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 clinical profiles of four patients with primary vulvar sarcomas are presented. Two patients had leiomyosarcoma in association with pregnancy, a third patient had a leiomyosarcoma with epithelioid elements, and a fourth had alveolar rhabdomyosarcoma. The natural history of the disease in the three leiomyosarcomas (including the case with epithelioid elements) was characterized by an indolent protracted course and frequent local recurrence, followed by distant fatal metastases. The patient with alveolar rhabdomyosarcoma is alive-with-disease. Surgery, chemotherapy, and radiotherapy achieved palliation rather than cure. Pregnancy did not seem to influence the prognosis.
Gynecol Oncol 1992 Sep
PMID:Vulvar sarcoma: a report of four cases. 152 19

We present here the results of the largest study of childhood cancer and ethnic group in Britain, based on 7,658 children treated at paediatric oncology centres throughout the country. Incidence rates could not be calculated and so relative frequencies were analysed by the log-linear modelling method of Kaldor et al. (1990) with allowance made for regional variations in the ages and diagnostic groups of the children included in the study. Children of Asian (Indian sub-continent) and West Indian ethnic origin had similar patterns of incidence for acute lymphoblastic leukaemia to White Caucasians. There was a significant excess of Hodgkin's disease among Asian children compared with Caucasians with an estimated relative risk (RR) of 2.09; this excess was greatest in the 0-4 age group (RR = 6.67). There were significant deficits of Wilms' tumour and rhabdomyosarcoma among Asian children, each with a frequency around half that among Caucasians, whereas West Indians had a significant excess of Wilms' tumour (RR = 2.55). Asian and West Indian children each had a non-significant twofold RR for unilateral retinoblastoma. The results suggest that the incidence of childhood acute lymphoblastic leukaemia is associated with environmental determinants in the country of residence which are most likely to relate to lifestyle factors. The occurrence of retinoblastoma, Wilms' tumour and Hodgkin's disease in early childhood is apparently related more to ethnicity than to geographical location and may reflect genetic factors or environmental exposures specific to the lifestyle of particular ethnic groups.
Br J Cancer 1991 Sep
PMID:Childhood cancer and ethnic group in Britain: a United Kingdom children's Cancer Study Group (UKCCSG) study. 165 82

Human immunodeficiency viruses (HIV) isolated from infected individuals show tremendous genetic and biologic diversity. To delineate the genetic determinants underlying specific biologic characteristics, such as rate of replication, cytopathic effects, and ability to infect macrophages and T4 lymphoid cells, generation of hybrid HIV using viruses which exhibit distinct biologic features is essential. To develop methods for generating hybrid HIV, we constructed truncated HIV proviral DNA plasmids. Upon digestion with restriction enzymes, these plasmid DNAs were cotransfected into human rhabdomyosarcoma cells to generate hybrid HIV. The hybrid HIVs derived by this method were infectious upon transmission to both phytohemagglutinin-stimulated peripheral blood lymphocytes and established human leukemic T-cell lines. The virus derived from molecular clone pHXB2 (HIVHTLV-III) productively infected CEMx174 cells. On the other hand, molecular clone pARV (HIVSF2)-derived virus did not show productive infection of CEMx174 cells when used as a cell-free virus. The hybrid HIV containing the 3' end of the genome from pARV and the 5' end of the genome from pHXB2 was effective in infecting CEMx174 cells, but the converse hybrid containing 5' pARV and 3' pHXB2 was not effective in infecting CEMx174 cells. These results suggest that differences in the genes outside of env and nef play a role in the ability of the virus to infect a certain cell type. The intracellular ligation method should be useful in the analysis of related and unrelated HIV-1 isolates with common restriction enzyme cleavage sites.
J Virol 1991 Sep
PMID:Generation of hybrid human immunodeficiency virus utilizing the cotransfection method and analysis of cellular tropism. 167 38

Fifteen patients with biopsy-proven mediastinal germ cell tumors treated with platinum-based chemotherapy were reviewed. They had a period of 4 to 6 weeks between the onset of symptoms and diagnosis. Four patients had sarcomatous elements in their tumor in association with common germ cell histologies. The sarcomatous components consisted of one angiosarcoma, one rhabdomyosarcoma, and two cases with mixed angiosarcoma and rhabdomyosarcoma. All patients with sarcomatous elements died; the median survival for these patients was 9 months. In contrast, six (54%) of the patients who did not have sarcomatous elements in their tumor are long-term disease-free survivors 5 to 8 years after diagnosis. The occurrence of sarcomatous elements in a mediastinal germ cell tumor is a poor prognostic sign, and therapy should be oriented to include drugs and regimens that may be effective against sarcoma.
Cancer 1990 Sep 15
PMID:Poor prognosis of mediastinal germ cell cancers containing sarcomatous components. 169 14

We characterized cDNA clones specific for the extracellular matrix glycoprotein undulin. Two sets of cDNA clones were isolated from a human placental lambda gt11 expression library and from a rhabdomyosarcoma cell line encoding two partially identical carboxyl-terminal polypeptides of 843 (Un1) and 443 (Un2) amino acids suggesting differential splicing of a single gene transcript. Northern blot analysis of human rhabdomyosarcoma cell poly (A) RNA with cDNA specific for Un1 identified transcripts of approximately 4.2, 6.5, and 8.5 kilobases, whereas a probe specific for Un2 detected a single mRNA of approximately 5 kilobases. Since a monoclonal antibody that is reactive with a sequence encoded by Un1 and not by Un2 detects the bands considered characteristic for undulin in Western blots, the mRNAs related to Un1 may code for the major part of the undulin molecule. The protein sequences deduced from Un1 and Un2 reveal an amino-terminal differentially spliced von Willebrand factor A domain, characteristic of proteins that interact with interstitial collagens, which is linked to fibronectin-like type III homology units by a unique sequence of 57 amino acids. Whereas Un2 encodes two complete and one incomplete type III homologies followed by a unique acidic carboxyl-terminal domain of 118 amino acids, Un1 codes for seven complete and one truncated type III homologies, followed by a short proline-rich carboxyl-terminal segment of 23 amino acids. Considering the 298 amino acids occurring in identical segments, the 989 different amino acid positions deduced from clones Un1 and Un2 represent an estimated 40% of the overall undulin sequence. In the context of 1) rotary shadowing electron microscopy data showing undulin as a structure composed of nodules that are interconnected by flexible rods of varying size, 2) the presence of three major bands of Mr 270,000, 190,000, and 180,000 in sodium dodecyl sulfate-polyacrylamide gel electrophoresis, with 3) common antigenic epitopes and similar peptide maps (Schuppan, D., Cantaluppi, M.C., Becker, J., Veit, A., Bunte, T., Troyer, D., Schuppan, F., Schmid, M., Ackermann, R., and Hahn, E.G. (1990) J. Biol. Chem. 265, 8823-8832), our finding of differentially spliced type III homology units, as found in tenascin and fibronectin, suggests that undulin is another member of the fibronectin-tenascin family of extracellular matrix glycoproteins. Furthermore, as in fibronectin and tenascin, undulin bears an additional subset of interactive domains tailored to specific structural and functional roles in development and differentiation.
J Biol Chem 1991 Sep 15
PMID:Undulin is a novel member of the fibronectin-tenascin family of extracellular matrix glycoproteins. 171 29

At necropsy, a 7-year-old Holstein cow, clinically diagnosed via rectal palpation as having malignant abdominal neoplasia was found to have many metastatic tumors of various sizes in the abdominal and thoracic cavities and in the liver, lung, kidney, adrenal, uterus, and lymph nodes. These tumors were examined by histologic and immunohistochemical methods using anti-human myoglobin, anti-bovine myoglobin, and anti-desmin sera. Tumors were diagnosed as undifferentiated alveolar rhabdomyosarcomas. The tumors in the abdominal and thoracic cavities differed histologically from the metastases in organs. The former tumors consisted of only undifferentiated cells, most of which did not stain positively for desmin or myoglobin. The metastatic tumors contained a few rhabdomyoblastic cells that stained positively for desmin and myoglobin. Tumors tissues from the cow were transplanted and propagated through six passages in athymic nude mice. After one passage, the transplanted tumor histologically resembled those from the abdominal serosa and consisted of cells with scanty cytoplasm with an alveolar arrangement. Cells from this tumor stained positively for desmin and negatively for myoglobin. After two passages through nude mice, tumor cells were of two distinct histologic types: those cells with scanty cytoplasm and elongated cells with plentiful eosinophilic cytoplasm. Both of these cells stained positively for both desmin and myoglobin. After six passages, cross striations were detected in neoplastic cells by electron microscopy. These findings illustrate that a highly undifferentiated bovine rhabdomyosarcoma, in which most of the cells in the original tumor lacked desmin and myoglobin, became better differentiated and stained positively for desmin and myoglobin after serial transplantation in nude mice.
Vet Pathol 1991 Sep
PMID:Bovine undifferentiated alveolar rhabdomyosarcoma and its differentiation in xenotransplanted tumors. 175 Jan 69

A case of testicular mixed germ cell tumor with teratoma in a 36-year-old Japanese male is reported. Histologically, it was strongly suggested that the rhabdomyosarcoma had originated from the mesenchymal element of the teratoma. A review of the literature revealed six definitive and two possible previous cases of testicular rhabdomyosarcoma with teratoma, either with or without other germ cell malignancies. However, the present case associated with seminoma as a germ cell tumor is the first of its kind to be reported. In contrast to the poor prognosis observed in the previous cases, the present patient remains free of disease 3 years and 3 months after orchiectomy, chemotherapy and irradiation.
Acta Pathol Jpn 1991 Sep
PMID:A testicular teratoma with rhabdomyosarcoma and seminoma. 177 72

We have been evaluating the role of all-trans-retinoic acid (RA) in the differentiation and growth of human rhabdomyosarcoma (RMS) cell lines. Treatment of both embryonal (RD) and alveolar (RH30) human RMS cell lines with all-trans-RA resulted in a dose-dependent inhibition of cell growth with a maximal inhibition of 92 and 66%, respectively, at 5 x 10(-6) M. When 13-cis-RA was used under identical experimental conditions, maximal growth inhibition was 41 and 37%, respectively. This stereo-specific growth inhibition was not associated with morphological or biochemical evidence of myogenic differentiation. Furthermore, all-trans-RA demonstrated no evidence of competition with binding of insulin-like growth factor II (IGF-II), an autocrine growth factor in RMS, to its membrane receptor as evaluated by an [125I]IGF-I-receptor-binding assay. Attempts to rescue all-trans-RA growth-inhibited RMS cells with exogenous IGF-II resulted in no increase in growth compared to cells treated with all-trans-RA alone. We conclude that RA inhibits the growth of human RMS cell lines in a dose-dependent, stereo-specific manner, is not associated with differentiation, and does not appear to be directly related to IGF-II.
Cancer Res 1991 Sep 15
PMID:All-trans-retinoic acid inhibits the growth of human rhabdomyosarcoma cell lines. 189 78

Cell adhesive activity of two animal lectins, frog (Rana catesbeiana) S-type 14K lectin and echinoidin (a C-type lectin from sea urchin plasma), was studied with human rhabdomyosarcoma (RD) cells. RD cells attached to and spread on plastic plates coated with each lectin. Cell adhesion by the frog lectin was completely inhibited by the addition of lactose or asialofetuin glycopeptide. Echinoidin-induced cell adhesion was only inhibited by peptide GRGDS. Since echinoidin is known to contain an RGD-sequence, our results clearly indicate that this sequence is active as the cell adhesive signal. These results suggest that some of the animal lectins may function as a cell adhesive molecule rather than using the carbohydrate-recognition mechanism.
FEBS Lett 1991 Sep 09
PMID:Cell adhesive activity of two animal lectins through different recognition mechanisms. 191 38

Infectious molecular clones of the human immunodeficiency virus (HIV) have been very important tools for the analysis of regulatory gene functions and the study of differential cell tropism. We have cloned and characterized a proviral sequence of HIVmn from mn strain infected H9 cells. This clone, called KP1, was found to be infectious for different cell lines and human peripheral blood lymphocytes (PBL). KP1 proviral DNA was detected in HUT-78 cells and human PBL by polymerase chain reaction (PCR) analysis after infection of these cells with cell-free supernatants from KP1 transfected human rhabdomyosarcoma (RD) cells. To the best of our knowledge, this is the first report of an infectious molecular clone of HIVmn which is a representative of one of the most prevalent strains of HIV-1 in North America and Europe. Biologically active clones of a broadly antigenic strain such as HIVmn will be extremely useful in therapeutic approaches for AIDS.
Biochem Biophys Res Commun 1991 Sep 30
PMID:Isolation and characterization of an infectious molecular clone of the MN strain of HIV-1. 193 Jan 83


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