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)

Adjuvant chemotherapy, established in the treatment of osteogenic sarcoma, Ewing's sarcoma, and rhabdomyosarcoma, currently remains controversial in adult soft-tissue sarcoma. Because the survival of the control arms in randomized studies is significantly better than historical controls, only randomized studies with nontreatment control arms will be analyzed here. None of the 11 reported adjuvant studies showed a significant survival advantage for chemotherapy overall. Although subset analyses are less reliable, the National Cancer Institute reported a significant prolonged survival and disease-free survival for the subset of chemotherapy-treated extremity primaries. The M.D. Anderson and Italian studies observed a significant disease-free survival advantage; however, survival was not significantly different in the former and survival statistics were not provided in the latter study. In the subset of central body lesions none of the studies revealed a significant survival advantage for chemotherapy. Adjuvant chemotherapy should be considered investigational for adult soft-tissue sarcomas.
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PMID:Adjuvant chemotherapy for soft-tissue sarcoma: a critical appraisal. 328 Dec 13

A case of embryonal rhabdomyosarcoma of the femur observed in an 11-year-old boy is reported, which simulated an osteosarcoma clinically and radiographically. To our knowledge, this is a second documented case of rhabdomyosarcoma of the long bone.
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PMID:Primary rhabdomyosarcoma of long bone. A case report. 345 39

Seven patients underwent resection of a sarcoma of the extremity requiring excision of a vital artery and revascularization. These included osteosarcoma of the pubis, osteosarcoma of the distal femur, undifferentiated sarcoma of the thigh, liposarcoma of the thigh, liposarcoma of the popliteal space, chondrosarcoma of the proximal tibia and rhabdomyosarcoma of the thumb. Preoperative evaluation included computed tomographic scan, magnetic resonance imaging and angiography. Operation involved excision of the iliac artery in one instance, femoral artery in three, popliteal artery in two instances and radial artery in one. Wide local resection including revascularization was undertaken when the tumor could be resected with the artery but separated from the nerve and it was a low grade malignant disease or the patient refused amputation for a high grade malignant disease. In five patients, the tumor margins were adequate. One patient with an osteosarcoma of the pubis with distant disease had a palliative resection and one patient had positive margins but refused amputation. Coverage of the soft tissue and vascular grafts was achieved using a distant pedicle flap in two patients. Amputation was avoided and each patient remained ambulatory. Five patients remained free of disease with patent grafts at six months to six years of follow-up study. One patient died of late myocardial infarction and one who underwent palliative resection died eight months later of metastatic disease. Involvement of the major arterial circulation does not preclude adequate resection of sarcomas of the extremity with limb salvage.
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PMID:Limb sparing operations for sarcomas of the extremities involving critical arterial circulation. 347 4

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

Studies of the presenting height of children with malignancies have produced conflicting results, from an excess of taller patients to an excess of shorter patients. The problems of measurement bias, inadequate comparison populations, small numbers of patients, subgroup analyses, and overreliance on simple significance tests are all possible reasons for the variation in results. To clarify this issue, we studied heights at diagnosis of 3657 children and adolescents aged under 18 years. Their malignancies included acute lymphoblastic leukaemia, non-Hodgkin's lymphoma, Hodgkin's disease, acute non-lymphoblastic leukaemia, osteosarcoma, retinoblastoma, neuroblastoma, Wilms' tumour, rhabdomyosarcoma, and Ewing's sarcoma. Compared with published standards for the heights of children in control populations, no significant deviation from population norms was found for patients in any of the 10 disease categories after proper adjustment for multiple significance testing.
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PMID:Height at diagnosis of malignancies. 360 84

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

We have evaluated the activity of ifosfamide in 75 patients with recurrent sarcomas and pediatric solid tumors. All patients had previously received cyclophosphamide in combination with other chemotherapeutic agents. Ifosfamide was administered as a continuous 5 day infusion at a dose of 1800 mg per M2, except in the last 14 patients who received the drug as a daily one hour infusion at the same dose level. Partial response was observed in 9 of 20 patients with Ewing's sarcoma, 2 of 9 patients with rhabdomyosarcoma, 3 of 17 patients with osteogenic sarcoma and 4 of 29 patients with various other neoplasms. A further 6 patients had stable disease, defined as the absence of progression for at least 6 cycles of therapy. Thus overall response rate was 24%, with the highest response rate of 45% being observed in Ewing's sarcoma. Toxicity was acceptable, although there was quite marked leucopenia (median nadir 700) with less profound thrombocytopenia (median nadir 87,000). Sepsis occurred in 3 patients but no patient died as a result of infection. Hematuria occurred in 43% of patients who did not receive mesna, and in 26% of patients who did, although prior pelvic irradiation was found to be a significant risk factor for hematuria. Only 1 of 14 patients without prior pelvic irradiation or hematuria developed hemorrhagic cystitis when treated with ifosfamide and mesna. Confusional states developed in 6 patients. We conclude that ifosfamide is an active agent in patients with relapsed sarcomas and childhood solid tumors, even when such patients have been previously treated with cyclophosphamide.
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PMID:A phase II study of ifosfamide in the treatment of recurrent sarcomas in young people. 381 17

The purpose of these studies was to select in vitro tumor cells that were resistant to macrophage-mediated lysis. Seven different heterogeneous murine neoplasms (four fibrosarcomas, a melanoma, a rhabdomyosarcoma, and an osteogenic sarcoma) and one cloned line of a fibrosarcoma were incubated in vitro with syngeneic tumoricidal macrophages. Surviving tumor cells were recovered and expanded to undergo subsequent interaction with tumoricidal macrophages. After six sequential interactions, all cell lines were examined for their susceptibility to lysis mediated by murine peritoneal exudate macrophages activated with liposomes containing muramyl tripeptide phosphatidylethanolamine. In all eight systems, no significant differences were detected between the parent tumor cells and cells that survived the sequential interactions. Neither macrophage infiltration into s.c. tumors nor the experimental or spontaneous metastatic potentials of the parental tumors differed from the lines established by cells surviving macrophage-mediated lysis. Collectively, the data suggest that tumor cell destruction by activated macrophages is nonselective and does not lead to the development of resistant tumor cells nor to cells with altered metastatic properties.
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PMID:Nonselective destruction of murine neoplastic cells by syngeneic tumoricidal macrophages. 385 80

Local control of the primary tumour is a fundamental requirement for clinical cure. Towards this aim, the primary tumour must be diagnosed early and identified histologically. The size, extension, and spread within the patient must be defined precisely. In planning effective local therapy, additional questions must be answered including resectability, mutilation, sensitivity to radio- and chemotherapy, anticipated morbidity from therapeutic measures, etc. For osteosarcoma there is no reasonable alternative to radical surgery. Because of the 20% local recurrence rate of Ewing's sarcoma following radiotherapy, radical surgical removal of the primary tumour should be attempted whenever possible. For rhabdomyosarcoma, particularly for its embryonal histology, non-radical removal of the primary sarcoma is still compatible with a cure, provided adequate radio- and chemotherapy is also administered. Primary irradiation is indicated in radiosensitive unresectable primary tumours and may convert these into an operable state. Chemotherapy is the domain of prevention and treatment of metastatic disease. It has, however, also a proved effect on primary tumours and, in several recent protocols, precedes local therapy.
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PMID:Local therapy of rhabdomyosarcoma, osteosarcoma and Ewing's sarcoma of children and adolescents. 386 80

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


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