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)

Sarcoma and normal tissue plasma membrane lectin-reactive glycoproteins were analyzed by sodium dodecyl sulfate polyacrylamide gel electrophoresis. Two peanut agglutinin-reactive N-acetylgalactosamine-containing glycoproteins of 1.05 x 10(6) and 1.25 x 10(5) Da and one lentil agglutinin-reactive mannose/N-acetylglucosamine(-fucose)/sialic acid-containing glycoprotein of 1.7 x 10(5) Da (Gp170) were detected in osteosarcoma and malignant fibrous histiocytoma (MFH), respectively. However, these glycoproteins were not detected in normal tissue plasma membranes. Concanavalin A, wheat germ and Ulex europaeus Type I agglutinins did not reveal any unique sarcoma-associated membrane glycoproteins. Preliminary studies on monoclonal antibodies (mAbs) generated against Gp170 (mAb 64-35-84) and against lentil-reactive glycoproteins from MFH (mAbs 67-34 and 67-117) revealed high specific binding to a number of membranes isolated from MFH and osteosarcoma tissues, with no crossreactivity to normal human tissues tested (liver, spleen and skin). Detailed analysis of mAb 67-102, which was generated against lentil-reactive glycoproteins isolated from MFH plasma membranes, exhibited significant binding to membranes isolated from osteosarcoma, liposarcoma and MFH; moderate binding to synovial sarcoma, aggressive fibromatosis and fibrosarcoma; and minimal to no binding to other soft tissue sarcoma plasma membranes. No binding was observed to twenty normal tissue specimens, with the exception of low positive binding to two of five fat and two of three colon specimens.
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PMID:Isolation and analysis of lectin-reactive sarcoma-associated membrane glycoproteins. 801 64

A radiograph of the second left metacarpal bone in a 34-year-old man showed irregular lytic areas in the diaphysis that was expanded with perpendicular periosteal reactions. Examination of the first biopsy specimen indicated a fibrous dysplasia. Examination of the second biopsy specimen revealed an unequivocal grade I intramedullary osteosarcoma, desmoid to fibrous dysplasia-like in histological pattern. Three years after an adequate en bloc resection of the metacarpal bone, the man was healthy. The long-term course is usually favorable.
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PMID:Low-grade osteosarcoma of metacarpal bone. 895 70

Between 1991 and 1994, 582 operations were performed in our service; 19 (3.26%) were on primitive tumors of the chest wall. We analyze the data for these patients, including age, sex, clinical findings, chest images, diagnoses, therapy and course. Ten tumors were benign and 9 were malignant. The most frequent clinical findings were pain and/or tumor. Diagnosis was achieved before surgery in only 2 cases. Except when there are clear macroscopic and X-ray signs that the tumor is benign, we performed broad exeresis of the chest wall, sometimes also resecting adjacent structures. The defect was repaired directly in 12 cases. The defects were covered by prostheses and/or muscle plasty in the remaining patients. The most frequent tumor was chondrosarcoma (3 cases), followed by 2 cases of osteoblastoma and osteochondroma. Only 1 each of the following tumors were found: plasmocytoma, chondroma, fibrous dysplasia, eosinophilic granuloma, osteosarcoma, Ewing's tumor, epithelioid sarcoma, fibrosarcoma, hemangioma, benign neurilemmoma, desmoid tumor and liposarcoma. Two patients with chondrosarcoma were operated on for recurrences and there was also recurrence in the patient with Ewing's tumor. We conclude that: 1) chest wall tumors are infrequent, 2) radical exeresis is the treatment of choice and prosthesis is often necessary, and 3) chondrosarcoma, with poor outcome in our patients, is the most frequent tumor.
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PMID:[Primary tumors of the thoracic wall (1991-1994)]. 1256 9

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

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

Adult head and neck soft tissue sarcomas (AHNSTS) are rare, and data concerning treatment results are spare. To assess clinico-pathological characteristics, management prognostic factors, and survival of AHNSTS, we reviewed our experience of 28 recent successive new cases. Data were collected from a retrospective database (1997-2002). Aggressive fibromatosis, dermatofibrosarcoma, Kaposi sarcoma, chondrosarcoma and osteogenic sarcoma were excluded. Univariate analysis for prognostic factors was performed with chi2 test with Yates correction. The median age was 45.7 years (range: 18-86). The male/female ratio was 15/13. The most common subtypes was rhabdomyosarcoma (seven cases). Twenty-two patients presented with previous inadequate resection performed elsewhere before admission. The most common location was neck muscles (11 cases). Nineteen patients had surgery (complete resection in 13 cases). Associated treatments were neoadjuvant chemotherapy, adjuvant chemotherapy and postoperative radiotherapy in respectively, 4, 3 and 10 cases. The 2-year overall survival was 56%. Rhabdomyosarcomas (p = 0.005) and inadequate resection (p = 0.04) were associated with poor outcome. Large resection of AHNSTS in a multimodality approach may afford the best chance of disease control.
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PMID:Head and neck soft tissue sarcomas of adult: prognostic value of surgery in multimodal therapeutic approach. 1538 Jan 66

Desmoplastic fibroma is a very rare primary tumor of bone, closely related to aggressive fibromatosis of soft tissue. Although considered a benign lesion, it can be very aggressive locally and has a high rate of local recurrence after incomplete surgical excision. Radiologically, the lesion shows no distinctive features, often simulating osteosarcoma, fibrous dysplasia, or fibrosarcoma. Rib involvement by desmoplastic fibroma is extremely rare. We present two cases of desmoplastic fibroma involving this unusual location with lytic costal lesion and chest wall extension. Histological examination after surgical resection revealed that the tumor consisted of spindle cells with small, elongated nuclei in a background of numerous collagen fibers and infiltrating lamellar bone. There was no evidence of nuclear atypia, mitotic activity, or necrosis. We also discuss histological differential diagnosis as well as clinical features and the radiological and pathologic findings of this rare disease. Recognition of this entity is important to ensure proper surgical treatment.
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PMID:[Desmoplastic fibroma of the rib: two case reports]. 1649 94

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

Sarcomas comprise a heterogeneous group of mesenchymal neoplasms. They can be grouped into 2 general categories, soft tissue sarcoma and primary bone sarcoma, which have different staging and treatment approaches. This review includes a discussion of both soft tissue sarcomas (malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, synovial sarcoma, dermatofibrosarcoma protuberans, angiosarcoma, Kaposi sarcoma, gastrointestinal stromal tumor, aggressive fibromatosis or desmoid tumor, rhabdomyosarcoma, and primary alveolar soft-part sarcoma) and primary bone sarcomas (osteosarcoma, Ewing sarcoma, giant cell tumor, and chondrosarcoma). The 3 most important prognostic variables are grade, size, and location of the primary tumor. The approach to a patient with a sarcoma begins with a biopsy that obtains adequate tissue for diagnosis without interfering with subsequent optimal definitive surgery. Subsequent treatment depends on the specific type of sarcoma. Because sarcomas are relatively uncommon yet comprise a wide variety of different entities, evaluation by oncology teams who have expertise in the field is recommended. Treatment and follow-up guidelines have been published by the National Comprehensive Cancer Network (www.nccn.org).
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PMID:Sarcoma. 1797 62

Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.
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PMID:Histology and imaging of soft tissue sarcomas. 1983 58


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