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Query: UMLS:C0029463 (osteosarcoma)
 16,637 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In a father and son who had familial Paget's disease of bone, secondary osteogenic sarcomas involving identical anatomic sites developed at about the same age. A review of the literature revealed 35 additional cases of familial osteogenic sarcoma arising in 14 families, mainly among siblings. The relationship of osteogenic sarcoma to Paget's disease and the familial aspects of both conditions are discussed. This is the first recorded instance of a vertical male-to-male transmission of Paget's disease and osteosarcoma.
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PMID:Familial osteogenic sarcoma occurring in pagetoid bone. 694 28

Twenty-seven osteosarcomas of bone were examined by electron microscopy. Eighteen cases were conventional osteosarcomas, one case was an osteosarcoma that developed in Paget's disease of bone, two cases were metastasis to the lungs of conventional osteosarcomas, five were parosteal osteosarcomas (and the recurrence of one of them), and one was periosteal osteosarcoma. In addition to the basic neoplastic cell, the osteoblast-like tumor cell, seven tumor cell types could be differentiated in varying numbers in the 27 cases examined: (1) chondroblast-like; (2) fibroblast-like; (3) histiocyte-like; (4) myofibroblast; (5) osteoclast-like; (6) malignant multinucleated osteoblast-like; and (7) atypical primitive mesenchymal cells. Moreover, endothelial cells and pericytes were also present. The tumor cells showed common neoplastic features in relation with general cell configuration and fine structures. No cytoplasmic organelle was considered to be cell-specific, and only the amount and distribution of the organelles allowed us to differentiate the above mentioned cell types. The presence of malignant appearing tumor cells with abundant and dilated cisternae of RER, in an intercellular matrix containing collagen fibers with areas of focal collections or puffs of hydroxyapatite crystals, are the most characteristic ultrastructural features of osteosarcoma. However, those features are not pathognomonic and do not allow in all cases the differential diagnosis with osteoid-osteoma and benign osteoblastoma. Fields of chondroblastic, fibroblastic, or fibrohitiocytic character are indistinguishable of chondrosarcomas, fibrosarcomas, or malignant fibrous histiocytoma of bone, respectively. The comparative study of parosteal and conventional osteosarcomas revealed a marked difference in the cell population: fibroblast-like cells and myofibroblasts were the main cell types in parosteal osteosarcoma; on the contrary, myofibroblasts were very scarce or absent in conventional osteosarcomas. This difference may correlate with the better outcome of parosteal osteosarcoma. The ultrastructural features of a periosteal osteosarcoma are reported for the first time. The tumor was comprised mostly of chondroblast-like tumor cells. However, in the more cellular peripheric areas of the neoplasm, the cells were predominantly of the fibroblast-like type and showed anaplastic features.
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PMID:The ultrastructure of conventional, parosteal, and periosteal osteosarcomas. 695 92

An account of primary malignant bone tumors in Africans is presented in this series. The study analyzes 170 Nigerians with bone neoplasms diagnosed over the 17-year period 1960-1976. It accounted for 1.28 percent of tumors registered in the Cancer Registry of the University College Hospital, Ibadan. There were 119 male and 51 female patients, with ages ranging from 4 to 75 years. The most common symptom was painful swelling. Trauma was associated with this condition. The pattern of behavior of these tumors conforms with other reports in the literature, but it showed some geographical variations. The most common tumor in the series was osteosarcoma, although there was no association of Paget's disease, which is very rare in Nigerians. Attention is also called to the presence of osteolytic conditions in the metaphysical region of a long bone, which may be due to osteomyelitis caused by Histoplasma duboisii and which has been confused with a malignant bone tumor in this environment.
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PMID:Primary malignant tumors of bone: incidence in Ibadan, Nigeria. 695 50

Sixty-nine patients with primary osteosarcoma of the bone are reviewed. There were 50 males and 19 female; a male predominance of 2.6:1. The patients presented in advanced stages. In agreement with other authors, we found a peak incidence in the second decade. No associated Paget's disease was found in any of the elderly patients. The long bones of the lower limbs were the most frequently affected. Treatment with amputation was not acceptable to the great majority of our patients.
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PMID:Osteosarcoma of the bone: clinico-pathologic features of sixty-nine cases in Ibadan, Nigeria. 696 47

Urinary glycosaminoglycan and hydroxyproline excretion was studied in 11 patients with clear evidence of Paget's disease of bone. Urinary hydroxyproline, cetyl pyridinium chloride (CPC)-precipitable uronic acid and CPC-precipitable hexosamine were expressed as ratios to urinary creatinine. Urine samples were concentrated x 1000 by vacuum dialysis and the glycosaminoglycans examined by electrophoresis on cellulose acetate followed by staining with alcian blue. All the cases studied showed markedly raised hydroxyproline excretion, whereas the uronic acid excretion was normal or only slightly raised in 10 of the 11 cases studied. One patient who had a raised uronic acid and raised hydroxyproline concentration was shown to have osteosarcoma as a complication of Paget's disease. THE VERY HIGH HYDROXYPROLINE: creatinine ratio in all cases of Paget's disease (mean 241.8 mmol hydroxyproline/mol creatinine) contrasted sharply with the cases of disseminated neoplasm, where the ratio was either normal or slightly raised (mean 29.3 mmol hydroxyproline/mol creatinine). The ratio of hydroxyproline to CPC-precipitable uronic acid was also markedly raised in cases of Paget's disease (mean 77.3 mmol hydroxyproline/mmol uronic acid) and was lower in the neoplastic group (mean 14.1 mmol hydroxyproline/mmol uronic acid) but showed no advantage over the hydroxyproline: creatinine ratio in differentiating the two groups. THE URINARY HYDROXYPROLINE: creatinine ratio promises to be of value in differentiating between Paget's disease of bone and neoplastic invasion of bone. A marked rise in CPC-precipitable uronic acid excretion alone is more suggestive of neoplastic invasion of bone, and if associated with a marked increase in hydroxyproline excretion, it raises the possibility of neoplastic change in Paget's disease of bone. The results of this study also suggest that bone collagen, rather than bone tissue in general, is primarily affected in Paget's disease.
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PMID:Urinary excretion of glycosaminoglycans and hydroxyproline in Paget's disease of bone, compared with neoplastic invasion of bone. 730 92

Skeletal metastases from prostate cancer is common and usually do not pose a diagnostic dilemma. This study reviews radiographic appearances of prostatic metastases to the appendicular skeleton in four patients where the appearances simulated osteosarcoma, Paget's disease and Paget's sarcoma. Prostatic metastases to long bones can produce appearances considered characteristic of other lesions and suggest misleading alternative diagnoses.
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PMID:Appendicular metastatic prostate cancer simulating osteosarcoma, Paget's disease, and Paget's sarcoma. 748 3

Using in situ hybridization we show that expression of the c-fos oncogene, a gene normally associated with osteosarcomas, is greatly elevated in osteoclasts of patients with Paget's disease. Immunohistochemical staining with c-fos antibodies also shows increased protein in pagetic osteoclasts. In light of transgenic mouse experiments showing a key role for c-fos in bone resorption, we propose that elevated c-fos gene expression in pagetic osteoclasts is an important component in producing the pagetic phenotype. Levels of c-fos gene and protein expression in pagetic osteoblasts are lower than those detected in osteoclasts but still higher than in nonpagetic osteoblasts. This may provide an explanation for the increased incidence of osteosarcomas in patients with Paget's disease because overexpression of c-fos in osteoblasts of transgenic mice induces osteosarcoma formation.
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PMID:Upregulation of c-fos protooncogene expression in pagetic osteoclasts. 797 1

Malignant neoplasms associated with Paget's disease of the bone are most commonly metastatic and rarely primary malignant transformations. Sarcomatous transformations, such as osteogenic sarcoma, predominant in Paget's disease. The authors discuss the synchronous and synostotic occurrence of a primary lymphoma of bone in association with Paget's disease. The simultaneous occurrence of isolated primary lymphoma of bone and its phenotype in association with Paget's disease in the femur, which had not been rigorously documented in the previous literature, is reported.
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PMID:Primary lymphoma and Paget's disease of the femur. 820 69

To determine the clinical presentation and manifestations of Paget's disease of the bone in patients older than 60 years, we reviewed the cases of 56 patients attending a bone clinic. Pain was the presenting symptom in 34 cases. It was attributed to the disease process in 21 cases, to osteoarthritis in 11, to trigeminal neuralgia in 1, and to osteosarcoma in 1. Other clinical manifestations included deformities (15 cases), diminished mobility and unsteady gait (9), hearing impairment (7), lethargy (4), diminished vision (3), cognitive deficit (3), sense of warmth in limbs (2), ill-fitting dentures (1), and fracture (1). We concluded that in patients older than 60 years, Paget's disease of the bone may present itself in a variety of ways, some of which may be mistakenly attributed to the "aging process" or some other disease. Furthermore, in this age group, osteoarthritis is responsible for the pain experienced by about one third of symptomatic patients.
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PMID:Clinical presentation of Paget's disease of the bone in older patients. 821 23

The authors treated 18 patients with Paget's disease of bone (12 men and 6 women, age 65 +/- 5 years) with pamidronate (bisphosphonate of the second generation). Three patients from this group were treated previously without success with calcitonin or bisphosphonate of the first generation (etidronate) 50% of the patients suffered from the polyostotic form of the disease. In one patient a rare combination of primary hyperparathyroidism with Paget's bone disease was found and in another patient later an osteosarcoma developed in the affected bone. To all patients sodium pamidronate was administered (Aredia, Ciba-Geigy) 30 mg per day by i.v. infusion for 2 hours during three days. Four patients developed fever, two patients phlebitis at the site of injection. These side-effects are described by the manufacturer. Two patients developed transient regional alopecia, not described so far. Subjective pain relief of the affected skeleton occurred in one patient after one month of treatment, after three months in 78%. Laboratory manifestations of activity of the disease (serum activity of alkaline phosphatase, tartrate resistant acid phosphatase and hydroxyprolinuria) declined gradually from the 1st to the 6th month after onset of treatment. There was a less marked decline of the osteocalcin serum concentration. The concentration of calcium, phosphorus and vitamin D metabolites did not change markedly. Twelve months after treatment 14.7% of the patients were inactive according to laboratory tests, 73% however experienced another rise of parameters of osteoresorption and osteoformation. Pamidronate treatment in patients with Paget's disease of bone is effective and safe.
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PMID:[Paget's disease of bone and treatment with pamidronate]. 837 65


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