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)

Five tumors (two myxoid malignant fibrous histiocytoma, two dermatofibrosarcoma protuberans, and one parosteal osteosarcoma) with ring chromosomes as the sole cytogenetic anomaly or as the only structural rearrangement were observed in a series of 60 karyotypically abnormal, nonlipogenic bone and soft tissue tumors (BST). All five tumors were of borderline or low malignancy. These findings support the suggestion that supernumerary ring chromosomes as the sole structural chromosomal aberration are not associated with any particular histopathologic diagnosis but may characterize a group of BST of borderline or low malignancy.
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PMID:Supernumerary ring chromosomes in five bone and soft tissue tumors of low or borderline malignancy. 131 82

In this study, two specimens of a parosteal osteosarcoma, a rare primary bone neoplasm comprising only 3-6% of all osteosarcomas, were cytogenetically analyzed utilizing standard techniques. In contrast to the complex karyotypes previously reported in osteosarcoma, this particular histologic subtype was characterized by a single chromosomal aberration, a ring chromosome. Ring chromosomes have been described as characteristic for two other low-grade malignant mesenchymal neoplasms, well-differentiated liposarcoma and dermatofibrosarcoma protuberans. We propose that the observation of a ring chromosome in osteosarcoma also correlates with a low-grade malignant potential.
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PMID:Ring chromosome in parosteal osteosarcoma. Clinical and diagnostic significance. 152 Dec 34

Authors operated on 6339 malignant breast tumors at the Department of Surgery, National Institute on Oncology between 1980 and 1994. The records of 14 patients with sarcomas of the breast were analyzed. These included 2 malignant phyllodes tumors, 3 malignant fibrous histiocytomas, 2-2 fibrosarcomas and carcinosarcomas and liposarcoma, angiosarcoma, leiomyosarcoma, osteosarcoma and dermatofibrosarcoma protuberans one of each. During this period 5 patients died, 9 are living without evidence of tumor. Analysing these 14 cases authors present their policy in surgical therapy of breast sarcomas compared with literature data. They emphasise the importance of wide resection margins for prevention of tumor progression. The above mentioned principle is valid for local recurrences so radicalization of the previous conservative breast surgery may be necessary in selected cases. Intraoperative histological examination is recommended to verify that the resection margins are tumor free. Axillary block dissection is not necessary except in cases when palpable, firm lymph nodes are present in the region. Depending on the histological grade adjuvant radiotherapy could be considered but its effectiveness is not proven.
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PMID:[Experience in the treatment of breast sarcomas]. 907 52

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

We described a case of Werner's syndrome associated with osteosarcoma. A 37-year-old Japanese man was diagnosed as having Werner's syndrome by the presence of juvenile cataracts, skin sclerosis and hyperpigmentation of the feet, high-pitched voice, characteristic bird-like appearance of the face with beak-shaped nose, thinning of the entire skin and hyperkeratoses on soles, hyperlipemia, hyperuricemia, diabetes melitus, and the mutated responsible gene (WRN). He had a 3-month history of a tumor on his left forearm. Histologically, the tumor included four histological patterns; a malignant fibrous histiocytoma-like, a desmoid-like, a dermatofibrosarcoma protuberans-like, and a chondrosarcoma-like pattern. Tumoral osteoid formation was also found in the tumor. Therefore, the tumor was diagnosed as osteosarcoma.
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PMID:A case of Werner's syndrome associated with osteosarcoma. 1055 36

This review discusses the spectrum of malignant soft tissue tumors usually found in adults. Lesions most commonly identified in the pediatric population, as well as tumorlike masses, are not covered. Rather than presenting a complete review, the focus of this discussion is on common lesions and those lesions in which the diagnosis may be suggested on the basis of imaging. Diagnoses covered include malignant fibrous histiocytoma, fibrosarcoma, dermatofibrosarcoma protuberans, liposarcoma, synovial sarcoma, malignant peripheral nerve sheath tumor, leiomyosarcoma, aggressive vascular tumors, extraskeletal chondrosarcoma and osteosarcoma, lymphoma, and metastatic carcinoma.
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PMID:Imaging of Soft Tissue Neoplasms in the Adult: Malignant Tumors. 1138 26

Low-affinity nerve growth factor receptor (p75) is a member of the tumor necrosis factor receptor family. It may modulate the binding of nerve growth factor (NGF) to the functional high-affinity receptor tyrosine kinase (trk) A. NGF is thought to be responsible for growth, apoptosis, and function of the nervous system. The presence of this receptor (p75) was determined in a large group of neural and nonneural tumors and fetal and adult tissues. One thousand one hundred fifty tumors were analyzed with monoclonal antibody for p75, along with selected normal fetal and adult tissues. Immunoreactivity for p75 was present in adult pericytes, perivascular fibroblasts, basal cells of several types of epithelia, perineurial cells, and dendritic reticulum cells. Additionally, a wide zone of subepithelial mesenchyme and skeletal muscle were positive in the first-trimester fetus, but were diminished or negative in the adult. Consistently positive nonneural mesenchymal tumors included dermatofibrosarcoma protuberans (DFSP), embryonal and alveolar rhabdomyosarcoma, synovial sarcoma, and spindle cell hemangio(endotheli)oma. Schwann cell tumors, ganglioneuroma, granular cell tumor, and malignant peripheral nerve sheath tumor (MPNST) were also p75 positive. Mesenchymal nonneural tumors that were variably positive (32% to 69%) for p75 included fibrosarcoma variants, solitary fibrous tumor, hemangiopericytoma, spindle cell lipoma, Ewing's sarcoma, mesenchymal chondrosarcoma, and malignant melanoma. Nervous system tumors such as paragangliomas, neuroblastoma, meningioma, and perineurioma and nonneural mesenchymal tumors, including extraskeletal osteosarcoma, benign fibrous histiocytomas, fibromas, alveolar soft part sarcoma, epithelioid sarcoma, smooth muscle and gastrointestinal stromal tumors, and angiosarcomas, were almost always negative for p75. Epithelial tumors that were consistently positive included mixed tumor and adenoid cystic carcinoma, whereas mesothelioma, adenocarcinomas, and most squamous cell carcinomas were negative. p75 is not a specific marker for nerve sheath tumors. It is present in a variety of other mesenchymal tumors including synovial sarcoma and in CD34-positive tumors such as DFSP, spindle cell lipoma, and hemangiopericytoma. The presence of p75 in nonneural tumors such as DFSP and rhabdomyosarcoma mimic its presence in early fetal mesenchyme and skeletal muscle, suggesting oncofetal expression in these tumors. p75 may be useful to distinguish DFSP from benign fibrous histiocytoma.
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PMID:Low-affinity nerve growth factor receptor (p75) in dermatofibrosarcoma protuberans and other nonneural tumors: a study of 1,150 tumors and fetal and adult normal tissues. 1156 28

Soft tissue and bone sarcomas in the head and neck are rare tumors. The 1000 to 1500 yearly cases in the United States are distributed among at least 10 main histologies and multiple head and neck subsites. Although this makes structured studies difficult to perform and high-level evidence-based treatment algorithms difficult to find, basic treatment recommendations can be made from the existing literature for most histologies and subsites. This paper discusses the epidemiology, natural history, and treatment approaches for several of the most common head and neck sarcomas, including the "adult soft tissue sarcomas," osteosarcoma, chondrosarcoma of the larynx, angiosarcoma, rhabdomyosarcoma, desmoid tumors, and dermatofibrosarcoma protuberans.
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PMID:Management of sarcomas of the head and neck. 1516 89

Microarrays began to be used to study gene expression profiles in the mid-1990s, but it was only after 2000 that serious attempts have been made to apply this technology to investigate sarcomas. Microarray technologies provide a comprehensive survey of active molecular pathways and potential molecular targets for diagnosis and treatment, but are challenging to use because of issues of specimen collection, cost, and complexities in experimental design and data analysis. As a discovery-based technique, microarray analyses are most valuable when framed around specific gaps in our knowledge of tumor etiology and progression, challenges in differential diagnosis, and pressing therapeutic needs. To date, microarray analyses of sarcomas support their division into molecularly defined and molecularly heterogeneous categories, and have provided useful diagnostic markers for entities such as gastrointestinal stromal tumors, synovial sarcoma, and dermatofibrosarcoma protuberans. Signatures predicting outcome and response to therapy have been published for Ewing sarcoma and osteosarcoma, and receptor tyrosine kinase expression patterns have suggested novel therapeutic approaches which may be applied to several types of sarcoma. Nevertheless, results need to be interpreted in the context of histopathology and validated by complementary technologies and/or other research groups.
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PMID:Microarray analysis of sarcomas. 1685 50

Sarcomas are mesenchymal cancers, which, in many cases, have distinctive molecular features. Limb-sparing surgery delivered at specialised sarcoma centres as part of a multidisciplinary approach has become the standard treatment for most patients and usually provides excellent local control. Preoperative treatment with chemotherapy is most common for patients with bone sarcomas. The ideal sequence of surgery and radiation for local management of soft-tissue sarcoma remains controversial on the basis of early versus late treatment complications, although preoperative radiation can provide the best results for improved long-term function. New methods for radiation delivery and tumour sensitisation might provide further improvements. However, metastatic disease is common, and conventional chemotherapy provides for only a narrow therapeutic window outside of a few responsive pathological subtypes. Targeting underlying molecular events in specific sarcomas can provide for dramatic benefits, as has been seen with imatinib treatment for gastrointestinal stromal tumours and dermatofibrosarcoma protuberans. Trials of agents targeting the cell cycle and angiogenesis in soft-tissue sarcomas, and of those targeting osteoclasts in bone sarcomas, are currently underway. Biological data and preclinical studies support trials using inhibitors of hedgehog signalling in chondrosarcoma, inhibitors of wnt/beta-catenin in osteosarcoma and aggressive fibromatosis, and inhibitors of histone deacetylases in synovial sarcoma and Ewing sarcoma. Pharmacogenetic approaches will be needed to identify individual determinants of response and outcome in order to maximise the benefits of targeting specific molecular events and keep side-effects to a minimum. Research in stem-cell biology and nanotechnology holds promise for additional novel treatment options in the future.
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PMID:Opportunities for improving the therapeutic ratio for patients with sarcoma. 1767 78


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