L, Locatelli F. MicroRNAs in rhabdomyosarcoma: pathogenetic implications and translational potentiality. Mol Cancer. 2011;10:120 pubmed publisher
    ..In recent years, microRNAs have emerged as prominent players in cancer as well. Rhabdomyosarcoma is a pediatric skeletal muscle-derived soft-tissue sarcoma that originates from myogenic precursors arrested ..
  11. Sher R, Cox G, Mills K, Sundberg J. Rhabdomyosarcomas in aging A/J mice. PLoS ONE. 2011;6:e23498 pubmed publisher
    ..These studies suggest that A strains of mice can be useful models for dissecting the molecular genetic basis for development, progression, and ultimately for testing novel anticancer therapeutic agents dealing with rhabdomyosarcoma.
  12. Mosquera J, Sboner A, Zhang L, Kitabayashi N, Chen C, Sung Y, et al. Recurrent NCOA2 gene rearrangements in congenital/infantile spindle cell rhabdomyosarcoma. Genes Chromosomes Cancer. 2013;52:538-50 pubmed publisher
    Spindle cell rhabdomyosarcoma (RMS) is a rare form of RMS with different clinical characteristics between children and adult patients...
  13. Sun M, Li J, Guo L, Xiao H, Dong L, Wang F, et al. TGF-?1 suppression of microRNA-450b-5p expression: a novel mechanism for blocking myogenic differentiation of rhabdomyosarcoma. Oncogene. 2014;33:2075-86 pubmed publisher
    Transforming growth factor beta 1 (TGF-?1) is the most potent inhibitor of myogenic differentiation (MyoD) of rhabdomyosarcoma (RMS); however, the underlying mechanisms of this inhibition remain unclear...
  14. Renshaw J, Taylor K, Bishop R, Valenti M, de Haven Brandon A, Gowan S, et al. Dual blockade of the PI3K/AKT/mTOR (AZD8055) and RAS/MEK/ERK (AZD6244) pathways synergistically inhibits rhabdomyosarcoma cell growth in vitro and in vivo. Clin Cancer Res. 2013;19:5940-51 pubmed publisher
    ..To provide rationale for using phosphoinositide 3-kinase (PI3K) and/or mitogen-activated protein kinase (MAPK) pathway inhibitors to treat rhabdomyosarcomas, a major cause of pediatric and adolescent cancer deaths...
  15. Ricard F, Cimarelli S, Deshayes E, Mognetti T, Thiesse P, Giammarile F. Additional Benefit of F-18 FDG PET/CT in the staging and follow-up of pediatric rhabdomyosarcoma. Clin Nucl Med. 2011;36:672-7 pubmed publisher
    The therapeutic management of rhabdomyosarcoma (RMS) is strongly dependent on initial staging...
  16. Taulli R, Foglizzo V, Morena D, Coda D, Ala U, Bersani F, et al. Failure to downregulate the BAF53a subunit of the SWI/SNF chromatin remodeling complex contributes to the differentiation block in rhabdomyosarcoma. Oncogene. 2014;33:2354-62 pubmed publisher
    b>Rhabdomyosarcoma (RMS), the most common soft tissue sarcoma in children and young adults, is characterized by a partially differentiated myogenic phenotype...
  17. Saab R, Spunt S, Skapek S. Myogenesis and rhabdomyosarcoma the Jekyll and Hyde of skeletal muscle. Curr Top Dev Biol. 2011;94:197-234 pubmed publisher
    b>Rhabdomyosarcoma, a neoplasm composed of skeletal myoblast-like cells, represents the most common soft tissue sarcoma in children...
  18. Kunigou O, Nagao H, Kawabata N, Ishidou Y, Nagano S, Maeda S, et al. Role of GOLPH3 and GOLPH3L in the proliferation of human rhabdomyosarcoma. Oncol Rep. 2011;26:1337-42 pubmed publisher
    ..However, the mechanism through which GOLPH3 is involved in the pathogenesis of rhabdomyosarcoma remains unidentified...
  19. Yamanaka H, Oue T, Uehara S, Fukuzawa M. Hedgehog signal inhibitor forskolin suppresses cell proliferation and tumor growth of human rhabdomyosarcoma xenograft. J Pediatr Surg. 2011;46:320-5 pubmed publisher
    ..In this study, we examined the effect of the Hh signal inhibitor forskolin on the growth of rhabdomyosarcoma (RMS) in vivo and in vitro and thereby elucidated the possibility of considering Hh signaling pathway as a ..
  20. Rossi S, Stoppani E, Puri P, Fanzani A. Differentiation of human rhabdomyosarcoma RD cells is regulated by reciprocal, functional interactions between myostatin, p38 and extracellular regulated kinase signalling pathways. Eur J Cancer. 2011;47:1095-105 pubmed publisher
    b>Rhabdomyosarcoma (RMS) includes heterogeneous tumours of mesenchymal derivation which are genetically committed to the myogenic lineage, but fail to complete terminal differentiation...
  21. Novak J, Vinklárek J, Bienertova Vasku J, Slaby O. MicroRNAs involved in skeletal muscle development and their roles in rhabdomyosarcoma pathogenesis. Pediatr Blood Cancer. 2013;60:1739-46 pubmed publisher
    ..b>Rhabdomyosarcoma (RMS) represents the most common soft tissue tumor in the pediatric population...
  22. Marchesi I, Fiorentino F, Rizzolio F, Giordano A, Bagella L. The ablation of EZH2 uncovers its crucial role in rhabdomyosarcoma formation. Cell Cycle. 2012;11:3828-36 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is a pediatric tumor that arises from muscle precursor cells...
  23. Fu W, Asp P, Canter B, Dynlacht B. Primary cilia control hedgehog signaling during muscle differentiation and are deregulated in rhabdomyosarcoma. Proc Natl Acad Sci U S A. 2014;111:9151-6 pubmed publisher
    ..However, a role for cilia in normal muscle development and rhabdomyosarcoma (RMS) has not been explored...
  24. Lambrou G, Zaravinos A, Adamaki M, Spandidos D, Tzortzatou Stathopoulou F, Vlachopoulos S. Pathway simulations in common oncogenic drivers of leukemic and rhabdomyosarcoma cells: a systems biology approach. Int J Oncol. 2012;40:1365-90 pubmed publisher
    ..gene expression is concerned, between the T-cell acute lymphoblastic leukemia (CCRF-CEM) and the rhabdomyosarcoma (TE-671) cell lines...
  25. Jacob A, O BRIEN D, Singh R, Comiskey D, Littleton R, Mohammad F, et al. Stress-induced isoforms of MDM2 and MDM4 correlate with high-grade disease and an altered splicing network in pediatric rhabdomyosarcoma. Neoplasia. 2013;15:1049-63 pubmed
    Pediatric rhabdomyosarcoma (RMS) is a morphologically and genetically heterogeneous malignancy commonly classified into three histologic subtypes, namely, alveolar, embryonal, and anaplastic...
  26. van Gaal J, Flucke U, Roeffen M, de Bont E, Sleijfer S, Mavinkurve Groothuis A, et al. Anaplastic lymphoma kinase aberrations in rhabdomyosarcoma: clinical and prognostic implications. J Clin Oncol. 2012;30:308-15 pubmed publisher
    The aim of this study is to investigate anaplastic lymphoma kinase (ALK) protein expression and underlying genetic aberrations in rhabdomyosarcoma (RMS), with special attention to clinical and prognostic implications.
  27. Post S, Lozano G. You can win by losing: p53 mutations in rhabdomyosarcomas. J Pathol. 2010;222:124-8 pubmed publisher
    ..and colleagues have generated elegant tissue-specific Cre/loxP-dependent mouse models that mimic pleomorphic rhabdomyosarcoma development in humans...
  28. Geoerger B, Kieran M, Grupp S, Perek D, Clancy J, Krygowski M, et al. Phase II trial of temsirolimus in children with high-grade glioma, neuroblastoma and rhabdomyosarcoma. Eur J Cancer. 2012;48:253-62 pubmed publisher
    A phase II study of temsirolimus was conducted in children and adolescents with high-grade glioma, neuroblastoma or rhabdomyosarcoma.
  29. Kawabata N, Ijiri K, Ishidou Y, Yamamoto T, Nagao H, Nagano S, et al. Pharmacological inhibition of the Hedgehog pathway prevents human rhabdomyosarcoma cell growth. Int J Oncol. 2011;39:899-906 pubmed publisher
    ..Recent studies have shown that mutation of the PTCH1 gene involved in the Hedgehog pathway affects rhabdomyosarcoma development...
  30. Turner J, Richmon J. Head and neck rhabdomyosarcoma: a critical analysis of population-based incidence and survival data. Otolaryngol Head Neck Surg. 2011;145:967-73 pubmed publisher
    To evaluate trends in incidence, survival, and treatment of rhabdomyosarcoma (RMS) of the head and neck.
  31. Hou J, Dong J, Sun L, Geng L, Wang J, Zheng J, et al. Inhibition of phosphorylated c-Met in rhabdomyosarcoma cell lines by a small molecule inhibitor SU11274. J Transl Med. 2011;9:64 pubmed publisher
    ..In this study, we investigated the role of c-Met in rhabdomyosarcoma (RMS) using its small molecule inhibitor SU11274, which has been hypothesized to be a potential therapeutic ..
  32. Lin X, Wang Y, Yu J, Liu Y, Wang L, Li Q, et al. Sclerosing rhabdomyosarcoma presenting in the masseter muscle: a case report. Diagn Pathol. 2013;8:18 pubmed publisher
    Sclerosing rhabdomyosarcoma (SRMS) is exceedingly rare, and may cause a great diagnostic confusion. Histologically, it is characterized by abundant extracellular hyalinized matrix mimicking primitive chondroid or osteoid tissue...
  33. Kojima Y, Hashimoto K, Ando M, Yonemori K, Yamamoto H, Kodaira M, et al. Comparison of dose intensity of vincristine, d-actinomycin, and cyclophosphamide chemotherapy for child and adult rhabdomyosarcoma: a retrospective analysis. Cancer Chemother Pharmacol. 2012;70:391-7 pubmed publisher
    The prognosis of adult rhabdomyosarcoma (RMS) has been considered dismal...
  34. Araki M, Maeda M, Motojima K. Hydrophobic statins induce autophagy and cell death in human rhabdomyosarcoma cells by depleting geranylgeranyl diphosphate. Eur J Pharmacol. 2012;674:95-103 pubmed publisher
    ..The results of our earlier work suggested that hydrophobic statins induce autophagy in cultured human rhabdomyosarcoma A204 cells...
  35. Tsioli P, Patsouris E, Giaginis C, Theocharis S. DNA repair systems in rhabdomyosarcoma. Histol Histopathol. 2013;28:971-84 pubmed publisher
    b>Rhabdomyosarcoma (RMS) represents the most common soft tissue sarcoma in children and adolescent population...
  36. Melguizo C, Prados J, Rama A, Ortiz R, Alvarez P, Fernández J, et al. Multidrug resistance and rhabdomyosarcoma (Review). Oncol Rep. 2011;26:755-61 pubmed publisher
    Classical cytotoxic treatment of rhabdomyosarcoma (RMS) is often accompanied by significant morbidity and poor response...
  37. Rodeberg D, Stoner J, Garcia Henriquez N, Randall R, Spunt S, ARNDT C, et al. Tumor volume and patient weight as predictors of outcome in children with intermediate risk rhabdomyosarcoma: a report from the Children's Oncology Group. Cancer. 2011;117:2541-50 pubmed publisher
    ..volume and patient weight versus traditional factors of tumor size (greatest dimension) and patient age and to determine which parameters best discriminated outcome among pediatric patients with intermediate-risk rhabdomyosarcoma (RMS).
  38. Preuss E, Hugle M, Reimann R, Schlecht M, Fulda S. Pan-mammalian target of rapamycin (mTOR) inhibitor AZD8055 primes rhabdomyosarcoma cells for ABT-737-induced apoptosis by down-regulating Mcl-1 protein. J Biol Chem. 2013;288:35287-96 pubmed publisher
    The PI3K/mammalian Target of Rapamycin (mTOR) pathway is often aberrantly activated in rhabdomyosarcoma (RMS) and represents a promising therapeutic target...
  39. Diao Y, Guo X, Jiang L, Wang G, Zhang C, Wan J, et al. miR-203, a tumor suppressor frequently down-regulated by promoter hypermethylation in rhabdomyosarcoma. J Biol Chem. 2014;289:529-39 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma found in children and young adults. It is characterized by the expression of a number of skeletal muscle-specific proteins, including MyoD and muscle ?-actin...
  40. Skapek S, Anderson J, Barr F, Bridge J, Gastier Foster J, Parham D, et al. PAX-FOXO1 fusion status drives unfavorable outcome for children with rhabdomyosarcoma: a children's oncology group report. Pediatr Blood Cancer. 2013;60:1411-7 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is divided into two major histological subtypes: alveolar (ARMS) and embryonal (ERMS), with most ARMS expressing one of two oncogenic genes fusing PAX3 or PAX7 with FOXO1 (P3F and P7F, respectively)...
  41. Abraham J, Prajapati S, Nishijo K, Schaffer B, Taniguchi E, Kilcoyne A, et al. Evasion mechanisms to Igf1r inhibition in rhabdomyosarcoma. Mol Cancer Ther. 2011;10:697-707 pubmed publisher
    ..Igf1r) is an approach being taken in clinical trials to overcome the dismal outcome for metastatic alveolar rhabdomyosarcoma (ARMS), an aggressive muscle cancer of children and young adults...
  42. Schneider G, Bryndza E, Abdel Latif A, Ratajczak J, Maj M, Tarnowski M, et al. Bioactive lipids S1P and C1P are prometastatic factors in human rhabdomyosarcoma, and their tissue levels increase in response to radio/chemotherapy. Mol Cancer Res. 2013;11:793-807 pubmed publisher
    ..S1P) and ceramide-1-phosphate (C1P) strongly enhanced the in vitro motility and adhesion of human rhabdomyosarcoma (RMS) cells...
  43. Peinemann F, Kroger N, Bartel C, Grouven U, Pittler M, Erttmann R, et al. High-dose chemotherapy followed by autologous stem cell transplantation for metastatic rhabdomyosarcoma--a systematic review. PLoS ONE. 2011;6:e17127 pubmed publisher
    Patients with metastatic rhabdomyosarcoma (RMS) have a poor prognosis...
  44. Aquino M, Gibson D, Bloom D. Paratesticular rhabdomyosarcoma with metastatic encasement of the abdominal aorta. Pediatr Radiol. 2011;41:1061-4 pubmed publisher
    Paratesticular rhabdomyosarcoma is a rare but aggressive malignancy in children and adolescents...
  45. Federico S, Spunt S, Krasin M, Billup C, Wu J, Shulkin B, et al. Comparison of PET-CT and conventional imaging in staging pediatric rhabdomyosarcoma. Pediatr Blood Cancer. 2013;60:1128-34 pubmed publisher
    Over the past decade, PET-CT has been used to assess rhabdomyosarcoma (RMS) in children. However, the role of PET-CT in staging RMS is unknown.
  46. Hosoyama T, Aslam M, Abraham J, Prajapati S, Nishijo K, Michalek J, et al. IL-4R drives dedifferentiation, mitogenesis, and metastasis in rhabdomyosarcoma. Clin Cancer Res. 2011;17:2757-66 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood...
  47. Hosur V, Kavirayani A, Riefler J, Carney L, Lyons B, Gott B, et al. Dystrophin and dysferlin double mutant mice: a novel model for rhabdomyosarcoma. Cancer Genet. 2012;205:232-41 pubmed publisher
    ..Not surprisingly, the double mutant mice develop severe MD symptoms and, moreover, develop rhabdomyosarcoma (RMS) at an average age of 12 months, with an incidence of >90%...
  48. Baruchel S, Pappo A, Krailo M, Baker K, Wu B, Villaluna D, et al. A phase 2 trial of trabectedin in children with recurrent rhabdomyosarcoma, Ewing sarcoma and non-rhabdomyosarcoma soft tissue sarcomas: a report from the Children's Oncology Group. Eur J Cancer. 2012;48:579-85 pubmed publisher
    To determine the toxicity, efficacy and pharmacokinetics of trabectedin given over 24h every 3 weeks to children with recurrent rhabdomyosarcoma, Ewing sarcoma, or non-rhabdomyosarcoma soft tissue sarcomas.
  49. Raimondi L, Ciarapica R, De Salvo M, Verginelli F, Gueguen M, Martini C, et al. Inhibition of Notch3 signalling induces rhabdomyosarcoma cell differentiation promoting p38 phosphorylation and p21(Cip1) expression and hampers tumour cell growth in vitro and in vivo. Cell Death Differ. 2012;19:871-81 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is a paediatric soft-tissue sarcoma arising from skeletal muscle precursors coexpressing markers of proliferation and differentiation. Inducers of myogenic differentiation suppress RMS tumourigenic phenotype...
  50. Mascarenhas L, Lyden E, Breitfeld P, Walterhouse D, Donaldson S, Paidas C, et al. Randomized phase II window trial of two schedules of irinotecan with vincristine in patients with first relapse or progression of rhabdomyosarcoma: a report from the Children's Oncology Group. J Clin Oncol. 2010;28:4658-63 pubmed publisher
    To compare response rates for two schedules of irinotecan with vincristine in patients with rhabdomyosarcoma at first relapse or disease progression...
  51. Zhang M, Truscott J, Davie J. Loss of MEF2D expression inhibits differentiation and contributes to oncogenesis in rhabdomyosarcoma cells. Mol Cancer. 2013;12:150 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is a highly malignant pediatric cancer that is the most common form of soft tissue tumors in children. RMS cells have many features of skeletal muscle cells, yet do not differentiate...
  52. Tarnowski M, Grymula K, Liu R, Tarnowska J, Drukala J, Ratajczak J, et al. Macrophage migration inhibitory factor is secreted by rhabdomyosarcoma cells, modulates tumor metastasis by binding to CXCR4 and CXCR7 receptors and inhibits recruitment of cancer-associated fibroblasts. Mol Cancer Res. 2010;8:1328-43 pubmed publisher
    ..Here, we report that human rhabdomyosarcoma (RMS) cell lines secrete MIF and that this chemokine (a) induces phosphorylation of mitogen-activated protein ..
  53. Castleman W, Toplon D, Clark C, Heskett T, Farina L, Lynch T, et al. Rhabdomyosarcoma in 8 horses. Vet Pathol. 2011;48:1144-50 pubmed publisher
    This multi-institutional report describes 8 cases of rhabdomyosarcoma in horses. Four neoplasms were in the tongue and other areas of the mouth or head, 2 were in the abdominal wall, and 1 each was in right shoulder muscles and heart...
  54. Guenther M, Graab U, Fulda S. Synthetic lethal interaction between PI3K/Akt/mTOR and Ras/MEK/ERK pathway inhibition in rhabdomyosarcoma. Cancer Lett. 2013;337:200-9 pubmed publisher
    b>Rhabdomyosarcoma (RMS) frequently exhibits concomitant activation of the PI3K/Akt/mTOR and the Ras/MEK/ERK pathways. Therefore, we investigated whether pharmacological cotargeting of these two key survival pathways suppresses RMS growth...
  55. Kriseman M, Wang W, Sullinger J, Schmeler K, Ramirez P, Herzog C, et al. Rhabdomyosarcoma of the cervix in adult women and younger patients. Gynecol Oncol. 2012;126:351-6 pubmed publisher
    Cervical rhabdomyosarcoma is extremely rare, and there is a paucity of literature on the subject. The purpose of this study was to describe the clinical and pathologic features of cervical rhabdomyosarcoma.
  56. Kang Z, Chen J, Yu Y, Li B, Sun S, Zhang B, et al. Drozitumab, a human antibody to death receptor 5, has potent antitumor activity against rhabdomyosarcoma with the expression of caspase-8 predictive of response. Clin Cancer Res. 2011;17:3181-92 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is a common pediatric soft-tissue tumor. In this study, we evaluated the efficacy and selectivity of drozitumab, a death receptor DR5-targeted therapeutic antibody, in RMS preclinical models...
  57. Li Y, Yu C, Wu S, Dai M, Lee H. Malignant mesenchymal tumor with leiomyosarcoma, rhabdomyosarcoma, chondrosarcoma, and osteosarcoma differentiation: case report and literature review. Diagn Pathol. 2011;6:35 pubmed publisher
    A case of malignant mesenchymoma of the bladder containing leiomyosarcoma, rhabdomyosarcoma, chondrosarcoma, osteosarcoma, and myxomatous components is described...
  58. O BRIEN D, Jacob A, Qualman S, Chandler D. Advances in pediatric rhabdomyosarcoma characterization and disease model development. Histol Histopathol. 2012;27:13-22 pubmed publisher
    b>Rhabdomyosarcoma (RMS), a form of soft tissue sarcoma, is one of the most common pediatric malignancies...
  59. Avirneni Vadlamudi U, GALINDO K, Endicott T, Paulson V, Cameron S, Galindo R. Drosophila and mammalian models uncover a role for the myoblast fusion gene TANC1 in rhabdomyosarcoma. J Clin Invest. 2012;122:403-7 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is a malignancy of muscle myoblasts, which fail to exit the cell cycle, resist terminal differentiation, and are blocked from fusing into syncytial skeletal muscle...
  60. Al Tahan A, Sarkis O, Harajly M, Baghdadi O, Zibara K, Boulos F, et al. Retinoic acid fails to induce cell cycle arrest with myogenic differentiation in rhabdomyosarcoma. Pediatr Blood Cancer. 2012;58:877-84 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Current treatment strategies do not cure most children with recurrent or high-risk disease, underlying the need for novel therapeutic approaches...
  61. Schoot R, McHugh K, van Rijn R, Kremer L, Chisholm J, Caron H, et al. Response assessment in pediatric rhabdomyosarcoma: can response evaluation criteria in solid tumors replace three-dimensional volume assessments?. Radiology. 2013;269:870-8 pubmed publisher
    ..Tumors [RECIST]) response assessments, (b) intermethod variability between EpSSG guidelines and RECIST, and (c) clinically relevant consequences of interobserver and intermethod variability in pediatric patients with rhabdomyosarcoma.
  62. Lee S, Kim J, Kim J, Lee K, Park J, Hur S. Malignant mixed mullerian tumor of the cervix including components of a rhabdomyosarcoma: case report and literature review. Eur J Gynaecol Oncol. 2010;31:462-6 pubmed
    ..For these reasons, MMMTs of the cervix may have a better prognosis compared to the uterine counterparts. A case of an immunohistochemically confirmed primary MMMT of the cervix, including components of a rhabdomyosarcoma, is reported.
  63. Tonelli R, McIntyre A, Camerin C, Walters Z, Di Leo K, Selfe J, et al. Antitumor activity of sustained N-myc reduction in rhabdomyosarcomas and transcriptional block by antigene therapy. Clin Cancer Res. 2012;18:796-807 pubmed publisher
    ..Our aim was to determine the prevalence of N-Myc protein expression and the potential therapeutic effects of reducing expression in rhabdomyosarcomas, including use of an antigene strategy that inhibits transcription...
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    b>Rhabdomyosarcoma is a pediatric malignancy thought to arise from the uncontrolled proliferation of myogenic cells...
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    ..We also demonstrated that inhibition of the STAT3 pathway led to apoptosis in human rhabdomyosarcoma cells...
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    ..CIITA overexpression can also activate the expression of these genes, indicating that the immunoproteasome genes LMP2 and LMP7 can be activated by both CIITA dependent and CIITA independent pathways...
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    The prognosis for children and adolescents with rhabdomyosarcoma (RMS) has improved with refinements in multi-modal therapy...
  68. Kaylani S, Xu J, Srivastava R, Kopelovich L, Pressey J, Athar M. Rapamycin targeting mTOR and hedgehog signaling pathways blocks human rhabdomyosarcoma growth in xenograft murine model. Biochem Biophys Res Commun. 2013;435:557-61 pubmed publisher
    ..Our data provide bases for using rapamycin either alone or in combination with traditional chemotherapeutic drugs to block the pathogenesis of high risk RMS...
  69. Keller C, Guttridge D. Mechanisms of impaired differentiation in rhabdomyosarcoma. FEBS J. 2013;280:4323-34 pubmed publisher
    b>Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma of childhood, with presumed skeletal muscle origins, because of its myogenic phenotype. RMS is composed of two main subtypes, embryonal RMS (eRMS) and alveolar RMS (aRMS)...
  70. Diaconescu S, Burlea M, Miron I, Aprodu S, Mihaila D, Olaru C, et al. Childhood rhabdomyosarcoma. Anatomo-clinical and therapeutic study on 25 cases. Surgical implications. Rom J Morphol Embryol. 2013;54:531-7 pubmed
    ..Despite advances in knowledge about biological pathways of tumorigenesis, risk stratification and multimodal treatment, the immediate and long-term prognosis of these lesions in many countries with limited resources is still poor...
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    Searching for new strategies to trigger apoptosis in rhabdomyosarcoma (RMS), we investigated the effect of two novel classes of apoptosis-targeting agents, i.e...
  72. MacQuarrie K, Yao Z, Fong A, Diede S, Rudzinski E, Hawkins D, et al. Comparison of genome-wide binding of MyoD in normal human myogenic cells and rhabdomyosarcomas identifies regional and local suppression of promyogenic transcription factors. Mol Cell Biol. 2013;33:773-84 pubmed publisher
    b>Rhabdomyosarcoma is a pediatric tumor of skeletal muscle that expresses the myogenic basic helix-loop-helix protein MyoD but fails to undergo terminal differentiation...