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 usually treated with intensive preoperative and postoperative chemotherapy and wide tumor resection, resulting in a 60% to 70% 5-year survival rate. Caffeine has a DNA-repair inhibiting effect. We therefore investigated the impact of caffeine given in conjunction with chemotherapy and limb-sparing surgery on survival and local tumor control in patients with nonmetastatic, high-grade osteosarcoma. Twenty-two patients were given 3 to 5 preoperative courses of intra-arterial cisplatin (120 mg/m2, 1 to 2 hours) and caffeine (1.5 g/m2/day x 3 days) with or without doxorubicin (30 mg/m2/day x 2 days). Following this treatment, limb-sparing surgery was performed by means of intentional marginal excision aiming at preservation of important structures such as major neurovascular bundles, tendons, ligaments and the epiphysis. Three courses of cisplatin and doxorubicin combined with caffeine, and high-dose methotrexate with vincristine and citrovorum factor rescue were given intravenously as postoperative chemotherapy for 21 patients and three courses of high-dose methotrexate and combination of ifosfamide, etoposide and methotrexate for 1 patient. Following the preoperative chemotherapy, there were no viable tumor cells in 19 patients, only scattered foci of viable cells in 2 patients, and some areas of viable tumor cells in 1. The 21 patients with a good chemotherapeutic response on radiographs underwent minimized marginal excision. Functional evaluation of the affected limbs was excellent for 17 patients, good for 3, fair for 1, and poor for 1. No local tumor recurrence was seen in this series. Eighteen patients remain disease-free with a mean follow-up of 61 months. Two patients died of metastatic disease, 1 died of chemotherapy-related complications, and 1 died of unknown causes. The overall 5-year cumulative survival rate was 90%, and the 5-year event-free survival rate was 75%. Chemotherapeutic caffeine enhanced tumor necrosis and improved the success rate of limb-sparing surgery using marginal procedure without any adverse impact on survival. The results of our limited clinical trial appear to justify further prospective, multicenter randomized trials of the benefits of caffeine combined with chemotherapy for nonmetastatic osteosarcoma and other malignant neoplasms.
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PMID:Caffeine-assisted chemotherapy and minimized tumor excision for nonmetastatic osteosarcoma. 958 49

The authors report on intentional marginal excision for osteosarcoma in conjunction with caffeine assisted chemotherapy for the purpose of preservation of good limb function. Twenty-seven patients with osteosarcoma (22 patients with Stage IIB and five with Stage IIIB) preoperatively were given three-to-five courses of intraarterial cisplatin and caffeine without or with doxorubicin. For 26 (96%) responders to the chemotherapy, limb salvage surgery was conducted by means of an intentional marginal procedure, which led to the preservation of important structures such as major neurovascular bundles, tendons, ligaments, muscles, and the epiphysis. Tumors were located in the distal femur in 11 patients, the proximal tibia in eight, the proximal fibula in four, the proximal humerus in two, and the proximal femur in one patient. The histologic response of these 26 patients to the preoperative chemotherapy showed no viable cells in 19 patients with Stage IIB osteosarcoma and only scattered foci of viable cells in two patients with Stage IIB and five patients with Stage IIIB osteosarcoma. As for reconstruction, distraction osteogenesis was performed in eight patients, allograft or autoclaved bone and prosthesis composite in four, autoclaved bone in two, osteochondral allograft in two, megaprosthesis in six, and resection alone in four patients. The average functional evaluation of the 26 patients was 91% of normal. Local tumor recurrence was seen in one patient, whereas 18 patients with Stage IIB osteosarcoma remain diseasefree with a mean followup of 61 months. Two patients with Stage IIB osteosarcoma and four patients with osteosarcoma Stage IIIB died of the disease. Intentional marginal excision for osteosarcoma in conjunction with caffeine assisted chemotherapy is advantageous because it results in the preservation of healthy important structures, with joint preservation possible in selected cases. This approach should help to improve the success rate of limb salvage surgery for osteosarcoma and to preserve the function of the affected limb.
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PMID:Marginal excision for osteosarcoma with caffeine assisted chemotherapy. 997 73

Giant cell tumor of bone (GCT) is a rare primary osteolytic tumor of bone that is characterized by massive tissue destruction at the epiphysis of long bones. There is no evidence that tumor cells themselves are capable of bone destruction; instead, it appears that the tumor cells of GCT act by promoting osteoclastogenesis and, as a consequence, osteoclastic bone resorption. However, the mechanism by which this is achieved is not understood. Here we attempted to determine whether osteoprotegerin ligand (OPGL), the factor that is necessary and essential for osteoclastogenesis, is involved in tumor cell-recruited osteoclast-like giant cell formation in GCT. Using fluorescence in situ hybridization, we sought to determine mRNA expression of OPGL, its receptor RANK, and its decoy receptor OPG in three major cell types of GCT. We demonstrated that OPG mRNA was expressed in all three cell types of GCT, OPGL transcripts were mainly detected in spindle-shaped stromal-like tumor cells, whereas RANK was expressed only in macrophage-like mononuclear cells and multinuclear osteoclast-like giant cells. By semiquantitative RT-PCR, we also showed that the level of OPGL mRNA in GCT is much higher than that in normal bone and osteogenic osteosarcoma. In contrast, a similar level of OPG transcripts was detected in these three kinds of tissues, and RANK mRNA was detectable only in GCT tissues. We have further examined the regulation of gene expression of OPGL and OPG in tumor cells in response to osteotropic hormones. Administration of 1,25(OH)(2)D(3) and dexamethasone resulted in maximum up-regulation of OPGL level and down-regulation of OPG level in cultured GCT stromal-like tumor cells and the mouse bone marrow-derived ST-2 stromal cell line. Furthermore, we have shown that tumor cells of GCT induce differentiation of RANK-expressing myeloid RAW(264.7) cells into osteoclast-like cells in the presence of 1,25(OH)(2)D(3) and dexamethasone. Our findings suggest that OPGL is involved in the tumor cell-induced osteoclast-like cell formation in GCT. The ratio of OPGL/OPG by tumor cells may contribute to the degree of osteoclastogenesis and bone resorption.
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PMID:Gene expression of osteoprotegerin ligand, osteoprotegerin, and receptor activator of NF-kappaB in giant cell tumor of bone: possible involvement in tumor cell-induced osteoclast-like cell formation. 1070 90

Fourteen boys (56%) and 11 girls (44%) 4 to 17 years of age (mean, 12.2 years) who had osteosarcoma and open epiphyseal plates were studied. A possible correlation between transepiphyseal spread of osteosarcoma and radiologic and histopathologic findings was investigated. Epiphyseal plate invasion was detected radiologically in only 11 patients (44%), whereas histopathologic examination showed transepiphyseal extension in 21 patients (84%). The authors conclude that the epiphyseal plate is not a barrier against tumor growth and strongly recommend that limb salvage surgery preserving the epiphysis be planned carefully.
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PMID:Epiphyseal plate involvement in osteosarcoma. 1081 Apr 60

Clear-cell chondrosarcoma is a rare, low-malignant bone tumor. The lesion most commonly occurs in adults, generally in the 3rd or 4th decade. Clear-cell chondrosarcoma has a predilection for the epiphyses of long tubular bones, particularly the femoral head. Radiologically, it is a sharply defined radiolucent lesion. Histologically, it is characterized by large tumor cells with distinct boundaries and a clear cytoplasm. In addition to areas of conventional chondrosarcoma, there are partially mineralized trabecular osteoid formations. On the basis of 16 cases of clear-cell chondrosarcoma, we present the observations of the Hamburg Bone Tumor Register. The cases were examined according to age distribution, location, and radiological and pathomorphological criteria. The proximal femur was the most frequent location; rare manifestations in the rib, os ilium, and distal phalanx of the hand were also present. In 50% of the cases high-malignant osteosarcoma or conventional chondrosarcoma was considered. In addition to the epiphysis of the proximal femur, clear-cell chondrosarcoma can also involve other, rather unusual sites in the skeleton. Knowledge of the histomorphology of this tumor is therefore of crucial importance for the diagnosis.
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PMID:[Clear-cell chondrosarcoma]. 1114 25

We report an osteoblastoma-like osteosarcoma in the right proximal fibula in a 22-year-old woman. Radiographs showed an irregular osteolytic lesion from the metaphysis to the epiphysis in the proximal fibula with partial destruction of cortical bone. Tissue from a biopsy indicated a typical osteoblastoma. Curettage and bone graft was performed. One year after the surgery, local recurrence occurred, and a wide excision was performed. Histological examination of the en-bloc surgical specimen revealed the tumor had permeated through the host bony trabeculae, although the nuclear atypia was not marked. Immunohistochemical expression of MIB-1 was detected in 9.0% of cells.
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PMID:Osteoblastoma-like osteosarcoma. 1120 Oct 36

This paper introduces an innovative technique of highly conservative limb-saving surgery for juxta-articular osteosarcoma. This technique consists of marginal tumour excision, joint preservation and reconstruction by distraction osteogenesis. Ten patients, with a mean age of 19.5 years and high-grade osteosarcoma, underwent this procedure. The distal femur and proximal tibia were affected in five patients each. After effective pre-operative chemotherapy, the tumour was excised with preservation of the epiphysis, the articular surface and the maximun amount of healthy soft tissue. This was followed by application of an external fixator. Bone transport was performed for seven patients and shortening-distraction for three. The limb function was rated excellent in seven patients, good in one and fair in two. At the final follow-up, three patients were dead after a mean of 25.3 months while seven patients remained free of disease with a mean follow-up of 55.4 months. Joint preservation and biological reconstruction through distraction osteogenesis can produce excellent and long-lasting functional results.
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PMID:The natural limb is best: joint preservation and reconstruction by distraction osteogenesis for high-grade juxta-articular osteosarcomas. 1217 29

In 11 patients juxta-articular osteosarcoma around the knee was treated by intraepiphyseal excision of the tumour and reconstruction of the bone defect by distraction osteogenesis. Preoperative and postoperative chemotherapy was given to eight patients with high-grade tumours. The articular cartilage of the epiphysis and a maximum of healthy soft tissues were preserved. Distraction osteogenesis was then carried out. The mean gain in length was 9.7 cm. Full function of the limb was preserved in all except one patient, with a mean follow-up of 53.8 months. Treatment of juxta-articular osteosarcomas around the knee with joint preservation and biological reconstruction using distraction osteogenesis can give excellent functional results.
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PMID:Osteosarcoma around the knee. Intraepiphyseal excision and biological reconstruction with distraction osteogenesis. 1246 63

Conventional osteosarcoma and Ewing sarcoma are the most common childhood malignant tumors of bone. These tumors arise in the metaphysis and diaphysis of long bones and may extend secondarily into the epiphysis. Primary epiphyseal location for these tumors is rare and may constitute a diagnostic and therapeutic challenge. The authors report two skeletally immature patients with an osteosarcoma and Ewing sarcoma limited to the epiphysis. Clinical, radiographic and histopathologic findings are discussed.
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PMID:Epiphyseal primary location for osteosarcoma and Ewing sarcoma in patients with open physis. 1282 57

We report a complicated case of osteosarcoma of the proximal tibia. A 15-year-old boy was referred to us and underwent distraction osteogenesis reconstruction. We administered preoperative chemotherapy for five cycles. Clinical response was determined to be complete by radiography. Marginal excision was then performed with preservation of the proximal tibial epiphysis. Metaphyseal reconstruction (type 2) was performed with distraction osteogenesis. Postoperative chemotherapy also was administered for five cycles. Two years later, the patient developed a deep infection. He underwent curettage and a pedicle peroneal flap transfer, which did not cure the infection. Infected tissues were excised, and shortening-distraction was carried out with the Ilizarov frame. The infection was cured; however, a leg length discrepancy and deformity resulted from frame instability. Four years after the initial operation, the patient fractured his reconstructed leg in a traffic accident. We performed osteosynthesis, deformity correction, and lengthening with the Ilizarov method. We were able to correct the defects using distraction osteogenesis, eventually restoring normal function. Epiphyseal preservation and reconstruction by distraction osteogenesis can provide an excellent outcome, resulting in a stable reconstruction that functionally restores the native limb. Distraction osteogenesis avoids some complications but may involve others, which require detection and appropriate management.
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PMID:Successful management of complications from distraction osteogenesis after osteosarcoma resection: a case report. 1622 85


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