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 definition of osteosarcoma requires that a malignant tumor of bone produce osteoid or bony matrix. With this as a basic definition, osteosarcoma of bone can be divided into several clinicopathologic entities based on clinical, roentgenographic, and pathologic features. The tumors can be broadly divided into those arising within the bone and those arising on the surface of bone. Most intraosseous osteosarcomas are high-grade malignant tumors that occur in children and adolescents. A small number occur in older patients, and they may be related to a preexisting condition, such as Paget's disease or radiation. The site of the lesion has prognostic importance. Osteosarcoma of the jaws is associated with an especially good prognosis, whereas the same kind of tumor involving the skull has a very poor prognosis. Most osteosarcomas of the surface of bone are well or moderately differentiated and are associated with an excellent prognosis.
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PMID:Osteosarcoma of bone. 973 64

The appearance of a sarcoma of bone is a well-recognized complication of Paget's disease. The most common type of such a sarcoma is osteosarcoma. Much less common are soft tissue lesions adjoining the pagetic bone that clinically and radiologically simulate sarcoma but histologically represent exaggerated periosteal bone formation as a manifestation of the basic pathologic process. We present a case of a bulky juxtacortical soft tissue mass in the thigh arising from a pagetic femur in a 62-year-old patient with polyostotic Paget's disease that was clinically and radiologically suspected to be a juxtacortical osteosarcoma. Microscopically, the lesion showed features of florid Paget's disease without any evidence of sarcomatous growth. It is important to be aware of this rare manifestation of Paget's disease to avoid unnecessary overtreatment.
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PMID:Pseudosarcoma in Paget's disease of bone. 1019 90

A 38-year-old male presented with an extremely rare primary osteogenic sarcoma, unassociated with Paget's disease or late effects of radiation, involving the sella and sphenoid sinus region. Complete excision of the tumor was achieved through an extended frontobasal approach. Postoperatively, six cycles of combination chemotherapy (adriamycin, ifosphamide, and cisplatin) followed by a total of 55 Gy local radiotherapy in 33 fractions was given. Primary osteogenic sarcoma should be considered in the differential diagnosis of the central skull base tumors. Osteogenic sarcoma, in general, has a bad prognosis, and should be managed aggressively with multimodality treatment including gross total surgical resection, combination chemotherapy, and radiotherapy.
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PMID:Primary osteogenic sarcoma involving sella-sphenoid sinus--case report. 1043 83

Sarcomatous transformation is the most dreaded complication of Paget's disease. We report on a case of post-Paget telangiectatic osteosarcoma of the skull, a variant of osteogenic osteosarcoma, in a 79-year-old woman. We discuss the radiological pattern in relationship to the differential diagnosis.
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PMID:Post-Paget telangiectatic osteosarcoma of the skull. 1048 18

The molecular mechanisms underlying Paget's disease and subsequent osteosarcoma formation are not well understood. In this study, we aim to delineate the function of the c-Fos oncogene in Paget's disease using transgenic mice, based on previous findings that c-Fos is highly expressed in Pagetic osteoclasts and that c-Fos is an essential gene for osteoclast differentiation and skeletal neoplasia. We have generated transgenic mice in which c-Fos is overexpressed specifically in osteoclasts using the tartrate-resistant acid phosphatase (TRAP) promoter, and five founder mice have been identified. All transgene-expressing animals developed severe bone remodeling lesions, some of which progressed to large bone tumors. Histopathologic analysis indicated that the lesions contained a marked increase in the number of osteoclasts that contained a large number of nuclei. Osteoclasts were identified by histochemical staining for TRAP and by in situ hybridization for matrix metalloproteinase-9 (MMP-9) expression. Moreover, transgenic osteoclasts, and in some cases, osteoblasts and chondrocytes, expressed high levels of c-Fos protein as judged by immunocytochemistry. This phenotype of increased osteoclast number and activity, together with an apparently high rate of bone turnover, resembles some characteristics of Paget's disease. These data therefore support an important function for c-Fos in the Pagetic phenotype, and further support the notion that this gene is important in osteoclastogenesis and in bone remodeling disorders.
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PMID:A putative role for c-Fos in the pathophysiology of Paget's disease. 1051 Feb 9

One of the most serious complications of Paget's disease is a significant increase in the incidence of osteosarcoma. Approximately 1% of Paget's patients develop osteosarcoma, an increase in risk that is several thousand-fold higher than the general population. This risk contributes significantly to the mortality and morbidity of Paget's disease patients. We examined several cases of pagetic and sporadic osteosarcoma for tumor-specific loss of constitutional heterozygosity (LoH) on chromosome 18q. Our analysis found that both pagetic and sporadic osteosarcoma tumors showed LoH for all or part of the distal portion of chromosome 18q. The pattern of LoH in both types of tumors identified a region between loci D18S60 and D18S42 that must contain the putative tumor suppressor locus. This region is tightly linked to familial Paget disease and familial expansile osteolysis (FEO). Our hypothesis is that the predisposition locus for Paget's disease and the tumor suppressor locus for osteosarcoma are either the same gene or that osteosarcoma in Paget's disease represents a deletion affecting two adjacent genes. In either model, localization of the osteosarcoma tumor suppressor gene would be of benefit in the eventual isolation of the predisposition locus for Paget's disease. We have begun to isolate and test candidate genes from within the region defined by both the familial Paget's disease families and the minimal region of LoH in osteosarcomas for evidence that one or more of them is responsible for predisposition to Paget's disease and/or osteosarcoma.
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PMID:Common mechanisms of osteosarcoma and Paget's disease. 1051 Feb 12

Pagetic sarcoma is a rare anaplastic malignancy with a peak incidence in the seventh and eighth decades of life; it usually occurs in patients with polyostotic Paget's disease. The most common tumor type is osteosarcoma. In one-third of the cases, presentation is a spontaneous pathologic fracture of an affected long bone. Amputation is the most appropriate form of surgical management in most cases because of the aggressive behavior of the sarcoma and its usually late presentation in this elderly population. However, selected patients with extremity lesions may be managed by pre- and postoperative chemotherapy and wide curative resection with limb salvage reconstruction. It is essential to differentiate pagetic sarcoma from metastatic carcinoma in pagetic bone and from a benign giant cell tumor.
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PMID:Surgical management of neoplastic complications of Paget's disease. 1051 Feb 13

Over a 25-year period we have treated 36 patients with osteosarcoma of the pelvis. Of the tumours, 24 (67%) were primary osteosarcomas and 12 (33%) arose either after irradiation or in association with Paget's disease. Six patients had a hindquarter amputation and 12 were treated by a limb-salvage procedure with intrapelvic excision. The five-year survival rate of all the patients with pelvic osteosarcoma was 18%, while for 17 treated by chemotherapy and surgery it was 41%. The prognosis for patients presenting with metastases or with secondary osteosarcoma was appalling and none survived after 29 months. No patient over the age of 50 years when seen initially survived for a year. Youth and a good response to chemotherapy along with complete surgical excision offer the best chance of cure.
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PMID:Osteosarcoma of the pelvis. 1053 Aug 39

Osteosarcoma is a malignant bone tumour of mesenchymal osteoid tissue origin. Osteosarcoma can be primitive, but it may also complicated a Paget's disease of bone. We described a 75yearold man with a right thigh mass discovered after a motoring accident. Histopathologic examination revealed the diagnosis of osteosarcoma. Plain film Xrays showed Paget's lesion of the right pelvis bone. CT and MRI allowed complete evaluation of this lesion. Final diagnosis was those of malignant transformation of Paget disease. The patient was refused for surgery in view of the size of the tumour and chimiotherapy followed.
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PMID:[Osteosarcoma manifesting as Paget's bone disease]. 1074 75

The differential diagnosis of an osteoblastic vertebral lesion (ivory vertebra) includes metastatic prostate cancer, lung cancer, lymphoma, osteosarcoma and Paget's disease. We report a case of a man who was initially diagnosed with Paget's disease on vertebral biopsy. He failed to respond to conventional bisphosphate therapy. The review of the original biopsy specimen showed metastatic carcinoid tumor involving the bone marrow. The various features of carcinoid tumors metastasizing to the skeleton are briefly reviewed.
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PMID:Case of an ivory vertebra. 1102 70


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