Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report a unique case of primary myxoid liposarcoma of the floor of the mouth, in which well-differentiated rhabdomyoblasts were present. The tumor was incompletely excised. The patient is free of recurrence or metastasis following a course of radiotherapy. Heterologous components found in a liposarcoma include leiomyosarcoma, rhabdomyosarcoma, chondrosarcoma, osteosarcoma, and angiosarcoma. These elements have been described mainly in dedifferentiated liposarcomas situated in the retroperitoneum. To our knowledge, there has been only one report of a liposarcoma with rhabdomyosarcomatous differentiation occurring in the absence of a dedifferentiated component. The clinical implications of the presence of heterologous components without dedifferentiation are unclear.
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PMID:Primary myxoid liposarcoma with rhabdomyoblastic differentiation. 970 38

The role and value of chemotherapy for soft tissue sarcomas remain unclear. Seventeen patients with pulmonary metastatic soft tissue sarcomas underwent treatment with chemotherapy, and the clinical efficacy and prognosis were studied. Six patients with synovial sarcomas, 4 with malignant fibrous histiocytomas, 4 with neurosarcomas, and the remaining 3 patients with leiomyosarcoma, extraskeletal osteosarcoma, and extraskeletal chondrosarcoma, were studied. Cases with small round cell sarcomas were excluded. The chemotherapy agents were ifosfamide in 10 cases, combination of ifosfamide and adriamycin in 5 cases, or cisplatin and adriamycin in 2 cases. Of the 17 patients, seven had partial responses radiographically and five had pulmonary metastases from synovial sarcoma. Eight patients underwent resection of pulmonary metastases following chemotherapy, and they were found to be residual tumor cells histologically. Twelve of the patients died of disease at 6-108 months (median, 30 months) from the time of the initial therapy, and five patients have survived from 1-53 months (median, 30 months). The absolute three-year survival rate, according to the Kaplan-Meier method, for all 17 patients was 39%. In the two cases with no change and progressive disease, all patients were dead within 2 years, while in the seven partial response cases, two patients were dead, four were alive with pulmonary metastases, and only one case was disease-free at this writing. The survival rate for patients with partial response was significantly higher than for patients with no response. Although the cure rate of pulmonary metastatic soft tissue sarcomas is still low, the combination of chemotherapy and surgery has been shown to result in prolonged survival.
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PMID:[Chemotherapy for pulmonary metastases of soft tissue sarcoma]. 975 95

A case of malignant mesenchymoma of the anterior abdominal wall, composed of leiomyosarcoma, chondrosarcoma and osteosarcoma is described. The tumour, arising in the old scar of a previous operation, was completely removed surgically. Eleven months after the operation the patient is well with no evidence of recurrence. There have been no previous reports of malignant mesenchymoma developing in operation scars. We believe that this traumatic factor played a significant role in the aetiology of this rare soft-tissue neoplasm.
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PMID:Malignant mesenchymoma arising in an incisional scar of the abdominal wall. 980 Sep 81

The aim of this study was the evaluation of p53/MDM-2 protein overexpression in different subtypes of human sarcomas, and their correlation with proliferative activity and patient outcome. We selected 40 cases of human sarcomas comprising 6 malignant fibrous histiocytomas (MFH), 1 fibrosarcoma, 1 dermatofibrosarcoma protuberans, 5 liposarcomas, 9 leiomyosarcomas, 1 rhabdomyosarcoma, 3 synovial sarcomas, 2 osteosarcomas, 1 chondrosarcoma, 4 Ewing's sarcomas, 2 Kaposi's sarcomas, 1 malignant haemangiopericytoma, 1 phylloides cystosarcoma, 1 neuroblastoma, 1 chordoma and 1 unclassified sarcoma. All the immunohistochemical markers, which had been used for the characterization of these sarcomas were re-examined. Additionally, the Streptavidin-Biotin peroxidase method was performed on paraffin sections using the monoclonal antibodies: anti-p53 antibody DO7, anti-MDM-2 antibody IF2 and anti-Ki-67 antibody MIB-1. According to our results, p53 protein nuclear expression was detected in 20% (8/40) of the tumours (1 fibrosarcoma, 2 liposarcomas, 1 leiomyosarcoma, 1 rhabdomyosarcoma, 2 Ewing's sarcomas and 1 unclassified sarcoma). MDM-2 nuclear staining was determined in 7.5% (3/40) of the cases (1 MFH and 2 liposarcomas). A high proliferative index was demonstrated in 27.5% (11/40) of the tumours (2 MFH, 4 leiomyosarcomas, 1 rhabdomyosarcoma, 1 osteosarcoma, 2 Ewing's sarcomas and 1 unclassified sarcoma). p53 overexpression was associated with high tumour grade (p < 0.05) and MIB-1 expression was correlated with reduced survival (p < 0.05), but p53 overexpression was not significantly associated with either MIB-1 score or with overall survival of the patients. In conclusion, from this limited and heterogeneous sample of cases, we suggest that the p53/MDM-2 pathway is involved in the tumourigenesis of several sarcoma subtypes, but it is unclear if the overexpression of these genes may become prognostic marker for patients affected with these highly aggressive tumours.
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PMID:p53/MDM-2 immunohistochemical expression correlated with proliferative activity in different subtypes of human sarcomas: a ten-year follow-up study. 989 39

To determine the usefulness of microsatellite analysis in the differential diagnosis of various sarcomas, we investigated microsatellite alterations at 12 microsatellite loci by polymerase chain reaction and electrophoresis in 39 Japanese patients with sarcomas. The sarcomas were: osteosarcoma, Ewing's sarcoma, chondrosarcoma, liposarcoma, leiomyosarcoma, epithelioid leiomyosarcoma, rhabdomyosarcoma, synovial sarcoma, and malignant fibrous histiocytoma. We also examined ten leiomyomas to contrast with leiomyosarcoma. No microsatellite instability (MSI) or loss of heterozygosity (LOH) were found in Ewing's sarcoma, chondrosarcoma, epithelioid leiomyosarcoma, malignant fibrous histiocytoma, and leiomyoma. Only three patients, one each with liposarcoma, leiomyosarcoma, and synovial sarcoma, manifested MSI, whereas, osteosarcoma, liposarcoma, leiomyosarcoma, rhabdomyosarcoma, and synovial sarcoma manifested LOH, with an incidence of 43%, 14%, 86%, 20%, and 75%, respectively. Interestingly, three patients showed unusual patterns of LOH, probably due to intratumoral heterogeneity. Kaplan-Meier analysis revealed that LOH on 11p was predictive of poor prognosis in osteosarcoma. The low incidence of MSI indicates that MSI is not necessary for neoplastic transformation in sarcomas. However, the very high incidence of LOH in leiomyosarcoma indicates that microsatellite analysis may serve for the differential diagnosis of leiomyosarcoma versus leiomyoma. Microsatellite analysis may also predict prognosis in osteosarcoma.
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PMID:Microsatellite alterations in various sarcomas in Japanese patients. 1037 Jan 64

Limb salvage is widely used but has different disadvantages. Attempting to establish a new limb conservative procedure, we treate patients with malignant bone tumor by intraoperative microwave heating on tumor bearing bone in place and neoadjvant chemotherapy. The tumors were localized at distal femur in 16, proximal tibia in 5, femur shaft in 2, and illia in 2. Pathological diagnosis showed osteosarcoma in 16 patents, parosteoal osteosarcoma in 4, chondrosarcoma in 3, and leiomyosarcoma in 2. The stage of tumor was II b in 18 patients, II a in 6, and III b in 3. Operative technique was wide resection for tumor in soft tissue, protecting the surrounding tissue from heat injury by copper net, and cheating tumor bone at 50 C for 15 minutes. Follow-up varied from 4 to 180 months (mean 57 months). The 5-, 10-years surviral rates were 70.19% and 54.83% respectively. Functional results of 21 patients were > 15 points by Enneking system. The advantages of the method and keeping the shape and continuity of the tumor bearing bone without osteotomy and internal fixation, controlling the time and temperature of heating treatment easily, benefiting for bone remodeling of heated bone. The indications, complications, and surgical procedure of this method were discussed in detail.
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PMID:[Microwave heating and neoadjuvant chemotherapy for malignant bone tumor]. 1037 34

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

Ionizing radiation is a well-known risk factor for sarcoma development. To investigate whether radiation-associated sarcomas are characterized by chromosome aberrations that distinguish them from de novo sarcomas, we identified those patients in our series of more than 500 cytogenetically abnormal sarcomas that fulfilled the following criteria: (1) each patient should have been irradiated for another malignancy at least 3 years prior to the sarcoma diagnosis, and (2) the sarcoma should have developed within the field of radiation. Ten patients fulfilling these criteria could be retrieved (median age at sarcoma diagnosis was 55 years, range 17-79; median latency period between primary tumor and radiation-associated sarcoma was 9 years, range 4-30). The diagnoses were typical for radiation-associated sarcomas: 2 each of malignant fibrous histiocytoma, leiomyosarcoma, and pleomorphic sarcoma, and 1 each of osteosarcoma, fibrosarcoma, myxofibrosarcoma, and spindle cell sarcoma. All 10 cases had relatively complex karyotypes with multiple, mostly unbalanced, structural rearrangements, similar to what has been reported in de novo sarcomas of the corresponding histologic subtypes. The only cytogenetic features that were unusually frequent among the radiation-associated sarcomas were the finding of unrelated clones in 3 cases, and loss of material from chromosome arm 3p, in particular 3p21-3pter, in 8 cases. Loss of the same chromosome segment has been described in 4 of the 8 previously published cases of radiation-associated sarcomas that have been analyzed after short-term culturing, which makes this imbalance significantly (P < 0.001) more frequent among radiation-associated sarcomas (12 of 18 cases) than among unselected cases of the corresponding histologic subtypes (74 of 282 cases). In contrast to the cytogenetic results, no 3p deletions were detected among the 6 cases of the present series that could be analyzed by comparative genomic hybridization (CGH). The most frequent imbalance detected by CGH was gain of 15cen-q15 (3 cases), followed by loss of chromosome 13 and gain of 5p, and 7cen-q22, each detected in 2 cases.
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PMID:Radiation-associated sarcomas are characterized by complex karyotypes with frequent rearrangements of chromosome arm 3p. 1064 Jan 39

Sarcomas are rare malignancies of mesenchymal origin. Computed tomographic and magnetic resonance imaging characteristics, as well as histologic findings and epidemiology, of sarcomas of the head and neck are reviewed. The sarcomas discussed include rhabdomyosarcoma, fibrosarcoma, osteosarcoma, chondrosarcoma, malignant fibrous histiocytoma, leiomyosarcoma, alveolar soft part sarcoma, Ewing's sarcoma, and synovial sarcoma. The imaging findings of these entities frequently are nonspecific. Imaging, particularly magnetic resonance imaging, has a major role in defining the extent of these tumors. This is important because complete surgical excision is the preferred method of treatment. Imaging also is useful in planning radiation therapy and determining prognosis.
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PMID:Sarcomas of the head and neck. 1064 80

Below knee amputation remains the treatment of choice for most patients with aggressive tumours of the distal tibia. We report the clinical and functional outcome of limb preserving surgery and endoprosthetic reconstruction of the distal tibia and ankle joint in five patients who declined amputation. The mean age was 32 years. Two had osteosarcoma, one Ewing's sarcoma, leiomyosarcoma and Giant cell tumour. Three patients developed significant complications including local recurrence, wound dehiscence and infection, and fibula impingement. Despite these complications the patients declined amputation even in the presence of significant discomfort. Early function was excellent in all patients but deteriorated with time. The patients still maintained an Enneking Score of more than 50%. Some patients are unwilling to undergo amputation for aggressive tumours of the distal tibia. For these, excision and reconstruction with endoprosthesis allow early functional recovery but there is significant medium term morbidity and functional deterioration.
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PMID:Endoprosthetic replacement of the distal tibia and ankle joint for aggressive bone tumours. 1065 97


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