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)

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

Giant cell tumor of the bone is usually located within the epiphysis of a long bone, the majority of the lesions occurring in the third and fourth decades of life. We report an unusual case of giant cell tumor (GCT) arising in the parietal skull bone of a 9-year-old girl. The tumor exhibited histologic findings typical for GCT, with conspicuous intravascular giant cells. Based on microscopic features, not only conditions like aneurysmal bone cyst or bone changes associated with hyperparathyroidism but also tumors such as chondroblastoma or osteosarcoma had to be considered. Immunohistochemistry revealed strong reactivity of the tumor giant cells and normal bone osteoclasts with CD68 but not Mac-387; tumor stromal cells were uniformly negative for both. The stromal cells exhibited two immunohistochemically distinct phenotypes. One, involving 50-80% of the tumor cells, exhibited negative lysozyme staining with positivity of proliferating cell nuclear antigen (PCNA) in about 30% of the nuclei. The other showed reactivity with lysozyme but negative PCNA staining. Immunohistochemistry thus helped to distinguish chondroblastoma and osteosarcoma, in which lysozyme positivity would reside in macrophages but not within stromal cells. Instead, chondroblastoma would exhibit protein S-100 positivity in the tumor cells. The biological behavior of GCT is difficult to predict based on morphology alone, although the malignant potential seems to rest in the stromal cells rather than the giant cells. Specifically, in reported cases, the intravascular occurrence of giant cells in GCT is not associated with an increased incidence of metastasis.
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PMID:Giant cell tumor in the skull of a 9-year-old child: immunohistochemistry to confirm a diagnosis rare for age and site. 859 62

Problems in diagnosis of bone tumors in routine laboratories concern very rare tumors, differentiation between chondroma and low grade chondrosarcoma, the recognition of lesions containing multinucleated giant cells, lesions with secondary cysts simulating iuvenile or aneurysmal bone cyst and sometimes the discrimination of callus from osteosarcoma. Real misdiagnoses are done in ossifying pseudotumors interpreted as osteosarcoma, metaphyseal fibrous defect confound with giant cell tumor and chondroblastoma misdiagnosed as giant cell tumor or osteosarcoma. All these mistakes are avoidable if the clinical and radiological features, especially age and exact site in bone studied on the x-rays, are taken into consideration.
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PMID:[Pitfalls and typical false interpretations in bone tumors. From the viewpoint of long-term consultation]. 868 89

Representing only about 1% of all primary bone tumors, chondroblastoma constitutes a very rare bone tumor entity. 56 cases of chondroblastoma, that had been collected by the Hamburg Bone Tumor Registry from 1972 to 1995, were examined histologically together with the radiological and clinical findings. In addition immunohistochemistry with antibodies against S 100, PGM1, LCA and the proliferationmarker MIB 1 was performed. The mean age was 20.4 years and male patients being the majority with a gender ratio of 2.7:1. Predominant localisation was the epiphyses of the long bones, although almost 40% of the tumors were located at untypical sites. Usually a well-circumscribed lysis could be seen on plain X-Ray examination, however partial cortical destruction could be observed in one third of the cases. Histologically characteristic was a polygonal cell component with a weblike chonroid matrix, sometimes with a plane-like appearance. 5 cases showed a distinct nuclear polymorphism making a distinction from osteosarcoma difficult. Using immunohistochemistry all tumors except for one showed positive reaction for S 100 protein. Although the histogenesis of chondroblastoma is not completely understood, morphological findings as well as the observed reactivity with the S 100 protein indicate the chondroid origin. No reactivity for PGM 1 (CD 68) or LCA could be detected. All chondroblastoma showed a low rate of proliferation, thereby being distinguishable from high malignant bone tumors. In general chondroblastoma show a benign biological behavior. Different behavior was observed in 2 cases. One relapse located in the pelvis revealed local aggressive growth while in another case in the humerus a malignant transformation had taken place.
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PMID:[Morphologic characteristics of chondroblastoma. A retrospective study of 56 cases of the Hamburg bone tumor register]. 868 93

The majority of skeletal lesions affecting the patella are benign and include entities such as chondroblastoma, giant cell tumor, osteomyelitis, and gout. Malignant processes involving the patella are distinctly unusual. Isolated occurrences of plasmacytoma, osteosarcoma, hemangiosarcoma, and metastatic disease have been reported. Malignant lymphoma involving the patella is extremely uncommon, although lymphomatous infiltration of the skeletal system is not a rare event, especially with the histiocytic lymphoma. The most frequent radiologic manifestations of skeletal lymphoma include osteolytic lesions with ill-defined margins involving the metaphysis of the long bones of the lower extremities. Involvement of the short tubular and flat bones, as well as the axial skeleton, occurs less commonly. The prognosis for lymphoma involving the skeleton is poor.
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PMID:Malignant lymphoma involving the patella. 895 29

The epiphyses of bone have a different physiologic development and pathophysiology compared to the other skeleton. Hereby the very low incidence of primary bone tumors has to be noted. The analysis of our tumor register, including 2758 cases, and of the literature confirms the chondroblastoma as the most frequent bone tumor especially in the second decade of life. Besides this we find chondromas of the epiphysis in infancy. Osteoid osteomas and osteoblastomas as well as tumor-like lesions and osteomyelitis occur extremely rare. We had no case of a primary malignant bone tumor in our register. Moreover less than 10 cases of osteosarcoma of the epiphysis have been reported in the literature. The border between metaphysis and epiphysis can not be distinguished any more after closure of the epiphyseal growth plate so that primary and secondary bone tumors can develop in this region now. An isolated occurrence of tumor in the epiphysis is even rare in adolescence.
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PMID:[Epiphyseal tumors]. 896 39

Eight cases of primary tumors of the patella were identified in a consecutive series of 587 patients treated surgically for benign or malignant bone tumors. Six cases were benign (5 giant cell tumors and 1 chondroblastoma), and there were 2 malignant tumors (osteosarcoma and malignant fibrous histiocytoma). Both patients with malignant lesions had Rothmund-Thomson syndrome. Benign pulmonary metastases developed in 2 of the patients with giant cell tumor and 1 patient now is deceased 72 months after surgery. Two patients were treated before referral to a tertiary care center; 1 with patellectomy before histologic diagnosis and 1 with curettage. Both resulted in extension of disease into the knee joint. Tumors of the patella require careful assessment before surgical management. Conservation of the bone can be achieved in benign lesions that have adequate residual bone remaining at the articular surface. Conservation of the extremity is possible in malignant tumors, but reconstruction of the extensor mechanism is difficult.
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PMID:Primary patellar tumors. 906 May 6

The expression of c-met proto-oncogene product (c-MET) has been reported to be related to invasive growth or tumor stage in some tumors, but little is known concerning the significance of c-MET expression in bone tumors. With use of formalin-fixed, paraffin-embedded tissue specimens and polyclonal antibody for c-MET, we studied the expression of c-MET in 122 cases of malignant bone tumors (43 osteosarcomas, 24 chondrosarcomas, 21 malignant fibrous histiocytomas of bone, 16 Ewing's sarcoma versus primitive neuroectodermal tumors, 18 chordomas), 65 cases of benign tumors and tumor-like lesions (including 8 giant cell tumors of bone, 8 chondroblastomas, 12 enchondromas, 7 osteochondromas, 10 fibrous dysplasias), 7 cases of articular cartilaginous tissue, and 10 cases of fetal vertebral tissue consisting of foci of enchondral ossification and notochordal tissue. In malignant tumors, c-MET expression was most frequently detected in chordoma (94.4%), followed by chondrosarcoma (54.2%) and osteosarcoma (23.3%). Among the osteosarcoma specimens, c-MET expression was frequently detected in the chondroblastic subtype (66.7%), but the incidence was low in the cases with other subtypes of osteosarcoma. We found no significant correlation between the c-MET expression and the histologic grade of malignancy in either osteosarcoma or chondrosarcoma. c-MET expression was either rarely observed or completely negative in malignant fibrous histiocytomas of bone (4.8%) and primitive neuroectodermal tumors (0%). In benign tumors and tumor-like lesions, c-MET expression was frequently detected in cartilaginous tumors, such as chondroblastoma (62.5%), enchondroma (66.7%), and osteochondroma (71.4%), but no expression was observed in giant cell tumors of bone or any other benign tumors or tumor-like lesions. In normal tissue, c-MET expression was frequently detected in the articular cartilage (100%) and notochord (70.0%) specimens examined. We conclude that c-MET expression as frequent as that observed in the notochordal tissue, chordomas, articular cartilage, and cartilaginous tumors is related to the development of both normal tissue and chondroid tumors.
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PMID:Expression of c-met proto-oncogene product (c-MET) in benign and malignant bone tumors. 926 27

Twenty-one patients with subacute osteomyelitis who were initially considered to have bone tumours were reviewed, with an average follow up of 3 years. The clinical symptoms were not specific and laboratory investigations were normal. The radiographic findings were limited osteolysis surrounded by bone sclerosis in 14 cases, osteolysis without definite borders in 6, and onion-layer periosteal bone formation in one. The preoperative diagnoses included osteoid osteoma, osteosarcoma, chondroblastoma, Ewing's sarcoma, giant cell tumour, fibrosarcoma, eosinophilic granuloma, and bone tumour of unknown aetiology. The definitive diagnosis was made by surgical biopsy, histology and cultures which grew staphylococcus in 9 cases. The gross specimens all showed lymphocytes, plasma cells and granulation tissue with osteogenesis. All the patients recovered completely; 17 were treated with antibiotics and immobilisation, and 4 did not need an antibiotic. There was no recurrence of infection after curettage and excision of the infected tissues.
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PMID:Subacute osteomyelitis presenting as a bone tumour. A review of 21 cases. 934 61

Except for clear cell carcinomas that metastasize to bone, with renal cell carcinoma being the principal representative of that group, clear cell osseous neoplasms are rare. The only distinct nosologic entity in this category that is primary in the bone is the clear cell chondrosarcoma (CCCS). This lesion, which is most often seen in the proximal femur or humerus, affects males more often than females and has a peak incidence during the third and fourth decades of life. Radiologic images of CCCS show a well-circumscribed, often calcified lytic lesion that may expand the bone, but only uncommonly breaches the cortex. Clear cell elements in CCCS are accompanied by "conventional" foci of chondrosarcoma in less than 50% of cases; noncartilaginous "secondary features," including areas of osteogenesis, osteoclast-like giant cells, and zones resembling aneurysmal bone cyst or giant cell tumor of bone, may be apparent as well. CCCS is a relatively indolent malignancy; roughly 25% of patients experience local recurrences of their tumors or suffer metastasis, but tumor-related death is uncommon, particularly when the lesion has been completely resected en bloc. Sporadic examples of other tumors in bone also may be focally or entirely composed of clear cells. These include osteosarcoma, chondroblastoma, chordoma, adamantinoma, Ewing's sarcoma, and primitive neuroectodermal tumor. The last two of these lesions represent the most common primary clear cell bone tumors in children, whereas metastatic renal clear cell sarcoma is the most frequent metastatic pediatric tumor in this category.
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PMID:Clear cell tumors of bone. 938 27


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