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)

The induction of rabbit rhabdomyosarcoma was obtained after intramuscular implantation of a large quantity of very pure nickel subsulphide, though until the present time the rabbit was considered refractory to Ni3S2 tumorigenesis. These tumors are similar to those induced in rats under the same conditions. Four different cell types were observed: small polygonal cells, small elongated cells, giant cells, and mature myofibers. Electron microscopy reveals a complete disorientation of myofibrils in mature myoblasts. Giant cells appear by pluripolar endomitosis and always contain myofibrillar structures, but M-lines and Z-lines are not present in these cells. Cylindrical laminated bodies were observed very often in all four cell types. They are formed of 4 nm fibrils arranged in crossed position in each lamella. Some of these paracrystalline structures were also observed in nuclei. The laminated bodies are considered to be abnormal formations of contractile proteins produced during tumoral myofibrillar differentiation.
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PMID:Cylindrical laminated bodies in nickel-subsulphide-induced rhabdomyosarcoma in rabbits. 48 25

Amplification and rearrangement of cellular proto-oncogenes are two of the several possible genetic alterations implicated in carcinogenesis and tumor progression. Although morphologically similar tumors may be heterogeneous at the level of the genome, some tumor types have shown relatively frequent and consistent abnormalities of specific oncogenes. In order to determine the frequency of oncogene amplification and rearrangement in several types of human sarcomas and to determine if histologically similar tumors have common genetic alterations, we analyzed 26 primary sarcomas by Southern hybridization. The oncogene probes utilized were N- and H-ras, sis, EGF-R (erb-B-1), neu (erb-B-2), fos, N- and c-myc, mos, and yes. The tumors studied included: five rhabdomyosarcomas (one alveolar, four embryonal), six malignant fibrous histiocytomas, six leiomyosarcomas, four liposarcomas, two Ewing's sarcomas, one osteosarcoma, and two fibrosarcomas. Oncogene abnormalities were identified in three tumors. One rhabdomyosarcoma showed 12-fold amplification and concurrent rearrangement of sis. This particular tumor also revealed rearrangement of H-ras and 15-fold amplification of c-myc. A second rhabdomyosarcoma revealed rearrangement of neu. A liposarcoma had a sis rearrangement. These findings suggest that many sarcomas show no common structural oncogene abnormalities. The presence of differing oncogene alterations within the rhabdomyosarcoma group indicates genetic heterogeneity among histologically similar sarcomas. The finding of a sis rearrangement in both a liposarcoma and a rhabdomyosarcoma, however, may suggest common oncogenesis among different tumor types.
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PMID:Genomic alterations in sarcomas: a histologic correlative study with use of oncogene panels. 149 46

Childhood rhabdomyosarcoma and the other soft tissue sarcomas of childhood are a heterogeneous group of tumors with many histologic subtypes, affecting multiple anatomic locations and requiring the care of subspecialists from a variety of disciplines. Epidemiologic studies of childhood sarcomas indicate that a familial predisposition to malignancy may contribute to tumorigenesis in a proportion of patients. Diagnosis of these tumors may be difficult; immunohistochemical and cytogenetic analysis is clearly important. Large cooperative group studies have answered important treatment questions in past years; these studies are now being analyzed for clues to effective treatment of specific histologic and anatomic subgroups. The Intergroup Rhabdomyosarcoma Study IV is now open and the questions addressed are reviewed. New developments in late effects of therapy for childhood sarcoma are considered.
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PMID:Rhabdomyosarcoma and other soft tissue sarcomas of childhood. 151 Oct 25

Moloney murine sarcoma virus (M-MSV) induces rapidly growing tumours in adult mice of most conventional strains. Rats are less susceptible to M-MSV oncogenesis, but the few rhabdomyosarcomas that do develop after viral inoculation of newborn animals closely resemble conventional malignancies: they develop after a long latency, grow progressively, and metastasize to regional lymph nodes and lungs. Southern blot analysis with a v-mos-specific probe of M-MSV-induced tumours in both species demonstrated an oligo-, monoclonal pattern of exogenous v-mos integration only in the rat system, while mouse tumours were not clonal in origin. Furthermore, the same type of analysis of lymph node and lung metastases showed that cell clones already present in the primary rat lesion colonized secondary sites during tumour progression. Apparently, Moloney murine leukemia virus (M-MuLV) was not involved in rhabdomyosarcoma pathogenesis since M-MuLV-specific DNA sequences could not be demonstrated in three of the six rat tumours. Finally, in all mouse tumours, unintegrated linear M-MSV proviruses could be readily detected.
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PMID:Tumours induced by Moloney murine sarcoma virus are clonal in rats, not clonal in mice. 173 35

The effects of short-term exposure to phorbol ester tumor promoters on epithelial and mesenchymal skin tumor induction, studied histologically in female S/RVCri mice, rendered precancerous by a subcutaneous (sc) injection of 3-methylcholanthrene (MCA). At 6 weeks after MCA injection, mice in Groups I to VI received topical twice weekly applications of 0.1 ml acetone, 1.8 nmol 12-O-tetradecanoylphorbol-13-acetate (TPA), phorbol didecanoate (PDD), phorbol dibenzoate (PDB), phorbol diacetate (PDA) or 6.1 nmol mezerein (MEZ) in 0.1 ml acetone, respectively, for four weeks. Animals were sacrificed after the development of palpable tumors. Data from various phorbol ester treatment groups were compared with the acetone control. Of the promoters tested, TPA treatment increased the percentage of tumor bearers and the number of combinations and expression of diverse neoplasms in the mixed growths. TPA-related phorbol esters such as PDD, PDB or MEZ did not alter the percentage of tumor bearers although the histological distribution into pure epithelial or mesenchymal and mixed tumor bearers differed significantly from the control treated with the solvent alone. However, PDA significantly inhibited MCA-induced skin tumorigenesis and increased the average time of tumor induction. All the promoters, except TPA, decreased the percentage of mixed tumor bearers, the development of hibernoma and fibrosarcoma, while allowing a selective expression of rhabdomyosarcoma.
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PMID:Influence of short-term topical exposure to phorbol ester tumor promoters on subcutaneously induced skin tumorigenesis in S/RVCri mice. 273 6

About 5% of Mendelian mutations displaying neoplastic tendencies are associated with chromosomal aberrations. The best established examples are retinoblastoma and del(13)(q14) and aniridia-Wilms' tumor and del(11)(p13). Evidence suggests that both mutations behave as dominant traits in the individual and as recessive traits in the cells. DNA analysis indicates that tumorigenesis arises from homozygosisty for the mutant allele at these loci, as a consequence of mitotic nondisjunction or from a mitotic recombination event. An additional argument for this conclusion is provided by the demonstration of duplication of 11p15 in some patients with the Beckwith-Wiedemann syndrome, which is complicated often by Wilms' tumor and other embryonal tumors. Data obtained with molecular probes have shown that also rhabdomyosarcoma and hepatoblastoma arise by homozygosity for a mutant allele at a locus on 11p, suggesting ontogenic relatedness of these tumor types. Additional examples of Mendelian mutations associated with chromosome deletions and neoplasia include Langer-Giedion syndrome with multiple exostoses and del(8)(q24.1), multiple endocrine neoplasia and del(20)(p12.2). While the presence of specific chromosome changes in subjects with high susceptibility to neoplasia does pinpoint the location of DNA sequences involved in the predisposition to certain types of cancers, selected Mendelian mutations associated with chromosome instability and cancer proneness may elucidate biological principles of cell proliferation and transformation. However, our current knowledge of mechanisms resulting in increased frequency of chromosome breakage and cancer susceptibility in ataxia-teleangiectasia, Fanconi's anemia, Bloom's syndrome, and similar conditions are still very incomplete.
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PMID:Cytogenetics of Mendelian mutations associated with cancer proneness. 382 76

The distribution of neutral, or asialosyl-, glycosphingolipids and gangliosides in a rhabdomyosarcoma of alveolar type have been studied. Histologically, this muscle tumor is composed primarily of two cell types: one with oval or round hyperchromatic nuclei and very little cytoplasm, and one a giant cell, with multiple, peripherally placed nuclei and weakly staining eosinophillic cytoplasm. In comparing glycolipids of the rhabdomyosarcoma with normal muscle from the same leg, the striking alteration in the tumor was a virtual disappearance of ganglioside GM2. There was also a slight increase in GM3 and a decrease in GD1a. The asialosyl derivative of GM2 (GalNac-Gal-Glc-Cer) was markedly increased in the tumor. A loss of glucosylceramide was also observed. The results are discussed in terms of glycolipid metabolic changes in muscle oncogenesis and their implications.
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PMID:Glycosphingolipids of an alveolar rhabdomyosarcoma and normal human muscle. A case study. 653 79

Skin tumors were induced in 6-week-old female Swiss albino mice by a single subcutaneous (SC) injection of 20-methylcholanthrene (MCA) in the right scapular region and the animals were then divided into four groups. Mice in group I did not receive further treatment. Six weeks after MCA injection, those in groups II and III received twice weekly applications of 0.1 ml acetone and 1.8 nmol 12-0-tetradecanoylphorbol-13-acetate (TPA) in 0.1 ml acetone, respectively, at the site of MCA injection until tumor development. Group IV animals were divided into four subsets and administered two, four, six, or eight TPA applications commencing 6 weeks after carcinogen injection. The effect of TPA pretreatment on MCA-induced tumorigenesis was studied in animals in group V. In mice treated with MCA alone, the most predominant mesenchymal tumor type is fibrosarcoma with induction of some rhabdomyosarcomas. Mixed mesenchymal tumors consisting of fibrosarcoma, rhabdomyosarcoma, or hibernoma were observed in only 12% of the animals. The number of animals bearing mixed mesenchymal tumors such as fibrosarcoma, rhabdomyosarcoma, hibernoma, and/or liposarcoma increased to 46% in mice receiving MCA + TPA until tumor development. Interestingly, liposarcomas were not found at all in animals treated with MCA alone. The data indicates that TPA application to precancerous mouse skin enhances mesenchymal tumorigenesis.
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PMID:12-0-Tetradecanoylphorbol-13-acetate-induced amplification of mesenchymal tumorigenesis in the mouse skin. 683 39

This review of the past year's literature summarizes the most relevant advances in the biology and therapy of rhabdomyosarcoma and other pediatric soft tissue sarcomas. The results of the third Intergroup Rhabdomyosarcoma Study clearly show that therapy based on specific risk factors offers the best chance of cure for children with rhabdomyosarcoma. The recent identification of nonrandom chromosomal translocations within distinct histologic subtypes of rhabdomyosarcoma and nonrhabdomyosarcoma soft tissue sarcomas offers a unique opportunity to improve our ability to diagnose, stage, and monitor these patients. Finally, identification of the genetic features that characterize these tumors will help us better understand the mechanisms involved in tumorigenesis and will facilitate the development of novel specific therapies.
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PMID:Rhabdomyosarcoma and other soft tissue sarcomas of childhood. 757 85

Expression of the RCK gene, which is a target gene on 11q23 of the t(11;14) (q23;q32) translocation in the B-cell lymphoma cell line RC-K8, was studied by Northern and Western blot analyses. The RCK gene product is a member of the D-E-A-D box protein/RNA helicase family. With the use of Northern blot analysis, a 7.5-kb transcript of the RCK gene was shown to be expressed ubiquitously in human and mouse tissues. Polyclonal antibodies against the RCK gene product were raised, and the RCK gene expression pattern was examined in human and mouse tissues. Two different polyclonal anti-rck antibodies detected a specific 54-kilodalton product named rck/p54 in the majority of human and mouse tissues tested by Western blot analysis. However, rck/p54 was shown to be very low in the human brain and was not detectable in lumbar muscle and lung tissues, although RCK mRNA is abundantly present in these tissues. It is of interest that malignant transformed human cells arising from tissues with low or no expression of rck/p54, such as neuroblastoma, glioblastoma, rhabdomyosarcoma, and lung cancer cell lines, produced a moderate amount of rck/p54 protein, suggesting that rck/p54 plays a role in tumorigenesis. In addition, the rck/p54 protein was localized to cytoplasm by immunostaining with the use of laser microscopy and by subcellular fractionation.
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PMID:The rck/p54 candidate proto-oncogene product is a 54-kilodalton D-E-A-D box protein differentially expressed in human and mouse tissues. 761 84


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