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)

Our focus was to develop an anti-angiogenic drug possessing the inhibitory activity of urokinase-type plasminogen activator (u-PA) production. During preliminary screening, the effects of 13 ozonides on the inhibition of u-PA production in human fibrosarcoma HT-1080 cells and on the inhibition of angiogenesis on chicken embryonic chorioallantoic membranes were determined. Of the ozonides tested, 9 inhibited in vitro u-PA production of HT-1080 cells and 7 of these 9 exhibited strong anti-angiogenic activity. Interestingly, 6 of the 13 ozonides also inhibited cathepsin B activity. 1-Phenyl-1, 4-epoxy-1H,4H-naphtho[1,8-de][1, 2]dioxepin (ANO-2) potently inhibited cathepsin B (IC(50) = 0.47 microM) as well as u-PA production. Consequently, ANO-2 was selected for further study. ANO-2 inhibited tube formation by human umbilical vein endothelial cells cultured on Matrigel while exhibiting no cytotoxicity. Additionally, in vivo administration of ANO-2 inhibited angiogenesis induced by mouse Sarcoma-180 cells tested using the mouse dorsal air sac assay. Moreover, ANO-2 also suppressed primary tumor growth and reduced the number of pulmonary metastases caused by Lewis lung carcinoma cells in mice. These in vitro and in vivo activities indicate that ANO-2 has considerable potential as a new and potent anti-angiogenic drug that inhibits both u-PA production and enzymatic activity of cathepsins, indicating that ANO-2 may be a multifunctional inhibitor of angiogenesis.
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PMID:Multifunctional anti-angiogenic activity of the cyclic peroxide ANO-2 with antitumor activity. 1211 73

Synovial sarcoma (SS), an aggressive neoplasm accounting for up to 14% of soft tissue sarcomas, was recently recognized as a primary tumor in the lung and pleura. SS is characterized by the chromosomal translocation t(X;18)(SYT-SSX) found in more than 95% of the tumors. We report a cooperative study from the French Sarcoma Group and the Mesopath Group on 40 t(X;18)(SYT-SSX)-positive primary intrathoracic SS. There were 22 males and 18 females, whose age ranged from 16 to 79 years (median, 47 years). Neoplasms were mostly circumscribed and of large size (median, 7.5 cm; range, 2-16 cm). Thirty-nine tumors were monophasic SS, including 24 (60%) monophasic fibrous and 15 (37.5%) poorly differentiated cases, and one lesion was a biphasic SS. A larger proportion of poorly differentiated tumors were observed among intrathoracic SS as compared with soft tissue SS. Immunohistochemically, 90% of the cases reacted with at least one epithelial marker. CD34 was focally expressed in 3 cases. SYT-SSX1 fusion transcripts were detected in 22 cases (56.4%) and SYT-SSX2 fusion transcripts in 17 cases. Median and 5-year disease-specific survival in 33 patients was 50 months and 31.6%. Median and 5-year disease-free survival was 24 months and 20.9%. Patient sex, age, tumor size, histologic subtype, grade, and SYS-SSX fusion type had no significant impact on outcome. In conclusion, intrathoracic SS are rare but aggressive tumors with poor prognosis. In this unusual location, the detection of SYT-SSX fusion transcripts is a valuable diagnostic adjunct.
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PMID:Primary intrathoracic synovial sarcoma: a clinicopathologic study of 40 t(X;18)-positive cases from the French Sarcoma Group and the Mesopath Group. 1572 2

Primary malignant neoplasms of the liver are some of the most uncommon malignancies in many parts of the world. They include hepatocellular carcinoma and stromal tumors such as hepatic angiosarcoma. It is a lethal tumor with life expectancy of less than six months. Once discovered, it is often too late for surgical intervention. Like other vascular tumors of the liver and spleen, intraperitoneal hemorrhage is a well-documented finding of angiosarcoma which can be lethal if not diagnosed and treated immediately. As in our case, intraperitoneal hemorrhage from primary tumor rupture was the only clinical presentation of this neoplasm. Approximately 15% of patients present with acute hemoperitoneum from either tumor rupture or peritoneal metastasis. Although several therapeutic options are available, we describe apalliative therapy for hepatic angiosarcoma utilizing transcatheter arterial chemoembolization (TACE) techniques incorporating the newer embolization agent Embospheres to locally target and treat this aggressive tumor.
Sarcoma 2007
PMID:Hepatic angiosarcoma presenting as an acute intraabdominal hemorrhage treated with transarterial chemoembolization. 1828 42

Purpose. Original articles and abstracts published between January 1991 and January 1997 were selected according to specified criteria and reviewed to provide answers to five interesting questions about the systemic treatment of metastatic osteosarcoma.Results.(1) In patients with metastatic disease at presentation, what is the outcome after intensive multi-agent chemotherapy?Historically, survival has been poor, but may be improving with the use of ifosfamide-containing regimens.(2) Can response to new agents be evaluated better in patients who have received no previous chemotherapy?Based on limited data, this is probably true.(3) Is the response to neo-adjuvant chemotherapy, as determined by histopathology, similar for the primary tumor and synchronous pulmonary metastases?With intensive multi-agent chemotherapy, good histological response rates are in the range 70-90% for both groups.(4) What is the outcome, after intensive combined modality treatment with chemotherapy and surgery, in patients relapsing with metastases after previous adjuvant chemotherapy, and what are the important prognostic factors?Outcome is highly variable, but 5-year survival ranges between 25 and 50% and a good outcome is more likely if recurrent disease is limited to resectable lung metastases.(5) Can a biological agent (L-MTP-PE) prolong the time to relapse in patients with resected metastatic osteosarcoma?Preliminary data suggest that this is possible, but more studies are required.
Sarcoma 1997
PMID:Metastatic osteosarcoma: a review of current issues in systemic treatment. 1852 Dec 13

Purpose. Angiosarcoma is a rare tumor with endothelial cell differentiation that may arise in any anatomic location.The purpose of this report was to identify prognostic factors on outcome in a group of prospectively followed patients with confirmed angiosarcoma.Subjects. Adult patients (>16 years old) with angiosarcoma treated between July 1982 and February 1998 were identified from a prospective database.Methods. Pathologic confirmation of all cases was performed prior to inclusion in this analysis. Various prognostic factors were evaluated for disease-specific survival. Survival was determined by the Kaplan- Meier method. Statistical significance was evaluated by log-rank test for univariate analysis and Cox stepwise regression for multivariate analysis (p<0.05).Results. Fifty patients were identified; at the initial evaluation, this group included 32 patients with a primary tumor, three with local recurrence and 15 with metastatic disease. Tumor sites included 16 head and neck and skin of head, eight extremity, seven trunk, six breast, five pelvis, four viscera and four thoracic. Median follow-up among survivors was 71 months (range, 38-191 months).Two- and 5-year disease-specific survival was 50 and 30%, respectively, with a median of 24 months. The factor predictive of tumor-related mortality was presentation status (p=0.001; relative risk, 5). Two-year disease-specific survival for patients presenting with recurrent or metastatic disease was 13%, compared with 70% for those with primary disease.
Sarcoma 2000
PMID:Confirmed angiosarcoma: prognostic factors and outcome in 50 prospectively followed patients. 1852 Dec 98

Purpose. Tc-99m tetrofosmin launched for myocardial studies has recently also shown a good detectability for several tumors. Data on PNET imaging by Tc-99m tetrofosmin are not yet available.Patient and method. In the case of a 21-year-old man suffering from pelvis PNET, Tc-99m tetrofosmin scintigraphy was performed additionally to CT and MRI.Results. The gluteal and iliac tumor masses were visualized by Tc-99m tetrofosmin according to the CT and MRI results.Discussion. Imaging with Tc-99m tetrofosmin could provide additional information to the available conventional radiological imaging modalities for diagnosis of PNET, and could be a useful tool for the restaging of the primary tumor.
Sarcoma 2001
PMID:Imaging of Primitive Neuroectodermal Tumor (PNET) by Technetium-99m Tetrofosmin. 1852 17

Background. Epstein-Barr virus (EBV)-related smooth muscle neoplasms (SMNs) have been associated with immune dysregulation, most notably in patients who have undergone solid organ transplantation or in patients with HIV/AIDS. Objective. to report our experience with EBV-related neoplasms as well as describing the first EBV-related SMN in the setting of administration of glucocorticoids and the tumor necrosis factor inhibitor etanercept. Design. We have case reports, of minimum 3-year follow-up, 2002-2005. Setting. It was held in an academic and tertiary referral cancer center. Patients. Patients are with dysregulated immunity after solid organ transplantation, HIV/AIDS, or with psoriasis after treatment with etanercept. Interventions. There were discontinuation of etanercept, right hepatic trisegmentectomy, and chemotherapy. Measurements. We use survival as a measurement here. Results. Patients who were able to withstand reduction in immunosuppression survived. Surgical resection or chemotherapy was successful in delaying progression of disease. Limitations. There was a relatively short follow-up for these slow-growing neoplasms. Conclusion. EBV-related SMNs have variable aggressiveness. While chemotherapy may slow disease progression, resection and improving the host immune status provide the best opportunity for primary tumor control.
Sarcoma 2008
PMID:EBV-Associated Smooth Muscle Neoplasms: Solid Tumors Arising in the Presence of Immunosuppression and Autoimmune Diseases. 1907 88

We report a case of extraskeletal osteosarcoma (ESOS) and autopsy findings. A 35-year-old man presented with an ossified tumor in the right thigh and lung metastasis. The lung tumors continued to develop despite multiagent chemotherapy and caused death within 8 months. Autopsy revealed many secondary lesions in the lungs, especially in the left lung. Histopathologically, the primary tumor and one of the secondary tumors showed proliferation of spindle-shaped tumor cells focally forming lace-like osteoid material. Therefore, we made a definite diagnosis of ESOS.
Sarcoma 2009
PMID:Extraskeletal osteosarcoma of the thigh: an autopsy case report. 1975 30

The Ewing Sarcoma Family Tumors (ESFT) consist of the classical pathologic entities of Ewing Sarcoma and peripheral Primitive Neuroectodermal Tumor. Occurring largely in the childhood through young adult years, these tumors have an unsurpassed propensity for metastasis and have no defined cell of origin. The biology of these aggressive malignancies centers around EWS/FLI1 and related EWS/ETS chimeric transcription factors, which are largely limited to this tumor class. Much progress has been made in the identification of a network of loci whose expression is modulated by EWS/FLI1 and its congeners. To date, little progress has been made in reconstructing the sequence of direct and indirect events that produce this network of modulated loci. The recent identification of GLI1 as an upregulated target of EWS/ETS transcription factors suggests a target which may be a more central mediator in the ESFT signaling network. In this paper, we further define the relationship of EWS/FLI1 expression and GLI1 upregulation in ESFT. This relationship is supported with data from primary tumor specimens. It is consistently observed across multiple ESFT cell lines and with multiple means of EWS/FLI1 inhibition. GLI1 inhibition affects tumor cell line phenotype whether shRNA or endogenous or pharmacologic inhibitors are employed. As is seen in model transformation systems, GLI1 upregulation by EWS/FLI1 appears to be independent of Hedgehog stimulation. Consistent with a more central role in ESFT pathogenesis, several known EWS/FLI1 targets appear to be targeted through GLI1. These findings further establish a central role for GLI1 in the pathogenesis of Ewing Tumors.
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PMID:GLI1 is a central mediator of EWS/FLI1 signaling in Ewing tumors. 1985 63

Osteosarcoma is the most frequent malignant primary bone tumor characterized by a high potency to form lung metastases which is the main cause of death. Unfortunately, the conventional chemotherapy is not fully effective on osteosarcoma metastases. The progression of a primary tumor to metastasis requires multiple processes, which are neovascularization, proliferation, invasion, survival in the bloodstream, apoptosis resistance, arrest at a distant organ, and outgrowth in secondary sites. Consequently, recent studies have revealed new insights into the molecular mechanisms of metastasis development. The understanding of the mechanism of molecular alterations can provide the identification of novel therapeutic targets and/or prognostic markers for osteosarcoma treatment to improve the clinical outcome.
Sarcoma 2012
PMID:Molecular alterations associated with osteosarcoma development. 2244 23


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