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)

Acute cold haemagglutinin disease, most commonly associated with underlying mycoplasma infection, is rare in children. A 3-year-old girl who developed this auto-immune disease under intensive cytotoxic treatment for rhabdomyosarcoma is presented. Clinically, a livedo reticularis skin pattern upon exposure to cold which was reversible at room temperature and a spontaneous red cell agglutination of blood samples in vitro led to the diagnosis. Together with bronchopneumonia the girl developed hyper-IgM, high antibody titres against Mycoplasma pneumoniae, as well as high titres of cold agglutinins. Laboratory signs of mild intravascular haemolysis were found. Positive direct antiglobulin test resulted from coating of red cells with C3d and C4. Three different antibodies were identified in serum: nonspecific cold agglutinins without complement activation, anti-I specific cold agglutinins with complement activation, as well as a weak biphasic Donath-Landsteiner haemolysin. Under antibiotic treatment and a short course of predisolone the clinical course was mild.
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PMID:Cold haemagglutinin disease complicating Mycoplasma pneumoniae infection in a child under cytotoxic cancer treatment. 162 72

Rhabdomyosarcoma of the biliary tree is a rare cause of biliary tract obstruction in childhood. A 3-year-old child is reported here after presenting with obstructive jaundice secondary to an embryonal rhabdomyosarcoma of the ampulla of Vater. He underwent pancreaticoduodenectomy followed by adjuvant chemotherapy and irradiation. He is now well and free of disease 5 years following treatment. This child appears to be the first long-term survivor who has required pancreaticoduodenal resection for this lesion.
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PMID:Embryonal rhabdomyosarcoma of the ampulla of Vater with long-term survival following pancreaticoduodenectomy. 228 99

A rapidly enlarging periocular mass in a child obligates the treating physician to rule out a malignancy, especially a rhabdomyosarcoma. A 3-year-old girl presented with a 5-day history of a rapidly growing episcleral mass superonasal to the globe, adjacent to the superior rectus muscle insertion. The lesion was locally excised. A sarcoma could not be excluded on frozen sections. Permanent sections and electron microscopy revealed nodular fasciitis, a benign lesion with a pseudosarcomatous histologic appearance. No recurrence has been noted at 9 months follow-up. Nodular fasciitis is a benign proliferation which should be considered in the differential diagnosis of a rapidly enlarging subconjunctival or orbital mass in a child.
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PMID:Nodular fasciitis presenting as a rapidly enlarging episcleral mass in a 3-year-old. 236 27

A 3-year-old girl had a large exophytic mass protruding from the nares, of seven months' duration. Routine microscopic studies demonstrated a tumor composed of solid sheets of small cells, with scattered foci lining alveola-like spaces. Immunohistochemical studies demonstrated the presence of desmin and myoglobin, and the absence of prekeratin, neuron-specific enolase, and leukocyte common antigen. These observations are consistent with the diagnosis of alveolar rhabdomyosarcoma.
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PMID:Alveolar rhabdomyosarcoma arising in the nasal cavity of a 3-year-old child. 323 83

A 3 year old boy presented with a presumably incurable form of rhabdomyosarcoma. Conventional treatment for advanced stage rhabdomyosarcoma (chemotherapy plus radiotherapy) was not used and intensive chemotherapy alone was given, resulting in an apparent cure. Our treatment rationale is discussed.
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PMID:Unexpected cure in metastatic rhabdomyosarcoma. 401 57

A 3 1/2-year-old boy had a neoplasm of mixed mesenchymal and neuroepithelial origin in the cerebellar vermis. The tumor was composed of rhabdomyosarcoma and medulloblastoma. There was additional differentiation within the neuroepithelium, including neoplastic neurons, neuroblasts, astroglia, oligodendroglia, ependymal cells as well as foci of glioblastomatous transformation. It is suggested that this combined tumor arose from the neural crest or combined neural crest and neural tube. The rhabdomyosarcoma could have arisen from the mesenchymal portion of the neural crest, and the medulloblastoma from the neuroectodermal part of the neural crest or from the neural tube itself. The two tumor types formed a neoplasm of mixed mesenchymal and neuroepithelial origin.
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PMID:Cerebellar neoplasm of mixed mesenchymal and neuroepithelial origin. Case report. 686 2

Cell populations of eight experimental tumors (murine and rat rhabdomyosarcomas induced with 20-methylcholantrene, transplantable murine rhabdomyosarcoma A-7, and transplantable rat lymphosarcoma) have been selected for the affinity of their cells to lung tissue. The level of the affinity was measured as the number of lung nodules per 100 000 tumor cells injected intravenously. A 3-4-fold selection appeared to be non-effective, whereas 10-fold or more prolonged selections resulted in a gradual enhancement of the affinity of tumor cells to lung tissue. Thus, the transplantable murine and rat rhabdomyosarcomas were obtained with an increased capacity of their cells of yielding lung nodules. The affinity of rat rhabdomyosarcoma was 200-300 times higher after 40 steps of selection compared to the initial tumor affinity. With the rhabdomyosarcoma of CC57W mice, the affinity increased by 5 times after 20 steps of selection. Using our technique of selection (without an in vitro cultivation), the capacity of cells of persisting in lung tissue and yielding lung nodules looks likely as a quantitative character with a rather low heritability. It has been concluded that in cell populations of tumors examined there are only a few genetic population variations in cell capacity of making non-random metastases.
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PMID:[Selection for affinity to lung tissue in cell populations of experimental neoplasms]. 689 67

A 3-year-old boy with inflammatory granulation tissue in the ear canal and typanometric evidence of middle-ear effusion was examined. Subsequently, rhabdomyosarcoma of the middle ear was diagnosed. The insidious, deceptive presentation of this common pediatric tumor in an uncommon location is discussed with indication that early diagnosis and aggressive multimodal antitumor therapy seems to be improving the hitherto gloomy prognosis for these highly lethal middle-ear-neoplasms.
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PMID:Rhabdomyosarcoma of the middle ear: a wolf in sheep's clothing. 737 37

ZT3, isolated from a murine muscle cell cDNA library by a low-stringency hybridization, encodes a zinc finger domain containing factor with a transcript of 5.0 kb. A 3' 2.5 kb partial nucleotide sequence contains an ORF of 1.5 kb where 17 canonical C2H2 zinc finger domains organized in tandem were identified. It maps on mouse chromosome 11, close to two mutations which affect skeletal formation. ZT3 expression depends upon differentiation of myogenic cells in culture, since it is upregulated with myogenin and inhibited in scr-transfected C2C12 cells. ZT3 is not expressed in NIH3T3 or C3H10T1/2 fibroblasts, but is induced when fibroblasts are myogenically converted by transfection with the muscle regulatory genes (MRFs). Its expression is also upregulated in the rhabdomyosarcoma cell line RD induced to myogenic differentiation by TPA treatment. In postimplantation embryos, ZT3 is diffusely expressed but higher expression is detectable in the neural tube and encephalic vesicles, in the somites and, at a high level, in the limb buds as they form. During further development ZT3 is expressed in many tissues of neuroectodermal and mesodermal origin, but its expression decreases during fetal development and in the adult it is restricted to skeletal and cardiac muscle and to spleen. This pattern of expression suggests a possible role played by ZT3 in differentiating skeletal muscle. Its expression in other tissues is compatible with the suggestion that members of this class of DNA-binding factors play different roles during post-implantation development and in the adult life.
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PMID:Differentiation dependent expression in muscle cells of ZT3, a novel zinc finger factor differentially expressed in embryonic and adult tissues. 880 10

A 3-year-old boy presented with a single episode of gross hematuria and no history of previous urinary tract disorder. Imaging studies revealed a large complex polypoid filling defect in the bladder lumen. Several attempts at transurethral biopsy and cytological examination of the urine revealed clumps of benign epithelial cells, but suspicion of a malignant neoplasm such as rhabdomyosarcoma remained high and the lesion was resected. The specimen measured 15 cm, had a narrow zone of attachment to the bladder mucosa, and was grossly botryoid. Changes typical of cystitis cystica et glandularis were present at and near all surfaces. Myxoid stroma contained scattered benign fibroblasts, myofibroblasts, and smooth muscle cells. Inexplicably, one of two karyotyped stromal cells demonstrated a translocation usually associated with rhabdomyosarcoma. This child is well without evidence of bladder abnormality 1.5 years after surgery.
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PMID:Giant botryoid fibroepithelial polyp of bladder with myofibroblastic stroma and cystitis cystica et glandularis. 1257 20


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