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)

A 70 year old woman was admitted for right ventricular failure and cyanosis of recent onset. Echocardiography showed a very large, homogenous, immobile, smooth-contoured mass filling the right atrium. Right atrial pressures were raised but the other intracardiac pressures were normal at catheterisation. Right heart angiography confirmed the voluminous right atrial mass and dilatation of the hepatic veins and showed early opacification of the left heart chambers. A right-to-left shunt was confirmed by oximetry which showed significant desaturation of the blood in the left atrium and ventricle (saturation 78% in the left ventricle). The tumour was also documented by a thoracic CT scan. At surgery, a very large, malignant right atrial tumour was resected which histological examination showed to be an angiosarcoma. The interatrial septum seemed to be intact: there was no true atrial septal defect but a persistent foramen ovale was found. After resection of the tumour the right atrium was reconstructed. The initial postoperative period was uncomplicated but the patient died nine months later of metastatic disease particularly affecting the liver and brain. The clinical presentation of malignant cardiac tumour is very variable but a right-to-left interatrial shunt through a patent foramen ovale has only been reported previously in 2 cases of primary malignant tumours (a rhabdomyosarcoma and an angiosarcoma) and in 1 case of a secondary cardiac metastasis.
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PMID:[Right-left shunt caused by sarcoma of the right atrium]. 211 45

Intermediate-sized filaments (IF) are among the most insoluble intracellular protein polymer structures. We have analyzed the small amounts of soluble vimentin, an IF protein, present in cytosol fractions obtained from lysis of cultured cells [rat RVF-SM cells, simian virus 40-transformed human fibroblasts, and human rhabdomyosarcoma (RD line) cells]. The molecular form of this soluble vimentin was determined by sucrose density gradient centrifugation, using vimentin-specific antibodies for subsequent ELISA and immunoblotting analyses. The majority of the soluble vimentin appeared in a distinct form indistinguishable in its sedimentation behavior from reconstituted tetrameric subunits of purified vimentin arrested at low ionic strength. The tetrameric coiled-coil nature of the soluble form of vimentin was indicated by the digestion pattern with chymotrypsin and by chemical crosslinking with copper-1,10-phenanthroline and dimethylsuberimidate. The competence of this soluble vimentin to assemble into IF at higher salt concentrations was demonstrated by electron microscopy. Pulse-chase experiments showed that the soluble form was not an exclusively posttranslational intermediate. We propose that in the living cell a small pool of a distinct soluble tetrameric form of vimentin exists which may exchange with polymeric IF vimentin.
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PMID:Identification of a distinct soluble subunit of an intermediate filament protein: tetrameric vimentin from living cells. 386 6

A 49-year-old man was admitted because of dyspnea and generalized edema. CT scan pulmonary scintigram and cardiac catheter examination revealed pulmonary arterial obstruction due to a tumor associated with right heart failure. The tumor was extirpated as much as possible by a pulmonary arteriotomy under cardiopulmonary bypass. Postoperatively, right ventricular pressure decreased to about 40 mmHg while PO2 increased to the normal level. Histological examination of the tumor revealed rhabdomyosarcoma arising from the pulmonary artery. Chemotherapy consisting of a single course of CDDP and adriamycin was administered.
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PMID:[A case report of primary pulmonary artery sarcoma]. 756 19

A seven-year-old male labrador retriever presented in right heart failure with weak femoral pulses, and pleural, abdominal and mild pericardial effusion. No diagnosis could be established initially. Two days later, the dog developed severe pericardial effusion causing cardiac tamponade. A tumour in the right ventricular wall was visualised on ultrasonographic examination. An exploratory thoracotomy was performed and biopsies of the mass submitted for histopathological examination. A diagnosis of rhabdomyosarcoma arising from the myocardium was established. Cardiac rhabdomyosarcoma has been reported in only two dogs. Neither report was associated with pericardial effusion.
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PMID:Pericardial effusion in a dog with rhabdomyosarcoma in the right ventricular wall. 873 9