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 case of a 66-year-old male with rhabdomyosarcoma of the right ventricle who presented first with a pleuritic chest pain and later developed congestive heart failure with A-V block is described. The total period of the disease was about 9 months. At autopsy the right ventricle and wall were found to be replaced by tumor tissue which was diagnosed histologically as rhabdomyosarcoma of the alveolar type. We described one case and 52 rhabdomyosarcomas of the heart from the literature; the ages ranged from 3 months to 92 years with 29 men and 23 women (unknown 1). The primary lesions were right ventricle 29%, right atrium 26%, left atrium 24%, left ventricle 6%, and others 15%.
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PMID:Rhabdomyosarcoma of the right ventricle. 713 97

Thirty-four cases of primary cardiac tumors, consisted of 11 men and 23 women, aged between 26 and 80 years have been operated on in our institution. A total of 30 myxomas and four malignant tumors were resected. The clinical presentations included congestive heart failure in 25 patients, tachyarrhythmia in 6, chest pain in 4, and embolism in 10. All 30 myxoma lesions were completely resected, although none of the malignant tumors could be resected completely because of invasion of the tumor to the surrounding tissues. Four patients with malignant tumors died of tumor recurrence within 9 months after operation. Histological diagnosis was rhabdomyosarcoma in two patients, round cell sarcoma in one and malignant fibrous histiocytoma in one. All patients with myxoma survived operation. Follow-up was completed in 28 patients (range 1 month to 15 years, mean 5.5 years): Twenty-four were in New York Heart Association Class I, and the remaining two in Class II. Actuarial survival rate was 89% at 15 years after operation. No recurrent myxoma have been identified clinically or by echocardiography. For malignant primary tumors, more effective adjuvant therapy with aggressive resection will be inevitable to improve long-term prognosis.
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PMID:[Surgical treatment of primary cardiac tumors]. 838 35

We report a case of cardiac rhabdomyosarcoma the initial clinical features of which were pericardial effusion, clinical symptoms of congestive heart failure and probable pulmonary thromboembolism, in which echocardiography constituted the first approach to the diagnosis of cardiac tumor and MRI confirmed it, precisely delimiting the tumoral extension and possible infiltration of pericardiac structures. A brief literature review of this entity is given, the MRI findings obtained in our case are described, and we discuss the advantages and limitations of this technique as compared with other alternatives of image diagnosis.
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PMID:Cardiac rhabdomyosarcoma: diagnosis by MR imaging. 1035 75

Urotensin-II (U-II) was identified as the natural ligand of the G protein-coupled receptor GPR14, which has been correspondingly renamed Urotensin-II receptor (U2R). The tissue distribution of U2R and the pharmacological effects of U-II suggest a novel neurohormonal system with potent cardiovascular effects. We here report the human rhabdomyosarcoma cell line TE-671 as the first natural and endogenous source of functional U2R in an immortalized cell line. In TE-671 cells, U-II stimulated extracellular signal regulated kinase phosphorylation and increased c-fos mRNA expression. Furthermore, we demonstrate that the expression of U2R mRNA and functional U-II high affinity binding sites are serum-responsive and that they are specifically up-regulated by interferon gamma (IFNgamma). We propose that IFNgamma contributes to the previously observed increase of U2R density in the heart tissue of congestive heart failure (CHF) patients and we suggest that U2R up-regulation, as a consequence of an inflammatory response, could lead to a clinical worsening of this disease.
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PMID:The expression of urotensin II receptor (U2R) is up-regulated by interferon-gamma. 1475 94

A rare case of left atrial rhabdomyosarcoma is presented in a patient with symptoms of congestive heart failure mimicking a hemodynamically obstructive mitral stenosis and secondary pulmonary hypertension. Although the diagnosis of a cardiac neoplasm is often difficult, it should be suspected in any patient with idiopathic heart failure refractory to conventional therapy, or with systemic or pulmonary emboli without an obvious source. The possibility of a "tumor plop" should always be considered during auscultation of a diastolic click. If clinically suspected, echocardiography will usually establish the diagnosis and allow follow-up for recurrences. If the tumor is benign, cardiac surgery will be curative and, if malignant, chemotherapy or radiotherapy should be considered.
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PMID:Left atrial rhabdomyosarcoma mimicking mitral valve stenosis. 1522 49

The present report describes the case of a 61-year-old woman with malignant fibrous histiocytoma of the left atrium originating from the left atrial free wall, operated on in emergency for a suspected large left atrial myxoma that, at the echo scan, was consistently protruding through the left atrioventricular orifice at each diastole and was almost completely occluding the left ventricular inflow, causing signs of congestive heart failure and severe dyspnea. Surgery was performed as radically as possible, but the histological examination of the specimen revealed the exact diagnosis of the neoplasm. About 75% of primary tumors are benign and 75% of these are atrial myxomas. The malignant tumors consist of various sarcomas: myxosarcoma, liposarcoma, angiosarcoma, fibrosarcoma, leiomyosarcoma, osteosarcoma, synovial sarcoma, rhabdomyosarcoma, undifferentiated sarcoma, reticulum cell sarcoma, neurofibrosarcoma, and malignant fibrous histiocytoma. The long-term results for sarcomas are very poor and there are few survivors after several months from surgery due to the extent of local spread and invasion or because of the frequent distant metastases. Malignant fibrous histiocytoma constitutes about 2% of all cardiac malignancies, which might grow within several localized areas, occasionally in the heart. Echocardiography represents the best examination procedure for both diagnosis and follow-up of patients with cardiac tumors.
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PMID:[A rare cardiac tumor: the malignant fibrous histiocytoma. Description of a case]. 1555 23

Although it is recognized that aggressive soft tissue sarcomas may give rise to cardiac metastases, these manifestations are usually late and clinically silent, being the prevailing finding in exceptional cases. This report describes the occurrence of a massive cardiac metastasis at diagnosis of leg rhabdomyosarcoma in a middle-age adult. This manifestation was the cause of rapidly progressive congestive heart failure and, together with the unusual occurrence of autoimmune thrombocytopenia, led to difficult patient care with a significantly negative influence on the outcome.
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PMID:Unusual manifestations of disseminated neoplasia at presentation: right-sided heart failure due to a massive cardiac metastasis and autoimmune thrombocytopenia in pleomorphic rhabdomyosarcoma of the adult. 1646 13


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