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 New South Wales Bone Tumour Registry records for malignant fibrous histiocytoma were reviewed. Thirty-eight cases were available for analysis, representing 8% of the 506 malignant bone tumours reported to the Registry over 15 years (1977-91). The review confirmed that malignant fibrous histiocytoma is a high-grade primary bone sarcoma occurring in a wide age range in a bimodal distribution. Tumours mainly occurred around the knee or the ends of other long bones. Twenty-six per cent of cases arose in a pre-existing lesion, 4 cases post-radiotherapy, 3 cases in Paget's disease, 2 cases in bone infarcts and 1 case each in osteoblastoma, osteosarcoma and giant cell tumour. Thirty-two patients were treated by amputation or wide excision and in 11 patients this was preceded by intralesional curettage. Three patients had marginal resections and three had no surgery. Five year survival was 53% overall. Age at presentation significantly affected survival; patients under 55 years had a 5 year survival rate of 70%, compared with 19% for patients over 55 (P < 0.05). Survival was 86% at 5 years if the initial procedure was radical or wide, but only 30% if marginal or intralesional (P < 0.01). Adjuvant chemotherapy as an isolated factor did not affect survival. Rigorous adherence to the principles of surgical staging and biopsy was found to be the most successful way to treat malignant fibrous histiocytoma of bone.
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PMID:Malignant fibrous histiocytoma of bone: the experience of the New South Wales Bone Tumour Registry. 838 25

Presented is a case report of osteosarcoma of the maxilla in a patient with Paget's disease. This case is unusual because of recurrent benign soft tissue enlargements at the site of eventual malignant disease.
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PMID:Recurrent enlargement of the left maxillary alveolus. 849 6

A 69 year old Japanese woman was hospitalized for emergency treatment of sudden onset of tetraplegia and somnolence. The patient had a long history of occipital pain without definite diagnosis. After admission, the patient progressively developed generalized palsy including respiratory paralysis, and died of bronchopneumonia. Autopsy revealed osteosarcoma of the cervical vertebrae with the features of Paget's disease involving the skull and the cervical vertebrae. Paget sarcoma is rare in Japan, where Paget's disease of the bone is an uncommon condition. A review of the world literature failed to reveal any reports describing Paget sarcoma of the cervical vertebrae. The present report indicates that the development of Paget sarcoma in the upper cervical vertebrae may cause life-threatening neurologic complications.
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PMID:Paget sarcoma of the cervical vertebrae: an autopsy case report and review of the literature. 858 Nov 54

Osteocalcin is one of the most abundant noncollagenous proteins found in adult bone. It is a highly conserved gamma-carboxyglutamic acid-containing protein that is believed to be produced exclusively by osteoblasts. In this study, intracellular and extracellular localization of osteocalcin in osteosarcoma was examined with anti-osteocalcin antibody and in situ hybridization using a synthetic oligonucleotide. Immunohistochemically, osteoblastic osteosarcomas were all positive for osteocalcin. The chondroblastic osteosarcomas were positive on the neoplastic chondrocytes. The five fibroblastic osteosarcomas out of seven were positive for osteocalcin immunostaining over the neoplastic spindle cells. Five cases of osteoblastic osteosarcomas out of seven were positive for osteocalcin in situ hybridization. Two cases of chondroblastic osteosarcomas and three cases of fibroblastic osteosarcomas were positive for in situ demonstration of osteocalcin. The malignant tumor giant cells were positive for osteocalcin immunostaining 83%. They were also positive for in situ hybridization. The benign giant cells in five giant cell tumors and five aneurysmal bone cysts were negative for osteocalcin immunostaining. The benign giant cells in three chondroblastoma and three Paget's disease were positive for osteocalcin. In this study, the osteocalcin in situ hybridization and immunostaining has very important meaning for making differential diagnoses of, especially giant cell rich bone forming tumors.
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PMID:Osteocalcin expression in primary bone tumors--in situ hybridization and immunohistochemical study. 859 6

Mandibular osteosarcoma is a rare neoplasm, appearing in young adults as a mass often accompanied by pain, occasionally with paraesthesias, gingival haemorrhages and mobility of teeth, and rarely with ulceration of the skin or mucosa. Roentgenological lytic and/or sclerotic findings are non-specific. Some patients have a previous history of Paget's disease, antecedent radiation treatment, or fibrous dysplasia. Malignant osteoid production can be minimal, resulting in diagnostic difficulties. We report a case of a 33 year-old woman, who, in the absence of a clinical soil associated with the condition, was presented with advanced disease. Immunohistochemical study using alpha-actin smooth muscle antibody disclosed bizarre tumor cells with "dendritic" appearance, to our knowledge, never described thus far. This report draws attention to this uncommon neoplasm and reviews the pertinent literature related to this entity.
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PMID:Mandibular osteosarcoma with unusual expression of alpha-actin smooth muscle antibody. 869 15

The incidence of Paget's disease in Germany is about 3%. Up to 10% of all affected individuals develop secondary osteosarcomas in the affected bone areas. Compared to primary osteosarcoma patients with Paget's sarcoma differ in regard to age, tumor localisation, therapy, prognosis and radiological symptoms. The following case report describes clinical features, therapy and the typical radiological findings.
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PMID:[Secondary osteosarcoma in Paget disease]. 892 52

Death certification data has shown that death rates due to Paget's disease of bone and osteosarcoma in older people (assumed to be attributable to Paget's) declined in the latter part of the 19th and in the early 20th century, suggesting that there may be a secular trend toward less severe disease. We have reviewed a 21 year experience in a clinic specializing in Paget's disease. Data from all 1041 patients attending the clinic in this period were reviewed. Despite an increase in the susceptible population and an increased rate of referral to the clinic over this time (p = 0.012), there was a fall in the absolute numbers of patients referred with severe disease, as judged by the initial plasma alkaline phosphatase activity at presentation. In the years 1973-1978 the initial plasma alkaline phosphatase was > 500 U/L in an average of 22 new patients per year and > 1000 U/L in 12 per year. In the years 1988-1993, the figures were 12 and 3 per year, respectively. During this period, there were no other facilities offering scintigraphy or intravenous treatment for Paget's disease in the Auckland region, making it unlikely that patients with severe disease were being seen and treated elsewhere. The average age of newly referred patients rose steadily from a mean 62 years, in 1971-1973 to 71 years in 1991-1993 (p < 0.001). 534 subjects had scintiscans (52%) from which the extent of skeletal involvement was calculated. Skeletal involvement showed a significant negative correlation with year of birth (p < 0.01) but not with age or year of presentation. The proportion of patients with > 20% skeletal involvement had fallen by a third in the cohort born after 1926, compared to the cohort born before 1915. Our data demonstrate that, on average, newly referred patients with Paget's disease have less severe disease and are significantly older at diagnosis than was the case two decades ago.
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PMID:Evidence for secular change in Paget's disease. 898 50

Pathological fracture in histologically proven post-Paget osteosarcoma of the humerus is a rare complication. Due to individual requests as well as age and comorbidity, a course of primary palliative treatment was chosen in the present case. Survival time after diagnosis was 9 months and the patient died of a tumor-independent disease. Even in combined treatment, consisting of surgery and (neo-)adjuvant radio-/chemotherapy, prognosis of osteosarcomas secondary to Paget's disease remains very disappointing. Therefore, in treatment of this highly lethal tumor the patient's individual requests and personal situation often require more consideration than in many other malignancies.
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PMID:[Pathologic humeral fracture in secondary osteosarcoma of the humerus: a rare complication of osteodystrophia deformans Paget]. 917 45

Paget's disease of bone is a common condition characterized by bone pain, deformity, pathological fracture, and an increased incidence of osteosarcoma. Genetic factors play a role in the pathogenesis of Paget's disease but the molecular basis of the disease remains unclear. Previous genetic linkage studies have mapped the rare Paget's disease-like bone dysplasia familial expansile osteolysis (FEO) to chromosome 18q21-22, and recent work has shown evidence of linkage between this locus and Paget's disease in one family. Here we studied the relationship between the 18q21-22 locus and Paget's disease in eight large multiplex families from diverse ethnic backgrounds with inherited Paget's disease. Paget's disease was inherited as an autosomal dominant trait in all families, with high penetrance by the sixth decade. Analysis of seven highly polymorphic markers from chromosome 18q21-22 showed positive summated two-point log10 odds ratio (lodscores) of +2.97 with the marker D18S42 at a recombination fraction (theta) = 0.05, and of +2.95 with the marker D18S60 at theta = 0.00, values which are close to the cut-off of +3.0, which is generally accepted as evidence of linkage. Segregation analysis of the haplotypes and formal statistical analysis using the HOMOG program provided evidence for genetic heterogeneity, however, with evidence for linkage in five families and against linkage in the remaining three families (chi square 8.82; df = 2; p < 0.025). Multipoint linkage analysis in the five linked families showed lodscores of above +3.5 across the whole susceptibility region and a maximum summated lodscore of 3.89 at the marker D18S465. In the three nonlinked families, negative multipoint results were obtained for the whole region, with lodscores below -2.0 in one family, excluding this as a candidate locus for the disease. Our studies demonstrate the importance of hereditary factors in the pathogenesis of Paget's disease and confirm evidence of linkage between Paget's disease and chromosome 18q21-22 in some families. This raises the possibility that Paget's disease and FEO may share a common molecular basis, perhaps due to different mutations in the same gene or family of genes. Data from three families did not support evidence of linkage to 18q21-22 however, indicating that Paget's disease is genetically heterogeneous and suggests the presence of at least one additional locus which remains to be discovered.
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PMID:Paget's disease of bone: evidence for a susceptibility locus on chromosome 18q and for genetic heterogeneity. 962 21

Paget's disease is a common entity but osteosarcoma arising in Paget's disease is an infrequent complication. Primary osteosarcomas are typically long bone tumours and the skull is rarely affected. A case with malignant transformation of Paget's disease of the skull and an unusual clinical presentation is reported. Computed tomography scans of the tumour area show interesting results. Main features are: an intensive sclerotic reaction of the host bone, an inner and outer spiculation of the calvarium, radial ossifications in the soft extracranial mass, and spotty calcifications and ossifications of the osteosarcomatous portion infiltrating the brain.
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PMID:Paget's disease of the skull with osteosarcoma and neurological symptoms associated. 972 28


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