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)

Primary cardiac rhabdomyosarcoma is rare and its extension to the mitral valve even rarer. We report a case of left atrial rhabdomyosarcoma involving the mitral valve. The patient was a 62-year-old man who complained of recurrent pre-syncopal episodes, dyspnoea often sudden in onset, asthenia and major weight loss (10 kg in one month). 2-D echocardiography revealed a 4.9 cm2 wide mass attached to the atrial side of the anterior mitral leaflet and to the adjacent inferior interatrial septum, where it seemed to have origin. CT scan and scintigraphy revealed bone, kidney and spleen metastases. The patient underwent emergency cardiac surgery because of increasing pre-syncopal and dyspnoeic episodes due to obstruction by the intracardiac mass. At surgery a tumor was found infiltrating the left atrial wall, the interatrial septum, the mitral anulus and the anterior mitral leaflet up to its tip. Invasion of mitral anulus did not allow mitral valve replacement, so that an excision of the intracardiac mass was performed as extensively as possible. Histology revealed a rhabdomyosarcoma. A post-operative chemotherapy cycle had to be stopped due to onset of atrial fibrillation and dyspnoea. 2-D echo monitoring revealed rapid new growth of the tumor across the basal portion of mitral valve leaflet to the atrioventricular orifice. After several episodes of increasing dyspnoea, the patient had a pulmonary oedema and died.
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PMID:[Primary cardiac rhabdomyosarcoma involving the mitral valve]. 129 26

There were 79 cases of cardiac tumors seen from 1957 to July 1988. 49 (62.0%) of them were benign and 30 (38.0%) malignant. All the 49 benign tumors except 2 were surgically excised and found to be myxoma. Of them, 18 patients were male and 31 female. 85.7% of the tumors were located in the left atrium, 12.2% in the right atrium and 2.0% in the left ventricle. Palpitation, dyspnea, chest oppression, fever, episodes of syncope and hemiplegia, cough, diastolic and systolic murmurs at the apical or tricuspid area were the common symptoms and signs. Atrial fibrillation was found only in 2 cases. Echocardiographic findings were diagnostic while ECG and X-ray findings were nonspecific. Four patients died after operation. Of the 30 cases of malignant tumors, 15 were secondary tumors metastasized mainly from the lung or mediastinal malignancies. Of 11 primary tumor cases (7 males and 4 females), 3 were malignant lymphoma, 2 mesothelioma of pericardium, 2 malignant myxoma, 1 angiosarcoma, 1 leiomyosarcoma, 1 fibrosarcoma and 1 rhabdomyosarcoma. Another 4 cases were not studied histopathologically. The clinical manifestations, ECG and X-ray findings of the 11 primary tumors were nonspecific but echocardiography was helpful to the diagnosis. Six patients were operated on and 1 died during hospitalization.
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PMID:Tumors of the heart. An analysis of 79 cases. 159 77

Rhabdomyosarcoma of the larynx is a malignant disease rarely seen in children. We present five cases (3 males, 2 females) observed among 126 patients with rhabdomyosarcoma of the head and neck (excluding the orbit) treated in the Institut Gustave-Roussy between 1955 and 1981. The patients were aged between 5 1/2 years and 13 1/2 years at the time of diagnosis. The presenting symptoms were dysphonia and/or dyspnea; tracheotomy was required in two cases. All five patients received chemotherapy consisting of vincristine, cyclophosphamide, actinomycin D + procarbazine or doxorubicin. Four patients also received radiotherapy (45 Gy during 5 weeks). Laryngectomy was not performed. Treatment sequelae included regional hypoplasia, huskiness, and subclinical thyroid insufficiency; two patients developed a benign thyroid adenoma. One patient presented a bilateral carotid stenosis subsequent to radiotherapy performed on "wide fields" portal designed to provided prophylactic treatment on uninvolved lymph nodes. All the patients are alive and relapse-free with a follow-up of between 13 and 17 years. This small series of RMS of the larynx shows that patients can be cured without laryngectomy, although long-term sequelae associated with radiotherapy are observed.
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PMID:Rhabdomyosarcoma of the larynx in children: a series of five patients treated in the Institut Gustave Roussy (Villejuif, France). 201 Oct 95

An autopsy case of a huge tumor in the right thorax is reported. A 71-year-old man complained of dyspnea and died after three months of hospitalization. The tumor, consisting of a circumscribed mass measuring 24 X 24 X 8 cm and weight 3.7 kg, originated from the right diaphragm and had no metastasis. Histologically, the tumor comprised multiple mesenchymal elements of fibrosarcoma, rhabdomyosarcoma, osteosarcoma and chondrosarcoma, and there was no distinct border between these different components. This tumor fulfills the criterion of malignant mesenchymoma (Stout). The histogenesis of the tumor is discussed with a survey of literature.
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PMID:[An autopsy case of malignant mesenchymoma of the diaphragm]. 403 73

Life-threatening airway obstruction from large mediastinal masses in children poses a difficult diagnostic and therapeutic dilemma, requiring the close coordination of a pediatric surgeon, anesthesiologist, radiologist, and oncologist. To focus on this problem, the anesthetic and surgical management of 50 consecutive children with mediastinal masses treated between 1978 and 1984 were reviewed. Thirty children presented with respiratory symptoms; nine had life-threatening respiratory compromise with dyspnea, orthopnea, and stridor. Thirteen of these symptomatic children had marked compression of the trachea and/or mainstem bronchi on radiographic studies. The tracheal cross-sectional area which was measured by computed tomography was decreased by 35% to 93% of the normal tracheal dimensions in these children. Nonresectable malignant neoplasms including lymphoma, Hodgkin's disease, rhabdomyosarcoma, and neuroblastoma were the eventual diagnoses in 10 of these patients. The other 3 patients were less than 4 years old and had benign lesions. General anesthesia was judged to be prohibitively risky in 5 of 13 patients. The diagnosis was established by node or needle biopsy under local anesthesia, and general anesthesia was deferred until the compromised airway was alleviated by radiation and chemotherapy. General anesthesia with endotracheal intubation was administered to 8 patients, 5 of whom developed total airway obstruction. Using a variety of maneuvers, ventilation was reestablished in all 5 patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Life-threatening airway obstruction as a complication to the management of mediastinal masses in children. 408 8

A case of rhabdomyosarcoma of the urinary bladder in an 11-month-old boy is presented. The chief complaint was complete urinary retention and histological examination showed embryonal rhabdomyosarcoma of the bladder. Initially, he was treated with vincristine, actinomycin-D and radiation therapy. This therapy was not effective, and he was next treated with vincristine, bleomycin, and cis-DDP. This therapy was significantly effective, and the tumor became non-palpable on physical examination. After six courses he was discharged and maintenance chemotherapy was continued until the tumor relapsed 1 year later. Finally he died of dyspnea due to diffuse pulmonary metastases approximately 2 years after the first diagnosis. We herein discuss several points which affect the prognosis and the usefulness of chemotherapy, especially combination therapy with vincristine, bleomycin, and cis-DDP for recurrent cases.
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PMID:[Rhabdomyosarcoma of the bladder in a child: report of a case]. 620 68

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

Thirty-five patients (10 men and 25 women) with a preoperative diagnosis of cardiac myxoma have undergone cardiac surgery since 1964 at the University of Louvain. The mean age of the patients was 49 (range 20-75) years. The most commonly encountered symptoms were: dyspnoea 49%; thoracic pain 26%; cough and peripheral embolism 17% each; stroke and preoperative atrial fibrillation 14% each; flutter 11%; expectoration, acute pulmonary oedema, syncope and transient ischaemic attack 6% each; and pulmonary embolism 3%. The different locations were: left atrium 66%; right atrium 26%; both atria 3%; right ventricle 3%: and retrohepatic vena cavae 3%. Septal implantation was found in 66%. Histological examination confirmed 28 myxomas but three 'tumours' were thrombi, two haemangiomas, one rhabdomyosarcoma and one liposarcoma. The follow-up has now reached 2829 months with an average of 81 months per patient (range 0-342 months). Three patients died early (9%) and there were four late deaths (11%). No cases were familial. Surgical resection is the correct treatment for cardiac myxomas and gives good long-term results.
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PMID:Cardiac myxoma. 807 15

A 63-year-old black man of Caribbean origin, seropositive for human T-cell lymphoma virus type I (HTLV-I), presented with a 4-week history of progressive dyspnea, and was found to have a tumor of the anterior mediastinum. Incisional biopsy revealed a malignant neoplasm with a solid pattern of glycogen-rich clear cells. Diffuse expression of vimentin was observed, whereas only rare cells were immunoreactive for muscle-specific actin and desmin. Ultrastructure revealed a large amount of mono-particulate glycogen in most cells and features of rhabdomyogenic differentiation in occasional cells. The autopsy revealed a 23 x 14-cm (1,345 g), soft and white mediastinal neoplasm bulging in the right thorax with right pleural metastases. HTLV-1 proviral genome was not detected within tumor cells by polymerase chain reaction. This rhabdomyosarcoma is best classified as the solid subtype of the alveolar variant, with an unusually large amount of cytoplasmic glycogen. Clear cell rhabdomyosarcoma could be potentially confused with more commonly encountered clear cell tumors, particularly in the mediastinum.
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PMID:Glycogen-rich clear cell rhabdomyosarcoma of the mediastinum. Potential diagnostic pitfall. 811 99

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


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