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)

Purpose. To report the outcome of 37 patients with metastatic osteosarcoma entered into a large randomized trial (EOI 80831/MRC B002) comparing two different regimens of chemotherapy in patients with osteosarcoma.Methods. Patients with biopsy-proven osteosarcoma localized and metastatic, age 40 years or younger, were randomized to receive either two-drug treatment with doxorubicin/cisplatin (DOX 25 mg m(-2) day(-1) x 3 + DDP 100 mg m(-2) on day 1 q 3 weeks x 6 courses) or three-drug treatment comprising high-dose methotrexate (HDMTX 8 mg m(-2) administered every 4.5 weeks x 4 courses) given 10 days before DOX/DDP.Results. Twenty-four patients with metastatic disease received the two-drug arm treatment and 13 received three-drug treatment. Despite chance imbalance in numbers, there were no major differences in age, sex, primary site or performance status. Baseline alkaline phosphatase (AP) was elevated more frequently (96 vs 42%) in the two-drug arm. Twenty-one of 24 patients in the two-drug arm and 11/13 patients in the three-drug arm had evaluable primary tumors concurrent with metastases. Respective clinical response rates for the two- and three-drug arms were 48% and 40% for primary tumors, and 33% and 55% for metastases. Respective survivals at 2 and 4 years were 36% and 9% for the two-drug arm, and 69% and 52% for the three-drug arm, and survival was better for patients with normal AP at presentation. When adjusted for AP, survival was not significantly different between the two treatments (hazard ratio 0.52, 95% confidence interval 0.22-1.23, p = 0.14). There were three long-term survivors among the metastatic patients, all of whom received the three-drug therapy.Discussion. It is likely that random bias in the population (small numbers, imbalance in size of groups, uneven distribution of AP) accounts for the difference in outcome favoring the three-drug treatment in patients with metastatic disease. More reliance can be placed on the finding that disease-free and overall survival in the adjuvant component of this study (Bramwell et al., J Clin Oncol 1992; 10: 1579-91) were better after two-drug treatment.
Sarcoma 1997
PMID:A Randomized Comparison of two Short Intensive Chemotherapy Regimens in Children and Young Adults With Osteosarcoma: Results in Patients With Metastases: A Study of the European Osteosarcoma Intergroup. 1852 Dec 18

Purpose. The purpose of this study was to analyse the height at diagnosis and growth in 72 skeletally immature children who had been treated for osteosarcoma in the area of the knee.Subjects. Of the patients, the average age at diagnosis was 10 years in girls and 12 years in boys. All children received neo-adjuvant chemotherapy, and had limb salvage by endoprosthetic replacement.Results and conclusion. The results of this study indicate that there is no evidence that children with osteosarcoma are taller at diagnosis than their normal counterparts. However, there was a marked retardation in growth in the year following the administration of cytotoxic chemotherapy. There were 19 children who reached skeletal maturity. The final height in these children was not significantly different from the normal population.
Sarcoma 1998
PMID:Longitudinal growth following treatment for osteosarcoma. 1852 Dec 42

Patient. We report a 22-year-old male presenting with extraskeletal osteosarcoma of the orbit.Discussion. Extra skeletal osteosarcomas are uncommon tumours, usually arising from the lower extremities or girdle. These are aggressive tumours with high metastatic potential and poor outcome. Optimal treatment is undefined, and the role of radical surgery, radiotherapy and aggressive chemotherapy is currently being evaluated. The orbit is a rare site for extraskeletal osteosarcoma, with the only previous case reported in an 11-year-old male, who was irradiated in infancy for a retinoblastoma.
Sarcoma 1998
PMID:Extraskeletal osteosarcoma of the orbit. 1852 Dec 43

Purpose. The feasibility of hyperthermic isolated limb perfusion (HILP) with tumor necrosis factor-alpha (TNFalpha ) and cisplatin for the management of osteosarcoma was studied in the canine model.Methods. During seven perfusions in six healthy mongrel dogs (weight 32+/-2 kg) technical aspects of HILP under mild hyperthermia (39- 40) were studied. In five experiments HILP was performed with TNFalpha alone (0.5 mg/l extremity volume), and in two experiments TNFalpha was combined with cisplatin (25 mg/l extremity volume). During the perfusions physiological parameters were monitored and TNFalpha and total cisplatin concentrations were determined.Results. Perfusion conditions (pH, PCO(2) , PO(2), flow and pressure) remained within physiological ranges.Three dogs died within 24 h despite a sublethal systemic concentration of TNFalpha that leaked from the perfusion circuit. Three dogs were terminated; one dog after the second experiment in accordance with Dutch ethical rules; one dog showed an invagination of the small bowel resulting in an ileus; one dog because of necrosis of the perfused limb.Conclusions. This feasibility study in healthy dogs demonstrated that HILP with TNFalpha and cisplatin was associated with a high mortality rate and does not allow us to treat dogs with spontaneous osteosarcoma with TNFalpha and cisplatin HILP. Therefore, an alternative model should be used in the search for the ideal combination of perfusion agents for limb sparing treatment in human osteosarcoma.
Sarcoma 1999
PMID:Hyperthermic Isolated Limb Perfusion with TNF alpha and Cisplatin in the Treatment of Osteosarcoma of the Extremities: A Feasibility Study in Healthy Dogs. 1852 Dec 69

Purpose. To present a case study involving the use of fast neutron therapy to treat an extensive unresectable osteogenic sarcoma arising from the left maxilla.Patient. A 14-year-old male presented with a massive tumor producing severe distortion of his facial structures. He had already received six courses of chemotherapy, which had reduced his pain, but had not measurably reduced the tumor.Methods. The patient was treated with 66 MeV fast neutrons to a dose of 20.4 Gy in 13 fractions over 35 days.Results. CT assessments indicate gradually increasing calcification and noticeable reduction of soft-tissue disease in the frontal sinus, orbit and maxillary antrum.There has been some recontouring of the facial structures.The boy conducts an active life, has no pain, and feels well. He was 17 years old at the last follow-up.Discussion. Fast neutrons have a greater biological effectiveness than conventional photon beams. Their use has been associated with improved chance for local control of unresectable disease.This case illustrates their effectiveness in controlling an unusual and aggressive osteogenic sarcoma of the facial bone and sinuses.
Sarcoma 1999
PMID:Osteogenic sarcoma of the maxilla: neutron therapy for unresectable disease. 1852 Dec 77

Purpose. Primary extra-osseous osteosarcomas are uncommon lesions, and those originating within the lung are especially rare, with few case reports existing in the literature.Patient. We report the case of a 48-year-old male smoker with a primary osteosarcoma of the right lower lung lobe.Results. Diagnosis was based on histopathological findings of a poorly differentiated sarcoma with malignant cellular components of osteoid and chondroid matrix, along with immunohistochemical and electron microscopy confirmation. Extensive clinical and radiographic evaluation failed to reveal any tumor at other anatomical sites.
Sarcoma 1999
PMID:Primary chondroblastic osteosarcoma of the lung. 1852 Dec 85

Purpose. Production of active transforming growth factor-beta (TGF-beta ) by human osteosarcoma may contribute to malignant progression through mechanisms that include induction of angiogenesis, immune suppression and autocrine growth stimulation of tumor cell growth.To study events associated with induction of cell proliferation by TGF-beta , we have evaluated the TGF-beta pathway in two murine osteosarcoma cell lines, K7 and K12.Results. Northern and immunohistochemical analyses show that each cell line expressesTGF-beta1 and TGF-beta3 mRNA and protein. Both cell lines secrete activeTGF-beta 1 and display a 30-50% reduction in growth when cultured in the presence of a TGF-beta blocking antibody. Expression of TGF-beta receptors TbetaRI, TbetaRII and TbetaRIII is demonstrated by affinity labeling with (125) -TGF-beta 1, and the intermediates, Smads 2, 3 and 4, are uniformly expressed. Smads 2 and 3 are phosphorylated in response toTGF-beta , while pRb phosphorylation in each osteosarcoma cell line is not affected by either exogenousTGF-beta or TGF-beta antibody.Conclusions. The data implicate events downstream of Smad activation, including impaired regulation of pRb, in the lack of a growth inhibitory response toTGF-beta , and indicate that this murine model of osteosarcoma is valid for investigating the roles of autocrineTGF-beta in vivo.
Sarcoma 2000
PMID:Autocrine Transforming Growth Factor-beta Growth Pathway in Murine Osteosarcoma Cell Lines Associated with Inability to Affect Phosphorylation of Retinoblastoma Protein. 1852 Dec 87

Purpose. Extraskeletal osteosarcoma represents an unusual soft-tissue sarcoma that historically is reported to carry an exceptionally poor prognosis.The objectives of this study were to use a prospectively gathered sarcoma database to test the prevailing clinical bias and more accurately describe the natural history, characterize the prognostic features, estimate survival and evaluate treatment strategies for this unusual sarcoma.Patients and methods. From a large database of nearly 4000 sarcomas at a single institution, 15 patients with pathologically confirmed extraskeletal osteosarcoma were analysed.Results and discussion. Extraskeletal osteosarcoma usually occurs as a large, deep, high-grade lesion in the lower extremity of older patients. Overall and disease-specific survival at 5 years was 50%, with a median follow-up of 35 months (range 3- 200 months). Use of adjuvant chemotherapy or radiation therapy did not appear to influence survival, but an effect may have been missed by the relatively low numbers in each group.When matched to a comparable group of patients with stage III extremity sarcomas, there was no significant difference in overall or disease-specific survival between groups. Treatment for extraskeletal osteosarcoma should follow established guidelines for treatment of soft-tissue sarcomas, with the decision regarding adjuvant therapy to be based on individual risk factors.
Sarcoma 2000
PMID:Extraskeletal osteosarcoma: analysis of outcome of a rare neoplasm. 1852 Dec 90

Purpose. We investigated self-reported quality of life (QoL), body image and daily competence of patients as a consequence of limb salvage surgery (LSS), primary or secondary amputation, and the views of patients following secondary amputation.Patients. Patients (n=37) had all been treated for osteosarcoma or Ewing's sarcoma in the lower limb.Methods. QoL was measured by questionnaire. A separate interview to determine satisfaction with decision-making was conducted with those treated for secondary amputation.Results. For the total group, QoL was below that expected from population norms. There were no differences in QoL between those undergoing LSS surgery compared with amputation. However, LSS reported better daily competence and were less likely to use a walking aid. For the total group, body image and daily competence were associated with better QoL.Discussion. All these patients are at risk of compromised QoL following surgery. Our data are in line with previous work suggesting outcomes may be better for LSS compared with amputation. Following secondary amputation, most patients (80%) did not regret initial LSS treatment, but felt that the time gained allowed them to come to terms with subsequent surgery.
Sarcoma 2001
PMID:Quality of life implications as a consequence of surgery: limb salvage, primary and secondary amputation. 1852 13

The most common primary malignant tumor of the bone is osteosarcoma. Primary involvement of the craniofacial bones in osteosarcoma is relatively rare. The mandible and the maxillae are the most commonly affected bones of the head. Here, we report a rare case of de novo high-grade osteogenic sarcoma of the mastoid region of the temporal bone and discuss the diagnostic and therapeutic properties.
Sarcoma 2002
PMID:De novo osteogenic sarcoma of mastoid bone. 1852 33


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