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)

Survivors of hereditary retinoblastoma are at increased risk for the development of second primary tumors, most commonly osteosarcoma. Recent molecular genetic data demonstrate that a pleiotrophic effect of the retinoblastoma gene may be responsible for the development of these sarcomas. This report describes the incidence of second nonocular malignancies among 53 infants seen at Stanford University Medical Center who have been followed a median of 11.7 years. Of these, 42 initially had bilateral disease and eleven had unilateral disease. Of 53 infants, 50 received irradiation either as part of the initial therapy or as treatment for recurrent disease. The actuarial survival for the entire group is 67% at 30 year follow-up with a median survival of 79% at 11.7 years. Eight patients developed eleven second primary tumors. All occurred in the group having hereditary retinoblastoma. Eight were within the previously irradiated field and three were at distant sites. The second tumors included seven osteosarcomas, one angiosarcoma, one rhabdomyosarcoma, one malignant fibrous histiocytoma, and one unclassifiable round blue cell tumor. The actuarial incidence of the development of a second primary malignancy was 6% at 10 years, 19% at 20 years, and 38% at 30 years. The latent period from treatment of retinoblastoma to the diagnosis of malignancy ranged from 5.2 years to 36.2 years (median 16 years). An aggressive approach with combined modality therapy including radical resection, re-irradiation and/or chemotherapy was used to treat these second primary tumors in five of eight patients. In four of the five, there was no evidence of disease at 22-72 months following treatment. In the three patients who did not receive aggressive combined treatment, there were no survivors. These data confirm the previously reported risk of developing a second primary tumor among survivors with hereditary retinoblastoma. Careful long-term follow-up for this genetically susceptible group is essential for early detection and implementation of curative therapy.
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PMID:Aggressive management of second primary tumors in survivors of hereditary retinoblastoma. 277 44

Local response of hyperthermia for soft tissue and bone tumors was investigated. Ten tumors were superficial tumors and 16 were deep seated tumors; 9 tumors were malignant fibrous histiocytoma, 5 were liposarcoma, 4 were neurogenic and 3 were myogenic sarcoma. The other five tumors were an angiosarcoma, a malignant mesenchymoma, an Ewing's sarcoma, a chordoma and an osteosarcoma. Some 23 tumors were heated in combination with radiation therapy, and 3 were combined with arterial infusion of ADR. Four of 10 superficial tumors disappeared (CR), and, 2 of 10 signified PR. Only one of 16 deep seated tumors showed CR, 3 were PR and 12 showed no response. But 4 of 12 tumors without regression in tumor volume indicated coagulation necrosis owing to histological examinations, and 5 of 12 were regarded as the same response from hypodensity area with CT examination after hyperthermia. Local response rate of of superficial tumors was 60% and that of deep-seated tumors was 81.4%.
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PMID:[Hyperthermia in bone and soft tissue tumors]. 283 94

A distinctly different entity from the now well-delineated malignant fibrous histiocytoma (MFH) of bone is the MFH histopathologic subtype of osteogenic sarcoma. Although uncommon, recently the authors have encountered six cases of this neoplasm, in each of which the soft tissue component was devoid of bone elements and was microscopically indistinguishable from MFH of bone or soft tissue. Neoplastic osteoid and woven bone were present in the osseous component of each tumor, however. Radiologically, the lesions generally were osteoblastic but focally osteolytic with features typical of osteogenic sarcoma. Pain was the most common presenting symptom. There was no age or sex predilection. Immunocytochemical staining showed strong positivity with alpha-1-antichymotrypsin within malignant bizarre giant cells and occasional neoplastic osteoblasts in five cases. The biological behavior followed a very aggressive course. Four of the six patients developed pulmonary metastases 6 to 12 months after initial surgery; one patient presented initially with pulmonary metastases. Adequate tumor sampling as well as optimal correlation with clinical and radiographic information are required to distinguish the MFH subtype of osteogenic sarcoma from MFH of bone, both being high-grade neoplasms, however.
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PMID:Osteogenic sarcoma. Malignant fibrous histiocytoma subtype. 284 Jan 91

Fifty-nine patients with osteogenic sarcomas arising in bones following exposure to x rays and 20 patients with postradiation malignant fibrous histiocytomas of bone arising as a direct consequence of irradiation were studied. These represent 5.5% of all osteogenic sarcomas and 4.9% of all malignant fibrous histiocytomas of bones. The sarcomas may affect any skeletal site, but most commonly they arose in bones of the pelvic and shoulder girdles or the distal end of the femur. Grounds for irradiation were either nonosseous conditions or preexistent skeletal lesions. Reasons for incidental osseous irradiation included Hodgkin's disease, carcinoma of cervix, breast or lung; bilateral retinoblastoma and others, and giant cell tumor predominated among the irradiated skeletal lesions. The mean and the median radiation doses were 6,040 cGy (rad) and 5,700 cGy (rad), respectively. The period of latency between irradiation and the appearance of the bone sarcoma ranged from 3.5 to 47 y with a mean of 16.5 and median of 14.5, respectively. The cumulative disease-free survival rate for malignant fibrous histiocytoma patients at 3 y was 58%. The cumulative disease-free survival rate at 5 y for patients with osteogenic sarcoma was 17%, with a median survival estimate of 1 y. Although all patients with malignant fibrous histiocytoma who received their radiation therapy for a preexistent bone lesion survived, only 27% of the patients whose bone was normal at the time of irradiation are alive and well at the 3-y mark.
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PMID:Postradiation sarcomas of bone. 284 96

Nude mice xenotransplants have been performed on human primary sarcomas of bone and soft tissues in order to delve into the cell heterogeneity of these neoplasms. Particular emphasis has been given to the group of small round blue cell sarcomas (Ewing's sarcomas and peripheral neuroectodermal tumors). Out of 31 xenotransplanted sarcomas, 16 cases have grown positively, and many of them continue to be transferred into nude mice on a regular time basis, being presently considered as fully established nude lines. Here we report the results of such a system, which has been followed with optical, electron microscopical, immunohistochemical and cytogenetic techniques. Osteosarcomas make up the group with the highest number of positivities taken (6 out of 9 transplanted cases). Diversity in growth rate, positive tumors and morphology are taken into consideration. Epithelial foci were seen in one of the transplanted neoplasms. Malignant fibrous histiocytoma and other mesenchymal sarcomas of bone and soft tissues are reviewed. Changes in immunohistochemical reactivity (alpha-1AT and alpha-1AQT) were observed in the transplanted neoplasms. Cytogenetic analysis performed on an undifferentiated (primitive) soft-tissue sarcoma provided clues to its synovial origin. Analysis of Ewing's sarcoma performed on nude mice transplanted tumors shows heterogeneous immunohistochemical response, with enhancement of cytokeratin positive cells, not previously seen in the primary neoplasms.
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PMID:Value of nude mice xenografts in the expression of cell heterogeneity of human sarcomas of bone and soft tissue. 285 74

By studying histochemically and ultrastructurally a case of malignant fibrous histiocytoma (MFH) of bone with neoplastic osteoid elements, the distinction of the tumor from osteosarcoma was discussed. MFH was likely to originate from undifferentiated mesenchymal cells that induced a histiocytic line of differentiation, and some histiocytes seemed to be transformed into well differentiated fibroblastic cells which accordingly differentiated into fibroblasts and osteoblasts. This may explain the existence of the osseous elements in MFH. Sarcoma showing the bimodal--histiocytic and fibroblastic--differentiation should be diagnosed as malignant fibrous histiocytoma, even if it contains tumorous osteoid; it must be clearly distinguished from osteosarcoma, in which the various cells only on a fibroblastic line of differentiation can be identified and the osteoblasts are the predominant cells.
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PMID:A study on the osseous elements in malignant fibrous histiocytoma of bone. 298 93

Ten tumours: 5 malignant fibrous histiocytomas (2 with a primary site in the bone and 3 in the soft tissue), 3 giant cell bone tumours (one of them was malignant with metastasis to the lung) and 2 osteogenic sarcomas were studied in organ culture. The blood was added into some tumour pieces with a subsequent examination of blood phagocytosis by means of checking the hemosiderin granule accumulation in the cell cytoplasm. Malignant fibrous histiocytoma and giant cell bone tumour were very similar by the pattern of their growth and phagocytic activity of their cells; both differed strikingly from the osteogenic sarcoma. This implies the histogenetic similarity of malignant fibrous histiocytoma and giant cell tumour of the bone.
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PMID:[Histogenesis of malignant fibrous histiocytoma and giant-cell tumor of bone]. 299 70

We have studied the possible origin of histiocytic cells, present in fibrous histiocytomas (MFH) by using immunohistochemistry to demonstrate lysozyme, alpha 1-antitrypsin, alpha 1-antichymotrypsin and receptors for peanut and soy bean agglutinin in tumour cells of MFH compared with their presence in tumour cells of malignant histiocytosis (MH) ('true' histiocytic lymphoma, 'true' histiocytic sarcoma). We included in this study a number of other soft tissue tumours (STT). Lysozyme was detected in half of the cases of malignant histiocytosis (n = 16) but in only two out of 77 MFH. alpha 1-Antitrypsin and alpha 1-antichymotrypsin usually occurred together although the latter was seen in more cases. Both markers were present in majority of cases of MH whereas they were detected in a minority of cases of MFH. MFH cases of the storiform subtype were less frequently stained than the pleomorphic or giant cell subtypes. Receptors for peanut or soy bean agglutinin were detected in nearly all MH cases, whereas their presence was only detected in a small number of MFH. Lysozyme was not detectable in other STT. alpha 1-Antitrypsin and alpha 1-antichymotrypsin were uncommonly present in other STT, except in osteosarcoma and rhabdomyosarcoma. These markers therefore have a limited value as indicators of a possible histiocytic origin of MFH. Lectins showed weak affinity for other STT. In accordance with others, we therefore conclude that the progenitor cell of MFH has to be sought within the undifferentiated mesenchymal cells and that histiocytes themselves probably do not give rise to MFH.
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PMID:Characterization of tumour cells in malignant fibrous histiocytomas and other soft tissue tumours in comparison with malignant histiocytes. I. Immunohistochemical study on paraffin sections. 299 65

Since 1927, 130 patients with well-documented malignant fibrous histiocytoma of bone have been diagnosed and treated at Memorial Hospital for Cancer and Allied Diseases. This sarcoma is 10 times less frequent than osteogenic sarcoma in this hospital. It most commonly occurred spontaneously (72%), whereas in the rest (28%) it followed previous radiation or various pre-existent osseous conditions, most often Paget's disease. The appendicular skeleton was the commonest site of involvement. The majority of the patients were middle-aged or older adults with a mean of 40.5 years of age; only 21.5% were 21 years or younger. Histologically, the lesions were subclassified as fibrous (62%), histiocytic or xanthomatous (30%), and malignant giant cell tumor (8%) variants. Older patients were more likely to have a secondary malignant fibrous histiocytoma, especially following radiation or Paget's disease. Overall survival estimates at 2 years and 5 years were 71% and 53%, respectively. Survival was not dependent on the histologic subtype of the lesion, but was strongly influenced by the histologic grade of malignancy. Important prognostic factors were the age of the patients and whether the lesions were primary de novo or secondary sarcomas: the older patients and those with secondary lesions did substantially worse.
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PMID:The pathology of malignant fibrous histiocytoma of bone. A study of 130 patients. 300 Feb 4

The treatment protocol of 15 patients with a primary tumor of the femur, including osteosarcoma, malignant fibrous histiocytoma and chondrosarcoma is presented. All patients had been selected for resection and reconstruction with an endoprosthesis. An endoprosthesis was implanted in 12 patients. The results of this type of treatment appear to be satisfactory. In eight osteosarcoma cases resection and reconstruction with an endoprosthesis combined with preoperative and postoperative chemotherapy, according to Rosen, were performed. Follow-up in all 15 patients, varying from 1.4 to 6.0 years, showed no evidence of disease in 12 patients. Three patients had died. Function of the involved leg was satisfactory in most cases. The advantage and disadvantages of the use of an endoprosthesis are discussed as well as complications in this series of patients.
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PMID:The treatment of primary tumors of the femur with chemotherapy (if indicated), resection and reconstruction with an endoprosthesis. 300 31


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