Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The role of radiotherapy and adjuvant chemotherapy in the primary treatment of osteogenic sarcomas and of Ewing's sarcoma is reviewed. In osteosarcoma radiotherapy can take the form of prophylactic total irradiation of the lung, but preoperative irradiation of the primary tumor has not proved successful. On the other hand, in Ewing's sarcoma primary and local irradiation is the therapy of choice, and is followed by adjuvant polychemotherapy over a long period.
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PMID:[The role of radiotherapy in the treatment of malignant bone tumors (author's transl)]. 106 Sep 7

Radiation-associated sarcomas are uncommon, constituting less than 5% of all sarcomas, and generally associated with a poor prognosis. We reviewed the medical records of 565 patients with sarcoma and a second malignancy seen at our institution between 1943 and 1989; 160 of these patients (28%) were considered to have a radiation-associated sarcoma. The most common diagnosis for which radiation had been given was breast cancer (26%), followed by lymphoma (25%) and carcinoma of the cervix (14%). The most common histologic types of radiation-associated sarcoma were osteogenic (21%), malignant fibrous histiocytoma (16%), and angiosarcoma/lymphangiosarcoma (15%). Most of the tumors were high grade (87%). Three variables had prognostic significance in multivariate analysis: the presence of metastatic disease, the completeness of operative resection in patients with localized disease, and the size of the primary tumor in patients who underwent complete resection of the sarcoma. Survival was independent of histologic subtype or site of disease.
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PMID:Radiation-associated sarcoma of bone and soft tissue. 136 80

From January 1978 to May 1983, 41 patients with primary high-grade osteogenic osteosarcoma of a limb were treated with a combination of intensive chemotherapy and prophylactic lung irradiation (PLI) intercalated between the first two cycles of chemotherapy. The primary tumor was treated according to its size and location by amputation, resection, high-dose radiotherapy, and salvage amputation for a tumor progressing under radiotherapy. Two weeks after surgery or simultaneously with radiotherapy, a three-drug regimen (cycle A) consisting of mitomycin C on day 1, vincristine followed by a 6-hour infusion of methotrexate on day 2 was given. Folinic acid rescue was started 6 hours after the end of the methotrexate infusion. A PLI of 20 G was given from day 10 to 22. On day 28, a four-drug regimen (cycle B) combining doxorubicin on day 1, vincristine on day 2 and dacarbazine with cyclophosphamide on days 3 to 6 was administered. Thereafter, five additional cycles of A and B were administered provided that the absolute number of polymorphonuclear cells and platelets had recovered. When these values were not attained, treatment was delayed until recovery. After a mean follow-up of 60.6 months, 16 patients have developed distant metastases, associated in four cases with local recurrence. Sixteen patients have died: 15 with metastases, one with no evidence of disease (toxic death). The overall survival of the entire group is 66% and the continuously disease-free survival 58% at 5 years. Alopecia, nausea, vomiting, asthenia, anorexia, and infraclinical and reversible impairment of lung ventilatory function were universal. A noticeable hematologic toxicity also was seen. One toxic death occurred after a pulmonary infection. Two patients developed cardiomyopathy. A multiparametic analysis of prognostic factors shows the very significant influence of age on treatment outcome. The continuous disease-free survival among the 17 patients younger than 15 years is 41% compared to 79% in older patients. The prognostic influence of age was independent of other factors. The delay (for more than two cycles) of methotrexate administration was the second independent prognostic factor. These results raise the question of using different protocols of adjuvant chemotherapy for patients younger or older than 15 years in order to optimize the curability/toxicity ratio.
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PMID:Age and dose of chemotherapy as major prognostic factors in a trial of adjuvant therapy of osteosarcoma combining two alternating drug combinations and early prophylactic lung irradiation. French Bone Tumor Study Group. 312 57

Eight patients who had large sarcomas in the hip, thigh, or shoulder girdle have been described. Three had osteogenic sarcomas, and one each had Ewing's sarcoma, biphasic synovial sarcoma, pleomorphic liposarcoma, undifferentiated spindling sarcoma, and malignant fibrous histiocytoma. All eight tumors showed evidence of regression after intraarterial infusion of cisplatin and Adriamycin (doxorubicin) given over 48 hours at 3-week intervals, for a total of between three and seven courses. Tru-cut needle biopsy specimens of five of the lesions were normal after chemotherapy. However, after resection of the regressed fibrotic tumor in seven of the patients, four contained foci of probably viable malignant cells. These cell foci were intraosseous in three cases and in the wall of a cyst in one case. In the remaining case, tumor in the distribution of the infused artery regressed, but tumor in a region supplied by an artery that was not infused continued to enlarge. In one patient with osteogenic sarcoma in the pelvis, despite a good response to intraarterial chemotherapy that was followed by surgical resection and radiotherapy, tumor recurred in an adjacent area in tissues supplied by an artery not infused. A hindquarter amputation subsequently was required. With the exception of the two cases in which adequate tumor arterial infusion was not achieved, local primary tumor control was accomplished by intraarterial infusion chemotherapy followed by local resection or radiotherapy and local resection in all patients. Four patients are well without evidence of residual or metastatic sarcoma 3.5 years after presentation in the case of an osteogenic sarcoma of shoulder, 2.5 years after presentation in the case of a large pleomorphic liposarcoma of thigh and groin, 20 months after presentation in the case of lower-thigh malignant fibrous histiocytoma, and 1 year after presentation in a child with an osteogenic sarcoma of lower femur.
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PMID:Regional chemotherapy with the use of cisplatin and doxorubicin as primary treatment for advanced sarcomas in shoulder, pelvis, and thigh. 347 53

In osteogenic sarcomas of the extremities, radical therapy of the primary tumor now seems more precisely delineated. In most cases, especially in large tumors with lytic lesions or fractures, and in children. Irradiation is indicated only in small tumors with condensation, and in tumors of the proximal metaphysis of the humerus. Conservative surgical treatment with carcinologically satisfactory resection of involved bone followed by reconstruction, is currently under evaluation. Such treatment is indicated in specific cases: children in the final stage of growth, and small tumors without major involvement of soft tissues that respond to preoperative chemotherapy.
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PMID:[Osteogenic sarcomas of the limbs. Radical treatment of the primary tumor: choosing between irradiation and amputation ]. 628 45

Twenty-nine patients with osteogenic osteosarcoma of a limb underwent both pulmonary radiotherapy and chemotherapy immediately after treatment of the primary tumor, mainly by radical surgery or radiotherapy (80 grays/tumor). Chemotherapy consisted of alternate A and B cycles every four weeks and BCG scarifications between cycles. Cycle A combines vincristine, ameticine, methotrexate and folinic acid; cycle B consists of adriamycin, vincristine, imidazole carboxamide and cyclophosphamide. A 20 gray irradiation was delivered to the thorax between the first A and B cycles. 50% of patients had no local recurrence or metastases after three years. 70% are alive at three years. Toxicity of this protocol is mainly hematologic and bronchopulmonary (with one fatal infection). Alopecia and minor digestive toxicity were recorded in all patients. Severe cardiotoxicity was seen in only one case. Longer follow-up is needed to evaluate long-term toxicity, particularly bronchopulmonary side-effects.
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PMID:[Adjuvant therapy of osteogenic osteosarcomas of the limb: results of the SO4 78 trial]. 629 Nov 57

During an 18 month period, 41 patients who had previously had a primary tumor removed, underwent 60 operations for resection of newly detected radiographic pulmonary nodules. Primary tumors included 16 osteogenic sarcomas, 9 malignant melanomas, 12 soft tissue sarcomas, 1 Ewing's sarcoma, 2 adenocarcinomas of the rectum, and 1 testicular teratocarcinoma. One hundred and eight-one pulmonary specimens were submitted for pathologic examination and 149 discrete nodules were found in these specimens by the pathologist (83%). Metastases represented 76-100% of nodules removed in 68% of the operations, 51-75% of nodules in 12%, 26-50% of nodules in 14%, and less than 25% of nodules in 6% of the cases. Metastatic disease was found in 90% of cases where nodules were greater than or equal of 0.5 cm in diameter, while metastases were found in on 33% of cases where the largest nodules removed were less than 0.5 cm. In only two cases did the histology of the pulmonary metastases differ from that of the primary tumor. We conclude that although benign lesions cannot be distinguished from metastases except by resection, nodules less than 0.5 cm in diameter probably should be observed for a further increase in size prior to operation.
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PMID:Histological evaluation of the nodules resected in the treatment of pulmonary metastatic disease. 695 74

This study was performed to analyze the relevance of iliac crest biopsy in patients with primary breast cancer with regard to metastases of the primary tumor and osteogenic disease. We performed intraoperative bilateral biopsy of the anterior iliac crests in 1465 patients with primary breast cancer. The bone specimens were histologically evaluated with regard to quality of the biopsy, tumor involvement, and osteogenic and hematogenic disease. Accurate and clear evaluation of the iliac crest biopsies was possible in 1365 patients (93%). Osteopenia was diagnosed in 48 patients (3.5%); 24 patients (1.7%) showed histological evidence of tumor involvement of the skeletal system. All these 24 patients received systemic (adjuvant) therapy after surgery. Ten patients had micrometastases, although in 5 of them both the postoperative bone scan and X-rays showed no pathological results. In 10 women with histologically negative bone biopsies, metastases to the bone were diagnosed by bone scan and radiological methods. Random perioperative iliac bone biopsy cannot be recommended in patients with primary breast cancer. Iliac crest biopsy is relevant in certain scenarios (e.g. suspected recurrence, doubtful bone scan).
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PMID:Results of iliac crest biopsies taken from 1465 patients with primary breast cancer. 949 34

Well-differentiated liposarcomas (WDLPS), especially those located in the retroperitoneum, may occasionally undergo dedifferentiation. Although this process is associated with a more aggressive clinical course, dedifferentiated liposarcomas rarely produces metastases. The case reported here is rather uncommon: A retroperitoneal WDLPS gave lung metastases that were diagnosed as highly malignant osteosarcomas. We used comparative genomic hybridization (CGH), fluorescence in situ hybridization (FISH), and Southern blot analyses to characterize the copy number changes and genetic aberrations occurring at different stages of the disease. In the primary tumor, the only detectable aberration was amplification of 12q13-q14, which was present only in a fraction of the cells and revealed by FISH analysis. High-level amplification of 12q13-q14, involving CDK4, MDM2, and HMGIC, was seen both in the relapse and the metastases. The second most common change, gain or high-level amplification of 1q22-q24, was detectable by CGH only in the osteogenic metastases, as was loss of the distal 2q. FISH analyses revealed considerable heterogeneity in the samples, and the percentage of cells showing aberrations was significantly higher in the metastatic samples. In particular, increased copy numbers of 789f2, a marker for 1q21 amplification in sarcomas, was observed in more than 65% of the cells in the metastatic samples, but in less than 10% of the cells from the recurrent samples. These observations could indicate that 1q amplification, in particular, may be indicative of a more malignant phenotype and ability of metastasis in WDLPS, as has also been suggested by others.
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PMID:Dedifferentiation of a well-differentiated liposarcoma to a highly malignant metastatic osteosarcoma: amplification of 12q14 at all stages and gain of 1q22-q24 associated with metastases. 1136 52

The SVEP1 protein comprises modules related to the selectin super family and other motifs found in cell surface molecules. Earlier, we demonstrated that SVEP1 is expressed in osteogenic cells both in vivo and in vitro; in the current study we elaborate on the regulation of SVEP1 by 17beta-Estradiol (17betaE2). SVEP1 message is expressed in vivo by bone marrow cells of sham-operated rats, but not in estrogen-depleted ovariectomized (OVX) rats. We demonstrated that 17betaE2 treatment increases the level of the SVEP1 expression in cultured osteoblasts. SVEP1 was identified also in breast carcinoma (BC) cells known to reside in bone when metastasized from the primary tumor. SVEP1 expression was demonstrated by immunohistochemistry and fluorescence-activated cell sorting (FACS) on various BC cell lines. The chromatin immunoprecipitation (ChIP) assay was applied to analyze the estrogen receptor (ER) binding to the putative SVEP1 promoter. We demonstrated that treatment with 17betaE2 or ICI 182,780 affects this binding and regulates the mRNA and protein levels of SVEP1 in BC cells. We propose that SVEP1 may serve as a useful biomarker for studying the mechanism of cells interactions within the local microenvironment affected by estrogen.
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PMID:SVEP1 expression is regulated in estrogen-dependent manner. 1713 25


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