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 56-year-old man was admitted to our hospital with right chest pain. Chest X-ray, CT scan and MRI revealed a chest wall tumor and enlarged mediastinal lymph nodes. Percutaneous lung biopsy was performed, and the pathological diagnosis of pleomorphic rhabdomyosarcoma was obtained. The only significant abnormal laboratory finding was elevation of serum NSE (24.5 ng/ml). Although chemotherapy (VAC-ADM) and radiation therapy were performed, the patient died about 7 months after admission. To our knowledge, only 17 cases of chest wall rhabdomyosarcoma have been reported in Japan.
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PMID:[A case of chest wall rhabdomyosarcoma]. 175 9

Malignant primary cardiac tumors have uniformly been associated with poor long-term survival. We reviewed data on 11 patients with such tumors seen between 1956 and 1986. There were five men and six women, with a mean age at presentation of 44 years. Nine (82 percent) of 11 patients presented initially with respiratory symptoms, and 45 percent (five) presented with chest pain. No patient was asymptomatic on initial presentation. Forty-five percent (five) of the patients had elevated jugular venous pressure or a systolic murmur. Cardiomegaly was present in eight (73 percent) of 11 patients. Nine of ten patients had a hemoglobin level less than 12 g/100 ml, and 30 percent (3/10) presented with thrombocytosis. Seventy percent (eight) of the patients underwent surgical biopsy, and in 30 percent (three), surgical excision of the tumor was attempted. Angiosarcoma was the most frequent type of tumor (four patients), followed by malignant fibrous histiocytoma (three patients), mesothelioma (two patients), rhabdomyosarcoma (one patient), and primary lymphoma (one patient). Long-term follow-up data reveals that ten (91 percent) of 11 patients had died, with a mean duration from diagnosis until death of 9.7 months. Newer pathologic techniques for identification of sarcomas, aggressive surgical resection, and advanced chemotherapy for certain subgroups may contribute to better survival for patients with these tumors in the future. In addition, cardiac transplantation may play an important role in the future for those patients with localized disease.
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PMID:Malignant primary cardiac tumors. The Cleveland Clinic experience, 1956 to 1986. 366 1

A case of polypoid sarcoma of the pulmonary trunk is described in an 80-year-old woman who had a 21-year history of episodic chest pain and hemoptysis. Ultrastructural examination revealed Z bands, characteristic of rhabdomyosarcoma, and leptomeric organelles. This is the 60th reported case of sarcoma of the pulmonary trunk. Symptoms in reported cases were variable, usually caused by tumor emboli to the lungs or by right ventricular outflow obstruction. Angiography was diagnostic. The tumor was characteristically polypoid, often multicentric, and by definition was fixed to the pulmonary trunk or valves. Non-differentiated sarcoma (often uniquely pleomorphic) was found in 37% of patients, leiomyosarcoma (once confirmed by electron microscopy) in 17%, myxosarcoma in 13%, elements of rhabdomyosarcoma in 8%, fibrosarcoma in 8%, elements of chondrosarcoma in 11%, and 5% were malignant mesenchymomas. The authors suggest that these tumors originate from the undifferentiated tissues of the bulbus cordis and propose the name myenchymoma for the tumor.
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PMID:Polypoid sarcoma of the pulmonary trunk: analysis of the literature and report of a case with leptomeric organelles and ultrastructural features of rhabdomyosarcoma. 738 74

Pulmonary artery rhabdomyosarcomas are rare lesions, with a small number of cases reported in the literature. We report a case of pulmonary artery rhabdomyosarcoma that arose in the right pulmonary artery. The patient presented with cough, chest pain, and hemoptysis and was found to have a sarcoma arising in the right pulmonary artery and extending distally within the pulmonary vasculature into the right lung. The gross pathologic features, microscopic appearance, immunohistochemical profile, and computed tomographic and electron microscopic findings are presented. The site of origin and differential diagnosis of these lesions are discussed.
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PMID:Pleomorphic rhabdomyosarcoma arising in association with the right pulmonary artery. 797 18

Four cases are presented of primary anterior mediastinal tumors in young adults that were characterized by solid, infiltrative lesions showing histologic and immunohistochemical features of rhabdomyoblastic differentiation. The patients were three men and one woman between 19 and 27 years of age (mean age, 23 years). All patients presented with symptoms referable to their tumors, including cough, chest pain, dyspnea, and left-sided pleural effusion. Grossly and radiographically, the lesions were characterized by their solid, infiltrative appearance. Histologically, two cases corresponded to the solid variant of alveolar rhabdomyosarcoma, one case was an embryonal rhabdomyosarcoma with a predominant spindle cell component, and the remaining case showed the features of a pleomorphic rhabdomyosarcoma. No glandular, epithelial, or other component could be identified in any of the tumors on extensive sampling. Immunohistochemical studies showed positive staining of the tumor cells with actin, desmin, and vimentin antibodies, with focal positivity for myoglobin in three cases and focal positive staining with S-100 protein in one case. Stains for low and high molecular weight keratin, carcinoembryonic antigen, alpha-fetoprotein, human chorionic gonadotropin, placental alkaline phosphatase, leukocyte-common antigen, and neuron-specific enolase were negative. All patients experienced rapid recurrence and metastases within the first 6 months after diagnosis. Three patients died within this period due to their tumors; the fourth patient has been lost to follow-up. Pure primary rhabdomyosarcomas of the anterior mediastinum are highly aggressive neoplasms that should be distinguished from germ cell, teratomatous, or carcinosarcomatous tumors with a focal rhabdomyoblastic component.
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PMID:Rhabdomyosarcomas of the anterior mediastinum: report of four cases unassociated with germ cell, teratomatous, or thymic carcinomatous components. 816 67

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

Rhabdomyosarcoma (RMS) rarely occurs in adult and intra thoracic activity. This is a report on a case we experienced of adult RMS, which was subjected to tumorectomy and lobectomy. The patient was a 23 years old male, who first consulted a hospital in his neighborhood due to anterior chest pain suffered since May 1995. Since the examination showed an abnormal shadow in right anterior mediastinum, the patient was referred to our department. By biopsy under CT, the patient was diagnosed as having embryonal type of RMS and underwent surgery. The outcome was good and the patient is currently in the course of chemotherapy using IRS (Inter-group Rhabdomyosarcoma study)-III.
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PMID:[Report on a case of adult rhabdomyosarcoma subjected to tumorectomy and lobectomy]. 915 36

We report on a case of embryonal rhabdomyosarcoma in an 11-yr-old boy investigated for mild chest pain after trauma. Chest radiography showed a massive right pleural effusion. Cytological analysis of the pleural fluid demonstrated the presence of malignant small undifferentiated cells. The rhabdomyoblastic nature of the cells was confirmed by positive immunostains of HHf35 actin, desmin, and skeletal muscle myosin; histological examination of a core biopsy confirmed the diagnosis of rhabdomyosarcoma. Computed tomography and gallium scan revealed the presence of an extensive anterior and lower chest wall mass involving the mediastinum, as well as retroperitoneal lymphadenopathy. Massive pleural effusion is a frequent presentation in malignant disease, but is rare in rhabdomyosarcoma. The immunochemical stain studies performed on cytological smears in this case proved to be very useful for making the definitive and accurate diagnosis. Diagn. Cytopathol. 1999;21:125-128.
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PMID:Rhabdomyosarcoma in a child with massive pleural effusion: cytological diagnosis from pleural fluid. 1042 51

We report a rare case of primary thoracic rhabdomyosarcoma in a girl who was referred with acute chest pain, hacking cough, and wheezing. A chest X-ray revealed a complete opacity of the right hemithorax. Ultrasound revealed a right-sided pleural effusion and a solid mass above the liver dome, suggesting a neoplastic disease, which quickly led to further specific examination. Use of CT and MRI together with bone scintigraphy completed the investigation. The biopsy specimen showed a pattern of alveolar rhabdomyosarcoma. This case was reported to emphasize the role of US in the evaluation of a child with hemithorax opacity.
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PMID:Integrated diagnostic imaging of primary thoracic rhabdomyosarcoma. 1128 60

We report a case of rhabdomyosarcoma which occurred in a mediastinal teratoma in a 44-year-old man. Presentation symptoms were chest pain, hoarseness and a cough. Diagnosis was fortuitous, performed by the histological and immunohistochemical study of a mediastinal tumour biopsy specimen that showed embryonal carcinoma and yolk sac tumour components associated with the rhabdomyosarcoma. After cisplatin-based chemotherapy (bleomycin-etoposide-cisplatin), surgical resection of the residual mediastinal tumour was performed. Histological and immunohistochemical study of this tumour confirmed the presence of mature teratoma and embryonal rhabdomyosarcoma. Evolution was marked by a local extension of the mediastinal tumour, occurrence of multiple metastases and bone marrow involvement. The patient died 8 months after diagnosis despite chemotherapy and radiotherapy. A review of the literature reveals that the development of rhabdomyosarcoma in primary mediastinal teratomas is unusual in adults. The diagnostic, therapeutic and prognostic implications of such an association are reviewed.
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PMID:Rhabdomyosarcoma arising in mediastinal teratoma in an adult man: a case report. 1188 12


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