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)

A through-knee amputation for suspected osteogenic sarcoma was performed on a patient with Paget's disease of the tibia. Plasma alkaline phosphatase (AP) activity fell to normal values, apparently as a single exponential function of time, with a half-life of 1.7 days. The daily turnover of bone-derived plasma AP was estimated to be approximately 200 I.U. from the Pagetic tibia and 20 I.U. from the rest of the skeleton.
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PMID:An estimate of the turnover rate of bone-derived plasma alkaline phosphatase in Paget's disease. 117 95

The authors report 12 cases of Paget's disease with sarcomatous degeneration that were observed among 311 patients with Paget's disease hospitalized in the Montpellier Rheumatology Clinic. They compare their experience with information they were able to extract from the literature in French and English. The frequency of such degeneration in cases of Paget's disease is difficult to determine but did not appear to exceed 1%. Degeneration rarely occurred before the age of 50 years, affected men twice as frequently as women, and occurred particularly in cases of diffuse Paget's disease, mainly in the femur or the humerus; the rachis was rarely affected. Pain was the main symptom, was practically constant, was remarkable because of its permanence and its intensity. Tumefaction was frequently seen. Pathological fractures were seen in almost a third of the patients with sarcomas of the long bones. Radiculo-medullary compression characterized the rare cases with involvement of the vertebral column or the sacrum. Radiography showed rupture of cortical layers with invasion of the soft parts without any periostal reaction. Histological investigation confirmed the diagnosis by demonstrating one of the three types of osteogenic sarcoma: osteosarcomas were the most frequent. Less frequently giant-cell sarcomas or reticulosarcomas were found. The affected patients nearly always died, survival at five years being rare. Treatment, amputation or radiotherapy, was disappointing.
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PMID:[Sarcomas associated with Paget's disease]. 122 55

Malignant change in Paget's disease of bone usually has the histological features of osteosarcoma, but follows a different clinical course from cases not associated with paget's disease of bone. Malignant fibrous histiocytoma is an uncommon neoplasm which may be associated with pagetic bone and its clinical course is not well defined. Seven cases of malignant fibrous histiocytoma in Paget's disease were reviewed from two Australian bone tumour registries. In stage IIB and III disease there was a three year survival rate of 57%, suggesting a better prognosis than for patients with other sarcomas in Paget's disease.
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PMID:Malignant fibrous histiocytoma in Paget's disease of bone. A report of seven cases. 131 2

Secondary tumors involving the temporal bone are comparatively rare. We report a case of secondary osteosarcoma presenting in the temporal bone which arose from a primary lesion in the tibia in a patient with Paget's disease. To our knowledge secondary osteosarcoma of the temporal bone has never previously been described.
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PMID:Metastatic spread of osteosarcoma to the temporal bone in a patient with Paget's disease: a case report. 158 4

The purpose of this study is to review survival, treatment methods and criteria for diagnosis of osteosarcoma at Royal Prince Alfred Hospital during two periods in the last 50 years. The records of 22 patients diagnosed with osteosarcoma and nine with osteosarcoma in Paget's disease (from 1939 to 1950) were reviewed. All but one had died within three years. One patient survived six years. The second series was taken from 1983 to 1990. Forty-nine patients, including three with Paget's sarcoma, were studied. Probability of survival was estimated by actuarial analysis using Kaplan-Meier curves. Overall survival was estimated at 45%. Those patients who were free of metastatic disease at the conclusion of their treatment were estimated to have a probability of survival of 85%.
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PMID:Osteosarcoma: then and now. A fifty year review at Royal Prince Alfred Hospital. 173 37

Serum osteocalcin did not show any response to the onset of osteosarcoma in Paget's disease of bone whereas serum alkaline phosphatase increased rapidly. This suggests that osteocalcin is not useful in the diagnosis and management of Paget's osteosarcoma and does not reflect the same osteoblastic processes in bone as serum alkaline phosphatase.
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PMID:Differential response of serum alkaline phosphatase and serum osteocalcin in Paget's osteosarcoma. 185 60

This report describes a family of three siblings with a long history of polyostotic Paget's disease. Because of the severe osseous involvement with Paget's disease, all three patients had problems that required orthopedic surgery. Subsequent to these surgeries, two patients developed osteogenic sarcoma at sites unrelated to their past procedures, one in the sacrum and one in the calvarium. Both patients died shortly after their diagnosis because of the aggressive spread of the tumor. Although the etiology of Paget's disease and its complication of osteogenic sarcoma still remain to be clarified, this and other case reports suggest a possible environmental or hereditary contribution to developing osteogenic sarcoma in Paget's disease. Patients with a familial clustering of polyostotic Paget's disease may benefit from more thorough screening tests to detect malignant transformation.
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PMID:Familial incidence of Paget's disease and secondary osteogenic sarcoma. A report of three cases from a single family. 200 72

Osteosarcoma associated with Paget's disease is very malignant and has an extremely severe prognosis. This is also related to the usually advanced age of the patient and to the consequent impossibility of carrying out suitable chemotherapy. The case presented here is exceptional because of the young age of the patient, which allowed us to use a neoadjuvant chemotherapy protocol.
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PMID:Osteosarcoma associated with Paget's disease: a description of a rare case. 209 33

A human osteosarcoma cell line derived from cells obtained from a patient with Paget's disease is shown to synthesize and secrete bone Gla protein (BGP); (osteocalcin), a noncollagenous bone matrix protein. Using a human BGP-specific RIA, we show that the human osteosarcoma cells synthesize significant amounts of BGP without any prior induction of BGP synthesis by 1,25-dihydroxyvitamin D. After specific immunoprecipitation of poly-A+ RNA in vitro translation products with antibodies to BGP, we found that BGP is synthesized as a precursor with an apparent mol wt of 13.5K, as demonstrated on 15% sodium dodecyl sulfate-polyacrylamide gels. Finally, pulse labeling of the osteosarcoma cells with [3H]proline reveals that the cells synthesize mature BGP of 12,000 mol wt as well as a higher mol wt precursor (13,500) of the protein.
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PMID:Constitutive biosynthesis of bone Gla protein in a human osteosarcoma cell line. 241 Feb 38

Since 1930, 117 patients with well-documented osteogenic sarcoma of the skeleton and the soft tissues occurring in patients older than 60 years have been diagnosed and treated at Memorial Hospital for Cancer and Allied Diseases. This number represents approximately 10% of all osteogenic sarcomas treated at this Medical Center. Slightly more men than women (68 versus 49, respectively) were affected, with a mean and a median age of 68 and 66 years, respectively, ranging from 60 to 86 years of age. One hundred one lesions occurred in the skeleton and 16 in extraosseous sites. In contrast to osteogenic sarcoma in children and adolescents, where more than half of the tumors arise in the fastest-growing bony sites around the knee, this region was involved in only 14.5% of the older patients. In this latter age group, the axial skeleton was the most commonly affected (27%), in addition to the craniofacial bones (13%) and extraskeletal sites (11%). Contrary to expectations, the lytic destructive skeletal lesions were three times more common than any other radiographic presentation, with the fibrous (38%), the osteoblastic (25%), and the cartilaginous (17%) sarcoma variants predominating over all others on histologic examination. Whereas 97% of osteogenic sarcomas occurring in patients younger than 21 years arise anew, without any pre-existent osseous disease, in this study's older population, sarcomas were more frequently (56%) secondary to other bony conditions, such as Paget's disease, or followed irradiation. Five-year survival estimates were strongly dependent on whether the patients had primary de novo osteogenic sarcomas (37%) or whether the sarcomas arose secondary to other skeletal conditions (7.5%). There are no significant survival differences between patients treated before or after 1974, when adjuvant and neoadjuvant chemotherapy started to make its positive impact in the treatment of osteogenic sarcoma in children and adolescents.
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PMID:Osteogenic sarcoma of bones and soft tissues in older persons. A clinicopathologic analysis of 117 patients older than 60 years. 241 41


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