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)

The use of adjuvant chemotherapy in soft tissue sarcomas (STS) continues to be an area of controversy; however, the group of investigators favoring the use of an anthracycline- and ifosfamide-based regimen for high-risk (American Joint Committee on Cancer stage III) extremity STS is steadily increasing. The historic 5-year survival rate of approximately 50% in this high-risk group treated with local therapy alone represents a poor standard of care, thus there is a need to incorporate systemic therapy early in the management of these patients. Published data from the meta-analysis of doxorubicin-based adjuvant chemotherapy trials and the prospective randomized data with epirubicin and ifosfamide from the Italian Sarcoma Group are frequently used as rationale for this approach. In a rare and heterogenous group of diseases, such as STS, physicians run into negative studies for various reasons that have little to do with the efficacy of the treatment being tested. The wisdom may be in capitalizing further on a positive lead as opposed to nihilism. It is appropriate to acknowledge that the chemotherapeutic agents have limited efficacy and are toxic, especially when used at full therapeutic doses. Selecting patients in whom there is some evidence of benefit, justifying the poor quality of life from receiving chemotherapy, becomes very important. This rationale, with the lessons learned from osteosarcoma research, forms the basis for neoadjuvant chemotherapy for STS. Until we reach the day when we have identified critical tumorigenic targets and their effective inhibitors for most of these tumors, we are obligated to use the available therapeutic armamentarium in the best possible sequence.
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PMID:Advances in neoadjuvant chemotherapy in soft tissue sarcomas. 1458 24

Sarcoma arising from fibrous dysplasia (FD) is rare and it is more common in polyostotic type. In this case report, we present a 41-year-old male patient who developed osteosarcoma of the left tibia 28 years after the initial diagnosis and 20 years after radiation therapy for FD. He underwent above-knee amputation, but died eight months after surgery. This case suggests that radiotherapy has no beneficial effect in the treatment of FD and that it may be associated with the development of sarcoma.
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PMID:[The development of osteosarcoma following radiotherapy for fibrous dysplasia]. 1572 18

All cases of high-grade osteosarcoma (OS) (n = 196) and Ewing's sarcoma of bone (ES) (n = 56) treated at the Norwegian Radium Hospital in the period 1980-1999 were analyzed retrospectively. They were allocated to consecutive ten-year periods by their time of diagnosis. Patient and tumour characteristics have been relatively stable. Eighty percent of all patients received surgical treatment and the amputation rate decreased from 64% to 23%. The percentage of patients receiving chemotherapy has remained around 80%. The use of radiotherapy in primary treatment decreased gradually from 33% to 18%. Sarcoma specific survival (SSS) at five years for all patients increased significantly from 39% to 53%. Similar trends for improvement were seen for both OS and ES. In multivariate analysis, independent prognostic factors for improved SSS were non-metastatic disease at diagnosis, age under 40, extremity tumours, small tumours and treatment from 1995 onwards. No major new treatment options have emerged over these 20 years. The improved outcome appears partly to be due to refinements in the use of existing modalities and improved quality and integration of multidisciplinary approaches. Improved formalized organisation of the sarcoma group and annual audited reports of its patient and research activity may also have contributed to improved focus and performance.
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PMID:Management of high-grade bone sarcomas over two decades: the Norwegian Radium Hospital experience. 1646 94

The aim was to evaluate the clinical impact of P-glycoprotein in primary non-metastatic high-grade osteosarcoma patients, treated with neoadjuvant chemotherapy protocols. P-glycoprotein was assessed by immunohistochemistry on paraffin-embedded tissue samples collected at time of diagnosis from 94 osteosarcoma patients, treated with the Italian Sarcoma Group/Scandinavian Sarcoma Group 1 (ISG/SSG 1) protocol. P-glycoprotein-positivity at diagnosis was found in 53/94 ISG/SSG 1 cases (56%) and emerged as the single factor significantly associated with an unfavourable outcome from survival and multivariate analyses. A comparative analysis of the subgroup of 94 patients considered for P-glycoprotein evaluation and the whole series of ISG/SSG 1 patients showed that this marker retained its prognostic value also in the latter group. In osteosarcoma patients treated with doxorubicin-based chemotherapy protocols, P-glycoprotein overexpression at diagnosis is an important adverse prognostic factor for outcome. P-glycoprotein evaluation can therefore constitute the basis for stratifying, at diagnosis, osteosarcoma patients for whom alternative treatments may be considered.
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PMID:May P-glycoprotein status be used to stratify high-grade osteosarcoma patients? Results from the Italian/Scandinavian Sarcoma Group 1 treatment protocol. 1708 85

Background. Multifocal osteosarcoma is usually described as the occurrence of the tumour at two or more sites in a patient without pulmonary metastases and may be synchronous or metachronous. Case report. A previously well 21-year old male, who presented with a swollen, painful right knee with no history of trauma, was found to have a high-grade osteosarcoma of the distal tibia and proximal femur. He underwent resection and prosthetic replacement of the distal femur and proximal tibia and remains well 19 months after diagnosis. Discussion. Multifocal osteosarcoma is a rare condition with a poor prognosis. There is debate about whether it represents multiple primary tumours or metastatic disease.
Sarcoma 2006
PMID:Synchronous multifocal osteosarcoma: case report and literature review. 1725 58

Extraskeletal osteosarcoma (EOS) is a highly aggressive and exceedingly rare mesenchymal tumor. Due to the rare nature of the disease, the diagnosis can be difficult and is often confirmed only after diagnostic laparotomy and histopathology. We describe the clinical history, radiologic and histomorphologic presentation, and clinical management of a 61-year-old patient who presented with abdominal pain. Abdominal ultrasound and computerized tomography (CT) scan revealed a calcified intra-abdominal mass. Following an explorative laparotomy, histology showed a large extraosseous osteosarcoma of the small bowel mesentery. Therapy according to the Cooperative Sarcoma Study-96 (COSS-96) was commenced. Diagnosis, management, and outcome in the context of the current literature are discussed. To our knowledge, this is the first description of an extraosseous osteosarcomas in the small bowel mesentery in the literature.
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PMID:Extraosseous osteosarcoma arising from the small intestinal mesentery. 1753 87

Purpose. The antimicrobial effect of a silver-coated tumor endoprosthesis has been proven in clinical and experimental trials. However, in the literature there are no reports concerning the effect of elementary silver on osteoblast behaviour. Therefore, the prosthetic stem was not silver-coated because of concerns regarding a possible inhibition of the osseointegration. The aim of the present study was to investigate the effect of 5-25 mg of elementary silver in comparison to Ti-6Al-4V on human osteosarcoma cell lines (HOS-58, SAOS). Methods. Cell viability was determined by measuring the MTT proliferation rate. Cell function was studied by measuring alkaline phosphatase (AP) activity and osteocalcine production. Results. In the HOS-58 cells, the AP activity was statistically significant (P < 0.05) higher at a supplement of 5-10 mg of silver than of Ti-6 Al-4V at the same doses. For both cell lines, a supplement above 10 mg of silver resulted in a reduced AP activity in comparision to the Ti-6 Al-4V group, but a statistically significant difference (P < 0.05) was observed at a dose of 25 mg for the SAOS cells only. At doses of 20-25 mg in the HOS-58 cells and 10-25 mg in the SAOS cells, the reduction of the proliferation rate by silver was statistically significant (P < 0.05) compared to the Ti-6 Al-4V supplement. Discussion. In conclusion, elementary silver exhibits no cytotoxicity at low concentrations. In contrast, it seems to be superior to Ti-6 Al-4V concerning the stimulation of osteogenic maturation at these concentrations, whereas at higher doses it causes the known cytotoxic properties.
Sarcoma 2007
PMID:The influence of elementary silver versus titanium on osteoblasts behaviour in vitro using human osteosarcoma cell lines. 1768 31

A murine model of osteosarcoma was developed to investigate the association between the expression of VEGF and the progression of osteosarcoma. Two human osteosarcoma cell lines with distinct VEGF expressions were introduced into proximal tibiae of immuno-deficient SCID mice, either by direct injection through the cortical bone or surgical exposing and drilling on the tibial metaphysis to seed tumor cells. Bone tumors were obvious on microCT within 4 weeks following osteosarcoma cell inoculation through surgical delivery. In contrast, direct injection without drilling often resulted in periosteal tumors. Although neoplasms were developed regardless of VEGF levels, orthotopic tumors derived from high VEGF-expressing cells were detected 2 weeks earlier on CT images than the ones from VEGF negative cells. At sacrifice, high VEGF tumors were distinctively larger in size and more frequently invaded the adjacent bone tissue. Multiple metastatic lesions were found in all the lung tissues at 8 weeks from high VEGF group, whereas only 1 of 7 VEGF negative tumors exhibited pulmonary metastasis. Overall, this model developed with the surgical tumor cell delivery results in histological and radiographic features more consistent with primary osteosarcoma. Interestingly, VEGF expression correlates with the early establishment, rapid tumor growth, and the development of pulmonary metastasis.
Sarcoma 2007
PMID:High VEGF with rapid growth and early metastasis in a mouse osteosarcoma model. 1827 12

Purpose. This paper describes the clinical history and radiographic appearance of second malignancies in patients with bilateral retinoblastoma.Subjects/methods. The imaging studies and clinical data of 14 patients with a history of bilateral retinoblastoma who were treated for second malignancies were reviewed.Results. A total of 17 tumors were identified in 14 patients during the period 1978-1996. The median age of occurrence of the second malignancy was 17 years (range 10-32 years). Fourteen of the 17 malignancies occurred in the facial structures and three developed in the lower extremities. The histologies included osteosarcoma (n = 5), malignant fibrous histiocytoma (n = 3), high-grade spindle cell sarcoma (n = 3), malignant mesenchymoma (n = 1), leiomyosarcoma (n = 4) and angiosarcoma (n = 1). The tumors were locally aggressive and had a similar appearance to those found in nonretinoblastoma patients. Six of the 14 patients are alive and disease free.Discussion. Most of the adolescent and young adult retinoblastoma survivors developed second malignancies in the irradiated facial structures but some occurred in distal sites. Radiologically, these tumors do not differ in appearance from those seen in non-retinoblastoma patients with the exception of their location.
Sarcoma 1997
PMID:The location and appearance of second malignancies in patients with bilateral retinoblastoma. 1852 Dec 7

Purpose. In a previous series of 25 human osteosarcoma samples studied for MYC gene amplification, we found amplification in two cases (8%), including one arising in association with Paget's disease (pagetic osteosarcoma). Based on this observation, we further investigated the prevalence of MYC gene amplification in pagetic osteosarcomas.Methods. MYC gene amplification was assessed by Southern blot analysis using frozen tissue samples in five cases of pagetic osteosarcoma and 53 cases of primary (non-pagetic) osteosarcoma. Amplification was considered present if the MYC copy number was six or greater.Results. Three out of five patients (60%) with pagetic osteosarcoma showed MYC gene amplification, whereas it was present in only 5/53 patients (9.4%) with primary osteosarcoma. The incidence of MYC amplification in pagetic osteosarcoma was thus significantly higher than that in primary osteosarcoma (p = 0.016).Discussion. The finding that MYC gene amplification may be more common in pagetic than primary osteosarcoma warrants further study and suggests pathogenetic differences between primary osteosarcomas and those arising in the setting of Paget's disease. Three of the four pagetic osteosarcomas from the present study were previously shown to be immunoreactive for p53, suggesting that p53 mutation may also be a frequent genetic lesion in these tumors.
Sarcoma 1997
PMID:Amplification of the MYC Gene in Osteosarcoma Secondary to Paget's Disease of Bone. 1852 Dec 14


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