Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Tumor invasion and metastasis are believed to involve the adhesive interaction of tumor cells with extracellular matrix (ECM). This study reports the expression of laminin, fibronectin, thrombospondin, tenascin, and CD44 in rhabdomyosarcoma cells taken from tumors derived from 12 pediatric patients. Twelve paraffin-embedded rhabdomyosarcomas consisting of five alveolar, six embryonal, and one botryoid subtype were examined. All tumors demonstrated positive staining for tenascin and thrombospondin, 10 were positive for fibronectin, 5 positive for laminin, and 4 positive for CD44. Both specimens without cellular staining for fibronectin were of embryonal subtype and no alveolar rhabdomyosarcomas were positive for CD44. No association of the expression of the studied ECM proteins and receptor with the presence of metastatic disease at clinical presentation or with the level of tumor differentiation was found.
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PMID:Cellular expression of adhesion factors in childhood rhabdomyosarcoma. 908 32

The expression pattern of CD44 standard and variant isoforms are prognostically significant in a number of malignancies. The aim of this study was to evaluate the role of the standard isoform of CD44 in predicting the clinical behaviour of rhabdomyosarcoma. Immunohistochemical analysis of CD44 was undertaken using a panel of antibodies recognizing the three core domains of the CD44 molecule. Labelling was repeated in triplicate and reported blind with respect to histological type and outcome. Tumours were characterized as positive in more than 60% of tumour cells labelled and negative if less than 40% of tumour cells labelled. Tumours with 40-60% of tumour cells labelling were considered indeterminate. Eleven of 20 favourable histology tumours were positive for CD44 compared with one of seven unfavourable tumours (P = 0.07). Eleven of 12 patients with CD44-positive tumours are alive in first remission compared with five of 15 CD44-negative tumours (P = 0.001). Expression of CD44 correlates directly with prognosis; however, larger studies are required so that multivariate analysis can be undertaken.
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PMID:Expression of CD44 by rhabdomyosarcoma: a new prognostic marker? 1036 Jun 76

The pathologic classification of rhabdomyosarcoma (RMS) into embryonal or alveolar subtype is an important prognostic factor guiding the therapeutic protocol chosen for an individual patient. Unfortunately, this classification is not always straightforward, and the diagnostic criteria are controversial in a subset of cases. Ancillary studies are used to aid in the classification, but their potential use as independent prognostic factors is rarely studied. The aim of this study is to identify immunohistochemical markers of potential prognostic significance in pediatric RMS and to correlate their expression with PAX-3/FKHR and PAX-7/FKHR fusion status. A single tissue microarray containing 71 paraffin-embedded pediatric RMSs was immunostained with antibodies against p53, bcl-2, Ki-67, CD44, myogenin, and MyoD1. The tissue microarray and whole paraffin blocks were studied for PAX-3/FKHR and PAX-7/FKHR gene fusions by fluorescence in situ hybridization and reverse transcription-polymerase chain reaction. Clinical follow-up data were available for each patient. Immunohistochemical staining results and translocation status were correlated with recurrence-free interval (RFI) and overall survival (OS) using the Kaplan-Meier method, the log-rank test, and Cox proportional hazard regression. The minimum clinical follow-up interval was 24 months (median follow-up=57 mo). On univariable analysis, immunohistochemical expression of myogenin, bcl-2, and identification of a gene fusion were associated with decreased 5-year RFI and 10-year OS (myogenin RFI P=0.0028, OS P=0.0021; bcl-2 RFI P=0.037, OS P=0.032; gene fusion RFI P=0.0001, OS P=0.0058). After adjustment for Intergroup Rhabdomyosarcoma Study-TNM stage, tumor site, age, tumor histology, and translocation status by multivariable analysis, only myogenin retained an independent association with RFI (P=0.034) and OS (P=0.0069). In this retrospective analysis, diffuse immunohistochemical reactivity for myogenin in RMS correlates with decreased RFI and OS, independent of histologic subtype, translocation status, tumor site, or stage.
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PMID:Diffuse myogenin expression by immunohistochemistry is an independent marker of poor survival in pediatric rhabdomyosarcoma: a tissue microarray study of 71 primary tumors including correlation with molecular phenotype. 1870 38