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 binding sites for human interferon-alpha (IFN-alpha) have been characterized on human lymphoblastoid, melanoma, rhabdomyosarcoma, and cervical carcinoma cells. Crosslinking of iodinated-recombinant DNA-derived IFN-alpha-Con1, an analog of the known IFN-alpha subtypes, to the cell surface with disuccinimidyl suberate yielded four IFN-receptor complexes of 118, 138, 159, and 260 kD on all cell lines that specifically bind IFN-alpha. Since IFN-alpha exists in solution as monomers, dimers, and trimers, and the three lower molecular weight IFN-alpha-receptor complexes differ by the molecular weight of IFN-alpha (20 kD), this suggests that the human IFN-alpha receptor of 100 kD binds more than one molecule of IFN-alpha. The higher molecular weight complex of 260 kD may result from dimerization of the receptor. None of these complexes was observed in a rhabdomyosarcoma subclone that does not specifically bind IFN-alpha. Pretreatment of cells with trypsin abolished the formation of these complexes. Pretreatment of cells with neuraminidase did not reduce IFN-alpha binding, but increased the electrophoretic mobility of all four IFN-alpha-receptor complexes. Other glycosidases (i.e., mannosidase, beta-galactosidase, and endoglycosidase F) had no effects on IFN-alpha binding or mobility of complexes. Thus, although the IFN-alpha receptor is a glycoprotein, the glycosylated portion is apparently not part of the IFN-alpha-binding domain. The formation of IFN-alpha-receptor complexes is independent of the duration of incubation with IFN (from 5 min to 1 h at 15 degrees C).(ABSTRACT TRUNCATED AT 250 WORDS)
J Interferon Res 1988 Dec
PMID:Characterization of interferon-alpha binding sites on human cell lines. 246 92

Nutritional rehabilitation of malnourished children with growth arrest is generally associated with a catch-up of growth but the occurrence of this compensatory phenomenon in adulthood is not well recognized. We investigated a case of maturation and growth acceleration secondary to nutritional intervention in a 22-y-old patient. After treatment for a rhabdomyosarcoma of the bladder at age 7 y, the patient developed severe malabsorption secondary to radiation enteritis and short bowel syndrome. As a result of profound malnutrition, growth and maturation were severely impaired. Initiation of home total parenteral nutrition at age 22 y led to an increase in height, substantial weight gain, advancement of bone age, and sexual maturation evidenced by appearance of secondary sex characteristics and normalization of hormone concentrations. The development of signs of puberty and a growth spurt appearing at this late age clearly show the potential for maturation and growth once malnutrition is corrected.
Am J Clin Nutr 1989 Dec
PMID:TPN-induced catch-up of growth in a 22-y-old male with radiation enteritis. 251 3

The present study reveals the dynamic distribution of membrane laminin receptors induced by laminin binding in a rat rhabdomyosarcoma cell line RMS S4. The treatment of the cells with soluble laminin did not modify cell adhesion to laminin-coated substrates in in vitro attachment assays. Fluorescent labeling of membrane-bound laminin revealed that occupied receptors were induced to cluster and cap. New free membrane binding sites were made evident after capping of bound laminin by a double labeling technique. Cytochalasin D (CD) treatment prevented the capping process. The adhesion of CD-treated cells to laminin-coated substrates was inhibited by cell preincubation with soluble laminin. Cycloheximide treatment had no effect on the ability of RMS S4 cells to adhere to adsorbed laminin after preincubation in the presence of soluble laminin. These results taken as a whole suggest that free receptors may arise from an intracellular pool that could be maintained by membrane receptor recycling. Since capping and motility seem related events, migration of RMS S4 cells on laminin was studied in the agarose drop assay. Immobilized laminin stimulated basic cell motility by more than 200%. E8 laminin fragment retained partially the motility stimulating property of laminin while P1 pepsinic fragment had no effect. The presence of constantly available receptors at the cell surface could be determinant in the ability of cells to migrate on laminin substrates.
Exp Cell Res 1989 Dec
PMID:Laminin-induced capping and receptor expression at cell surface in a rat rhabdomyosarcoma cell line: involvement in cell adhesion and migration on laminin substrates. 253 49

From 1980 to 1987, 162 consecutive children with soft tissue and osseous sarcoma were reviewed to determine the frequency and types of neurologic complications seen. Neurologic complications occurred in 43 of 162 (26.5%) patients. Children with poorly differentiated sarcomas and rhabdomyosarcoma were more likely to have neurologic complications, which occurred in 39% of patients at risk. The types of complications seen included: metastatic spinal cord compression (11%); symptomatic peripheral neuropathy (10%); intracranial metastatic disease (7.5%); seizures (6%); and acute and chronic methotrexate-related neurologic dysfunction (2.5%). Spinal cord compression frequently occurred early in disease whereas brain metastases was almost always a late finding. Symptomatic peripheral neuropathy occurred primarily in children with rhabdomyosarcoma and Ewing's sarcoma. The advent of increasingly successful therapies for children with sarcoma and the frequency of severe neurologic complications indicate that a heightened level of surveillance for neurologic compromise is required.
Cancer 1989 Dec 15
PMID:Neurologic complications in children with soft tissue and osseous sarcoma. 255 41

Patterns of intermediate filament expression of 10 malignant fibrous histiocytomas (MFHs) were immunohistochemically evaluated using acetone-fixed frozen sections. Seven cases represented the storiform-pleomorphic subtype, 2 were of myxoid type, and 1 was of giant-cell type. All cases had been studied by electron microscopy, and no proof for the diagnoses of liposarcoma, rhabdomyosarcoma, and leiomyosarcoma could be obtained. All tumors showed prominent vimentin immunoreactivity in the tumor cells. Cytokeratin-positive neoplastic cells were found in 2 cases, and in the majority of tumor cells in 1 of these. The 68k neurofilament-positive cells were found in 2 cases. Desmin was not found beyond doubt in the neoplastic cells in any cases, and all cases were negative for glial fibrillary acidic protein. The expression of several types of intermediate filament indicates divergent differentiation properties in MFH and may suggest the heterogeneity of this entity, but more cases should be studied to elaborate any possible consistent patterns of intermediate filament expression in different types of MFH. The expression of multiple types of intermediate filament proteins in MFH can alternatively signify random activation of the corresponding genes in the primitive tumor cells. The complex patterns of intermediate filament proteins in morphologically defined MFHs should be taken into account in the practical immunohistologic analysis of tumors.
Arch Pathol Lab Med 1989 Dec
PMID:Malignant fibrous histiocytoma. Heterogeneous patterns of intermediate filament proteins by immunohistochemistry. 255 88

A melphalan-resistant human rhabdomyosarcoma xenograft, TE-671 MR, was established in athymic mice by serial melphalan treatment of the parent xenograft, TE-671, at the 10% lethal dosage (LD10); significant resistance was evident after ten passages of the tumor. TE-671 MR demonstrated a doubling time of 3.5 days and a latency period to 1000-mm3 tumors of 27.5 days. The glutathione level of TE-671 MR was 2.36 mumol/g tumor, wet weight, 2-fold higher than the parent line. The glutathione S-transferase activity of TE-671 MR was 117.8 mumol/min/mg protein, essentially unchanged from the parent line. Although TE-671 MR demonstrated cross-resistance to vincristine, dot blot analysis did not reveal an elevated expression of mdr1 mRNA in the resistant line. TE-671 MR demonstrated a 9.7-day growth delay following treatment with melphalan at the LD10 (compared to 20.9 days for the parent line). Treatment with L-buthionine-SR-sulfoximine (BSO) resulted in increased sensitivity to melphalan subsequently administered at 50% of the LD10 (melphalan alone, growth delays of 3.7 and 4.6 days in duplicate trials; melphalan plus BSO, growth delays of 7.2 and 9.8 days). Sensitivity to melphalan equal to that of the parent line TE-671 was not achieved, however. Treatment with BSO did not result in significantly enhanced sensitivity to subsequently administered vincristine (50% of the LD10) (vincristine alone, growth delays of 6.8 and 6.9 days in duplicate trials; vincristine plus BSO, growth delays of 10.9 and 7.5 days). These results suggest that generation of melphalan resistance may be associated with development of cross-resistance to vincristine; this resistance may be associated with (although not necessarily mediated by) glutathione elevation; this resistance may be partially overcome by BSO-mediated depletion of glutathione.
Cancer Res 1989 Dec 15
PMID:Establishment of a melphalan-resistant rhabdomyosarcoma xenograft with cross-resistance to vincristine and enhanced sensitivity following buthionine sulfoximine-mediated glutathione depletion. 258 34

Three clonal subpopulations (A, B, C) isolated from the same rhabdomyosarcoma of the rat were tested and compared for their susceptibility to differentiation induction using retinoic acid (RA), dimethylformamide (DMF), and N-monomethylformamide (NMF). These subpopulations differ in that a block to spontaneous differentiation is imposed at different stages which are characteristic for each subpopulation. Whereas tumor cell proliferation was significantly inhibited (P less than 0.001) in all three subpopulations, the effects of RA, DMF, and NMF on tumor cell differentiation were strikingly heterogeneous. The response was most marked in subpopulation C, as evidenced by a significant increase in the number of terminally differentiated myotube-like giant cells (P less than 0.001) and in biochemical differentiation, as indicated by the creatine kinase activity (P less than 0.05). Between 5% (DMF and NMF) and 30% (RA) of the mononuclear cells in subpopulation C exhibited thick and thin myofilaments, which were never observed in the mononuclear cells of the control. In contrast, subpopulation A and B responded to RA, DMF, and NMF quite heterogeneously with an increase in biochemical differentiation, whereas terminally differentiated myotube-like giant cells were never observed. These results demonstrate that the therapeutic potential of differentiation induction in malignant tumors may be impaired by tumor heterogeneity.
Cancer Res 1989 Dec 15
PMID:Heterogeneous response to differentiation induction in different clonal subpopulations of a rat rhabdomyosarcoma cell line (BA-HAN-1). 258 54

The correction of marked blepharoptosis in patients with severe or potential keratopathy will worsen the keratopathy and possibly lead to the complications of corneal ulceration and endophthalmitis. The conjunctival flap--cosmetic shell--ptosis procedure is well suited to this difficult management problem. Patients are initially treated with a conjunctival flap to protect their cornea. Subsequently they are fit with a cosmetic shell, and finally they undergo surgery to correct their ptosis. This three-stage procedure has produced excellent cosmetic and functional results in two patients, one of whom had ptosis and severe radiation-induced keratopathy following the treatment of a rhabdomyosarcoma; the other patient had severe ptosis associated with lack of corneal sensation and orbicularis function following removal of a cerebral meningioma.
Arch Ophthalmol 1989 Dec
PMID:Conjunctival flap-cosmetic shell-ptosis procedure. Treatment of blepharoptosis in severe keratopathy. 259 74

A unique case of embryonal rhabdomyosarcoma arising at the left pleura of a 7-year-old Japanese girl is reported. The present case was characterized by persistent pleural effusion, and the malignant cells incidentally found in it were the first diagnostic clue. The tumor showed a rare growth pattern involving diffuse thickening of the parietal pleura. Biopsy of the thickened parietal pleura upon thoracotomy revealed embryonal rhabdomyosarcoma largely composed of immature mesenchymal cells. Immunohistochemical demonstration of creatinine phosphokinase-MM was most helpful among several types of immunostain for the histopathological diagnosis. Ultrastructurally, thin filaments with primitive Z bands could be seen in some tumor cells. Intensive clinical examination revealed only diffuse thickening of the parietal pleura, which was reduced by chemotherapy. This is the first documented case of rhabdomyosarcoma arising at the pleura. Previous reports of rhabdomyosarcoma arising at unusual sites are reviewed and the histogenesis of this tumor is briefly discussed.
Acta Pathol Jpn 1989 Dec
PMID:Diffuse pleural rhabdomyosarcoma with persistent pleural effusion. 262 6

A 14-year-old girl with a large left ovarian mass underwent excision of the tumor which on histologic examination revealed dysgerminoma mixed with elements of rhabdomyosarcoma. A subsequent staging laparotomy revealed a solitary 1-cm nodule along the pelvic wall. This was composed of rhabdomyosarcoma. Review of literature revealed that soft tissue sarcomas of several types may on occasion be seen in association with gonadal and extragonadal mixed germ cell tumors or in spermatocytic seminoma of the testis. However, no previously published report of a sarcoma arising in a pure dysgerminoma was found in the literature.
Cancer 1989 Dec 01
PMID:Dysgerminoma of the ovary with rhabdomyosarcoma. Report of a case. 268 53


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