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)

Two hundred soft tissue sarcomas, accrued consecutively over a 4-year period, were examined by light and electronmicroscopy and by routine immunohistochemistry. The commonest tumour type was malignant fibrous histiocytoma. Fibrosarcoma, composed only of fibroblasts, was diagnosed in only one case; three others, composed also of myofibroblasts, could be regarded as fibrosarcomas or myofibrosarcomas. Immunohistochemistry was of most value in the diagnosis of rhabdomyosarcoma, leiomyosarcoma, malignant peripheral nerve sheath tumour of Schwann cell type and epithelioid sarcoma. Electronmicroscopy was of most use for the diagnosis of malignant peripheral nerve sheath tumour of perineurial cell type and marker-negative monophasic synovial sarcoma, and for confirming malignant fibrous histiocytoma. Fifteen of 19 marker-negative spindle cell tumours (79%) were diagnosable by electronmicroscopy. A combination of techniques resulted in a specific diagnosis in 193 cases (96.5%). The routine use of electronmicroscopy in sarcoma diagnosis can improve accuracy of diagnosis, establish the true frequency of marker-positivity for each ultrastructurally confirmed tumour type and minimise the number of unclassifiable cases.
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PMID:The value of electronmicroscopy and immunohistochemistry in the diagnosis of soft tissue sarcomas: a study of 200 cases. 169 15

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

Five patients with nonlymphomatous sarcoma metastatic to the brain are presented and the literature reviewed. In 50 patients, brain metastasis was verified pathologically and some case data were available, including the five reported here. In five patients, there was pathologic verification but no case data. Thirty-nine other patients were found without such verification. Reportedly, most types of sarcomas are able to metastasize to the brain, and are represented in these 94 patients. Fibrosarcoma and alveolar soft-part sarcoma tumors metastasize to the brain without regard to treatment technique. There may be a group of tumors, including malignant fibrous histiocytoma, rhabdomyosarcoma, and perhaps leiomyosarcoma and osteosarcoma, in which the incidence of brain metastases has increased with improved sarcoma chemotherapy (CT). In this group particularly, but also in alveolar soft-part sarcoma and others, the presence of lung metastases may increase the probability of brain metastasis occurring subsequently.
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PMID:Sarcoma metastatic to the brain. 327 83

Ninety-four soft-tissue sarcomas of head and neck occurring among 10,700 malignancies, seen during the period 1953-1980 in the head-and-neck surgical department, were studied with reference to age, sex, ethnic origin, site, histological type and survival. The objective was to review our own experience with sarcomas in these sites seen in the Department of Pathology, A.C. Camargo Hospital. The predominant site was the head. Fibrosarcoma was the most frequent type in adults and rhabdomyosarcoma, located exclusively in the head, was the most frequent in children.
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PMID:Soft-tissue sarcomas of the head and neck in adults and children: experience at a single institution with a review of literature. 327 33

From 1964 to 1978, 313 patients, 192 males and 121 females, with soft tissue sarcoma underwent surgery in our hospital. All patients have been followed up for over 5 years. One hundred ninety patients (60.7%) had recurrence of the tumor after previous surgery. The tumor was located in the head in 55 cases, in the trunk in 136, and in the extremities in 122 cases. Regional lymph node metastases occurred in 23 patients (7.4%). Fibrosarcoma, neurofibrosarcoma, and rhabdomyosarcoma were the types most commonly encountered in this series. The 5-year survival rate, according to the extent of surgical resection for 117 patients with local resection, was 47.9%; for 75 patients with wide local resection, 62.7%; for 43 patients with amputation, 18.6%; and for 78 patients with local resection combined with irradiation, 48.7%. For the whole series, the 5- and 10-year survival rates were 47.6% and 35.8%, respectively. The 5-year survival rates of stages I, II, III, and IV (TNM classification) were 60%, 46.9%, 40%, and 22.2%, respectively.
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PMID:Surgical management of soft tissue sarcomas, with an analysis of 313 cases. 335 24

347 children with all types of soft tissue sarcoma (STS) have been entered between 1981 and April 1986 into the CWS-81 study. Excluding 229 patients with rhabdomyosarcoma, 118 patients with other STS were analysed in the histological subgroups, separately. 33 synovial sarcomas (SyS), 15 undifferentiated sarcomas (US) and 13 extrasceletal Ewing's sarcoma (EES) were treated with a multimodale trial including chemotherapy and radiotherapy after initial not-mutilating resection or biopsy only, Relapse-free survival rate by Kaplan-Meier was 66% in SyS, 48% in EES and 38% in US, respectively. Patients treated according to the guidelines of the CWS-protocol showed better prognosis than patients with major protocol violations or patients with recurrence when entered into the trial. In SyS only 2 of the 7 patients with recurrence developed metastases. The tumor response rate within 7-9 weeks chemotherapy exclusively given was 71% in patients with stage III and IV. Non-responding tumors to chemotherapy had bad prognosis, irrespective of histological subtype or further treatment. EES and US developed earlier recurrence and more often metastases than SyS or rhabdomyosarcomas. The administered VACA-chemotherapy seems not to be very effective in EES and US. In all other varieties of STS recurrence at the primary site assumed relatively greater importance. Chemotherapy and radiation were not administered in all patients. Fibrosarcoma (FS) was diagnosed in 13 patients. Six of these were entered in the trial at the time of local recurrence and 4 of these 6 relapsed again locally, one with metastasis concurrently.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Results of the treatment of non-rhabdomyosarcomatous soft tissue neoplasms within the scope of the CWS 81 study]. 362 23

Primary retroperitoneal tumors are rare (0.05-0.2% of all tumors), often malignant and characterized by a poor and non-specific symptomatology and by a late diagnosis. Complete resection is possible in only a few patients, while recurrence is very common. The records of 29 adult patients who underwent operative treatment at Surgical Oncology Institute-University of Cagliari between November 1973 and June 1992 were reviewed; 9 were males, 20 females, median age 46.4 years (range 12-82). There were 4 benign tumors (13.8%) and 25 malignant (86.2%). Fibrosarcoma (9 cases, 31%) and liposarcoma (3 cases, 10.3%) were the most frequent histologic types. There were also two fibroleiomyomas, leiomyosarcomas, malignant fibrous histiocytomas and neuroblastomas, one case of fibroma, neurofibroma, rhabdomyosarcoma and schwannoma. Five sarcoma were not otherwise specified. Abdominal mass (25 cases, 86.2%), flank or abdominal pain (15 cases, 51.7%) and weight loss (8 cases, 27.6%) were most common symptoms; change in bowel habit and constipation (6 cases, 20.7%), fever (5 cases, 17.2%), urinary disorders (4 cases, 13.8%) nausea and vomiting (2 cases, 6.7%) were less common. Diagnosis was made by ultrasonography, computed tomography and traditional radiographic studies. median interval between first symptoms and diagnosis was 11 months. Complete surgical resection was possible in only 13 cases (46.4%): 10 of the 25 malignant tumors (40%) and 3 of the 4 benign tumors (75%).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Primary retroperitoneal tumors. Our experience]. 751 58

Malignancy occurring during the neonatal period (defined as the first 28 days of life) is over 3 times the incidence of other pediatric age groups. Of all neoplasia occurring in infants, benign and malignant, 25% are soft tissue tumors. Differentiating the benign lesions from the 15% that are malignant can be difficult. This article discusses the epidemiology, differential diagnosis, evaluation, and treatment of infants with soft tissue sarcomas. Fibrosarcoma and rhabdomyosarcoma are also discussed at length. The authors review other rare tumors as well. The impact on diagnosis of molecular techniques is included when appropriate. A multidisciplinary team approach for treatment of these infants is recommended.
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PMID:Soft tissue sarcomas of infancy. 1047 43

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

This research was carried out on soft tissue sarcomas processed in the Department of Pathology of University of Ilorin Teaching Hospital over a period of 18 years (1979-1996). Tumours in which pathognomonic features necessary for diagnosis were present on haematoxylin and eosin sections were diagnosed accordingly while special stains were employed to highlight characteristic features of some other tumours. Also a panel of special stains was performed on the poorly differentiated tumours. The three commonest tumours were fibrosarcoma, rhabdomyosarcoma and liposarcoma in decreasing order of frequency. Fibrosarcoma and leiomyosarcoma were commonest in middle age while rhabdomyosarcoma was commonest in childhood and early adult life. There was a male preponderance of the tumours with a male to female ratio of 1.3:1. With the use of special stains, typical diagnostic histological characteristics were found in 3 out of the 13 tumours initially diagnosed as poorly differentiated thereby reducing their number to 10. Plans are underway to do immunohistochemical studies and chromosomal analysis on the tumours through collaborative studies.
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PMID:Histopathological and histochemical patterns of soft tissue sarcomas in Ilorin, Nigeria. 1503 86


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