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 retrospective study of 88 cases of extraskeletal osteosarcoma revealed that this tumor affects adults almost exclusively, with a high incidence in patients older than 50 years, and is slightly more common in males (58%) than in female patients. The tumor occurred principally as a soft tissue mass in an extremity, with a predilection for the thighs (lower extremity, 46.6%; upper extremity, 20.5%) and the retroperitoneum (17%). Most were deep-seated and were firmly attached to the fascia, but occasionally they were freely movable and confined to the subcutis or dermis. Nearly all presented as an insidiously growing mass rarely causing pain or tenderness. The preoperative duration of symptoms ranged from 2 weeks to 25 years (median, 6 months). In 17 cases, it exceeded 2 years. A history of prior trauma to the site of the tumor was stated in 11 of the 88 cases (12.5%) and of radiation in five cases (5.7%). Microscopically, the tumors contained varying amounts of neoplastic osteoid and bone, sometimes together with islands of malignant-appearing cartilage. Like osteosarcoma of bone, extraskeletal osteosarcoma showed a striking variation in histologic appearance and focally resembled malignant fibrous histiocytoma, fibrosarcoma, and malignant schwannoma. Follow-up information was available for 65 patients. Eight (12.3%) patients were alive with no evidence of recurrence; 12 (18.5%) patients were alive with one or more recurrences; and five (7.6%) were alive with metastases. Twenty-eight of the tumors (43%) recurred and 39 (63%) metastasized. Forty (61.5%) of the patients with follow-up information had died, 36 from the tumor and four from miscellaneous causes. The prevailing sites of metastases were the lung, the regional lymph nodes, and bone.
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PMID:Extraskeletal osteosarcoma. 347 57

Twenty-nine consecutive patients 2-35 years old underwent serial thoracic CT evaluations for metastatic disease. Thymic volumes were determined for each patient during cycles of chemotherapy and were compared with the patient's clinical status. This group included patients with Hodgkin's disease (13 patients), osteogenic sarcoma (five), testicular neoplasm (four), Wilms' tumor (three), rhabdomyosarcoma (two), malignant fibrous histiocytoma (one), and Ewing's sarcoma (one). Seven patients with mediastinal lymphoma had tumor involvement of the thymus and therefore were excluded. The 22 remaining patients showed cyclic thymic volume changes in response to chemotherapy or its discontinuance. During the first course of chemotherapy the thymic volume decreased by an average of 43% in 20 of 22 patients. Between the first and second course, regrowth was observed in all 20 of these patients. Among the six patients who received a second course of therapy, an average volume decrease of 36% was observed during the second course with regrowth again occurring during recovery from chemotherapy. Thymic rebound (regrowth 50% greater than baseline volume) occurred in five patients, three of whom were in clinical remission. The thymus appears to atrophy during the administration of chemotherapy and regrow during the recovery phase of chemotherapy in 90% of the patients studied. Thymic hyperplasia or rebound is a relatively common phenomenon occurring in 25% of patients. The size of the thymus appears to be extremely sensitive to chemotherapy.
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PMID:Thymic atrophy and regrowth in response to chemotherapy: CT evaluation. 349 49

A material of 87 consecutive patients with Ewing's sarcoma referred for treatment in the period 1962-1983 was retrospectively analysed. Thirteen patients had metastases at the time of diagnosis. Of the remainder, 71 received radiation therapy and 32 adjuvant chemotherapy. Survival rate was not influenced by age, sex or treatment delay. Metastatic disease predictably shortened survival (median 6 months vs. 23 months for localized disease). Tumour site did not significantly influence survival rate, although pelvic localization was associated with a slightly shorter median survival. Both pain and objective impairment of movement at presentation correlated to a poorer prognosis, possibly because of larger tumours or soft tissue extension. Adjuvant chemotherapy prolonged recurrence-free survival from a median of 6 months to 16 months, but survival was not improved significantly. Local failure occurred in about 40 per cent, regardless of radiation dose and tumour site. At the time of evaluation, 13 patients (15%) were alive with no evidence of disease and a median follow-up time of 68 months (range 16-196). So far, 2 patients have developed secondary malignancies in irradiated areas (one malignant fibrous histiocytoma and one osteogenic sarcoma).
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PMID:Ewing's sarcoma. A retrospective study of prognostic factors and treatment results. 368 82

During a 37-year period, 26 patients were seen who had chondrosarcoma with additional mesenchymal components ("dedifferentiated low-grade chondrosarcoma"). Sixteen were men and 10 were women aged 30 to 85 years (median, 61 years). The tumors' chondroid areas were of borderline or low-grade malignancy. The additional mesenchymal component was histologically classified as malignant fibrous histiocytoma (16), rhabdomyosarcoma (4), low-grade fibrosarcoma (3), osteosarcoma (2), and undifferentiated sarcoma (1). Preferred locations were pelvis (10) and femur (8). Symptoms had been present for 1 year or less in most cases. Pain was the most common symptom. In 15 of 26, major amputation was the primary treatment. Twelve patients received chemotherapy, usually after developing metastatic disease, but only one achieved a partial response. Median disease-free interval after diagnosis was 4 months, median survival was 6 months, and 19 patients died within 1 year. Of 4 who survived longer than 18 months, 3 presented with a low-grade fibrosarcoma. Survival and development of metastasis appeared unrelated to cell type, initial treatment, or chemotherapy, except when the tumor's initial nonchondroid component was low-grade fibrosarcoma.
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PMID:Chondrosarcoma with additional mesenchymal component (dedifferentiated chondrosarcoma). I. A clinicopathologic study of 26 cases. 371 21

Sarcomas of childhood rank fifth in incidence of malignant tumors in children younger than 15 years. Among the soft tissue sarcomas, approximately 50% are rhabdomyosarcomas. The remainder represent a heterogeneous group of diverse sarcomas which are not unique to children and include fibrosarcoma, synoviosarcoma, malignant fibrous histiocytoma, malignant schwannoma, angiosarcoma, leiomyosarcoma, and others. The most common bone cancers in childhood are osteosarcoma and Ewing's sarcoma. Although a multidisciplinary approach utilizing surgery, irradiation, and combination chemotherapy is routinely used in management of virtually all children with solid tumors, the value of adjuvant chemotherapy in select bone and rare soft tissue sarcomas is currently being tested. Multiagent chemotherapy including vincristine, dactinomycin, cyclophosphamide, and Adriamycin (doxorubicin) contribute to cure rates in 65% to 75% of children with localized rhabdomyosarcoma, Stages I to III, when combined with surgery and/or irradiation. Other drugs which hold promise include platinum, DTIC, methotrexate, and VP-16. The efficacy of similar drugs in the rarer pediatric soft tissue sarcomas other than rhabdomyosarcoma and its variants requires prospective randomized trials evaluating histologic grade, tumor size, and nodal status. It has been suggested that the high-grade sarcomas presenting with minimal tumor bulk are most sensitive to combined radiotherapy-chemotherapy, whereas the low-grade sarcomas are more resistant to such therapy. Tumor cell heterogeneity contributes to biologic diversity and response to treatment. Chemotherapy as adjuvant therapy to irradiation is currently recommended and utilized for Ewing's sarcoma with survival rates approaching 80%, and disease-free survival of approximately 75% for those with localized disease. Children with widespread and metastatic disease at presentation fare less well. Although multiple single agents exhibit response rates ranging from 40% to 60%, including cyclophosphamide, Adriamycin, dactinomycin, BCNU, mithramycin, and 5-fluorouracil, new and more effective agents are needed. Controversy regarding the value of multiagent chemotherapy in osteosarcoma has stimulated prospective randomized trials. Evaluation of local control rates as well as sites and occurrence of metastases are essential in assessing the contribution of aggressive combined modality therapy in the pediatric sarcomas. Emphasis on refinement of therapy in determining the risk/benefit ratio from adjuvant chemotherapy in pediatric sarcomas is mandatory. Enhancement of early local reactions is apparent when adjuvant chemotherapy is used with local radiotherapy.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:The value of adjuvant chemotherapy in the management of sarcomas in children. 388 37

Sixteen patients are presented who had sarcomas of the chest wall at a site where a prior malignancy had been irradiated. The first malignancies included breast cancer (ten cases), Hodgkin's disease (four cases), and others (two cases). Radiation doses varied from 4200 to 5500 R (mean, 4900 R). The latency period ranged from 5 to 28 years (mean, 13 years). The histologic types of the radiation-induced sarcomas were as follows: malignant fibrous histiocytoma, nine cases; osteosarcoma, six cases; and malignant mesenchymoma, one case. The only long-term survivor is alive and well 12 years after resection of a clavicular chondroblastic osteosarcoma. Three cases were recently diagnosed. Despite aggressive multimodality treatment, the remaining 13 patients have all died from their sarcomas (mean survival, 13.5 months). All patients have apparently been cured of their first malignancies. Chemotherapy was ineffective. No treatment, including forequarter amputation, appeared to palliate the patients with supraclavicular soft tissue sarcomas. Major chest wall resection offered good palliation for seven of eight patients with sarcomas arising in the sternum or lateral chest wall. Close follow-up is needed to detect signs of these sarcomas in the ever-increasing number of patients receiving therapeutic irradiation.
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PMID:Radiation-induced sarcomas of the chest wall. 394 97

Histochemical staining for three hydrolytic enzymes were performed in 35 bone tumours and 43 soft tissue tumours, malignant as well as benign. Osteosarcoma, intra-osseous as well as extra-osseous, revealed characteristic rich staining for alkaline phosphatase, no matter how dedifferentiated the tumour was. Haemangioendothelioma (and normal endothelium), too, showed strong reaction for alkaline phosphatase whereas haemangiopericytoma did not. Alkaline phosphatase furthermore was found in slight to moderate amounts in fibrous proliferations. All other tumours examined were negative. Acid phosphatase was found in almost every tumour investigated except Ewing sarcoma and chondromyxoid fibroma. However, high activity was characteristic of giant cell tumours and malignant fibrous histiocytoma. The inhibition of acid phosphatase by tartrate was complete except in osteosarcoma and giant cell tumours, where only a partial inhibition was seen. There were non-specific esterase reactions in a variety of tumours, but very strong reactions were characteristic of malignant fibrous histiocytoma and giant cell tumours. The reaction could be completely inhibited by the addition of fluoride. In an era of increasing application of immunohistologic techniques in surgical pathology it might be of value to remember that simple enzyme histochemical stainings may provide helpful diagnostic features in the classification of bone and soft tissue tumours.
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PMID:Enzyme histochemical investigations on bone and soft tissue tumours. 398 37

We will report the result obtained from sensitivity tests on various anti-cancer agents for malignant bone and soft-tissue tumors based on SDI (Succinic Dehydrogenase Inhibition Test) method with the use of enzymic activities as marker since 1976. Our study comprised 27 cases altogether 15 cases of osteosarcoma, one case each of Ewing's sarcoma, malignant fibrous histiocytoma and malignant lymphoma, 3 cases of metastatic bone tumor and one case each of angiosarcoma, fibrosarcoma, rhabdomyosarcoma, liposarcoma, 2 cases of metastatic lung tumor among soft-tissue sarcomas. In all cases, sensitivity tests were done on the tumor tissues according to SDI method at the same time as biopsy for the determination of the appropriate medications. Four to six weeks of pre-operative intra-arterial infusion was done followed by radical operation. The results obtained are as follows. Observing the long-term results between subjects that applied anti-cancer agents decided by sensitivity test and those without sensitivity test. The 5 years cumulative survival rate jumped from 30.5% to 50.5%, showing a clear improvement.
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PMID:[Clinical evaluation on the sensitivity test for anti-cancer agents in malignant bone and soft-tissue tumors]. 608 33

The data on 35 cases of bone fibrosarcoma treated at the Center clinic in 1955-1982 are presented. A retrospective morphological investigation left diagnosis unchanged in 17 cases (48.6%). In 9 cases diagnosis was changed to fibrous histiocytoma of the bone, in 2--parostal sarcoma and in one case--"dedifferentiated" chondrosarcoma. In 6 cases, diagnosis could not be improved and neoplasms were identified as poly-morphocellular sarcoma. Clinically, a more favorable course and prognosis are distinguishing features of fibrosarcoma as compared with osteogenic sarcoma and malignant fibrous histiocytoma.
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PMID:[Clinico-morphological analysis of bone fibrosarcomas (a retrospective study)]. 609 41

The histological diagnosis of malignant fibrous histiocytoma (MFH) seems to have become recently fashionable among pathologists, although its histogenesis and diagnostic criteria are not entirely settled as yet. For practical purposes the differential diagnosis with other easily mistakable mesenchymal tumors should be strictly made with great caution, because of variable histological features of this tumor. The authors attempted to elucidate the differential points from other tumors in a review of 189 cases of malignant soft tissue tumors. Some cases of carcinoma mimicking MFH were also reviewed. No single criterion for making the histological diagnosis of MFH was obtained. Its histological features and differential points from pleomorphic rhabdomyosarcoma and fibrosarcoma were tabulated. The recognition of a true tumor osteoid was emphasized as a single differential point between osteosarcoma and MFH often with fibrous areas mimicking osteoid. Renal cell carcinoma metastasizing to bone, which was misinterpreted as MFH on biopsy tissue, was also described and its differential point was stated.
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PMID:Malignant fibrous histiocytoma with special reference to its differential diagnosis. 625 39


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