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)

Osteosarcoma is a fatal disease. Neoadjuvant chemotherapy combined with irradiation treatment provide a better survival. In the Faculty of Medicine, Ramathibodi Hospital, the overall 9 year survival probability was 55 per cent among 130 cases of more than Enneking stage II osteosarcoma. Between 15 cases of bony metastases, there were 2 cases which were classified as multifoci osteosarcoma or osteosarcomatosis. These two cases developed second bone disease, 32 and 38 months after initial diagnosis and survived for 84 and 88 months with one patient also developing pulmonary metastasis. Both of them are still alive and in very good health.
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PMID:7 year-survival of 2 cases of multicentric or metastatic osteosarcoma. 973 34

Multiple myeloma is associated with unbalanced bone remodeling causing lytic bone lesions. Interleukin-11 (IL-11) promotes osteoclast formation and inhibits osteoblast activity and may, thus, be one factor involved in cancer-induced bone destruction. We have previously shown that myeloma cells produce hepatocyte growth factor (HGF). We now report that HGF induces IL-11 secretion from human osteoblast-like cells and from the osteosarcoma cell lines Saos-2 and HOS. In coculture experiments, both the myeloma cell line JJN-3 and primary myeloma cells from 3 patients induced IL-11 secretion from osteoblasts, whereas no induction was observed with the non-HGF producing myeloma cell line OH-2. Enhanced IL-11 induction was observed with physical contact between osteoblasts and myeloma cells as compared with experiments in which contact was prohibited by tissue inserts. Anti-HGF serum strongly reduced the myeloma cell-induced IL-11 secretion. Furthermore, we show that JJN-3 cells express HGF on the cell-surface. Removal of surface-bound HGF on JJN-3 cells reduced IL-11 production induced in cocultures. Transforming growth factor beta1 and IL-1 potentiated the effect of HGF on IL-11 secretion, whereas an additive effect was observed with tumor necrosis factor. Thus, myeloma-derived HGF can influence the bone marrow environment both as a soluble and a surface-bound factor. Furthermore, HGF emerges as a possible factor involved in myeloma bone disease by its ability to induce IL-11.
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PMID:Hepatocyte growth factor (HGF) induces interleukin-11 secretion from osteoblasts: a possible role for HGF in myeloma-associated osteolytic bone disease. 1057 4

Paget's disease of bone (PDB) is one of the most common bone disorders in the western world. PDB is characterized by focal areas of increased osteoclastic bone resorption and bone formation, which leads to the formation of poorly structured bone. These abnormalities of bone turnover and structure predispose affected individuals to various complications including bone pain, deformity, pathological fracture, and an increased risk of osteosarcoma. One of the main mechanisms of osteoclast formation and activation involves the receptor activator of nuclear factor -kappaB (RANK)/RANK ligand (RANKL)/osteoprotegerin (OPG) pathway, where binding of RANKL to RANK results in the differentiation of osteoclast precursors. OPG, on the other hand, acts as an inhibitor of osteoclastogenesis by serving as a decoy receptor for RANKL. Recently, mutations in the RANK gene have been shown to cause familial expansile osteolysis, a rare bone disorder showing great similarity to PDB. We performed mutation analysis in the RANK and OPG genes in 28 PDB patients to investigate whether mutations in these genes could be responsible for PDB. Our data suggest that RANK is not directly involved in PDB in our set of patients, as no mutations in the RANK coding region could be identified and allele frequencies of RANK polymorphisms did not differ in PDB patients as compared with the random population. Also, in the OPG gene, we could not detect PDB-causing mutations. However, of the several polymorphisms identified, one (400 + 4 C/T in intron 2), showed a statistically significant increased frequency for the C allele in PDB patients, suggesting that individuals harboring this allele may be more susceptible for developing PDB.
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PMID:Evaluation of the role of RANK and OPG genes in Paget's disease of bone. 1116 49

Novel imaging of the fine structures of the ribs of a pig and a specimen of human osteosarcoma utilizing the spatial coherence of X-rays was successfully performed with an incident X-ray energy of 30 keV at SPring-8, Japan. The image contrast appearing at the periphery of trabecular bone, small calcifications and small fractures is caused by the phase shift of the X-rays at the boundary of these objects that have different X-ray refractive indices. The image is recorded on mammography film without an intensifying screen. Comparison of the image contrast using different sample-to-film distances, Z, such as Z = 5 m and Z approximately 0 m, showed that the former images were always more informative, i.e. better in resolution and/or image contrast when imaging trabecular bone, bone marrow and small fractures in compact bone, and for imaging cartilage. Radiography using synchrotron X-rays for phase objects should be a powerful tool for diagnosis in orthopedics, especially for bone disease.
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PMID:Application of synchrotron X-ray imaging to phase objects in orthopedics. 1197 68

Fibrous dysplasia is a rare bone disease that can be misdiagnosed because of the slowness of its symptoms. Although it is a benign disease its clinical behaviour can be severe, with possibility of local growth and malignant degeneration into osteosarcoma. An uncommon case of fibrous dysplasia is reported, affecting only the middle turbinate. Diagnostic and therapeutic problems are discussed.
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PMID:[An exceptional form of cranio-facial fibrous dysplasia: fibrous dysplasia of middle turbinate]. 1218 6

Benign and malignant tumors of bone often have common musculoskeletal manifestations mimicking rheumatic disorders. The detection and resolution of mimicking symptoms require knowledge, skills, and a problem-solving attitude for musculoskeletal disorders. Before engaging in an extensive investigation, a careful history and full physical examination must be done. This review addresses the recent literature from June 2001 to May 2002 on musculoskeletal manifestations of benign and malignant tumors of bone using "red flag" rubrics: nonspecific pain pattern, atypical soft-tissue or bony swellings, pathologic fracture, spinal paresis, osteolytic x-ray findings, and unexpected results of laboratory tests. Early diagnosis (appropriate use of imaging techniques) and multidisciplinary management have improved considerably the survival of patients with primary malignant bone disease (eg, osteosarcoma). For some benign bone tumors (eg, osteoid osteoma), interstitial laser photocoagulation is now the treatment of choice with a success rate comparable with that of other treatments.
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PMID:Musculoskeletal manifestations of benign and malignant tumors of bone. 1249 13

We describe four individuals of an African-American family with a predominantly diaphyseal bone disease associated with familial gigantiform cementoma (FGC), a disorder typically seen in Caucasians. The mother and her children presented with deformities of the jaws, abnormalities of the long bones, and pre-pubertal pathologic fractures. The index patient carried the diagnosis of osteosarcoma (OS). In addition, we provide a possible explanation for the jaw abnormalities of King Tutankhamen's father in the 18th dynasty in Egypt around 1350 BC.
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PMID:Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma: a possible explanation of an ancient mystery. 1560 17

The oncophilic complex of technetium-99m labeled pentavalent dimercaptosuccinic acid (99mTc(V)-DMSA) has been successfully used for the detection of primary and metastatic medullary thyroid cancer and for imaging various soft tissue tumors like lung, brain and prostate cancer. In this article, the role of 99mTc(V)-DMSA in the diagnosis of the primary tumor and metastases of osteosarcoma patients as compared to the 99mTc-MDP scan and the CT scan was studied. Twenty-eight patients with bone disease were referred to the Nuclear Medicine Department of Saint Savas Oncology Hospital in Athens from the Orthopedics Department of the same Hospital. From them, 18 (Group A) had osteosarcoma, 7 (Group B) osteomyelitis and 3 (Group C) bone fractures. The final diagnosis was made after fine needle aspiration biopsy. All patients were subjected to the 99mTc(V)-DMSA scan, the standard bone scan (99mTc-MDP) and CT scan. Group A patients showed a selective uptake of 99mTc(V)-DMSA in the primary tumor region. No abnormal 99mTc(V)-DMSA uptake was observed in the patients of Groups B and C. The 99mTc(V)-DMSA scan was found to be superior to the 99mTc-MDP and the CT scans in identifying metastases of osteosarcoma. Sensitivity was 100%, 86% and 98% respectively.
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PMID:The role of 99mTc(V)-DMSA scan as compared to 99mTc-MDP and CT scans in imaging the primary tumor and metastases of osteosarcoma. 1933 Jan 92

Bisphosphonates are known to inhibit osteoclast-mediated bone resorption and osteoblast differentiation and are currently used in the treatment of Paget's disease, osteoporosis, metastatic and osteolytic bone disease and hypercalcaemia of malignancy. The parathyroid hormone-related peptide (PTHrP) and type 1 PTH/PTHrP receptor (PTH.1R) bioregulation systems mediate a wide range of local paracrine/autocrine and intracrine functions in various tissues and modify the actions of pharmaceutical agents on target tissues, both in vivo and in vitro. In addition, bone microenvironment-related growth factors, such as insulin-like growth factor 1 (IGF-1), transforming growth factor beta 1 (TGF beta 1), basic fibroblast growth factor (bFGF) and interleukin 6 (IL-6), can modify the actions of various pharmaceutical agents, including cytotoxic drugs in malignant cell lines. Whether IGF-1, TGF beta 1, bFGF, IL-6 and zoledronic acid affect the expressions of PTHrP and PTH.1R in MG-63 osteoblast-like osteosarcoma cells was investigated in this study. Relative quantitative-PCR (expression at mRNA level) and immunofluorescence analysis (localization of the expression at protein level) were employed to assess PTHrP and PTH.IR expressions. Our data showed that primarily IGF-1, TGF beta 1 and IL-6 (up to 25 ng/ml for 48 h) increased PTHrP mRNA expression and modified its perinuclear localization, while zoledronic acid (up to 100 microM for 48 h) inhibited cell proliferation and suppressed PTHrP expression in the MG-63 osteosarcoma cells. These growth factors were incapable of reversing the zoledronic acid decrease of the expression of PTHrP in the MG-63 cells, suggesting that zoledronic acid and the growth factors affect PTHrP expression via an independent intracellular signal transduction pathway in these cells. However, no appreciable modulation of the PTH.1R expression by IGF-1, TGF beta 1, bFGF, IL-6 or zoledronic acid was detected in MG-63 cells. Therefore, we conclude that PTHrP expression possibly mediates the action of bone microenvironment-related growth factors and of zoledronic acid in MG-63 cells.
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PMID:Bone-related growth factors and zoledronic acid regulate the PTHrP/PTH.1 receptor bioregulation systems in MG-63 human osteosarcoma cells. 1647 8

Historically, the etiology of local bone pathologies, such as avascular necrosis, has been related to intravascular occlusion. Recent reports have highlighted the occlusion of arteries, venules, and/or capillaries in bone tissue. Endothelium of bone presumably participates locally in the formation of the microvascular thrombosis. It is also known that endothelial cells (ECs) play a central role in angiogenesis, a process seen in osteosarcoma, amongst other bone diseases. Given the well-recognized heterogeneity of ECs throughout the body, investigations of local bone disease related to endothelium processes may be more appropriately targeted on bone ECs rather than other primary ECs or an immortalized EC line. In the current study, mechanical and enzymatic methods are described to isolate ECs from cancellous human bone tissue followed by immunomagnetic bead separation to purify the cell populations. The human bone-derived endothelial cells (hBDECs) were characterized based on endothelial cell antigen expression and functional assays. This study is the first report of isolation and expansion of ECs from human bone tissue. Isolation of hBDECs in human vascular bone diseases may facilitate the study of the molecular and/or genetic abnormalities in the vasculature system that contributes to the initiation and/or progression of the disease.
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PMID:Isolation and characterization of human bone-derived endothelial cells. 1749 68


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