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1

Ewing sarcoma  

MedlinePLUS

Anderson ME, Randall RL, Springfield DS, Gebhardt MC. Sarcomas of bone. In: Niederhuber JE, Armitage JO, Doroshow JH, et al., eds. Abeloff's Clinical Oncology . 5th ed. Philadelphia, Pa: Elsevier Churchill Livingstone; ...

2

Collecting and Storing Biological Samples From Patients With Ewing Sarcoma  

ClinicalTrials.gov

Askin Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

2014-08-26

3

Primary Ewing's Sarcoma of the Lung  

PubMed Central

Most cases of Ewing's sarcoma are reported in the bone, and extraosseous Ewing's sarcoma is an extremely rare disease. Here, we report a rare case of primary pulmonary Ewing's sarcoma in a patient with hemoptysis. The patient underwent right upper lung lobe lobectomy with adjuvant chemotherapy and radiation therapy and has been free of recurrent disease for 4 years. PMID:24570867

Hwang, Su Kyung; Park, Seung-Il; Kim, Yong-Hee; Kim, Hyeong Ryul

2014-01-01

4

CENTER FOR SARCOMA AND BONE ONCOLOGY EWING'S SARCOMA  

E-print Network

for a particular subtype of ovarian cancer have yielded promising results, encouraging further clinical development Hospital into a clinical trial testing a new class of therapeutic drug against Ewing's sarcoma. For reasons still unknown, PARP inhibition led to a uniquely powerful anti- cancer effect in Ewing's sarcoma

Liu, Xiaole Shirley

5

Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors  

ClinicalTrials.gov

Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

2014-06-09

6

WT1 regulates angiogenesis in Ewing Sarcoma  

PubMed Central

Angiogenesis is required for tumor growth. WT1, a protein that affects both mRNA transcription and splicing, has recently been shown to regulate expression of vascular endothelial growth factor (VEGF), one of the major mediators of angiogenesis. In the present study, we tested the hypothesis that WT1 is a key regulator of tumor angiogenesis in Ewing sarcoma. We expressed exogenous WT1 in the WT1-null Ewing sarcoma cell line, SK-ES-1, and we suppressed WT1 expression using shRNA in the WT1-positive Ewing sarcoma cell line, MHH-ES. Suppression of WT1 in MHH-ES cells impairs angiogenesis, while expression of WT1 in SK-ES-1 cells causes increased angiogenesis. Different WT1 isoforms result in vessels with distinct morphologies, and this correlates with preferential upregulation of particular VEGF isoforms. WT1-expressing tumors show increased expression of pro-angiogenic molecules such as VEGF, MMP9, Ang-1, and Tie-2, supporting the hypothesis that WT1 is a global regulator of angiogenesis. We also demonstrate that WT1 regulates the expression of a panel of pro-angiogenic molecules in Ewing sarcoma cell lines. Finally, we found that WT1 expression is correlated with VEGF expression, MMP9 expression, and microvessel density in samples of primary Ewing sarcoma. Thus, our results demonstrate that WT1 expression directly regulates tumor angiogenesis by controlling the expression of a panel of pro-angiogenic genes. PMID:24810959

Katuri, Varalakshmi; Gerber, Stephanie; Qiu, Xiaofei; McCarty, Gregory; Goldstein, Seth D.; Hammers, Hans; Montgomery, Elizabeth; Chen, Allen R.; Loeb, David M.

2014-01-01

7

MicroRNAs in Ewing Sarcoma  

PubMed Central

MicroRNAs (miRs) have emerged recently as important regulators of gene expression in the cell. Frequently dysregulated in cancer, miRs have shed new light on molecular mechanisms of oncogenesis, and have generated substantial interest as biomarkers, and novel therapeutic agents and targets. Recently, a number of studies have examined miR biology in Ewing sarcoma. Findings indicate that alterations in miR expression in Ewing Sarcoma are widespread, involve both EWS/Ets oncogenic fusion-dependent and independent mechanisms, and contribute to malignant phenotypes. miRs with prognostic potential have been identified, and several preclinical studies suggest that miR manipulation could be therapeutically useful in this aggressive disease. These and future studies of miR biology stand to expand our understanding of Ewing sarcoma pathogenesis, and may identify new biomarkers and treatment options. PMID:23543617

Dylla, Layne; Moore, Colin; Jedlicka, Paul

2013-01-01

8

Primary cutaneous Ewing sarcoma - Case report*  

PubMed Central

Ewing sarcoma is a primitive neuroectodermal tumor rarely occurs in the skin and sobcutaneous tissues. Generally Ewing's sarcoma is a primary bone tumor, but when present in soft tissues it characterizes an extremely uncommon clinical picture. It usually involves the deep subcutaneous tissue or muscles, and more rarely occurs like a primary skin cancer. Most patients are white, women, and in the second decade of life. The clinical features are a superficial mass, in average measuring 2-3 cm, of soft consistency, freely mobile and sometimes painful. The more affected locations are upper and lower extremities, trunk, head, neck or multiple lesions. The presence of metastases is very rare. PMID:24937829

de Oliveira, Jayme; Tebet, Ana Carolina Franco; de Oliveira, Anna Rita Ferrante Mitidieri; Nasser, Kassila

2014-01-01

9

Novel Combination Chemotherapy for Localized Ewing Sarcoma  

Cancer.gov

In this clinical trial, researchers will test whether the addition of the drug combination vincristine, topotecan, and cyclophosphamide to a standard chemotherapy regimen improves overall survival and event-free survival in newly diagnosed patients with non-metastatic Ewing sarcoma of the bone or soft tissue (excluding the soft tissue of the skull).

10

Cluster analysis of immunohistochemical profiles in synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma  

Microsoft Academic Search

As a result of overlapping morphologic and immunohistochemical features, it can be difficult to distinguish synovial sarcoma, malignant peripheral nerve sheath tumor, and Ewing sarcoma\\/primitive neuroectodermal tumor in core biopsies. To analyze and compare immunohistochemical profiles, we stained tissue microarrays of 23 synovial sarcomas, 23 malignant peripheral nerve sheath tumors, and 27 Ewing sarcomas with 22 antibodies potentially useful in

Stephen H Olsen; Dafydd G Thomas; David R Lucas

2006-01-01

11

An Unusual Location of Extraosseous Ewing's Sarcoma  

PubMed Central

Ewing's sarcoma (ES) is the second most common malignant bone tumor in children and young adults. ES also occurs as a primary soft tissue neoplasm without involvement of bone. We report the second case of extraosseous (EO) ES emerging from the omentum and a review of the relevant literature. EO ES should be included in the differential diagnosis of soft tissue neoplasms in the abdomen. PMID:23898272

Geens, Lisanne; Robays, Johan Van; Geert, Verswijvel; der Speeten, Kurt Van

2013-01-01

12

Combination Chemotherapy in Treating Patients With Non-Metastatic Extracranial Ewing Sarcoma  

ClinicalTrials.gov

Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Childhood Supratentorial Primitive Neuroectodermal Tumor; Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Extraosseous Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Peripheral Primitive Neuroectodermal Tumor of the Kidney; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

2014-09-11

13

Cytological characterization and histogenesis of Ewing's sarcoma.  

PubMed

In addition to a light-microscopical and histochemical investigation of primary and metastic lesions in 27 cases of Ewing's sarcoma, biopsy materials from the primary bone lesions of 7 patients with this neoplasm were examined histochemically, enzyme-cytochemically and electron microscopically to elucidate the histogenesis and nature of the neoplasm. Ultrastructural observation has revealed that besides intracytoplasmic and extracellular deposition of glycogen the tumor cells possess several cytological features characterized by intracytoplasmic microfilaments of varying thickness up to 80 A, occasional appearance of dense patches, fat droplets, desmosomal connections and reminiscent attachment bodies. The tumor cells are mostly round, oval or polygonal in shape, but spindle or elongated cells are intermingled and occasionally contain well-developed rough endoplasmic reticulum, resembling pericytes or fibroblasts. In the intercellular spaces amongst the tumor cells, varying amounts of variable-shaped amorphous materials are found, which are compatible with acid mucopolysaccharides and glycoproteins histochemically verified. These findings may suggest that Ewing's sarcoma is a highly malignant neoplasm originating from a transitional cell developed from pericytes to vascular smooth muscle cells in the bone marrow. PMID:132079

Kojima, M

1976-03-01

14

Ewing's sarcoma of maxilla: A rare case report  

PubMed Central

Ewing's sarcoma is uncommon malignancy of childhood, frequently involving the mandible. The occurrence in maxilla is rare. It is histopathologically characterized by sheets of round cells positive for CD99. Although the prognosis is poor but early diagnosis and long term follow up can improve the survival. This article presents a rare case of Ewing's sarcoma of maxilla in a 15 year old male patient showing excessive fibro-osseous response which is not a frequent presentation. A retrospective analysis of cases of Ewings sarcoma of maxilla published in the English litreture is reviewed. In our case, diagnosis was confirmed by immunohistochemistry where sheets of round tumor cells were positive for CD 99. Ewings sarcoma of maxilla is a rare and aggressive tumor. Hence early diagnosis, combined therapy and long term follow up is suggested in such cases.

Jairamdas Nagpal, Deepak Kumar; Prabhu, Prashant Ramesh; Palaskar, Sangeeta Jayant; Patil, Swati

2014-01-01

15

Ewing's Sarcoma: An Uncommon Breast Tumor  

PubMed Central

Ewing’s sarcoma/primitive neuroectodermal tumors (EWS/PNET) are rare malignant and aggressive tumors, usually seen in the trunk and lower limbs of children and young adults. They are uncommon in the breast. We report a case of a 43-year-old woman who developed a painless breast mass. An initial core needle biopsy concluded to a fibrocystic dystrophy contrasting with a rapidly growing mass; thus a large lumpectomy was done. Diagnosis of primary PNET of the breast was established, based on both histopathological examination and immunohistochemical findings. Surgical margins were positive, therefore, left modified radical mastectomy with axillary lymph nodes dissection was performed. The patient was given 6 cycles of adjuvant chemotherapy containing cyclophosphamide, adriamycin and vincristine. Twenty months later, she is in life without recurrence or metastasis. EWS/PNET may impose a diagnostic challenge. Indeed, mammography and ultrasonography features are non specific. The histopathological pattern is variable depending on the degree of neuroectodermal differentiation. Immuno-phenotyping is necessary and genetic study is the only confirmatory tool of diagnosis showing a characteristic cytogenetic anomaly; t (11; 22) translocation. PMID:25332765

Meddeb, Sawsen; Rhim, Mohamed Salah; Kouira, Mouna; Mestiri, Sarra; Bibi, Mohamed; Yacoubi, Mohamed Tahar

2014-01-01

16

Poly(ADP-ribose) polymerase inhibitors in Ewing sarcoma  

PubMed Central

Purpose of review In 2012, two publications revealed a particular sensitivity of Ewing sarcoma cells to the inhibition of poly(ADP-ribose) polymerase (PARP). This review updates the reader on PARP function, the development of PARP inhibitors (PARPi) and the evidence for targeting PARP in Ewing sarcoma. It concludes with a description of ongoing/emerging PARPi clinical trials in patients with Ewing sarcoma. Recent findings PARP has a major role in DNA repair, and is a transcription regulator. The oncoprotein in Ewing sarcoma, EWS-FLI1, is proposed to interact with PARP-1, driving PARP-1 expression, which further promotes transcriptional activation by EWS-FLI1. Thus, there are two rationales for PARPi in the treatment of Ewing sarcoma: to disrupt the interaction between EWS-FLI1 and PARP, and for chemo-potentiation or radio-potentiation. The first clinical trial with a single agent PARPi failed to show significant responses, but preclinical evidence for combinations of PARPi with chemotherapy or radiotherapy is very promising. Summary Despite initial excitement for the potential of PARPi as single agent therapy in Ewing sarcoma, the emerging preclinical data now strongly support testing PARPi in combination with chemo/radiotherapy clinically. PMID:24840521

Vormoor, Britta; Curtin, Nicola J.

2014-01-01

17

BCOR-CCNB3 (Ewing-like) sarcoma: a clinicopathologic analysis of 10 cases, in comparison with conventional Ewing sarcoma.  

PubMed

BCOR-CCNB3 fusion transcripts resulting from an X-chromosomal paracentric inversion were recently identified in a series of unclassifiable soft tissue and bone sarcomas with Ewing sarcoma-like morphology. The morphologic and clinical features of these sarcomas are, as yet, not well characterized. Here we describe the clinicopathologic features of 10 cases of BCOR-CCNB3 sarcoma and compare their clinical course with typical Ewing sarcoma. Nine of 10 patients were male, and all were 11 to 18 years of age. Seven tumors were located in the bone and 3 in the deep soft tissues. The histomorphologic spectrum was quite wide, with 7 tumors predominately showing small primitive cell morphology with angulated nuclei simulating so-called atypical Ewing sarcoma and 3 predominately showing spindle cell morphology. Recurrent and metastatic lesions showed increased cellularity and marked pleomorphism. Immunohistochemistry showed expression of CCNB3 (100%), bcl2 (90%), CD99 (60%), and CD117 (60%). Reverse transcription polymerase chain reaction for BCOR-CCNB3 fusion transcripts was positive in all 9 cases, which yielded sufficient extracted RNA. Five- and 10-year survival rates were 75% and 56%, respectively. BCOR-CCNB3 sarcomas located in axial skeleton and soft tissues showed a significantly shorter survival. The Ewing sarcoma overall survival was not statistically different, although there was a trend for longer survival of patients with BCOR-CCNB3 sarcomas in the extremities. In conclusion, this study provides a detailed description of the histologic spectrum, immunohistochemical features, and clinical characteristic of BCOR-CCNB3 sarcoma justifying distinction from Ewing sarcoma with its typical EWS/FUS-ETS translocations. Ideally immunohistochemistry is used in combination with reverse transcription polymerase chain reaction for definitive diagnosis. PMID:24805859

Puls, Florian; Niblett, Angela; Marland, Gillian; Gaston, Czar Louie L; Douis, Hassan; Mangham, D Chas; Sumathi, Vaiyapuri P; Kindblom, Lars-Gunnar

2014-10-01

18

Ewing's Sarcoma: An Uncommon Breast Tumor.  

PubMed

Ewing's sarcoma/primitive neuroectodermal tumors (EWS/PNET) are rare malignant and aggressive tumors, usually seen in the trunk and lower limbs of children and young adults. They are uncommon in the breast. We report a case of a 43-year-old woman who developed a painless breast mass. An initial core needle biopsy concluded to a fibrocystic dystrophy contrasting with a rapidly growing mass; thus a large lumpectomy was done. Diagnosis of primary PNET of the breast was established, based on both histopathological examination and immunohistochemical findings. Surgical margins were positive, therefore, left modified radical mastectomy with axillary lymph nodes dissection was performed. The patient was given 6 cycles of adjuvant chemotherapy containing cyclophosphamide, adriamycin and vincristine. Twenty months later, she is in life without recurrence or metastasis. EWS/PNET may impose a diagnostic challenge. Indeed, mammography and ultrasonography features are non specific. The histopathological pattern is variable depending on the degree of neuroectodermal differentiation. Immuno-phenotyping is necessary and genetic study is the only confirmatory tool of diagnosis showing a characteristic cytogenetic anomaly; t (11; 22) translocation. PMID:25332765

Meddeb, Sawsen; Rhim, Mohamed Salah; Kouira, Mouna; Mestiri, Sarra; Bibi, Mohamed; Yacoubi, Mohamed Tahar

2014-06-18

19

A Molecular Function Map of Ewing's Sarcoma  

PubMed Central

Background EWS-FLI1 is a chimeric ETS transcription factor that is, due to a chromosomal rearrangement, specifically expressed in Ewing's sarcoma family tumors (ESFT) and is thought to initiate the development of the disease. Previous genomic profiling experiments have identified EWS-FLI1–regulated genes and genes that discriminate ESFT from other sarcomas, but so far a comprehensive analysis of EWS-FLI1–dependent molecular functions characterizing this aggressive cancer is lacking. Methodology/Principal Findings In this study, a molecular function map of ESFT was constructed based on an integrative analysis of gene expression profiling experiments following EWS-FLI1 knockdown in a panel of five ESFT cell lines, and on gene expression data from the same platform of 59 primary ESFT. Out of 80 normal tissues tested, mesenchymal progenitor cells (MPC) were found to fit the hypothesis that EWS-FLI1 is the driving transcriptional force in ESFT best and were therefore used as the reference tissue for the construction of the molecular function map. The interrelations of molecular pathways were visualized by measuring the similarity among annotated gene functions by gene sharing. The molecular function map highlighted distinct clusters of activities for EWS-FLI1 regulated genes in ESFT and revealed a striking difference between EWS-FLI1 up- and down-regulated genes: EWS-FLI1 induced genes mainly belong to cell cycle regulation, proliferation, and response to DNA damage, while repressed genes were associated with differentiation and cell communication. Conclusions/Significance This study revealed that EWS-FLI1 combines by distinct molecular mechanisms two important functions of cellular transformation in one protein, growth promotion and differentiation blockage. By taking MPC as a reference tissue, a significant EWS-FLI1 signature was discovered in ESFT that only partially overlapped with previously published EWS-FLI1–dependent gene expression patterns, identifying a series of novel targets for the chimeric protein in ESFT. Our results may guide target selection for future ESFT specific therapies. PMID:19404404

Kofler, Reinhard; Walker, Bob; Davis, Sean; Meltzer, Paul; Kovar, Heinrich

2009-01-01

20

Ewing sarcoma superimposed on a previous osteochondroma in multiple osteochondromatosis.  

PubMed

It has been reported that patients with hereditary multiple exostoses (called multiple osteochondromatosis by the World Health Organization) are at increased risk for malignant transformation of osteochondromas to secondary chondrosarcomas. A review of the literature found 14 cases showing transformation of osteochondromas into osteosarcomas; however, Ewing sarcoma has never been reported superimposed on an osteochondroma. This article presents the case of a boy who underwent biopsy of a previously existent osteochondroma for which the pathology report showed cytologic and immunohistochemical properties consistent with Ewing sarcoma. A 13-year-old boy with hereditary multiple exostoses (multiple osteochondromatosis) presented to an orthopedic clinic because of waxing and waning pain superficial to a previous osteochondroma on the lateral aspect of the right leg, below the knee, of 1 month's duration. On examination, inflammation was noted over a bony mass associated with tenderness to palpation of the affected area. There was no evidence of penetrating injury or trauma, and the patient reported no constitutional symptoms, including fever. Radiographs showed marked osteolysis and signs of periosteal reaction. Magnetic resonance imaging showed evidence of cortical bone erosion and extension of the mass into soft tissue. Malignant transformation was suspected, and the patient underwent biopsy. The pathology findings were consistent with Ewing sarcoma. The highly uncommon presentation of this malignancy must serve as a red flag to other physicians who treat patients with hereditary multiple exostoses. Ewing sarcoma tends to be of higher grade and have a worse prognosis than other malignancies that are more commonly seen in these patients. PMID:24762849

Marrero Barrera, Pablo A; Marrero Ortiz, Pablo V

2014-04-01

21

Non-metastatic Ewing's sarcoma of the ribs  

Microsoft Academic Search

From 1984 to 1997, 57 consecutive patients with non-metastatic Ewing's sarcoma of the ribs were treated according to multimodal French Society of Pediatric Oncology (SFOP) protocols EW 84, EW 88 and EW 93. The results of treatment were reviewed and analysed. Median age was 12 years. 34 patients had large tumours (greatest tumour dimension ?8 cm); pleural effusion was noted

N Sirvent; J Kanold; C Levy; J Dubousset; J. M Zucker; T Philip; M. C Demaille; A Robert; J. P Vannier; O Oberlin

2002-01-01

22

Prognostic implication of immunodetection of P glycoprotein in Ewing's sarcoma  

Microsoft Academic Search

Increased expression of P glycoprotein is associated with multidrug resistance in many cell lines. P glycoprotein has been detected in different human tumors. To assess the implication of multidrug resistance in the prognosis of Ewing's sarcoma the expression of P glycoprotein was studied immunohistochemically in pre- and post-therapeutic tumor tissues of 21 cases treated according to the CESS 81 or

A. Roessner; Y. Ueda; B. Bockhorn-Dworniczak; S. Blasius; A. Peters; P. Wuisman; J. Ritter; M. Paulussen; H. Jürgens; W. Böcker

1993-01-01

23

18F-FLT Positron Emission Tomography and Diffusion-Weighted Magnetic Resonance Imaging in Planning Surgery and Radiation Therapy and Measuring Response in Patients With Newly Diagnosed Ewing Sarcoma  

ClinicalTrials.gov

Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

2014-01-22

24

Primary Pulmonary Ewing's Sarcoma: Rare Cause of Superior Vena Cava Syndrome in Children  

PubMed Central

Ewing’s sarcoma is a common malignant bone tumour presenting in children and young adults. Rarely extra- skeletal soft tissues and visceral organs can also be the site of origin of Ewing’s sarcoma. Primary pulmonary Ewing’s sarcoma is an extremely rare malignancy which occurs in the paediatric population. We report an unusual case of primary pulmonary Ewing’s sarcoma in a nine year old girl who presented with features of superior vena cava syndrome in the emergency department. The diagnosis was confirmed pathologically both by light microscopy and immunohistochemistry. The patient was put on chemotherapy and surgery was planned but the patient expired within three days of starting chemotherapy.

Atwal, Swapndeep Singh; Garga, Umesh Chandra

2014-01-01

25

Review of therapeutic strategies for osteosarcoma, chondrosarcoma, and Ewing's sarcoma  

PubMed Central

Summary The most prevalent forms of bone cancer are osteosarcoma, chondrosarcoma, and Ewing’s sarcoma. Although chemotherapy and radiotherapy have replaced traditional surgical treatments, survival rates have undergone only marginal improvements. Current knowledge of the molecular pathways involved in each type of cancer has led to better approaches in cancer treatment. A number of cell signaling molecules are involved in tumorigenesis, and specific targets have been identified based on these signal transducers. This review highlights some of the important cellular pathways and potential therapeutic targets, tumor site-specific irradiation techniques, and novel drug delivery systems used to administer these drugs. PMID:21804475

Dai, Xing; Ma, Wei; He, Xijing; Jha, Rajiv Kumar

2011-01-01

26

Dilemmas Associated With Congenital Ewing Sarcoma Family Tumors  

PubMed Central

The case report by Meazza et al describes the rapidly fatal outcome of a child born with a congenital peripheral primitive neuroectodermal tumor (pPNET). The case report prompted us to ask several questions. (1) Is there such an entity as congenital pPNET? (2) Do translocation-negative Ewing sarcoma family tumors (ESFT) exist? (3) What is the outcome for newborns with congenital ESFT and how does this affect treatment options? PMID:18176172

Kim, Su Young; Tsokos, Maria; Helman, Lee J.

2009-01-01

27

RNAi phenotype profiling of kinases identifies potential therapeutic targets in Ewing's sarcoma  

Microsoft Academic Search

BACKGROUND: Ewing's sarcomas are aggressive musculoskeletal tumors occurring most frequently in the long and flat bones as a solitary lesion mostly during the teen-age years of life. With current treatments, significant number of patients relapse and survival is poor for those with metastatic disease. As part of novel target discovery in Ewing's sarcoma, we applied RNAi mediated phenotypic profiling to

Shilpi Arora; Irma M Gonzales; R. Tanner Hagelstrom; Christian Beaudry; Ashish Choudhary; Chao Sima; Raoul Tibes; Spyro Mousses; David O Azorsa

2010-01-01

28

N-(4-Hydroxyphenyl) Retinamide Potentiated Anti-tumor Efficacy of Genistein in Human Ewing’s Sarcoma Xenografts  

PubMed Central

Background Ewing’s sarcoma is a pediatric tumor that mainly occurs in soft tissues and bones. New therapeutic strategies are urgently needed for treatment of Ewing’s sarcoma. We examined for the first time the efficacy of N-(4-hydroxyphenyl) retinamide (4-HPR) and genistein (GST) alone and also in combination for controlling growth of human Ewing’s sarcoma SK-N-MC and RD-ES xenografts. Methods Efficacy of combination therapy was evaluated using histopathological parameters. Molecular mechanisms of combination therapy were detected using Western blotting and immunofluorescence microscopy. Results Histopathological examination of tumor sections showed that control group maintained characteristic growth of tumors, 4-HPR alone caused differentiation of tumor cells, GST alone induced apoptosis to some extent, and combination of 4-HPR and GST significantly induced apoptosis in both Ewing’s sarcoma xenografts. Time-dependent reductions in body weight, tumor volume, and tumor weight were also found. Combination therapy increased Bax:Bcl-2 ratio to trigger mitochondrial release of Smac/Diablo into the cytosol to down regulate the baculovirus inhibitor-of-apoptosis repeat containing (BIRC) proteins such as BIRC-2 and BIRC-3 and thereby promote apoptosis. Activation of caspase-3 and mitochondrial release of apoptosis-inducing factor (AIF) occurred in course of apoptosis. Down regulation of the survival factor NF-?B and the angiogenic factors VEGF and FGF2 and increase in caspase-3 activity controlled tumor growth. In situ immunofluorescent labelings showed overexpression of calpain, caspase-12, and caspase-3, and AIF in xenografts, indicating induction of cysteine proteases and AIF for apoptosis. Conclusions Results revealed that combination of 4-HPR and GST could be highly effective treatment for inhibiting Ewing’s sarcomas in vivo. PMID:21822457

Karmakar, Surajit; Choudhury, Subhasree Roy; Banik, Naren L.; Ray, Swapan K.

2011-01-01

29

Ewing Sarcoma Protein: A Key Player in Human Cancer  

PubMed Central

The Ewing sarcoma protein (EWS) is a well-known player in cancer biology for the specific translocations occurring in sarcomas. The EWS-FLI1 gene fusion is the prototypical translocation that encodes the aberrant, chimeric transcription factor, which is a landmark of Ewing tumors. In all described Ewing sarcoma oncogenes, the EWS RNA binding domains are completely missing; thus RNA binding properties are not retained in the hybrid proteins. However, it is currently unknown whether the absence of EWS function in RNA metabolism plays a role in oncogenic transformation or if EWS plays a role by itself in cancer development besides its contribution to the translocation. In this regard, recent reports have highlighted an essential role for EWS in the regulation of DNA damage response (DDR), a process that counteracts genome stability and is often deregulated in cancer cells. The first part of this review will describe the structural features of EWS and its multiple roles in the regulation of gene expression, which are exerted by coordinating different steps in the synthesis and processing of pre-mRNAs. The second part will examine the role of EWS in the regulation of DDR- and cancer-related genes, with potential implications in cancer therapies. Finally, recent advances on the involvement of EWS in neuromuscular disorders will be discussed. Collectively, the information reviewed herein highlights the broad role of EWS in bridging different cellular processes and underlines the contribution of EWS to genome stability and proper cell-cycle progression in higher eukaryotic cells. PMID:24082883

2013-01-01

30

Ewing sarcoma of the proximal phalanx: case report.  

PubMed

Abstract We report a case of primary Ewing sarcoma of the proximal phalanx of the right middle finger in an 18-year-old boy. He was treated with neoadjuvant chemotherapy, followed by ray amputation. To restore maximum function, the index ray was transferred to the base of the third metacarpal bone and fixed with a plate. The function of his right hand after the operation was excellent and the cosmetic appearance acceptable. There was no evidence of local recurrence or metastasis after 20 months follow up. PMID:23822186

Fujii, Hiromasa; Honoki, Kanya; Kobata, Yasunori; Yajima, Hiroshi; Kido, Akira; Takakura, Yoshinori

2014-12-01

31

Proton Radiotherapy for Pediatric Ewing's Sarcoma: Initial Clinical Outcomes  

SciTech Connect

Purpose: Proton radiotherapy (PT) has been prescribed similarly to photon radiotherapy to achieve comparable disease control rates at comparable doses. The chief advantage of protons in this setting is to reduce acute and late toxicities by decreasing the amount of normal tissue irradiated. We report the preliminary clinical outcomes including late effects on our pediatric Ewing's sarcoma patients treated with PT at the Francis H. Burr Proton Therapy Center at Massachusetts General Hospital (Boston, MA). Methods and Materials: This was a retrospective review of the medical records of 30 children with Ewing's sarcoma who were treated with PT between April 2003 and April 2009. Results: A total of 14 male and 16 female patients with tumors in several anatomic sites were treated with PT at a median age of 10 years. The median dose was 54 Gy (relative biological effectiveness) with a median follow-up of 38.4 months. The 3-year actuarial rates of event-free survival, local control, and overall survival were 60%, 86%, and 89%, respectively. PT was acutely well tolerated, with mostly mild-to-moderate skin reactions. At the time of writing, the only serious late effects have been four hematologic malignancies, which are known risks of topoisomerase and anthracyline exposure. Conclusions: Proton radiotherapy was well tolerated, with few adverse events. Longer follow-up is needed to more fully assess tumor control and late effects, but the preliminary results are encouraging.

Rombi, Barbara [ATreP (Provincial Agency for Proton Therapy), Trento (Italy); DeLaney, Thomas F.; MacDonald, Shannon M. [Department of Radiation Oncology, Massachusetts General Hospital-Harvard Medical School, Boston, MA (United States); Huang, Mary S.; Ebb, David H. [Department of Pediatric Hematology and Oncology, Massachusetts General Hospital-Harvard Medical School, Boston, MA (United States); Liebsch, Norbert J. [Department of Radiation Oncology, Massachusetts General Hospital-Harvard Medical School, Boston, MA (United States); Raskin, Kevin A. [Department of Orthopaedic Surgery, Massachusetts General Hospital-Harvard Medical School, Boston, MA (United States); Yeap, Beow Y. [Department of Medicine, Massachusetts General Hospital-Harvard Medical School, Boston, MA (United States); Marcus, Karen J. [Division of Radiation Oncology, Children's Hospital Boston, MA (United States); Tarbell, Nancy J. [Department of Radiation Oncology, Massachusetts General Hospital-Harvard Medical School, Boston, MA (United States); Yock, Torunn I., E-mail: tyock@partners.org [Department of Radiation Oncology, Massachusetts General Hospital-Harvard Medical School, Boston, MA (United States)

2012-03-01

32

Microarray-based DNA methylation study of Ewing's sarcoma of the bone  

PubMed Central

Alterations in DNA methylation patterns are a hallmark of malignancy. However, the majority of epigenetic studies of Ewing’s sarcoma have focused on the analysis of only a few candidate genes. Comprehensive studies are thus lacking and are required. The aim of the present study was to identify novel methylation markers in Ewing’s sarcoma using microarray analysis. The current study reports the microarray-based DNA methylation study of 1,505 CpG sites of 807 cancer-related genes from 69 Ewing’s sarcoma samples. The Illumina GoldenGate Methylation Cancer Panel I microarray was used, and with the appropriate controls (n=14), a total of 92 hypermethylated genes were identified in the Ewing’s sarcoma samples. The majority of the hypermethylated genes were associated with cell adhesion, cell regulation, development and signal transduction. The overall methylation mean values were compared between patients who survived and those that did not. The overall methylation mean was significantly higher in the patients who did not survive (0.25±0.03) than in those who did (0.22±0.05) (P=0.0322). However, the overall methylation mean was not found to significantly correlate with age, gender or tumor location. GDF10, OSM, APC and HOXA11 were the most significant differentially-methylated genes, however, their methylation levels were not found to significantly correlate with the survival rate. The DNA methylation profile of Ewing’s sarcoma was characterized and 92 genes that were significantly hypermethylated were detected. A trend towards a more aggressive behavior was identified in the methylated group. The results of this study indicated that methylation may be significant in the development of Ewing’s sarcoma.

PARK, HYE-RIM; JUNG, WOON-WON; KIM, HYUN-SOOK; PARK, YONG-KOO

2014-01-01

33

Combining PARP-1 inhibition and radiation in Ewing sarcoma results in lethal DNA damage.  

PubMed

Ewing sarcomas (ES) harbor a chromosomal translocation that fuses the EWS gene to an ETS transcription factor, most commonly Friend leukemia integration 1 (FLI1). The EWS-FLI1 fusion protein acts in a positive feedback loop to maintain the expression of PARP-1, which is involved in repair of DNA damage. Here, we examine the effects of PARP-1 inhibition and radiation therapy on Ewing sarcomas. In proliferation assays, the Ewing sarcoma cell lines RD-ES and SK-N-MC were much more sensitive than non-Ewing sarcoma cell lines to the PARP-1 inhibitor olaparib (Ola; IC50 0.5-1 ?mol/L vs. >5 ?mol/L) and to radiation (IC50 2-4 Gy vs. >6 Gy). PARP-1 inhibition with short hairpin RNA (shRNA) or Ola sensitized Ewing sarcoma cells, but not non-Ewing sarcoma cells, to radiation therapy in both proliferation and colony formation assays. Using the Comet assay, radiation of Ewing sarcoma cells with Ola, compared to without Ola, resulted in more DNA damage at 1 hour (mean tail moment 36-54 vs. 26-28) and sustained DNA damage at 24 hours (24-29 vs. 6-8). This DNA damage led to a 2.9- to 4.0-fold increase in apoptosis and a 1.6- to 2.4-fold increase in cell death. The effect of PARP-1 inhibition and radiation therapy on Ewing sarcoma cells was lost when EWS-FLI1 was silenced by shRNA. A small dose of radiation therapy (4 Gy), when combined with PARP-1 inhibition, stopped the growth of SK-N-MC flank tumors xenografts. In conclusion, PARP-1 inhibition in Ewing sarcomas amplifies the level and duration of DNA damage caused by radiation therapy, leading to synergistic increases in apoptosis and cell death in a EWS-FLI1-dependent manner. PMID:23966622

Lee, Hae-June; Yoon, Changhwan; Schmidt, Benjamin; Park, Do Joong; Zhang, Alexia Y; Erkizan, Hayriye V; Toretsky, Jeffrey A; Kirsch, David G; Yoon, Sam S

2013-11-01

34

A Novel Role for Keratin 17 in Coordinating Oncogenic Transformation and Cellular Adhesion in Ewing Sarcoma  

PubMed Central

Oncogenic transformation in Ewing sarcoma is caused by EWS/FLI, an aberrant transcription factor fusion oncogene. Glioma-associated oncogene homolog 1 (GLI1) is a critical target gene activated by EWS/FLI, but the mechanism by which GLI1 contributes to the transformed phenotype of Ewing sarcoma was unknown. In this work, we identify keratin 17 (KRT17) as a direct downstream target gene upregulated by GLI1. We demonstrate that KRT17 regulates cellular adhesion by activating AKT/PKB (protein kinase B) signaling. In addition, KRT17 is necessary for oncogenic transformation in Ewing sarcoma and accounts for much of the GLI1-mediated transformation function but via a mechanism independent of AKT signaling. Taken together, our data reveal previously unknown molecular functions for a cytoplasmic intermediate filament protein, KRT17, in coordinating EWS/FLI- and GLI1-mediated oncogenic transformation and cellular adhesion in Ewing sarcoma. PMID:24043308

Sankar, Savita; Tanner, Jason M.; Bell, Russell; Chaturvedi, Aashi; Randall, R. Lor; Beckerle, Mary C.

2013-01-01

35

Characterization of Ewing sarcoma associated cancer/testis antigens  

PubMed Central

The prognosis of patients suffering from tumors of the Ewing family (EFT) is still poor. Immunotherapy strategies are pursued and EFT-specific antigens have to be identified as targets for cytotoxic T-lymphocytes (CTL). Due to the lack of expression of cancer/testis antigens (CTA) in normal tissues, these antigens are partially able to induce immune responses in cancer patients. Therefore, they are promising targets for immunotherapy. EFT are characterized by chromosomal rearrangements involving members of the TET (translocated in liposarcoma, Ewing sarcoma breakpoint region 1, TATA box binding protein-associated factor 15) family of RNA binding proteins and members of the E-26 (ETS) family of transcription factors. The resulting onco-fusion proteins are highly specific for EFT and downstream targets of TET-ETS represent candidate tumor specific antigens. In order to identify new EFT-associated CTA, we analyzed microarray-data sets from EFT and normal tissues from the Gene Expression Omnibus (GEO) database. The impact of TET-ETS on expression of CTA was analyzed using GEO data sets from transgenic mesenchymal stem cells. One CTA with high specificity for EFT is lipase I (LIPI, membrane-associated phospholipase A1-?). CTL specific for LIPI-derived peptides LDYTDAKFV and NLLKHGASL were able to lyse HLA-A2 positive EFT cells in vitro which confirms the possible role of LIPI and other CTA for EFT-immunotherapy. PMID:23291981

Mahlendorf, Dorothea E.; Staege, Martin Sebastian

2013-01-01

36

Characterization of Ewing sarcoma associated cancer/testis antigens.  

PubMed

The prognosis of patients suffering from tumors of the Ewing family (EFT) is still poor. Immunotherapy strategies are pursued and EFT-specific antigens have to be identified as targets for cytotoxic T-lymphocytes (CTL). Due to the lack of expression of cancer/testis antigens (CTA) in normal tissues, these antigens are partially able to induce immune responses in cancer patients. Therefore, they are promising targets for immunotherapy. EFT are characterized by chromosomal rearrangements involving members of the TET (translocated in liposarcoma, Ewing sarcoma breakpoint region 1, TATA box binding protein-associated factor 15) family of RNA binding proteins and members of the E-26 (ETS) family of transcription factors. The resulting onco-fusion proteins are highly specific for EFT and downstream targets of TET-ETS represent candidate tumor specific antigens. In order to identify new EFT-associated CTA, we analyzed microarray-data sets from EFT and normal tissues from the Gene Expression Omnibus (GEO) database. The impact of TET-ETS on expression of CTA was analyzed using GEO data sets from transgenic mesenchymal stem cells. One CTA with high specificity for EFT is lipase I (LIPI, membrane-associated phospholipase A1-?). CTL specific for LIPI-derived peptides LDYTDAKFV and NLLKHGASL were able to lyse HLA-A2 positive EFT cells in vitro which confirms the possible role of LIPI and other CTA for EFT-immunotherapy. PMID:23291981

Mahlendorf, Dorothea E; Staege, Martin Sebastian

2013-03-01

37

Disseminated Intracranial Ewing's Sarcoma in an Adult: A Rare and Difficult Diagnosis  

PubMed Central

The Ewing sarcoma family of tumors comprises a rare class of cancers of mesenchymal origin. Cases of Ewing's sarcoma in the central nervous system – specifically, intracranial Ewing's – are extremely rare. Almost all reported cases have occurred in children. However, this rare presentation can also occur in the adult population. It is important to distinguish these tumors from primitive neuroectodermal tumors at the time of diagnosis. Testing for EWSR1(22q12) gene rearrangement using fluorescence in situ hybridization is a useful tool for making the distinction between these 2 similar but distinct entities. We present here the case of a middle-aged male patient with intracranial Ewing's sarcoma, and discuss diagnostic challenges and potential new treatment approaches for this rare disease. PMID:22933997

Lou, Emil; Sumrall, Ashley L.; Cummings, Thomas J.; Korones, David N.; Weaver, Susan A.; Peters, Katherine B.

2012-01-01

38

Diagnosis of osteosarcoma in a patient previously treated for Ewing sarcoma.  

PubMed

Primary malignant bone tumors, whether Ewing sarcoma or osteosarcoma, are a rare type of tumor. The sequential occurrence of two bone sarcomas, Ewing sarcoma and high-grade osteosarcoma, in the same patient at two different locations is an exceptionally rare phenomenon. We present the case of a 13-year-old girl who presented with a high-grade osteoblastic osteosarcoma of the distal femur, 7 years after treatment for Ewing sarcoma of the left pelvis. She did not receive radiation therapy. Following the recent developing multidisciplinary therapy, long-term follow-up for monitoring latent treatment-related adverse effects may be necessary for survivors of primary malignant bone tumors. PMID:24722657

Hoshi, Manabu; Ieguchi, Makoto; Yamato, Kazumi; Tokimasa, Sadao; Nakamura, Hiroaki

2014-09-01

39

Salient features of mesenchymal stem cells--implications for Ewing sarcoma modeling  

PubMed Central

Despite a heightened appreciation of the many defining molecular aberrations in Ewing sarcoma, the cooperative genetic environment and permissive cell of origin essential for EWS/ETS-mediated oncogenesis remain elusive. Consequently, inducible animal and in vitro models of Ewing sarcoma from a native cellular context are unable to fully recapitulate malignant transformation. Despite these shortcomings, human, and murine mesenchymal stem cells (MSCs) are the closest working in vitro systems available. MSCs are tolerant of ectopic EWS/FLI expression, which is accompanied by a molecular signature most similar to Ewing sarcoma. Whether MSCs are the elusive cell of origin or simply a tolerant platform of the EWS/FLI transcriptome, these cells have become an excellent molecular tool to investigate and manipulate oncogenesis in Ewing sarcoma. Our understanding of the biological complexity and heterogeneity of human MSCs (hMSCs) has increased substantially over time and as such, appreciation and utilization of these salient complexities may greatly enhance the efficient use of these cells as surrogate models for Ewing sarcoma tumorigenesis. PMID:23443465

Monument, Michael J.; Bernthal, Nicholas M.; Randall, R. Lor

2013-01-01

40

Primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old woman: a case report  

Microsoft Academic Search

INTRODUCTION: Primary Ewing's sarcoma or primitive neuroectodermal tumor of the genital tract of women is uncommon. Rarer still is its occurrence in the vagina, with only five cases described so far. Out of these, only one case was confirmed using molecular analysis. CASE PRESENTATION: We present an extremely rare case of Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old

Bharat Rekhi; Sajid Qureshi; Ranjan Basak; Sangeeta B Desai; Seema Medhi; Purna Kurkure; Santosh Menon; Amita Maheshwari; Nirmala A Jambhekar

2010-01-01

41

The ganglioside antigen GD2 is surface-expressed in Ewing sarcoma and allows for MHC-independent immune targeting  

PubMed Central

Background: Novel treatment strategies are needed to cure disseminated Ewing sarcoma. Primitive neuroectodermal features and a mesenchymal stem cell origin are both compatible with aberrant expression of the ganglioside antigen GD2 and led us to explore GD2 immune targeting in this cancer. Methods: We investigated GD2 expression in Ewing sarcoma by immunofluorescence staining. We then assessed the antitumour activity of T cells expressing a chimeric antigen receptor specific for GD2 against Ewing sarcoma in vitro and in vivo. Results: Surface GD2 was detected in 10 out of 10 Ewing sarcoma cell lines and 3 out of 3 primary cell cultures. Moreover, diagnostic biopsies from 12 of 14 patients had uniform GD2 expression. T cells specifically modified to express the GD2-specific chimeric receptor 14. G2a-28? efficiently interacted with Ewing sarcoma cells, resulting in antigen-specific secretion of cytokines. Moreover, chimeric receptor gene-modified T cells from healthy donors and from a patient exerted potent, GD2-specific cytolytic responses to allogeneic and autologous Ewing sarcoma, including tumour cells grown as multicellular, anchorage-independent spheres. GD2-specific T cells further had activity against Ewing sarcoma xenografts. Conclusion: GD2 surface expression is a characteristic of Ewing sarcomas and provides a suitable target antigen for immunotherapeutic strategies to eradicate micrometastatic cells and prevent relapse in high-risk disease. PMID:22374462

Kailayangiri, S; Altvater, B; Meltzer, J; Pscherer, S; Luecke, A; Dierkes, C; Titze, U; Leuchte, K; Landmeier, S; Hotfilder, M; Dirksen, U; Hardes, J; Gosheger, G; Juergens, H; Rossig, C

2012-01-01

42

Huntsman Cancer Institute researchers discover possible new treatment for Ewing sarcoma  

Cancer.gov

Discovery of a new drug with high potential to treat Ewing sarcoma, an often deadly cancer of children and young adults, and the previously unknown mechanism behind it, come hand-in-hand in a new study by researchers from Huntsman Cancer Institute (HCI) at the University of Utah. In the lab, researchers found that an enzyme, called lysine specific demethylase (LSD-1), interacts with EWS/FLI to turn off gene expression in Ewing sarcoma. By turning off specific genes, the EWS/FLI-LSD1 complex causes Ewing sarcoma development. The team is now working to further test LSD inhibitors in animal models as they work toward approval of a first-in-man clinical trial.

43

The Ca, Cl, Mg, Na, and P mass fractions in human bone affected by Ewing's sarcoma.  

PubMed

The Ca, Cl, Mg, Na, and P contents and Ca/P, Ca/Mg, Ca/Na, Cl/Ca, and Cl/Na ratios in samples of intact bone, inflamed bone, and Ewing's sarcoma tissue were investigated by neutron activation analysis with high-resolution spectrometry of short-lived radionuclides. In Ewing's sarcoma tissue, the mass fractions of Cl and Na are higher and the mass fractions of Ca and Mg are lower than those of both normal and inflamed bone tissues. It was shown that the levels of Ca and Cl mass fractions and also levels of the Ca/Cl and Cl/Na ratios can be used as an additional test for differential diagnosis between inflamed (or normal) bone and Ewing's sarcoma. PMID:24723217

Zaichick, Vladimir; Zaichick, Sofia

2014-06-01

44

ZEB2 Represses the Epithelial Phenotype and Facilitates Metastasis in Ewing Sarcoma  

PubMed Central

The vast majority of cancer-related deaths are attributable to metastasis. Effective treatment of metastatic disease will be improved by a better understanding of the molecular mechanisms contributing to this phenomenon. Much of the work in this field has focused on metastasis of carcinomas, tumors of epithelial origin, while metastasis of sarcomas, tumors of mesenchymal origin, remains poorly understood. Experimental evidence from studies in carcinomas, coupled with clinical observations, highlights the importance of both epithelial and mesenchymal characteristics in these cancer cells that make them competent for metastasis. We set out to test if similar cellular plasticity contributes to sarcoma metastasis. We found that the transcription factor, ZEB2, repressed epithelial gene expression in Ewing sarcoma cells, and this, in turn, repressed the epithelial phenotype. When ZEB2 was experimentally reduced in these cells, epithelial characteristics including decreased migratory ability and cytoskeleton rearrangements were observed. Furthermore, ZEB2 reduction in Ewing sarcoma cells resulted in a decreased metastatic potential using a mouse metastasis model. Our data show that Ewing sarcoma cells may have more epithelial plasticity than previously appreciated. This coupled with previous data demonstrating Ewing sarcoma cells also have mesenchymal features primes these cells to successfully metastasize. This is clinically relevant for 2 important reasons. First, this may offer a therapeutic opportunity to induce characteristics of one cell type or the other depending on the stage of the disease. Second, and more broadly, this raises questions about the cell of origin in Ewing sarcoma and may inform future animal models of the disease. PMID:24386509

Wiles, Elizabeth T.; Bell, Russell; Thomas, Dafydd; Beckerle, Mary

2013-01-01

45

Ewing sarcoma dissemination and response to T-cell therapy in mice assessed by whole-body magnetic resonance imaging  

PubMed Central

Background: Novel treatment strategies in Ewing sarcoma include targeted cellular therapies. Preclinical in vivo models are needed that reflect their activity against systemic (micro)metastatic disease. Methods: Whole-body magnetic resonance imaging (WB-MRI) was used to monitor the engraftment and dissemination of human Ewing sarcoma xenografts in mice. In this model, we evaluated the therapeutic efficacy of T cells redirected against the Ewing sarcoma-associated antigen GD2 by chimeric receptor engineering. Results: Of 18 mice receiving intravenous injections of VH-64 Ewing sarcoma cells, all developed disseminated tumour growth detectable by WB-MRI. All mice had lung tumours, and the majority had additional manifestations in the bone, soft tissues, and/or kidney. Sequential scans revealed in vivo growth of tumours. Diffusion-weighted whole-body imaging with background signal suppression effectively visualised Ewing sarcoma growth in extrapulmonary sites. Animals receiving GD2-targeted T-cell therapy had lower numbers of pulmonary tumours than controls, and the median volume of soft tissue tumours at first detection was lower, with a tumour growth delay over time. Conclusion: Magnetic resonance imaging reliably visualises disseminated Ewing sarcoma growth in mice. GD2-retargeted T cells can noticeably delay tumour growth and reduce pulmonary Ewing sarcoma manifestations in this aggressive disease model. PMID:23839490

Liebsch, L; Kailayangiri, S; Beck, L; Altvater, B; Koch, R; Dierkes, C; Hotfilder, M; Nagelmann, N; Faber, C; Kooijman, H; Ring, J; Vieth, V; Rossig, C

2013-01-01

46

Ewing sarcoma inhibition by disruption of EWSR1-FLI1 transcriptional activity and reactivation of p53.  

PubMed

Translocations involving ETS-transcription factors, most commonly leading to the EWSR1-FLI1 fusion protein, are the hallmark of Ewing sarcoma. Despite knowledge of this driving molecular event, an effective therapeutic strategy is lacking. To test potential treatment regimes, we established a novel Ewing sarcoma zebrafish engraftment model allowing time-effective, dynamic quantification of Ewing sarcoma progression and tumour burden in vivo, applicable for screening of single and combined compounds. In Ewing sarcoma the tumour-suppressor gene TP53 is commonly found to be wild-type, thus providing an attractive target for treatment. Here, we study TP53 wild-type (EW7, CADO-ES1 and TC32) and TP53-deleted (SK-N-MC) Ewing sarcoma cell lines to investigate the potentiating effect of p53 reactivation by Nutlin-3 on treatment with YK-4-279 to block transcriptional activity of EWSR1-FLI1 protein. Blocking EWSR1-FLI1 transcriptional activity reduced Ewing sarcoma tumour cell burden irrespective of TP53 status. We show that simultaneous YK-4-279 treatment with Nutlin-3 to stabilize p53 resulted in an additive inhibition of TP53 wild-type Ewing sarcoma cell burden, whilst not affecting TP53-deleted Ewing sarcoma cells. Improved inhibition of proliferation and migration by combinatorial treatment was confirmed in vivo by zebrafish engraftments. Mechanistically, both compounds together additively induced apoptosis of tumour cells in vivo by engaging distinct pathways. We propose reactivation of the p53 pathway in combination with complementary targeted therapy by EWSR1-FLI1 transcriptional activity disruption as a valuable strategy against p53 wild-type Ewing sarcoma. PMID:24974828

van der Ent, Wietske; Jochemsen, Aart G; Teunisse, Amina F A S; Krens, S F Gabriel; Szuhai, Karoly; Spaink, Herman P; Hogendoorn, Pancras C W; Snaar-Jagalska, B Ewa

2014-08-01

47

Low Levels of Common Enzyme Key to Resistance in Ewing's Sarcoma  

Cancer.gov

A study from scientists at the University of Freiburg, Germany, and their collaborators at the NCI, has pinpointed a potential mechanism for resistance of Ewing's sarcoma, a type of bone cancer, to a protein that may be useful in fighting cancer -- and a possible method for overcoming this resistance.

48

Targeting the Insulin-Like Growth Factor 1 Receptor in Ewing's Sarcoma: Reality and Expectations  

PubMed Central

Ewing's sarcoma family of tumours comprises a group of very aggressive diseases that are potentially curable with multimodality treatment. Despite the undoubted success of current treatment, approximately 30% of patients will relapse and ultimately die of disease. The insulin-like growth factor 1 receptor (IGF-1R) has been implicated in the genesis, growth, proliferation, and the development of metastatic disease in Ewing's sarcoma. In addition, IGF1-R has been validated, both in vitro and in vivo, as a potential therapeutic target in Ewing's sarcoma. Phase I studies of IGF-1R monoclonal antibodies reported several radiological and clinical responses in Ewing's sarcoma patients, and initial reports of several Phase II studies suggest that about a fourth of the patients would benefit from IGF-1R monoclonal antibodies as single therapy, with approximately 10% of patients achieving objective responses. Furthermore, these therapies are well tolerated, and thus far severe toxicity has been rare. Other studies assessing IGF-1R monoclonal antibodies in combination with traditional cytotoxics or other targeted therapies are expected. Despite, the initial promising results, not all patients benefit from IGF-1R inhibition, and consequently, there is an urgent need for the identification of predictive markers of response. PMID:21647361

Olmos, David; Martins, Ana Sofia; Jones, Robin L.; Alam, Salma; Scurr, Michelle; Judson, Ian R.

2011-01-01

49

N-(4-Hydroxyphenyl) Retinamide Potentiated Anti-tumor Efficacy of Genistein in Human Ewing's Sarcoma Xenografts.  

PubMed

BACKGROUND: Ewing's sarcoma is a pediatric tumor that mainly occurs in soft tissues and bones. New therapeutic strategies are urgently needed for treatment of Ewing's sarcoma. We examined for the first time the efficacy of N-(4-hydroxyphenyl) retinamide (4-HPR) and genistein (GST) alone and also in combination for controlling growth of human Ewing's sarcoma SK-N-MC and RD-ES xenografts. METHODS: Efficacy of combination therapy was evaluated using histopathological parameters. Molecular mechanisms of combination therapy were detected using Western blotting and immunofluorescence microscopy. RESULTS: Histopathological examination of tumor sections showed that control group maintained characteristic growth of tumors, 4-HPR alone caused differentiation of tumor cells, GST alone induced apoptosis to some extent, and combination of 4-HPR and GST significantly induced apoptosis in both Ewing's sarcoma xenografts. Time-dependent reductions in body weight, tumor volume, and tumor weight were also found. Combination therapy increased Bax:Bcl-2 ratio to trigger mitochondrial release of Smac/Diablo into the cytosol to down regulate the baculovirus inhibitor-of-apoptosis repeat containing (BIRC) proteins such as BIRC-2 and BIRC-3 and thereby promote apoptosis. Activation of caspase-3 and mitochondrial release of apoptosis-inducing factor (AIF) occurred in course of apoptosis. Down regulation of the survival factor NF-?B and the angiogenic factors VEGF and FGF2 and increase in caspase-3 activity controlled tumor growth. In situ immunofluorescent labelings showed overexpression of calpain, caspase-12, and caspase-3, and AIF in xenografts, indicating induction of cysteine proteases and AIF for apoptosis. CONCLUSIONS: Results revealed that combination of 4-HPR and GST could be highly effective treatment for inhibiting Ewing's sarcomas in vivo. PMID:21822457

Karmakar, Surajit; Choudhury, Subhasree Roy; Banik, Naren L; Ray, Swapan K

2011-04-01

50

Long noncoding RNA EWSAT1-mediated gene repression facilitates Ewing sarcoma oncogenesis.  

PubMed

Chromosomal translocation that results in fusion of the genes encoding RNA-binding protein EWS and transcription factor FLI1 (EWS-FLI1) is pathognomonic for Ewing sarcoma. EWS-FLI1 alters gene expression through mechanisms that are not completely understood. We performed RNA sequencing (RNAseq) analysis on primary pediatric human mesenchymal progenitor cells (pMPCs) expressing EWS-FLI1 in order to identify gene targets of this oncoprotein. We determined that long noncoding RNA-277 (Ewing sarcoma-associated transcript 1 [EWSAT1]) is upregulated by EWS-FLI1 in pMPCs. Inhibition of EWSAT1 expression diminished the ability of Ewing sarcoma cell lines to proliferate and form colonies in soft agar, whereas EWSAT1 inhibition had no effect on other cell types tested. Expression of EWS-FLI1 and EWSAT1 repressed gene expression, and a substantial fraction of targets that were repressed by EWS-FLI1 were also repressed by EWSAT1. Analysis of RNAseq data from primary human Ewing sarcoma further supported a role for EWSAT1 in mediating gene repression. We identified heterogeneous nuclear ribonucleoprotein (HNRNPK) as an RNA-binding protein that interacts with EWSAT1 and found a marked overlap in HNRNPK-repressed genes and those repressed by EWS-FLI1 and EWSAT1, suggesting that HNRNPK participates in EWSAT1-mediated gene repression. Together, our data reveal that EWSAT1 is a downstream target of EWS-FLI1 that facilitates the development of Ewing sarcoma via the repression of target genes. PMID:25401475

Marques Howarth, Michelle; Simpson, David; Ngok, Siu P; Nieves, Bethsaida; Chen, Ron; Siprashvili, Zurab; Vaka, Dedeepya; Breese, Marcus R; Crompton, Brian D; Alexe, Gabriela; Hawkins, Doug S; Jacobson, Damon; Brunner, Alayne L; West, Robert; Mora, Jaume; Stegmaier, Kimberly; Khavari, Paul; Sweet-Cordero, E Alejandro

2014-12-01

51

Ewing's Sarcoma of the Sacroiliac Joint Presenting as Tubercular Sacroiliitis: A Diagnostic Dilemma.  

PubMed

We report a case of Ewing's sarcoma of the sacroiliac joint in a 21-year-old male mimicking tubercular sacroiliitis, a rare entity not reported in literature. He presented with pain in the lower back radiating to the right lower limb along with constitutional symptoms of 3 months duration. On examination, the right sacroiliac joint was tender. The laboratory investigations showed anaemia, leukocytosis and raised erythrocyte sedimentation rate. On X-ray, features of right sacroiliitis were seen. This was further investigated with magnetic resonance imaging (MRI), which showed features consistent with tubercular sacroiliitis. Patient was then started on antitubercular treatment, but the improvement was not consistent. So, a contrast MRI was done, which indicated features of primary sarcoma. It was then further confirmed by a computed tomography-guided biopsy, which showed features consistent with Ewing's sarcoma of the sacroiliac joint. PMID:24596610

Garg, Bhavuk; Jalan, Divesh; Kotwal, Prakash P

2014-02-01

52

Ewing's Sarcoma of the Sacroiliac Joint Presenting as Tubercular Sacroiliitis: A Diagnostic Dilemma  

PubMed Central

We report a case of Ewing's sarcoma of the sacroiliac joint in a 21-year-old male mimicking tubercular sacroiliitis, a rare entity not reported in literature. He presented with pain in the lower back radiating to the right lower limb along with constitutional symptoms of 3 months duration. On examination, the right sacroiliac joint was tender. The laboratory investigations showed anaemia, leukocytosis and raised erythrocyte sedimentation rate. On X-ray, features of right sacroiliitis were seen. This was further investigated with magnetic resonance imaging (MRI), which showed features consistent with tubercular sacroiliitis. Patient was then started on antitubercular treatment, but the improvement was not consistent. So, a contrast MRI was done, which indicated features of primary sarcoma. It was then further confirmed by a computed tomography-guided biopsy, which showed features consistent with Ewing's sarcoma of the sacroiliac joint. PMID:24596610

Garg, Bhavuk; Kotwal, Prakash P

2014-01-01

53

Differentially Expressed miRNAs in Ewing Sarcoma Compared to Mesenchymal Stem Cells: Low miR-31 Expression with Effects on Proliferation and Invasion  

PubMed Central

Ewing sarcoma, the second most common bone tumor in children and young adults, is an aggressive malignancy with a strong potential to metastasize. Ewing sarcoma is characterised by translocations encoding fusion transcription factors with an EWSR1 transactivation domain fused to an ETS family DNA binding domain. microRNAs are post-transcriptional regulators of gene expression and aberrantly expressed microRNAs have been identified as tumor suppressors or oncogenes in most cancer types. To identify potential oncogenic and tumor suppressor microRNAs in Ewing sarcoma, we determined and compared the expression of 377 microRNAs in 40 Ewing sarcoma biopsies, 6 Ewing sarcoma cell lines and mesenchymal stem cells, the putative cellular origin of Ewing sarcoma, from 6 healthy donors. Of the 35 differentially expressed microRNAs identified (fold change >4 and q<0.05), 19 were higher and 16 lower expressed in Ewing sarcoma. In comparisons between Ewing sarcoma samples with EWS-FLI or EWS-ERG translocations, with differing dissemination characteristics and of primary samples and metastases no significantly differential expressed microRNAs were detected using various stringency criteria. For miR-31, the microRNA with lowest expression in comparison to mesenchymal stem cells, functional analyses were performed to determine its potential as a tumor suppressor in Ewing sarcoma. Two of four miR-31 transfected Ewing sarcoma cell lines showed a significantly reduced proliferation (19% and 33% reduction) due to increased apoptosis in one and increased length of G1-phase in the other cell line. All three tested miR-31 transfected Ewing sarcoma cell lines showed significantly reduced invasiveness (56% to 71% reduction). In summary, we identified 35 microRNAs differentially expressed in Ewing sarcoma and demonstrate that miR-31 affects proliferation and invasion of Ewing sarcoma cell lines in ex vivo assays. PMID:24667836

Karnuth, Bianca; Dedy, Nicolas; Spieker, Tilmann; Lawlor, Elizabeth R.; Gattenlohner, Stefan; Ranft, Andreas; Dirksen, Uta; Jurgens, Heribert; Brauninger, Andreas

2014-01-01

54

Incidence and management of secondary malignancies in patients with retinoblastoma and Ewing's sarcoma  

SciTech Connect

Childhood cancer survivors at highest risk of developing a secondary malignancy are those with hereditary retinoblastoma. The majority of such secondary cancers will be sarcomas, most commonly of bone. One-third of these occur outside a typical radiation field, commonly in an extremity. Bone sarcoma is also the most commonly reported secondary cancer to develop among survivors of Ewing's sarcoma. In this group, radiation doses greater than 60 Gy as well as alkylating agent chemotherapy have been identified as contributors to the increased risk. The prognosis for patients with a secondary sarcoma has been poor, with few cures reported to date. However, an aggressive, combined modality approach, including radical resection, postoperative radiation, and adjuvant chemotherapy, may improve the survival rate.

Smith, L.M.; Donaldson, S.S. (Department of Radiation Oncology, Stanford University Medical Center, CA (United States))

1991-05-01

55

Analysis of the p16INK4, p14ARF, p15, TP53, and MDM2 Genes and Their Prognostic Implications in Osteosarcoma and Ewing Sarcoma  

Microsoft Academic Search

We examined alterations of the p16INK4, p14ARF, p15, TP53, and MDM2 genes in 30 osteosarcomas and 24 Ewing sarcomas. Among 21 osteosarcomas and 24 Ewing sarcomas, p16INK4, p14ARF, and p15 abnormalities were found in 4 (19%), 2 (9%), and 3 (14%) osteosarcomas, respectively, and in 4 (17%), 3 (13%), and 4 (17%) Ewing sarcomas, respectively. The alterations of p16INK4, p14ARF,

Takashi Tsuchiya; Ki-ichi Sekine; Shin-ichi Hinohara; Takeshi Namiki; Tsutomu Nobori; Yasuhiko Kaneko

2000-01-01

56

A Novel CIC-FOXO4 Gene Fusion in Undifferentiated Small Round Cell Sarcoma: A Genetically Distinct Variant of Ewing-like Sarcoma.  

PubMed

Differential diagnosis of small round cell sarcomas (SRCSs) grouped under the Ewing sarcoma family of tumors (ESFT) can be a challenging situation for pathologists. Recent studies have revealed that some groups of Ewing-like sarcoma show typical ESFT morphology but lack any EWSR1-ETS gene fusions. Here we identified a novel gene fusion, CIC-FOXO4, in a case of Ewing-like sarcoma with a t(X;19)(q13;q13.3) translocation. The patient was a 63-year-old man who had an asymptomatic, 30-mm, well-demarcated, intramuscular mass in his right posterior neck, and imaging findings suggested a diagnosis of high-grade sarcoma. He was treated with complete resection and subsequent radiotherapy and chemotherapy. He was alive without local recurrence or distant metastasis 6 months after the operation. Histologic examination revealed SRCS with abundant desmoplastic fibrous stroma suggesting a desmoplastic small round cell tumor. Immunohistochemical analysis showed weak to moderate and partial staining for MIC2 (CD99) and WT1, respectively. High-throughput transcriptome sequencing revealed a gene fusion, and the genomic rearrangement between the CIC and FOXO4 genes was identified by fluorescence in situ hybridization. Aside from the desmoplastic stroma, the CIC-FOXO4 fusion sarcoma showed morphologic and immunohistochemical similarity to ESFT and Ewing-like sarcomas, including the recently described CIC-DUX4 fusion sarcoma. Although clinicopathologic analysis with additional cases is necessary, we conclude that CIC-FOXO4 fusion sarcoma is a new type of Ewing-like sarcoma that has a specific genetic signature. These findings have important implications for the differential diagnosis of SRCS. PMID:25007147

Sugita, Shintaro; Arai, Yasuhito; Tonooka, Akiko; Hama, Natsuko; Totoki, Yasushi; Fujii, Tomoki; Aoyama, Tomoyuki; Asanuma, Hiroko; Tsukahara, Tomohide; Kaya, Mitsunori; Shibata, Tatsuhiro; Hasegawa, Tadashi

2014-11-01

57

High ALDH Activity Identifies Chemotherapy-Resistant Ewing's Sarcoma Stem Cells That Retain Sensitivity to EWS-FLI1 Inhibition  

PubMed Central

Background Cancer stem cells are a chemotherapy-resistant population capable of self-renewal and of regenerating the bulk tumor, thereby causing relapse and patient death. Ewing's sarcoma, the second most common form of bone tumor in adolescents and young adults, follows a clinical pattern consistent with the Cancer Stem Cell model – remission is easily achieved, even for patients with metastatic disease, but relapse remains frequent and is usually fatal. Methodology/Principal Findings We have isolated a subpopulation of Ewing's sarcoma cells, from both human cell lines and human xenografts grown in immune deficient mice, which express high aldehyde dehydrogenase (ALDHhigh) activity and are enriched for clonogenicity, sphere-formation, and tumor initiation. The ALDHhigh cells are resistant to chemotherapy in vitro, but this can be overcome by the ATP binding cassette transport protein inhibitor, verapamil. Importantly, these cells are not resistant to YK-4-279, a small molecule inhibitor of EWS-FLI1 that is selectively toxic to Ewing's sarcoma cells both in vitro and in vivo. Conclusions/Significance Ewing's sarcoma contains an ALDHhigh stem-like population of chemotherapy-resistant cells that retain sensitivity to EWS-FLI1 inhibition. Inhibiting the EWS-FLI1 oncoprotein may prove to be an effective means of improving patient outcomes by targeting Ewing's sarcoma stem cells that survive standard chemotherapy. PMID:21085683

Gul, Naheed; Katuri, Varalakshmi; O'Neill, Alison; Kong, Yali; Brown, Milton L.; Toretsky, Jeffrey A.; Loeb, David M.

2010-01-01

58

Anchorage-independent growth of Ewing sarcoma cells under serum-free conditions is not associated with stem-cell like phenotype and function.  

PubMed

Novel treatment strategies for Ewing sarcoma aim to eliminate residual tumor cells that have maintained the capacity to reinitiate tumor growth after intensive conventional therapy. Preclinical models that more closely mimic in vivo tumor growth than standard monolayer cultures are needed. Sphere formation under anchorage-independent, serum-free conditions has been proposed to enrich for cells with tumor-initiating, stem cell-like properties in various solid cancers. In the present study, we assessed the phenotype and functional stem cell characteristics of Ewing sarcoma spheres. Spheres were generated under serum-free culture conditions from four Ewing sarcoma cell lines and four relapse tumor biopsies. Standard monolayer cultures were established as controls. Median levels of surface expression of the Ewing sarcoma marker CD99 as well as the supposed stem cell marker CD133 and the neural crest marker CD57 were comparable between spheres and monolayers. Ewing sarcoma spheres from individual tumors failed to continuously self-renew by secondary sphere formation. They contained variable proportions of side populations (SPs). Sphere culture did not enhance the in vivo tumorigenicity of Ewing sarcoma cells in a murine xenograft model. We conclude that sphere formation under serum-free conditions is not a reliable tool to enrich for cells with stem cell characteristics in Ewing sarcoma. By mimicking the anchorage-independent, multicellular growth of Ewing sarcoma micrometastases, in vitro sphere growth may still add value as a preclinical tool to evaluate the efficacy of novel therapeutics. PMID:24927333

Leuchte, Katharina; Altvater, Bianca; Hoffschlag, Simeon; Potratz, Jenny; Meltzer, Jutta; Clemens, Dagmar; Luecke, Andrea; Hardes, Jendrik; Dirksen, Uta; Juergens, Heribert; Kailayangiri, Sareetha; Rossig, Claudia

2014-08-01

59

EWS-FLI1 inhibits TNF{alpha}-induced NF{kappa}B-dependent transcription in Ewing sarcoma cells  

SciTech Connect

Research highlights: {yields} EWS-FLI1 interferes with TNF-induced activation of NF{kappa}B in Ewing sarcoma cells. {yields} EWS-FLI1 knockdown in Ewing sarcoma cells increases TNF-induced NF{kappa}B binding to DNA. {yields} EWS-FLI1 reduces TNF-stimulated NF{kappa}B-dependent transcriptional activation. {yields} Constitutive NF{kappa}B activity is not affected by EWS-FLI1. {yields} EWS-FLI1 physically interacts with NF{kappa}B p65 in vivo. -- Abstract: Ewing sarcoma is primarily caused by a t(11;22) chromosomal translocation encoding the EWS-FLI1 fusion protein. To exert its oncogenic function, EWS-FLI1 acts as an aberrant transcription factor, broadly altering the gene expression profile of tumor cells. Nuclear factor-kappaB (NF{kappa}B) is a tightly regulated transcription factor controlling cell survival, proliferation and differentiation, as well as tumorigenesis. NF{kappa}B activity is very low in unstimulated Ewing sarcoma cells, but can be induced in response to tumor necrosis factor (TNF). We wondered whether NF{kappa}B activity could be modulated by EWS-FLI1 in Ewing sarcoma. Using a knockdown approach in Ewing sarcoma cells, we demonstrated that EWS-FLI1 has no influence on NF{kappa}B basal activity, but impairs TNF-induced NF{kappa}B-driven transcription, at least in part through inhibition of NF{kappa}B binding to DNA. We detected an in vivo physical interaction between the fusion protein and NF{kappa}B p65, which could mediate these effects. Our findings suggest that, besides directly controlling the activity of its primary target promoters, EWS-FLI1 can also indirectly influence gene expression in tumor cells by modulating the activity of key transcription factors such as NF{kappa}B.

Lagirand-Cantaloube, Julie, E-mail: julie.cantaloube@crbm.cnrs.fr [UMR8113 CNRS, LBPA, Ecole Normale Superieure, Cachan (France)] [UMR8113 CNRS, LBPA, Ecole Normale Superieure, Cachan (France); Laud, Karine, E-mail: karine.laud@curie.fr [U830 INSERM, Institut Curie, Paris (France) [U830 INSERM, Institut Curie, Paris (France); Institut Curie, Genetique et biologie des cancers, Paris (France); Lilienbaum, Alain, E-mail: alain.lilienbaum@univ-paris-diderot.fr [EA300 Universite Paris 7, Stress et pathologies du cytosquelette, Paris (France)] [EA300 Universite Paris 7, Stress et pathologies du cytosquelette, Paris (France); Tirode, Franck, E-mail: franck.tirode@curie.fr [U830 INSERM, Institut Curie, Paris (France) [U830 INSERM, Institut Curie, Paris (France); Institut Curie, Genetique et biologie des cancers, Paris (France); Delattre, Olivier, E-mail: olivier.delattre@curie.fr [U830 INSERM, Institut Curie, Paris (France) [U830 INSERM, Institut Curie, Paris (France); Institut Curie, Genetique et biologie des cancers, Paris (France); Auclair, Christian, E-mail: auclair@lbpa.ens-cachan.fr [UMR8113 CNRS, LBPA, Ecole Normale Superieure, Cachan (France)] [UMR8113 CNRS, LBPA, Ecole Normale Superieure, Cachan (France); Kryszke, Marie-Helene, E-mail: kryszke@lbpa.ens-cachan.fr [UMR8113 CNRS, LBPA, Ecole Normale Superieure, Cachan (France)] [UMR8113 CNRS, LBPA, Ecole Normale Superieure, Cachan (France)

2010-09-03

60

The Genomic Landscape of the Ewing Sarcoma Family of Tumors Reveals Recurrent STAG2 Mutation  

PubMed Central

The Ewing sarcoma family of tumors (EFT) is a group of highly malignant small round blue cell tumors occurring in children and young adults. We report here the largest genomic survey to date of 101 EFT (65 tumors and 36 cell lines). Using a combination of whole genome sequencing and targeted sequencing approaches, we discover that EFT has a very low mutational burden (0.15 mutations/Mb) but frequent deleterious mutations in the cohesin complex subunit STAG2 (21.5% tumors, 44.4% cell lines), homozygous deletion of CDKN2A (13.8% and 50%) and mutations of TP53 (6.2% and 71.9%). We additionally note an increased prevalence of the BRCA2 K3326X polymorphism in EFT patient samples (7.3%) compared to population data (OR 7.1, p?=?0.006). Using whole transcriptome sequencing, we find that 11% of tumors pathologically diagnosed as EFT lack a typical EWSR1 fusion oncogene and that these tumors do not have a characteristic Ewing sarcoma gene expression signature. We identify samples harboring novel fusion genes including FUS-NCATc2 and CIC-FOXO4 that may represent distinct small round blue cell tumor variants. In an independent EFT tissue microarray cohort, we show that STAG2 loss as detected by immunohistochemistry may be associated with more advanced disease (p?=?0.15) and a modest decrease in overall survival (p?=?0.10). These results significantly advance our understanding of the genomic and molecular underpinnings of Ewing sarcoma and provide a foundation towards further efforts to improve diagnosis, prognosis, and precision therapeutics testing. PMID:25010205

Brohl, Andrew S.; Solomon, David A.; Chang, Wendy; Wang, Jianjun; Song, Young; Sindiri, Sivasish; Patidar, Rajesh; Hurd, Laura; Chen, Li; Shern, Jack F.; Liao, Hongling; Wen, Xinyu; Gerard, Julia; Kim, Jung-Sik; Lopez Guerrero, Jose Antonio; Machado, Isidro; Wai, Daniel H.; Picci, Piero; Triche, Timothy; Horvai, Andrew E.; Miettinen, Markku; Wei, Jun S.; Catchpool, Daniel; Llombart-Bosch, Antonio; Waldman, Todd; Khan, Javed

2014-01-01

61

The genomic landscape of the Ewing Sarcoma family of tumors reveals recurrent STAG2 mutation.  

PubMed

The Ewing sarcoma family of tumors (EFT) is a group of highly malignant small round blue cell tumors occurring in children and young adults. We report here the largest genomic survey to date of 101 EFT (65 tumors and 36 cell lines). Using a combination of whole genome sequencing and targeted sequencing approaches, we discover that EFT has a very low mutational burden (0.15 mutations/Mb) but frequent deleterious mutations in the cohesin complex subunit STAG2 (21.5% tumors, 44.4% cell lines), homozygous deletion of CDKN2A (13.8% and 50%) and mutations of TP53 (6.2% and 71.9%). We additionally note an increased prevalence of the BRCA2 K3326X polymorphism in EFT patient samples (7.3%) compared to population data (OR 7.1, p?=?0.006). Using whole transcriptome sequencing, we find that 11% of tumors pathologically diagnosed as EFT lack a typical EWSR1 fusion oncogene and that these tumors do not have a characteristic Ewing sarcoma gene expression signature. We identify samples harboring novel fusion genes including FUS-NCATc2 and CIC-FOXO4 that may represent distinct small round blue cell tumor variants. In an independent EFT tissue microarray cohort, we show that STAG2 loss as detected by immunohistochemistry may be associated with more advanced disease (p?=?0.15) and a modest decrease in overall survival (p?=?0.10). These results significantly advance our understanding of the genomic and molecular underpinnings of Ewing sarcoma and provide a foundation towards further efforts to improve diagnosis, prognosis, and precision therapeutics testing. PMID:25010205

Brohl, Andrew S; Solomon, David A; Chang, Wendy; Wang, Jianjun; Song, Young; Sindiri, Sivasish; Patidar, Rajesh; Hurd, Laura; Chen, Li; Shern, Jack F; Liao, Hongling; Wen, Xinyu; Gerard, Julia; Kim, Jung-Sik; Lopez Guerrero, Jose Antonio; Machado, Isidro; Wai, Daniel H; Picci, Piero; Triche, Timothy; Horvai, Andrew E; Miettinen, Markku; Wei, Jun S; Catchpool, Daniel; Llombart-Bosch, Antonio; Waldman, Todd; Khan, Javed

2014-07-01

62

Bone- and cartilage-forming tumors and ewing sarcoma: an update with a gnathic emphasis.  

PubMed

Over the past decade, there have been remarkable advances in bone tumor pathology. Insights into the genetic basis and pathobiology of many tumor types have impacted diagnosis, classification, and treatment. However, because gnathic lesions may comprise only a small proportion of cases overall for many tumors, clinicopathologic features and management considerations specific to this subset may be overlooked. Here we provide a summary of recent developments in the following tumor types: osteosarcoma (OS), chondrosarcoma (CS), osteoid osteoma (OO), osteoblastoma (OB), and Ewing sarcoma (ES). In particular, we will give special consideration to cases arising in the jaws. PMID:25409851

Stewart, Brian D; Reith, John D; Knapik, Jacquelyn A; Chi, Angela C

2014-12-01

63

CT and MR findings in a neuroforaminal extraskeletal Ewing sarcoma mimicking benign nerve sheath tumor  

Microsoft Academic Search

Introduction  We report the CT and MR findings in a 30-year-old man with extraskeletal Ewing sarcoma (EES) involving the left neural foramen\\u000a at L5–S1 level.\\u000a \\u000a \\u000a \\u000a \\u000a Materials and methods  The patient was evaluated with preoperative lumbosacral CT and MR imaging and postoperative lumbosacral MR imaging.\\u000a \\u000a \\u000a \\u000a Results   The lesion was hyperdense on CT, isointense on T1- and T2-weighted MR images, and enhanced homogeneously after

S. Avcu; H. N. Özcan; M. ?zmirli; M. Lemmerling

64

Primary Ewing sarcoma/primitive neuroectodermal tumor of the renal pelvis: a case report.  

PubMed

Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a childhood malignancy, typically occurring in the bone and rarely in any other part of the body. We herein present a case of ES/PNET of the renal pelvis. A 37-year-old male patient presented with a chief complaint of pain in the left flank and gross hematuria. The tumor had caused moderate hydronephrosis, and ureteroscopic biopsy findings were highly suspicious of sarcoma. Subsequently, radical nephroureterectomy was performed. On the basis of the pathological and cytogenetic findings, a final diagnosis of primary ES/PNET of left renal pelvis was made. Adjuvant chemotherapy with adriamycin and ifosfamide was initiated as ES/PNET often exhibits aggressive biological behavior. The patient was disease-free at his last regular follow-up visit 18 months after the surgery. To our knowledge, this is the first reported case of primary ES/PNET of the renal pelvis. PMID:25242447

Liu, Zhihong; Wang, Xianding; Lu, You; Chen, Libo; Lu, Yiping

2014-01-01

65

A small molecule blocking oncogenic protein EWS-FLI1 interaction with RNA helicase A inhibits growth of Ewing's sarcoma  

Microsoft Academic Search

Many sarcomas and leukemias carry nonrandom chromosomal translocations encoding tumor-specific mutant fusion transcription factors that are essential to their molecular pathogenesis. Ewing's sarcoma family tumors (ESFTs) contain a characteristic t(11;22) translocation leading to expression of the oncogenic fusion protein EWS-FLI1. EWS-FLI1 is a disordered protein that precludes standard structure-based small-molecule inhibitor design. EWS-FLI1 binding to RNA helicase A (RHA) is

Hayriye V Erkizan; Yali Kong; Melinda Merchant; Silke Schlottmann; Julie S Barber-Rotenberg; Linshan Yuan; Ogan D Abaan; Tsu-hang Chou; Sivanesan Dakshanamurthy; Milton L Brown; Aykut Üren; Jeffrey A Toretsky

2009-01-01

66

Detection and characterization of side population in Ewing's sarcoma SK-ES-1 cells in vitro  

SciTech Connect

Dye exclusion is a valuable technique to isolate cancer stem cells (CSCs) based on an ability of stem cell to efflux fluorescent DNA-binding dye, especially for tumors without unique surface markers. It has been proven that side population (SP) cells that exclude Hoechst 33342 dye are enriched with stem-like cells in several cancer cell lines. In this study, we isolated and characterized SP cells from human Ewing's sarcoma cell line SK-ES-1 in vitro. SP cells were detected in SK-ES-1 and comprised 1.2% of total cell population. Only SP cells had the capacity to regenerate both SP and non-SP cells. The proliferation rates were similar between SP and non-SP cells. However, the clonogenicity and invasiveness of SP cells were significantly higher than that of non-SP cells. Further characterization of this SP phenotype presented other properties. SP cells exhibited increased multi-drug resistance and the ATP binding cassette protein (ABC) transporters were up-regulated in SP population. These findings suggest that SP cells derived from Ewing's sarcoma play the critical role in tumor metastasis and recurrence and might be an ideal target for clinical therapy.

Yang, Min [Institute of Orthopaedics and Traumatology of PLA of China, Xijing Hospital, The Fourth Military Medical University, Xi'an, Shaanxi 710032 (China)] [Institute of Orthopaedics and Traumatology of PLA of China, Xijing Hospital, The Fourth Military Medical University, Xi'an, Shaanxi 710032 (China); Zhang, Rui [Department of Biochemistry and Molecular Biology, The Fourth Military Medical University, Xi'an, Shaanxi 710032 (China)] [Department of Biochemistry and Molecular Biology, The Fourth Military Medical University, Xi'an, Shaanxi 710032 (China); Yan, Ming; Ye, Zhengxu; Liang, Wei [Institute of Orthopaedics and Traumatology of PLA of China, Xijing Hospital, The Fourth Military Medical University, Xi'an, Shaanxi 710032 (China)] [Institute of Orthopaedics and Traumatology of PLA of China, Xijing Hospital, The Fourth Military Medical University, Xi'an, Shaanxi 710032 (China); Luo, Zhuojing, E-mail: luozhuojing@hotmail.com [Institute of Orthopaedics and Traumatology of PLA of China, Xijing Hospital, The Fourth Military Medical University, Xi'an, Shaanxi 710032 (China)] [Institute of Orthopaedics and Traumatology of PLA of China, Xijing Hospital, The Fourth Military Medical University, Xi'an, Shaanxi 710032 (China)

2010-01-01

67

Intensive combined modality therapy including low-dose TBI in high-risk Ewing's sarcoma patients  

SciTech Connect

Twenty-four high-risk Ewing's sarcoma patients were treated on an intensive combined modality protocol including low-dose fractionated total body irradiaiton (TBI) and autologous bone marrow infusion (ABMI). Twenty patients (83%) achieved a complete clinical response to the primary and/or metastatic sites following induction therapy. The median disease-free interval was 18 months, and nine patients remain disease-free with a follow-up of 22 to 72 months. Local failure as a manifestation of initial relapse occurred in only three patients (15%), each having synchronous distant failure. Eight patients failed initially with only distant metastases, usually within 1-2 years following a complete clinical response. Two patterns of granulocyte recovery following consolidative therapy (including TBI and ABMI) were recognized. The time to platelet recovery was different for the groups with early and late granulocyte recovery. Patients with late recovery did not tolerate maintenance chemotherapy. However, there was no difference in disease-free and overall survival, when comparing the groups with early and late granulocyte recovery. It is concluded that these high-risk Ewing's sarcoma patients remain a poor-prognosis group in spite of intensive combined modality therapy including low-dose TBI. The control of microscopic systemic disease remains the major challenge to improving the cure rate. A new combined modality protocol with high-dose 'therapeutic' TBI (800 rad/2 fractions) is being used and the protocol design is outlined.

Kinsella, T.J.; Glaubiger, D.; Diesseroth, A.; Makuch, R.; Waller, B.; Pizzo, P.; Glatstein, E.

1983-12-01

68

Hypoxia shifts activity of neuropeptide Y in Ewing sarcoma from growth-inhibitory to growth-promoting effects  

E-print Network

Ewing sarcoma (ES) is an aggressive malignancy driven by an oncogenic fusion protein, EWS-FLI1. Neuropeptide Y (NPY), and two of its receptors, Y1R and Y5R are up-regulated by EWS-FLI1 and abundantly expressed in ES cells. ...

Lu, Congyi

69

Ewing Sarcoma Protein Ewsr1 Maintains Mitotic Integrity and Proneural Cell Survival in the Zebrafish Embryo  

PubMed Central

Background The Ewing sarcoma breakpoint region 1 gene (EWSR1), also known as EWS, is fused to a number of different partner genes as a result of chromosomal translocation in diverse sarcomas. Despite the involvement of EWSR1 in these diverse sarcomas, the in vivo function of wild type EWSR1 remains unclear. Principal Findings We identified two zebrafish EWSR1 orthologues, ewsr1a and ewsr1b, and demonstrate that both genes are expressed maternally, and are expressed ubiquitously throughout zebrafish embryonic development. Morpholino induced knockdown of both zebrafish ewsr1 genes led to mitotic defects with multipolar or otherwise abnormal mitotic spindles starting from the bud stage (10 hour post-fertilization (hpf)). The abnormalities in mitotic spindles were followed by p53-mediated apoptosis in the developing central nervous system (CNS) leading to a reduction in the number of proneural cells, disorganization of neuronal networks, and embryonic lethality by 5 days post-fertilization. siRNA silencing of EWSR1 in Hela cells resulted in mitotic defects accompanied by apoptotic cell death, indicating that the role of EWSR1 is conserved between zebrafish and human. Conclusions Ewsr1 maintains mitotic integrity and proneural cell survival in early zebrafish development. PMID:17912356

Azuma, Mizuki; Embree, Lisa J.; Sabaawy, Hatem; Hickstein, Dennis D.

2007-01-01

70

Long-term results in 144 localized Ewing's sarcoma patients treated with combined therapy.  

PubMed

The results of 144 previously untreated cases of primary Ewing's sarcoma of bone are reported with a minimum follow-up of 5 years. This series was treated between 1972 and 1982 at Istituto Ortopedico Rizzoli with a combined therapy. The local control of the disease consisted of amputation (ten cases), resection followed by radiation therapy (35-45 Gy) (48 cases) and radiation therapy alone (40-60 Gy) (86 cases). Adjuvant chemotherapy, rigorously standardized, was performed according two different protocols: the first (85 cases treated in the period 1972-1978) consisted of vincristine (VCR) Adriamycin (doxorubicin) (ADM), and cyclophosphamide (EDX); the second (59 cases treated in the period 1979-1982) of VCR, ADM, EDX and dactinomycin (DACT). At a follow-up of 5 to 16 years (median, 9), 59 patients (41%) are continuously disease-free (CDF), 81 (56%) developed metastatic disease and/or local recurrence, and four (3%) had a second malignancy. Three factors seem to be correlated to prognosis: the site of the initial lesion (only 23% of the pelvic lesions are represented in the CDF group versus 46% of the other locations); the chemotherapy protocol (32% of the cases in the first protocol are CDF versus 54% in the second); the type of local treatment (60% of the patients treated with amputation or resection plus radiotherapy versus 28% of those treated with radiation therapy alone are CDF). A local recurrence was observed in 24% of the patients (8% in the group locally treated with surgery or surgery plus radiation therapy versus 36% in the group treated with radiation therapy alone). These data suggest that even though adjuvant chemotherapy can improve the long-term results in localized Ewing's sarcoma patients, this disease still represents, in a high percentage of cases, a lethal process whose final prognosis widely depends on the local control of the lesion. Due to the questionable effect of the radiation therapy alone in controlling the primary lesion and its important side effects, the role of surgery in treating Ewing's sarcoma of bone should be extended. PMID:2924256

Bacci, G; Toni, A; Avella, M; Manfrini, M; Sudanese, A; Ciaroni, D; Boriani, S; Emiliani, E; Campanacci, M

1989-04-15

71

Local control of Ewing's sarcoma of bone with radiotherapy and combination chemotherapy  

SciTech Connect

Between 1964 and 1977, 94 patients with Ewing's sarcoma of bone were treated at the National Cancer Institute. They received 5000 rad to the whole bone and progressively more aggressive chemotherapy protocols. The patients were divided according to site of primary lesion into central, proximal and distal lesions, with 19%, 33% and 57%, respectively, alive and well. The local control rate is high (93%), with good functional results in the distal lesions, and no changes are needed in radiation therapy dose or volume. Control is not as satisfactory for central and proximal lesions and efforts need to be made to increase control at these sites. We are at present attempting to define more accurately the extent of soft tissue disease, increasing the dose to 6000 rad for central lesions, and using a more aggressive chemotherapy program, in the hope of increasing the local control in these more aggressive tumors.

Tepper, J.; Glaubiger, D.; Lichter, A.; Wackenhut, J.; Glatstein, E.

1980-11-01

72

In vitro radiation studies on Ewing's sarcoma cell lines and human bone marrow: application to the clinical use of total body irradiation (TBI)  

SciTech Connect

Patients with Ewing's sarcoma who present with a central axis or proximal extremity primary and/or with metastatic disease have a poor prognosis despite aggressive combination chemotherapy and local irradiation. In this high risk group of patients, total body irradiation (TBI) has been proposed as a systemic adjuvant. To aid in the design of a clinical TBI protocol, the authors have studied in the in vitro radiation response of two established cell lines of Ewing's sarcoma and human bone marrow CFUc. The Ewing's lines showed a larger D/sub 0/ and anti-n compared to the bone marrow CFU. No repair of potentially lethal radiation damage (PLDR) was found after 4.5 Gy in plateau phase Ewing's sarcoma cells. A theoretical split dose survival curve for both the Ewing's sarcoma lines and human bone marrow CFUc using this TBI schedule shows a significantly lower surviving fraction (10/sup -4/-10/sup -5/) for the bone marrow CFUc. Based on these in vitro results, two 4.0 Gy fractions separated by 24 hours is proposed as the TBI regimen. Because of the potentially irreversible damage to bone marrow, autologous bone marrow transplantation following the TBI is felt to be necessary. The details of this clinical protocol in high risk Ewing's sarcoma patients are outlined.

Kinsella, T.J.; Mitchell, J.B.; McPherson, S.; Miser, J.; Triche, T.; Glatstein, E.

1984-07-01

73

EWS-Fli1 antisense oligodeoxynucleotide inhibits proliferation of human Ewing's sarcoma and primitive neuroectodermal tumor cells.  

PubMed Central

The translocation t(11;22) is a common chromosomal abnormality detected both in Ewing's sarcoma and in primitive neuroectodermal tumor cells. The translocation results in an EWS-Fli1 fusion gene, made up of the 5' half of the EWS gene on chromosome 22 fused to the 3' half of the Fli1 gene on chromosome 11. Recent studies have evaluated possible roles of the fusion gene products. However, the biological significance of EWS-Fli1 is still unknown. Using a competitive polymerase chain reaction technique, we show here that there might be a correlation between the expression levels of the EWS-Fli1 fusion gene and the proliferative activities of Ewing's sarcoma and primitive neuroectodermal tumor cells. When the EWS-Fli1 expression is inhibited by antisense oligodeoxynucleotides against the fusion RNA, the growth of the tumor cells is significantly reduced both in vitro and in vivo. The data further indicate the growth inhibition of the cells by the antisense sequence might be mediated by G0/G1 block in the cell cycle progression. These results suggest that EWS-Fli1 may play an important role in the proliferation of the tumor cells, and the EWS-Fli1 fusion RNA could be used as a target to inhibit the growth of Ewing's sarcoma and primitive neuroectodermal tumor with the specific antisense oligonucleotide. PMID:9005992

Tanaka, K; Iwakuma, T; Harimaya, K; Sato, H; Iwamoto, Y

1997-01-01

74

Chest Wall Ewing Sarcoma Family of Tumors: Long-Term Outcomes  

SciTech Connect

Purpose: To review the 40-year University of Florida experience treating Ewing sarcoma family of tumors of the chest wall. Methods and Materials: Thirty-nine patients were treated from 1966 to 2006. Of the patients, 22 were treated with radiotherapy (RT) alone, and 17 patients were treated with surgery with or without RT. Of 9 patients with metastatic disease, 8 were treated with RT alone. The risk profiles of each group were otherwise similar. The median age was 16.6 years, and the most frequent primary site was the rib (n = 17). The median potential follow-up was 19.2 years. Results: The 5-year actuarial overall survival (OS), cause-specific survival (CSS), and local control (LC) rates were 34%, 34%, and 72%, respectively. For the nonmetastatic subset (n = 30), the 5-year OS, CSS, and LC rates were 44%, 44%, and 79%, respectively. LC was not statistically significantly different between patients treated with RT alone (61%) vs. surgery + RT (75%). None of the 4 patients treated with surgery alone experienced local failure. No patient or treatment variable was significantly associated with local failure. Of the patients, 26% experienced Common Toxicity Criteria (CTC) Grade 3+ toxicity, including 2 pulmonary deaths. Modern intensive systemic therapy helped increase the 5-year CSS from 7% to 49% in patients treated after 1984 (p = 0.03). Conclusions: This is the largest single-institution series describing the treatment of chest wall Ewing tumors. Despite improvements in survival, obtaining local control is challenging and often accompanied by morbidity. Effort should be focused on identifying tumors amenable to combined-modality local therapy and to improving RT techniques.

Indelicato, Daniel J., E-mail: dindelicato@floridaproton.org [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL, and University of Florida Proton Therapy Institute, Jacksonville, FL (United States); Keole, Sameer R. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL, and University of Florida Proton Therapy Institute, Jacksonville, FL (United States); Lagmay, Joanne P. [Department of Pediatrics, University of Florida College of Medicine, Gainesville, FL (United States); Morris, Christopher G. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL, and the University of Florida Proton Therapy Institute, Jacksonville, FL (United States); Gibbs, C. Parker; Scarborough, Mark T. [Department of Orthopedic Surgery, University of Florida College of Medicine, Gainesville, FL (United States); Islam, Saleem [Department of Surgery at the University of Florida College of Medicine, Gainesville, FL (United States); Marcus, Robert B. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL, and the University of Florida Proton Therapy Institute, Jacksonville, FL (United States)

2011-09-01

75

Contribution of magnetic resonance imaging in the diagnosis of talus skip metastases of Ewing's sarcoma of the calcaneus in a child: a case report  

PubMed Central

Introduction Ewing's sarcoma of the calcaneus is rare. About thirty cases with calcaneus involvement have been reported in the literature. Talus skip metastases have rarely been described in the available literature Case presentation We report a case of a 14-year-old Moroccan boy, who presented with Ewing's sarcoma of his right calcaneus, diagnosed by swelling of the calcaneus evolving over a year. Radiography, computed tomography and magnetic resonance imaging showed an important tumoral process of the calcaneus and talus skip metastases. The diagnosis was confirmed with histology after a biopsy. In spite of amputation and postoperative chemotherapy, our patient died six months later due to secondary respiratory distress after lung metastasis. Conclusion Imaging, especially magnetic resonance, is important in the diagnosis of Ewing sarcoma and skeletal skip metastases. Treatment of Ewing's sarcoma consists of chemotherapy, radiation therapy and surgical resection depending on the stage and extent of the disease. With the exception of lesions in the calcaneus, the prognosis for disease-free survival of Ewing's sarcoma of the foot is excellent. PMID:21910875

2011-01-01

76

EWS/FLI and its Downstream Target NR0B1 Interact Directly to Modulate Transcription and Oncogenesis in Ewing's Sarcoma  

PubMed Central

Most Ewing's sarcomas harbor chromosomal translocations that encode fusions between EWS and ETS family members. The most common fusion, EWS/FLI, consists of an EWSR1-derived strong transcriptional activation domain fused, in frame, to the DNA binding domain-containing portion of FLI1. EWS/FLI functions as an aberrant transcription factor to regulate genes that mediate the oncogenic phenotype of Ewing's sarcoma. One of these regulated genes, NR0B1, encodes a co-repressor protein, and likely plays a transcriptional role in tumorigenesis. However, the genes that NR0B1 regulates and the transcription factors it interacts with in Ewing's sarcoma are largely unknown. We used transcriptional profiling and chromatin immunoprecipitation to identify genes that are regulated by NR0B1, and compared these data to similar data for EWS/FLI. While the transcriptional profile overlapped as expected, we also found that the genome-wide localization of NR0B1and EWS/FLI overlapped as well, suggesting that they regulate some genes coordinately. Further analysis revealed that NR0B1 and EWS/FLI physically interact. This protein-protein interaction is likely to be relevant for Ewing's sarcoma development because mutations in NR0B1 that disrupt the interaction have transcriptional consequences and also abrogate oncogenic transformation. Taken together, these data suggest that EWS/FLI and NR0B1 physically interact, coordinately modulate gene expression, and mediate the transformed phenotype of Ewing's sarcoma. PMID:19920188

Kinsey, Michelle; Smith, Richard; Iyer, Anita K.; McCabe, Edward R.B.; Lessnick, Stephen L.

2009-01-01

77

Molecular inversion probe analysis detects novel copy number alterations in Ewing sarcoma.  

PubMed

Ewing sarcoma (ES) is the second most common bone tumor in children and young adults, with dismal outcomes for metastatic and relapsed disease. To better understand the molecular pathogenesis of ES and to identify new prognostic markers, we used molecular inversion probes (MIPs) to evaluate copy number alterations (CNAs) and loss of heterozygosity (LOH) in formalin-fixed paraffin-embedded (FFPE) samples, which included 40 ES primary tumors and 12 ES metastatic lesions. CNAs were correlated with clinical features and outcome, and validated by immunohistochemistry (IHC). We identified previously reported CNAs, in addition to SMARCB1 (INI1/SNF5) homozygous loss and copy neutral LOH. IHC confirmed SMARCB1 protein loss in 7-10% of clinically diagnosed ES tumors in three separate cohorts (University of Utah [N = 40], Children's Oncology Group [N = 31], and University of Michigan [N = 55]). A multifactor copy number (MCN)-index was highly predictive of overall survival (39% vs. 100%, P < 0.001). We also identified RELN gene deletions unique to 25% of ES metastatic samples. In summary, we identified both known and novel CNAs using MIP technology for the first time in FFPE samples from patients with ES. CNAs detected by microarray correlate with outcome and may be useful for risk stratification in future clinical trials. PMID:22868000

Jahromi, Mona S; Putnam, Angelica R; Druzgal, Colleen; Wright, Jennifer; Spraker-Perlman, Holly; Kinsey, Michelle; Zhou, Holly; Boucher, Kenneth M; Randall, R Lor; Jones, Kevin B; Lucas, David; Rosenberg, Andrew; Thomas, Dafydd; Lessnick, Stephen L; Schiffman, Joshua D

2012-01-01

78

Identification of a tripartite import signal in the Ewing Sarcoma protein (EWS)  

SciTech Connect

The Ewing Sarcoma (EWS) protein is a ubiquitously expressed RNA processing factor that localises predominantly to the nucleus. However, the mechanism through which EWS enters the nucleus remains unclear, with differing reports identifying three separate import signals within the EWS protein. Here we have utilized a panel of truncated EWS proteins to clarify the reported nuclear localisation signals. We describe three C-terminal domains that are important for efficient EWS nuclear localization: (1) the third RGG-motif; (2) the last 10 amino acids (known as the PY-import motif); and (3) the zinc-finger motif. Although these three domains are involved in nuclear import, they are not independently capable of driving the efficient import of a GFP-moiety. However, collectively they form a complex tripartite signal that efficiently drives GFP-import into the nucleus. This study helps clarify the EWS import signal, and the identification of the involvement of both the RGG- and zinc-finger motifs has wide reaching implications.

Shaw, Debra J.; Morse, Robert; Todd, Adrian G. [Clinical Neurobiology, IBCS, Peninsula College of Medicine and Dentistry, Exeter EX1 2LU (United Kingdom)] [Clinical Neurobiology, IBCS, Peninsula College of Medicine and Dentistry, Exeter EX1 2LU (United Kingdom); Eggleton, Paul [Inflammation and Musculoskeletal Disease, IBCS, Peninsula College of Medicine and Dentistry, Exeter EX1 2LU (United Kingdom) [Inflammation and Musculoskeletal Disease, IBCS, Peninsula College of Medicine and Dentistry, Exeter EX1 2LU (United Kingdom); MRC Immunochemistry Unit, University of Oxford, Oxford OX1 3QU (United Kingdom); Lorson, Christian L. [Department of Veterinary Pathobiology, Bond Life Sciences Center, 1201 Rollins Road, University of Missouri, Columbia, MO 65211 (United States)] [Department of Veterinary Pathobiology, Bond Life Sciences Center, 1201 Rollins Road, University of Missouri, Columbia, MO 65211 (United States); Young, Philip J., E-mail: philip.young@pms.ac.uk [Clinical Neurobiology, IBCS, Peninsula College of Medicine and Dentistry, Exeter EX1 2LU (United Kingdom)

2009-12-25

79

Ewing sarcoma gene EWS is essential for meiosis and B lymphocyte development  

PubMed Central

Ewing sarcoma gene EWS encodes a putative RNA-binding protein with proposed roles in transcription and splicing, but its physiological role in vivo remains undefined. Here, we have generated Ews-deficient mice and demonstrated that EWS is required for the completion of B cell development and meiosis. Analysis of Ews–/– lymphocytes revealed a cell-autonomous defect in precursor B lymphocyte (pre–B lymphocyte) development. During meiosis, Ews-null spermatocytes were deficient in XY bivalent formation and showed reduced meiotic recombination, resulting in massive apoptosis and complete arrest in gamete maturation. Inactivation of Ews in mouse embryonic fibroblasts resulted in premature cellular senescence, and the mutant animals showed hypersensitivity to ionizing radiation. Finally, we showed that EWS interacts with lamin A/C and that loss of EWS results in a reduced lamin A/C expression. Our findings reveal essential functions for EWS in pre–B cell development and meiosis, with proposed roles in DNA pairing and recombination/repair mechanisms. Furthermore, we demonstrate a novel role of EWS in cellular senescence, possibly through its interaction and modulation of lamin A/C. PMID:17415412

Li, Hongjie; Watford, Wendy; Li, Cuiling; Parmelee, Alissa; Bryant, Mark A.; Deng, Chuxia; O'Shea, John; Lee, Sean Bong

2007-01-01

80

Development of a Preclinical Orthotopic Xenograft Model of Ewing Sarcoma and Other Human Malignant Bone Disease Using Advanced In Vivo Imaging  

PubMed Central

Ewing sarcoma and osteosarcoma represent the two most common primary bone tumours in childhood and adolescence, with bone metastases being the most adverse prognostic factor. In prostate cancer, osseous metastasis poses a major clinical challenge. We developed a preclinical orthotopic model of Ewing sarcoma, reflecting the biology of the tumour-bone interactions in human disease and allowing in vivo monitoring of disease progression, and compared this with models of osteosarcoma and prostate carcinoma. Human tumour cell lines were transplanted into non-obese diabetic/severe combined immunodeficient (NSG) and Rag2?/?/?c?/? mice by intrafemoral injection. For Ewing sarcoma, minimal cell numbers (1000–5000) injected in small volumes were able to induce orthotopic tumour growth. Tumour progression was studied using positron emission tomography, computed tomography, magnetic resonance imaging and bioluminescent imaging. Tumours and their interactions with bones were examined by histology. Each tumour induced bone destruction and outgrowth of extramedullary tumour masses, together with characteristic changes in bone that were well visualised by computed tomography, which correlated with post-mortem histology. Ewing sarcoma and, to a lesser extent, osteosarcoma cells induced prominent reactive new bone formation. Osteosarcoma cells produced osteoid and mineralised “malignant” bone within the tumour mass itself. Injection of prostate carcinoma cells led to osteoclast-driven osteolytic lesions. Bioluminescent imaging of Ewing sarcoma xenografts allowed easy and rapid monitoring of tumour growth and detection of tumour dissemination to lungs, liver and bone. Magnetic resonance imaging proved useful for monitoring soft tissue tumour growth and volume. Positron emission tomography proved to be of limited use in this model. Overall, we have developed an orthotopic in vivo model for Ewing sarcoma and other primary and secondary human bone malignancies, which resemble the human disease. We have shown the utility of small animal bioimaging for tracking disease progression, making this model a useful assay for preclinical drug testing. PMID:24409320

Batey, Michael A.; Almeida, Gilberto S.; Wilson, Ian; Dildey, Petra; Sharma, Abhishek; Blair, Helen; Hide, I. Geoff; Heidenreich, Olaf; Vormoor, Josef; Maxwell, Ross J.; Bacon, Chris M.

2014-01-01

81

Quantification of the Heterogeneity of Prognostic Cellular Biomarkers in Ewing Sarcoma Using Automated Image and Random Survival Forest Analysis  

PubMed Central

Driven by genomic somatic variation, tumour tissues are typically heterogeneous, yet unbiased quantitative methods are rarely used to analyse heterogeneity at the protein level. Motivated by this problem, we developed automated image segmentation of images of multiple biomarkers in Ewing sarcoma to generate distributions of biomarkers between and within tumour cells. We further integrate high dimensional data with patient clinical outcomes utilising random survival forest (RSF) machine learning. Using material from cohorts of genetically diagnosed Ewing sarcoma with EWSR1 chromosomal translocations, confocal images of tissue microarrays were segmented with level sets and watershed algorithms. Each cell nucleus and cytoplasm were identified in relation to DAPI and CD99, respectively, and protein biomarkers (e.g. Ki67, pS6, Foxo3a, EGR1, MAPK) localised relative to nuclear and cytoplasmic regions of each cell in order to generate image feature distributions. The image distribution features were analysed with RSF in relation to known overall patient survival from three separate cohorts (185 informative cases). Variation in pre-analytical processing resulted in elimination of a high number of non-informative images that had poor DAPI localisation or biomarker preservation (67 cases, 36%). The distribution of image features for biomarkers in the remaining high quality material (118 cases, 104 features per case) were analysed by RSF with feature selection, and performance assessed using internal cross-validation, rather than a separate validation cohort. A prognostic classifier for Ewing sarcoma with low cross-validation error rates (0.36) was comprised of multiple features, including the Ki67 proliferative marker and a sub-population of cells with low cytoplasmic/nuclear ratio of CD99. Through elimination of bias, the evaluation of high-dimensionality biomarker distribution within cell populations of a tumour using random forest analysis in quality controlled tumour material could be achieved. Such an automated and integrated methodology has potential application in the identification of prognostic classifiers based on tumour cell heterogeneity. PMID:25243408

Yu, Haiyue; Branford White, Harriet; Schafer, Karl L.; Llombart-Bosch, Antonio; Machado, Isidro; Picci, Piero; Hogendoorn, Pancras C. W.; Athanasou, Nicholas A.; Noble, J. Alison; Hassan, A. Bassim

2014-01-01

82

Ewing sarcoma-primitive neuroectodermal tumor of the uterus: a clinicopathologic, immunohistochemical and ultrastructural study of one case  

Microsoft Academic Search

Introduction  Ewing sarcoma-primitive neuroectodermal tumors (ES\\/PNET) constitute a family of neoplasms characterized by a continuum of\\u000a neuroectodermal differentiation. ES\\/PNET of the uterus is rare. There are 43 cases published in the English literature as\\u000a far as we know. We describe an additional case.\\u000a \\u000a \\u000a \\u000a \\u000a Case report  A 56-year-old woman presented with a 2-month history of irregular menopausal vaginal bleeding. After surgical excision, microscopic,

Ya-Li RenXiu-Ying; Xiu-Ying Tang; Ting Li

2011-01-01

83

Characterization and drug resistance patterns of Ewing's sarcoma family tumor cell lines.  

PubMed

Despite intensive treatment with chemotherapy, radiotherapy and surgery, over 70% of patients with metastatic Ewing's Sarcoma Family of Tumors (EFT) will die of their disease. We hypothesize that properly characterized laboratory models reflecting the drug resistance of clinical tumors will facilitate the application of new therapeutic agents to EFT. To determine resistance patterns, we studied newly established EFT cell lines derived from different points in therapy: two established at diagnosis (CHLA-9, CHLA-32), two after chemotherapy and progressive disease (CHLA-10, CHLA-25), and two at relapse after myeloablative therapy and autologous bone marrow transplantation (post-ABMT) (CHLA-258, COG-E-352). The new lines were compared to widely studied EFT lines TC-71, TC-32, SK-N-MC, and A-673. These lines were extensively characterized with regard to identity (short tandem repeat (STR) analysis), p53, p16/14 status, and EWS/ETS breakpoint and target gene expression profile. The DIMSCAN cytotoxicity assay was used to assess in vitro drug sensitivity to standard chemotherapy agents. No association was found between drug resistance and the expression of EWS/ETS regulated genes in the EFT cell lines. No consistent association was observed between drug sensitivity and p53 functionality or between drug sensitivity and p16/14 functionality across the cell lines. Exposure to chemotherapy prior to cell line initiation correlated with drug resistance of EFT cell lines in 5/8 tested agents at clinically achievable concentrations (CAC) or the lower tested concentration (LTC): (cyclophosphamide (as 4-HC) and doxorubicin at CAC, etoposide, irinotecan (as SN-38) and melphalan at LTC; P<0.1 for one agent, and P<0.05 for four agents. This panel of well-characterized drug-sensitive and drug-resistant cell lines will facilitate in vitro preclinical testing of new agents for EFT. PMID:24312454

May, William A; Grigoryan, Rita S; Keshelava, Nino; Cabral, Daniel J; Christensen, Laura L; Jenabi, Jasmine; Ji, Lingyun; Triche, Timothy J; Lawlor, Elizabeth R; Reynolds, C Patrick

2013-01-01

84

Characterization and Drug Resistance Patterns of Ewing's Sarcoma Family Tumor Cell Lines  

PubMed Central

Despite intensive treatment with chemotherapy, radiotherapy and surgery, over 70% of patients with metastatic Ewing's Sarcoma Family of Tumors (EFT) will die of their disease. We hypothesize that properly characterized laboratory models reflecting the drug resistance of clinical tumors will facilitate the application of new therapeutic agents to EFT. To determine resistance patterns, we studied newly established EFT cell lines derived from different points in therapy: two established at diagnosis (CHLA-9, CHLA-32), two after chemotherapy and progressive disease (CHLA-10, CHLA-25), and two at relapse after myeloablative therapy and autologous bone marrow transplantation (post-ABMT) (CHLA-258, COG-E-352). The new lines were compared to widely studied EFT lines TC-71, TC-32, SK-N-MC, and A-673. These lines were extensively characterized with regard to identity (short tandem repeat (STR) analysis), p53, p16/14 status, and EWS/ETS breakpoint and target gene expression profile. The DIMSCAN cytotoxicity assay was used to assess in vitro drug sensitivity to standard chemotherapy agents. No association was found between drug resistance and the expression of EWS/ETS regulated genes in the EFT cell lines. No consistent association was observed between drug sensitivity and p53 functionality or between drug sensitivity and p16/14 functionality across the cell lines. Exposure to chemotherapy prior to cell line initiation correlated with drug resistance of EFT cell lines in 5/8 tested agents at clinically achievable concentrations (CAC) or the lower tested concentration (LTC): (cyclophosphamide (as 4-HC) and doxorubicin at CAC, etoposide, irinotecan (as SN-38) and melphalan at LTC; P<0.1 for one agent, and P<0.05 for four agents. This panel of well-characterized drug-sensitive and drug-resistant cell lines will facilitate in vitro preclinical testing of new agents for EFT. PMID:24312454

May, William A.; Grigoryan, Rita S.; Keshelava, Nino; Cabral, Daniel J.; Christensen, Laura L.; Jenabi, Jasmine; Ji, Lingyun; Triche, Timothy J.; Lawlor, Elizabeth R.; Reynolds, C. Patrick

2013-01-01

85

Pharmacokinetic modeling optimizes inhibition of the 'undruggable' EWS-FLI1 transcription factor in Ewing Sarcoma  

PubMed Central

Transcription factors have long been deemed ‘undruggable’ targets for therapeutics. Enhanced recognition of protein biochemistry as well as the need to have more targeted approaches to treat cancer has rendered transcription factors approachable for therapeutic development. Since transcription factors lack enzymatic domains, the specific targeting of these proteins has unique challenges. One challenge is the hydrophobic microenvironment that affects small molecules gaining access to block protein interactions. The most attractive transcription factors to target are those formed from tumor specific chromosomal translocations that are validated oncogenic driver proteins. EWS-FLI1 is a fusion protein that results from the pathognomonic translocation of Ewing sarcoma (ES). Our past work created the small molecule YK-4-279 that blocks EWS-FLI1 from interacting with RNA Helicase A (RHA). To fulfill long-standing promise in the field by creating a clinically useful drug, steps are required to allow for in vivo administration. These investigations identify the need for continuous presence of the small molecule protein-protein inhibitor for a period of days. We describe the pharmacokinetics of YK-4-279 and its individual enantiomers. In vivo studies confirm prior in vitro experiments showing (S)-YK-4-279 as the EWS-FLI1 specific enantiomer demonstrating both induction of apoptosis and reduction of EWS-FLI1 regulated caveolin-1 protein. We have created the first rat xenograft model of ES, treated with (S)-YK-4-279 dosing based upon PK modeling leading to a sustained complete response in 2 of 6 ES tumors. Combining laboratory studies, pharmacokinetic measurements, and modeling has allowed us to create a paradigm that can be optimized for in vivo systems using both in vitro data and pharmacokinetic simulations. Thus, (S)-YK-4-279 as a small molecule drug is ready for continued development towards a first-in-human, first-in-class, clinical trial. PMID:24481407

Hong, S. Peter; Kallakury, Bhaskar; Monroe, Phillip; Erkizan, Hayriye V; Barber-Rotenberg, Julie S.; Houghton, Peter; Uren, Aykut; Toretsky, Jeffrey A.

2014-01-01

86

miR-125b develops chemoresistance in Ewing sarcoma/primitive neuroectodermal tumor  

PubMed Central

Background Diverse functions of microRNAs (miRNAs), including effects on tumorigenesis, proliferation, and differentiation, have been reported, and several miRNAs have also been demonstrated to play an important role in apoptosis. In this study, we investigated the possible role that miRNAs may play in the development of chemoresistance in Ewing sarcoma/primitive neuroectodermal tumor (EWS). Methods We screened doxorubicin (Dox)-resistant EWS cells to identify any distinct miRNA sequences that may regulate the chemoresistance of EWS cells. The effects of miRNAs were evaluated using a chemosensitivity assay. The possible target genes of the miRNAs were predicted using a web-based prediction program. Results We found miR-125b to be upregulated in two different Dox-resistant EWS cell lines. The upregulation of miR-125b was also confirmed in the EWS tumors having survived chemotherapy regimen which includes doxorubicin. When miR-125b was knocked down in EWS cells, both the Dox-resistant and parental cells showed an enhanced sensitivity to doxorubicin, which was associated with the upregulation of the pro-apoptotic molecules, p53 and Bak. Inversely, the overexpression of miR-125b in parental EWS cells resulted in enhanced drug resistance, not only to doxorubicin, but also to etoposide and vincristine. Conclusions Our findings suggest that miR-125b may play a role in the development of chemoresistance in EWS by suppressing the expression of the apoptotic mediators, such as p53 and Bak. PMID:23497288

2013-01-01

87

Myeloablative therapy against high risk Ewing's sarcoma: A single institution experience and literature review  

PubMed Central

Background Attempts to improve survival outcomes of patients with high risk Ewing's sarcoma (ES) have focused on chemotherapy dose intensification strategies. Aim The objective of this study is to retrospectively evaluate clinical characteristics and outcome of pediatric patients with high risk ES treated at a single institution. Materials and methods From 1995 to 2008, seventeen patients (male:female, 14:3) were treated with dose-intensive therapy in our institution. Median age at diagnosis was 10 years (range: 2–15). Seven patients had metastases at diagnosis (lung in 6 cases and bone in one case). Eleven patients presented with unresectable disease. Fifteen (88.2%) received the Spanish Society of Pediatric Oncology protocol which includes six cycles of vincristine, doxorubicin, ifosfamide and etoposide. Two out of the six cases that were resectable received postoperative radiation. In addition, eleven patients received definitive radiation therapy. Finally, twelve (70.5%) out of 17 patients received myeloablative therapy with melphalan/etoposide. The rest of patients (N = 5) received busulfan/melphalan. Results Median follow-up was 78 months (range: 15–155 months). Initial responses were complete in all patients, but 9 of them developed progression disease. Seven patients became long-term event-free survivors. No patient died of toxicity after transplantation. The 2- and 5-year overall survival rates for all patients were 93% and 73%, respectively. Event-free survival rates were 74% and 54% at 2 and 5 years, respectively. Conclusion This single-institution experience suggests that myeloablative therapy against high risk ES is effective and safe. PMID:24376974

Lopez, Jose Luis; Perez, Concepcion; Marquez, Catalina; Cabrera, Patricia; Perez, Jose Maria; Ramirez, Gema Lucia; Ordonez, Rafael; Praena-Fernandez, Juan Manuel; Ortiz, Maria Jose

2011-01-01

88

Successful complete resection of Ewing sarcoma arising from the bladder in a 10-year-old boy after chemotherapy.  

PubMed

Ewing sarcoma (ES) of the bladder is extremely rare. Here we report the successful treatment of the youngest case of ES arising from bladder encountered to date and a literature review. A 10-year-old boy who presented with polyuria and lower abdominal swelling was found to have a 13 cm mass arising from the bladder localized to the center of the lower abdomen. Biopsy confirmed ES. Following chemotherapy, the mass shrank to 5 cm and was found to be localized to the right side of the bladder with invasion of the right vas deferens. One-third of the right side of the bladder and part of the right vas deferens were resected. No viable cancer cells were detected in the resected specimen, and the surgical margins were reported to be negative. The patient is currently well with no recurrence or metastases after 11 months. There are 12 cases of Ewings sarcoma arising from the bladder reported in the English literature; two cases in children. Our case will be the third pediatric case and the youngest. PMID:25080033

Sueyoshi, Ryo; Okawada, Manabu; Fujimura, Junya; Saito, Masahiro; Koga, Hiroyuki; Lane, Geoffrey J; Yamataka, Atsuyuki

2014-09-01

89

Trial of Dasatinib in Advanced Sarcomas  

ClinicalTrials.gov

Rhabdomyosarcoma; Malignant Peripheral Nerve Sheath Tumors; Chondrosarcoma; Sarcoma, Ewing's; Sarcoma, Alveolar Soft Part; Chordoma; Epithelioid Sarcoma; Giant Cell Tumor of Bone; Hemangiopericytoma; Gastrointestinal Stromal Tumor (GIST)

2014-06-17

90

[Where does Ewing sarcoma end and begin - two cases of unusual bone tumors with t(20;22)(EWSR1-NFATc2) alteration].  

PubMed

The authors present two cases of Ewing-like sarcoma of the humerus and femur of a 12-year-old boy and a 28-year-old male, respectively. Identical morphology in both tumors consisted of multiple solid nests with a mosaic collection of small, round, uniform cells with clear cytoplasm and no apparent nuclear atypia. A monotonous structural arrangement, including both rich vascularity of bordering septae and significant admixtures of eosinophil leucocytes, resulted in a final organoid "neuroendocrine-like" pattern. Immunohistochemistry revealed diffuse strong CD10, CD99 and CD138 positivity. Detailed molecular analysis in both tumors confirmed translocation t(20;22) resulting in an EWSR1-NFATc2 fusion gene. Additionally, this translocation was accompanied by amplification of the proximal part of the genes and surrounding areas. Clinically, both neoplasms behaved aggressively and they were primarily chemoresistant. Four years later, the patient with the lesion in the humerus developed a massive local recurrence with a disruption of osteosynthesis. The last follow-up disclosed suspicious metastatic deposits in the lung. The boy with the femoral tumor underwent a total femoral prosthesis and there are no signs of local or systemic recurrence after 11 months of follow-up. The authors discuss the taxonomic placement of these rare examples of Ewing-like sarcoma family in the light of new molecular discoveries. Keywords: bone - humerus - femur - small blue round cell tumor - Ewing sarcoma - Ewing-like sarcoma - t(20;22)(EWSR1-NFATc2) - amplification. PMID:24758504

Kinkor, Zden?k; Vane?ek, Tomáš; Svajdler, Marián; Mukenšnabl, Petr; Veselý, Karel; Baxa, Jan; Kokavec, Milan

2014-04-01

91

Lysyl Oxidase Is Downregulated by the EWS/FLI1 Oncoprotein and Its Propeptide Domain Displays Tumor Supressor Activities in Ewing Sarcoma Cells  

PubMed Central

Ewing sarcoma is the second most common bone malignancy in children and young adults. It is driven by oncogenic fusion proteins (i.e. EWS/FLI1) acting as aberrant transcription factors that upregulate and downregulate target genes, leading to cellular transformation. Thus, identificating these target genes and understanding their contribution to Ewing sarcoma tumorigenesis are key for the development of new therapeutic strategies. In this study we show that lysyl oxidase (LOX), an enzyme involved in maintaining structural integrity of the extracellular matrix, is downregulated by the EWS/FLI1 oncoprotein and in consequence it is not expressed in Ewing sarcoma cells and primary tumors. Using a doxycycline inducible system to restore LOX expression in an Ewing sarcoma derived cell line, we showed that LOX displays tumor suppressor activities. Interestingly, we showed that the tumor suppressor activity resides in the propeptide domain of LOX (LOX-PP), an N-terminal domain produced by proteolytic cleavage during the physiological processing of LOX. Expression of LOX-PP reduced cell proliferation, cell migration, anchorage-independent growth in soft agar and formation of tumors in immunodeficient mice. By contrast, the C-terminal domain of LOX, which contains the enzymatic activity, had the opposite effects, corroborating that the tumor suppressor activity of LOX is mediated exclusively by its propeptide domain. Finally, we showed that LOX-PP inhibits ERK/MAPK signalling pathway, and that many pathways involved in cell cycle progression were significantly deregulated by LOX-PP, providing a mechanistic explanation to the cell proliferation inhibition observed upon LOX-PP expression. In summary, our observations indicate that deregulation of the LOX gene participates in Ewing sarcoma development and identify LOX-PP as a new therapeutic target for one of the most aggressive paediatric malignancies. These findings suggest that therapeutic strategies based on the administration of LOX propeptide or functional analogues could be useful for the treatment of this devastating paediatric cancer. PMID:23750284

Garcia-Garcia, Laura; de la Parra, Juan; Alonso, Javier

2013-01-01

92

Pathobiologic Markers of the Ewing Sarcoma Family of Tumors: State of the Art and Prediction of Behaviour  

PubMed Central

Over the past three decades, the outcome of Ewing sarcoma family tumor (ESFT) patients who are nonmetastatic at presentation has improved considerably. The prognosis of patients with metastatic disease at the time of diagnosis and recurrence after therapy remains dismal. Drug-resistant disease at diagnosis or at relapse remains a major cause of mortality among patients diagnosed with ESFT. In order to improve the outcome for patients with potential relapse, there is an urgent need to find reliable markers that either predict tumor behaviour at diagnosis or identify therapeutic molecular targets at the time of recurrence. An improved understanding of the cell of origin and the molecular pathways that regulate tumorigenicity in ESFT should aid us in the search for novel therapies for ESFT. The purpose of this paper is thus to outline current concepts of sarcomagenesis in ESFT and to discuss ESFT patterns of differentiation and molecular markers that might affect prognosis or direct future therapeutic development. PMID:20981347

Pinto, Alfredo; Dickman, Paul; Parham, David

2011-01-01

93

Plerixafor plus G-CSF in combination with chemotherapy for stem cell mobilization in a pediatric patient with Ewing's sarcoma.  

PubMed

Some malignant tumors in childhood require high-dose chemotherapy with stem cell support to achieve a cure. In patients heavily pretreated with myelosuppressive chemotherapy or irradiation, granulocyte colony-stimulating factor (G-CSF) may fail to mobilize stem cells from the bone marrow. Based on the experience with lymphoma and myeloma patients in whom peripheral blood-derived stem cell (PBSC) collection following mobilization with G-CSF failed, we successfully employed plerixafor in a 14-year-old female diagnosed with Ewing's sarcoma in early relapse treated with three lines of chemotherapy in whom PBSC could not be mobilized using either G-CSF alone or G-CSF following chemotherapy. No side effects were observed. Plerixafor may be an effective and safe agent for stem cell collection in pediatric patients with solid tumors, although new studies addressed to evaluate its effectiveness and safety are needed. PMID:22566276

Vives, Susana; Sancho, Juan-Manuel; Almazán, Francisco; Juncà, Jordi; Grifols, Joan-Ramon; Ribera, Josep-Maria

2012-11-01

94

Distinct Transcriptional Signature and Immunoprofile of CIC-DUX4-Fusion Positive Round Cell Tumors Compared to EWSR1-Rearranged Ewing Sarcomas - Further Evidence Toward Distinct Pathologic Entities  

PubMed Central

Round cell sarcomas harboring CIC-DUX4 fusions have recently been described as highly aggressive soft tissue tumors of children and young adults. Due to partial morphologic and immunohistochemical overlap with Ewing sarcoma (ES), CIC-DUX4-positive tumors have generally been classified as Ewing sarcoma-like and managed similarly, however, a systematic comparison at the molecular and immunohistochemical levels between these two groups has not yet been conducted. Based on an initial observation that CIC-DUX4-positive tumors show nuclear immunoreactivity for WT1 and ETS transcription factors, FLI1 and ERG, we performed a detailed immunohistochemical and molecular analysis including these markers, to further investigate the relationship between CIC-DUX4 tumors and ES. The study group included 21 CIC-DUX4-positive sarcomas and 20 EWSR1-rearranged ES. Immunohistochemically, CIC-DUX4 sarcomas showed membranous CD99 positivity in 18 (86%) cases, but only 5 (24%) with a diffuse pattern, while WT1 and FLI1 were strongly positive in all cases. ERG was positive in 18% of cases. All ES expressed CD99 and FLI1, while ERG positivity was only seen in EWSR1-ERG fusion positive ES. WT1 was negative in all ES. Expression profiling validated by q-PCR revealed a distinct gene signature associated with CIC-DUX4 fusion, with upregulation of ETS transcription factors (ETV4, ETV1 and ETV5) and WT1, among top overexpressed genes compared to ES, other sarcomas and normal tissue. In conclusion, the distinct gene signature and immunoprofile of CIC-DUX4 sarcomas suggest a distinct pathogenesis from ES. The consistent WT1 expression may provide a useful clue in the diagnosis in the context of round cell sarcomas negative for EWSR1-rearrangement. PMID:24723486

Specht, Katja; Sung, Yun-Shao; Zhang, Lei; Richter, Gunther H. S.; Fletcher, Christopher D.; Antonescu, Cristina R.

2014-01-01

95

Using Targeted Virotherapy to Treat a Resistant Ewing Sarcoma Model: From the Bedside to the Bench and Back  

PubMed Central

Metastatic Ewing sarcoma (EWS) is often resistant to current multimodal chemotherapeutic regimens. Oncolytic virus therapy (OV) is a novel therapeutic platform whereby viruses can selectively infect as well as replicate in and kill tumor cells, while sparing normal tissues. The purpose of this study is to investigate the efficacy of the biotherapeutic oncolytic agent, vesicular stomatitis virus (VSV?M51), to kill EWS cells that are resistant to conventional therapy. Our hypothesis is that systemic delivery of VSV?M51 can demonstrate tumor-specific killing of resistant EWS cells, as well as a significant decrease of tumor burden in EWS bearing mice. Methods. A biopsy sample was obtained from a patient with metastatic EWS and was used to establish a novel EWS cell line. In vitro assays evaluated the oncolytic effect of vesicular stomatitis virus (VSV?M51) on this cell line. EWS xenograft mice model bearing either lung or subcutaneous tumors was established to evaluate the antitumor specific oncolytic effect of VSV?M51 after local and systemic delivery. Results. The established EWS cell line shared similar molecular and genetic traits to the patient's original tumor specimen. VSV?M51 effectively infected and killed EWS cells in vitro. In vivo, VSV?M51 selectively infected and killed EWS and led to significant delay in tumor growth. Conclusion. This study has been designed to implement a translational link between the bedside and the bench, where a specific challenging clinical scenario guided this basic science research. This research demonstrated that a sarcoma, which is resistant to current conventional standard therapies, is still susceptible to an alternative therapeutic platform, such as OV. Adding OV to the armamentarium of sarcoma treatment can enhance the future therapeutic approach towards these cancer patients. PMID:24672293

Brown, Christopher W.; Werier, Joel; Bell, John

2014-01-01

96

Zoledronic acid inhibits pulmonary metastasis dissemination in a preclinical model of Ewing's sarcoma via inhibition of cell migration  

PubMed Central

Background Ewing’s sarcoma (ES) is the second most frequent primitive malignant bone tumor in adolescents with a very poor prognosis for high risk patients, mainly when lung metastases are detected (overall survival <15% at 5 years). Zoledronic acid (ZA) is a potent inhibitor of bone resorption which induces osteoclast apoptosis. Our previous studies showed a strong therapeutic potential of ZA as it inhibits ES cell growth in vitro and ES primary tumor growth in vivo in a mouse model developed in bone site. However, no data are available on lung metastasis. Therefore, the aim of this study was to determine the effect of ZA on ES cell invasion and metastatic properties. Methods Invasion assays were performed in vitro in Boyden’s chambers covered with Matrigel. Matrix Metalloproteinase (MMP) activity was analyzed by zymography in ES cell culture supernatant. In vivo, a relevant model of spontaneous lung metastases which disseminate from primary ES tumor was induced by the orthotopic injection of 106 human ES cells in the tibia medullar cavity of nude mice. The effect of ZA (50 ?g/kg, 3x/week) was studied over a 4-week period. Lung metastases were observed macroscopically at autopsy and analysed by histology. Results ZA induced a strong inhibition of ES cell invasion, probably due to down regulation of MMP-2 and ?9 activities as analyzed by zymography. In vivo, ZA inhibits the dissemination of spontaneous lung metastases from a primary ES tumor but had no effect on the growth of established lung metastases. Conclusion These results suggest that ZA could be used early in the treatment of ES to inhibit bone tumor growth but also to prevent the early metastatic events to the lungs. PMID:24612486

2014-01-01

97

Biomarkers in Ewing Sarcoma: The Promise and Challenge of Personalized Medicine. A Report from the Children's Oncology Group  

PubMed Central

A goal of the COG Ewing Sarcoma (ES) Biology Committee is enabling identification of reliable biomarkers that can predict treatment response and outcome through the use of prospectively collected tissues and correlative studies in concert with COG therapeutic studies. In this report, we aim to provide a concise review of the most well-characterized prognostic biomarkers in ES, and to provide recommendations concerning design and implementation of future biomarker studies. Of particular interest and potentially high clinical relevance are studies of cell-cycle proteins, sub-clinical disease, and copy number alterations. We discuss findings of particular interest from recent biomarker studies and examine factors important to the success of identifying and validating clinically relevant biomarkers in ES. A number of promising biomarkers have demonstrated prognostic significance in numerous retrospective studies and now need to be validated prospectively in larger cohorts of equivalently treated patients. The eventual goal of refining the discovery and use of clinically relevant biomarkers is the development of patient specific ES therapeutic modalities. PMID:23761859

Shukla, Neerav; Schiffman, Joshua D.; Reed, Damon; Davis, Ian J.; Womer, Richard B.; Lessnick, Stephen L.; Lawlor, Elizabeth R.

2013-01-01

98

Identification of target genes for the Ewing's sarcoma EWS/FLI fusion protein by representational difference analysis.  

PubMed Central

The EWS/FLI-1 fusion gene results from the 11;22 chromosomal translocation in Ewing's sarcoma. The product of the gene is one of a growing number of structurally altered transcription factors implicated in oncogenesis. We have employed a subtractive cloning strategy of representational difference analysis in conjunction with a model transformation system to identify genes transcribed in response to EWS/FLI. We have characterized eight transcripts that are dependent on EWS/FLI for expression and two transcripts that are repressed in response to EWS/FLI. Three of the former were identified by sequence analysis as stromelysin 1, a murine homolog of cytochrome P-450 F1 and cytokeratin 15. Stromelysin 1 is induced rapidly after expression of EWS/FLI, suggesting that the stromelysin 1 gene may be a direct target gene of EWS/FLI. These results demonstrate that expression of EWS/FLI leads to significant changes in the transcription of specific genes and that these effects are at least partially distinct from those caused by expression of germ line FLI-1. The representational difference analysis technique can potentially be applied to investigate transformation pathways activated by a broad array of genes in different tumor systems. PMID:7623854

Braun, B S; Frieden, R; Lessnick, S L; May, W A; Denny, C T

1995-01-01

99

Identification of a self-association domain in the Ewing's sarcoma protein: a novel function for arginine-glycine-glycine rich motifs?  

PubMed Central

The Ewing’s sarcoma (EWS) protein is a ubiquitously expressed RNA chaperone. The EWS protein localizes predominantly to the nucleus. Previous reports have suggested that the EWS protein is capable of dimerizing. However, to date this has not been confirmed. Here, using a novel panel of recombinant proteins, we have performed an in vitro biomolecular interaction analysis of the EWS protein. We have demonstrated that all three arginine-glycine-glycine (RGG) motifs are capable of binding directly to the survival motor neuron protein, a Tudor domain containing EWS binding partner. We have also confirmed EWS is capable of self-associating, and we have mapped this binding domain to the RGG motifs. We have also found that self-association may be required for EWS nuclear import. This is the first direct evidence of RGG domains being involved in self-association and has implications on all RGG-containing proteins. PMID:20211855

Shaw, Debra J.; Morse, Robert; Todd, Adrian G.; Eggleton, Paul; Lorson, Christian L.; Young, Philip J.

2010-01-01

100

Common Ewing sarcoma-associated antigens fail to induce natural T cell responses in both patients and healthy individuals.  

PubMed

Disseminated or relapsed Ewing sarcoma (EwS) has remained fatal in the majority of patients. A promising approach to preventing relapse after conventional therapy is to establish tumor antigen-specific immune control. Efficient and specific T cell memory against the tumor depends on the expansion of rare T cells with native specificity against target antigens overexpressed by the tumor. Candidate antigens in EwS include six-transmembrane epithelial antigen of the prostate-1 (STEAP1), and the human cancer/testis antigens X-antigen family member 1 (XAGE1) and preferentially expressed antigen in melanoma (PRAME). Here, we screened normal donors and EwS patients for the presence of circulating T cells reactive with overlapping peptide libraries of these antigens by IFN-? Elispot analysis. The majority of 22 healthy donors lacked detectable memory T cell responses against STEAP1, XAGE1 and PRAME. Moreover, ex vivo detection of T cells specific for these antigens in both blood and bone marrow were limited to a minority of EwS patients and required nonspecific T cell prestimulation. Cytotoxic T cells specific for the tumor-associated antigens were efficiently and reliably generated by in vitro priming using professional antigen-presenting cells and optimized cytokine stimulation; however, these T cells failed to interact with native antigen processed by target cells and with EwS cells expressing the antigen. We conclude that EwS-associated antigens fail to induce efficient T cell receptor (TCR)-mediated antitumor immune responses even under optimized conditions. Strategies based on TCR engineering could provide a more effective means to manipulating T cell immunity toward targeted elimination of tumor cells. PMID:24973179

Altvater, Bianca; Kailayangiri, Sareetha; Theimann, Nadine; Ahlmann, Martina; Farwick, Nicole; Chen, Christiane; Pscherer, Sibylle; Neumann, Ilka; Mrachatz, Gabriele; Hansmeier, Anna; Hardes, Jendrik; Gosheger, Georg; Juergens, Heribert; Rossig, Claudia

2014-10-01

101

Anti-VEGFR2 and anti-IGF-1R-Adnectins inhibit Ewing's sarcoma A673-xenograft growth and normalize tumor vascular architecture.  

PubMed

Increasing experimental evidence suggests that IGF-1 may modulate tumor angiogenesis via activation of the expression of VEGF in Ewing sarcomas and rhabdomyosarcomas. This study investigates the effects of the PEGylated Adnectins™ CT-322, a VEGFR2-inhibitor and AT580Peg40, an IGF-1R inhibitor, as monotherapy and in combination in a murine A673 xenograft tumor model. The combination of Adnectins CT-322 and AT580Peg40 revealed a 83% reduction in tumor growth, a nearly 5 times lower vessel density, less necrotic areas and less appearance of intussusceptive angiogenesis. Monotherapy with IGF-1R or CT-322 revealed equally a significant inhibition of tumor and vessel growth. Combinatory inhibition of IGF-1R and VEGFR2 shows a downregulation of IGF-binding protein 2 and a compensatory upregulation of VEGF levels. Immunohistological analysis showed remodeling vascular effects of CT-322-treatment or combination therapy. The vascular architecture in Adnectin-treated tumors was characterized by a strong normalization of vasculature. 3D-evaluation in microvascular corrosion casts showed significantly higher intervascular and interbranching distances in Adnectin-treated tumors. CT-322-treatment and combinatory inhibition reveal a significant reduction of intussusceptive angiogenesis. These pronounced effects on tumor vasculature suggest potential therapeutic benefit of combinatorial IGF1- and VEGF-pathways inhibition in Ewing's sarcoma. PMID:22914877

Ackermann, Maximilian; Morse, Brent A; Delventhal, Vera; Carvajal, Irvith M; Konerding, Moritz A

2012-12-01

102

Combined use of expression and CGH arrays pinpoints novel candidate genes in Ewing sarcoma family of tumors  

PubMed Central

Background Ewing sarcoma family of tumors (ESFT), characterized by t(11;22)(q24;q12), is one of the most common tumors of bone in children and young adults. In addition to EWS/FLI1 gene fusion, copy number changes are known to be significant for the underlying neoplastic development of ESFT and for patient outcome. Our genome-wide high-resolution analysis aspired to pinpoint genomic regions of highest interest and possible target genes in these areas. Methods Array comparative genomic hybridization (CGH) and expression arrays were used to screen for copy number alterations and expression changes in ESFT patient samples. A total of 31 ESFT samples were analyzed by aCGH and in 16 patients DNA and RNA level data, created by expression arrays, was integrated. Time of the follow-up of these patients was 5–192 months. Clinical outcome was statistically evaluated by Kaplan-Meier/Logrank methods and RT-PCR was applied on 42 patient samples to study the gene of the highest interest. Results Copy number changes were detected in 87% of the cases. The most recurrent copy number changes were gains at 1q, 2, 8, and 12, and losses at 9p and 16q. Cumulative event free survival (ESFT) and overall survival (OS) were significantly better (P < 0.05) for primary tumors with three or less copy number changes than for tumors with higher number of copy number aberrations. In three samples copy number imbalances were detected in chromosomes 11 and 22 affecting the FLI1 and EWSR1 loci, suggesting that an unbalanced t(11;22) and subsequent duplication of the derivative chromosome harboring fusion gene is a common event in ESFT. Further, amplifications on chromosomes 20 and 22 seen in one patient sample suggest a novel translocation type between EWSR1 and an unidentified fusion partner at 20q. In total 20 novel ESFT associated putative oncogenes and tumor suppressor genes were found in the integration analysis of array CGH and expression data. Quantitative RT-PCR to study the expression levels of the most interesting gene, HDGF, confirmed that its expression was higher than in control samples. However, no association between HDGF expression and patient survival was observed. Conclusion We conclude that array CGH and integration analysis proved to be effective methods to identify chromosome regions and novel target genes involved in the tumorigenesis of ESFT. PMID:19144156

2009-01-01

103

miR-375 Mediated Acquired Chemo-Resistance in Cervical Cancer by Facilitating EMT  

PubMed Central

Acquired chemo-resistance is one of the key causal factors in cancer death. Emerging evidences suggest that miRNA and epithelial–mesenchymal transition play critical roles in the chemo-resistance in cancers. Here, we showed the association of paclitaxel-resistance with miR-375 over-expression and epithelial–mesenchymal transition inducement in cervical cancer. Using different cervical cancer cell models, we found that paclitaxel transiently induced up-regulation of miR-375 expression, proliferation inhibition, transition from epithelial to mesenchymal phenotype, and consequently impaired paclitaxel sensitivity. Forced over-expression of miR-375 may suppress Ecadherin expression by a directly targeting pathway, which led to paclitaxel resistance. Contrarily, re-expression of Ecadherin partly reversed epithelial–mesenchymal transition phenotype and miR-375 induced paclitaxel-resistance. Our findings suggest that paclitaxel-induced miR-375 over-expression facilitates epithelial–mesenchymal transition process via directly targeting Ecadherin, proliferation inhibition, and consequently results in chemo-resistance in cervical cancer cells. A reversion of miR-375 or Ecadherin expression may be a novel therapeutic approach for overcoming chemo-resistance in cervical cancer. PMID:25330011

Shen, Yuanming; Zhou, Jiansong; Li, Yang; Ye, Feng; Wan, Xiaoyun; Lu, Weiguo; Xie, Xing; Cheng, Xiaodong

2014-01-01

104

A phase II study to determine the efficacy and safety of oral treosulfan in patients with advanced pre-treated Ewing sarcoma ISRCTN11631773.  

PubMed

We report a prospective Phase II study of efficacy and toxicity for oral treosulfan in advanced Ewing sarcoma. Twenty patients, median age 19 years (range 7-39) from five UK sites, were treated with oral treosulfan 1?g/m(2) daily for 7 days in 28. Primary endpoint was objective response rate. Best response was stable disease in one patient. All patients died of progressive disease, after median 6.41 months. Median progression free survival was 1.8 months. Toxicity was minimal. No activity was demonstrated for treosulfan at this dose. Progression free survival data should be able to be used for comparison when planning future clinical trials. Pediatr Blood Cancer 2015;62:158-159. © 2014 Wiley Periodicals, Inc. PMID:25284019

Michelagnoli, M; Whelan, J; Forsyth, S

2015-01-01

105

Fine-needle aspiration as a diagnostic technique in 50 cases of primary Ewing sarcoma/peripheral neuroectodermal tumor. Institut Curie's experience.  

PubMed

Fine-needle aspiration (FNA) followed by a core-needle biopsy during general anesthesia is a method for diagnosing pediatric tumors in our Institute. To complete the diagnosis in the case of round cell sarcomas, cytology material is also used for genomic analyses, that is, karyotyping and molecular biology-derived techniques. Fifty primary Ewing sarcomas/peripheral neuroectodermal tumors (ES/PNET) in 50 patients were sampled. Cytological diagnoses were "malignant" in all cases and accurate (ES/PNET) in 46 (92%). Two (4%) cases were misdiagnosed as neuroblastoma, and two others (4%) as rhabdomyosarcoma and nephroblastoma. No suspicious or false-negative results were rendered. Karyotyping was performed in 20 (40%) cases and was interpretable in 17 cases but not in three cases. Molecular search for ES/PNET fusion transcripts were performed in all cases and were detected in 44 (88%) cases, but not in six (12%) cases. In conclusion, FNA assisted by genomic techniques is powerful methods to accurate diagnose ES/PNET. PMID:22180234

Klijanienko, Jerzy; Couturier, Jérôme; Bourdeaut, Franck; Fréneaux, Paul; Ballet, Stelly; Brisse, Hervé; Lagacé, Réal; Delattre, Olivier; Pierron, Gaëlle; Vielh, Philippe; Sastre-Garau, Xavier; Michon, Jean

2012-01-01

106

Cytotoxic effect of the pentacyclic oxindole alkaloid mitraphylline isolated from Uncaria tomentosa bark on human Ewing's sarcoma and breast cancer cell lines.  

PubMed

Preparations from Uncaria tomentosa, a South American Rubiaceae, have been used in the Peruvian traditional medicine for the treatment of infective, inflammatory and tumoral processes. In this study, the pentacyclic oxindole alkaloid mitraphylline was isolated from the dried inner bark of this plant species, and its structure elucidated by analysis of NMR spectroscopic data. Mitraphylline was differentially identified from its stereoisomeric pair isomitraphylline by (15)N-NMR. Its antiproliferative and cytotoxic effects have been tested on human Ewing's sarcoma MHH-ES-1 and breast cancer MT-3 cell lines, using cyclophosphamide and vincristine as reference controls. A Coulter counter was used to determine viable cell numbers, followed by the application of the tetrazolium compound MTS [3-(4,5-dimethylthiazol-2-yl)-5-(3-carboxymethoxy phenyl)-2-(4-sulfophenyl)-2H-tetrazolium] an inner salt. A colorimetric method was employed to evaluate cell viability in this cytotoxic assay. Micromolar concentrations of mitraphylline (5 microM to 40 microM) inhibited the growth of both cell lines in a dose-dependent manner. The IC (50) +/- SE values were 17.15 +/- 0.82 microM for MHH-ES-1 and 11.80 +/- 1.03 microM for MT-3 for 30 hours, smaller than those obtained for the reference compounds. This action suggests that the pentacyclic oxindole alkaloid mitraphylline might be a new promising agent in the treatment of both human sarcoma and breast cancer. PMID:19724995

García Giménez, Dolores; García Prado, Elena; Sáenz Rodríguez, Teresa; Fernández Arche, Angeles; De la Puerta, Rocío

2010-02-01

107

Targeted therapy for sarcomas  

PubMed Central

Sarcomas are tumors of mesenchymal origin that make up approximately 1% of human cancers. They may arise as primary tumors in either bone or soft tissue, with approximately 11,280 soft tissue tumors and 2,650 bone tumors diagnosed each year in the United States. There are at least 50 different subtypes of soft tissue sarcoma, with new ones described with ever-increasing frequency. One way to look at sarcomas is to divide them into categories on the basis of their genetic make-up. One group of sarcomas has an identifiable, relatively simple genetic signature, such as the X:18 translocation seen in synovial sarcoma or the 11:22 translocation seen in Ewing’s sarcoma. These specific abnormalities often lead to the presence of fusion proteins, such as EWS-FLI1 in Ewing’s sarcoma, which are helpful as diagnostic tools and may become therapeutic targets in the future. Another group of sarcomas is characterized by complex genetic abnormalities as seen in leiomyosarcoma, osteosarcoma, and undifferentiated sarcoma. It is important to keep these distinctions in mind when contemplating the development of targeted agents for sarcomas. Different abnormalities in sarcoma could be divided by tumor subtype or by the molecular or pathway abnormality. However, some existing drugs or drugs in development may interfere with or alter more than one of the presented pathways. PMID:24669185

Forscher, Charles; Mita, Monica; Figlin, Robert

2014-01-01

108

Expression of MYCN in pediatric synovial sarcoma  

Microsoft Academic Search

Synovial sarcoma accounts for between 6 and 10% of childhood sarcomas and histological diagnosis can be challenging, even for experienced pathologists. Several other tumors enter the differential diagnosis, including malignant peripheral nerve sheath tumor, Ewing sarcoma\\/primitive neuroectodermal tumor and undifferentiated sarcoma. Several recent reports utilizing expression array techniques have documented expression of the MYCN oncogene in synovial sarcoma. In order

Gino R Somers; Maria Zielenska; Shaker Abdullah; Christopher Sherman; Suzanne Chan; Paul S Thorner

2007-01-01

109

Suppression of FOXO1 is responsible for a growth regulatory repressive transcriptional sub-signature of EWS-FLI1 in Ewing sarcoma  

PubMed Central

The Ewing sarcoma (ES) EWS-FLI1 chimeric oncoprotein is a prototypic aberrant ETS transcription factor with activating and repressive regulatory functions. We report that EWS-FLI1-repressed promoters are enriched in forkhead box (FOX) recognition motifs, and identify FOXO1 as a EWS-FLI1-suppressed regulator orchestrating a major subset of EWS-FLI1-repressed genes. In addition to FOXO1 regulation by direct promoter binding of EWS-FLI1, its subcellular localization and activity is regulated by cyclin-dependent kinase 2- and AKT-mediated phosphorylation downstream of EWS-FLI1. Restoration of nuclear FOXO1 expression in ES cells impaired proliferation and significantly reduced clonogenicity. Gene-expression profiling revealed a significant overlap between EWS-FLI1-repressed and FOXO1-activated genes. As a proof of principle for a potential therapeutic application of our findings, the treatment of ES cell lines with methylseleninic acid (MSA) reactivated endogenous FOXO1 in the presence of EWS-FLI1 in a dose- and time-dependent manner and induced massive cell death dependent on FOXO1. In an orthotopic xenograft mouse model, MSA increased FOXO1 expression in the tumor paralleled by a significant decrease in ES tumor growth. FOXO1 reactivation by small molecules may therefore serve as a promising strategy for a future ES-specific therapy. PMID:23995784

Niedan, S; Kauer, M; Aryee, D N T; Kofler, R; Schwentner, R; Meier, A; Potschger, U; Kontny, U; Kovar, H

2014-01-01

110

DNA-binding and transcriptional activation properties of the EWS-FLI-1 fusion protein resulting from the t(11;22) translocation in Ewing sarcoma.  

PubMed Central

The 5' half of the EWS gene has recently been described to be fused to the 3' regions of genes encoding the DNA-binding domain of several transcriptional regulators, including ATF1, FLI-1, and ERG, in several human tumors. The most frequent occurrence of this situation results from the t(11;22)(q24;q12) chromosome translocation specific for Ewing sarcoma (ES) and related tumors which joins EWS sequences to the 3' half of FLI-1, which encodes a member of the Ets family of transcriptional regulators. We show here that this chimeric gene encodes an EWS-FLI-1 nuclear protein which binds DNA with the same sequence specificity as the wild-type parental FLI-1 protein. We further show that EWS-FLI-1 is an efficient sequence-specific transcriptional activator of model promoters containing FLI-1 (Ets)-binding sites, a property which is strictly dependent on the presence of its EWS domain. Comparison of the properties of the N-terminal activation domain of FLI-1 to those of the EWS domain of the fusion protein indicates that EWS-FLI-1 has altered transcriptional activation properties compared with FLI-1. These results suggest that EWS-FLI-1 contributes to the transformed phenotype of ES tumor cells by inducing the deregulated and/or unscheduled activation of genes normally responsive to FLI-1 or to other close members of the Ets family. ES and related tumors are characterized by an elevated level of c-myc expression. We show that EWS-FLI-1 is a transactivator of the c-myc promoter, suggesting that upregulation of c-myc expression is under control of EWS-FLI-1. Images PMID:8164678

Bailly, R A; Bosselut, R; Zucman, J; Cormier, F; Delattre, O; Roussel, M; Thomas, G; Ghysdael, J

1994-01-01

111

Prognostic factors in patients with localized Ewing's sarcoma: the effect on survival of actual received drug dose intensity and of histologic response to induction therapy.  

PubMed

To bring to the fore the most important prognostic factors in Ewing's sarcoma (ES) with current protocols, we studied the classical prognostic factors, dose intensity (DI) of actual received drugs, age and histological response to induction therapy and their correlation in 39 patients with localized ES treated from 11/85 to 06/95 to identify eventual predictors of event-free survival (EFS). Inclusion criteria were age 35 yr or less, definitive local treatment by our team and chemotherapy including at least 4 drugs: vincristine (VCR), dactinomycin (DACT), doxorubicin (DOXO) cyclophosphamide (CPX). The endpoint was the absence of relapse. Parameters related to the status of patients were tested using the Chi square test or Fisher's exact test. The non parametric Kruskal-Wallis test was used for quantitative data. When necessary stratified analysis was done using the Mantel Cox test. With a median follow-up of 7 yr, overall survival (OS) and EFS were both 67% at 7 yr. According to univariate analysis, the significant predictors of survival were the DI of VCR and DACT, the histological response to preoperative chemotherapy (CT), the patient's age (< 18 yr DFS: 84%; > 18 yr DFS: 38%). The risk of metastases was almost tenfold higher in patients with low received DI of VCR (DFS 40% versus 95%) and of DACT (DFS 48% versus 94%). The prognostic value of primary tumor characteristics (tumoral volume or location) was erased by the comprehensive treatment. Following multivariate analysis, the actual received DI of VCR (p < 0.02) and DACT (p < 0.03) and the histological response to preoperative CT (p < 0.05) were retained as the only significant independent predictors of EFS. Taking into account the actual received DI of VCR and DACT, the prognostic value of age disappears. In conclusion, this study points out the main role of the drug DI in ES (particularly VCR and DACT) and of histological response to preoperative CT. PMID:9373791

Delepine, N; Delepine, G; Cornille, H; Voisin, M C; Brun, B; Desbois, J C

1997-10-01

112

Role of radiation therapy in the multidisciplinary management of Ewing's Sarcoma of bone in pediatric patients: An effective treatment for local control  

PubMed Central

Background Radiotherapy (RT) plays an important role in the multidisciplinary management of Ewing's Sarcoma (ES), especially in unresectable cases. Aim Assessment of efficacy of RT in terms of local control in pediatric patients with primary ES of bone. Materials and methods Thirty-six patients younger than 17 years old with ES treated with combined RT and chemotherapy with (N = 14) or without (N = 22) prior surgery from 1981 to 2008 were retrospectively reviewed. Since 1995, they were all treated according to the Spanish Society of Pediatric Oncology protocol (55.5% cases). Those patients received vincristine, ifosfamide, doxorubicin and etoposide. The TNM classification was as follows: 17 T1, 18 T2 and 1 T3; 36 N0; 29 M0, 5 M1a and 2 M1b. Analysis was stratified by treatment: definitive RT or pre/postoperative RT. Results The 36 patients (21 male; 15 female) had a median age of 10 years (range 2–17 years). Median follow-up of living patients was 105 months. The 2-year local control (LC) rate for all patients was 88%. Five-year LC rates for patients treated with definitive and pre/postoperative RT were 91% and 86%, respectively. Two-year overall survival and disease-free survival rates for all patients were 68% and 66%, respectively. Low phosphatase alkaline levels and local and distant recurrences were significantly predictive of worse prognosis (P = 0.021, P = 0.011, P = 0.007, respectively). Conclusion Radiotherapy with and without surgery is a highly effective local treatment option in the multidisciplinary management of ES in pediatric patients. PMID:24376965

Lopez, Jose Luis; Cabrera, Patricia; Ordonez, Rafael; Marquez, Catalina; Ramirez, Gema Lucia; Praena-Fernandez, Juan Manuel; Ortiz, Maria Jose

2011-01-01

113

Chemo-resistant choriocarcinoma metastatic to colon cured by low-anterior resection  

PubMed Central

The role of surgery in the treatment of patients with metastatic choriocarcinoma has diminished. We present a case of chemo-resistant metastatic choriocarcinoma salvaged by surgery. A 48-year-old patient presented with uterine perforation and severe intractable hemorrhage, and histological examination revealed a choriocarcinoma. After 6 years of disease-free state, recurrence occurred in the rectosigmoid colon. Seven cycles of EMACO chemotherapy was administered, and the human chorionic gonadotropin level was normalized. Three months after the chemotherapy, the rectosigmoid colon metastasis progressed. Low anterior resection with lymphadenectomy up to the level of the inferior mesenteric artery was conducted. After the operation, the human chorionic gonadotropin level decreased to within the normal range. There has been no evidence of disease for 13 months since the operation. Local resection of metastases seems to play a significant role in curing the disease in a small subset of patients. PMID:21998764

Ryu, Ju Hyun; Choi, Chel Hun; Kim, Tae-Joong; Lee, Jeong-Won; Kim, Byoung-Gie

2011-01-01

114

Central Nervous System Involvement in Children with Sarcoma  

Microsoft Academic Search

Objectives: To summarize and analyze the experience in CNS involvement (CNSI) in children with sarcomas treated in the above-mentioned institutions. Patients and Methods: From 1990 to 2001, all medical charts were retrospectively reviewed: 19 sarcoma patients (12 boys and 7 girls) were diagnosed with CNSI (4 osteogenic sarcomas, 11 Ewing sarcomas, 2 rhabdomyosarcomas, 1 alveolar soft part sarcoma and 1

S. Postovsky; S. Ash; I. N. Ramu; Y. Yaniv; R. Zaizov; B. Futerman; R. Elhasid; A. Ben Barak; A. Halil; M. W. Ben Arush

2003-01-01

115

Formation and Rupture of the Internal Carotid Artery Aneurysm after Multiple Courses of Intensity-Modulated Radiation Therapy for Management of the Skull Base Ewing Sarcoma/PNET: Case Report  

PubMed Central

Background?Aneurysm formation after stereotactic irradiation of skull base tumors is rare. The formation and rupture of an internal carotid artery (ICA) aneurysm in a patient with skull base Ewing sarcoma/primitive neuroectodermal tumor (PNET), who underwent surgery followed by multiple courses of intensity-modulated radiation therapy (IMRT) and chemotherapy, is described. Case Description?A 25-year-old man presented with a sinonasal tumor with intraorbital and intracranial growth. At that time cerebral angiography did not reveal any vascular abnormalities. The lesion was resected subtotally. Histopathologic diagnosis was Ewing sarcoma/PNET. The patient underwent multiple courses of chemotherapy and three courses of IMRT at 3, 28, and 42 months after initial surgery. The total biologically effective dose delivered to the right ICA was 220.2 Gy. Seven months after the third IMRT, the patient experienced profound nasal bleeding that resulted in hypovolemic shock. Angiography revealed a ruptured right C4–C5 aneurysm and irregular stenotic changes of the ICA. Lifesaving endovascular trapping of the right ICA was done. The patient recovered well after surgery but died due to tumor recurrence 6 months later. Conclusion?Excessive irradiation of the ICA may occasionally result in aneurysm formation, which should be borne in mind during stereotactic irradiation of malignant skull base tumors. PMID:24303346

Tamura, Manabu; Kogo, Kasei; Masuo, Osamu; Oura, Yoshinori; Matsumoto, Hiroyuki; Fujita, Koji; Nakao, Naoyuki; Uematsu, Yuji; Itakura, Toru; Chernov, Mikhail; Hayashi, Motohiro; Muragaki, Yoshihiro; Iseki, Hiroshi

2013-01-01

116

The combination of CD99 and NKX2.2, a transcriptional target of EWSR1-FLI1, is highly specific for the diagnosis of Ewing sarcoma.  

PubMed

Ewing sarcoma (ES) is a high-grade malignant neoplasm primarily affecting children and young adults. The diagnosis of ES is often difficult because of its broad differential diagnosis comprising a diverse group of small round cell tumors (SRCTs). Although the identification of tumor type-specific fusion genes by molecular testing is the gold standard for the diagnosis of ES, such approaches are not always available in a routine pathology practice. Thus, a reliable immunohistochemical marker is required. A recent study using a limited number of tumor samples has shown that NKX2.2, a putative transcriptional target of EWSR1-FLI1, is a useful marker for the diagnosis of ES. In the present study, the immunohistochemical expression of NKX2.2 was evaluated on 46 genetically confirmed ES and 85 non-ES SRCTs, together with comparative assessment of CD99 and other molecular targets of EWSR1-FLI1, including NR0B1, E2F3, and EZH2. NKX2.2 was expressed in 37 (80 %) of the ES samples with a mostly diffuse and strong staining pattern, and 14 (16 %) of the non-ES SRCTs, including olfactory neuroblastomas, extraskeletal myxoid chondrosarcoma, mesenchymal chondrosarcoma, small cell carcinomas, and Merkel cell carcinoma, also expressed this marker. The sensitivity and specificity of the NKX2.2 expression in this cohort were 80 and 84 %, respectively. The specificity when combined with CD99 was 98 %, with exceptional expression of both markers in only two non-ES SRCTs, including one case each of mesenchymal chondrosarcoma and small cell carcinoma. NR0B1, E2F3, and EZH2 were less sensitive for specific markers for ES when applied singly or in any combination. In conclusion, the study reinforces that NKX2.2 is a useful immunohistochemical marker for ES, and that the combination of CD99 and NKX2.2 is a powerful diagnostic tool that can differentiate ES from other SRCTs. PMID:25031013

Shibuya, Ryo; Matsuyama, Atsuji; Nakamoto, Mitsuhiro; Shiba, Eisuke; Kasai, Takahiko; Hisaoka, Masanori

2014-11-01

117

General Information about Ewing Sarcoma  

MedlinePLUS

... and given back to the patient through an infusion . These reinfused stem cells grow into (and restore) ... them from spreading. Monoclonal antibodies are given by infusion. They may be used alone or to carry ...

118

Insulin-Like Growth Factor 1 Receptor as a Therapeutic Target in Ewing Sarcoma: Lack of Consistent Upregulation or Recurrent Mutation and a Review of the Clinical Trial Literature  

PubMed Central

The insulin-like growth factor 1 receptor (IGF-1R) has been considered an important therapeutic target in Ewing sarcoma (ES), generating a need to identify the subset of patients most likely to respond to IGF-1R inhibitors. We assessed IGF-1R expression in ES cell lines and patient tumors to understand the variable clinical responses to anti-IGF-1R therapy. Using ligand-binding displacement, we measured between 13,000 and 40,000 receptors per cell in ES cell lines. We used ELISA to quantify IGF-1R in patient tumors, which expressed 4.8%??± 3.7 to 20.0%??± 0.2 of the levels in a positive control cell line overexpressing IGF-1R. Flow cytometry showed markedly reduced IGF-1R expression in ES cell lines compared to a standard positive control cell line. The IGF1R gene was sequenced in 47 ES tumor samples and 8 ES cell lines; only one tumor sample showed a nonsynonymous mutation, R1353H, in a region with low functional impact. Finally, we assessed IGF-1R pathway activity in the ES stem cell (ESSC) population, to characterize its potential for resistance to anti-IGF-1R therapy, using Luminex technology. We found no significant differences in IGF-1R pathway activity between ESSCs and the total cell population. Overall, our findings suggest that IGF-1R as a therapeutic target in this sarcoma may require reevaluation. PMID:23431249

O'Neill, Alison; Shah, Nilay; Zitomersky, Naamah; Ladanyi, Marc; Shukla, Neerav; Uren, Aykut; Loeb, David; Toretsky, Jeffrey

2013-01-01

119

Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma  

ClinicalTrials.gov

Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Conjunctival Kaposi Sarcoma; Dermatofibrosarcoma Protuberans; Gastrointestinal Stromal Tumor; Metastatic Adult Malignant Fibrous Histiocytoma of Bone; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Malignant Fibrous Histiocytoma of Bone; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Kaposi Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Sarcoma; Small Intestine Leiomyosarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma

2014-10-09

120

74. Specific Delivery of hTRAIL and Inhibition of Ewing's Sarcoma Growth by Transplantation of Viral Vector-Transduced Bone Marrow-Derived Mesenchymal Stem Cells (MSC)  

Microsoft Academic Search

For the majority of pediatric patients with soft tissue tumors, some form of selective systemic therapy is required. Here we show that bone marrow-derived non-hematopoietic mesenchymal stem cells (MSCs) integrate into pediatrics soft tissue sarcoma xenografts as stromal fibroblasts. Importantly, exogenously administered MSC preferentially engraft at the sites of disseminated tumor nodules throughout the body. These findings suggest the development

Jeffrey S. Bartlett; Wenfang Shi; Carmel Lawrencia

2005-01-01

121

The Epidemiology of Sarcoma  

PubMed Central

Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, and an interaction between the two. HIV-positive individuals are at an increased risk for Kaposi’s sarcoma, even though HHV8 is the causative virus. Radiation exposure from radiotherapy has been strongly associated with secondary sarcoma development in certain cancer patients. In fact, the risk of malignant bone tumors increases as the cumulative dose of radiation to the bone increases (p for trend <0.001). A recent meta-analysis reported that children with a history of hernias have a greater risk of developing Ewing’s sarcoma (adjusted OR 3.2, 95% CI 1.9, 5.7). Bone development during pubertal growth spurts has been associated with osteosarcoma development. Occupational factors such as job type, industry, and exposures to chemicals such as herbicides and chlorophenols have been suggested as risk factors for sarcomas. A case-control study found a significant increase in soft tissue sarcoma risk among gardeners (adjusted OR 4.1, 95% CI 1.00, 14.00), but not among those strictly involved in farming. A European-based study reported an increased risk in bone tumors among blacksmiths, toolmakers, or machine-tool operators (adjusted OR 2.14, 95% CI 1.08, 4.26). Maternal and paternal characteristics such as occupation, age, smoking status, and health conditions experienced during pregnancy also have been suggested as sarcoma risk factors and would be important to assess in future studies. The limited studies we identified demonstrate significant relationships with sarcoma risk, but many of these results now require further validation on larger populations. Furthermore, little is known about the biologic mechanisms behind each epidemiologic association assessed in the literature. Future molecular epidemiology studies may increase our understanding of the genetic versus environmental contributions to tumorigenesis in this often deadly cancer in children and adults. PMID:23036164

2012-01-01

122

Basic fibroblast growth factor in the bone microenvironment enhances cell motility and invasion of Ewing's sarcoma family of tumours by activating the FGFR1–PI3K–Rac1 pathway  

PubMed Central

Background: Ewing's sarcoma family of tumours (ESFT) is a malignant small round-cell tumour of the bone and soft tissues. It is characterised by a strong tendency to invade and form metastases. The microenvironment of the bone marrow is a large repository for many growth factors, including the basic fibroblast growth factor (bFGF). However, the role of bFGF in the invasive and metastatic phenotype of ESFT has not been investigated. Methods: The motility and invasion of ESFT cells were assessed by a wound-healing assay, chemotaxis assay, and invasion assay. The expression and activation of FGF receptors (FGFRs) in ESFT cell lines and clinical samples were detected by RT–PCR, western blotting, and immunohistochemistry. The morphology of ESFT cells was investigated by phase-contrast microscopy and fluorescence staining for actin. Activation of Rac1 was analysed by a pull-down assay. Results: bFGF strongly induced the motility and invasion of ESFT cells. Furthermore, FGFR1 was found to be expressed and activated in clinical samples of ESFT. Basic FGF-induced cell motility was mediated through the FGFR1–phosphatidylinositol 3-kinase (PI3K)–Rac1 pathway. Conditioned medium from bone marrow stromal cells induced the motility of ESFT cells by activating bFGF/FGFR1 signalling. Conclusion: The bFGF–FGFR1–PI3K–Rac1 pathway in the bone microenvironment may have a significant role in the invasion and metastasis of ESFT. PMID:20606682

Kamura, S; Matsumoto, Y; Fukushi, J-i; Fujiwara, T; Iida, K; Okada, Y; Iwamoto, Y

2010-01-01

123

The role of testicular nuclear receptor 4 in chemo-resistance of docetaxel in castration-resistant prostate cancer.  

PubMed

Docetaxel-based therapy is one of the first-line options for castration-resistant prostate cancer (CRPC). However, a large proportion of CRPC patients show different extents of docetaxel resistance. The current study aims to investigate the role of testicular nuclear receptor 4 (TR4) in docetaxel resistance in CRPC. TR4 expression level in prostate biopsy samples from CRPC patients treated with docetaxel was measured by immunohistochemistry (IHC). Alternation of TR4 expression in prostate cancer (PCa) cell line PC3 was applied to find out the influence of TR4 on half-maximal inhibitory concentration (IC50), cell viability and cell apoptosis. Patients who failed to achieve prostate-specific antigen (PSA) response (<50% PSA reduction from baseline) after docetaxel-based chemotherapy had a comparatively higher TR4 expression than those who achieved PSA response (?50% PSA reduction from baseline). Knocking down TR4 in PC3 cells led to a lower IC50 dose, poorer cell viability and more cell apoptosis when treated with docetaxel, whereas overexpression of TR4 in PC3 led to a higher IC50 dose, better cell viability and less cell apoptosis. TR4 enhances the chemo-resistance of docetaxel in CRPC. It may serve as a biomarker to determine the prognosis of docetaxel-based therapy and as a potential therapy target to combine with docetaxel to better suppress CRPC. PMID:25104727

Chen, B; Yu, S; Ding, X; Jing, C; Xia, L; Wang, M; Matro, E; Rehman, F; Niu, Y; Li, G; Chang, C

2014-10-01

124

Mir-34a Mimics Are Potential Therapeutic Agents for p53-Mutated and Chemo-Resistant Brain Tumour Cells  

PubMed Central

Chemotherapeutic drug resistance and relapse remains a major challenge for paediatric (medulloblastoma) and adult (glioblastoma) brain tumour treatment. Medulloblastoma tumours and cell lines with mutations in the p53 signalling pathway have been shown to be specifically insensitive to DNA damaging agents. The aim of this study was to investigate the potential of triggering cell death in p53 mutated medulloblastoma cells by a direct activation of pro-death signalling downstream of p53 activation. Since non-coding microRNAs (miRNAs) have the ability to fine tune the expression of a variety of target genes, orchestrating multiple downstream effects, we hypothesised that triggering the expression of a p53 target miRNA could induce cell death in chemo-resistant cells. Treatment with etoposide, increased miR-34a levels in a p53-dependent fashion and the level of miR-34a transcription was correlated with the cell sensitivity to etoposide. miR-34a activity was validated by measuring the expression levels of one of its well described target: the NADH dependent sirtuin1 (SIRT1). Whilst drugs directly targeting SIRT1, were potent to trigger cell death at high concentrations only, introduction of synthetic miR-34a mimics was able to induce cell death in p53 mutated medulloblastoma and glioblastoma cell lines. Our results show that the need of a functional p53 signaling pathway can be bypassed by direct activation of miR-34a in brain tumour cells. PMID:25250818

Fan, Yuen Ngan; Meley, Daniel; Pizer, Barry; See, Violaine

2014-01-01

125

Cancer Stem Cells in Pediatric Sarcomas  

PubMed Central

Sarcomas represent a clinically and biologically diverse group of malignant connective tissue tumors. Despite aggressive conventional therapy, a large proportion of sarcoma patients experience disease recurrence which will ultimately result in mortality. The presence of a unique population of cells, referred to as cancer stem cells (CSCs), have been proposed to be responsible for refractory responses to current chemotherapies as well underlying the basis for metastasis and relapse of disease – clinical corollaries to what has been termed the CSC hypothesis. The presence of CSCs have been suggested in a variety of hematologic and solid malignancies, and only more recently in sarcomas. Based on our current understanding of normal stem cell biology and evidence obtained from the study of malignant hematopoietic and solid tumors, researchers have identified candidate cell surface markers (CD133, CD117, Stro-1), biochemical markers (aldehyde dehydrogenase activity), and cytological characteristics (side population and spherical colony formation) that may identify putative sarcoma CSCs. In this review, we explore the current state of evidence that may suggest the existence of sarcoma CSCs. We present research in osteosarcoma, the Ewing’s sarcoma family of tumors, rhabdomyosarcoma, as well as other sarcoma subtypes to describe commonly used molecular and biochemical markers, as well as techniques, used in the identification, isolation, and characterization of candidate sarcoma CSCs. We will also discuss the current controversies and challenges that face research in sarcoma CSC. PMID:23819111

Dela Cruz, Filemon S.

2013-01-01

126

Sarcoma Subtypes  

MedlinePLUS

... Leiomyosarcoma Uterine Leiomyosarcoma Liposarcoma Malignant Fibro Histiocytoma (MFH) Malignant Peripheral Nerve Sheath Tumor (MPNST) Osteosarcoma Rhabdomyosarcoma Synovial Sarcoma Sarcoma Foundation of ...

127

Uncommon sarcomas of the uterine cervix: a review of selected entities  

PubMed Central

Sarcomas constitute less than 1% of all cervical malignancies. With over 150 reported cases, rhabdomyosarcomas represent the most commonly reported sarcoma at this location. In this report, a select group of the more uncommon sarcomas of the uterine cervix are reviewed, including all previously reported examples of leiomyosarcoma, liposarcoma, alveolar soft part sarcoma, Ewing sarcoma/primitive neuroectodermal tumor, undifferentiated endocervical sarcoma, and malignant peripheral nerve sheath tumor (MPNST). Emphasis is placed on any distinctive clinicopathologic features of these entities at this unusual location. PMID:16981999

Fadare, Oluwole

2006-01-01

128

Rare Cause of Stricture Esophagus-Sarcoma: A Case Report and Review of the literature.  

PubMed

Adenocarcinoma and squamous cell carcinoma account for the vast majority of oesophageal malignancies. Other malignancies known to occur in the oesophagus include melanoma, sarcoma, and lymphoma. Among the sarcomas, carcinosarcoma is the commonest with both carcinomatous and sarcomatous elements followed by leiomyosarcoma of mesenchymal origin. Other sarcomas reported in the literature are liposarcoma, synovial sarcoma, myxofibrosarcoma, Ewing's sarcoma, granulocytic sarcoma, histiocytic sarcoma, schwannoma rhabdomyosarcoma, and epithelioid sarcoma. We report a case of malignant spindle cell tumour of oesophagus. Sarcomas of esophagus present as a polypoid exophytic soft tissue mass. Our patient presented with a stricture which is a rare presentation. Locally aggressive treatment with surgery is beneficial, and local palliative treatment including radiotherapy is worthwhile. PMID:22606414

Patricia, S; Saikat, Das; Rajesh, B; Rajesh, I; Selvamani, B; Subhashini, John

2011-01-01

129

Retroperitoneal Sarcomas  

Microsoft Academic Search

\\u000a Retroperitoneal sarcomas are rare, accounting for approximately 10–15% of all soft-tissue sarcomas with an estimated 1,500\\u000a cases occurring annually in USA [1, 2]. The most common histologic subtypes include liposarcoma, leiomyosarcoma, and malignant\\u000a fibrohistiocytoma [3]. Approximately 33–50% of retroperitoneal sarcomas are low grade, in contrast to 19–26% of extremity\\u000a and truncal sarcomas [4, 5], possibly explaining the lower incidence or

Brian Czito; John Donohue; Christopher G. Willett; Douglas Tyler; Ivy A. Petersen; Robert Krempien; Kenneth S. Hu; Felipe A. Calvo; Matthew D. Callister; Kaled M. Alektiar; Michael Eble; Ana Alvarez

130

Type I interferon and TNF? cooperate with type II interferon for TRAIL induction and triggering of apoptosis in SK-N-MC EWING tumor cells  

Microsoft Academic Search

Ewing's sarcoma is the second most common human bone tumor in childhood. Here, we investigated the sensitivity of the Ewing tumor cell line, SK-N-MC, to the apoptotic effect of type I (IFN?) and type II (IFN?) interferons and TNF?. We demonstrate that although IFN? and TNF? alone are unable to induce cell death, they act in synergy with IFN? to

Annie Abadie; Françoise Besançon; Juana Wietzerbin; J Wietzerbin

2004-01-01

131

Bone Sarcomas: From Biology to Targeted Therapies  

PubMed Central

Primary malignant bone tumours, osteosarcomas, and Ewing sarcomas are rare diseases which occur mainly in adolescents and young adults. With the current therapies, some patients remain very difficult to treat, such as tumour with poor histological response to preoperative CT (or large initial tumour volume for Ewing sarcomas not operated), patients with multiple metastases at or those who relapsed. In order to develop new therapies against these rare tumours, we need to unveil the key driving factors and molecular abnormalities behind the malignant characteristics and to broaden our understanding of the phenomena sustaining the metastatic phenotype and treatment resistance in these tumours. In this paper, starting with the biology of these tumours, we will discuss potential therapeutic targets aimed at increasing local tumour control, limiting metastatic spread, and finally improving patient survival. PMID:23226965

Gaspar, Nathalie; Di Giannatale, Angela; Geoerger, Birgit; Redini, Francoise; Corradini, Nadege; Enz-Werle, Natacha; Tirode, Franck; Marec-Berard, Perrine; Gentet, Jean-Claude; Laurence, Valerie; Piperno-Neumann, Sophie; Oberlin, Odile; Brugieres, Laurence

2012-01-01

132

ERG expression in epithelioid sarcoma: a diagnostic pitfall.  

PubMed

ERG transcription factor is constitutively expressed in endothelial cells. Because benign and malignant vascular endothelia retain the ERG expression, ERG is considered a useful marker for angiosarcomas and related tumors. ERG is also expressed in a subset of prostate carcinomas and Ewing sarcomas due to ERG-involved translocations; therefore, this marker is also of high interest in the study of these malignancies. In this study, we evaluated 109 epithelioid sarcomas for ERG expression, on the basis of an initial observation of an ERG-positive case. We also studied expression of other endothelial antigens in epithelioid sarcoma. ERG was expressed in 38% of epithelioid sarcomas (41/109), usually with a uniform nuclear staining, similar to that seen in angiosarcomas. However, all epithelioid sarcomas were negative for ERG gene rearrangement indicating that ERG expression is not likely related to ERG-involving translocations in epithelioid sarcoma. Other endothelial markers, CD31, claudin 5, and Prox1, were absent in epithelioid sarcomas. The only exception was a pulmonary metastasis of epithelioid sarcoma showing focal CD31 expression, which probably resulted from antigen adsorption onto tumor cell surfaces. However, podoplanin was commonly (7/9) expressed in epithelioid sarcoma; therefore, this marker is not useful in distinguishing epithelioid sarcoma from angiosarcoma. INI1/SMARCB1 gene product was absent in all epithelioid sarcomas (considered here a definitional feature) but was absent from only 1 epithelioid angiosarcoma, indicating its relative specificity for epithelioid sarcoma in this differential diagnostic setting. ERG expression is fairly common in epithelioid sarcoma and should be recognized as a diagnostic pitfall in the differential diagnosis of epithelioid sarcoma and epithelioid angiosarcoma. General lack of endothelial cell-specific markers in epithelioid sarcoma helps in this distinction. PMID:23774169

Miettinen, Markku; Wang, Zengfeng; Sarlomo-Rikala, Maarit; Abdullaev, Ziedulla; Pack, Svetlana D; Fetsch, John F

2013-10-01

133

ERG EXPRESSION IN EPITHELIOID SARCOMA - A DIAGNOSTIC PITFALL  

PubMed Central

ERG transcription factor is constitutively expressed in endothelial cells. Because benign and malignant vascular endothelia retain the ERG-expression, ERG is considered a useful marker for angiosarcomas and related tumors. ERG is also expressed in a subset of prostate carcinomas and Ewing sarcomas due to ERG-involving translocations, so that this marker is also of high interest for the study of these malignancies. In this study, we evaluated 109 epithelioid sarcomas for ERG expression, based on an initial observation of an ERG-positive case. We also studied expression of other endothelial antigens in epithelioid sarcoma. ERG was expressed in 38% of epithelioid sarcomas (41/109), usually with a uniform nuclear staining, similar to that seen in angiosarcomas. However, all epithelioid sarcomas were negative for ERG gene rearrangement indicating that that ERG expression is not likely related to ERG involving translocations in epithelioid sarcoma. Other endothelial markers, CD31, claudin 5, and Prox1 were absent in epithelioid sarcomas. The only exception was a pulmonary metastasis of epithelioid sarcoma showing focal CD31 expression, which was probably resulting from antigen adsorption onto tumor cell surfaces. However, podoplanin was commonly (7/9) expressed in epithelioid sarcoma, so that this marker is not useful in the distinction of epithelioid sarcoma and angiosarcoma. INI1/SMARCB1 gene product was absent in all epithelioid sarcomas (considered here a definitional feature) but was absent from only one epithelioid angiosarcoma, indicating its relative specificity for epithelioid sarcoma in this differential diagnostic setting. ERG expression is fairly common in epithelioid sarcoma, and should be recognized as a diagnostic pitfall in the differential diagnosis of epithelioid sarcoma and epithelioid angiosarcoma. General lack of endothelial cell specific markers in epithelioid sarcoma helps in this distinction. PMID:23774169

Miettinen, Markku; Wang, Zengfeng; Sarlomo-Rikala, Maarit; Abdullaev, Ziedulla; Pack, Svetlana D.; Fetsch, John F.

2013-01-01

134

Combination of 5-fluorouracil and genistein induces apoptosis synergistically in chemo-resistant cancer cells through the modulation of AMPK and COX-2 signaling pathways  

SciTech Connect

5-Fluorouracil (5-FU) is one of the widely used chemotherapeutic drugs targeting various cancers, but its chemo-resistance remains as a major obstacle in clinical settings. In the present study, HT-29 colon cancer cells were markedly sensitized to apoptosis by both 5-FU and genistein compared to the 5-FU treatment alone. There is an emerging evidence that genistein, soy-derived phytoestrogen, may have potential as a chemotherapeutic agent capable of inducing apoptosis or suppressing tumor promoting proteins such as cyclooxygenase-2 (COX-2). However, the precise mechanism of cellular cytotoxicity of genistein is not known. The present study focused on the correlation of AMPK and COX-2 in combined cytotoxicity of 5-FU and genistein, since AMPK is known as a primary cellular homeostasis regulator and a possible target molecule of cancer treatment, and COX-2 as cell proliferation and anti-apoptotic molecule. Our results demonstrated that the combination of 5-FU and genistein abolished the up-regulated state of COX-2 and prostaglandin secretion caused by 5-FU treatment in HT-29 colon cancer cells. These appear to be followed by the specific activation of AMPK and the up-regulation of p53, p21, and Bax by genistein. Under same conditions, the induction of Glut-1 by 5-FU was diminished by the combination treatment with 5-FU and genistein. Furthermore, the reactive oxygen species (ROS) was found as an upstream signal for AMPK activation by genistein. These results suggested that the combination of 5-FU and genistein exert a novel chemotherapeutic effect in colon cancers, and AMPK may be a novel regulatory molecule of COX-2 expression, further implying its involvement in cytotoxicity caused by genistein.

Hwang, Jin-Taek [Department of Biochemistry and Molecular Biology, Medical Research Center for Bioreaction to Reactive Oxygen Species, Kyung Hee University College of Medicine, Seoul 130-701 (Korea, Republic of); Ha, Joohun [Department of Biochemistry and Molecular Biology, Medical Research Center for Bioreaction to Reactive Oxygen Species, Kyung Hee University College of Medicine, Seoul 130-701 (Korea, Republic of); Park, Ock Jin [Department of Food and Nutrition, Hannam University, 133 Ojeong-dong Daedeok-gu, Daejeon 306-791 (Korea, Republic of)]. E-mail: ojpark@hannam.ac.kr

2005-07-01

135

Effect of Indirect Nonequilibrium Atmospheric Pressure Plasma on Anti-Proliferative Activity against Chronic Chemo-Resistant Ovarian Cancer Cells In Vitro and In Vivo  

PubMed Central

Purpose Nonequilibrium atmospheric pressure plasma (NEAPP) therapy has recently been focused on as a novel medical practice. Using cells with acquired paclitaxel/cisplatin resistance, we elucidated effects of indirect NEAPP-activated medium (NEAPP-AM) exposure on cell viability and tumor growth in vitro and in vivo. Methods Using chronic paclitaxel/cisplatin-resistant ovarian cancer cells, we applied indirect NEAPP-exposed medium to cells and xenografted tumors in a mouse model. Furthermore, we examined the role of reactive oxygen species (ROS) or their scavengers in the above-mentioned EOC cells. Results We assessed the viability of NOS2 and NOS3 cells exposed to NEAPP-AM, which was prepared beforehand by irradiation with NEAPP for the indicated time. In NOS2 cells, viability decreased by approximately 30% after NEAPP-AM 120-sec treatment (P<0.01). The growth-inhibitory effects of NEAPP-AM were completely inhibited by N-acetyl cysteine treatment, while L-buthionine-[S, R]-sulfoximine, an inhibitor of the ROS scavenger used with NEAPP-AM, decreased cell viability by 85% after NEAPP-AM 60-sec treatment(P<0.05) and by 52% after 120 sec, compared to the control (P<0.01). In the murine subcutaneous tumor-formation model, NEAPP-AM injection resulted in an average inhibition of the NOS2 cell-inoculated tumor by 66% (P<0.05) and NOS2TR cell-inoculated tumor by 52% (P<0.05), as compared with the control. Conclusion We demonstrated that plasma-activated medium also had an anti-tumor effect on chemo-resistant cells in vitro and in vivo. Indirect plasma therapy is a promising treatment option for EOC and may contribute to a better patient prognosis in the future. PMID:24367486

Utsumi, Fumi; Kajiyama, Hiroaki; Nakamura, Kae; Tanaka, Hiromasa; Mizuno, Masaaki; Ishikawa, Kenji; Kondo, Hiroki; Kano, Hiroyuki; Hori, Masaru; Kikkawa, Fumitaka

2013-01-01

136

High-dose chemotherapy with autologous peripheral blood stem cell transplantation for bone and soft-tissue sarcomas  

Microsoft Academic Search

The role of high-dose chemotherapy (HDCT) with PBSCT in the treatment of bone and soft-tissue sarcomas is not established. In total, 27 patients (15 female, median age at TPL 30.6 years (range: 13–59)) were analyzed (Ewing sarcoma family n=8, osteosarcoma n=6, MPNST (malignant peripheral nerve sheath tumor) n=4, synovial sarcoma n=3, liposarcoma n=2, leiomyosarcoma n=2, rhabdomyosarcoma n=1, meningosarcoma n=1). Following

B Kasper; T Lehnert; L Bernd; G Mechtersheimer; H Goldschmidt; A D Ho; G Egerer

2004-01-01

137

ErbB Receptors as Prognostic and Therapeutic Drug Targets in Bone and Soft Tissue Sarcomas.  

PubMed

ErbB receptors have been intensely studied to understand their importance in cancer biology and as therapeutic targets, and many ErbB inhibitors are now used in the clinical setting. A large number of studies have been conducted to examine the expression of ErbB family members in bone and soft tissue sarcomas, including osteosarcomas, synovial sarcomas, Ewing sarcomas, rhabdomyosarcomas, and so on. Nevertheless, the clinical implications of ErbB receptors remain elusive. To illustrate the potential of ErbB family members as prognostic and therapeutic drug targets in bone and soft tissue sarcomas, we summarized the molecular evidence and observations from clinical and basic trials. PMID:25347730

Wang, Hongsheng; Yang, Qingbo; Fu, Zeze; Zuo, Dongqing; Hua, Yingqi; Cai, Zhengdong

2014-12-01

138

Epithelioid Sarcoma  

PubMed Central

Epithelioid sarcoma is a rare, highgrade, soft tissue tumor that has a known propensity for local recurrence, regional lymph node involvement, and distant metastases. We review the clinical and histological presentations of epithelioid sarcoma. Because epithelioid sarcoma presents innocuously, it is often mistaken as a benign process, which can result in insufficient treatment. Therefore, we emphasize the need for clinicians to consider this diagnosis when a slowgrowing tumor is found on the distal extremity of a young male as the malignancy inherently portends a poor prognosis. Prognostic factors, such as local recurrence, regional metastatic disease, and tumor width, are discussed along with current treatment modalities, which include radical excision, sentinel lymph node biopsy, and radiation. PMID:20729965

Sobanko, Joseph F.; Meijer, Lindsay; Nigra, Thomas P.

2009-01-01

139

Kaposi Sarcoma  

Cancer.gov

DCEG researchers conduct studies on Kaposi sarcoma (KS). Infection with KS-associated herpesvirus (KSHV, also known as human herpesvirus-8 [HHV-8]) is necessary for KS to occur, but other factors, such as HIV infection, greatly increase the risk of the disease.

140

Preclinical evaluation of nanoparticle albumin-bound paclitaxel for treatment of pediatric bone sarcoma.  

PubMed

The combination of docetaxel and gemcitabine is frequently used to treat recurrent bone sarcoma. Nanoparticle albumin-bound paclitaxel (nab-paclitaxel) is less toxic and more active than docetaxel or paclitaxel for breast cancer patients. The combination of nab-paclitaxel and gemcitabine has preclinical synergy and is approved to treat pancreatic cancer. We observed growth inhibition and improved survival with nab-paclitaxel in a Ewing sarcoma xenograft, and activity was additive with gemcitabine in an osteosarcoma model. Primary Ewing sarcoma tumors expressed the transport protein SPARC, previously associated with nab-paclitaxel activity. These findings provide rationale for further evaluation of nab-paclitaxel with gemcitabine for bone sarcoma. Pediatr Blood Cancer 2014;61:2096-2098. © 2014 Wiley Periodicals, Inc. PMID:24753077

Wagner, Lars M; Yin, Hong; Eaves, David; Currier, Mark; Cripe, Timothy P

2014-11-01

141

Synovial sarcoma.  

PubMed Central

Of 34 cases with synovial sarcoma, the five-year survival rate was 36%. A high local recurrence rate results when local excision is performed. Wide excision which may necessitate amputation is the treatment of choice. Prophylactic and even therapeutic node dissections are ineffective in increasing survival because of the disease. Evaluation of radiation therapy was impossible, although some patients obtained significant paliation. Adriamycin appears to have a tumoricidal effect and provided clinically significant responses in several patients. PMID:164156

Gerner, R E; Moore, G E

1975-01-01

142

Comorbidity in adult bone sarcoma patients: a population-based cohort study.  

PubMed

Background. Comorbidity is an important prognostic factor for survival in different cancers; however, neither the prevalence nor the impact of comorbidity has been investigated in bone sarcoma. Methods. All adult bone sarcoma patients from western Denmark treated at the Aarhus Sarcoma Centre in the period from 1979 to 2008 were identified through a validated population-based database. Charlson Comorbidity Index scores were computed, using discharge diagnoses from the Danish National Patient Registry. Survival was assessed as overall and disease-specific mortality. The impact of comorbidity was examined as rates according to the level of comorbidity as well as uni- and multivariately using proportional hazard models. Results. A total of 453 patients were identified. The overall prevalence of comorbidity was 19%. The prevalence increased with age and over the study period. In patients with Ewing/osteosarcoma, comorbidity was not associated with an increased overall or disease-specific mortality. However, patients with bone sarcomas other than Ewing/osteosarcoma had increased overall mortality. Independent prognostic factors for disease-specific survival were age, tumor size, stage at diagnosis, soft tissue involvement, grade, and surgery. Conclusion. The prevalence of comorbidity in bone sarcoma patients is low. Comorbidity impaired survival in patients with non-Ewing/nonosteosarcoma, histology. This emphasizes the importance of not only treating the sarcoma but also comorbidity. PMID:24723789

Aggerholm-Pedersen, Ninna; Maretty-Nielsen, Katja; Keller, Johnny; Baerentzen, Steen; Safwat, Akmal

2014-01-01

143

Horton and Ewing Medalists  

NASA Astrophysics Data System (ADS)

AGU has announced the recipients of the 1988 Robert E. Horton and Maurice Ewing medals. The recipient of the Robert E. Horton Medal for outstanding contributions to the geophysical aspects of hydrology will be Peter S. Eagleson of the Massachusetts Institute of Technology. Eagleson is the immediate Past-President of AGU.The recipient of the Maurice Ewing Medal for significant original contributions to understanding physical, geophysical, and geological processes in the ocean; and/or significant original contributions to scientific ocean engineering, technology, and instrumentation; and/or outstanding service to marine sciences is Wolfgang H. Berger of the Scripps Institution of Oceanography. The medal is presented jointly by the U.S Navy and AGU.

144

Development of IGF-IR inhibitors in pediatric sarcomas  

Microsoft Academic Search

For approximately two decades, the insulin-like growth factor (IGF) has been implicated in the pathogenesis of numerous pediatric\\u000a malignancies, including osteosarcoma, Ewing sarcoma, and rhabdomyosarcoma. In the past, major toxicities have limited the\\u000a clinical development of IGF-targeted therapies. However, recent interest has been heightened by the availability of increasingly\\u000a specific small molecules and antibodies directed at IGF-I receptor. Preclinical data

E. Anders Kolb; Richard Gorlick

2009-01-01

145

Relationships Among Ewe Milk Production and Ewe and Lamb Forage Intake in Targhee Ewes Nursing Single or Twin Lambs  

Microsoft Academic Search

Targhee ewes with single or twin lambs were used in four trials during early lactation through weaning to evaluate ewe milk production (MP), kilograms of lamb weaned, and forage intake by ewes and lambs. During Trial 1, ewes with lambs were individually penned, fed .45 kg of barley-ewe-l-d-l and allowed ad libitum access to chopped alfalfa hay. Trials 2, 3,

W. S. Ramsey; P. G. Hatfields; J. D. Wallace; G. M. Southward

2010-01-01

146

Region Based Image Fusion for Detection of Ewing Sarcoma  

Microsoft Academic Search

In the medical image processing different sources of images are providing complementary information so fusion of different source images will give more details for diagnosis of patients. In this paper an automatic region based image fusion algorithm is proposed which is applied on the registered Magnetic Resonance (MR) image of human brain. The aim of this paper is to detect

Tanish Zaveri; Mukesh A. Zaveri

2009-01-01

147

Optimal Learning for Drug Discovery in Ewing's Sarcoma Diana Negoescu  

E-print Network

' prognosis and avoid many of the side effects caused by chemotherapy and radiation. This compound operates usually consists of chemotherapy followed by surgery or radiation therapy, often causing damaging side effects. While the impact of the disease is mitigated by its relatively rarity, with approximately 200

Powell, Warren B.

148

Constitutive Activation of Neuregulin\\/ERBB3 Signaling Pathway in Clear Cell Sarcoma of Soft Tissue1  

Microsoft Academic Search

Clear cell sarcoma of soft tissue (CCSST) represents a highly malignant tumor of the musculoskeletal system that is characterized by the chromosomal translocation t(12;22)(q13;q12) of the Ewing sarcoma gene (EWSR1) and activating transcription factor 1 (ATF1). In a former microarray expression study, we identified ERBB3 ,a member oftheepidermal growthfactor receptor(EGFR) family, as a promising new diagnostic marker in the differential

Karl-Ludwig Schaefer; Kristin Brachwitz; Yvonne Braun; Raihanatou Diallo; Daniel H. Wai; Susanne Zahn; Dominik T. Schneider; Cornelius Kuhnen; Arabel Vollmannb; Gero Brockhoff; Helmut E. Gabbert; Christopher Poremba

149

Neurologic Complications of Sarcoma  

Microsoft Academic Search

Sarcomas are a heterogeneous group of tumors that rarely involve the nervous system. Neurologic effects of sarcoma are more\\u000a often due to tumors outside of the central nervous system. However, as long-term survival rates in childhood sarcoma improves,\\u000a reports of late neurologic complications have increased. With recent advances in treating local sarcomas with targeted molecular\\u000a therapies, the incidence of late

Santosh Kesari; Lara J. Kunschner

150

Vascular sarcomas.  

PubMed

Vascular sarcomas are soft-tissue tumors that arise from the endothelium with a malignant potential. This review discusses the management of epithelioid hemangioendothelioma (EHE) and angiosarcoma. EHE is a vascular tumor of intermediate malignant potential with an indolent course. EHE arising from the liver, lung, or bone tends to be multifocal and the rate of progression is slow and often unpredictable. Treatment should be considered in patients with significant symptomatic deterioration and/or progressive disease on imaging studies. Various cytotoxic and targeted therapies are available for management, with disease stabilization as the most common outcome. Angiosarcoma is an aggressive vascular tumor with a high malignant potential. Multidisciplinary care is critical for the management of localized disease, and the best outcomes are often observed in patients when a combination of systemic and local therapy options is used. Metastatic angiosarcoma is treated primarily with systemic therapy, and several cytotoxic and targeted therapies are available, alone or in combination. The choice of therapy depends on several factors, such as cutaneous location of the tumor, performance status of the patient, toxicity of the treatment, and patient goals. PMID:23852636

Ravi, Vinod; Patel, Shreyaskumar

2013-08-01

151

Ewing Marion Kauffman Foundation  

NSDL National Science Digital Library

Founded in 1966, the Kauffman Foundation was the brainchild of Ewing Kauffman who displayed a great curiosity about the world and who also happened to be a great believer in the importance of philanthropy. Over the past forty years, the Foundation has worked on a variety of initiatives, including work on supporting early education, entrepreneurship, and school reform. On the homepage, visitors will find five primary sections, including "Advancing Innovation", "Education", and "Research & Policy". The first place to start is the "Research & Policy" area. Here, visitors can find data reports and analysis papers on national entrepreneurship trends and technology innovation strategies. In each section, visitors can also view media clips featuring commentary from Kauffman Foundation scholars and experts. Moving on, the "Grants" area is a great way to learn about grant opportunities and recipients listed by date and name. Finally, the "Stay Connected" area contains a place where visitors can sign up to receive their various e-newsletters.

152

Relationships among ewe milk production and ewe and lamb forage intake in Targhee ewes nursing single or twin lambs.  

PubMed

Targhee ewes with single or twin lambs were used in four trials during early lactation through weaning to evaluate ewe milk production (MP), kilograms of lamb weaned, and forage intake by ewes and lambs. During Trial 1, ewes with lambs were individually penned, fed .45 kg of barley-ewe-1.d-1 and allowed ad libitum access to chopped alfalfa hay. Trials 2, 3, and 4 were conducted on three different types of Western range environments. Forage intake of ewes in Trials 2, 3, and 4 and of lambs in Trials 3 and 4 was estimated using chromic oxide. Estimates of milk production were obtained by hand-milking. Average lamb age was 4, 50, 78, and 106 d at the beginning of Trials 1, 2, 3, and 4, respectively. Milk production was greater (P = .01) for ewes nursing twins than for those nursing singles at d 4 and 14 in Trial 1. In Trials 2, 3, and 4, all ewes had similar (P > .18) milk production. Ewe DMI during Trials 1 and 2 was greater (P < .05) for ewes nursing twin lambs than for those nursing singles. There was no difference (P > .88) in DMI between ewes with single and those with twin lambs in Trials 3 and 4. Forage DMI by twin lambs, expressed as either kilograms.ewe-1.day-1 or percentage of lamb BW, was higher (P = .01) than intake by single lambs in Trials 3 and 4.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:8014144

Ramsey, W S; Hatfield, P G; Wallace, J D; Southward, G M

1994-04-01

153

[Soft tissue sarcoma--problems of diagnosis and treatment].  

PubMed

Soft tissue sarcomas are a rare group of cancers compromising 1% of all malignancies and there has been a slight increase in incidence. We present 3 cases of soft tissue sarcomas (the tumors were located to the right axilary region, perianal and dorsale face of the left leg) hospitalized in 2nd Surgical Clinic of Emergency Hospital of Craiova and we discuss the difficulties of diagnosis and treatment. The classification and characterization of soft-tissue sarcomas have evolved as the information supplied by histologic analysis has been supplemented with that provided by immunohistochemical analysis. Surgical resection involving wide margins, with or without radiotherapy, offers the best chance of cure in the absence of metastatic disease. There is little evidence that local recurrence increases the likelihood of metastatic spread, although debate on this point continues. Except for rhabdomyosarcomas and Ewing's sarcomas, the use of adjuvant chemotherapy generally does little to influence the natural history of the disease. In conclusion surgical treatment is mainstay of treatment for soft-tissue sarcomas and is usefull the prompt diagnosis for decrease the risk of local recurrence and metastatic disease. PMID:20540243

Me?in?, C; Vasile, I; Vîlcea, I D; Pa?alega, M; Pârv?nescu, H; Calot?, F; Georgescu, C V; Ghilu?i, M; Dumitrescu, T; Mirea, C; Mogoan??, S; Moraru, E

2010-01-01

154

NY-ESO-1 expression in synovial sarcoma and other mesenchymal tumors: significance for NY-ESO-1-based targeted therapy and differential diagnosis.  

PubMed

A promising targeted therapy against NY-ESO-1 (CTAG 1B) using genetically modified T-cells in synovial sarcomas was recently demonstrated in a clinical trial at the NCI. To investigate the role of NY-ESO-1 immunohistochemistry in patient selection and gain better insight into the incidence of NY-ESO-1 expression in synovial sarcomas and other mesenchymal tumors, we evaluated NY-ESO-1 expression by immunohistochemistry in 417 tumors. This collection of samples included: 50 SS18/SSX1/2 fusion positive synovial sarcomas, 155 gastrointestinal stromal tumors (GIST), 135 other spindle cell sarcomas as well as 77 other sarcomas (chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, rhabdomyosarcoma, angiosarcoma, malignant mesothelioma, and Ewing's sarcoma). We report that 76% of synovial sarcomas expressed NY-ESO-1 in a strong and diffuse pattern (2-3+, >50-70% of tumor cells). In contrast, only rare cases of other spindle cell mesenchymal tumor expressed NY-ESO-1 (GIST (2/155), malignant peripheral nerve sheath tumors (1/34), and dermatofibrosarcoma protuberans (2/20)). Individual cases of other sarcomas (angiosarcoma, malignant mesothelioma, chondrosarcoma, osteosarcoma, dedifferentiated liposarcoma, alveolar soft part sarcoma, and Ewing's sarcoma) were positive for NY-ESO-1. However, no positive cases were identified amongst our cohort of leiomyosarcomas (0/24), hemangiopericytoma/solitary fibrous tumors (0/40), and cellular schwannomas (0/17). In summary, we find that NY-ESO-1 is strongly and diffusely expressed in a majority of synovial sarcomas, but only rarely in other mesenchymal lesions. Beyond its role in patient selection for targeted therapy, immunohistochemistry for NY-ESO-1 may be diagnostically useful for the distinction of synovial sarcoma from other spindle cell neoplasms. PMID:22388761

Lai, Jin-Ping; Robbins, Paul F; Raffeld, Mark; Aung, Phyu Phyu; Tsokos, Maria; Rosenberg, Steven A; Miettinen, Markku M; Lee, Chyi-Chia Richard

2012-06-01

155

Carbon Ion Radiotherapy for Unresectable Retroperitoneal Sarcomas  

SciTech Connect

Purpose: To evaluate the applicability of carbon ion radiotherapy (CIRT) for unresectable retroperitoneal sarcomas with regard to normal tissue morbidity and local tumor control. Methods and Materials: From May 1997 to February 2006, 24 patients (17 male and 7 female) with unresectable retroperitoneal sarcoma received CIRT. Age ranged from 16 to 77 years (median, 48.6 years). Of the patients, 16 had primary disease and 8 recurrent disease. Histologic diagnoses were as follows: malignant fibrous histiocytoma in 6, liposarcoma in 3, malignant peripheral nerve sheath tumor in 3, Ewing/primitive neuroectodermal tumor (PNET) in 2, and miscellaneous in 10 patients. The histologic grades were as follows: Grade 3 in 15, Grade 2-3 in 2, Grade 2 in 3, and unknown in 4. Clinical target volumes ranged between 57 cm{sup 3} and 1,194 cm{sup 3} (median 525 cm{sup 3}). The delivered carbon ion dose ranged from 52.8 to 73.6 GyE in 16 fixed fractions over 4 weeks. Results: The median follow-up was 36 months (range, 6-143 months). The overall survival rates at 2 and 5 years were 75% and 50%, respectively. The local control rates at 2 and 5 years were 77% and 69%. No complications of the gastrointestinal tract were encountered. No other toxicity greater than Grade 2 was observed. Conclusions: Use of CIRT is suggested to be effective and safe for retroperitoneal sarcomas. The results obtained with CIRT were a good overall survival rate and local control, notwithstanding the fact that most patients were not eligible for surgical resection and had high-grade sarcomas.

Serizawa, Itsuko, E-mail: s_itsuko@nirs.go.j [Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba (Japan); Kagei, Kenji; Kamada, Tadashi; Imai, Reiko; Sugahara, Shinji; Okada, Tohru; Tsuji, Hiroshi [Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba (Japan); Ito, Hisao [Department of Radiology, Chiba University, Chiba (Japan); Tsujii, Hirohiko [Research Center Hospital for Charged Particle Therapy, National Institute of Radiological Sciences, Chiba (Japan)

2009-11-15

156

Whole-lung irradiation in the treatment of metastatic synovial sarcoma.  

PubMed

Whole-lung irradiation (WLI) is standard of care in the treatment of patients with rhabdomyosarcoma, Ewing sarcoma, and Wilms tumor and pulmonary metastases. However, it is not routinely utilized in the treatment of pulmonary metastases arising from other soft tissue sarcoma histologies. A patient presented with synovial sarcoma of his groin and punctate pulmonary metastases. After completion of multimodality treatment to his primary lesion, he received WLI. The patient is without evidence of disease at 3.8 years. This case demonstrates the need for further study of WLI in synovial sarcoma as it may improve outcomes in patients with this disease. Pediatr Blood Cancer 2014;61:2092-2093. © 2014 Wiley Periodicals, Inc. PMID:24939451

Gerber, Naamit K; Meyers, Paul A; LaQuaglia, Michael P; Wolden, Suzanne L

2014-11-01

157

Pineal region myeloid sarcoma.  

PubMed

The overwhelming majority of pineal region tumors are malignant germ cell tumors, pineal cell tumors, or glial tumors. To our knowledge we report the first patient with myeloid sarcoma in the pineal region. Myeloid sarcomas are composed of immature granulocytic precursor cells and are associated with acute myelogenous leukemia. Thus, myeloid sarcoma should be considered in the differential diagnosis of pineal region masses in patients with a known history of acute myelogenous leukemia. PMID:22560847

Wilson, Thomas J; Than, Khoi D; Parmar, Hemant A; Lieberman, Andrew P; Valdivia, Juan M; Sullivan, Stephen E

2012-07-01

158

Metformin as an Adjuvant Drug against Pediatric Sarcomas: Hypoxia Limits Therapeutic Effects of the Drug  

PubMed Central

Metformin, a well-known insulin-sensitizer commonly used for type 2 diabetes therapy, has recently emerged as potentially very attractive drug also in oncology. It is cheap, it is relatively safe and many reports have indicated effects in cancer prevention and therapy. These desirable features are particularly interesting for pediatric sarcomas, a group of rare tumors that have been shown to be dependent on IGF and insulin system for pathogenesis and progression. Metformin exerts anti-mitogenic activity in several cancer histotypes through several molecular mechanisms. In this paper, we analyzed its effects against osteosarcoma, Ewing sarcoma and rhabdomyosarcoma, the three most common pediatric sarcomas. Despite in vitro metformin gave remarkable antiproliferative and chemosensitizing effects both in sensitive and chemoresistant cells, its efficacy was not confirmed against Ewing sarcoma xenografts neither as single agent nor in combination with vincristine. This discrepancy between in vitro and in vivo effects may be due to hypoxia, a common feature of solid tumors. We provide evidences that in hypoxia conditions metformin was not able to activate AMPK and inhibit mTOR signaling, which likely prevents the inhibitory effects of metformin on tumor growth. Thus, although metformin may be considered a useful complement of conventional chemotherapy in normoxia, its therapeutic value in highly hypoxic tumors may be more limited. The impact of hypoxia should be considered when novel therapies are planned for pediatric sarcomas. PMID:24391834

Garofalo, Cecilia; Capristo, Mariantonietta; Manara, Maria Cristina; Mancarella, Caterina; Landuzzi, Lorena; Belfiore, Antonino; Lollini, Pier-Luigi; Picci, Piero; Scotlandi, Katia

2013-01-01

159

A unique pattern of INI1 immunohistochemistry distinguishes synovial sarcoma from its histologic mimics.  

PubMed

The absence of INI1 (SMARCB1, hSNF5, BAF47) immunohistochemical reactivity is a central feature of malignant rhabdoid tumor, renal medullary carcinoma, and epithelioid sarcoma. We characterized INI1 immunoreactivity in synovial sarcoma (49 cases) in comparison with its closest histologic mimics (68 cases). We observed a unique pattern of decreased INI1 immunoreactivity with a high specificity (100%) and sensitivity (86%) for synovial sarcoma and particular sensitivity for poorly differentiated subtypes of synovial sarcoma (94%; 16/17 cases). Decreased INI1 immunoreactivity was not seen in any of the other lesions we examined, including 14 cases of Ewing sarcoma and 22 cases of malignant peripheral nerve sheath tumor. Furthermore, decreased INI1 immunoreactivity is distinct from the complete absence of INI1 immunoreactivity seen in malignant rhabdoid tumor or other INI1-negative neoplasms. We propose that this distinct INI1 immunohistochemical pattern serves as a useful diagnostic tool to provide preliminary results before molecular test results are available, especially in cases of poorly differentiated synovial sarcoma and in cases where limited material precludes confirmatory molecular studies. Awareness of this unique pattern is critical to avoid misinterpreting decreased INI1 immunoreactivity as a complete absence of INI1 and, consequently, misdiagnosing synovial sarcoma as an INI1-negative neoplasm. PMID:23245672

Arnold, Michael A; Arnold, Christina A; Li, Guang; Chae, Uisoo; El-Etriby, Rana; Lee, Chyi-Chia Richard; Tsokos, Maria

2013-05-01

160

Molecular Alterations in Pediatric Sarcomas: Potential Targets for Immunotherapy  

PubMed Central

Purpose/results/discussion. Recurrent chromosomal translocations are common features of many human malignancies. While such translocations often serve as diagnostic markers, molecular analysis of these breakpoint regions and the characterization of the affected genes is leading to a greater understanding of the causal role such translocations play in malignant transformation. A common theme that is emerging from the study of tumor-associated translocations is the generation of chimeric genes that, when expressed, frequently retain many of the functional properties of the wild-type genes from which they originated. Sarcomas, in particular, harbor chimeric genes that are often derived from transcription factors, suggesting that the resulting chimeric transcription factors contribute to tumorigenesis. The tumor-specific expression of the fusion proteins make them likely candidates for tumor-associated antigens (TAA) and are thus of interest in the development of new therapies. The focus of this review will be on the translocation events associated with Ewing's sarcomas/PNETs (ES), alveolar rhabdomyosarcoma (ARMS), malignant melanoma of soft parts (MMSP) (clear cell sarcoma), desmoplastic small round cell tumor (DSRCT), synovial sarcoma (SS), and liposarcoma (LS), and the potential for targeting the resulting chimeric proteins in novel immunotherapies. PMID:18521238

Goletz, Theresa J.; Mackall, Crystal L.; Berzofsky, Jay A.

1998-01-01

161

SYMPOSIUM: MOLECULAR GENETICS IN SARCOMA Synovial Sarcoma  

E-print Network

2030 East, Room 5440, Salt Lake City, UT 84112-5331, USA e-mail: mario.capecchi@genetics.utah.edu M. Haldar, M. R. Capecchi Howard Hughes Medical Institute, University of Utah School of Medicine, Salt Lake City, UT, USA R. L. Randall Huntsman Cancer Institute Sarcoma Services, University of Utah, Salt Lake

Capecchi, Mario R.

162

Donor Cell Myeloid Sarcoma  

PubMed Central

Donor cell derived malignancies are a rare and interesting complication of allogeneic bone marrow transplantation. We present a case of a 56-year-old male with donor cell myeloid sarcoma of the stomach and myocardium. PMID:24822132

Walshauser, Mark A.; Sojitra, Payal

2014-01-01

163

Radiation-induced sarcoma  

Microsoft Academic Search

Opinion statement  Radiation-induced sarcomas can originate in either the irradiated bone or soft tissues. Most of these tumors are high-grade.\\u000a The most common histologic subtypes are malignant fibrous histiocytoma (MFH) and osteosarcoma, although other histologies\\u000a (eg, angiosarcoma, rhabdomyosarcoma) can occur. Tumor size and grade are the two most important prognostic factors for soft tissue\\u000a sarcomas, including those associated with radiation therapy.

Shreyaskumar R. Patel

2000-01-01

164

Lactation curves of commercial ewes rearing lambs.  

PubMed

Three-hour milk production measurements determined by machine milking at 3-d intervals throughout a 63-d lactation period were used to describe lactation curves for crossbred ewes lambing at 1 and 2 yr of age and rearing single and twin lambs. Age of ewe, type of rearing, and day of lactation affected (P < 0.05) milk production. Over the 63-d lactation, average daily milk production was 2.56 and 2.63 kg, respectively, for 1- and 2-yr-old ewes rearing single lambs and 2.73 and 3.47 kg, respectively, for 1- and 2-yr-old ewes rearing twins. Milk production of 2-yr-old ewes rearing twin lambs peaked at 21 d of lactation, and that of 1- and 2-yr-old ewes rearing singles peaked between 27 and 30 d of lactation. The largest differences in the lactation curves among age and rearing ewe classes were found in early lactation. These differences were reduced by midlactation, and by late lactation, milk production for all ewes was similar. Diurnal variation in milk production by ewes was evaluated in an 8 x 8 Latin square design. Diurnal variation in milk yield measurements of eight mature ewes, each bearing and rearing twin lambs, was similar between d 21 and 24 of lactation. Time of milk production measurements within a day did not affect yield determinations. Extrapolation from 3-h production estimates to daily milk production is valid in determining a ewe's milk contribution in support of lamb growth. PMID:11831522

Cardellino, R A; Benson, M E

2002-01-01

165

Expression Profiling of t(12;22) Positive Clear Cell Sarcoma of Soft Tissue Cell Lines Reveals Characteristic Up-Regulation of Potential New Marker Genes Including ERBB3  

Microsoft Academic Search

Clear cell sarcoma of soft tissue (CCSST), also known as malignant melanoma of soft parts, represents a rare lesion of the musculoskeletal system usually affecting adolescents and young adults. CCSST is typified by a chromosomal t(12;22)(q13;q12) translocation resulting in a fusion between the Ewing sarcoma gene (EWSR1) and activating transcription factor 1 (ATF1), of which the activity in nontransformed cells

Karl-Ludwig Schaefer; Kristin Brachwitz; Daniel H. Wai; Yvonne Braun; Raihanatou Diallo; Eberhard Korsching; Martin Eisenacher; Reinhard Voss; Frans van Valen; Claudia Baer; Barbara Selle; Laura Spahn; Shuen-Kuei Liao; Kevin A. W. Lee; Pancras C. W. Hogendoorn; Guido Reifenberger; Helmut E. Gabbert; Christopher Poremba

2004-01-01

166

Synovial sarcoma in the neck.  

PubMed

Synovial sarcomas are very uncommon and highly malignant tumours. This uncommon malignant tumour of mesenchymal origin may rarely present in the neck. Since 1954, nearly 80 cases of synovial sarcoma, located in the head and neck region have been reported. Synovial sarcoma is most prevalent in adolescents and young adults between 15 and 40 years of age. In this report we present a case of synovial sarcoma in the neck of a 10-year-old patient. PMID:8390971

Onerci, M; Sarioglu, T; Gedikoglu, G; Hosal, S; Ruacan, S

1993-05-01

167

Suicide gene therapy of sarcoma cell lines using recombinant adeno-associated virus 2 vectors.  

PubMed

Soft-tissue sarcomas are mesenchymal tumors that respond poorly to systemic chemotherapy. Suicide gene therapy may be an alternative treatment strategy. Here we show a high susceptibility of human sarcoma cell lines for recombinant adeno-associated virus 2 (rAAV-2) suicide vectors: connective tissue sarcoma (HS-1), fibrosarcoma (HT-1080), Ewing sarcoma (RD-ES), Askin tumor (SK-N-MC), rhabdomyosarcoma (A-204) and soft-tissue sarcoma (WSKL-1). Several vectors containing the thymidine kinase (TK) gene under the control of either the cytomegalovirus promoter or the elongation-factor 1 alpha (EF1alpha) promoter were cloned and tested. Higher expression levels of the transgene were observed in the sarcoma lines when using the EF1alpha-suicide gene-containing vectors. A complete eradication of rAAV-2-EF1alpha-TK/eGFP (TK/enhanced green fluorescent protein fusion gene)-transduced tumor cells was shown following exposure to ganciclovir (2.5 microg/ml) in vitro, while at this dose level > 90% of mock-transduced tumor cells survived. Xenotransplantation tumor models (intraperitoneal, subcutaneous) for the human sarcoma cell line HS-1 were established in nonobese diabetic/severe-combined immunodeficient mice. Mice transplanted with rAAV-2-EF1alpha-TK/eGFP-transduced and ganciclovir-exposed tumor cells survived > 5 months while in the nontransduced group all mice had died approximately 1 month after inoculation. These data hold promise for further development of rAAV-2-based suicide gene therapy of sarcomas. PMID:15280909

Veldwijk, Marlon R; Berlinghoff, Simone; Laufs, Stephanie; Hengge, Ulrich R; Zeller, W Jens; Wenz, Frederik; Fruehauf, Stefan

2004-08-01

168

Intraarticular epithelioid sarcoma.  

PubMed

A case of an intraarticular epithelioid sarcoma is presented. The patient was a 35 year old man who presented with a 10 months history of a chronic monoarthritis. The MRI showed a diffuse lesion involving the synovial membrane of the knee. There was a marked increased signal on T2 weighted images. Most epithelioid sarcomas involve the subcutaneous tissues of the hands or feet. This presentation is unusual and this entity should be considered in the differential diagnosis of an intraarticular proliferative process. PMID:9765140

Hurtado, R M; McCarthy, E; Frassica, F; Holt, P A

1998-08-01

169

17-N-Allylamino-17-Demethoxygeldanamycin in Treating Patients With Advanced Epithelial Cancer, Malignant Lymphoma, or Sarcoma  

ClinicalTrials.gov

AIDS-related Peripheral/Systemic Lymphoma; AIDS-related Primary CNS Lymphoma; Anaplastic Large Cell Lymphoma; Angioimmunoblastic T-cell Lymphoma; Chondrosarcoma; Extranodal Marginal Zone B-cell Lymphoma of Mucosa-associated Lymphoid Tissue; Intraocular Lymphoma; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Nodal Marginal Zone B-cell Lymphoma; Ovarian Sarcoma; Primary Central Nervous System Non-Hodgkin Lymphoma; Recurrent Adult Burkitt Lymphoma; Recurrent Adult Diffuse Large Cell Lymphoma; Recurrent Adult Diffuse Mixed Cell Lymphoma; Recurrent Adult Diffuse Small Cleaved Cell Lymphoma; Recurrent Adult Hodgkin Lymphoma; Recurrent Adult Immunoblastic Large Cell Lymphoma; Recurrent Adult Lymphoblastic Lymphoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Adult T-cell Leukemia/Lymphoma; Recurrent Cutaneous T-cell Non-Hodgkin Lymphoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Grade 1 Follicular Lymphoma; Recurrent Grade 2 Follicular Lymphoma; Recurrent Grade 3 Follicular Lymphoma; Recurrent Mantle Cell Lymphoma; Recurrent Marginal Zone Lymphoma; Recurrent Mycosis Fungoides/Sezary Syndrome; Recurrent Osteosarcoma; Recurrent Small Lymphocytic Lymphoma; Recurrent Uterine Sarcoma; Small Intestine Lymphoma; Splenic Marginal Zone Lymphoma; Stage IV Adult Burkitt Lymphoma; Stage IV Adult Diffuse Large Cell Lymphoma; Stage IV Adult Diffuse Mixed Cell Lymphoma; Stage IV Adult Diffuse Small Cleaved Cell Lymphoma; Stage IV Adult Hodgkin Lymphoma; Stage IV Adult Immunoblastic Large Cell Lymphoma; Stage IV Adult Lymphoblastic Lymphoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Adult T-cell Leukemia/Lymphoma; Stage IV Cutaneous T-cell Non-Hodgkin Lymphoma; Stage IV Grade 1 Follicular Lymphoma; Stage IV Grade 2 Follicular Lymphoma; Stage IV Grade 3 Follicular Lymphoma; Stage IV Mantle Cell Lymphoma; Stage IV Marginal Zone Lymphoma; Stage IV Mycosis Fungoides/Sezary Syndrome; Stage IV Small Lymphocytic Lymphoma; Stage IV Uterine Sarcoma; Unspecified Adult Solid Tumor, Protocol Specific

2013-02-06

170

LEUKEMIA, SARCOMA AND RADIOACTIVITY  

Microsoft Academic Search

Data are reviewed on the induction of leukemia, osteogenic sarcoma, ; thyroid cancer, and skin cancer by exposure to ionizing radiation. Available ; data on man are compared with experimental data from laboratory animals. The ; radiation danger from fallout for human populations is considered. (43 ; references.) (C.H.);

1962-01-01

171

Sorafenib for Kaposi's Sarcoma  

Cancer.gov

In this trial, patients with either AIDS-related or non-AIDS-related Kaposi's sarcoma (KS) will be treated with varying doses of the drug sorafenib (Nexavar®) for up to 54 weeks to examine the safety of the drug and determine how it is processed in patients with KS who are receiving antiretroviral therapy.

172

Recombinant Interferon Gamma in Treating Patients With Soft Tissue Sarcoma  

ClinicalTrials.gov

Adult Liposarcoma; Adult Synovial Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Stage IA Adult Soft Tissue Sarcoma; Stage IB Adult Soft Tissue Sarcoma; Stage IIA Adult Soft Tissue Sarcoma; Stage IIB Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

2014-05-29

173

Exogenous oxytocin dilates the cervix in ewes.  

PubMed

Cervical anatomy in ewes usually prevents nonsurgical, intrauterine AI and transcervical embryo transfer (ET), which limits their commercial use in sheep. This study was conducted to determine whether oxytocin would dilate the cervix in ewes and permit passage of a stainless steel rod into the uterus. In Exp. 1, at 44 and 52 h after removal of progestogenated pessaries, ewes were injected i.v. with 0 (saline), 200, 400, or 600 USP units of oxytocin. Immediately before and after treatments, stainless steel rods were used to evaluate cervical dilation and determine whether the uterus could be entered. A rod could not be passed through the cervix and into the uterus in any of the saline-treated ewes. All doses of oxytocin given at 44 and 52 h after pessary removal dilated the cervix and permitted easy passage of a rod into the uterus. At both 44 and 52 h, a stainless steel rod was passed into the uterus in 33 of 43 (77%) of the oxytocin-treated ewes. In 93% (40/43) of these ewes, a rod could be passed into the uterus during either the 44-h or during the 52-h attempt. In Exp. 2, on d 9 after pessary removal, ewes were injected i.v. with oxytocin (400 USP units) at 6 or 12 h after i.v. estradiol-17 beta (0, 100, or 200 micrograms). Cervical dilation was evaluated as in Exp. 1. Dose of estradiol x time of oxytocin affected (P less than .01) the proportion of ewes in which a rod could be passed transcervically into the uterus.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1582916

Khalifa, R M; Sayre, B L; Lewis, G S

1992-01-01

174

Effects of helium pneumoperitoneum in pregnant ewes  

Microsoft Academic Search

  Background: Previous animal studies have demonstrated that a carbon dioxide (CO2) pneumoperitoneum in pregnant ewes causes\\u000a maternal and fetal acidosis, decreased uterine blood flow (UtBF), and fetal hypertension. This study was undertaken to determine\\u000a whether helium (He) produces these same effects when used as an insufflating gas. Methods: Six gravid ewes, at 116 to 120\\u000a days gestation, underwent catheterization of

M. J. Curet; D. M. Weber; A. Sae; J. Lopez

2001-01-01

175

Spinal and Paraspinal Ewing Tumors  

SciTech Connect

Purpose: To perform a review of the 40-year University of Florida experience treating spinal and paraspinal Ewing tumors. Patients and Methods: A total of 27 patients were treated between 1965 and 2007. For local management, 21 patients were treated with radiotherapy (RT) alone and 6 with surgery plus RT. All patients with metastatic disease were treated with RT alone. The risk profiles of each group were otherwise similar. The median age was 17 years, and the most frequent subsite was the sacral spine (n = 9). The median potential follow-up was 16 years. Results: The 5-year actuarial overall survival, cause-specific survival, and local control rate was 62%, 62%, and 90%, respectively. For the nonmetastatic subset (n = 22), the 5-year overall survival, cause-specific survival, and local control rate was 71%, 71%, and 89%, respectively. The local control rate was 84% for patients treated with RT alone vs. 100% for those treated with surgery plus RT. Patients who were >14 years old and those who were treated with intensive therapy demonstrated superior local control. Of 9 patients in our series with Frankel C or greater neurologic deficits at presentation, 7 experienced a full recovery with treatment. Of the 27 patients, 37% experienced Common Toxicity Criteria Grade 3 or greater toxicity, including 2 deaths from sepsis. Conclusion: Aggressive management of spinal and paraspinal Ewing tumors with RT with or without surgery results in high toxicity but excellent local control and neurologic outcomes. Efforts should be focused on identifying disease amenable to combined modality local therapy and improving RT techniques.

Indelicato, Daniel J., E-mail: dindelicato@floridaproton.or [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); University of Florida Proton Therapy Institute, Jacksonville, FL (United States); Keole, Sameer R. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); University of Florida Proton Therapy Institute, Jacksonville, FL (United States); Shahlaee, Amir H. [Department of Pediatrics, University of Florida College of Medicine, Gainesville, FL (United States); Morris, Christopher G. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); University of Florida Proton Therapy Institute, Jacksonville, FL (United States); Gibbs, C. Parker; Scarborough, Mark T. [Department of Orthopedic Surgery, University of Florida College of Medicine, Gainesville, FL (United States); Pincus, David W. [Department of Neurosurgery, University of Florida College of Medicine, Gainesville, FL (United States); Marcus, Robert B. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); University of Florida Proton Therapy Institute, Jacksonville, FL (United States)

2010-04-15

176

Do We Know What Causes Kaposi Sarcoma?  

MedlinePLUS

... Kaposi sarcoma be prevented? Do we know what causes Kaposi sarcoma? Kaposi sarcoma (KS) is caused by ... as Epstein-Barr virus (EBV), the virus that causes infectious mononucleosis (mono) and is linked to several ...

177

Stages of Adult Soft Tissue Sarcoma  

MedlinePLUS

... Soft Tissue Sarcoma After adult soft tissue sarcoma has been diagnosed, tests are done to find out ... and Stage III Adult Soft Tissue Sarcoma That Has Not Spread to Lymph Nodes Treatment of stage ...

178

Relationships among ewe milk production and ewe and lamb forage intake in Suffolk and Targhee ewes nursing single or twin lambs.  

PubMed

Suffolk and Targhee ewes (30 each) with single or twin lambs were used in four periods beginning in late gestation and continuing through weaning to evaluate breed differences in milk production, lamb BW, and DMI by ewes and lambs. In Periods 1 (late gestation) and 2 (early lactation), ewes (Period 1) and ewes with lambs (Period 2) were individually penned, fed .45 kg of barley x ewe(-1) x d(-1) and allowed ad libitum access to chopped alfalfa. Ewes and lambs grazed native range in Periods 3 and 4. Grazed forage DMI was estimated using chromic oxide. Estimates of milk production were obtained by handmilking. Average lamb age was 4, 45, and 73 d at the beginning of Periods 2, 3, and 4, respectively. Milk production tended (P = .20) to be greater for Suffolk than for Targhee ewes. Targhee ewes produced 85% more (P = .001) wool than Suffolk ewes. From 33 d prepartum to 89 d postpartum, Suffolk ewes consistently weighed more (P = .001) than Targhee ewes. Suffolk ewe BW loss (-.15 kg/d) was greater (P = .01) than Targhee ewe BW loss (-.02 kg/d) from 33 d prepartum to 6 d postpartum. From 6 to 89 d postpartum BW gain did not differ (P = .69; .05 kg/d) between breeds. From birth to 89 d postpartum, Suffolk lambs consistently weighed more than Targhee lambs (P = .003). From birth to 89 d postpartum, ADG was greater for Suffolk than for Targhee lambs (P = .006). Targhee ewes consumed 25% more (P = .01) feed over the course of the study than did Suffolk ewes. Grazed forage DMI by Targhee lambs was 26% greater (P = .01) than DMI by Suffolk lambs. When meat production is the primary income from sheep, one potential advantage of Suffolks compared with Targhees is more rapid gain with less feed intake. PMID:9621929

Ramsey, W S; Hatfield, P G; Wallace, J D

1998-05-01

179

Targeted Therapy of Sarcoma  

Microsoft Academic Search

Sarcomas represent a heterogeneous group of tumors that are composed of a wide range of tumor types with different natural\\u000a histories and therapeutic approaches. Recent discoveries have identified specific molecular alterations in the pathogenesis\\u000a of many of these tumors. These specific molecular alterations acquired during sarcomagenesis lead to the phenotypic changes\\u000a of malignancy, namely proliferation, survival, invasion, metastasis, and angiogenesis.

Joseph Ludwig; Jonathan C. Trent

180

An aza-macrocycle containing maltolic side-arms (maltonis) as potential drug against human pediatric sarcomas  

PubMed Central

Background Identification of new drugs against paediatric sarcomas represents an urgent clinical need that mainly relies on public investments due to the rarity of these diseases. In this paper we evaluated the in vitro and in vivo efficacy of a new maltol derived molecule (maltonis), belonging to the family of molecules named hydroxypyrones. Methods Maltonis was screened for its ability to induce structural alteration of DNA molecules in comparison to another maltolic molecule (malten). In vitro antitumour efficacy was tested using a panel of sarcoma cell lines, representative of Ewing sarcoma, osteosarcoma and rhabdomyosarcoma, the three most common paediatric sarcomas, and in normal human mesenchymal primary cell cultures. In vivo efficacy was tested against TC-71 Ewing sarcoma xenografts. Results Maltonis, a soluble maltol-derived synthetic molecule, was able to alter the DNA structure, inhibit proliferation and induce apoptotic cell death in paediatric sarcoma cells, either sensitive or resistant to some conventional chemotherapeutic drugs, such as doxorubicin and cisplatin. In addition, maltonis was able to induce: i) p21, p15 and Gadd45a mRNA upregulation; ii) Bcl-2, survivin, CDK6 and CDK8 down-regulation; iii) formation of ?-H2AX nuclear foci; iv) cleavage of PARP and Caspase 3. Two independent in vivo experiments demonstrated the tolerability and efficacy of maltonis in the inhibition of tumour growth. Finally maltonis was not extruded by ABCB1, one of the major determinants of chemotherapy failure, nor appeared to be a substrate of the glutathione-related detoxification system. Conclusions Considering that treatment of poorly responsive patients still suffers for the paucity of agents able to revert chemoresistance, maltonis may be considered for the future development of new therapeutic approaches for refractory metastatic patients. PMID:24575739

2014-01-01

181

Cleavage of ova in New Zealand romney ewes  

Microsoft Academic Search

The onset of oestrus was observed in 99 Romney ewes injected with pregnant mares' serum (PMS) on day 12 or 13 of the cycle; in 34 untreated cyclic ewes; and in 26 anoestrous ewes, following treatment with progestagen and PMS. All ewes were mated to entire rams and in vivo recovery of ova attempted 36-101 hours after onset of oestrus.Thirty-eight

H. R. Tervit; M. F. McDonald

1968-01-01

182

THE TRANSPORT OF SHEEP AND GOAT SPERMATOZOA IN THE EWE  

Microsoft Academic Search

Summary. Counts were made of the numbers of spermatozoa in the Fallopian tubes of ewes 24 hr after mating or after insemination with either sheep or goat semen. Spermatozoa were recovered from all five mated ewes (10\\/10 tubes), from all eight ewes inseminated with sheep semen (13\\/15 tubes) and from four of eight ewes inseminated with goat semen (7\\/16 tubes).

J. L. HANCOCK; P. T. McGOVERN

1968-01-01

183

Strategies for rapid rebreeding of lactating ewes in the spring.  

PubMed

Rapid rebreeding of winter- and spring-lambing ewes is essential if ewes are to lamb more than once per year, but fertility of lactating ewes is often low and early weaning of lambs may be undesirable in forage-based production systems. Selection to improve fertility in spring matings has been successful and led to development of ewes with a reduced seasonal anestrus. Potential for rapid rebreeding of lactating out-of-season breeding (OOS) ewes was tested in three studies. In Experiment 1, effects of short-term lamb removal on rebreeding was evaluated over 2 years using 71 January-lambing OOS ewes. At an average of 63 days postpartum, 36 ewes had lambs removed for 72 h, and all ewes were joined with rams. Circulating progesterone levels indicated that 74% of ewes ovulated before lamb separation; 91% of ewes mated within 5 weeks of ram exposure, 85% were diagnosed as pregnant and 75% lambed. The average interval between lambings was 225 days. In contrast to results observed in cattle, none of the measured variables was affected by lamb separation (P>0.20). Experiment 2 compared rebreeding performance of 24 OOS and 23 St. Croix ewes that lambed in January and averaged 60 days postpartum at ram introduction. More OOS ewes ovulated, mated and became pregnant during the first 21 days of ram exposure (83.3%, 58.3%, and 41.7%, respectively; P<0.001) compared with St. Croix ewes (26.1%, 0%, and 0%, respectively). After 39 days of ram exposure, pregnancy rates still favored OOS ewes (66.7% v. 39.1%; P=0.06), but the percentage of ewes that lambed did not differ (P>0.20) between OOS (47.8%) and St. Croix ewes (34.8%). In the third study, 34 March-lambing OOS ewes were exposed to rams on May 3 at an average of 40 days postpartum to characterize their reproductive performance. After 39 days of ram exposure, 52.9±8.7% of the ewes had mated, and 38.2±8.5% were diagnosed as pregnant. However, only 20.6±7.0% of the ewes produced viable lambs, suggesting a high level of uterine insufficiency. Spring fertility of lactating OOS ewes in these studies was one of the highest reported in the literature and indicated that selection for fertility in spring mating would improve reproductive performance in accelerated lambing programs. However, exposure of lactating OOS ewes to rams at 30 to 50 days postpartum was associated with high prenatal lamb mortality. PMID:24622342

Goff, K J; Notter, D R; Vanimisetti, H-B; Knight, J W

2014-06-01

184

[Intraosseous highly differentiated osteogenic sarcoma].  

PubMed

A man of 20 developed well-differentiated intraosseous osteogenic sarcoma of the femur. Clinico-roentgenologic, computed tomography and histological findings revealed a number of features uncommon for the tumor: (1) a torpid course, (2) histological appearance typical for parosteal sarcoma, (3) low malignant potential. PMID:3196181

Petrovichev, N N; Khmelev, O N; Luk'ianchenko, A B; Karapetian, R M

1988-01-01

185

Primary sarcomas of the lung  

Microsoft Academic Search

Background: Chest physicians have a limited experience of primary pulmonary sarcomas, which represent a particular entity among rare intrathoracic neoplasms. Design: Retrospective review of medical records. Purpose: To study patients with primary sarcomas of the lung diagnosed in our pathology department in order to define their clinical characteristics, treatment, and prognosis. Patients: The study group consisted of 12 patients, with

Bénédicte Etienne-Mastroianni; Lionel Falchero; Lara Chalabreysse; Robert Loire; Dominique Ranchère; Pierre-Jean Souquet; Jean-François Cordier

2002-01-01

186

Pediatric Nonrhabdomyosarcoma Soft Tissue Sarcomas  

Microsoft Academic Search

The nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) are a heterogeneous group of mesenchymal cell neoplasms that account for about 4% of childhood cancers. Because each histologic subtype of NRSTS is rare, they have been poorly studied and little is known about their biology, natural history, or optimal treat- ment. Data from adults with soft tissue sarcomas pro- vide some helpful insight,

SHERI L. SPUNT; STEPHEN X. SKAPEK; CHERYL M. COFFINc

187

The effect of ewe size and nutritional regimen beginning in early pregnancy on ewe and lamb performance to weaning  

Microsoft Academic Search

Ewe size, pregnancy nutrition and pregnancy rank are known to affect the productive performance of ewes and their offspring. The aim of the present study was to compare the effects of two nutritional regimens, from day 21 to day 140 of pregnancy, in singleton? and twin?bearing ewes of two different mean weights, taken from a single flock. The study included

P. R. Kenyon; H. T. Blair; C. M. C. Jenkinson; S. T. Morris; D. D. S. Mackenzie; S. W. Peterson; E. C. Firth; P. L. Johnston

2009-01-01

188

Soft tissue sarcoma subtypes exhibit distinct patterns of acquired uniparental disomy  

PubMed Central

Background Soft tissue sarcomas (STS) are heterogeneous mesenchymal tumors with diverse subtypes. STS can be classified into two main categories according to the type of genomic alteration: recurrent translocation driven STS, and non-recurrent translocations. However, little has known about acquired uniparental disomy in STS. Methods In this study, we analyzed SNP microarray data to determine the frequency and distribution patterns of acquired uniparental disomy (aUPD) in major soft tissue sarcoma (STS) subtypes using CNAG and R softwares. Results We identified recurrent aUPD regions specific to alveolar rhabdomyosarcoma with the most frequent at 11p15.4, gastrointestinal stromal tumor at 1p36.11-p35.3, leiomyosarcoma at 17p13.3-p13.1, myxofibrosarcoma at 1p35.1-p34.2 and 16q23.3-q24.1, and pleomorphic liposarcoma at 13q13.2-q13.3 and 13q14.11-q14.2. In contrast, specific recurrent aUPD regions were not identified in dedifferentiated liposarcoma, Ewing sarcoma, myxoid/round cell liposarcoma, and synovial sarcoma. Strikingly total, centromeric and segmental aUPD regions are more frequent in STS that do not exhibit recurrent translocation events. Conclusions Our study yields a detailed map of aUPD across 9 diverse STS subtypes and suggests the potential location of several novel tumor suppressor genes and oncogenes. PMID:23217126

2012-01-01

189

Adult Pelvic Sarcomas: A Heterogeneous Collection of Sarcomas?  

PubMed Central

Introduction. Adult pelvic soft tissue sarcomas are a rare group of heterogeneous malignancies. These sarcomas differ from extremity and trunk soft tissue sarcomas in presentation, characteristics and response to treatment. Methods. A retrospective analysis of patient and tumor characteristics, treatment and prognosis and prognostic factors was performed. Results. Between 1977 and 1997, a total of 33 adult patients with soft tissue sarcomas involving the pelvis but excluding uterine leiomyosarcoma were identified. Leiomyosarcomas (18), including six GIST, and rhabdomyosarcomas (eight) were the most commonly seen tumors. At first presentation, nine patients already had metastases. The mean follow-up was 52 months (1–200). Recurrences developed in 15 of the 24 cases (63%) with tumors without metastases at first presentation; in six (25%) recurrence was locally only, in nine distant metastases occurred. The nine patients with metastatic disease at first presentation died of the disease, while eight of the 24 patients with localized disease at presentation died. One patient died of an unrelated cause, four were alive with disease, and 11 patients were alive and free of disease. The only identifiable prognostic factor of disease-free interval and overall survival was histological grade. Conclusion. Soft tissue sarcomas of the pelvis appear to be associated with increased rate of metastasis at the time of diagnosis and higher rates of local recurrence. In this study, multi-modality treatment for most primary tumors did not show a significant benefit in recurrence rate, DFI and OST, when compared to single modality approach. Although the number of patients in this study is small, and different types of sarcomas were studied, the only identifiable predictor for survival was low histological grade of the tumors. The differences of this heterogeneous group of pelvic sarcomas with retroperitoneal, trunk and extremity sarcomas should be taken into consideration in the management of these sarcomas. PMID:18521389

Keyzer-Dekker, Claudia M. G.; Houtkamp, Richard G.; Peterse, Johannes L.

2004-01-01

190

Immunohistochemical validation of TLE1, a novel marker, for synovial sarcomas  

PubMed Central

Background & objectives: Logistic and financial constraints limit application of several available immunohistochemical (IHC) markers and molecular analysis in every case of synovial sarcoma, diagnosed in our settings. Recently, TLE1 has been recognized as a robust IHC marker for diagnosing a synovial sarcoma. Here, we present IHC features of synovial sarcomas, including TLE1 expression in these cases and in some other tumours. Methods: Conventional sections from 42 synovial sarcomas (30 retrospective & 12 prospectively diagnosed) were subjected to TLE1 IHC staining, including 21 tumours confirmed with molecular testing. TLE1 immunostaining was graded from 0, 1+, 2+, 3+, with 2+ or 3+ grades interpreted as positive staining. Results: Of the 42 tumours, 26 (61.9%) were of monophasic spindle cell type, 13 biphasic type (30.9%), two (4.7%) calcifying type and remaining one (2.3%) was a poorly differentiated synovial sarcoma. On immunohistochemistry (IHC), tumours were positive for epithelial membrane antigen (EMA) (26/34, 76.4%), cytokeratin (CK)7 (6/10, 60%), CK/MNF116 (6/21, 28.6%), B cell lymphoma 2 (BCL2) (36/37, 97.3%), cluster of differentiation molecule 99 (MIC2) (23/31, 74.1%) and transducin-like enhancer of split 1 (TLE1) (40/42, 95.2%), while negative for CD34 in all 21 tumours, wherever performed. TLE1 was also positive in tumour controls, including schwannomas (5/5, 100%), neurofibromas (2/2, 100%), malignant peripheral nerve sheath tumors (2/12, 17%) and Ewing sarcomas (4/10, 40%). TLE1 sensitivity for diagnosis of synovial sarcomas was 95.2 per cent. Its overall specificity was 63.7 per cent, whereas with regards to tumors forming its closest differential diagnoses, its specificity was 72 per cent. Interpretation & conclusions: Although molecular confirmation is the diagnostic gold standard for synovial sarcoma, TLE1, in view of its high sensitivity may be a useful marker within the optimal IHC panel comprising EMA, BCL2, MIC2, CD34 and CK7, especially on small biopsy samples, for substantiating a diagnosis of synovial sarcoma. Awareness of TLE1 expression in other tumours and its correct interpretation are necessary. PMID:23287123

Rekhi, Bharat; Basak, Ranjan; Desai, Sangeeta B.; Jambhekar, Nirmala A.

2012-01-01

191

Memo from the desk of Sally Ewing  

E-print Network

the University Commencement and the School of Communication Convocation. The School of Communication conducts. We at the School of Communication are immensely proud of your achievements here. While weMemo from the desk of Sally Ewing Assistant Dean for Advising and Student Affairs School

Contractor, Anis

192

Preoperative Radiotherapy for Retroperitoneal Sarcoma  

Cancer.gov

In this trial, researchers are testing whether radiotherapy before surgery will help patients with retroperitoneal sarcoma survive longer without relapse of their cancer than when treated with surgery alone.

193

Drugs Approved for Kaposi Sarcoma  

Cancer.gov

This page lists cancer drugs approved by the Food and Drug Administration (FDA) for Kaposi sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

194

Synovial sarcoma in childhood  

SciTech Connect

The clinical and radiologic findings in seven children with synovial sarcoma are described. The five boys and two girls had a mean age at presentation of 4.4 years. All seven had the lesion situated in an extremity. Plain radiographs in four revealed the presence of a soft-tissue mass with no calcification or bone and joint involvement. In two patients studied with computed tomography (CT), the primary lesions had peripheral irregular areas of enhancement with central areas of poor enhancement, reflecting the necrotic, cystic, and hemorrhagic changes found in the centers of these tumors. Although the exact margins of these lesions were difficult to define accurately even with intravenous contrast enhancement, CT is still recommended as the best imaging method for assessing the local extent of the primary tumor and is a useful tool in the planning of appropriate therapy as well as the gauging of the tumor response to ongoing treatment.

Israels, S.J.; Chan, H.S.L.; Daneman, A.; Weitzman, S.S.

1984-04-01

195

Clear cell sarcoma.  

PubMed

Malignant melanoma (MM) of soft tissue, also called clear cell sarcoma (CCS) of tendons and aponeuroses, derives from the neural crest. CCS is similar morphologically to MM but has no precursor skin lesion, and instead, has a characteristic chromosomal translocation. Prognosis is related to the tumor size. Early recognition and initial radical surgery is the key to a favorable outcome. The tumor has to be differentiated from other benign and malignant lesions of the soft tissues, such as fibrosarcoma. The demonstration of melanin and a positive immunohistochemical reaction for S-100 protein and HMB-45 can assist in the differential diagnosis. We report the case of a 58-year-old woman with CCS arising from the soft tissue of her little finger. PMID:25396137

Ozuguz, Pinar; Kocak, Mukadder; Atasoy, Pinar; Vargel, Ibrahim; Cavusoglu, Tarik

2014-10-01

196

Reproductive performance of ewe lambs from ewes from different selection practices with or without induced estrus.  

PubMed

Three groups of ewe lambs born in May (experiment 1; n=211) or April (experiment 2; n=174) were used to evaluate the effects of selection line and induction of estrus on pregnancy rate. Experiment 1 was a single factor experiment with induction of estrus as the main effect. In early December, May-born Targhee (n=82) and Rambouillet x Targhee (n=129) ewes were randomly assigned within body weight to one of two treatment groups: control or induction of estrus. Experiment 2 was designed in a 2x2 factorial array with the main effects of induction of estrus or selection line. In early November, April-born Targhee lambs (n=174) from two distinct selection lines were either treated as controls or received an estrus induction treatment. The two lines included an unselected control line of randomly bred ewes and a line that had been selected since 1976, based on the weight of lamb weaned. Ewes from each line were randomly assigned within body weight to one of the treatment groups. In experiments 1 and 2, estrus was induced using MAP pessaries. Pessaries were inserted for 12 days. At the time of pessary removal, ewe lambs received 400 IU eCG i.m. All ewe lambs were bred in multi-sire pens. Pregnancy rate and fetal numbers were determined either by lambing data or real-time ultrasound. Body weight, lambing date and fetal numbers were analyzed by GLM, and remaining variables were analyzed by CATMOD. For experiment 1, estrus induction increased (P<0.01) pregnancy rates (61 versus 31%) and number of fetuses estimated by real-time ultrasound (79 versus 35%) compared to control ewe lambs. Pregnancy rate and fetal number were increased (P<0.01) for the 1st year compared to the 2nd year. For experiment 2, estrus induction tended to increase (P<0.07) pregnancy rate, and pregnancy rate differed (P<0.01) between selection lines. Estrus induction increased (P<0.05) fetal numbers (0.96) compared to controls (0.77). Fetal numbers were greater (P<0.01) for the selected line (1.06) compared to random bred controls (0.67). Average date of lambing was earlier in both experiments for the estrus-induced ewe lambs compared to controls. These results indicate that induction of estrus can be recommended if increased reproduction is desired for ewe lambs. PMID:11348781

Stellflug, J N; Hatfield, P G; Wulster-Radcliffe, M C; Walker, J W

2001-05-31

197

Sorafenib in Treating Patients With Metastatic, Locally Advanced, or Recurrent Sarcoma  

ClinicalTrials.gov

Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Leiomyosarcoma; Adult Malignant Fibrous Histiocytoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

2014-05-07

198

Experimental ornithosis in ewes (Ovis aries  

E-print Network

Station. TASLE OF CONTENTS INTRODUCTION. REVIEW OF LITERATURE. 0BS ERVATI ONS, DISCUSSION CONCLUSIONS AND SUHMARY. REFERENCES. TABLES TABLE I. OBSERVATIONS ON ABORTED OVINE PETUSES, . . 11 II, SEROLOGICAL DATA ON ENES. CONPLEHENT PIXATION TITERS... by abortion during the latter part of gestatioa, or birch of deed or weak laabs that die 35 within 48 hours was reported in 1950 . This disease aay infect any ewe, regardless of age, and aay produce no evidence of infection other than in the fetal tissues...

Pierce, Kenneth Ray

2012-06-07

199

Radiotherapy for Kaposi's sarcoma  

SciTech Connect

Between 1954 and 1976, 60 patients with Kaposi's sarcoma were treated in the Department of Radiotherapy of the Lahey Clinic Foundation at the High Voltage Research Laboratory of Massachusetts Institute of Technology. Only 2 patients were free of clinical disease in the lower extremities at the time of initial presentation, and 40 patients (69%) had cutaneous lesions involving areas extending above the knees. Eight patients (13%) also presented with mucous membrane involvement in addition to skin disease. Twenty-one patients were treated only with megavoltage electrons during the initial course of radiotherapy, and 12 patients were treated with supervoltage photons alone. The remaining 27 patients were treated with a combination of electrons and photons; in 17 patients, the same tumor sites were irradiated with both modalities. Eleven patients received whole-body surface electron irradiation. The choice of treatment modalities was based on the extent and distribution of cutaneous disease and depth of the lesions. The overall response rate was 93% after a single fractionated course of radiotherapy. Twenty-five patients achieved complete regression and 18 were in remission for 2 to 13 years. Response rates were also analyzed with respect to the three subgroups in terms of treatment modalities. A single dose of 800 to 1200 rads or its equivalent was required to control local cutaneous lesions. Widespread visceral metastasis was the most common cause of failure and death; the incidence of second malignancies was increased. Trial of systemic chemotherapy and immunotherapy would seem to be a reasonable therapeutic adjunct.

Lo, T.C.; Salzman, F.A.; Smedal, M.I.; Wright, K.A.

1980-02-15

200

Ewing Sarcoma Protein Ewsr1 Maintains Mitotic Integrity and Proneural Cell Survival in the Zebrafish Embryo  

E-print Network

, disorganization of neuronal networks, and embryonic lethality by 5 days post-fertilization. siRNA silencing of EWSR1 in Hela cells resulted in mitotic defects accompanied by apoptotic cell death, indicating that the role of EWSR1 is conserved between zebrafish....pone.0000979.g004 Ewsr1 in Mitosis PLoS ONE | www.plosone.org 4 October 2007 | Issue 10 | e979 Loss of mitotic integrity accompanied by mislocalization of Aurora B proteins in EWSR1 deficient Hela cells The Ewsr1 knockdown in zebrafish embryos induced mitotic...

Azuma, Mizuki; Embree, Lisa J.; Sabaawy, Hatem; Hickstein, Dennis D.

2007-10-03

201

Immunomic analysis of human sarcoma  

PubMed Central

The screening of cDNA expression libraries from human tumors with serum antibody (SEREX) has proven to be a powerful method for identifying the repertoire of tumor antigens recognized by the immune system of cancer patients, referred to as the cancer immunome. In this regard, cancer/testis (CT) antigens are of particular interest because of their immunogenicity and restricted expression patterns. Synoivial sarcomas are striking with regard to CT antigen expression, with >80% of specimens homogeneously expressing NY-ESO-1 and MAGE-A3. In the present study, 54 sarcoma patients were tested for serum antibodies to NY-ESO-1, SSX2, MAGE-A1, MAGE-A3, MAGE-A4, MAGE-A10, CT7, and CT10. Two patients had detectable antibodies to CT antigens, and this seroreactivity was restricted to NY-ESO-1. Thus, although highly expressed in sarcoma, CT antigens do not induce frequent humoral immune responses in sarcoma patients. Sera from these two patients were used to immunoscreen cDNA libraries from two synovial sarcoma cell lines and normal testis, resulting in the identification of 113 distinct antigens. Thirty-nine antigens were previously identified by SEREX analysis of other tumor types, and 23/39 antigens (59%) had a serological profile that was not restricted to cancer patients, indicating that only a proportion of SEREX-defined antigens are cancer-related. A novel CT antigen, NY-SAR-35, mapping to chromosome Xq28 was identified among the cancer-related antigens, and encodes a putative extracellular protein. In addition to testis-restricted expression, NY-SAR-35 mRNA was expressed in sarcoma, melanoma, esophageal cancer, lung cancer and breast cancer. NY-SAR-35 is therefore a potential target for cancer vaccines and monoclonal antibody-based immunotherapies. PMID:12601173

Lee, Sang-Yull; Obata, Yuichi; Yoshida, Masahiro; Stockert, Elisabeth; Williamson, Barbara; Jungbluth, Achim A.; Chen, Yao-Tseng; Old, Lloyd J.; Scanlan, Matthew J.

2003-01-01

202

Reproductive performance of postpartum Awassi ewes under different lambing regimes  

Microsoft Academic Search

Reproductive performance was studied in 40 multiparous Awassi ewes, weaned during their first week postpartum (Regime A), or at 2 months postpartum (Regime B). In both regimes, ewes were simultaneously exposed to rams after weaning, for a period of 48 days. In Regime A, early weaning did not improve reproductive performance in fertility and prolificacy; however, it indicated their potential

S. K. Hamadeh; E. K. Barbour; M. Abi Said; K. Daadaa

1996-01-01

203

and Christina C. Leslie Bonventre, Heather Ewing, Michael H. Gelb  

E-print Network

-Lung Hsu§ , Joseph V. Bonventre¶ , Heather Ewing , Michael H. Gelb **, and Christina C. Leslie §§1 Fromand Christina C. Leslie Bonventre, Heather Ewing, Michael H. Gelb V.Benjamin F. Cravatt, Ku-Lung Hsu, Joseph Bogeon Yun, HeeJung Lee, Moumita Ghosh, Permeability Transition Pore Formation Overload

Gelb, Michael

204

Prognostic impact of chromosomal aberrations in Ewing tumours  

Microsoft Academic Search

Although greater than 50% of Ewing tumours contain non-random cytogenetic aberrations in addition to the pathognomonic 22q12 rearrangements, little is known about their prognostic significance. To address this question, tumour samples from 134 Ewing tumour patients were analysed using a combination of classical cytogenetics, comparative genomic and fluorescence in situ hybridisation. The evaluation of the compiled data revealed that gain

C M Hattinger; U Pötschger; M Tarkkanen; J Squire; M Zielenska; S Kiuru-Kuhlefelt; L Kager; P Thorner; S Knuutila; F K Niggli; P F Ambros; H Gadner; D R Betts

2002-01-01

205

Induction of ovulation in chronically hypophysectomized Booroola ewes  

Microsoft Academic Search

Summary. Booroola Merino ewes, with (F+; N = 17) and without (++; N = 13) a copy of the fecundity gene were hypophysectomized and 6 weeks later were given an i.m. injection of PMSG (high, medium or low dose) followed by hCG. The induced ovulation rates were observed laparoscopically. Ovulation rates were significantly higher (P < 0\\\\m=.\\\\01)in Booroola F+ ewes

R. C. Fry; I. J. Clarke; J. T. Cummins; B. M. Bindon; L. R. Piper; L. P. Cahill

1988-01-01

206

Rare sarcoma presented as sinusitis.  

PubMed

Myxofibrosarcoma (MFS) also known as myxoid variant of malignant fibrous histocytoma is one of the most common soft tissue sarcomas of the extremities in adult and elderly patients with rare occurrences in head and neck region. Low-grade MFS is unusual among low-grade sarcomas because it often recurs relentlessly and multiplies despite wide local resection with gross negative margins. We report a case of extreme rarity and a tumour of aggressive nature in the maxillary sinus, which presented with non-specific sinonasal symptoms and we present a review of the radiological and histopathological characteristics of this rare tumour and recent evidence of management. PMID:22927267

Taghi, Ali S; Ali, Ahmed; Kuchai, Romana; Saleh, Hesham

2012-01-01

207

LAMB PRODUCTION OF TARGHEE AND SUFFOLK-TARGHEE RANGE EWES 1  

Microsoft Academic Search

SUMMARY This study was conducted under primarily range conditions in southwestern Utah during an 8 year period from 1967 to 1974. The total sample size was 3,355 lambs and 2,255 ewes. Three breeding groups were included. Targhee ewes bred to Targhee rams (Tt), Targhee ewes bred to Suffolk rams (Ts), and Suffolk x Targhee crossbred ewes bred to Suffolk rams

D. H. Matthews; M. A. Madsen; J. A. Bennett; W. C. Foote

208

Original article Responses of ewes to B. melitensis Rev1 vaccine  

E-print Network

Original article Responses of ewes to B. melitensis Rev1 vaccine administered by subcutaneous the innocuousness and the humoral immune response of B. melitensis Rev1 vaccine was carried out in pregnant ewes. Thirty-three ewes were vaccinated on day 55 of pregnancy : 9 ewes received 2 x 109 Rev1 of French origin

Paris-Sud XI, Université de

209

Gemcitabine Hydrochloride, Docetaxel, and Radiation Therapy in Treating Patients With Uterine Sarcoma That Has Been Removed By Surgery  

ClinicalTrials.gov

Stage IA Uterine Sarcoma; Stage IB Uterine Sarcoma; Stage IC Uterine Sarcoma; Stage IIA Uterine Sarcoma; Stage IIB Uterine Sarcoma; Stage IIIA Uterine Sarcoma; Stage IIIB Uterine Sarcoma; Stage IIIC Uterine Sarcoma; Stage IVA Uterine Sarcoma; Stage IVB Uterine Sarcoma; Uterine Leiomyosarcoma

2014-05-09

210

Somatic cell counts of ewes' milk.  

PubMed

The somatic cell counts of ewes' milk were determined by an electronic particle counter (Coulter Counter). Of 1408 apparently normal milk samples, 98.2% had a somatic cell count lower than 1.0 x 10(6) cells/ml and 85.8% of 254 bacteriologically positive samples had a count higher than 1.0 x 10(6) cells/ml. Values exceeding 1.0 x 10(6) cells/ml are indicative of subclinical mastitis, if samples were collected from clinically healthy mammary glands. PMID:1777802

Fthenakis, G C; el-Masannat, E T; Booth, J M; Jones, J E

1991-01-01

211

Postradiation sarcoma involving the spine  

SciTech Connect

Postradiation sarcomas arising many years after treatment of cancer are long term sequelae of therapy. We describe the clinical features, radiographic findings, and results of treatment in 13 patients with such sarcomas encountered over a 6-year period. Of these patients, 9 had bone sarcomas and the remaining 4 had paraspinal tumors arising from adjacent soft tissue and nerve. The primary cancer for which radiation was given included Hodgkin's disease (4 patients), breast cancer (2 patients), cervix cancer (2 patients), and a variety of others (5 patients). The latent interval to the occurrence of the second neoplasm varied from 6 to 30 years (median, 10 years) after treatment of the original tumor. Despite aggressive treatment, the overall prognosis was poor. The median survival was 8 months, with only 3 surviving more than 2 years. Although rare, postradiation sarcoma should be considered in the differential diagnosis of patients presenting with late onset of spinal pain or neurological symptoms after clinical remission of an original cancer.

Sundaresan, N.; Huvos, A.G.; Krol, G.; Hughes, J.E.; Cahan, W.G.

1986-06-01

212

What's New in Soft Tissue Sarcomas Research and Treatment?  

MedlinePLUS

... Topic Additional resources for soft tissue sarcoma What`s new in soft tissue sarcoma research and treatment? Research ... develop. This information is already being applied to new tests to diagnose and classify sarcomas. This is ...

213

CASE REPORT Open Access Isolated granulocytic sarcoma of the pancreas  

E-print Network

CASE REPORT Open Access Isolated granulocytic sarcoma of the pancreas: A tricky diagnostic sarcoma of the pancreas that were diagnosed on the surgical specimen. Atypical clinical and morphological and therapeutic strategies. Keywords: Granulocytic sarcoma, Chloroma, Myeloid tumor, Pancreas. Background

Boyer, Edmond

214

A Primary Synovial Sarcoma of Lung  

PubMed Central

Primary pulmonary synovial sarcoma is an extremely rare tumor. The diagnosis is established only after sarcoma like primary lung malignancies and metastatic sarcoma have been excluded. It has four subtypes: monophasic fibrous, monophasic epithelial, biphasic, and poorly differentiated subtypes, We report a case of a 55-year-old man, who complained of left-sided chest pain and shortness of breath, had a large heterogeneous mass, occupying most of left hemithorax, associated with ipsilateral pleural effusion, seen on contrast enhanced computed tomogram of thorax. Computed tomography guided tru-cut biopsy revealed spindle cell sarcoma. On immunohistochemistry, tumor cells expressed epithelial membrane antigen, CD99, bcl-2 and Calponin and were immunonegative for cytokeratin. So, final diagnosis was primary pulmonary synovial sarcoma, Primary pulmonary synovial sarcoma is a rarely reported case of malignant neoplasm of lung. Histopathology, immunohistochemistry, and cytogenetics, if possible, are essential for confirmation of its diagnosis. PMID:22655286

Roy, PP; Das, A; Sarkar, A; Dwari, AK; Datta, S

2012-01-01

215

Bronchopulmonary Kaposi's sarcoma in patients with AIDS.  

PubMed Central

BACKGROUND: Kaposi's sarcoma is the most common secondary neoplasm to complicate HIV infection and may cause pulmonary disease. METHODS: A prospective study was carried out in 140 consecutive patients who were HIV seropositive and required bronchoscopy for new respiratory symptoms of at least two weeks' duration, with either a chest radiographic abnormality or abnormality of pulmonary function. The patients were classified into those with single local endobronchial lesions of Kaposi's sarcoma or generalised widespread lesions. Before bronchoscopy all patients had routine simple pulmonary function tests and chest radiography. RESULTS: Thirty nine (21%) patients had evidence of cutaneous Kaposi's sarcoma. Nineteen of the 39 were found to have endobronchial Kaposi's sarcoma lesions at bronchoscopy, but none of those who did not have cutaneous Kaposi's sarcoma. Respiratory symptoms of cough and breathlessness and radiographic abnormalities were attributed to Kaposi's sarcoma in this group, except in four patients who had concomitant pneumocystis pneumonia. Eight patients had local endobronchial Kaposi's sarcoma lesions and 11 had extensive lesions. Patients with extensive lesions had more widespread radiographic abnormalities; four of the patients with local endobronchial lesions had normal chest radiographs. All patients had reduced transfer factor for carbon monoxide and transfer coefficient, whereas patients with extensive endobronchial lesions also had reductions in forced expiratory volume in one second and forced vital capacity. Median survival (with palliative chemotherapy with vincristine and bleomycin) was only seven months. In three patients who needed further diagnostic bronchoscopy endobronchial lesions had regressed while they were having chemotherapy. CONCLUSIONS: This study suggests that endobronchial Kaposi's sarcoma is a relatively common finding in patients with AIDS and is particularly common in patients with cutaneous Kaposi's sarcoma who present with respiratory illness. Endobronchial Kaposi's sarcoma causes respiratory disease and abnormalities of pulmonary function. Pulmonary Kaposi's sarcoma should be considered as a possible cause for respiratory illness in any patient with cutaneous Kaposi's sarcoma. PMID:1440468

Mitchell, D M; McCarty, M; Fleming, J; Moss, F M

1992-01-01

216

Effects of time of weaning, pasture allowance, and shearing time on ewe and lamb liveweight, wool growth, and subsequent ovulation rate of the ewe  

Microsoft Academic Search

The effects of weaning lambs from ewes at either 8 or 14 weeks after lambing on subsequent ewe performance were investigated for 2 feeding levels and 2 shearing times. Pasture allowance levels for the high and low groups were about 4.5 and 2 kg green DM\\/day per ewe throughout the trial, which ran from the time of early weaning to

D. C. Smeaton; R. M. W. Sumner; T. W. Knight; T. K. Wadams

1983-01-01

217

Synovial sarcoma of the foot.  

PubMed

We report the case of a 75-year-old male who underwent lung lobectomy for presumed lung cancer. Thereafter, he presented with a painful mass between the third and fourth metatarsal heads in the foot that was assumed to be Morton's neuroma. After extensive oncologic evaluation, the foot mass was diagnosed as a synovial sarcoma. In retrospect, his lung lesion was understood to be metastatic disease. PMID:23632071

Bekarev, Mikhail; Elsinger, Elisabeth C; Villanueva-Siles, Esperanza; Borzykowski, Ross M; Geller, David S

2013-01-01

218

Soft tissue sarcomas in children  

Microsoft Academic Search

\\u000a Investigators from Europe and the United States meet periodically to define prognostically significant classifications for\\u000a the soft tissue sarcomas (STS) of children. International teams of pathologists have developed classification systems based\\u000a on classic histologic features, including that of the International Society of Pediatric Oncology (SIOP). Accurate classification\\u000a facilitates both the diagnosis and assessment of the probability of metastasis [1–5].

Charles B. Pratt; Larry E. Kun

219

Phase II multicenter trial of imatinib in 10 histologic subtypes of sarcoma using a bayesian hierarchical statistical model.  

PubMed

PURPOSE The purpose of this trial was to assess the efficacy of imatinib in patients with one of 10 different subtypes of advanced sarcoma. PATIENTS AND METHODS Eligible patients were treated daily with imatinib dosed at 300 mg twice a day (for body-surface area > or = 1.5 m(2)). The primary end point was response (clinical benefit response [CBR]), defined as complete (CR) or partial response (PR) at 2 months, or stable disease, CR, or PR at 4 months. Rules for early termination within each disease type were based on a Bayesian hierarchical probability model (BHM) accounting for correlation of the responses of the 10 subtypes. Available tissue samples were analyzed for molecules within the KIT/platelet-derived growth factor receptor (PDGFR) signal transduction pathway. Results One hundred eighty-five assessable patients with one of 10 subtypes of sarcoma were treated. One CR and three PRs were achieved. A CBR was achieved in 28 patients treated overall and by subtype: two angiosarcomas (n = 16), 0 Ewing (n = 13), one fibrosarcoma (n = 12), six leiomyosarcomas (n = 29), seven liposarcomas (n = 31), three malignant fibrous histiocytomas (n = 30), five osteosarcomas (n = 27), one malignant peripheral-nerve sheath tumor (n = 7), 0 rhabdomyosarcoma (n = 2), and three synovial sarcomas (n = 22). Variable expression and mutations within the KIT/PDGFR pathway were observed. CONCLUSION This is the first phase II study of a new agent in sarcoma to include sufficient patients with each of the common histologic subtypes to permit generalizable conclusions. The BHM is an effective method for studying rare diseases and their subtypes, when it is reasonable to assume that their response rates are exchangeable. Although rare dramatic responses were seen, imatinib is not an active agent in advanced sarcoma in these subtypes. PMID:19451433

Chugh, Rashmi; Wathen, J Kyle; Maki, Robert G; Benjamin, Robert S; Patel, Shreyaskumar R; Meyers, Paul A; Myers, Paul A; Priebat, Dennis A; Reinke, Denise K; Thomas, Dafydd G; Keohan, Mary L; Samuels, Brian L; Baker, Laurence H

2009-07-01

220

Potential for Early Breeding of Kivircik (Western Thrace) Ewes  

Microsoft Academic Search

Koyuncu, M., KaraUzun, S. and Ozis, S. 2007. Potential for early breeding of Kivircik (Western Thrace) ewes. J. Appl. Anim. Res., 32: 61–64.This study was carried out to investigate the possibility of early breeding of ewe lambs in Kivircik sheep breed. Mating and reproduction performances were investigated in normally mated (15–16 months old) and early mated (7 months old) females.

M. Koyuncu; S. KaraUzun; S. Ozis

2007-01-01

221

Utility of a Monoclonal ERG/FLI1 Antibody for Immunohistochemical Discrimination of Ewing's Family Tumors  

PubMed Central

Ewing family tumors (EFTs) and prostate carcinomas (PCa) are characterized by rearrangement of ETS genes, most commonly FLI1 (EFTs) and ERG (PCa). Previously, we characterized an antibody against ERG (EPR3864) for detecting ERG-rearranged PCa. EPR3864 also cross reacts with FLI1, thus, here we evaluated the utility of EPR3864 for discriminating EFTs from other small round blue cell tumors (SRBCTs) by immunohistochemistry. Of 57 evaluable EFTs, 47 (82%) demonstrated at least moderate, diffuse, nuclear ERG/FLI1 staining (including 89% and 100% of cases with confirmed EWSR1:FLI1 and EWSR1:ERG fusions, respectively), of which 1, 3 and 43 showed negative, cytoplasmic or membranous CD99 staining, respectively. Amongst other SRBCTs (n=61 cases, 6 types), at least moderate, diffuse, nuclear EPR3864 staining was seen in all precursor-B-lymphoblastic lymphomas/leukemias and subsets of Burkitt’s lymphomas (10%) and synovial sarcomas (45%). In summary, EPR3864 may have utility for detecting EWSR1:FLI1 and EWSR1:ERG rearranged EFTs, in addition to PCa. PMID:23690120

Tomlins, Scott A.; Palanisamy, Nallasivam; Brenner, J. Chad; Stall, Jennifer N.; Siddiqui, Javed; Thomas, Dafydd G.; Lucas, David R.; Chinnaiyan, Arul M.; Kunju, Lakshmi P.

2013-01-01

222

Radiation therapy for soft tissue sarcoma.  

PubMed

Sarcomas are relatively uncommon tumors of mesenchymal origin. They may arise anywhere within the musculoskeletal system and are typically classified by clinical behavior into benign, borderline, or malignant. This article focuses primarily on the role of radiation for malignant soft tissue sarcoma and selected borderline tumors. PMID:12916463

Ballo, Matthew T; Zagars, Gunar K

2003-04-01

223

Notch signaling in pediatric soft tissue sarcomas  

PubMed Central

Pediatric soft tissue sarcomas are rare tumors of childhood, frequently characterized by specific chromosome translocations. Despite improvements in treatment, their clinical management is often challenging due to the low responsiveness of metastatic forms and aggressive variants to conventional therapeutic approaches, which leads to poor overall survival. It is widely thought that soft tissue sarcomas derive from mesenchymal progenitor cells that, during embryonic life, have developed chromosomal aberrations with de-regulation of the main pathways governing tissue morphogenesis. The Notch signaling pathway is one of the most important molecular networks involved in differentiation processes. Emerging evidence highlights the role of Notch signaling de-regulation in the biology of these pediatric sarcomas. In this review, we present an outline of recently gathered evidence on the role of Notch signaling in soft tissue sarcomas, highlighting its importance in tumor cell biology. The potential challenges and opportunities of targeting Notch signaling in the treatment of pediatric soft tissue sarcomas are also discussed. PMID:23158439

2012-01-01

224

Socioeconomic factors and the risk for sarcoma.  

PubMed

Sarcomas are a heterogeneous group of rare malignancies arising from mesenchymal tissue. Although several occupational exposures have been evaluated in association with sarcoma, little is known about the role of socioeconomic indicators such as education. Socioeconomic status has been found to be associated with risk of development of several types of cancers, primarily lung, gastric, and cervical cancers. We conducted a hospital-based case-control study to evaluate the association of socioeconomic level with the risk for sarcoma. A total of 371 incident cases of sarcoma were matched in terms of age, sex, and year of enrollment in the study with 742 cancer-free controls. Education and income levels were evaluated as the indicators of socioeconomic status. Higher education (college level) was associated with a significantly lower risk for sarcoma [odds ratio (OR)=0.48, 95% confidence interval (CI)=0.29-0.80], even after adjusting for important confounders. After stratifying by sex, significantly lower risk for sarcoma was observed among men who had college level education compared with men with a level of education of eighth grade or lower (OR=0.38, 95% CI=0.19-0.74). A significant association between education and the risk for sarcoma remained after stratifying by income (OR=0.49, 95% CI=0.28-0.86, among the low income group). When analyzed as a composite exposure, individuals with high education and high income status had significantly lower risk for sarcoma compared with those with low income and low education status (OR=0.41, 95% CI=0.23-0.71). Thus, socioeconomic factors may play a significant role in determining the risk for sarcoma and should be explored further to elucidate the underlying factors that may explain these sociodemographic inequalities related to sarcoma. PMID:24356343

Hampras, Shalaka S; Moysich, Kirsten B; Marimuthu, Sathiya P; Ravi, Vinod; Jayaprakash, Vijayvel

2014-11-01

225

Diagnosis and treatment of Kaposi's Sarcoma. Oncology overview  

SciTech Connect

Oncology Overviews are a service of the International Cancer Research Data Bank (ICRDB) Program of the National Cancer Institute, intended to facilitate and promote the exchange of information between cancer scientists by keeping them aware of literature related to their research being published by other laboratories throughout the world. Each Oncology Overview represents a survey of the literature associated with a selected area of cancer research. It contains abstracts of articles which have been selected and organized by researchers associated with the field. Contents: Kaposi's sarcoma diagnosis; Kaposi's sarcoma clinical immunology; Kaposi's sarcoma chemotherapy; Kaposi's sarcoma radiotherapy; Kaposi's sarcoma other treatment modalities; Kaposi's sarcoma case reports; Kaposi's sarcoma etiology; Kaposi's sarcoma epidemiology; Kaposi's sarcoma relation to acquired immune deficiency syndrome; Kaposi's sarcoma reviews.

Not Available

1983-11-01

226

Neuronal Differentiation of Synovial Sarcoma and Its Therapeutic Application  

Microsoft Academic Search

Synovial sarcoma is a rare sarcoma of unknown histologic origin. We previously reported the gene expression profile of synovial\\u000a sarcoma was closely related to that of malignant peripheral nerve sheath tumors, and the fibroblast growth factor (FGF) signal\\u000a was one of the main growth signals in synovial sarcoma. Here we further demonstrate the neural origin of synovial sarcoma\\u000a using primary

Tatsuya Ishibe; Tomitaka Nakayama; Tomoki Aoyama; Takashi Nakamura; Junya Toguchida

2008-01-01

227

Reproductive responses following royal jelly treatment administered orally or intramuscularly into progesterone-treated Awassi ewes  

Microsoft Academic Search

An experiment was conducted to determine whether natural royal jelly (RJ) paste administered orally or intramuscularly (i.m.) in conjunction with exogenous progesterone is associated with improved reproductive responses in ewes. Thirty 3–6-year-old Awassi ewes were randomly allocated into three (RJ-capsule, RJC; RJ-injection, RJI and control, CON) groups of 10 ewes each. All ewes were treated with intravaginal progesterone sponges for

M. Q Husein; R. T Kridli

2002-01-01

228

Yellow grease as an alternative energy source for nursing Awassi ewes and their suckling lambs  

Microsoft Academic Search

Thirty Awassi ewes with an initial body weight (BW) of 52.0kg (SD=8.4) nursing single lambs with an initial BW of 6.2kg (SD=1.0) were utilized to study yellow grease, by partially replacing barley, as an alternative energy source. The ewes aged 3–7 years and parity ranged from 2 to 6. All ewes gave birth 6–8 days before starting the experiment. Ewes

M. S. Awawdeh; B. S. Obeidat; R. T. Kridli

2009-01-01

229

Mesenchymal stem cell transformation and sarcoma genesis  

PubMed Central

MSCs are hypothesized to potentially give rise to sarcomas after transformation and therefore serve as a good model to study sarcomagenesis. Both spontaneous and induced transformation of MSCs have been reported, however, spontaneous transformation has only been convincingly shown in mouse MSCs while induced transformation has been demonstrated in both mouse and human MSCs. Transformed MSCs of both species can give rise to pleomorphic sarcomas after transplantation into mice, indicating the potential MSC origin of so-called non-translocation induced sarcomas. Comparison of expression profiles and differentiation capacities between MSCs and sarcoma cells further supports this. Deregulation of P53- Retinoblastoma-, PI3K-AKT-and MAPK pathways has been implicated in transformation of MSCs. MSCs have also been indicated as cell of origin in several types of chromosomal translocation associated sarcomas. In mouse models the generated sarcoma type depends on amongst others the tissue origin of the MSCs, the targeted pathways and genes and the differentiation commitment status of MSCs. While some insights are glowing, it is clear that more studies are needed to thoroughly understand the molecular mechanism of sarcomagenesis from MSCs and mechanisms determining the sarcoma type, which will potentially give directions for targeted therapies. PMID:23880362

2013-01-01

230

Individual Differences, Parasites, and the Costs of Reproduction for Bighorn Ewes (Ovis canadensis)  

E-print Network

Individual Differences, Parasites, and the Costs of Reproduction for Bighorn Ewes (Ovis canadensis-795 INDIVIDUAL DIFFERENCES, PARASITES, AND THE COSTSOF REPRODUCTIONFOR BIGHORN EWES (OVIS CANADENSIS) BY MARCO marked bighorn ewes (Ovis canadensis)were examined over 8 years in south-western Alberta, Canada. (2

Lazzaro, Brian

231

Use of Melengestrol Acetate and Gonadotropins to Induce Fertile Estrus in Seasonally Anestrous Ewes''2  

Microsoft Academic Search

Ewes of three genotypes (Hamp- shire, n = 59; Rambouillet, n = 36; crossbred, n = 57) were used to determine the efficiency of melengestrol acetate (MGA) and(or1 PG-600 (a combination of pregnant mare's serum gonadotropin and human chorionic gonadotropin) in inducing fertile estrus in seasonally anestrus ewes. Ewes were assigned randomly, within geno- type, to treatments in a 2

T. J. Safranski; W. R. Lamberson; D. H. Keisler

2010-01-01

232

UDDER CONFORMATION, MILK YIELD AND MILK FRACTIONATION IN THE DAIRY EWE  

E-print Network

UDDER CONFORMATION, MILK YIELD AND MILK FRACTIONATION IN THE DAIRY EWE R. SAGI M. MORAG Faculty SUMMARY Dairy ewes of the Assaf breed were classified into 4 groups according to udder conformation in dairy sheep farming is the unsatisfactory fractionation of milk yield in machine milked ewes. Machine

Paris-Sud XI, Université de

233

Udder support as a means for improving milk fractionation in dairy ewes  

E-print Network

Udder support as a means for improving milk fractionation in dairy ewes R. SAGI Faculty supporting arm as a means for increasing the machine milk fraction in dairy ewes was tested. Four groups were not affected. Introduction The efficiency of the machine milking of dairy ewes is, quite often

Paris-Sud XI, Université de

234

Responses of dairy ewes before and after parturition, to different nutritional regimes during pregnancy  

E-print Network

Responses of dairy ewes before and after parturition, to different nutritional regimes during pregnancy 1. - Ewe body weight, uterine contents, and lamb birth weight (1) D. STERN J. H. ADLER H. TAGARI E Summary A study was made of the effects of nutritional levels in pregnant dairy ewes in which three basic

Paris-Sud XI, Université de

235

Responses of dairy ewes before and after parturition to different nutritional regimes during pregnancy.  

E-print Network

Responses of dairy ewes before and after parturition to different nutritional regimes during pregnancy. III. — The concentration of some metabolites in the blood during pregnancy D. STERN, J. H of several metabolites in the blood of ewes in late pregnancy are described. The ewes were included in a 3 x

Paris-Sud XI, Université de

236

Rhodotorula minuta fungemia in a ewe lamb.  

PubMed

An 8-month-old crossbred ewe, normal upon physical examination, was humanely euthanized for tissue collection. After approximately 3 weeks in tissue culture, fungi began budding out of cells obtained from the choroid plexus. After an additional 3 weeks, budding was observed in kidney cell cultures and eventually in monocyte cultures as well. Serum from the lamb was submitted to the Veterinary Diagnostic Laboratory at Colorado State University for fungal diagnosis and was found negative for Aspergillus, Blastomyces, Coccidioidomycosis and Histoplasmosis. DNA was isolated from fungi collected from tissue culture supernatants and used in a set of pan-fungal PCR assays with DNA from Candida acting as a positive control. PCR products were sequenced and BLAST analysis performed. The unknown fungal sequence aligned with 100% identity to Rhodotorula minuta an emerging opportunistic pathogen. Samples were submitted to The Fungal Testing Laboratory at The University of Texas Health Science Center at San Antonio for additional validation. We believe this to be the first report of Rhodotorula fungemia in a sheep in the United States. PMID:23347360

Chitko-McKown, C G; Leymaster, K A; Heaton, M P; Griffin, D D; Veatch, J K; Jones, S A; Clawson, M L

2014-12-01

237

Molecular Diagnosis in Ewing Family Tumors  

PubMed Central

The Ewing's family of tumors (EFTs) are characterized by chimeric transcripts generated by specific chromosomal rearrangements. The most common fusions are between the EWSR1 gene on chromosome 22 and the ETS family of transcription factors; rarely, FUS (on chromosome 16) substitutes for EWSR1. The detection of specific translocations using molecular analysis is now a routine part of the pathological examination of EFT. Here, we report our experience with molecular diagnosis of EFT during the 4 years (2006–2009) at the Rizzoli Institute. We analyzed 222 consecutive tumors with a presumptive diagnosis of EFT using molecular techniques and IHC. We found five distinct types of EWSR1-FLI1 fusion transcripts resulting from translocation t(11;22), three types of EWSR1-ERG transcripts resulting from t(21;22), and one type of t(2;22) resulting in EWSR1-FEV fusion. Molecular investigation validated 92% of cases ultimately diagnosed as EFT; IHC validated 76% of the cases. Thus, despite the difficulties and limitations associated with both molecular and IHC analysis on fresh and formalin-fixed, paraffin-embedded tissue, a combination of these techniques is the best approach to enhancing the accuracy of EFT diagnosis. We also present our method for choosing which molecular techniques to apply. Finally, we collected the most prevalent breakpoints reported in the literature, indicating which exons are involved, the sequence breakpoints, and the NCBI reference sequences. PMID:21458383

Gamberi, Gabriella; Cocchi, Stefania; Benini, Stefania; Magagnoli, Giovanna; Morandi, Luca; Kreshak, Jennifer; Gambarotti, Marco; Picci, Piero; Zanella, Licciana; Alberghini, Marco

2011-01-01

238

Kaposi's sarcoma-associated herpesvirus-like DNA sequences (KSHV\\/HHV-8) in oral AIDS-Kaposi's sarcoma  

Microsoft Academic Search

Recently, a new human herpesvirus (KSHV\\/HHV-8) has been identified in classic, transplant, endemic, and AIDS Kaposi's sarcoma that may be involved in the pathogenesis of Kaposi's sarcoma. The purpose of this study was to evaluate oral AIDS-Kaposi's sarcoma for detection of KSHV\\/HHV-8 DNA. DNA extracted from 54 oral AIDS-Kaposi's sarcoma lesions (47 initial, 7 postvinblastine treated), 5 non-Kaposi's sarcoma HIV-positive

Catherine M Flaitz; Ying-Tai Jin; M. John Hicks; C. Mark Nichols; Yih-Weng Wang; Ih-Jen Su

1997-01-01

239

Angiosarcomas and other sarcomas of endothelial origin.  

PubMed

Although benign hemangiomas are among the most common diagnoses among connective tissue tumors, angiosarcomas and other sarcomas arising from blood vessels are rare, even among sarcomas. Because endothelial tumors have unique embryonal derivation compared with other sarcomas, it is not surprising they have unique characteristics. Herein are reviewed some of these unique characteristics and therapeutic options for patients with some of these diagnoses, highlighting the potential of new agents for these tumors, which will in all likelihood also impact treatment on more common cancers. PMID:24093171

Cioffi, Angela; Reichert, Sonia; Antonescu, Cristina R; Maki, Robert G

2013-10-01

240

Therapeutic Angiotensin-(1-7) in Treating Patients With Metastatic Sarcoma That Cannot Be Removed By Surgery  

ClinicalTrials.gov

Bone Cancer; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma

2013-12-10

241

Regulation of endometrial granulocyte macrophage-colony stimulating factor (GM-CSF) in the ewe.  

PubMed

Granulocyte macrophage-colony stimulating factor (GM-CSF) increases ovine interferon-tau (oIFNtau) secretion by ovine conceptuses, but endometrial production of GM-CSF has not been characterized. Endometrial GM-CSF expression was evaluated in ovariectomized ewes implanted with estradiol-17beta (E(2)) and/or progesterone (P(4)) for 14 days, in day 14 cyclic and day 14 pregnant ewes. Relative levels of endometrial GM-CSF mRNA were 3-fold higher in E(2)- and E(2)/P(4)-treated ewes than that of control or P(4)-treated ovariectomized ewes. Levels of endometrial GM-CSF mRNA for cyclic ewes were similar to E(2)- and E(2)/P(4)-treated ewes, but amounts of GM-CSF mRNA in pregnant ewes were 2-fold higher. GM-CSF concentrations in endometrial culture media, determined by GM-CSF bioassay, for cyclic and E(2)/P(4)-treated ovariectomized ewes were 3-fold higher than those of control, E(2)- and P(4)-treated ovariectomized ewes; however, amounts of GM-CSF in pregnant ewes were 2-fold higher. Immunoreactive GM-CSF, examined by western blot, was detected in the culture medium from E(2)/P(4)-treated ovariectomized, cyclic and pregnant ewes. Luminal and glandular epithelia and stromal regions were determined to be sites of GM-CSF expression by immunohistochemistry and in situ hybridization techniques. Data indicate that combined E(2) and P(4) treatment of ovariectomized ewes is sufficient to restore GM-CSF expression to the level found in cyclic ewes; however, GM-CSF mRNA and protein in pregnant ewes is 2-fold greater than in ovariectomized or cyclic ewes. These data suggest that the conceptus, in addition to steroids, may play a role in the regulation of endometrial production of GM-CSF. PMID:12206872

McGuire, W J; Imakawa, K; Tamura, K; Meka, C S R; Christenson, R K

2002-10-01

242

Ziv-aflibercept in Treating Patients With Locally Advanced, Unresectable, or Metastatic Gynecologic Soft Tissue Sarcoma  

ClinicalTrials.gov

Fallopian Tube Cancer; Female Reproductive Cancer; Ovarian Carcinosarcoma; Ovarian Sarcoma; Recurrent Ovarian Epithelial Cancer; Recurrent Uterine Sarcoma; Stage III Ovarian Epithelial Cancer; Stage III Uterine Sarcoma; Stage IV Ovarian Epithelial Cancer; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

2013-05-07

243

Protection of Ewing’s sarcoma family tumor (ESFT) cell line SK-N-MC from betulinic acid induced apoptosis by ?- dl-tocopherol  

Microsoft Academic Search

Betulinic acid (BA) is known to induce apoptosis in melanoma neuroectodermal and malignant brain cancer cell lines. Present report describes the role of antioxidants on the BA-induced toxicity to human cell line SK-N-MC. Hydrophilic antioxidants viz., l-ascorbic acid (VitC) and N-acetyl-l-cysteine (l-NAC) had no protective effect on BA-induced apoptosis at the maximal concentrations tested. The lipophilic antioxidant, ?-dl-tocopherol (VitE) showed

D. V. Raghuvar Gopal; A. A. Narkar; Y. Badrinath; K. P. Mishra; D. S. Joshi

2004-01-01

244

Clinical mastitis in ewes; bacteriology, epidemiology and clinical features  

PubMed Central

Background Clinical mastitis is an important disease in sheep. The objective of this work was to identify causal bacteria and study certain epidemiological and clinical features of clinical mastitis in ewes kept for meat and wool production. Methods The study included 509 ewes with clinical mastitis from 353 flocks located in 14 of the 19 counties in Norway. Clinical examination and collection of udder secretions were carried out by veterinarians. Pulsed-field gel electrophoresis (PFGE) was performed on 92 Staphylococcus aureus isolates from 64 ewes. Results and conclusion S. aureus was recovered from 65.3% of 547 clinically affected mammary glands, coagulase-negative staphylococci from 2.9%, enterobacteria, mainly Escherichia coli, from 7.3%, Streptococcus spp. from 4.6%, Mannheimia haemolytica from 1.8% and various other bacteria from 4.9%, while no bacteria were cultured from 13.2% of the samples. Forty percent of the ewes with unilateral clinical S. aureus mastitis also had a subclinical S. aureus infection in the other mammary gland. Twenty-four of 28 (86%) pairs of S. aureus isolates obtained from clinically and subclinically affected mammary glands of the same ewe were indistinguishable by PFGE. The number of identical pairs was significantly greater than expected, based on the distribution of different S. aureus types within the flocks. One-third of the cases occurred during the first week after lambing, while a second peak was observed in the third week of lactation. Gangrene was present in 8.8% of the clinically affected glands; S. aureus was recovered from 72.9%, Clostridium perfringens from 6.3% and E. coli from 6.3% of the secretions from such glands. This study shows that S. aureus predominates as a cause of clinical ovine mastitis in Norway, also in very severe cases. Results also indicate that S. aureus is frequently spread between udder halves of infected ewes. PMID:17892567

M?rk, Tormod; Waage, Steinar; Tollersrud, Tore; Kvitle, Bj?rg; Sviland, Stale

2007-01-01

245

Phase II Study of High-Dose Photon/Proton Radiotherapy in the Management of Spine Sarcomas  

SciTech Connect

Purpose: Radiotherapy (XRT) for spine sarcomas is constrained by spinal cord, nerve, and viscera tolerance. Negative surgical margins are uncommon; hence, doses of {>=}66 Gy are recommended. A Phase II clinical trial evaluated high-dose photon/proton XRT for spine sarcomas. Methods and Materials: Eligible patients had nonmetastatic, thoracic, lumbar, and/or sacral spine/paraspinal sarcomas. Treatment included pre- and/or postoperative photon/proton XRT with or without radical resection; patients with osteosarcoma and Ewing's sarcoma received chemotherapy. Shrinking fields delivered 50.4 cobalt Gray equivalent (Gy RBE) to subclinical disease, 70.2 Gy RBE to microscopic disease in the tumor bed, and 77.4 Gy RBE to gross disease at 1.8 Gy RBE qd. Doses were reduced for radiosensitive histologies, concurrent chemoradiation, or when diabetes or autoimmune disease present. Spinal cord dose was limited to 63/54 Gy RBE to surface/center. Intraoperative boost doses of 7.5 to 10 Gy could be given by dural plaque. Results: A total of 50 patients (29 chordoma, 14 chondrosarcoma, 7 other) underwent gross total (n = 25) or subtotal (n = 12) resection or biopsy (n = 13). With 48 month median follow-up, 5-year actuarial local control, recurrence-free survival, and overall survival are: 78%, 63%, and 87% respectively. Two of 36 (5.6%) patients treated for primary versus 7/14 (50%) for recurrent tumor developed local recurrence (p < 0.001). Five patients developed late radiation-associated complications; no myelopathy developed but three sacral neuropathies appeared after 77.12 to 77.4 Gy RBE. Conclusions: Local control with this treatment is high in patients radiated at the time of primary presentation. Spinal cord dose constraints appear to be safe. Sacral nerves receiving 77.12-77.4 Gy RBE are at risk for late toxicity.

DeLaney, Thomas F. [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States)], E-mail: tdelaney@partners.org; Liebsch, Norbert J. [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); Pedlow, Francis X. [Department of Orthopedic Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); Adams, Judith; Dean, Susan [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); Yeap, Beow Y. [Department of Biostatistics, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); McManus, Patricia [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); Rosenberg, Andrew E.; Nielsen, G. Petur [Department of Pathology, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); Harmon, David C. [Department of Medicine, Division of Hematology Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); Spiro, Ira J. [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); Raskin, Kevin A. [Department of Orthopedic Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); Suit, Herman D. [Department of Radiation Oncology, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); Yoon, Sam S. [Department of Surgery (Section of Surgical Oncology), Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States); Hornicek, Francis J. [Department of Orthopedic Surgery, Massachusetts General Hospital, Harvard Medical School, Boston, MA (United States)

2009-07-01

246

Drugs Approved for Soft Tissue Sarcoma  

Cancer.gov

This page lists cancer drugs approved by the Food and Drug Administration (FDA) for soft tissue sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

247

Nutritional status of adult ewes during early and mid-pregnancy. 2. Effects of supplementation with selenised yeast on ewe reproduction and offspring performance to weaning.  

PubMed

The objective of this study was to determine the effects of selenium (Se) supplementation of mature ewes in the period from day -14 to day 90 post mating on Se status, productivity and viability of ewes and their offspring. Multiparous crossbred ewes (n = 82) were randomly assigned to receive a standard dried grass-based diet (control) or dried grass diet supplemented with 1 g of selenised yeast (Selplex®), providing 0.5 mg Se per ewe per day. After day 90 post mating, all ewes were offered grass-based diets supplemented with a standard multivitamin and mineral mix, up to lambing. Ewes that were fed additional Se had increased (P < 0.001) activity of glutathione peroxidase enzyme (GPx) and increased plasma Se concentrations throughout the treatment period. At lambing, GPx activity of supplemented ewes was higher (P < 0.001) than that of controls. Twenty-four hours after birth, lambs of ewes given Se had higher (P < 0.001) concentration of Se in plasma, greater (P < 0.001) activity of GPx and better (P < 0.01) immune status. Lambs of ewes that received supplementation showed a faster progression to stand than control lambs (P < 0.05), independent of maternal behaviour which was not affected. Supplementation reduced perinatal lamb mortality (0.04 v. 0.17; P < 0.05). However, overall mortality from birth to weaning was unaffected (P > 0.05). Supplemented ewes weaned lambs on average 2 kg heavier than control ewes, due to the higher (P < 0.05) growth rates achieved by their offspring. In conclusion, for ewes with a marginal Se status, an organic source of Se supplemented throughout pregnancy, compared with supplementation only in the last trimester, positively affected measures of lamb viability and survival. PMID:22444964

Muñoz, C; Carson, A F; McCoy, M A; Dawson, L E R; O'Connell, N E; Gordon, A W

2008-01-01

248

Primary epithelioid sarcoma of the oesophagus  

Microsoft Academic Search

Epithelioid sarcoma is a rare soft tissue tumour presenting two main variants: the ‘classical’ distal type and the more recently\\u000a described proximal type. The latter is distinguished from the former by occurrence in elderly patients, more axial and deep\\u000a location, prominent atypical and pleomorphic appearance and eventually aggressive clinical behaviour with poor outcome. To\\u000a date, only few perivisceral epithelioid sarcomas

Francesca Maggiani; Maria Debiec-Rychter; Nadine Ectors; Antoon Lerut; Raf Sciot

2007-01-01

249

NY-ESO-1 expression in sarcomas  

PubMed Central

NY-ESO-1 (CTAG 1B) is highly expressed in the majority of synovial sarcomas and myxoid/round cell liposarcomas as well as in a subset of melanomas, but only rarely in other mesenchymal tumors. This points to a potential for using NY-ESO-1 in the differential diagnosis of these lesions. Furthermore, promising results have been obtained in clinical trials testing NY-ESO-1-targeted immunotherapy in subsets of melanoma and synovial sarcoma patients. PMID:23243610

Lai, Jin-Ping; Rosenberg, Avi Z.; Miettinen, Markku M.; Lee, Chyi-Chia R.

2012-01-01

250

Bronchopulmonary Kaposi's sarcoma in patients with AIDS.  

PubMed Central

BACKGROUND: Kaposi's sarcoma in HIV antibody positive patients may affect the lungs. This study describes the presentation, chest radiographic appearances, and pulmonary function test abnormalities in patients with AIDS who had tracheobronchial Kaposi's sarcoma. METHODS AND RESULTS: Twenty nine (8%) of 361 consecutive HIV antibody positive patients undergoing bronchoscopy for respiratory symptoms had tracheobronchial Kaposi's sarcoma. Eight patients had intercurrent infections and one had previously received chemotherapy for cutaneous Kaposi's sarcoma; these patients were excluded. Seven of the remaining 20 patients had localised Kaposi's sarcoma (lesions confined to the trachea or the subsegments of one lobe) and 13 had widespread Kaposi's sarcoma (affecting the trachea and one lobe or the subsegments of more than one lobe); 19 patients also had cutaneous and palatal Kaposi's sarcoma. Seven patients, four with widespread disease, had a normal radiograph. All patients had reduced transfer factor (TLCO) and transfer coefficient (KCO) but only those with widespread disease had reductions in forced expiratory volume in one second (FEV1), forced vital capacity (FVC), and peak expiratory flow (PEF). Follow up pulmonary function testing in seven patients (median three months later) showed further reductions in TLCO. All four patients who received no treatment had progressive radiographic abnormalities; bronchoscopy in two patients showed progressive tracheobronchial disease, and two patients had further reductions in FEV1 and FVC. In three patients treated with chemotherapy palliation of symptoms was achieved but two had further reductions in FEV1 and FVC and the radiograph deteriorated. Bronchoscopy showed regression of disease in only one patient. CONCLUSION: Pulmonary Kaposi's sarcoma produces abnormalities of TLCO even in patients with localised disease; airflow obstruction may occur in patients with widespread disease. Bronchoscopic reassessment of the extent of disease may not accurately reflect response to chemotherapy. PMID:1440467

Miller, R F; Tomlinson, M C; Cottrill, C P; Donald, J J; Spittle, M F; Semple, S J

1992-01-01

251

Primary synovial sarcoma of lung  

PubMed Central

A synovial sarcoma (SS) is a rare form of cancer which usually occurs near the joints of the arm, neck, or leg, but has been documented in most human tissues and organs, including the brain, prostate, and heart. Primary pulmonary SS is an extremely rare tumor. We report a case of primary SS of lung who presented with severe chest pain and a large right lung mass with right-sided pleural effusion in computed tomography (CT) scan of thorax. The diagnosis was made on the basis of CT-guided core biopsy and immunohistochemistry. On immunohistochemistry, tumor cell expressed epithelial membrane antigen, bcl 2, Vimentin and smooth muscle actin and were immunonegative for S100 and cytokeratin. So, the final diagnosis was primary SS. PMID:25125819

Devleena; Bansal, Vikram; Chaudhuri, Tamohan; Roy, Shravasti

2014-01-01

252

Isolated Limb Perfusion of Melphalan With or Without Tumor Necrosis Factor in Treating Patients With Soft Tissue Sarcoma of the Arm or Leg  

ClinicalTrials.gov

Stage IVB Adult Soft Tissue Sarcoma; Stage IIB Adult Soft Tissue Sarcoma; Stage IIC Adult Soft Tissue Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Stage IVA Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma

2012-03-14

253

Does mass change of primiparous bighorn ewes reflect reproductive effort?  

E-print Network

analyzed 26 years of data on marked bighorn (Ovis canadensis) ewes to assess how variation in first�es de Mouflons d'Am�rique (Ovis canadensis), nous avons v�rifi� de quelle mani�re l'effort reproducteur

Festa-Bianchet, Marco

254

Bighorn ewes transfer the costs of reproduction to their lambs.  

PubMed

Several studies of large mammals report no direct reproductive costs for females. Individual heterogeneity may hide fitness costs of reproduction, but mothers could also transfer some costs to their offspring. Using data on 442 lambs weaned by 146 bighorn sheep (Ovis canadensis) ewes at Ram Mountain, Alberta, we studied how reproductive effort varied with environmental and maternal conditions. During summer, lactating ewes should gain enough mass to survive the winter and to support their next gestation, while nursing their current lamb. We measured reproductive effort as summer mass gain by lambs corrected for maternal mass in June and maternal mass gain during summer. Females lowered their reproductive effort when population density increased and if they had weaned a lamb the previous year. A reduction in reproductive effort led to lower winter survival by lambs. Bighorn ewes have a conservative reproductive tactic and always favor their own body condition over that of their lambs. When resources are limited, ewes appear to transfer reproductive costs to their lambs, as expected from the much greater relative fitness consequences of a reduction in maternal than in offspring survival. PMID:20735260

Martin, Julien G A; Festa-Bianchet, Marco

2010-10-01

255

and Christina C. Leslie Bonventre, Heather Ewing, Michael H. Gelb  

E-print Network

and Christina C. Leslie Bonventre, Heather Ewing, Michael H. Gelb V.Benjamin F. Cravatt, Ku-Lung Hsu, Joseph Bogeon Yun, HeeJung Lee, Moumita Ghosh, Permeability Transition Pore Formation Overload formation* Bogeon Yun1 , HeeJung Lee1 , Moumita Ghosh1 , Benjamin F. Cravatt2 , Ku-Lung Hsu2 , Joseph V

Gelb, Michael

256

Mixed grazing trial with suckling cows and dry pregnant ewes  

E-print Network

Mixed grazing trial with suckling cows and dry pregnant ewes N Grenet1 J Billant2 'Institut de l'Elevage, Theix, 63122 St-Genès-Champanelle ; 2LEGTA, 52903 Chaumont Cedex 9, France Mixed grazing of sheep be profitable if suckled calves' growth is maintained or improved. During three successive grazing seasons (from

Paris-Sud XI, Université de

257

Influence of supplemental monensin on gestating and lactating ewes  

E-print Network

were grouped into singles and twins by sonogram results, allotted to pens in pairs and assigned to either an ionophore (diet I with 70 mg daily) or non-ionophore (diet 2) treatment groups in a 2 x 2 factorial arrangement. All ewes were fed according...

Peel, Richard Kraig

2012-06-07

258

Induction of cervical dilation for transcervical embryo transfer in ewes  

PubMed Central

Background A major limitation in the application of assisted reproductive technologies in sheep arises from the inability to easily traverse the uterine cervix. The cervix of the non-pregnant ewe is a narrow and rigid structure, with 5–7 spiral folds and crypts that block its lumen. The first two folds closest to the vagina appear to be the greatest obstacle for the instrument insertion into the sheep cervix. Therefore, the dilation of the distal part of the cervix could provide the conformational change necessary to perform non-invasive transcervical procedures. The present study set out to assess the efficacy of Cervidil®, a patented dinoprostone (PgE2)-containing vaginal insert with a controlled-release mechanism, to safely induce sufficient cervical dilation for the purpose of transcervical embryo transfer (TCET) in cyclic ewes. Methods The transfer of frozen-thawed ovine embryos was attempted in 22 cross-bred Rideau Arcott x Polled Dorset ewes, with or without the pre-treatment with Cervidil® for 12 or 24 h prior to TCET. Results Cervical penetration rate was significantly improved after Cervidil® pre-treatment, with 55% (6/11) of treated versus 9% (1/11) of control animals successfully penetrated (?2-test, p?ewes that were penetrated, 67% (4/6) had been exposed to Cervidil(R) for 24 h and 33% (2/6) had had a 12-h exposure (p?>?0.05). Variations in the age, weight, genotype, parity, lifetime lamb production (LLP) and post-partum interval (PPI) between penetrated and non-penetrated ewes were not significant (p?>?0.05). The time taken to traverse the uterine cervix was negatively correlated (p?ewes, but no fetuses were detected ultrasonographically 55 days post-TCET. Conclusions The present results indicate a significant benefit of using Cervidil® for inducing cervical dilation during the mid-luteal phase in ewes but the reason(s) for impaired fertility after the transfer of frozen-thawed ovine embryos remains to be elucidated. PMID:24467737

2014-01-01

259

Establishment of a novel clear cell sarcoma cell line (Hewga-CCS), and investigation of the antitumor effects of pazopanib on Hewga-CCS  

PubMed Central

Background Clear cell sarcoma (CCS) is a therapeutically unresolved, aggressive, soft tissue sarcoma (STS) that predominantly affects young adults. This sarcoma is defined by t(12;22)(q13;q12) translocation, which leads to the fusion of Ewing sarcoma gene (EWS) to activating transcription factor 1 (ATF1) gene, producing a chimeric EWS-ATF1 fusion gene. We established a novel CCS cell line called Hewga-CCS and developed an orthotopic tumor xenograft model to enable comprehensive bench-side investigation for intensive basic and preclinical research in CCS with a paucity of experimental cell lines. Methods Hewga-CCS was derived from skin metastatic lesions of a CCS developed in a 34-year-old female. The karyotype and chimeric transcript were analyzed. Xenografts were established and characterized by morphology and immunohistochemical reactivity. Subsequently, the antitumor effects of pazopanib, a recently approved, novel, multitargeted, tyrosine kinase inhibitor (TKI) used for the treatment of advanced soft tissue sarcoma, on Hewga-CCS were assessed in vitro and in vivo. Results Hewga-CCS harbored the type 2 EWS-ATF1 transcript. Xenografts morphologically mimicked the primary tumor and expressed S-100 protein and antigens associated with melanin synthesis (Melan-A, HMB45). Pazopanib suppressed the growth of Hewga-CCS both in vivo and in vitro. A phospho-receptor tyrosine kinase array revealed phosphorylation of c-MET, but not of VEGFR, in Hewga-CCS. Subsequent experiments showed that pazopanib exerted antitumor effects through the inhibition of HGF/c-MET signaling. Conclusions CCS is a rare, devastating disease, and our established CCS cell line and xenograft model may be a useful tool for further in-depth investigation and understanding of the drug-sensitivity mechanism. PMID:24946937

2014-01-01

260

What's New in Kaposi Sarcoma Research and Treatment?  

MedlinePLUS

... Next Topic Additional resources for Kaposi sarcoma What’s new in Kaposi sarcoma research and treatment? A great ... once it has developed. Treatment Researchers are studying new and different ways to treat KS. Imiquimod (Aldara) ...

261

Progesterone administration reduces the behavioural and physiological responses of ewes to abrupt weaning of lambs.  

PubMed

Abrupt weaning, a usual management in sheep productive systems, may provoke behavioural and physiological responses indicative of stress in ewes and lambs. Progesterone (P4) has anxiolytic and sedative effects through the union of its metabolites that contain 3?-hydroxyl group to the ?-aminobutyricacidA receptor. Our first aim was to determine whether P4 administration reduces the behavioural and physiological responses of ewes to abrupt weaning of lambs. A complementary aim was to determine whether P4 treatment affects the milk yield and composition of ewes, and the BW of their lambs. In experiment 1, seven ewes received P4 treatment for 32 days (group E1-P4), and eight ewes remained as an untreated control group (group E1-C). BW of the lambs was recorded during P4 treatment. Lambs were weaned at 59 days (Day 0 = weaning). The main behaviours of the ewes before and after weaning were recorded using 10 min scan sampling. The ewes' serum total protein, albumin and globulin concentrations were measured before and after weaning of the lambs. In experiment 2, milk yield and composition were determined in two different groups of six ewes treated with P4 (group E2-P4) for 16 days and in five untreated controls (group E2-C). The BW of lambs increased with time (P = 0.001) in both groups and did not differ. The percentage of observations in which the ewes were seen pacing on Day 0 was greater in the E1-C group than in the E1-P4 group (P = 0.0007). Similarly, the percentage of observations in which the ewes were recorded vocalizing on Day 0 was greater in the E1-C group than in the E1-P4 group (P = 0.04). The percentage of observations in which E1-C ewes were recorded lying did not change from Days 0 to 1; however, it increased in E1-P4 ewes. Total serum protein concentration did not change in E1-P4 ewes from Days 0 to 3, although a decrease was seen in E1-C ewes (P = 0.04). Serum globulin concentration was greater in E1-P4 ewes on Day 3 than in E1-C ewes (P = 0.0008). In experiment 2, there were no differences between E2-P4 and E2-C ewes in terms of milk yield, protein, fat and lactose content. Progesterone administration reduced the behavioural and physiological responses of ewes to abrupt weaning of lambs, and this effect was not mediated by changes in milk yield and composition, or by lambs' BW. PMID:23597263

Freitas-de-Melo, A; Banchero, G; Hötzel, M J; Damián, J P; Ungerfeld, R

2013-08-01

262

Pregnancy rates and gravid uterine parameters in single, twin and triplet pregnancies in naturally bred ewes and ewes after transfer of in vitro produced embryos  

Microsoft Academic Search

The objectives of this study were to: (1) evaluate the pregnancy rates after transfer of embryos produced in the presence or absence of epidermal growth factor (EGF) during in vitro maturation, and (2) compare several variables of the gravid uterus on day 140 after fertilization in single, twin and triplet pregnancies in ewes (n=12) bred naturally and in ewes (n=18)

Anna T. Grazul-Bilska; Disha Pant; Justin S. Luther; Pawel P. Borowicz; Chainarong Navanukraw; Joel S. Caton; Marcy A. Ward; Dale A. Redmer; Lawrence P. Reynolds

2006-01-01

263

Isolation of 16L Virus: A Rapidly Transforming Sarcoma Virus from an Avian Leukosis Virus-Induced Sarcoma  

Microsoft Academic Search

We have isolated a replication-defective rapidly transforming sarcoma virus (designated 16L virus) from a fibro-sarcoma in a chicken infected with td107A, a transformation-defective deletion mutant of subgroup A Schmidt-Ruppin Rous sarcoma virus. 16L virus transforms fibroblasts and causes sarcomas in infected chickens within 2 wk. Its genomic RNA is 6.0 kilobases and contains sequences homologous to the transforming gene (fps)

Benjamin G. Neel; Lu-Hai Wang; Bernard Mathey-Prevot; Teruko Hanafusa; Hidesaburo Hanafusa; William S. Hayward

1982-01-01

264

The Inter-Regional Epidemiological Study of Childhood Cancer (IRESCC): case control study of children with bone and soft tissue sarcomas.  

PubMed Central

The Inter-Regional Epidemiological Study of Childhood Cancer included 43 cases of soft tissue and 30 cases of bone sarcomas, together with their 146 matched controls. Analysis of a wide range of aetiological factors revealed few risk factors relating to events during the index pregnancy, the earlier medical experiences of the case child, or parental medical, occupational and smoking history. Associations which did emerge included: lower birth weight in children with Ewing's tumour, an excess of mothers of children with soft tissue sarcoma with symptoms of toxaemia in pregnancy; and more children with rhabdomyosarcoma who received antibiotics soon after birth. There was some evidence that mothers of children with soft tissue sarcoma may have had reduced fertility with a significant excess of the case mothers having no other pregnancies. Slight excesses of congenital malformations in the case children and of malignant and benign/borderline neoplastic disease in the older mothers were consistent with the existence of a degree of genetic predisposition in the development of the tumours in this series. PMID:3224086

Hartley, A. L.; Birch, J. M.; McKinney, P. A.; Teare, M. D.; Blair, V.; Carrette, J.; Mann, J. R.; Draper, G. J.; Stiller, C. A.; Johnston, H. E.

1988-01-01

265

Primary cardiac sarcoma after breast cancer.  

PubMed

Primary cardiac sarcomas are rare tumours carrying poor prognosis. Postradiation sarcoma has been reported in patients with breast, cervical and head and neck cancers. We report a case of a 56-year-old woman with stage IIA breast cancer diagnosed in 1997, submitted to mastectomy, adjuvant chemotherapy, radiotherapy and hormonotherapy. Pulmonary metastasis were detected in 2008 and treated with chemotherapy and hormonotherapy, being in complete remission since August 2009. She was admitted in December 2009 with a 3-week history of fever, dyspnoea, polyarthralgias and leg oedema. An echocardiography showed a mass in the left atrium. She was submitted to a surgical tumour resection and the histology revealed a sarcoma of intermediate degree of differentiation. Chemoradiation therapy was started and she remains alive after 3 years, without tumour regrowth or metastasis. This case is a therapeutic challenge, because the previous therapies for breast cancer hampered the options for extra chemoradiation therapy. PMID:23608855

Ramalho, Joana; Nunes, Sandra; Marques, Irene; Marques, Franklim

2013-01-01

266

Constitutive Activation of Neuregulin/ERBB3 Signaling Pathway in Clear Cell Sarcoma of Soft Tissue1  

PubMed Central

Abstract Clear cell sarcoma of soft tissue (CCSST) represents a highly malignant tumor of the musculoskeletal system that is characterized by the chromosomal translocation t(12;22)(q13;q12) of the Ewing sarcoma gene (EWSR1) and activating transcription factor 1 (ATF1). In a former microarray expression study, we identified ERBB3, a member of the epidermal growth factor receptor (EGFR) family, as a promising new diagnostic marker in the differential diagnosis of CCSST. Here we show that, besides ErbB3, all CCSST cell lines (n = 8) also express the ErbB2 receptor or the ErbB4 receptor, representing an adequate coreceptor of ErbB3. The phosphorylation status of ErbB3 revealed these receptor pairs to be either constitutively activated in CCSST cells with high neuregulin-1 (NRG1) expression (n = 4) or activatable by exogenic NRG1 in cells showing low amounts of NRG1 mRNA (n = 4). Exogenous NRG1 stimulated the growth of a subset of CCSST cells but did not affect the kinetics of another subset. This difference was not strictly dependent on endogenous NRG1 expression; however, the growth-inhibiting effect of the pan-ErbB tyrosine kinase inhibitor CI-1033 or PD158780 clearly correlated with NRG1 expression indicating an autocrine growth stimulation loop, which may constitute an interesting target of new therapeutic strategies in this tumor entity. PMID:16867224

Braun, Yvonne; Diallo, Raihanatou; Wai, Daniel H.; Zahn, Susanne; Schneider, Dominik T; Kuhnen, Cornelius; Vollmann, Arabel; Brockhoff, Gero; Gabbert, Helmut E.; Poremba, Christopher

2006-01-01

267

Observation, Radiation Therapy, Combination Chemotherapy, and/or Surgery in Treating Young Patients With Soft Tissue Sarcoma  

ClinicalTrials.gov

Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

2014-09-08

268

[Surgical therapy of abdominal and trunk soft tissue sarcomas].  

PubMed

Soft tissue sarcomas are rare tumours of mesenchymal cells which can arise in all parts of the body. Sarcomas of the trunk and abdominal cavity are a very heterogeneous group of tumours. This review focuses on retroperitoneal sarcomas, which tend to be asymptomatic for a long time, resulting in an advanced stage at presentation in most patients. Surgical resection is the basis for cure in these patients, possibly combined with radiation. In contrast to extremity sarcomas, the majority of patients with retroperitoneal sarcomas succumb to an isolated local recurrence of the disease. In case of local recurrence or distant metastases, surgical resection should again be contemplated. PMID:19221701

Schimmack, S; Büchler, M W; Weitz, J

2009-03-01

269

Multimodality therapy for metastatic sarcomas confined to the lung  

PubMed Central

Metastectomy or resection of sarcomas which have metastasized to the lung from other sites has a long and established history. At present, there are more than forty different drugs with activity in soft tissue sarcomas. A number of sarcomas demonstrate differential sensitivities to chemotherapy and targeted agents. Intimate knowledge of the biological behavior of each distinct type of sarcoma should predicate what treatment or protocol is most suitable. Certain patients might benefit from either neoadjuvant or adjuvant therapy following the resection of metastatic lesions. Much remains to be learned about the differential sensitivities of various sarcomas to different treatment regimens. PMID:23205068

GOLLARD, RUSSELL P.; TURNER, J. FRANCIS

2012-01-01

270

RUMEN-PROTECTED BYPASS FAT FOR DAIRY EWE COMMERCIAL MILK PRODUCTION  

Microsoft Academic Search

The effects of fat supplementation and weaning system on commercial milk yield and milk composition were determined on 129 East Friesian crossbred ewes. Prior to lambing, ewes were randomly assigned to one of two weaning systems. The DY1 system involved weaning of ewes from their lambs within 24 to 36 hr post-partum and then twice-daily machine milking. In the MIX

Brett C. McKusick; Yves M. Berger; David L. Thomas

271

Comparison of Several Enzymes and Components of Milk, Blood, and Tissues Between Cows and Ewes1  

Microsoft Academic Search

Two ewes and three cows were compared in certain biochemical parameters which might help to explain the greater content of protein and fat in milk of the ewe. Ewe's milk contained 1.25 times as much protein as cow's milk. The RNA\\/DNA ratio, )encine incorporation, acetate supply, and substrate supply (a-amino nitrogen) all were cousistent with higher levels of protein in

I. M. Yousef; R. S. Emery; E. W. Askew; J. D. Benson; J. W. Thomas; J. T. Huber

1969-01-01

272

Immunization against androstenedione and biological activity of luteinizing hormone in the ewe  

E-print Network

Introduction Materials and Methods Results Discussion . 15 . 18 . 28 . 36 IV CONCLUSION REFERENCES APPENDIX . 44 . 53 VITA . 72 vj1 LIST OF FIGURES Figure Page The effects of active immunization of ewes against androstenedione (A) or BSA (C... ewes, 4 yr of age, were randomly assigned to one of four groups described below. The ewes were main- tained under range conditions at the Winters Ranch in Brady, Texas . Pre aration of the Immuno en Control animals received bovine serum albumin (BSA...

Norris, Therese Ann

2012-06-07

273

Molecular piracy of Kaposi's sarcoma associated herpesvirus.  

PubMed

Kaposi's Sarcoma associated Herpesvirus (KSHV) is the most recently discovered human tumor virus and is associated with the pathogenesis of Kaposi's sarcoma, primary effusion lymphoma, and Multicentric Casttleman's disease. KSHV contains numerous open reading frames with striking homology to cellular genes. These viral gene products play a variety of roles in KSHV-associated pathogenesis by disrupting cellular signal transduction pathways, which include interferon-mediated anti-viral responses, cytokine-regulated cell growth, apoptosis, and cell cycle control. In this review, we will attempt to cover our understanding of how viral proteins deregulate cellular signaling pathways, which ultimately contribute to the conversion of normal cells to cancerous cells. PMID:11325605

Choi, J; Means, R E; Damania, B; Jung, J U

2001-01-01

274

Effect of plane of nutrition of 1- and 2-year-old ewes in early and mid-pregnancy on ewe reproduction and offspring performance up to weaning.  

PubMed

The objective of the present study was to determine the effect of plane of nutrition in early pregnancy (EP) and mid-pregnancy (MP), on the productive performance of 1- and 2-year-old ewes and their offspring. Over 2 successive years, between days 0 and 39 after synchronized mating (EP), 1- (n = 117) and 2- (n = 52) year-old ewes were allowed 60% (low, L-EP), 100% (medium, M-EP) or 200% (high, H-EP) of requirements for maintenance (M). Between days 40 and 90 (MP), 1-year-old ewes were allowed 140% (M-MP) or 200% (H-MP), while 2-year-old ewes were allowed 80% (M-MP) or 140% (H-MP) of their M requirement. After day 90, all ewes were fed to meet requirements for late pregnancy. Increasing the plane of nutrition between days 0 and 39 resulted in increases in live weight (LW) (P < 0.001) and body condition score (BCS) (P < 0.001) during the EP period (H-EP > M-EP > L-EP), differences that in 1-year-old ewes were sustained to lambing (P < 0.05). On day 42 of gestation H-EP ewes had lower plasma progesterone concentrations than L-EP or M-EP ewes in 1- (P < 0.01) and 2- (P < 0.001) year olds. This was concomitant with diet H-EP tending to reduce the number of lambs born per ewe in both age groups (P = 0.06 and 0.07, respectively). Foetuses from 1-year-old L-EP ewes had smaller cranial (P < 0.01) and abdominal (P < 0.05) diameters at day 53 of gestation, with H-EP lambs tending to be heaviest at birth (P = 0.07). Similar findings were recorded for 2-year-old ewes. One-year-old ewes offered diet L-EP presented negative maternal behaviours more frequently (P < 0.05), while the incidence of lamb mortality at 6 weeks tended to be greater for L-EP lambs (P = 0.07). In MP, 1-year-old ewes offered diet M-MP were associated with foetuses with bigger abdominal diameters at day 78 (P < 0.05). However, there were no differences in lamb weight or size at term (P > 0.05). These ewes exhibited more positive maternal behaviours (e.g. increased grooming frequency and duration; P < 0.05) than ewes offered diet H-MP, and their offspring were more successful in suckling (P < 0.05). Results suggest that in young ewes, a temporary nutrient restriction in EP resulted in increased prolificacy. However, ewes and their offspring were lighter at birth and ewe maternal behaviour was poorer, resulting in increased lamb mortality. In MP, a medium plane of nutrition offered to 1-year-old ewes led to improved maternal and offspring behaviour. PMID:22444443

Muñoz, C; Carson, A F; McCoy, M A; Dawson, L E R; O'Connell, N E; Gordon, A W

2009-05-01

275

Effect of Fluorogestone Acetate (cronolone) on the performance of fine-wool ewes  

E-print Network

. &"" One ewe was not bred, ass Includes 3 embryos, ova recovery from thin and/or old ewes, by excision of the tubes, was of limited success. In other words, recovery was made from the uterine horns following hysterectomy. This was not the case in young.... &"" One ewe was not bred, ass Includes 3 embryos, ova recovery from thin and/or old ewes, by excision of the tubes, was of limited success. In other words, recovery was made from the uterine horns following hysterectomy. This was not the case in young...

Ayachi, Salah

2012-06-07

276

Thermoregulation of nutrient-restricted hair ewes subjected to heat stress during late pregnancy.  

PubMed

In summers with severe hot environmental temperatures, thermoregulation is expected to be critical during the last third of gestation of hair ewes, and this effect can be overstated by suboptimal feeding conditions. Twenty-four multiparous hair sheep ewes with approximately 100d of pregnancy were randomly assigned to two treatments to evaluate the effects of nutritional restriction (NR) during late gestation on some serum metabolites, physiological variables, and thyroid hormone concentrations under intense heat load (maximum temperature 42-45°C). Treatments were as follows: (1) control, ewes fed free choice wheat straw plus a concentrate at a rate of 500g/d, and (2) nutritionally-restricted (NR) ewes, which were offered wheat straw only ad libitum. Body weight of NR ewes dropped 5.1kg (P<0.05), while control ewes gained 4.7kg during the study period. Rectal temperature was not affected (P>0.05) by feeding regime either in the morning or afternoon hours. Respiration rate was greater (P<0.05) in control ewes (20 and 40breaths/min higher in the day and night, respectively) on d 130 and 145 of pregnancy compared to NR ewes. Skin temperatures during the morning and afternoon were affected (P<0.05) by feeding regime at d 130 and 145 of pregnancy. In the morning, NR ewes presented greater (P<0.05) head and rump temperature at day 145 of gestation, and lower (P<0.05) udder temperatures at d 130 and 145 than control ewes. In the afternoon, skin temperatures of NR ewes were higher (P<0.05) in head and right flank on d 130 and 145 of pregnancy, and in udder at day 145 compared to control ewes. Serum glucose was higher (P<0.05) in NR ewes than control animals at day 145 of pregnancy. Serum cholesterol, triglyceride and thyroid hormones were not affected by nutritional restriction. Overall, it was found that nutritionally restricted ewes were less affected by intense heat loads than well-fed ewes, apparently due to the lower metabolic heat produced by this underfed animals. Also, it was apparent that the lower respiration rate of NR ewes was compensated by a greater body surface temperature. PMID:24229797

Macías-Cruz, U; Álvarez-Valenzuela, F D; Correa-Calderón, A; Díaz-Molina, R; Mellado, M; Meza-Herrera, C; Avendaño-Reyes, L

2013-01-01

277

Effect of estrous synchronization on the histology of the oviduct and uterus of the ewe  

E-print Network

. , in a study of the effects of estrogen and progesterone upon the genital tract of the ewe, reported that estra- diol caused an increase in the height of the epitheliuxn of the uterine glands and uterine surface of spayed ewes. The effect was rapidly.... , in a study of the effects of estrogen and progesterone upon the genital tract of the ewe, reported that estra- diol caused an increase in the height of the epitheliuxn of the uterine glands and uterine surface of spayed ewes. The effect was rapidly...

Hoermann, Albert LeRoy

2012-06-07

278

Dairy and suckled milk production of Dorset ewes  

Microsoft Academic Search

Two experiments were carried out (1976–77 and 1977–78) to measure both dairy and suckled milk production of Dorset ewes by machine milking. Dairy production was 71% of suckled yield during the corresponding 10 weeks of lactation in 1976–77 and 75% during 14 weeks of lactation in 1977–78. In the 1976–77 experiment varying lamb suckling regimes, which involved partial or complete

K. G. Geenty

1980-01-01

279

TAG composition of ewe's milk fat. Detection of foreign fats  

Microsoft Academic Search

The TAG composition of 45 samples of ewe's milk, collected throughout the year from five Spanish breeds, was analyzed according\\u000a to their carbon number by using short capillary column GC. The TAG content had a bimodal distribution with maxima at C38 (12.8%) and C52 (8.4%). The TAG composition did not vary significantly with respect to the time of year of

Hanane Goudjil; Javier Fontecha; Ma Jesús Fraga; Manuela Juárez

2003-01-01

280

What regulates placental steroidogenesis in 90-day pregnant ewes?  

Microsoft Academic Search

By day-90, the placenta secretes half of the circulating progesterone and 85% of the circulating estradiol-17? [Weems YS, Vincent D, Tanaka Y, et al. Effects of prostaglandin F2? on sources of progesterone and pregnancy in intact, ovariectomized, and hysterectomized 90–100 day pregnant ewes. Prostaglandins 1992;43:203–22; Weems YS, Vincent DL, Nusser K, et al. Effects of prostaglandin F2? (PGF2?) on secretion

Yoshie S. Weems; Laurie Kim; Vicki Tsuda; Chaoquan Yin; Charles W. Weems

2007-01-01

281

KINETICS OF EXPERIMENTAL SALMONELLA ABORTUS OVIS INFECTION IN EWES  

E-print Network

KINETICS OF EXPERIMENTAL SALMONELLA ABORTUS OVIS INFECTION IN EWES F LANTIER Station de Pathologie/12/86 / accepted 15/03/87 Résumé CIN�TIQUE D'UNE INFECTION EXP�RIMENTALE PAR SALMONELLA ABORTUS OVlS CHEZ LA BRE- BIS. - La multiplication et la dissémination de Salmonella abortus ovis, un sérotype de salmonella spé

Paris-Sud XI, Université de

282

SARCOMAS IN HAMSTERS AFTER INJECTION WITH ROUS CHICKEN TUMOR MATERIAL  

PubMed Central

Newborn hamsters were injected subcutaneously with a suspension of finely minced Rous chicken sarcoma (Schmidt-Ruppin strain). After an interval of about 2 weeks, progressively growing sarcomas developed at the site of injection in almost all animals. Also in adult hamsters inoculated intramuscularly with the same material sarcomas developed at the site of injection within 2 to 4 months. Secondary growths appeared on the peritoneal surface, in the retroperitoneal and mediastinal lymph nodes and in the lungs. The sarcomas usually had a pleomorphic appearance and showed a certain resemblance to rhabdomyosarcoma, but sometimes they had the character of spindle cell sarcomas of varying degree of maturity. Sarcomas were not only obtained in hamsters injected with cellular material from the Rous chicken sarcoma but were also seen in hamsters which were injected at birth or when 2 months' old with supernatant fluid obtained by repeated centrifugation of suspensions of homogenized chicken sarcoma, and presumed to be cell-free. The hamster sarcoma was transplanted to a newborn hamster and could then without difficulties be passed in series in hamsters. All attempts to transfer the sarcoma from hamster to hamster by means of cell-free material from the hamster sarcoma failed. On the other hand, material from the hamster sarcomas inoculated into chickens induced rapidly growing Rous sarcomas at the site of inoculation. This proved possible not only with material from the first but also from later passages of the tumor in hamsters. It is concluded that the strain of Rous virus used has the capacity to induce sarcomas not only in chickens but also in hamsters. PMID:13859724

Ahlstrom, C. G.; Forsby, Nils

1962-01-01

283

Primary monophasic synovial sarcoma of the pancreas.  

PubMed

We report a case of synovial sarcoma of the pancreas in a 44-year-old male who presented with multiple episodes of retroperitoneal hemorrhage; the diagnosis was confirmed by histology. The patient underwent distal pancreatectomy without complication, and the hospital stay was nine days. No adjuvant treatment was administered. The patient is alive at 1 year. PMID:23491856

Luc, G; Collet, D; Reich, S; Stanislas, S; Sa-Cunha, A

2013-04-01

284

Pulmonary Artery Sarcoma Mimicking Pulmonary Embolism  

PubMed Central

Primary sarcomas that arise from major blood vessels are exceedingly rare, and some of the published cases have been autopsy reports. Most patients are adults. We report a case of pulmonary artery sarcoma in a 77-year-old man who presented with acute onset of dyspnea. Magnetic resonance imaging of the chest revealed a large mass within the pulmonary trunk and its main branches. Because massive pulmonary embolism was suspected, both anticoagulant and thrombolytic therapies were initiated. The patient responded poorly to these therapies, which then necessitated resection of both the mass and the pulmonary valve. A bioprosthetic porcine valve replaced the native valve, and we reconstructed the right ventricular outflow tract with a Dacron patch. Histopathologic examination revealed a high-grade sarcoma with focal myogenic and chondrogenic differentiation. The patient tolerated the procedure well and was discharged from the hospital on postoperative day 7. He was subsequently treated with chemotherapy and radiation and continued to show no evidence of disease. The diagnosis of pulmonary artery sarcoma should be suspected in patients who present with manifestations of pulmonary embolism, especially when there is no evidence of deep venous thrombosis and poor response to anticoagulant therapy. Multimodal therapy can provide prolonged survival. PMID:25425986

Aftab, Muhammad; Al-Najjar, Raed M.; de la Cruz, Kim I.; Benjamin, Robert S.; Hallman, Charles H.

2014-01-01

285

Biological Therapy to Treat Kaposi Sarcoma  

Cancer.gov

Researchers with this study are investigating whether bevacizumab (Avastin®), a type of biological agent that blocks the formation and growth of new blood vessels, is effective in the treatment of Kaposi sarcoma (KS). Blood vessel cells are the main component of KS lesions.

286

Pulmonary artery sarcoma and chronic thromboembolism  

Microsoft Academic Search

Pulmonary artery sarcoma is a rare and highly lethal disease whose clinical findings are often indistinguishable from those of chronic thromboembolic pulmonary hypertension. Partial improvement after thrombolytic therapy has suggested that thromboembolic phenomena may be superimposed on the tumor, but, to date, a well-documented statement of these events has not been provided.

Simone G. Ramos; Luiz G. C. Salvatti; Federico G. Cipriano; Jarbas S. Roriz-Filho; Fernando D. Matos; Marcos A. Rossi

2008-01-01

287

Pulmonary artery sarcoma and chronic thromboembolism.  

PubMed

Pulmonary artery sarcoma is a rare and highly lethal disease whose clinical findings are often indistinguishable from those of chronic thromboembolic pulmonary hypertension. Partial improvement after thrombolytic therapy has suggested that thromboembolic phenomena may be superimposed on the tumor, but, to date, a well-documented statement of these events has not been provided. PMID:18037248

Ramos, Simone G; Salvatti, Luiz G C; Cipriano, Federico G; Roriz-Filho, Jarbas S; Matos, Fernando D; Rossi, Marcos A

2008-01-01

288

Prostatic stromal sarcoma with neuroectodermal differentiation  

PubMed Central

Abstract Prostatic stromal sarcoma is a fairly rare tumor that constitutes approximately 0.1–0.2% of all prostatic cancers. Detailed characteristics of the tumor are still unclear due to its rarity. We describe a case of prostatic stromal sarcoma in a 63 year-old man who suffered from urinary obstructive symptoms. Palliative transuterine resection was performed and the preliminary histopathological diagnosis was neuroendocrine carcinoma. After chemotherapy, total pelvic exenteration was performed. Histopathologically, the tumor was composed of monotonously proliferating small to medium-sized round cells, which existed in compact islands with loose or dense fibrovascular networks. Immunohistochemically, the tumor cells were widely positive for vimentin, CD56, CD99 and focally positive for synaptophysin, CD10, progesterone receptor, desmin and CD34, but negative for EMA, cytokeratin, estrogen receptor, S-100 and myoglobin. Most of the previously reported tumors exhibited positive stainability for CD10 and progesterone receptor. In addition to these markers, expressions of CD56, CD99 and synaptophysin were characteristically detected in our case. To the best of our knowledge, we present the first case of prostatic stromal sarcoma with characteristic immunohistochemical staining properties. Although the biological characteristics of this rare tumor have not yet been elucidated, these findings suggest prostatic stromal sarcoma can potentially show neuroectodermal differentiation. Virtual slide The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/7291874028051262 PMID:23217062

2012-01-01

289

Pathogenesis of reproductive failure induced by Trypanosoma vivax in experimentally infected pregnant ewes  

PubMed Central

The present study was aimed at investigating the effect of experimental infection by Trypanosoma vivax in different stages of pregnancy, determining the pathogenesis of reproductive failure, and confirming transplacental transmission. We used 12 pregnant ewes distributed into four experimental groups: G1, was formed by three ewes infected with T. vivax in the first third of pregnancy (30 days); G2 comprised three infected ewes in the final third of pregnancy (100 days); G3 and G4 were composed of three non-infected ewes with the same gestational period, respectively. Each ewe of G1 and G2 was inoculated with 1.25 × 105 tripomastigotes. Clinical examination, determination of parasitemia, serum biochemistry (albumin, total protein, glucose, cholesterol, and urea), packed cell volume (PCV), serum progesterone, and pathological examination were performed. Placenta, amniotic fluid, blood and tissues from the fetuses and stillbirths were submitted to PCR. Two ewes of G1 (Ewe 1 and 3) presented severe infection and died in the 34th and 35th days post-infection (dpi), respectively; but both fetuses were recovered during necropsy. In G2, Ewe 5 aborted two fetuses on the 130th day (30 dpi) of pregnancy; and Ewe 6 aborted one fetus in the 140th day (40 dpi) of gestation. Ewes 2 and 4 delivered two weak lambs that died five days after birth. Factors possibly involved with the reproductive failure included high parasitemia, fever, low PCV, body score, serum glucose, total protein, cholesterol, and progesterone. Hepatitis, pericarditis, and encephalitis were observed in the aborted fetuses. The presence of T. vivax DNA in the placenta, amniotic fluid, blood, and tissues from the fetuses confirms the transplacental transmission of the parasite. Histological lesion in the fetuses and placenta also suggest the involvement of the parasite in the etiopathogenesis of reproductive failure in ewes. PMID:23289625

2013-01-01

290

A cytological study of the maturation process of the ovum of the ewe during normal and induced ovulations  

E-print Network

A CYTOLOGICAL STUDY OP THE MATURATION PROCESS OF THE OVUM OP THE EWE DURING NORMAL AND INDUCED OVULATIONS A Dissertation By Harry P. Savery Approved as to style and content by: Chairman of Committee Head of department of Animal Husbandry... ovulations in anestrous ewes ? ? ? ? ? ? 34 3 Cytological data of artificially ovulated ova recovered from anestrous ewes. ? ? ? ? ? ? ? ? ? 35 4 Cytological data of induced follicular ova recovered from anestrous ewes. ? ? ? ? ? ? ? ? ? 36 5 Induced...

Savery, Harry P.

2013-10-04

291

Many eyes or many ewes: vigilance tactics in female bighorn sheep Ovis canadensis vary according to reproductive status  

E-print Network

Many eyes or many ewes: vigilance tactics in female bighorn sheep Ovis canadensis vary according patterns in bighorn sheep Ovis canadensis ewes. We also test whether the observed vigilance patterns status can lead to individual differences in vigilance tactics in bighorn sheep Ovis canadensis ewes

Festa-Bianchet, Marco

292

Successful pregnancies after transfer of embryos recovered from ewes induced to ovulate 24-29 days post partum  

Microsoft Academic Search

CIDR-device withdrawal and eggs recovered on Day 3 of the cycle. Ovum recovery and fertilization rates were higher in lactating than in early weaned ewes, with fertilization being achieved as early as 24 days post partum in both groups. Of the 7 early weaned and 11 lactating ewes yielding eggs, fertilization occurred in 4 and 7 ewes respectively. A total

J. M. Wallace; J. J. Robinson; R. P. Aitken

1989-01-01

293

Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma  

ClinicalTrials.gov

Adult Rhabdomyosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Previously Untreated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

2014-08-12

294

Effects of shade and flaxseed supplementation on the welfare of lactating ewes under high ambient temperatures  

Microsoft Academic Search

The objectives of this study were to evaluate the effects of protection from solar radiation and whole flaxseed supplementation on the welfare of ewes under high ambient temperatures. The experiment was carried out during the summer (July and August) of 2007: 40 Sarda ewes were divided into four groups of ten each, and either exposed (EXP; not offered shade) or

M. Caroprese; M. Albenzio; A. Bruno; G. Annicchiarico; R. Marino; A. Sevi

295

Failure of immunisation against sporidesmin or a structurally related compound to protect ewes against facial eczema.  

PubMed

Sixty ewes, divided randomly into four groups of 15, were immunised subcutaneously against sporidesmin (sdm) -bovine thyroglobulin (BTG) or 2-amino-5-chloro-3,4-dimethoxy benzyl alcohol (ACDMBA) coupled to heat killed staphylococci or to bovine gamma globulin. Fifteen ewes served as untreated controls. Approximately 10 weeks after inoculation ewes were dosed orally with sdm at a rate of 0.1 mg/kg body weight/day for three consecutive days. Sdm antibody binding values.in plasma collected before dosing were higher in ewes immunised with sdm-BTG than ewes given the ACDMBA-complexes. Levels in the 15 untreated ewes were all very low. However, despite the presence of antibodies, the immunised ewes were not protected against sdm challenge; and cholesterol and bilirubin levels in serum and liver and urinary bladder damage scores, at slaughter, were all significantly higher (P<0.05) in the immunised compared to the control ewes six weeks after dosing. It is concluded from these results that subcutaneous immunisation against sdm or the structurally related substance used did not protect sheep against sdm dosing. PMID:16031065

Fairclough, R J; Ronaldson, J W; Jonas, W W; Mortimer, P H; Erasmuson, A G

1984-07-01

296

Responses to saline drinking water in offspring born to ewes fed high salt during pregnancy  

Microsoft Academic Search

We have studied the fetal programming of lambs born to ewes exposed to high salt during pregnancy. In the present study, we hypothesise that salt-programmed lambs may not need to drink as much saline water as control lambs and that voluntary feed intake of salt-programmed lambs would be reduced. We used two groups of lambs born to ewes fed either

S. N. Digby; D. Blache; D. G. Masters; D. K. Revell

2010-01-01

297

Original article Iodine nutrition in ewes. 2. Effects of low to high iodine  

E-print Network

Original article Iodine nutrition in ewes. 2. Effects of low to high iodine intake by ewes on the iodine (I) levels. Dietary I contents (mg/kg dry matter [DM]) in pregnancy and lactation, respectively; Nutrition en iode des brebis. 2. Effet de différents niveaux d'ingestion d'iode par des brebis, sur les

Boyer, Edmond

298

Original article Iodine nutrition in ewes : effects of low to high iodine  

E-print Network

Original article Iodine nutrition in ewes : effects of low to high iodine intake on iodine content, for pregnancy and lactation. In a second experiment, 3 groups of 10 nullipa- rous ewes received diets which), and 10.77 and 8.88 mg/kg dry matter (group E), respecti- vely, for pregnancy and lactation. Observations

Boyer, Edmond

299

Comparison between two methods of measuring the grazing time of ewes  

E-print Network

Comparison between two methods of measuring the grazing time of ewes G Béchet, JP Brun M Petit INRA to measure daily grazing time of ewes were compared : the first method used a portable electronic device (APEC : Brun JP et al, 1984, 5th Meet Eur Grazing Workshop), and the second used visual observations

Paris-Sud XI, Université de

300

The effects of exogenous melatonin on wool quality and thyroid function in Rasa Aragonesa ewes  

Microsoft Academic Search

The effect of melatonin on wool quality and thyroid function was studied. Ten ewes received a melatonin implant (M) on March 2004, and 10 ewes which were not implanted served as control (C). At monthly intervals over 12 months, fibre length and growth were calculated, and plasma thyroxine (T4) concentrations were measured. Wool samples (~N = 2000 fibres) were collected

J. A. Abecia; I. Palacín; F. Forcada

301

LOW FERTILITY IN NURSING EWES DURING THE NON-BREEDING SEASON  

E-print Network

LOW FERTILITY IN NURSING EWES DURING THE NON-BREEDING SEASON Y. COGNIE, M. HERNANDEZ-BARRETO J, regardless of the month of the year. However, the fertility after artificial insemination at the induced-lambings-per-year programme the lowest fertility is found in nursing ewes during the period of lactation anoestrus. When

Paris-Sud XI, Université de

302

Genetic correlations between body weight change and reproduction traits in Merino ewes depend on age.  

PubMed

Merino sheep in Australia experience periods of variable feed supply. Merino sheep can be bred to be more resilient to this variation by losing less BW when grazing poor quality pasture and gaining more BW when grazing good quality pasture. Therefore, selection on BW change might be economically attractive but correlations with other traits in the breeding objective need to be known. The genetic correlations (rg) between BW, BW change, and reproduction were estimated using records from approximately 7,350 fully pedigreed Merino ewes managed at Katanning in Western Australia. Number of lambs and total weight of lambs born and weaned were measured on approximately 5,300 2-yr-old ewes, approximately 4,900 3-yr-old ewes, and approximately 3,600 4-yr-old ewes. On a proportion of these ewes BW change was measured: approximately 1,950 2-yr-old ewes, approximately 1,500 3-yr-old ewes, and approximately 1,100 4-yr-old ewes. The BW measurements were for 3 periods. The first period was during mating period over 42 d on poor pasture. The second period was during pregnancy over 90 d for ewes that got pregnant on poor and medium quality pasture. The third period was during lactation over 130 d for ewes that weaned a lamb on good quality pasture. Genetic correlations between weight change and reproduction were estimated within age classes. Genetic correlations were tested to be significantly greater magnitude than 0 using likelihood ratio tests. Nearly all BW had significant positive genetic correlations with all reproduction traits. In 2-yr-old ewes, BW change during the mating period had a positive genetic correlation with number of lambs weaned (rg = 0.58); BW change during pregnancy had a positive genetic correlation with total weight of lambs born (rg = 0.33) and a negative genetic correlation with number of lambs weaned (rg = -0.49). All other genetic correlations were not significantly greater magnitude than 0 but estimates of genetic correlations for 3-yr-old ewes were generally consistent with these findings. The direction of the genetic correlations mostly coincided with the energy requirements of the ewes and the stage of maturity of the ewes. In conclusion, optimized selection strategies on BW changes to increase resilience will depend on the genetic correlations with reproduction and are dependent on age. PMID:24879756

Rose, G; Mulder, H A; van der Werf, J H J; Thompson, A N; van Arendonk, J A M

2014-08-01

303

Progesterone improves the maturation of male-induced preovulatory follicles in anoestrous ewes.  

PubMed

The first ovulation induced by male effect in sheep during seasonal anoestrus usually results in the development of a short cycle that can be avoided by progesterone priming before ram introduction. In elucidating the involvement of the hypothalamic-pituitary-gonadal axis in the occurrence of short cycles, the effects of progesterone and the time of anoestrus on the development of male-induced preovulatory follicles were investigated in anoestrous ewes using morphological, endocrine and molecular approaches. Ewes were primed with progesterone for 2 (CIDR2) or 12 days (CIDR12) and untreated ewes used as controls during early (April) and late (June) anoestrus. The duration of follicular growth and the lifespan of the male-induced preovulatory follicles were prolonged by ?1.6 days in CIDR12 ewes compared with the controls. These changes were accompanied by a delay in the preovulatory LH and FSH surges and ovulation. Intra-follicular oestradiol concentration and mRNA levels of LHCGR and STAR in the granulosa and theca cells of the preovulatory follicles were higher in CIDR12 ewes than the control ewes. The expression of mRNA levels of CYP11A1 and CYP17A1 also increased in theca cells of CIDR12 ewes. CIDR2 ewes gave intermediate results. Moreover, ewes ovulated earlier in June than in April, without changes in the duration of follicular growth, but these effects were unrelated to the lifespan of corpus luteum. Our results give the first evidence supporting the positive effect of progesterone priming on the completion of growth and maturation of preovulatory follicles induced by male effect in seasonal anoestrous ewes, thereby preventing short cycles. PMID:25062803

Adib, Achraf; Freret, Sandrine; Touze, Jean-Luc; Lomet, Didier; Lardic, Lionel; Chesneau, Didier; Estienne, Anthony; Papillier, Pascal; Monniaux, Danielle; Pellicer-Rubio, Maria-Teresa

2014-10-01

304

Olfactory bulb removal does not prevent gonadotropin or prolactin responses to changing photoperiod in the ewe.  

PubMed

The purpose of this study was to determine if bilateral olfactory bulb removal (Bulbx) alters photoperiod-induced changes of gonadotropin and prolactin secretion in the ewe. Ovariectomized (group 1; n = 12) or ovariectomized estradiol-treated (group 2; n = 12) Suffolk ewes underwent Bulbx (7 per group) or sham operations (5 per group). All ewes subsequently were placed into photochambers and exposed to a photoregimen of alternating 16 h light/8 h dark and 10 h light/14 h dark photoperiods. Plasma concentrations of luteinizing hormone (LH), follicle-stimulating hormone (FSH) and prolactin were determined in blood samples taken twice weekly from group 2 ewes. At the end of each 90-day photoregimen blood samples from all ewes in both groups were taken at frequent intervals for 4 h to determine LH pulse parameters. During the initial 16 h light/8 h dark photoperiod, plasma melatonin concentrations were determined for group 2 ewes during a 24-hour period. The completeness of Bulbx was determined at time of necropsy for all Bulbx ewes. In addition, the functional completeness of Bulbx was determined in group 2 ewes by exposing them to ram's wool and measuring changes in LH secretion. Bulbx did not affect either basal or photoperiod-induced changes in LH, FSH and prolactin in group 2 ewes. LH pulse parameters varied with photoperiod but were not significantly (p > 0.05) affected by Bulbx in either group 1 or group 2 ewes. The normal nocturnal elevation in plasma melatonin concentrations was unaffected by Bulbx.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:8321417

Jansen, H T; Jackson, G L

1993-03-01

305

Primary Kaposi Sarcoma of Penis in HIV Negative Patient  

PubMed Central

Primary Kaposi sarcoma of penis is very rare. We will introduce a 47 years old male patient referred to our clinic from dermatology service, in this report. The patient suffered from itchy penile papules around coronal region. The lab tests had revealed a negative serology of HIV but tissue PCR was positive for Human Herpesvirus-8 (HHV8). Histological findings were compatible with Kaposi sarcoma. Primary Kaposi sarcoma of penis is rare but could occur in HIV negative patients.

Karami, Hossein; Bagher-Tabrizi, Alireza; Yaghoobi, Mohammad

2012-01-01

306

Familial Kaposi's Sarcoma: A Report of Five Cases from Greece  

PubMed Central

Introduction. Familial cases of Kaposi's sarcoma have rarely been reported. Kaposi's sarcoma is not uncommon in Greece; its incidence is estimated at 0.20 per 100.000 habitants, showing an increased predominance in the Peloponnese, in Southern Greece. Case Report. We describe five cases of familial clustering of KS originating from Greece. Discussion. The pathogenesis of familial Kaposi's sarcoma is still far from being completely understood. Genetic, environmental, and infectious factors have been incriminated. PMID:25093124

Armyra, Kalliopi; Kouris, Anargyros; Xanthinaki, Arsinoi; Stratigos, Alexandros; Potouridou, Irene

2014-01-01

307

Post-treatment sarcoma in breast cancer patients  

Microsoft Academic Search

Background: Many patients treated for breast cancer with radiotherapy will survive their disease and be at risk for treatment-related sarcoma for many years.\\u000aMethods: In order to identify patients with post-treatment sarcoma and define this disease, we examined the records of 99 patients treated for sarcoma with a history of antecedent breast carcinoma. Of these patients, 51 were felt to

Mary S. Brady; Carol F. Garfein; Jeanne A. Petrek; Murray F. Brennan

1994-01-01

308

Refractory ulcerative colitis and iatrogenic colorectal Kaposi's sarcoma.  

PubMed

Colorectal Kaposi's sarcoma, a human herpes virus-8 associated mesenchymal tumour, is exceedingly rare in human immunodeficiency virus-negative subjects and almost always reported in association with severe, refractory, inflammatory bowel disease. In this paper we report a case--the second from Italy--of a colorectal Kaposi's sarcoma in a human immunodeficiency virus-negative, heterosexual man with severe refractory ulcerative colitis. Kaposi's sarcoma developed after starting glucocorticosteroid therapy, supporting the theory that colorectal Kaposi's sarcoma associated with ulcerative colitis is iatrogenic. PMID:18054849

Girelli, C M; Serio, G; Rocca, E; Rocca, F

2009-02-01

309

Isolated myeloid sarcoma presenting in all four eyelids.  

PubMed

Myeloid sarcoma is an unusual manifestation of acute leukemia. These leukemic tumors have been described in multiple organ systems. Leukemia is almost always present, but may be undiagnosed when the myeloid sarcoma comes to medical attention. We present a clinicopathologic report of a previously healthy young woman with conjunctival myeloid sarcomas of all 4 eyelids without evidence of an underlying hematologic disorder over 15 months' follow-up, despite refusal of consolidation chemotherapy. Isolated, multifocal myeloid sarcoma of the periorbital region is a very rare manifestation of acute leukemia. PMID:17667120

Thomas, Scott A; Durairaj, Vikram D

2007-01-01

310

GEIS 2013 guidelines for gastrointestinal sarcomas (GIST).  

PubMed

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal soft tissue sarcoma of the gastrointestinal tract. Correct diagnosis with thorough use of pathologic and molecular tools of GIST mutations has been of the foremost importance. GIST are usually (95 %) KIT positive and harbor frequent KIT or platelet-derived growth factor receptor ?-activating mutations. This deep molecular understanding has allowed the correct classification into risk groups with implications regarding prognosis, essential use in the development of targeted therapies and even response prediction to this drugs. Treatment has been evolving and an update to include lessons learned from recent trials in advanced disease as well as controversies in the adjuvant setting that are changing daily practice, is reviewed here. An effort from the Spanish Group for Sarcoma Research with investigators from the group has been undertaken to launch this third version of the GIST guidelines and provide a practical means for the different disciplines that treat this complex disease. PMID:25193432

Poveda, Andrés; Del Muro, Xavier García; López-Guerrero, Jose Antonio; Martínez, Virginia; Romero, Ignacio; Valverde, Claudia; Cubedo, Ricardo; Martín-Broto, Javier

2014-11-01

311

Multimodality Local Therapy for Retroperitoneal Sarcoma  

SciTech Connect

Purpose: Soft-tissue sarcomas of the retroperitoneum are rare tumors comprising less than 1% of all malignancies. Although surgery continues as the mainstay of treatment, the large size of these tumors coupled with their proximity to critical structures make resection with wide margins difficult to achieve. The role and timing of radiotherapy are controversial. This study updates our institutional experience using multimodality local therapy for resectable retroperitoneal sarcoma and identifies prognostic factors impacting disease control and survival. Methods and Materials: Between 1974 and 2007, 58 patients with nonmetastatic retroperitoneal sarcoma were treated with surgery and radiation at University of Florida. The median age at radiotherapy was 57 years old (range, 18-80 years). Forty-two patients received preoperative radiotherapy and 16 received postoperative radiotherapy. Nineteen patients received 1.8 Gy once daily and 39 patients received 1.2 Gy twice daily. Variables analyzed for prognostic value included age, grade, kidney involvement, histology, de novo versus recurrent presentation, tumor diameter, margin status, radiotherapy sequencing (preoperative vs. postoperative), total radiation dose, fractionation scheme, and treatment era. Results: The 5-year overall survival, cause-specific survival, and local control rates were 49%, 58%, and 62%, respectively. Nearly two-thirds of disease failures involved a component of local progression. On multivariate analysis, only margin status was significantly associated with improved 5-year local control (85%, negative margins; 63%, microscopic positive margins; 0%, gross positive margins; p < 0.0001) and 5-year overall survival (64%, negative margins; 56%, microscopic positive margins; 13%, gross positive margins; p = 0.0012). Thirty-one Grade 3 or greater toxicities were observed in 22 patients, including two treatment-related deaths (3%). Conclusion: For retroperitoneal sarcoma, local control remains a challenge and combined-modality therapy may be associated with significant acute and late morbidity. Our patterns of failure data suggest that improvements in local control may translate into a survival benefit.

Paryani, Nitesh N.; Zlotecki, Robert A.; Swanson, Erika L.; Morris, Christopher G. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); Grobmyer, Stephen R.; Hochwald, Steven N. [Department of General Surgery, University of Florida College of Medicine, Gainesville, FL (United States); Marcus, Robert B. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); University of Florida Proton Therapy Institute, Jacksonville, FL (United States); Indelicato, Daniel J., E-mail: dindelicato@floridaproton.org [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); University of Florida Proton Therapy Institute, Jacksonville, FL (United States)

2012-03-01

312

Soft tissue sarcoma, version 2.2014.  

PubMed

These NCCN Guidelines Insights highlight the important updates to the NCCN Guidelines for Soft Tissue Sarcoma (STS) specific to the role of radiation therapy in the management of patients with retroperitoneal/intra-abdominal STS. The guidelines have also included recommendations for genetic testing and counseling for patients with a clinical and/or family history of genetic cancer syndromes associated with a predisposition for the development of STS. PMID:24717567

von Mehren, Margaret; Randall, R Lor; Benjamin, Robert S; Boles, Sarah; Bui, Marilyn M; Casper, Ephraim S; Conrad, Ernest U; Delaney, Thomas F; Ganjoo, Kristen N; George, Suzanne; Gonzalez, Ricardo J; Heslin, Martin J; Kane, John M; Mayerson, Joel; McGarry, Sean V; Meyer, Christian; O'Donnell, Richard J; Pappo, Alberto S; Paz, I Benjamin; Pfeifer, John D; Riedel, Richard F; Schuetze, Scott; Schupak, Karen D; Schwartz, Herbert S; Van Tine, Brian A; Wayne, Jeffrey D; Bergman, Mary Anne; Sundar, Hema

2014-04-01

313

Proteomic analysis of soft tissue sarcoma  

Microsoft Academic Search

Soft tissue sarcomas are a heterogeneous group of malignant mesenchymal tumors, and novel tumors biomarkers and therapeutic\\u000a targets are required to achieve early diagnosis and optimize therapy for individual patients. Global protein expression studies,\\u000a an approach known as “proteomics”, can offer important clues for understanding tumor biology that cannot be obtained by other\\u000a approaches. Proteomic studies, such as two-dimensional difference

Yoshiyuki Suehara

2011-01-01

314

Effect of oestrogen and an LH-RH agonist on the release of gonadotrophins in ovariectomized ewes deprived of LH-RH  

Microsoft Academic Search

Summary. Endogenous LH-RH in ewes was inhibited by active immunization or by injection of LH-RH antiserum. Plasma levels of LH and FSH were elevated in 3 ovariectomized control ewes but low in 3 LH-RH immunized ovariectomized ewes. Oestradiol benzoate (50 \\\\g=m\\\\gi.m.) caused a marked rise in LH concentrations in control ewes but not in the immunized ewes. In the immunized

H. M. Fraser; I. J. Clarke; A. S. McNeilly

1981-01-01

315

Glutathione peroxidase (GPX) activity in blood of ewes on farms in different scrapie categories in Iceland  

PubMed Central

Background Preliminary studies indicated decreased glutathione peroxidase (GPX) activity in blood of ewes on scrapie-afflicted farms. Other studies have shown decreased GPX activity in brain of prion-infected mice and in prion-infected cells in vitro. The aim of this study was to examine the GPX activity in blood as well as the distribution of GPX-activity levels from ewes on farms in scrapie-afflicted areas in Iceland. Methods Blood samples were collected from 635 ewes (non-pregnant [n = 297] and pregnant [n = 338]) on 40 farms in scrapie-afflicted areas during the years 2001–2005, for analysis of GPX activity. The farms were divided into three categories: 1. Scrapie-free farms (n = 14); 2. Scrapie-prone farms (earlier scrapie-afflicted, restocked farms) (n = 12); 3. Scrapie-afflicted farms (n = 14). For comparison, 121 blood samples were also collected from non-pregnant ewes on one farm (farm A) in a scrapie-free area (scrapie never registered). Chi-square test was used to test for normal distribution of GPX-results, and Kruskal-Wallis test to compare GPX-results between categories. Results The GPX-results appeared to be biphasically distributed in ewes in all three scrapie categories and on farm A. The presumptive breaking point was about 300 units g Hb-1. About 30–50% of the GPX-results from ewes in all three scrapie categories were below 300 units g Hb-1 but only about 13% of the GPX-results from ewes on farm A. The mean GPX activity was highest on farm A, and was significantly lower on scrapie-prone farms than on scrapie-free or scrapie-afflicted farms (non-pregnant and pregnant ewes: P < 0.005, respectively; non-pregnant and pregnant ewes combined: P < 0.0005). Conclusions 1) the distribution of GPX-results in blood of Icelandic ewes apparently has a biphasic character; 2) the GPX-results were higher in ewes on one farm in a scrapie-free area than in ewes on farms in the scrapie-afflicted areas; 3) GPX-activity levels were significantly lowest on earlier scrapie-afflicted, restocked farms, which might have a bearing on the recurrence of sporadic scrapie on these farms; 4) further study on the possible role of GPX activity in the occurrence of scrapie in Iceland is warranted. PMID:18573202

Gudmundsdottir, Kristin B; Kristinsson, Jakob; Sigurdarson, Sigurdur; Eiriksson, Tryggvi; Johannesson, Torkell

2008-01-01

316

Response of fat-tailed Syrian Awassi ewes to accelerated lambing systems  

Microsoft Academic Search

Fifty cyclic fat-tailed Syrian Awassi ewes aged 2–4 years, with a mean weight of 51.4 kg, were used for 4 years to assess\\u000a the accelerated lambing system (three lambings in 2 years). Ewes were divided into two groups: treated (T) and untreated (C).\\u000a Ewes in the T group were treated with flugestone acetate for 14 days and injected intramuscularly at sponge withdrawal with\\u000a 500 IU of

Moutaz Zarkawi

2011-01-01

317

Treatment of patients with sarcoma of the uterus.  

PubMed

In the present series of 156 patients with sarcomas of the uterus the therapeutic modalities employed were evaluated. The 5-year survival rate of 51.3% was obtained for this series including 41.2% for leiomyosarcomas, 57.1% for endometrial stromal sarcomas, 50.0% for mixed mesodermal tumors and 40.0% for carcinosarcomas. The prognostic significance of the extent of disease, histological type and the age of patients is emphasised. Clinical features of uterine sarcomas specific for different histological types were shown: leiomyosarcomas were characterized by a rapid tumor growth while uterine bleeding in the background of neuroendocrine disturbances similar to those observed in endometrial carcinomas were related to endometrial stromal sarcomas and mixed mesodermal tumors. In respect to early diagnosis of sarcomas an ultrasonic pelvic examination and studies of aspirate specimens taken from the uterine cavity are recommended to be performed for the groups at high risk. An individual treatment policy was developed for each sarcoma patient depending on the histological type, the stage of disease and general condition of the patient. Surgical approach varied ranging from a total hysterectomy and adnexectomy, done for leiomyosarcomas, to an extended hysterectomy and pelvic lymphadenectomy for mixed mesodermal tumors and endometrial stromal sarcomas. Postoperative distant and/or endovaginal radiation are indicated for all histological types of sarcomas excepting leiomyosarcomas. Application of adjuvant chemotherapy with carminomycin or adriamycin improves the results of treatment. PMID:2209643

Bokhman, J V; Yakovleva, I A; Urmanchejeva, A F

1990-01-01

318

Two Types of Soft Tissue Sarcoma of Uncertain Histogenesis  

PubMed Central

A brief account is given of two types of rare soft tissue sarcoma of uncertain histogenesis. The danger of mistaking the epitheloid sarcoma for a benign lesion is emphasized. ImagesFigs. 3-4Figs. 5-6Figs. 7-8Figs. 1-2Figs. 9-10 PMID:5144520

Mackenzie, D. H.

1971-01-01

319

KIRURGIJA SARKOMOV RETROPERITONEJA SURGICAL TREATMENT OF THE RETROPERITONEAL SARCOMA  

Microsoft Academic Search

Abstract - Abstract - Abstract - Abstract - Background. Retroperitoneal sarcomas are mali- gnant tumors with an aggressive course of disease. Cause of death is local disease in 50% and disseminated disease in only 25%. We made a retrospective analysis of surgical treatment of retroperitoneal sarcomas in order assess the effect of this treatment modality on the course of the

Janez Novak

2003-01-01

320

The multiple sclerotic osteogenic sarcoma of early childhood.  

PubMed

Two cases of sclerosing osteogenic sarcoma that occurred in early childhood are reported. They and the similar cases reviewed are unusual in their development at an unusually early age, their multicentric presentation and their densely sclerotic nature. These are uncommon manifestations of osteogenic sarcoma and are considered due to proliferative metastatic osteoid formation in areas of rapid bone growth. PMID:1065446

Cremin, B J; Heselson, N G; Webber, B L

1976-05-01

321

Kaposi's sarcoma involving the thyroid in a patient with AIDS  

SciTech Connect

A 30-year-old man with acquired immune deficiency syndrome (AIDS) and Kaposi's sarcoma had a palpable thyroid mass and cervical lymphadenopathy. Nuclear medicine and ultrasound scans revealed multiple thyroid nodules. Results of biopsy showed Kaposi's sarcoma metastatic to the thyroid.

Krauth, P.H.; Katz, J.F.

1987-11-01

322

Kaposi's sarcoma in acquired immune deficiency syndrome (AIDS)  

Microsoft Academic Search

Of 22 patients with Kaposi's sarcoma, 16 had the acquired immune deficiency syndrome (AIDS). The histological pattern in AIDS differs from the more familiar classical Kaposi's sarcoma. The features most useful in making the diagnosis are: dissection of collagen; lymphatic vessel like spaces; angiomatoid lesions; premonitory sign; and spindle cell proliferation. It is important to examine multiple levels of small

N D Francis; J M Parkin; J Weber; A W Boylston

1986-01-01

323

Optimal management of primary retroperitoneal sarcoma: an update.  

PubMed

Soft tissue sarcomas are a group of heterogeneous neoplasms with more than 50 histological subtypes exhibiting major differences in terms of pathogenesis, genetic alterations and clinical behavior. Sarcomas represent approximately 1% of malignancies with retroperitoneal sarcomas representing 10-15% of all soft tissue sarcomas. Surgery is currently the only modality which offers the chance of cure. Surgery for retroperitoneal sarcomas presents specific challenges due their location in a complex space surrounded by vital structures and visceral organs often prohibiting resection with wide margins. Furthermore, even after complete resection local recurrence is common and the leading cause of death. In this article the authors describe the initial investigations, prognostic factors and optimal surgical management. The evidence and current research as regards the role of multimodality treatment is reviewed and discussed. PMID:24524274

Miah, Aisha B; Hannay, Jonathan; Benson, Charlotte; Thway, Khin; Messiou, Christina; Hayes, Andrew J; Strauss, Dirk C

2014-05-01

324

Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-Rhabdomyosarcoma Soft Tissue Sarcomas That Can be Removed by Surgery  

ClinicalTrials.gov

Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Mesenchymoma; Adult Synovial Sarcoma; Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Epithelioid Sarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Synovial Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Stage IB Adult Soft Tissue Sarcoma; Stage IIB Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

2014-10-22

325

Undifferentiated pleiomorphic sarcoma simultaneously occuring with thymoma  

PubMed Central

We report here a case of thymoma simultaneously associated with neuroendocrine tumor. A 65-year-old male, presented with cough. Radiographic studies showed a mediastinal mass. On fine needle aspiration cytology and histopathological examination, a diagnosis of thymoma with coexisting undifferentiated pleomorphic sarcoma was made. Although thymoma is associated with many extrathymic malignancies, its association with neuroendocrine tumor is rare. This case is being reported on to reinforce that clinicians should bear in mind the possibility of extrathymic malignancies in patients with thymoma.

Tsolakis, Nikolaos

2014-01-01

326

Disseminated Kaposi's sarcoma without cutaneous involvement  

PubMed Central

Kaposi's sarcoma (KS) is a low-grade vascular tumor caused by infection with human herpesvirus 8. Prior to the AIDS epidemic, KS was rare in the United States. With the advent of highly active antiretroviral therapy, KS has become far less common, now occurring at a rate of about 6 cases per million people each year. It is still seen most commonly in those infected with HIV, and cutaneous manifestations represent the most common presentation. In this case, we describe a patient with disseminated AIDS-associated KS lacking cutaneous manifestations. PMID:24381399

Shepard, Benjamin; Tompkins, Dustin; Baker, Damon

2014-01-01

327

The roles of estradiol-17 beta and prolactin in uterine gland development in the neonatal ewe  

E-print Network

Endometrial glands are required for adult uterine function and develop post-natally in mammalian species. Therefore, studies were conducted using neonatal ewes as a model to determine: 1) the roles of estradiol-17-alpha and estrogen receptor...

Carpenter, Karen Denise

2005-11-01

328

Mass-dependent reproductive strategies in wild bighorn ewes: a quantitative genetic approach  

E-print Network

; maternal effects; maternal expenditure; Ovis canadensis; population density; ungulates. Abstract In the Ram Mountain bighorn sheep (Ovis canadensis) population, ewes differing by more than 30% in body mass weaned

Réale, Denis

329

vol. 176, no. 4 the american naturalist october 2010 Bighorn Ewes Transfer the Costs of  

E-print Network

transfer some costs to their offspring. Using data on 442 lambs weaned by 146 bighorn sheep (Ovis canadensis) ewes at Ram Mountain, Alberta, we studied how reproductive effort varied with environmental

Festa-Bianchet, Marco

330

Effects of supplementary treatment with bovine growth hormone on hormonal and ovulatory responses to inhibin immunization in ewes.  

PubMed

The aim of this study was to determine whether supplementary treatment with recombinant bovine growth hormone(rbGH) can enhance the ovulatory response of ewes to inhibin immunization. Crossbred ewes (n = 20) were actively immunized against bovine inhibin a1-29 peptide conjugate while 20 ewes served as controls. Oestrus was synchronized using progestagen sponges and ewes were allocated to four groups: control ewes (n = 10); control ewes given rbGH (n = 10); inhibin-immunized ewes (n = 10) and inhibin-immunized ewes given rbGH (n = 10). A single s.c. dose of rbGH (50 mg) was given 7 days before sponge removal. Blood was collected for measurement of inhibin antibody titre, and concentrations of insulin-like growth factor I (IGF-I), FSH, oestradiol and progesterone. Ovulation, pregnancy and lambing rates were also recorded. All inhibin-immunized ewes produced antibodies that bound 125I-labelled (32 kDa) inhibin. The concentration of FSH in the plasma of the ewes after the second booster inhibin immunization was higher than that in control ewes (P < 0.005). Treatment with rbGH promoted a 2-3-fold increase in plasma concentration of IGF-I (P < 0.001); the response was less (P < 0.01) in immunized compared with control ewes. Treatment with rbGH alone had no significant effect on the concentration of FSH or oestradiol or on ovulation rate or litter size. Overall, inhibin-immunized ewes had higher mean FSH concentrations (P < 0.002), higher preovulatory oestradiol surges (P < 0.05) and higher progesterone concentrations in the luteal phase (P < 0.0001). Treatment with rbGH reduced the effects of immunization on FSH (P < 0.01) and progesterone (P < 0.02) concentrations. Immunized ewes showed a threefold increase in ovulation rate (P < 0.001) and a 1.8-fold increase in litter size (P < 0.05) compared with control ewes. In immunized ewes given rbGH, ovulation rate was increased by a factor of 2.2 and litter size by a factor of 1.8. In conclusion, these data do not support the hypothesis that supplementary treatment of ewes with rbGH to raise plasma IGF-I concentrations (and presumably intraovarian IGF-I) can enhance the ovulatory response to inhibin immunization. PMID:9306979

Tannetta, D S; Fray, M D; Wrathall, J H; Bleach, E C; Glencross, R G; Knight, P G

1997-07-01

331

Equine chorionic gonadotrophin administration to rams improves their effectiveness to stimulate anoestrous ewes (the "ram effect").  

PubMed

Ewes' response to ram effect is related to the strength of androgen-dependent ram signals. Experiment 1 aimed to determine if the administration of a single dose of 1000IU of eCG to rams three days before joining them with ewes enhance their ability to stimulate females. Based on the results of Experiment 1, in a second experiment rams received two doses seven and three days before their introduction to females. In Experiment 1, rams treated or not with eCG were joined with ewes, and estrous was recorded until Day 5 (Day 0=rams and ewes were joined), and from Day 15 to Day 23. In addition, serum testosterone concentration was measured in all rams in the first recorded period. Testosterone values were greater in eCG-E1 than in Con-E1 rams on Days 0 and 2. The percentage of ewes in estrus was similar in both groups. In Experiment 2, rams were treated with two doses of eCG on Days -7 and -3 or remained as untreated controls. Estrous was recorded until Day 5, and pregnancy rate on Day 46; testosterone was measured in samples collected from all rams. Testosterone concentration was greater in eCG-E2 than Con-E2 rams from Day -5 to Day 1, and tended to do so on Day 2. More eCG-E2 than Con-E2 ewes came into estrus and became pregnant. It was concluded that treatment of rams with two high doses of eCG before joining them with anestrous ewes, enhanced their ability to induce ewes' cyclic activity (the "ram effect"). PMID:25059200

Ungerfeld, Rodolfo; Clemente, Neftali; Bonjour, Lorena; Orihuela, Agustin

2014-10-01

332

Phenotypic relationships among early life traits and lifetime ewe production efficiency.  

PubMed

In least-squares analyses of variance, 10 ewe lamb traits were used, singly and in various combinations, to predict lifetime production efficiency. Ewes belonged to eight crossbred groups and were raised on two management systems for either 4 or 5 yr of production. Lifetime production efficiency components were total feed and ewe ownership cost, gross income and net revenue. A ewe lamb's type of birth, actual and adjusted weaning weight (WWt and AWWt), postweaning weight (PWWt) and first year gross income (FGROS) were significantly phenotypically related to one or more components of lifetime production efficiency. While triplets were better than either of the other two birth types, single-born ewes were better than twin-born ewes for lifetime production efficiency. Any of the three ewe lamb weights (AWWt, WWt or PWWt) appeared to be an accurate estimator of lifetime production efficiency. The effect of first year productivity was largely a part-whole relationship with lifetime production because the regression coefficients of subsequent lifetime production efficiency (not including first year production) on first year productivity were near zero. Postweaning average daily gain, ewe age at first estrus and date of first lambing (at approximately 12 mo of age) did not have significant relationships with lifetime production efficiency. When type of birth, date of birth, weaning weight and first year gross income were analyzed simultaneously, weaning weight and FGROS had the greatest effect on lifetime production efficiency. When AWWt and type of birth were considered simultaneously, both had significant relationships with lifetime production efficiency. PMID:6436218

Saoud, N B; Hohenboken, W D

1984-09-01

333

Placental development and fetal growth in growth hormone-treated ewes  

Microsoft Academic Search

The effects of recombinant bovine growth hormone (bGH) treatment of pregnant ewes on maternal metabolism, placental development and fetal growth were examined in two studies. In a preliminary study (experiment one), single-bearing ewes were treated by twice-daily subcutaneous injection for 7 days with bGH (n= 8) at a dose of 0.15 mg\\/kg LW\\/day or with saline (n= 8) between days

C. M. C. Jenkinson; S. H. Min; D. D. S. Mackenzie; S. N. McCutcheon; B. H. Breier; P. D. Gluckman

1999-01-01

334

Reproductive responses in ewes treated with eCG or increasing doses of royal jelly  

Microsoft Academic Search

This experiment was conducted to evaluate the effect of administering increasing doses of royal jelly (RJ) on reproductive parameters in ewes. Additionally, this study compared using RJ vs. equine chorionic gonadotropin (eCG) in estrous cycle control. In May (transitional period between anestrous and the breeding season) 37 multiparous, winter-lambing Awassi ewes 3–6 years of age (average body weight of 53±1.2kg)

R. T. Kridli; S. S. Al-Khetib

2006-01-01

335

Effect of selenium yeast supplementation on naturally acquired parasitic infection in ewes.  

PubMed

Gastrointestinal parasites cause substantial economic losses in pasture-based sheep production systems. Supranutritional organic selenium (Se) supplementation may be beneficial because it improves immune responses to pathogens. To evaluate the effect of Se-yeast supplementation on gastrointestinal parasite load, 30 ewes per treatment group were drenched weekly with no Se, 4.9 mg Se/week as Se yeast (maximum FDA-allowed concentration), or supranutritional concentrations of Se yeast (14.7 and 24.5 mg Se/week) starting early fall for 85 weeks. Fecal samples were collected at weeks 63, 66, 78, and 84 and counted for total trichostrongyle-type eggs and Haemonchus contortus eggs (in samples with ?200 trichostrongyle eggs/g feces). During breeding season (fall), ewes were kept on pasture; ewes receiving 24.5 mg Se/week had lower fecal trichostrongyle egg counts (93?±?40 eggs/g feces) compared with ewes receiving no Se (537?±?257 eggs/g feces; P?=?0.007) or ewes receiving 4.9 mg Se/week as Se yeast (398?±?208 eggs/g feces; P?=?0.03). In winter, fecal trichostrongyle egg counts decreased, and group differences were not apparent. During lambing season (spring), ewes were kept in the barn and fecal trichostrongyle egg counts increased, although no group differences were observed. However, none of the ewes receiving supranutritional Se yeast, and with trichostrongyle egg counts ?200 eggs/g of feces, but four of the ewes receiving lower Se dosages had H. contortus egg counts ?1,000 eggs/g feces (P?=?0.04). Our results suggest that supranutritional Se-yeast supplementation may enhance resistance to naturally occurring H. contortus gastrointestinal parasitism in sheep. PMID:25256922

Hooper, Kathryn J; Bobe, Gerd; Vorachek, William R; Bishop-Stewart, Janell K; Mosher, Wayne D; Pirelli, Gene J; Kent, Michael L; Hall, Jean A

2014-12-01

336

The induction of breeding activity in lactating ewes during anestrus by the use of hormones  

E-print Network

THE INDUCTION OF BREEDING ACTIVITY IN LACTATING EWES DURING ANESTRUScBY THE USE OF HORMONES A Thesis By Shams Uddin Ahmed Submitted to the Graduate School of the Agricultural and Mechanical College of Texas in partial fulfillment... of the requirements for the degree of MASTER OF SCIENCE January, 1963 Major Subject: Physiology of Reproduction 11 THE INDUCTION OF BREEDING ACTIVITY IN LACTATING EWES DURING ANESTRUS BY THE USE OF HORMONES A Thesis By Shams Uddin Ahmed Approved as to style...

Ahmed, Shams Uddin

2012-06-07

337

Ethanol kinetics during pregnancy. Study in ewes and their fetuses.  

PubMed

1. Kinetics of placental transfer and elimination of ethanol in maternal and fetal blood and amniotic fluid were studied after iv infusion of ethanol in 3 ewes in the third trimester of pregnancy. 2. Ethanol was transferred rapidly from the maternal to the fetal compartment, and more slowly from these to the amniotic fluid. 3. The kinetics of ethanol elimination from the maternal and fetal circulations were similar, indicating rapid bidirectional transfer between them and elimination from the former only. 4. There was significantly slower elimination of ethanol from the amniotic fluid, with increasing concentrations long after these had started to decline in the maternal and fetal blood. This suggests that the amniotic fluid can act as a reservoir for ethanol storage. PMID:7202228

Ng, P K; Cottle, M K; Baker, J M; Johnson, B; van Muyden, P; van Petten, G R

1982-01-01

338

Staphylococcus-associated abortions in ewes with long-term central venous catheterization.  

PubMed

Forty-two ewes had an intravenous catheter sewn in place in a prepared area over the jugular vein and beginning at 60 days of gestation received an infusion 3 times daily. The infusion consisted of sterile saline or sterile saline containing arginine. Twenty-six ewes in both control and treatment groups aborted between 81 days of gestation and term. Fetuses from 16 ewes that aborted were examined. Most were autolyzed or had early mummification. Macroscopic placentitis and noncollapsing lungs were noted. Large numbers of coagulase-positive Staphylococcus were isolated from fetal abomasal content, lungs, brains, or placentas. Histologically, suppurative placentitis with necrosis and pulmonary aspiration of meconium and amniotic debris often with suppurative bronchopneumonia were observed in abortuses. Four ewes euthanized and examined after abortion had suppurative endometritis. Three ewes had severe, chronic, jugular thrombophlebitis from which coagulase-positive Staphylococcus was isolated. The fourth ewe had mild phlebitis, and Staphylococcus aureus was isolated from both the catheter and the blood. Catheter-associated staphylococcal abortion was diagnosed. PMID:18984790

Edwards, J F; Lassala, A L; Spencer, T E

2008-11-01

339

The biology of uterine sarcomas: A review and update  

PubMed Central

Uterine sarcoma is a rare neoplasm, accounting for only 5% of uterine malignancies. The pathogenesis of uterine sarcoma remains largely unknown, although recent basic science and pre-clinical animal models have provided a better understanding of tumor biology. The aim of this study was to review the clinical features, imaging characteristics, genetic aberrations and therapeutic approaches in uterine sarcoma. This study reviewed the English-language literature on clinical and basic studies on uterine sarcoma. The common variants of uterine sarcoma are carcinosarcoma, leiomyosarcoma and endometrial stromal sarcoma (ESS). Genetic profiling efforts have identified amplification, overexpression and mutation, while the molecular mechanisms of tumorigenesis driven by these genomic and genetic aberrations have yet to be fully elucidated yet. Recent genome-wide studies have also identified complex chromosomal rearrangements as oncogenic mechanisms. The cell cycle regulators, p16 and p53, are frequently over-expressed and appear to be involved in key modifications of sarcomagenesis. Molecular-targeted therapy has now been evaluated in clinical trials for certain subtypes. In conclusion, aberrations of cell cycle control would be a critical step in the development of uterine sarcoma. This review has provided new areas of study targeting molecular and genetic pathways. PMID:24649216

KOBAYASHI, HIROSHI; UEKURI, CHIAKI; AKASAKA, JURIA; ITO, FUMINORI; SHIGEMITSU, AIKO; KOIKE, NATSUKI; SHIGETOMI, HIROSHI

2013-01-01

340

Gastrointestinal Kaposi's Sarcoma Presenting as Ileocolic Intussusception  

PubMed Central

Context: Kaposi's sarcoma (KS) is the most common neoplasm in patients with acquired immune deficiency syndrome (AIDS). Gastrointestinal (GI) involvement with KS commonly occurs in association with cutaneous lesions or lymph node involvement, with GI tract involvement alone occurring in only 3.5% of cases. There are several case reports described in the literature about asymptomatic intestinal KS with skin manifestations. Although GI KS is usually asymptomatic, hemorrhages from the oral cavity, esophagus, stomach, and large bowel have been reported in this disease. Case Report: Our case is unique, in a way that the patient does not have skin manifestation, and also is that the first manifestation presented as acute intestinal intussusception and obstruction with nodular mass lesions of the stomach and GI tract due to GI KS. Conclusion: As a differential diagnosis of KS, nonHodgkin lymphomas frequently involve the gut in AIDS patients. Furthermore, tumors of the gut with spindle-shaped cells such as leiomyomas, rhabdomyosarcomas, high-grade pleomorphic sarcomas, or GI stromal tumors have to be considered in the differential diagnosis. Overall, the visceral involvement of the KS is usually associated with poor prognosis. Our case illustrates the importance of physicians to recognize GI KS as a differential diagnosis for HIV positive patients with recurrent abdominal pain. It is commonly occurs in association with cutaneous lesions or lymph node involvement and rarely presents with GI involvement alone, which is makes it a challenge to the physician. PMID:24404547

Nidimusili, Amara J.; Eisa, Naseem; Shaheen, Khaldoon

2013-01-01

341

Effect of time of weaning on the reproductive performance of Barbados Blackbelly ewes and lamb growth reared in the tropics  

Microsoft Academic Search

The effects of time of weaning on subsequent reproductive performance of ewes and the growth of lambs were evaluated in Barbados Blackbelly hair sheep, under a tropical photoperiod. Two experiments were conducted to assess the effect of time of weaning on subsequent reproductive performance of the ewe. In Experiment 1, lactating ewes (n=56) were weaned early (WE1; 72.6±1.3 days postpartum;

M. Knights; N. Siew; R. Ramgattie; D. Singh-Knights; G. Bourne

342

Dopaminergic-like activity in toxic fescue alters prolactin but not growth hormone or thyroid stimulating hormone in ewes.  

PubMed

Studies were conducted to determine the specificity and cause of altered pituitary hormone secretion when ewes ingest endophyte-infected (Acremonium coenophialum) GI-307 tall fescue (toxic fescue). Plasma concentrations of prolactin (PRL) but not growth hormone (GH) or thyroid stimulating hormone (TSH) in ewes grazing toxic fescue were significantly lower (P less than .01) than concentrations measured in ewes grazing orchardgrass (OG). Comparing hormone secretory responses of ewes grazing each grasstype, ewes on toxic fescue released less PRL following thyrotropin releasing hormone (TRH) challenge than ewes on OG. TSH responses to TRH were not affected by grasstype. At this dose of TRH, GH secretion was not significantly affected in either group of ewes. In a separate study, dopamine hydrochloride (DA) was infused into control ewes to define the effect of a pure dopamine agonist on basal and TRH-stimulated secretion of PRL, GH and TSH. DA depressed both basal and TRH-stimulated secretion of PRL without affecting the basal concentrations or responses of GH or TSH. Based on the assumption that the active agent in toxic fescue responsible for the observed hypoprolactinemia was a dopaminergic agonist, haloperidol (HAL), a DA receptor blocking drug, was administered to ewes grazing toxic fescue or OG. HAL evoked significant PRL secretion unaccompanied by any GH or TSH effect in both toxic fescue and OG ewes. Administration of HAL resulted in a gradual increase over 4 hr in PRL in toxic fescue ewes and prolonged the duration of the PRL response to TRH. No differences in circulating plasma concentrations of DA, epinephrine or norepinephrine were measured in ewes on troxic fescue or OG. Alterations in pituitary hormone secretion due to toxic factors in fescue were confined to PRL. Hormone secretory responses to TRH and HAL suggest that the effects on PRL are mediated through dopamine-like activity in toxic fescue. PMID:3507894

Elsasser, T H; Bolt, D J

1987-10-01

343

Effect of royal jelly and GnRH on the estrus synchronization and pregnancy rate in ewes using intravaginal sponges  

Microsoft Academic Search

The influence of natural royal jelly (RJ) paste and gonadotropin releasing hormone (GnRH) on reproductive responses of Awassi ewes was evaluated. In May 1998, 20 Awassi ewes (aged 2–5 years and weighing 52kg) were treated with 40mg fluorogestone acetate (FGA) impregnated intravaginal sponges for a period of 12 days. Half of the ewes were administered orally a total of 3g

R. T. Kridli; M. Q. Husein; W. D. Humphrey

2003-01-01

344

Effects of pre?lamb shearing on feed intake and associated productivity of May? and August?lambing ewes  

Microsoft Academic Search

The effects of pre?lamb shearing on feed intake and wool\\/lamb production of May (autumn)? and August (spring)?lambing ewes were examined. Mixed age Border Leicester x Romney ewes mated to lamb in May or August (60 ewes\\/ lambing policy) were divided at random into two groups (balanced for pregnancy status and liveweight). One group was shorn on Day 118 of pregnancy

N. Dabiri; S. T. Morris; M. Wallentine; S. N. Mccutcheon; W. J. Parker; G. A. Wickham

1996-01-01

345

Pulmonary Artery Sarcoma Masquerading as Chronic Pulmonary Thromboembolism  

PubMed Central

We describe the case of a 60-year-old woman who presented with pulmonary artery sarcoma, a very rare tumor of the cardiovascular system. Her tumor was initially misdiagnosed as chronic pulmonary thromboembolism, and she underwent pulmonary endarterectomy. Early diagnosis of primary pulmonary artery sarcoma is crucial. That alternative should always be considered before settling on a diagnosis of pulmonary embolism. Suspicion should be aroused by the failure of anticoagulant treatment to alleviate pulmonary perfusion abnormalities and systemic symptoms. Surgical resection of the tumor—preferably by pulmonary endarterectomy, followed by reconstruction as needed—is currently the most promising treatment for pulmonary artery sarcoma. PMID:25425987

Coskun, Ugur; Calpar, Ilknur; Yildizeli, Bedrettin; Yanartas, Mehmet; Filinte, Deniz; Kucukoglu, Mehmet Serdar

2014-01-01

346

Targeting Signal Transduction Pathways for the Treatment of Kaposi Sarcoma  

Microsoft Academic Search

\\u000a Kaposi sarcoma (KS) is a multi-focal angioproliferative disease driven by infection of cells by Kaposi sarcoma herpesvirus\\/human\\u000a herpesvirus-8 (KSHV\\/HHV8). KSHV\\/HHV8 infection activates numerous sequential and parallel signaling pathways creating an angiogenic–inflammatory\\u000a state that leads to the development of Kaposi sarcoma. These pathways can be grouped into KSHV\\/HHV8- specific pathways and\\u000a cellular growth\\/angiogenic pathways that are “pirated” by KSHV\\/HHV8. Advances in

Janet L. Douglas; Henry B. Koon; Ashlee V. Moses

347

Thymoma with Coexisting Undifferentiated Pleomorphic Sarcoma: A Case Report  

PubMed Central

We report here a case of thymoma simultaneously associated with undifferentiated pleomorphic sarcoma. A 45-year-old male presented with axillary lump. Radiographic studies showed a mediastinal mass. On fine needle aspiration cytology and histopathological examination, a diagnosis of thymoma with coexisting undifferentiated pleomorphic sarcoma was made. Although thymomas are associated with many extrathymic malignancies, it's association with undifferentiated pleomorphic sarcoma is rare. This case is being reported on to reinforce that clinicians should bear in mind the possibility of extrathymic malignancies in patients with thymomas. PMID:22937421

Varshney, Manoranjan; Shahid, Mohammad; Maheshwari, Veena; Mubeen, Aysha; Siddiqui, Mohammed Azfar

2011-01-01

348

Primary pericardial sarcoma: a case report and a brief review.  

PubMed

There are very few cases of primary pericardial sarcomas reported in the English literature. Pericardial tumors, like other cardiac tumors, are most likely to be metastatic in nature and are an extension of primary tumors from the surrounding structures. Sarcomas are the most common primary pericardial tumors. Surgical eradication of the tumor is considered to be the treatment of choice. We are presenting a case of a primary pericardial, high-grade pleomorphic undifferentiated sarcoma that was diagnosed at our institution. We discuss the available diagnostic modalities and also shed light on alternative therapies when patients are not ideal surgical candidates. PMID:21559228

Pakala, Aneesh; Gupta, Raghav; Lazzara, Ralph

2011-01-01

349

Primary Pericardial Sarcoma: A Case Report and a Brief Review  

PubMed Central

There are very few cases of primary pericardial sarcomas reported in the English literature. Pericardial tumors, like other cardiac tumors, are most likely to be metastatic in nature and are an extension of primary tumors from the surrounding structures. Sarcomas are the most common primary pericardial tumors. Surgical eradication of the tumor is considered to be the treatment of choice. We are presenting a case of a primary pericardial, high-grade pleomorphic undifferentiated sarcoma that was diagnosed at our institution. We discuss the available diagnostic modalities and also shed light on alternative therapies when patients are not ideal surgical candidates. PMID:21559228

Pakala, Aneesh; Gupta, Raghav; Lazzara, Ralph

2011-01-01

350

Biological Extremity Reconstruction after Sarcoma Resection: Past, Present, and Future  

PubMed Central

In sarcoma surgery besides a wide local resection, limb salvage became more and more important. Reconstruction of bone and soft tissue defects after sarcoma resection poses a major challenge for surgeons. Nowadays a broad range of reconstructive methods exist to deal with bony defects. Among these are prostheses, bone autografts, or bone allografts. Furthermore a variety of plastic reconstructive techniques exist that allow soft tissue reconstruction or coverage after sarcoma resection. Here we discuss the historical highlights, the present role, and possible future options for biological reconstruction. PMID:23840167

Holzer, Lukas A.; Leithner, Andreas

2013-01-01

351

A case of interdigitating dendritic cell sarcoma/histiocytic sarcoma – a diagnostic pitfall  

PubMed Central

Interdigitating dendritic cell sarcoma (IDCS) and histiocytic sarcoma (HS) are two distinct rare hematolymphoid neoplasms, and HS derived from a likely pre-existing IDCS has never been reported in the English literature. Diagnosis of such entities in excised specimens is difficult, but becomes more difficult with the scant amount of materials obtained with fine needle aspiration (FNA) and core needle biopsy. Here we present an interesting and unique case of an IDCS located within a mesenteric mass, which was initially diagnosed as IDCS from the cytology of FNA and core needle biopsy specimens. After brief chemotherapy, the patient again developed abdominal pain, and a HS was diagnosed based on the excised segmental small intestinal specimen. While the exact relationship between the IDCS and HS cannot be ascertained, it is most likely that the HS is derived from the IDCS, although co-existing HS in addition to IDCS from the cytology specimen cannot be completely ruled out. PMID:24427360

Johnson, Rebecca L; Boisot, Saskia; Ball, Edward D; Wang, Huan-You

2014-01-01

352

Molecular mechanisms of endometrial stromal sarcoma and undifferentiated endometrial sarcoma as premises for new therapeutic strategies.  

PubMed

Endometrial stromal sarcoma (ESS) and undifferentiated endometrial sarcoma (UES) are very rare gynecologic malignancies. Due to the rarity and heterogeneity of these tumors, little is known about their epidemiology, pathogenesis, and molecular pathology. Our previous studies have described deregulation of histone deacetylases expression in ESS/UES samples. Some of these enzymes can be inhibited by substances which are already approved for treatment of cutaneous T-cell lymphoma. On the basis of published data, they may also provide a therapeutic option for ESS/UES patients. Our review focuses on molecular mechanisms of ESS/UES. It describes various aspects with special emphasis on alteration of histone deacetylation and its possible relevance for novel therapies. PMID:25128649

Hrzenjak, Andelko; Dieber-Rotheneder, Martina; Moinfar, Farid; Petru, Edgar; Zatloukal, Kurt

2014-11-01

353

Primary central nervous system histiocytic sarcoma presenting as a postradiation sarcoma: case report and literature review.  

PubMed

Histiocytic sarcoma (HS) is a rare neoplasm that occurs most commonly in the intestinal tract, skin, soft tissue, and lymph node. The incidence of primary central nervous system (CNS) HS is even rarer, with a total of 6 cases reported in the literature. An etiologic link has not been identified for CNS HS, and the current case of primary CNS HS is unique in that an etiologic link to prior radiation therapy is identified, associated with complex cytogenetic abnormalities in the tumor. Although radiation-associated sarcomas can present as any number of different pathologic entities, this is the first reported case of a radiation-associated CNS HS. The pathologic and immunophenotypic characteristics of this case, with a nearly obscuring heavy inflammatory infiltrate and expression of monocytic/histiocytic markers (CD163, CD68, CD4, fascin), are characteristic of CNS HS. A discussion of the differential diagnosis and review of relevant literature are presented. PMID:23356953

Wu, Winnie; Tanrivermis Sayit, Asli; Vinters, Harry V; Pope, Whitney; Mirsadraei, Leili; Said, Jonathan

2013-06-01

354

Restricted Expression of Kaposi Sarcoma-Associated Herpesvirus (Human Herpesvirus 8) Genes in Kaposi Sarcoma  

Microsoft Academic Search

Kaposi sarcoma (KS) is the leading neoplasm of HIV-infected patients and is also found in several HIV-negative populations. Recently, DNA sequences from a novel herpesvirus, termed KS-associated herpesvirus (KSHV), or human herpesvirus 8 (HHV-8) have been identified within KS tissue from both HIV-positive and HIV-negative cases; infection with this agent has been proposed as a possible factor in the etiology

Weidong Zhong; Hao Wang; Brian Herndier; Don Ganem

1996-01-01

355

Ifosfamide plus etoposide combined with regional hyperthermia in patients with locally advanced sarcomas: a phase II study.  

PubMed

From July 1986 to July 1989, 40 patients (92% pretreated) with deep-seated, advanced soft tissue sarcomas (STS, 25 patients), Ewing's sarcomas (ES, eight patients), osteosarcomas (OS, three patients) and chondrosarcomas (ChS, four patients) were treated at the University of Munich in a protocol involving regional hyperthermia (RHT) combined with ifosfamide plus etoposide. A total of 265 RHT treatments (mean, 6.6 RHT per patient) were applied including 33 pelvic, four extremity, and three abdominal sites. The mean tumor volume was 537 cc (range, 50 to 2,980 cc). For systemic chemotherapy, all patients received ifosfamide (1.5 g/m2, days 1 to 5), etoposide (100 mg/m2, days 1, 3, and 5), and mesna (300 mg/m2 x 4, days 1 to 5) with RHT given only on days 1 and 5 in repeated cycles every 4 weeks. Acute toxicity consisted primarily of pain (57%) combined with local discomfort within the annular phased array applicator (AA) of the BSD hyperthermia system (BSD Medical Corp, Salt Lake City, UT). The average maximum systemic temperature was 37.4 +/- 0.5 degrees C, and there was no indication of enhanced bone marrow toxicity due to the addition of RHT to the systemic chemotherapy. Detailed thermal mapping by invasive thermometry was performed in all patients. In 38 assessable patients, the overall objective response rate was 37%: six complete responses (CRs), four partial responses (PRs), and four favorable histologic responses (FHRs) (95% confidence limits, 22% to 54%). Complete responders are alive and disease-free at 40, 35, 23, 19, 19, and 8 months. Of patients with PR and FHR, two died from metastatic disease after 4 and 17 months and one died from other disease after 27 months. The remaining five patients are stable at 37, 25, 21, 13, and 8 months. Eleven patients showed no change (NC), and 13 patients showed local tumor progression (PD). The mean observation time for all patients was 11.6 months. The time-averaged temperatures (Ts) of all RHT treatments calculated as 20% (T20), 50% (T50), or 90% (T90) of measured tumor sites differed significantly between responders and nonresponders (T20, P = .003; T50, P = .006; and T90, P = .004; respectively). These data support activity for ifosfamide-etoposide combined with RHT in pretreated patients with advanced sarcomas. PMID:2121910

Issels, R D; Prenninger, S W; Nagele, A; Boehm, E; Sauer, H; Jauch, K W; Denecke, H; Berger, H; Peter, K; Wilmanns, W

1990-11-01

356

Soft-tissue sarcoma of the foot  

PubMed Central

Background We reviewed cases of soft-tissue sarcoma of the foot to gain insight into the presentation, treatments and outcomes for this rare disease and to determine whether limb-salvage surgery yields reasonable functional and oncological outcomes. Methods We reviewed the cases of 16 patients treated by 2 of us (R.T. and M.I.) for soft-tissue sarcoma of the foot over a 15-year period. We extracted the following information from each patient’s medical record: disease status at presentation, prior treatment, histological diagnosis, American Joint Committee on Cancer (AJCC) stage, details of treatment, oncological outcome and functional outcome. Functional outcome was assessed with the Toronto Extremity Salvage Score (TESS) and the Musculoskeletal Tumor Society (MSTS 1987). Results Follow-up averaged 6 (range 2–15) years. Eight patients presented after unplanned excision. Histological diagnosis was synovial sarcoma for 7 of 16 patients. The tumours were evenly distributed among the hindfoot, midfoot and forefoot. Most patients (n = 13) presented with AJCC stage II or III disease. Amputation was necessary for 3 patients, whereas limb salvage was possible for the other 13. Free tissue transfer (n = 9) and radiation therapy (n = 12) were used in most cases. Surgical margins were microscopically positive in 4 of the 13 patients treated with limb salvage. Local disease recurred in 2 patients. Lung metastases occurred in 4 patients. At last follow-up, 11 of 16 patients were alive without disease, 2 with disease and 3 had died of their disease. Functional assessment with MSTS 1987 and the TESS averaged 28% and 90%, respectively, after limb salvage. Conclusion In this series, we found that, first, patients frequently presented after unplanned excision, and this may have led to worse oncological outcomes compared with patients who presented primarily. Second, limb salvage was usually possible, but it required accepting marginal resections, relying on free tissue transfer to obtain coverage and using radiation therapy to obtain local control. Third, this combination yielded an acceptable local control rate and very good functional outcomes. PMID:21092437

Latt, L. Daniel; Turcotte, Robert E.; Isler, Marc H.; Wong, Cynthia

2010-01-01

357

What Happens After Treatment for Soft Tissue Sarcomas?  

MedlinePLUS

... Article Close Push escape to close saved articles window. My Saved Articles » My ACS » Sarcoma: Adult Soft Tissue Cancer + - Text Size Download Printable Version [PDF] » After Treatment TOPICS Document Topics GO » SEE A LIST » What ...

358

Intra-articular calcifying synovial sarcoma mimicking synovial chondromatosis.  

PubMed

A case of intra-articular calcifying synovial sarcoma of the right knee joint in a 27-year-old man is reported. Prior to admission the patient had been suffering pain in the right knee for 5 years. Plain radiographs showed multiple calcified densities in the anterior aspect of the right knee closely mimicking synovial chondromatosis. Histological examination revealed that the lesion was a synovial sarcoma with extensive calcification subjacent to the synovial surface. Although intra-articular calcifying synovial sarcoma is very rare, it is important to be aware of the fact that synovial sarcoma, as well as synovial chondromatosis, can be present as a calcified lesion in the joint cavity. PMID:8958625

Ishida, T; Iijima, T; Moriyama, S; Nakamura, C; Kitagawa, T; Machinami, R

1996-11-01

359

What Are the Risk Factors for Uterine Sarcoma?  

MedlinePLUS

... to 25 years after exposure to the radiation. Race Uterine sarcomas are about twice as common in ... College Relay For Life Relay Recess Donate a Car About ACS About Us Contact Us Local Offices ...

360

Familial Kaposi's sarcoma and Paget's disease of bone.  

PubMed

Familial Kaposi's sarcoma and familial Paget's disease of bone have not previously been reported to occur in the one patient or the one family. We report on an 82-year-old female of Lebanese descent who was recently diagnosed with Kaposi's sarcoma and Paget's disease. Of the patient's eight siblings, seven had Paget's disease and two of these also had Kaposi's sarcoma. Histocompatibility leucocyte antigen (HLA) class I and II typing of the patient showed: A2, A3; B35, Bx; Bw6; Cw4; DR beta 1*1101 (an HLA-DR5 subtype), DR beta 3 and DQ beta 1*0301. Previous reports have described possible associations of familial Kaposi's sarcoma with HLA-DR5 and Paget's disease with DR2. DR beta 1*1104, DP beta 1*04 and DQw1. Genetic factors and possible viral aetiologies for each condition are reviewed. PMID:9838721

Hale, L R; Kelly, J W

1998-11-01

361

Thalidomide Shows Activity Against AIDS-Related Kaposi's Sarcoma  

Cancer.gov

The drug thalidomide, used in the 1950s and 1960s as a sedative which led to birth defects in newborns of mothers who took the drug, has now been shown to have clinical activity against Kaposi's sarcoma (KS).

362

An Insidious Cardiac Sarcoma Presenting with Progressive Neurologic Dysfunction  

PubMed Central

Central nervous system metastases as the presentation of primary cardiac sarcoma are a very rare entity, with only a few previously reported cases. Sarcomas specifically make up 10 to 20% of all primary cardiac tumors. Patients with primary cardiac tumors typically present with cardiac symptomatology that may include arrhythmias, obstruction to blood flow and valve function, or symptoms of heart failure. We report a unique case of a patient with a primary cardiac sarcoma who presented with progressive neurologic dysfunction secondary to brain metastases without any preceding cardiac symptoms. We describe our novel management of these unique cases and discuss the current medical and surgical approaches to treating neurologic metastases from cardiac sarcoma. PMID:24416490

Pasalic, Dario; Hegerova, Livia T.; Gonsalves, Wilson I.; Robinson, Steven

2013-01-01

363

NCI Researchers Identify Novel Mechanism for Spread of Sarcoma Tumors  

Cancer.gov

A team of researchers at the National Cancer Institute (NCI), using a mouse model, have uncovered a novel protein interaction that promotes the spread of cancer cells (a process known as metastasis) in a class of tumors collectively called sarcomas.

364

What Are the Risk Factors for Soft Tissue Sarcoma?  

MedlinePLUS

... soft tissue sarcoma. Exposure to dioxin and to herbicides that contain phenoxyacetic acid at high doses (such ... known for certain. There is no evidence that herbicides (weed killers) or insecticides, at levels encountered by ...

365

Short-term nutritional treatments grazing legumes or feeding concentrates increase prolificacy in Corriedale ewes.  

PubMed

We tested whether short periods of increased nutrition will improve ovulation rate and prolificacy, irrespective of the method used to synchronise the cycles of the ewes. In Experiment 1, we used 138 Corriedale ewes to evaluate two factors: synchronization treatment (sponges versus a single injection of prostaglandin) and type of pasture (native versus improved with Lotus corniculatus). Ewes were mated at the end of the grazing period and prolificacy was evaluated at lambing. Grazing Lotus corniculatus for 12 days tended to increase the number of twin lambs born (P=0.09). The percentage of ewes showing oestrus during a 9-day period was similar among synchronization treatments. Animals in Experiments 2 (n=282) and 3 (n=288) were allocated to a control group or a group fed a supplement of corn grain and soybean meal for 7 days. Ewes received 2 prostaglandin injections and the supplement was fed from Days 11 to 17 after the second prostaglandin. Ovulation rate was measured in 65 (Experiment 2) and 61 (Experiment 3) ewes that were confirmed to have consumed the supplement and showed oestrus in a 4-day period. The supplement increased ovulation rate by 14% in both experiments (P<0.05). We conclude that Corriedale ewes can respond with increases in prolificacy to a 12-day period grazing Lotus corniculatus and in ovulation rate to 7 days feeding with a supplement rich in energy and protein. Moreover, in these studies, prostaglandin was as effective as sponges for synchronising oestrus, an important factor in future decisions about hormonal management of fertility. PMID:18639397

Viñoles, C; Meikle, A; Martin, G B

2009-07-01

366

Uterine artery embolization in an undiagnosed uterine sarcoma  

Microsoft Academic Search

Background: Uterine sarcomas are rare malignancies that resemble benign uterine leiomyomata. Uterine artery embolization is offered increasingly for treatment of uterine leiomyomata, which might lead to embolization of undiagnosed uterine sarcoma.Case: A 52-year-old woman, gravida 7, para 6, with perimenopausal menometrorrhagia was diagnosed with uterine leiomyomata after physical examination and transvaginal ultrasound. An endometrial biopsy was negative for malignancy. After

Ahmed Al-Badr; Wylam Faught

2001-01-01

367

Molecular virology of Kaposi's sarcoma-associated herpesvirus  

Microsoft Academic Search

Kaposi's sarcoma-associated herpesvirus (KSHV), the most recently discovered human tumour virus, is the causative agent of Kaposi's sarcoma, primary e¡usion lymphoma and some forms of Castleman's disease. KSHV is a rhadinovirus, and like other rhadinoviruses, it has an extensive array of regulatory genes obtained from the host cell genome. These pirated KSHV proteins include homologues to cellular CD21, three di¡erent

Patrick S. Moore; Yuan Chang

2001-01-01

368

Sarcoma Metastatic to the Brain: A Series of 15 Cases  

Microsoft Academic Search

MethodsWe report on 15 patients surgically treated for intraparenchymal brain metastases from sarcoma, including six osteosarcomas, five leiomyosarcomas, two malignant fibrous histiocytomas, and two alveolar soft-part sarcomas (ASPS).ResultMedian survival after craniotomy was 9.3 months. Patients with a preoperative Karnofsky performance score of > 70 survived for 12.8 versus 5.3 months for those with a Karnofsky performance score < 70 (p

Maurizio Salvati; Luigi Cervoni; Riccardo Caruso; Franco Maria Gagliardi; Roberto Delfini

1998-01-01

369

Non-neoplastic soft tissue masses that mimic sarcoma.  

PubMed

Non-neoplastic soft tissue masses may mimic soft tissue sarcoma in a wide variety of clinical settings. Systematic and thorough review of patient history, physical examination, imaging, laboratory results, and biopsy will allow the clinician to differentiate between the two in most cases. We describe several common non-neoplastic entities that may mimic soft tissue sarcoma in case presentation format along with the characteristics that help distinguish them. PMID:24684918

Colman, Matthew W; Lozano-Calderon, Santiago; Raskin, Kevin A; Hornicek, Francis J; Gebhardt, Mark

2014-04-01

370

Myxoinflammatory Fibroblastic Sarcoma: An Uncommon Tumour at an Unusual Site  

PubMed Central

Myxoinflammatory fibroblastic sarcoma is a low grade sarcoma that is composed of a mixed inflammatory infiltrate along with spindled, epithelioid and bizarre appearing cells in a background of hyaline and myxoid zones. Seen affecting the distal extremities commonly, with an equal sex predilection, these tumors are rare and require an extensive immunohistochemical work up for proper diagnosis. They have a tendency to recur. PMID:24891666

Mallya, Varuna; Singh, Avninder; Siraj, Fouzia; Ramesh, V

2014-01-01

371

Mast cell sarcoma of the larynx.  

PubMed Central

A 74 year old woman presented with a primary subglottic tumour. Neither cutaneous mastocytosis (urticaria pigmentosa) nor spread to the bone marrow, liver, or spleen were detected. About two years after initial manifestation of the tumour nodular skin metastases appeared, as well as local recurrence in the larynx. Despite chemotherapy and radiation the disease progressed and was fatal. The terminal phase was characterised by generalisation of the mast cell tumour with diffuse infiltration of bone marrow and, shortly before death, leukaemic transformation. The patient died four years after onset of disease with symptoms of a hemorrhagic diathesis. As far as we know this is the first case of mast cell sarcoma to be reported in man. Images PMID:3088063

Horny, H P; Parwaresch, M R; Kaiserling, E; Muller, K; Olbermann, M; Mainzer, K; Lennert, K

1986-01-01

372

Kaposi Sarcoma, CSR 1975-2003  

Cancer.gov

Under 65 51.9 52.1 45.5f 55.0 54.8 64.4f 42.2 43.1 24.4f 65 and over 85.7f 84.6f 82.8f 86.3f 86.0f 83.5f 72.0g - - Table X-6 KAPOSI SARCOMA (Invasive) SURVIVAL RATES, BY RACE, SEX, DIAGNOSIS YEAR, STAGE AND AGE SEER Cancer Statistics Review 1975-2003 National Cancer Institute a Survival rates are relative rates expressed as percents. b Rates are from the SEER 9 areas (San Francisco, Connecticut, Detroit, Hawaii, Iowa, New Mexico, Seattle, Utah, and Atlanta).

373

Kaposi Sarcoma, CSR 1975-2005  

Cancer.gov

Under 65 54.2 54.5 43.2f 56.6 56.5 62.9f 43.1 44.2 19.7f 65 and over 85.9 87.0 78.5f 87.7 87.5 81.2f 75.3g 64.4g - Table X-6 KAPOSI SARCOMA SURVIVAL RATES, BY RACE, SEX, DIAGNOSIS YEAR, STAGE AND AGE SEER Cancer Statistics Review 1975-2005 National Cancer Institute a Survival rates are relative rates expressed as percents. b Rates are from the SEER 9 areas (San Francisco, Connecticut, Detroit, Hawaii, Iowa, New Mexico, Seattle, Utah, and Atlanta).

374

Kaposi Sarcoma, CSR 1975-2004  

Cancer.gov

Under 65 53.2 53.4 44.9f 55.3 55.2 63.6f 42.3 43.1 25.7f 65 and over 84.0 86.4f 77.3f 85.9 87.4f 80.8f 66.5g 39.8g - Table X-6 KAPOSI SARCOMA SURVIVAL RATES, BY RACE, SEX, DIAGNOSIS YEAR, STAGE AND AGE SEER Cancer Statistics Review 1975-2004 National Cancer Institute a Survival rates are relative rates expressed as percents. b Rates are from the SEER 9 areas (San Francisco, Connecticut, Detroit, Hawaii, Iowa, New Mexico, Seattle, Utah, and Atlanta).

375

[Brachytherapy for extremity soft tissue sarcomas].  

PubMed

The standard of care of local treatment for extremities soft tissue sarcomas relies on conservative surgery combined with external beam radiotherapy. Brachytherapy can be realized instead of external beam radiotherapy in selected cases, or more often used as a boost dose on a limited volume on the area at major risk of relapse, especially if a microscopic positive resection is expected. In these cases, this combination allows to obtain the best local control rates published. Close interaction and communication between radiation oncologist and surgeon are mandatory at the time of implantation to limit the risk of side effects. Long-term results are available for low-dose rate brachytherapy. Nowadays, pulsed dose rate is more often used. More limited experience has been reported for high dose rate. PMID:23402876

Delannes, M; Thomas, L; Brun, T; David, I; Ducassou, A

2013-04-01

376

Production by ewes rearing single or twin lambs: effects of dietary crude protein percentage and supplemental zinc methionine.  

PubMed

Eighty Targhee ewes with single or twin lambs were used to investigate the effects of chelated zinc methionine and level of dietary CP on milk and wool production, DMI, and ewe and lamb BW change. Treatments were arranged as a 2 x 2 x 2 factorial. Dietary treatments started 30 d prepartum and continued until 42 d postpartum. Milk production was estimated at 4, 10, 18, and 28 d postpartum. During gestation, DMI by ewes receiving zinc methionine and the 14.9% CP diet was greater (P < .10) than DMI by ewes not supplemented with zinc methionine and ewes fed the 11.3% CP diet. Ewes fed the 14.9% CP diet tended (P < .14) to gain more BW from 30 d prepartum to 4 d postpartum and lose more BW from 4 to 42 d postpartum than ewes fed the 11.3% CP diet. Ewes fed zinc methionine and the 14.9% CP diet produced more (P < .10) milk on d 28 than their counterparts. Ewes rearing twins produced more (P < .05) milk on d 4, 10, and 18 than ewes rearing single lambs. However, milk production on d 28 was not affected by number of lambs reared (P = .68). Lamb ADG at 28, 42, and 59 d postpartum was greater (P < .05) for both the zinc methionine treatment and the 14.9% CP diet. Zinc methionine (P = .11) and the 14.9% CP diet (P = .02) increased weaning weights by 6 and 9%, respectively. We conclude that both the zinc methionine supplement and the 14.9% CP diet resulted in more persistent milk production. In addition, when ewes and lambs were fed for an average of 42 d during early lactation, a 14.9% CP diet, and to a lesser degree, a chelated zinc methionine supplement, lamb weaning weights were improved. PMID:7665352

Hatfield, P G; Snowder, G D; Head, W A; Glimp, H A; Stobart, R H; Besser, T

1995-05-01

377

Soft tissue sarcoma and occupational exposures  

SciTech Connect

The associations between soft tissue sarcoma (STS) and occupational exposures were studied in a case-referent study in the southeast of Sweden. Exposure information was obtained through mailed questionnaires to 96 cases, 450 randomly selected population referents, and 200 cancer referents. Odds ratios (OR), were calculated for various occupational groups, and particularly, for occupations with potential exposure to chlorinated phenoxy herbicides and chlorophenols. In the analyses based on population referents, increased risks for soft tissue sarcoma were seen for especially gardeners (OR = 4.1), but also railroad workers (OR = 3.1); construction workers with exposure to impregnating agents (OR = 2.3), asbestos (OR = 1.8), or pressure impregnating agents (OR = 1.7); and unspecified chemical workers with potential exposure to phenoxy herbicides and/or chlorophenols (OR = 1.6). A similar pattern appeared when cancer referents were used although the numerical values of the odds ratios became different. A grouping of jobs resulted in Mantel-Haensel OR from 1.5 to 1.9 for farmers and forestry workers, dependent on referents used and even more increased OR for railroad workers and unspecified chemical workers with potential exposure to phenoxy herbicides and chlorophenols. The results of the study confirm rather than refute that phenoxy herbicides and chlorophenols could be of etiologic importance for STS; the high risk for gardeners, although based on a small number of individuals, was unexpected and remains unclear. Also, since other cancers were used as referents, no definite problems of recall bias should obtain in this material. None of the exposed groups had a higher proportion of smokers than the unexposed group.

Wingren, G.; Fredrikson, M.; Brage, H.N.; Nordenskjoeld, B.A.; Axelson, O. (University Hospital, Linkoeping (Sweden))

1990-08-15

378

Testicular Synovial Sarcoma: A Case Report  

PubMed Central

This paper reports a case of testicular synovial sarcoma with molecular genetic analysis. A 24-year-old male presented with painless scrotal mass. Ultrasonography showed a heterogeneous mass of 66 mm × 34 mm in size involving the inguinal region. Histological examination of a surgical biopsy showed a grade III monophasic growth pattern of spindle cell proliferation. Immunohistochemical analyses indicated positive staining for pancytokeratine and epithelial membrane antigen. Cytogenetic analysis showed the presence of CYT-SSX1 mutation, and CT scan showed non-specific pleural micro-nodules with a size of 7.5 mm. The patient had an extended left orchidectomy but was lost to follow-up for 1 year. A local recurrent scrotal mass of 32 mm × 25 mm, multiple inguinal lymph nodes, and increased pleural nodules, which were confirmed by histological examination, were treated with three cycles of adriamycine and ifosfamide chemotherapy, surgical resection, and radiotherapy with complete response. After 3 months, the patient developed local recurrence and pulmonary metastases that did not respond to second-line chemotherapy based on gemcitabine and paclitaxel. The patient had dyspnea at the time of this writing and chest pain, and is under third-line chemotherapy based on Deticene after 30 months of following up. This patient died on November 16, 2012 after a resperatory failure and malignant pelural effusion. Synovial sarcoma should be considered in the differential diagnosis of soft tissue tumor and it should be aggressively treated to improve prognosis. Although our patient has shown numerous factors of bad prognosis, he has had a relatively long survival time. PMID:23691490

Nesrine, Mejri; Sellami, Rym; Doghri, Raoudha; Rifi, Hela; Raies, Henda; Mezlini, Amel

2012-01-01

379

Advances in the Systemic Treatment of Cutaneous Sarcomas  

PubMed Central

Cutaneous sarcomas are a rare sub-set of soft tissue sarcomas. These tumors are managed with definitive surgical resection however upon unresectable recurrence or metastatic spread, systemic therapy is warranted in their treatment. As with other sarcomas, these treatments have classically included cytotoxic chemotherapy programs that were associated with variable response rates and poor overall survival. Recently, major advances have been made in the understanding of the molecular biology these tumors and as such treatment paradigms are changing. Multiple growth pathways have been documented to be important in the growth of cutaneous sarcomas, including receptor tyrosine kinases such as platelet-derived growth factor receptor, insulin-like growth factor receptor and c-KIT. Dysregulated angiogenesis, through VEGF and other pathways, is associated with the growth of these tumors. As such, new treatments have entered the clinical arena and a host of novel therapies are in clinical development. In this review, we discuss the current standard therapies of cutaneous sarcoma and discuss the recent advances and on-going investigations into cutaneous sarcoma biology. PMID:22484189

Luke, Jason J; Keohan, Mary-Louise

2013-01-01

380

Weaning and the ram-effect on fertility, serum luteinizing hormone and prolactin levels in spring rebreeding of postpartum Awassi ewes  

Microsoft Academic Search

Spring rebreeding activity was investigated in 44 multiparous Awassi ewes allocated to one of three treatments. The first treatment entailed ewes suckling lambs for 45 days and then continuously exposed to fertile rams (Group 1); ewes of the second group were weaned at 14 days of age and then were either exposed continuously (Group 2) or intermittently (Group 3) to

S. K. Hamadeh; M. Abi Said; F. Tami; E. K. Barbour

2001-01-01

381

The Relationship between Selenium and T3 in Selenium Supplemented and Nonsupplemented Ewes and Their Lambs  

PubMed Central

Twenty pregnant ewes were selected and classified into two groups. The first group received subcutaneous selenium supplementation (0.1 mg of sodium selenite/kg BW) at the 8th and 5th weeks before birth and 1st week after birth while the other was control group without selenium injection. Maternal plasma and serum samples were collected weekly from the 8th week before birth until the 8th week after birth and milk samples were taken from ewes weekly, while plasma and serum samples were collected at 48 hours, 1st, 2nd, 3rd, 5th, and 8th weeks after birth from the newborn lambs. Results demonstrated significant positive relationship between maternal plasma selenium and serum T3 in supplemented and control ewes (r = 0.69 to 0.72, P < 0.05). There was significant (P < 0.001) increase in T3 in supplemented ewes and their lambs until the 8th week after birth. There was positive relationship between milk, selenium concentration, and serum T3 in the newborn lambs of the supplemented group (r = 0.84, P < 0.01), while the relationship was negative in the control one (r = ?0.89, P < 0.01). Muscular and thyroid pathological changes were independent of selenium supplementation. Selenium supplementation was important for maintaining T3 in ewes and newborn lambs until the 8th week after birth. PMID:24660087

Hefnawy, Abd Elghany; Youssef, Seham; Aguilera, P. Villalobos; Rodriguez, C. Valverde; Perez, J. L. Tortora

2014-01-01

382

Quantification of progesterone binding in mammary tissue of pregnant ewes  

SciTech Connect

Progestin-binding sites in mammary tissue from 14 prepartum, multiparous ewes at 50, 80, 115, and 140 d of gestation were demonstrated by the binding of (/sup 3/H) R5020 (17,21-dimethyl-19-nor-4,9-pregnadiene-3,20-dione) to ovine mammary cytosol in the presence of sodium molybdate and excess cortisol. Homogenization extracted 89% of total mammary receptors (nuclear) into cytosol. Binding was specific for progestins and was of high affinity. The average dissociation constant for (/sup 3/H) R5020 specifically bound to receptors extracted into mammary cytosol was 1.9 (+/- .4) x 10/sup -9/ M (n = 14) and did not change significantly over the test period. However, binding capacities (fmol/mg cytosolic protein) differed according to stage of gestation with averages of 125 +/- 53, 149 +/- 26, 656 +/- 216, 57 +/- 22 at 50, 80, 115, and 140 d of pregnancy, respectively. Increased number of progestin-binding sites at 115 d of gestation (whether data are expressed per unit of tissue weight, DNA, or cytosolic protein) suggests that an increase per mammary epithelial cell may be necessary to produce the full lobuloalveolar proliferation observed at this stage of gestation.

Smith, J.J.; Capuco, A.V.; Akers, R.M.

1987-06-01

383

Radiation-associated sarcoma: A review of 23 patients with postradiation sarcoma over a 50-year period  

SciTech Connect

Between 1934 and 1983, 23 patients with well-documented diagnosis of radiation-associated sarcoma (RAS) were seen at the University of Michigan Medical Center. The median latent period from irradiation to diagnosis of RAS was 13 years with a minimum latent period of 3 and a maximum of 34 years. All sarcomas originated in previously normal tissues within the irradiated field. Pathology slides available in all patients were reviewed by the same pathologist for the purpose of the study, and the diagnosis of sarcoma was confirmed histologically. There were five bone sarcomas and 18 soft tissue sarcomas. Thirteen patients developed radiation-associated sarcoma following megavoltage treatment with a minimum total radiation dose of 25 Gy in 2 1/2 weeks. The other 10 patients received orthovoltage and/or brachytherapy irradiation alone or combined with external beam radiation. In this group, the radiation doses ranged from 25 Gy to 72 Gy except for one patient who received 8 Gy delivered by orthovoltage irradiation as treatment of knee arthritis. Four patients were originally treated for benign conditions. All the other patients (n = 19) received radiation therapy for a variety of primary malignancies including carcinoma of the cervix (n = 4), brain gliomas (n = 13), Wilm's tumors (n = 2) and retinoblastomas (n = 2), among others.

Amendola, B.E.; Amendola, M.A.; McClatchey, K.D.; Miller, C.H. Jr. (Hahnemann Univ., Philadelphia, PA (USA))

1989-10-01

384

The Effect of Ewe Body Condition at Lambing on Colostral Immunoglobulin G Concentration and Lamb Performance1,2  

Microsoft Academic Search

Body condition was scored at lambing (BCSL) on 101 mature ( 4 to 7 yr old) Polypay ewes and related to colostral immunoglobulin G (IgG) concentration and lamb performance. Colostrum sam- ples were collected from each ewe within 12 h of lambing and litters of more than two lambs were reduced to two within 2 h of lambing. Colostral IgG

T. A. Al-Sabbagh; L. V. Swanson; J. M. Thompson

385

The effect of condensed tannins in Lotus corniculatus upon reproductive efficiency and wool production in ewes during autumn  

Microsoft Academic Search

A grazing experiment, conducted for 81 days (from 13 February to 4 May) in the late summer\\/autumn of 1999 at Massey University, Palmerston North, New Zealand, compared the reproductive efficiency and wool growth of ewes grazing Lotus corniculatus (birdsfoot trefoil) or perennial ryegrass (Lolium perenne)\\/white clover (Trifolium repens) pasture (referred to as pasture). Half the ewes grazing L. corniculatus were

B. R Min; J. M Fernandez; T. N Barry; W. C McNabb; P. D Kemp

2001-01-01

386

Managing anthelmintic resistance: Untreated adult ewes as a source of unselected parasites, and their role in reducing parasite populations  

Microsoft Academic Search

AIMS: To test the hypotheses that when untreated adult ewes are rotationally grazed (follow behind) on pastures after lambs receiving routine anthelmintic treatments, the ewes can function as a source of unselected parasites in refugia, capable of slowing the development of anthelmintic resistance, and suppress the build-up of parasites resulting from the development of anthelmintic resistance.METHODS: Firstly, the potential of

DM Leathwick; CM Miller; DS Atkinson; NA Haack; TS Waghorn; A-M Oliver

2008-01-01

387

Nutritional status of adult ewes during early and mid-pregnancy. 1. Effects of plane of nutrition on ewe reproduction and offspring performance to weaning.  

PubMed

The objective of this study was to determine the effects of plane of nutrition during early and mid-pregnancy on the performance of mature ewes and their offspring. From day 0 to day 39 post mating (early pregnancy, EP), 82 multiparous ewes were fed to provide either 60% (low, L), 100% (medium, M) or 200% (high, H) of predicted metabolisable energy (ME) requirements for maintenance, following a synchronised mating. From day 40 to day 90 (mid-pregnancy, MP), ewes were provided with either 80% (M) or 140% (H) of ME requirements. After 90 days of gestation, all ewes were fed to meet requirements for late pregnancy. During EP, mean live weight (LW) and body condition score (BCS) change of ewes were -6.3, -0.8 and +6.0 kg and -0.02, +0.10 and +0.22 units in the L-, M- and H-EP treatments, respectively. During MP, mean LW and BCS change were -0.8 and +4.9 kg and -0.09 and +0.09 units in the M- and H-MP treatments, respectively (P < 0.001). Treatments had no effect (P > 0.05) on conception rate, although there tended to be an inverse relationship (P = 0.085) between plane of nutrition in EP and plasma progesterone concentrations at day 42 of gestation. EP nutrition influenced foetal development with lambs from ewes offered diet L-EP being smaller (P < 0.01) at day 56 than M- or H-EP lambs. However, at parturition L-EP lambs were heavier (P < 0.05) and tended to have higher (P = 0.056) immunoglobulin status 24 h after birth. Mortality rates at weaning were reduced (P < 0.05) for lambs born from ewes offered diet L-EP compared with M- or H-EP lambs. Diet M during mid-pregnancy resulted in larger (P < 0.05) foetuses at day 80 of gestation. At parturition, these lambs had longer head and crown-rump lengths than H-MP lambs (P < 0.05). Lambs born to ewes offered diet M-MP tended to progress faster to attempting to suckle than H-MP lambs (P = 0.089). There was an interaction between plane of nutrition in early and mid-pregnancy, whereby the highest number of lambs weaned was a result of diet L-EP followed by diet M-MP. These results indicate that in adult ewes, temporary nutrient restriction during early pregnancy results in better lamb survival; and mild nutrient restriction in mid-pregnancy tends to improve neonatal behaviour and results in lambs with longer skeletal size. PMID:22444963

Muñoz, C; Carson, A F; McCoy, M A; Dawson, L E R; O'Connell, N E; Gordon, A W

2008-01-01

388

Cross Species Genomic Analysis Identifies a Mouse Model as Undifferentiated Pleomorphic Sarcoma/Malignant Fibrous Histiocytoma  

E-print Network

Undifferentiated pleomorphic sarcoma/Malignant Fibrous Histiocytoma (MFH) is one of the most common subtypes of human soft tissue sarcoma. Using cross species genomic analysis, we define a geneset from the LSL-Kras[superscript ...

Jacks, Tyler E.

389

Comparison of weigh-suckle-weigh and machine milking for measuring ewe milk production.  

PubMed

Thirteen crossbred ewes were used to compare weigh-suckle-weigh (WSW) and machine milking (MM) methods for determining milk production of ewes that were rearing single or twin lambs. At parturition, ewes were 13 mo of age and produced six single lambs and seven pairs of twin lambs. Milk production estimates were initiated on d 6 of lactation and a 3-d rotation of the two techniques was implemented. On d 6, milk production was measured using WSW; on d 7, MM was used. No measurement was made on d 8. The 3-d rotation was repeated 20 times throughout a 63-d lactation, resulting in 20 point estimates of milk production for each method of measurement for each ewe. The WSW procedure consisted of a 3-h period in which lambs were withheld from suckling their dams. This was followed by a suckling period, a second 3-h withholding period, and a second suckling period. Differences in pre- and postsuckling lamb weights of the second suckling period were defined as milk consumption and, indirectly, 3-h milk production. The MM procedure included an administration of 10 IU of oxytocin (i.v.), followed by evacuation of the udder with a machine using commercially available sheep milking equipment, and the milk was discarded. Lambs were withheld from suckling the ewes for a 3-h period, followed by a repetition of the oxytocin and machine milking procedures. Milk from the second milking was weighed. Milk production estimates determined using the WSW and MM techniques were similar (P = .42). Average 3-h milk production was 340 and 351 g for WSW and MM, respectively. Machine milking provides a reliable tool in evaluating the milk-producing ability of ewes that are rearing single or twin lambs. PMID:10492436

Benson, M E; Henry, M J; Cardellino, R A

1999-09-01

390

Effect of feeding flax or linseed meal on progesterone clearance rate in ovariectomized ewes.  

PubMed

Ovariectomized ewes (n=22; 68.76+/-2.34 kg initial body weight; 2.9+/-0.1 initial body condition score) were individually fed one of three diets: (1) control (phytoestrogen-free; n=7), (2) flax containing diet (n=8), or (3) linseed meal (LSM) containing diet (n=7) to investigate the rate of progesterone (P4) clearance. On day 20 of feeding (day 0=initiation of treatment), a P4 releasing device (CIDR) was placed in the vagina and jugular blood samples were obtained prior to CIDR insertion and 15, 30, 60, and 120 min following CIDR insertion. Further, blood samples were obtained daily between days 21 and 24. On day 25, blood samples were retrieved prior to CIDR removal and 2, 5, 10, 15, 30, 60, 120, and 360 min following CIDR removal. There was no difference in initial or final body weight or body condition score and there were no time by diet interactions on P4 clearance. The fractional rate of P4 uptake measured prior to CIDR insertion through day 4 following insertion tended to be greater (P=0.07) in LSM fed ewes (508.75+/-71.37%/min) compared to flax (295.39+/-66.76%/min) and control fed (287.54+/-71.37%/min) ewes. Diet tended (P=0.10) to influence P4 clearance rate when measured from prior to CIDR removal through 120 min following CIDR removal with LSM fed ewes having a greater (1.26+/-0.2) fractional rate constant than flax (0.929+/-0.09) and control fed (0.922+/-0.09) ewes. Flax fed ewes also had more (P<0.01) omega-3 fatty acids and total fatty acids in plasma. Reports of increased pregnancy rates in dairy cows fed flax may relate to P4 metabolism. PMID:18555637

Galbreath, Collin W; Scholljegerdes, Eric J; Lardy, Gregory P; Odde, Kenneth G; Wilson, Matthew E; Schroeder, Jerome W; Vonnahme, Kimberly A

2008-08-01

391

Evaluation of the effect of progesterone CIDR Devices on circulating levels of progesterone in cyclic ewes  

E-print Network

removal. Circulating progesterone levels were determined for each ewe through daily blood collection via jugular venipuncture. Each ewe underwent a pretreatment 25 day sampling period (Period 1), a 12 day treatment period characterized by the presence... iv Period 3: 1.3 ? 0.1 ng/ml versus 1.4 ? 0.1 ng/ml, respectively). Data collected in this study revealed that treatment with exogenous progesterone via CIDR for a 12- day treatment period does not influence circulating levels of progesterone...

Satterfield, Michael Carey

2005-02-17

392

Homology Exists among the Transforming Sequences of Avian and Feline Sarcoma Viruses  

Microsoft Academic Search

Fujinami sarcoma virus (FSV) of chickens does not contain nucleotide sequences related to the src gene of Rous sarcoma virus, but it carries unique sequences of at least 3000 bases, which are likely to code for the transforming protein of this virus. Using radioactive DNA complementary to FSV-unique sequences, we investigated the relatedness of FSV to other sarcoma-leukemia retroviruses in

M. Shibuya; T. Hanafusa; H. Hanafusa; J. R. Stephenson

1980-01-01

393

Fujinami Sarcoma Virus: An Avian RNA Tumor Virus with a Unique Transforming Gene  

Microsoft Academic Search

The oncogenic properties and RNA of the Fujinami avian sarcoma virus (FSV) and the protein it encodes were investigated and compared to those of other avian tumor viruses with sarcomagenic properties such as Rous sarcoma virus and the acute leukemia viruses MC29 and erythroblastosis virus. Cloned stocks of FSV caused sarcomas in all chickens inoculated and were found to contain

Wen-Hwa Lee; Klaus Bister; Anthony Pawson; Terry Robins; Carlo Moscovici; Peter H. Duesberg

1980-01-01

394

Pulmonary Kaposi's sarcoma in patients with acquired immune deficiency syndrome: a clinicopathological study  

Microsoft Academic Search

Pulmonary Kaposi's sarcoma may contribute to respiratory dysfunction in patients with acquired immune deficiency syndrome (AIDS) and features of pneumonitis. Opportunistic infections are readily recognised in endoscopic material, but pulmonary Kaposi's sarcoma is easily missed, so that patients are deprived of specific treatment. The clinical and pathological findings from nine cases of pulmonary Kaposi's sarcoma have been reviewed; these were

P J Fouret; J L Touboul; C M Mayaud; G M Akoun; J Roland

1987-01-01

395

Synovial Sarcoma Involving the Calcaneus and Plantar Compartment of the Foot: A Case Report  

Microsoft Academic Search

Synovial sarcoma arising in the foot is a rare finding. We report a 64-year-old female with synovial sarcoma involving the calcaneus and central plantar compartment. The patient presented with a 2-year history of painful heel with soft tissue mass presentation 21 months after initial pain. We performed an incisional biopsy with frozen section; histopathology was consistent with synovial sarcoma.

John Haight; Russell Caprioli; Michael Esposito; Antonio Macias; Maria Lucchese; Elijah Davis

2010-01-01

396

Progress in the molecular biology of ewing tumors.  

PubMed

Purpose/results/discussion. Rearrangement of the EWS gene with an ETS oncogene by chromosomal translocation is a hallmark of the Ewing family of tumors (EFT). Detectability, incidence, tumor specificity and variability of this aberration have been matters of intense investigation in recent years. A number of related alterations have also been found in other malignancies. The common consequence of these gene rearrangements is the generation of an aberrant transcription factor. In EFT, the ETS partner is responsible for target recognition. However, synergistic and possibly tissue-restricted transcription factors interacting with either the EWS or the ETS portion may influence target selection. Minimal domains of both fusion partners were defined that have proved necessary for the in vitro transformation of murine fibroblasts. These functional studies suggest a role for aberrant transcriptional regulation of transforming target genes by the chimeric transcription factors. Also, fusion of the two unrelated protein domains may affect overall protein conformation and consequently DNA binding specificity. Recent evidence suggests that EWS, when fused to a transcription factor, interacts with different partners than germ-line EWS. Variability in EWS-ETS gene fusions has recently been demonstrated to correlate with clinical outcome. This finding may reflect functional differences of the individual chimeric transcription factors. Alternatively, type and availability of specific recombinases at different time-points of stem cell development or in different stem cell lineages may determine fusion type. Studies on EFT cell lines using EWS-ETS antagonists do suggest a rate-limiting essential role for the gene rearrangement in the self-renewal capacity of EFT cells. The presence of additional aberrations varying in number and type that may account for immortalization and full transformation is postulated. Knowledge about such secondary alterations may provide valuable prognostic markers that could be used for treatment stratification. PMID:18521227

Kovar, H

1998-01-01

397

Cytogenetics and Molecular Genetics of Myxoid Soft-Tissue Sarcomas  

PubMed Central

Myxoid soft-tissue sarcomas represent a heterogeneous group of mesenchymal tumors characterized by a predominantly myxoid matrix, including myxoid liposarcoma (MLS), low-grade fibromyxoid sarcoma (LGFMS), extraskeletal myxoid chondrosarcoma (EMC), myxofibrosarcoma, myxoinflammatory fibroblastic sarcoma (MIFS), and myxoid dermatofibrosarcoma protuberans (DFSP). Cytogenetic and molecular genetic analyses have shown that many of these sarcomas are characterized by recurrent chromosomal translocations resulting in highly specific fusion genes (e.g., FUS-DDIT3 in MLS, FUS-CREB3L2 in LGFMS, EWSR1-NR4A3 in EMC, and COL1A1-PDGFB in myxoid DFSP). Moreover, recent molecular analysis has demonstrated a translocation t(1;?10)(p22;?q24) resulting in transcriptional upregulation of FGF8 and NPM3 in MIFS. Most recently, the presence of TGFBR3 and MGEA5 rearrangements has been identified in a subset of MIFS. These genetic alterations can be utilized as an adjunct in diagnostically challenging cases. In contrast, most myxofibrosarcomas have complex karyotypes lacking specific genetic alterations. This paper focuses on the cytogenetic and molecular genetic findings of myxoid soft-tissue sarcomas as well as their clinicopathological characteristics. PMID:22567356

Nishio, Jun; Iwasaki, Hiroshi; Nabeshima, Kazuki; Naito, Masatoshi

2011-01-01

398

Sarcomas arising after radiotherapy for peptic ulcer disease  

SciTech Connect

Therapeutic gastric irradiation has been used to reduce peptic juice secretion in patients with peptic ulcer disease. Between 1937 and 1968 a total of 2049 patients received such therapy at the University of Chicago. Three of these patients are known to have developed sarcomas in the field of radiation. Two gastric leiomyosarcomas of the stomach were diagnosed 26 and 14 years after treatment and a malignant fibrous histiocytoma of the anterior chest wall was removed six years after gastric irradiation. Of 743 peptic ulcer patients treated without irradiation and constituted as a control group for the study of therapeutic gastric radiation, none is known to have developed sarcoma. As the incidence of sarcoma in these patient groups is known only from the tumor registry of the University of Chicago, other cases of sarcoma may exist in the groups. While an increased incidence of sarcoma has not been proven to occur in patients who received therapeutic gastric irradiation for peptic ulcer disease, the possibility of such a risk should be borne in mind by physicians caring for such patients.

Lieber, M.R.; Winans, C.S.; Griem, M.L.; Moossa, R.; Elner, V.M.; Franklin, W.A.

1985-06-01

399

Novel pathways and molecular targets for the treatment of sarcoma.  

PubMed

Sarcomas collectively represent over 100 different subtypes of bone and soft tissue tumors of mesenchymal origin. The low response rate to cytotoxic chemotherapies has necessitated the need for development of either histologically driven or pathway-specific targeted therapies. As our understanding of the molecular mechanisms driving certain subtypes is rapidly advancing, the number of targeted therapies is also increasing. Recently identified novel druggable targets include the MDM2 amplifications in well-differentiated and dedifferentiated liposarcomas, the new translocation NAB2:STAT6 of solitary fibrous tumors, the angiopoeitin-TIE2 pathway in angiosarcoma, the suppression of Mcl1 in X:18/synovial sarcomas, the mTOR pathway in malignant peripheral nerve sheath tumors, CDK4 in alveolar rhabdomyosarcoma, cMET regulation in alveolar soft parts sarcoma, the metabolic abnormalities in wild-type/SHD GIST, and the lack of argininosuccinate synthetase 1 expression seen in most sarcomas. It is through a fundamental understanding of sarcoma biology that clinical trials based on molecular targets can be developed. PMID:23661264

Frith, Ashley E; Hirbe, Angela C; Van Tine, Brian A

2013-08-01

400

Myxoinflammatory fibroblastic sarcoma: morphologic and genetic updates.  

PubMed

Myxoinflammatory fibroblastic sarcoma (MIFS) is a malignant mesenchymal neoplasm most frequently arising in the distal extremities of adults, which usually behaves in a low-grade manner but is capable of metastasizing to local and distant sites, rarely leading to death. It is a rare tumor whose unusual morphology can lead to erroneous histologic diagnosis, either as a nonneoplastic (infectious or inflammatory) process or as a variety of neoplastic diseases. While its exact origin is uncertain, ultrastructural studies have shown at least some of the constituent cells to be modified fibroblasts. Distinct and reproducible genetic abnormalities identified in MIFS are translocation t(1;10)(p22:q24), with rearrangements of the TGFBR3 and MGEA5 genes associated with increased levels of FGF8, and formation of marker/ring chromosome 3, with amplification of the VGLL3 locus. Because these genetic abnormalities are shared by both MIFS and hemosiderotic fibrohistiocytic lipomatous tumor, it is thought that these 2 morphologically distinct neoplasms may comprise a spectrum of disease defined by these genetics. We review the literature on MIFS and discuss morphology (including that of MIFS/hemosiderotic fibrohistiocytic lipomatous tumor hybrid lesions), immunohistochemistry, the differential diagnosis, and recent molecular genetic developments. PMID:25268202

Ieremia, Eleni; Thway, Khin

2014-10-01

401

A Case of Inguinal Sparganosis Mimicking Myeloid Sarcoma  

PubMed Central

We report here a case of inguinal sparganosis, initially regarded as myeloid sarcoma, diagnosed in a patient undergone allogeneic hematopoietic transplantation (HSCT). A 56-year-old male patient having myelodysplastic syndrome was treated with allogeneic HSCT after myeloablative conditioning regimen. At day 5 post-HSCT, the patient complained of a painless palpable mass on the left scrotum and inguinal area. Pelvic magnetic resonance imaging and computed tomography revealed suspected myeloid sarcoma. Gun-biopsy was performed, and the result revealed eosinophilic infiltrations without malignancy. Subsequent serologic IgG antibody test was positive for sparganum. Excisional biopsy as a therapeutic diagnosis was done, and the diagnosis of sparganosis was confirmed eventually. This is the first report of sparganosis after allogeneic HSCT mimicking myeloid sarcoma, giving a lesson that the physicians have to consider the possibility of sparganosis in this clinical situation and perform adequate diagnostic and therapeutic approaches. PMID:23230335

Yeo, Jin Yeob; Han, Jee Young; Lee, Jung Hwan; Park, Young Hoon; Lim, Joo Han; Lee, Moon Hee; Kim, Chul Soo

2012-01-01

402

Trabectedin: novel insights in the treatment of advanced sarcoma.  

PubMed

Soft tissue sarcomas are a heterogenous group of malignancies with relatively high mortality rates. The outlook for these patients has been poor, with only a few drugs showing measurable activity. Trabectedin is a new alkylating agent with significant activity in sarcomas, but particularly in liposarcomas and leiomyosarcomas, both as a single agent or in combination with other drugs. Phase I and II studies of trabectedin have shown measurable benefit. Currently there are several Phase III trials which have completed accrual to better study its use as a single agent or in combination therapy, although outcomes have not yet been reported. Trabectedin (Yondelis) is approved for the treatment of sarcomas by the EMEA, but is not yet approved by the FDA, pending the results of the currently maturing phase III trials. PMID:24756367

Lopez, Jay Patrick; Gajdos, Csaba; Elias, Anthony

2014-06-01

403

Novel treatment targets in sarcoma: more than just the GIST.  

PubMed

Sarcomas are rare tumors comprising a heterogeneous group of more than 50 histologic subtypes, the majority of which do not respond well to cytotoxic chemotherapy. This has fueled research into the distinct molecular mechanisms of tumorigenesis and disease progression for various sarcoma subtypes. Gastrointestinal stromal tumors and liposarcomas are presented as paradigms of molecular classification that have led to the rational development of novel therapeutic strategies for those tumors. Recent advances in understanding of growth signaling pathways, metabolic reprogramming, and immune therapy have identified new treatment targets for many sarcomas. These investigations will form the foundation for further improvements in our ability to care for patients with these tumors and may offer clinical insights into a wide range of other tumors. PMID:24857144

Shoushtari, Alexander N; Van Tine, Brian A; Schwartz, Gary K

2014-01-01

404

Heart failure resulting from giant left atrial synovial sarcoma metastasis.  

PubMed

Synovial sarcoma metastasis affecting the heart and infiltrating the mitral valve is a very rare pathology. We report the case of a 44-year-old male treated with chemotherapy for atypical synovial sarcoma of the oral mucosa who presented to our clinic after cardiac decompensation with a presumptive diagnosis of myxoma of the left atrium. A large necrotic tumour positive for CK 22, EMA, CD 99 and BCL-2 but negative for translocation in COBRA-FISH analysis by break-apart probe could be excised and revealed a very rare subtype of synovial sarcoma metastasis arising from the endocard of the left atrium. The tumour was resected and the mitral valve reconstructed through ring annuloplasty. PMID:21425056

Winkler, B; Grapow, M; Seeberger, M; Matt, P; Aulitzky, W; Eckstein, F

2012-02-01

405

Bacteriological and epidemiological findings during examination of the uterine content of ewes with retention of fetal membranes.  

PubMed

We included 92 pairs of ewes with or without retention of fetal membranes in a cohort study of 25 flocks in Southern Greece. We obtained two uterine content samples under aseptic conditions, by introducing a swab into the uterus of these ewes, on the 2nd-4th and the 5th-9th day after lambing. We used conventional bacteriological techniques to isolate and identify bacteria and to carry out antimicrobial agents susceptibility testing. The prevalence of bacterial intrauterine contamination among ewes with retention was 24% on the first and 46% on the second sampling (P < 0.0001) and that among ewes without retention was 8 and 2% (P > 0.05), respectively. Clinical signs accompanying the retention of fetal membranes were more frequently observed among ewes with intrauterine contamination than among those without (P = 0.0007). The odds of an ewe having an intrauterine contamination increased multiplicatively by 1.06 when the median duration of retention in the flock increased by 6 h. The principal bacteria isolated from the ewes with retention were Arcanobacterium pyogenes and Escherichia coli; 21% of 73 isolates tested were found resistant to at least one antimicrobial agent. PMID:12041685

Tzora, A; Leontides, L S; Amiridis, G S; Manos, G; Fthenakis, G C

2002-04-15

406

Effects of addition of different vegetable oils to lactating dairy ewes' diet on meat quality characteristics of suckling lambs reared on the ewes' milk.  

PubMed

The effect of different vegetable oils used in the diet of lactating ewes on the meat quality of their suckling lambs has been evaluated. Lambs (males and females) were slaughtered at 11 kg. Fortyeight lactating Churra ewes (prolificacy 1.5) and their suckling lambs were assigned to four treatments according to the oil added (3% on weight basis) to the ewes' daily ration: palm oil as control (CON); olive oil (OLI); soybean oil (SOY); and linseed oil (LIN). Analyses of pH, colour, thiobarbituric acid reactive substances (TBARS), tocopherol levels, volatile compounds and a sensory evaluation were carried out on suckling lambs' meat. Results showed no substantial effect on pH, colour, TBARS and tocopherol levels. Volatiles typically derived from lipid oxidation were higher in SOY group. However, panellists were only able to correctly identify samples from LIN group. Furthermore, the meat from LIN group showed lower scores towards odour and flavour quality and overall liking than that from the rest of treatments. PMID:22381704

Vieira, Ceferina; Fernández-Diez, Ana; Mateo, Javier; Bodas, Raul; Soto, Sergio; Manso, Teresa

2012-07-01

407

Maternal obesity and overnutrition alter fetal growth rate and cotyledonary vascularity and angiogenic factor expression in the ewe  

PubMed Central

In pregnant sheep, maternal:fetal exchange occurs across placentomes composed of placental cotyledonary and uterine caruncular tissues. Recently, we reported that fetal weights of obese (OB) ewes [fed a diet of 150% of National Research Council (NRC) recommendations] were ?30% greater than those of control (C) ewes (fed a diet 100% of NRC recommendations) at midgestation (MG), but fetal weights were similar in late gestation (LG). Transplacental nutrient exchange is dependent on placental blood flow, which itself is dependent on placental vascularity. The current study investigated whether the observed initial faster and subsequent slower fetal growth rate of OB compared with C was associated with changes in cotyledonary vascularity and expression of angiogenic factors (vascular endothelial growth factor, fibroblast growth factor-2, placental growth factor, angiopoietin-1 and -2). Cotyledonary arteriole diameters were markedly greater (P < 0.05) in OB than C ewes at MG, but while arteriole diameter of C ewes increased (P < 0.05) from MG to LG, they remained unchanged in OB ewes. Cotyledonary arterial angiogenic factors mRNA and protein expression were lower (P < 0.05) in OB than C ewes at MG and remained low from MG to LG. In contrast, mRNA levels of angiogenic factors in C ewes declined from high levels at MG to reach those of OB ewes by LG. The increase in cotyledonary arteriole diameter in early to MG may function to accelerate fetal growth rate in OB ewes, while the decreased cotyledonary arterial angiogenic factors from MG-LG may function to protect the fetus from excessive placental vascular development, increased maternal nutrient delivery, and excessive weight gain. PMID:20427725

Ma, Yan; Zhu, Mei J.; Zhang, Liren; Hein, Sarah M.; Nathanielsz, Peter W.

2010-01-01

408

Maternal obesity and overnutrition alter fetal growth rate and cotyledonary vascularity and angiogenic factor expression in the ewe.  

PubMed

In pregnant sheep, maternal:fetal exchange occurs across placentomes composed of placental cotyledonary and uterine caruncular tissues. Recently, we reported that fetal weights of obese (OB) ewes [fed a diet of 150% of National Research Council (NRC) recommendations] were approximately 30% greater than those of control (C) ewes (fed a diet 100% of NRC recommendations) at midgestation (MG), but fetal weights were similar in late gestation (LG). Transplacental nutrient exchange is dependent on placental blood flow, which itself is dependent on placental vascularity. The current study investigated whether the observed initial faster and subsequent slower fetal growth rate of OB compared with C was associated with changes in cotyledonary vascularity and expression of angiogenic factors (vascular endothelial growth factor, fibroblast growth factor-2, placental growth factor, angiopoietin-1 and -2). Cotyledonary arteriole diameters were markedly greater (P < 0.05) in OB than C ewes at MG, but while arteriole diameter of C ewes increased (P < 0.05) from MG to LG, they remained unchanged in OB ewes. Cotyledonary arterial angiogenic factors mRNA and protein expression were lower (P < 0.05) in OB than C ewes at MG and remained low from MG to LG. In contrast, mRNA levels of angiogenic factors in C ewes declined from high levels at MG to reach those of OB ewes by LG. The increase in cotyledonary arteriole diameter in early to MG may function to accelerate fetal growth rate in OB ewes, while the decreased cotyledonary arterial angiogenic factors from MG-LG may function to protect the fetus from excessive placental vascular development, increased maternal nutrient delivery, and excessive weight gain. PMID:20427725

Ma, Yan; Zhu, Mei J; Zhang, Liren; Hein, Sarah M; Nathanielsz, Peter W; Ford, Stephen P

2010-07-01

409

Isolated Gastric Myeloid Sarcoma: A Case Report and Review of the Literature  

PubMed Central

Myeloid sarcoma represents the proliferation of myeloblasts of acute myeloid leukemia (AML) at extramedullary sites. While extramedullary involvement in AML is uncommon in itself, isolated myeloid sarcomas, that is, myeloid sarcomas without any bone marrow involvement, are extremely rare and pose a diagnostic and therapeutic challenge. Here, we present the case of a middle-aged woman with isolated myeloid sarcoma in the stomach—an organ seldom involved by this disease. Additionally, the literature on the epidemiology, diagnosis, pathology, prognosis, and therapeutic options in myeloid sarcomas has been reviewed. PMID:25105036

Bose, Prithviraj

2014-01-01

410

The value of radiation therapy in uterine sarcoma.  

PubMed

Fifty-eight cases of leiomyosarcoma of the uterus and 47 endometrial sarcomas of the uterus are reviewed based on the pertinent information related to recurrence patterns. The following conclusions are made: 1) Cases of endometrial sarcoma should receive preoperative pelvic irradiation, or postoperative pelvic irradiation if a diagnosis is not made until the postoperative period. This plan will probably be of most benefit, if at all, in stage 1 disease. 2) A case for pelvic irradiation as an adjuvant to surgery in leiomyosarcoma cannot be made from a review of this material, either from a viewpoint of recurrence patterns or radio-responsiveness. PMID:1110823

Gilbert, H A; Kagan, A R; Lagasse, L; Jacobs, M R; Tawa, K

1975-01-01

411

Langerhans cell sarcoma: case report and review of world literature.  

PubMed

Langerhans cell sarcoma is a rare malignancy with only 1 pediatric case (less than 15 y of age) reported. Here, we report the second case of Langerhans cell sarcoma in a child who presented with cord compression. This patient was treated with extensive surgical resection, postoperative chemotherapy, and involved-field radiation therapy. She completed therapy and remains in remission for 27 months. A review and analysis of all 53 cases published in the world literature is provided to help guide physicians treating this disease. Recently discovered genetic mutation involving BRAF is also discussed. PMID:24942035

Zwerdling, Ted; Won, Eric; Shane, Lisa; Javahara, Ramin; Jaffe, Ronald

2014-08-01

412

Guidelines for the Management of Soft Tissue Sarcomas  

PubMed Central

These guidelines were drawn up following a consensus meeting of UK sarcoma specialists convened under the auspices of the British Sarcoma Group and are intended to provide a framework for the multidisciplinary care of patients with soft tissue sarcomas. The guidelines published by the European Society of Medical Oncology (ESMO) and the National Comprehensive Cancer Network (NCCN) were used as the basis for discussion and adapted according to UK clinical practice and local requirements. Note was also taken of the National Institute for Health and Clinical Excellence (NICE) improving outcomes guidance (IOG) for people with sarcoma and existing technology appraisals. The guidelines are not intended to challenge NICE guidance but discrepancies may exist where current guidance does not reflect an international standard of care owing to the ever-evolving nature of cancer treatment. It is acknowledged that these guidelines will require updating on a regular basis. An appendix lists the key recommendations which are summarised below. Any patient with a suspected soft tissue sarcoma should be referred to a diagnostic centre and managed by a specialist sarcoma multidisciplinary team. Surgical excision followed by post operative radiotherapy is the standard management of high grade limb sarcomas although occasionally amputation remains the only option. Pre-operative treatment with chemotherapy or radiotherapy should be considered for patients with borderline resectable tumours. Isolated limb perfusion may permit limb salvage in some cases where amputation is the only other option. Adjuvant chemotherapy is not routinely recommended but may be considered in certain specific situations. Regular follow up is recommended to assess local control and the development of metastatic disease. Single agent doxorubicin is the standard first line therapy for metastatic disease. Ifosfamide is an alternative if anthracyclines are contraindicated. Combination therapy may be considered in individual patients. Second line agents include ifosfamide, dacarbazine, trabectedin and the combination of gemcitabine + docetaxel. Surgical resection of local recurrence and pulmonary metastases should be considered in individual patients. There is specific guidance on the management of retroperitoneal and uterine sarcomas. PMID:20634933

Grimer, Robert; Judson, Ian; Peake, David; Seddon, Beatrice

2010-01-01

413

Primary Pulmonary Synovial Sarcoma: A Very Rare Presentation  

PubMed Central

Synovial sarcoma (SS) is a rare tumor originating from mesenchymal tissue and accounting for approximately 5–10% of all soft tissue sarcomas. A rare case of primary pulmonary SS in an asymptomatic 18-year-old man admitted to our hospital for investigation of a 6 × 6.5?cm, oval-shaped, well-delineated pleural based peripheral mass in the left lower lobe in his thorax CT is presented. Left lower lobectomy was done. Immunohistochemically, tumor cells were positive for cytokeratin, epithelial membrane antigen (EMA), and vimentin so that the histopathological diagnosis was compatible with biphasic spindle cell type SS in the lung. PMID:25152824

Seyhan, Ekrem Cengiz; Sokucu, Sinem Nedime; Gunluoglu, Gulsah; Veske, Nurdan Simsek; Altin, Sedat

2014-01-01

414

Prognostic factors and staging for soft tissue sarcomas: an update.  

PubMed

Soft tissue sarcoma (STS) staging is a constantly evolving process. Grading is still of utmost importance and has been adapted into a three-tier system. The STS most difficult to categorize are those with uncertain malignant potential, such as solitary fibrous tumors, gastrointestinal stromal tumors, and glomus tumors, some of which have developed completely separate staging systems and may not even be considered sarcomas. Beyond the current TNM staging system, a multitude of prognostic factors for STS will continue to be discovered and ultimately incorporated into future revisions of the staging system. PMID:22365514

Jones, Natalie B; Iwenofu, Hans; Scharschmidt, Thomas; Kraybill, William

2012-04-01

415

Massive left atrial sarcoma presenting with severe congestive heart failure.  

PubMed

Primary intimal sarcomas of the left atrium are extremely rare and highly aggressive tumours. To our knowledge, only 4 cases have been reported to date. We report on a 42-year-old man who presented with signs of congestive heart failure and was found to have an enormous left atrial bilobed tumour with obstruction of the mitral valve. The patient underwent a surgical excision of the sarcoma. The tumour recurred 8.5 months later and the patient required right pneumonectomy. PMID:25108492

Kuurstra, Emily J; Mullen, John C; MacArthur, Roderick G

2014-10-01

416

SARC: Development and Support of a Sarcoma Research Consortium Infrastructure  

SciTech Connect

SARC is a non-for-profit organization whose mission and vision is to advocate for the collaboration on the design of clinical trials on sarcoma, to further the knowledge regarding the diagnosis and treatment of sarcoma and provide accurate and up to date information to physicians, patients and families. The objectives are to assist in the development of the infrastructure for the continued growth and spectrum of clinical research, to facilitate biannual meeting of investigators, and to develop a preclinical research base that would design and conduct research that would improve the process of drug treatments selected for clinical research trials.

Arkison, Jim

2007-10-29

417

TRIM8 anti-proliferative action against chemo-resistant renal cell carcinoma.  

PubMed

In some tumours, despite a wild-type p53 gene, the p53 pathway is inactivated by alterations in its regulators or by unknown mechanisms, leading to resistance to cytotoxic therapies. Understanding the mechanisms of functional inactivation of wild-type p53 in these tumours may help to define prospective targets for treating cancer by restoring p53 activity. Recently, we identified TRIM8 as a new p53 modulator, which stabilizes p53 impairing its association with MDM2 and inducing the reduction of cell proliferation. In this paper we demonstrated that TRIM8 deficit dramatically impairs p53-mediated cellular responses to chemotherapeutic drugs and that TRIM8 is down regulated in patients affected by clear cell Renal Cell Carcinoma (ccRCC), an aggressive drug-resistant cancer showing wild-type p53. These results suggest that down regulation of TRIM8 might be an alternative way to suppress p53 activity in RCC. Interestingly, we show that TRIM8 expression recovery in RCC cell lines renders these cells sensitive to chemotherapeutic treatments following p53 pathway re-activation. These findings provide the first mechanistic link between TRIM8 and the drug resistance of ccRCC and suggest more generally that TRIM8 could be used as enhancer of the chemotherapy efficacy in cancers where p53 is wild-type and its pathway is defective. PMID:25277184

Caratozzolo, Mariano Francesco; Valletti, Alessio; Gigante, Margherita; Aiello, Italia; Mastropasqua, Francesca; Marzano, Flaviana; Ditonno, Pasquale; Carrieri, Giuseppe; Simonnet, Hélène; D'Erchia, Anna Maria; Ranieri, Elena; Pesole, Graziano; Sbisà, Elisabetta; Tullo, Apollonia

2014-09-15

418

TRIM8 anti-proliferative action against chemo-resistant renal cell carcinoma  

PubMed Central

In some tumours, despite a wild-type p53 gene, the p53 pathway is inactivated by alterations in its regulators or by unknown mechanisms, leading to resistance to cytotoxic therapies. Understanding the mechanisms of functional inactivation of wild-type p53 in these tumours may help to define prospective targets for treating cancer by restoring p53 activity. Recently, we identified TRIM8 as a new p53 modulator, which stabilizes p53 impairing its association with MDM2 and inducing the reduction of cell proliferation. In this paper we demonstrated that TRIM8 deficit dramatically impairs p53-mediated cellular responses to chemotherapeutic drugs and that TRIM8 is down regulated in patients affected by clear cell Renal Cell Carcinoma (ccRCC), an aggressive drug-resistant cancer showing wild-type p53. These results suggest that down regulation of TRIM8 might be an alternative way to suppress p53 activity in RCC. Interestingly, we show that TRIM8 expression recovery in RCC cell lines renders these cells sensitive to chemotherapeutic treatments following p53 pathway re-activation. These findings provide the first mechanistic link between TRIM8 and the drug resistance of ccRCC and suggest more generally that TRIM8 could be used as enhancer of the chemotherapy efficacy in cancers where p53 is wild-type and its pathway is defective. PMID:25277184

Gigante, Margherita; Aiello, Italia; Mastropasqua, Francesca; Marzano, Flaviana; Ditonno, Pasquale; Carrieri, Giuseppe; Simonnet, Helene; D'Erchia, Anna Maria; Ranieri, Elena; Pesole, Graziano; Sbisa, Elisabetta; Tullo, Apollonia

2014-01-01

419

Estimation of dry matter intake by ewes at pasture, using the n-alkanes method  

E-print Network

Estimation of dry matter intake by ewes at pasture, using the n-alkanes method G Béchet, A Kerkeb alkanes as external and internal markers (RW Mayes et al, 1986). Three groups of 3 dry and non.05), and between N and VT, or T and VT groups (Palkane method shows, in spite

Paris-Sud XI, Université de

420

The periparturient relaxation of immunity in Merino ewes infected with Trichostrongylus colubriformis: Parasitological and immunological responses  

Microsoft Academic Search

The temporal association between the periparturient rise (PPR) in worm egg count (WEC) of grazing Merino ewes to infection with Trichostrongylus colubriformis and the underlying causal mechanisms was investigated in an experiment that incorporated two levels of pregnancy (pregnant or unmated), two levels of infection (infected with 6000 T. colubriformis L3\\/week or uninfected) and, following lambing, three levels of lactation

A. M. Beasley; L. P. Kahn; R. G. Windon

2010-01-01