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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A human tumour cell line, designated RMS-YM, was established from a childhood rhabdomyosarcoma. The monolayer cells were polygonal, round or spindle-shaped. The cells became multilayered and formed many focal piles when confluent. RMS-YM became stable with a doubling time of about 30 h and has been maintained for 104 passages to date. Tumourigenicity of the cells was confirmed by heterotransplantation into nude mice. Morphological features were similar to those of the primary tumour, and myofibrils were found by electron microscopy. The expression of desmin and human myoglobin, and high levels of striated muscle system specific enzymes were recognised. Chromosomal analysis revealed possible gene amplification in the form of homogeneously staining regions. Oncogene analysis was performed on the primary tumour and the cell line, but neither N-myc nor N-ras genes were amplified, nor were Ki-ras, Ha-ras or N-ras genes mutated at the 12th, 13th and 61st codons. The RMS-YM cell line may provide a system to identify novel genes which are amplified in rhabdomyosarcoma.
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PMID:Cytogenetic and cellular characteristics of a human embryonal rhabdomyosarcoma cell line, RMS-YM. 190 91

We studied the human embryonal rhabdomyosarcoma cell line RD and 8 derivatives obtained in our laboratory either by cell cloning or by culturing in vitro cells from tumors or secondaries grown in nude mice. The expression of desmin and of the embryonic isoform of myosin and the formation of multinuclear myotube-like structures were studied as specific markers of myogenic differentiation. During continuous growth, each derivative contained a proportion (ranging from 5 to 80% among derivatives) of desmin-positive cells and a small number of myosin-positive or multinuclear elements. Cells from continuous cultures were injected intravenously in nude mice, producing lung and kidney/adrenal nodules. No correlation was found between the proportion of cells expressing desmin and metastatic capacity. When cultures were grown in differentiation medium (Dulbecco's minimal essential medium + 2% horse serum) some derivatives (designated type A) showed a strong increase in the proportion of myosin-positive cells, while others (type B) showed no increase. In vitro differentiation significantly reduced the metastatic ability of type A cells, while no modification was observed in type B after growth in differentiation medium. The proliferative ability of type A and type B cells grown in differentiation medium did not correlate with the proportion of myosin-positive cells, and extensive formation of multinuclear myotubes was never observed. It was concluded that reduction of experimental metastatic ability was mediated by events related to late, though not necessarily terminal, differentiation of rhabdomyosarcoma cells.
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PMID:Reduced metastatic ability of in vitro differentiated human rhabdomyosarcoma cells. 191 85

Sites in the head and neck region (orbit, nasopharynx, nasal cavity, etc.) are among the most frequent locations for juvenile (embryonal and alveolar) rhabdomyosarcomas in patients younger than 15 years; however, comparable neoplasms in adults are very uncommon. A clinicopathologic and immunohistochemical study of 12 juvenile rhabdomyosarcomas in patients between the ages of 18 and 36 years is presented. There was a female:male ratio of 2:1. The orbit with or without contiguous paranasal sinus involvement, nasal cavity, sphenoid sinus, middle ear, and soft tissues of the neck and preauricular region were the primary sites. Seven tumors involved a parameningeal site and eight cases were alveolar rhabdomyosarcomas which together contributed to the adverse outcome. Only two patients were long-term, disease-free survivors. Six patients have died of tumor and two others are alive with persistent disease. Immunohistochemical study in 11 cases demonstrated reactivity for vimentin and muscle-specific actin (HHF-35) and desmin in ten cases. Juvenile rhabdomyosarcoma rarely presents in the head and neck of adults but should be considered in the differential diagnosis of a small cell neoplasm in patients during the third and fourth decades of life.
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PMID:Juvenile (embryonal and alveolar) rhabdomyosarcoma of the head and neck in adults. A clinical, pathologic, and immunohistochemical study of 12 cases. 199 Dec 49

In vitro cultures and clonal derivatives have been established from rat rhabdomyosarcomas induced by Moloney-Murine Sarcoma Virus (MSV) or by nickel sulfide; differentiation ability has been studied as expression of desmin, embryonic and adult myosin isoforms, alpha-actin isoforms and cellular fusion. The two rhabdomyosarcoma models showed different levels of myogenic differentiation. Multinucleated myotube-like structures were frequently observed in cultures derived from nickel-induced tumours. Desmin was present in 50-80% of cells and embryonic myosin in up to 10%. In MSV-tumour-derived cultures and in their metastases or clonal derivatives two cell types are present in different ratios: spindle-shaped cells, adherent to plastic surfaces, and rounded cells, loosely attached or floating free in the medium. These cultures showed features of myogenic differentiation (10-80% desmin-positive cells), but embryonic myosin expression and production of multinucleated myotube-like structures were very rare events. Cultures from autochthonous lymph node and lung metastatic cells showed similar patterns of differentiation. Retinoic acid increased differentiated features (myotube formation and embryonic myosin expression) only in nickel-induced rhabdomyosarcoma cells. The two models described here mimic the heterogeneity in differentiation pattern found among human rhabdomyosarcomas. Myogenic differentiation ability was retained at a good level by nickel-induced tumours, whereas it was strongly impaired in MSV-induced tumours.
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PMID:In vitro differentiation of rhabdomyosarcomas induced by nickel or by Moloney murine sarcoma virus. 203 98

The cytologic and histologic findings in two cases of the extremely rare alveolar rhabdomyosarcoma of the vulva are reported. The tumor cells in fine needle aspiration smears from one case and tumor imprints from both cases were isolated or were in sheets or reticular patterns. The nuclei were round to oval; only a few cells were multinucleated. The chromatinic material was increased in amount and finely granular. Many mitotic figures were observed. The cytoplasm was scanty in general, but some cells had abundant cytoplasm; cross striations were not recognized. The tumor cells were positive with immunocytochemical stains for desmin, vimentin and myoglobin. Similar findings were observed in biopsy and surgical specimens.
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PMID:Alveolar rhabdomyosarcoma of the vulva. Report of two cases. 204 36

Rhabdomyoblasts demonstrating immunoreactivity for muscle-specific actin, desmin, and myoglobin were identified in smears obtained by aspiration from a large retroperitoneal mass in a 14-mo-old girl. Following a tentative diagnosis of a rhabdomyogenous neoplasm, retroperitoneal exploration and adrenalectomy demonstrated a stromal poor neuroblastoma with extensive rhabdomyogenous differentiation. The presence of a subpopulation of rhabdomyoblasts was not diagnostic of rhabdomyosarcoma when obtained by fine-needle aspiration from a retroperitoneal tumor composed predominantly of primitive small round cells.
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PMID:Rhabdomyosarcomatous differentiation in a neuroblastoma: a potential pitfall in the cytologic diagnosis of small round-cell tumors of childhood. 206 76

We present cytogenetic and molecular genetic analyses of two cases of alveolar rhabdomyosarcoma. The characteristic translocation between chromosomes 2 and 13, t(2;13)(q35;q14), has been identified in both cases. Using cell lines derived from these tumor specimens, we have performed Southern blot analysis to investigate the possibility of rearrangement of 14 candidate genes mapping to the relevant regions of 2q and 13q. These candidate genes can be divided into 5 groups: signal transduction proteins (RB1, inhibin alpha, FLT1, and HOX4B), muscle-specific products [myosin light chain, desmin, and nicotinic cholinergic receptor subunits gamma and delta (CHRNG and CHRND)], extracellular matrix proteins (collagen type VI alpha 3 chain, elastin, and fibronectin), transformation-associated products (intestinal alkaline phosphatase and L-plastin), and other genes (esterase D). Conventional gel electrophoresis followed by Southern blot analysis indicated no evidence of rearrangement within or near these genes except for a rearrangement in the CHRNG-CHRND locus, which occurred only in a subpopulation of the late recurrence tumor cells of one patient. In addition, we employed pulsed-field gel electrophoresis-Southern blot analysis to demonstrate the absence of detectable rearrangements within a larger region around each of these genes.
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PMID:Molecular and cytogenetic analysis of chromosomal arms 2q and 13q in alveolar rhabdomyosarcoma. 206 13

An immunohistochemical analysis using a series of mono- and polyclonal antibodies against myoglobin, desmin, vimentin, actin, NSE, S-100 protein and keratin was done on alveolar rhabdomyosarcoma (3 cases), embryonal (3) and pleomorphic rhabdomyosarcoma of the orbit (1). All tumors were stained by monoclonal antibodies to desmin and vimentin, whereas 6 of the 7 cases were stained by myoglobin. The results indicate that desmin and vimentin are highly sensitive immunohistochemical markers for the diagnosis of rhabdomyosarcoma, especially in poorly differentiated ones.
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PMID:Orbital rhabdomyosarcoma. Immunohistochemical studies of seven cases. 211 61

We report on an alveolar rhabdomyosarcoma occurring in a 17-year-old girl. Histologically, the tumour exhibited a proliferation of small, round cells and formed alveolar structures. Immunohistochemical studies of the tumour cells showed desmin and creatine kinase MM positivity. In electron-microscopic studies, the tumour cells showed an abundance of glycogen granules, and myofilaments were recognized in the cytoplasm. Chromosome analysis revealed a translocation, t(2;13)(q37;q14), which is though to be common in this subtype of rhabdomyosarcoma. Conventional ultrastructural and immunohistochemical investigations and chromosome analysis thus appear to be a highly promising combination of methods for improved pathological diagnosis of alveolar rhabdomyosarcoma.
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PMID:A case of alveolar rhabdomyosarcoma with a chromosomal translocation, t(2;13)(q37;q14). 212 83

In murine sarcomas induced by 20-methylcholanthrene, histological features of malignant fibrous histiocytoma (MFH) as well as rhabdomyosarcoma were found in the same tumour both at light microscopy and at ultrastructural level. The areas showing rhabdomyomatous differentiation expressed vimentin, desmin, muscle-specific alpha-actinin, and sometimes myoglobin, but in the MFH areas only vimentin was expressed. A series of allografts in athymic mice, using tumour areas of both histological types, showed in every case a mixed pattern of tumour growth, whether the transplanted tissue was of MFH or rhabdomyosarcomatous type. This suggests that the MFH areas in the original experimental sarcomas were modulated disguised rhabdomyosarcomas. The significance of MFH-like areas in non-related soft tissue sarcomas is also discussed.
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PMID:Experimental rhabdomyosarcoma with regions like malignant fibrous histiocytoma (MFH)--a true double phenotypic pattern? 215 83


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