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Sample records for chemo-resistant ewing sarcoma

  1. Ewing sarcoma

    International Nuclear Information System (INIS)

    Ewing sarcoma is the second most frequent primary bone cancer affecting children or young adults. Advances in molecular biology have revealed common chromosomal translocations such as EWS-FLI1 among Ewing sarcoma and related diseases such as primitive neuroectodermal tumor (PNET), so these are considered as Ewing sarcoma family tumor (ESFT). Although fewer than 10% of patients with ESFT survived before establishment of modern multiagent chemotherapy, the multimodal therapeutic regimens including combination chemotherapy, radiotherapy, and surgery can cure 60% of patients with localized disease, due to the collaborative research in European-American or the international trials. The standard chemotherapy for localized ESFT now comprises vincristine, actinomycin D, cyclophosphamide and doxorubicin (VACD) in Europe or vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VDC-IE) in North America. Meanwhile, those with metastatic disease have a much worse outcome with an approximately 10-30% 5-year event-free survival rate. New American-European collaborative trials such as EURO-E.W.I.N.G. 99 are in progress for further improvement of the cure rate in localized and metastatic ESFT. In Japan, Japan Ewing Sarcoma Study Group (JESS) phase II clinical trial for localized ESFT, and some clinical trials including new drugs are ongoing and waiting for results. (author)

  2. Stages of Ewing Sarcoma

    Science.gov (United States)

    ... such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained ... cells. The disease is metastatic Ewing sarcoma, not lung cancer. Recurrent Ewing Sarcoma Recurrent Ewing sarcoma is cancer ...

  3. Treatment Option Overview (Ewing Sarcoma)

    Science.gov (United States)

    ... such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained ... cells. The disease is metastatic Ewing sarcoma, not lung cancer. Recurrent Ewing Sarcoma Recurrent Ewing sarcoma is cancer ...

  4. Imaging characteristics of primary cranial Ewing sarcoma

    International Nuclear Information System (INIS)

    Ewing sarcoma accounts for 10-15% of all childhood malignant bone tumours and is second in prevalence to osteosarcoma. The skull bones are an unusual site of origin of primary Ewing sarcoma in children. Previous reports concentrate on the neurosurgical aspects and relatively good outcome compared to other bone tumours of the calvarium. Reported cases mainly describe the imaging characteristics on CT. To describe the MRI and CT features of primary cranial Ewing sarcoma. The neuroimaging of three cases of primary cranial Ewing sarcoma are reviewed. Our three cases show an extra-axial mass that is high attenuation on CT and low signal on T2-weighted MRI. Haemorrhagic components, dural extension and contrast enhancement are also characteristic features. CT attenuation and magnetic resonance signal characteristics reflect sheets of densely packed cells seen in Ewing sarcoma. (orig.)

  5. Cell Cycle Deregulation in Ewing's Sarcoma Pathogenesis

    Directory of Open Access Journals (Sweden)

    Ashley A. Kowalewski

    2011-01-01

    Full Text Available Ewing's sarcoma is a highly aggressive pediatric tumor of bone that usually contains the characteristic chromosomal translocation t(11;22(q24;q12. This translocation encodes the oncogenic fusion protein EWS/FLI, which acts as an aberrant transcription factor to deregulate target genes necessary for oncogenesis. One key feature of oncogenic transformation is dysregulation of cell cycle control. It is therefore likely that EWS/FLI and other cooperating mutations in Ewing's sarcoma modulate the cell cycle to facilitate tumorigenesis. This paper will summarize current published data associated with deregulation of the cell cycle in Ewing's sarcoma and highlight important questions that remain to be answered.

  6. MicroRNAs in Ewing Sarcoma

    OpenAIRE

    PaulJedlicka; LayneDylla

    2013-01-01

    MicroRNAs have emerged recently as important regulators of gene expression in the cell. Frequently dysregulated in cancer, microRNAs 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 microRNA biology in Ewing Sarcoma. Findings indicate that alterations in microRNA expression in Ewing Sarcoma are widespread, involve both EWS/Ets oncogenic fusion...

  7. Procholecystokinin as marker of human Ewing sarcomas

    DEFF Research Database (Denmark)

    Reubi, Jean Claude; Koefoed, Pernille; Hansen, Thomas von O; Stauffer, Edouard; Rauch, Daniel; Nielsen, Finn C; Rehfeld, Jens F

    2004-01-01

    PURPOSE: Ewing sarcoma is a rapidly growing mesenchymal tumor in young adults. Although it was shown previously to express the cholecystokinin (CCK) gene, it is unknown whether CCK gene expression is detectable at protein level in Ewing sarcoma tumor cell lines, in tumor tissue, and in plasma fro...... metabolism in human cancer; Ewing sarcomas synthesize and secrete proCCK that can be identified in plasma as circulating tumor marker....... found in plasma from patients before treatment and after tumor recurrence, whereas successful chemotherapy reduced proCCK to basal concentrations. Plasma concentrations of proCCK paralleled the respective tumor size. CONCLUSIONS: This is the first study that consistently documents an altered CCK...

  8. Ewing's Sarcoma of the Stomach; Rare Case of Ewing's Sarcoma and Suggestion of New Treatment Strategy

    OpenAIRE

    Kim, Hyo-Sin; Kim, Sungsoo; Min, Young-Don; KEE, KEUN-HONG; HONG, RAN

    2012-01-01

    Ewing's sarcoma is a neoplasm of the undifferenciated small round cells, which generally affects the bone and deep soft tissues of children and adolescents. We present a case of gastric Ewing's sarcoma; a 35-year-old female who had no symptoms. While she was at a routine medical checkup, a protruding mass in her gastric antrum was incidentally found on esophagogastroduodenoscopy. Endoscopic ultrasonogram showed a submucosal mass on the same lesion and a laparosopic wedge resection was done. P...

  9. Ewing sarcoma of the thoracic wall

    International Nuclear Information System (INIS)

    Ewing's sarcomas in the chest wall have in common the facts that the ribs are mainly changed asteolytically; sclerosing is initially seen very seldom, it usually occurs after the therapy; and periosteal detachment with stratification or spicula formation was not observed. All our children had considerably larger intrathoracic tumors than tumors palpable at the chest wall and a notably low number of symptoms in regard of the partially gigantic tumors. (orig.)

  10. Identifying Targetable Liabilities in Ewing Sarcoma

    OpenAIRE

    Vallurupalli, Mounica

    2014-01-01

    Background: Despite multi-modality therapy, the majority of patients with metastatic or recurrent Ewing sarcoma (ES), the second most common pediatric bone malignancy, will die of their disease. ES tumors express aberrantly activated ETS transcription factors through translocations that fuse the EWS gene to ETS family genes FLI1 or ERG. The aberrant activation of ETS transcription factors promotes malignant transformation and proliferation. While, FLI1 or ERG cannot be readily targeted, ther...

  11. Potential molecular targets for Ewing's sarcoma therapy.

    Science.gov (United States)

    Jully, Babu; Rajkumar, Thangarajan

    2012-10-01

    Ewing's sarcoma (ES) is a highly malignant tumor of children and young adults. Modern therapy for Ewing's sarcoma combines high-dose chemotherapy for systemic control of disease, with advanced surgical and/or radiation therapeutic approaches for local control. Despite optimal management, the cure rate for localized disease is only approximately 70%, whereas the cure rate for metastatic disease at presentation is less than 30%. Patients who experience long-term disease-free survival are at risk for significant side-effects of therapy, including infertility, limb dysfunction and an increased risk for second malignancies. The identification of new targets for innovative therapeutic approaches is, therefore, strongly needed for its treatment. Many new pharmaceutical agents have been tested in early phases of clinical trials in ES patients who have recurrent disease. While some agents led to partial response or stable disease, the percentages of drugs eliciting responses or causing an overall effect have been minimal. Furthermore, of the new pharmaceuticals being introduced to clinical practice, the most effective agents also have dose-limiting toxicities. Novel approaches are needed to minimize non-specific toxicity, both for patients with recurrence and at diagnosis. This report presents an overview of the potential molecular targets in ES and highlights the possibility that they may serve as therapeutic targets for the disease. Although additional investigations are required before most of these approaches can be assessed in the clinic, they provide a great deal of hope for patients with Ewing's sarcoma. PMID:23580819

  12. Ewing sarcoma versus osteomyelitis: differential diagnosis with magnetic resonance imaging

    International Nuclear Information System (INIS)

    To find and evaluate characteristic magnetic resonance imaging (MRI) patterns for the differentiation between Ewing sarcoma and osteomyelitis. We identified 28 consecutive patients referred to our department for MRI (1.5 T) of an unclear bone lesion with clinical symptoms suggestive of Ewing sarcoma or osteomyelitis. MRI scans were re-evaluated by two experienced radiologists, typical MR imaging features were documented and a diagnostic decision between Ewing sarcoma and osteomyelitis was made. Statistical significance of the association between MRI features and the biopsy-based diagnosis was assessed using Fisher's exact test. The most clear-cut pattern for determining the correct diagnosis was the presence of a sharp and defined margin of the bone lesion, which was found in all patients with Ewing sarcoma, but in none of the patients with osteomyelitis (P < 0.0001). Contrast enhancing soft tissue was present in all cases with Ewing sarcoma and absent in 4 patients with osteomyelitis (P = 0.0103). Cortical destruction was found in all patients with Ewing sarcoma, 4 patients with osteomyelitis did not present any cortical reaction (P = 0.0103). Cystic or necrotic areas were identified in 13 patients with Ewing sarcoma and in 1 patient with osteomyelitis (P = 0.004). Interobserver reliability was very good (kappa = 1) in Ewing sarcoma and moderate (kappa = 0.6) in patients with osteomyelitis. A sharp and defined margin, optimally visualized on T1-weighted images in comparison to short tau inversion recovery (STIR) images, is the most significant feature of Ewing sarcoma in differentiating from osteomyelitis. (orig.)

  13. Ewing sarcoma versus osteomyelitis: differential diagnosis with magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Henninger, B.; Glodny, B.; Rudisch, A.; Trieb, T.; Loizides, A.; Judmaier, W.; Schocke, M.F. [Innsbruck Medical University, Department of Radiology, Innsbruck (Austria); Putzer, D. [Innsbruck Medical University, Department of Nuclear Medicine, Innsbruck (Austria)

    2013-08-15

    To find and evaluate characteristic magnetic resonance imaging (MRI) patterns for the differentiation between Ewing sarcoma and osteomyelitis. We identified 28 consecutive patients referred to our department for MRI (1.5 T) of an unclear bone lesion with clinical symptoms suggestive of Ewing sarcoma or osteomyelitis. MRI scans were re-evaluated by two experienced radiologists, typical MR imaging features were documented and a diagnostic decision between Ewing sarcoma and osteomyelitis was made. Statistical significance of the association between MRI features and the biopsy-based diagnosis was assessed using Fisher's exact test. The most clear-cut pattern for determining the correct diagnosis was the presence of a sharp and defined margin of the bone lesion, which was found in all patients with Ewing sarcoma, but in none of the patients with osteomyelitis (P < 0.0001). Contrast enhancing soft tissue was present in all cases with Ewing sarcoma and absent in 4 patients with osteomyelitis (P = 0.0103). Cortical destruction was found in all patients with Ewing sarcoma, 4 patients with osteomyelitis did not present any cortical reaction (P = 0.0103). Cystic or necrotic areas were identified in 13 patients with Ewing sarcoma and in 1 patient with osteomyelitis (P = 0.004). Interobserver reliability was very good (kappa = 1) in Ewing sarcoma and moderate (kappa = 0.6) in patients with osteomyelitis. A sharp and defined margin, optimally visualized on T1-weighted images in comparison to short tau inversion recovery (STIR) images, is the most significant feature of Ewing sarcoma in differentiating from osteomyelitis. (orig.)

  14. Primary Ewing's sarcoma of the vertebral column

    International Nuclear Information System (INIS)

    To determine the demographics, imaging findings, clinical symptoms, and prognosis of primary vertebral Ewing's sarcoma (PVES). A retrospective review of medical records and radiological studies of patients diagnosed with PVES from 1936 through 2001 in our institution and Department of Pathology consultation files was undertaken. Metastatic and soft tissue Ewing's sarcoma cases were excluded. From a total of 1,277 cases of Ewing's sarcoma, 125 (9.8%) had a primary vertebral origin. There were 48 females and 76 males. Patient ages ranged from 4 to 54 (mean 19.3, standard deviation 10.7, median 16) years. Vertebral column distribution was four cervical (3.2%), 13 thoracic (10.5%), 31 lumbar (25%), and 67 sacrum (53.2%). More than one vertebral segment was involved in ten cases (8%). Satisfactory imaging studies were available in 51 patients: 49 radiographs, 27 computerized tomography (CT), and 23 magnetic resonance imaging (MRI) studies. The majority of tumors were lytic (93%). Three cases were mixed lytic and sclerotic (6%) and one sclerotic. In the nonsacral spine, the majority of lesions (12/20) involved the posterior elements with extension into the vertebral body. Five cases were centered in the vertebral body with extension into the posterior elements. Two cases were limited to the posterior elements, and one case solely involved the vertebral body. Ala was the most frequently affected site in the sacrum (18/26). Spinal canal invasion was frequent (91%). Detailed clinical information was available in 53 patients. Duration of symptoms ranged from 1 to 30 (mean 7) months. Local pain was the first symptom and seen in all cases. Neurological deficits were present in 21 (40%) cases. All patients received radiation in various dosages; 70% additionally received chemotherapy. Twenty-five patients had surgery, and two patients received bone marrow transplantation. Forty-five patients had follow-up; the five-year disease-free survival probability is 0.53. Disease

  15. Ewing Sarcoma of the External Ear Canal.

    Science.gov (United States)

    Binnetoglu, Adem; Baglam, Tekin; Tokuc, Gulnur; Kecelioglu Binnetoglu, Kiymet; Gerin, Fatma; Sari, Murat

    2016-01-01

    Background. Ewing sarcoma (ES) is a high-grade malignant tumor that has skeletal and extraskeletal forms and consists of small round cells. In the head and neck region, reported localization of extraskeletal ES includes the larynx, thyroid gland, submandibular gland, nasal fossa, pharynx, skin, and parotid gland, but not the external ear canal. Methods. We present the unique case of a 2-year-old boy with extraskeletal ES arising from the external ear canal, mimicking auricular hematoma. Results. Surgery was performed and a VAC/IE (vincristine, adriamycin, cyclophosphamide alternating with ifosfamide, and etoposide) regimen was used for adjuvant chemotherapy for 12 months. Conclusion. The clinician should consider extraskeletal ES when diagnosing tumors localized in the head and neck region because it may be manifested by a nonspecific clinical picture mimicking common otorhinolaryngologic disorders. PMID:27313930

  16. Transarticular spread of Ewing sarcoma mimicking septic arthritis

    Energy Technology Data Exchange (ETDEWEB)

    Jordanov, Martin I.; Block, John J. [Vanderbilt University Medical Center, Department of Radiology and Radiological Sciences, Nashville, TN (United States); Gonzalez, Adriana L. [Vanderbilt University Medical Center, Department of Pathology, Nashville, TN (United States); Green, Neil E. [Vanderbilt Children' s Hospital, Department of Pediatric Orthopaedics, Nashville, TN (United States)

    2009-04-15

    Transarticular spread of tumor is rare; it has only been reported in the sacroiliac joint, intervertebral disk spaces, and facet joints. The anatomic and kinetic characteristics of the sacroiliac joint, as well as the changes the joint undergoes during a lifetime, make it particularly vulnerable to transarticular tumor invasion. Although extremely rare, Ewing sarcoma can extend through the sacroiliac joint and be virtually indistinguishable radiologically from septic arthritis. Furthermore, the clinical presentation of a child with Ewing sarcoma can be similar to that of a child with osteomyelitis. Laboratory values are quite nonspecific and are not always helpful in differentiating between the entities. Therefore, the possibility of sacroiliac joint transarticular Ewing sarcoma should be considered in a child presenting with hip pain, despite clinical, radiological and laboratory findings suggesting an infectious process. (orig.)

  17. Transarticular spread of Ewing sarcoma mimicking septic arthritis

    International Nuclear Information System (INIS)

    Transarticular spread of tumor is rare; it has only been reported in the sacroiliac joint, intervertebral disk spaces, and facet joints. The anatomic and kinetic characteristics of the sacroiliac joint, as well as the changes the joint undergoes during a lifetime, make it particularly vulnerable to transarticular tumor invasion. Although extremely rare, Ewing sarcoma can extend through the sacroiliac joint and be virtually indistinguishable radiologically from septic arthritis. Furthermore, the clinical presentation of a child with Ewing sarcoma can be similar to that of a child with osteomyelitis. Laboratory values are quite nonspecific and are not always helpful in differentiating between the entities. Therefore, the possibility of sacroiliac joint transarticular Ewing sarcoma should be considered in a child presenting with hip pain, despite clinical, radiological and laboratory findings suggesting an infectious process. (orig.)

  18. Adult Intramedullary Ewing Sarcoma of the Proximal Hip

    Directory of Open Access Journals (Sweden)

    Preetam Gongidi

    2014-01-01

    Full Text Available Ewing sarcoma of bone is classically a permeative lesion in the diaphysis of long bones in children. While they occur primarily in children and adolescents, they can be seen in young adults in their 20s, but these are typically seen in flat bones. The permeative nature of the lesion can elicit new bone formation creating a partially sclerotic appearance, cortical expansion presenting as a “Codman triangle,” or have an “onion-skin” type of aggressive periosteal reaction/periostitis. Ewing sarcoma is rarely seen without an associated soft-tissue mass and is even rarer to just have benign-appearing periostitis (e.g., thick, uniform, or wavy cortex. We present such a case of Ewing sarcoma in a young adult confined to just the medullary metadiaphysis without cortical erosion or soft-tissue mass. To the best of our knowledge, this is the first case to be reported in the radiology literature.

  19. Adult intramedullary ewing sarcoma of the proximal hip.

    Science.gov (United States)

    Gongidi, Preetam; Jasti, Siva; Rafferty, William; Barshay, Veniamin; Lackman, Richard

    2014-01-01

    Ewing sarcoma of bone is classically a permeative lesion in the diaphysis of long bones in children. While they occur primarily in children and adolescents, they can be seen in young adults in their 20s, but these are typically seen in flat bones. The permeative nature of the lesion can elicit new bone formation creating a partially sclerotic appearance, cortical expansion presenting as a "Codman triangle," or have an "onion-skin" type of aggressive periosteal reaction/periostitis. Ewing sarcoma is rarely seen without an associated soft-tissue mass and is even rarer to just have benign-appearing periostitis (e.g., thick, uniform, or wavy cortex). We present such a case of Ewing sarcoma in a young adult confined to just the medullary metadiaphysis without cortical erosion or soft-tissue mass. To the best of our knowledge, this is the first case to be reported in the radiology literature. PMID:25024862

  20. Copy Number Alterations and Methylation in Ewing's Sarcoma

    Directory of Open Access Journals (Sweden)

    Mona S. Jahromi

    2011-01-01

    Full Text Available Ewing's sarcoma is the second most common bone malignancy affecting children and young adults. The prognosis is especially poor in metastatic or relapsed disease. The cell of origin remains elusive, but the EWS-FLI1 fusion oncoprotein is present in the majority of cases. The understanding of the molecular basis of Ewing's sarcoma continues to progress slowly. EWS-FLI1 affects gene expression, but other factors must also be at work such as mutations, gene copy number alterations, and promoter methylation. This paper explores in depth two molecular aspects of Ewing's sarcoma: copy number alterations (CNAs and methylation. While CNAs consistently have been reported in Ewing's sarcoma, their clinical significance has been variable, most likely due to small sample size and tumor heterogeneity. Methylation is thought to be important in oncogenesis and balanced karyotype cancers such as Ewing's, yet it has received only minimal attention in prior studies. Future CNA and methylation studies will help to understand the molecular basis of this disease.

  1. Cytotoxicity of the saponin TTB2 on Ewing sarcoma cells

    OpenAIRE

    Huang, Wenfeng; Zou, Kun

    2015-01-01

    The steroidal saponin TTB2 can be isolated from the n-BuOH extracts of Trillium tschonoskii Maxim. The aim of the present study was to observe whether this saponin exerted any cytotoxic effects on malignant sarcoma cells, and to further investigate the possible underlying molecular mechanisms. The cell viability, cell cycle arrest and phosphorylation of certain important signal molecules in the sarcoma cell line were investigated. It was found that TTB2 inhibited the growth of the Ewing sarco...

  2. Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumor of breast

    Science.gov (United States)

    Srivastava, Smita; Arora, Jyoti; Parakh, Anushri; Goel, Ruchika Kumar

    2016-01-01

    Extraskeletal Ewing's sarcoma (EES) is a rare soft tissue tumor that is morphologically indistinguishable from skeletal ES. We report a case of a 25-year-old female with recurrent EES/primitive neuroectodermal tumor of right breast with imaging findings on mammogram, ultrasound, magnetic resonance imaging breast, and positron emission tomography–computed tomography.

  3. Microsatellites with Macro-Influence in Ewing Sarcoma

    Directory of Open Access Journals (Sweden)

    Stephen L. Lessnick

    2012-07-01

    Full Text Available Numerous molecular abnormalities contribute to the genetic derangements involved in tumorigenesis. Chromosomal translocations are a frequent source of these derangements, producing unique fusion proteins with novel oncogenic properties. EWS/ETS fusions in Ewing sarcoma are a prime example of this, resulting in potent chimeric oncoproteins with novel biological properties and a unique transcriptional signature essential for oncogenesis. Recent evidence demonstrates that EWS/FLI, the most common EWS/ETS fusion in Ewing sarcoma, upregulates gene expression using a GGAA microsatellite response element dispersed throughout the human genome. These GGAA microsatellites function as enhancer elements, are sites of epigenetic regulation and are necessary for EWS/FLI DNA binding and upregulation of principal oncogenic targets. An increasing number of GGAA motifs appear to substantially enhance EWS/FLI-mediated gene expression, which has compelling biological implications as these GGAA microsatellites are highly polymorphic within and between ethnically distinct populations. Historically regarded as junk DNA, this emerging evidence clearly demonstrates that microsatellite DNA plays an instrumental role in EWS/FLI-mediated transcriptional regulation and oncogenesis in Ewing sarcoma. This unprecedented role of GGAA microsatellite DNA in Ewing sarcoma provides a unique opportunity to expand our mechanistic understanding of how EWS/ETS fusions influence cancer susceptibility, prognosis and transcriptional regulation.

  4. 18q-syndrome and extraskeletal Ewing's sarcoma.

    OpenAIRE

    Machin Valtueña, M; Garcia-Sagredo, J M; Muñoz Villa, A; Lozano Giménez, C; Aparicio Meix, J M

    1987-01-01

    Cytogenetic studies carried out in a boy with multiple congenital anomalies showed a partial deletion of the long arm of chromosome 18. The child later developed an extraskeletal Ewing's sarcoma. The possible association of the tumour with 18q- syndrome is discussed.

  5. Novel Combination Chemotherapy for Localized Ewing Sarcoma

    Science.gov (United States)

    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 in patients with extracranial Ewing

  6. 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

    Science.gov (United States)

    2016-03-15

    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

  7. FOXM1 is an oncogenic mediator in Ewing Sarcoma.

    Directory of Open Access Journals (Sweden)

    Laura Christensen

    Full Text Available Ewing Family Tumors (Ewing Sarcoma and peripheral Primitive Neuroectodermal Tumor are common bone and soft tissue malignancies of childhood, adolescence and young adulthood. Chromosomal translocation in these tumors produces fusion oncogenes of the EWS/ETS class, with EWS/FLI1 being by far the most common. EWS/ETS chimera are the only well established driver mutations in these tumors and they function as aberrant transcription factors. Understanding the downstream genes whose expression is modified has been a central approach to the study of these tumors. FOXM1 is a proliferation associated transcription factor which has increasingly been found to play a role in the pathogenesis of a wide range of human cancers. Here we demonstrate that FOXM1 is expressed in Ewing primary tumors and cell lines. Reduction in FOXM1 expression in Ewing cell lines results in diminished potential for anchorage independent growth. FOXM1 expression is enhanced by EWS/FLI1, though, unlike other tumor systems, it is not driven by expression of the EWS/FLI1 target GLI1. Thiostrepton is a compound known to inhibit FOXM1 by direct binding. We show that Thiostrepton diminishes FOXM1 expression in Ewing cell lines and this reduction reduces cell viability through an apoptotic mechanism. FOXM1 is involved in Ewing tumor pathogenesis and may prove to be a useful therapeutic target in Ewing tumors.

  8. Adult Intramedullary Ewing Sarcoma of the Proximal Hip

    OpenAIRE

    Preetam Gongidi; Siva Jasti; William Rafferty; Veniamin Barshay; Richard Lackman

    2014-01-01

    Ewing sarcoma of bone is classically a permeative lesion in the diaphysis of long bones in children. While they occur primarily in children and adolescents, they can be seen in young adults in their 20s, but these are typically seen in flat bones. The permeative nature of the lesion can elicit new bone formation creating a partially sclerotic appearance, cortical expansion presenting as a “Codman triangle,” or have an “onion-skin” type of aggressive periosteal reaction/periostitis. Ewing sarc...

  9. Recent advances in targeted therapy for Ewing sarcoma [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Kathleen I. Pishas

    2016-08-01

    Full Text Available Ewing sarcoma is an aggressive, poorly differentiated neoplasm of solid bone that disproportionally afflicts the young. Despite intensive multi-modal therapy and valiant efforts, 70% of patients with relapsed and metastatic Ewing sarcoma will succumb to their disease. The persistent failure to improve overall survival for this subset of patients highlights the urgent need for rapid translation of novel therapeutic strategies. As Ewing sarcoma is associated with a paucity of mutations in readily targetable signal transduction pathways, targeting the key genetic aberration and master regulator of Ewing sarcoma, the EWS/ETS fusion, remains an important goal.

  10. BONE TUMOR ENVIRONMENT AS POTENTIAL THERAPEUTIC TARGET IN EWING SARCOMA

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    Françoise eREDINI

    2015-12-01

    Full Text Available Ewing sarcoma is the second most common pediatric bone tumor, with three cases per million worldwide. In clinical terms, ES is an aggressive, rapidly fatal malignancy that mainly develops in osseous sites (85%, but also in extraskeletal soft tissue. It spreads naturally to the lungs, bones and bone marrow with poor prognosis in the two latter cases. Bone lesions from primary or secondary (metastases tumors are characterized by extensive bone remodeling, more often due to osteolysis. Osteoclast activation and subsequent bone resorption is responsible for the clinical features of bone tumors including pain, vertebral collapse and spinal cord compression. Based on the vicious cycle concept of tumor cells and bone resorbing cells, drugs which target osteoclasts may be promising agents as adjuvant setting for treating bone tumors, including Ewing sarcoma. There is also increasing evidence that cellular and molecular protagonists present in the bone microenvironment play a part in establishing a favorable niche for tumor initiation and progression. The purpose of this review is to discuss the potential therapeutic value of drugs targeting the bone tumor microenvironment in Ewing Sarcoma. The first part of the review will focus on targeting the bone resorbing function of osteoclasts by means of bisphosphonates (BPs or drugs blocking the pro-resorbing cytokine Receptor Activator of NF-kappa B Ligand (RANKL. Second, the role of this peculiar hypoxic microenvironment will be discussed in the context of resistance to chemotherapy, escape from the immune system, or neo-angiogenesis. Therapeutic interventions based on these specificities could be then proposed in the context of Ewing sarcoma.

  11. Establishment of a Ewing's sarcoma mouse model: JAK/STAT signalling in Ewing's sarcoma

    International Nuclear Information System (INIS)

    The Ewing's sarcoma family of tumours (ESFT) comprises paediatric cancers of bone and soft tissue which presumably originate from mesenchymal progenitor cells(MPC). One hallmark of ESFT is the presence of a chromosomal translocation. In 90% of the cases chromosome 11 fuses with chromosome 22. This translocation generates the EWS/FLI-1 fusion which acts as an aberrant transcription factor deregulating many genes involved in tumour development. Surgery and/or radiotherapy combined with chemotherapy are the usual forms of treatment for ESFT. But since there is only little progress in the field of chemotherapy the need for an animal model for pre-clinical drug testing is evident. Thus, the main focus of this thesis was to establish a mouse model that develops sarcomas resembling the phenotype of ESFT. We used a conditional EWS/FLI-1 mouse model, which upon Cre activity (controlled by a tissue specific promotor) expressed EWS/FLI-1 in the targeted cells. Since ESFT arises in bone and surrounding soft tissue we decided to direct expression of EWS/FLI-1 to the mesenchymal lineage by using different Cre lines. Only when using the Prx1Cre, double transgenic mice tolerated EWS/FLI-1 expression. We observed developmental abnormalities with severe skeletal deformations. Bone formation was impaired due to the absence of mature chondrocytes and osteoblasts and hence a lack of calcified bone. The lack of mature bone cells in EWS/FLI-1 expressing Prx1Cre mice supports in vitro data showing that EWS/FLI-1 impedes differentiation of murine mesenchymal progenitor cells. Currently, the project is extended to analysis of an inducible Prx1Cre system which circumvents the early lethality of Prx1Cre EF mice. This should provide the basis for tumour formation in these mice and hence the development of an appropriate mouse model for pre-clinical research. In the second project of my PhD thesis, the role of the Janus Kinase/Signal Transducer and Activator of Transcription (JAK

  12. Variable expression of PIK3R3 and PTEN in Ewing Sarcoma impacts oncogenic phenotypes.

    Directory of Open Access Journals (Sweden)

    Brian F Niemeyer

    Full Text Available Ewing Sarcoma is an aggressive malignancy of bone and soft tissue affecting children and young adults. Ewing Sarcoma is driven by EWS/Ets fusion oncoproteins, which cause widespread alterations in gene expression in the cell. Dysregulation of receptor tyrosine kinase signaling, particularly involving IGF-1R, also plays an important role in Ewing Sarcoma pathogenesis. However, the basis of this dysregulation, including the relative contribution of EWS/Ets-dependent and independent mechanisms, is not well understood. In the present study, we identify variable expression of two modifiers of PI3K signaling activity, PIK3R3 and PTEN, in Ewing Sarcoma, and examine the consequences of this on PI3K pathway regulation and oncogenic phenotypes. Our findings indicate that PIK3R3 plays a growth-promotional role in Ewing Sarcoma, but suggest that this role is not strictly dependent on regulation of PI3K pathway activity. We further show that expression of PTEN, a well-established, potent tumor suppressor, is lost in a subset of Ewing Sarcomas, and that this loss strongly correlates with high baseline PI3K pathway activity in cell lines. In support of functional importance of PTEN loss in Ewing Sarcoma, we show that re-introduction of PTEN into two different PTEN-negative Ewing Sarcoma cell lines results in downregulation of PI3K pathway activity, and sensitization to the IGF-1R small molecule inhibitor OSI-906. Our findings also suggest that PTEN levels may contribute to sensitivity of Ewing Sarcoma cells to the microtubule inhibitor vincristine, a relevant chemotherapeutic agent in this cancer. Our studies thus identify PIK3R3 and PTEN as modifiers of oncogenic phenotypes in Ewing Sarcoma, with potential clinical implications.

  13. Whole-Body Radiation Therapy, Systemic Chemotherapy, and High-Dose Chemotherapy Followed By Stem Cell Rescue in Treating Patients With Poor-Risk Ewing Sarcoma

    Science.gov (United States)

    2015-01-07

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

  14. Skull-base Ewing sarcoma with multifocal extracranial metastases

    Directory of Open Access Journals (Sweden)

    Sumit Thakar

    2012-01-01

    Full Text Available Intracranial occurrence of Ewing sarcoma (ES is unusual, with a skull-base location being anecdotal. We report a 29-year-old man who presented with rapidly progressive ophthalmoplegia, and was found to be harboring an infiltrative lesion involving the sphenoid sinus, sella, and clivus. He underwent trans-sphenoidal decompression of the lesion which was histologically suggestive of ES. He developed paraparesis 2 weeks after commencing adjuvant therapy. Imaging revealed two thoracic extradural lesions and florid vertebral and pulmonary metastases. This is the first report in indexed literature of a primary intracranial ES on the skull-base with disseminated extracranial disease.

  15. Magnetic resonance imaging of Ewing sarcoma: morphology and tumour delineation

    International Nuclear Information System (INIS)

    Magnetic resonance images (spin-echo techniques; T1-w pre/post gadolinium, T2-w) of 59 patients as part of a multicenter study were evaluated retrospectively. Qualitative image analysis was performed: signal intensity (point of reference extraosseous: muscle, intraosseous: bone marrow), enhancement patterns, lesion delineation and differentiation between tumor and oedema. Morphology of Ewing sarcoma in magnetic resonance imaging is rather uniform. The lesion is intra- and extraosseous sharply delineated, though tumour and oedema can be rarely differentiated. (orig./MG)

  16. Combined chemotherapy and radiotherapy of localized and metastasizing Ewing's sarcoma

    International Nuclear Information System (INIS)

    Between 1973 and 1978 combined radiotherapy and chemotherapy were given to 22 patients with histologically proven Ewing's sarcoma. The combined chemotherapy consisted of cyclophosphamide, vincristin, adriamycin, as well as dacarbazine in some cases. The neoplasm was a localized one at the beginning of treatment in 14 of the 22. These patients received high-voltage rediotherapy to the primary focus at a focal dose between 42 and 55 Gray (4200-5500 rad), followed by chemotherapy. After 6-8 treatment cycles, adriamycin was replaced by methotrexate. Nine of the 14 patients survived without recurrence for 12 to over 59 months. Eight patients had extensive metastases at the beginning of treatment: they at first received only chemotherapy, followed by radiotherapy or operation, as indicated. Full clinical remission was achieved in five of them: in three this remission has now lasted for more than 18, 40 and 44 months, respectively. These results indicate that (1) additional chemotherapy improves the prognosis of localized Ewing's sarcoma, and (2) even in farprogressed cases the combination of chemotherapy and radiotherapy can achieve lasting remission, which may in fact be a true cure. (orig.)

  17. Radiographic features of Ewing's sarcoma of the bones of the hands and feet

    International Nuclear Information System (INIS)

    The radiographic features of Ewing's sarcoma of the bones of the hands and feet are reviewed utilizing cases obtained from the Mayo Clinic patient files and the consultation files of Drs. D.C. Dahlin and K.K. Unni. This series consists of a total of 43 cases of pathologically proven Ewing's sarcoma involving the small bones of the hands and feet. The classic radiographic features of Ewing's sarcoma in the long bones, including lytic, permeative destruction, aggressive periosteal reaction, cortical violation, and a soft tissue mass, are also seen in the bones of the hands and feet, with similar frequency. These classic features are most commonly present in lesions affecting the short tubular bones. Lesions affecting the tarsal bones more often demonstrate atypical radiographic features. These atypical radiographic appearances may play a role in the reported delay in diagnosis of Ewing's sarcoma within the tarsal bones. (orig.)

  18. Actuarial risk of isolated CNS involvement in Ewing's sarcoma following prophylactic cranial irradiation and intrathecal methotrexate

    International Nuclear Information System (INIS)

    Records of 154 patients with Ewing's sarcoma treated at the National Cancer Institute were reviewed to assess the incidence and risk of developing isolated central nervous system (CNS) Ewing's sarcoma. Sixty-two of the 154 patients had received CNS irradiation and intrathecal (i.t.) methotrexate as part of their initial therapy to prevent the occurrence of isolated CNS Ewing's sarcoma. The risk of developing isolate CNS Ewing's sarcoma was greatest within the first two years after diagnosis and was approximately 10%. The overall risk of CNS recurrence in the group of patients receiving DNS treatment was similar to the group receiving no therapy directed to the CNS. The occurrence of isolated CNS involvement was not prevented by the use of CNS irradiation and i.t. methotrexate. Because of a lack of efficacy to the CNS irradiation regimen, current treatment regimens do not include therapy directed to CNS

  19. Primary Ewing sarcoma of the coronoid process of mandible.

    Science.gov (United States)

    Sabir, Husain; Kumbhare, S; Pande, S; Sachdeva, S; Gajbhiye, N

    2016-01-01

    Ewing sarcoma (ES) is a rare, primary malignancy of the bone that occurs mainly in childhood and early adolescence. ES usually occurs in long bones of the axial skeleton. Although uncommon in the jaws, ES at this site is most likely to occur in the posterior mandible. The outcome for patients with localised disease has improved over the decades, due to better combination chemotherapies and better methods of local control. We present the clinicopathologic features and management of a case of ES that developed in the left coronoid process of the mandible of a 31-year-old male. Chemotherapy and, later, a segmental mandibulectomy were used to achieve local control. A fibula-free flap repair was performed with good aesthetic results. This case elucidates the importance of the interdisciplinary approach required for the evaluation and treatment of this aggressive neoplasm. PMID:27514181

  20. Primitive Neuroectodermal Tumor/Ewing Sarcoma Presenting with Pulmonary Nodular Lesions

    Directory of Open Access Journals (Sweden)

    Selvi Asker

    2015-01-01

    Full Text Available Primitive neuroectodermal tumors (PNETs and Ewing sarcoma (EWS belong to the same family of malignant, small, round cell neoplasms of soft tissue or bone origin. EWS-PNETs that arise in the lung parenchyma involvement are extremely rare in adults. A case of a 32-year-old male presenting with chest pain and diffuse pulmonary nodules on chest X-ray and diagnosed with Ewing sarcoma-PNETs will be presented here.

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

    OpenAIRE

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

    2013-01-01

    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 recep...

  2. The Neurological Compromised Spine Due to Ewing Sarcoma. What First: Surgery or Chemotherapy? Therapy, Survival, and Neurological Outcome of 15 Cases With Primary Ewing Sarcoma of the Vertebral Column

    NARCIS (Netherlands)

    Mirzaei, L.; Kaal, S.E.J.; Schreuder, H.W.B.; Bartels, R.H.M.A.

    2015-01-01

    BACKGROUND: The vertebral column is an infrequent site of primary involvement in Ewing sarcoma. Yet when Ewing sarcoma is found in the spine, the urge for decompression is high because of the often symptomatic compression of neural structures. It is unclear in alleviating a neurological deficit whet

  3. Intracranial Dural Metastasis of Ewing's Sarcoma: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Eung Yeop; Lee, Seung Koo; Kim, Dong Joon; Kim, Jin Na; Lee, Kyu Sung; Jung, Woo Hee; Kim, Dong Ik [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2008-02-15

    Ewing's sarcoma is a malignant bone tumor that can occur anywhere in the body, but it is most commonly observed in the long bones of the arms and legs, the pelvis and in the chest. The predominant sites of metastasis include the lung (38%), bone (including the spine; 31%), and the bone marrow (11%). Metastasis of Ewing's sarcoma to the central nervous system (CNS) is relatively rare, and most of the previous reports have demonstrated involvement of the bony calvarium or brain parenchyma. We describe here the imaging findings of dural metastasis of Ewing's sarcoma, and these imaging findings have not been previously reported on in the medical literature. In conclusion, dural metastasis of Ewing's sarcoma is very rare and its imaging characteristics are similar to those of a primary tumor, which mimic the findings of a schwannoma or meningioma. Despite its rarity, secondary Ewing's sarcoma may be included in the differential diagnosis of extra-axial dural masses.

  4. Ewing sarcoma in adults treated with modern radiotherapy techniques

    International Nuclear Information System (INIS)

    Background and purpose: To evaluate local control and survival outcomes in adults with Ewing sarcoma (ES) treated with radiotherapy (RT). Material and methods: Retrospective review of all 109 patients age ⩾18 treated for ES with RT to the primary site at Memorial Sloan Kettering Cancer Center between 1990 and 2011. RT was used as the definitive local control modality in 44% of patients, preoperatively for 6%, and postoperatively for 50%. Results: Median age at diagnosis was 27 years (range, 18–67). The 5-year local failure (LF) was 18%. Differences in LF were not identified when evaluated by modality of local control (RT versus combined surgery and RT), RT dose, fractionation, and RT technique. However, margin status at time of resection significantly predicted LF. The 5-year event-free survival and overall survival rates were 44% and 66% for patients with localized disease, compared with 16% and 26% for metastatic disease (p = 0.0005 and 0.0002). Tumor size, histopathologic response to chemotherapy, and treatment on or according to a protocol were also significantly associated with survival. Conclusions: This series of adults treated with modern chemotherapy and RT had prognostic factors and outcomes similar to adolescents with ES. All adults with ES should be treated with an aggressive, multidisciplinary approach

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

    Science.gov (United States)

    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

    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-28ζ 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

  6. Expression of CCL21 in Ewing sarcoma shows an inverse correlation with metastases and is a candidate target for immunotherapy.

    Science.gov (United States)

    Sand, Laurens G L; Berghuis, Dagmar; Szuhai, Karoly; Hogendoorn, Pancras C W

    2016-08-01

    Ewing sarcoma is an aggressive neoplasm predominantly occurring in adolescents and has a poor prognosis when metastasized. For patients with metastatic disease in particular, immunotherapy has been proposed as possible beneficial additive therapy. CCL21 activation-based immunotherapy was successful in preclinical studies in other tumor types; therefore, we investigated CCL21 expression in Ewing sarcoma as potential target for immunotherapy. The CCL21 RNA expression was determined in 21 Ewing sarcoma cell lines and 18 primary therapy-naive Ewing sarcoma samples. In the tumor samples, this was correlated with the number and CD4(+)/CD8(+) ratio of infiltrating T cells and clinical parameters. Higher RNA expression levels of CCL21 significantly correlated with a lower CD4(+)/CD8(+) T cell ratio (P = 0.009), good chemotherapeutic response (P = 0.01) and improved outcome (P factor. Protein expression analysis of CCL21 and its receptor CCR7 in 24 therapy-naïve tumors showed that there was no expression in all bar one Ewing sarcoma cells. In conclusion, CCL21 is expressed in clinical Ewing sarcoma samples by nontumor-infiltrating immune cells. The observed positive correlation with survival implies that CCL21 might be a potential prognostic marker for Ewing sarcoma and marks the potential of CCL21 immunotherapy for use in Ewing sarcoma. PMID:27369431

  7. Melatonin Cytotoxicity Is Associated to Warburg Effect Inhibition in Ewing Sarcoma Cells

    Science.gov (United States)

    Sanchez-Sanchez, Ana M.; Antolin, Isaac; Puente-Moncada, Noelia; Suarez, Santos; Gomez-Lobo, Marina; Rodriguez, Carmen; Martin, Vanesa

    2015-01-01

    Melatonin kills or inhibits the proliferation of different cancer cell types, and this is associated with an increase or a decrease in reactive oxygen species, respectively. Intracellular oxidants originate mainly from oxidative metabolism, and cancer cells frequently show alterations in this metabolic pathway, such as the Warburg effect (aerobic glycolysis). Thus, we hypothesized that melatonin could also regulate differentially oxidative metabolism in cells where it is cytotoxic (Ewing sarcoma cells) and in cells where it inhibits proliferation (chondrosarcoma cells). Ewing sarcoma cells but not chondrosarcoma cells showed a metabolic profile consistent with aerobic glycolysis, i.e. increased glucose uptake, LDH activity, lactate production and HIF-1α activation. Melatonin reversed Ewing sarcoma metabolic profile and this effect was associated with its cytotoxicity. The differential regulation of metabolism by melatonin could explain why the hormone is harmless for a wide spectrum of normal and only a few tumoral cells, while it kills specific tumor cell types. PMID:26252771

  8. Synovial sarcoma with radiological appearances of primitive neuroectodermal tumour/Ewing sarcoma: differentiation by molecular genetic studies

    International Nuclear Information System (INIS)

    Synovial sarcoma (SS) arises in soft tissues but may invade adjacent bone. We describe a case of SS presenting as aggressive lysis of the proximal ulna, the imaging of which suggested a primary bone lesion. Needle biopsy showed a 'small round blue cell tumour', and a primitive neuroectodermal tumour (PNET)/Ewing sarcoma was suggested on the basis of the imaging appearances. The definitive diagnosis of synovial sarcoma was made following molecular genetic studies, which demonstrated a fusion product incorporating the genes SYT and SSX1. The importance of correct diagnosis to guide appropriate management, and, therefore, the necessity for molecular genetic studies, is discussed. (orig.)

  9. Synovial sarcoma with radiological appearances of primitive neuroectodermal tumour/Ewing sarcoma: differentiation by molecular genetic studies

    Energy Technology Data Exchange (ETDEWEB)

    O' Donnell, P. [Royal National Orthopaedic Hospital NHS Trust, Department of Radiology, Stanmore, Middlesex (United Kingdom); Diss, T.C. [University College Hospital, Department of Histopathology, London (United Kingdom); Whelan, J. [University College Hospital, Meyerstein Institute of Oncology, London (United Kingdom); Flanagan, A.M. [University College Hospital, Department of Histopathology, London (United Kingdom); Royal National Orthopaedic Hospital, Department of Histopathology, Stanmore, Middlesex (United Kingdom); University College, Institute of Orthopaedics, London (United Kingdom)

    2006-04-15

    Synovial sarcoma (SS) arises in soft tissues but may invade adjacent bone. We describe a case of SS presenting as aggressive lysis of the proximal ulna, the imaging of which suggested a primary bone lesion. Needle biopsy showed a 'small round blue cell tumour', and a primitive neuroectodermal tumour (PNET)/Ewing sarcoma was suggested on the basis of the imaging appearances. The definitive diagnosis of synovial sarcoma was made following molecular genetic studies, which demonstrated a fusion product incorporating the genes SYT and SSX1. The importance of correct diagnosis to guide appropriate management, and, therefore, the necessity for molecular genetic studies, is discussed. (orig.)

  10. Evaluation of NKX2-2 expression in round cell sarcomas and other tumors with EWSR1 rearrangement: imperfect specificity for Ewing sarcoma.

    Science.gov (United States)

    Hung, Yin P; Fletcher, Christopher D M; Hornick, Jason L

    2016-04-01

    Ewing sarcoma shows considerable histologic overlap with other round cell tumors. NKX2-2, a homeodomain transcription factor involved in neuroendocrine/glial differentiation and a downstream target of EWSR1-FLI1, has been reported as an immunohistochemical marker for Ewing sarcoma. We assessed the specificity of NKX2-2 for Ewing sarcoma compared with other round cell malignant neoplasms and other soft tissue tumors with EWSR1 translocations. We evaluated whole-tissue sections from 270 cases: 40 Ewing sarcomas (4 with atypical/large cell features), 20 CIC-DUX4 sarcomas, 5 BCOR-CCNB3 sarcomas, 9 unclassified round cell sarcomas, 10 poorly differentiated synovial sarcomas, 10 lymphoblastic lymphomas, 10 alveolar rhabdomyosarcomas, 10 embryonal rhabdomyosarcomas, 10 Merkel cell carcinomas, 10 small cell carcinomas, 20 melanomas, 5 NUT midline carcinomas, 10 Wilms tumors, 10 neuroblastomas, 10 olfactory neuroblastomas, 12 mesenchymal chondrosarcomas, 10 angiomatoid fibrous histiocytomas, 10 clear cell sarcomas, 5 gastrointestinal clear cell sarcoma-like tumors, 5 desmoplastic small round cell tumors, 10 extraskeletal myxoid chondrosarcomas, 10 soft tissue and cutaneous myoepitheliomas, and 19 myoepithelial carcinomas. NKX2-2 positivity was defined as moderate-to-strong nuclear immunoreactivity in at least 5% of cells. NKX2-2 was positive in 37/40 (93%) Ewing sarcomas, including all atypical Ewing sarcomas and cases with known EWSR1-FLI1 or EWSR1-ERG fusion; 85% of Ewing sarcomas showed diffuse (>50%) staining. NKX2-2 was positive in 9 (75%) mesenchymal chondrosarcomas, 8 (80%) olfactory neuroblastomas, 1 CIC-DUX4 sarcoma, 1 poorly differentiated synovial sarcoma, 1 neuroblastoma, 2 unclassified round cell sarcomas, and 3 small cell carcinomas; all other EWSR1-associated tumors were negative for NKX2-2, apart from 1 desmoplastic small round cell tumor, 1 myoepithelioma, and 1 myoepithelial carcinoma. In summary, NKX2-2 is a sensitive but imperfectly specific marker for

  11. Gene Expression Music Algorithm-Based Characterization of the Ewing Sarcoma Stem Cell Signature

    Science.gov (United States)

    2016-01-01

    Gene Expression Music Algorithm (GEMusicA) is a method for the transformation of DNA microarray data into melodies that can be used for the characterization of differentially expressed genes. Using this method we compared gene expression profiles from endothelial cells (EC), hematopoietic stem cells, neuronal stem cells, embryonic stem cells (ESC), and mesenchymal stem cells (MSC) and defined a set of genes that can discriminate between the different stem cell types. We analyzed the behavior of public microarray data sets from Ewing sarcoma (“Ewing family tumors,” EFT) cell lines and biopsies in GEMusicA after prefiltering DNA microarray data for the probe sets from the stem cell signature. Our results demonstrate that individual Ewing sarcoma cell lines have a high similarity to ESC or EC. Ewing sarcoma cell lines with inhibited Ewing sarcoma breakpoint region 1-Friend leukemia virus integration 1 (EWSR1-FLI1) oncogene retained the similarity to ESC and EC. However, correlation coefficients between GEMusicA-processed expression data between EFT and ESC decreased whereas correlation coefficients between EFT and EC as well as between EFT and MSC increased after knockdown of EWSR1-FLI1. Our data support the concept of EFT being derived from cells with features of embryonic and endothelial cells. PMID:27446218

  12. Gene Expression Music Algorithm-Based Characterization of the Ewing Sarcoma Stem Cell Signature

    Directory of Open Access Journals (Sweden)

    Martin Sebastian Staege

    2016-01-01

    Full Text Available Gene Expression Music Algorithm (GEMusicA is a method for the transformation of DNA microarray data into melodies that can be used for the characterization of differentially expressed genes. Using this method we compared gene expression profiles from endothelial cells (EC, hematopoietic stem cells, neuronal stem cells, embryonic stem cells (ESC, and mesenchymal stem cells (MSC and defined a set of genes that can discriminate between the different stem cell types. We analyzed the behavior of public microarray data sets from Ewing sarcoma (“Ewing family tumors,” EFT cell lines and biopsies in GEMusicA after prefiltering DNA microarray data for the probe sets from the stem cell signature. Our results demonstrate that individual Ewing sarcoma cell lines have a high similarity to ESC or EC. Ewing sarcoma cell lines with inhibited Ewing sarcoma breakpoint region 1-Friend leukemia virus integration 1 (EWSR1-FLI1 oncogene retained the similarity to ESC and EC. However, correlation coefficients between GEMusicA-processed expression data between EFT and ESC decreased whereas correlation coefficients between EFT and EC as well as between EFT and MSC increased after knockdown of EWSR1-FLI1. Our data support the concept of EFT being derived from cells with features of embryonic and endothelial cells.

  13. Acute lymphoblastic leukemia with multiple cytogenetic abnormalities secondary to treatment of Ewing's sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Al-Homaidhi, A.M. [Department of Medicine, Princess Margaret Hospital and The University of Toronto, 610 University Ave, Rm. 4-429, Toronto, Ont. M5G 2M9 (Canada); Patterson, B. [Department of Pathology, Princess Margaret Hospital and The University of Toronto, 610 University Ave, Rm. 4-429, Toronto, Ont. M5G 2M9 (Canada); Rubin, S. [Moncton Hospital, Moncton, New Brunswick (Canada); Lipton, J.H. [Department of Medicine, Princess Margaret Hospital and The University of Toronto, 610 University Ave, Rm. 4-429, Toronto, Ont. M5G 2M9 (Canada)

    1999-06-01

    We report the case of a 22-year-old man with Ewing's sarcoma who attained a complete remission (CR) after combination radiotherapy and chemotherapy. Secondary acute lymphoblastic leukemia with multiple cytogenetic abnormalities involving chromosome 5 and 7 developed 16 years later. The patient underwent induction chemotherapy and entered a CR. Peripheral blood stem cell transplantation from a matched sibling was performed successfully and he is in complete remission of both ALL and Ewing's sarcoma. (Copyright (c) 1999 Elsevier Science B.V., Amsterdam. All rights reserved.)

  14. Acute lymphoblastic leukemia with multiple cytogenetic abnormalities secondary to treatment of Ewing's sarcoma

    International Nuclear Information System (INIS)

    We report the case of a 22-year-old man with Ewing's sarcoma who attained a complete remission (CR) after combination radiotherapy and chemotherapy. Secondary acute lymphoblastic leukemia with multiple cytogenetic abnormalities involving chromosome 5 and 7 developed 16 years later. The patient underwent induction chemotherapy and entered a CR. Peripheral blood stem cell transplantation from a matched sibling was performed successfully and he is in complete remission of both ALL and Ewing's sarcoma. (Copyright (c) 1999 Elsevier Science B.V., Amsterdam. All rights reserved.)

  15. Sarcoma de Ewing extraesquelético que semeja abdomen agudo

    OpenAIRE

    Gilberto Guzmán Valdivia-Gómez; María Teresa Soto-Guerrero; María Isabel Cedillo-de la Cruz

    2010-01-01

    Introducción: El sarcoma de Ewing extraóseo es un tumor raro de origen neuroectodérmico que se ha presentado principalmente en las partes blandas de las extremidades y del tórax; histológicamente es similar al sarcoma de Ewing en el tejido óseo. Caso clínico: Paciente con abdomen agudo y leucocitosis, en quien por imagen (ultrasonido y tomografía axial computarizada) se diagnosticó enfermedad diverticular complicada del colon, por lo que fue intervenido quirúrgicamente, encontrando lesión loc...

  16. Molecular pathogenesis and targeted therapeutics in Ewing sarcoma/primitive neuroectodermal tumours

    Directory of Open Access Journals (Sweden)

    Kelleher Fergal C

    2012-02-01

    Full Text Available Abstract Background Ewing sarcoma/PNET is managed with treatment paradigms involving combinations of chemotherapy, surgery, and sometimes radiation. Although the 5-year survival rate of non-metastatic disease approaches 70%, those cases that are metastatic and those that recur have 5-year survival rates of less than 20%. Molecularly targeted treatments offer the potential to further improve treatment outcomes. Methods A PUBMED search was performed from 1997 to 2011. Published literature that included the topic of the Ewing sarcoma/PNET was also referenced. Results Insulin-like growth factor-1 receptor (IGF-1R antagonists have demonstrated modest single agent efficacy in phase I/II clinical trials in Ewing sarcoma/PNET, but have a strong preclinical rationale. Based on in vitro and animal data, treatment using antisense RNA and cDNA oligonucleotides directed at silencing the EWS-FLI chimera that occurs in most Ewing sarcoma/PNET may have potential therapeutic importance. However drug delivery and degradation problems may limit this therapeutic approach. Protein-protein interactions can be targeted by inhibition of RNA helicase A, which binds to EWS/FLI as part of the transcriptional complex. Tumour necrosis factor related apoptosis inducing ligand induction using interferon has been used in preclinical models. Interferons may be incorporated into future chemotherapeutic treatment paradigms. Histone deacetylase inhibitors can restore TGF-β receptor II allowing TFF-β signalling, which appears to inhibit growth of Ewing sarcoma/PNET cell lines in vitro. Immunotherapy using allogeneic natural killer cells has activity in Ewing sarcoma/PNET cell lines and xenograft models. Finally, cyclin dependent kinase inhibitors such as flavopiridol may be clinically efficacious in relapsed Ewing sarcoma/PNET. Conclusion Preclinical evidence exists that targeted therapeutics may be efficacious in the ESFT. IGF-1R antagonists have demonstrated efficacy in phase I

  17. Renal artery stenosis after radiotherapy for Ewing's sarcoma

    International Nuclear Information System (INIS)

    Background: the fact that therapeutic irradiation can induce significant stenosis in the arteries of the head, neck, and chest, as welt as in the aorta and the iliac arteries, is familiar in daily practice and well documented in the literature. By contrast, radiation-induced renal artery stenosis seems to be a less widely known complication. Patients and methods: the sudden onset of medically refractory arterial hypertension and coma in a 27-year-old man is reported, who had been treated at age 20 with chemotherapy and radiotherapy for Ewing's sarcoma in the lumbar region. This treatment had been performed at the hospital of Sion, Switzerland in 2001. Also, the relevant literature from 1965 to 2007 is reviewed to underscore various aspects of this problem and to demonstrate the clinical relevance of renal artery stenosis as a potential long-term sequela of radiotherapy. Conclusion: radiation-induced renal artery stenosis has only rarely been described in the literature, but arterial hypertension due to radiation-induced renal artery stenosis is a serious long-term sequela that can appear at a latency of up to 20 years after treatment. The paucity of reports presumably reflects the lesser frequency of radiotherapy for retroperitoneal tumors as compared to head-and-neck cancers, as well as lower awareness of the problem due to diagnostic bias in the era before CT and MRI were in routine use: at that time, carotid artery stenosis was easy to diagnose by ultrasonography, while radiation-induced renal artery stenosis, whose real incidence may well be higher, probably often went undetected. Thus, when a patient with a history of abdominal or retroperitoneal radiotherapy unexpectedly develops intractable hypertension, radiation-induced renal artery stenosis must be included in the differential diagnosis. (orig.)

  18. Very Late Local Relapse of Ewing's Sarcoma of the Head and Neck treated with Aggressive Multimodal Therapy

    Directory of Open Access Journals (Sweden)

    A. Thyss

    2008-05-01

    Full Text Available Ewing's sarcoma's relapse rarely occurs more than two years after the initial diagnosis. We report the case of a 26-year-old man with a history of Ewing's sarcoma of the left maxillary sinus at the age of 10 who presented with a very late local relapse, 16 years after the first occurrence of disease. Ultimate control was achieved after multimodal therapy including surgery, high-dose chemotherapy, and radiotherapy. This report indicates that local relapses of Ewing's sarcoma can be treated with curative intent in selected cases.

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

    OpenAIRE

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

    2012-01-01

    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 E...

  20. Therapeutic opportunities in Ewing sarcoma: EWS-FLI inhibition via LSD1 targeting.

    Science.gov (United States)

    Theisen, Emily R; Pishas, Kathleen I; Saund, Ranajeet S; Lessnick, Stephen L

    2016-04-01

    Ewing sarcoma is an aggressive primary pediatric bone tumor, often diagnosed in adolescents and young adults. A pathognomonic reciprocal chromosomal translocation results in a fusion gene coding for a protein which derives its N-terminus from a FUS/EWS/TAF15 (FET) protein family member, commonly EWS, and C-terminus containing the DNA-binding domain of an ETS transcription factor, commonly FLI1. Nearly 85% of cases express the EWS-FLI protein which functions as a transcription factor and drives oncogenesis. As the primary genomic lesion and a protein which is not expressed in normal cells, disrupting EWS-FLI function is an attractive therapeutic strategy for Ewing sarcoma. However, transcription factors are notoriously difficult targets for the development of small molecules. Improved understanding of the oncogenic mechanisms employed by EWS-FLI to hijack normal cellular programming has uncovered potential novel approaches to pharmacologically block EWS-FLI function. In this review we examine targeting the chromatin regulatory enzymes recruited to conspire in oncogenesis with a focus on the histone lysine specific demethylase 1 (LSD1). LSD1 inhibitors are being aggressively investigated in acute myeloid leukemia and the results of early clinical trials will help inform the future use of LSD1 inhibitors in sarcoma. High LSD1 expression is observed in Ewing sarcoma patient samples and mechanistic and preclinical data suggest LSD1 inhibition globally disrupts the function of EWS-ETS proteins. PMID:26848860

  1. The second European interdisciplinary Ewing sarcoma research summit – A joint effort to deconstructing the multiple layers of a complex disease

    OpenAIRE

    Kovar, Heinrich; Amatruda, James; Brunet, Erika; Burdach, Stefan; Cidre-Aranaz, Florencia; de Alva, Enrique; Dirksen, Uta; van der Ent, Wietske; Grohar, Patrick; Grunewald, Thomas G. P.; Helman, Lee; Houghton, Peter; Iljin, Kristiina; Korsching, Eberhard; Ladanyi, Marc

    2016-01-01

    Despite multimodal treatment, long term outcome for patients with Ewing sarcoma is still poor. The second “European interdisciplinary Ewing sarcoma research summit” assembled a large group of scientific experts in the field to discuss their latest unpublished findings on the way to the identification of novel therapeutic targets and strategies. Ewing sarcoma is characterized by a quiet genome with presence of an EWSR1-ETS gene rearrangement as the only and defining genetic aberration. RNA-seq...

  2. Hypoxia and hypoglycaemia in Ewing's sarcoma and osteosarcoma: regulation and phenotypic effects of Hypoxia-Inducible Factor

    International Nuclear Information System (INIS)

    Hypoxia regulates gene expression via the transcription factor HIF (Hypoxia-Inducible Factor). Little is known regarding HIF expression and function in primary bone sarcomas. We describe HIF expression and phenotypic effects of hypoxia, hypoglycaemia and HIF in Ewing's sarcoma and osteosarcoma. HIF-1α and HIF-2α immunohistochemistry was performed on a Ewing's tumour tissue array. Ewing's sarcoma and osteosarcoma cell lines were assessed for HIF pathway induction by Western blot, luciferase assay and ELISA. Effects of hypoxia, hypoglycaemia and isoform-specific HIF siRNA were assessed on proliferation, apoptosis and migration. 17/56 Ewing's tumours were HIF-1α-positive, 15 HIF-2α-positive and 10 positive for HIF-1α and HIF-2α. Expression of HIF-1α and cleaved caspase 3 localised to necrotic areas. Hypoxia induced HIF-1α and HIF-2α in Ewing's and osteosarcoma cell lines while hypoglycaemia specifically induced HIF-2α in Ewing's. Downstream transcription was HIF-1α-dependent in Ewing's sarcoma, but regulated by both isoforms in osteosarcoma. In both cell types hypoglycaemia reduced cellular proliferation by ≥ 45%, hypoxia increased apoptosis and HIF siRNA modulated hypoxic proliferation and migration. Co-localisation of HIF-1α and necrosis in Ewing's sarcoma suggests a role for hypoxia and/or hypoglycaemia in in vivo induction of HIF. In vitro data implicates hypoxia as the primary HIF stimulus in both Ewing's and osteosarcoma, driving effects on proliferation and apoptosis. These results provide a foundation from which to advance understanding of HIF function in the pathobiology of primary bone sarcomas

  3. Extraosseous Ewing's sarcoma / primitive neuroectodermal tumor of the sacral nerve plexus

    International Nuclear Information System (INIS)

    We report an unusual case of Ewing's sarcoma / primitive neuroectodermal tumor (PNET) of the sacral nerve plexus in a 9-year-old boy who presented with a soft tissue swelling and severe piercing pain in the lower back region. MRI of the lumbosacral spine showed a lobulated soft tissue mass with clubbed finger-like projections along the path of the sacral nerves, which had caused widening of the spinal canal and the sacral foramina (S2–S4 level). There was presacral extension and posterior scalloping of the sacral vertebrae. Histopathology of the lesion confirmed Ewing's sarcoma / PNET of the sacral spinal nerve plexus. The patient responded favorably to chemotherapy and radiotherapy, showing clinical and radiological improvement

  4. Correlation between radiologic evaluation modalities and histologic tumor response in chemotherapy-treated Ewing sarcoma

    International Nuclear Information System (INIS)

    In Ewing sarcoma, the addition of preoperative and postoperative chemotherapy has dramatically raised the 5-year survival rate. Radiologic evaluation of chemotherapy response becomes important so that the treatment plan can be altered in cases of poor response. The authors evaluated sequential examinations, including plain radiographs, Tc-99m skeletal scintigrams, and CT scans in 48 patients with Ewing sarcoma of bone. In 31 patients, biopsy material was obtained for histologic grading of treatment response. Good tumor response (grades 3 and 4) led over the ensuing 1-3 months to disappearance of the soft-tissue tumor component, solid transformation of the previously lamellated or spiculated periosteal reaction, and filling in of the lytic regions. Insufficient tumor response (grades 1 and 2) demonstrated persistence of soft-tissue tumor component and lamellated or spiculated periosteal reaction as well as absence, filling in, or even enlargement of lytic regions

  5. Microarrays in Molecular Profiling of Ewing Sarcoma Family of Tumors and CDKN2A Aberrations

    OpenAIRE

    Savola, Suvi

    2009-01-01

    Background: The Ewing sarcoma family of tumors (ESFT) are rare but highly malignant neoplasms that occur mainly in bone or but also in soft tissue. ESFT affects patients typically in their second decade of life, whereby children and adolescents bear the heaviest incidence burden. Despite recent advances in the clinical management of ESFT patients, their prognosis and survival are still disappointingly poor, especially in cases with metastasis. No targeted therapy for ESFT patients is currentl...

  6. PARP-1 inhibition as a targeted strategy to treat Ewing's sarcoma

    OpenAIRE

    Brenner, J. Chad; Feng, Felix Y.; Han, Sumin; Patel, Sonam; Goyal, Siddharth V.; Bou-Maroun, Laura M.; Liu, Meilan; Lonigro, Robert; Prensner, John R.; Tomlins, Scott A.; Chinnaiyan, Arul M.

    2012-01-01

    Ewing's sarcoma family tumors (ESFTs) are aggressive malignancies which frequently harbor characteristic EWS-FLI1 or EWS-ERG genomic fusions. Here we report that these fusion products interact with the DNA damage response protein and transcriptional co-regulator PARP-1. ESFT cells, primary tumor xenografts and tumor metastases were all highly sensitive to PARP1 inhibition. Addition of a PARP1 inhibitor to the second-line chemotherapeutic agent temozolamide resulted in complete responses of al...

  7. Characterization of Ewing sarcoma associated cancer/testis antigens

    OpenAIRE

    Mahlendorf, Dorothea E.; Staege, Martin Sebastian

    2013-01-01

    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 involv...

  8. Ewing`s sarcoma treatment in Scandinavia 1984-1990. Ten-year results of the Scandinavian sarcoma group protocol SSGIV

    Energy Technology Data Exchange (ETDEWEB)

    Nilbert, M.; Alvegaard, T.A. [University Hospital Lund (Sweden). Dept. of Oncology; Saeter, G. [University Hospital Lund (Sweden). Dept. of Pediatric Oncology]|[Norwegian Radium Hospital, Oslo (Norway). Dept. of Oncology; Elomaa, I. [University Hospital, Helsinki (Finland). Dept. of Oncology; Monge, O.R. [Haukeland Hospital, Bergen (Norway). Dept. of Oncology; Wiebe, T

    1998-12-31

    A report on the long-term follow up of the first cooperative Scandinavian Sarcoma Group study in Ewing`s sarcoma of bone is presented. Fifty-two previously untreated patients entered the study between 1984 and 1990. Half of the tumors were located in the extremities and one quarter in the pelvis. The combined modality treatment consisted of 5 cycles of chemotherapy - including vincristine, methotrexate, doxorubicin, cyclophosphamide, bleomycin and dactinomycin. The first two cycles were followed by local resection or amputation in 35 patients and by radiotherapy alone in 17 patients. When surgery was not performed, was incomplete or yielded poor margins radiotherapy was given at a dose of 40-60 Gy. Local tumor relapses developed in 10 patients and in all but one patient were accompanied by metastatic disease. Five patients had metastasis at diagnosis and distant metastases developed after primary treatment in 27 patients after a median of 14 months. The median follow-up time for the 20 surviving patients is 10 years. At 5 years the tumor-related survival was 46% and the metastasis-free survival 43%. Late tumor relapses occurred in 4 patients, which reduced the 10-year tumor related survival to 41% and the metastasis-free survival to 38%. Histopathological tumour response correlated with survival with 5-year metastasis-free survival rates of 73% for the good responders and 35% for the poor responders. (orig.)

  9. Ewing's sarcoma in mandibular similar to dental abscess

    OpenAIRE

    Forouz Keshani; Gholamreza Jahanshahi; Bijan Movahedian Attar; Mahsa Kalantari; Seyed Mohammad Razavi; Zahra Hashemzade; Payam Tavakoli

    2014-01-01

    Ewing′s sarcoma is a rare malignant neoplasm that comprises approximately 4-6% of primary bone tumors. In most cases, femur and pelvis are affected, and less commonly the head and neck areas (in the jaws, usually the mandible). These tumors have been reported more frequently in males, mostly aged 5-20 years old. Systemic symptoms and signs such as fever, weight loss, anemia, leukocytosis, and elevated erythrocyte sedimentation rate (ESR) may be the first signs in oral Ewing′s sarcoma. Such...

  10. High ALDH activity identifies chemotherapy-resistant Ewing's sarcoma stem cells that retain sensitivity to EWS-FLI1 inhibition.

    Directory of Open Access Journals (Sweden)

    Ola Awad

    Full Text Available 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 (ALDH(high activity and are enriched for clonogenicity, sphere-formation, and tumor initiation. The ALDH(high 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 ALDH(high 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.

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

    Directory of Open Access Journals (Sweden)

    Kurkure Purna

    2010-03-01

    Full Text Available Abstract 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 Indian girl. She presented with a vaginal mass that was initially diagnosed as a malignant round cell tumor. Immunohistochemistry showed diffuse positivity for vimentin, membranous positivity for MIC2, and positivity for BCL2 and FLI-1. On the other hand, she was negative for cytokeratin, epithelial membrane antigen, desmin, Myo D-1, myogenin and smooth muscle actin. A diagnosis of primitive neuroectodermal tumor was thus offered. Furthermore, a molecular analysis of our patient using reverse transcription-polymerase chain reaction technique showed positivity for t(11; 22 (q24; q12 (EWSR1-FLI1, thus confirming the diagnosis of a Ewing's sarcoma/primitive neuroectodermal tumor. Our patient was offered chemotherapy on Institutional protocol EFT 2001. Conclusion This is a rare case of primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor, which was confirmed with molecular analysis, in the youngest patient known so far. This study reinforces the value of integrating morphological features with membranous MIC2 positivity, along with application of molecular techniques in objective identification of an Ewing's sarcoma or primitive neuroectodermal tumor at uncommon sites.

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

    Science.gov (United States)

    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

    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

  13. Establishing a panel of chemo-resistant mesothelioma models for investigating chemo-resistance and identifying new treatments for mesothelioma

    OpenAIRE

    Hudson, Amanda L.; Weir, Chris; Moon, Elizabeth; Harvie, Rozelle; Klebe, Sonja; Clarke, Stephen J.; Pavlakis, Nick; Howell, Viive M.

    2014-01-01

    Mesothelioma is inherently chemo-resistant with only 50% of patients responding to the standard of care treatments, and consequently it has a very grim prognosis. The aim of this study was to establish a panel of chemo-resistant mesothelioma models with clinically relevant levels of resistance as tools for investigating chemo-resistance and identifying new treatments for mesothelioma. Chemo-resistant cell lines were established in vitro and characterized in vivo using syngeneic Fischer rats. ...

  14. EWS-FLI1 inhibits TNFα-induced NFκB-dependent transcription in Ewing sarcoma cells

    International Nuclear Information System (INIS)

    Research highlights: → EWS-FLI1 interferes with TNF-induced activation of NFκB in Ewing sarcoma cells. → EWS-FLI1 knockdown in Ewing sarcoma cells increases TNF-induced NFκB binding to DNA. → EWS-FLI1 reduces TNF-stimulated NFκB-dependent transcriptional activation. → Constitutive NFκB activity is not affected by EWS-FLI1. → EWS-FLI1 physically interacts with NFκB 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 (NFκB) is a tightly regulated transcription factor controlling cell survival, proliferation and differentiation, as well as tumorigenesis. NFκB activity is very low in unstimulated Ewing sarcoma cells, but can be induced in response to tumor necrosis factor (TNF). We wondered whether NFκB 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 NFκB basal activity, but impairs TNF-induced NFκB-driven transcription, at least in part through inhibition of NFκB binding to DNA. We detected an in vivo physical interaction between the fusion protein and NFκB 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 NFκB.

  15. Primary spinal epidural extraosseous Ewing's sarcoma: Report of five cases and literature review

    International Nuclear Information System (INIS)

    Ewing's sarcoma is the most common malignant bone tumour occurring in children and adolescents and exists in two different clinico pathological entities: osseous Ewing's sarcoma (OES) and extraosseous Ewing's sarcoma (EES). Five cases of primary epidural EES are described, which presented with non-specific symptoms leading to a long diagnostic delay. The median age at diagnosis was 22 years (range 13-36 years). The median diagnostic delay was 3 months. All patients had one or more neurological deficits. All underwent surgical exploration with a laminectomy and partial resection followed by adjuvant radiotherapy to a dose of 46-50 Gy and chemotherapy with VAC (vincristine, adriamycin and cyclophosphamide) alternating with ICE (ifosphamide, cisplatin and etoposide) for at least six cycles. The mean follow-up period is 21.2 months (range 11-32 months). Four of the five patients achieved a complete remission and are disease free at the time of writing this report. Two patients have a residual neurological deficit - both having presented with long history of neurological deficit. Primary spinal epidural EES should be suspected whenever young patients present with back pain and/or radicular pain, have abnormal neurology and an extradural mass is demonstrated on MRI. Surgical excision followed by adjuvant radiotherapy (50 Gy) and combination chemotherapy (VAC alternating with ICE) achieved local and systemic control in these patients. A greater number of patients and longer follow up are required to evolve a generally accepted treatment policy for this aggressive but potentially curable malignancy. Copyright (2001) Blackwell Science Pty Ltd

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

    Directory of Open Access Journals (Sweden)

    Andrew S Brohl

    2014-07-01

    Full Text Available 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.

  17. A Radiological Review of Ewing's Sarcoma of Mandible: A Case Report with One Year Follow-up.

    Science.gov (United States)

    Krishna, Kb Bimal; Thomas, Valsa; Kattoor, Jayasree; Kusumakumari, P

    2013-05-01

    Ewing's sarcoma (ES) is an uncommon round cell tumor with an aggressive course affecting mainly children and young adults. Only 1% of cases is reported with jaw involvement and have mandibular predilection. Radiographic finding in ES reflect many destructive nature of the lesion, like osteolysis, cortical erosion, periostitis and soft tissue mass. A case of ES of the mandible is reported with special consideration to the radiological appearance. How to cite this article: Krishna KBB, Thomas V, Kattoor J, Kusumakumari P. A Radiological Review of Ewing's Sarcoma of Mandible: A Case Report with One Year Follow-up. Int J Clin Pediatr Dent 2013;6(2):109-114. PMID:25206203

  18. Ewing's sarcoma of the humerus mimicking fibrous dysplasia on imaging and biological behavior

    International Nuclear Information System (INIS)

    We present the case of a 12-year-old girl who presented with a pathological fracture through a benign-appearing osteolytic lesion that was presumed to represent fibrous dysplasia. The fracture healed, and over the next 2.5 years there was further refracturing and healing with progressive osteolysis. A biopsy was performed and revealed Ewing's sarcoma. The disease was locally controlled by neoadjuvant chemotherapy and radiation therapy. The patient is disease free with excellent function 6 years following the discovery of the lesion. We illustrate and discuss the sequence of events. (orig.)

  19. Coverage of Megaprosthesis with Human Acellular Dermal Matrix after Ewing's Sarcoma Resection: A Case Report

    Directory of Open Access Journals (Sweden)

    Robert M. Whitfield

    2011-01-01

    Full Text Available A 23-year-old female with Ewing's Sarcoma underwent tibial resection and skeletal reconstruction using proximal tibial allograft prosthetic reconstruction with distal femur endoprosthetic reconstruction and rotating hinge. Human acellular dermal matrix, (Alloderm, LifeCell, Branchburg, NJ, USA, was used to wrap the skeletal reconstruction. Soft tissue reconstruction was completed with a rotational gastrocnemius muscle flap and skin graft. Despite prolonged immobilization, the patient quickly regained full range of motion of her skeletal reconstruction. Synthetic mesh, tapes and tubes are used to perform capsule reconstruction of megaprosthesis. This paper describes the role of human acellular dermal matrix in capsule reconstruction around a megaprosthesis.

  20. Programs of radiation and drug therapy for lung metastases of Ewing's sarcoma in children

    International Nuclear Information System (INIS)

    As a result of total-local irradiation of the lung combined with polychemotherapy in patients with solitary metastases to the lungs, a 3-year survival in children was 52.7+-12.1%, after local irradiation of the lungs and chemotherapy 30+-11.8% of the patients. Combined treatment was Well tolerated by pediatric patients. The results of the stUdy have shown that radiation therapy of metastases of Ewing sarcoma to the lungs with simultaneoUs polychemotherapy does not disturb external respiratory function even in a later period, the clinical manifestations of lung radiation reactions were seldom observed

  1. [Secondary TFE3-associated renal cell carcinoma in a child treated for Ewing sarcoma].

    Science.gov (United States)

    Fedhila Ben Ayed, F; Rhayem, S; Doghri, R; Ben Hassine, L; Khemiri, M; Mrad, K; Bellagha, I; Barsaoui, S

    2016-02-01

    Renal cell carcinoma is a rare pediatric malignant tumor of the kidney. Unlike Wilms tumor, the efficacy of chemotherapy and radiation therapy in pediatric renal cell carcinoma remains uncertain. Surgery is the best treatment and prognosis is favorable when the tumor is localized and completely eradicated. We report an exceptional observation in a 7-year-old girl with renal cell carcinoma who had been treated 20 months previously for Ewing sarcoma with chemotherapy and radiotherapy. The renal tumor was revealed by abdominal pain without hematuria. She underwent a radical nephrectomy, and histopathology concluded in renal carcinoma associated with translocation Xp 11.2 grade 3 of Furhrman pT3a N1. No adjuvant therapy was given. After 3 years of follow-up, there is no evidence of local or metastatic recurrence. This observation is significant given the very young age of this patient, the occurrence after Ewing sarcoma with a short disease-free interval. It seems that translocation renal cell carcinoma is associated with previous exposure to chemotherapy, particularly topoisomerase II inhibitors or alkylating agents. PMID:26702489

  2. Lung irradiation for Ewing's sarcoma with pulmonary metastases at diagnosis: results of the CESS-studies

    International Nuclear Information System (INIS)

    Background: In the German CESS-studies, Ewing's sarcoma patients with pulmonary metastases at diagnosis were considered as candidates for additional bilateral lung irradiation. We have retrospectively analyzed the impact of radiotherapy on survival. Material and methods: Out of 42 patients presenting with pulmonary metastases of Ewing's sarcoma between 1981 and June 1992, 30 were evaluable. One patient died of progressive disease prior to lung irradiation. The other patients had a complete radiographic remission either after chemotherapy (n=25) or chemotherapy plus resection of lung metastases (n=4). 22 patients received bilateral lung irradiation with doses of 12 to 21 Gy (once daily 1.5 Gy or twice daily 1.25 Gy), six had no further treatment and one had bone marrow transplantation for consolidation. Results: Ten patients are in complete remission, nine of them had received lung irradiation and one had a complete resection of three lung metastases. Two patients are alive with disease, two have died from treatment-related complications and 16 have died from systemic disease with or without local relapse. NED-patients had received significant higher lung doses than relapsed patients p=0.028. Moreover, a dose-response relationship was detectable: 1/6 patients without lung irradiation vs. 4/10 with 12 to 16 Gy vs. 5/6 with 18 to 21 Gy lung irradiation were in remission. (orig.)

  3. Zoledronic acid negatively affects the expansion of in vitro activated human NK cells and their cytolytic interactions with Ewing sarcoma cells.

    Science.gov (United States)

    Mueller, Sarah-Kristin; Altvater, Bianca; Chen, Christiane; Kailayangiri, Sareetha; Ahlmann, Martina; Dirksen, Uta; Juergens, Heribert; Rossig, Claudia

    2013-06-01

    Disseminated Ewing sarcoma remains a fatal disease despite advanced multimodal treatment regimens. Immunotherapies as well as novel drugs and biologicals are currently being explored to eliminate minimal residual disease after conventional therapy thereby rescuing patients at a high risk for relapse. Insights into the interactions between novel therapies provide the basis for the development of effective combination strategies. We investigated the effects of the aminobisphosphonate zoledronic acid (ZA) on the in vitro expansion of human natural killer (NK) cells and their cytolytic activity against Ewing sarcoma cells. ZA significantly impaired the in vitro expansion of activated NK cells from both healthy donors and Ewing sarcoma patients in a dose-dependent manner. Expression of differentiation markers and activating receptors was unaffected by the drug. Activated NK cells from both healthy donors and patients had potent degranulation responses to Ewing sarcoma cells. In the presence of ZA at concentrations reflecting pharmaceutical serum levels, the in vitro antitumor activity of NK cells from Ewing sarcoma patients was significantly impaired. We conclude that ZA can impede in vitro NK cell expansion and cytolytic NK cell responses to Ewing sarcoma. These observations raise caution against the combination of adoptive NK cell transfer with ZA maintenance therapy in Ewing sarcoma. Future studies aim to identify potentiating interactions of novel drugs with cellular therapies. PMID:23525469

  4. The clinical use of biomarkers as prognostic factors in Ewing sarcoma

    Directory of Open Access Journals (Sweden)

    van Maldegem Annmeik M

    2012-02-01

    Full Text Available Abstract Ewing Sarcoma is the second most common primary bone sarcoma with 900 new diagnoses per year in Europe (EU27. It has a poor survival rate in the face of metastatic disease, with no more than 10% survival of the 35% who develop recurrence. Despite the remaining majority having localised disease, approximately 30% still relapse and die despite salvage therapies. Prognostic factors may identify patients at higher risk that might require differential therapeutic interventions. Aside from phenotypic features, quantitative biomarkers based on biological measurements may help identify tumours that are more aggressive. We audited the research which has been done to identify prognostic biomarkers for Ewing sarcoma in the past 15 years. We identified 86 articles were identified using defined search criteria. A total of 11,625 patients were reported, although this number reflects reanalysis of several cohorts. For phenotypic markers, independent reports suggest that tumour size > 8 cm and the presence of metastasis appeared strong predictors of negative outcome. Good histological response (necrosis > 90% after treatment appeared a significant predictor for a positive outcome. However, data proposing biological biomarkers for practical clinical use remain un-validated with only one secondary report published. Our recommendation is that we can stratify patients according to their stage and using the phenotypic features of metastases, tumour size and histological response. For biological biomarkers, we suggest a number of validating studies including markers for 9p21 locus, heat shock proteins, telomerase related markers, interleukins, tumour necrosis factors, VEGF pathway, lymphocyte count, and a number of other markers including Ki-67.

  5. Intraoperative Electron-Beam Radiation Therapy for Pediatric Ewing Sarcomas and Rhabdomyosarcomas: Long-Term Outcomes

    Energy Technology Data Exchange (ETDEWEB)

    Sole, Claudio V., E-mail: csole@iram.cl [Department of Radiation Oncology, Instituto de Radiomedicina, Santiago (Chile); School of Medicine, Complutense University, Madrid (Spain); Calvo, Felipe A. [School of Medicine, Complutense University, Madrid (Spain); Department of Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); Polo, Alfredo [Service of Radiation Oncology, Hospital Universitario Ramón y Cajal, Madrid (Spain); Cambeiro, Mauricio [Service of Radiation Oncology, Clínica Universidad de Navarra, Pamplona (Spain); Gonzalez, Carmen [School of Medicine, Complutense University, Madrid (Spain); Service of Radiation Oncology, Hospital General Universitario Gregorio Marañón, Madrid (Spain); Desco, Manuel [School of Medicine, Complutense University, Madrid (Spain); Department of Experimental Surgery and Medicine, Hospital General Universitario Gregorio Marañón, Madrid (Spain); Martinez-Monge, Rafael [Service of Radiation Oncology, Clínica Universidad de Navarra, Pamplona (Spain)

    2015-08-01

    Purpose: To assess long-term outcomes and toxicity of intraoperative electron-beam radiation therapy (IOERT) in the management of pediatric patients with Ewing sarcomas (EWS) and rhabdomyosarcomas (RMS). Methods and Materials: Seventy-one sarcoma (EWS n=37, 52%; RMS n=34, 48%) patients underwent IOERT for primary (n=46, 65%) or locally recurrent sarcomas (n=25, 35%) from May 1983 to November 2012. Local control (LC), overall survival (OS), and disease-free survival were estimated using Kaplan-Meier methods. For survival outcomes, potential associations were assessed in univariate and multivariate analyses using the Cox proportional hazards model. Results: After a median follow-up of 72 months (range, 4-310 months), 10-year LC, disease-free survival, and OS was 74%, 57%, and 68%, respectively. In multivariate analysis after adjustment for other covariates, disease status (P=.04 and P=.05) and R1 margin status (P<.01 and P=.04) remained significantly associated with LC and OS. Nine patients (13%) reported severe chronic toxicity events (all grade 3). Conclusions: A multimodal IOERT-containing approach is a well-tolerated component of treatment for pediatric EWS and RMS patients, allowing reduction or substitution of external beam radiation exposure while maintaining high local control rates.

  6. Intraoperative Electron-Beam Radiation Therapy for Pediatric Ewing Sarcomas and Rhabdomyosarcomas: Long-Term Outcomes

    International Nuclear Information System (INIS)

    Purpose: To assess long-term outcomes and toxicity of intraoperative electron-beam radiation therapy (IOERT) in the management of pediatric patients with Ewing sarcomas (EWS) and rhabdomyosarcomas (RMS). Methods and Materials: Seventy-one sarcoma (EWS n=37, 52%; RMS n=34, 48%) patients underwent IOERT for primary (n=46, 65%) or locally recurrent sarcomas (n=25, 35%) from May 1983 to November 2012. Local control (LC), overall survival (OS), and disease-free survival were estimated using Kaplan-Meier methods. For survival outcomes, potential associations were assessed in univariate and multivariate analyses using the Cox proportional hazards model. Results: After a median follow-up of 72 months (range, 4-310 months), 10-year LC, disease-free survival, and OS was 74%, 57%, and 68%, respectively. In multivariate analysis after adjustment for other covariates, disease status (P=.04 and P=.05) and R1 margin status (P<.01 and P=.04) remained significantly associated with LC and OS. Nine patients (13%) reported severe chronic toxicity events (all grade 3). Conclusions: A multimodal IOERT-containing approach is a well-tolerated component of treatment for pediatric EWS and RMS patients, allowing reduction or substitution of external beam radiation exposure while maintaining high local control rates

  7. Pelvic Ewing sarcomas. Three-dimensional conformal vs. intensity-modulated radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Mounessi, F.S.; Lehrich, P.; Haverkamp, U.; Eich, H.T. [Muenster Univ. (Germany). Dept. of Radiation Oncology; Willich, N. [Muenster Univ. (Germany). Dept. of Radiation Oncology; Universitaetsklinikum Muenster (Germany). RiSK - Registry for the Evaluation of Late Side Effects after Radiotherapy in Childhood and Adolescence; Boelling, T. [Center for Radiation Oncology, Osnabrueck (Germany)

    2013-04-15

    The goal of the present work was to assess the potential advantage of intensity-modulated radiotherapy (IMRT) over three-dimensional conformal radiotherapy (3D-CRT) planning in pelvic Ewing's sarcoma. A total of 8 patients with Ewing sarcoma of the pelvis undergoing radiotherapy were analyzed. Plans for 3D-CRT and IMRT were calculated for each patient. Dose coverage of the planning target volume (PTV), conformity and homogeneity indices, as well as further parameters were evaluated. Results The average dose coverage values for PTV were comparable in 3D-CRT and IMRT plans. Both techniques had a PTV coverage of V{sub 95} > 98 % in all patients. Whereas the IMRT plans achieved a higher conformity index compared to the 3D-CRT plans (conformity index 0.79 {+-} 0.12 vs. 0.54 {+-} 0.19, p = 0.012), the dose distribution across the target volumes was less homogeneous with IMRT planning than with 3D-CRT planning. This difference was statistically significant (homogeneity index 0.11 {+-} 0.03 vs. 0.07 {+-} 0.0, p = 0.035). For the bowel, D{sub mean} and D{sub 1%}, as well as V{sub 2} to V{sub 60} were reduced in IMRT plans. For the bladder and the rectum, there was no significant difference in D{sub mean}. However, the percentages of volumes receiving at least doses of 30, 40, 45, and 50 Gy (V{sub 30} to V{sub 50}) were lower for the rectum in IMRT plans. The volume of normal tissue receiving at least 2 Gy (V{sub 2}) was significantly higher in IMRT plans compared with 3D-CRT, whereas at high dose levels (V{sub 30}) it was significantly lower. Compared to 3D-CRT, IMRT showed significantly better results regarding dose conformity (p = 0.012) and bowel sparing at dose levels above 30 Gy (p = 0.012). Thus, dose escalation in the radiotherapy of pelvic Ewing's sarcoma can be more easily achieved using IMRT. (orig.)

  8. The efficacy of low-dose radiation therapy and surgical resection for Ewing's sarcoma

    International Nuclear Information System (INIS)

    Purpose: To evaluate the effect of low-dose radiation therapy and surgery on local control in patients with Ewing's Sarcoma. Materials and Methods: Between 1979 and 1995 24 patients (ages 4-47) with Ewing's sarcoma were treated with combined modality therapy that included multi agent chemotherapy, surgical resection, and radiation therapy. Twelve patients had metastatic disease at the time of presentation (pleura = 3, lung = 5, bone 4). Tumor location was central in 16 and peripheral in 8. Multi agent chemotherapy consisted of vincristine/actinomycin D/cyclophosphamide/doxorubicin in 19. The same agents with the addition of ifosfamide and etoposide were given in 5. The extent of resection was considered to be radical in 14 and of the 16 patients who received preoperative chemotherapy, 5 were found to have a pathologic complete response. All patients with lung metastases at diagnosis received whole-lung irradiation to 1400 cGy in 10 fractions. Radiation therapy to the primary site ranged from 2400 - 3000 cGy although only 4 patients received less than 3000 cGy. Conventional fractionation (180-200) cGy was employed. Results: With a median follow-up of 88 months (range 6 - 182) local control was achieved in 95 % of patients. Local failure occurred in only one patient who had a pathologic partial response to chemotherapy and had undergone gross total resection of a metatarsal primary. Distant failure occurred in 6 of the 12 patients who did not have metastatic disease at presentation. Twelve patients have died of disease, 7 from progression of distant metastases identified at presentation, 4 from distant metastases, and one from local failure followed by distant metastases. The median time to distant failure or progression of initially diagnosed metastatic disease was 28 months. All 5 patients with a pathologic complete response are alive and disease free. Only 4 of 10 patients with a partial response to chemotherapy are alive. Discussion: The margins of resection and

  9. Atypical growth on MRI in a case of Ewing's sarcoma despite lower SUV on PET

    International Nuclear Information System (INIS)

    Ewing's sarcoma is a rare primary bone malignancy of small round blue cells. Treatment typically consists of neoadjuvant chemotherapy, surgical resection, and adjuvant chemotherapy. The disease response to chemotherapy can be followed with fluorodeoxyglucose (FDG) positron emission tomography (PET), which measures the metabolic activity of the tumor, and by magnetic resonance imaging (MRI), which measures tumor size. We present a unique case in which the tumor grew in size following neoadjuvant chemotherapy but decreased in metabolic activity, making it difficult to judge efficacy of the chemotherapy. An atypical response to chemotherapy in this case caused tumor growth due to a fibrotic reaction while viable tumor cells were eradicated. This case highlights the ability of FDG-PET scan to identify the uncommon situation in which a tumor that increased in size may have had a favorable response to chemotherapy. This possibility should be considered in similar cases in which FDG-PET scan shows diminishing metabolic activity despite tumor growth. (orig.)

  10. Proliferation of Ewing sarcoma cell lines is suppressed by the receptor tyrosine kinase inhibitors gefitinib and vandetanib

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    Åman Pierre

    2008-01-01

    Full Text Available Abstract Background Tyrosine kinase inhibitors (TKIs have gained much attention in recent years as targeted agents for the treatment of a wide range of human cancers. We have investigated the effect of the TKIs gefitinib and vandetanib on tumor cell lines derived from Ewing sarcoma, a highly malignant tumor affecting bone and soft tissue in children and young adults. Gefitinib is an inhibitor of epidermal growth factor receptor tyrosine kinase activity (EGFR and vandetanib selectively targets vascular endothelial growth factor receptor-2 (VEGFR-2 with additional activity against VEGFR-3, EGFR and RET kinase receptors. Results Two Ewing sarcoma cell lines investigated showed high levels of nuclear EGFR expression as well as moderate expression in plasma membrane and cytoplasm. When treated with concentrations of 5 μM and more of either gefitinib or vandetanib, we observed a significant decrease in cell proliferation. However, there were no detectable changes in p44/42 MAPK and Akt-1 phosphorylation, or in the expression of cyclin D1 or c-Myc following gefitinib or vandetanib treatment. Conclusion We conclude that Ewing sarcoma tumor cell proliferation is not highly sensitive to inhibition of EGFR signaling alone or the simultaneous inhibition of VEGFR receptors, EGFR and RET kinase. Decreased tumor cell proliferation could be achieved with gefitinib and vandetanib, but only at higher doses where non-specific effects of the compounds may be overriding. As Ewing tumor cells do not seem to depend on EGFR and VEGFR pathways for survival, other key factors in the cellular signaling of Ewing sarcoma should be targeted in order to obtain a potent therapeutic response.

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

    International Nuclear Information System (INIS)

    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

  12. A general aspect on soft-tissue sarcoma and c-kit expression in primitive neuroectodermal tumor and Ewings sarcoma

    International Nuclear Information System (INIS)

    Within soft-tissue sarcoma, primitive neuroectodermal tumors have been shown to cover a wide spectrum of small round cell sarcomas, including Ewings sarcomas (Es) and primitive neuroectodermal tumors (PET). The role of the stem cell factor/kit pathway has been investigated in different human tumors especially in chronic metallically leukemia and gastrointestinal stromal tumor and an autocrine loop has been assumed in small cell lung carcinoma, and recently in Es and PET. Our aim is to investigate the c-kit expression in Es and PET and also to assessed if c-kit has any role in disease process. We thoroughly searched the archives of the Department of Pathology, Faculty of Medicine, Cukurova University Turkey, between 2000 and 2004; and found 14 ES and 14 PNET paraffin embedded tissues. We carried out the detection of the c-kit expression by immunohistochemical staining. The patients median age was 23.7 +/-14.6 (12 male and 16 female). Five were diagnosed as metastatic disease whereas 23 were diagnosed as non-metastatic disease at admission. The mean follow up period was 38.9 +/- 22.3 months. The main localization of the disease was lower extremity (32.1%), and others were as follows: head and neck 25%, thorax and abdomen 14.3%, pelvic and upper extremity 7.1% (11 were localized skeletal and 17 were extraskeletal). According to treatment modalities, 10 were treated with surgery alone, 11 with surgery and chemotherapy, and 7 with surgery, radiation therapy and also with chemotherapy. The primary tumor was lower than 5 cm in its dimension in 21 patients. While in 5 patients, tumor was more than 5 cm but did not exceed 10 cm, it was >10 cm in 2 patients. The c-kit expression was positive in 7 patients both cytoplasmic and membranously, whereas 8 patients were positive cytoplasmically. In 5 PNET patients, c-kit expression were stained immunohistochemically in over 50% and in 3 of ES patients. There was no significant correlation between c-kit expression and gender

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

    OpenAIRE

    Ola Awad; Jason T Yustein; Preeti Shah; Naheed Gul; Varalakshmi Katuri; Alison O'Neill; Yali Kong; Brown, Milton L.; Toretsky, Jeffrey A.; Loeb, David M.

    2010-01-01

    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 h...

  14. A Radiological Review of Ewing's Sarcoma of Mandible: A Case Report with One Year Follow-up

    OpenAIRE

    Krishna, KB Bimal; Thomas, Valsa; Kattoor, Jayasree; Kusumakumari, P

    2013-01-01

    ABSTRACT Ewing's sarcoma (ES) is an uncommon round cell tumor with an aggressive course affecting mainly children and young adults. Only 1% of cases is reported with jaw involvement and have mandibular predilection. Radiographic finding in ES reflect many destructive nature of the lesion, like osteolysis, cortical erosion, periostitis and soft tissue mass. A case of ES of the mandible is reported with special consideration to the radiological appearance. How to cite this article: Krishna KBB,...

  15. Genomic EWS-FLI1 fusion sequences in Ewing sarcoma resemble breakpoint characteristics of immature lymphoid malignancies.

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    Manfred Berger

    Full Text Available Chromosomal translocations between the EWS gene and members of the ETS gene family are characteristic molecular features of the Ewing sarcoma. The most common translocation t(11;22(q24;q12 fuses the EWS gene to FLI1, and is present in 85-90% of Ewing sarcomas. In the present study, a specifically designed multiplex long-range PCR assay was applied to amplify genomic EWS-FLI1 fusion sites from as little as 100 ng template DNA. Characterization of the EWS-FLI1 fusion sites of 42 pediatric and young adult Ewing sarcoma patients and seven cell lines revealed a clustering in the 5' region of the EWS-breakpoint cluster region (BCR, in contrast to random distribution of breakpoints in the FLI1-BCR. No association of breakpoints with various recombination-inducing sequence motifs was identified. The occurrence of small deletions and duplications at the genomic junction is characteristic of involvement of the non-homologous end-joining (NHEJ repair system, similar to findings at chromosomal breakpoints in pediatric leukemia and lymphoma.

  16. Local control and functional results after twice-daily radiotherapy for Ewing's sarcoma of the extremities

    International Nuclear Information System (INIS)

    Purpose: Radiotherapy (RT) has been the predominant local treatment for Ewing's sarcoma of bone at the University of Florida. Twice-daily hyperfractionated RT was initiated in 1982 to improve local control and functional outcome. This retrospective review compares the results of once-daily vs. twice-daily RT in patients with primary Ewing's sarcoma of an extremity, with emphasis on functional outcome. Methods and Materials: Between June 1971 and January 1990, 37 patients were treated at the University of Florida for nonmetastatic Ewing's sarcoma of bone with a primary lesion in an extremity. Three patients underwent amputation. Of 34 patients treated with RT, 31 had RT alone and 3 had a combination of RT and local excision. Before 1982, 14 patients received once-daily RT; since 1982, 17 patients have received twice-daily RT. Doses of once-daily RT varied from 47 to 61 Gy at 1.8-2 Gy per fraction. Doses of twice-daily RT varied, depending on the response of the soft-tissue component of the tumor to chemotherapy, and ranged from 50.4 to 60 Gy at 1.2 Gy per fraction. Some patients in the twice-daily RT group also received total body irradiation 1-3 months after local RT as part of a conditioning regimen before marrow-ablative therapy with stem cell rescue. They received either 8 Gy in two once-daily fractions or 12 Gy in six twice-daily fractions. The six patients who received surgery were excluded from local control analysis. Local control rates were calculated using the Kaplan-Meier (actuarial) method. Fifteen patients had a formal functional evaluation. Results: In the 31 patients treated with RT alone, the actuarial local control rate at 5 years was 81% for patients treated twice daily and 77% for those treated once daily (p = NS). No posttreatment pathologic fractures occurred in patients treated twice daily, whereas five fractures occurred in those treated once daily (p = 0.01). On functional evaluation, less loss in range of motion (15 deg. vs. 28 deg. of loss

  17. The second European interdisciplinary Ewing sarcoma research summit – A joint effort to deconstructing the multiple layers of a complex disease

    Science.gov (United States)

    2016-01-01

    Despite multimodal treatment, long term outcome for patients with Ewing sarcoma is still poor. The second “European interdisciplinary Ewing sarcoma research summit” assembled a large group of scientific experts in the field to discuss their latest unpublished findings on the way to the identification of novel therapeutic targets and strategies. Ewing sarcoma is characterized by a quiet genome with presence of an EWSR1-ETS gene rearrangement as the only and defining genetic aberration. RNA-sequencing of recently described Ewing-like sarcomas with variant translocations identified them as biologically distinct diseases. Various presentations adressed mechanisms of EWS-ETS fusion protein activities with a focus on EWS-FLI1. Data were presented shedding light on the molecular underpinnings of genetic permissiveness to this disease uncovering interaction of EWS-FLI1 with recently discovered susceptibility loci. Epigenetic context as a consequence of the interaction between the oncoprotein, cell type, developmental stage, and tissue microenvironment emerged as dominant theme in the discussion of the molecular pathogenesis and inter- and intra-tumor heterogeneity of Ewing sarcoma, and the difficulty to generate animal models faithfully recapitulating the human disease. The problem of preclinical development of biologically targeted therapeutics was discussed and promising perspectives were offered from the study of novel in vitro models. Finally, it was concluded that in order to facilitate rapid pre-clinical and clinical development of novel therapies in Ewing sarcoma, the community needs a platform to maintain knowledge of unpublished results, systems and models used in drug testing and to continue the open dialogue initiated at the first two Ewing sarcoma summits. PMID:26802024

  18. Characterization of Human Mesenchymal Stem Cells from Ewing Sarcoma Patients. Pathogenetic Implications

    Science.gov (United States)

    Amaral, Ana Teresa; Manara, Maria Cristina; Berghuis, Dagmar; Ordóñez, José Luis; Biscuola, Michele; Lopez-García, Maria Angeles; Osuna, Daniel; Lucarelli, Enrico; Alviano, Francesco; Lankester, Arjan; Scotlandi, Katia; de Álava, Enrique

    2014-01-01

    Background Ewing Sarcoma (EWS) is a mesenchymal-derived tumor that generally arises in bone and soft tissue. Intensive research regarding the pathogenesis of EWS has been insufficient to pinpoint the early events of Ewing sarcomagenesis. However, the Mesenchymal Stem Cell (MSC) is currently accepted as the most probable cell of origin. Materials and Methods In an initial study regarding a deep characterization of MSC obtained specifically from EWS patients (MSC-P), we compared them with MSC derived from healthy donors (MSC-HD) and EWS cell lines. We evaluated the presence of the EWS-FLI1 gene fusion and EWSR1 gene rearrangements in MSC-P. The presence of the EWS transcript was confirmed by q-RT-PCR. In order to determine early events possibly involved in malignant transformation, we used a multiparameter quantitative strategy that included both MSC immunophenotypic negative/positive markers, and EWS intrinsic phenotypical features. Markers CD105, CD90, CD34 and CD45 were confirmed in EWS samples. Results We determined that MSC-P lack the most prevalent gene fusion, EWSR1-FLI1 as well as EWSR1 gene rearrangements. Our study also revealed that MSC-P are more alike to MSC-HD than to EWS cells. Nonetheless, we also observed that EWS cells had a few overlapping features with MSC. As a relevant example, also MSC showed CD99 expression, hallmark of EWS diagnosis. However, we observed that, in contrast to EWS cells, MSC were not sensitive to the inhibition of CD99. Conclusions In conclusion, our results suggest that MSC from EWS patients behave like MSC-HD and are phenotypically different from EWS cells, thus raising important questions regarding MSC role in sarcomagenesis. PMID:24498265

  19. Local control and survival of Ewing's sarcoma in children with radiotherapy and chemotherapy without radical surgery

    International Nuclear Information System (INIS)

    The present study was undertaken in 27 children with localized Ewing's sarcoma treated at our hospital. In 9 cases the lesion was central and in 18 peripheral. Treatment consisted of: 1) Chemotherapy with 6 drugs (vincristine, cyclophosphamide, methotrexate, bleomycin, dactinomycin and doxorubicin) for one year in all patients; 2) Radiotherapy in 24 patients at a dose of 30-40 Gy in the entire bone with a booster dose of 10-15 Gy in the lesion. In 7 patients debulking surgery was performed followed by irradiation. Two cases with tumor in expendable bones and one with extensive destructive lesion in the tibia underwent radical surgery. One patient suffered local recurrence (femur) and 6 developed distant metastases. The remaining 20 (74%) patients were alive and tumor-free after follow-up periods of 17-80 months. Among the 12 patients with extremity lesions who were alive, 7 (58%) had minimal or mild treatment sequelae, 2 moderate, and 3 severe sequelae but no amputation has been required. Our study shows that local control can be achieved in almost all patients without radical surgery and with acceptable extremity function in the majority. (orig.)

  20. Axial Skeletal Location Predicts Poor Outcome in Ewing's Sarcoma: A Single Institution Experience

    Directory of Open Access Journals (Sweden)

    Kurt R. Weiss

    2011-01-01

    Full Text Available Introduction. Ewing's sarcomas (EWSs of bone and soft tissue are neuroectodermal tumors that affect both axial and appendicular locations. We hypothesized that axial location predicted poor outcome in EWS patients. Materials and Methods. Sixty-seven patients (57 with bone EWS and 10 with soft tissue EWS were identified from our database. Thirty-four (51% had axial EWS and 33 (49% had appendicular EWS. Statistical analyses identified predictors of poor outcome. Results and Discussion. Axial location, large size, metastases at presentation, lack of definitive treatment, and positive surgical margins all correlated with poor outcome in univariate analysis. In multivariate analysis, axial location still predicted poor outcome when adjusted for pretreatment variables. Axial location was not statistically predictive of poor outcome when adjusted for treatment variables. Conclusions. Anatomic location has a negative effect on outcome in EWS that cannot be completely explained by pretreatment or treatment factors. Additional studies are required to determine if there is a biologic difference between axial and appendicular EWS.

  1. IGF1 is a common target gene of Ewing's sarcoma fusion proteins in mesenchymal progenitor cells.

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    Luisa Cironi

    Full Text Available BACKGROUND: The EWS-FLI-1 fusion protein is associated with 85-90% of Ewing's sarcoma family tumors (ESFT, the remaining 10-15% of cases expressing chimeric genes encoding EWS or FUS fused to one of several ets transcription factor family members, including ERG-1, FEV, ETV1 and ETV6. ESFT are dependent on insulin-like growth factor-1 (IGF-1 for growth and survival and recent evidence suggests that mesenchymal progenitor/stem cells constitute a candidate ESFT origin. METHODOLOGY/PRINCIPAL FINDINGS: To address the functional relatedness between ESFT-associated fusion proteins, we compared mouse progenitor cell (MPC permissiveness for EWS-FLI-1, EWS-ERG and FUS-ERG expression and assessed the corresponding expression profile changes. Whereas all MPC isolates tested could stably express EWS-FLI-1, only some sustained stable EWS-ERG expression and none could express FUS-ERG for more than 3-5 days. Only 14% and 4% of the total number of genes that were respectively induced and repressed in MPCs by the three fusion proteins were shared. However, all three fusion proteins, but neither FLI-1 nor ERG-1 alone, activated the IGF1 promoter and induced IGF1 expression. CONCLUSION/SIGNIFICANCE: Whereas expression of different ESFT-associated fusion proteins may require distinct cellular microenvironments and induce transcriptome changes of limited similarity, IGF1 induction may provide one common mechanism for their implication in ESFT pathogenesis.

  2. Ewing's sarcoma. Radiographic pattern of healing and bony complications in patients with long-term survival

    International Nuclear Information System (INIS)

    The radiographic appearance of Ewing's sarcoma was studied retrospectively in 22 patients who survived 5 years or longer after diagnosis and treatment. Expected changes from treatment, including regression of the extraosseous soft tissue mass, periostitis, and reconstitution of the cortex, occurred in all patients. Local recurrence occurred in one patient 10 years after complete remission whereas secondary osteosarcoma occurred more than 5 years after complete remission in two other cases. Both recurrent and secondary tumors presented as new lytic foci at the site of the original primary lesion. Lytic changes from radiation (radiation osteitis) may develop more than 2 years after treatment and in this sample; such findings were widely distributed in the radiation port. The authors conclude that bone remodeling and postradiation changes occur slowly over 2 years after treatment, and that any localized lysis at the primary site is suspicious for recurrence or secondary neoplasm. Knowledge of the expected changes and patterns of local recurrence and secondary neoplasms helps one to detect any significant change in its early phase

  3. Comprehensive analysis of published phase I/II clinical trials between 1990-2010 in osteosarcoma and Ewing sarcoma confirms limited outcomes and need for translational investment

    Directory of Open Access Journals (Sweden)

    van Maldegem Annemiek M

    2012-01-01

    Full Text Available Abstract Background High grade primary bone sarcomas are rare cancers that affect mostly children and young adults. Osteosarcoma and Ewing sarcoma are the most common histological subtypes in this age group, with current multimodality treatment strategies achieving 55-70% overall survival. As there remains an urgent need to develop new therapeutic interventions, we have reviewed published phase I/II trials that have been reported for osteosarcoma and Ewing sarcoma in the last twenty years. Results We conducted a literature search for clinical trials between 1990 and 2010, either for trials enrolling bone sarcoma patients as part of a general sarcoma indication or trials specifically in osteosarcoma and Ewing sarcoma. We identified 42 clinical trials that fulfilled our search criteria for general sarcoma that enrolled these patient groups, and eight and twenty specific trials for Ewing and osteosarcoma patients, respectively. For the phase I trials which enrolled different tumour types our results were incomplete, because the sarcoma patients were not mentioned in the PubMed abstract. A total of 3,736 sarcoma patients were included in these trials over this period, 1,114 for osteosarcoma and 1,263 for Ewing sarcoma. As a proportion of the worldwide disease burden over this period, these numbers reflect a very small percentage of the potential patient recruitment, approximately 0.6% for Ewing sarcoma and 0.2% for osteosarcoma. However, these data show an increase in recent activity overall and suggest there is still much room for improvement in the current trial development structures. Conclusion Lack of resources and commercial investment will inevitably limit opportunity to develop sufficiently rapid improvements in clinical outcomes. International collaboration exists in many well founded co-operative groups for phase III trials, but progress may be more effective if there were also more investment of molecular and translational research into

  4. Localization of genetic elements of intact and derivative chromosome 11 and 22 territories in nuclei of Ewing sarcoma cells

    Czech Academy of Sciences Publication Activity Database

    Taslerová, R.; Kozubek, Stanislav; Bártová, Eva; Jirsová, Pavla; Kodet, R.; Kozubek, M.

    2006-01-01

    Roč. 155, č. 3 (2006), s. 493-504. ISSN 1047-8477 R&D Projects: GA AV ČR(CZ) 1QS500040508; GA ČR(CZ) GA202/04/0907; GA MZd(CZ) 1A8241; GA AV ČR(CZ) IAA5004306; GA MŠk(CZ) LC535 Institutional research plan: CEZ:AV0Z50040507 Keywords : chromatin structure * chromosome territory * Ewing sarcoma Subject RIV: BO - Biophysics Impact factor: 3.496, year: 2006

  5. CT and Positron Emission Tomography/CT Findings of Mediastinal Extraskeletal Ewing's Sarcoma with Extensive Distant Metastasis

    International Nuclear Information System (INIS)

    Extraskeletal Ewing's sarcoma (EES) is a rare aggressive malignant tumor, usually occurring in the chest wall of children and young adults. Although EES shows aggressive growth pattern, it seldom demonstrates distant metastasis at diagnosis. Herein, we present computed tomography (CT) and positron emission tomography computed tomography (PET/CT) findings of EES in anterior mediastinum in a 68-year-old man, showing multi-organ distant metastasis at diagnosis. It is another atypical case with unusual presentation in point of old age, mediastinal location and distant metastasis at diagnosis, showing PET/CT findings.

  6. Safety considerations for transplanting cryopreserved ovarian tissue to restore fertility in female patients who have recovered from Ewing's sarcoma

    DEFF Research Database (Denmark)

    Sørensen, Stine D; Greve, Tine; Wielenga, Vera Timmermans;

    2014-01-01

    Ewing's sarcoma (EWS) is a highly malignant cancer in children, adolescents and young adults. The chemotherapy required to treat female EWS patients may cause primary ovarian insufficiency and infertility as a side effect. Cryopreservation of ovarian tissue before the start of chemotherapy can...... potentially preserve fertility. When the patient has been cured and primary ovarian insufficiency has developed, transplantation of frozen/thawed ovarian tissue can restore ovarian function. The tissue is usually collected before chemotherapy is initiated, and malignant cells may contaminate the stored...... EWS patients and presents a new case of malignant cells in an ovarian biopsy from a girl with EWS....

  7. Primary Ewing's sarcoma/primitive neuroectodermal tumor of the urogenital tract in children

    Institute of Scientific and Technical Information of China (English)

    SONG Hong-cheng; SUN Ning; ZHANG Wei-ping; HUANG Cheng-ru

    2012-01-01

    Background Primary Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of urogenital tract is a rare condition with non-specific clinical presentations,which can make it difficult to diagnose.In this study,we summarize the clinical presentation,pathological features,therapeutic strategies,and prognosis of ES/PNET.Methods Clinical information on two cases of ES/PNET in the penis and ureter was analyzed,and relevant literature was reviewed.Results ES/PNET was confirmed pathologically,immunohistochemically and via molecular biology techniques in the penis (n=1) and ureter (n=1).In one case,a tumor was found at the base of penis,which had invaded the corpus cavernosum,and resulted in a massive enlargement of the penis.This tumor was initially diagnosed as an endocrine disorder.However,a confirmed diagnosis was made 11 months later when massive metastases in both lungs were noted.A tumor biopsy was performed to confirm the diagnosis,and chemotherapy with a CAV (cyclophosphamide+doxorubicin+vincristine) + IE (ifosfamide+ etoposide) regimen for 9 months was prescribed.In the second case,a child was admitted due to abdominal pain and a hydroureter in the right kidney,as determined by ultrasonography.A tumor was found in the right ureter at the level of iiiac vessels.Removal of the tumor and ureteral anastomosis were performed,and chemotherapy with CAV+IE for 8 months were prescribed.Both patients are currently being followed-up closely.Conclusions ES/PNET is a highly malignant tumor and has poor prognosis.Pre-operative diagnosis of ES/PNET of urogenital tract is difficult and largely depends on pathology,immunohistochemistry,and,if applicable,molecular biology.Comprehensive therapy may include surgery,chemotherapy and radiotherapy.

  8. Ewing sarcoma of the bone in children under 6 years of age.

    Directory of Open Access Journals (Sweden)

    Maria Antonietta De Ioris

    Full Text Available BACKGROUND: Ewing Sarcoma Family Tumours (ESFT are rare in early childhood. The aim of this study was to report the clinical characteristics and outcome of children under 6 years of age affected by ESFT of the bone in Italy. METHODS: The records of all the children diagnosed with osseous ESFT in centres members of the Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP from 1990 to 2008 were reviewed. The Kaplan-Meier method was used for estimating overall and progression-free survival (OS, PFS curves; multivariate analyses were performed using Cox proportional hazards regression model. RESULTS: This study includes 62 patients. An axial primary localization was present in 66% of patients, with the primary site in the chest wall in 34%. Fourteen (23% patients presented metastatic disease. The 5-year OS and PFS were 73% (95% confidence interval, CI, 58-83% and 72% (95% CI 57-83% for patients with localized disease and 38% (95% CI 17-60% and 21% (95% CI 5-45% for patients with metastatic disease. Metastatic spread, skull/pelvis/spine primary localization, progression during treatment and no surgery predicted worse survival (P<0.01, while patients treated in the last decade had better survival (P = 0.002. In fact, the 5-year OS and PFS for patients diagnosed in the period 2000-2008 were 89% (95% CI 71-96% and 86% (95% CI 66-94%, respectively. CONCLUSION: The axial localization is the most common site of ESFT in pre-scholar children. Patients treated in the most recent period have an excellent outcome.

  9. Whole Lung Irradiation for Adults With Pulmonary Metastases From Ewing Sarcoma

    International Nuclear Information System (INIS)

    Purpose: To evaluate feasibility and patterns of failure in adult patients with Ewing sarcoma (ES) treated with whole lung irradiation (WLI) for pulmonary metastases. Methods and Materials: Retrospective review of all ES patients treated at age 18 or older with 12-15 Gy WLI for pulmonary metastases at a single institution between 1990 and 2014. Twenty-six patients met the study criteria. Results: The median age at WLI was 23 years (range, 18-40). The median follow-up time of the surviving patients was 3.8 years (range, 1.0-9.6). The 3-year cumulative incidence of pulmonary relapse (PR) was 55%, with a 3-year cumulative incidence of PR as the site of first relapse of 42%. The 3-year event-free survival (EFS) and overall survival (OS) were 38 and 45%, respectively. Patients with exclusively pulmonary metastases had better outcomes than did those with extrapulmonary metastases: the 3-year PR was 45% in those with exclusively lung metastases versus 76% in those with extrapulmonary metastases (P=.01); the 3-year EFS was 49% versus 14% (P=.003); and the 3-year OS was 61% versus 13% (P=.009). Smoking status was a significant prognostic factor for EFS: the 3-year EFS was 61% in nonsmokers versus 11% in smokers (P=.04). Two patients experienced herpes zoster in the radiation field 6 and 12 weeks after radiation. No patients experienced pneumonitis or cardiac toxicity, and no significant acute or late sequelae were observed among the survivors. Conclusion: WLI in adult patients with ES and lung metastases is well tolerated and is associated with freedom from PR of 45% at 3 years. Given its acceptable toxicity and potential therapeutic effect, WLI for pulmonary metastases in ES should be considered for adults, as it is in pediatric patients. All patients should be advised to quit smoking before receiving WLI

  10. Severe gonadotoxic insult manifests early in young girls treated for Ewing sarcoma.

    Science.gov (United States)

    Mörse, Helena; Elfving, Maria; Turkiewicz, Aleksandra; Andersen, Claus Yding; Øra, Ingrid

    2016-08-01

    We prospectively investigated anti-Müllerian hormone (AMH) as a measure of ovarian insult in young females during and after treatment for Wilms tumor (WT), osteosarcoma (OS), and Ewing sarcoma (ES).Twenty-one female childhood cancer patients, with a mean age of 7.9 years (range 0.6-17), entered the study. Levels of AMH, follicle-stimulating hormone (FSH), and luteinizing hormone were monitored at diagnosis and every 3 to 4 months during, and regularly for a mean of 2.6 years after treatment.A profound decline in AMH was seen in the majority of the 21 study patients 3 to 4 months after the beginning of treatment, the exception being patients with WT, of whom 60% showed no such decline. During the remaining treatment, all patients except those with WT not treated with whole abdominal radiotherapy or stem cell transplantation (SCT) had AMH below detection limit.After completion of treatment, patients with OS and WT (without whole abdominal radiotherapy and SCT) recovered in AMH and had FSH in the normal range. In contrast, ES patients showed no AMH recovery and highly fluctuating FSH in the first years of follow-up, except for the 2 youngest patients, who had a late, slow AMH recovery.In conclusion, young female ES patients already showed signs of severe ovarian dysfunction during the first years after cancer treatment similar to patients treated with SCT and abdominal radiotherapy, in contrast to females with WT and OS. Fertility counseling and information concerning fertility preservation procedures should be considered before starting to treat young females with ES. PMID:27537574

  11. Somatic mutations of the mitochondrial genome in Chinese patients with Ewing sarcoma.

    Science.gov (United States)

    Yu, Man; Wan, Yanfang; Zou, Qinghua

    2013-07-01

    Somatic mutations in mitochondrial DNA (mtDNA) have been long proposed to drive initiation and progression of human malignancies. Our previous study revealed a high prevalence of somatic mutations in the D-loop region of mtDNA in Ewing sarcoma (EWS). However, it is unclear whether somatic mutations also occur in the coding regions of mtDNA in EWS. To test this possibility, in the present study, we sequenced the whole mitochondrial genome from 20 cases of EWS specimens and their corresponding peripheral blood samples. We identified a total of 6 somatic mutations in the mtDNA coding regions in our EWS series, and 5 of them were missense or frame-shift mutations that have the potential to directly influence proper mitochondrial function. In combination with our earlier observations on the D-loop fragment, 70% (14/20) of EWS tissues appeared to harbor somatic mtDNA mutations. Among the identified 25 somatic mutations, 19 (76%) were located in the D-loop control region, 1 (4%) was in the sequence of the tRNA(Val) gene, 1 (4%) was in the mitochondrial ATP synthase subunit 6 gene, and 4 (16%) occurred in genes encoding components of the mitochondrial respiratory complexes. In addition, patients carrying somatic mtDNA mutations did not show significant association with their clinicopathologic characteristics. Together, these findings suggest that somatic mtDNA mutations occur in both protein coding and noncoding regions of mtDNA, which may play critical roles in the pathogenesis of EWS and should be further explored for its possible use as a novel marker for monitoring EWS occurrence and advancement. PMID:23375644

  12. Myeloablative therapy against high risk Ewings sarcoma: A single institution experience and literature review

    International Nuclear Information System (INIS)

    Background: Attempts to improve survival outcomes of patients with high risk Ewings 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. (authors)

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

    Directory of Open Access Journals (Sweden)

    William A May

    Full Text Available 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.

  14. The association between let-7, RAS and HIF-1α in Ewing Sarcoma tumor growth.

    Science.gov (United States)

    Hameiri-Grossman, Michal; Porat-Klein, Adi; Yaniv, Isaac; Ash, Shifra; Cohen, Ian J; Kodman, Yona; Haklai, Ronit; Elad-Sfadia, Galit; Kloog, Yoel; Chepurko, Elena; Feinmesser, Meora; Issakov, Josephine; Sher, Osnat; Luria, Drorit; Kollender, Yehuda; Weizman, Avraham; Avigad, Smadar

    2015-10-20

    Ewing Sarcoma (ES) is the second most common primary malignant bone tumor in children and adolescents. microRNAs (miRNAs) are involved in cancer as tumor suppressors or oncogenes. We studied the involvement of miRNAs located on chromosomes 11q and 22q that participate in the most common translocation in ES. Of these, we focused on 3 that belong to the let-7 family.We studied the expression levels of let-7a, and let-7b and detected a significant correlation between low expression of let-7b and increased risk of relapse. let-7 is known to be a negative regulator of the RAS oncogene. Indeed, we detected an inverse association between the expression of let-7 and RAS protein levels and its downstream target p-ERK, following transfection of let-7 mimics and inhibitors. Furthermore, we identified let-7 as a negative regulator of HIF-1α and EWS-FLI-1. Moreover, we were able to show that HIF-1α directly binds to the EWS-FLI-1 promoter. Salirasib treatment in-vitro resulted in the reduction of cell viability, migration ability, and in the decrease of cells in S-phase. A significant reduction in tumor burden and in the expression levels of both HIF-1α and EWS-FLI-1 proteins were observed in mice after treatment.Our results support the hypothesis that let-7 is a tumor suppressor that negatively regulates RAS, also in ES, and that HIF-1α may contribute to the aggressive metastatic behavior of ES. Moreover, the reduction in the tumor burden in a mouse model of ES following Salirasib treatment, suggests therapeutic potential for this RAS inhibitor in ES. PMID:26393682

  15. Mechanisms of transcriptional repression by EWS-FLl1 in Ewing Sarcoma

    International Nuclear Information System (INIS)

    The EWS-FLI1 chimeric oncoprotein characterizing Ewing Sarcoma (ES) is a prototypic aberrant ETS transcription factor with activating and repressive gene regulatory functions. Mechanisms of transcriptional regulation, especially transcriptional repression by EWS-FLI1, are poorly understood. We report that EWS-FLI1 repressed promoters are enriched in forkhead box recognition motifs, and identify FOXO1 as a EWS-FLI1 suppressed master regulator responsible for a significant subset of EWS-FLI1 repressed genes. In addition to transcriptional FOXO1 regulation by direct promoter binding of EWS-FLI1, its subcellular localization and activity is regulated by CDK2 and AKT mediated phosphorylation downstream of EWS-FLI1. Functional 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. Treatment of ES cell lines with Methylseleninic acid (MSA) evoked reactivation of endogenous FOXO1 in the presence of EWS-FLI1 in a dose- and time-dependent manner and induced massive cell death which was found to be partially FOXO1-dependent. In an orthotopic xenograft mouse model, MSA increased FOXO1 expression in the tumor paralleled by a significant decrease in ES tumor growth. Together, these data suggest that a repressive sub-signature of EWS-FLI1 repressed genes precipitates suppression of FOXO1. FOXO1 re-activation by small molecules may therefore constitute a novel therapeutic strategy in the treatment of ES. (author)

  16. Molecular localization of the t(11;22)(q24;q12) translocation of Ewing sarcoma by chromosomal in situ suppression hybridization

    International Nuclear Information System (INIS)

    Chromosome translocations are associated with a variety of human leukemias, lymphomas, and solid tumors. To localize molecular markers flanking the t(11;22)(q24;q12) breakpoint that occurs in virtually all cases of Ewing sarcoma and peripheral neuroepithelioma, high-resolution chromosomal in situ suppression hybridization was carried out using a panel of cosmid clones localized and ordered on chromosome 11q. The location of the Ewing sarcoma translocation breakpoint was determined relative to the nearest two cosmid markers on 11q, clones 23.2 and 5.8, through the analysis of metaphase chromosome hybridization. By in situ hybridization to interphase nuclei, the approximate physical separation of these two markers was determined. In both Ewing sarcoma and peripheral neuroepithelioma, cosmid clone 5.8 is translocated from chromosome 11q24 to the derivative chromosome 22 and a portion of chromosome 22q12 carrying the leukemia inhibitory factor gene is translocated to the derivative chromosome 11. The physical distance between the flanking cosmid markers on chromosome 11 was determined to be in the range of 1,000 kilobases, and genomic analysis using pulsed-field gel electrophoresis showed no abnormalities over a region of 650 kilobases in the vicinity of the leukemia inhibitory factor gene on chromosome 22. This approach localizes the Ewing sarcoma breakpoint to a small region on chromosome 11q24 and provides a rapid and precise technique for the molecular characterization of chromosomal aberrations

  17. Extraosseal Ewing sarcoma as a rare cause of the blueberry muffin baby syndrome: A case report and the review of the literature

    Czech Academy of Sciences Publication Activity Database

    Křenová, Z.; Křen, L.; Blatný, J.; Falk, Martin; Kazakov, D.; Grossmann, P.; Shimada, H.; Štěrba, J.

    2011-01-01

    Roč. 33, č. 7 (2011), s. 733-735. ISSN 0193-1091 Grant ostatní: GA MŠk(CZ) LM2010004 Institutional research plan: CEZ:AV0Z50040507; CEZ:AV0Z50040702 Keywords : blueberry muffin baby syndrome * Ewing sarcoma * fluorescence in situ hybridization (FISH) Subject RIV: BO - Biophysics Impact factor: 1.197, year: 2011

  18. LGR5 is expressed by Ewing sarcoma and potentiates Wnt/β-catenin signaling

    Directory of Open Access Journals (Sweden)

    ElizabethRLawlor

    2013-04-01

    Full Text Available Ewing sarcoma (ES is an aggressive bone and soft tissue tumor of putative stem cell origin that predominantly occurs in children and young adults. Although most patients with localized ES can be cured with intensive therapy, the clinical course is variable and up to one third of patients relapse following initial remission. Unfortunately, little is yet known about the biologic features that distinguish low-risk from high-risk disease or the mechanisms of ES disease progression. Recent reports have suggested that putative cancer stem cells exist in ES and may contribute to an aggressive phenotype. The cell surface receptor LGR5 (leucine-rich repeat-containing G-protein coupled receptor 5 is a somatic stem cell marker that functions as an oncogene in several human cancers, most notably colorectal carcinoma. LGR5 is a receptor for the R-spondin (RSPO family of ligands and RSPO-mediated activation of LGR5 potentiates Wnt/β-catenin signaling, contributing to stem cell proliferation and self-renewal. Given its presumed stem cell origin, we investigated whether LGR5 contributes to ES pathogenesis. We found that LGR5 is expressed by ES and that its expression is relatively increased in cells and tumors that display a more aggressive phenotype. In particular, LGR5 expression was increased in putative cancer stem cells. We also found that neural crest-derived stem cells express LGR5, raising the possibility that expression of LGR5 may be a feature of ES cells of origin. LGR5-high ES cells showed nuclear localization of ß-catenin and robust activation of TCF-reporter activity when exposed to Wnt ligand and this was potentiated by RSPO. However, modulation of LGR5 or exposure to RSPO had no impact on proliferation confirming that Wnt/ β-catenin signaling in ES cells does not recapitulate signaling in epithelial cells. Together these studies show that the RSPO-LGR5-WNT- β-catenin axis is present and active in ES and may contribute to tumor pathogenesis.

  19. [Cytogenetics of bone sarcomas].

    Science.gov (United States)

    Vagner-Capodano, A M; Poitout, D

    There has been much progress in the cytogenesis, and molecular biology of bone tumours such as Ewing sarcoma and osteosarcomas, greatly improving diagnostic possibilities and prognosis. Ewing's sarcoma is an indifferentiated sarcoma with round cells which usually occurs in children or adolescents. Ewing's sarcoma corresponds to 6% of all bone tumours. Histologically Ewing's sarcoma belongs to a group of small round cell tumours including neuroblastoma, embryon and alveolar rhabdomyosarcoma and non-Hodgkin's lymphoma. Differential diagnosis is difficult. Cytogenetic examinations can now differentiate Ewing's sarcoma from other small round cell tumours. There is a specific 11:12 translocation (q24; q12) which can be used as a marker. PMID:8785922

  20. Histone deacetylase inhibitors enhance expression of NKG2D ligands in Ewing sarcoma and sensitize for natural killer cell-mediated cytolysis

    Directory of Open Access Journals (Sweden)

    Berghuis Dagmar

    2012-02-01

    Full Text Available Abstract Background Ewing sarcoma patients have a poor prognosis despite multimodal therapy. Integration of combination immunotherapeutic strategies into first-/second-line regimens represents promising treatment options, particularly for patients with intrinsic or acquired resistance to conventional therapies. We evaluated the susceptibility of Ewing sarcoma to natural killer cell-based combination immunotherapy, by assessing the capacity of histone deacetylase inhibitors to improve immune recognition and sensitize for natural killer cell cytotoxicity. Methods Using flow cytometry, ELISA and immunohistochemistry, expression of natural killer cell receptor ligands was assessed in chemotherapy-sensitive/-resistant Ewing sarcoma cell lines, plasma and tumours. Natural killer cell cytotoxicity was evaluated in Chromium release assays. Using ATM/ATR inhibitor caffeine, the contribution of the DNA damage response pathway to histone deacetylase inhibitor-induced ligand expression was assessed. Results Despite comparable expression of natural killer cell receptor ligands, chemotherapy-resistant Ewing sarcoma exhibited reduced susceptibility to resting natural killer cells. Interleukin-15-activation of natural killer cells overcame this reduced sensitivity. Histone deacetylase inhibitor-pretreatment induced NKG2D-ligand expression in an ATM/ATR-dependent manner and sensitized for NKG2D-dependent cytotoxicity (2/4 cell lines. NKG2D-ligands were expressed in vivo, regardless of chemotherapy-response and disease stage. Soluble NKG2D-ligand plasma concentrations did not differ between patients and controls. Conclusion Our data provide a rationale for combination immunotherapy involving immune effector and target cell manipulation in first-/second-line treatment regimens for Ewing sarcoma.

  1. FOXO1 is a direct target of EWS-Fli1 oncogenic fusion protein in Ewing's sarcoma cells

    International Nuclear Information System (INIS)

    Research highlights: → Inducible and reversible siRNA knockdown of an oncogenic fusion protein such as EWS-Fli1 is feasible and more advantageous than other siRNA methods. → The tumor suppressor gene FOXO1 is a new EWS-Fli1 target. → While trans-activators are known for the FOXO1 gene, there has been no report on negative regulators of FOXO1 transcription. → This study provides first evidence that the EWS-Fli1 oncogenic fusion protein can function as a transcriptional repressor of the FOXO1 gene. -- Abstract: Ewing's family tumors are characterized by a specific t(11;22) chromosomal translocation that results in the formation of EWS-Fli1 oncogenic fusion protein. To investigate the effects of EWS-Fli1 on gene expression, we carried out DNA microarray analysis after specific knockdown of EWS-Fli1 through transfection of synthetic siRNAs. EWS-Fli1 knockdown increased expression of genes such as DKK1 and p57 that are known to be repressed by EWS-Fli1 fusion protein. Among other potential EWS-Fli1 targets identified by our microarray analysis, we have focused on the FOXO1 gene since it encodes a potential tumor suppressor and has not been previously reported in Ewing's cells. To better understand how EWS-Fli1 affects FOXO1 expression, we have established a doxycycline-inducible siRNA system to achieve stable and reversible knockdown of EWS-Fli1 in Ewing's sarcoma cells. Here we show that FOXO1 expression in Ewing's cells has an inverse relationship with EWS-Fli1 protein level, and FOXO1 promoter activity is increased after doxycycline-induced EWS-Fli1 knockdown. In addition, we have found that direct binding of EWS-Fli1 to FOXO1 promoter is attenuated after doxycycline-induced siRNA knockdown of the fusion protein. Together, these results suggest that suppression of FOXO1 function by EWS-Fli1 fusion protein may contribute to cellular transformation in Ewing's family tumors.

  2. A rare case of extraskeletal Ewing's sarcoma/primitive neuroectodermal tumor developing in maxillary sinus of an old patient.

    Science.gov (United States)

    Kulkarni, Maithili Mandar; Khandeparkar, Siddhi Gaurish Sinai; Joshi, Avinash R; Barpande, Chitrangi

    2016-01-01

    Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) family of tumors is an uncommon group of malignant neoplasms that may present in both skeletal and extraskeletal sites. PNET outside the central nervous system is called peripheral PNET (pPNET) developing from migrating embryonal cells of the neural crest. Very few cases of pPNET of the maxilla are reported in English literature. These tumors may be difficult to diagnose due to their primitive morphology. These tumors occur predominantly in infancy or early childhood. The occurrence of extraskeletal ES/PNET in the maxillary sinus in an old age is very rare. We report a case of extraskeletal ES/PNET developing in maxillary sinus in a 60-year-old woman. The ES/PNET should be included in the differential diagnosis of a small round cell tumor and immunohistochemical analysis with a panel of immunomarkers should be done for correct diagnosis and proper treatment. PMID:27601837

  3. Case Report: Intracranial peripheral primitive neuroectodermal tumor – Ewing's sarcoma of dura with transcalvarial–subgaleal extension: An unusual radiological presentation

    International Nuclear Information System (INIS)

    The occurrence of the intracranial, peripheral, primitive, neuroectodermal tumor, Ewing's sarcoma (pPNET-ES) of the dura, is very rare. Immunophenotypical as well as genetic analyses play key roles in its diagnosis and differentiation from central PNET. We describe here the CT scan and MRI findings of an interesting case of intracranial pPNET-ES arising from the anterior falx cerebri with a trancalvarial–subgaleal extension

  4. Gene Expression Profiling of Ewing Sarcoma Tumors Reveals the Prognostic Importance of Tumor-Stromal Interactions: A Report from the Children’s Oncology Group

    OpenAIRE

    Volchenboum, Samuel L.; Andrade, Jorge; Huang, Lei; Barkauskas, Donald A; Krailo, Mark; Womer, Richard B.; Ranft, Andreas; Potratz, Jenny; Dirksen, Uta; Triche, Timothy J.; Lawlor, Elizabeth R.

    2015-01-01

    Relapse of Ewing sarcoma (ES) can occur months or years after initial remission and salvage therapy for relapsed disease is usually ineffective. Thus, there is great need to develop biomarkers that can predict which patients are at risk for relapse so that therapy and post-therapy evaluation can be adjusted accordingly. For the current study we performed whole genome expression profiling on two independent cohorts of clinically annotated ES tumors in an effort to identify and validate prognos...

  5. Prognostic Factors and Patterns of Relapse in Ewing Sarcoma Patients Treated With Chemotherapy and R0 Resection

    International Nuclear Information System (INIS)

    Purpose: To identify prognostic factors and patterns of relapse for patients with Ewing sarcoma who underwent chemotherapy and R0 resection without radiation therapy (RT). Methods and Materials: We reviewed the medical records of patients who underwent surgical resection at our institution between 2000 and 2013 for an initial diagnosis of Ewing sarcoma. The associations of demographic and clinical factors with local control (LC) and patient outcome were determined by Cox regression. Time to events was measured from the time of surgery. Survival curves were estimated by the Kaplan-Meier method and compared by the log-rank test. Results: A total of 66 patients (median age 19 years, range 4-55 years) met the study criteria. The median follow-up was 5.6 years for living patients. In 43 patients (65%) for whom imaging studies were available, the median tumor volume reduction was 73%, and at least partial response by Response Evaluation Criteria in Solid Tumors was achieved in 17 patients (40%). At 5 years, LC was 78%, progression-free survival (PFS) was 59%, and overall survival (OS) was 65%. Poor histologic response (necrosis ≤95%) was an independent predictor of LC (hazard ratio [HR] 6.8, P=.004), PFS (HR 5.2, P=.008), and OS (HR 5.0, P=.008). Metastasis on presentation was also an independent predictor of LC (HR 6.3, P=.011), PFS (HR 6.8, P=.002), and OS (HR 6.7, P=.002). Radiologic partial response was a predictor of PFS (HR 0.26, P=.012), and postchemotherapy tumor volume was associated with OS (HR 1.06, P=.015). All deaths were preceded by distant relapse. Of the 8 initial local-only relapses, 5 (63%) were soon followed by distant relapse. Predictors of poor postrecurrence survival were time to recurrence <1 year (HR 11.5, P=.002) and simultaneous local and distant relapse (HR 16.8, P=.001). Conclusions: Histologic and radiologic response to chemotherapy were independent predictors of outcome. Additional study is needed to determine the role of adjuvant

  6. Prognostic Factors and Patterns of Relapse in Ewing Sarcoma Patients Treated With Chemotherapy and R0 Resection

    Energy Technology Data Exchange (ETDEWEB)

    Pan, Hubert Y. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Morani, Ajaykumar [Department of Radiology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Wang, Wei-Lien [Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Hess, Kenneth R. [Department of Biostatistics, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Paulino, Arnold C. [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Ludwig, Joseph A. [Department of Medical Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Lin, Patrick P. [Department of Orthopedic Surgery, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Daw, Najat C. [Department of Pediatrics, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States); Mahajan, Anita, E-mail: amahajan@mdanderson.org [Department of Radiation Oncology, The University of Texas MD Anderson Cancer Center, Houston, Texas (United States)

    2015-06-01

    Purpose: To identify prognostic factors and patterns of relapse for patients with Ewing sarcoma who underwent chemotherapy and R0 resection without radiation therapy (RT). Methods and Materials: We reviewed the medical records of patients who underwent surgical resection at our institution between 2000 and 2013 for an initial diagnosis of Ewing sarcoma. The associations of demographic and clinical factors with local control (LC) and patient outcome were determined by Cox regression. Time to events was measured from the time of surgery. Survival curves were estimated by the Kaplan-Meier method and compared by the log-rank test. Results: A total of 66 patients (median age 19 years, range 4-55 years) met the study criteria. The median follow-up was 5.6 years for living patients. In 43 patients (65%) for whom imaging studies were available, the median tumor volume reduction was 73%, and at least partial response by Response Evaluation Criteria in Solid Tumors was achieved in 17 patients (40%). At 5 years, LC was 78%, progression-free survival (PFS) was 59%, and overall survival (OS) was 65%. Poor histologic response (necrosis ≤95%) was an independent predictor of LC (hazard ratio [HR] 6.8, P=.004), PFS (HR 5.2, P=.008), and OS (HR 5.0, P=.008). Metastasis on presentation was also an independent predictor of LC (HR 6.3, P=.011), PFS (HR 6.8, P=.002), and OS (HR 6.7, P=.002). Radiologic partial response was a predictor of PFS (HR 0.26, P=.012), and postchemotherapy tumor volume was associated with OS (HR 1.06, P=.015). All deaths were preceded by distant relapse. Of the 8 initial local-only relapses, 5 (63%) were soon followed by distant relapse. Predictors of poor postrecurrence survival were time to recurrence <1 year (HR 11.5, P=.002) and simultaneous local and distant relapse (HR 16.8, P=.001). Conclusions: Histologic and radiologic response to chemotherapy were independent predictors of outcome. Additional study is needed to determine the role of adjuvant

  7. Englerin A Inhibits EWS-FLI1 DNA Binding in Ewing Sarcoma Cells.

    Science.gov (United States)

    Caropreso, Vittorio; Darvishi, Emad; Turbyville, Thomas J; Ratnayake, Ranjala; Grohar, Patrick J; McMahon, James B; Woldemichael, Girma M

    2016-05-01

    High-throughput screening of extracts from plants, marine, and micro-organisms led to the identification of the extract from the plant Phyllanthus engleri as the most potent inhibitor of EWS-FLI1 induced luciferase reporter expression. Testing of compounds isolated from this extract in turn led to the identification of Englerin A (EA) as the active constituent of the extract. EA induced both necrosis and apoptosis in Ewing cells subsequent to a G2M accumulation of cells in the cell cycle. It also impacted clonogenic survival and anchorage-independent proliferation while also decreasing the proportion of chemotherapy-resistant cells identified by high ALDH activity. EA also caused a sustained increase in cytosolic calcium levels. EA appears to exert its effect on Ewing cells through a decrease in phosphorylation of EWS-FLI1 and its ability to bind DNA. This effect is mediated, at least in part, through a decrease in the levels of the calcium-dependent protein kinase PKC-βI after a transient up-regulation. PMID:26961871

  8. Correlation of non-mass-like abnormal MR signal intensity with pathological findings surrounding pediatric osteosarcoma and Ewing's sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Masrouha, Karim Z.; Haidar, Rachid; Saghieh, Said [American University of Beirut Medical Center, Department of Surgery, Beirut (Lebanon); Musallam, Khaled M. [American University of Beirut Medical Center, Internal Medicine Division of Hematology and Oncology, Beirut (Lebanon); Samra, Alexis Bou; Tawil, Ayman; Chakhachiro, Zaher [American University of Beirut Medical Center, Pathology, Beirut (Lebanon); Abdallah, Abeer; Khoury, Nabil J. [American University of Beirut Medical Center, Diagnostic Radiology, Beirut (Lebanon); Saab, Raya; Muwakkit, Samar; Abboud, Miguel R. [American University of Beirut Medical Center, Children' s Cancer Center of Lebanon, Beirut (Lebanon)

    2012-11-15

    The aim of this work was to determine the role of MRI in interpreting abnormal signals within bones and soft tissues adjacent to tumor bulk of osteosarcoma and Ewing's sarcoma in a pediatric population by correlating MR findings with histopathology. Thirty patients met the inclusion criteria, which included (1) osteosarcoma or Ewing's sarcoma, (2) MR studies no more than 2 months prior to surgery, (3) presence of abnormal MR signal surrounding the tumor bulk, (4) pathological material from resected tumor. The patients received standard neoadjuvant chemotherapy. Using grid maps on gross pathology specimens, the abnormal MR areas around the tumor were matched with the corresponding grid sections. Histopathology slides of these sections were then analyzed to determine the nature of the regions of interest. The MR/pathological correlation was evaluated using Mann-Whitney U test and Fisher's exact test. Twenty-seven patients had osteosarcoma and three patients had Ewing's sarcoma. Of the studied areas, 17.4% were positive for tumor (viable or necrotic). There was no statistically significant correlation between areas positive for tumor and age, gender, signal extent and intensity on MRI, or tissue type. There was, however, a statistically significant correlation between presence of tumor and the appearance of abnormal soft tissue signals. A feathery appearance correlated with tumor-negative areas whereas a bulky appearance correlated with tumor-positive regions. MR imaging is helpful in identifying the nature of abnormal signal areas surrounding bone sarcomas that are more likely to be tumor-free, particularly when the signal in the soft tissues surrounding the tumor is feathery and edema-like in appearance. (orig.)

  9. Atypical growth on MRI in a case of Ewing's sarcoma despite lower SUV on PET

    Energy Technology Data Exchange (ETDEWEB)

    Sanford, Zachary [Marshall University, Joan C. Edwards School of Medicine (JCESOM), Huntington, WV (United States); Marshall University, Department of Orthopaedic Surgery, Joan C. Edwards School of Medicine JCESOM, Huntington, WV (United States); Israelsen, Stanford [Marshall University, Department of Orthopaedic Surgery, Joan C. Edwards School of Medicine JCESOM, Huntington, WV (United States); Sehgal, Rajesh [Marshall University, Department of Medicine, Division of Oncology, JCESOM, Huntington, WV (United States); Joan C. Edwards School of Medicine, Edwards Comprehensive Cancer Center, Huntington, WV (United States); Cheung, Felix H. [Marshall University, Department of Orthopaedic Surgery, Joan C. Edwards School of Medicine JCESOM, Huntington, WV (United States); Marshall University, Department of Orthopaedic Surgery, Joan C. Edwards School of Medicine, Edwards Comprehensive Cancer Center, Huntington, WV (United States)

    2014-06-15

    Ewing's sarcoma is a rare primary bone malignancy of small round blue cells. Treatment typically consists of neoadjuvant chemotherapy, surgical resection, and adjuvant chemotherapy. The disease response to chemotherapy can be followed with fluorodeoxyglucose (FDG) positron emission tomography (PET), which measures the metabolic activity of the tumor, and by magnetic resonance imaging (MRI), which measures tumor size. We present a unique case in which the tumor grew in size following neoadjuvant chemotherapy but decreased in metabolic activity, making it difficult to judge efficacy of the chemotherapy. An atypical response to chemotherapy in this case caused tumor growth due to a fibrotic reaction while viable tumor cells were eradicated. This case highlights the ability of FDG-PET scan to identify the uncommon situation in which a tumor that increased in size may have had a favorable response to chemotherapy. This possibility should be considered in similar cases in which FDG-PET scan shows diminishing metabolic activity despite tumor growth. (orig.)

  10. Ewing's sarcoma. Radiographic pattern of healing and bony complications in patients with long-term survival

    Energy Technology Data Exchange (ETDEWEB)

    Ehara, S.; Kattapuram, S.V.; Egglin, T.K. (Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston (United States))

    1991-10-01

    The radiographic appearance of Ewing's sarcoma was studied retrospectively in 22 patients who survived 5 years or longer after diagnosis and treatment. Expected changes from treatment, including regression of the extraosseous soft tissue mass, periostitis, and reconstitution of the cortex, occurred in all patients. Local recurrence occurred in one patient 10 years after complete remission whereas secondary osteosarcoma occurred more than 5 years after complete remission in two other cases. Both recurrent and secondary tumors presented as new lytic foci at the site of the original primary lesion. Lytic changes from radiation (radiation osteitis) may develop more than 2 years after treatment and in this sample; such findings were widely distributed in the radiation port. The authors conclude that bone remodeling and postradiation changes occur slowly over 2 years after treatment, and that any localized lysis at the primary site is suspicious for recurrence or secondary neoplasm. Knowledge of the expected changes and patterns of local recurrence and secondary neoplasms helps one to detect any significant change in its early phase.

  11. Primary Ewing sarcoma of vulva, confirmed with molecular cytogenetic analysis: A rare case report with diagnostic and treatment implications

    Directory of Open Access Journals (Sweden)

    Bharat Rekhi

    2015-01-01

    Full Text Available Primary vulvar Ewing sarcoma (ES/PNET is an uncommonly documented tumor, especially with molecular results. A 10-year-old girl presented with left vulvar swelling, a year ago. Her abdominopelvic ultrasound revealed a 12 cm × 8 cm sized, mixed echogenic blood-filled lesion in the left vulva; radiologically considered as a hematoma. Vulvectomy revealed a multinodular grey-brown tumor, microscopically comprising malignant round cells. Immunohistochemically, tumor cells diffusely expressed MIC2/CD99 and Fli1 and subsequently displayed EWSR1 rearrangement, confirming diagnosis of ES/PNET. Subsequently, PET-CT scan revealed residual local lesion with lung metastases. The patient was induced on EFT 2001 chemotherapy protocol. Three months after chemotherapy completion, there was no metabolically active disease on PET scan. Four months later, MRI disclosed recurrent primary and metastatic pulmonary lesions. She was planned for scar excision and adjuvant radiotherapy, but unfortunately defaulted further treatment. This forms the eighth case of primary vulvar ES/PNET confirmed with molecular cytogenetic result, underscoring therapeutic value of objective diagnosis in such cases.

  12. {sup 18}F-FDG PET response to neoadjuvant chemotherapy for Ewing sarcoma and osteosarcoma are different

    Energy Technology Data Exchange (ETDEWEB)

    Gaston, Louie L. [Philippine General Hospital, Department of Orthopedics, Manila (Philippines); Di Bella, Claudia [Saint Vincent' s Hospital, Department of Orthopedics, East Melbourne, VIC (Australia); Peter MacCallum Cancer Centre, Bone and Soft Tissue Sarcoma Service, East Melbourne, VIC (Australia); Slavin, John [Saint Vincent' s Hospital, Department of Pathology, Melbourne (Australia); Hicks, Rodney J. [The Peter MacCallum Cancer Centre, Centre for Cancer Imaging, Translational Research Group, Molecular Imaging and Targeted Therapeutics Laboratory, East Melbourne, VIC (Australia); Choong, Peter F.M. [Saint Vincent' s Hospital, Department of Orthopedics, East Melbourne, VIC (Australia); Peter MacCallum Cancer Centre, Bone and Soft Tissue Sarcoma Service, East Melbourne, VIC (Australia); University of Melbourne, Department of Surgery, Melbourne (Australia)

    2011-08-15

    Ewing sarcoma (ES) and osteosarcoma (OS) have different biological characteristics and respond differently to chemotherapy. We reviewed {sup 18}F-FDG PET imaging characteristics of ES and OS patients at baseline and following treatment to determine whether this biological variation is reflected in their imaging phenotype. A retrospective review of ES and OS patients treated with neoadjuvant chemotherapy and surgery was done, correlating PET results with histologic response to chemotherapy. Change in the maximum standardized uptake value (SUVmax) between baseline and post-treatment scanning was not significantly associated with histologic response for either ES or OS. Metabolic tumor volume (MTV) and the percentage of injected {sup 18}F-FDG dose (%ID) in the primary tumor were found to be different for ES and OS response subgroups. A 50% reduction in MTV (MTV2:1 < 0.5) was found to be significantly associated with favorable histologic response in OS. Using the same criteria for ES incorrectly predicted good responders. Increasing the cut-off values for ES to a 90% reduction in MTV (MTV2:1 < 0.1) resulted in association with favorable histologic response. Response to neoadjuvant chemotherapy as reflected by changes in PET characteristics should be interpreted differently for ES and OS. (orig.)

  13. Upregulation of NKX2.2, a target of EWSR1/FLI1 fusion transcript, in primary renal Ewing sarcoma

    Directory of Open Access Journals (Sweden)

    Yoshinari Yamamoto

    2015-01-01

    Full Text Available Renal Ewing sarcoma (ES is a rare malignant tumor characterized by fusion of the EWSR1 gene with a member of the ETS family of oncogenes, arising at a specific chromosomal translocation. Diagnosis of ES can be problematic, especially from cytological or small bioptical specimens because the differential diagnoses comprising a diverse group of small round blue cell tumors (SRBCTs. We report a case of primary renal ES in a young male, which had a t(11;22 (q24;q12 chromosome translocation encoding a type2 EWSR1/FLI1 fusion transcript. The tumor cells showed diffuse cytoplasmic immunoreactivity for CD99 and diffuse nuclear immunoreactivity for NKX2.2, an important oncogenic transcriptional target of EWSR1/FLI1, not only in the histological, but also in the cytological specimens. From the results of this case, we speculate that NKX2.2, in combination with CD99, may be a useful immunocytochemical marker to distinguish renal ES from other SRBCTs of kidney.

  14. Expression of Cathepsin L in tumor cells and tumor-associated macrophages in patients with Ewing sarcoma family of tumors: A pilot study

    Directory of Open Access Journals (Sweden)

    Bivas Biswas

    2015-01-01

    Full Text Available Background: Cysteine protease Cathepsin L is involved in bone remodeling and expressed in activated macrophages. It is highly expressed in metastatic tumor tissue, especially with bone metastases. Aims: We evaluated immunohistochemical expression of Cathepsin L in tumor cells and tumor-associated macrophages (TAMs in chemo-naive Ewing sarcoma. Settings and Design: Retrospective evaluation of archived specimens of Ewing sarcoma. Materials and Methods: Immunohistochemical staining was performed on archived blocks of chemo-naive patients with Ewing sarcoma treated with uniform chemotherapy at our institute between January 2009 and November 2011. Statistical Analysis: Immunohistochemical expression was co-related with baseline demographics and survival. Results: During the study period, we had evaluable baseline samples from 62 patients with median age 15 years (range: 2-40; 26 (42% had metastases. Cathepsin L expression in tumor cells was observed in 8/62 (13% specimens. None of the baseline clinical characteristics correlated with Cathepsin L expression. Cathepsin L positivity was associated with poor response to neoadjuvant chemotherapy (NACT (P = 0.05, but did not influence either event-free-survival (EFS or overall survival. Cathepsin L was expressed in TAMs in all specimens. Grade 3 TAMs (>10 TAMs/high power field was associated with better response to NACT (P = 0.05. On univariate analysis Grade 3 TAMs predicted superior EFS (median EFS 28.5 months in those with Grade 3 TAMs versus 14.8 months in those with grade ½ TAMs [P = 0.04]. Conclusions: Cathepsin L expression by immunohistochemistry was low in our patient cohort, and it did not affect the outcome. In addition, Grade 3 TAMs with Cathepsin L expression was associated with improved EFS.

  15. Mecanismo de acción de edelfosina en sarcoma de ewing y cáncer de cabeza y cuello

    OpenAIRE

    Bonilla Forero, Ximena Marcela

    2014-01-01

    [ES] Los tumores sólidos de sarcoma de Ewing y cáncer de cabeza y cuello son tumores altamente resistentes a la terapia convencional; además estos tumores emplean mecanismos moleculares que permiten la evasión de la respuesta apoptótica como mecanismo de supervivencia. Los lípidos antitumorales (ATLs) son compuestos ampliamente estudiados como posible herramienta terapéutica para el tratamiento de varios tipos de tumores, cuyo mecanismo de acción implica la activación de la ...

  16. CRM1 Inhibition Promotes Cytotoxicity in Ewing Sarcoma Cells by Repressing EWS-FLI1-Dependent IGF-1 Signaling.

    Science.gov (United States)

    Sun, Haibo; Lin, De-Chen; Cao, Qi; Guo, Xiao; Marijon, Helene; Zhao, Zhiqiang; Gery, Sigal; Xu, Liang; Yang, Henry; Pang, Brendan; Lee, Victor Kwan Min; Lim, Huey Jin; Doan, Ngan; Said, Jonathan W; Chu, Peiguo; Mayakonda, Anand; Thomas, Tom; Forscher, Charles; Baloglu, Erkan; Shacham, Sharon; Rajalingam, Raja; Koeffler, H Phillip

    2016-05-01

    Ewing sarcoma (EWS) is an aggressive bone malignancy that mainly affects children and young adults. The mechanisms by which EWS (EWSR1) fusion genes drive the disease are not fully understood. CRM1 (XPO1) traffics proteins from the nucleus, including tumor suppressors and growth factors, and is overexpressed in many cancers. A small-molecule inhibitor of CRM1, KPT-330, has shown therapeutic promise, but has yet to be investigated in the context of EWS. In this study, we demonstrate that CRM1 is also highly expressed in EWS. shRNA-mediated or pharmacologic inhibition of CRM1 in EWS cells dramatically decreased cell growth while inducing apoptosis, cell-cycle arrest, and protein expression alterations to several cancer-related factors. Interestingly, silencing of CRM1 markedly reduced EWS-FLI1 fusion protein expression at the posttranscriptional level and upregulated the expression of the well-established EWS-FLI1 target gene, insulin-like growth factor binding protein 3 (IGFBP3), which inhibits IGF-1. Accordingly, KPT-330 treatment attenuated IGF-1-induced activation of the IGF-1R/AKT pathway. Furthermore, knockdown of IGFBP3 increased cell growth and rescued the inhibitory effects on IGF-1 signaling triggered by CRM1 inhibition. Finally, treatment of EWS cells with a combination of KPT-330 and the IGF-1R inhibitor, linsitinib, synergistically decreased cell proliferation both in vitro and in vivo Taken together, these findings provide a strong rationale for investigating the efficacy of combinatorial inhibition of CRM1 and IGF-1R for the treatment of EWS. Cancer Res; 76(9); 2687-97. ©2016 AACR. PMID:26956669

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

    Science.gov (United States)

    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

    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-γ 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

  18. Vorinostat Enhances Cytotoxicity of SN-38 and Temozolomide in Ewing Sarcoma Cells and Activates STAT3/AKT/MAPK Pathways.

    Directory of Open Access Journals (Sweden)

    Valerie B Sampson

    Full Text Available Histone deacetylase inhibitors (HDACi have been evaluated in patients with Ewing sarcoma (EWS but demonstrated limited activity. To better understand the potential for HDACi in EWS, we evaluated the combination of the HDACi vorinostat, with DNA damaging agents SN-38 (the active metabolite of irinotecan and topoisomerase 1 inhibitor plus the alkylating agent temozolomide (ST. Drugs were evaluated in sequential and simultaneous combinations in two EWS cell lines. Results demonstrate that cell viability, DNA damage and reactive oxygen species (ROS production are dependent on the sequence of drug administration. Enhanced cytotoxicity is exhibited in vitro in EWS cell lines treated with ST administered before vorinostat, which was modestly higher than concomitant treatment and superior to vorinostat administered before ST. Drug combinations downregulate cyclin D1 to induce G0/G1 arrest and promote apoptosis by cleavage of caspase-3 and PARP. When ST is administered before or concomitantly with vorinostat there is activation of STAT3, MAPK and the p53 pathway. In contrast, when vorinostat is administered before ST, there is DNA repair, increased AKT phosphorylation and reduced H2B acetylation. Inhibition of AKT using the small molecule inhibitor MK-2206 did not restore H2B acetylation. Combining ST with the dual ALK and IGF-1R inhibitor, AZD3463 simultaneously inhibited STAT3 and AKT to enhance the cytotoxic effects of ST and further reduce cell growth suggesting that STAT3 and AKT activation were in part mediated by ALK and IGF-1R signaling. In summary, potent antiproliferative and proapoptotic activity were demonstrated for ST induced DNA damage before or simultaneous with HDAC inhibition and cell death was mediated through the p53 pathway. These observations may aid in designing new protocols for treating pediatric patients with high-risk EWS.

  19. Plasma hydrogenated cationic detonation nanodiamonds efficiently deliver to human cells in culture functional siRNA targeting the Ewing sarcoma junction oncogene.

    Science.gov (United States)

    Bertrand, Jean-Rémi; Pioche-Durieu, Catherine; Ayala, Juan; Petit, Tristan; Girard, Hugues A; Malvy, Claude P; Le Cam, Eric; Treussart, François; Arnault, Jean-Charles

    2015-03-01

    The expression of a defective gene can lead to major cell dysfunctions among which cell proliferation and tumor formation. One promising therapeutic strategy consists in silencing the defective gene using small interfering RNA (siRNA). In previous publications we showed that diamond nanocrystals (ND) of primary size 35 nm, rendered cationic by polyethyleneimine-coating, can efficiently deliver siRNA into cell, which further block the expression of EWS/FLI-1 oncogene in a Ewing sarcoma disease model. However, a therapeutic application of such nanodiamonds requires their elimination by the organism, particularly in urine, which is impossible for 35 nm particles. Here, we report that hydrogenated cationic nanodiamonds of primary size 7 nm (ND-H) have also a high affinity for siRNA and are capable of delivering them in cells. With siRNA/ND-H complexes, we measured a high inhibition efficacy of EWS/FLI-1 gene expression in Ewing sarcoma cell line. Electron microscopy investigations showed ND-H in endocytosis compartments, and especially in macropinosomes from which they can escape before siRNA degradation occurred. In addition, the association of EWS/FLI-1 silencing by the siRNA/ND-H complex with a vincristine treatment yielded a potentiation of the toxic effect of this chemotherapeutic drug. Therefore ND-H appears as a promising delivery agent in anti-tumoral gene therapy. PMID:25662499

  20. Influence of the Internalization Pathway on the Efficacy of siRNA Delivery by Cationic Fluorescent Nanodiamonds in the Ewing Sarcoma Cell Model

    Science.gov (United States)

    Alhaddad, Anna; Durieu, Catherine; Dantelle, Géraldine; Le Cam, Eric; Malvy, Claude; Treussart, François; Bertrand, Jean-Rémi

    2012-01-01

    Small interfering RNAs (siRNAs) are powerful tools commonly used for the specific inhibition of gene expression. However, vectorization is required to facilitate cell penetration and to prevent siRNA degradation by nucleases. We have shown that diamond nanocrystals coated with cationic polymer can be used to carry siRNAs into Ewing sarcoma cells, in which they remain traceable over long periods, due to their intrinsic stable fluorescence. We tested two cationic polymers, polyallylamine and polyethylenimine. The release of siRNA, accompanied by Ewing sarcoma EWS-Fli1 oncogene silencing, was observed only with polyethylenimine. We investigated cell penetration and found that the underlying mechanisms accounted for these differences in behavior. Using drugs selectively inhibiting particular pathways and a combination of fluorescence and electronic microscopy, we showed that siRNA gene silencing occurred only if the siRNA:cationic nanodiamond complex followed the macropinocytosis route. These results have potential implications for the design of efficient drug-delivery vectors. PMID:23284935

  1. Molecular detection of EWS-Ets fusion transcripts and their clinicopathologic significance in Ewing's sarcoma/peripheral primitive neuroectodermal tumor

    Institute of Scientific and Technical Information of China (English)

    WANG Hua; ZHENG Jie; WANG Yu-ping; YANG Yu; YOU Jiang-feng

    2005-01-01

    Background Ewing's sarcoma/peripheral primitive neuroectodermal tumor (ES/pPNET) is often difficult to distinguish from other small round cell tumors. The EWS-Ets gene fusions that result from chromosomal translocations in this tumor provide potential molecular diagnostic markers. To apply these molecular markers to commonly available archival materials, we evaluated the feasibility of detecting EWS-Ets including EWS-Fli1 and EWS-ERG fusion transcripts in paraffin-embedded tissues and its diagnostic value for detecting ES/pPNET.Methods Thirteen paraffin-embedded samples of ES/pPNETs were retrieved from archives. Thirteen cases of other tumors with small round cell features (including rhabdomyosarcoma, neuroblastoma, lymphoma, small cell carcinoma, and desmoplastic small round cell tumor) were used as negative controls. Β-actin and β2-microglobulin were used as internal controls. A nested reverse transcriptase-polymerase chain reaction (RT-PCR)-based assay was performed to detect the EWS-Fli1 and EWS-ERG fusion transcripts.Results β-actin and β2-microglobulin were detected in 10/13 and 13/13 ES/pPNETs, respectively. EWS-Fli1 fusion transcripts were detected in 11 of 13 (85%) ES/pPNETs. Three chimeric transcripts, all EWS-Fli1, were detected in ES/pPNET samples. Among 11 EWS-Fli1-positive cases, 7 cases had a typeⅠfusion transcript involving fusion of EWS exon 7 with Fli1 exon 6, 2 cases had a typeⅡfusion transcript involving EWS exon 7 with Fli1 exon 5, and 2 cases expressed fusion transcripts involving EWS exon 7 and Fli1 exon 8. Type Ⅰ EWS-Fli1 fusion predominated over other types. Fusion types could not be distinguished in the remaining 2 cases. Thirteen negative controls did not show detectable chimeric messages. There was a significant relationship between EWS-Fli1 fusion transcripts and CD99 expression. Conclusions Molecular detection of EWS-Fli1 fusion transcripts in formalin-fixed paraffin-embedded material by nested RT-PCR is feasible and is

  2. FOXO1 is a direct target of EWS-Fli1 oncogenic fusion protein in Ewing's sarcoma cells

    Energy Technology Data Exchange (ETDEWEB)

    Yang, Liu, E-mail: lyang@u.washington.edu [Department of Orthopedics, University of Washington, Seattle, WA 98195 (United States); Medical Research Service, VA Puget Sound Health Care System, Seattle, WA 98108 (United States); Hu, Hsien-Ming; Zielinska-Kwiatkowska, Anna; Chansky, Howard A. [Department of Orthopedics, University of Washington, Seattle, WA 98195 (United States); Medical Research Service, VA Puget Sound Health Care System, Seattle, WA 98108 (United States)

    2010-11-05

    Research highlights: {yields} Inducible and reversible siRNA knockdown of an oncogenic fusion protein such as EWS-Fli1 is feasible and more advantageous than other siRNA methods. {yields} The tumor suppressor gene FOXO1 is a new EWS-Fli1 target. {yields} While trans-activators are known for the FOXO1 gene, there has been no report on negative regulators of FOXO1 transcription. {yields} This study provides first evidence that the EWS-Fli1 oncogenic fusion protein can function as a transcriptional repressor of the FOXO1 gene. -- Abstract: Ewing's family tumors are characterized by a specific t(11;22) chromosomal translocation that results in the formation of EWS-Fli1 oncogenic fusion protein. To investigate the effects of EWS-Fli1 on gene expression, we carried out DNA microarray analysis after specific knockdown of EWS-Fli1 through transfection of synthetic siRNAs. EWS-Fli1 knockdown increased expression of genes such as DKK1 and p57 that are known to be repressed by EWS-Fli1 fusion protein. Among other potential EWS-Fli1 targets identified by our microarray analysis, we have focused on the FOXO1 gene since it encodes a potential tumor suppressor and has not been previously reported in Ewing's cells. To better understand how EWS-Fli1 affects FOXO1 expression, we have established a doxycycline-inducible siRNA system to achieve stable and reversible knockdown of EWS-Fli1 in Ewing's sarcoma cells. Here we show that FOXO1 expression in Ewing's cells has an inverse relationship with EWS-Fli1 protein level, and FOXO1 promoter activity is increased after doxycycline-induced EWS-Fli1 knockdown. In addition, we have found that direct binding of EWS-Fli1 to FOXO1 promoter is attenuated after doxycycline-induced siRNA knockdown of the fusion protein. Together, these results suggest that suppression of FOXO1 function by EWS-Fli1 fusion protein may contribute to cellular transformation in Ewing's family tumors.

  3. Prevention of central nervous system involvement with intrathecal 198Au colloid and methotrexate in non-Hodgkin lymphoma, acute non-lymphatic leukaemia and Ewing's sarcoma

    International Nuclear Information System (INIS)

    Intrathecal 198Au colloid and methotrexate were administered to 27 children (between 1972 and 1981) with non-lymphatic leukaemia, 21 with non-Hodgkin lymphoma and two with Ewing's sarcoma to prevent CNS involvement. In one boy with non-lymphatic leukaemia a stable remission after a three-year period of cytostatic treatment ended with isolated CNS involvement. No isolated CNS recurrence occurred in children with non-Hodgkin lymphoma receiving regular radiogold administration. Combined iris and CNS recurrence occurred in one child with non-Hodkin lymphoma. Eleven of 21 children with non-Hodgkin lymphoma have been in complete initial remission for 4-39 months without cytostatic treatment. Late cerebral complications have not been observed after 198Au colloid and methotrexate. (orig.)

  4. CD99 regulates neural differentiation of Ewing sarcoma cells through miR-34a-Notch-mediated control of NF-κB signaling.

    Science.gov (United States)

    Ventura, S; Aryee, D N T; Felicetti, F; De Feo, A; Mancarella, C; Manara, M C; Picci, P; Colombo, M P; Kovar, H; Carè, A; Scotlandi, K

    2016-07-28

    Sarcomas are mesenchymal tumors characterized by blocked differentiation process. In Ewing sarcoma (EWS) both CD99 and EWS-FLI1 concur to oncogenesis and inhibition of differentiation. Here, we demonstrate that uncoupling CD99 from EWS-FLI1 by silencing the former, nuclear factor-κB (NF-κB) signaling is inhibited and the neural differentiation program is re-established. NF-κB inhibition passes through miR-34a-mediated repression of Notch pathway. CD99 counteracts EWS-FLI1 in controlling NF-κB signaling through the miR-34a, which is increased and secreted into exosomes released by CD99-silenced EWS cells. Delivery of exosomes from CD99-silenced cells was sufficient to induce neural differentiation in recipient EWS cells through miR-34a inhibition of Notch-NF-κB signaling. Notably, even the partial delivery of CD99 small interfering RNA may have a broad effect on the entire tumor cell population owing to the spread operated by their miR-34a-enriched exosomes, a feature opening to a new therapeutic option. PMID:26616853

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

    Science.gov (United States)

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

    2010-02-01

    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

  6. t(4;11 (q21;q23 in acute myeloid leukemia-M0 following treatment [EW92 Protocol] for Ewing's sarcoma Leucemia mielóide aguda-M0 com t(4;11 (q21;q23 após tratamento para sarcoma de Ewing com o protocolo EW92

    Directory of Open Access Journals (Sweden)

    Terezinha J. Marques Salles

    2004-01-01

    Full Text Available We report on a 7-year-old girl with Ewing's Sarcoma (ES who developed a poorly differentiated acute myeloid leukemia (AML-M0 20 months after beginning the EW92 protocol for the treatment of the primary tumor. She received a total dose of 1500 mg of etoposide, a tumor cumulative radiation dose of 35Gy and granulocyte colony-stimulating factor (G-CSF was as predicted in the protocol regimen. At onset of secondary malignancy her laboratorial analysis revealed immature blast cells characterized by CD34+/CD33-/a-MPO+ and a t(4;11(q21;q23 abnormality. This serious complication of ES treatment, which associates etoposide, irradiation and G-CSF schedule, should be weighed against its therapeutic benefits.Nós descrevemos o caso clínico de uma criança do sexo feminino, com 7 anos de idade, portadora de sarcoma de Ewing, que evoluiu com leucemia aguda mielóide pouco diferenciada (LMA-M0 após vinte meses de tratamento utilizando o protocolo EW92. Ela recebeu uma dose total de 1.500 mg de etoposídio, irradiação tumoral na dose total de 35G, e fator de estimulação de colônia granulocítica (G-CSF conforme programação do protocolo terapêutico. Os exames laboratoriais, por ocasião do diagnóstico da segunda malignidade, mostraram células blásticas imaturas caracterizadas pela expressão de CD34+/CD33-/aMPO+ e a translocação t(4;11 (q 21;q23. A exclusão do G-CSF nos esquemas terapêuticos que associam etoposídio e irradiação tumoral se justifica devido a esta séria complicação no tratamento do sarcoma de Ewing.

  7. Ovarian tissue cryopreserved for fertility preservation from patients with Ewing or other sarcomas appear to have no tumour cell contamination

    DEFF Research Database (Denmark)

    Greve, Tine; Wielenga, Vera Timmermans; Grauslund, Morten; Sørensen, Nils; Christiansen, Dorte Bang; Rosendahl, Mikkel; Yding Andersen, Claus

    2013-01-01

    The chemotherapy required to treat patients with sarcoma may as a side-effect induce infertility in girls and young women. If these patients have ovarian cortical tissue cryopreserved prior to chemotherapy, they may, if necessary, have the tissue transplanted and restore their fertility. The aim of...

  8. Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma

    Science.gov (United States)

    2014-08-26

    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 Rhabdomyosarcoma; Adult Synovial Sarcoma; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  9. Ionizing radiation induced production of tumor necrosis factor {alpha} in the Ewing`s sarcoma cell line RM 82 in vitro and in vivo; Nachweis einer strahleninduzierten Produktion von Tumor-Nekrose-Faktor alpha im Ewing-Sarkom RM 82 in vitro und in vivo

    Energy Technology Data Exchange (ETDEWEB)

    Ruebe, C. [Muenster Univ. (Germany). Klinik und Poliklinik fuer Strahlentherapie - Radioonkologie; Finke, C. [Muenster Univ. (Germany). Klinik und Poliklinik fuer Strahlentherapie - Radioonkologie; Valen, F. van [Zellbiologisches Lab., Klinik und Poliklinik fuer Orthopaedie, Muenster Univ. (Germany); Schaefer, K.L. [Muenster Univ. (Germany). Gerhard-Domagk-Inst. fuer Pathologie; Dockhorn-Dworniczak, B. [Muenster Univ. (Germany). Gerhard-Domagk-Inst. fuer Pathologie; Willich, N. [Muenster Univ. (Germany). Klinik und Poliklinik fuer Strahlentherapie - Radioonkologie

    1997-08-01

    Aim: The expression of cytokines plays an important role in the transmission of the effects of ionizing radiation to tumor cells and normal tissue. Tumor necrosis factor alpha (TNF {alpha}), a pleiotropic monokine, is of special interest because of its cytotoxic effect on tumor cells and the induction of hemorrhagic necrosis in tumors. We examined the influence of ionizing radiation on TNF {alpha} production in a human Ewing`s sarcoma cell line in vitro and in vivo. Methods: The protein and mRNA levels of the Ewing`s sarcoma cell line RM 82 were examined in vitro with `Enhanced Amplified Sensitivity Immunoassay` (EASIA) and semiquantitative RT-PCR before and after treatment with single doses of 2 to 40 Gy, 1 to 72 hours after irradiation. After successful transplantation to nude mice, the time and dose correlation of TNF {alpha} mRNA production was examined in vivo. Results: In vitro, RM 82 had a basal protein level of TNF {alpha} of 20.1 {+-} 4.3 pg/ml/10{sup 6} cells. We observed a time- and dose-dependent increase of TNF {alpha} expression with a maximum of 125 pg/ml/10{sup 6} (5.9fold) 24 hours after irradiation with 20 Gy. At the mRNA level, the maximal up-regulation occurred 6 to 12 hours after 10 Gy. In vivo, the xenograft tumor maintained the capacity of TNF {alpha} expression. Time- and dose-dependency in mRNA production showed a maximum increase 6 hours after treatment with 10 Gy. (orig.) [Deutsch] Fragestellung: Die Expression von Zytokinen aus Tumor- und Normalgewebszellen spielt bei der Vermittlung der Strahlenwirkung auf Tumoren eine wichtige Rolle. Tumor-Nektrose-Faktor alpha (TNF {alpha}), ein pleiotropes, primaer als Monokin beschriebenes Polypeptid, ist durch seine zytotoxische Wirkung auf Tumorzellen sowie durch die Induktion haemorrhagischer Nekrosen von Tumoren von besonderem Interesse. Die vorliegende Arbeit untersucht den Einfluss ionisierender Strahlen auf die tumorzelleigene Expression von TNF {alpha} an einer humanen Ewing

  10. Process development of a human recombinant diabody expressed in E. coli: engagement of CD99-induced apoptosis for target therapy in Ewing's sarcoma.

    Science.gov (United States)

    Moricoli, Diego; Carbonella, Damiano Cosimo; Dominici, Sabrina; Fiori, Valentina; Balducci, Maria Cristina; Guerzoni, Clara; Manara, Maria Cristina; Pasello, Michela; Laguardia, Maria Elena; Cianfriglia, Maurizio; Scotlandi, Katia; Magnani, Mauro

    2016-05-01

    Ewing's sarcoma (EWS) is the second most common primary bone tumor in pediatric patients characterized by over expression of CD99. Current management consists in extensive chemotherapy in addition to surgical resection and/or radiation. Recent improvements in treatment are still overshadowed by severe side effects such as toxicity and risk of secondary malignancies; therefore, more effective strategies are urgently needed. The goal of this work was to develop a rapid, inexpensive, and "up-scalable" process of a novel human bivalent single-chain fragment variable diabody (C7 dAbd) directed against CD99, as a new therapeutic approach for EWS. We first investigated different Escherichia coli constructs of C7 dAbd in small-scale studies. Starting from 60 % soluble fraction, we obtained a yield of 25 mg C7 dAbd per liter of bacterial culture with the construct containing pelB signal sequence. In contrast, a low recovery of C7 dAbd was achieved starting from periplasmic inclusion bodies. In order to maximize the yield of C7 dAbd, large-scale fermentation was optimized. We obtained from 75 % soluble fraction 35 mg C7 dAbd per L of cell culture grown in a synthetic media containing 3 g/L of vegetable peptone and 1 g/L of yeast extract. Furthermore, we demonstrated the better efficacy of the cell lysis by homogenization versus periplasmic extraction, in reducing endotoxin level of the C7 dAbd. For gram-scale purification, a direct aligned two-step chromatography cascade based on binding selectivity was developed. Finally, we recovered C7 dAbd with low residual process-related impurities, excellent reactivity, and apoptotic ability against EWS cells. PMID:26685854

  11. Role of radiation therapy in the multidisciplinary management of Ewings Sarcoma of bone in pediatric patients: An effective treatment for local control

    International Nuclear Information System (INIS)

    Background: Radiotherapy (RT) plays an important role in the multidisciplinary management of Ewings 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. (authors)

  12. High-dose Chemotherapy and Autologous Peripheral Blood Stem Cell Transplantation in the Treatment of Children and Adolescents with Ewing Sarcoma Family of Tumors

    Directory of Open Access Journals (Sweden)

    Juhee Seo

    2013-09-01

    Full Text Available Purpose: We performed a pilot study to determine the benefit of high-dose chemotherapy and autologous peripheral blood stem cell transplantation (HDCT/autoPBSCT for patients with Ewing sarcoma family of tumors. Methods: We retrospectively analyzed the data of patients who received HDCT/autoPBSCT at Korea Cancer Center Hospital. Patients with relapsed, metastatic, or centrally located tumors were eligible for the study. Results: A total of 9 patients (3 male, 6 female, with a median age at HDCT/autoPBSCT of 13.4 years (range, 7.1 to 28.2 years, were included in this study. Patients underwent conventional chemotherapy and local control either by surgery or radiation therapy, and had achieved complete response (CR, n=7, partial response (n=1, or stable disease (n=1 prior to HDCT/autoPBSCT. There was no transplant-related mortality. However, the median duration of overall survival and event-free survival after HDCT/autoPBSCT were 13.3 months (range, 5.3 to 44.5 months and 6.2 months (range, 2.1 to 44.5 months, respectively. At present, 4 patients are alive and 5 patients who experienced adverse events (2 metastasis, 2 local recur, and 1 progressive disease survived for a median time of 2.8 months (range, 0.1 to 10.7 months. The 2-year survival after HDCT/autoPBSCT was 44.4%±16.6% and disease status at the time of HDCT/autoPBSCT tended to influence survival (57.1%±18.7% of cases with CR vs. 0% of cases with non-CR, P =0.07. Conclusion: Disease status at HDCT/autoPBSCT tended to influence survival. Further studies are necessary to define the role of HDCT/autoPBSCT and to identify subgroup of patients who might benefit from this investigational treatment.

  13. Ewing Sarcoma With ERG Gene Rearrangements: A Molecular Study Focusing on the Prevalence of FUS-ERG and Common Pitfalls in Detecting EWSR1-ERG Fusions by FISH

    Science.gov (United States)

    Chen, Sonja; Deniz, Kemal; Sung, Yun-Shao; Zhang, Lei; Dry, Sarah; Antonescu, Cristina R.

    2016-01-01

    The genetics of Ewing sarcoma (ES) are characterized by a canonical fusion involving EWSR1 gene and a member of the ETS family of transcription factors, such as FLI1 and ERG. In fact, ERG gene rearrangements represent the second most common molecular alteration, with EWSR1-ERG being identified in 5–10% of cases, while only a handful of reports document a FUS-ERG fusion. In this study, we focus on ES with ERG gene abnormalities, specifically to investigate the prevalence and clinicopathologic features of FUS-ERG fusions in a large cohort of small blue round cell tumors (SBRCTs) and compare to the eight reported FUS-positive ES. Among the 85 SBRCTs tested, seven (8.2%) cases harbored FUS gene rearrangements; six fused to ERG and one with FEV. During this investigation we came across a number of ERG-rearranged ES lacking both EWSR1 and FUS abnormalities by FISH. In one case, RNA sequencing identified an EWSR1-ERG transcript despite the negative EWSR1 rearrangements by FISH. Additional 3-color FISH fusion assay demonstrated the fusion of EWSR1 and ERG signals in all four cases negative for break-apart EWSR1 FISH. These results emphasize a potential pitfall of relying on EWSR1 FISH assay alone for diagnosis of ES. In cases with classic morphology and/or strong CD99 and ERG immunoreactivity, additional molecular testing should be applied, such as ERG FISH or RT-PCR/next generation sequencing, for a more definitive diagnosis. Although our study group is small, there were no differences noted between the clinical, morphologic features and immunoprofile of the different subsets of ERG-rearranged SBRCTs. PMID:26690869

  14. Diagnostic accuracy of 18F-FDG-PET and PET/CT in patients with Ewing sarcoma family tumours: a systematic review and a meta-analysis

    International Nuclear Information System (INIS)

    To systematically review and meta-analyse literature data on the diagnostic performance of fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography (PET) and positron emission tomography/computed tomography (PET/CT) in patients with Ewing sarcoma family tumours (ESFT). PubMed/MEDLINE, Embase and Scopus databases were searched for articles that evaluated FDG-PET and PET/CT in patients with ESFT from inception to 31 May 2011. Studies that fulfilled the three following criteria were included in the systematic review: FDG-PET or PET/CT performed in patients with ESFT; articles about the diagnostic accuracy of FDG-PET and PET/CT; sample size of at least 10 patients with ESFT were included. Studies in which there were sufficient data to reassess sensitivity and specificity of FDG-PET or PET/CT in ESFT were included in the meta-analysis, excluding duplicate publications. Finally, pooled sensitivity, pooled specificity and area under the receiver operating characteristic (ROC) curve of FDG-PET or PET/CT in ESFT were calculated. We found 13 studies comprising a total of 342 patients with ESFT. The main findings of the studies included are presented. The meta-analysis of five selected studies provided these results about FDG-PET and PET/CT in ESFT: pooled sensitivity: 96% (95% confidence interval [CI] 91-99%); pooled specificity: 92% (95% CI 87-96%); area under the ROC curve: 0.97. With regard to the staging and restaging of patients with ESFT, the sensitivity, specificity and accuracy of FDG-PET and PET/CT are high; the combination of FDG-PET or PET/CT with conventional imaging is a valuable tool for the staging and restaging of ESFT and has a relevant impact on the treatment strategy plan. (orig.)

  15. General Information about Ewing Sarcoma

    Science.gov (United States)

    ... Español 1-800-4-CANCER Live Chat Publications Dictionary Menu Contact Dictionary Search About Cancer Causes and Prevention Risk Factors ... Contacts Other Funding Find NCI funding for small business innovation, technology transfer, and contracts Training Cancer Training ...

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

    Science.gov (United States)

    2015-05-22

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

  17. Targeted therapy for sarcomas

    Directory of Open Access Journals (Sweden)

    Forscher C

    2014-03-01

    Full Text Available Charles Forscher,1 Monica Mita,2 Robert Figlin3 1Sarcoma Program, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 2Experimental Therapeutics Program, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 3Academic Development Program, Samuel Oschin Comprehensive Cancer Institute, and Division of Hematology/Oncology, Cedars-Sinai Medical Center, Los Angeles, CA, USA Abstract: 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. Keywords: sarcoma, targeted agents, tyrosine kinase inhibitors, mTor inhibition

  18. The histopathology of a human mesenchymal stem cell experimental tumor model: support for an hMSC origin for Ewing's sarcoma?

    DEFF Research Database (Denmark)

    Burns, J S; Abdallah, B M; Schrøder, Henrik Daa;

    2008-01-01

    Sarcomas display varied degrees of karyotypic abnormality, vascularity and mesenchymal differentiation. We have reported that a strain of telomerized adult human bone marrow mesenchymal stem cells (hMSC-TERT20) spontaneously evolved a tumorigenic phenotype after long-term continuous culture. We...... asked to what extent our hMSC-TERT20 derived tumors reflected events found in human sarcomas using routine histopathological procedures. Early versus late passage hMSC-TERT20 cultures persistently expressed mesenchymal lineage proteins e.g. CD105, CD44, CD99 and vimentin. However, late passage cultures...... hypothesis that sarcomas may arise from hMSC, providing a unique diploid model for exploring human sarcoma biology....

  19. Cixutumumab and Temsirolimus in Treating Younger Patients With Recurrent or Refractory Sarcoma

    Science.gov (United States)

    2015-03-19

    Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Gliosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma

  20. Efficacy of amifostine combined with chemotherapy for patients with Ewing sarcoma or PNET%氨磷汀联合化疗治疗Ewing肉瘤/PNET的临床观察

    Institute of Scientific and Technical Information of China (English)

    王翌庆; 刘云霞

    2012-01-01

    目的 探讨氨磷汀联合化疗治疗Ewing肉瘤/PNET的疗效、不良反应和安全性.方法 32例Ewing肉瘤/PNET患者分为化疗加氨磷汀组(观察组)及单纯化疗组(对照组),观察组12例,对照组20例.行IFO-IFO-DDP-ADM化疗2周期后评价疗效、不良反应.结果 观察组完全缓解(CR)及非常好的部分缓解率(VGPR)为91.6%,对照组为90.0%,两组比较差异无统计学意义(P>0.05).比较两组各种Ⅰ度~Ⅳ度不良反应发生率,差异无统计学意义(P>0.05).比较两组发生的Ⅳ度不良反应,观察组Ⅳ度白细胞减少为25.0%,对照组为70.0%;观察组Ⅳ度粒细胞减少25.0%,对照组为75.0%,差异均有统计学意义(P<0.05).结论 氨磷汀联合化疗治疗Ewing肉瘤/PNET不改变化疗疗效,不增加各种常见不良反应发生率;可以明显减少化疗后Ⅳ度白细胞及粒细胞减少的发生率,安全性好.%Objective To investigate the efficacy and adverse reactions of chemotherapy combined with amifostine for patients with Ewing sarcoma or primary neuroectodermal tumor ( PNET). Methods Thirty-two patients with Ewing sarcoma/PNET were divided into Group A ( amifostine group, n = 12) and Group B ( control group, n =20). After two cycles of IFO-IFO-DDP-ADM chemotherapy, the efficacy and adverse reaction of chemotherapy were observed. Results The rate of complete remission (CR) and very good partial response ( VGPR) was 91. 6% in Group A, and 90. 0% in Group B (P > 0. 05 ) . There was no significant difference in rate of adverse reaction I ~ IV between two groups (P > 0. 05 ) . The rate of IV leukopenia in Group A was 25. 0% and 70. 0% in Group B (P < 0. 05 ). The rate of W neutropenia in Group A was 25. 0% and 75. 0% in Group B ( P < 0. 05 ). Conclusion Amifostine combined with chemotherapy for patients with Ewing sarcoma/PNET may not change the efficacy or adverse reaction, while the rate of IV leukopenia and neutropenia would be reduced.

  1. A high proportion of bone marrow T cells with regulatory phenotype (CD4+CD25hiFoxP3+) in Ewing sarcoma patients is associated with metastatic disease.

    Science.gov (United States)

    Brinkrolf, Peter; Landmeier, Silke; Altvater, Bianca; Chen, Christiane; Pscherer, Sibylle; Rosemann, Annegret; Ranft, Andreas; Dirksen, Uta; Juergens, Heribert; Rossig, Claudia

    2009-08-15

    Immunosuppressive CD4+CD25(hi)FoxP3+ T cells (T(reg) cells) have been found at increased densities within the tumor microenvironment in many malignancies and interfere with protective antitumor immune responses. Osseous Ewing sarcomas (ESs) are thought to derive from a bone marrow (BM) mesenchymal cell of origin, and microscopic marrow involvement defines a subpopulation of patients at a high risk of relapse. We hypothesized that BM-resident T cells may contribute to a permissive milieu for immune escape of ESs. Using 6-color-flow cytometry, we investigated the pattern of immune cell subset distribution including NK cells, gammadelta T cells, central and effector memory CD8+ and CD4+ T cells as well as T cells with regulatory phenotype (T(reg) cells) in BM obtained at diagnosis from 45 primary or relapsed ES patients treated within standardized protocols. Although patients at relapse had an inverted CD4:CD8 T-cell ratio, neither CD8+ effector/memory T-cell subsets nor T(reg) cells significantly differed from patients at diagnosis. No significant associations of innate and effector/memory T-cell subpopulations with known risk factors were found, including age, gender, tumor site, primary metastases and histological tumor response. By contrast, T(reg) cells were found at significantly higher frequencies in patients with primary metastatic disease compared with localized ESs (5.0 vs. 3.3%, p = 0.01). Thus, increased BM T(reg) cells in patients with metastasized ES may reflect an immune escape mechanism that contributes to the development of metastatic disease. Immunotherapeutic strategies will have to adequately consider the regulatory milieu within areas of Ewing tumor-immune interactions. PMID:19480009

  2. Type 1 (11; 22)(q24: q12) translocation is common in Ewing's sarcoma/peripheral neuroectodermal tumour in south Indian patients

    Indian Academy of Sciences (India)

    T Parija; S Shirley; S Uma; K R Rajalekshmy; S Ayyappan; T Rajkumar

    2005-06-01

    The Ewing’s sarcoma family can present diagnostic difficulties. In the past the basis of diagnosis has been a exclusion. Identification of a specific translocation especially t(11; 22) (EWS-FLI 1 fusion gene), which is seen in nearly 85% of Ewing’s sarcoma cases can help in precise diagnosis. We have carried out a study on twenty patient samples diagnosed to have Ewing’s sarcoma/peripheral neuroectodermal tumour (PNET)/small round cell malignant tumour. The study involved RT-PCR analysis for the fusion transcript, followed by sequencing to identify the specific type of fusion. Ninety percent (18/20) of the samples tested were found to be t(11; 22) translocations involving EWS-FLI 1 genes. Sixty-one percent (11/18) were found to be type 1 fusion and seven were type 2 (39%). This is the first study in India with quantitative information about the types of EWS-FLI 1 translocations present in Ewing’s family of tumours in south Indian patients.

  3. Local recurrence, rate and sites of metastases, and time to relapse as a function of treatment regimen, size of primary and surgical history in 62 patients presenting with non-metastatic Ewing's sarcoma of the pelvic bones

    International Nuclear Information System (INIS)

    This report reviews the experience of 62 patients who presented between 1972 and 1978 with non-metastatic Ewing's sarcoma of the pelvis and were entered on IESS I. Seventeen patients (27%) developed a local recurrence, 38 patients (61%) demonstrated metastases and 21 (34%) neither. In the dose range 4000 rad to 6000 rad no dose response could be detected for local control of tumor. Forty-six patients (74%) had a biopsy or exploratory surgery only, 5 patients (8%) had an incomplete resection and 11 patients (18%) has a complete resection of their tumor. In the 46 patients having a biopsy only, 13 developed a local recurrence (28%) as compared to 2 of 11 patients undergoing a complete resection (18%). The most common sites for metastases were lung in 19 patients (31%) and bone in 23 patients (37%). No significant difference was noted in the frequency of overall metastases or metastases to any site between those patients receiving one of the three treatment regimens used in IESS I: VAC and Adriamycin (regime I), VAC alone (regimen II) and VAC plus bilateral pulmonary irradiation (regimen III). At a median follow-up of 135 weeks no significant difference in median survival could be detected in patients with pelvic primaries between regimens I, II and III. The possible reasons for the poor prognosis of pelvic primary patients are discussed together with treatment policies that might improve the survival of this group of patients

  4. Sulforaphane reverses chemo-resistance to temozolomide in glioblastoma cells by NF-κB-dependent pathway downregulating MGMT expression.

    Science.gov (United States)

    Lan, Fengming; Yang, Yang; Han, Jing; Wu, Qiaoli; Yu, Huiming; Yue, Xiao

    2016-02-01

    The survival benefits of patients with glioblastoma (GBM) remain unsatisfactory due to the intrinsic or acquired resistance to temozolomide (TMZ). We elucidated the mechanisms of sulforaphane (SFN) reverse TMZ resistance in TMZ-inducing cell lines by inhibiting nuclear factor-κB (NF-κB) transcriptional activity. TMZ-resistant cell lines (U87-R and U373-R) were generated by stepwise (6 months) exposure of parental cells to TMZ. Luciferase reporter assay, biochemical assays and subcutaneous tumor establishment were used to characterize the antitumor effect of SFN. MGMT expression and 50% inhibiting concentration (IC50) values of TMZ in GBM cell lines were assessed. Next, we established that U87-R and U373-R cells presenting high IC50 of TMZ, activated NF-κB transcription and significantly increased MGMT expression compared with untreated cells. Furthermore, we revealed that SFN could significantly suppress proliferation of TMZ-resistant GBM cells. In addition, SFN effectively inhibited activity of NF-κB signaling pathway and then reduced MGMT expression to reverse the chemo-resistance to TMZ in T98G, U87-R and U373-R cell lines. Sequential combination with TMZ synergistically inhibited survival capability and increased the induction of apoptosis in TMZ-resistant GBM cells. Finally, a nude mouse model was established with U373-R cell subcutaneous tumor-bearing mice, and results showed that SFN could remarkably suppress cell growth and enhance cell death in chemo-resistant xenografts in the nude mouse model. Collectively, the present study suggests that the clinical efficacy of TMZ-based chemotherapy in TMZ-resistant GBM may be improved by combination with SFN. PMID:26648123

  5. Epidemiology and therapies for metastatic sarcoma

    Directory of Open Access Journals (Sweden)

    Amankwah EK

    2013-05-01

    Full Text Available Ernest K Amankwah,1 Anthony P Conley,2 Damon R Reed2 1Department of Cancer Epidemiology, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA; 2Sarcoma Department, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA Abstract: Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subcutaneous tissue, and lymph nodes. Additionally, many sarcoma patients presenting initially with localized disease may relapse at metastatic sites. While localized sarcomas can often be cured through surgery and often radiation, controversies exist over optimal management of patients with metastatic sarcoma. Combinations of chemotherapy are the most effective in many settings, and many promising new agents are under active investigation or are being explored in preclinical models. Metastatic sarcomas are excellent candidates for novel approaches with additional agents as they have demonstrated chemosensitivity and affect a portion of the population that is motivated toward curative therapy. In this paper, we provide an overview on the common sarcomas of childhood (rhabdomyosarcoma, adolescence, and young adults (osteosarcoma, Ewing sarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor and older adults (leiomyosarcoma, liposarcoma, and undifferentiated high grade sarcoma in terms of the epidemiology, current therapy, promising therapeutic directions and outcome with a focus on metastatic disease. Potential advances in terms of promising therapy and biologic insights may lead to more effective and safer therapies; however, more clinical trials and research are needed for patients with metastatic sarcoma. Keywords: chemotherapy, pediatric sarcoma, rhabdomyosarcoma, osteosarcoma, Ewing sarcoma, synovial sarcoma

  6. APRIL is a novel clinical chemo-resistance biomarker in colorectal adenocarcinoma identified by gene expression profiling

    International Nuclear Information System (INIS)

    5-Fluorouracil(5FU) and oral analogues, such as capecitabine, remain one of the most useful agents for the treatment of colorectal adenocarcinoma. Low toxicity and convenience of administration facilitate use, however clinical resistance is a major limitation. Investigation has failed to fully explain the molecular mechanisms of resistance and no clinically useful predictive biomarkers for 5FU resistance have been identified. We investigated the molecular mechanisms of clinical 5FU resistance in colorectal adenocarcinoma patients in a prospective biomarker discovery project utilising gene expression profiling. The aim was to identify novel 5FU resistance mechanisms and qualify these as candidate biomarkers and therapeutic targets. Putative treatment specific gene expression changes were identified in a transcriptomics study of rectal adenocarcinomas, biopsied and profiled before and after pre-operative short-course radiotherapy or 5FU based chemo-radiotherapy, using microarrays. Tumour from untreated controls at diagnosis and resection identified treatment-independent gene expression changes. Candidate 5FU chemo-resistant genes were identified by comparison of gene expression data sets from these clinical specimens with gene expression signatures from our previous studies of colorectal cancer cell lines, where parental and daughter lines resistant to 5FU were compared. A colorectal adenocarcinoma tissue microarray (n = 234, resected tumours) was used as an independent set to qualify candidates thus identified. APRIL/TNFSF13 mRNA was significantly upregulated following 5FU based concurrent chemo-radiotherapy and in 5FU resistant colorectal adenocarcinoma cell lines but not in radiotherapy alone treated colorectal adenocarcinomas. Consistent withAPRIL's known function as an autocrine or paracrine secreted molecule, stromal but not tumour cell protein expression by immunohistochemistry was correlated with poor prognosis (p = 0.019) in the independent set

  7. Complex treatment of localized bone marrow sarcoma in children

    International Nuclear Information System (INIS)

    The retrospective analysis included the results of the treatment of 67 children suffering from localized sarcomas of bone (Ewing's sarcoma, lymphosarcoma). The advantage was demonstrated in patients, received combination of chemotherapy and radiotherapy on the involved bone. The resection of the primary tumor in combination with radio-chemotherapy improves the 10-year survival

  8. MUC5B silencing reduces chemo-resistance of MCF-7 breast tumor cells and impairs maturation of dendritic cells.

    Science.gov (United States)

    García, Enrique P; Tiscornia, Inés; Libisch, Gabriela; Trajtenberg, Felipe; Bollati-Fogolín, Mariela; Rodríguez, Ernesto; Noya, Verónica; Chiale, Carolina; Brossard, Natalie; Robello, Carlos; Santiñaque, Federico; Folle, Gustavo; Osinaga, Eduardo; Freire, Teresa

    2016-05-01

    Mucins participate in cancer progression by regulating cell growth, adhesion, signaling, apoptosis or chemo-resistance to drugs. The secreted mucin MUC5B, the major component of the respiratory tract mucus, is aberrantly expressed in breast cancer, where it could constitute a cancer biomarker. In this study we evaluated the role of MUC5B in breast cancer by gene silencing the MUC5B expression with short hairpin RNA on MCF-7 cells. We found that MUC5B-silenced MCF-7 cells have a reduced capacity to grow, adhere and form cell colonies. Interestingly, MUC5B knock-down increased the sensitivity to death induced by chemotherapeutic drugs. We also show that MUC5B silencing impaired LPS-maturation of DCs, and production of cytokines. Furthermore, MUC5B knock-down also influenced DC-differentiation and activation since it resulted in an upregulation of IL-1β, IL-6 and IL-10, cytokines that might be involved in cancer progression. Thus, MUC5B could enhance the production of LPS-induced cytokines, suggesting that the use of MUC5B-based cancer vaccines combined with DC-maturation stimuli, could favor the induction of an antitumor immune response. PMID:26984395

  9. Cabozantinib-S-Malate in Treating Younger Patients With Recurrent, Refractory, or Newly Diagnosed Sarcomas, Wilms Tumor, or Other Rare Tumors

    Science.gov (United States)

    2016-08-15

    Adrenal Cortex Carcinoma; Adult Alveolar Soft Part Sarcoma; Adult Clear Cell Sarcoma of Soft Parts; Adult Hepatocellular Carcinoma; Adult Rhabdomyosarcoma; Adult Soft Tissue Sarcoma; Childhood Alveolar Soft Part Sarcoma; Childhood Central Nervous System Neoplasm; Childhood Clear Cell Sarcoma of Soft Parts; Childhood Hepatocellular Carcinoma; Childhood Rhabdomyosarcoma; Childhood Soft Tissue Sarcoma; Childhood Solid Neoplasm; Ewing Sarcoma; Hepatoblastoma; Hepatocellular Carcinoma; Recurrent Adrenal Cortex Carcinoma; Recurrent Adult Hepatocellular Carcinoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Alveolar Soft Part Sarcoma; Recurrent Childhood Central Nervous System Neoplasm; Recurrent Childhood Hepatocellular Carcinoma; Recurrent Childhood Soft Tissue Sarcoma; Recurrent Ewing Sarcoma; Recurrent Hepatoblastoma; Recurrent Renal Cell Carcinoma; Recurrent Rhabdomyosarcoma; Relapsed Solid Neoplasm; Renal Cell Carcinoma; Thyroid Gland Medullary Carcinoma; Wilms Tumor

  10. Mir-34a mimics are potential therapeutic agents for p53-mutated and chemo-resistant brain tumour cells.

    Directory of Open Access Journals (Sweden)

    Yuen Ngan Fan

    Full Text Available 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.

  11. Immunotherapy of Childhood Sarcomas.

    Science.gov (United States)

    Roberts, Stephen S; Chou, Alexander J; Cheung, Nai-Kong V

    2015-01-01

    Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing's family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis. New therapies for these patients are urgently needed. Immunotherapy is an established treatment modality for both liquid and solid tumors, and in pediatrics, most notably for neuroblastoma and osteosarcoma. In the past, immunomodulatory agents such as interferon, interleukin-2, and liposomal-muramyl tripeptide phosphatidyl-ethanolamine have been tried, with some activity seen in subsets of patients; additionally, various cancer vaccines have been studied with possible benefit. Monoclonal antibody therapies against tumor antigens such as disialoganglioside GD2 or immune checkpoint targets such as CTLA-4 and PD-1 are being actively explored in pediatric sarcomas. Building on the success of adoptive T cell therapy for EBV-related lymphoma, strategies to redirect T cells using chimeric antigen receptors and bispecific antibodies are rapidly evolving with potential for the treatment of sarcomas. This review will focus on recent preclinical and clinical developments in targeted agents for pediatric sarcomas with emphasis on the immunobiology of immune checkpoints, immunoediting, tumor microenvironment, antibody engineering, cell engineering, and tumor vaccines. The future integration of antibody-based and cell-based therapies into an overall treatment strategy of sarcoma will be discussed. PMID:26301204

  12. Kaposi sarcoma

    Science.gov (United States)

    ... HIV, a weakened immune system, and the human herpesvirus-8 (HHV-8). Kaposi sarcoma has been linked ... on foot References Kaye KM. Kaposi's sarcoma-associated herpesvirus (human herpesvirus type 8). In: Mandell GL, Bennett ...

  13. Sarcomas of Soft Tissue and Bone.

    Science.gov (United States)

    Ferrari, Andrea; Dirksen, Uta; Bielack, Stefan

    2016-01-01

    The definition of soft tissue and bone sarcomas include a large group of several heterogeneous subtypes of mesenchymal origin that may occur at any age. Among the different sarcomas, rhabdomyosarcoma, synovial sarcoma, Ewing sarcoma and osteosarcoma are aggressive high-grade malignancies that often arise in adolescents and young adults. Managing these malignancies in patients in this age bracket poses various clinical problems, also because different therapeutic approaches are sometimes adopted by pediatric and adult oncologists, even though they are dealing with the same condition. Cooperation between pediatric oncologists and adult medical oncologists is a key step in order to assure the best treatment to these patients, preferably through their inclusion into international clinical trials. PMID:27595362

  14. Detection of fusion genes and fusion proteins in sarcoma : methodological and clinical aspects

    OpenAIRE

    Nilsson, Gunnar

    1999-01-01

    Ewing's sarcoma/PNET and synovial sarcoma, all regarded as high-grade tumours, have their peak incidence during the second decade of life, thus affecting children and adolescents. Both tumour types have specific chromosomal translocations, i.e. t(11;22) and t(X;18), respectively, resulting in fusion genes coding for chimeric proteins. The t(11;22) translocation, present in 85% of Ewing's sarcoma/PNET, results in the fusion of the EWS gene to the FLI- 1 gene forming EWS-FLI- ...

  15. Synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Sucari S.C. Vlok

    2014-12-01

    Full Text Available Synovial sarcoma is a malignant, predominantly juxta-articular, soft-tissue tumour representing approximately 10% of all soft-tissue sarcomas. Frequently initially incorrectly diagnosed as a benign lesion, it should be considered as a diagnosis when a young adult patient presents with a calcified juxta-articular soft-tissue mass of insidious onset.

  16. MicroRNA调控肿瘤耐药的研究进展%Advances in Tumor Chemo-resistance Regulated by MicroRNA

    Institute of Scientific and Technical Information of China (English)

    林高阳(综述); 徐克(审校)

    2014-01-01

    化学药物治疗(简称化疗)是目前治疗恶性肿瘤的主要手段之一,但肿瘤的多药耐药(multidurg resistance, MDR)是临床化疗失败的主要原因之一,然而肿瘤的耐药机制至今尚未被完全阐明。microRNA是近年来生命科学新的研究热点之一,microRNA通过在转录后水平调控基因表达而参与调控一系列的生命活动,包括细胞增殖、凋亡、脂肪代谢、神经元发育、激素分泌、肿瘤血管生成、干细胞分化、肿瘤细胞浸润及转移等多种生理和病理过程。最近的研究表明,microRNA对多基因表达的调控具有高效性和特异性,对靶基因的异常调控可能构成肿瘤耐药机制,是肿瘤耐药复杂性调控的重要构成部分。因此,对microRNA与肿瘤耐药性的研究具有现实意义。本文对近年来肿瘤耐药性的研究,microRNA调控肿瘤耐药机制及microRNA作为肿瘤耐药治疗潜在靶点作一综述。%Chemotherapy is one of the primary treatment for malignant tumors. Tumor multidrug resistance (MDR) is a major cause of clinical failure of chemotherapy;however the mechanisms of chemo-resistance have not been fully elucidated. Recently, microRNA is one of the new hotspots in life science. MicroRNA regulates the expression of genes and plays roles a series of life events by post-transcriptional regulations, including cell proliferation, apoptosis, fat metabolism, ner-vous development, hormone secretion, tumor vessels generation, stem cell differentiation, tumor cell invasion and metastasis, and other physiological and pathological processes. Recent studies show that microRNA regulates the expression of multiple genes with high efficiency and specificity. The abnormal regulation of target genes by microRNA is responsible for tumor chemo-resistance, this may be an important component of the complexity of the regulation of chemo-resistance. hTerefore, the study of microRNA and tumor drug resistance has

  17. The potential palliative role and possible immune modulatory effects of low-dose total body irradiation in relapsed or chemo-resistant non-Hodgkin's lymphoma

    International Nuclear Information System (INIS)

    In a group of 35 patients with relapsed and/or chemo-resistant non-Hodgkin's lymphoma (NHL), low-dose total body irradiation (LTBI) (+involved-field radiotherapy to bulky sites) achieved a complete remission rate of 29%, 2-years progression-free survival of 32% and a median progression-free survival of 12 months. The 2-year survival was 42% and the median survival was 17 months. Immuno-staining and flow cytometry of peripheral blood in 14 patients showed that LTBI leads to a significant increase in the percentage of CD4+ cells with a consequent significant increase in the CD4+/CD8+ ratio. High lymphocytic percent and a high percentage of CD4+ cells before LTBI were significantly correlated with longer response duration and overall survival. These data may suggest that the palliative potential of LTBI should be investigated as an alternative to chemotherapy in NHL patients. The pre-treatment percentage of lymphocytes and CD4+ cells may be used as predictors for response to LTBI

  18. MR criteria for differentiation of 'pseudo tumourous' lesions from bone sarcomas of the extremities

    International Nuclear Information System (INIS)

    The MRT scans of 57 patients with Ewing or osteosarcomas and 34 patients with haematogenous osteomyelitis or periostitis/stress fractures were examined in order to determine whether a distinction between benign or malignant lesions is possible. Four criteria were evaluated: The margin of the bone marrow component; intensity and homogeneity of the T1-weighted signal in the bone marrow; presence of an extraosseus structured soft tissue mass and/or soft tissue edema. It was found that central osteosarcomas and Ewing's sarcomas reduced signal intensity of the marrow to become muscle-isointense with a well defined margin. In acute haematogenous osteomyelitis and periostitis/stress fracture the marrow lesion was not sharply demarcated. In contrast to patients with bone sarcomas only one case of osteomyelitis showed an extrosseus structured soft tissue mass. On the basis of these findings we believe that acute haematogenous osteomyelitis can be distinguished with high degree of accuracy from Ewing's sarcoma and central osteosarcomas. (orig.)

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

    International Nuclear Information System (INIS)

    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

  20. Sensitization of Chemo-Resistant Human Chronic Myeloid Leukemia Stem-Like Cells to Hsp90 Inhibitor by SIRT1 Inhibition

    Science.gov (United States)

    Kim, Hak-Bong; Lee, Su-Hoon; Um, Jee-Hyun; Kim, Mi-Ju; Hyun, Suh-Kyung; Gong, Eun-Ji; Oh, Won Keun; Kang, Chi-Dug; Kim, Sun-Hee

    2015-01-01

    Development of effective therapeutic strategies to eliminate cancer stem-like cells (CSCs), which play a major role in drug resistance and disease recurrence, is critical to improve cancer treatment outcomes. The current investigation was undertaken to examine the effectiveness of the combination treatment of Hsp90 inhibitor and SIRT1 inhibitor in inhibiting the growth of chemo-resistant stem-like cells isolated from human chronic myeloid leukemia K562 cells. Inhibition of SIRT1 by use of SIRT1 siRNA or SIRT1 inhibitors (amurensin G and EX527) effectively potentiated sensitivity of Hsp90 inhibitors (17-AAG and AUY922) in CD44high K562 stem-like cells expressing high levels of CSC-related molecules including Oct4, CD34, β-catenin, c-Myc, mutant p53 (mut p53), BCRP and P-glycoprotein (P-gp) as well as CD44. SIRT1 depletion caused significant down-regulation of heat shock factor 1 (HSF1)/heat shock proteins (Hsps) as well as these CSC-related molecules, which led to the sensitization of CD44high K562 cells to Hsp90 inhibitor by SIRT1 inhibitor. Moreover, 17-AAG-mediated activation of HSF1/Hsps and P-gp-mediated efflux, major causes of Hsp90 inhibitor resistance, was suppressed by SIRT1 inhibitor in K562-CD44high cells. Our data suggest that combined treatment with Hsp90 inhibitor and SIRT1 inhibitor could be an effective therapeutic approach to target CSCs that are resistant to current therapies. PMID:26157347

  1. Sarcoma Foundation of America

    Science.gov (United States)

    ... Mission The mission of the Sarcoma Foundation of America (SFA) is to advocate for sarcoma patients by ... behalf of everyone at the Sarcoma Foundation of America (SFA),THANK YOU! The Celebration of Life drew ...

  2. Clinical and prognostic significance of PD-1 and PD-L1 expression in sarcomas.

    Science.gov (United States)

    Paydas, Semra; Bagir, Emine Kilic; Deveci, Mehmet Ali; Gonlusen, Gulfiliz

    2016-08-01

    Programmed death-1 (PD-1) and programmed death-ligand 1 (PD-L1) are new targets in cancer immunotherapy in recent years. The aim of this study is to evaluate the PD-1/PD-L1 expressions in sarcomas and to determine association between PD-1/PD-L1 expressions and clinical/pathological properties in some sarcoma subtypes. Formalin-fixed, paraffin-embedded tissue samples from 65 cases with sarcomas were analyzed. Immunohistochemical staining was performed to detect the PD-1 and PD-L1 expressions in tumor tissue and microenvironment, separately. PD-1 expression in tumor tissue and microenvironment was detected in 11 (17 %) and 8 (12 %) cases, respectively. PD-L1 expression in tumor tissue and microenvironment was detected in 19 (29 %) and 20 cases (30 %), respectively. None of the 5 Ewing sarcomas involving bone showed PD-1/PD-L1 expression, while 2 of 3 cases with Ewing sarcomas involving soft tissue showed PD-1 and PD-L1 expression. Among 5 cases with Kaposi sarcoma, four showed PD-1 and/or PD-L1 expression in tumor or microenvironment. PD-1/PD-L1 expressions were detected 3 of 6 cases with pleomorphic sarcoma, 2 of 4 cases with peripheral nerve sheath tumors and 1 of 4 cases with synovial sarcoma. Interestingly, strongest PD-1/PD-L1 expressions in our study group were detected in 2 sarcoma cases with the history of giant cell tumor. PD-1 and PD-L1 expressions are up to 30 % of the cases with sarcomas. It may be rational to target programmed death pathway in Kaposi sarcoma, pleomorphic sarcoma and peripheral nerve sheath tumors. Strong expression of PD-1/PD-L1 in cases with previous giant cell bone tumor has been found to be interesting and must be studied in giant cell tumor samples. PMID:27421997

  3. Sarcoma Immunotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Gouw, Launce G., E-mail: launce.gouw@hsc.utah.edu [Departments of Oncology, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Jones, Kevin B. [Departments of Orthopaedic Surgery, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Sharma, Sunil [Departments of Oncology, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Randall, R. Lor [Departments of Orthopaedic Surgery, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States)

    2011-11-10

    Much of our knowledge regarding cancer immunotherapy has been derived from sarcoma models. However, translation of preclinical findings to bedside success has been limited in this disease, though several intriguing clinical studies hint at the potential efficacy of this treatment modality. The rarity and heterogeneity of tumors of mesenchymal origin continues to be a challenge from a therapeutic standpoint. Nonetheless, sarcomas remain attractive targets for immunotherapy, as they can be characterized by specific epitopes, either from their mesenchymal origins or specific alterations in gene products. To date, standard vaccine trials have proven disappointing, likely due to mechanisms by which tumors equilibrate with and ultimately escape immune surveillance. More sophisticated approaches will likely require multimodal techniques, both by enhancing immunity, but also geared towards overcoming innate mechanisms of immunosuppression that favor tumorigenesis.

  4. Sarcoma Immunotherapy

    International Nuclear Information System (INIS)

    Much of our knowledge regarding cancer immunotherapy has been derived from sarcoma models. However, translation of preclinical findings to bedside success has been limited in this disease, though several intriguing clinical studies hint at the potential efficacy of this treatment modality. The rarity and heterogeneity of tumors of mesenchymal origin continues to be a challenge from a therapeutic standpoint. Nonetheless, sarcomas remain attractive targets for immunotherapy, as they can be characterized by specific epitopes, either from their mesenchymal origins or specific alterations in gene products. To date, standard vaccine trials have proven disappointing, likely due to mechanisms by which tumors equilibrate with and ultimately escape immune surveillance. More sophisticated approaches will likely require multimodal techniques, both by enhancing immunity, but also geared towards overcoming innate mechanisms of immunosuppression that favor tumorigenesis

  5. Radiotherapy and high-dose chemotherapy in advanced Ewing's tumors

    International Nuclear Information System (INIS)

    Background: Ewing's tumors are sensitive to radio- and chemotherapy. Patients with multifocal disease suffer a poor prognosis. Patients presenting primary bone marrow involvement or bone metastases at diagnosis herald a 3-year disease-free survival below 15%. The European Intergroup Cooperative Ewing's Sarcoma Study (EICESS) has established the following indications for high-dose therapy in advanced Ewing's tumors: Patients with primary multifocal bone disease, patients with early (<2 years after diagnosis) or multifocal relapse. Patients and Method: As of 1987, 83 patients have been treated in the EICESS group, 39 of them at the transplant center in Duesseldorf, who have been analyzed here. All individuals received 4 courses of induction chemotherapy with EVAJA and stem cell collection after course 3 and 4. Consolidation radiotherapy of the involved bone compartments was administered in a hyperfractionated regimen 2 times 1.6 Gy per day, up to 22.4 Gy simultaneously to course 5 and 22.4 Gy to course 6 of chemotherapy. The myeloablative chemotherapy consisted of melphalan and etoposide (ME) in combination with 12 Gy TBI (Hyper-ME) oder Double-ME with whole lung irradiation up to 18 Gy (without TBI). Results: The survival probability at 40 months was 31% (44% DOD; 15% DOC). Pelvic infiltration did not reach prognostic relevance in this cohort. Radiotherapy encompassed 75% of the bone marrow at maximum (average 20%). Engraftment was not affected by radiotherapy. Conclusion: High-dose chemotherapy can improve outcome in poor prognostic advanced Ewing's tumors. The disease itself remains the main problem. The expected engraftment problems after intensive radiotherapy in large volumes of bone marrow can be overcome by stem cell reinfusion. (orig.)

  6. Intra articular synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Sistla Radha

    2010-01-01

    Full Text Available Synovial sarcoma is a soft tissue neoplasm with a characteristic biphasic pattern. Incidence in soft tissues is 5-10%. Intra articularly synovial sarcoma is extremely rare. Fewer than 5% of all synovial sarcomas arise within the joint space. We report a case of intra articular synovial sarcoma in a young male who presented as internal derangement of the knee.

  7. Intra articular synovial sarcoma

    OpenAIRE

    Sistla Radha; Tameem Afroz; Vidyasagar JVS

    2010-01-01

    Synovial sarcoma is a soft tissue neoplasm with a characteristic biphasic pattern. Incidence in soft tissues is 5-10%. Intra articularly synovial sarcoma is extremely rare. Fewer than 5% of all synovial sarcomas arise within the joint space. We report a case of intra articular synovial sarcoma in a young male who presented as internal derangement of the knee.

  8. The diagnostic utility of reduced immunohistochemical expression of SMARCB1 in synovial sarcomas: a validation study.

    Science.gov (United States)

    Ito, Junko; Asano, Naofumi; Kawai, Akira; Yoshida, Akihiko

    2016-01-01

    Synovial sarcoma is a malignant mesenchymal neoplasm of uncertain histogenesis, characterized by a specific SS18-SSX fusion. The diagnosis of synovial sarcoma can be challenging based on morphology and conventional immunohistochemistry alone, and identification of the fusion gene by molecular genetics may be necessary for diagnosis. Several recent studies have demonstrated the diagnostic utility of the reduced expression of SMARCB1 in synovial sarcomas as measured using immunohistochemistry. Therefore, we undertook a validation study using synovial sarcomas and other spindle or round cell tumors that could enter differential diagnosis of monophasic or poorly differentiated synovial sarcomas. Among 36 synovial sarcomas that were successfully evaluated, the expression of SMARCB1 was diffusely reduced in 33 cases (92%) at variable degrees. In contrast, the expression of SMARCB1 was not reduced in any of the 93 evaluable non-synovial sarcoma tumors (5 thymomas, 5 sarcomatoid mesotheliomas, 10 schwannomas, 9 mesenchymal chondrosarcomas, 20 solitary fibrous tumors, 19 Ewing sarcomas, and 25 malignant peripheral nerve sheath tumors). A few schwannomas and malignant peripheral nerve sheath tumors showed mosaic or complete loss of SMARCB1 expression. Reduced expression of SMARCB1 immunoreactivity was therefore found to be highly sensitive and specific for synovial sarcoma, and can be useful for rapidly and accurately confirming the diagnosis of synovial sarcoma. This reduction in SMARCB1 expression likely reflects the BAF47 ejection mechanism of the SS18-SSX fusion product and can therefore be viewed as an indirect visualization of this fusion product. PMID:26520417

  9. [Treatments of Soft Tissue Sarcomas by Orthopaedic Surgeons in Japan].

    Science.gov (United States)

    Tanaka, Kazuhiro

    2016-01-01

    In Japan, the treatment of soft tissue sarcomas (STS) has been performed mainly by orthopaedic surgeons. The standard therapy for all cases of STS is surgical resection of the tumor. The prognosis of patients with unresectable tumors or distant metastases is poor despite treatment with intensive chemotherapy. Adjuvant chemotherapy is indicated for patients with resectable tumors. Round-cell STS, including extraskeletal Ewing sarcoma and rhabdomyosarcoma, have high sensitivity to chemotherapy. The standard treatment for round-cell STS is multimodal therapy with surgery and chemotherapy, with or without radiotherapy. On the other hand, non-round cell STS, including leiomyosarcoma, synovial sarcoma, and liposarcoma, have low sensitivity to chemotherapy. Thus, the standard treatment for non-round cell STS is essentially, surgery. Large and high-grade non-round cell STS are also treated using adjuvant chemotherapy along with surgery. In this review, the standard therapies for STS and the future perspective in Japan are discussed. PMID:26809525

  10. Diagnosis and treatment of sarcomas and related tumors

    Energy Technology Data Exchange (ETDEWEB)

    1978-01-01

    The Cancergram focuses on clinical aspects of sarcomas involving soft tissue and bone, and also includes abstracts on related malignant and benign tumors. Soft tissues are considered as all non-epithelial extra-skeletal tissues of the body, with the exception of the reticuloendothelial system, the neuroglia, and visceral and parenchymal organs. Included, therefore, are sarcomas of the vascular system, fatty tissue, muscle tissues, connective tissues, and synovial tissues. Bone tumors included are osteosarcoma, chondrosarcoma, adamantinoma, chrondroblastoma, Ewing's sarcoma, and other benign and malignant disorders of the bone. This Cancergram excludes disorders of the bone marrow, which are the subjects of separate Cancergrams (see series CT03 for leukemias, and series CT12 for multiple myeloma). The scope includes diagnosis and staging, supportive care, evaluation, and therapy. Selected abstracts concerning epidemiology, etiology and other pre-clinical studies will also be included where they have direct clinical relevance.

  11. The Epidemiology of Sarcoma

    Directory of Open Access Journals (Sweden)

    Burningham Zachary

    2012-10-01

    Full Text Available Abstract 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

  12. Adult soft tissue sarcoma

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/007654.htm Adult soft tissue sarcoma To use the sharing features on this page, please enable JavaScript. Soft tissue sarcoma is cancer that forms in the soft ...

  13. What Is Kaposi Sarcoma?

    Science.gov (United States)

    ... with a certain virus (the Kaposi sarcoma associated herpesvirus or KSHV ) are more likely to develop KS. ... who already have a KSHV (Kaposi sarcoma associated herpesvirus) infection are more likely to develop KS. Endemic ( ...

  14. Primary Renal Synovial Sarcoma

    OpenAIRE

    Halil Ciftci; Adem Altunkol; Ismail Ozdemir; Dilek Mil; Ilyas Ozardali; Murat Savas; Ercan Yeni; Mehmet Gulum

    2011-01-01

    Synovial sarcomas are generally deep-seated tumors that most often occur in the proximity of large joints of adolescents and young adults. We describe two cases of primary renal synovial sarcoma that were treated successfully by radical nephrectomy. Synovial sarcoma originating from the kidney is extremely rare and the histogenesis is uncertain. Surgical resection and ifosfamide based chemotherapy are the mainstay for the management of renal synovial sarcoma. Fewer than 40 patients have been ...

  15. Chromatin H3K27me3/H3K4me3 histone marks define gene sets in high-grade serous ovarian cancer that distinguish malignant, tumour-sustaining and chemo-resistant ovarian tumour cells.

    Science.gov (United States)

    Chapman-Rothe, N; Curry, E; Zeller, C; Liber, D; Stronach, E; Gabra, H; Ghaem-Maghami, S; Brown, R

    2013-09-19

    In embryonic stem (ES) cells, bivalent chromatin domains containing H3K4me3 and H3K27me3 marks silence developmental genes, while keeping them poised for activation following differentiation. We have identified gene sets associated with H3K27me3 and H3K4me3 marks at transcription start sites in a high-grade ovarian serous tumour and examined their association with epigenetic silencing and malignant progression. This revealed novel silenced bivalent marked genes, not described previously for ES cells, which are significantly enriched for the PI3K (P<10(-7)) and TGF-β signalling pathways (P<10(-5)). We matched histone marked gene sets to gene expression sets of eight normal fallopian tubes and 499 high-grade serous malignant ovarian samples. This revealed a significant decrease in gene expression for the H3K27me3 and bivalent gene sets in malignant tissue. We then correlated H3K27me3 and bivalent gene sets to gene expression data of ovarian tumour 'stem cell-like' sustaining cells versus non-sustaining cells. This showed a significantly lower expression for the H3K27me3 and bivalent gene sets in the tumour-sustaining cells. Similarly, comparison of matched chemo-sensitive and chemo-resistant ovarian cell lines showed a significantly lower expression of H3K27me3/bivalent marked genes in the chemo-resistant compared with the chemo-sensitive cell line. Our analysis supports the hypothesis that bivalent marks are associated with epigenetic silencing in ovarian cancer. However it also suggests that additional tumour specific bivalent marks, to those known in ES cells, are present in tumours and may potentially influence the subsequent development of drug resistance and tumour progression. PMID:23128397

  16. SYNOVIAL CELL SARCOMA

    OpenAIRE

    Farzan, M

    1997-01-01

    Ten cases of synovial cell sarcoma are reported. The youngest patient was a 2'A years old boy with synovial cell sarcoma of the knee and the oldest one was a man with synovial cell sarcoma of the elbow.

  17. SYNOVIAL CELL SARCOMA

    Directory of Open Access Journals (Sweden)

    M. Farzan

    1997-06-01

    Full Text Available Ten cases of synovial cell sarcoma are reported. The youngest patient was a 2'A years old boy with synovial cell sarcoma of the knee and the oldest one was a man with synovial cell sarcoma of the elbow.

  18. Extra-osseous Ewing′s sarcoma of sciatic nerve masquerading as an infected hemangioma: A rare case report

    OpenAIRE

    Dhua, Anjan K; Ravindhra Bharathi; Chokka Mahesh Kiran; Pappu Paramartha Lingam; Manoj Joshi

    2014-01-01

    Extra-osseous Ewing's Sarcoma (EES) arising from the peripheral nerve is rarely reported in children. Here, we report an instance of EES arising from the left sciatic nerve mimicking an infected hemangioma. This case highlights the need for a high index of suspicion and early histological diagnosis to avoid diagnostic delay.

  19. Synovial sarcoma

    International Nuclear Information System (INIS)

    Authors present the material of 12 patients with synovial sarcoma treated in Surgical Oncology Dept. Medical University of Gdansk between 1973-1993. In all cases the main symptom was a growing tumor; tumor accompanied by pain in 3 of them. Average time for symptoms was about 9 months. Tumors were classified according to GTNM and Enneking Classification. 9 cases were classified as G2T1NOMO and 3 as G2T2NOMO. According to Enneking Classification 1 case only was recognized as intracompartmental and 11 as extracompartmental. In 9 tumors cytogenetic analysis was done. In 8 of them specific chromosome aberration was discovered - translocation between X and 18 chromosomes: t (18;X)(p11;q11). The following procedures were performed: enucleation of tumor (non-radical) in 2 cases, wide excision in 8 cases, extremity amputation in 1 case. Local recurrence was observed in 9 cases. 30 months without recurrence was noticed as an average. The wide local excision was the method of treatment of local recurrence in 4 cases (followed by RTG-therapy in one case). In another 5 cases extremity amputation was performed (followed by CyVADic chemotherapy in one case). In our material 5-year survival was observed in 36% of cases. The longest notice follow-up was 30 years. (author)

  20. STAT3 Regulates Proliferation and Immunogenicity of the Ewing Family of Tumors In Vitro

    Directory of Open Access Journals (Sweden)

    Sam Behjati

    2012-01-01

    Full Text Available The Ewing sarcoma family of tumors (ESFT represents an aggressive spectrum of malignant tumour types with common defining histological and cytogenetic features. To evaluate the functional activation of signal transducer and activator of transcription 3 (STAT3 in ESFT, we evaluated its activation in primary tissue sections and observed the functional consequences of its inhibition in ESFT cell lines. STAT3 was activated (tyrosine 705-phosphorylated in 18 out of 31 primary tumours (58%, either diffusely (35% or focally (23%. STAT3 was constitutively activated in 3 out of 3 ESFT cell lines tested, and its specific chemical inhibition resulted in complete loss of cell viability. STAT3 inhibition in ESFT cell lines was associated with several consistent changes in chemokine profile suggesting a role of STAT3 in ESFT in both cell survival and modification of the cellular immune environment. Together these data support the investigation of STAT3 inhibitors for the Ewing family of tumors.

  1. FDG PET/CT imaging in primary osseous and soft tissue sarcomas: a retrospective review of 212 cases

    Energy Technology Data Exchange (ETDEWEB)

    Charest, Mathieu [Lakeshore General Hospital, Service of Nuclear Medicine, Pointe-Claire (Canada); Hickeson, Marc; Lisbona, Robert; Novales-Diaz, Javier A.; Derbekyan, Vilma [McGill University Health Center, Department of Nuclear Medicine, Montreal (Canada); Turcotte, Robert E. [McGill University Health Center, Department of Orthopedic Surgery, Montreal (Canada)

    2009-12-15

    The aims of this study are to evaluate the sensitivity of FDG PET/CT for detection of soft tissue and osseous sarcomas on the basis of FDG avidity. We retrospectively evaluated 212 consecutive patients with known soft tissue or osseous sarcoma who had undergone a FDG PET/CT study for the initial staging or assessment of recurrence of disease. The maximum standardized uptake value (SUV{sub max}) of each primary and/or most intense metastatic lesion was measured and compared with the histological data provided in the final pathological reports. An SUV{sub max} of 2.5 or greater was considered positive for our analysis. Sufficient histopathological data were available for 160 soft tissue sarcomas and 52 osseous sarcomas. FDG PET/CT detected 93.9% of all sarcomas with a sensitivity of 93.7% for soft tissue sarcomas and 94.6% for osseous sarcomas. The sensitivities of the most common sarcoma histologies were 100% for leiomyosarcomas, 94.7% for osteosarcomas, 100% for Ewing's sarcomas, 88.9% for liposarcomas, 80.0% for synovial sarcomas, 100% for gastrointestinal stromal tumors, 87.5% for malignant peripheral nerve sheath tumors, 100% for fibroblastic and myoblastic sarcomas, and 100% for malignant fibrohistiocytic tumors. The receiver-operating characteristic curve revealed an area under the curve of 94% for the discrimination of low-grade and high-grade sarcomas imaged for initial staging by FDG PET/CT. The combined metabolic and morphological information of FDG PET/CT imaging allows high sensitivity for the detection of various sarcomas and accurate discrimination between newly diagnosed low-grade and high-grade sarcomas. (orig.)

  2. FDG PET/CT imaging in primary osseous and soft tissue sarcomas: a retrospective review of 212 cases

    International Nuclear Information System (INIS)

    The aims of this study are to evaluate the sensitivity of FDG PET/CT for detection of soft tissue and osseous sarcomas on the basis of FDG avidity. We retrospectively evaluated 212 consecutive patients with known soft tissue or osseous sarcoma who had undergone a FDG PET/CT study for the initial staging or assessment of recurrence of disease. The maximum standardized uptake value (SUVmax) of each primary and/or most intense metastatic lesion was measured and compared with the histological data provided in the final pathological reports. An SUVmax of 2.5 or greater was considered positive for our analysis. Sufficient histopathological data were available for 160 soft tissue sarcomas and 52 osseous sarcomas. FDG PET/CT detected 93.9% of all sarcomas with a sensitivity of 93.7% for soft tissue sarcomas and 94.6% for osseous sarcomas. The sensitivities of the most common sarcoma histologies were 100% for leiomyosarcomas, 94.7% for osteosarcomas, 100% for Ewing's sarcomas, 88.9% for liposarcomas, 80.0% for synovial sarcomas, 100% for gastrointestinal stromal tumors, 87.5% for malignant peripheral nerve sheath tumors, 100% for fibroblastic and myoblastic sarcomas, and 100% for malignant fibrohistiocytic tumors. The receiver-operating characteristic curve revealed an area under the curve of 94% for the discrimination of low-grade and high-grade sarcomas imaged for initial staging by FDG PET/CT. The combined metabolic and morphological information of FDG PET/CT imaging allows high sensitivity for the detection of various sarcomas and accurate discrimination between newly diagnosed low-grade and high-grade sarcomas. (orig.)

  3. Chemotherapy for Soft Tissue Sarcomas

    Science.gov (United States)

    ... Next Topic Targeted therapy for soft tissue sarcoma Chemotherapy for soft tissue sarcomas Chemotherapy (chemo) is the use of drugs given into ... Depending on the type and stage of sarcoma, chemotherapy may be given as the main treatment or ...

  4. Pediatric Soft Tissue Sarcomas

    OpenAIRE

    Loeb, David M.; Thornton, Katherine; Shokek, Ori

    2008-01-01

    Soft tissue sarcomas in children are relatively rare. Approximately 850 to 900 children and adolescents are diagnosed each year with rhabdomyosarcoma (RMS) or one of the non-RMS soft tissue sarcomas (NRSTS). Of these, 350 are cases of RMS. RMS is the most common soft tissue sarcoma in children 14 years old and younger, and NRSTS is more common in adolescents and young adults. Infants also get NRSTS, but their tumors constitute a distinctive set of histologies, including infantile fibrosarcoma...

  5. Four year experience of sarcoma of soft tissues and bones in a tertiary care hospital and review of literature

    Directory of Open Access Journals (Sweden)

    Ansari Tayyaba Z

    2011-05-01

    Full Text Available Abstract Background Sarcoma encompasses an uncommon group of cancer and the data is insufficient from Pakistan. We report our four years experience of Sarcoma of soft tissues and bones. Methods This cross sectional study was carried out at Aga Khan University Hospital from 2004 to 2008. The patients were divided into two groups from the outset i.e. initially diagnosed and relapsed group and separate sub group analysis was conducted. Results Out of 93 newly diagnosed patients, 58 belonged to bone sarcoma and 35 to soft tissue sarcoma group. While for relapsed patients, 5 had soft tissue sarcoma and 9 had bone sarcoma. Mean age was 32.5 years. At presentation, approximately two third patients had localised disease while remaining one third had metastatic disease. The Kaplan Meier estimate of median recurrence free survival was 25 months, 35 months, and 44 months for Osteogenic sarcoma, Ewing's sarcoma and Chondrosarcoma respectively. For Leiomyosarcoma and Synovial sarcoma, it was 20 and 19 months respectively. The grade of the tumour (p = 0.02 and surgical margin status (p = 0.001 were statistically significant for determination of relapse of disease. Conclusion The median recurrence free survival of patients in our study was comparable to the reported literature but with significant lost to follow rate. Further large-scale, multi centre studies are needed to have a more comprehensive understanding of this heterogeneous disease in our population.

  6. [Primary pulmonary sarcomas].

    Science.gov (United States)

    Jakubcová, T; Jakubec, P

    2009-01-01

    Primary pulmonary sarcomas are rare diseases unlike lung carcinomas. The occurence of these sarcomas is between 0.013-0.40% of all malignant lung tumours. There are malignant mesenchymal tumours. They are flowing from the soft tissue of lung. The pulmonary sarcomas are heterogenic group with various biological behaviour. Their morfologic structure does not digger from the sarcomas of soft tissue. The primary pulmonary sarcomas occur more often in childhood and in young people unlike lung carcinomas. Radiation and some toxic substances are noted risk factors. Some gene mutations, infectious pathoghens and contraception have a possible impact on the origin of some types of the sarcomas. The current hypothesis is, that most of the sarcomas, if not all sarcomas, stem from primitive multipotent mesenchymal cell by malignant transformation in one or more lines. The diagnostic standard is biopsy from tumour with histologic and immunohistochemistry examination of a sample. The basic diagnostic problem is exclusion of a secondary origin of sarcomatic cells in the lung, because pulmonary metastasis of extrapulmonary sarcomas are more often than the primary pulmonary involvement.The optimal treatment is a resection of the tumour.The other therapeutic modalities are radiotherapy and chemotherapy, but results of these modalities are unsatisfactory. There are various chemotherapeutic regimes, monotherapy or combination regimes. The basic cytostatics are doxorubicine, iphosphamide, dacarbazine. Problems of the chemotherapy are high toxicity and relatively low curative effect about 20%.The first studies with biological treatment of the sarcomas of soft tissue have been published recently.This types of drugs could be a part of the complex management of these primary pulmonary tumours in the future. The primary pulmonary sarcomas have mostly aggresive course and often recur. Their prognosis is usually not very good. The survival median is 48 months and 5-years survival ranges

  7. Profiling of potential driver mutations in sarcomas by targeted next generation sequencing.

    Science.gov (United States)

    Andersson, Carola; Fagman, Henrik; Hansson, Magnus; Enlund, Fredrik

    2016-04-01

    Comprehensive genetic profiling by massively parallel sequencing, commonly known as next generation sequencing (NGS), is becoming the foundation of personalized oncology. For sarcomas very few targeted treatments are currently in routine use. In clinical practice the preoperative diagnostic workup of soft tissue tumours largely relies on core needle biopsies. Although mostly sufficient for histopathological diagnosis, only very limited amounts of formalin fixated paraffin embedded tissue are often available for predictive mutation analysis. Targeted NGS may thus open up new possibilities for comprehensive characterization of scarce biopsies. We therefore set out to search for driver mutations by NGS in a cohort of 55 clinically and morphologically well characterized sarcomas using low input of DNA from formalin fixated paraffin embedded tissues. The aim was to investigate if there are any recurrent or targetable aberrations in cancer driver genes in addition to known chromosome translocations in different types of sarcomas. We employed a panel covering 207 mutation hotspots in 50 cancer-associated genes to analyse DNA from nine gastrointestinal stromal tumours, 14 synovial sarcomas, seven myxoid liposarcomas, 22 Ewing sarcomas and three Ewing-like small round cell tumours at a large sequencing depth to detect also mutations that are subclonal or occur at low allele frequencies. We found nine mutations in eight different potential driver genes, some of which are potentially actionable by currently existing targeted therapies. Even though no recurrent mutations in driver genes were found in the different sarcoma groups, we show that targeted NGS-based sequencing is clearly feasible in a diagnostic setting with very limited amounts of paraffin embedded tissue and may provide novel insights into mesenchymal cell signalling and potentially druggable targets. Interestingly, we also identify five non-synonymous sequence variants in 4 established cancer driver genes in DNA

  8. Ulnar joint synovial sarcoma

    International Nuclear Information System (INIS)

    Statement of the diagnosis of synovial sarcoma in early period on the example of one patient is analyzed. It is too difficult to diagnose synovial sarcoma. Comprehensive X-ray ultrasonic examination is the most informative in detection of tumor. Surgical removal of revealed formation has been conducted to the patient. The formation is dense, joined to muscular fibers, is not connected with the joint. Histological investigation of the formation determines dissemination of malignant tumor, presented by round or oval cells. Tumor tissue is devided by thick fibrous layers, surrounded by fibrous capsula. The following conclusion is given: synovial sarcoma

  9. Primary renal synovial sarcoma

    OpenAIRE

    Bakhshi, Girish D.; Arshad S. Khan; Shaikh, Aftab S; Khan, Mohammad Ashraf A.; Mohammad Adil A. Khan; Jamadar, Nilofar M.

    2012-01-01

    Primary Renal Sarcoma is rare tumor comprising only 1% of all renal tumours. Synovial sarcomas are generally deep-seated tumors arising in the proximity of large joints of adolescents and young adults and account for 5-10% of all soft tissue tumours. Primary synovial sarcoma of kidney is rare and has poor prognosis. It can only be diagnosed by immunohistochemistry. It should be considered as a differential in sarcomatoid and spindle cell tumours. We present a case of 33-year-old female, who u...

  10. Classic Kaposi Sarcoma

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  11. Epidemic Kaposi Sarcoma

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  12. Primary renal synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Girish D. Bakhshi

    2012-03-01

    Full Text Available Primary Renal Sarcoma is rare tumor comprising only 1% of all renal tumours. Synovial sarcomas are generally deep-seated tumors arising in the proximity of large joints of adolescents and young adults and account for 5-10% of all soft tissue tumours. Primary synovial sarcoma of kidney is rare and has poor prognosis. It can only be diagnosed by immunohistochemistry. It should be considered as a differential in sarcomatoid and spindle cell tumours. We present a case of 33-year-old female, who underwent left sided radical nephrectomy for renal tumour. Histopathology and genetic analysis diagnosed it to be primary renal synovial sarcoma. Patient underwent radiation therapy and 2 years follow up is uneventful. A brief case report with review of literature is presented.

  13. Feline injection site sarcomas

    OpenAIRE

    Nóbrega, C.; Mesquita, Jr.; Cruz, R; C. Coelho; Esteves, F.; Mega, A. C.; Santos, C.; Vala, Helena

    2016-01-01

    Feline injection site sarcoma (FISS), formerly known as Vaccine Associated Sarcoma (VAS) is a rare, but life-threatening disease. The incidence estimates have varied from 1 case of FISS per 1,000-10,000 cats vaccinated in North America and between 1 per 5,000-12,500 vaccination visits in United Kingdom. It has been primarily associated with vaccine administration, but several other injectable materials/substances have been implicated as aetiologic agents, namely lufenuron, penicilin, meti...

  14. Chronic osteomyelitis mimicking sarcoma

    OpenAIRE

    Gulmann, C; Young, O.; Tolan, M.; O’Riordan, D.; Leader, M

    2003-01-01

    This report describes a rare case of chronic osteomyelitis in a 60 year old man mimicking a soft tissue sarcoma. Chronic osteomyelitis is an infrequent cause of a soft tissue mass and is usually diagnosed clinically by a combination of radiology and microbiology. Rarely, COM can mimic a primary bony neoplasm, but this is the first reported case where it mimicked a soft tissue sarcoma. The clinical, radiological, and histological appearances of this case will be discussed.

  15. Anti-Endosialin Antibody-Drug Conjugate: Potential in Sarcoma and Other Malignancies.

    Science.gov (United States)

    Rouleau, Cecile; Gianolio, Diego A; Smale, Robert; Roth, Stephanie D; Krumbholz, Roy; Harper, Jay; Munroe, Kenneth J; Green, Tessa L; Horten, Bruce C; Schmid, Steven M; Teicher, Beverly A

    2015-09-01

    Endosialin/TEM1/CD248 is a cell surface protein expressed at high levels by the malignant cells of about 50% of sarcomas and neuroblastomas. The antibody-drug conjugate (ADC) anti-endosialin-MC-VC-PABC-MMAE was selectively cytotoxic to endosialin-positive cells in vitro and achieved profound and durable antitumor efficacy in preclinical human tumor xenograft models of endosialin-positive disease. MC-VC-PABC-MMAE was conjugated with anti-endosialin with 3-4 MMAE molecules per ADC. The anti-endosialin-MC-VC-PABC-MMAE conjugate was tested for activity in four human cell lines with varied endosialin levels. The HT-1080 fibrosarcoma cells do not express endosialin, A-673 Ewing sarcoma cells and SK-N-AS neuroblastoma cells are moderate expressers of endosialin, and SJSA-1 osteosarcoma cells express very high levels of endosialin. To determine whether endosialin expression was maintained in vivo, A-673 Ewing sarcoma, SK-N-AS neuroblastoma, and SJSA-1 osteosarcoma cells were grown as xenograft tumors in nude mice. The SK-N-AS neuroblastoma and the A-673 Ewing sarcoma lines were selected for in vivo efficacy testing of the anti-endosialin-MC-VC-PABC-MMAE conjugate. The treatment groups included a vehicle control, unconjugated anti-endosialin, an admix control consisting of anti-endosialin and a dose of free MMAE equivalent to the dose administered as the ADC, and the anti-endosialin-MC-VC-PABC-MMAE conjugate. The unconjugated anti-endosialin had no antitumor activity and resulted in similar tumor growth as the vehicle control. The admix control produced a modest tumor growth delay. Administration of the anti-endosialin-MC-VC-PABC-MMAE conjugate resulted in a marked prolonged tumor response of both xenograts. These proof-of-concept results break new ground and open a promising drug discovery approach to these rare and neglected tumors. PMID:26184481

  16. Primary Intracranial Synovial Sarcoma

    Science.gov (United States)

    Li, Luyuan; Sinson, Grant; Mueller, Wade

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression.

  17. Primary Intracranial Synovial Sarcoma.

    Science.gov (United States)

    Patel, Mohit; Li, Luyuan; Nguyen, Ha Son; Doan, Ninh; Sinson, Grant; Mueller, Wade

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. PMID:27247811

  18. Primary Intracranial Synovial Sarcoma

    Directory of Open Access Journals (Sweden)

    Mohit Patel

    2016-01-01

    Full Text Available Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18(p11.2;q11.2 chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression.

  19. To Find a Safe Dose and Show Early Clinical Activity of Weekly Nab-paclitaxel in Pediatric Patients With Recurrent/ Refractory Solid Tumors

    Science.gov (United States)

    2016-08-10

    Neuroblastoma;; Rhabdomyosarcoma;; Ewing's Sarcoma;; Ewing's Tumor;; Sarcoma, Ewing's;; Sarcomas, Epitheliod;; Sarcoma, Soft Tissue;; Sarcoma, Spindle Cell;; Melanoma;; Malignant Melanoma;; Clinical Oncology;; Oncology, Medical;; Pediatrics, Osteosarcoma;; Osteogenic Sarcoma;; Osteosarcoma Tumor;; Sarcoma, Osteogenic;; Tumors;; Cancer;; Neoplasia;; Neoplasm;; Histiocytoma;; Fibrosarcoma;; Dermatofibrosarcoma

  20. Immunotherapeutic Intervention against Sarcomas

    Directory of Open Access Journals (Sweden)

    Paolo Pedrazzoli, Simona Secondino, Vittorio Perfetti, Patrizia Comoli, Daniela Montagna

    2011-01-01

    Full Text Available Advances in systemic therapy for sarcoma have produced, over the last two decades, relatively short-term benefits for the majority of patient. Among the novel biologic therapeutics that will likely increase our ability to cure human cancer in the years to come, immunotherapy is one of the most promising approaches. While past attempts to use immunotherapy have failed to dramatically shift the paradigm of care for the treatment of patients with sarcoma, major advances in basic and translational research have resulted, in more recent years, in clinical trial activity that is now beginning to generate promising results. However, to move from “proof of principle” to large scale clinical applicability, we need well-designed, multi-institutional clinical trials, along with continuous laboratory research to explore further the immunological characteristics of individual sarcoma subtypes and the consequent tailoring of therapy.

  1. Study the ovarian function in Awassi ewes

    International Nuclear Information System (INIS)

    An experiment was carried out on local Awassi ewes to study the reproductive performance, progesteron concentrations, accuracy of pregnacy diagnosis and other reproduction related criteria of Awassi ewes using radioimunoassay (RIA). A total of 18 nulliparous Awassi ewes, aged about 1 year and weighing on average 36.9 Kg was used. Live-weight changes were recorded weekly. Blood samples (5 ml) were collected from the jugular vein for about 1 year at 10.00h. Sera were prepared and stored at - 20 deg until assyed. The FAO/IAEA progesterone RIA kits were used. Two ewes aborted after about three-and-a-half months of pregnancy due to mechanical reasons. The remaining ewes (16) lambed between 30th November 1994 and 22nd February 1995. Sex ratio was 1:1 (8 males: 8 females). Average birth weights for both sexes was 5210 ± 525 g. Average birth-weight was higher in males than females. Average male birth-weights was 5338 ± 515 g, (4600 - 6000 g) whereas was 5083 ± 538 g, (4180 - 5700 g) for female lambs. Average basal pre-mating progesterone concentration was 0.60 ± 0.42 n mol/l (0-1.7 n mol/l). Average maximum progesterone concentration during the luteal phase in non-pregnant ewes was 11.1 ± 3.1 n mol/l (5.8 -18.00 n mol/l). Average maximum progesterone concentration during gestation was 58.4 ± 20.9 n mol/l (29.25 - 100.00 n mol/l). The accuracy of pregnancy diagnosis at around 21 days post mating using RIA was 100%. It is concluded that RIA could be used for progesterone determination in Awassi sheep successfully, and that more research work on other aspects of reproduction in Awassi sheep should be carried out. (Author). 29 refs., 47 tabs., 5 figs

  2. Intravascular synovial sarcoma.

    OpenAIRE

    Robertson, N. J.; Halawa, M H; Smith, M E

    1998-01-01

    A case of intravascular biphasic synovial sarcoma arising from the wall of the left femoral vein in a 34 year old woman is described. This is the third case of an intravascular synovial sarcoma known to be reported in the medical literature. The two previous cases arose from the left femoral vein and inferior vena cava in women of 34 and 31 years old, respectively. A characteristic clinical pattern appears to be emerging--that is, location in large veins of the lower extremity and trunk in yo...

  3. Definitive intraoperative radiotherapy for musculoskeletal sarcomas and malignant lymphoma in combination with surgical excision

    International Nuclear Information System (INIS)

    The purpose was to estimate retrospectively the outcome of patients with musculoskeletal sarcoma or malignant lymphoma treated with intraoperative radiotherapy (IORT). Between 1988 and 1999, definitive IORT in combination with surgical excision was performed in 24 patients with musculoskeletal sarcoma (malignant fibrous histiocytoma, 10; osteosarcoma, 6; liposarcoma, 2; chondrosarcoma, 1; synovial sarcoma, 1; Ewing's sarcoma, 1; angiosarcoma, 1; epithelioid sarcoma, 1; malignant schwannoma, 1; and 3 patients with malignant lymphoma. The tumor was excised by marginal margin excision, intralesional margin excision, or wide margin excision; 15-45 Gy electrons was then delivered to the affected sites. In the 8 patients without distant metastases at the first visit, 4 patients are alive 6.5-11.5 years after IORT, and 4 patients died <4.5 years after IORT. The incidence of local recurrence was 13%. In the 19 patients with distant metastases at the first visit, 3 patients are alive 2.5-6.7 years after IORT, and 16 patients died 0.2-5.7 years after IORT. The incidence of local recurrence was 45%. Complications after IORT were found in 5 patients: neuropathy, 1 patient; skin necrosis, 1 patient; myelopathy, 1 patient; enteritis ileus, 1 patient; and edema, 1 patient. IORT with a radiation dose of 15-45 Gy in combination with surgical excision appeared to be useful for local control and to be more effective in patients without distant metastases at the first visit than in patients with distant metastases. (author)

  4. Synovial sarcoma: current perspectives

    Directory of Open Access Journals (Sweden)

    Brennan B

    2016-04-01

    Full Text Available Bernadette Brennan Department of Paediatric Oncology, Royal Manchester Children's Hospital, Manchester, UK Abstract: Synovial sarcoma (SS is a distinct soft tissue sarcoma, occurring across all ages, from young children to the elderly, but the incidence of SS peaks in young adults. Recently, its biology, specifically the biomarker genomic index, may prove to be the most important prognostic marker, explaining findings such as the positive effect of younger age on outcome. While believed to be a chemosensitive soft tissue sarcoma, surgery remains the most important modality of treatment for many people, especially in localized disease, plus or minus radiotherapy to improve local control. Moving forward, the demonstration that SS has multiple therapeutic targets such as vascular endothelial growth factor, and new emerging targets, allows us to start to consider different systemic therapeutic interventions other than just chemotherapy. This is particularly important for advanced/metastatic SS which, with conventional chemotherapy only, continues to have a very poor outcome. The way forward, therefore, is an all-age trial combining perhaps anti-angiogenesis agents with chemotherapy, focusing on those SS cases whose outcome, as determined by biology, site, or metastatic status, is poor with just conventional sarcoma chemotherapy. This also allows prospective evaluation of the role of genomic index and other biomarkers. Keywords: children/adolescents, genomic index, targeted therapy, MAMS design

  5. Synovial sarcoma mechanisms

    DEFF Research Database (Denmark)

    Svejstrup, Jesper Q

    2013-01-01

    Human synovial sarcoma is caused by a chromosome translocation, which fuses DNA encoding SSX to that encoding the SS18 protein. Kadoch and Crabtree now show that the resulting cellular transformation stems from disruption of the normal architecture and function of the human SWI/SNF (BAF) complex....

  6. Immunotherapy of childhood Sarcomas

    Directory of Open Access Journals (Sweden)

    Stephen S Roberts

    2015-08-01

    Full Text Available Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing’s family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis. New therapies for these patients are urgently needed. Immunotherapy is an established treatment modality for both liquid and solid tumors, and in pediatrics, most notably for neuroblastoma and osteosarcoma. In the past, immunomodulatory agents such as interferon, interleukin-2, and Liposomal-muramyl  tripeptide phosphatidyl-ethanolamine (L-MTP have been tried, with some activity seen in subsets of patients; additionally, various cancer vaccines have been studied with possible benefit. Monoclonal antibody therapies against tumor antigens such as disialoganglioside GD2 or immune checkpoint targets such as CTLA4 and PD-1 are being actively explored in pediatric sarcomas. Building on the success of adoptive T cell therapy for EBV-related lymphoma, strategies to redirect T cells using chimeric antigen receptors and bispecific antibodies are rapidly evolving with potential for the treatment of sarcomas. This review will focus on recent preclinical and clinical developments in targeted agents for pediatric sarcomas with emphasis on the immunobiology of immune checkpoints, immunoediting, tumor microenvironment, antibody engineering, cell engineering, and tumor vaccines. The future integration of antibody based and cell based therapies into an overall treatment strategy of sarcoma will be discussed.

  7. Ewe characteristics associated with neonatal loss in Norwegian sheep.

    Science.gov (United States)

    Holmøy, Ingrid H; Waage, Steinar; Gröhn, Yrjö T

    2014-06-01

    A case-control study was conducted to identify ewe characteristics that affect the risk of a ewe losing at least one lamb during the first 5 days post lambing. Data were from a national sheep registry, and only ewes that lambed in the spring of 2010 belonging to flocks that reported disease events were included. Ewes registered with abortion or stillbirth were excluded. Cases (n=4850) and controls (n=85,354) from 1153 flocks were studied using logistic regression models, accounting for within flock correlation. The odds of losing at least one lamb increased substantially when litter size exceeded two. For example, in 3-year-old ewes, the odds were 6 times greater for those with 3 lambs than for those with 1 lamb. However, the effect of litter size depended on the age of the ewe; for example for ewes giving birth to triplet lambs, the odds of losing at least one lamb were 2.7 times greater in 1-year-old ewes than in 3-year-old ewes. Dystocia was associated with increased risk of losing at least one lamb, but the effect varied by litter size. In ewes with single lambs, the odds of lamb loss were 5 times greater in those that experienced dystocia than in those that did not, while within subgroups of ewes with twins, triplets or >3 lambs, the corresponding odds ratio (OR) of losing one or more lambs was 2.2, 1.5 and 1.3, respectively. Compared with ewes of the Norwegian White breed, ewes of old Norwegian breeds were less likely to lose lambs (OR=0.8). We also examined the effects of several diseases experienced by the ewe during pregnancy or shortly postpartum on the risk of subsequent neonatal lamb loss. Significantly increased risk was found for ewes with abdominal hernia (OR=2.5) and for ewes treated for moderate to severe clinical mastitis (OR=1.6) when compared with ewes without these disorders. In conclusion, our large study population allowed for a detailed analysis of the combined effect of important ewe factors that affected survival of their lambs in the

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

    International Nuclear Information System (INIS)

    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. 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. 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 γ-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. 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

  9. Thyroid Synovial Sarcoma: A Case Report

    OpenAIRE

    Ali Ghafouri; Taha Anbara; Ali Mir; Marzieh Lashkari; Mohammad Nazari

    2013-01-01

    Sarcomas are rare tumors account for about 1% of cancers in adult. Soft tissue sarcomas are the most common one. Synovial sarcoma's incident is about 10% of all sarcomas and most commonly rise from para-articular regions in young adults. Based on our knowledge there have been only two reports of thyroid synovial sarcoma in medical literature. We report a 44-year old woman presented with a rapid growing neck mass. The pathology report revealed sarcoma and the immunohistochemistry (IHC) was com...

  10. Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma

    Science.gov (United States)

    2016-03-07

    Sarcoma, Soft Tissue; Soft Tissue Sarcoma; Undifferentiated Pleomorphic Sarcoma; Leiomyosarcoma; Liposarcoma; Synovial Sarcoma; Myxofibrosarcoma; Angiosarcoma; Fibrosarcoma; Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma

  11. Paget's sarcoma of bone

    International Nuclear Information System (INIS)

    The most sinister complication of Paget's disease is malignant degeneration. The true etiology of Paget's disease remains unproven and unknown. The relative incidence of sarcoma ranges from 0.9% to 20% depending upon the population source. The anatomical distribution of lesions is similar to that of uncomplicated Paget's disease except for the high incidence in the humerus and rare occurrence in the spine. Approximately 50% of the lesions histologically represent osteosarcomas, with fibrosarcoma, fibrous histiocytoma, chondrosarcoma and giant cell tumor rarely occurring. The radiographic presentation is most commonly lytic, but sclerotic and mixed patterns occur. Cortical destruction and soft tissue mass are the cardinal radiographic signs. Malignant periosteal response is seldom found associated with Paget's sarcoma. The most common sites are femur, humerus, pelvis, skull and tibia. The prognosis is fatal at a very rapid rate. There are but a few isolated cases of long-term survival reported. (orig.)

  12. Radiotherapy and high-dose chemotherapy in advanced Ewing's tumors

    Energy Technology Data Exchange (ETDEWEB)

    Pape, H.; Glag, M.; Gripp, S.; Wittkamp, M.; Schmitt, G. [Duesseldorf Univ. (Germany). Klinik und Poliklinik fuer Strahlentherapie und Radiologische Onkologie; Laws, H.J.; Kaik, B. van; Goebel, U. [Duesseldorf Univ. (Germany). Abt. Paediatrische Haematologie und Onkologie; Burdach, S. [Halle Univ. (Germany). Abt. Paediatrie; Juergens, H. [Muenster Univ. (Germany). Abt. Paediatrische Hematologie und Onkologie

    1999-10-01

    Background: Ewing's tumors are sensitive to radio- and chemotherapy. Patients with multifocal disease suffer a poor prognosis. Patients presenting primary bone marrow involvement or bone metastases at diagnosis herald a 3-year disease-free survival below 15%. The European Intergroup Cooperative Ewing's Sarcoma Study (EICESS) has established the following indications for high-dose therapy in advanced Ewing's tumors: Patients with primary multifocal bone disease, patients with early (<2 years after diagnosis) or multifocal relapse. Patients and Method: As of 1987, 83 patients have been treated in the EICESS group, 39 of them at the transplant center in Duesseldorf, who have been analyzed here. All individuals received 4 courses of induction chemotherapy with EVAJA and stem cell collection after course 3 and 4. Consolidation radiotherapy of the involved bone compartments was administered in a hyperfractionated regimen 2 times 1.6 Gy per day, up to 22.4 Gy simultaneously to course 5 and 22.4 Gy to course 6 of chemotherapy. The myeloablative chemotherapy consisted of melphalan and etoposide (ME) in combination with 12 Gy TBI (Hyper-ME) oder Double-ME with whole lung irradiation up to 18 Gy (without TBI). Results: The survival probability at 40 months was 31% (44% DOD; 15% DOC). Pelvic infiltration did not reach prognostic relevance in this cohort. Radiotherapy encompassed 75% of the bone marrow at maximum (average 20%). Engraftment was not affected by radiotherapy. Conclusion: High-dose chemotherapy can improve outcome in poor prognostic advanced Ewing's tumors. The disease itself remains the main problem. The expected engraftment problems after intensive radiotherapy in large volumes of bone marrow can be overcome by stem cell reinfusion. (orig.) [German] Hintergrund: Ewing-Tumoren sind radio- und chemosensibel. Im metastasierten Stadium ist die Prognose schlecht. Patienten mit Knochen- oder Knochenmarkinfiltration haben nach drei Jahren eine

  13. Immunotherapy of Childhood Sarcomas

    OpenAIRE

    Roberts, Stephen S.; Chou, Alexander J.; Cheung, Nai-Kong V.

    2015-01-01

    Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing’s family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis....

  14. Ewe: a computer model for ultrasonic inspection

    International Nuclear Information System (INIS)

    The computer program EWE simulates the propagation of elastic waves in solids and liquids. It has been applied to ultrasonic testing to study the echoes generated by cracks and other types of defects. A discussion of the elastic wave equations is given, including the first-order formulation, shear and compression waves, surface waves and boundaries, numerical method of solution, models for cracks and slot defects, input wave generation, returning echo construction, and general computer issues

  15. Comparison of seasonal effects on some hematological and biochemical parameters between ewes with subclinical mastitis and healthy ewes

    OpenAIRE

    R.N. Sani; M. Moezifar

    2016-01-01

    This study was conducted to compare the effects of season on some hematological and biochemical parameters in ewes with subclinical mastitis and healthy ewes. A total of 1192 milk and blood samples were collected from ewes between February and April (791 samples in spring), August and October (401 samples in summer). California Mastitis Test (CMT), milk culture, hematologic and serum biochemical parameters were obtained from the medical records. Samples which were positive by bacterial cultur...

  16. Soft tissue sarcoma subtypes exhibit distinct patterns of acquired uniparental disomy

    Directory of Open Access Journals (Sweden)

    Tuna Musaffe

    2012-12-01

    Full Text Available Abstract 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.

  17. Pan-European Sarcoma Trials: Moving Forward in a Climate of Increasing Economic and Regulatory Pressure

    Directory of Open Access Journals (Sweden)

    Dorothe Carrle

    2007-06-01

    Full Text Available Advances in sarcoma treatment are largely based on investigator-initiated, multicentric and interdisciplinary clinical trials. The EU's Good Clinical Practice Directive 2001/20/EC, effective since 2004, was meant to harmonize the conditions for clinical trials across Europe, but, instead, the challenge of initiating and running multinational, noncommercial clinical trials has become greater than ever. Institutions participating in existing noncommercial Pan-European studies are struggling to cope with increased administrative and financial burdens, and few new studies are initiated any more. The aim of a conference entitled “Pan-European Sarcoma Trials: Moving Forward in a Climate of Increasing Economic and Regulatory Pressure,” held in Stuttgart, Germany, 30 November–2 December 2006 as part of the European Science Foundation's ECT-program, was not only to provide an overview of currently active and planned multinational studies on osteo-, Ewing's, and soft tissue sarcoma, but also to draw on areas of synergy between various established sarcoma groups in Europe to define plausible survival strategies for collaborative, interdisciplinary, patient-oriented research.

  18. Sarcoma risk after radiation exposure

    Directory of Open Access Journals (Sweden)

    Berrington de Gonzalez Amy

    2012-10-01

    Full Text Available Abstract Sarcomas were one of the first solid cancers to be linked to ionizing radiation exposure. We reviewed the current evidence on this relationship, focusing particularly on the studies that had individual estimates of radiation doses. There is clear evidence of an increased risk of both bone and soft tissue sarcomas after high-dose fractionated radiation exposure (10 + Gy in childhood, and the risk increases approximately linearly in dose, at least up to 40 Gy. There are few studies available of sarcoma after radiotherapy in adulthood for cancer, but data from cancer registries and studies of treatment for benign conditions confirm that the risk of sarcoma is also increased in this age-group after fractionated high-dose exposure. New findings from the long-term follow-up of the Japanese atomic bomb survivors suggest, for the first time, that sarcomas can be induced by acute lower-doses of radiation (

  19. Retroperitoneal synovial sarcoma presenting as paraneoplastic hypoglycaemia

    Directory of Open Access Journals (Sweden)

    Reddy VV

    2015-01-01

    Full Text Available Synovial sarcoma is a well-recognized malignant mesenchymal neoplasm. Primary retroperitoneal synovial sarcoma is extremely rare and has poor prognosis. There are sparse reports in the literature on the secretory synovial sarcomas. In this report, we present the case of a patient with retroperitoneal synovial sarcoma who presented with recurrent attacks of hypoglycaemia.

  20. Retroperitoneal synovial sarcoma presenting as paraneoplastic hypoglycaemia

    OpenAIRE

    Reddy VV; Sarala S; Mathai V; Madhu; Sreedhar Babu KV

    2015-01-01

    Synovial sarcoma is a well-recognized malignant mesenchymal neoplasm. Primary retroperitoneal synovial sarcoma is extremely rare and has poor prognosis. There are sparse reports in the literature on the secretory synovial sarcomas. In this report, we present the case of a patient with retroperitoneal synovial sarcoma who presented with recurrent attacks of hypoglycaemia.

  1. [Synovial sarcoma. Case report].

    Science.gov (United States)

    Deme, Dániel; Abdulfatah, Bishr; Telekes, András

    2016-02-01

    In 2013 there were 94,770 new cancer patients reported in Hungary. Synovial sarcoma accounts for 0.05-0.1% of all cancers and, therefore its incidence is predicted to be 47-94 patients/year in Hungary. The authors report the history of a 18-year-old man who was operated on a right upper abdominal wall tumor with R1 resection. During the next 5 months the tumor grew up to 8 cm in largest diameter. Histology revealed monophasic synovial sarcoma. Immunohistochemistry showed bcl2, focal CD99 and high molecular weight cytokeratin positivity, while smooth muscle actin, S100 and CD34 immunostainings were negative. Becose of this reoperation was not possible, curative six cycles of doxorubicine and ifosfamide with granulocyte colony stimulating factor support and 60 Gy radiotherapy was given to the tumor bed. After these treatments computed tomography scan was negative and the patient attended regular imaging every 3 months. At the age of 20 years the patient developed two neoplastic lesions in the surgical scar measuring 10 mm and 45 × 10 mm in size. R0 resection, partial rib resection and abdominal wall reconstruction were performed. Histology confirmed residual monophasic synovial sarcoma. Radiotherapy was not given because of a risk of intestinal wall perforation. Staging positron emission tomography-computed tomography proved to be negative. At the age of 22 years magnetic resonance imaging scans indicated no tumor recurrence, but after one month a rapidly growing tumorous lesion was found on ultrasound in the surgical scar measuring 20 × 20 × 12 mm in size. Cytology confirmed local recurrence and fluorescence in situ hibridization indicated t(x;18). R0 exstirpation and partial mesh resection were performed and histology showed the same monophasic synovial sarcoma. Because of the presence of vascular invasion and a close resection margin (1 mm) the patient underwent 3 cycles of adjuvant chemotherapy (doxorubicine and ifosfamide) with granulocyte colony stimulating

  2. Synovial Sarcoma: Laryngopharynx a Challenge

    OpenAIRE

    Verma, Ravinder; Verma, Ravneet Ravinder; Verma, Rohan Ravinder; Sardana, N. K.

    2013-01-01

    Synovial sarcoma is a rare malignant tumor. It derives from a mesenchymal precursor stem cell that is unrelated to mature synovial tissue. Synovial sarcoma classically affects lower limbs between the ages of 15 and 40 years and the proportion of male-to-female patients is 3:2. It is very rare in the head and neck region especially in laryngopharynx. Till date, only six cases of synovial sarcoma involving laryngopharynx have been reported in the English literature. Painless mass, hoarseness, u...

  3. An integrated analysis of miRNA and gene copy numbers in xenografts of Ewing's sarcoma

    Directory of Open Access Journals (Sweden)

    Mosakhani Neda

    2012-03-01

    Full Text Available Abstract Background Xenografts have been shown to provide a suitable source of tumor tissue for molecular analysis in the absence of primary tumor material. We utilized ES xenograft series for integrated microarray analyses to identify novel biomarkers. Method Microarray technology (array comparative genomic hybridization (aCGH and micro RNA arrays was used to screen and identify copy number changes and differentially expressed miRNAs of 34 and 14 passages, respectively. Incubated cells used for xenografting (Passage 0 were considered to represent the primary tumor. Four important differentially expressed miRNAs (miR-31, miR-31*, miR-145, miR-106 were selected for further validation by real time polymerase chain reaction (RT-PCR. Integrated analysis of aCGH and miRNA data was performed on 14 xenograft passages by bioinformatic methods. Results The most frequent losses and gains of DNA copy number were detected at 9p21.3, 16q and at 8, 15, 17q21.32-qter, 1q21.1-qter, respectively. The presence of these alterations was consistent in all tumor passages. aCGH profiles of xenograft passages of each series resembled their corresponding primary tumors (passage 0. MiR-21, miR-31, miR-31*, miR-106b, miR-145, miR-150*, miR-371-5p, miR-557 and miR-598 showed recurrently altered expression. These miRNAS were predicted to regulate many ES-associated genes, such as genes of the IGF1 pathway, EWSR1, FLI1 and their fusion gene (EWS-FLI1. Twenty differentially expressed miRNAs were pinpointed in regions carrying altered copy numbers. Conclusion In the present study, ES xenografts were successfully applied for integrated microarray analyses. Our findings showed expression changes of miRNAs that were predicted to regulate many ES associated genes, such as IGF1 pathway genes, FLI1, EWSR1, and the EWS-FLI1 fusion genes.

  4. Reconstruction of the elbow and forearm for Ewing sarcoma of ulna: A new biological technique

    Science.gov (United States)

    Puri, Ajay; Gulia, Ashish; Byregowda, Suman; Ramanujan, Vishnu

    2016-01-01

    Primary bone tumors around the elbow represent ulnar synostosis with the creation of a single bone forearm. We attempted to achieve a mobile elbow and stable wrist joint with the radio-ulnar union at the proximal articular surface of the ulna resulting in a single bone forearm. The procedure involves an oblique osteotomy preserving the olecranon process (after taking adequate margins based on oncological principles) and its articular cartilage along with the attachment of the triceps tendon. Then the radial head was partially denuded of its cartilage using a burr, leaving cartilage only on the volar side, and then fused to the remnant olecranon. Osteosynthesis was done using compression screw and tension band wiring. The advantages of this procedure are that the mobility at wrist and elbow are retained, it requires minimal hardware and allows for primary closure of the wound.

  5. Gene Expression Music Algorithm-Based Characterization of the Ewing Sarcoma Stem Cell Signature

    OpenAIRE

    Staege, Martin Sebastian

    2016-01-01

    Gene Expression Music Algorithm (GEMusicA) is a method for the transformation of DNA microarray data into melodies that can be used for the characterization of differentially expressed genes. Using this method we compared gene expression profiles from endothelial cells (EC), hematopoietic stem cells, neuronal stem cells, embryonic stem cells (ESC), and mesenchymal stem cells (MSC) and defined a set of genes that can discriminate between the different stem cell types. We analyzed the behavior ...

  6. Cutaneous Ewing's sarcoma secondary to chemotherapy given for testis tumor: Case report

    Directory of Open Access Journals (Sweden)

    Serhat Tanık

    2014-01-01

    CONCLUSION: Secondary tumors can emerge months or years after primary tumor therapies, and are not related with the primary tumors. Any lesion or sign should be investigated carefully. Early diagnosis and correct treatment could prevent dramatic results.

  7. Treatment Options for Kaposi Sarcoma

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  8. General Information about Kaposi Sarcoma

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  9. Treatment Option Overview (Kaposi Sarcoma)

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  10. Post irradiation maxillary sinus sarcoma

    International Nuclear Information System (INIS)

    Radiation-induced cancer of the head and neck is well known, but radiation-induced sarcoma is rare. Only nine cases have been reported in the Japanese literature. We treated a patient with post irradiation sarcoma arising in the maxillary sinus. She is now 60 years old and had received radiation therapy for left maxillary carcinoma in 1952. In 1972, left maxillary sarcoma was found and she received postoperative radiation therapy again. Then right nasal carcinoma was found in 1980, and she again received postoperative radiation therapy. Then left maxillary sarcoma was found in 1992. There have been no previous reports of three consecutive radiation-induced malignancies developing in a patient. (author)

  11. Drugs Approved for Kaposi Sarcoma

    Science.gov (United States)

    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.

  12. Can Kaposi Sarcoma Be Prevented?

    Science.gov (United States)

    ... KS) is caused by the Kaposi sarcoma associated herpesvirus (KSHV). There are no vaccines at this time ... HIV-infected people who take drugs to treat herpesvirus infections (such as ganciclovir or foscarnet) are less ...

  13. Trabectedin in Soft Tissue Sarcomas

    OpenAIRE

    Petek, Bradley J.; Loggers, Elizabeth T.; Pollack, Seth M.; Jones, Robin L.

    2015-01-01

    Soft tissue sarcomas are a group of rare tumors derived from mesenchymal tissue, accounting for about 1% of adult cancers. There are over 60 different histological subtypes, each with their own unique biological behavior and response to systemic therapy. The outcome for patients with metastatic soft tissue sarcoma is poor with few available systemic treatment options. For decades, the mainstay of management has consisted of doxorubicin with or without ifosfamide. Trabectedin is a synthetic ag...

  14. Synovial sarcoma--a misnomer.

    OpenAIRE

    Miettinen, M.; Virtanen, I

    1984-01-01

    For an evaluation of the putative histogenetic relationship of synovia and synovial sarcomas, normal synovia, villonodular synovitis, and synovial sarcomas were compared for their patterns of expression of intermediate filaments of keratin and vimentin type and epithelial membrane antigen and for lectin binding sites. The lining cells in both normal synovia and villonodular synovitis reacted with anti-vimentin antibodies, but not with antibodies to different types of keratins or epithelial me...

  15. Synovial sarcoma in childhood

    International Nuclear Information System (INIS)

    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

  16. Primary renal synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Vandana U Grampurohit

    2011-01-01

    Full Text Available Primary synovial sarcoma (SS of kidney is very rare and difficult to diagnose. Here, we present a case of a 21-year-old female clinically diagnosed as renal cell carcinoma. Right nephrectomy specimen showed a cystic tumor in the upper pole of kidney with areas of hemorrhage and solid growth. Histologically, it showed poorly differentiated cells with hemangiopericytoma-like vascular pattern. Morphologic and immunohistochemical features were compatible with the diagnosis of poorly differentiated SS of kidney. Primary renal SS is a recently described entity. To the best of our knowledge, approximately 34 cases have been reported till date and this is the eighth documented case of poorly differentiated variant. Most of the time, poorly differentiated SS of kidney exhibits hemangiopericytoma like histology. Reverse transcriptase-polymerase chain reaction analysis to demonstrate SYT-SSX fusion gene transcript helps to confirm the diagnosis.

  17. Synovial sarcoma in childhood

    Energy Technology Data Exchange (ETDEWEB)

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

    1984-04-01

    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.

  18. Clear cell sarcoma

    Directory of Open Access Journals (Sweden)

    Pinar Ozuguz

    2014-01-01

    Full Text Available 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.

  19. Radiosensitivity of soft tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Hirano, Toru; Iwasaki, Katsuro; Suzuki, Ryohei; Monzen, Yoshio; Hombo, Zenichiro

    1989-05-01

    The correlation between the effectiveness of radiation therapy and the histology of soft tissue sarcomas was investigated. Of 31 cases with a soft tissue sarcoma of an extremity treated by conservative surgery and postoperative radiation of 3,000-6,000 cGy, local recurrence occurred in 12; 5 out of 7 synovial sarcomas, 4 of 9 MFH, one of 8 liposarcomas, none of 4 rhabdomyosarcomas and 2 of 3 others. As for the histological subtyping, the 31 soft tissue sarcomas were divided into spindle cell, pleomorphic cell, myxoid and round cell type, and recurrence rates were 75%, 33.3%, 16.7% and 0%, respectively. From the remarkable difference in recurrent rate, it was suggested that round cell and myxoid type of soft tissue sarcomas showed a high radiosensitivity compared to the spindle cell type with low sensitivity. Clarifying the degree of radiosensitivity is helpful in deciding on the management of limb salvage in soft tissue sarcomas of an extremity. (author).

  20. A case report of CIC-rearranged undifferentiated small round cell sarcoma in the cerebrum.

    Science.gov (United States)

    Ito, Mayumi; Ishikawa, Misawo; Kitajima, Masateru; Narita, Jun; Hattori, Shinya; Endo, Otone; Goto, Keisuke

    2016-10-01

    CIC-rearranged undifferentiated small round cell sarcoma (CIC-rearranged USRCS) is a recently established type of Ewing-like small round cell sarcomas, characterized by CIC gene rearrangement, most commonly CIC-DUX4 fusion. This report presents the second case of CIC-rearranged USRCS arising primarily in the cerebrum. A 64-year-old otherwise healthy woman presented with a 1 × 1 cm sized hemorrhagic subcortical tumor in the left temporo-parietal lobe. The tumor repeatedly recurred, and the patient underwent three surgeries, chemotherapy with doxorubicin and ifosfamide, and radiotherapy, as well as gamma knife surgery. Systemic examination revealed no other extracranial masses. Imprint cytology revealed small to moderate-sized round-to-ovoid tumor cells with mild pleomorphism and variations in size and shape. The nuclei contained finely granular chromatin, and some had easily-recognizable nucleoli. The tumor exhibited a mainly cytoplasmic pattern of CD99 immunostaining, rather than a diffuse membranous pattern. The tumor also exhibited diffuse positivity for calretinin and p16, as well as partial positivity for WT1 (nuclear and cytoplasmic staining pattern) and D2-40. FISH assessment showed CIC split signals. In conclusion, CIC-rearranged USRCSs can occur primarily in the cerebrum. It would be impossible to diagnose them through cytology alone, but cytology would be useful to rule out other small round cell brain tumors including gliomas, lymphomas, carcinomas, and germinoma. Immunohistochemical analysis including tests for CD99, calretinin, and WT1 would help to suggest CIC-rearranged USRCSs and distinguish them from Ewing sarcomas. Additionally, immunohistochemistry for p16 might be useful in the diagnosis. Diagn. Cytopathol. 2016;44:828-832. © 2016 Wiley Periodicals, Inc. PMID:27324529

  1. Effects of Different Progesterone Plugs on Estrous Control in Ewe

    Institute of Scientific and Technical Information of China (English)

    WU Hongda

    2009-01-01

    In breeding season and non-breeding season, the effects of three kinds of progesterone plugs on estrous control in ewes were studied. Meanwhile, the advantages and the disadvantages of the progesterone plugs were analyzed. The results showed that there were no significant difference among three progesterone plugs on modulating the ewes' estrus and pregnancy, and affecting estrous rate and fecundation rate (P>0.05). It was observed that the progesterone plug produced by Animal Husbandry Research Center of Heilongjiang Academy of Agricultural Sciences could modulate the ewes' estrus and pregnancy effectively, and the low cost and convenience in operation made it popularize in the reproducation of sheep widely.

  2. Genetic Parameters of Some Economic Traits in Arabi Ewes

    OpenAIRE

    S F. Al-Dabbagh; A K.Mohammed; R K.Abdulla

    2013-01-01

    Data were analyzed of Arabi ewes flock (153) within productive years 1990-1999 to estimate the overall mean and genetic parameters which are heritability, repeatability, genetic and phenotypic correlations for fertility, litter size, ewes weight, lambs birth weights, milk and wool yield for this breed. The overall mean for fertility, litter size, ewes weight, lambs birth weights, daily milk yield and greasy fleece weight were 70.07%, 1.14, 50.24 kg, 4.52 kg, 0.59 kg and 1.67 kg respectively....

  3. Comparison of seasonal effects on some hematological and biochemical parameters between ewes with subclinical mastitis and healthy ewes

    Directory of Open Access Journals (Sweden)

    R.N. Sani

    2016-06-01

    Full Text Available This study was conducted to compare the effects of season on some hematological and biochemical parameters in ewes with subclinical mastitis and healthy ewes. A total of 1192 milk and blood samples were collected from ewes between February and April (791 samples in spring, August and October (401 samples in summer. California Mastitis Test (CMT, milk culture, hematologic and serum biochemical parameters were obtained from the medical records. Samples which were positive by bacterial culture and CMT as were deemed to have glands with subclinical mastitis (SCM. The prevalence of subclinical mastitis in our study was around 12% (in one year. Of 144 (18.2% and 47 (11.72% positive samples in CMT, 134 (16.94% and 44 (10.97% samples were positive for bacterial culture in spring and summer, respectively. Comparison of results of hematological and serum biochemical parameters in ewes with SCM and healthy ewes in spring and summer showed that WBC counts, total protein concentrations and plasma fibrinogen in both groups of ewes were significantly higher in spring than summer (P˂0.05. The results of the present study indicated that when interpreting hematological and serum biochemical parameters in ewes with SCM should be consider the effect of season on these parameters.

  4. Comparison of seasonal effects on some hematological and biochemical parameters between ewes with subclinical mastitis and healthy ewes

    Directory of Open Access Journals (Sweden)

    R.N. Sani

    2016-04-01

    Full Text Available This study was conducted to compare the effects of season on some hematological and biochemical parameters in ewes with subclinical mastitis and healthy ewes. A total of 1192 milk and blood samples were collected from ewes between February and April (791 samples in spring, August and October (401 samples in summer. California Mastitis Test (CMT, milk culture, hematologic and serum biochemical parameters were obtained from the medical records. Samples which were positive by bacterial culture and CMT as were deemed to have glands with subclinical mastitis (SCM. The prevalence of subclinical mastitis in our study was around 12% (in one year. Of 144 (18.2% and 47 (11.72% positive samples in CMT, 134 (16.94% and 44 (10.97% samples were positive for bacterial culture in spring and summer, respectively. Comparison of results of hematological and serum biochemical parameters in ewes with SCM and healthy ewes in spring and summer showed that WBC counts, total protein concentrations and plasma fibrinogen in both groups of ewes were significantly higher in spring than summer (P˂0.05. The results of the present study indicated that when interpreting hematological and serum biochemical parameters in ewes with SCM should be consider the effect of season on these parameters.

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

    Science.gov (United States)

    2014-05-07

    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

  6. Management of pulmonary Kaposi's sarcoma: new perspectives

    International Nuclear Information System (INIS)

    Pulmonary Kaposi's sarcoma (KS) is a frequent complication of human immunodeficiency virus infection. This article focuses on the current approach to diagnosis and treatment of Kaposi's sarcoma involving the respiratory system. (author). 27 refs., 3 figs., 5 tabs

  7. Pericardial Synovial Sarcoma: A Rare Clinical Entity.

    Science.gov (United States)

    Goldblatt, Joshua; Saxena, Pankaj; McGiffin, David C; Zimmet, Adam

    2015-11-01

    Synovial sarcoma is an extremely rare form of primary malignancy of the pericardium. We present a case of primary synovial sarcoma of the pericardium followed by a review of the literature. PMID:26347295

  8. Treatment Options by Stage (Uterine Sarcoma)

    Science.gov (United States)

    ... Cancer Prevention Endometrial Cancer Screening Research Uterine Sarcoma Treatment (PDQ®)–Patient Version General Information About Uterine Sarcoma ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  9. Soft Tissue Sarcomas and Agent Orange

    Science.gov (United States)

    ... ZIP code here Soft Tissue Sarcomas and Agent Orange VA presumes some soft tissue sarcomas in Veterans are related to their exposure to Agent Orange or other herbicides during military service. The soft ...

  10. Soft tissue sarcomas of the kidney

    OpenAIRE

    Olivia Köhle; Dominik Abt; Christian Rothermundt; Christian Öhlschlegel; Christiane Brugnolaro; Hans-Peter Schmid

    2015-01-01

    Soft tissue sarcomas are rare mesenchymal tumors. Amongst others, primitive neuroectodermal tumors (PNET) of the kidney and synovial sarcoma of the kidney belong to the group of soft tissue sarcomas. Synovial sarcomas can occur almost anywhere in the body, most frequently, however, in the lower (62%) or upper extremities (21%). Metastases occur in 50-70% of cases, and thus the prognosis is poor. PNETs are rare, highly aggressive neoplastic lesions which mainly occur in the torso or axial skel...

  11. Synovial sarcoma of the sellar region

    OpenAIRE

    Scheithauer, BW; Silva, AI; Kattner, K; Seibly, J; Oliveira, AM; Kovacs, K.

    2007-01-01

    Primary sarcomas of the sellar region are uncommon, although a wide variety have been reported. To date, no cases of primary synovial sarcoma have been described as occurring at this site. We report an immunohistochemically and molecular genetically confirmed primary synovial sarcoma involving the sellar/parasellar region and cavernous sinus in an adult male. Subtotal resection and radiosurgery proved to be efficacious. The spectrum of primary sellar region sarcomas is summarized.

  12. Primary clear cell sarcoma of bone

    International Nuclear Information System (INIS)

    Clear cell sarcoma is a rare soft tissue sarcoma of young adults with melanocytic differentiation. It occurs predominantly in the soft tissue of extremities, typically involving tendons and aponeuroses. Primary clear cell sarcoma of bone is extremely rare. We report a case of primary clear cell sarcoma of the right first metatarsal in a 48-year-old woman and provide a literature review of the entity. (orig.)

  13. Management of high-grade bone sarcomas over two decades: The Norwegian Radium Hospital experience

    Energy Technology Data Exchange (ETDEWEB)

    Hege Aksnes, Liv; Sundby Hall, Kirsten; Folleraas, Gunnar; Stenwig, Anna Elisabeth; Bjerkehagen, Bodil; Taksdal, Ingeborg; Winderen, Mette; Bruland, Oeyvind Sverre; Saeter, Gunnar [The Norwegian Radium Hospital, Montebello, Oslo (Norway)

    2006-02-15

    All cases of high-grade osteosarcoma (OS) (n=196) and Ewing's sarcoma of bone (ES) (n=56) treated at the Norwegian Radium Hospital in the period 1980-1999 were analyzed retrospectively. They were allocated to consecutive ten-year periods by their time of diagnosis. Patient and tumour characteristics have been relatively stable. Eighty percent of all patients received surgical treatment and the amputation rate decreased from 64% to 23%. The percentage of patients receiving chemotherapy has remained around 80%. The use of radiotherapy in primary treatment decreased gradually from 33% to 18%. Sarcoma specific survival (SSS) at five years for all patients increased significantly from 39% to 53%. Similar trends for improvement were seen for both OS and ES. In multivariate analysis, independent prognostic factors for improved SSS were non-metastatic disease at diagnosis, age under 40, extremity tumours, small tumours and treatment from 1995 onwards. No major new treatment options have emerged over these 20 years. The improved outcome appears partly to be due to refinements in the use of existing modalities and improved quality and integration of multidisciplinary approaches. Improved formalized organisation of the sarcoma group and annual audited reports of its patient and research activity may also have contributed to improved focus and performance.

  14. MR criteria for differentiation of 'pseudo tumourous' lesions from bone sarcomas of the extremities. MRT-Kriterien zur Differenzierung 'pseudotumoroeser' Laesionen von Knochensarkomen der Extremitaeten

    Energy Technology Data Exchange (ETDEWEB)

    Lehner, K. (Technische Univ. Muenchen, Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik (Germany)); Rechl, H. (Technische Univ. Muenchen, Klinikum rechts der Isar, Orthopaedische Klinik (Germany)); Daschner, H. (Technische Univ. Muenchen, Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik (Germany)); Kutschker, C. (Technische Univ. Muenchen, Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik (Germany))

    1993-05-01

    The MRT scans of 57 patients with Ewing or osteosarcomas and 34 patients with haematogenous osteomyelitis or periostitis/stress fractures were examined in order to determine whether a distinction between benign or malignant lesions is possible. Four criteria were evaluated: The margin of the bone marrow component; intensity and homogeneity of the T[sub 1]-weighted signal in the bone marrow; presence of an extraosseus structured soft tissue mass and/or soft tissue edema. It was found that central osteosarcomas and Ewing's sarcomas reduced signal intensity of the marrow to become muscle-isointense with a well defined margin. In acute haematogenous osteomyelitis and periostitis/stress fracture the marrow lesion was not sharply demarcated. In contrast to patients with bone sarcomas only one case of osteomyelitis showed an extrosseus structured soft tissue mass. On the basis of these findings we believe that acute haematogenous osteomyelitis can be distinguished with high degree of accuracy from Ewing's sarcoma and central osteosarcomas. (orig.)

  15. Hormonal levels after ovarian X-irradiation of ewes

    International Nuclear Information System (INIS)

    The effects of X-irradiation on ovulation rate, cyclicity and progesterone and FSH levels in Ile-de-France ewes (4 control and 16 irradiated) after they were treated during the breeding season were reported. The doses used (800 and 2400 R) destroyed 50% of all size classes of the follicular population. Ovulation occurred in 87% of the treated ewes (ovulation rate = 1) when they were irradiated 24 h after luteolysis; 78% of the corpora lutea resulting from these ovulations were normal as to length and progesterone production. FSH in treated ewes started to increase 20 h after treatment and remained higher than in the controls until ovulation time. Later, while these levels were similar between groups on the day of ovulation, high ovulatory levels persisted in irradiated ewes. In the next cycles, the length of the follicular phases, ovulation rate and progesterone and FSH levels were similar between groups

  16. Hormonal levels after ovarian X-irradiation of ewes

    Energy Technology Data Exchange (ETDEWEB)

    Driancourt, M.A.; Blanc, M.R.; Mariana, J.C. (I.N.R.A., Monnaie (France))

    1983-01-01

    The effects of X-irradiation on ovulation rate, cyclicity and progesterone and FSH levels in Ile-de-France ewes (4 control and 16 irradiated) after they were treated during the breeding season were reported. The doses used (800 and 2400 R) destroyed 50% of all size classes of the follicular population. Ovulation occurred in 87% of the treated ewes (ovulation rate = 1) when they were irradiated 24 h after luteolysis; 78% of the corpora lutea resulting from these ovulations were normal as to length and progesterone production. FSH in treated ewes started to increase 20 h after treatment and remained higher than in the controls until ovulation time. Later, while these levels were similar between groups on the day of ovulation, high ovulatory levels persisted in irradiated ewes. In the next cycles, the length of the follicular phases, ovulation rate and progesterone and FSH levels were similar between groups.

  17. Postradiation sarcomas: importance of surgery

    International Nuclear Information System (INIS)

    Purpose: To evaluate the role of surgery in the treatment of Post-radiation sarcomas Materials. Post-radiation sarcomas is a rare entity and large series have rarely been reported. In order to improve knowledge about this entity the Radiotherapist group of the French Cancer Centres (FNCLCC) decided to collect retrospectively the cases treated in their institutions. In order to be sure of the histology, all the cases were reviewed by a panel of pathologists of the FNCLCC Pathologist group. A total of 129 cases of sarcomas, and 108 were reviewed; analysis of 8 is in progress, and no material was obtained in the other 11 cases. The diagnosis of sarcomas was confirmed in 80 cases. All patients (60 F, 20 M) have received radiation therapy (median dose 50 Gy; 9-110 Gy) for the treatment of the primary tumor. At this time the age was 44 years (6-83 y). Diagnoses included: breast C. 42%, Lymphomas 11.5%, gynaecological C. 10% benign lesions 5% miscellaneous. Sarcomas developed after a mean interval of 12 years (3-64 y), in bone in 30% of the cases and in soft tissue in 70%. The majority of lesions (90%) developed in the irradiated field (dose received was between 50 Gy and 60 Gy). Histologically there were 29% Malignant HistiocytofibroSarcomas, 19% OsteoSarcomas, 15% FibroSarcomas, 9% LipoSarcomas, 6% LeiomyoSarcomas, miscellaneous sarcomas 22%. Treatment included: Surgery 28 cases, Surgery+Chemotherapy 17 cases, Chemotherapy only 16 cases, Radiation therapy only 1 case, surgery + Radiation therapy 5 cases, Radiation therapy +chemotherapy 6 cases, Surgery + Radiation therapy + Chemotherapy 7 cases, no treatment 5 cases. Results. The outcome is known for all but 3 patients. 51 patients have died (44 of their sarcoma, 4 of the primary tumour, 2 of other cause and 1 iatrogenic). Median survival is 23 months (95% confidence interval 16-29 mo) but 9 patients survived 5 yr or more. Median survival was 43 mo for patients treated by surgery (28p), 6 mo for chemotherapy group (16 p

  18. Synovial sarcoma of the abdominal wall

    International Nuclear Information System (INIS)

    A case report of synovial sarcoma arising in the abdominal wall is presented. A brief review of the clinical and radiological features of synovial sarcoma is made. Pre-operative diagnosis of an abdominal wall synovial sarcoma is virtually impossible, but should be considered when a soft tissue swelling is found to show amorphous stippled calcification X-ray. (author)

  19. Synovial sarcoma; Maziowczak zlosliwy

    Energy Technology Data Exchange (ETDEWEB)

    Kopacz, A.; Jastrzebski, T.; Jaskiewicz, J. and others [Klinika Chirurgii Onkologicznej, Akademia Medyczna, Gdansk (Poland)

    1995-12-31

    Authors present the material of 12 patients with synovial sarcoma treated in Surgical Oncology Dept. Medical University of Gdansk between 1973-1993. In all cases the main symptom was a growing tumor; tumor accompanied by pain in 3 of them. Average time for symptoms was about 9 months. Tumors were classified according to GTNM and Enneking Classification. 9 cases were classified as G2T1NOMO and 3 as G2T2NOMO. According to Enneking Classification 1 case only was recognized as intracompartmental and 11 as extracompartmental. In 9 tumors cytogenetic analysis was done. In 8 of them specific chromosome aberration was discovered - translocation between X and 18 chromosomes: t (18;X)(p11;q11). The following procedures were performed: enucleation of tumor (non-radical) in 2 cases, wide excision in 8 cases, extremity amputation in 1 case. Local recurrence was observed in 9 cases. 30 months without recurrence was noticed as an average. The wide local excision was the method of treatment of local recurrence in 4 cases (followed by RTG-therapy in one case). In another 5 cases extremity amputation was performed (followed by CyVADic chemotherapy in one case). In our material 5-year survival was observed in 36% of cases. The longest notice follow-up was 30 years. (author) 20 refs, 3 tabs, 1 fig

  20. Myeloid Sarcoma of the Skin

    Directory of Open Access Journals (Sweden)

    Ruksan Elal

    2013-06-01

    Full Text Available Myeloid sarcoma (MS (granulocytic sarcoma, extramedullary myeloid tumor, chloroma is a rare malignant extramedullary neoplasms of myeloid precursor cells. Skin is one of the most common localization of MS. The tumor may be isolated or associated with acute myeloid leukemia, chronic myeloid leukemia, primary myelofibrosis, hypereosinophilic syndrome and polycythemia vera. MS is a disease that is rare and difficult to diagnose. Perhaps the most important factor in the diagnosis is suggestion of MS. In this article, clinicopathological features of skin localized MS case are presented.

  1. Magnetic resonance imaging features of extremity sarcomas of uncertain differentiation

    International Nuclear Information System (INIS)

    The purpose of this review is to illustrate the pertinent clinical and imaging features of extremity sarcomas of uncertain differentiation, including synovial sarcoma, epithelioid sarcoma, clear-cell sarcoma, and alveolar soft part sarcoma. These tumours should be considered in the differential diagnosis when a soft-tissue mass is encountered in the extremity of an adolescent or young adult

  2. Magnetic resonance imaging features of extremity sarcomas of uncertain differentiation

    Energy Technology Data Exchange (ETDEWEB)

    Stacy, G.S. [Department of Radiology, University of Chicago Hospitals, Chicago, Illinois (United States)], E-mail: sstacy@radiology.bsd.uchicago.edu; Nair, L. [Department of Radiology, University of Chicago Hospitals, Chicago, Illinois (United States)

    2007-10-15

    The purpose of this review is to illustrate the pertinent clinical and imaging features of extremity sarcomas of uncertain differentiation, including synovial sarcoma, epithelioid sarcoma, clear-cell sarcoma, and alveolar soft part sarcoma. These tumours should be considered in the differential diagnosis when a soft-tissue mass is encountered in the extremity of an adolescent or young adult.

  3. Para-oesophageal synovial sarcoma

    International Nuclear Information System (INIS)

    An unusual case of mediastinal synovial sarcoma with secondary invasion of the oesophagus simulating an intra-oesophageal mass is reported. The location and radiological appearance of this tumour are exceptional, and, to the authors' knowledge, have not been reported previously. (orig.)

  4. Synovial sarcoma of the larynx

    OpenAIRE

    Bhandari, Anita; Sharma, Man Prakash; Bapna, A. S.

    1998-01-01

    A 23 years old male presented with a soft cystic mass in the left paralaryngeal space since 3 months. Indirect laryngoscopy revealed a bulging of the left lateral pharyngeal wall. Histopathologic al examination of the biopsy proved it to be a synovial sarcoma. The case is the seventh case reported in literature.

  5. Para-oesophageal synovial sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Pulpeiro, J.R.; Cruz, R.; Arenas, A.; Perez-Espejo, G.

    1988-05-01

    An unusual case of mediastinal synovial sarcoma with secondary invasion of the oesophagus simulating an intra-oesophageal mass is reported. The location and radiological appearance of this tumour are exceptional, and, to the authors' knowledge, have not been reported previously.

  6. Immunotherapy in Sarcoma: Future Horizons.

    Science.gov (United States)

    Burgess, Melissa; Gorantla, Vikram; Weiss, Kurt; Tawbi, Hussein

    2015-11-01

    Immunologic approaches to cancer are over a century old. Over the years, the strategy has been fine-tuned from inciting infections in subjects to inhibiting negative regulatory signals from the innate immune system. Sarcomas are among the first tumors to be considered for immune interventions. From Coley's toxin to cytokine-based therapies to adoptive cell therapy, there have been numerous immunotherapeutic investigations in this patient population. A promising strategy includes adoptive T cell therapy which has been studied in small cohorts of synovial sarcoma, a subtype that is known to widely express the cancer testis antigen, NY-ESO-1. Additionally, recent data in metastatic melanoma and renal cell carcinoma demonstrate the utility and tremendous efficacy of immune checkpoint blockade with increased rates of durable responses compared to standard therapies. Responses in traditionally "non-immunogenic" tumors, such as lung and bladder cancers, provide ample rationale for the study of immune checkpoint inhibitors in sarcoma. While immunotherapy has induced some responses in sarcomas, further research will help clarify optimal patient selection for future clinical trials and new combinatorial immunotherapeutic strategies. PMID:26423769

  7. Kaposi's sarcoma in South Africa.

    Science.gov (United States)

    Sitas, F; Newton, R

    2001-01-01

    Kaposi's sarcoma was endemic in South Africa even before the advent of the human immunodeficiency virus (HIV). Between 1988 and 1996, the incidence of Kaposi's sarcoma in South Africa has risen at least threefold and continues to increase as the HIV epidemic grows. Research from South Africa has shown that infection with human herpesvirus 8 (HHV8) is associated with Kaposi's sarcoma but not with any other major cancer site or type. In addition, the risk of Kaposi's sarcoma increases with increasing antibody titer to HHV8, but, for a given titer, the risk is greater in HIV-seropositive compared with HIV-seronegative individuals. The age- and sex-standardized seroprevalence of HHV8 in black South African hospital patients was found to be slightly more than 30%; the seroprevalence of HHV8 increased with age and was similar in men and in women. The modes of transmission of HHV8 are yet to be fully elucidated. Limited evidence exists for sexual transmission in black South African adults, but mother-to-child and person-to-person transmission in childhood is also likely. Furthermore, the seroprevalence of HHV8 decreases with increasing levels of education and is lower in whites than in blacks, suggesting that factors associated with poverty may be important determinants of transmission. Future research should focus on risk factors for Kaposi's sarcoma in HHV8-infected individuals, on determinants and mode of transmission of HHV8, and on the elucidation of the effect of primary HHV8 infection in adults and in children. PMID:11158199

  8. Comparative Clinical Observations on Trypanosoma vivax Infected Pregnant Yankasa and West African Dwarf Ewes

    Directory of Open Access Journals (Sweden)

    S.A.S. Olorunju

    2005-01-01

    Full Text Available Three groups of pregnant Yankasa (YK and West African Dwarf (WAD ewes, made up of 6pregnant YK and 6 pregnant WAD ewes in each group, were assigned at random to first, second and third trimester of pregnancy, to study the clinical manifestation of. T. vivax infection at each trimester of pregnancy. A fourth group made up of 3 pregnant YK and 3 pregnant WAD ewes served as the non-infected controls for the study. Pre- infection mean rectal temperatures of the YK and WAD ewes were 38.6 Co and 38.7 Crespectively, while post-infection temperatures were as high as 41.5 C and 41.6 C for the YK and WAD ewes respectively. The infection was severe in the infected YK and WAD ewes in the second and third trimesters of pregnancy and most severe in YK than in the WAD ewes. The infected YK and WAD ewes exhibited pyrexia, signs of anaemia shown by pale mucus membrane, decrease in packed cell volume (PCV values, decrease in total plasma protein (TP values, weight loss, lethargy, dullness, abortions and death of ewes. The severity of the infection increased as the pregnancy advanced in the ewes. WAD ewes in the first trimester were least susceptible to the infection and self-cure was observed in one WAD ewe. The infected WAD ewes controlled the effects of the infection on abortions and mortality more than the YK ewes. It was concluded from the study that the trimester of pregnancy and breed of ewe influenced the clinical manifestation of T. vivax infection on pregnant YK and WAD ewes.

  9. Plasma Fatty Acid Profile of Gestating Ewes Supplemented with Fishmeal

    Directory of Open Access Journals (Sweden)

    Mamun M. Or-Rashid

    2012-01-01

    Full Text Available Problem statement: The very long chain n-3 polyunsaturated fatty acids (>18C cannot be adequately synthesized by ruminant tissues to meet their requirements; therefore, their concentration in body depends on the supply through feed. It may be possible to improve the essential fatty acid status of ruminant animals, during gestation by manipulating the maternal diet with Fishmeal (FM. The objectives of this research were to (1 determine the effect of fishmeal supplementation on the plasma fatty acid profile of ewes during late gestation and (2 determine the status of the plasma docosahexaenoic acid (22:6n3 of lambs born to these ewes. Approach: Eight gestating ewes [Rideau-Arcott, 97±5 kg initial body weight, 100 days of gestation] were used in a completely randomized design. Ewes were individually-housed and fed either a control diet (supplemented with soybean meal or a fishmeal supplemented diet. Blood samples were collected via jugular venipuncture for plasma fatty acids analysis on 100, 114, 128 and 142 days of “gestation”. Blood samples from the lambs were also collected via jugular venipuncture immediately after birth and before receiving their mothers’ colostrum. Plasma fatty acids were analyzed by gas-liquid chromatography. Results: The ewes from both groups, i.e., control and fishmeal supplemented, had a similar fatty acid profile prior to supplementation (at 100 days, p>0.05. Thereafter, there was an increase in eicosapentaenoic acid (20:5n3, docosahexaenoic acid, total n3-PUFA and total very long chain n3-PUFA (>C18 contents in plasma for the fishmeal supplemented ewes compared to the control (p0.05 in total saturated fatty acids, total monounsaturated fatty acids, total conjugated linoleic acid, total trans-18:1, total cis-18:1, or total n6-PUFA contents in ewe plasma between control and fishmeal supplemented groups. Lambs born to ewes fed the fishmeal supplemented diet had greater (pConclusion: The ewes supplemented

  10. Sapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic Sarcoma

    Science.gov (United States)

    2016-06-01

    High Grade Sarcoma; Metastatic Leiomyosarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Recurrent Leiomyosarcoma; Recurrent Malignant Peripheral Nerve Sheath Tumor; Recurrent Synovial Sarcoma; Recurrent Undifferentiated Pleomorphic Sarcoma; Uterine Corpus Leiomyosarcoma

  11. The clone wars - revenge of the metastatic rogue state: the sarcoma paradigm

    Directory of Open Access Journals (Sweden)

    Holly Lynn Spraker

    2012-01-01

    Full Text Available Ewing sarcoma (ES is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES likely is metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: 1 selection of chemotherapy-resistant clones, 2 signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or 3 genetic changes within the ES cells themselves due to a combination of therapy-related selection and DNA-damaging chemotherapeutic agents. These possibilities and the evidence base to support them are explored.

  12. Primary retroperitoneal synovial sarcoma in CT and MRI

    Directory of Open Access Journals (Sweden)

    Alhazzani Abdulmajeed

    2010-01-01

    Full Text Available Synovial sarcomas are most commonly localized in the extermities, especially the lower thigh and knee areas. Retroperitoneal synovial sarcoma is very rare. We decribe the radiological findings of an adult retroperitoneal synovial sarcoma.

  13. Primary retroperitoneal synovial sarcoma in CT and MRI

    OpenAIRE

    Alhazzani Abdulmajeed; El-sharkawy Mohamed; Hassan Hamdy

    2010-01-01

    Synovial sarcomas are most commonly localized in the extermities, especially the lower thigh and knee areas. Retroperitoneal synovial sarcoma is very rare. We decribe the radiological findings of an adult retroperitoneal synovial sarcoma.

  14. Intraparenchymal Myeloid Sarcoma and Subsequent Spinal Myeloid Sarcoma for Acute Myeloblastic Leukemia

    OpenAIRE

    Eom, Ki Seong; Kim, Tae Young

    2011-01-01

    Myeloid sarcoma is a solid, extramedullary tumor composed of leukemic myeloblasts or immature myeloid cells. Intraparenchymal myeloid sarcoma without the involvement of the skull or meninges is extremely rare. Here, we present the case of a 49-year-old man who developed intraparenchymal myeloid sarcoma on the left cerebellum after allogeneic bone marrow transplantation (BMT). He received radiotherapy after complete removal of intraparenchymal myeloid sarcoma, but he was diagnosed spinal myelo...

  15. Patterns of care for patients with advanced soft tissue sarcoma: experience from Australian sarcoma services

    OpenAIRE

    Bae, Susie; Crowe, Philip; Gowda, Raghu; Joubert, Warren; Carey-Smith, Richard; Stalley, Paul; Desai, Jayesh

    2016-01-01

    Background There is a paucity of data on the current management of patients with advanced soft tissue sarcoma (STS) in the Australian health care setting. This study utilised the Australian sarcoma database to evaluate the patterns of care delivered to patients with advanced STS at Australian sarcoma services. Methods Prospectively collected data from six sarcoma centres in Australia were sourced to identify patients diagnosed with advanced STS between 1 January 2010 and 31 December 2012. Des...

  16. Neuronal Differentiation of Synovial Sarcoma and Its Therapeutic Application

    OpenAIRE

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

    2008-01-01

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

  17. Evaluating Dactinomycin and Vincristine in Young Patients With Cancer

    Science.gov (United States)

    2015-08-26

    Childhood Acute Lymphoblastic Leukemia; Childhood Rhabdomyosarcoma; Childhood Soft Tissue Sarcoma; Ewing Sarcoma; Ewing Sarcoma of Bone; Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor (PNET); Unspecified Childhood Solid Tumor, Protocol Specific; Wilms Tumor and Other Childhood Kidney Tumors

  18. Clinical mastitis in ewes; bacteriology, epidemiology and clinical features

    Directory of Open Access Journals (Sweden)

    Kvitle Bjørg

    2007-09-01

    Full Text Available Abstract 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.

  19. Primary pleuropulmonary synovial sarcoma: a case report

    OpenAIRE

    Yuan, Lianfang; Guan, Zhiyu; Dai, Xuan; Jie XU

    2015-01-01

    Pleuropulmonary synovial sarcoma (PPSS) is an extremely rare malignant tumor, which is increasingly recognized as a subtype of sarcoma with a distinctive chromosomal translocation specific to synovial sarcoma. It is often presents like any thoracic tumor with symptoms such as chest pain or cough. Here we report a case of PPSS in a 49-year-old woman presenting with cough, shortness of breath and chest pain. And who were found upon histologic examination of the resection specimen to have cystic...

  20. Primary synovial sarcoma of the maxilla

    OpenAIRE

    Manikkath Aparna; Jayalakshmi Natarajan; Chakravarty Arumugam; Raghu Radhakrishnan

    2014-01-01

    An innocuously appearing gingival mass in the maxilla revealed extensive osteolysis on radiographic examination. A rare clinical presentation of synovial sarcoma, appropriate diagnostic strategies and suitable treatment protocol in a 21-year-old male is reported herewith. It is only the third case of primary monophasic synovial sarcoma of the maxilla to be reported to the best of our knowledge and the first to have occurred in a male patient. The importance of considering synovial sarcoma in ...

  1. Synovial Sarcoma of the Thyroid Gland

    OpenAIRE

    Ryu, Chang Hwan; Cho, Kyung-Ja; Choi, Seung-Ho

    2011-01-01

    Primary synovial sarcoma of the thyroid is an extremely rare condition which has only been reported twice in the literature. We herein report a case of highly aggressive and rapidly lethal primary synovial sarcoma of the thyroid. A 72-year-old woman presented with extensive local invasion, rapid progression, and early distant metastasis secondary to primary thyroid synovial sarcoma. The tumor exhibited an atypical histologic and immunohistochemical staining pattern. Detection of SYT/SSX fusio...

  2. Intraneural synovial sarcoma of the median nerve

    OpenAIRE

    Rahul Kasukurthi; Pruzansky, Mark E; Mackinnon, Susan E.; Lipira, Angelo B; Ray, Wilson Z.

    2010-01-01

    Synovial sarcomas are soft-tissue malignancies with a poor prognosis and propensity for distant metastases. Although originally believed to arise from the synovium, these tumors have been found to occur anywhere in the body. We report a rare case of synovial sarcoma arising from the median nerve. To our knowledge, this is the twelfth reported case of intraneural synovial sarcoma, and only the fourth arising from the median nerve. Because the diagnosis may not be apparent until after pathologi...

  3. A Primary Synovial Sarcoma of Lung

    OpenAIRE

    Roy, P. P.; Das, A.; A. Sarkar; A K Dwari; Datta, S.

    2012-01-01

    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 pl...

  4. Cytomorphology of epithelioid sarcoma: A diagnostic enigma

    Directory of Open Access Journals (Sweden)

    Yogesh K Yadav

    2012-01-01

    Full Text Available Epithelioid sarcoma is an uncommon soft tissue malignancy with a known propensity for recurrence as well as metastasis. We report epithelioid sarcoma in 50 year old male with a recurrent ulcerative nodule over the right ankle since five months, initially misdiagnosed as malignant fibrous histiocytoma. The article aims at assessing the key cytological features of epithelioid sarcoma and their reliability in the diagnosis of the lesion. Also described are its histopathological and immunohistochemical features.

  5. Intraneural synovial sarcoma of the median nerve

    Directory of Open Access Journals (Sweden)

    Rahul Kasukurthi

    2010-06-01

    Full Text Available Synovial sarcomas are soft-tissue malignancies with a poor prognosis and propensity for distant metastases. Although originally believed to arise from the synovium, these tumors have been found to occur anywhere in the body. We report a rare case of synovial sarcoma arising from the median nerve. To our knowledge, this is the twelfth reported case of intraneural synovial sarcoma, and only the fourth arising from the median nerve. Because the diagnosis may not be apparent until after pathological examination of the surgical speci­men, synovial sarcoma should be kept in mind when dealing with what may seem like a benign nerve tumor.

  6. Proton Radiotherapy for Pediatric Sarcoma

    International Nuclear Information System (INIS)

    Pediatric sarcomas represent a distinct group of pathologies, with approximately 900 new cases per year in the United States alone. Radiotherapy plays an integral role in the local control of these tumors, which often arise adjacent to critical structures and growing organs. The physical properties of proton beam radiotherapy provide a distinct advantage over standard photon radiation by eliminating excess dose deposited beyond the target volume, thereby reducing both the dose of radiation delivered to non-target structures as well as the total radiation dose delivered to a patient. Dosimetric studies comparing proton plans to IMRT and 3D conformal radiation have demonstrated the superiority of protons in numerous pediatric malignancies and data on long-term clinical outcomes and toxicity is emerging. In this article, we review the existing clinical and dosimetric data regarding the use of proton beam radiation in malignant bone and soft tissue sarcomas

  7. Irradiation for conjunctival granulocytic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Fleckenstein, K.; Geinitz, H.; Grosu, A.; Molls, M. [Dept. of Radiation Oncology, Technical Univ. of Munich (Germany); Goetze, K. [Dept. of Hematology and Oncology, Technical Univ. of Munich (Germany); Werner, M. [Dept. of Pathology, Technical Univ. of Munich (Germany)

    2003-03-01

    Case History and Findings: A 73-year-old woman with a history of myeloproliferative syndrome (MPS) presented with bilateral chemosis, redness and burning of the eyes. The ocular motility was severely impaired. Ophthalmological examination revealed markedly distended conjunctivas on both sides. Biopsy disclosed conjunctival granulocytic sarcoma as an initial symptom of acute myelogenous leukemia (AML). Diagnosis was confirmed by peripheral blood smear and bone marrow aspiration. Treatment and Outcome: The orbital tumor disappeared completely after local external beam irradiation with a total dose of 30 Gy and no further orbital recurrence occurred. With chemotherapy following irradiation transient hematological remission was achieved. 5 months after diagnosis the patient died of respiratory failure following atypical pneumonia as a consequence of her underlying disorder. Conclusion: Detection of orbital granulocytic sarcoma, even in the absence of typical leukemic symptoms is of practical importance, because treatment with irradiation can lead to stabilization or improvement in the patient's vision. (orig.)

  8. Irradiation for conjunctival granulocytic sarcoma

    International Nuclear Information System (INIS)

    Case History and Findings: A 73-year-old woman with a history of myeloproliferative syndrome (MPS) presented with bilateral chemosis, redness and burning of the eyes. The ocular motility was severely impaired. Ophthalmological examination revealed markedly distended conjunctivas on both sides. Biopsy disclosed conjunctival granulocytic sarcoma as an initial symptom of acute myelogenous leukemia (AML). Diagnosis was confirmed by peripheral blood smear and bone marrow aspiration. Treatment and Outcome: The orbital tumor disappeared completely after local external beam irradiation with a total dose of 30 Gy and no further orbital recurrence occurred. With chemotherapy following irradiation transient hematological remission was achieved. 5 months after diagnosis the patient died of respiratory failure following atypical pneumonia as a consequence of her underlying disorder. Conclusion: Detection of orbital granulocytic sarcoma, even in the absence of typical leukemic symptoms is of practical importance, because treatment with irradiation can lead to stabilization or improvement in the patient's vision. (orig.)

  9. [Sarcomas of the small intestine].

    Science.gov (United States)

    Beyrouti, M L; Abid, M; Beyrouti, R; Ben Amar, M; Gargouri, F; Frikha, F; Affes, N; Boujelbene, S; Ghorbel, A

    2005-03-12

    Sarcomas of the small intestine are rare, clearly differentiated, malignant, mesenchymatous tumours that can be of smooth muscle, Schwann cell or fibroblastic origin. From a clinical point of view, the pain and abdominal mass are the 2 types of symptoms that frequently reveal the disease. In rare cases, sarcomas of the small intestine are manifested by an acute complication. No imaging method can clearly confirm the diagnosis. Before immunohistochemistry, differential diagnosis was made on undifferentiated mesenchymatous "stromal" tumours, which are also rare. Exeresis must be complete and without perforation of the tumour because of the risk of locoregional relapse. The benefits provided by chemotherapy and radiotherapy are limited because of the low mitotic activity of the tumour cells and its weak vascularisation. Long-term survival is limited by poor prognosis criteria: high grade malignancy, size greater than 5 cm, tumour extension, perforation of the tumour, quality of surgical resection and histological type. PMID:15859576

  10. Epithelioid sarcoma: A diagnostic challenge

    Directory of Open Access Journals (Sweden)

    Pai Kanthilatha

    2006-01-01

    Full Text Available Epithelioid sarcoma is an uncommon slow-growing soft tissue malignancy, associated with a high incidence of local recurrence and metastasis. We report a 26-year-old male with epithelioid sarcoma on the right palm with a long history of over seven years, which was initially misdiagnosed as cutaneous tuberculosis and epithelioid hemangioendothelioma, as a result of which the treatment was delayed. No metastasis was found in our patient. The patient was referred to the oncology centre where he underwent wide excision of the lesion followed by radiotherapy. The review of the literature including clinical and histological differential diagnosis is presented as it mimics inflammatory, benign tumors as well as other malignant conditions.

  11. Proton Radiotherapy for Pediatric Sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Ladra, Matthew M.; Yock, Torunn I., E-mail: tyock@partners.org [Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA 02114 (United States)

    2014-01-14

    Pediatric sarcomas represent a distinct group of pathologies, with approximately 900 new cases per year in the United States alone. Radiotherapy plays an integral role in the local control of these tumors, which often arise adjacent to critical structures and growing organs. The physical properties of proton beam radiotherapy provide a distinct advantage over standard photon radiation by eliminating excess dose deposited beyond the target volume, thereby reducing both the dose of radiation delivered to non-target structures as well as the total radiation dose delivered to a patient. Dosimetric studies comparing proton plans to IMRT and 3D conformal radiation have demonstrated the superiority of protons in numerous pediatric malignancies and data on long-term clinical outcomes and toxicity is emerging. In this article, we review the existing clinical and dosimetric data regarding the use of proton beam radiation in malignant bone and soft tissue sarcomas.

  12. Reticular schwannoma mimicking myxoid sarcoma

    OpenAIRE

    Chaurasia, Jai Kumar; Afroz, Nishat; Sahoo, Biswajit; Naim, Mohammed

    2014-01-01

    Reticular/microcystic schwannoma is a recently described rare distinctive variant of schwannoma with a predilection for gastrointestinal tract. Its occurrence in soft tissue is extremely rare. We report a case of reticular/microcystic variant of schwannoma in a 55-year-old Indian woman who presented with a painless slow-growing swelling in the right forearm for the past 6 months. MRI findings suggested myxoid sarcoma. However, histological and immunohistochemical findings in the excised speci...

  13. MR findings of synovial sarcoma

    International Nuclear Information System (INIS)

    MR images of seven patients with histologically documented synovial sarcoma were reviewed to find the suggestive or pathognomonic findings of the disease. MR appearance of the tumor was inhomogeneous, multisepatated mass with various degree of internal septation and infiltrative tumor margin. The signal intensity of the mass was slightly hyperintense relative to muscle on T1-weighted image and hyperintense relative to subcutaneous fatty tissue on T2-weighted image

  14. MR findings of synovial sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jin Joo; Byun, Hong Sik; Kim, Kie Hwan; Yoon, Il Yoon; Chin, Soo Yil [Korea Cancer Center Hospital, Seoul (Korea, Republic of)

    1993-09-15

    MR images of seven patients with histologically documented synovial sarcoma were reviewed to find the suggestive or pathognomonic findings of the disease. MR appearance of the tumor was inhomogeneous, multisepatated mass with various degree of internal septation and infiltrative tumor margin. The signal intensity of the mass was slightly hyperintense relative to muscle on T1-weighted image and hyperintense relative to subcutaneous fatty tissue on T2-weighted image.

  15. Synovial sarcoma of the foot

    International Nuclear Information System (INIS)

    The case of a 29 year-old female patient who had experienced pain in the right midfoot for 5 years which was diagnosed as a degenerative or rheumatic change and treated by physiotherapy and medication. By means of magnetic resonance imaging we identified a soft-tissue tumor of the midfoot. Histology provided the findings of a monophasic fibrous synovial sarcoma. The case history is reported together with a presentation of the disease and its radiological diagnosis. (orig.)

  16. Epithelioid sarcoma of the orbit

    OpenAIRE

    Alkatan, Hind M.; Chaudhry, Imtiaz; Al-Qahtani, Abdullah

    2011-01-01

    Epithelioid sarcoma is an aggressive and rare malignancy first recognized by Enzinger in 1970. It is known most commonly to affect the distal upper extremities in young adults. The classical “distal” form has a male predominance and can also involve other less frequent sites including lower extremities, proximal upper extremities, and the trunk. The “proximal” variant of this tumor is deep seated, tends to occur in older patients and predominantly develops in the pelvis, perineum, and genital...

  17. Synovial sarcoma of the foot.

    Science.gov (United States)

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

    2013-01-01

    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

  18. Fibromyxoid sarcoma of the pancreas

    Directory of Open Access Journals (Sweden)

    Čolović Radoje

    2008-01-01

    Full Text Available Introduction Fibromyxoid sarcoma is a rare mesenchymal neoplasm, usually appearing in the soft tissue of the extremities, less frequently in the groin, trunk, neck, and upper extremities. Within the abdomen, the tumour is usually localised within the retroperitoneum. Case OutlineWe present a 56-year-old woman in whom, during the routinely performed investigation for atacks of choking with lots of bronchial secretion, and arterial hypertension, an ultrasonographer found a tumour within the head of the pancreas 6×6 cm in diameter. At operation, a dark pink, lobulated soft tumour, surrounded by a tiny capsule, clearly different from the completely normal pancreatic tissue of the posterior side of the head of the pancreas, was easily and ideally excised.The postoperative recovery was stormy. She developed postoperative pancreatitis, temporary biliary and duodenal fistula, which all settled by conservative treatment. The histology of the 80 g weighing tumour showed a circumscribed fibromyxoid sarcoma of low malignancy. Immunohistochemistry showed diffuse vimentin and CD34 strong positivity, as well as focal anti-SMA and anti-EMA immunopositivity. Six months after surgery, she died with signs of cerebrovascular insult, asthmatic status, and recurrent suppurative abdominal fistula, probably related to the previous pancreatitis. Ultrasonography showed a possible liver secondary. The exact cause of death was not confirmed as the autopsy was refused by the family. Conclusion Primary sarcomas of the pancreas are very rare, but should be considered in differential diagnosis of pancreatic neoplasms. To the best of our knowledge, there has been no previously described fibromyxoid sarcoma of the pancreas. .

  19. Intensive combined modality therapy of small round cell and undifferentiated sarcomas in children and young adults

    International Nuclear Information System (INIS)

    Seventy-five patients (ages 4-35 years) with the following small round cell tumors and undifferentiated sarcoma were treated at the National Cancer Institute: Ewing's sarcome (n=32), peripheral neuroepithelioma (n=14), rhabdomyosarcoma (n=24), undifferentiated sarcoma (n=5). Most patients had poor prognostic features including 36 (48%) with metastatic disease, and 42 (56%) with central (truncal) tumors (22 in the pelvis). Treatment included 5 cycles of intensive induction chemotherapy with vincristine, cyclophosphamide and adriamycin, plus aggressive local radiation therapy using simulation and computerized treatment planning for all patients. Thereafter, complete clinical responses were consolidated with intensive chemotherapy, total body irradiation and autologous bone marrow transplantation. There were three local only failures, 10 local plus distant failures, 36 distant only failures, 3 treatment-related deaths, and one intercurrent death. Overall actuarial survival and event-free survival at 4 years are 49 and 29%, respectively. Actuarial freedom from local progression was seen in 74% of patients at 4 years, quite remarkable considering the bulk and location of most of these tumors. Without aggressive surgery, many of these high risk patients had satisfactory outcomes, but better systemic treatments are still needed.(author). 44 refs.; 8 figs.; 6 tabs

  20. Constitutive Activation of Neuregulin/ERBB3 Signaling Pathway in Clear Cell Sarcoma of Soft Tissue

    Directory of Open Access Journals (Sweden)

    Karl-Ludwig Schaefer

    2006-07-01

    Full Text Available 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(g13;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 Cl-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.

  1. Genetic analysis of milking ability in Lacaune dairy ewes

    Directory of Open Access Journals (Sweden)

    Barillet Francis

    2006-02-01

    Full Text Available Abstract The milking ability of Lacaune ewes was characterised by derived traits of milk flow patterns, in an INRA experimental farm, from a divergent selection experiment in order to estimate the correlated effects of selection for protein and fat yields. The analysis of selected divergent line effects (involving 34 616 data and 1204 ewes indicated an indirect improvement of milking traits (+17% for maximum milk flow and -10% for latency time with a 25% increase in milk yield. Genetic parameters were estimated by multi-trait analysis with an animal model, on 751 primiparous ewes. The heritabilities of the traits expressed on an annual basis were high, especially for maximum flow (0.54 and for latency time (0.55. The heritabilities were intermediate for average flow (0.30, time at maximum flow (0.42 and phase of increasing flow (0.43, and low for the phase of decreasing flow (0.16 and the plateau of high flow (0.07. When considering test-day data, the heritabilities of maximum flow and latency time remained intermediate and stable throughout the lactation. Genetic correlations between milk yield and milking traits were all favourable, but latency time was less milk yield dependent (-0.22 than maximum flow (+0.46. It is concluded that the current dairy ewe selection based on milk solid yield is not antagonistic to milking ability.

  2. Nasopharyngeal Synovial Sarcoma - An Oncological Surprise

    OpenAIRE

    Ram Abhinav Kannan; Kirthi Koushik; Nalini Kilara; Aparna Gangoli

    2014-01-01

    Synovial sarcoma is a rare and aggressive high-grade neoplasm with poor prognosis. It is usually encountered in the lower extremities of young adults; only a few cases have been reported in the head and neck region. We report the fourth case of synovial sarcoma of the nasopharynx, treated with neoadjuvant chemotherapy, complete surgical excision and adjuvant radiation with extensive review of literature.

  3. Delays in the management of retroperitoneal sarcomas

    DEFF Research Database (Denmark)

    Seinen, Jojanneke; Almquist, Martin; Styring, Emelie;

    2010-01-01

    Retroperitoneal sarcomas are rare and treatment should optimally be centralized. Despite successful centralization with 90% of the patients referred prior to surgery, delays occur, which led us to assess lead times in a population-based series. Method. Patients diagnosed with retroperitoneal sarc...... indicate that development of coordinated diagnostic packages could shorten delays at the sarcoma centre....

  4. Functional imaging techniques for evaluation of sarcomas

    OpenAIRE

    Hicks, Rodney J.

    2005-01-01

    Sarcomas are often characterised by significant histopathologic heterogeneity, both between and within tumours. This heterogeneity reflects physiologic, biochemical and genetic processes that are amenable to characterisation by functional imaging. Although anatomically based imaging modalities such as plain radiography, X-ray computed tomography (CT) and magnetic resonance imaging (MRI) remain the primary diagnostic modalities for staging sarcomas, nuclear medicine approaches including gamma ...

  5. Primary stromal sarcoma of the breast

    Directory of Open Access Journals (Sweden)

    Radovanović Aleksandra

    2012-01-01

    Full Text Available Primary sarcomas of the breast are rare and there are only a few hundred cases reported in the literature; hence, their significance is primarily educational. We report a case of a 57-year old patient who developed breast sarcoma without previous history on any breast diseases.

  6. Histomorphological changes in follicular apparatus of ewe ovaries following irradiation

    International Nuclear Information System (INIS)

    Histological changes in primary follicles of ewes after a five-day protracted exposure to gamma rays were studied by qualitative and micrometric methods. The experiment was carried out in the anoestrous period with 21 ewes of the Slovak Merino breed, divided into three groups. The first control group (five ewes) was not irradiated. The second and third groups (each included eight ewes) were irradiated with 60Co gamma rays for a period of five days to the total dose of 4.8 Gy. All ewes including the control ones were given Ampicillin Spofa 250 mg per head/day during the period of ten days after irradiation. The third group was administered apart from this a mixture of vitamins, Roboran H, at the dose of 10 g per head/day. The animals were slaughtered on the fifth day of irradiation and on the tenth day after the end of irradiation. The ovaries processed by a routine histological method were cut in 7 μm slices in a series of 70 μm and stained with hematoxylin-eosine. By qualitative histomorphological analysis of the oocytes of primary follicles, chromatin aggregation, pycnosis of nuclei, pronounced acidophilia of oocyte cytoplasm, their shrinking and disintegration were determined. In intact primary follicles, mitotic division of follicular cells stopped and the proportion of follicular cells with pycnotic nuclei increased after irradiation. The results show that the five-day protracted exposure to gamma rays to the total dose of 4.8 Gy caused pronounced degenerative changes in the anoestrous period. Administration of antibiotics or vitamins had no significant effect on the stated histomorphological changes. (author)

  7. Changes in ovaries of X-irradiated ewes

    International Nuclear Information System (INIS)

    The qualitative histological alterations of ovaries and caryometric changes in the corpora lutea (CL) were studied in the ewes of the Slovak Merino breed after irradiation with X-rays. The experiments were conducted on 12 ewes divided into three groups of four animals. The first group included the control ewes, the second, experimental group was exposed to X-rays applied directly to the ovaries after laparotomy at a rate of 64.4 mC per kg (250 R), and the ewes in the third group were locally irradiated in the hypothalamo-hypophysial region at a rate of 516.5 mC per kg (2000 R). On the 10th day following the irradiation, the animals were killed, ovarian excisions were taken from the carcasses and processed. Changes in the blood vessels of the ovaries of the ewes in the second group were revealed by qualitative microscopic analysis. Pronounced subintimal changes, reminding of foamy cells described in available literature as a consequence of radiation damage to the blood vessels, were observed in the arteries and veins of the marrow and porta of the ovaries. A pronounced alteration of follicular structures was induced by single local irradiation of the ovaries which, at the same time, stimulated CL production. The irradiation of the hypothalamo-hypophysial region resulted in a multiplication of normal tertiary follicles and in an increase of the total number of follicles. No other histological changes were observed in the tissues of ovaries after irradiation of the hypothalamo-hypophysial region

  8. Penile epithelioid sarcoma: MR imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Sirikci, A.; Bayram, M.; Demirci, M. [Department of Radiology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey); Bakir, K. [Department of Pathology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey); Sarica, K. [Department of Urology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey)

    1999-10-01

    Magnetic resonance imaging findings of a 38-year-old man with epithelioid sarcoma of the penis is presented. It started as a firm, painless and slowly growing nodule at the base of his penis 6 months previously which caused pain radiating to the testis during coitus. It has been well known that sarcomas may well mimic reactive processes. Initial presentation of epithelioid sarcoma may provoke considerable diagnostic difficulty, and its differentiation from benign lesions, such as Peyronie`s disease and chronic inflammation, may be a clinical problem. In our present report the MR findings are compared with those of the epithelioid sarcomas of various locations reported in the literature and differential diagnosis of the entity is discussed. To our knowledge, this is the first report regarding the MR findings of the epithelioid sarcoma of penis. (orig.) With 3 figs., 16 refs.

  9. Angiography of histopathologic variants of synovial sarcoma

    International Nuclear Information System (INIS)

    Synovial sarcomas are rare soft tissue tumors which histopathologically can be divided into monophasic, biphasic and mixed variants. As part of a protocol for intra-arterial chemotherapy 12 patients with biopsy proven synovial sarcoma underwent angiography. The angiograms on these patients were reviewed to determine whether synovial sarcomas and their variants demonstrated a characteristic angiographic appearance. Synovial sarcomas appeared angiographically as soft tissue masses which showed a fine network of tumor vessels with an inhomogeneous capillary blush. Their degree of vascularity varied according to their histopathology. Monophasic synovial sarcomas demonstrated in general a higher degree of neovascularity than the biphasic form. This finding was also suggested by histopathologic analysis of the vessels in the tumor. Although angiography did not show a distinctive vascular pattern it may be useful to evaluate tumor size and vascularity. (orig.)

  10. Penile epithelioid sarcoma: MR imaging findings

    International Nuclear Information System (INIS)

    Magnetic resonance imaging findings of a 38-year-old man with epithelioid sarcoma of the penis is presented. It started as a firm, painless and slowly growing nodule at the base of his penis 6 months previously which caused pain radiating to the testis during coitus. It has been well known that sarcomas may well mimic reactive processes. Initial presentation of epithelioid sarcoma may provoke considerable diagnostic difficulty, and its differentiation from benign lesions, such as Peyronie's disease and chronic inflammation, may be a clinical problem. In our present report the MR findings are compared with those of the epithelioid sarcomas of various locations reported in the literature and differential diagnosis of the entity is discussed. To our knowledge, this is the first report regarding the MR findings of the epithelioid sarcoma of penis. (orig.)

  11. Synovial Sarcoma in the Rectovesical Space: A Case Report

    International Nuclear Information System (INIS)

    Synovial sarcoma is an uncommon soft tissue malignancy usually arising in the extremities of young adults. Synovial sarcomas at unusual anatomic locations have been reported; however, to the best of our knowledge, there are no reports on primary synovial sarcoma in the rectovesical space. Here, we describe the radiologic findings of primary synovial sarcoma in the rectovesical space and review relevant literature.

  12. Synovial Sarcoma in the Rectovesical Space: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kil, Min Chul; Cho, Bum Sang; Han, Gi Seok; Park, Kil Sun; Kim, Sung Jin; Cha, Sang Hoon; Lee, Seung Young; Kang, MIn Ho [Dept. of Radiology, Chungbuk National University Hospital, Chunju (Korea, Republic of); Lee, Ok Jun [Dept. of Pathology, Chungbuk National University Hospital, Chunju (Korea, Republic of)

    2011-08-15

    Synovial sarcoma is an uncommon soft tissue malignancy usually arising in the extremities of young adults. Synovial sarcomas at unusual anatomic locations have been reported; however, to the best of our knowledge, there are no reports on primary synovial sarcoma in the rectovesical space. Here, we describe the radiologic findings of primary synovial sarcoma in the rectovesical space and review relevant literature.

  13. Late Gestational Undernutrition Alters Plasma IGF-1 Concentration During Subsequent Lactation in Ewe

    Directory of Open Access Journals (Sweden)

    Ali Kiani

    2011-12-01

    Full Text Available The objective of this study was to investigate the effects of undernutrition during late gestation on plasma concentration of insulin-like growth factor (IGF1, leptin, insulin and glucose in pregnant and lactating ewes. Ten twin-bearing shropshire ewes were fed either adequately (AN; 100% energy and protein requirements or restrictedly (RN; about 60% of energy and protein requirements fed during the last six weeks of gestation. Ewe’s blood samples were taken at 50, 28 and 10 days pre-partum as well as at lambing and 7, 17 and 35 days post-partum. At lambing plasma glucose concentrations sharply increased in both groups and it was significantly lower in RN ewes in comparison with AN ewes. Plasma concentrations of insulin and leptin were not affected by late gestational undernutrition. Plasma IGF1 concentrations in RN ewes (78 ±8 ng/ml was significantly lower (P<0.05 than that in AN ewes (110 ±8 ng/ml. Plasma IGF1 decreased in RN ewes during late gestation and then increased sharply at lambing and during first month of lactation. In contrast IGF1 concentration was relatively constant both pre and postnataly in AN ewes. IGF1 values in restricted fed ewes were significantly lower than values in adequately fed ewes during gestation. Surprisingly IGF1 plasma in RN ewes was significantly lower at 35 days (110 vs. 164 ng/ml post-partum in comparison with those in AN ewes. In conclusion, results showed late gestational undernutrion causes a decrease in plasma glucose and IGF1 at parturition as well as during late gestation. In addition, late gestational undernutrition seems to have longer term effect on plasma IGF1 even when ewes are adequately fed during lactation.

  14. Late Gestational Undernutrition Alters Plasma IGF-1 Concentration During Subsequent Lactation in Ewe

    Directory of Open Access Journals (Sweden)

    Ali Kiani

    2011-10-01

    Full Text Available The objective of this study was to investigate the effects of undernutrition during late gestation on plasma concentration of insulin-like growth factor (IGF1, leptin, insulin and glucose in pregnant and lactating ewes. Ten twin-bearing shropshire ewes were fed either adequately (AN; 100% energy and protein requirements or restrictedly (RN; about 60% of energy and protein requirements fed during the last six weeks of gestation. Ewe’s blood samples were taken at 50, 28 and 10 days pre-partum as well as at lambing and 7, 17 and 35 days post-partum. At lambing plasma glucose concentrations sharply increased in both groups and it was significantly lower in RN ewes in comparison with AN ewes. Plasma concentrations of insulin and leptin were not affected by late gestational undernutrition. Plasma IGF1 concentrations in RN ewes (78 ±8 ng/ml was significantly lower (P<0.05 than that in AN ewes (110 ±8 ng/ml. Plasma IGF1 decreased in RN ewes during late gestation and then increased sharply at lambing and during first month of lactation. In contrast IGF1 concentration was relatively constant both pre and postnataly in AN ewes. IGF1 values in restricted fed ewes were significantly lower than values in adequately fed ewes during gestation. Surprisingly IGF1 plasma in RN ewes was significantly lower at 35 days (110 vs. 164 ng/ml post-partum in comparison with those in AN ewes. In conclusion, results showed late gestational undernutrion causes a decrease in plasma glucose and IGF1 at parturition as well as during late gestation. In addition, late gestational undernutrition seems to have longer term effect on plasma IGF1 even when ewes are adequately fed during lactation.

  15. Effect of PMSG dosage on the reproductive performance of adult ewes and ewe lambs bred at a progestagen-PMSG synchronized estrus.

    Science.gov (United States)

    Ainsworth, L; Shrestha, J N

    1985-11-01

    The effect of dose of pregnant mares' serum gonadotropin (PMSG) on the reproductive performance of adult ewes and ewe lambs and lamb survival at birth after treatment with fluorogestone acetate (FGA)-impregnated intravaginal sponges and PMSG (250 IU or 500 IU) to synchronize estrus was evaluated. Ewes were exposed to rams for breeding at the synchronized and subsequent estrous cycles. The flock, comprised of three synthetic strains and two control breeds, was maintained in a controlled environment and exposed to an artificial light regimen which alternated at 4-mo intervals from 16h of light daily to 9h of light daily. Trials were conducted during January, May and September at the end of a 9-h daylength cycle. Adult ewes were bred in May and 8 mo later in January. Ewe lambs were bred in September at 6.5 to 7.5 mo of age. The overall reproductive performance of the adult ewes was similar at the two breedings: fertility approximately 90%, prolificacy approximately 2.7, fecundity approximately 240% and lambs born alive approximately 2.4. Dosage of PMSG had no effect. Reproductive performance of ewe lambs was lower and there was a strain x treatment interaction, suggesting greater variability in response. The results indicate there is no advantage to using a higher dose of PMSG in ewes with a natural relatively high fecundity. Moreover, the use of the artificial photoperiod appears to overcome the natural seasonal variation in reproductive performance. PMID:16726102

  16. Synovial sarcoma: MR evaluation in 23 patients

    International Nuclear Information System (INIS)

    The synovial sarcoma is one of the most common soft tissue sarcomas. MR is the technique of choice to determine to local extension of malignant soft tissue tumors. To assess the clinical and MR imaging parameters associated with synovial sarcomas that aid in establishing their diagnosis. We review the clinical findings and images of 23 histologically confirmed synovial sarcomas that were studied by MR. Synovial sarcomas usually develop in young adults as soft tissue tumors, preferentially in the deep tissues of an extremity in close proximity to a joint. They are characterized as having a lobulated contour and septa, frequently infiltrating neighboring tissues at some point, and are heterogeneous. The presence of hemorrhage, as well as infiltration of the fascia in subcutaneous tumors, suggests the diagnosis of synovial sarcoma. The development of perilesional edema is not uncommon. Although, logically, the clinical and radiological features of synovial sarcomas can overlap with those of other soft tissue tumors, the findings described here are fairly characteristic of these lesions: thus, when present, they should serve to orient the diagnostic process. (Author) 16 refs

  17. Relationship between energy intake and chewing index of diets fed to pregnant ewes

    DEFF Research Database (Denmark)

    Nielsen, Mette Vestergaard; Nadeau, E.; Markussen, Bo; Eknæs, M.; Nørgaard, Peder

    2015-01-01

    .001), with different within-experiment intercepts in relation to week before lambing (P < 0.001), supporting the existence of a linear relationship between MEI and CIcor for pregnant ewes in the last 4 weeks before lambing. CTmax could be calculated for the ewes and had a mean of 1066 min/d. The model......The objective was to determine whether a linear relationship exists between the metabolizable energy (ME) intake of pregnant ewes and a dietary chewing index (CI). The relationship was studied using five feeding trials with intake data from 108 pregnant ewes, 4 to 1 weeks before lambing, giving a...... total of 324 observations. All ewes were fed grass silage ad libitum, supplemented with concentrates either separately or in a total mixed ration (TMR). The ewes were of different breeds, were between 2 and 7 years old, had a mean body weight (BW) in the 4th week before lambing of 95.1 kg (SD = 9...

  18. Hypereosinophilia associated with genital sarcomas

    International Nuclear Information System (INIS)

    Eosinophils are phagocytic leukocytes, regulators reactions Mast cell mediated hypersensitivity. toxicity and primarily responsible antiparasitic. Predominate in epithelial tissues near the interface surface (skin, digestive tract) .The cytotoxic reaction exerted by deposit cell surface substances from the granules themselves: peroxidases, neurotoxins, and other cationic proteins. Hypereosinophilia is defined as the increase in eosinophils above 1500 / m m3. The most common causes are parasitic infections and reactions allergic. About 60% of tumors may be associated with an elevation eosinophil discrete but marked eosinophilia in these patients is little frequent. Tumors are most associated lung cancer and tumors hematology. There are few reports of this entity associated with uterine sarcomas. Although the pathophysiologic mechanism is unclear, it is assumed that the base is the increased secretion of cytokines eosinofilopoiétics: interleukins (Il) I L-3, Il-5 and G M-CSFR among other possible. Self-morbidity is primarily maintained hypereosinophilic heart, and is derived from the cytotoxic action, with endomyocardial fibrosis and thrombosis. Treatment should be directed at the control of the underlying disease, as good Tumor response was associated with the account and normalizaciónd eosinófiles. Los corticosteroids prednisone, 60 mg / day po) may be effective because antagonize The stimulatory effect of cytokines. In the vast majority of cases, the disease is associated with hypereosinophilia disseminated and poor overall prognosis We present a case of vaginal sarcoma with pulmonary metastases and hypereosinophilia seniors who responded to treatment with chemotherapy

  19. Soft tissue sarcomas or intramuscular haematomas?

    Energy Technology Data Exchange (ETDEWEB)

    Taieb, Sophie [Department of Radiology, Centre Oscar Lambret, 03, rue Frederic Combemale, BP 307, 59020 Lille Cedex (France)], E-mail: s-taieb@o-lambret.fr; Penel, Nicolas [Department of Oncology, Centre Oscar Lambret, 03, rue Frederic Combemale, BP 307, 59020 Lille Cedex (France); Vanseymortier, Luc [Department of Surgery, Centre Oscar Lambret, 03, rue Frederic Combemale, BP 307, 59020 Lille Cedex (France); Ceugnart, Luc [Department of Radiology, Centre Oscar Lambret, 03, rue Frederic Combemale, BP 307, 59020 Lille Cedex (France)

    2009-10-15

    Haematomas are common and sarcomas are rare. However the absence of trauma or a light trauma should alert the clinician to the possibility that the abnormality may represent haemorrhage into a tumor and not just haematoma, even in a haemophilic patient. Clinical findings, sonography with Doppler assessment and magnetic resonance images with contrast administration will help in the differential diagnosis. The diagnosis of a high grade sarcoma must be considered in these patients and any doubt should be resolved with a biopsy to avoid tragic consequences of missed sarcoma.

  20. Intraosseous Synovial Sarcoma of the Proximal Tibia

    OpenAIRE

    Beck, Sarah E.; G. Petur Nielsen; Raskin, Kevin A.; Schwab, Joseph H.

    2011-01-01

    Synovial Sarcoma is a malignant mesenchymal tumor that comprises 5–10% of all soft tissue sarcomas. The mean age of onset is thirty years old. Intraosseous presentation is very rare and has only been documented a few times. We report herein a case of a 53-year-old man with synovial sarcoma arising in the left proximal tibia. The patient underwent a wide surgical resection and reconstruction, followed by adjuvant chemotherapy. Three years later, the patient developed a local recurrence that re...

  1. Ovarian Follicular Atresia of Ewes during Spring Puerperium

    Directory of Open Access Journals (Sweden)

    Radoslava Vlčková

    2012-01-01

    Full Text Available The distribution of healthy and atretic follicles on the ovarian surface of improved Valachian ewes 17, 24, and 32 days postpartum is reported in this study. The number of healthy follicles was higher on day 24 postpartum and their mean diameter tended to increase to day 32 (P<0.05 with the greatest diameter of 5 mm. 78–81% of atretic follicles ≥3 mm in diameter was observed where apoptosis began in the follicular cells situated at the follicular cavity. The early atretic follicles are characterized by the presence of mitotic pictures. In one ewe 24 days postpartum, small regressive follicular cysts were observed. Contracting atresia is characterized by thickening of the theca interna even to 190 μm. Progesterone and oestradiol-17β concentrations were maintained at relatively low levels, but with no significant difference between the days postpartum.

  2. Dystocia due to schistosomus reflexus (cojoined twins in a Yankassa ewe

    Directory of Open Access Journals (Sweden)

    Ambali Hauwa Motunrayo

    2015-04-01

    Full Text Available A two years' old, white and brown Yankassa ewe, weighing 20 kg was presented with dystocia of about 48 h duration at Mabs Veterinary Centre Ltd, Lagos. On thorough physical and vaginal examination, there were evidences of feotal parts and foul smelling discharges per vagina. A systematic and calculated traction and retropulsion revealed schistosomus reflexus cojoined twins. There was a complete recovery of the ewe 3 days post operative management. Finally, this case reported a true schistosomus reflexus cojoined twins in Yankassa ewe which was successfully relieved through traction and retropulsion despite the supposedly small birth canal which precluded the procedure in ewes except for caesarian section or fetotomy

  3. Dystocia due to schistosomus reflexus (cojoined twins) in a Yankassa ewe

    Institute of Scientific and Technical Information of China (English)

    Ambali Hauwa Motunrayo; Nwoha Rosemary Ijeoma Ogechi; Garba Danjuma Bire; Kantiyok Josiah Tagwai; Omamegbe Joseph Omolathebu

    2015-01-01

    A two years' old, white and brown Yankassa ewe, weighing 20 kg was presented with dystocia of about 48 h duration at Mabs Veterinary Centre Ltd, Lagos. On thorough physical and vaginal examination, there were evidences of feotal parts and foul smelling discharges per vagina. A systematic and calculated traction and retropulsion revealed schistosomus reflexus cojoined twins. There was a complete recovery of the ewe 3 days post operative management. Finally, this case reported a true schistosomus reflexus cojoined twins in Yankassa ewe which was successfully relieved through traction and retropulsion despite the supposedly small birth canal which precluded the procedure in ewes except for caesarian section or fetotomy.

  4. Prions in milk from ewes incubating natural scrapie.

    Directory of Open Access Journals (Sweden)

    Caroline Lacroux

    2008-12-01

    Full Text Available Since prion infectivity had never been reported in milk, dairy products originating from transmissible spongiform encephalopathy (TSE-affected ruminant flocks currently enter unrestricted into the animal and human food chain. However, a recently published study brought the first evidence of the presence of prions in mammary secretions from scrapie-affected ewes. Here we report the detection of consistent levels of infectivity in colostrum and milk from sheep incubating natural scrapie, several months prior to clinical onset. Additionally, abnormal PrP was detected, by immunohistochemistry and PET blot, in lacteal ducts and mammary acini. This PrP(Sc accumulation was detected only in ewes harbouring mammary ectopic lymphoid follicles that developed consequent to Maedi lentivirus infection. However, bioassay revealed that prion infectivity was present in milk and colostrum, not only from ewes with such lympho-proliferative chronic mastitis, but also from those displaying lesion-free mammary glands. In milk and colostrum, infectivity could be recovered in the cellular, cream, and casein-whey fractions. In our samples, using a Tg 338 mouse model, the highest per ml infectious titre measured was found to be equivalent to that contained in 6 microg of a posterior brain stem from a terminally scrapie-affected ewe. These findings indicate that both colostrum and milk from small ruminants incubating TSE could contribute to the animal TSE transmission process, either directly or through the presence of milk-derived material in animal feedstuffs. It also raises some concern with regard to the risk to humans of TSE exposure associated with milk products from ovine and other TSE-susceptible dairy species.

  5. Fungal diversity in cow, goat and ewe milk.

    Science.gov (United States)

    Delavenne, Emilie; Mounier, Jerome; Asmani, Katia; Jany, Jean-Luc; Barbier, Georges; Le Blay, Gwenaelle

    2011-12-01

    Knowledge of fungal diversity in the environment is poor compared with bacterial biodiversity. In this study, we applied the denaturing high-performance liquid chromatography (D-HPLC) technique, combined with the amplification of the ITS1 region from fungal rDNA, for the rapid identification of major fungal species in 9 raw milk samples from cow, ewe and goat, collected at different periods of the year. A total of 27 fungal species were identified. Yeast species belonged to Candida, Cryptococcus, Debaryomyces, Geotrichum, Kluyveromyces, Malassezia, Pichia, Rhodotorula and Trichosporon genera; and mold species belonged to Aspergillus, Chrysosporium, Cladosporium, Engyodontium, Fusarium, Penicillium and Torrubiella genera. Cow milk samples harbored the highest fungal diversity with a maximum of 15 species in a single sample, whereas a maximum of 4 and 6 different species were recovered in goat and ewe milk respectively. Commonly encountered genera in cow and goat milk were Geotrichum candidum, Kluyveromyces marxianus and Candida spp. (C. catenulata and C. inconspicua); whereas Candida parapsilosis was frequently found in ewe milk samples. Most of detected species were previously described in literature data. A few species were uncultured fungi and others (Torrubiella and Malassezia) were described for the first time in milk. PMID:21944758

  6. Histology and imaging of soft tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Kind, Michele [Departement d' Imagerie Medicale, Institut Bergonie, 229 cours de l' Argonne, 33076 Bordeaux Cedex (France)], E-mail: kind@bergonie.org; Stock, Nathalie; Coindre, Jean Michel [Departement de Pathologie, Institut Bergonie, 229 cours de l' Argonne, 33076 Bordeaux Cedex (France); Universite Victor Segalen Bordeaux 2, 146 rue Leo Saignat, 33076 Bordeaux Cedex (France)

    2009-10-15

    Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.

  7. Histology and imaging of soft tissue sarcomas

    International Nuclear Information System (INIS)

    Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.

  8. Biphasic synovial sarcoma in mandibular region

    Directory of Open Access Journals (Sweden)

    Vijay Wadhwan

    2011-01-01

    Full Text Available The term synovioma was coined by Smith in 1927, and later in 1936 Knox suggested the name synovial sarcoma. It occurs primarily in the paraarticular regions, usually in close association with tendon sheaths, bursae, and joint capsules. On rare occasions it may be seen in areas without any apparent relationship to synovial structures as in parapharyngeal region or the abdominal cavity. The first description of synovial sarcoma in the head and neck region was by Pack and Ariel in 1950. The majority of these tumors seem to take origin from paravertebral connective tissue spaces and manifest as solitary retropharyngeal or parapharyngeal masses near the carotid bifurcation. Synovial sarcoma has been reported in soft palate, tongue, maxillofacial region, angle of mandible, sternoclavicular region, scapular region, and the esophagus. We report a case of 28-year-old male patient with synovial sarcoma in mandibular region with biphasic pattern.

  9. [Grading of soft tissue and bone sarcomas].

    Science.gov (United States)

    Petersen, I; Wardelmann, E

    2016-07-01

    Malignancy grading is an essential element in the classification of sarcomas. It correlates with the prognosis of the disease and the risk of metastasis. This article presents the grading schemes for soft tissue, bone and pediatric sarcomas. It summarizes the histological criteria of the Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) system and the Pediatric Oncology Group as well as the grading of bone tumors by the College of American Pathologists (CAP). Furthermore, the potential relevance of gene expression signatures, the complexity index in sarcoma (CINSARC) and single genetic alterations (p53, MDM2, p16, SWI/SNF, EWSR1 fusions and PAX3/PAX7-FOXO1 fusions) for the prognosis of sarcomas are discussed. PMID:27384333

  10. General Information about Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... sarcoma include the following: Past treatment with radiation therapy for certain cancers. Being exposed to certain chemicals , such as Thorotrast (thorium dioxide), vinyl chloride , or arsenic . Having swelling ( lymphedema ) in the arms or legs for a long ...

  11. Stages of Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... sarcoma include the following: Past treatment with radiation therapy for certain cancers. Being exposed to certain chemicals , such as Thorotrast (thorium dioxide), vinyl chloride , or arsenic . Having swelling ( lymphedema ) in the arms or legs for a long ...

  12. Treatment Option Overview (Adult Soft Tissue Sarcoma)

    Science.gov (United States)

    ... sarcoma include the following: Past treatment with radiation therapy for certain cancers. Being exposed to certain chemicals , such as Thorotrast (thorium dioxide), vinyl chloride , or arsenic . Having swelling ( lymphedema ) in the arms or legs for a long ...

  13. Treatment Options for Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... sarcoma include the following: Past treatment with radiation therapy for certain cancers. Being exposed to certain chemicals , such as Thorotrast (thorium dioxide), vinyl chloride , or arsenic . Having swelling ( lymphedema ) in the arms or legs for a long ...

  14. Immunosuppressive Therapy-Related Kaposi Sarcoma

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  15. Childhood Soft Tissue Sarcoma: Treatment Information

    Science.gov (United States)

    ... Kidney/Wilms Tumor Liver Cancer Lymphoma (Non-Hodgkin) Lymphoma (Hodgkin) Neuroblastoma Osteosarcoma Retinoblastoma Rhabdomyosarcoma Skin Cancer Soft Tissue Sarcoma Thyroid Cancer Understanding Children's Cancer Anxiety Around Procedures Childhood Cancer Statistics Late ...

  16. Pulmonary metastasectomy from soft tissue sarcomas

    OpenAIRE

    Rodrigo Afonso da Silva Sardenberg; Luiz Poli de Figueiredo; Fábio José Haddad; Jefferson Luiz Gross; Riad Naim Younes

    2010-01-01

    INTRODUCTION: Isolated pulmonary metastases from soft tissue sarcomas occur in 20-50% of these (the issue is about metastases, not lung cancer) patients, and 70% of these patients will present disease limited only to the lungs. Surgical resection is well accepted as a standard approach to treat metastases from soft tissue sarcomas isolated in the lungs, and many studies investigating this technique have reported an overall 5-year survival ranging from 30-40%. The most consistent predictor of ...

  17. Primary biphasic synovial sarcoma of the orbit.

    OpenAIRE

    Ratnatunga, N.; Goodlad, J. R.; Sankarakumaran, N.; Seimon, R.; Nagendran, S.; Fletcher, C D

    1992-01-01

    Synovial sarcoma is one of the most common soft tissue malignancies of adolescents and young adults. Despite its name, it is no longer thought to be histogenetically derived from the synovium. What seems to be the first case of synovial sarcoma to arise in the orbit presented in a 21 year old woman as a slowly enlarging subconjunctival mass. Although this tumour was typically biphasic, the monophasic spindle cell variant arising at this site could easily be confused with less aggressive orbit...

  18. Biphasic synovial sarcoma in mandibular region

    OpenAIRE

    Vijay Wadhwan; Sangeeta Malik; Nitin Bhola; Minal Chaudhary

    2011-01-01

    The term synovioma was coined by Smith in 1927, and later in 1936 Knox suggested the name synovial sarcoma. It occurs primarily in the paraarticular regions, usually in close association with tendon sheaths, bursae, and joint capsules. On rare occasions it may be seen in areas without any apparent relationship to synovial structures as in parapharyngeal region or the abdominal cavity. The first description of synovial sarcoma in the head and neck region was by Pack and Ariel in 1950. The majo...

  19. Bronchopulmonary Kaposi's sarcoma in patients with AIDS.

    OpenAIRE

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

    1992-01-01

    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...

  20. Surgical management of soft tissue sarcomas

    International Nuclear Information System (INIS)

    This volume reflects the latest thinking in surgical and adjuvant forms of therapy that can be offered to the sarcoma patient. Based on their analysis of sarcoma patients, the authors stress management based on site of origin, and discuss tumors on and about the shoulder girdle, hip joint, extremity, retroperitoneum, etc. Coverage includes methods for limb preservation; techniques for regional node resection; indications and methods for arterial perfusion, cryosurgery and isotope implantation; pre- and post-operative immunotherapy chemotherapy and radiation therapy

  1. Local Recurrence of Extremity Soft Tissue Sarcoma.

    Science.gov (United States)

    Guerrero, Whitney M; Deneve, Jeremiah L

    2016-10-01

    The management of recurrent soft tissue sarcoma is a challenging problem for clinicians and has a significant physical, mental, emotional, and oncologic impact for the patient. Despite excellent limb-preservation therapies, approximately one-quarter of patients may eventually develop recurrence of disease. How to most appropriately manage these patients is a matter of debate. Several treatment options exist, including surgical resection, irradiation, systemic chemotherapy, amputation, and regional therapies. This article highlights the management of recurrent extremity soft tissue sarcoma. PMID:27542648

  2. Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas

    Science.gov (United States)

    2016-05-13

    Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Recurrent Childhood Soft Tissue Sarcoma

  3. Undifferentiated embryonal sarcoma of liver

    Directory of Open Access Journals (Sweden)

    Avyakta Kallam

    2015-12-01

    Full Text Available Undifferentiated embryonal sarcoma of the liver (UESL is a rare malignant hepatic tumor. A 47 year old male presented with symptoms of sour taste in his mouth, occasional nausea, indigestion and 15-pound weight loss over two months. He had an unremarkable upper gastrointestinal endoscopy. Imaging showed a large liver mass in the left hepatic lobe that was resected and then reported as UESL. He went on to develop lung metastases and was initially treated with doxorubicin and ifosfamide followed by switching of therapy to gemcitabine and docetaxel due to progression of disease. He had a good response after two cycles and went on to receive four more cycles, achieving stable disease. We can therefore conclude that the combination of gemcitabine and docetaxel is a potential therapeutic option for patients with UESL.

  4. Undifferentiated Embryonal Sarcoma of Liver.

    Science.gov (United States)

    Kallam, Avyakta; Krishnamurthy, Jairam; Kozel, Jessica; Shonka, Nicole

    2015-12-29

    Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant hepatic tumor. A 47 year old male presented with symptoms of sour taste in his mouth, occasional nausea, indigestion and 15-pound weight loss over two months. He had an unremarkable upper gastrointestinal endoscopy. Imaging showed a large liver mass in the left hepatic lobe that was resected and then reported as UESL. He went on to develop lung metastases and was initially treated with doxorubicin and ifosfamide followed by switching of therapy to gemcitabine and docetaxel due to progression of disease. He had a good response after two cycles and went on to receive four more cycles, achieving stable disease. We can therefore conclude that the combination of gemcitabine and docetaxel is a potential therapeutic option for patients with UESL. PMID:26788276

  5. Primary synovial sarcoma of lung

    Directory of Open Access Journals (Sweden)

    Devleena

    2014-01-01

    Full Text Available 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.

  6. Synovial sarcoma of the mandible

    Directory of Open Access Journals (Sweden)

    Maryam Khalili

    2012-01-01

    Full Text Available Synovial sarcoma (SS is a relatively common soft tissue tumor but only 6%-7% of cases are diagnosed in the head and neck region. It typically occurs in young adults and is slightly more common in males. The most common sites in the head and neck region are hypopharynx and parapharyngeal spaces. However, SS can also occur in tonsils, tongue, and orofacial soft tissues. It is not difficult to diagnose SS microscopically with its classic biphasic appearance, but the diagnosis of monophasic forms is more challenging especially in unusual locations. In this article, we report a rare case of monophasic SS of the mandible. The clinical, histopathological, and immunohistochemical features are discussed and compared with previously reported cases in the literature. To our knowledge, only six primary involvements have been reported in the jaws. Therefore, our case represents the seventh reported case of SS in the area.

  7. Synovial sarcoma of the heart

    International Nuclear Information System (INIS)

    A young male presented with dyspnoea and was found to have a diastolic murmur at the left lower sternal edge. Trans-thoracic and trans oesophageal echocardiograms found a large mass attached to the tricuspid valve, which was projecting into the right atrium and the right ventricle. The mass was causing significant obstruction of the right ventricular inflow. Emergency surgery had to be performed because the patient developed severe vomiting (of unknown cause) leading to haemodynamic compromise. This condition was not responding to fluid resuscitation as there was obstruction to right ventricular inflow. Intraoperatively the mass was removed and the tricuspid valve was repaired. The histological and immunohistochemical examination of the excised tissue confirmed the rare diagnosis of synovial sarcoma of the heart. Post surgical imaging showed no metastases. The patient received postoperative radio therapy and chemotherapy. The tumour recurred after 6 months and the patient succumbed to his illness and expired. (author)

  8. Primary Synovial Cell Sarcoma of the Heart: A Rare Case

    OpenAIRE

    Anil Sharma; Sunil Dixit; Mohit Sharma; Neeraj Sharma; Omeshwar Sharma

    2015-01-01

    A synovial sarcoma (also known as malignant synovioma) is a rare form of cancer that usually occurs near the joints of the arm, neck, or leg. It is one of the soft tissue sarcomas. Primary cardiac neoplasms are rare. Most common site for synovial sarcoma is lower limb. Synovial sarcoma of the heart is extremely rare. Occurrence of synovial sarcoma at extra synovial site is very uncommon. It is two times common in right side heart than left side so left side synovial sarcoma is rarest of rare,...

  9. Imaging sarcomas of the great vessels and heart.

    Science.gov (United States)

    Bendel, Emily C; Maleszewski, Joseph J; Araoz, Philip A

    2011-10-01

    Primary sarcomas of the aorta, pulmonary artery, superior vena cava, inferior vena cava, and the heart are rare neoplasms. Aortic sarcomas are broadly categorized as either primarily luminal or primarily mural, with luminal sarcomas more likely to be misdiagnosed as thrombus. Pulmonary artery sarcomas are often mistaken for pulmonary embolism both clinically and at imaging. Vena caval sarcomas appear as intraluminal or extraluminal masses connecting to or filling the veins. The most common are leiomyosarcomas of the inferior vena cava. Primary sarcomas of the heart are rare and usually appear as heterogeneous aggressive masses. PMID:21963161

  10. Carbon ion radiotherapy for sarcomas

    International Nuclear Information System (INIS)

    Principles of heavy ion therapy, its application to bone and soft tissue sarcomas and outline of its general state are described. The heavy ion therapy has advantages of its high dose distribution to the target and strong biological effect due to the Bragg peak formation and high linear energy transfer, respectively. The authors use carbon ion generated by Heavy Ion Medical Accelerator in Chiba (HIMAC) for the therapy of performance state 0-2 patients with the sarcomas unresectable, diagnosed pathologically, and of 60 y, 45% and teens, 8%) have been treated, whose tumor site has been the pelvis in 73%, volume >600 mL in 63%, tissue type of bone tumor in 70% (where cordoma has amounted to>200 cases). Five-year local control rate is found 71% and survival, 59%. In 175 therapeutically fresh cases with sacral cordoma of median age 67 y, with median clinical target volume 9 cm, treated with median dose 70.4 GyE/16 irradiations, the 8-y local control rate is found to be 69% and survival, 74%, within the median follow-up 54 months; with severe skin ulcer in 2 cases and deterioration of nervous dysfunction in 15 cases; suggesting the therapy is as effective and useful as surgical resection. At present, the therapy is not applicable to Japan health insurance. In the author's hospital, the heavy ion therapy has been conducted to total of >6,000 patients, which amounting to the largest number in the world. Now, 3 Japanese facilities can do the therapy as well and 3 countries in the world.(T.T.)

  11. Sarcoma sinovial anorretal: relato de caso

    Directory of Open Access Journals (Sweden)

    Hernán Augusto Centurión Sobral

    2006-03-01

    Full Text Available Os sarcomas são neoplasias que se originam das células mesenquimais primitivas, sendo raros na região anorretal. O objetivo é relatar um caso de sarcoma sinovial anorretal, neoplasia extremamente rara nesta localização. É descrito o caso de uma paciente de 77 anos que apresentava nodulação anal dolorosa e sangrante às evacuações, associada a puxo, tenesmo e perda ponderal. A lesão foi biopsiada e o estudo imunohistoquímico evidenciou sarcoma sinovial anorretal. A paciente foi submetida a amputação abdomino-perineal do reto, encontra-se assintomática, sem sinais de recidiva e em seguimento ambulatorial.Originated from mesenchymal cells, the sarcoma is rare in the anorectal area. The authors report a case of anorectal sinovial sarcoma, extremely rare in this location with no previous reports on literature. It's described a case of a 77 years old patient presenting an anal tumor, associated to pain, bleeding, tenesmus and weight loss. A synovial sarcoma was diagnosed after biopsy and imunohistochemical study. The pacient was submitted to a Miles procedure being assymptomatic and without any signs of disease in the last seven months.

  12. Time-to-event analysis of mastitis at first-lactation in Valle del Belice ewes

    NARCIS (Netherlands)

    Portolano, B.; Firlocchiaro, R.; Kaam, van J.B.C.H.M.; Riggio, V.; Maizon, D.O.

    2007-01-01

    A time-to-event study for mastitis at first-lactation in Valle del Belice ewes was conducted, using survival analysis with an animal model. The goals were to evaluate the effect of lambing season and level of milk production on the time from lambing to the day when a ewe experienced a test-day with

  13. Genetic evaluation of the probability of lambing in yearling Targhee ewes

    Science.gov (United States)

    The objective of this study was to determine the additive genetic control of lambing percentage in yearling Targhee ewes. The records of 3,103 ewe lambs born from 1989 to 2011 and mated at approximately 7.5 mo of age were analyzed. Records included sire, dam, weaning weight, breeding pen, age of dam...

  14. Effect of zinc from zinc sulfate on trace mineral concentrations of milk in Varamini ewes

    NARCIS (Netherlands)

    Zali, A.; Ganjkhanlou, M.

    2009-01-01

    This study was conducted to evaluate the effect of feeding supplemental zinc (zinc sulfate) in different levels (15, 30, or 45 mg/kg) on trace mineral concentrations in milk of ewes. Thirty lactating Varaminni ewes were assigned to three experimental groups according to their live body weights, milk

  15. Effect of vitamin E on the immune system of ewes during late pregnancy and lactation

    Science.gov (United States)

    The present experiment was designed to determine the effects of a regimen of repeated, intramuscular (i.m.) injections of vitamin E (VE) on innate and humoral components of the immune response of pregnant and lactating ewes. Pregnant ewes were randomly assigned to two treatments consisting of i.m. i...

  16. Measurements repeated in time carried out by ultrasound in discard ewes in different physiological stages

    Directory of Open Access Journals (Sweden)

    Rafael Silvio Bonilha Pinheiro

    2014-09-01

    Full Text Available Some measurements carried out by ultrasound in the Longissimus dorsi between the 12th and 13th ribs were monitored, aiming to know the changes in the muscular and adipose tissues in that muscle and the connections of these measurements with body weight (BW and body condition score (BCS of ewes in different stages (physiological stages and experimental periods. Ewes were arranged into the following treatments: EL = ewes which remained in lactation for 60 days with their respective lambs and slaughtered one day after weaning; EWL = ewes which remained in lactation for 60 days with their respective lambs and one more period of approximately 30 days without the lambs and slaughtered afterwards; and ENC = ewes which did not give birth during the year. Ultrasound measurements were carried out in the beginning, middle and end of the experiment (every 21 days, as well as BW and BCS of ewes. There was not interaction between periods and experimental treatments for BW and BCS of ewes. It was concluded that the use of ultrasound allows monitoring changes that occur with the subcutaneous fat and Longissimus dorsi of ewes. Those changes occur more frequently in intervals of 42 days (muscular and adipose tissues.

  17. Do We Know What Causes Soft Tissue Sarcomas?

    Science.gov (United States)

    ... Topic Can soft tissue sarcomas be prevented? Do we know what causes soft tissue sarcomas? Scientists still ... the instructions for nearly everything our cells do. We usually look like our parents because they are ...

  18. Primary synovial cell sarcoma of the heart: A rare case

    Directory of Open Access Journals (Sweden)

    Anil Sharma

    2015-01-01

    Full Text Available A synovial sarcoma (also known as malignant synovioma is a rare form of cancer that usually occurs near the joints of the arm, neck, or leg. It is one of the soft tissue sarcomas. Primary cardiac neoplasms are rare. Most common site for synovial sarcoma is lower limb. Synovial sarcoma of the heart is extremely rare. Occurrence of synovial sarcoma at extra synovial site is very uncommon. It is two times common in right side heart than left side so left side synovial sarcoma is rarest of rare, only very limited cases are reported. Here, we report a case of primary synovial cell sarcoma of the left side of the heart in a 26-year-old female. She presented with dyspnea and weakness only. She was operated for left-sided mass covering mitral valve; further histo-pathology revealed the mass as synovial cell sarcoma. In Indian scenario, this type of rare case needs documentation.

  19. What Should You Ask Your Doctor about Uterine Sarcoma?

    Science.gov (United States)

    ... after treatment for uterine sarcoma? What should you ask your doctor about uterine sarcoma? It is important ... your work schedule. You may also want to ask about second opinions or about clinical trials for ...

  20. What Should You Ask Your Doctor about Kaposi Sarcoma?

    Science.gov (United States)

    ... after treatment for Kaposi sarcoma? What should you ask your doctor about Kaposi sarcoma? As you cope ... honest, open discussions with your doctor. You should ask any question on your mind no matter how ...

  1. Soft tissue sarcoma - diagnosis and treatment

    International Nuclear Information System (INIS)

    Significant progress in the treatment of soft tissue sarcoma (STS), both primary tumor and local recurrences/metastatic disease, has been achieved in recent years. Surgery is essential modality, but the use of combined treatment (standard combination of surgery with adjuvant radiotherapy, chemotherapy in selected cases and perioperative rehabilitation) in highly-experienced centers increased possibility of cure and limitations of extent of local surgery. Current combined therapy together with the use of reconstructive methods allows for limb-sparing surgery in majority of soft tissue sarcoma patients (amputation in 10% of cases as compared to approximately 50% in the 1960 - 70s). The slow, but constant, increase of rate of soft tissue sarcoma patients with long-term survival has been observed. Contemporary 5-year overall survival rate in patients with extremity soft tissue sarcomas is 55 -78%. In case of diagnosis of metastatic disease the prognosis is still poor (survival of approximately 1 year). Good results of local therapy may be expected only after planned (e.g., after preoperative biopsy - tru - cut or incisional) radical surgical excision of primary tumor with pathologically negative margins (R0 resection). Following appropriate diagnostic check-up, adjuvant radiotherapy is necessary in the majority of patients treated with radical surgery need, as well as long-term rehabilitation and follow-up examinations in treating center are needed for at least 5 years. The progress is due to the introduction of targeted therapy acting on molecular or genetic cellular disturbances detected during studies on etiopathogenetic mechanisms of sarcoma subtypes. In view of rarity of sarcomas and necessity of multidisciplinary therapy, the crucial issue is that management of these tumors should be hold in experienced oncological sarcoma centers. (authors)

  2. Synovial sarcoma of kidney in a child: A rare presentation

    OpenAIRE

    Venkatraman Radhakrishnan; Manikandan Dhanushkodi; Kathiresan Narayanswamy; Anand Raja; Shirley Sundersingh; Tenali Sagar

    2016-01-01

    There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms’ tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms’ tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with chang...

  3. Primary Synovial Sarcoma of the Parietal Pleura: A Case Report

    OpenAIRE

    Kang, Min-Kyun; Cho, Kwang-Hyun; Lee, Yang-Haeng; Han, Il-Yong; Yoon, Young Chul; Park, Kyung-Taek; Kang, Do Kyun; Kim, Bo-Mi

    2013-01-01

    Synovial sarcoma is a malignant soft tissue tumor that most commonly occurs in the extremities of young and middle-aged adults, in the vicinity of large joints. Although synovial sarcoma is frequently associated with joints, it may arise in unexpected sites, such as the mediastinum, heart, lung, pleura, or chest wall. Primary synovial sarcoma of the pleura is rare. To date, nearly 36 cases of primary synovial sarcoma of the pleura have been reported since Gaertner et al. published the first c...

  4. Expression of Smooth Muscle Calponin in Synovial Sarcoma

    OpenAIRE

    Katsuhito Takahashi; Hiroshi Sonobe; Masaharu Tatsuta; Nobuhito Araki; Nobuyuki Hashimoto; Yumiko Koyanagi; Masayuki Manou; Shingo Ishiguro; Hiroko Funai; Ikuo Kudawara; Takafumi Ueda; Hisako Yamamura; Hidefumi Ono; Hideki Yoshikawa

    1999-01-01

    Purpose. Histogenesis of synovial sarcoma remains controversial and reliable molecular markers for diagnosis are necessary. Expression of basic calponin, a smooth muscle differentiation-specific actin-binding protein, was studied in synovial sarcoma. Subjects and Methods. The basic calponin gene and the gene product were analyzed by reverse transcription PCR analysis (RT-PCR) and immunohistochemistry in 14 synovial sarcomas and a human synovial sarcoma cell line (HS-SY-II). Results and Discus...

  5. Epithelioid sarcoma : Still an only surgically curable disease

    NARCIS (Netherlands)

    de Visscher, Sebastiaan A. H. J.; van Ginkel, Robbert J.; Wobbes, Theo; Veth, Rene P. H.; ten Heuvel, Suzanne E.; Suurmeijer, Albert J. H.; Hoekstra, Harad J.

    2006-01-01

    BACKGROUND. Epithelioid sarcoma is a rare soft tissue sarcoma with a known high propensity for locoregional recurrence and distant metastases. The clinical behavior and prognostic factors that influence the survival of patients with epithelioid sarcoma were studied. METHODS. Twenty-three patients, i

  6. Synovial sarcoma arising in association with a popliteal cyst

    International Nuclear Information System (INIS)

    Synovial sarcoma is a relatively common soft tissue sarcoma particularly in the adolescent and young adult. We report an unusual case of a synovial sarcoma arising within a popliteal cyst in a 13-year-old female presenting with bilateral popliteal cysts. MR imaging demonstrated the cyst with evidence of subacute haemorrhage and a discrete nodule of tumour. (orig.)

  7. Primary Synovial Sarcoma of Lung: A Rare Tumor.

    Science.gov (United States)

    Raj, Prince; Kumar, Parveen; Sarin, Yogesh Kumar

    2016-01-01

    Synovial sarcoma of lung is a rare tumor with few case reports in literature. Though named synovial sarcoma due to its resemblance to synovium on light microscopy, it arises from mesenchymal tissue. Here, we present a case of synovial sarcoma of lung in a 7-year old boy, with main emphasis on difficulty faced in the management. PMID:27170917

  8. Synovial sarcoma arising in association with a popliteal cyst

    Energy Technology Data Exchange (ETDEWEB)

    Ayoub, K.S.; Grimer, R.J. [Royal Orthopaedic Hospital, Birmingham (United Kingdom). Dept. of Orthopaedic Oncology; Davies, A.M. [MRI Centre, Royal Orthopaedic Hospital, Birmingham (United Kingdom); Mangham, D.C. [Dept. of Pathology, Royal Orthopaedic Hospital, Birmingham (United Kingdom); Twiston Davies, C.W. [Jersey General Hospital (United Kingdom). Dept. of Orthopaedic Surgery

    2000-12-01

    Synovial sarcoma is a relatively common soft tissue sarcoma particularly in the adolescent and young adult. We report an unusual case of a synovial sarcoma arising within a popliteal cyst in a 13-year-old female presenting with bilateral popliteal cysts. MR imaging demonstrated the cyst with evidence of subacute haemorrhage and a discrete nodule of tumour. (orig.)

  9. Monophasic synovial sarcoma of the pharynx: a case report

    OpenAIRE

    Betal, Dibendu; Babu, Ramesh; Mehmet, Veysi

    2009-01-01

    Synovial sarcomas are a rare form of soft tissue sarcomas. We present a case of a 62 year-old male presenting with a left thyroid lump initially though to be a thyroid adenoma but subsequently diagnosed as a monophasic synovial sarcoma of the pharynx. We discuss the diagnosis and treatment of this case.

  10. A case of epithelioid sarcoma arising in the vulva

    OpenAIRE

    Kim, Ju Hyun; Choi, Youn Seok; Lee, Tae Sung

    2008-01-01

    Epithelioid sarcoma of vulva is extremely rare. Only 20 cases have been reported in the literature so far. Early diagnosis is difficult because of its benign appearance as a painless subcutaneous nodule. Therefore treatment can be delayed, although epithelioid sarcoma requires prompt surgery. We report a case of epithelioid sarcoma that occurred in the left vulva with a brief review of literature.

  11. [Demonstration and evolution of chorionic gonadotropin in ewes].

    Science.gov (United States)

    Lacroix, M C; Martal, J

    1979-02-26

    Occurrence of ovine Chorionic Gonadotropin (oCG) is demonstrated in placenta and amniotic fluid with the use of a radioreceptor assay (corpus luteum membranes) in ewes. Identification of oCG is possible as early as 15th day of pregnancy. It should be secreted at a constant rate, and its maximum concentration is recorded on the 130th day. This hormonal factor might be one of the major components accountable for high progesteronemia observed during the 100 last days of pregnancy. PMID:110484

  12. Somatic cell count control strategies in dairy ewes

    OpenAIRE

    Spanu, Carlo

    2010-01-01

    The consumption of milk products, especially made from raw milk, have been reported to be associated with food borne diseases. Since most sheep’s milk products are made from raw milk, it is clear how udder health is an important prerequisite to produce hygienic milk. Ewes with mastitis, particularly in their subclinical form, serve as reservoir of pathogens that can be shed into the milk and constitute a potential risk for human health. Milk somatic cell count (SCC) is not a public health con...

  13. Surrogate reactions: the Weisskopf-Ewing approximation and its limitations

    International Nuclear Information System (INIS)

    A brief description of the Surrogate reaction method, an indirect approach for determining compound-nuclear reaction cross sections, is presented. The Weisskopf-Ewing limit, an approximation scheme that is typically employed in the analysis of Surrogate experiments, is considered and its validity and limitations are discussed. Particularly the assumption in the surrogate-reaction process that the result of the initial direct reaction is a fully-equilibrated compound nucleus is not obviously correct and needs to be checked by appropriate calculations

  14. Weisskopf-Ewing calculations: neutron-induced reactions

    International Nuclear Information System (INIS)

    The cross sections of several neutron-induced reactions on 55Mn, sup(54,56)Fe, 59Co, sup(58,60)Ni and sup(63,65)Cu are calculated for energies below 20 MeV using the Weisskopf-Ewing theory and compared with experimental data. The total (n,p) and (n, α) cross sections are generally well fitted, especially when they are dominant channels. At the higher energies the (n,p) cross sections have important contributions from pre-equilibrium processes, and these are fitted using the theory of Feshbach, Kerman and Koonin. (author)

  15. Further tests of the Weisskopf-Ewing exciton model

    International Nuclear Information System (INIS)

    A phenomenological model wherein the statistical contribution to the cross section is evaluated from the Weisskopf-Ewing theory and the pre-equilibrium part from the exciton model is tested for neutron, proton and α-particle induced reactions on several nuclei. Using global prescriptions for the model parameters, this model is shown to provide a simple and reliable method for a fast calculation of the excitation functions and emission spectra in a wide energy range. The spectra of emitted particles obtained within this model are shown to be consistent with the data and with the more fundamental Feshbach, Kerman and Koonin theory. (author)

  16. Ovarian Follicular Atresia of Ewes during Spring Puerperium

    OpenAIRE

    Radoslava Vlčková; Drahomíra Sopková; Ján Pošivák; Igor Valocký

    2012-01-01

    The distribution of healthy and atretic follicles on the ovarian surface of improved Valachian ewes 17, 24, and 32 days postpartum is reported in this study. The number of healthy follicles was higher on day 24 postpartum and their mean diameter tended to increase to day 32 (P < 0.05) with the greatest diameter of 5 mm. 78–81% of atretic follicles ≥3 mm in diameter was observed where apoptosis began in the follicular cells situated at the follicular cavity. The early atretic follicles are cha...

  17. Synovial sarcoma: Clinicopathologic evaluation of 82 cases

    Directory of Open Access Journals (Sweden)

    Taner AKALIN

    2006-09-01

    Full Text Available The current retrospective study examined a relatively large group of synovial sarcoma patients of all ages who were evaluated at a single institution over a 21 year period.There were 42 females and 40 males, whose age ranged from 13 to 75 (median 34 years. The neoplasms measured from 1,5 to 24 cm in greatest diameter (median 8,5 cm. Tumor locations were as follows; lower extremities (53 cases, upper extremities (16 cases, trunk (7 cases and head-neck (3 cases.One hundred thirty six material belonging to 82 cases were evaluated histologically. Of these cases, 58 were monophasic fibrous type, 18 were biphasic type and 6 were poorly differentiated type synovial sarcoma. Immunohistochemically, monophasic fibrous type and poorly differentiated type synovial sarcoma cases (64 cases were positive for EMA or cytokeratin. Of these cases, 14 were EMA positive but cytokeratin negative, however six were cytokeratin positive but EMA negative.Follow up was available for 43 patients with a duration of median 47 months. Of the 43 patients, 12 died of disease, nine had metastatic disease, nine had local reccurence and 13 (30% had no evidence of disease.In conclusion, synovial sarcoma is an aggressive neoplasm and is affected from radiochemotherapy in varying proportions. Most of the synovial sarcoma cases can be diagnosed by the help of morphology and immunohistochemistry (by applying both EMA and cytokeratin.

  18. Sarcomas cutâneos primários Primary cutaneous sarcomas

    Directory of Open Access Journals (Sweden)

    Luiz Fernando Fróes Fleury Jr

    2006-06-01

    Full Text Available Os sarcomas com apresentação cutânea primária são tumores raros e de grande heterogeneidade histológica. Com a evolução da oncologia cutânea e da cirurgia dermatológica, os dermatologistas têm sido cada vez mais requisitados para o diagnóstico e orientação terapêutica de tumores menos freqüentes. Este artigo de revisão analisa os sarcomas cutâneos primários observando suas características clínicas, etiopatogênicas e histológicas, bem como aspectos do tratamento e evolução. Enfatiza os sarcomas de maior relevância para o dermatologista, como angiossarcoma, dermatofibrossarcoma protuberans, fibroxantoma atípico, leiomiossarcoma, lipossarcoma, tumor maligno de bainha de nervo periférico e sarcoma epitelióide. O sarcoma de Kaposi não é abordado devido a suas características individuais específicas.Soft tissue tumors represent a heterogeneous group of mesenchymal and neural lesions. The cutaneous presentation of these tumours is rare. With the evolution of dermatologic surgery and cutaneous oncology, dermatologists have emerged as specialists for skin cancer management. This article reviews primary cutaneous sarcomas with particular emphasis on the epidemiologic, clinical, and histological features of diagnosis, as well as treatment modalities and prognosis. The most frequent cutaneous sarcomas were reviewed, including angiosarcoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, leiomyosarcoma, liposarcoma, malignant nerve sheath tumor, and epithelioid sarcoma. Kaposi's sarcoma, due to specific characteristics, was omitted from this review.

  19. Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma

    Science.gov (United States)

    2016-04-05

    Adult Alveolar Soft Part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Undifferentiated Pleomorphic Sarcoma; Malignant Adult Hemangiopericytoma; Recurrent Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  20. Efficacy of trabectedin in advanced soft tissue sarcoma: beyond lipo- and leiomyosarcoma

    Directory of Open Access Journals (Sweden)

    De Sanctis R

    2015-10-01

    pleomorphic sarcoma (n=5, Ewing/peripheral primitive neuroectodermal tumor (n=1, and malignant peripheral nerve sheath tumor (n=1. Any grade AEs were noncumulative, reversible, and manageable. G3/G4 AEs included anemia (n=1, 1.4%, neutropenia (n=7, 9.6%, liver toxicity (n=6, 8.3%, and fatigue (n=2, 2.8%. With a median follow-up time of 11 (range, 2–23 months, median progression-free survival and OS of the entire cohort were 2.97 months and 16.5 months, respectively.Conclusion: Our experience confirms trabectedin as an effective therapeutic option for metastatic lipo- and leiomyosarcoma and suggests promise in synovial sarcomas and high-grade undifferentiated pleomorphic sarcoma. Keywords: trabectedin, soft tissue sarcoma, liposarcoma, leiomyosarcoma

  1. Generalized intramuscular granulocytic sarcoma mimicking polymyositis

    International Nuclear Information System (INIS)

    We report a case of granulocytic sarcoma exclusively manifesting as diffuse intramuscular infiltration of the proximal upper and lower limb girdle and the torso muscles in a patient with previous history of acute myelogenous leukemia 5a. Whole-body CT showed widespread distribution of ill-defined intramuscular, homogeneously enhancing lesions. On whole-body MRI, lesions were homogeneously hyperintense on fat saturated T2-weighted images, isointense on T1-weighted images and strongly enhancing after intravenous gadolinium contrast administration. Histopathology revealed muscular infiltration of blast cells with identical immunochemistry to the initial manifestation of leukemia, diagnostic for an extramedullary relapse manifesting as granulocytic sarcoma. CT and MRI characteristics of this previously undocumented manifestation of granulocytic sarcoma should assist in the identification of such cases. (orig.)

  2. Biphasic synovial sarcoma of oral cavity

    International Nuclear Information System (INIS)

    Synovial sarcoma is a mesenchymal spindle cell tumour, which is unrelated to synovium and shows variable epithelial differentiation. Typically, synovial sarcoma arises in the soft tissues of the extremities but cases in the head and neck region are less common and oral cavity involvement is extremely rare. A 17-year-old girl presented with a gradually increasing swelling on the right cheek for 2 years, which on biopsy, revealed a biphasic tumour comprising fascicles of spindle shaped cells with gland formation by epithelial cells and scattered masts cells. Histological diagnosis of biphasic synovial sarcoma was confirmed on immunohistochemistry by strong positivity for EMA, S-100 and CD-99 in both epithelial as well as spindle cell areas. (author)

  3. Generalized intramuscular granulocytic sarcoma mimicking polymyositis

    Energy Technology Data Exchange (ETDEWEB)

    Fritz, Jan; Claussen, Claus D.; Pereira, Philippe L.; Horger, Marius S. [Eberhard-Karls-University, Department of Diagnostic Radiology, Tuebingen (Germany); Vogel, Wichard [Eberhard-Karls-University, Department of Internal Medicine-Oncology, Tuebingen (Germany); Wehrmann, Martin [Eberhard-Karls-University, Department of Pathology, Tuebingen (Germany)

    2007-10-15

    We report a case of granulocytic sarcoma exclusively manifesting as diffuse intramuscular infiltration of the proximal upper and lower limb girdle and the torso muscles in a patient with previous history of acute myelogenous leukemia 5a. Whole-body CT showed widespread distribution of ill-defined intramuscular, homogeneously enhancing lesions. On whole-body MRI, lesions were homogeneously hyperintense on fat saturated T2-weighted images, isointense on T1-weighted images and strongly enhancing after intravenous gadolinium contrast administration. Histopathology revealed muscular infiltration of blast cells with identical immunochemistry to the initial manifestation of leukemia, diagnostic for an extramedullary relapse manifesting as granulocytic sarcoma. CT and MRI characteristics of this previously undocumented manifestation of granulocytic sarcoma should assist in the identification of such cases. (orig.)

  4. Clinical Pathological Analysis of Synovial Sarcoma

    Institute of Scientific and Technical Information of China (English)

    2007-01-01

    OBJECTIVE To investigate the clinical diagnosis and differential diagnosis of synovial sarcoma (SS).METHODS A total of 41 paraffin-embedded synovial sarcoma samples were examined by H&E staining, immunohistochemistry staining and the reverse transcriptase polymerase chain reaction (RT-PCR), in order to provide a scientific bases for diagnosis and differential diagnosis.RESULTS Twelve cases were a biphasic type, 22 cases were a monophasic fibrous type, and 7 cases were a poorly differentiated type. Thirty-six cases were both CK (and/or EMA) and Vim positive. Five cases were only Vim positive. A SYT-SSX fusion gene was detected in 18 cases by RT-PCR.CONCLUSION By observation of the histomorphology, immunohistochemistry markers and detection of a SYT-SSX fusion gene, we can make a clinical pathological diagnosis of synovial sarcoma.

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

    Science.gov (United States)

    2014-09-08

    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

  6. Synovial sarcoma of kidney in a child: A rare presentation

    Directory of Open Access Journals (Sweden)

    Venkatraman Radhakrishnan

    2016-01-01

    Full Text Available There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms′ tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms′ tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with change in her neoadjuvant chemotherapy regimen and surgery. The diagnosis of synovial sarcoma was confirmed by demonstrating the t (X, 18 translocation using polymerase chain reaction.

  7. Synovial sarcoma of kidney in a child: A rare presentation.

    Science.gov (United States)

    Radhakrishnan, Venkatraman; Dhanushkodi, Manikandan; Narayanswamy, Kathiresan; Raja, Anand; Sundersingh, Shirley; Sagar, Tenali

    2016-01-01

    There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms' tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms' tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with change in her neoadjuvant chemotherapy regimen and surgery. The diagnosis of synovial sarcoma was confirmed by demonstrating the t (X, 18) translocation using polymerase chain reaction. PMID:27046979

  8. Gastrointestinal transport of calcium and glucose in lactating ewes.

    Science.gov (United States)

    Klinger, Stefanie; Schröder, Bernd; Gemmer, Anja; Reimers, Julia; Breves, Gerhard; Herrmann, Jens; Wilkens, Mirja R

    2016-06-01

    During lactation, mineral and nutrient requirements increase dramatically, particularly those for Ca and glucose. In contrast to monogastric species, in ruminants, it is rather unclear to which extend this physiological change due to increased demand for milk production is accompanied by functional adaptations of the gastrointestinal tract (GIT). Therefore, we investigated potential modulations of Ca and glucose transport mechanisms in the GIT of lactating and dried-off sheep. Ussing-chamber technique was applied to determine the ruminal and jejunal Ca flux rates. In the jejunum, electrophysiological properties in response to glucose were recorded. Jejunal brush-border membrane vesicles (BBMV) served to characterize glucose uptake via sodium-linked glucose transporter 1 (SGLT1), and RNA and protein expression levels of Ca and glucose transporting systems were determined. Ruminal Ca flux rate data showed a trend for higher absorption in lactating sheep. In the jejunum, small Ca absorption could only be observed in lactating ewes. From the results, it may be assumed that lactating ewes compensate for the Ca loss by increasing bone mobilization rather than by increasing supply through absorption from the GIT Presence of SGLT1 in the jejunum of both groups was shown by RNA and protein identification, but glucose uptake into BBMV could only be detected in lactating sheep. This, however, could not be attributed to electrogenic glucose absorption in lactating sheep under Ussing-chamber conditions, providing evidence that changes in jejunal glucose uptake may include additional factors, that is, posttranslational modifications such as phosphorylation. PMID:27273883

  9. Ovarian responses to undernutrition in pregnant ewes, USA

    Directory of Open Access Journals (Sweden)

    Vonnahme Kimberly A

    2003-02-01

    Full Text Available Abstract In most mammals oogonia proliferate by mitosis and begin meiotic development during fetal life. Previous studies indicated that there is a delay in the progression to the first stage of meiotic arrest in germ cells of female fetuses of undernourished ewes. We report that underfeeding (50% NRC requirement beginning on Day 28 of pregnancy provokes an increase in oxidative base lesions within DNA of mid-gestational (Day 78 fetal oogonia; this condition was associated with up-regulation of the tumor suppressor/cell-cycle arrest modulator p53, antiapoptotic factor Bcl-2, and base-excision repair polymerase β. Fetal ovarian weights and germ cell concentrations were not altered by nutrient deprivation. Ovaries of ewes on control diets (100% NRC contained more tertiary follicles than their restricted counterparts; however, peripheral venous estradiol-17β was not different between groups. There was no effect of treatment on p53 accumulation in maternal oocytes. Luteal structure-function was not perturbed by undernutrition. No fetal losses were attributed to the dietary restriction. It is proposed that DNA of interphase fetal oogonia is vulnerable to oxidative insults perpetrated by a nutritional stress to the dam, and that multiple/integrated adaptive molecular response mechanisms of cell-cycle inhibition (providing the time required for base repairs and survival hence sustain the genomic integrity and population stability of the germline.

  10. Ewe welfare and ovine milk and cheese quality

    Directory of Open Access Journals (Sweden)

    A. Sevi

    2010-04-01

    Full Text Available Causes of welfare reduction in dairy sheep flocks are presented and their impact on ovine milk and cheese quality is discussed. Attention is focused on climatic extremes, poor housing and milking hygiene, and nutritional imbalance: mechanisms are outlined through which stress-induced reduction of immune function can result in poor milk composition, deteriorated renneting ability of milk and altered proteolysis in cheese during ripening. In particular, the impact is brought out of exposure to high ambient temperature on the nutritional properties of ewe milk, in terms of increased short-chain and saturated fatty acids, and decreased unsaturated to saturated fatty acid ratio. As well, the relationship is highlighted between ewe welfare and udder health. Especially under poor hygiene conditions the risk of mastitis markedly increases due to reduction of the natural defense mechanisms of the teat and mammary gland and increased number and pathogenicity of the micro-organisms in contact with the entrance of the teat canal. Evidence is provided that rise in milk somatic cell count, in response to bacteria penetration into the udder, can lead to decreased milk yield and altered composition of milk and cheese, due to extensive epithelium secretory cell damage.

  11. Machine milking management and milk nitrogen fractions in primiparous ewes

    Directory of Open Access Journals (Sweden)

    G. Pulina

    2010-04-01

    Full Text Available Little work has been carried out on management techniques to reduce stress of first machine milking in ewes. The aim of this work was to evaluate the effect of milking parlour training before weaning on sheep milk yield and milk protein fractions. One week before weaning, an experimental group (EG was introduced into the machine milking parlour and hand-milked once a day, to adapt them to the new environment and noise of the milking machine, while a control group (CG was hand-milked once a day in a traditional fold parlour until weaning. After weaning, both groups were machine milked, separately, twice a day in the milking parlour. Milk yield and nitrogen fractions were recorded for the first 10 days of machine milking. Differences between samplings within group were observed for all parameters considered. Only on the first day of machine milking, differences between groups occurred for total nitrogen, total protein and casein. Higher content of nitrogen fractions in the milk of EG was due to its lower milk production. In fact, daily production of nitrogen fractions was higher in CG than in EG. High variability for most parameters was observed during the first 5 days of machine milking in both groups. A week of training to machine milking parlour was not enough to reduce the negative effects of weaning and machine milking stresses on primiparous ewes. (interaction?

  12. Cadmium and lead elimination through milk in the ewe

    Energy Technology Data Exchange (ETDEWEB)

    Houpert, P.; Mehennaoui, S.; Federspiel, B.; Milhaud, G. [Equipe associee INRA-ENV Alfort, 94 - Maisons-Alfort (France)

    1997-12-31

    Cadmium and lead are heavy metals widely distributed in industrialized countries, and known for their cumulative properties in continental ecological cycling. The purpose of the experiments was to study lead and cadmium transfer from ewe feed to their milk and meat, in view of protecting human consumers. Ewes were given daily oral administrations of lead, cadmium or associations of lead-cadmium, lead-cadmium-zinc for 52 days. Lead and cadmium were measured in blood, milk and tissues. About 0.1 pc of the total lead and 0.002-0.004 pc of the total cadmium dose was excreted via milk during the lactation, with milk concentrations of about 120 microgram per liter for lead and about 3.5 micro-g./l for cadmium. Sixty days after the last administration, 0.16 pc of the total cadmium dose was retained in liver and 0.09 pc in kidneys. Cadmium and lead concentrations in the muscle remained low

  13. Peripheral blood and milk leukocytes subsets of lactating Sarda ewes

    Directory of Open Access Journals (Sweden)

    Piero Bonelli

    2013-05-01

    Full Text Available Leukocytes subpopulations in blood and milk of lactating Sarda ewes were investigated. Animals characterized by a SSC level <500×103cells/mL and a negative bacteriological examination were sampled in early, mid and late lactation. Milk differential cell count evidenced that macrophage represented the main population (42.8%±3.5 followed by lymphocytes (40.2%±3.4 and neutrophils (8,6%±2.1. Flow cytometry analysis showed that lymphocytes subsets in milk were quite different from blood. High CD8+ and low CD4+ lymphocytes percentages determined a CD4/CD8 ratio inversion in milk compared to blood (0.3%±0.03 vs 1.8%±0.08. CD8+ decreased while, conversely, CD4+ increased in late lactation. γδ T cells were more represented in milk (12.6%±1.3 than in blood (6.8%±0.3 and their proportions appeared similar throughout lactation in both compartments. IL-2 receptor was mainly expressed in milk on T cytotoxic lymphocytes. Data obtained in uninfected mammary glands could allow an early discrimination between physiological and pathological changes occurring in ewe milk. Further phenotypical and functional studies on milk leukocytes subsets might help to understand defense mechanisms of the ovine mammary gland against IMI.

  14. Primary Renal Synovial Sarcoma: An Oncologic Surprise

    Directory of Open Access Journals (Sweden)

    H. Krishna Moorthy

    2014-09-01

    Full Text Available Primary renal synovial sarcoma is a rare tumor having a specific chromosomal translocation t(X; 18 (p11.2; q11.2. The clinical features of this tumor and radiologic appearances are quite similar to those of renal cell carcinoma. Confirmatory diagnosis requires fluorescent in situ hybridization or reverse transcriptase polymerase chain reaction validation for differentiating the tumors from sarcomatoid renal cell carcinoma. We present a case of primary renal synovial sarcoma that was diagnosed in a middle-aged man.

  15. Primary pulmonary monophasic synovial sarcoma: Evading diagnosis.

    Science.gov (United States)

    Taylor, Marcus; Srinivasan, Lakshmi; Abid, Qamar

    2016-02-01

    Primary pulmonary synovial sarcoma is a very rare tumor, thus there is no consensus as to the most appropriate management. A 78-year-old man presented with nonspecific symptoms of weight loss and shortness of breath. Imaging confirmed a large right-sided mass and accompanying pleural effusion. Strong 18F-fluorodeoxyglucose uptake was found on positron-emission tomography. The preoperative work-up and intraoperative frozen section were inconclusive. Immunohistochemistry and molecular analysis confirmed the diagnosis of primary pulmonary monophasic synovial sarcoma. PMID:26612959

  16. Primary Synovial Sarcoma of the Thyroid Gland

    OpenAIRE

    Jang, Ki-Seok; Min, Kyueng-Whan; Jang, Si-Hyong; Paik, Seung Sam; Tae, Kyung; Jang, Se Jin; Park, Moon Hyang

    2007-01-01

    Synovial sarcoma is a rare but distinct soft tissue neoplasm, most commonly occurring in para-articular regions of the extremities of young adults and also occurring in the head and neck region. To the best of our knowledge, only one case of primary synovial sarcoma of the thyroid has been previously reported. Here, we report a 15-yr-old man who had a chief complaint of a palpable neck mass. The neck computed tomography revealed a relatively well-demarcated solid mass in the left thyroid glan...

  17. [Adjuvant chemotherapy of adults soft tissue sarcomas].

    Science.gov (United States)

    Bui-Nguyen, B; Italiano, A; Delva, F; Toulmond, M

    2010-06-01

    The main progress in the management of soft tissue sarcomas have been obtained in the field of local control. Although the main evolutive, vital, risk of these diseases is metastatic dissemination, efficacy of adjuvant chemotherapy remains a controversial issue. Thus, adjuvant chemotherapy cannot be considered as a standard for any situation. The last results of clinical trials, meta-analysis and population studies are presented and discussed in this article. New therapeutic strategies are to be developed to prevent metastases in soft tissue sarcomas. This needs a better understanding of the biology of those tumors, of metastases risk factors and of the determinants of systemic therapies efficacy in these tumors. PMID:20547481

  18. Soft tissue sarcoma of the extremity.

    LENUS (Irish Health Repository)

    Cooper, T M

    2012-02-03

    A retrospective review of 33 cases of soft tissue sarcoma of the extremity presenting over a 10 year period was undertaken. The history, patterns of referral, diagnostic investigations, procedures undertaken and outcomes were studied. We found there was a frequent delay in diagnosis and sometimes misinterpretation of biopsy specimens. Patients were seen by a variety of specialists from disciplines such as general surgery, plastic surgery, orthopaedic surgery and rheumatology. Considerable progress has been made in the treatment of soft tissue sarcomas, often allowing local control of the tumour without amputation. We believe there should be early referral of patients having these tumours to a centre where a combined multidisciplinary approach can be undertaken.

  19. Feline intraocular sarcoma associated with phthisis bulbi

    Directory of Open Access Journals (Sweden)

    E. Perlmann

    2011-06-01

    Full Text Available Two cases of feline intraocular sarcoma were reported in stray cats that presented blindness and hypotonia of the affected eye for years before the tumor development. Phthisis bulbi, a final stage of a severe inflammation of the eye, is frequently unmonitored because eyes are blind, small, opaque, and not painful. Yet, this report shows that monitoring and early enucleation of eyes of cats with phthisis bulbi are important and should be considered as a treatment option, because feline intraocular sarcoma is an aggressive tumor that significantly decreases live expectancy.

  20. Circalunar Rhythms Induced Changes in the Body Functions and Reproductive Performance in Pregnant Ewes

    International Nuclear Information System (INIS)

    A total number of 136 pregnant ewes with the same age and parity were used in the present study. The experimental ewes reared during Arabic months or lunar months, from the 1st Gomady to Zol-hega, 1432 AH. The available data for each animal were obtained from the records of Sheep Farm, Project of Experimental Farms. Lambing season started at months, Shawal to Zol-hega. After lambing, the ewes were divided into three groups according to moon phases. Ewes in the 1st group laboured in new moon phase period. The ewes in the 2nd and the 3rd groups laboured in full and old moon phases, respectively. The study was conducted to determine the changes in the body functions and reproductive of pregnant ewes due to the effect of lunar cycle which causes the increase of births by up to 72% in the full moon phase. The results showed that, a significant increase in feed and water consumption, the activity of liver enzymes, concentration of cholesterol, triglyceride, kidney functions and also the hormone thyroxine and triiodothyronine was recorded during full moon, on the contrary, there was a significant decrease in the concentration of total protein, albumin, glucose, calcium, phosphorus and cortisol in the blood of pregnant ewes was recorded during full moon phases followed by new and old moon. On the other hand, reproductive traits including litter size at birth and weaning and mortality rate were recorded during different moon phases.

  1. Targeting protein kinases to reverse multidrug resistance in sarcoma.

    Science.gov (United States)

    Chen, Hua; Shen, Jacson; Choy, Edwin; Hornicek, Francis J; Duan, Zhenfeng

    2016-02-01

    Sarcomas are a group of cancers that arise from transformed cells of mesenchymal origin. They can be classified into over 50 subtypes, accounting for approximately 1% of adult and 15% of pediatric cancers. Wide surgical resection, radiotherapy, and chemotherapy are the most common treatments for the majority of sarcomas. Among these therapies, chemotherapy can palliate symptoms and prolong life for some sarcoma patients. However, sarcoma cells can have intrinsic or acquired resistance after treatment with chemotherapeutics drugs, leading to the development of multidrug resistance (MDR). MDR attenuates the efficacy of anticancer drugs and results in treatment failure for sarcomas. Therefore, overcoming MDR is an unmet need for sarcoma therapy. Certain protein kinases demonstrate aberrant expression and/or activity in sarcoma cells, which have been found to be involved in the regulation of sarcoma cell progression, such as cell cycle, apoptosis, and survival. Inhibiting these protein kinases may not only decrease the proliferation and growth of sarcoma cells, but also reverse their resistance to chemotherapeutic drugs to subsequently reduce the doses of anticancer drugs and decrease drug side-effects. The discovery of novel strategies targeting protein kinases opens a door to a new area of sarcoma research and provides insight into the mechanisms of MDR in chemotherapy. This review will focus on the recent studies in targeting protein kinase to reverse chemotherapeutic drug resistance in sarcoma. PMID:26827688

  2. Reproduction Performance of Pelibuey Ewes in Response to Estrus Synchronization and Artificial Insemination in Northwestern Mexico

    Directory of Open Access Journals (Sweden)

    M. Cruz-Villegas

    2007-01-01

    Full Text Available Artificial insemination is a valuable technique for improving reproductive performance in sheep as well as providing a resource to introduce new genetics. The purpose of the present study was to evaluate the induction of estrous, gestation length, fertility and prolificacy in Pelibuey ewes in response to Laparoscopic Artificial Insemination (LAI using frozen semen from 3 hair sheep breeds in a northwest region of Mexico during 2 consecutive years. In year one, 36 Pelibuey ewes were randomly distributed in three groups to be inseminated with three hair sheep breeds (Katahdin, K, n = 11; Dorper, D, n = 13; Pelibuey, P, n = 12. In year two, 43 Pelibuey ewes were randomly distributed in 3 groups using the same breeds (K, n = 14; D, n = 15; P, n = 14. Gestation length and prolificacy were analyzed using analysis of variance and induction of estrus, fertility and fecundity using chi-square tests. All analyses were performed through SAS procedures. Estrus was induced in all ewes of the three different groups. Gestation length was longer (p0.05 to those with D and K semen. Fertility rates of ewes inseminated with D semen (65% were higher (p0.05 in fertility rates between ewes inseminated with D and P semen. Fecundity rate was higher (p< 0.05 in ewes inseminated with D (164% than those with P (131% or K (108%. Pelibuey ewes inseminated with P semen had the lowest (p< 0.05 prolificacy (2.1 lpe than those with K (2.7 and D (2.6 lpe semen. These results suggest that estrus synchronization resulted in an efficient technique to induce estrus in Pelibuey ewes. Also, data from LAI in this desert conditions had similar results in fertility than those in other regions of the world. Although differences were observed in prolificacy, more information is needed to evaluate the convenience of introducing new sheep hair breeds using LAI in northwestern Mexico.

  3. Physiological Changes in Ewes Conceived Single or Twins Fetuses Related with Survivability of Lambs

    International Nuclear Information System (INIS)

    The number of 239 (137 in year 2009 and 102 in 2010) pregnant ewes with the same age and average live body weight were used in the present study. The experimental does reared under same conditions during May to September in 2009 and August to December in 2010, respectively. The available data for each animal were obtained from the recodes. The classified of breeds and total numbers used in this study were (Ossimi =49, Barki =47, Rahmani =60 and crossbred =83). Season of lambing started in October to November in 2009 and January and February in 2010. After parturition, the does were classified according to litter size into two groups. The ewes in the 1st and 2nd groups were labor single or twins lamb, respectively. The results showed that progressed pregnancy stages related with decreases in concentrations of immunity function (total proteins, albumin and globulin), cholesterol, glucose and calcium in serum of pregnant ewes. On the other hand, enzymes activities of liver functions (GOT, GPT and γGT), creatinine, triglycerides and phosphorus increased in serum of pregnant ewes with progressed pregnancy stages. Progesterone and parathormone concentrations showed opposite trends to those obtained levels with progressed pregnancy stages. Comparison between ewes conceived single or twins fetuses showed that most blood components values are related with survivability of lambs. In ewes conceived single fetus recorded higher values of total proteins, albumin, globulin, glucose and calcium than ewes conceived twins fetuses. On the other hand, GOT, GPT and γGT, cholesterol, triglycerides, creatinine, urea and phosphorus concentrations were lower in ewes conceived single fetus than in ewes conceived twins fetuses. On the opposite, progesterone and parathormone concentrations were lower in single than in twins fetuses

  4. Vitamin E supplementation of undernourished ewes pre- and post-lambing reduces weight loss of ewes and increases weight of lambs.

    Science.gov (United States)

    Rosales Nieto, César Augusto; Meza-Herrera, César Alberto; Moron Cedillo, Felipe de Jesús; Flores Najera, Manuel de Jesús; Gámez Vázquez, Hector Guillermo; Ventura Pérez, Felipe de Jesús; Liu, Shimin

    2016-03-01

    The aim of this study was to test if vitamin E supplementation during late gestation and early lactation would affect the weight of ewes under nutritional restriction and the performance of their lambs. Mature Rambouillet ewes (n = 37) were fed a diet that supplied 70 % of the energy and 80 % of recommended protein requirements and randomly assigned to either vitamin E (vit E, n = 20, 4 IU of α-tocopherol kg(-1) of live weight) or control (n = 17, without vitamin E supplementation). During the experimental period, the mean weight of ewes decreased from 74.6 ± 2.4 to 58.1 ± 2.2 kg. Weight loss of ewes was slightly less for the vit E than the control (-65 vs -124 g day(-1), SEM = 46; P = 0.07). Lambs born from vit E-supplemented ewes were heavier than lambs from the control and grew significantly faster (239 vs 195 g day(-1), SEM = 29.3, P < 0.05) with heavier weights at weaning (16.5 vs 13.5 kg, SEM = 1.8, P < 0.05). Besides, birth weight, weaning weight and daily weight gain favoured to single lambs and to male lambs (P < 0.05). Weaning weight was positively correlated to birth weight (P < 0.05) and weight gain (P < 0.001). When the nutrient requirements for ewes are not met, supplementation of vitamin E during late gestation and early lactation might be an effective strategy to minimise ewe weight loss as well as to increase lamb growth. PMID:26894501

  5. A typical case of hydrallantois accompanied by fetal monstrosity in a local ewe of Kashmir

    Directory of Open Access Journals (Sweden)

    Hiranya Kumar Bhattacharyya

    2012-09-01

    Full Text Available A full termed local ewe with the history of continuous straining with labored breathing for last 24 hours was presented. The animal was disinclined to move with tense and round abdomen which developed rapidly during last two weeks. Caesarean section revealed hydrallantois accompanied by multiple fetal congenital abnormalities. The ewe was under observation for four weeks. Metritis developed 12 days post-operation and was treated successfully. The ewe was found active on 25 days post-surgery with gain of extra 3 kg bodyweight.

  6. Continuous urea-molasses supplementation for Sumatra thin tail ewes grazing in rubber plantation : Reproductive performances.

    OpenAIRE

    Simon P Ginting; L.P Batubara; M.D Sanchez; K.R Pond

    1999-01-01

    The reproductive responses of local Sumatra ewes on the continuous urea-molasses supplementation was studied in 116 ewes for 21 months. The animals were divided into two groups and randomly allocated to urea-molasses supplement and grazing in rubber plantation or grazing in rubber plantation only (control). Both groups were allowed to graze for 8 hours a day. The urea-molasses mixture contained 3% urea (kg/kg) and were fed to the supplemented ewes ad libitum. The number of lambs born from sup...

  7. Faecal particle-size distribution from ewes fed grass silages harvested at different stages of maturity

    DEFF Research Database (Denmark)

    Jalali, Alireza; Nørgaard, Peder; Nadeau, E.;

    2008-01-01

    The aim of this experiment was to study the effect of maturity stage of grass at harvest on particle size in faeces from ewes fed grass silage ad libitum. Eighteen pregnant Swedish ewes bearing two foetuses were given one of three treatments as their only feed. The treatments were early (ECS...... pore size. The proportions of particles in the B, C, D, S and O fractions were affected by cutting time of the silaage (P < 0.003). In conclusion, stage of maturity at harvest strongly affects the distribution of particle size in faeces from ewes fed grass silages....

  8. Primary pulmonary sarcoma with morphologic features of biphasic synovial sarcoma: a case report.

    OpenAIRE

    Yoon, G. S.; Park, S Y; Kang, G. H.; Kim, O. J.

    1998-01-01

    We report an unusual primary case of pulmonary sarcoma that developed in the lung of a 36-year-old woman. The tumor had histologic, immunologic and ultrastructural features identical to those of biphasic synovial sarcoma of the soft tissue. It consisted of an intimate admixture of cytokeratin and epithelial membrane antigen(EMA)-positive neoplastic epithelial cells and vimentin-positive fibroblast-like spindle cells with areas of hyalinization. The patient had a lobectomy and showed no eviden...

  9. Circannual changes in progesterone secretion in intact ewes, luteinizing hormone secretion in ovariectomized estradiol-implanted ewes, and prolactin secretion in three sheep breeds anticipated to differ in seasonality of reproduction.

    Science.gov (United States)

    Goff, Katherine J; Knight, James W; Pelzer, Kevin D; Akers, R Michael; Notter, David R

    2013-05-01

    Changes in progesterone secretion in intact ewes (7 or 9 per breed) and luteinizing hormone secretion in ovariectomized, estradiol-implanted ewes (9 or 10 per breed) were monitored for 12 mo in Suffolk, tropically adapted St. Croix, and OOS ewes. The OOS line is a composite population of 50% Dorset, 25% Rambouillet, and 25% Finnish Landrace breeding that was selected for 10 yr for ability to lamb in October and early November. Ewes were isolated from rams, and blood samples were collected twice weekly. Circulating prolactin concentrations were also determined from blood samples collected near the summer and winter solstice and vernal and autumnal equinox. Intact OOS ewes entered anestrus later, began the subsequent breeding season sooner, and had a shorter seasonal anestrus than Suffolk and St. Croix ewes (P ≤ 0.005). St. Croix ewes did not differ from Suffolk ewes in date of onset or cessation of breeding or duration of anestrus (P ≥ 0.06). Breed differences in duration of luteinizing hormone inhibition in ovariectomized ewes were essentially identical to those observed for duration of anestrous. Prolactin concentrations varied during the year: annual changes were larger in relatively seasonal Suffolk ewes than in tropically-derived St. Croix ewes (P<0.01), and OOS ewes were intermediate to, and tended to differ from (P<0.10), the other two breeds. We conclude that OOS ewes developed by selection for fertility in spring matings had an abbreviated seasonal anestrus that is one of the shortest ever reported for temperate breeds, and that tropical St. Croix sheep did not have a shorter seasonal anestrus than Suffolk sheep under temperate conditions and ram isolation. PMID:23528712

  10. Resistance and perspectives in soft tissue sarcomas

    NARCIS (Netherlands)

    Komdeur, Rudy

    2003-01-01

    Soft tissue sarcomas are rare malignancies originating from mesenchymal origin. They may occur at any age, but the incidence increases with age: about 50% of the patients are over 60 years of age. A distinct peak incidence is made up by embryonal rhabdomyosarcomas that mostly afflict children at age

  11. The Value of Surgery for Retroperitoneal Sarcoma

    Directory of Open Access Journals (Sweden)

    Sepideh Gholami

    2009-01-01

    Full Text Available Introduction. Retroperitoneal sarcomas are uncommon large malignant tumors. Methods. Forty-one consecutive patients with localized retroperitoneal sarcoma were retrospectively studied. Results. Median age was 58 years (range 20–91 years. Median tumor size was 17.5 cm (range 4–41 cm. Only 2 tumors were <5 cm. Most were liposarcoma (44% and high-grade (59%. 59% were stage 3 and the rest was stage 1. Median followup was 10 months (range 1–106 months. Thirty-eight patients had an initial complete resection; 15 (37% developed recurrent sarcoma and 12 (80% had a second complete resection. Patients with an initial complete resection had a 5-year survival of 46%. For all patients, tumor grade affected overall survival (=.006. Complete surgical resection improved overall survival for high-grade tumors (=.03. Conclusions. Tumor grade/stage and complete surgical resection for high-grade tumors are important prognostic variables. Radiation therapy or chemotherapy had no significant impact on overall or recurrence-free survival. Complete surgical resection is the treatment of choice for patients with initial and locally recurrent retroperitoneal sarcoma.

  12. Osteogenic sarcoma with skeletal muscle metastases

    Energy Technology Data Exchange (ETDEWEB)

    Peh, W.C.G. [Department of Diagnostic Radiology, The University of Hong Kong, Queen Mary Hospital (Hong Kong); Shek, T.W.H. [Department of Pathology, The University of Hong Kong, Queen Mary Hospital (Hong Kong); Wang Shihchang [Department of Diagnostic Imaging, National University of Singapore, National University Hospital (Singapore); Wong, J.W.K.; Chien, E.P. [Department of Orthopaedic Surgery, The University of Hong Kong, Queen Mary Hospital (Hong Kong)

    1999-05-01

    Two cases of osteogenic sarcoma with skeletal muscle metastases are described. A 40-year-old woman presented with progressive swelling of both calves and a soft tissue back lump. She had been diagnosed with mandibular chondroblastic osteogenic sarcoma 6 years earlier. Radiographs showed calcified masses. MRI scans and bone scintigraphy revealed multiple soft tissue masses in both calves. Bone scintigraphy also showed uptake in the back lump, right thigh and left lung base. Biopsy confirmed metastatic chondroblastic osteogenic sarcoma, which initially responded well to chemotherapy. However, the metastatic disease subsequently progressed rapidly and she died 21 months after presentation. The second case concerns a 20-year-old man who presented with a pathologic fracture of the humerus, which was found to be due to osteoblastic osteogenic sarcoma. He developed cerebral metastases 17 months later, followed by metastases at other sites. Calcified masses were subsequently seen on radiographs of the abdomen and chest. CT scans confirmed the presence of densely calcified muscle metastases in the abdominal wall, erector spinae and gluteal muscles. The patient`s disease progressed rapidly and he died 30 months after presentation. (orig.) With 6 figs., 29 refs.

  13. What Is a Soft Tissue Sarcoma?

    Science.gov (United States)

    ... breast after radiation therapy and in limbs with lymphedema . Clear cell sarcoma is a rare cancer that often develops in tendons of the arms or legs. Under the microscope, it has some features of malignant melanoma , a type of cancer that develops from ...

  14. Delays in the management of retroperitoneal sarcomas

    DEFF Research Database (Denmark)

    Seinen, Jojanneke; Almquist, Martin; Styring, Emelie; Rydholm, Anders; Nilbert, Mef

    2010-01-01

    Retroperitoneal sarcomas are rare and treatment should optimally be centralized. Despite successful centralization with 90% of the patients referred prior to surgery, delays occur, which led us to assess lead times in a population-based series. Method. Patients diagnosed with retroperitoneal sarc...

  15. Primary pleomorphic sarcoma of the liver

    Energy Technology Data Exchange (ETDEWEB)

    Mani, S.; Naik, L.; Shet, S.; Vora, I.M.; Rananavare, R. [BYL Nail Hospital, Bombay (India). Departments of Radiology and Pathology

    1998-02-01

    A 35-year-old woman presented with abdominal distension and a palpable liver mass. Ultrasonography and computed tomography revealed a large well-delineated liver mass with bilobar involvement. Based on autopsy and immunohistochemical findings, a final diagnosis of primary pleomorphic liver sarcoma with myogenic differentiation W established. Copyright (1998) Blackwell Science Pty Ltd 8 refs., 5 figs.

  16. Prostatic stromal sarcoma with neuroectodermal differentiation

    Directory of Open Access Journals (Sweden)

    Yamazaki Hitoshi

    2012-12-01

    Full Text Available 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

  17. Effect of season and mating system in Awassi ewes superovulated with FSH on fertilization rate and embryo recovery

    Directory of Open Access Journals (Sweden)

    M. K. M. A. Al-Mola

    2010-01-01

    Full Text Available The aim of the present study was to evaluate the contribution of laparoscopic intrauterine insemination to the improvement of fertilization and embryo recovery in Awassi ewes superovulated with FSH in breeding and non-breeding season. Twelve nonpregnant and cycling Awassi ewes of 3-4 years of age were randomly allocated in equal numbers (n = 6 to two groups. Each ewe was treated with a progesterone impregnated intravaginal sponge for 12 days. All ewes were superovulated with FSH in eight reducing doses for four days in the morning and evening from two days prior to sponge withdrawal. Ewes of group 1 were mated naturally at least two times with Awassi rams of proven fertility. Ewes of group 2 had intrauterine insemination and were conducted 44-46 h after sponge removal, under laparoscopic visualization of uterine horns, depositing 1 ml of semen in the distal portion of each uterine horn. Ovarian response was assessed by determining number of corpora lutea by laparoscopy on day 6 after mating. Embryo recovery was performed by hand assisted laparoscopy and by flushing both uterine horns. Ovarian response of the ewes superovulated with FSH was decreased to half in the non-breeding season. High number of unfertilized ova (P<0.05 was observed in ewes superovulated with FSH in the non-breeding season when naturally inseminated compared to ewes inseminated intrauterine using laparoscopic technique. Higher rates of embryo recovery (P<0.05 were achieved in superovulated ewes in the breeding season when ewes were inseminated by laparoscopic intrauterine insemination. The fertilization rates in ewes inseminated intrauterine using laparoscopic techniques in breeding season and non-breeding season were 88.1% and 37.98%, respectively. It could be concluded from the results of the present study that the use of FSH to induce superovulation in Awassi ewes combined with laparoscopic intrauterine insemination can increase the fertilization rate in the breeding

  18. GLUT1 Expression in Synovial Sarcomas

    Directory of Open Access Journals (Sweden)

    Gülşah KAYGUSUZ

    2009-09-01

    Full Text Available Objective: In this study, the role of GLUT1 expression in synovial sarcomas and its association with disease pathogenesis were examined.Materials and Methods: Twenty two cases of synovial sarcoma were included in this study. The clinicopathological features of the cases, such as age, sex, localization of tumor, information of primary or metastatic tumor, histopathological type were recorded. The tissue microarray paraffin block containing tumor tissues was built by using tissue microarrayer. GLUT1 expression was analyzed on tissue sections by immunohistochemistry.Results: A total of 22 cases (mean age 36 years; range 14-54 years were analyzed. All cases except one were primary tumors. The tumors showed monophasic histological type in 13 cases and biphasic type in 9 cases. GLUT1 expression was found in 3 cases with biphasic type (14%. The cytoplasmic and incomplete membranous GLUT1 expression was seen in the tumor cells showing epithelial-glandular differentiation, whereas spindled cells were negative.Conclusion: Although GLUT1 expression is a diagnostic marker for juvenile capillary hemangioma and perineural tumors, both of which included in the group of mesenchymal tumors, it can be seen in a subset of synovial sarcomas. In our series, the observation of GLUT1 expression especially in the epithelial component of biphasic synovial sarcomas suggests that; i GLUT1 may be relatively used by tumoral cells composing epithelial component of the tumor, and ii the spindle cell component of the tumor would have been positive for other glucose transporters. The finding of uncommon GLUT1 expression in synovial sarcomas is indirectly consistent with the reported results of decreased standardized uptake value by Positron emission tomography with 2-[18F]fluoro-2-deoxy-D-glucose method in the literature.

  19. The importance of PET/CT in the evaluation of patients with Ewing tumors

    Energy Technology Data Exchange (ETDEWEB)

    Guimaraes, Julio Brandao; Rigo, Leticia; Lewin, Fabio; Emerick, Andre, E-mail: juliobrandaoguimaraes@hotmail.com [Hospital Sao Jose-Beneficincia Portuguesa de Sao Paulo, SP (Brazil)

    2015-05-15

    The effective evaluation for the treatment of patients with Ewing tumors depends on the accuracy in the determination of the primary tumor extent and the presence of metastatic disease. Currently, no universally accepted staging system is available to assess Ewing tumors. The present study aimed at discussing the use of PET/CT as a tool for staging, restaging and assessment of therapeutic response in patients with Ewing tumors. In spite of some limitations of PET/CT as compared with anatomical imaging methods, its relevance in the assessment of these patients is related to the capacity of the method to provide further physiological information, which often generates important clinical implications. Currently, the assessment of patients with Ewing tumor should comprise a study with PET/CT combined with other anatomical imaging modalities, such as radiography, computed tomography and magnetic resonance imaging. (author)

  20. HEALTH AND HYGIENIC CONDITIONS OF EWE'S MILK PROCESSING FROM THE ASPECT OF FOOD SAFETY

    Directory of Open Access Journals (Sweden)

    Jana Pukáčová

    2010-07-01

    Full Text Available Totally, 47 strains of S. aureus and 578 coagulase negative staphylococci were detected in samples from raw ewe milk. The 35 out 47 isolates of S. aureus from ewe milk were positive for the presence of staphylococcal enterotoxin genes: sea(4 %, sec (48 % a sed (48 %. Staphylococcus epidermis (33.04%, Staphylococcus caprae (21.28% were more prevalent. Staphylococcus chromogenes (7.44 %, Staphylococcus hominis (7.09%, Staphylococcus xylosus (6,92 %, a Staphylococcus warneri (6.40 % were isolated also in ewes milk. Staphylococcus haemolyticus (3.11 %, Staphylococcus capitis (2.94 %, Staphylococcus simulans (2.08 % and Staphylococcus saprophyticus (1.73 % were isolated very rarely from the taken individual milk ewe samples. Sporadically, only in few cases, the others coagulase negative staphylococci were isolated (Staphylococcus cohnii cohnii, Staphylococcus sciuri, Staphylococcus closii, Staphylococcus lugdunensis, Staphylococcus auricularis and Staphylococcus equorum.   doi:10.5219/24

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

    Science.gov (United States)

    2016-03-18

    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

  2. Reproduction Performance of Pelibuey Ewes in Response to Estrus Synchronization and Artificial Insemination in Northwestern Mexico

    OpenAIRE

    M. Cruz-Villegas; RODRIGUEZ-GARCIA, J; A. Correa-Calderon; R. Rangel-Santos; L. Molina-Ramirez; F.D. Alvarez-Valenzuela; L. Avendano-Reyes; Robinson, P H; Famula, T. R.

    2007-01-01

    Artificial insemination is a valuable technique for improving reproductive performance in sheep as well as providing a resource to introduce new genetics. The purpose of the present study was to evaluate the induction of estrous, gestation length, fertility and prolificacy in Pelibuey ewes in response to Laparoscopic Artificial Insemination (LAI) using frozen semen from 3 hair sheep breeds in a northwest region of Mexico during 2 consecutive years. In year one, 36 Pelibuey ewes were randomly ...

  3. Screening of antibiotic residues in ewe milk destined to cheese by a commercial microbiological inhibition assay

    OpenAIRE

    Yamaki, Miho; Berruga, M. Isabel; Althaus, Rafael Lisandro; Molina, Pilar; Molina, Ana

    2006-01-01

    Abstract Bulk ewe milk from Spanish dairy farms situated in the Castilla-La Mancha region, and destined to Protected Denomination of Origin (PDO) Manchego cheese, were analysed each month for antimicrobial residues during one year. For this study a microbiological assay specific for ewe milk (Eclipse ?100ov??) was used. The number of positive samples by the Eclipse ?100ov?? test was 2.6%. A second examination of positives following heat treatment at 82?C for 10 min showed a reduc...

  4. HEALTH AND HYGIENIC CONDITIONS OF EWE'S MILK PROCESSING FROM THE ASPECT OF FOOD SAFETY

    OpenAIRE

    Jana Pukáčová; Lucia Poľaková; Eva Dudriková

    2010-01-01

    Totally, 47 strains of S. aureus and 578 coagulase negative staphylococci were detected in samples from raw ewe milk. The 35 out 47 isolates of S. aureus from ewe milk were positive for the presence of staphylococcal enterotoxin genes: sea(4 %), sec (48 %) a sed (48 %). Staphylococcus epidermis (33.04%), Staphylococcus caprae (21.28%) were more prevalent. Staphylococcus chromogenes (7.44 %), Staphylococcus hominis (7.09%), Staphylococcus xylosus (6,92 %), a Staphylococcus warneri (6.40 %) wer...

  5. The effect of ewes relocation on milk composition and milk flow kinetics

    Directory of Open Access Journals (Sweden)

    Lucia Jackuliaková

    2014-02-01

    Full Text Available The investigation of an influence of ewes relocation and milking in other parlour (treatment on milk flow kinetics, milkability and milk composition was the aim of this study. In total 34 ewes of two breeds and crosbreeds Tsigai (14 heads and Improved Valachian (20 heads with Lacaune were tested. Two weeks after lamb weaning the ewes were milked in parallel milking parlour (1x16 stalls under shelter. On the last evening milking (first experimental milking, EB before relocation of flock to another parlour, and during next three continuous evening milkings (E0 - second, E1 - third and E2 - fourth milking of exp. after relocation the milk flow kinetics were measured using electronic collection jar. On day E0 after morning milking the flock was moved on a pasture and milked in other parlour (1x24-stalls. During E0 we recorded a significant decrease of total milk yield in comparison with EB (0.527 ±0.04 and 0.647 ±0.04 L. Significant differences were also recorded in machine milk yield, machine stripping, milking time and in maximum milk flow rate. During E0 there was a higher number of nonbimodal and lower numbers of bimodal flow types. The response of ewe to E0 depended on its response to EB. Ewes with bimodal flow at EB responded more negatively to E0 than ewes with nonbimodal or plateau flow. During E2 there were significantly increased protein content and solids not fat in milk. Thus the treatment significantly influenced the milkability of ewes in a negative way, but more clear response was found out in ewes with bimodal flow response to machine milking before treatment. We could assume that relocation to other milking conditions caused only short-term changes in milk flow kinetic and milk yield. Normal 0 21 false false false SK X-NONE X-NONE

  6. On-farm supplements for lactating manchega ewe's grazing sown irrigated pastures. Milk composition

    OpenAIRE

    Caballero, R.; Rioperez, Juan; Fernandez, E.; Amigo, Lourdes; Fontecha, F. Javier

    1991-01-01

    Manchega ewes grazing sown irrigated pastures in Central Spain during the autum or spring lambing season were, or were not, supplemented with up to 1 kg ewe-1 d-1 of starchrich ingredients (barley and field beans) until mid-lactation with the aim of assessing the influence of supplementation in grazing conditions on milk composition and cheese making suitability. Five sampling periods in 1986 and six in 1988 were controlled beginning one week after parturiton. Milk samples were taken at 15day...

  7. Effect of toxoplasmosis and brucellosis on some biochemical parameters in ewes

    OpenAIRE

    N. A. J. Al- Hussary; A. S. M. Al- Zuhairy

    2010-01-01

    The present study was conducted to evaluate the effects of infection of ewes with toxoplasmosis and/or brucellosis on someserum biochemical parameters. Ninety six samples of blood were collected from aborted ewes at different stages of gestation,suspected to be infected with toxoplasmosis and /or brucellosis from different regions in Nineveh governorate. The percentageof toxoplasma and brucella infection depending on Latex Agglutination Test (LAT) and Rose Bengal Test were 21.88% and23.96% re...

  8. Clinical findings related to intramammary infections in meat-producing ewes

    OpenAIRE

    Maiara G. Blagitz; Fernando N. Souza; Camila F. Batista; Diniz, Soraia A.; Haddad, João Paulo A.; Nilson R. Benites; Melville, Priscilla A.; Alice M.M.P. Della Libera

    2013-01-01

    The aim of this study was to investigate the relationship between clinical findings and bacterial isolation in milk samples of meat-producing ewes. The study was conducted in 17 commercial flocks and 550 udder halves from suckling Santa Ines ewes. Initially, the clinical examination of the mammary glands and teats was performed by visual inspection and palpation of the teats and udder halves; then a scoring system was devised for all the findings. After that, the strip cup test and the Califo...

  9. Optimizing the interval between G-CSF therapy and F-18 FDG PET imaging in children and young adults receiving chemotherapy for sarcoma

    International Nuclear Information System (INIS)

    Granulocyte colony-stimulating factors (G-CSF) speed recovery from chemotherapy-induced myelosuppression but the marrow stimulation they cause can interfere with interpretation of F-18 fluorodeoxyglucose positron emission tomography (F-18 FDG PET) exams. To assess the frequency of interfering G-CSF-induced bone marrow activity on FDG PET imaging in children and young adults with Ewing sarcoma and rhabdomyosarcoma and to define an interval between G-CSF administration and FDG PET imaging that limits marrow interference. Blinded, retrospective review of FDG PET exams performed in patients treated with long-acting G-CSF as part of their chemotherapeutic regimen. Exams were subjectively scored by two reviewers (R1 and R2) who assessed the level of marrow uptake of FDG and measured standardized uptake values in the marrow, liver, spleen and blood pool. FDG PET findings were correlated with time since G-CSF administration and with blood cell counts. Thirty-eight FDG PET exams performed in 17 patients were reviewed with 47.4% (18/38) of exams having marrow uptake of FDG sufficient to interfere with image interpretation. Primary predictors of marrow uptake of FDG were patient age (P = 0.0037) and time since G-CSF exposure (P = 0.0028 for subjective marrow uptake of FDG, P = 0.008 [R1] and P = 0.004 [R2] for measured maximum standardized uptake value (SUVmax)). The median interval between G-CSF administration and PET imaging in cases with marrow activity considered normal or not likely to interfere was 19.5 days (range: 7-55 days). In pediatric and young adult patients with Ewing sarcoma and rhabdomyosarcoma, an interval of 20 days between administration of the long-acting form of G-CSF and FDG PET imaging should limit interference by stimulated marrow. (orig.)

  10. Optimizing the interval between G-CSF therapy and F-18 FDG PET imaging in children and young adults receiving chemotherapy for sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Trout, Andrew T.; Sharp, Susan E.; Gelfand, Michael J. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Turpin, Brian K. [Cincinnati Children' s Hospital Medical Center, Cancer and Blood Diseases Institute, Division of Oncology, Cincinnati, OH (United States); Zhang, Bin [Cincinnati Children' s Hospital Medical Center, Division of Biostatistics and Epidemiology, Cincinnati, OH (United States)

    2015-07-15

    Granulocyte colony-stimulating factors (G-CSF) speed recovery from chemotherapy-induced myelosuppression but the marrow stimulation they cause can interfere with interpretation of F-18 fluorodeoxyglucose positron emission tomography (F-18 FDG PET) exams. To assess the frequency of interfering G-CSF-induced bone marrow activity on FDG PET imaging in children and young adults with Ewing sarcoma and rhabdomyosarcoma and to define an interval between G-CSF administration and FDG PET imaging that limits marrow interference. Blinded, retrospective review of FDG PET exams performed in patients treated with long-acting G-CSF as part of their chemotherapeutic regimen. Exams were subjectively scored by two reviewers (R1 and R2) who assessed the level of marrow uptake of FDG and measured standardized uptake values in the marrow, liver, spleen and blood pool. FDG PET findings were correlated with time since G-CSF administration and with blood cell counts. Thirty-eight FDG PET exams performed in 17 patients were reviewed with 47.4% (18/38) of exams having marrow uptake of FDG sufficient to interfere with image interpretation. Primary predictors of marrow uptake of FDG were patient age (P = 0.0037) and time since G-CSF exposure (P = 0.0028 for subjective marrow uptake of FDG, P = 0.008 [R1] and P = 0.004 [R2] for measured maximum standardized uptake value (SUVmax)). The median interval between G-CSF administration and PET imaging in cases with marrow activity considered normal or not likely to interfere was 19.5 days (range: 7-55 days). In pediatric and young adult patients with Ewing sarcoma and rhabdomyosarcoma, an interval of 20 days between administration of the long-acting form of G-CSF and FDG PET imaging should limit interference by stimulated marrow. (orig.)

  11. Non-genetic factors of udder morphology traits in Istrian ewes

    Directory of Open Access Journals (Sweden)

    Valentino Držaić

    2013-05-01

    Full Text Available Considering the facts that Istrian sheep is Croatian indigenous breed with the highest milk production, and increasing interest of local farmers for machine milking implementation, the aim of this study was to determine the factors affecting udder morphology traits of Istrian ewes. Ninetythree purebred Istrian ewes with symmetrical udders and no signs of clinical mastitis, from second to fourth lactation, were used for this study. Average udder depth of Istrian ewes was 15.85 cm, udder width 13.05 cm, udder circumference 39.66 cm, cistern height 1.44 cm, teat position 2.85 (scored from 1 to 5, teat angle 47.32°, teat length 3.43 cm and teat width 2.03 cm. The traits related to udder size (depth, width, and circumference were significantly (P<0.01 affected by lactation milk yield. These traits were also in high and positive correlations (P<0.01 with daily milk yield. With the increase of parity, the morphological aptitude of udder to mechanical milking become worse, with an increase (P<0.01 of cistern height, teat angle and position. As lactation of Istrian ewes continued morphological traits defining udder aptitude for machine milking improved (decrease of cistern height, teat angle and position. Ewes with two and more lambs had larger udder (P<0.05 than ewes with a single lamb.

  12. Reproductive performance of ewes grazing lucerne during different periods around mating.

    Science.gov (United States)

    Robertson, S M; Clayton, E H; Friend, M A

    2015-11-01

    High intake of lucerne pastures or feeding of other high quality diets during early pregnancy may increase embryo mortality, negating any benefit of improved nutrition on ovulation rate in ewes. This study was conducted to determine whether grazing ewes on lucerne (Medicago sativa) pastures for 7 days prior to and throughout joining would result in greater foetal numbers than if ewes were removed 7 days after the commencement of joining, or if ewes grazed senescent pasture throughout the joining period. Merino ewes (300) were allocated to two replicates of three treatments, grazing pastures between Days -7 and 36 of an unsynchronised, natural autumn joining. Grazing lucerne to Day 7 of joining resulted in 30% more (P0.05) among all treatment groups, suggesting no differences between treatments in embryo mortality. Grazing naturally cycling ewes on lucerne prior to and during joinings in autumn is recommended as a means to increase the number of lambs born, although additional gains may not be obtained by grazing past day seven of joining. PMID:26454684

  13. Improvement of fertility in artificially inseminated ewes following vaginal treatment with misoprostol plus terbutaline sulphate.

    Science.gov (United States)

    Horta, A E M; Barbas, J P; Marques, C C; Baptista, M C; Vasques, M I; Pereira, R M; Mascarenhas, R D; Cavaco-Gonçalves, S

    2010-12-01

    The effect of vaginal administration of misoprostol plus terbutaline sulphate 6 h prior to artificial insemination (AI) upon the site of AI (vaginal or cervical) and fertility was studied using a total of 87 estrous synchronized Serra da Estrela ewes (control n = 42 and treated n = 45). Artificial insemination was performed using refrigerated semen at 54-55 h after sponge removal. Lambing rate (fertility) and prolificacy were compared between control and treated ewes. The effect of the site of semen deposition on fertility was also evaluated. Prolificacy rate was not different between control (1.5) and treated (1.59) ewes. The proportion of cervical AI achieved in control (45.2%) and treated (37.8%) ewes was not significantly different. Overall, fertility was significantly lower in control than in treated ewes (42.9% vs 64.4%; p terbutaline sulphate 6 h prior to artificial insemination did not affect the proportion of cervical inseminations but significantly improved the fertility of treated ewes. Although needing confirmation, it was hypothesized that drugs might have induced local secretory modifications leading to an increase of cervical ability to retain more viable spermatozoa for fertilization. PMID:20210884

  14. Lymphangiectatic Kaposi's sarcoma in a patient with AIDS.

    Science.gov (United States)

    Santos, Mônica; Vilasboas, Virginia; Mendes, Luciana; Talhari, Carolina; Talhari, Sinésio

    2013-01-01

    Kaposi's sarcoma is a malignant disease that originates in the lymphatic endothelium. It has a broad spectrum of clinical manifestations. Its four distinct clinical forms are: classic, endemic, iatrogenic and epidemic Kaposi's sarcoma. In non-HIV-associated Kaposi's sarcoma, the disease is typically limited to the lower extremities, but in immunodeficient patients, it is a multifocal systemic disease. The clinical course of the disease differs among patients, ranging from a single or a few indolent lesions to an aggressive diffuse disease. Advanced Kaposi's sarcoma lesions, typically those on the lower extremities, are often associated with lymphedema. In this paper, we report a case of a patient with a rare form of AIDS-associated Kaposi sarcoma called lymphangiectatic Kaposis's sarcoma. PMID:23739700

  15. Concentrate supplementation during pregnancy and lactation of ewes affects the growth rate of lambs from a variety of crosses

    OpenAIRE

    Jacira Neves da Costa Torreão; Arturene Marques Rocha; Carlo Aldrovandi Torreão Marques; Leilson Rocha Bezerra; Fernanda Patrícia Gottardi; Marcos Jácome de Araújo; Edmilson Lúcio de Souza Júnior; Ronaldo Lopes Oliveira

    2014-01-01

    The objective of this study was to determine how concentrate supplementation during late third gestation and lactation affects ewe and offspring performance from birth to weaning. Thirty-six ewes and their offspring (n = 32) were used. Eighteen Morada Nova and 18 Santa Inês ewes were artificially inseminated with semen from Dorper ram and distributed in a completely randomized factorial arrangement (2 × 2) consisting of two supplementation levels (5 and 15 g kg-1 of BW) and two breeds (Morada...

  16. MIB-1 expression and iododeoxyuridine labelling in soft tissue sarcomas

    DEFF Research Database (Denmark)

    Jensen, V; Høyer, M; Sørensen, Flemming Brandt;

    1996-01-01

    We investigated the relationship between immunohistochemical estimates of proliferative activity and expression of bcl-2 protein and mutant p53 protein in 23 cases of soft tissue sarcoma. Furthermore, the reproducibility of estimates of proliferative activity was analysed and correlations between...... activity and might improve the accuracy of conventional malignancy grading of soft tissue sarcomas. Furthermore, the results indicate that neither mutant p53 protein nor bcl-2 oncogene alone are sufficient to induce increased proliferation in these sarcomas....

  17. Primary Kaposi Sarcoma of Penis in HIV Negative Patient

    OpenAIRE

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

    2012-01-01

    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.

  18. Management of Monophasic Synovial Sarcoma of the Small Intestine

    OpenAIRE

    Eriksen, Calvin; Burns, Lance; Bohlke, Angela; Haque, Salima; Slakey, Douglas P

    2010-01-01

    Background: Reports of primary intraabdominal synovial sarcomas are extremely rare. Methods: A literature review using PubMed was performed. A retrospective review of the one known case at our institution was completed. Results: Even the most experienced pathologists report that synovial sarcomas can be very difficult to diagnose correctly. One cytogenic abnormality that is common (>90%) and pathognomonic for synovial sarcoma is a characteristic chromosomal translocation resulting in the SYT/...

  19. Synovial sarcoma of the vulva: a case report

    OpenAIRE

    van Schalkwyk Gerhard; Asher Viren; Bali Anish

    2011-01-01

    Abstract Introduction Contrary to its name, synovial sarcoma does not arise from the synovial membrane but from multipotent stem cells and can present in any part of the body. Very few cases of vulval synovial sarcoma have been reported in the literature; we report on such a presentation. These tumors can present as painless lumps, which must be completely excised to give the best prognosis. Therefore the diagnosis of synovial sarcoma should always be kept in mind in the management of vulval ...

  20. Synovial Sarcoma of the Buccal Mucosa: A Rare Case Report

    OpenAIRE

    Kumar T. S. Mahesh; Indira Annamalai Ponnuswamy; Maria Priscilla David; Peeyush Shivhare; Mahalakshmi Ikkanur Puttaranganayak; Pooja Sinha

    2013-01-01

    Synovial sarcoma (SS) is a rare malignant neoplasm that arises most commonly in joint capsules and articular tendons, but its relationship to the synovium is not always obvious. Synovial sarcoma is a malignant soft tissue tumor representing 5.6% to 10% of all soft tissue sarcomas. They are termed SS because of their histologic resemblance to the synovium, but they rarely involve a synovial structure and are thought to arise from pluripotential mesenchymal cells. The tumor usually occurs in cl...

  1. Lymphangiectatic Kaposi's sarcoma in a patient with AIDS *

    OpenAIRE

    Santos, Mônica; Vilasboas, Virginia; Mendes, Luciana; Talhari, Carolina; Talhari, Sinésio

    2013-01-01

    Kaposi's sarcoma is a malignant disease that originates in the lymphatic endothelium. It has a broad spectrum of clinical manifestations. Its four distinct clinical forms are: classic, endemic, iatrogenic and epidemic Kaposi's sarcoma. In non-HIV-associated Kaposi's sarcoma, the disease is typically limited to the lower extremities, but in immunodeficient patients, it is a multifocal systemic disease. The clinical course of the disease differs among patients, ranging from a single or a few in...

  2. UK guidelines for the management of bone sarcomas

    OpenAIRE

    Gerrand, Craig; Athanasou, Nick; Brennan, Bernadette; Grimer, Robert; Judson, Ian; Morland, Bruce; Peake, David; Seddon, Beatrice; Whelan, Jeremy; ,

    2016-01-01

    This document is an update of the British Sarcoma Group guidelines published in 2010. The aim is to provide a reference standard for the clinical care of patients in the UK with bone sarcomas. Recent recommendations by the European Society of Medical Oncology, The National Comprehensive Cancer Network and The National Institute for Health and Care Excellence have been incorporated, and the literature since 2010 reviewed. The standards represent a consensus amongst British Sarcoma Group member...

  3. MLN0128, an ATP-Competitive mTOR Kinase Inhibitor, with Potent In vitro and In vivo Antitumor Activity as Potential Therapy for Bone and Soft-Tissue Sarcoma

    Science.gov (United States)

    Slotkin, Emily K.; Patwardhan, Parag P.; Vasudeva, Shyamprasad Deraje; de Stanchina, Elisa; Tap, William D.; Schwartz, Gary K.

    2014-01-01

    The mammalian target of rapamycin (mTOR) is a serine/threonine protein kinase that exists in two complexes (mTORC1 and mTORC2) and integrates extracellular and intracellular signals to act as a master regulator of cell growth, survival, and metabolism. The PI3K/AKT/mTOR pro-survival pathway is often dysregulated in multiple sarcoma subtypes. First-generation allosteric inhibitors of mTORC1 (rapalogues) have been extensively tested with great pre-clinical promise, but have had limited clinical utility. Here we report that MLN0128, a second-generation, ATP-competitive, pan-mTOR kinase inhibitor, acts on both mTORC1 and mTORC2, and has potent in vitro and in vivo anti-tumor activity in multiple sarcoma subtypes. In vitro, MLN0128 inhibits mTORC1/2 targets in a concentration dependent fashion, and shows striking anti-proliferative effect in rhabdomyosarcoma (RMS), Ewing sarcoma (ES), malignant peripheral nerve sheath tumor, synovial sarcoma, osteosarcoma, and liposarcoma. Unlike rapamycin, MLN0128 inhibits phosphorylation of 4EBP1 and NDRG1 as well as prevents the reactivation of pAKT that occurs via negative feedback release with mTORC1 inhibition alone. In xenograft models, MLN0128 treatment results in suppression of tumor growth with two dosing schedules (1 mg/kg daily and 3 mg/kg BID TIW). At the 3 mg/kg dosing schedule, MLN0128 treatment results in significantly better tumor growth suppression than rapamycin in RMS and ES models. Additionally, MLN0128 induces apoptosis in models of RMS both in vitro and in vivo. Results from our study strongly suggest that MLN0128 treatment should be explored further as potential therapy for sarcoma. PMID:25519700

  4. An Australasian perspective on sarcoma research, translational biology and clinical trials: the Australasian Sarcoma Study Group (ASSG).

    Science.gov (United States)

    Bae, Susie; Caruso, Denise; Desai, Jayesh

    2014-02-01

    Each year approximately 800 Australians are diagnosed with sarcoma, accounting for less than 1% of cancer diagnoses overall. A significant proportion of these sarcoma cases are in children and adolescents. The rarity and heterogeneity of this group of tumours, coupled with Australasia's relative geographical isolation, pose significant challenges in developing locoregional basic, translational and clinical research. The Australasian Sarcoma Study Group (ASSG) was established in 2008 as a Cooperative Cancer Clinical Research Group and is now the peak body for sarcoma research in Australasia, providing a mechanism to drive and coordinate collaborative research, promote education and assist with advocating for sarcoma within the region. This paper describes the development of ASSG and examines the current state of play with regard to sarcoma research in Australasia. PMID:24378392

  5. Clinical outcomes for patients with synovial sarcoma of the hand

    OpenAIRE

    Outani, Hidetatsu; Hamada, Kenichiro; Oshima, Kazuya; Joyama, Susumu; Naka, Norifumi; Araki, Nobuhito; Ueda, Takafumi; Yoshikawa, Hideki

    2014-01-01

    Purpose Soft tissue sarcoma of the hand is rare, and one of the most common histological diagnosis is synovial sarcoma. We report the clinical outcomes of patients with synovial sarcoma of the hand and discuss treatment strategies. Methods We reviewed five patients with synovial sarcoma of the hand treated at our institutions from 1983 to 2013. The mean patient age at the time of diagnosis was 36.6 years (range, 20–62 years). Two patients underwent marginal excision after neoadjuvant chemothe...

  6. Refractory ulcerative colitis and iatrogenic colorectal Kaposi's sarcoma.

    Science.gov (United States)

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

    2009-02-01

    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

  7. Evaluation of an accelerated lambing system in Syrian Awassi ewes, using hormonal treatments inside and outside the breeding season

    International Nuclear Information System (INIS)

    An experiment was carried out on Syrian Awassi ewes to assess the accelerated lambing system (three lambings in 2 years) by synchronising and induction of oestrus and increasing ovulation rate using hormonal treatments (intravaginal sponges: FGA + equine chorionic gonadotrophin: eCG). Fifty intact cyclic Syrian Awassi ewes aged 2- 4 years with an average live weight of 51.4 kg were used for 4 years (6 lambings). Ewes were divided into 2 groups: 40 ewes in the treated (T) and 10 in the control (C). Ewes in the T group were treated with FGA for 14 days and injected intramuscularly at sponge withdrawal with 500 IU eCG. Results indicated that, throughout the 6 breeding periods, oestrus induction rate was 100%, and all ewes in the T group were mated within 1-5 days post sponge removal as compared to 10-11 days for ewes in the C group. Treated ewes had higher rates of lambing, multiple birth and fecundity. Repeated administration of eCG (6 times at 8 months interval) had no negative effect on fertility of Syrian Awassi ewes. However, anti-eCG antibodies were produced following eCG injections. (author)

  8. Fertility Rates of Ewes Treated with Medroxyprogesterone and Injected with Equine Chorionic Gonadotropin plus Human Chorionic Gonadotropin in Anoestrous Season

    OpenAIRE

    I.W. Santos; Binsfeld, L. C.; Weiss, R. R.; Kozicki, L. E.

    2010-01-01

    The aim of the present paper was to investigate the efficiency of the equine chorionic gonadotropin (eCG) plus human chorionic gonadotropin (hCG) associated with medroxyprogesterone acetate (MAP) to estrous ewes synchronization. Ninety Texel ewes were investigated during seasonal anoestrous. The ewes received intravaginal sponges containing MAP (60 mg) for nine days. At the time of sponges' withdrawal, the ewes were divided into three groups (G): (1) receiving 2 mL of saline i.m. (n = 30), (2...

  9. Primary synovial sarcoma of the kidney

    Directory of Open Access Journals (Sweden)

    Shwetank Mishra

    2015-01-01

    Full Text Available Primary synovial sarcoma (SS of the kidney is a very rare disease as well as a diagnostic dilemma. Here, we present a case of a 60-year-old male clinically diagnosed as renal cell carcinoma. The radical nephrectomy specimen showed a well-circumscribed renal mass of approximately 13 cm × 9 cm × 7 cm. The cut-surface of the mass was tawny and firm, with cystic areas, and also showed focal hemorrhage and necrosis. Histologically, the tumor was composed of spindle cells arranged in the intersecting fascicles, alternating with hypocellular areas suggestive of monophasic synovial sarcoma. Morphological and immunohistochemical features were compatible with the diagnosis of SS of the kidney.

  10. Thyroid carcino-sarcoma in a dog

    Directory of Open Access Journals (Sweden)

    Antonio Giuliano

    2013-04-01

    Full Text Available An adult male greyhound was diagnosed with a thyroid carcino-sarcoma by means of histopathology and positive immuno-histochemistry staining for cytokeratin and vimentin. Surgery and radiotherapy of the area were successful in local tumour control. Adjuvant chemotherapy was recommended to treat and prevent further metastasis. The use of carboplatin, metronomic cyclophosphamide chemotherapy and toceranib failed to control the progression of distant metastasis. The survival time was seven months from the time of diagnosis. This is the eighth case of carcino-sarcoma of the thyroid documented in veterinary medicine and the first one treated with a multimodal approach based on surgery, radiotherapy and chemotherapy. As documented in human medicine, chemotherapy appeared to be ineffective to prevent or delay the progression of the metastatic disease in this case.

  11. Thyroid carcino-sarcoma in a dog

    Directory of Open Access Journals (Sweden)

    Antonio Giuliano

    2013-02-01

    Full Text Available An adult male greyhound was diagnosed with a thyroid carcino-sarcoma by means of histopathology and positive immuno-histochemistry staining for cytokeratin and vimentin. Surgery and radiotherapy of the area were successful in local tumour control. Adjuvant chemotherapy was recommended to treat and prevent further metastasis. The use of carboplatin, metronomic cyclophosphamide chemotherapy and toceranib failed to control the progression of distant metastasis. The survival time was seven months from the time of diagnosis. This is the eighth case of carcino-sarcoma of the thyroid documented in veterinary medicine and the first one treated with a multimodal approach based on surgery, radiotherapy and chemotherapy. As documented in human medicine, chemotherapy appeared to be ineffective to prevent or delay the progression of the metastatic disease in this case.

  12. Thyroid carcino-sarcoma in a dog.

    Science.gov (United States)

    Giuliano, Antonio; Grant, Jessica; Benoit, Jerome

    2013-01-01

    An adult male greyhound was diagnosed with a thyroid carcino-sarcoma by means of histopathology and positive immuno-histochemistry staining for cytokeratin and vimentin. Surgery and radiotherapy of the area were successful in local tumour control. Adjuvant chemotherapy was recommended to treat and prevent further metastasis. The use of carboplatin, metronomic cyclophosphamide chemotherapy and toceranib failed to control the progression of distant metastasis. The survival time was seven months from the time of diagnosis. This is the eighth case of carcino-sarcoma of the thyroid documented in veterinary medicine and the first one treated with a multimodal approach based on surgery, radiotherapy and chemotherapy. As documented in human medicine, chemotherapy appeared to be ineffective to prevent or delay the progression of the metastatic disease in this case. PMID:23718201

  13. Lymphangiectatic Kaposi's sarcoma in a patient with AIDS Sarcoma de Kaposi linfangiectásico em paciente com Aids

    Directory of Open Access Journals (Sweden)

    Mônica Santos

    2013-04-01

    Full Text Available Kaposi's sarcoma is a malignant disease that originates in the lymphatic endothelium. It has a broad spectrum of clinical manifestations. Its four distinct clinical forms are: classic, endemic, iatrogenic and epidemic Kaposi's sarcoma. In non-HIV-associated Kaposi's sarcoma, the disease is typically limited to the lower extremities, but in immunodeficient patients, it is a multifocal systemic disease. The clinical course of the disease differs among patients, ranging from a single or a few indolent lesions to an aggressive diffuse disease. Advanced Kaposi's sarcoma lesions, typically those on the lower extremities, are often associated with lymphedema. In this paper, we report a case of a patient with a rare form of AIDS-associated Kaposi sarcoma called lymphangiectatic Kaposis's sarcoma.O sarcoma de Kaposi é uma neoplasia originária do endotélio linfatico, que apresenta um amplo espectro de manifestações, com quatro formas clínicas: sarcoma de Kaposi clássico, endêmico, iatrogêncio e epidêmico ou associado ao HIV. Em pacientes imunocompetentes, a doença é tipicamente limitada às extremidades. Porém em pacientes imunideprimidos, o sarcoma de Kaposi é uma doença sistêmica multifocal. Apresenta cursos clínicos diferentes, desde simples lesões cutâneas isoladas até lesões agressivas e difusas, com ou sem envolvimento sistêmico. Lesões avançadas de sarcoma de Kaposi, principalmente as localizadas nas extremidades, podem apresentar linfedema. Neste trabalho, reportamos caso de paciente com forma rara de Sarcoma de Kaposi associado a Aids, chamada de sarcoma de Kaposi linfangiectásico.

  14. Lymphangiectatic Kaposi's sarcoma in a patient with AIDS Sarcoma de Kaposi linfangiectásico em paciente com Aids

    OpenAIRE

    Mônica Santos; Virginia Vilasboas; Luciana Mendes; Carolina Talhari; Sinésio Talhari

    2013-01-01

    Kaposi's sarcoma is a malignant disease that originates in the lymphatic endothelium. It has a broad spectrum of clinical manifestations. Its four distinct clinical forms are: classic, endemic, iatrogenic and epidemic Kaposi's sarcoma. In non-HIV-associated Kaposi's sarcoma, the disease is typically limited to the lower extremities, but in immunodeficient patients, it is a multifocal systemic disease. The clinical course of the disease differs among patients, ranging from a single or a few in...

  15. GEIS 2013 guidelines for gastrointestinal sarcomas (GIST)

    OpenAIRE

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

    2014-01-01

    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 α-activating mutations. This deep molecular understanding has allowed the correct classification into risk groups with implications regarding prog...

  16. Primary Pulmonary Synovial Sarcoma in Pregnancy

    OpenAIRE

    Bunch, K; Deering, S. H.

    2012-01-01

    Background. Primary pulmonary synovial sarcoma is a rare malignancy with a poor prognosis. Surgical resection and postoperative management of these tumors has not been previously described in pregnancy. Case. A 38-year-old pregnant woman was admitted for evaluation of a right thoracic mass found on chest radiography at 26 weeks of gestation. A computed tomography-guided biopsy was subsequently completed and demonstrated a high-grade neoplasm. A right pneumonectomy was performed at 28 weeks of...

  17. MRI of perineural extramedullary granulocytic sarcoma

    International Nuclear Information System (INIS)

    Granulocytic sarcoma is an extramedullary solid tumour consisting of myelogenous leukaemic blast cells, usually seen in acute myeloid leukaemia and less commonly in patients with chronic myeloid leukaemia or myeloproliferative disorders. Blast cells have a predilection for periosteal and perineural regions and rarely precede evidence of systemic disease. We present two patients, aleukaemic on peripheral blood counts, both at presentation and during subsequent treatment. We present the MRI features of this rare but important condition. (orig.)

  18. MRI of perineural extramedullary granulocytic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Graham, A. [Rehabilitation Medicine, Hunters Moor Neurological Rehabilitation Centre, Newcastle-Upon-Tyne (United Kingdom); Hodgson, T. [Neuroradiology Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom); Jacubowski, J. [Neurosurgical Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom); Norfolk, D. [Haematology Department, Leeds General Infirmary, Leeds LS1 3EX (United Kingdom); Smith, C. [Pathology Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom)

    2001-06-01

    Granulocytic sarcoma is an extramedullary solid tumour consisting of myelogenous leukaemic blast cells, usually seen in acute myeloid leukaemia and less commonly in patients with chronic myeloid leukaemia or myeloproliferative disorders. Blast cells have a predilection for periosteal and perineural regions and rarely precede evidence of systemic disease. We present two patients, aleukaemic on peripheral blood counts, both at presentation and during subsequent treatment. We present the MRI features of this rare but important condition. (orig.)

  19. Multimodality Local Therapy for Retroperitoneal Sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    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

    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

  20. Pediatric rhabdomyosarcomas and nonrhabdomyosarcoma soft tissue sarcoma

    OpenAIRE

    Agarwala Sandeep

    2006-01-01

    Tumors arising from the soft tissues are uncommon in children, accounting for about 6% of all childhood malignancies. More than half (53%) of these originate from the striated muscles and are called rhabdomyosarcomas (RMS) the remaining are nonrhabdomyosarcoma soft tissue sarcomas (NRSTS). Almost two-thirds of RMS cases are diagnosed in children < 6 years of age. They can arise at varied locations like the head and neck region, genitourinary tract, extremities, trunk and retrope...

  1. Nonrhabdomyosarcomatous abdominopelvic sarcomas: Analysis of prognostic factors

    Science.gov (United States)

    Iqbal, Nida; Shukla, Nootan K.; Deo, S. V. S.; Agarwala, Sandeep; Sharma, D. N.; Sharma, Meher C.; Bakhshi, Sameer

    2016-01-01

    Background: Data concerning treatment outcome and prognostic factors in sarcomas of abdomen and pelvis are sparse in literature. Methods and Results: Of 696 patients with nonrhabdomyosarcomatous soft tissue sarcoma registered at our center between June 2003 and December 2012, 112 (16%) patients of sarcomas arising from abdomen and pelvis were identified, of which 88 patients were analyzed for treatment outcome and prognostic factors. The median age was 40 years (range: 1–78 years) with a male: female ratio of 0.7:1. Twenty-one (24%) patients were metastatic at baseline. The most common tumor sites were retroperitoneum in 70% patients and abdominal wall in 18% patients. Leiomyosarcoma was the most common histological subtype in 36% patients followed by liposarcoma in 17% patients. Thirty-five (40%) patients had Grade III tumors. Forty-six (52%) patients underwent surgical resection. At a median follow-up of 43 months (range: 2–94 months), the 5-year event-free survival (EFS) and overall survival (OS) were 35% and 42%, with a median of 22 months and 43 months, respectively. Multivariate analysis identified male gender (P - 0.03, hazard ratio [HR] - 0.46, 95% confidence interval [CI] - 0.23–0.92), baseline metastatic disease (P - 0.01, HR - 2.98, 95% CI - 1.27–6.98) and Grade III tumors (P - 0.02, HR - 1.84, 95% CI - 1.08–3.13) as factors associated with poor EFS, whereas baseline metastatic disease (P < 0.001, HR - 5.45, 95% CI - 2.31–12.87) and unresectability (P - 0.01, HR - 2.72, 95% CI - 1.27–5.83) were associated with poor OS. Conclusion: This is a single-institutional study of patients with abdominopelvic sarcomas where gender was identified as a new factor affecting survival apart from baseline presentation, histologic grade, and surgical resection. PMID:27168708

  2. Multimodality Local Therapy for Retroperitoneal Sarcoma

    International Nuclear Information System (INIS)

    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

  3. Thyroid carcino-sarcoma in a dog

    OpenAIRE

    Antonio Giuliano; Jessica Grant; Jerome Benoit

    2013-01-01

    An adult male greyhound was diagnosed with a thyroid carcino-sarcoma by means of histopathology and positive immuno-histochemistry staining for cytokeratin and vimentin. Surgery and radiotherapy of the area were successful in local tumour control. Adjuvant chemotherapy was recommended to treat and prevent further metastasis. The use of carboplatin, metronomic cyclophosphamide chemotherapy and toceranib failed to control the progression of distant metastasis. The survival time was seven months...

  4. Cutaneous myeloid sarcoma of the penile foreskin.

    Science.gov (United States)

    Afrose, Ruquiya; Nebhnani, Deepa; Wadhwa, Neelam

    2015-01-01

    Myeloid sarcoma, considered to herald the onset of a blast crisis in the setting of chronic myeloproliferative neoplasm/dysplasia, typically presents during the course of the disorder. Cutaneous involvement is uncommon and lesions on genital skin are seldom seen. We present a case of a well-differentiated myeloid sarcoma in the penile foreskin in an apparently healthy 29-year-old male presenting with phimosis. The unusual composition of the inflammatory cell infiltrate, and characteristic sparing of dermal blood vessels, nerves and smooth muscle fibres led to the correct diagnosis. Absence of commonly observed changes in the circumcision skin like those of balanitis xerotica was also helpful. Detailed hematological work up revealed a previously undiagnosed chronic myeloid leukemia in chronic phase. The patient also had simultaneous priapism, another rare presentation of chronic myeloid leukemia. One year hence, the patient is in hematological remission with no evidence of extramedullary disease. Although priapism has been described as a rare presenting symptom in chronic myeloid leukemia, the present case is unique as this is the first time a cutaneous myeloid sarcoma has been documented in the penile foreskin. PMID:24913300

  5. CT imaging of primary pleuropulmonary synovial sarcoma

    International Nuclear Information System (INIS)

    Aim: To evaluate the computed tomography (CT) imaging findings of primary pleuropulmonary synovial sarcoma. Materials and methods: Five cases of synovial sarcoma confirmed by histopathology and cytogenetic study were retrospectively analysed. All patients had undergone chest radiography and unenhanced and contrast-enhanced CT examinations, and three had also undergone multiphase CT enhancement examinations. Image characteristics, including shape, size, margin, and attenuation of each lesion before and after contrast enhancement, were analysed. Results: The chest radiographs of the five patients showed well-defined or partly well-defined masses, which were homogeneous and without associated calcification or lymphadenopathy. Pneumothorax was present in one patient. The unenhanced CT images showed well-defined, heterogeneous masses with patchy low density in all five patients. The contrast-enhanced CT images showed heterogeneous enhancement in all cases, three of which demonstrated cystic and necrotic areas. The tumour showed no prolonged or delayed enhancement in three cases using multiphase CT. There were small pleural effusions in four cases. No calcification was observed in any of the cases. There was no evidence of hilar or mediastinal lymphadenopathy. Conclusions: In these five patients, primary pleuropulmonary synovial sarcoma presented as a well-defined mass with patchy low density and heterogeneous enhancement, with no evidence of regional lymphadenopathy. It should be included in the differential diagnosis of regional tumours.

  6. Phosphoproteomics in translational research: a sarcoma perspective.

    Science.gov (United States)

    Noujaim, J; Payne, L S; Judson, I; Jones, R L; Huang, P H

    2016-05-01

    Phosphoproteomics has been extensively used as a preclinical research tool to characterize the phosphorylated components of the cancer proteome. Advances in the field have yielded insights into new drug targets, mechanisms of disease progression and drug resistance, and biomarker discovery. However, application of this technology to clinical research has been challenging because of practical issues relating to specimen integrity and tumour heterogeneity. Beyond these limitations, phosphoproteomics has the potential to play a pivotal role in translational studies and contribute to advances in different tumour groups, including rare disease sites like sarcoma. In this review, we propose that deploying phosphoproteomic technologies in translational research may facilitate the identification of better defined predictive biomarkers for patient stratification, inform drug selection in umbrella trials and identify new combinations to overcome drug resistance. We provide an overview of current phosphoproteomic technologies, such as affinity-based assays and mass spectrometry-based approaches, and discuss their advantages and limitations. We use sarcoma as an example to illustrate the current challenges in evaluating targeted kinase therapies in clinical trials. We then highlight useful lessons from preclinical studies in sarcoma biology to demonstrate how phosphoproteomics may address some of these challenges. Finally, we conclude by offering a perspective and list the key measures required to translate and benchmark a largely preclinical technology into a useful tool for translational research. PMID:26802162

  7. Plasma urea nitrogen and progesterone concentrations and follicular dynamics in ewes fed proteins of different degradability

    Directory of Open Access Journals (Sweden)

    Gustavo Bianchi Lazarin

    2012-07-01

    Full Text Available The effects of overfeeding with protein of different degradability on body condition, plasma urea nitrogen and progesterone concentrations, ovulation number and follicular dynamics were assessed in Santa Ines ewes. Twelve ewes were assigned to a randomized block design according to body weight and received overfeeding with soybean meal or with corn gluten meal or maintenance diet for 28 days before ovulation and during the next estrous cycle. Blood samples were taken on days 7, 14, 21, and 28 after the beginning of treatments for analysis of plasma urea nitrogen and on days 3, 6, 9, 12, and 15 into the estrous cycle for analysis of plasma urea nitrogen and progesterone. Follicular dynamics was monitored daily by ultrasound during one estrous cycle. Dry matter and crude protein intake, weight gain, plasma urea nitrogen concentration before ovulation, number of ovulations, diameter of the largest follicle of the 1st and of the 2nd waves and the growth rate of the largest follicle of the 1st wave were higher in the ewes that received overfeeding. The growth rate of the largest follicle of the 3rd wave was higher in the ewes fed maintenance diet. The back fat thickness, plasma urea nitrogen before ovulation and progesterone concentrations, diameter of the largest follicle of the 2nd wave and growth rate of the largest follicle of the 3rd wave were higher in ewes that received overfeeding with soybean meal. The growth rate of the largest follicle of the 1st wave was higher in ewes that received overfeeding with corn gluten meal. Overfeeding with protein-rich feeds may increase the ovulation number and with soybean meal, it may be effective in increasing plasma progesterone concentration in ewes.

  8. Implantação intracerebral experimental de sarcomas Experimental intracerebral implantation of sarcomas

    Directory of Open Access Journals (Sweden)

    Alexandre Alencar

    1972-01-01

    Full Text Available Estudo histopatológico da implantação intracerebral de sarcomas, realizado em ratos (fibrosarcoma e em camundongos (sarcoma 180. A implantação intracerebral de fibrosarcoma desenvolveu, em cerca de 45 dias, neoplasia relativamente bem delimitada, com pequena infiltração do parênquima nervoso adjacente, nunca se propagando a maiores distãncias devido a forte coesão entre as suas células, com grande diferenciação de fibrilas reticulares e de colágeno. Ao contrário, a inoculação do sarcoma 180, principalmente sob a forma ascítica, levou rapidamente a um quadro de forte hipertensão intracraniana, com disseminação das células neoplásticas pelos espaços subaracnoideanos e intraventriculares. Ambas as neoplasias propagavam-se pelos espaços subaracnoideanos e intraventriculares. Ambas as neoplasias propagavam-se pelos espaços perivasculares, porém o faziam de maneira diversa; o sarcoma 180 tinha uma disseminação muito intensa e rápida, que se fazia a longas distâncias, enquanto que o fibrossarcoma somente se disseminava nos vasos próximos à neoplasia em desenvolvimento. Tomando por base observações próprias e outras colhidas na bibliografia especializada, conclui-se que os agentes etiológicos destes tumores exercem sua ação sobre tipos celulares diferentes.Histopathological study of intracerebral implantation of sarcomas, performed in rats (fibrosarcomas and mice (sarcoma 180. The intracerebral implantation of fibrosarcoma has developed in about 45 days a well limited tumor, with little infiltration of the adjacent nervous parenchyma, never streading to longer distances because the strong cohesion between its cells, with great differentiation of reticular fibers and collagen. On the contrary, the innoculation of sarcoma 180, chiefly of the ascitical form, has rapidly lead to a strong intracranial hipertension, with dissemination of neoplastic cells through the subarachnoid and intraventricular spaces. Both

  9. Interval compressed vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, etoposide in patients with advanced Ewing’s and other Small Round Cell Sarcomas

    Directory of Open Access Journals (Sweden)

    Whelan Jeremy

    2012-09-01

    Full Text Available Abstract Background To evaluate tolerability and maintenance of dose intensity of 2 weekly treatment with vincristine, doxorubicin, cyclophosphamide alternating with ifosfamide, etoposide (VDC/IE in patients with advanced small round cell sarcomas including Ewing family tumours (EFT, desmoplastic small round cell tumours (DSRCT and undifferentiated high grade round cell sarcomas (UHGRCS. Methods Retrospective review of 16 patients treated at a single centre with VDC/IE. Dose received, treatment delay, toxicity and clinical outcome were recorded for each cycle up to a maximum of 14 cycles. Results A total 193 cycles of VDC/IE were administered to 10 patients with EFT, 4 with DSRCT and 2 with UHGRCS. Median age was 22 years with 75% over 18 years. Metastases were present in 14 patients. The mean duration of each cycle was 16.7 days. Febrile neutropenia occurred in 14 % of cycles, and grade 3/4 haematologic toxicity including anaemia and thrombocytopenia in 16 % and 11 % of cycles respectively. Seven patients had a dose reduction. Five patients discontinued VDC/IE early due to toxicity. Conclusions This schedule of VDC/IE is feasible in patients with EFT and DSRCT including adults and those with metastases. Its comparison with other standard regimens for these diseases is justified.

  10. Estrus response and fertility of Menz and crossbred ewes to single prostaglandin injection protocol.

    Science.gov (United States)

    Mekuriaw, Zeleke; Assefa, Habtemariam; Tegegne, Azage; Muluneh, Dagne

    2016-01-01

    Natural lambing in sheep in Ethiopia occurs throughout the year in a scattered manner negatively affecting survival and growth rates of the lambs born during the unfavorable season of the year. Thus, controlling the time of mating artificially using exogenous source of hormones is considered as one of the ways to mitigated problems related to haphazard lambing. To this end, an experiment was conducted to evaluate efficacy of prostaglandin-based estrus synchronization protocol in local and crossbred ewes. A total of 160 ewes (80 local and 80 crossbreds) which lambed at least once and aged 3-5 years were used. Lutalyse® (dinoprost tromethamine sterile solution equivalent to 5 mg dinoprost per ml) and its analog, Synchromate® (cloprostenol sodium equivalent to 0.250 mg cloprostenol per ml), were tested at different doses. The treatments used were intramuscular injection of (1) 2.50 ml of Lutalyse® (12.5 mg dinoprost tromethamine), (2) 2 ml of Lutalyse® (10.0 mg dinoprost tromethamine), (3) 1 ml of Synchromate® (0.25 mg of cloprostenol Sodium), and (4) 0.8 ml of Synchromate® (0.20 mg of cloprostenol Sodium). Forty ewes (20 local and 20 crossbreds) were allocated per treatment. Following injection of the respective hormones, rams of known fertility were introduced into the flock for the duration of 96 h at the ratio of one ram to 10 ewes. All estrus synchronization protocols except treatment 4 (0.8 ml of Synchromate®) induced estrus (heat) in majority (55-65%) of local and crossbred ewes within 96 h post-hormone injection. The time interval from hormone administration to onset of estrus was also more or less similar for all treatment groups except for treatment group 4 which showed heat quicker. The highest lambing rate was recorded in local ewes (84.62% (11/13) treated with 2.5 ml of Lutalyse®, whereas the least was obtained in crossbreds (33.33% (3/9) treated with 0.8 ml Synchromate®. In conclusion, even though 2.5 ml and 2 ml of Lutalyse® or 1 ml of

  11. Electromagnetic Dissociation Cross Sections using Weisskopf-Ewing Theory

    Science.gov (United States)

    Adamczyk, Anne M.; Norbury, John W.

    2011-01-01

    It is important that accurate estimates of crew exposure to radiation are obtained for future long-term space missions. Presently, several space radiation transport codes exist to predict the radiation environment, all of which take as input particle interaction cross sections that describe the nuclear interactions between the particles and the shielding material. The space radiation transport code HZETRN uses the nuclear fragmentation model NUCFRG2 to calculate Electromagnetic Dissociation (EMD) cross sections. Currently, NUCFRG2 employs energy independent branching ratios to calculate these cross sections. Using Weisskopf-Ewing (WE) theory to calculate branching ratios, however, is more advantageous than the method currently employed in NUCFRG2. The WE theory can calculate not only neutron and proton emission, as in the energy independent branching ratio formalism used in NUCFRG2, but also deuteron, triton, helion, and alpha particle emission. These particles can contribute significantly to total exposure estimates. In this work, photonuclear cross sections are calculated using WE theory and the energy independent branching ratios used in NUCFRG2 and then compared to experimental data. It is found that the WE theory gives comparable, but mainly better agreement with data than the energy independent branching ratio. Furthermore, EMD cross sections for single neutron, proton, and alpha particle removal are calculated using WE theory and an energy independent branching ratio used in NUCFRG2 and compared to experimental data.

  12. Pazopanib in the management of advanced soft tissue sarcomas

    Science.gov (United States)

    Cranmer, Lee D; Loggers, Elizabeth T; Pollack, Seth M

    2016-01-01

    Therapy of soft tissue sarcomas represents an area of significant unmet need in oncology. Angiogenesis has been explored as a potential target both preclinically and clinically, with suggestions of activity. Pazopanib is a multitargeted tyrosine kinase inhibitor with prominent antiangiogenic effects. In a Phase II study, pazopanib demonstrated activity in strata enrolling patients with leiomyosarcomas, synovial sarcomas, or other sarcomas but not those enrolling adipocytic sarcomas. PALETTE, the pivotal Phase III trial, demonstrated improved progression-free survival versus placebo in pazopanib-treated patients previously treated for advanced soft tissue sarcomas. No survival benefit was observed, and adipocytic sarcomas were excluded. Health-related quality-of-life assessments indicated significant decrements in several areas affected by pazopanib toxicities, but no global deterioration. Cost-effectiveness analyses indicate that pazopanib therapy may or may not be cost-effective in different geographic settings. Pazopanib provides important proof-of-concept for antiangiogenic therapy in soft tissue sarcomas. Its use can be improved by further biological studies of its activity profile in sarcomas, studies of biological rational combinations, and clinicopathologic/biological correlative studies of activity to allow better drug targeting. PMID:27354810

  13. Mediastinal Synovial Sarcoma: A Case Report and Literature Review

    OpenAIRE

    Linda SL Cheng; Gary MK Tse; Wilson WL Li; Lee TW; Anthony PC Yim

    2003-01-01

    Synovial sarcomas are uncommon soft tissue tumours. Immunohistochemistry and cytogenetic techniques are essential for proper diagnosis and differentiation from other spindle cell neoplasms. A case of mediastinal synovial sarcoma is described, of which the unusual location, diagnosis and treatment form the basis of this report.

  14. Synovial Sarcoma Involving the Median Nerve: A Case Report

    Directory of Open Access Journals (Sweden)

    Anna Lisa TOSI

    2012-09-01

    Full Text Available Synovial sarcoma may arise from different and unusual sites. Here a case of biphasic synovial sarcoma arising or invading the radial nerve in a 59-year-old female classically showing chromosomal reciprocal translocation (X; 18 is reported. The differential diagnosis from similar tumors is discussed.

  15. Synovial Sarcoma Involving the Median Nerve: A Case Report

    OpenAIRE

    Anna Lisa TOSI; Orcioni, Giulio Fraternali; Biase, Dario de; Sara COSTANTINI; Ishikawa, Yuko; Eusebi, Vincenzo

    2012-01-01

    Synovial sarcoma may arise from different and unusual sites. Here a case of biphasic synovial sarcoma arising or invading the radial nerve in a 59-year-old female classically showing chromosomal reciprocal translocation (X; 18) is reported. The differential diagnosis from similar tumors is discussed.

  16. Pulmonary metastasis of synovial sarcoma simulating residuals granuloma

    International Nuclear Information System (INIS)

    An unusual case of a synovial sarcoma of the left forearm with calcified pulmonary metastases is reported. Three calcified pulmonary nodules were found two years after resection of the patient's primary synovial sarcoma. Earch nodule showed the same pattern of calcification, resembling granulomas and simulating bening lesions. (author)

  17. Chemotherapy of synovial cell sarcoma in a dog

    International Nuclear Information System (INIS)

    A synovial cell sarcoma of the tarsus in a 6-year-old dog was treated with doxorubicin HCl and cyclophosphamide. The tumor regressed after treatment. There was no recurrence 3 years after initiation of treatment. Previously, synovial cell sarcoma has not been reported to be responsive to chemotherapeutic agents, and the treatment of choice has been amputation

  18. Strategic study on energy-protein requirements for local sheep: 5. Ewes during lactation phase

    Directory of Open Access Journals (Sweden)

    I-W Mathius

    2004-03-01

    Full Text Available Thirty-six Javanese thin-tail ewes in the end of late pregnancy phase were set out to study the energy and crude protein requirements during the first eight-week of lactation phase. The ewes were penned individually in doors and randomly assigned to a 3 x 3 factorial arrangement, consisting of three levels of energy (low, medium and high and three levels of crude protein (low, medium and high diets with four ewes per treatment. The diets were pelleted and offered four times daily in approximately equal amount. Feed intake, nutrient digestibility, body weight and milk production were recorded. Results showed that, total lamb birth weights was not affected, but protein content on the ration treatments significantly altered (P0.05, while crude protein content on the ration highly significantly affected (P<0.01. Based on data recorded, the energy and protein requirements for ewes during lactation phase are highly significantly depended on ewes’ live weight, milk production and the ratio of energy metabolism and crude protein of the ration. It was concluded that in order to fulfil the crude protein and energy needs of the ewes during lactation phase, the ration given should contain crude protein and energy as much as 16% (based on dry matter and 13.4 MJ/kg dry matter respectively.

  19. Reproductive performance of Ile de France ewes under dietary supplementation before and during the breeding season

    Directory of Open Access Journals (Sweden)

    Luis Gabriel Alves Cirne

    2016-02-01

    Full Text Available The aim of this study was to evaluate the reproductive performance of Ile de France ewes undergoing dietary supplementation before and during the breeding season, with and without association with management conditions (pre-mating shearing. Thirty-six ewes with an average body weight of 66 kg were used in the experiment. Treatments involved ewes receiving or not receiving concentrate supplementation (flushing, with groups subdivided according to the management condition to which animals were subjected: shearing or lack of it. Thus, ewes were divided into four treatments: flushed and shorn; flushed and unshorn; unflushed and shorn; and unflushed and unshorn. Flushing increased weight gain and body condition score, and when associated with shearing, it promoted anticipation of estrus. Fertility rate (86.05%, calving rate (77.77%, birth rate (113.83%, and type of birth (single: 82.29% and twin: 17.71% were not influenced. Birth weight (3.96 kg and prolificacy (1.25% also were not affected. Despite the lack of changes in reproductive traits, flushing adopted during the breeding season associated with shearing anticipated estrus in ewes.

  20. Dietary -carbamylglutamate and rumen-protected -arginine supplementation ameliorate fetal growth restriction in undernourished ewes.

    Science.gov (United States)

    Zhang, H; Sun, L W; Wang, Z Y; Deng, M T; Zhang, G M; Guo, R H; Ma, T W; Wang, F

    2016-05-01

    This study was conducted with an ovine intrauterine growth restriction (IUGR) model to test the hypothesis that dietary -carbamylglutamate (NCG) and rumen-protected -Arg (RP-Arg) supplementation are effective in ameliorating fetal growth restriction in undernourished ewes. Beginning on d 35 of gestation, ewes were fed a diet providing 100% of NRC-recommended nutrient requirements, 50% of NRC recommendations (50% NRC), 50% of NRC recommendations supplemented with 20 g/d RP-Arg (providing 10 g/d of Arg), and 50% of NRC recommendations supplemented with 5 g/d NCG product (providing 2.5 g/d of NCG). On d 110, maternal, fetal, and placental tissues and fluids were collected and weighed. Ewe weights were lower ( effect on concentrations of lactate and GH. Maternal RP-Arg or NCG supplementation markedly improved ( amniotic fluids within nutrient-restricted ewes. These novel results indicate that dietary NCG and RP-Arg supplementation to underfed ewes ameliorated fetal growth restriction, at least in part, by increasing the availability of AA in the conceptus and provide support for its clinical use to ameliorate IUGR in humans and sheep industry production. PMID:27285704