Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

100 cases of undifferentiated soft tissue sarcomas were studied using cell markers by immunoperoxidase technique with DAB as substrate. Vimentin, Desmin, Myoglobin, Actin, Keratin, Epithelial Membrane Antigen, S-100 protein, F VIII R Ag, A1 Antitrypsin, A1 Antichymotrypsin, Collagen-IV and UEA-1 lectin were used as markers. Fibrosarcoma was consistently positive for Vimentin and Collagen-IV. The undifferentiated Rhabdomyosarcoma showed strong and consistent positivity for Vimentin Actin and Myoglobin. Desmin positivity was the hallmark of leiomyosarcoma, whereas the malignant schwannomas were identified by their S-100 positivity. This marker along with A1AT and A1ACT reactivity was of great use in the identification of malignant fibrous histiocytoma. Angiosarcoma/malignant haemangioendothelioma could be identified with great accuracy by their strong positivity for F VIII RAg and UEA-1 lectin. Other miscellaneous sarcomas also could be identified by their specific reactivity to the markers used. We consider immunohistochemistry to be an important and essential adjunct to routine stains in the diagnosis of undifferentiated soft tissue sarcomas.
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PMID:Cell marker studies in undifferentiated soft tissue sarcoma. 175 49

We report 2 cases of angiosarcoma of the prostate gland. The patients were 42 and 60 years old, which is in sharp contrast to the presentation of rhabdomyosarcoma, which occurs primarily in children. Immunoperoxidase staining for factor VIII associated antigen was positive in both cases, and was particularly useful in confirming the diagnosis in 1 case that was otherwise poorly differentiated. One patient is alive without evidence of recurrence 2 years after surgical excision, while the other presented with unresectable tumor and died 6 months after presentation. Angiosarcoma represents less than 2 per cent of sarcomas of the prostate but it should be considered in the differential diagnosis of poorly differentiated sarcomas, particularly those occurring in adults.
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PMID:Angiosarcoma of the prostate: report of 2 cases and review of the literature. 351 Dec 94

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

Primary cardiac malignancies are rare tumors that are difficult to diagnose clinically. Different primary cardiac malignancies may have different clinical, morphologic, and radiologic features and intracardiac locations. Angiosarcoma is the most common primary cardiac malignancy. It tends to occur in the right atrium and involve the pericardium. Because of its tendency to hemorrhage, angiosarcoma often demonstrates areas of increased signal intensity with T1-weighted sequences. Undifferentiated sarcomas typically occur in the left atrium and have variable epidemiologic and radiologic features. Rhabdomyosarcoma is the most common primary cardiac malignancy in children and is more likely than other primary cardiac sarcomas to involve the valves. Primary cardiac osteogenic sarcoma almost always occurs in the left atrium and frequently demonstrates calcification. Certain features (eg, broad base of attachment, origin at a site other than the atrial septum) help differentiate this tumor from left atrial myxoma. Leiomyosarcoma favors the left atrium and tends to invade the pulmonary veins and mitral valve. Fibrosarcoma also tends to occur in the left atrium and is often necrotic. Liposarcoma is very rare and usually manifests as a large, infiltrating mass. Foci of macroscopic fat are occasionally seen. Primary cardiac lymphoma occurs more commonly in immunocompromised patients, frequently involves the pericardium, and, unlike other primary cardiac malignancies, may respond to chemotherapy. The advent of cross-sectional imaging has allowed earlier detection of primary cardiac malignancies as well as more accurate diagnosis and characterization.
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PMID:CT and MR imaging of primary cardiac malignancies. 1055 66

Primary sarcomas of the thorax are rare. The diagnosis is established only after sarcomalike primary lung malignancies and metastatic disease have been excluded. Primary sarcomas of the thorax are classified according to their histologic features and constitute a large group of tumors that occur in the lung, mediastinum, pleura, and chest wall. Angiosarcoma, leiomyosarcoma, rhabdomyosarcoma, and mesothelioma (sarcomatoid variant) are the most common primary intrathoracic sarcomas. Ewing sarcoma, primitive neuroectodermal tumor, chondrosarcoma, malignant fibrous histiocytoma, osteosarcoma, synovial sarcoma, and fibrosarcoma usually arise in the chest wall. Although primary thoracic sarcomas commonly manifest as large, heterogeneous masses, they have a wide spectrum of radiologic manifestations, including solitary pulmonary nodules, central endobronchial tumors, and intraluminal masses within the pulmonary arteries. The different histologic types of sarcomas are frequently indistinguishable at radiologic analysis. However, differences in clinical presentation and the location of the tumor, as well as morphologic features such as calcification within the mass and rib involvement, can be useful in suggesting the appropriate diagnosis. For example, a large rib mass in a child with fever and malaise indicates a Ewing sarcoma, a mass with a calcified matrix is likely a chondrosarcoma or osteosarcoma, and a pulmonary artery mass is likely a leiomyosarcoma.
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PMID:Primary thoracic sarcomas. 1200 91

Cardiovascular magnetic resonance imaging (CMRI) is the reference noninvasive imaging technique for assessment and characterization of a suspected cardiac or juxta-cardiac mass. The multiplanar assessment of anatomy, tissue composition, and functional impact afforded by CMRI allows for early differentiation between a nonneoplastic mass and a tumor mass, be it benign or malignant. Malignant cardiac tumors have a poor prognosis; however, early detection and characterization confer some survival advantage, enabling early instigation of chemotherapy and/or consideration of a surgical debulking procedure. Cardiac metastases are far more common than primary tumors and are an important consideration in patients with disseminated disease. Angiosarcoma accounts for the majority of primary malignant lesions. Less common primary malignant cardiac tumors include sarcomas with myofibroblastic differentiation, lymphoma, rhabdomyosarcoma, pericardial mesothelioma, and pericardial synovial sarcoma. A number of benign masses and normal anatomical variants can cause confusion to the inexperienced observer and must be recognized to avoid unnecessary intervention. These include intracardiac thrombus, bronchogenic and pericardial cysts, and anatomical structures, such as the Crista terminalis and moderator band.
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PMID:Magnetic resonance imaging of cardiac tumors: part 2, malignant tumors and tumor-like conditions. 2161 79

Objectives To investigate clinicopathologic and treatment factors associated with survival in adult head and neck sarcomas in the National Cancer Database (NCDB). To analyze whether treatment settings and therapies received influence survival outcomes and to compare trends in utilization via an aggregated national data set. Study Design Prospectively gathered data. Setting NCDB. Subjects and Methods The study comprised a total of 6944 adult patients treated for a head and neck sarcoma from January 2004 to December 2013. Overall survival (OS) was the primary outcome. Results Increased age and tumor size, nodal involvement, and poorly differentiated histology had significantly reduced OS ( P < .001). Angiosarcoma, malignant nerve sheath tumor, malignant fibrous histiocytoma, osteosarcoma, and rhabdomyosarcoma histologic subtypes had significantly reduced OS, while liposarcoma, chondrosarcoma, and chordoma had improved OS ( P < .001). Utilization of surgical therapy was associated with improved OS, while positive surgical margins were associated with treatment at a community-based cancer program and had reduced OS ( P < .001). On multivariate analysis, treatment with radiation and/or chemotherapy was not significantly associated with OS; however, primary treatment with definitive chemoradiotherapy had significantly reduced OS. Patients treated at academic/research cancer programs (n = 3874) had significantly improved 5- and 10-year OS (65% and 54%, respectively) when compared with patients treated at community-based cancer programs (n = 3027; 49% and 29%; P < .001). The percentage utilization of these programs (56% vs 44%) did not change over the study period. Conclusion For adult head and neck sarcomas, treatment at an academic/research cancer program was associated with improved survival; however, despite increasing medical specialization, the percentage utilization of these programs for this rare tumor remains constant.
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PMID:Academic Facility Utilization and Survival Outcomes in Adult Head and Neck Sarcomas: An NCDB Analysis. 2966 Oct 49