Disease relevance of Rhabdomyosarcoma, Embryonal

• CEA plasma values greater than 3.35 ng/ml were found in 35% and 24% of patiens with neuroblastoma and embryonal rhabdomyosarcoma, respectively [1].
• On average, alveolar rhabdomyosarcomas expressed at least threefold more myogenin than embryonal rhabdomyosarcomas [2].
• Immunohistochemical staining revealed a subgroup of fetal rhabdomyomas and embryonal rhabdomyosarcomas (12/34) lacking PTCH immunoreactivity [3].
• Lactoferrin inhibits enterovirus 71 infection of human embryonal rhabdomyosarcoma cells in vitro [4].
• Furthermore, distinguishing these neoplasms from neuroblastoma, embryonal rhabdomyosarcoma, small cell osteogenic sarcoma, and non-Hodgkin's lymphoma can be difficult [5].

High impact information on Rhabdomyosarcoma, Embryonal

• To test this we have characterized total and allele-specific mRNA expression levels and DNA methylation of the 11p15.5 KIP2 gene in normal human tissues, WTs and embryonal rhabdomyosarcoma (RMS) [6].
• The results show that 35% (5/14) embryonal rhabdomyosarcomas investigated contain mutations in the N-ras or K-ras genes [7].
• One of two embryonal rhabdomyosarcomas that strongly stained for myogenin was retrospectively found to be positive for Pax3/FKHR transcripts [2].
• We propose that embryonal rhabdomyosarcomas result from an early block in myogenesis, before the expression of myogenin [2].
• In embryonal rhabdomyosarcomas, desmin prevailed in the large, apparently well-differentiated rhabdomyoblasts; whereas the smaller, less differentiated tumor cells preferentially contained vimentin [8].

Chemical compound and disease context of Rhabdomyosarcoma, Embryonal

• RD/18 clone cells (derived from the embryonal rhabdomyosarcoma cell line RD) treated with 100 nM dexamethasone showed an almost complete block of differentiation: about 5% myosin-positive cells were observed after 2 weeks of culture in dexamethasone-supplemented differentiation medium, compared to 20% of untreated cultures [9].
• Ifosfamide, doxorubicin and dacarbazine chemotherapy, and adjunctive laparoscopic cystoprostatectomy and external beam radiotherapy for prostatic embryonal rhabdomyosarcoma [10].
• To solve these problems, fluorescent in situ hybridization (FISH) technique was used on free nuclei isolated from formalin-fixed paraffin-embedded embryonal rhabdomyosarcoma (RMS) tissue using our modification of Hedley's method for isolation of nuclei [11].
• The IRSG has reported improvement in the prognosis of both nonmetastatic and metastatic embryonal rhabdomyosarcoma as attributable to three, three-drug regimens that use cyclophosphamide at 2.2 g/m2 in either maintenance or induction and maintenance therapy [12].
• We conclude that exposure of this human embryonal rhabdomyosarcoma cell line to low concentrations of actinomycin D released the neoplastic cells from their blockade, allowing them to recover normal myogenic development [13].

Biological context of Rhabdomyosarcoma, Embryonal

• Four of nine informative fetal rhabdomyomas and embryonal rhabdomyosarcomas showed loss of heterozygosity (LOH) in the PTCH region with two of these (one fetal rhabdomyoma and one embryonal rhabdomyosarcoma) also showing LOH in the SUFU region [3].
• Characterization of an embryonal rhabdomyosarcoma cell line showing amplification and over-expression of the N-myc oncogene [14].

Anatomical context of Rhabdomyosarcoma, Embryonal

• Similarly, MYCN protein was detected in all cell lines by western blot analysis, with higher levels of expression in alveolar than in embryonal rhabdomyosarcoma cells [15].
• Desmin positivity was seen in 10/11 cases of embryonal rhabdomyosarcoma, whereas immunopositivity with leukocyte common antigen was detected in both cases of inflammatory pseudotumor [16].

Gene context of Rhabdomyosarcoma, Embryonal

• To explore molecular pathways altered by this oncoprotein, we generated an inducible form by fusing PAX3-FKHR to a modified estrogen receptor ligand-binding domain and expressed this construct in the RD embryonal rhabdomyosarcoma cell line [17].
• PAX7 expression in embryonal rhabdomyosarcoma suggests an origin in muscle satellite cells [18].
• Thus, we suggest that the significant difference (p < 0.01) in the less aggressive clinical behavior of embryonal rhabdomyosarcomas in comparison to other subtypes may be due to differences in telomerase expression [19].
• Here, we show that a single solid malignancy, i.e., a human embryonal rhabdomyosarcoma, produces in vivo at least three biologically active angiogenesis stimulators (vascular endothelial growth factor, basic fibroblast growth factor and interleukin-8) [20].
• In one case of embryonal rhabdomyosarcoma nearly all tumor cells were stained, but in the remaining cases few or no tumor cells were positive with the vimentin antibody [21].

Analytical, diagnostic and therapeutic context of Rhabdomyosarcoma, Embryonal

• Tissue-specific expression of neural cell adhesion molecule (NCAM) may allow differential diagnosis of neuroblastoma from embryonal rhabdomyosarcoma [22].
• The value of the embedding technique for ultrastructural analysis and immunohistochemistry in the demonstration of desmin in aspirates is emphasized in the diagnosis of embryonal rhabdomyosarcoma [23].

References