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Sample records for chemo-resistant ewing sarcoma

  1. Ewing sarcoma

    Science.gov (United States)

    Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... this tissue to help determine how aggressive the cancer is and what treatment may be best.

  2. Collecting and Storing Biological Samples From Patients With Ewing Sarcoma

    Science.gov (United States)

    2016-11-21

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

  3. Diagnostic Study of Tumor Characteristics in Patients With Ewing's Sarcoma

    Science.gov (United States)

    2013-06-20

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

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

    Science.gov (United States)

    2016-08-25

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

  5. Ewing's Sarcoma and Second Malignancies

    Directory of Open Access Journals (Sweden)

    Joshua D. Schiffman

    2011-01-01

    Full Text Available Ewing's sarcoma (ES is a rare tumor that is most common in children and young adults. Late effects of ES therapy include second cancers, a tragic outcome for survivors of such a young age. This paper will explore the frequencies and types of malignancies that occur after ES. Additionally, it will review how second malignancies have changed with the shift in treatment from high-dose radiation to chemotherapy regimens including alkylators and epipodophyllotoxins. The risk of additional cancers in ES survivors will also be compared to survivors of other childhood cancers. Finally, the possible genetic contribution to ES and second malignancies will be discussed.

  6. Extraosseus Ewing sarcoma: An uncommon periclavicular location

    Directory of Open Access Journals (Sweden)

    Fabrizio Albarello, MD

    2015-01-01

    Full Text Available A rapidly enlarging right sternoclavicular mass in a young male was labeled as a nonspecific mass. MRI played a crucial role in characterizing the lesion, helping to define the possible mesenchymal origin and the relative involvement of the surrounding structures. We also discuss the differential diagnosis of an extraosseus Ewing sarcoma (ES, with its imaging findings.

  7. Is There a Predisposition Gene for Ewing's Sarcoma?

    Directory of Open Access Journals (Sweden)

    R. L. Randall

    2010-01-01

    Full Text Available Ewing's sarcoma is a highly malignant tumor of children and young adults. The molecular mechanisms that underlie Ewing's Sarcoma development are beginning to be understood. For example, most cases of this disease harbor somatic chromosomal translocations that fuse the EWSR1 gene on chromosome 22 with members of the ETS family. While some cooperative genetic events have been identified, such as mutations in TP53 or deletions of the CDKN2A locus, these appear to be absent in the vast majority of cases. It is therefore uncertain whether EWS/ETS translocations are the only consistently present alteration in this tumor, or whether there are other recurrent abnormalities yet to be discovered. One method to discover such mutations is to identify familial cases of Ewing's sarcoma and to then map the susceptibility locus using traditional genetic mapping techniques. Although cases of sibling pairs with Ewing's sarcoma exist, familial cases of Ewing's sarcoma have not been reported. While Ewing's sarcoma has been reported as a 2nd malignancy after retinoblastoma, significant associations of Ewing's sarcoma with classic tumor susceptibility syndromes have not been identified. We will review the current evidence, or lack thereof, regarding the potential of a heritable condition predisposing to Ewing's sarcoma.

  8. Ewing Sarcoma of the Posterior Fossa in an Adolescent Girl

    Directory of Open Access Journals (Sweden)

    Andreas M. Stark

    2014-01-01

    Full Text Available Medulloblastoma, astrocytoma, and ependymoma represent the most common infratentorial tumors in childhood, while Ewing sarcomas in that localization are extremely rare. A large left infratentorial space-occupying lesion was diagnosed in a 12-year-old girl with signs of increased intracranial pressure. Following total tumor resection, histological and molecular examination revealed Ewing sarcoma with rearranged EWSR-1 gene. The patient achieved complete remission following adjuvant chemotherapy and radiotherapy according to Euro-EWING 2008 treatment protocol. Intracranial Ewing sarcoma, although rare, should be an important differential diagnosis of intracranial tumors in childhood which requires aggressive multimodal treatment.

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

    Science.gov (United States)

    2017-02-08

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

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

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    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.)

  11. Primary extraosseous Ewing sarcoma of the orbit.

    Science.gov (United States)

    Alio, Jorge L; Sales-Sanz, Marco; Vaz, Maria A; Barrancos, Constanza; Reguero, Maria E; Diamantopoulus, Jorge; Poveda, Pedro

    2013-01-01

    A 40-year-old man presented with painless, progressive vision loss and mild proptosis of the OD. CT revealed a right intraconal mass with slight penetration of the optic canal not contiguous with any bony structure. Incisional biopsy through a transfrontal orbitotomy revealed a diffuse growth of homogeneous, small, round cells. Immunohistochemical stains were positive for vimentin and MIC2 (CD99), and the translocation at EWS gene (22q12) was detected. Metastatic workup and a full-body bone scan were negative, confirming primary orbital extraosseous Ewing sarcoma. The patient received neoadjuvant chemotherapy and an orbital exenteration with preservation of eyelids and conjunctiva. He also received adjuvant chemotherapy and local radiotherapy, and he has remained disease-free for almost 3 years.

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

    Science.gov (United States)

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

    2014-10-01

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

  13. Perigastric extraskeletal Ewing's sarcoma: A case report

    Institute of Scientific and Technical Information of China (English)

    Radoje B Colovic; Nikica M Grubor; Marjan T Micev; Slavko V Matic; Henry Dushan Edward Atkinson; Stojan M Latincic

    2009-01-01

    Ewing's sarcoma (ES) is a neoplasm of undifferentiated small round cells, which occurs in the bones and deep soft tissues of children and adolescents. We present a rare case of a 44-year-old woman with gastric ES presenting with epigastric pain and weight loss. Ultrasound and computed tomography scans indicated a solid/cystic mass in the pancreatic tail. At laparotomy, the tumor was found attached to the posterior surface of the stomach, completely free from the pancreas, with no lymphadenopathy or local metastases. The polynodal, partly pseudocystic, dark-red soft tumor was excised. Histopathology revealed an anaplastic small-round-cell tumor with strong membranous CD99 immunoexpression. Additionally, there was patchy immunostaining for S-100 protein, vimentin, protein gene product (PGP) 9.5 and neuron-specific enolase, and weak focal CD117 cytoplasmic immunoreactivity. The patient had no adjuvant chemotherapy; her postoperative recovery was uneventful, and she remains symptom-free, and without any sign of recurrence at 20 mo. To the best of our knowledge, this is only the third ever case of gastric ES.

  14. Adult Intramedullary Ewing Sarcoma of the Proximal Hip

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    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.

  15. Transarticular spread of Ewing sarcoma mimicking septic arthritis

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    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.)

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

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    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.

  17. Ewing sarcoma in adolescents and young adults: diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Davis LE

    2014-09-01

    Full Text Available Lara E Davis,1,2 Suman Malempati2 1Department of Medicine, 2Department of Pediatrics, Oregon Health and Science University, Portland, OR, USA Abstract: Ewing sarcoma (ES is a rare tumor that most often occurs in adolescents and young adults. This review discusses the diagnosis, prognosis, and treatment of localized and metastatic ES, with an emphasis on the care of adolescent and young adult patients. The pathobiology is reviewed. Particular attention is given to recent and current clinical trials, and an introduction to future directions for therapy of ES is provided. Keywords: Ewing, sarcoma, primitive neuroectodermal tumor, PNET

  18. Efficacy of ATR inhibitors as single agents in Ewing sarcoma

    DEFF Research Database (Denmark)

    Nieto-Soler, Maria; Morgado-Palacin, Isabel; Lafarga, Vanesa;

    2016-01-01

    Ewing sarcomas (ES) are pediatric bone tumors that arise from a driver translocation, most frequently EWS/FLI1. Current ES treatment involves DNA damaging agents, yet the basis for the sensitivity to these therapies remains unknown. Oncogene-induced replication stress (RS) is a known source...... efficacy in ES xenografts as single agents. Expression of EWS/FLI1 or EWS/ERG oncogenic translocations sensitizes non-ES cells to ATR inhibitors. Our data shed light onto the sensitivity of ES to genotoxic agents, and identify ATR inhibitors as a potential therapy for Ewing Sarcomas....

  19. Combinatorial Drug Screening Identifies Ewing Sarcoma-specific Sensitivities

    DEFF Research Database (Denmark)

    Radic-Sarikas, Branka; Tsafou, Kalliopi P; Emdal, Kristina B.;

    2017-01-01

    including approved drugs. We were able to retrieve highly synergistic drug combinations specific for Ewing sarcoma and identified signaling processes important for Ewing sarcoma cell proliferation determined by EWS-FLI1 We generated a molecular target profile of PKC412, a multikinase inhibitor with strong...... and IGF1R inhibitors. The mechanism of the drug synergy between these inhibitors is different from the sum of the mechanisms of the single agents. The combination effectively inhibited pathway crosstalk and averted feedback loop repression, in EWS-FLI1-dependent manner. Mol Cancer Ther; 16(1); 88...

  20. Targeting the p53 Pathway in Ewing Sarcoma

    OpenAIRE

    Neilsen, Paul M.; Pishas, Kathleen I.; Callen, David F; Thomas, David M.

    2011-01-01

    The p53 tumour suppressor plays a pivotal role in the prevention of oncogenic transformation. Cancers frequently evade the potent antitumour surveillance mechanisms of p53 through mutation of the TP53 gene, with approximately 50% of all human malignancies expressing dysfunctional, mutated p53 proteins. Interestingly, genetic lesions in the TP53 gene are only observed in 10% of Ewing Sarcomas, with the majority of these sarcomas expressing a functional wild-type p53. In addition, the p53 downs...

  1. Microsatellites with Macro-Influence in Ewing Sarcoma

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    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.

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

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    Smita Srivastava

    2016-01-01

    Full Text Available 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. Targeting the p53 Pathway in Ewing Sarcoma

    Directory of Open Access Journals (Sweden)

    Paul M. Neilsen

    2011-01-01

    Full Text Available The p53 tumour suppressor plays a pivotal role in the prevention of oncogenic transformation. Cancers frequently evade the potent antitumour surveillance mechanisms of p53 through mutation of the TP53 gene, with approximately 50% of all human malignancies expressing dysfunctional, mutated p53 proteins. Interestingly, genetic lesions in the TP53 gene are only observed in 10% of Ewing Sarcomas, with the majority of these sarcomas expressing a functional wild-type p53. In addition, the p53 downstream signaling pathways and DNA-damage cell cycle checkpoints remain functionally intact in these sarcomas. This paper summarizes recent insights into the functional capabilities and regulation of p53 in Ewing Sarcoma, with a particular focus on the cross-talk between p53 and the EWS-FLI1 gene rearrangement frequently associated with this disease. The development of several activators of p53 is discussed, with recent evidence demonstrating the potential of small molecule p53 activators as a promising systemic therapeutic approach for the treatment of Ewing Sarcomas with wild-type p53.

  4. Ewing sarcoma of the oral cavity. A review

    Science.gov (United States)

    Bagán, José; Poveda-Roda, Rafael

    2017-01-01

    Objectives A review is made of the clinical, diagnostic, therapeutic and survival characteristics of Ewing sarcoma (ES) of the oral cavity. Material and Methods A systematic literature search was carried out, with restrictions referred to time (1960-2014), language (English and Spanish) and type of study (case reports, letters, datasets, reviews). The following MeSH terms and boolean operators were used: Ewing AND Sarcoma AND [tongue, jaw, maxilla, cheek, condyle OR temporomandibular, floor AND mouth, gum OR gingiva, palate OR palatal, lip, uvula, head AND neck]. Results Seventy-one cases of ES of the oral cavity were documented from 53 articles. The main differences versus ES of other locations were a younger age at manifestation, a shorter time from symptoms onset to diagnosis, and swelling as the most frequent clinical manifestation versus swelling and pain in the rest of disease locations. The way in which ES manifests in the oral cavity is varied and comprises dental displacement (19.7%), dental mobility (7%), root reabsorption (5.6%), destruction of the dental follicle (4.2%), premature exfoliation (4.2%) and paresthesia of the chin (2.8%). Metastatic neck adenopathies appear in 11.3% of the cases. Significant differences in survival are observed between patients with a complete diagnosis of ES (hematoxylin-eosin staining, PAS positivity, CD99 positivity) and those with an incomplete diagnosis. Conclusions Ewing sarcoma of the oral cavity presents a series of specific features that distinguish it from ES of other locations. Key words:Primitive neuroectodermal tumor, PNET, Ewing sarcoma, Ewing tumor, sarcoma, oral cavity. PMID:28210452

  5. Extraosseous Ewing's sarcoma: review of a case. Sarcoma de Ewing extraoseo: revision a proposito de un caso

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    Quevedo Moreno, P.; Hernandez Moreno, L.; Perez Diaz, M.; Lafuente Martinez, J.L.; Arozamena Laso, M.

    1994-01-01

    Extraosseous Ewing's sarcoma (EES) is an uncommon lesion included in the group of soft tissue tumors. We present a case in a 19-year-old woman in which the diagnosis was not initially suspected because of the absence of clinical and radiological evidence. (Author)

  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. 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

  8. 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.

  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. Primitive neuroectodermal tumor/Ewing sarcoma of the retina.

    Science.gov (United States)

    Grossniklaus, Hans E; Shehata, Bahig; Sorensen, Poul; Bergstrom, Chris; Hubbard, G Baker

    2012-07-01

    An 11-year-old boy underwent enucleation of his left eye for an intraocular tumor. Examination showed a small, round blue cell tumor arising in the peripheral retina near the ciliary body. Immunohistochemical stain results were positive for neuron-specific enolase, synaptophysin, cluster of differentiation 99 (CD99), Friend leukemia integration 1, and CD56. Ultrastructural findings included occasional intracytoplasmic dense core granules. Polymerase chain reaction of the tumor showed a Ewing sarcoma/Friend leukemia integration gene fusion product. The tumor was classified as a primitive neuroectodermal tumor/Ewing sarcoma of the retina and should be distinguished from retinoblastoma. To our knowledge, this is the first case of primary primitive neuroectodermal tumor of the retina.

  11. Ewing sarcoma mimicking a peripheral nerve sheath tumor.

    Science.gov (United States)

    Mitchell, B D; Fox, B D; Viswanathan, A; Mitchell, A H; Powell, S Z; Cech, D A

    2010-10-01

    We describe the first patient with an extradural, extramedullary Ewing's sarcoma tumor mimicking a nerve sheath tumor with no overt evidence of metastasis. A 28-year-old woman with no past medical history presented with a progressive 3-year history of low back pain and right-sided lower extremity radiculopathy after having failed conservative therapies. MRI of the lumbar spine revealed a right-sided enhancing, dumbbell-shaped lesion at the right neural foramen appearing to originate from the L4 nerve root, suspicious for a peripheral nerve sheath tumor or schwannoma. The patient and findings are discussed in the context of the literature, including an update on the relatively recent diagnostic redesignation of the Ewing's sarcoma family tumors.

  12. RNA helicase DDX3: a novel therapeutic target in Ewing sarcoma.

    Science.gov (United States)

    Wilky, B A; Kim, C; McCarty, G; Montgomery, E A; Kammers, K; DeVine, L R; Cole, R N; Raman, V; Loeb, D M

    2016-05-19

    RNA helicase DDX3 has oncogenic activity in breast and lung cancers and is required for translation of complex mRNA transcripts, including those encoding key cell-cycle regulatory proteins. We sought to determine the expression and function of DDX3 in sarcoma cells, and to investigate the antitumor activity of a novel small molecule DDX3 inhibitor, RK-33. Utilizing various sarcoma cell lines, xenografts and human tissue microarrays, we measured DDX3 expression at the mRNA and protein levels, and evaluated cytotoxicity of RK-33 in sarcoma cell lines. To study the role of DDX3 in Ewing sarcoma, we generated stable DDX3-knockdown Ewing sarcoma cell lines using DDX3-specific small hairpin RNA (shRNA), and assessed oncogenic activity. DDX3-knockdown and RK-33-treated Ewing sarcoma cells were compared with wild-type cells using an isobaric mass-tag quantitative proteomics approach to identify target proteins impacted by DDX3 inhibition. Overall, we found high expression of DDX3 in numerous human sarcoma subtypes compared with non-malignant mesenchymal cells, and knockdown of DDX3 by RNA interference inhibited oncogenic activity in Ewing sarcoma cells. Treatment with RK-33 was preferentially cytotoxic to sarcoma cells, including chemotherapy-resistant Ewing sarcoma stem cells, while sparing non-malignant cells. Sensitivity to RK-33 correlated with DDX3 protein expression. Growth of human Ewing sarcoma xenografts expressing high DDX3 was inhibited by RK-33 treatment in mice, without overt toxicity. DDX3 inhibition altered the Ewing sarcoma cellular proteome, especially proteins involved in DNA replication, mRNA translation and proteasome function. These data support further investigation of the role of DDX3 in sarcomas, advancement of RK-33 to Ewing sarcoma clinical trials and development of RNA helicase inhibition as a novel anti-neoplastic strategy.

  13. Primary Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of the penis: a report of an extraordinarily unusual site and a literature review of extraskeletal Ewing's sarcoma.

    Science.gov (United States)

    Ma, Zheng; Brimo, Fadi; Zeizafoun, Nebras

    2013-02-01

    Ewing's sarcomas/primitive neuroectodermal tumors (ES/PNETs) arise from a multipotent progenitor cell and are considered to be of neuroectodermal derivation. Most tumors commonly arise in the skeletal system, which are the classic ES/PNET and occasionally occur in the soft tissue of extraskeletal sites, which are named extraskeletal Ewing's sarcomas (EES/PNET). This study reports a case of a 28-year-old man with primary EES/PNET of the penis.

  14. 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

  15. 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.

  16. Skull-base Ewing sarcoma with multifocal extracranial metastases

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    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.

  17. Extensive primary Ewings' sarcoma in the greater wing of the sphenoid bone.

    Science.gov (United States)

    Apostolopoulos, Kostas; Ferekidis, Eleftherios

    2003-01-01

    We describe a rare case of an extensive primary cranial Ewing's sarcoma located in the greater wing of the sphenoid bone with extension to the orbit, the endocranium, the parapharyngeal and infratemporal space. The patient presented with diplopia, anosmia and prolapse of the left eye. He was given chemo- and radiotherapy and was free of symptoms on re-examination 1.5 years later. The prognosis of Ewing's sarcoma in the absence of surgery is uncertain, but prompt treatment appears to have a satisfactory therapeutic outcome. In the future, more cases should be studied in order to investigate the biological behaviour of a primary cranial Ewing's sarcoma.

  18. Primary Ewing's sarcoma of the vertebral column

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    Ilaslan, Hakan; Sundaram, Murali [Department of Radiology, Mayo Clinic, Ch2-290 200 First Street, SW, Rochester, 55905, MN (United States); Unni, K.Krishnan [Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, 55905, Rochester, MN (United States); Dekutoski, Mark B. [Department of Orthopedic Surgery, Mayo Clinic, 200 First Street, SW, 55905, Rochester, MN (United States)

    2004-09-01

    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

  19. Combination Chemotherapy With or Without Ganitumab in Treating Patients With Newly Diagnosed Metastatic Ewing Sarcoma

    Science.gov (United States)

    2017-03-27

    Metastatic Ewing Sarcoma; Metastatic Malignant Neoplasm in the Bone; Metastatic Malignant Neoplasm in the Bone Marrow; Metastatic Malignant Neoplasm in the Lung; Metastatic Peripheral Primitive Neuroectodermal Tumor of Bone; Peripheral Primitive Neuroectodermal Tumor of Soft Tissues

  20. Ten-Year Follow-Up of a Patient with Metastatic Ewing's Sarcoma of the Pelvis

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    Robert U. Ashford

    2002-01-01

    Full Text Available Patient: We report a 32-year-old women with a pelvic Ewing's sarcoma, who developed skeletal metastases within 20 months of diagnosis but following treatment remains disease-free at 10 years.

  1. Targeting the DNA repair pathway in Ewing sarcoma.

    Science.gov (United States)

    Stewart, Elizabeth; Goshorn, Ross; Bradley, Cori; Griffiths, Lyra M; Benavente, Claudia; Twarog, Nathaniel R; Miller, Gregory M; Caufield, William; Freeman, Burgess B; Bahrami, Armita; Pappo, Alberto; Wu, Jianrong; Loh, Amos; Karlström, Åsa; Calabrese, Chris; Gordon, Brittney; Tsurkan, Lyudmila; Hatfield, M Jason; Potter, Philip M; Snyder, Scott E; Thiagarajan, Suresh; Shirinifard, Abbas; Sablauer, Andras; Shelat, Anang A; Dyer, Michael A

    2014-11-06

    Ewing sarcoma (EWS) is a tumor of the bone and soft tissue that primarily affects adolescents and young adults. With current therapies, 70% of patients with localized disease survive, but patients with metastatic or recurrent disease have a poor outcome. We found that EWS cell lines are defective in DNA break repair and are sensitive to PARP inhibitors (PARPis). PARPi-induced cytotoxicity in EWS cells was 10- to 1,000-fold higher after administration of the DNA-damaging agents irinotecan or temozolomide. We developed an orthotopic EWS mouse model and performed pharmacokinetic and pharmacodynamic studies using three different PARPis that are in clinical development for pediatric cancer. Irinotecan administered on a low-dose, protracted schedule previously optimized for pediatric patients was an effective DNA-damaging agent when combined with PARPis; it was also better tolerated than combinations with temozolomide. Combining PARPis with irinotecan and temozolomide gave complete and durable responses in more than 80% of the mice.

  2. MR findings of primary Ewing's sarcoma of greater wing of sphenoid.

    Science.gov (United States)

    Singh, Paramjeet; Jain, Manoj; Singh, D P; Kalra, Naveen; Khandelwal, N; Suri, S

    2002-12-01

    Primary Ewing's sarcoma of the skull is a very rare entity. We report MRI findings in a case of Ewing's sarcoma of the greater wing of sphenoid in a 4-year-old patient. Magnetic resonance imaging showed markedly heterogenous signal intensity with areas of haemorrhage and necrosis. It also demonstrated the exact extent of tumour due to its multiplanar capabilities and was, therefore, helpful in planning surgery.

  3. EWS/FLI1 Target Genes and Therapeutic Opportunities in Ewing Sarcoma

    Science.gov (United States)

    Cidre-Aranaz, Florencia; Alonso, Javier

    2015-01-01

    Ewing sarcoma is an aggressive bone malignancy that affect children and young adults. Ewing sarcoma is the second most common primary bone malignancy in pediatric patients. Although significant progress has been made in the treatment of Ewing sarcoma since it was first described in the 1920s, in the last decade survival rates have remained unacceptably invariable, thus pointing to the need for new approaches centered in the molecular basis of the disease. Ewing sarcoma driving mutation, EWS–FLI1, which results from a chromosomal translocation, encodes an aberrant transcription factor. Since its first characterization in 1990s, many molecular targets have been described to be regulated by this chimeric transcription factor. Their contribution to orchestrate Ewing sarcoma phenotype has been reported over the last decades. In this work, we will focus on the description of a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance. We will also discuss their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development. PMID:26258070

  4. 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

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

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    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.

  6. Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Stomach

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    Safi Khuri

    2016-11-01

    Full Text Available Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation. These findings were compatible with gastric ES/PNET. The fluorescence in situ hybridization analysis result for the EWSR1 gene rearrangement (11: 22 translocation was positive. The patient refused neoadjuvant treatment and thus underwent an operation during which a mass at the lesser curvature of the stomach was found. The mass was adhering to the pancreatic tail and to the mesentery of the transverse and descending colon. Total gastrectomy, distal pancreatectomy, splenectomy, and left adrenalectomy were done. The patient refused adjuvant treatment. She is free of disease 3 years after surgery.

  7. Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Stomach

    Science.gov (United States)

    Khuri, Safi; Gilshtein, Hayim; Sayidaa, Sa'd; Bishara, Bishara; Kluger, Yoram

    2016-01-01

    Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation). These findings were compatible with gastric ES/PNET. The fluorescence in situ hybridization analysis result for the EWSR1 gene rearrangement (11: 22 translocation) was positive. The patient refused neoadjuvant treatment and thus underwent an operation during which a mass at the lesser curvature of the stomach was found. The mass was adhering to the pancreatic tail and to the mesentery of the transverse and descending colon. Total gastrectomy, distal pancreatectomy, splenectomy, and left adrenalectomy were done. The patient refused adjuvant treatment. She is free of disease 3 years after surgery. PMID:27920700

  8. Targeting the DNA Repair Pathway in Ewing Sarcoma

    Directory of Open Access Journals (Sweden)

    Elizabeth Stewart

    2014-11-01

    Full Text Available Ewing sarcoma (EWS is a tumor of the bone and soft tissue that primarily affects adolescents and young adults. With current therapies, 70% of patients with localized disease survive, but patients with metastatic or recurrent disease have a poor outcome. We found that EWS cell lines are defective in DNA break repair and are sensitive to PARP inhibitors (PARPis. PARPi-induced cytotoxicity in EWS cells was 10- to 1,000-fold higher after administration of the DNA-damaging agents irinotecan or temozolomide. We developed an orthotopic EWS mouse model and performed pharmacokinetic and pharmacodynamic studies using three different PARPis that are in clinical development for pediatric cancer. Irinotecan administered on a low-dose, protracted schedule previously optimized for pediatric patients was an effective DNA-damaging agent when combined with PARPis; it was also better tolerated than combinations with temozolomide. Combining PARPis with irinotecan and temozolomide gave complete and durable responses in more than 80% of the mice.

  9. Nonmetastatic Ewing's Sarcoma of the Lumbar Spine in an Adult Patient

    Science.gov (United States)

    Iacoangeli, Maurizio; Dobran, Mauro; Di Rienzo, Alessandro; di Somma, Lucia Giovanna Maria; Alvaro, Lorenzo; Moriconi, Elisa; Nocchi, Niccolò; Gladi, Maurizio; Scerrati, Massimo

    2012-01-01

    Although the spine is frequently involved in metastatic Ewing's sarcoma, primary involvement of the spine, beside sacrum, is much less frequent, especially in adult patients. Because of the low incidence of these tumors, there are currently no clinical guidelines outlining their management and a multitude of therapeutic strategies have been employed with varying success. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include the combination of three main modalities: aggressive surgery, radiotherapy, and combined chemotherapy. Whenever possible, en bloc spondylectomy or extralesional resection is preferable, providing a better oncological result with a longer survival and a better preservation of the spine biomechanics. This is the lesson we learned about the case, we present here, of nonmetastatic lumbar localization by Ewing's sarcoma in as adult patient. PMID:23133768

  10. Primary Ewing's sarcoma of the greater wing of the sphenoid bone.

    Science.gov (United States)

    Sharma, R R; Netalkar, A; Lad, S D

    2000-02-01

    Primary Ewing's sarcoma is an uncommon lethal tumour of the long bones and pelvic girdle mainly affecting children and young adults. An origin in the cranial bones is extremely rare. We report a unique case of primary involvement of the greater wing of sphenoid bone in a 16-year-old patient. Aggressive management using microsurgical resection, radiotherapy and chemotherapy was curative. Localized, primary Ewing's sarcoma of the cranial bones should be considered as a distinct clinicopathological entity with an extremely low rate of dural penetration and metastases, and with a relatively better prognosis as compared with those of long bones and pelvic girdle. In neurosurgical practice, primary Ewing's sarcoma of the cranial bones requires early aggressive management to achieve adequate long-term prognosis and cure.

  11. Exploring the surfaceome of Ewing sarcoma identifies a new and unique therapeutic target

    Science.gov (United States)

    Town, Jennifer; Pais, Helio; Harrison, Sally; Bataille, Carole; Bunjobpol, Wilawan; Zhang, Jing; Rabbitts, Terence H.

    2016-01-01

    The cell surface proteome of tumors mediates the interface between the transformed cells and the general microenvironment, including interactions with stromal cells in the tumor niche and immune cells such as T cells. In addition, the cell surface proteome of individual cancers defines biomarkers for that tumor type and potential proteins that can be the target of antibody-mediated therapy. We have used next-generation deep RNA sequencing (RNA-seq) coupled to an in-house database of genes encoding cell surface proteins (herein referred to as the surfaceome) as a tool to define a cell surface proteome of Ewing sarcoma compared with progenitor mesenchymal stem cells. This subtractive RNA-seq analysis revealed a specific surfaceome of Ewing and showed unexpectedly that the leucine-rich repeat and Ig domain protein 1 (LINGO1) is expressed in over 90% of Ewing sarcoma tumors, but not expressed in any other somatic tissue apart from the brain. We found that the LINGO1 protein acts as a gateway protein internalizing into the tumor cells when engaged by antibody and can carry antibody conjugated with drugs to kill Ewing sarcoma cells. Therefore, LINGO1 is a new, unique, and specific biomarker and drug target for the treatment of Ewing sarcoma. PMID:26979953

  12. 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

  13. 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.

  14. Camptothecin-Based Regimens for Treatment of Ewing Sarcoma: sPast Studies and Future Directions

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    Lars Wagner

    2011-01-01

    Full Text Available New therapies are needed to improve survival for patients with Ewing sarcoma. Over the past decade, camptothecin agents such as topotecan and irinotecan have demonstrated activity against Ewing sarcoma, especially in combination with alkylating agents. Previous studies have shown camptothecin-based combinations to be tolerable outpatient strategies that are attractive for salvage therapy. This paper highlights important issues related to drug dosing, schedule of administration, pharmacokinetics, toxicity, and activity of commonly used camptothecin-based regimens. Also discussed are strategies for incorporating these regimens into therapy for newly diagnosed patients, including several potential possibilities for combination with targeted agents.

  15. Growth-promoting role of the miR-106a~363 cluster in Ewing sarcoma.

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    Layne Dylla

    Full Text Available MicroRNAs (miRs have been identified as potent regulators of both normal development and the hallmarks of cancer. Targeting of microRNAs has been shown to have preclinical promise, and select miR-based therapies are now in clinical trials. Ewing Sarcoma is a biologically aggressive pediatric cancer with little change in clinical outcomes despite improved chemotherapeutic regimens. There is a substantial need for new therapies to improve Ewing Sarcoma outcomes and to prevent chemotherapy-related secondary sequelae. Most Ewing Sarcoma tumors are driven by the EWS/Fli-1 fusion oncoprotein, acting as a gain-of-function transcription factor causing dysregulation of a variety of targets, including microRNAs. Our previous studies, and those of others, have identified upregulation of miRs belonging to the related miR-17~92a, miR-106b~25, and miR-106a~363 clusters in Ewing Sarcoma. However, the functional consequences of this have not been characterized, nor has miR blockade been explored as an anti-cancer strategy in Ewing Sarcoma. To simulate a potential therapeutic approach, we examined the effects of blockade of these clusters, and their component miRs. Using colony formation as a read-out, we find that blockade of selected individual cluster component miRs, using specific inhibitors, has little or no effect. Combinatorial inhibition using miR "sponge" methodology, on the other hand, is inhibitory to colony formation, with blockade of whole clusters generally more effective than blockade of miR families. We show that a miR-blocking sponge directed against the poorly characterized miR-106a~363 cluster is a particularly potent inhibitor of clonogenic growth in a subset of Ewing Sarcoma cell lines. We further identify upregulation of miR-15a as a downstream mechanism contributing to the miR-106a~363 sponge growth-inhibitory effect. Taken together, our studies provide support for a pro-oncogenic role of the miR-106a~363 cluster in Ewing Sarcoma, and

  16. Ewings sarcoma of 4th metacarpal bone of right hand, a rare case report

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    Basharat Mubeen

    2014-05-01

    Full Text Available Ewings sarcoma of the hand is relatively rare. Ewings sarcoma can present with minimal pain and swelling of the affected digit. The Erythrocyte sedimentation rate and C-reactive protein may be high. Because of these features, this can be confused with an inflammatory lesion. This leads to starting of inappropriate treatment and delay in diagnosis. Radiologically, Ewings sarcoma can present with a plethora of features from permeative bone destruction to expansile lesions with or without periosteal reaction. We present a case of 10 year old male, with complaints of pain and swelling in right hand, which on X-ray showed periosteal reaction, giving a sun burst appearance. So the provisional diagnosis of osteosarcoma was made. The diagnosis of ewings sarcoma was made on FNAC [fine needle aspiration cytology] which was later on confirmed on histopathology. We intend to report this case, because it is very rare location and the radiological features can mimic other lesions which commonly occur in this location like chronic osteomyelitis, osteosarcoma so it can be easily missed especially at preliminary evaluations.

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

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    J. Thariat

    2008-01-01

    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.

  18. Promoter Methylation Analysis Reveals that KCNA5 Ion Channel Silencing Supports Ewing Sarcoma Cell Proliferation

    Science.gov (United States)

    Ryland, Katherine E; Hawkins, Allegra G.; Weisenberger, Daniel J.; Punj, Vasu; Borinstein, Scott C.; Laird, Peter W.; Martens, Jeffrey R.; Lawlor, Elizabeth R.

    2015-01-01

    Polycomb proteins are essential regulators of gene expression in stem cells and development. They function to reversibly repress gene transcription via post-translational modification of histones and chromatin compaction. In many human cancers, genes that are repressed by polycomb in stem cells are subject to more stable silencing via DNA methylation of promoter CpG islands. Ewing sarcoma is an aggressive bone and soft tissue tumor that is characterized by over-expression of polycomb proteins. This study investigates the DNA methylation status of polycomb target gene promoters in Ewing sarcoma tumors and cell lines and observes that the promoters of differentiation genes are frequent targets of CpG-island DNA methylation. In addition, the promoters of ion channel genes are highly differentially methylated in Ewing sarcoma compared to non-malignant adult tissues. Ion channels regulate a variety of biological processes, including proliferation, and dysfunction of these channels contributes to tumor pathogenesis. In particular, reduced expression of the voltage-gated Kv1.5 channel has been implicated in tumor progression. These data show that DNA methylation of the KCNA5 promoter contributes to stable epigenetic silencing of Kv1.5 channel. This epigenetic repression is reversed by exposure to the DNA methylation inhibitor decitabine, which inhibits Ewing sarcoma cell proliferation through mechanisms that include restoration of Kv1.5 channel function. Implications This study demonstrates that promoters of ion channels are aberrantly methylated in Ewing sarcoma and that epigenetic silencing of KCNA5 contributes to tumor cell proliferation, thus providing further evidence of the importance of ion channel dyregulation to tumorigenesis. PMID:26573141

  19. Ewing sarcoma EWS protein regulates midzone formation by recruiting Aurora B kinase to the midzone.

    Science.gov (United States)

    Park, Hyewon; Turkalo, Timothy K; Nelson, Kayla; Folmsbee, Stephen Sai; Robb, Caroline; Roper, Brittany; Azuma, Mizuki

    2014-01-01

    Ewing sarcoma is a malignant bone cancer that primarily occurs in children and adolescents. Eighty-five percent of Ewing sarcoma is characterized by the presence of the aberrant chimeric EWS/FLI1 fusion gene. Previously, we demonstrated that an interaction between EWS/FLI1 and wild-type EWS led to the inhibition of EWS activity and mitotic dysfunction. Although defective mitosis is considered to be a critical step in cancer initiation, it is unknown how interference with EWS contributes to Ewing sarcoma formation. Here, we demonstrate that EWS/FLI1- and EWS-knockdown cells display a high incidence of defects in the midzone, a midline structure located between segregating chromatids during anaphase. Defects in the midzone can lead to the failure of cytokinesis and can result in the induction of aneuploidy. The similarity among the phenotypes of EWS/FLI1- and EWS siRNA-transfected HeLa cells points to the inhibition of EWS as the key mechanism for the induction of midzone defects. Supporting this observation, the ectopic expression of EWS rescues the high incidence of midzone defects observed in Ewing sarcoma A673 cells. We discovered that EWS interacts with Aurora B kinase, and that EWS is also required for recruiting Aurora B to the midzone. A domain analysis revealed that the R565 in the RGG3 domain of EWS is essential for both Aurora B interaction and the recruitment of Aurora B to the midzone. Here, we propose that the impairment of EWS-dependent midzone formation via the recruitment of Aurora B is a potential mechanism of Ewing sarcoma development.

  20. Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: A Rare and Lethal Entity.

    Science.gov (United States)

    Celli, Romulo; Cai, Guoping

    2016-03-01

    Ewing sarcoma/primitive neuroectodermal tumor represents a spectrum of undifferentiated tumors with similar biology that together represent the second most common sarcoma in the pediatric-young adult age range. Very rarely, this tumor presents as a primary neoplasm of the kidney. The clinical presentation of this tumor is not specific, and other renal tumors may present with a similar histologic appearance. Establishing the correct diagnosis is critical because renal Ewing sarcoma/primitive neuroectodermal tumor carries a strikingly dismal prognosis and thus dictates a specific treatment strategy. A low threshold for the use of ancillary molecular tests is recommended, particularly in diagnostically problematic cases. Important considerations with regards to morphology, immunohistochemistry, and molecular alterations will be reviewed here and should be taken into account before rendering this rare and lethal diagnosis.

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

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    Trigg, M.E.; Makuch, R.; Glaubiger, D.

    1985-04-01

    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.

  2. Expression of neural cell adhesion molecules and neurofilament protein isoforms in Ewing's sarcoma of bone and soft tissue sarcomas of other than rhabdomyosarcoma

    NARCIS (Netherlands)

    Molenaar, W.M.; Muntinghe, F.L.H.

    1999-01-01

    In a previous study, it was shown that rhabdomyosarcomas widely express "neural" markers, such as neural cell adhesion molecules (N-CAM) and neurofilament protein isoforms, In the current study, a series of Ewing's sarcomas of bone and soft tissue sarcomas other than rhabdomyosarcoma was probed for

  3. Ewing sarcoma cells secrete EWS/Fli-1 fusion mRNA via microvesicles.

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    Masanori Tsugita

    Full Text Available Tumours defined as Ewing sarcoma (ES constitute a group of highly malignant neoplasms that most often affect children and young adults in the first 2 decades of life. The EWS/Fli-1 fusion gene, a product of the translocation t(11;22 (q24; 12, is detected in 95% of ES patients. Recently, it was validated that cells emit a heterogeneous mixture of vesicular, organelle-like structures (microvesicles, MVs into their surroundings including blood and body fluids, and that these MVs contain a selected set of tumor-related proteins and high levels of mRNAs and miRNAs. In this present study, we detected the Ewing sarcoma-specific EWS/Fli-1 mRNA in MVs from the culture medium of ES cell lines carrying t(11;22 (q24; 12. Also, we detected this fusion gene in approximately 40% of the blood samples from mice inoculated with xenografts of TC135 or A673 cells. These findings indicate the EWS/Fli-1 mRNA in MVs might be a new non-invasive diagnostic marker for specific cases of Ewing sarcoma.

  4. Massive Pleural Effusion in an 18-Year-Old Girl with Ewing Sarcoma

    Directory of Open Access Journals (Sweden)

    Cengiz Özge

    2004-01-01

    Full Text Available Ewing sarcoma is a bone tumour that commonly appears between ages five and 10 in the diaphysis of the long bones and predominantly presents with pain and swelling. The case of an 18-year-old girl who presented with back pain, cough, dyspnea, weakness and fever is described. Chest radiograph showed a homogenous density in the middle and inferior zones of the right hemithorax. Thoracic computed tomography revealed a diffuse pleural effusion and a 6.99 cmx4.45 cm solid mass composed of lobulated, small cystic lesions and calcifications in the right hemithorax. Biochemical analysis of pleural fluid showed hemorrhagic effusion and exudate. A pleural needle biopsy demonstrated solid uniform tumour cells with narrowed cytoplasm, round nuclei and uncertain nucleoli. All of the tumour cell cytoplasms stained with CD99. The pathological examination supported Ewing sarcoma. Three-phase Tc-99m methylene diphosphonate scintigraphy of the whole body showed pathological tracer uptake in a broad area of the eighth costal bone and in smaller areas of the ninth and 10th costal bones. This case is reported because Ewing sarcoma is a rare cause of pleural effusion in clinical practice among younger adults.

  5. Preoperative evaluation and monitoring chemotherapy in patients with high-grade osteogenic and Ewing`s sarcoma: review of current imaging modalities

    Energy Technology Data Exchange (ETDEWEB)

    Woude, H.-J. van der; Bloem, J.L. [Department of Radiology, Leiden University Medical Centre, C2-S, P.O. Box 9600, NL-2300 RC Leiden (Netherlands); Hogendoorn, P.C.W. [Department of Pathology, Leiden University Medical Centre, Leiden (Netherlands)

    1998-02-01

    Diagnostic imaging is pivotal in the initial detection, characterization, staging and post-treatment follow-up of patients with high-grade osteogenic and Ewing`s sarcoma. In the present review article, conventional and new imaging modalities are discussed with regard to the monitoring of the effect of neoadjuvant chemotherapy in such patients. Presurgical monitoring of response to chemotherapy may have an impact on modification of neoadjuvant treatment protocols, on patient selection for the performance and timing of limb-salvage surgery and on planning of radiation therapy (in non-operated Ewing`s sarcomas) and selection of postoperative chemotherapy regimens. Dynamic contrast-enhanced MR imaging, as part of a routine MR protocol, assists in the detection of the most viable parts of the tumour and serves as an initial standard for follow-up of the metabolic activity of the tumour during and after chemotherapy, both in small intraosseous tumours and in tumours with an associated soft tissue mass. In combination with selected morphological features, dynamic imaging parameters are therefore advocated for monitoring the effect of neoadjuvant chemotherapy in patients with osteogenic and Ewing`s sarcoma. (orig.) With 9 figs., 2 tabs., 62 refs.

  6. 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.

  7. An Interaction with Ewing's Sarcoma Breakpoint Protein EWS Defines a Specific Oncogenic Mechanism of ETS Factors Rearranged in Prostate Cancer.

    Science.gov (United States)

    Kedage, Vivekananda; Selvaraj, Nagarathinam; Nicholas, Taylor R; Budka, Justin A; Plotnik, Joshua P; Jerde, Travis J; Hollenhorst, Peter C

    2016-10-25

    More than 50% of prostate tumors have a chromosomal rearrangement resulting in aberrant expression of an oncogenic ETS family transcription factor. However, mechanisms that differentiate the function of oncogenic ETS factors expressed in prostate tumors from non-oncogenic ETS factors expressed in normal prostate are unknown. Here, we find that four oncogenic ETS (ERG, ETV1, ETV4, and ETV5), and no other ETS, interact with the Ewing's sarcoma breakpoint protein, EWS. This EWS interaction was necessary and sufficient for oncogenic ETS functions including gene activation, cell migration, clonogenic survival, and transformation. Significantly, the EWS interacting region of ERG has no homology with that of ETV1, ETV4, and ETV5. Therefore, this finding may explain how divergent ETS factors have a common oncogenic function. Strikingly, EWS is fused to various ETS factors by the chromosome translocations that cause Ewing's sarcoma. Therefore, these findings link oncogenic ETS function in both prostate cancer and Ewing's sarcoma.

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

    Energy Technology Data Exchange (ETDEWEB)

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

    2010-09-03

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

  9. 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.

  10. Ewing sarcoma ewsa protein regulates chondrogenesis of Meckel's cartilage through modulation of Sox9 in zebrafish.

    Directory of Open Access Journals (Sweden)

    Chris Merkes

    Full Text Available Ewing sarcoma is the second most common skeletal (bone and cartilage cancer in adolescents, and it is characterized by the expression of the aberrant chimeric fusion gene EWS/FLI1. Wild-type EWS has been proposed to play a role in mitosis, splicing and transcription. We have previously shown that EWS/FLI1 interacts with EWS, and it inhibits EWS activity in a dominant manner. Ewing sarcoma is a cancer that specifically develops in skeletal tissues, and although the above data suggests the significance of EWS, its role in chondrogenesis/skeletogenesis is not understood. To elucidate the function of EWS in skeletal development, we generated and analyzed a maternal zygotic (MZ ewsa/ewsa line because the ewsa/wt and ewsa/ewsa zebrafish appeared to be normal and fertile. Compared with wt/wt, the Meckel's cartilage of MZ ewsa/ewsa mutants had a higher number of craniofacial prehypertrophic chondrocytes that failed to mature into hypertrophic chondrocytes at 4 days post-fertilization (dpf. Ewsa interacted with Sox9, which is the master transcription factor for chondrogenesis. Sox9 target genes were either upregulated (ctgfa, ctgfb, col2a1a, and col2a1b or downregulated (sox5, nog1, nog2, and bmp4 in MZ ewsa/ewsa embryos compared with the wt/wt zebrafish embryos. Among these Sox9 target genes, the chromatin immunoprecipitation (ChIP experiment demonstrated that Ewsa directly binds to ctgfa and ctgfb loci. Consistently, immunohistochemistry showed that the Ctgf protein is upregulated in the Meckel's cartilage of MZ ewsa/ewsa mutants. Together, we propose that Ewsa promotes the differentiation from prehypertrophic chondrocytes to hypertrophic chondrocytes of Meckel's cartilage through inhibiting Sox9 binding site of the ctgf gene promoter. Because Ewing sarcoma specifically develops in skeletal tissue that is originating from chondrocytes, this new role of EWS may provide a potential molecular basis of its pathogenesis.

  11. Lurbinectedin Inactivates the Ewing Sarcoma Oncoprotein EWS-FLI1 by Redistributing It within the Nucleus.

    Science.gov (United States)

    Harlow, Matt L; Maloney, Nichole; Roland, Joseph; Guillen Navarro, Maria Jose; Easton, Matthew K; Kitchen-Goosen, Susan M; Boguslawski, Elissa A; Madaj, Zachary B; Johnson, Ben K; Bowman, Megan J; D'Incalci, Maurizio; Winn, Mary E; Turner, Lisa; Hostetter, Galen; Galmarini, Carlos María; Aviles, Pablo M; Grohar, Patrick J

    2016-11-15

    There is a great need to develop novel approaches to target oncogenic transcription factors with small molecules. Ewing sarcoma is emblematic of this need, as it depends on the continued activity of the EWS-FLI1 transcription factor to maintain the malignant phenotype. We have previously shown that the small molecule trabectedin interferes with EWS-FLI1. Here, we report important mechanistic advances and a second-generation inhibitor to provide insight into the therapeutic targeting of EWS-FLI1. We discovered that trabectedin functionally inactivated EWS-FLI1 by redistributing the protein within the nucleus to the nucleolus. This effect was rooted in the wild-type functions of the EWSR1, compromising the N-terminal half of the chimeric oncoprotein, which is known to be similarly redistributed within the nucleus in the presence of UV light damage. A second-generation trabectedin analogue lurbinectedin (PM01183) caused the same nuclear redistribution of EWS-FLI1, leading to a loss of activity at the promoter, mRNA, and protein levels of expression. Tumor xenograft studies confirmed this effect, and it was increased in combination with irinotecan, leading to tumor regression and replacement of Ewing sarcoma cells with benign fat cells. The net result of combined lurbinectedin and irinotecan treatment was a complete reversal of EWS-FLI1 activity and elimination of established tumors in 30% to 70% of mice after only 11 days of therapy. Our results illustrate the preclinical safety and efficacy of a disease-specific therapy targeting the central oncogenic driver in Ewing sarcoma. Cancer Res; 76(22); 6657-68. ©2016 AACR.

  12. The role of FLI-1-EWS, a fusion gene reciprocal to EWS-FLI-1, in Ewing sarcoma

    OpenAIRE

    Elzi, David J.; Song, Meihua; Houghton, Peter J.; Chen, Yidong; Shiio, Yuzuru

    2015-01-01

    Ewing sarcoma is a cancer of bone and soft tissue in children that is characterized by a chromosomal translocation involving EWS and an Ets family transcription factor, most commonly FLI-1. The EWS-FLI-1 fusion oncogene is widely believed to play a central role in Ewing sarcoma. The EWS-FLI-1 gene product regulates the expression of a number of genes important for cancer progression, can transform mouse cells such as NIH3T3 and C3H10T1/2, and is necessary for proliferation and tumorigenicity ...

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

    Energy Technology Data Exchange (ETDEWEB)

    Sundaram, Murali [Mayo Clinic, Department of Radiology, Rochester, Minnesota (United States); Cleveland Clinic, Diagnostic Radiology, Cleveland (United States); Inwards, Carrie Y. [Mayo Clinic, Department of Surgical Pathology, Rochester, Minnesota (United States); Shives, Thomas E. [Mayo Clinic, Department of Orthopedic Surgery, Rochester, Minnesota (United States); Anderson, Peter M. [Mayo Clinic, Department of Pediatric and Adolescent Medicine and Internal Medicine, Rochester, Minnesota (United States)

    2005-05-01

    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.)

  14. 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.

  15. 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...

  16. Prostatic sarcoma of the Ewing family in a 33-year-old male – A case report and review of the literature

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    Lukas Esch

    2016-04-01

    Full Text Available Ewing sarcoma is the second most common primary bone tumor seen in children and adolescents, typically presenting between 10 and 20 years of age. Extraosseous sarcomas of the Ewing family in adults are rare. We report a manifestation of this tumor entity in the periprostatic tissue of a 33-year-old male and discuss our treatment approach. Transrectal biopsy is a feasible and simple diagnostic tool for unclear pelvic masses. Multi-modal therapy and central registries are needed to gain knowledge of rare pelvic tumors like Ewing sarcoma.

  17. 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.

  18. 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.

  19. 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.)

  20. Aggressive Ewing's sarcoma appearing as a cold lesion on bone scan; Sarcome d'Ewing agressif apparaissant comme une lesion froide sur la scintigraphie osseuse

    Energy Technology Data Exchange (ETDEWEB)

    Chatti, K.; Guezguez, M.; Maha Ben Fredj, M.; Sfar, R.; Essabbah, H. [Hopital Universitaire de Sahloul, Dept. de Medecine Nucleaire, Sahloul (Tunisia); Mtaoumi, M. [Hopital Universitaire de Sahloul, Dept. d' Orthopedie, Sousse (Tunisia); Chatti, K. [Faculte de Medecine de Monastir, Lab. de Biophysique, Monastir (Tunisia)

    2009-10-15

    Ewing's sarcoma classically presents as a hot spot on bone scan as a result of increased vascularity of the tumor and new bone formation. Purpose We report and analyze an uncommon pattern of a 'cold' lesion in Ewing's sarcoma on bone scan and its pathophysiologic significance. Case report A 15-year-old boy complaining of thigh pain. CT scan evoked Ewing's sarcoma or osteitis. MRI evoked chronic osteitis. Scintigraphy showed a fairly intense and heterogeneous uptake on the femoral lesion and no abnormal uptake elsewhere. Biopsy showed none pathologic pattern. Three months later, a second biopsy concluded to Ewing's sarcoma. Bone scan showed a larger lesion with peripheral intense uptake centered by enlarged 'cold' area in the left femoral diaphysis and no evident bone metastasis. The patient underwent chemotherapy and surgery. Three months later, bone scan showed extensive skeletal metastasis. Conclusion Ewing's sarcoma appears usually as an intense lesion on bone scan. Nevertheless, decreased radiopharmaceutical uptake or 'cold' lesion may be seen in aggressive Ewing's sarcoma with lytic tumor, growth of which is very rapid and bony reaction is minimal. (authors)

  1. Ewing sarcoma family of tumors: a model for the new era of integrated laboratory diagnostics.

    Science.gov (United States)

    Khoury, Joseph D

    2008-01-01

    The Ewing sarcoma family of tumors (ESFT) represents one of the best models illustrating the multifaceted approach to the diagnosis of cancer that has evolved over the past decade. ESFT encompasses tumors that arise in bone or soft tissues and may have disparate histologic features. As a result, it was not until the discovery that these tumors share a common underlying molecular pathogenesis (chromosomal translocations involving the EWS gene and one of several members of the ETS family of transcription factors) that significant advances in the diagnosis and therapy of ESFT became possible. As a result, ESFT has come to embody the amalgamation of classical diagnostic tools, such as histology and routine microscopy, with newer techniques, such as immunohistochemistry and molecular techniques; the latter include PCR-based methods and fluorescence in situ hybridization. This review will address the features of ESFT and how it has emerged as a model for the new era of integrated diagnostics.

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

    Directory of Open Access Journals (Sweden)

    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.

  3. Antagonism pattern detection between microRNA and target expression in Ewing's sarcoma.

    Directory of Open Access Journals (Sweden)

    Loredana Martignetti

    Full Text Available MicroRNAs (miRNAs have emerged as fundamental regulators that silence gene expression at the post-transcriptional and translational levels. The identification of their targets is a major challenge to elucidate the regulated biological processes. The overall effect of miRNA is reflected on target mRNA expression, suggesting the design of new investigative methods based on high-throughput experimental data such as miRNA and transcriptome profiles. We propose a novel statistical measure of non-linear dependence between miRNA and mRNA expression, in order to infer miRNA-target interactions. This approach, which we name antagonism pattern detection, is based on the statistical recognition of a triangular-shaped pattern in miRNA-target expression profiles. This pattern is observed in miRNA-target expression measurements since their simultaneously elevated expression is statistically under-represented in the case of miRNA silencing effect. The proposed method enables miRNA target prediction to strongly rely on cellular context and physiological conditions reflected by expression data. The procedure has been assessed on synthetic datasets and tested on a set of real positive controls. Then it has been applied to analyze expression data from Ewing's sarcoma patients. The antagonism relationship is evaluated as a good indicator of real miRNA-target biological interaction. The predicted targets are consistently enriched for miRNA binding site motifs in their 3'UTR. Moreover, we reveal sets of predicted targets for each miRNA sharing important biological function. The procedure allows us to infer crucial miRNA regulators and their potential targets in Ewing's sarcoma disease. It can be considered as a valid statistical approach to discover new insights in the miRNA regulatory mechanisms.

  4. Xenograft and genetically engineered mouse model systems of osteosarcoma and Ewing's sarcoma: tumor models for cancer drug discovery

    Science.gov (United States)

    Sampson, Valerie B; Kamara, Davida F; Kolb, E Anders

    2014-01-01

    Introduction There are > 75 histological types of solid tumors that are classified into two major groups: bone and soft-tissue sarcomas. These diseases are more prevalent in children, and pediatric sarcomas tend to be highly aggressive and rapidly progressive. Sarcomas in adults may follow a more indolent course, but aggressive tumors are also common. Sarcomas that are metastatic at diagnosis, or recurrent following therapy, remain refractory to current treatment options with dismal overall survival rates. A major focus of clinical trials, for patients with sarcoma, is to identify novel and more effective therapeutic strategies targeted to genomic or proteomic aberrations specific to the malignant cells. Critical to the understanding of the potential for targeted therapies are models of disease that are representative of clinical disease and predictive of relevant clinical responses. Areas covered In this article, the authors discuss the use of mouse xenograft models and genetically engineered mice in cancer drug discovery. The authors provide a special focus on models for the two most common bone sarcomas: osteosarcoma (OS) and Ewing's sarcoma (ES). Expert opinion Predicting whether a new anticancer agent will have a positive therapeutic index in patients with OS and ES remains a challenge. The use of mouse sarcoma models for understanding the mechanisms involved in the response of tumors to new treatments is an important step in the process of drug discovery and the development of clinically relevant therapeutic strategies for these diseases. PMID:23844615

  5. Renal artery stenosis after radiotherapy for Ewing's sarcoma; Radiotherapieinduzierte Nierenarterienstenose nach Behandlung eines Ewing-Sarkoms

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    Tacconi, S.; Bieri, S. [Abt. fuer Radioonkologie, Centre Hospitalier du Centre du Valais (CHCVs), Hopital de Sion (Switzerland)

    2008-09-15

    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.)

  6. Epigenetic reprogramming and re-differentiation of a Ewing sarcoma cell line

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    Joseph Brady Moore IV

    2015-03-01

    Full Text Available Developmental reprogramming techniques have been used to generate induced pluripotent stem (iPS cells from both normal and malignant cells. The derivation of iPS cells from cancer has the potential to provide a unique scientific tool to overcome challenges associated with the establishment of cell lines from primary patient samples and a readily expandable source of cells that may be used to model the initial disease. In the current study we developmentally reprogrammed a metastatic Ewing sarcoma (EWS cell line to a meta-stable embryonic stem (ES-like state sharing molecular and phenotypic features with previously established ES and iPS cell lines. EWS-iPS cells exhibited a pronounced drug resistant phenotype despite persistent expression of the oncogenic EWS-FLI1 fusion transcript. This included resistance to compounds that specifically target downstream effector pathways of EWS-FLI1, such as MAPK/ERK and PI3K/AKT, which play an important role in EWS pathogenesis. EWS-iPS cells displayed tumor initiation abilities in vivo and formed tumors exhibiting characteristic Ewing histopathology. In parallel, EWS-iPS cells re-differentiated in vitro recovered sensitivity to molecularly targeted chemotherapeutic agents, which reiterated pathophysiological features of the cells from which they were derived. These data suggest that EWS-iPS cells may provide an expandable disease model that could be used to investigate processes modulating oncogenesis, metastasis, and chemotherapeutic resistance in EWS.

  7. Pre-clinical efficacy of PU-H71, a novel HSP90 inhibitor, alone and in combination with bortezomib in Ewing sarcoma.

    Science.gov (United States)

    Ambati, Srikanth R; Lopes, Eloisi Caldas; Kosugi, Kohji; Mony, Ullas; Zehir, Ahmet; Shah, Smit K; Taldone, Tony; Moreira, Andre L; Meyers, Paul A; Chiosis, Gabriela; Moore, Malcolm A S

    2014-03-01

    Ewing sarcoma is characterized by multiple deregulated pathways that mediate cell survival and proliferation. Heat shock protein 90 (HSP90) is a critical component of the multi-chaperone complexes that regulate the disposition and activity of a large number of proteins involved in cell-signaling systems. We tested the efficacy of PU-H71, a novel HSP90 inhibitor in Ewing sarcoma cell lines, primary samples, benign mesenchymal stromal cells and hematopoietic stem cells. We performed cell cycle analysis, clonogenic assay, immunoblot analysis and reverse phase protein array in Ewing cell lines and in vivo experiments in NSG and nude mice using the A673 cell line. We noted a significant therapeutic window in the activity of PU-H71 against Ewing cell lines and benign cells. PU-H71 treatment resulted in G2/M phase arrest. Exposure to PU-H71 resulted in depletion of critical proteins including AKT, pERK, RAF-1, c-MYC, c-KIT, IGF1R, hTERT and EWS-FLI1 in Ewing cell lines. Our results indicated that Ewing sarcoma tumor growth and the metastatic burden were significantly reduced in the mice injected with PU-H71 compared to the control mice. We also investigated the effects of bortezomib, a proteasome inhibitor, alone and in combination with PU-H71 in Ewing sarcoma. Combination index (CI)-Fa plots and normalized isobolograms indicated synergism between PU-H71 and bortezomib. Ewing sarcoma xenografts were significantly inhibited when mice were treated with the combination compared to vehicle or either drug alone. This provides a strong rationale for clinical evaluation of PU-H71 alone and in combination with bortezomib in Ewing sarcoma.

  8. EWS/FLI-l peptide-pulsed dendritic cells induces the antitumor immunity in a murine Ewing's sarcoma cell model.

    Science.gov (United States)

    Peng, Wei; Huang, Xunwu; Yang, Dazhi

    2014-08-01

    An increasing number of T-cell epitopes derived from various tumor-associated antigens have been reported, and they proved to play significant roles for tumor rejection both in vivo and in vitro. Over 85% of Ewing's sarcoma family of tumors (ESFTs) express tumor-specific chimeric protein EWS/FLI-1, making it an attractive target for therapeutic cytotoxic T-lymphocyte responses. Here, we identified a novel peptide epitope derived from the EWS/FLI-1 protein and demonstrated that effectors induced by the peptide could specifically secrete IFN-γ and lyse the tumor cell line of EWS/FLI-1-positive and HLA-matched cells. In addition, mice treated with dendritic cells pulsed with the EWS/FLI-1 epitope were able to reject a lethal tumor inoculation of the Ewing's sarcoma A673 cells. Therefore, these data provide evidence for the use of the EWS/FLI-l peptide epitope in T cell-based immunotherapeutic concepts against Ewing's sarcoma cell in vitro and in vivo.

  9. Chimeric EWSR1-FLI1 regulates the Ewing sarcoma susceptibility gene EGR2 via a GGAA microsatellite

    Science.gov (United States)

    Grünewald, Thomas G. P.; Bernard, Virginie; Gilardi-Hebenstreit, Pascale; Raynal, Virginie; Surdez, Didier; Aynaud, Marie-Ming; Mirabeau, Olivier; Cidre-Aranaz, Florencia; Tirode, Franck; Zaidi, Sakina; Perot, Gaëlle; Jonker, Anneliene H.; Lucchesi, Carlo; Le Deley, Marie-Cécile; Oberlin, Odile; Marec-Bérard, Perrine; Véron, Amélie S.; Reynaud, Stephanie; Lapouble, Eve; Boeva, Valentina; Frio, Thomas Rio; Alonso, Javier; Bhatia, Smita; Pierron, Gaëlle; Cancel-Tassin, Geraldine; Cussenot, Olivier; Cox, David G.; Morton, Lindsay M.; Machiela, Mitchell J.; Chanock, Stephen J.; Charnay, Patrick; Delattre, Olivier

    2015-01-01

    Deciphering the ways in which somatic mutations and germline susceptibility variants cooperate to promote cancer is challenging. Ewing sarcoma is characterized by fusions between EWSR1 and members of the ETS gene family, usually EWSR1-FLI1, leading to the generation of oncogenic transcription factors that bind DNA at GGAA motifs1–3. A recent genome-wide association study4 identified susceptibility variants near EGR2. Here we found that EGR2 knockdown inhibited proliferation, clonogenicity and spheroidal growth in vitro and induced regression of Ewing sarcoma xenografts. Targeted germline deep sequencing of the EGR2 locus in affected subjects and controls revealed 291 Ewing-associated SNPs. At rs79965208, the A risk allele connected adjacent GGAA repeats by converting an interspaced GGAT motif into a GGAA motif, thereby increasing the number of consecutive GGAA motifs and thus the EWSR1-FLI1–dependent enhancer activity of this sequence, with epigenetic characteristics of an active regulatory element. EWSR1-FLI1 preferentially bound to the A risk allele, which increased global and allele-specific EGR2 expression. Collectively, our findings establish cooperation between a dominant oncogene and a susceptibility variant that regulates a major driver of Ewing sarcomagenesis. PMID:26214589

  10. 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

  11. 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

  12. Primary Ewing's Sarcoma of the Spine in a Two-Year-Old Boy

    Science.gov (United States)

    Electricwala, Jaffer T.

    2016-01-01

    Ewing's Sarcoma (ES) is a highly malignant bone tumour. It may involve any part of the skeleton but the most frequent parts are the ilium and diaphysis of femur and tibia (Alfeeli et al., 2005; Zhu et al., 2012). Primary ES of the spine is extremely rare (Yan et al., 2011). It accounts for only 3.5 to 14.9 percent of all primary bone sarcomas. The age of presentation ranges from 12 to 24 years (median 21 years) (Ferguson, 1999; Sharafuddin et al., 1992; Klimo Jr. et al., 2009). We report an unusual case of primary ES of the spine in a two-year-old boy, who presented to us with paraparesis and features of cauda equina syndrome. MRI scan showed a tumour mass arising from the pedicle of L4 vertebra invading the spinal canal. Tc-99 bone scan showed increased tracer uptake in L4 vertebra and normal tracer uptake elsewhere in the skeleton. After reaching the diagnosis of a space occupying lesion invading the lumber spinal canal, we performed a decompressive laminectomy and a biopsy was sent which confirmed the diagnosis of ES. Immunohistochemistry showed tumour cells staining positive for CD-99 (specific stain for ES). Gene testing showed an EWS-FLI 1 chimera. Surgery was followed by good improvement in motor signs. The child was then referred to a specialized oncotherapy centre for further treatment, radiation, and chemotherapy. To the best of our knowledge, we are the first to report primary ES of the spine at the age of two years. PMID:27895949

  13. Connexin 43 Is a Potential Prognostic Biomarker for Ewing Sarcoma/Primitive Neuroectodermal Tumor

    Directory of Open Access Journals (Sweden)

    Marilyn M. Bui

    2011-01-01

    Full Text Available Connexins (Cxs are building unit proteins of gap junctions (GJs that are prognostic markers in carcinomas. To investigate the role of Cx in Ewing sarcoma (EWS/primitive neuroectodermal tumor (PNET, we examined the expression of Cx43 and Cx26 in 36 EWS/PNETs and found (1 cytoplasmic Cx43 reactivity in 28/36 (78% cases. (2 Cx43 score was significantly correlated with overall survival (P=.025. The average scores for patients alive and dead at 3 years are 46.08 and 96.98 (P=.004 at 5 years are 46.06 and 96.42 (P=.002. (3 Metastasis had a significant effect on the overall survival (P=.003. (4 Cytoplasmic Cx26 reactivity was detected in 2 of 36 (6% patients who died with metastasis. Our results suggest a possible oncogenic and prognostic role for Cx43 and Cx26 in EWS/PNET. The lack of membranous immunoreactivity suggests that the effect of Cx in EWS/PNET is via a GJ function-independent mechanism.

  14. 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.

  15. Medication Exposures and Subsequent Development of Ewing Sarcoma: A Review of FDA Adverse Event Reports

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    Judith U. Cope

    2015-01-01

    Full Text Available Background. Ewing sarcoma family of tumors (ESFT are rare but deadly cancers of unknown etiology. Few risk factors have been identified. This study was undertaken to ascertain any possible association between exposure to therapeutic drugs and ESFT. Methods. This is a retrospective, descriptive study. A query of the FDA Adverse Event Reporting System (FAERS was conducted for all reports of ESFT, January 1, 1998, through December 31, 2013. Report narratives were individually reviewed for patient characteristics, underlying conditions and drug exposures. Results. Over 16 years, 134 ESFT reports were identified, including 25 cases of ESFT following therapeutic drugs and biologics including immunosuppressive agents and hormones. Many cases were confounded by concomitant medications and other therapies. Conclusions. This study provides a closer look at medication use and underlying disorders in patients who later developed ESFT. While this study was not designed to demonstrate any clear causative association between ESFT and prior use of a single product or drug class, many drugs were used to treat immune-related disease and growth or hormonal disturbances. Further studies may be warranted to better understand possible immune or neuroendocrine abnormalities or exposure to specific classes of drugs that may predispose to the later development of ESFT.

  16. Targeting the EWS-ETS transcriptional program by BET bromodomain inhibition in Ewing sarcoma.

    Science.gov (United States)

    Hensel, Tim; Giorgi, Chiara; Schmidt, Oxana; Calzada-Wack, Julia; Neff, Frauke; Buch, Thorsten; Niggli, Felix K; Schäfer, Beat W; Burdach, Stefan; Richter, Günther H S

    2016-01-12

    Ewing sarcomas (ES) are highly malignant bone or soft tissue tumors. Genetically, ES are defined by balanced chromosomal EWS/ETS translocations, which give rise to chimeric proteins (EWS-ETS) that generate an oncogenic transcriptional program associated with altered epigenetic marks throughout the genome. By use of an inhibitor (JQ1) blocking BET bromodomain binding proteins (BRDs) we strikingly observed a strong down-regulation of the predominant EWS-ETS protein EWS-FLI1 in a dose dependent manner. This was further enhanced by co-treatment with an inhibitor of the PI3K pathway. Microarray analysis further revealed JQ1 treatment to block a typical ES associated expression program. The effect on this expression program was mimicked by RNA interference with BRD3 or BRD4 expression, indicating that the EWS-FLI1 mediated expression profile is at least in part mediated via such epigenetic readers. Consequently, contact dependent and independent proliferation of different ES lines was strongly inhibited. Mechanistically, treatment of ES resulted in a partial arrest of the cell cycle as well as induction of apoptosis. Tumor development was suppressed dose dependently in a xeno-transplant model in immune deficient mice, overall indicating that ES may be susceptible to treatment with epigenetic inhibitors blocking BET bromodomain activity and the associated pathognomonic EWS-ETS transcriptional program.

  17. Localizing potentially active post-transcriptional regulations in the Ewing's sarcoma gene regulatory network

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    Delyon Bernard

    2010-11-01

    Full Text Available Abstract Background A wide range of techniques is now available for analyzing regulatory networks. Nonetheless, most of these techniques fail to interpret large-scale transcriptional data at the post-translational level. Results We address the question of using large-scale transcriptomic observation of a system perturbation to analyze a regulatory network which contained several types of interactions - transcriptional and post-translational. Our method consisted of post-processing the outputs of an open-source tool named BioQuali - an automatic constraint-based analysis mimicking biologist's local reasoning on a large scale. The post-processing relied on differences in the behavior of the transcriptional and post-translational levels in the network. As a case study, we analyzed a network representation of the genes and proteins controlled by an oncogene in the context of Ewing's sarcoma. The analysis allowed us to pinpoint active interactions specific to this cancer. We also identified the parts of the network which were incomplete and should be submitted for further investigation. Conclusions The proposed approach is effective for the qualitative analysis of cancer networks. It allows the integrative use of experimental data of various types in order to identify the specific information that should be considered a priority in the initial - and possibly very large - experimental dataset. Iteratively, new dataset can be introduced into the analysis to improve the network representation and make it more specific.

  18. 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

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    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

  19. Uncommon bone tumors of the skull: Ewing's sarcoma and Triton's tumor; Tumores osseos raros da calota craniana: sarcoma de Ewing e tumor de Triton

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    Rosa, Ana Claudia Ferreira; Carvalho, Claudio Sobral de [Hospital Sirio-Libanes, Sao Paulo, SP (Brazil). Dept. de Radiologia; Machado, Marcio Martins [Sao Paulo Univ., SP (Brazil). Faculdade de Medicina. Dept. de Radiologia; Figueiredo, Marco Antonio Junqueira; Albertotti, Cesar Jose [Hospital Sirio-Libanes, Sao Paulo, SP (Brazil). Servico de Tomografia Computadorizada e Ressonancia Magnetica; Cerri, Giovanni Guido [Hospital Sirio-Libanes, Sao Paulo, SP (Brazil). Centro de Diagnostico]. E-mail: giovanni.cerri@hcnet.usp.br

    2002-04-01

    Ewing's sarcoma and Triton's tumor are two uncommon bone tumors of the skull that have nonspecific clinical and imaging features. However, imaging methods are important in the detection of the lesions during the diagnostic investigation in order to evaluate the extent of the bone lesions, involvement of the soft tissues and brain, and to determine the presence of local recurrence and metastases. The confirmatory diagnosis relies on histological studies and immunohistochemistry. The authors report two cases of patients with these tumors and present the radiological findings. (author)

  20. Activation of Wnt/β-Catenin in Ewing Sarcoma Cells Antagonizes EWS/ETS Function and Promotes Phenotypic Transition to More Metastatic Cell States.

    Science.gov (United States)

    Pedersen, Elisabeth A; Menon, Rajasree; Bailey, Kelly M; Thomas, Dafydd G; Van Noord, Raelene A; Tran, Jenny; Wang, Hongwei; Qu, Ping Ping; Hoering, Antje; Fearon, Eric R; Chugh, Rashmi; Lawlor, Elizabeth R

    2016-09-01

    Ewing sarcomas are characterized by the presence of EWS/ETS fusion genes in the absence of other recurrent genetic alterations and mechanisms of tumor heterogeneity that contribute to disease progression remain unclear. Mutations in the Wnt/β-catenin pathway are rare in Ewing sarcoma but the Wnt pathway modulator LGR5 is often highly expressed, suggesting a potential role for the axis in tumor pathogenesis. We evaluated β-catenin and LGR5 expression in Ewing sarcoma cell lines and tumors and noted marked intra- and inter-tumor heterogeneity. Tumors with evidence of active Wnt/β-catenin signaling were associated with increased incidence of tumor relapse and worse overall survival. Paradoxically, RNA sequencing revealed a marked antagonism of EWS/ETS transcriptional activity in Wnt/β-catenin-activated tumor cells. Consistent with this, Wnt/β-catenin-activated cells displayed a phenotype that was reminiscent of Ewing sarcoma cells with partial EWS/ETS loss of function. Specifically, activation of Wnt/β-catenin induced alterations to the actin cytoskeleton, acquisition of a migratory phenotype, and upregulation of EWS/ETS-repressed genes. Notably, activation of Wnt/β-catenin signaling led to marked induction of tenascin C (TNC), an established promoter of cancer metastasis, and an EWS/ETS-repressed target gene. Loss of TNC function in Ewing sarcoma cells profoundly inhibited their migratory and metastatic potential. Our studies reveal that heterogeneous activation of Wnt/β-catenin signaling in subpopulations of tumor cells contributes to phenotypic heterogeneity and disease progression in Ewing sarcoma. Significantly, this is mediated, at least in part, by inhibition of EWS/ETS fusion protein function that results in derepression of metastasis-associated gene programs. Cancer Res; 76(17); 5040-53. ©2016 AACR.

  1. Development of a preclinical orthotopic xenograft model of ewing sarcoma and other human malignant bone disease using advanced in vivo imaging.

    Directory of Open Access Journals (Sweden)

    Britta Vormoor

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

  2. Cirugía de salvamento de extremidad en sarcoma de Ewing supervivencia, complicaciones y factores de riesgo

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    Álvarez San Nicolás, Jordi

    2015-01-01

    INTRODUCCIÓN:La introducción de pautas de quimioterapia supuso el aumento de la supervivencia en pacientes con sarcoma de Ewing (ES),con el consecuente desarrollo de las técnicas locales de tratamiento como la cirugía de salvamento de extremidad (LS).La supervivencia y eventos adversos han sido ampliamente estudiada en series generales de ES, así como las complicaciones locales en técnicas LS en series generales de tumores óseos. Sin embargo,no existen series exclusivas de ES tratados mediant...

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

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    Casey, Dana L.; Alektiar, Kaled M.; Gerber, Naamit K.; Wolden, Suzanne L., E-mail: woldens@mskcc.org

    2014-08-01

    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.

  4. 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.

  5. Ewing sarcoma/primitive neuroectodermal tumor of the kidney: clinicopathologic analysis of 34 cases.

    Science.gov (United States)

    Karpate, Arti; Menon, Santosh; Basak, Ranjan; Yuvaraja, Thyavihalli B; Tongaonkar, Hemant B; Desai, Sangeeta B

    2012-08-01

    The present study describes the clinicopathologic analysis of 34 cases of Ewing sarcoma/primitive neuroectodermal tumor occurring in the kidney. The patients were 21 males and 13 females with an age range of 6 to 44 years. Clinically, patients presented with multiple symptoms including hematuria, pain, and/or lump in the abdomen. Nephrectomy was performed in most of the cases. Grossly, whole of the renal parenchyma was involved by a variegated tumor. Histologically, the tumor was composed of monomorphic, small, and round cells arranged in a variety of patterns. Rosettes, geographical areas of necrosis, and arborizing vascular pattern were the prominent histologic features. The nucleus was monomorphic and round. Anisonucleosis was also noted in some cases. The nucleus was mostly hyperchromatic. A mixture of hyperchromatic and powdery chromatin was noted in few cases. Immunohistochemically, MIC2 (CD99) was positive in 32 of 34 cases followed by neuron-specific enolase (9/12 cases), vimentin (8/14 cases), synaptophysin (1/8 cases), and S-100 protein (1/4 cases). Molecular analysis by reverse transcriptase-polymerase chain reaction that was carried out in 26 cases revealed presence of EWS-FLI-1 type 1 translocation in 12 cases, EWS-FLI-1 type 2 translocation in 10 cases, and both type 1 and type 2 EWS-FLI-1 translocation in 2 cases. Two cases did not demonstrate any translocation. Follow-up data were available for 17 of 34 cases. Local recurrence of the tumor was seen in 4 patients, and 10 patients were recorded to have distant metastasis in various organs, such as lung, bone, and lymph node, during the course of the disease.

  6. PET surveillance of patients with ewing sarcomas of the trunk. Must the lower legs be included?

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    Vrachimis, A.; Stegger, L.; Weckesser, M. [Klinik und Poliklinik fuer Nuklearmedizin, Univ. Muenster (Germany); Dirksen, U.; Juergens, H. [Klinik und Poliklinik fuer Kinder- und Jugendmedizin - Paediatrische Haematologie und Onkologie, Univ. Muenster (Germany); Wessling, J. [Inst. fuer Klinische Radiologie, Univ. Muenster (Germany); Wenning, C.; Schober, O. [Klinik und Poliklinik fuer Nuklearmedizin, Univ. Muenster (Germany); European Inst. of Molecular Imaging, Westfaelische Wilhelms Univ. Muenster (Germany); Franzius, C. [MR- und PET/CT-Zentrum Bremen Mitte, Bremen (Germany)

    2010-07-01

    Aim: FDG-PET(/CT) is frequently used in surveillance of Ewing sarcoma (ES) patients. Since ES and PNET (primitive neuroectodermal tumours) may cause peripheral metastases some centers routinely recommend whole body PET acquisition from head to toe what may necessitate repositioning of the patient and thus extending examination time. It is not clear yet whether inclusion of lower leg adds to the diagnostic accuracy of PET scanning, especially in primary tumors of the trunk. Patients, method: 40 patients with ES and PNET of the trunk who were referred for surveillance after primary therapy with complete remission, were evaluated retrospectively: 27 men, 13 women; mean age at diagnosis 16.3 (3-35) years. At the time of diagnosis 28 patients had localized and 12 metastatic disease. Almost all of the patients had undergone a combined chemotherapy with surgery or/and radiotherapy. 156 follow-up PET scans of the legs of these patients were evaluated retrospectively. Results: Only in three (1.9%) of 156 scans a pathologic FDG accumulation was attributed to metastatic disease of the lower extremities. In these cases the observation of metastatic disease in the legs did not alter therapy, since in all three cases a multifocal disease progression was observed. Conclusion: Scanning of the lower legs may be omitted during follow-up in patients in whom the primary tumor was located in the trunk and in whom no clinical signs pointing to metastases in the lower legs are present. This provides a sufficient diagnostic power and a shorter examination time, thus increasing patient comfort and scanner availability. (orig.)

  7. Let-7a is a direct EWS-FLI-1 target implicated in Ewing's sarcoma development.

    Directory of Open Access Journals (Sweden)

    Claudio De Vito

    Full Text Available Ewing's sarcoma family tumors (ESFT are the second most common bone malignancy in children and young adults, characterized by unique chromosomal translocations that in 85% of cases lead to expression of the EWS-FLI-1 fusion protein. EWS-FLI-1 functions as an aberrant transcription factor that can both induce and suppress members of its target gene repertoire. We have recently demonstrated that EWS-FLI-1 can alter microRNA (miRNA expression and that miRNA145 is a direct EWS-FLI-1 target whose suppression is implicated in ESFT development. Here, we use miRNA arrays to compare the global miRNA expression profile of human mesenchymal stem cells (MSC and ESFT cell lines, and show that ESFT display a distinct miRNA signature that includes induction of the oncogenic miRNA 17-92 cluster and repression of the tumor suppressor let-7 family. We demonstrate that direct repression of let-7a by EWS-FLI-1 participates in the tumorigenic potential of ESFT cells in vivo. The mechanism whereby let-7a expression regulates ESFT growth is shown to be mediated by its target gene HMGA2, as let-7a overexpression and HMGA2 repression both block ESFT cell tumorigenicity. Consistent with these observations, systemic delivery of synthetic let-7a into ESFT-bearing mice restored its expression in tumor cells, decreased HMGA2 expression levels and resulted in ESFT growth inhibition in vivo. Our observations provide evidence that deregulation of let-7a target gene expression participates in ESFT development and identify let-7a as promising new therapeutic target for one of the most aggressive pediatric malignancies.

  8. Ewing Sarcoma of the Bone in Children under 6 Years of Age

    Science.gov (United States)

    De Ioris, Maria Antonietta; Prete, Arcangelo; Cozza, Raffaele; Podda, Marta; Manzitti, Carla; Pession, Andrea; Schiavello, Elisabetta; Contoli, Benedetta; Balter, Rita; Fagioli, Franca; Bisogno, Gianni; Amoroso, Loredana

    2013-01-01

    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. PMID:23382839

  9. 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.

  10. Cytokeratin-positive meningeal peripheral PNET/Ewing's sarcoma of the cervical spinal cord: diagnostic value of genetic analysis.

    Science.gov (United States)

    Woestenborghs, Heidi; Debiec-Rychter, Maria; Renard, Marleen; Demaerel, Philippe; Van Calenbergh, Frank; Van Gool, Stefaan; Sciot, Raf

    2005-01-01

    Peripheral primitive neuroectodermal tumor (PNET)/Ewing's sarcoma (ES) of the central nervous system is extremely rare and should be differentiated from central PNET and other small blue round cell tumors. We describe a case of a meningeal peripheral PNET/ES of the spinal cord in an 11-year-old boy. Immunohistochemically, the small blue round cell tumor showed expression of epithelial markers and of CD99, thus posing an important differential diagnostic problem with a poorly differentiated synovial sarcoma. Fluorescence in situ hybridization revealed rearrangement of the EWS gene, as seen in peripheral PNET/ES. Peripheral PNET/ES does occur in the central nervous system, but its diagnosis can be extremely difficult on morphologic and immunohistochemical grounds alone. Genetic analysis plays a key role in its distinction from other small blue round cell tumors.

  11. Ewing sarcoma and primitive neuroectodermal tumor of the esophagus: report of a case and review of literature.

    Science.gov (United States)

    Johnson, Andrew D; Pambuccian, Stefan E; Andrade, Rafael S; Dolan, Michelle M; Aslan, Deniz L

    2010-10-01

    This study presents a case of Ewing sarcoma and primitive neuroectodermal tumor arising in the esophagus of a 44-year-old woman who presented with progressive dysphagia. Imaging studies demonstrated a polypoid lesion in the esophagus. The tumor was characterized by corded and pseudopapillary architecture, cytologic monotony, and low proliferative activity. Immunohistochemical stains were positive for CD99, neuron-specific enolase, vimentin, cyclin D1, p53, and FLI1 gene product. Fluorescence in situ hybridization demonstrated a 22q12 translocation, associated with primitive neuroectodermal tumor in the tumor cells, whereas reverse transcription polymerase chain reaction conformed expression of Ewing sarcoma/FLI1 fusion transcript in the patient's bone marrow aspirate. Although this is a rare site for this type of tumor to occur, primitive neuroectodermal tumor should be considered in the differential diagnosis of mesenchymal tumors of the esophagus. Genetic analysis is crucial to establish the diagnosis and can be successfully performed on formalin-fixed, paraffin-embedded material and hematopoietic tissue.

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

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

    1983-12-01

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

  13. Signature-based small molecule screening identifies cytosine arabinoside as an EWS/FLI modulator in Ewing sarcoma.

    Directory of Open Access Journals (Sweden)

    Kimberly Stegmaier

    2007-04-01

    Full Text Available BACKGROUND: The presence of tumor-specific mutations in the cancer genome represents a potential opportunity for pharmacologic intervention to therapeutic benefit. Unfortunately, many classes of oncoproteins (e.g., transcription factors are not amenable to conventional small-molecule screening. Despite the identification of tumor-specific somatic mutations, most cancer therapy still utilizes nonspecific, cytotoxic drugs. One illustrative example is the treatment of Ewing sarcoma. Although the EWS/FLI oncoprotein, present in the vast majority of Ewing tumors, was characterized over ten years ago, it has never been exploited as a target of therapy. Previously, this target has been intractable to modulation with traditional small-molecule library screening approaches. Here we describe a gene expression-based approach to identify compounds that induce a signature of EWS/FLI attenuation. We hypothesize that screening small-molecule libraries highly enriched for FDA-approved drugs will provide a more rapid path to clinical application. METHODS AND FINDINGS: A gene expression signature for the EWS/FLI off state was determined with microarray expression profiling of Ewing sarcoma cell lines with EWS/FLI-directed RNA interference. A small-molecule library enriched for FDA-approved drugs was screened with a high-throughput, ligation-mediated amplification assay with a fluorescent, bead-based detection. Screening identified cytosine arabinoside (ARA-C as a modulator of EWS/FLI. ARA-C reduced EWS/FLI protein abundance and accordingly diminished cell viability and transformation and abrogated tumor growth in a xenograft model. Given the poor outcomes of many patients with Ewing sarcoma and the well-established ARA-C safety profile, clinical trials testing ARA-C are warranted. CONCLUSIONS: We demonstrate that a gene expression-based approach to small-molecule library screening can identify, for rapid clinical testing, candidate drugs that modulate previously

  14. Trial of Dasatinib in Advanced Sarcomas

    Science.gov (United States)

    2016-10-12

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

  15. Picropodophyllin inhibits the growth of Ewing's sarcoma cells through the insulin‑like growth factor‑1 receptor/Akt signaling pathway.

    Science.gov (United States)

    Wu, Yong-Tao; Wang, Bao-Jun; Miao, Sheng-Wu; Gao, Jian-Jun

    2015-11-01

    Ewing's sarcoma (ES) is the second most common type of pediatric bone tumor, and is associated with a poor prognosis. Picropodophyllin (PPP), a novel selective inhibitor of insulin‑like growth factor‑1 receptor (IGF‑1R), is able to strongly inhibit various types of cancers. However, the effect of IGF‑1R on ES remains unclear. Following treatment with various concentrations of PPP for various times, cell viability was determined using an MTT assay. In addition, cell proliferation and apoptosis was investigated separately by bromodeoxyuridine staining and flow cytometry, respectively. The PPP‑associated signaling pathway was also investigated. The results of the present study suggested that PPP inhibited cell proliferation and viability of A673 and SK‑ES‑1 human Ewing's sarcoma cells in a dose- and time‑dependent manner. In addition, cell apoptosis rates were increased following treatment with PPP. Further investigation of the underlying mechanism revealed that PPP inhibited Akt phosphorylation. Fumonisin B1, an Akt‑specific activator, reversed the inhibitory effects of PPP on cell growth. Furthermore, the results suggested that PPP decreased the expression levels of IGF‑1R, a common activator of Akt signaling. PPP inhibited the growth of human Ewing's sarcoma cells by targeting the IGF‑1R/Akt signaling pathway. Therefore, PPP may prove useful in the development of an effective strategy for the treatment of Ewing's sarcoma.

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

    Directory of Open Access Journals (Sweden)

    Christopher A Scannell

    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.

  17. Long-term results following multidisciplinary treatment of localized Ewing's sarcoma in children and adolescents; Ergebnisse der multidisziplinaeren Behandlung lokalisierter Ewing-Sarkome bei Kindern und Jugendlichen

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    Ullmann, C.; Sauer, R.; Grabenbauer, G.G. [Universitaetsklinikum Erlangen (Germany). Strahlenklinik; Beck, J.D.; Holter, W. [Universitaetsklinikum Erlangen (Germany). Klinik fuer Kinder und Jugendliche; Petsch, S. [Tumorzentrum Erlangen-Nuernberg (Germany); Dunst, J. [Universitaetsklinikum Schleswig-Holstein, Campus Luebeck (Germany). Klinik fuer Strahlentherapie

    2008-03-15

    Purpose: To identify results and prognostic factors on long-term survival and local control following treatment of localized Ewing's sarcoma. Patients and Methods: Between 1979 and 2004, a total of 60 children and young adults with Ewing's sarcoma were treated. Patients with distant metastases at presentation (n = 6) and recurrent cases (n = 2) were excluded from this analysis. Patients were exclusively treated within ongoing national and international protocols CESS-81, CESS-86, EICESS-92, EURO-EWING-99. All patients received local irradiation with a total dose of 45-60 Gy; in addition, 41 (79%) of the patients had local surgical procedures, 27 (52%) of them with clear margins. Results: Overall survival rates at 5 and 10 years were 56% and 45%, respectively. Patients {<=} 14 years at diagnosis had overall survival rates of 66% and 61% at 5 and 10 years compared to older patients with corresponding survival rates of 47% and 31%, respectively (p = 0.05). Patients with tumors of lower volume ({<=} 100 ml) had significantly better survival rates at 5 and 10 years: 82% and 60% versus 42% und 39% (p = 0.03). Seven of 52 (14%) patients experienced local failure; no significant impact was noted for any of the following factors: treatment protocol, radiation dose, surgery. Conclusion: Radiotherapy with and without surgery of Ewing's sarcoma is a highly effective local treatment option. It seems preferable to mutilating surgery. (orig.)

  18. Ewing's sarcoma, fibrogenic tumors, giant cell tumor, hemangioma of bone. Radiology and pathology; Ewing-Sarkom, fibrogene Tumoren, Riesenzelltumor, Haemangiom des Skeletts. Radiologie und Pathologie

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    Freyschmidt, J. [Beratungsstelle und Referenzzentrum fuer Osteoradiologie, Bremen (Germany); Ostertag, H. [Klinikum Region Hannover GmbH, Pathologisches Institut, Hannover (Germany)

    2016-06-15

    Radiological imaging only reflects the anatomy and its pathological abnormalities. Therefore, the radiologist should be able to recognize the basic features of the pathological anatomy of bone tumors. This can only be learned working closely with a pathologist who is experienced in this field. On the other hand, the pathologist needs from the radiologist their diagnostic assessment with information on size, location, aggressiveness and the existence of a bone tumor's matrix, of the whole lesion, because he usually only receives a small part for examination in the form of a biopsy. In this article, the features and fundamentals (standards) of radiological-pathological cooperation as the mainstay for a precise diagnosis in bone tumors are outlined. The radiological appearance and the histopathological features behind it are presented for Ewing's sarcoma, fibrogenic tumors, giant cell tumor, and hemangioma of the bone. (orig.) [German] Radiologische Bilder spiegeln nichts anderes als die Anatomie und ihre pathologischen Abweichungen wider. Deshalb sollte der Radiologe die Grundzuege der pathologischen Anatomie auch von Knochentumoren kennen. Das kann er nur durch eine enge Zusammenarbeit mit einem auf diesem Gebiet erfahrenen Pathologen erlernen. Andererseits braucht der Pathologe vom Radiologen dessen diagnostische Einschaetzung mit Informationen ueber die Groesse, Lage, Aggressivitaet und das Vorhandensein einer Matrix eines Knochentumors und zwar von der gesamten Laesion, denn er bekommt inform einer Biopsie i. d. R. nur einen mehr oder weniger kleinen Teil zur Untersuchung. In diesem Beitrag werden die Grundzuege und Standards der radiologisch-pathologischen Zusammenarbeit aufgezeigt, auf denen eine praezise Diagnosestellung beruht. Radiologisches Erscheinungsbild und die dahintersteckenden - und erklaerenden - histopathologischen Merkmale werden fuer das Ewing-Sarkom, fuer fibrogene Tumoren, den Riesenzelltumor und das Haemangiom des Knochens

  19. Detection of EWS/FLI-1 by Immunostaining. An Adjunctive Tool in Diagnosis of Ewing's Sarcoma and Primitive Neuroectodermal Tumour on Cytological Samples and Paraffin-Embedded Archival Material

    Directory of Open Access Journals (Sweden)

    Gunnar Nilsson

    1999-01-01

    Full Text Available Purpose. Recently we showed that the 68-kDa fusion protein derived from the EWS/FLI1 hybrid gene can be specifically detected by Western blotting using a polyclonal antibody to the C-terminal of FLI1 on biopsy material from Ewing's sarcoma. The aim of this study was to investigate whether this antibody also could be used for immunocytochemistry and immunohistochemistry in diagnosis of Ewing's sarcoma.

  20. Clinical and biochemical function of polymorphic NR0B1 GGAA-microsatellites in Ewing sarcoma: a report from the Children's Oncology Group.

    Directory of Open Access Journals (Sweden)

    Michael J Monument

    Full Text Available The genetics involved in Ewing sarcoma susceptibility and prognosis are poorly understood. EWS/FLI and related EWS/ETS chimeras upregulate numerous gene targets via promoter-based GGAA-microsatellite response elements. These microsatellites are highly polymorphic in humans, and preliminary evidence suggests EWS/FLI-mediated gene expression is highly dependent on the number of GGAA motifs within the microsatellite.Here we sought to examine the polymorphic spectrum of a GGAA-microsatellite within the NR0B1 promoter (a critical EWS/FLI target in primary Ewing sarcoma tumors, and characterize how this polymorphism influences gene expression and clinical outcomes.A complex, bimodal pattern of EWS/FLI-mediated gene expression was observed across a wide range of GGAA motifs, with maximal expression observed in constructs containing 20-26 GGAA motifs. Relative to white European and African controls, the NR0B1 GGAA-microsatellite in tumor cells demonstrated a strong bias for haplotypes containing 21-25 GGAA motifs suggesting a relationship between microsatellite function and disease susceptibility. This selection bias was not a product of microsatellite instability in tumor samples, nor was there a correlation between NR0B1 GGAA-microsatellite polymorphisms and survival outcomes.These data suggest that GGAA-microsatellite polymorphisms observed in human populations modulate EWS/FLI-mediated gene expression and may influence disease susceptibility in Ewing sarcoma.

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

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    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

  2. Sorafenib in Treating Patients With Soft Tissue Sarcomas (Extremity Sarcoma Closed to Entry as of 5/30/07)

    Science.gov (United States)

    2014-04-01

    Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

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

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    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.)

  4. 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.

  5. DNA Methylation and Gene Expression Profiling of Ewing Sarcoma Primary Tumors Reveal Genes That Are Potential Targets of Epigenetic Inactivation

    Directory of Open Access Journals (Sweden)

    Nikul Patel

    2012-01-01

    Full Text Available The role of aberrant DNA methylation in Ewing sarcoma is not completely understood. The methylation status of 503 genes in 52 formalin-fixed paraffin-embedded EWS tumors and 3 EWS cell lines was compared to human mesenchymal stem cell primary cultures (hMSCs using bead chip methylation analysis. Relative expression of methylated genes was assessed in 5-Aza-2-deoxycytidine-(5-AZA-treated EWS cell lines and in a cohort of primary EWS samples and hMSCs by gene expression and quantitative RT-PCR. 129 genes demonstrated statistically significant hypermethylation in EWS tumors compared to hMSCs. Thirty-six genes were profoundly methylated in EWS and unmethylated in hMSCs. 5-AZA treatment of EWS cell lines resulted in upregulation of expression of hundreds of genes including 162 that were increased by at least 2-fold. The expression of 19 of 36 candidate hypermethylated genes was increased following 5-AZA. Analysis of gene expression from an independent cohort of tumors confirmed decreased expression of six of nineteen hypermethylated genes (AXL, COL1A1, CYP1B1, LYN, SERPINE1, and VCAN. Comparing gene expression and DNA methylation analyses proved to be an effective way to identify genes epigenetically regulated in EWS. Further investigation is ongoing to elucidate the role of these epigenetic alterations in EWS pathogenesis.

  6. 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.

  7. {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.)

  8. Chemotherapy-related toxicity in patients with non-metastatic Ewing sarcoma: influence of sex and age.

    Science.gov (United States)

    Paioli, Anna; Luksch, Roberto; Fagioli, Franca; Tamburini, Angela; Cesari, Marilena; Palmerini, Emanuela; Abate, Massimo Eraldo; Marchesi, Emanuela; Balladelli, Alba; Pratelli, Loredana; Ferrari, Stefano

    2014-02-01

    Influence of age and sex on chemotherapy-related toxicity was evaluated in children (3-9 years), adolescents (10-17 years), and adults (up to 40 years) with localized Ewing sarcoma (ES) enrolled in the ISG/SSG III protocol. Treatment was based on vincristine, doxorubicin, cyclophosphamide, ifosfamide, dactinomycin, and etoposide. High-dose chemotherapy with busulfan and melphalan was given in poor responder patients. The analysis was based on 2191 courses of standard chemotherapy and 230 patients. A lower risk of G4 leukopenia and thrombocytopenia, hospitalization, febrile neutropenia, and red blood cell (RBC) transfusions was observed in males. Use of granulocyte colony-stimulating factor (G-CSF) was more frequent in adults, while children more often received RBC transfusions. A significant correlation between sex and chemotherapy-related toxicity was observed in the study, whereas no significant differences in terms of bone marrow toxicity can be expected according to patient age. Further studies should analyse the role of pharmacokinetics, pharmacogenomics, and clinical characteristics.

  9. Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution

    Science.gov (United States)

    Huh, Jimi; Park, Seong Joon; Kim, Hyoung Jung; Lee, Jong Seok; Ha, Hyun Kwon; Tirumani, Sree Harsha; Ramaiya, Nikhil H.

    2015-01-01

    Objective To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. Materials and Methods We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for ≥ 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. Results Among the 70 patients (mean age, 35.8 ± 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). Conclusion Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites. PMID:26175577

  10. Primary cutaneous Ewing's sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, Won-Jong [The Catholic University of Korea, Department of Orthopedic Surgery, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Chang, Eun-Deok; Lee, An-Hee [The Catholic University of Korea, Department of Pathology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Bae, Jung Min; Kim, Jin Woo [The Catholic University of Korea, Department of Dermatology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Chun, Kyung-Ah [The Catholic University of Korea, Department of Radiology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Rho, Sang-Young [The Catholic University of Korea, Department of Hemato-oncology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea)

    2010-06-15

    Extraskeletal Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are widely regarded as clinically and histologically identical tumors which consist of small blue round cells. Extraskeletal ESs/PNETs usually occur in the deep soft tissues of the paraspinal region, chest wall, or lower extremities. However, superficially located cases, so-called cutaneous ESs/PNETs, are exceedingly rare, and the vast majority of the reported cases present as a single small mass. We present magnetic resonance imaging (MRI) findings and clinical course of a unique case of primary cutaneous ES/PNET presenting as numerous huge masses with severe ulceration on them. (orig.)

  11. The Ewing's sarcoma EWS/FLI-1 fusion gene encodes a more potent transcriptional activator and is a more powerful transforming gene than FLI-1.

    OpenAIRE

    May, W A; Lessnick, S L; Braun, B S; Klemsz, M; Lewis, B. C.; Lunsford, L B; Hromas, R; Denny, C T

    1993-01-01

    EWS/FLI-1 is a chimeric protein formed by a tumor-specific 11;22 translocation found in both Ewing's sarcoma and primitive neuroectodermal tumor of childhood. EWS/FLI-1 has been shown to be a potent transforming gene, suggesting that it plays an important role in the genesis of these human tumors. We now demonstrate that EWS/FLI-1 has the characteristics of an aberrant transcription factor. Subcellular fractionation experiments localized the EWS/FLI-1 protein to the nucleus of primitive neuro...

  12. 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 ...

  13. 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.

  14. 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

  15. 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

  16. Ewing's sarcoma in children: twenty-five years of experience at the Instituto Portugês de Oncologia de Francisco Gentil (I.P.O.F.G.).

    Science.gov (United States)

    Patricio, M B; Vilhena, M; Neves, M; Raposo, S; Catita, J; De Sousa, V; Martins, A G

    1991-05-01

    Fifty children with Ewing's sarcoma were consecutively treated from 1962 to 1987 and retrospectively analyzed at the I.P.O.F.G. of Lisbon. At first diagnosis, 10 cases had distant metastases. The remaining 40 patients had clinically localized disease, and different protocols were followed over the years. The best results were obtained with chemotherapy and radiotherapy with or without surgery; and for these children the two-year survival rate was 42.8% vs. 8.3% for the group of patients submitted to local treatment alone. Besides the treatment modality, other factors influenced the prognosis, such as inflammatory signs, sex, tumor volume, and tumor site as well as evidence of distant metastases.

  17. 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.

  18. Stages of Ewing Sarcoma

    Science.gov (United States)

    ... available from the NCI website . Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is ... of Health FOLLOW US Facebook Twitter Instagram YouTube Google+ LinkedIn GovDelivery RSS CONTACT INFORMATION Contact Us LiveHelp ...

  19. 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.

  20. 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.

  1. 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;

    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 ...

  2. R1507, an anti-insulin-like growth factor-1 receptor (IGF-1R antibody, and EWS/FLI-1 siRNA in Ewing's sarcoma: convergence at the IGF/IGFR/Akt axis.

    Directory of Open Access Journals (Sweden)

    Helen J Huang

    Full Text Available A subset of patients with Ewing's sarcoma responds to anti-insulin-like growth factor-1 receptor (IGF-1R antibodies. Mechanisms of sensitivity and resistance are unknown. We investigated whether an anti-IGF-1R antibody acts via a pathway that could also be suppressed by small interfering (si RNA against the EWS/FLI-1 fusion protein, the hallmark of Ewing's sarcoma. The growth of two Ewing's sarcoma cell lines (TC-32 and TC-71 was inhibited by the fully human anti-IGF-1R antibody, R1507 (clonogenic and MTT assays. TC-32 and TC-71 cells express high levels of IGF-2, while RD-ES and A4573 Ewing's cell lines, which were less responsive to R1507 in our assays, express low or undetectable IGF-2, respectively. TC-71 cells also expressed high levels of IGF-1R, and R1507 decreased steady-state levels of this receptor by internalization/degradation, an effect which was associated with a decrease in p-IGF-1R, p-IRS-1, and p-Akt. EWS/FLI-1 siRNA also decreased p-Akt, due to its ability to increase IGF-BP3 levels and subsequently decrease IGF-1 and IGF-2 levels, thus inhibiting signaling through p-IGF-1R. This inhibition correlated with growth suppression and apoptosis. The attenuation of Akt activation was confirmed in TC-71 and HEK-293 (human embryonic kidney cells by transfecting them with IGF-1R siRNA. We conclude that antibodies and siRNA to IGF-1R, as well as siRNA to EWS/FLI-1, act via intersecting IGF/IGF-1R signals that suppress a common point in this pathway, namely the phosphorylation of Akt.

  3. Candida tropicalis arthritis of the elbow in a patient with Ewing's sarcoma that successfully responded to itraconazole

    OpenAIRE

    Seung Youn Kim; Jung Sub Lim; Dong Hwan Kim; Hyeon Jeong Lee; Joon Bum Cho; Jn Ah Lee; Dong Ho Kim

    2011-01-01

    Fungal infections are rarely responsible for arthritis. Few cases of fungal arthritis have been reported, even in immunocompromised hosts susceptible to low-virulence organisms. Herein, the authors report the first case of Candida tropicalis arthritis in a child with a solid tumor. A 13-year-old boy with Ewing’s sarcoma developed arthritis in his elbow during the neutropenic period after chemotherapy. Despite treatment with broad-spectrum antibiotics, his condition did not improve and serial ...

  4. Clinicopathological and molecular spectrum of ewing sarcomas/PNETs, including validation of EWSR1 rearrangement by conventional and array FISH technique in certain cases.

    Science.gov (United States)

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

    2014-07-01

    Over the years, a wide clinicopathological spectrum has been identified within Ewing family of tumors (EFTs). As these tumors are chemosensitive, their correct and timely identification is necessary. The aims of this study were (1) to present the diverse clinicopathological and molecular profile of EFTs in our settings, (2) to identify a pragmatic approach for diagnosing EFTs, especially for application of ancillary techniques, namely RT-PCR for specific transcripts (EWS-FLI1, EWS-ERG) and FISH for EWSR1 gene rearrangement, in certain cases and (3) to show the utility of tissue microarray in establishing a new FISH test. Fifty-eight EFTs were identified in 38 males and 20 females within an age-range of 1-65 years (median, 16), mostly in lower extremities (14) (24.1 %). Therapeutically, most patients underwent neoadjuvant chemotherapy with subsequent surgery. Histopathologically, diagnosis of EFTs was initially offered in 41/58 (70.6 %) tumors. On review, 59 % tumors showed diffuse pattern, while 41 % displayed rosettes. Immunohistochemically, tumor cells were mostly diffusely positive for CD99 (48/52) (92.3 %); FLI-1 (17/18) (94.4 %); variably for BCL2 (16/18) (88.8 %), synaptophysin (6/20) (35 %), S100-P (2/7) (28.5 %), CD56 (2/5) (40 %), NSE (2/5) (40 %), calponin (3/4) (75 %), EMA (5/24) (20.8 %) and CK (3/24) (12.5 %), the latter two mostly focally. Fifty five tumors were EWS-FLI1 positive, while a single tumor was EWS-ERG positive. Sensitivity for PCR was 61 %. EWSR1 rearrangement was detected by FISH in 12/13 Ewing sarcomas/PNETs. Sensitivity for EWSR1 test was 92.3 % and specificity was 100 %. Thirty-eight tumors, including 14 molecular confirmed EFTs and 21 other tumors were tested for EWSR1 rearrangement. Among 21 unrelated tumors, EWSR1 rearrangement was detected in few myoepithelial tumors, occasional desmoplastic small round cell tumor and an extraskeletal myxoid chondrosarcoma. Further, a tissue microarray with a separate set of 8 EFTs, confirmed at

  5. Cyclin D1 is a useful marker for soft tissue Ewing's sarcoma/peripheral Primitive Neuroectodermal Tumor in children and adolescents: A comparative immunohistochemical study with rhabdomyosarcoma.

    Science.gov (United States)

    Magro, Gaetano; Brancato, Franca; Musumeci, Giuseppe; Alaggio, Rita; Parenti, Rosalba; Salvatorelli, Lucia

    2015-01-01

    Cyclin D1 amplification and/or overexpression contribute to the loss of the regulatory circuits that govern G1-S transition phase of the cell cycle, playing pivotal roles in different human malignant tumors, including breast, colon, prostate cancer, lymphoma, melanoma and neuroblastoma. In vitro studies have shown that cyclin D1 is overexpressed in Ewing's sarcoma (EWS)/peripheral Primitive Neuroectodermal Tumor (pPNET), but not in rhabdomyosarcoma cell lines. Only a few immunohistochemical studies are available on cyclin D1 expression in EWS/pPNET, which confirmed its expression only in a limited number of cases. The aim of the present study was a comparative immunohistochemical analysis of the expression and distribution of cyclin D1 in a large series of pediatric/adolescent soft tissue EWS/pPNETs and rhabdomyosarcomas (both embryonal and alveolar subtypes) to assess its potential usefulness in their differential diagnosis. Notably cyclin D1 was strongly and diffusely expressed in all cases (20/20) of EWS/pPNET, while it was lacked in all cases (15/15) of rhabdomyosarcomas. Immunohistochemical overexpression of cyclin D1 in EWS/pPNET is a novel finding which could be exploitable as a diagnostic immunomarker for this tumor. Although highly sensitive, cyclin D1 is not specific for EWS/pPNET, and thus it should not be evaluated alone but in the context of a wide immunohistochemical panel. Accordingly, we first emphasize that when pathologists are dealing with a small round blue cell tumor of soft tissues in pediatric/adolescent patients, a strong and diffuse nuclear expression of cyclin D1 is of complementary diagnostic value to CD99 and FLI-1 in confirming diagnosis of EWS/pPNET and in ruling out rhabdomyosarcoma.

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

    Energy Technology Data Exchange (ETDEWEB)

    Treglia, Giorgio [Institute of Nuclear Medicine, Catholic University of the Sacred Heart, Department of Bioimaging and Radiological Sciences, Rome (Italy); Institute of Nuclear Medicine, Positron Emission Tomography Centre, Catholic University of the Sacred Heart, Department of Bioimaging and Radiological Sciences, Rome (Italy); Salsano, Marco; Stefanelli, Antonella; Mattoli, Maria Vittoria; Giordano, Alessandro [Institute of Nuclear Medicine, Catholic University of the Sacred Heart, Department of Bioimaging and Radiological Sciences, Rome (Italy); Bonomo, Lorenzo [Institute of Radiology, Catholic University of the Sacred Heart, Department of Bioimaging and Radiological Sciences, Rome (Italy)

    2012-03-15

    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.)

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

    Science.gov (United States)

    2016-06-09

    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

  8. 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

  9. 脊柱尤文肉瘤的临床及病理特征和MRI表现%Ewing's sarcoma of vertebral column:clinical,pathological and MRI manifestations

    Institute of Scientific and Technical Information of China (English)

    洪国斌; 梁碧玲; 邬伟明; 冯晓新; 谢玉妍

    2009-01-01

    Objective To analyze the clinical,pathological and MR imaging features of Ewing's sarcoma of vertebral column.Methods Nine cases of Ewing's sarcoma of vertebral column was proved by surgical or biopsy pathology.6 of 9 patients underwent MR imaging.Their clinical and pathological features and MR imaging manifestations were retrospectively analyzed.Results In 6 patients with MR imaging,2 tumors arose each in cervical,thoracic and lumbar vertebrae,respectively,the remaining 3 tumors in sacral vertebrae.The tumor showed isointensity in 4 cases and slight hypointensity in 2 cases on T1-weighted imaging,and heterogeneous hyperintensity signal on T2-weighted imaging and heterogeneously enhanced after contrast enhancement.Soft tissue mass were found in 6 tumors and no calcification and periosteal reaction were seen.Hematoxylin-Eosin staining demonstrated uniform small round cells in all cases and without Homer-Wright(H-W) rosettes.PAS staining were positive in 6 cases.2 cases were slightly positive for NSE.Conclusions The Ewing's sarcoma of vertebral column has some characteristic manifestations but not pathognomonic on MR imaging.The Ewing's sarcoma of vertebral column should be reminded when the patients are children or adolescent,and the lesions show obviously osteolysis of vertebral body and a concurrent large soft tissue mass.%目的 探讨脊柱尤文肉瘤的临床、病理及MRI特点.方法 回顾性分析9例经手术病理或穿刺活检证实的脊柱尤文肉瘤的临床、病理特征及MRI表现.结果 9例脊柱尤文肉瘤中,发生于颈椎、胸椎和腰椎各2例,骶椎3例.6例进行了MRI检查,均显示椎体骨骼信号异常,在T1WI上4例表现为中等信号,2例表现为稍低信号;在T2WI上表现为稍高信号,钆喷替酸葡甲胺(Gd-DTPA)增强后不均匀强化.3例表现为溶骨性骨质破坏、椎体压缩变扁;3例表现为椎体轻度膨胀性溶骨性破坏.相邻的椎间隙无狭窄、椎间盘信号无异常;均见周围明显

  10. Treatment Option Overview (Ewing Sarcoma)

    Science.gov (United States)

    ... available from the NCI website . Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is ... of Health FOLLOW US Facebook Twitter Instagram YouTube Google+ LinkedIn GovDelivery RSS CONTACT INFORMATION Contact Us LiveHelp ...

  11. General Information about Ewing Sarcoma

    Science.gov (United States)

    ... available from the NCI website . Chimeric antigen receptor (CAR) T-cell therapy CAR T-cell therapy is ... of Health FOLLOW US Facebook Twitter Instagram YouTube Google+ LinkedIn GovDelivery RSS CONTACT INFORMATION Contact Us LiveHelp ...

  12. Genetics Home Reference: Ewing sarcoma

    Science.gov (United States)

    ... and young adults. Affected individuals usually feel stiffness, pain, swelling, or tenderness of the bone ... of genetic change, called a somatic mutation, is not inherited. The protein produced from ...

  13. Treatment of pelvic Ewing's sarcoma in children and the effect on the skeletal growth and development%儿童骨盆尤文肉瘤的治疗以及对骨骼生长发育的影响

    Institute of Scientific and Technical Information of China (English)

    付军; 郭征; 王臻; 栗向东; 李靖; 陈国景; 吴智钢

    2012-01-01

    Objective To explore the effect of neo-adjuvant chemotherapy and computer-assisted surgery on children and adolescents with primary pelvic Ewing's sarcoma,and assess the therapeutic effect on the pelvic skeletal growth and development.Methods This is a retrospective analysis of 10 children with primary pelvic Ewing's sarcoma treated between Jan 2001 and Oct 2010 at the Department of Oncologic Orthopaedics at Xijing Hospital.There were 3 girls and 7 boys in the age of 7 to 16 years (average 12.7 years).All patients were pathologically diagnosed as Ewing's sarcoma.There were two cases in the sacroiliac joint,one in the ilium,one in the pubic bone,and 6 cases in peri-acetabular area including 5 below the triradiate cartilage and one above the triradiate cartilage,without cartilage invasion.All patients underwent neo-adjuvant chemotherapy,resection and reconstruction surgery and postoperative chemotherapy.CDP,ADM and IFO regimen chemotherapy were given as the main treatment.Five cases were treated by traditional resection and reconstruction,and after 2008,five cases were treated by computer-assisted surgery.During the reconstruction,the hip rotation center was put at a depressed location.All of the 10 cases underwent postoperative radiotherapy in a dose of 45-55 Gy.Results All patients were followed-up for 12-72 months (mean:37.8 months).One child had tumor recurrence and lung metastasis and 9 patients had no evidence of disease (NED).After neo-adjuvant chemotherapy,the oncologic statuses (RECIST)were:1 CR,8 PR and 1 SD.The functional recoveries after surgery (Enneking's) were:4 cases excellent,4 good,1 fair and 1 poor.Five cases who underwent computer-assisted surgery achieved a good reconstruction without local recurrence.There were no effects on skeletal growth in 8 cases.An unbalanced hip rotational center occurred in one case,and a compemsatory scoliosis was found in another case.There were no serious complications in all patients.Conclusions The comprehensive

  14. 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.

  15. RING1B contributes to Ewing sarcoma development by repressing the NaV1.6 sodium channel and the NF-κB pathway, independently of the fusion oncoprotein

    Science.gov (United States)

    Hernandez-Muñoz, Inmaculada; Rodriguez, Eva; Fernández-Mariño, Ana Isabel; Pardo-Pastor, Carlos; Bahamonde, María Isabel; Fernández-Fernández, José M.; García-Domínguez, Daniel J.; Hontecillas-Prieto, Lourdes; Lavarino, Cinzia; Carcaboso, Angel M.; de Torres, Carmen; Tirado, Oscar M.; de Alava, Enrique; Mora, Jaume

    2016-01-01

    Ewing sarcoma (ES) is an aggressive tumor defined by EWSR1 gene fusions that behave as an oncogene. Here we demonstrate that RING1B is highly expressed in primary ES tumors, and its expression is independent of the fusion oncogene. RING1B-depleted ES cells display an expression profile enriched in genes functionally involved in hematological development but RING1B depletion does not induce cellular differentiation. In ES cells, RING1B directly binds the SCN8A sodium channel promoter and its depletion results in enhanced Nav1.6 expression and function. The signaling pathway most significantly modulated by RING1B is NF-κB. RING1B depletion results in enhanced p105/p50 expression, which sensitizes ES cells to apoptosis by FGFR/SHP2/STAT3 blockade. Reduced NaV1.6 function protects ES cells from apoptotic cell death by maintaining low NF-κB levels. Our findings identify RING1B as a trait of the cell-of-origin and provide a potential targetable vulnerability. PMID:27317769

  16. Full-term newborn after repeated ovarian tissue transplants in a patient treated for Ewing sarcoma by sterilizing pelvic irradiation and chemotherapy

    Science.gov (United States)

    Rodriguez-Wallberg, Kenny A; Karlström, Per-Olof; Rezapour, Masoumeh; Castellanos, Enrique; Hreinsson, Julius; Rasmussen, Carsten; Sheikhi, Mona; Ouvrier, Bettina; Bozóky, Béla; Olofsson, Jan I; Lundqvist, Monalill; Hovatta, Outi

    2015-01-01

    We report the first successful transplantation of cryopreserved ovarian cortical tissue into heavily irradiated tissues in a patient who had received sterilizing pelvic radiotherapy (54 Gy) and 40 weeks of intensive high-dose chemotherapy for the treatment of Ewing’s sarcoma 14 years earlier. Repeated transplantation procedures were required to obtain fully functional follicular development. Enlargement of the transplants over time and increase of the size of the uterus were demonstrated on sequential ultrasonographic examinations. Eggs of good quality that could be fertilized in vitro were obtained only after a substantial incremental increase of the amount of ovarian tissue transplanted. Single embryo replacement resulted in a normal pregnancy and the birth of a healthy child by cesarean section at full-term. No neonatal or maternal postoperative complications occurred. Women facing high-dose pelvic radiotherapy should not be systematically excluded from fertility preservation options, as is currently the trend. PMID:25545009

  17. Human HLA-A*02:01/CHM1+ allo-restricted T cell receptor transgenic CD8+ T cells specifically inhibit Ewing sarcoma growth in vitro and in vivo.

    Science.gov (United States)

    Blaeschke, Franziska; Thiel, Uwe; Kirschner, Andreas; Thiede, Melanie; Rubio, Rebeca Alba; Schirmer, David; Kirchner, Thomas; Richter, Günther H S; Mall, Sabine; Klar, Richard; Riddell, Stanley; Busch, Dirk H; Krackhardt, Angela; Grunewald, Thomas G P; Burdach, Stefan

    2016-07-12

    The endochondral bone protein Chondromodulin-I (CHM1) provides oncogene addiction in Ewing sarcoma (ES). We pre-clinically tested the targetability of CHM1 by TCR transgenic, allo-restricted, peptide specific T cells to treat ES. We previously generated allo-restricted wildtype CD8+ T cells directed against the ES specific antigen CHM1319 causing specific responses against ES. However, utilization of these cells in current therapy protocols is hampered due to high complexity in production, relatively low cell numbers, and rapid T cell exhaustion.In order to provide off-the-shelf products in the future, we successfully generated HLA-A*02:01-restricted T cell receptor (TCR) transgenic T cells directed against CHM1319 by retroviral transduction.After short-term expansion a 100% purified CHM1319-TCR-transgenic T cell population expressed a CD62L+/CD45RO and CD62L+/CD45RA+ phenotype. These cells displayed specific in vitro IFNg and granzyme B release in co-culture with HLA-A*02:01+ ES cell lines expressing CHM1. When co-injected with ES cells in Rag2-/-É£c-/- mice, CHM1-specific TCR-transgenic T cells significantly inhibited the formation of lung and liver metastases in contrast to control mice. Lungs and livers of representative mice displayed CD8+ T cell infiltration in the presence (control group treated with unspecific T cells) and in the absence (study group) of metastatic disease, respectively. Furthermore, mice receiving unspecific T cells showed signs of graft-versus-host-disease in contrast to all mice, receiving CHM1319-TCR-transgenic T cells.CHM1319 specific TCR-transgenic T cells were successfully generated causing anti-ES responses in vitro and in vivo. In the future, CHM1319-TCR-transgenic T cells may control minimal residual disease rendering donor lymphocyte infusions more efficacious and less toxic.

  18. 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

  19. Osteopontin is involved in the development of acquired chemo-resistance of cisplatin in small cell lung cancer.

    Science.gov (United States)

    Gu, Tao; Ohashi, Rina; Cui, Ri; Tajima, Ken; Yoshioka, Masakata; Iwakami, Shinichiro; Sasaki, Shinichi; Shinohara, Atsuko; Matsukawa, Takehisa; Kobayashi, Jun; Inaba, Yutaka; Takahashi, Kazuhisa

    2009-11-01

    Osteopontin (OPN) is a multi-functional cytokine involved in cell survival, migration and adhesion which is associated with tumorigenesis, progression and metastasis. However, the role of OPN in chemo-sensitivity of human lung cancer has not yet been elucidated. The purpose of this study is to investigate the role of OPN in chemo-sensitivity of lung cancer cells. We developed a stable OPN transfectant (SBC-3/OPN) and a control transfectant (SBC-3/NEO) from human small cell lung cancer cell line, SBC-3. SBC-3/OPN cells were more resistant to cisplatin than SBC-3/NEO cells. Multi-drug resistance-associated protein (MRP) does not appear to be involved in the development of acquired chemo-resistance, since MRP inhibitor did not alter chemo-sensitivity. After exposure to cisplatin, the apoptotic SBC-3/OPN cells were reduced in number compared to SBC-3/NEO cells. Treatment with cisplatin revealed that the expression of anti-apoptotic protein, bcl-2, was down-regulated in SBC-3/NEO cells, while that of SBC-3/OPN cells was not altered. In contrast, pro-apoptotic protein, bax, was not altered in both SBC-3/OPN and SBC-3/NEO cells, thus bcl-2/bax ratio was decreased in SBC-3/NEO but not altered in SBC-3/OPN cells. Activation of caspase-3 and caspase-9 was increased in SBC-3/NEO cells, but not in SBC-3/OPN cells. Our results suggest that OPN enhances chemo-resistance of cisplatin in SBC-3 cells by suppressing bcl-2 protein down-regulation, thereby blocking the caspase-9- and caspase-3-dependent cell apoptosis.

  20. The role of SH3GL3 in myeloma cell migration/invasion, stemness and chemo-resistance.

    Science.gov (United States)

    Chen, Ruoying; Zhao, Hong; Wu, Dan; Zhao, Chen; Zhao, Weiling; Zhou, Xiaobo

    2016-11-08

    Multiple myeloma (MM) is an incurable cancer characterized by clonal expansion of malignant plasma cells in the bone marrow and their egress into peripheral blood. The mechanisms of myeloma cells migration/invasion have remained unclear. Herein, we found SH3GL3 was highly expressed in the CD138-negative (CD138-) myeloma cells. The migration/invasion capability of CD138- cells was significantly higher than that in the CD138-positive (CD138+) cells. Silencing SH3GL3 using shRNA reduced myeloma cells migration/invasion. Conversely, overexpression of SH3GL3 increased myeloma cells migration/invasion. Moreover, SH3GL3 is also associated with the stemness and chemo-resistance of CD138- myeloma cells. Elevated expression of stem cell and multi-drug resistant markers were seen in the myeloma cells with overexpressed SH3GL3; while knocking-down SH3GL3 reduced the expression of these markers. A marked increase in p-PI3K and p-FAK was observed in the cells with overexpressed SH3GL3. To test if FAK/PI3K signaling pathway was involved in the SH3GL3-mediated myeloma cells migration, the cells transfected w/wo SH3GL3 cDNA were treated with FAK inhibitor 14 and PI3K inhibitor LY294002. Inhibition of FAK and PI3K attenuated SH3GL3-mediated migration /invasion. Our findings indicate that SH3GL3 plays an important role in myeloma cell migration/invasion, stemness and chemo-resistance. The SH3GL3-mediated myeloma cell migration/invasion is mediated by FAK/PI3K signaling pathway.

  1. Indian data on bone and soft tissue sarcomas: A summary of published study results

    Directory of Open Access Journals (Sweden)

    Anant Ramaswamy

    2016-01-01

    Full Text Available Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS, chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation. Soft tissue sarcomas are a rare heterogeneous group of tumors, more than 50 different tumor entities. The common subtypes identified in India include Ewing sarcoma and synovial sarcoma. Valuable work regarding brachytherapy has been done by radiation oncologists from the TMH, especially in pediatric patients.

  2. 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.

  3. 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.

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

    Directory of Open Access Journals (Sweden)

    Fadare Oluwole

    2006-09-01

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

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

    Directory of Open Access Journals (Sweden)

    S. Patricia

    2011-01-01

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

  6. 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.

  7. Retroperitoneal Sarcomas.

    Science.gov (United States)

    Porpiglia, Andrea S; Reddy, Sanjay S; Farma, Jeffrey M

    2016-10-01

    Retroperitoneal sarcomas are rare tumors, representing only 15% of all sarcomas. The mainstay of therapy is surgical resection with negative margins. However, this is challenging because of the late presentation of many of these tumors and involvement with adjacent structures. Decisions on radiation therapy and chemotherapy should be made in a multidisciplinary setting at a tertiary referral center.

  8. Primary Ewing’s Sarcoma of the Kidney in a 73-Year-Old Man

    Directory of Open Access Journals (Sweden)

    T. B. Wedde

    2011-01-01

    Full Text Available Objective. Ewing’s sarcoma of the kidney is rare and is usually found in young adults. We present here a single case study of Ewing's sarcoma found in an elderly man. Material and methods. A 73-year-old man underwent routine surgery for hydrocoele of the testis. He developed urinary obstruction symptoms, and radiological examinations revealed a tumour in the right kidney. Results. Microscopical, immunohistochemical, and molecular pathological analysis of the tumour was consistent with Ewing's sarcoma. FISH showed rearrangement of chromosomes 22q12 (EWSR1. The patient subsequently underwent nephrectomy followed by 6 adjuvant chemotherapy cycles. Follow-up after 7 months shows no recurrence. Conclusion. This case report presents not only the rare finding of Ewing's sarcoma in the kidney, but also the occurrence of this tumour entity in an elderly patient. Treatment options for the different types of renal tumours are vastly different and the need for a correct diagnosis is, therefore, vital.

  9. What Is Kaposi Sarcoma?

    Science.gov (United States)

    ... Treatment? Kaposi Sarcoma About Kaposi Sarcoma What Is Kaposi Sarcoma? Cancer starts when cells in the body begin ... the lungs may cause trouble breathing. Types of Kaposi sarcoma The different types of KS are defined by ...

  10. Vascularized fibula grafts for reconstruction of bone defects after resection of bone sarcomas

    DEFF Research Database (Denmark)

    Petersen, Michael Mørk; Hovgaard, Dorrit; Elberg, Jens Jørgen

    2010-01-01

    We evaluated the results of limb-sparing surgery and reconstruction of bone defects with vascularized fibula grafts in 8 consecutive patients (mean age at operation 13.6 years (range 4.1-24.2 years), female/male = 6/2) with bone sarcomas (BS) (osteosarcoma/Ewing's sarcoma/chondrosarcoma= 4....../3/1) operated on form 2000 to 2006. The bone defects reconstructed were proximal femoral diaphysis and epiphysis (n = 2), humeral diaphysis (n = 2), humeral proximal diaphysis and epiphysis (n = 1), femoral diaphysis (n = 1), ulnar diaphysis (n = 1), and tibial diaphysis (n = 1). One patient with Ewing......'s sarcoma had an early hip disarticulation, developed multiple metastases, and died 9 months after the operation. The remaining patients (n = 7) are all alive 50 months (range 26-75 months) after surgery. During the follow-up the following major complications were seen: 1-2 fractures (n = 4), pseudarthrosis...

  11. Histiocytic sarcoma

    Directory of Open Access Journals (Sweden)

    Eduardo Silva Machado

    2011-01-01

    Full Text Available A 59-year-old white woman, SC, after being treated for pneumonia, presented with an increase in the size of lymph nodes. The immunohistochemical examination diagnosed histiocytic sarcoma. Relapse occurred 12 months after starting chemotherapy. The patient evolved with febrile neutropenia, septic shock and death.

  12. Postradiation sarcoma of bone: review of 78 Mayo Clinic cases

    Energy Technology Data Exchange (ETDEWEB)

    Weatherby, R.P.; Dahlin, D.C.; Ivins, J.C.

    1981-05-01

    Postradiation sarcoma of bone is an uncommon but serious sequela of radiation therapy. Seventy-eight Mayo Clinic patients have been treated for sarcomas arising in irradiated bones. They received their initial radiotherapy for a wide variety of nonneoplastic and neoplastic conditions, both benign and malignant. Thirty-five sarcomas arose in bone that was normal at the time of radiotherapy, and 43 arose in irradiated preexisting osseous lesions. The latent period between radiotherapy and diagnosis of sarcoma averaged 14.3 years. Ninety percent of the postradiation sarcomas were either osteosarcomas or fibrosarcomas; chondrosarcoma, malignant (fibrous) histiocytoma, malignant lymphoma, Ewing's tumor, and metastasizing chondroblastoma also occurred. Prompt radical surgery, when feasible, is usually the treatment of choice for the sarcoma. About 30% of patients with sarcomas of the extremities or craniofacial bones survived 5 years without recurrence; there were no disease-free survivors among patients with tumors of the vertebral column, pelvis, or shoulder girdle. The low risk of sarcoma following radiotherapy for the treatment of cancer should not be a contraindication to its use in these patients; however, radiation therapy for benign bone tumors should be reserved for lesions that are not amenable to surgical treatment. An unusual case is also reported herein in which a fibrosarcoma was discovered in the humerus of a patient who had received radiotherapy 55 years previously for a verified osteosarcoma in the same site.

  13. 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.

  14. Patología Molecular de los sarcomas

    OpenAIRE

    Álava, Enrique

    2005-01-01

    Los sarcomas de hueso y tejidos blandos son un grupo poco frecuente de tumores. Su prevalencia es inferior a 1 caso por 10000 habitantes, lo que los convierte en una enfermedad rara. Algunos de estos tumores, como el sarcoma sinovial, el tumor de Ewing o el osteosarcoma, son más habituales en los adolescentes o en los adultos jóvenes, mientras que existen neoplasias como el leiomiosarcoma o el liposarcoma, más frecuentes en pacientes de edad superior a los 55 años. Desde el punto de vista his...

  15. Pediatric Sarcoma in Central America: Outcomes, Challenges and Plans for Improvement

    Science.gov (United States)

    Friedrich, Paola; Ortiz, Roberta; Strait, Kelly; Fuentes, Soad; Gamboa, Yéssica; Arambú, Ingrid; Ah-Chu-Sanchez, María; London, Wendy; Rodríguez-Galindo, Carlos; Antillón-Klussmann, Federico; Báez, Fulgencio

    2012-01-01

    Background Children with cancer in middle-income countries have inferior outcomes to those in high-income countries. The magnitude and drivers for this survival gap are not well understood. We sought to describe patterns of clinical presentation, magnitude of treatment abandonment, and survival in children with sarcoma in Central America. Methods Retrospective review of hospital-based registries from national pediatric oncology referral centers. Patients with newly diagnosed osteosarcoma, Ewing sarcoma (Ewing), rhabdomyosarcoma (RMS), and soft tissue sarcomas (STS) between 1/1/00-12/31/09 were included. Survival analysis was performed using standard definitions of overall and event-free survival (OS and EFS) and with abandonment included as an event (AOS and AEFS). Results A total of 785 new cases of pediatric sarcoma were reported (264 osteosarcoma, 175 Ewing, 240 RMS, and 106 STS). Metastatic disease at presentation was high (osteosarcoma 38%, Ewing 39%, RMS 29% and STS 21%). Treatment abandonment rate was high, particularly among patients with extremity bone sarcomas (osteosarcoma 30%, Ewing 15%, RMS 25% and STS 15%). Of 559 patients experiencing a first event, 59% had either relapse or progressive disease. The 4-year OS was 40% (SE±3%) and EFS was 30% (SE±2%), but further decreased to 31% (SE±2%) and 24% (SE±2%), when abandonment was taken into account. Conclusion High rate of metastases and treatment abandonment, and difficulty with upfront treatment effectiveness are important contributors to poor survival of children with pediatric sarcomas in Central America. Initiatives for early diagnosis, psychosocial support, quality improvement, and multidisciplinary care are warranted to improve outcomes. PMID:22972687

  16. 经“Y”型软骨截骨髋臼挽救术治疗儿童和青少年TypeⅡ型骨盆尤文肉瘤%Acetabulum salvage by osteotomy through triradiate cartilage for surgical management of type Ⅱ pelvic Ewing sarcoma in children and adolescents

    Institute of Scientific and Technical Information of China (English)

    范宏斌; 王臻; 郭征; 付军; 吴志钢; 陈国景; 栗向东; 李靖

    2014-01-01

    sarcoma.Methods Data of 8 patients (male:6; female:2) with type Ⅱ pelvic Ewing sarcoma receiving treatment in our department from January 2001 to October 2010 were retrospectively analyzed.The mean age was 12.7 years (range,7-16 years).The tumors were located above,anteiro-inferior,and posterior-inferior to triradiate cartilage in 3,4,and 1 case,respectively.All tumors were localized and no metastasis was found.All patients received X-ray film,CT scan,and MRI.The diagnosis was determined by biopsy.All patients had undergone neoadjuvant chemotherapy and radiotherapy (post operation).All patients received 1 cycle of IFO-ADM+CDP-IFO and 2 cycles of same reagents before and after surgery.All patients received radio-therapy after surgery.In these consecutive patients,three received conventional surgery and another 5 were operated with the help of computer-assisted surgical navigation system.Results According to response evaluationg criteria in solid tumors (RECIST),CR was in 1 case,PR in 6 cases and SD in 1 case.No patient was recorded as PD.All patients were followed up for 12 to 72 months (mean,37.8 months).There were 7 patients surviving from 24 to 60 months (mean,39 months) with disease-free survival.One patient died of metastasis at 24 months postoperatively.The cumulative survival rate at 2 and 5 years were 100% and 87.5%,respectively.The MSTS score ranged from 18 to 27 (mean,25.4) and radiological score of International Society of Limb Salvage (ISOLS) ranged from 76% to 94% (mean,90.1%).Conclusion Osteotomy through acetabular triradiate cartilage in combination with allograft reconstruction can maximally restore the hip function and increase the survival rate.The triradiate cartilage may be a natural barrier to prevent tumor invasion.

  17. Horton and Ewing Medalists

    Science.gov (United States)

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

  18. Chemotherapy for Soft Tissue Sarcomas

    Science.gov (United States)

    ... Stage Soft Tissue Sarcoma Treating Soft Tissue Sarcomas 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 ...

  19. 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

  20. Do We Know What Causes Kaposi Sarcoma?

    Science.gov (United States)

    ... and Prevention Do We Know What Causes Kaposi Sarcoma? Kaposi sarcoma (KS) is caused by infection with a ... Sarcoma? Can Kaposi Sarcoma Be Prevented? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...

  1. Nonmetastatic Ewing’s Sarcoma of the Lumbar Spine in an Adult Patient

    Directory of Open Access Journals (Sweden)

    Maurizio Iacoangeli

    2012-01-01

    Full Text Available Although the spine is frequently involved in metastatic Ewing's sarcoma, primary involvement of the spine, beside sacrum, is much less frequent, especially in adult patients. Because of the low incidence of these tumors, there are currently no clinical guidelines outlining their management and a multitude of therapeutic strategies have been employed with varying success. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include the combination of three main modalities: aggressive surgery, radiotherapy, and combined chemotherapy. Whenever possible, en bloc spondylectomy or extralesional resection is preferable, providing a better oncological result with a longer survival and a better preservation of the spine biomechanics. This is the lesson we learned about the case, we present here, of nonmetastatic lumbar localization by Ewing’s sarcoma in as adult patient.

  2. Intracranial Ewings Sarcoma/peripheral primitive neuroectodermal tumor

    Directory of Open Access Journals (Sweden)

    R Amita

    2014-01-01

    Full Text Available Central nervous system primitive neuroectodermal tumours (CNS PNET are aggressive embryonal tumours composed of undifferentiated or poorly differentiated neuroepithelial cells seen in the pediatric age group. This is rare and only a handful of cases of ES/pPNET in CNS are reported. We report such a case in a 3 year old child. Reporting of more such cases is needed to better define these rare tumours of the dura.

  3. Immunostaining for SYT protein discriminates synovial sarcoma from other soft tissue tumors: analysis of 146 cases.

    Science.gov (United States)

    He, Rui; Patel, Rajiv M; Alkan, Serhan; Hammadeh, Rasheed; Weiss, Sharon W; Goldblum, John R; Venkataraman, Girish; Baila, Horea

    2007-05-01

    Synovial sarcoma in its classic biphasic form can be distinguished readily from other soft tissue lesions; however, monophasic and poorly differentiated forms are diagnostically more problematic. For this reason, we assessed the efficacy of immunostaining for SYT and SSX1 proteins, the gene products resulting from unique synovial sarcoma translocation, to distinguish synovial sarcoma from other soft tissue lesions. A total number of 146 cases were analyzed, including 47 synovial sarcoma cases (all of which were verified by FISH to have t(X; 18) translocation and SYT-SSX fusion gene) and 99 soft tissue tumors of various types. A polyclonal IgG antibody against SYT was used to stain formalin-fixed paraffin embedded tissues. Forty-one out of 47 (87%) synovial sarcoma displayed strong positive nuclear staining (ranging from 80 to 90% of the tumor cells) for SYT antibody. Nineteen of 99 (19%) non-synovial sarcoma cases showed variable nuclear and cytoplasmic staining with SYT, which ranged from 20 to 60% of tumor nuclei, and included malignant peripheral nerve sheath tumor (5/25), solitary fibrous tumor (2/14), Ewing sarcoma (2/6), low grade fibromyxoid tumor (2/4), extraskeletal mesenchymal chondrosarcoma (2/6), gastrointestinal tumor (4/17), epithelioid sarcoma (2/2). The remaining non-synovial sarcomas were negative. This is the first study demonstrating SYT protein expression in tissue sections of synovial sarcoma. This method could provide an easy, rapid and widely applicable means of assisting in the diagnosis of synovial sarcoma, particularly when material and/or resources are unavailable for PCR or FISH-based testing. However, as variable weak staining for SYT may be encountered in a small percentage of non-synovial sarcoma sarcomas, a positive interpretation should be made only when the staining is strong, nuclear and present in the majority of cells.

  4. 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.

  5. Workshop Report on the European Bone Sarcoma Networking Meeting: Integration of Clinical Trials with Tumor Biology.

    Science.gov (United States)

    Thomas, David M; Wilhelm, Miriam; Cleton-Jansen, Anne-Marie; Dirksen, Uta; Entz-Werlé, Natacha; Gelderblom, Hans; Hassan, Bass; Jürgens, Heribert; Koster, Jan; Kovar, Heinrich; Lankester, Arjan C; Lewis, Ian J; Myklebost, Ola; Nathrath, Michaela H M; Picci, Piero; Whelan, Jeremy S; Hogendoorn, Pancras C W; Bielack, Stefan S

    2011-09-01

    A key workshop was held in The Netherlands in June 2011, hosted by several European bone sarcoma networks and with a broad range of stakeholders from Europe and Australia. The purpose of the meeting was to identify the strengths and weaknesses in current clinical trials for bone sarcomas and to make recommendations as to how to accelerate progress in this field. Two areas of particular interest were discussed. First, all participants agreed upon the importance of tumor biology to understanding clinical responses for all types of bone sarcoma. Various barriers to biobanking tumor and germline specimens were canvassed and are outlined in this paper. Second, there was consideration of the particular challenges of dealing with adolescent and young adult cancers, exemplified by bone sarcomas. Participants recommended greater engagement of both pediatric and adult sarcoma trial organizations to address this issue. Specific opportunities were identified to develop biological sub-studies within osteosarcoma, focused on understanding germ line risk and pharmacogenomics defining toxicity and biological responses. In Ewing sarcoma, it was harder to define opportunities for biological insights. There was agreement that the results for insulin-like growth factor pathway inhibition in Ewing family tumors were disappointing, but represented a clear indication of the need for companion biologic studies to develop predictive biomarkers. The meeting ended with broad commitment to working together to make progress in this rare but important subgroup of cancers.

  6. 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.)

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

    Science.gov (United States)

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

    2012-06-01

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

  8. Breast sarcomas. Literature review

    Directory of Open Access Journals (Sweden)

    D. A. Ryabchikov

    2014-01-01

    Full Text Available The article presents an overview of the literature about breast sarcomas (nonepithelial malignances. Primary sarcomas are extremely rare, with less than 1 % of all malignant tumors of the breast. Breast carcinomas cause an increased interest of the scientists due to their unique clinical and pathological features and unpredictable prognosis.

  9. 耐药和复发性妊娠滋养细胞肿瘤的临床病例分析%Clinical analysis of patients with relapsed and chemo-resistant gestational trophoblastic neoplasia

    Institute of Scientific and Technical Information of China (English)

    周莹; 冯凤芝; 向阳; 万希润

    2010-01-01

    Objective To analyze and compare the clinical characteristics and the treatment outcome of the patients with chemo-resistant and relapsed gestational trophoblastic neoplasia (GTN).Methods The clinical records of the patients with refractory GTN treated at the Peking Union Medical College Hospital (PUMCH) from Jan 2005 to Dec 2007 were retrospectively reviewed.According to the reasons for referral, all cases were classified as chemo-resistant GTN group who had never a normal serum human chorionic gonadotropin-beta subunit (β-hCG) level during their previous treatment, relapsed GTN group who had elevated serum β-hCG levels in the absence of the pregnancy after finished treatment 3 months or more, and undetermined GTN group who had elevated serum β-hCG levels in the absence of the pregnancy less than 3 months after completed treatment.The clinical features and treatment outcomes were compared between undetermined GTN group and chemo-resistant GTN group and also between undetermined GTN group and relapsed GTN group, respectively.Results Of 81 patients with refractory GTN, 32 cases were defined as undetermined GTN, 38 cases as chemo-resistant GTN and 11 cases as relapsed GTN.The median number of previous chemotherapy regimens, the rate of serologic complete remission ( SCR), the patients who needed to change regimens due to resistance, and the patients who needed to change regimens in the undetermined GTN group and the chemo-resistant GTN group were 2.3 versus 3.1 ( P = 0.010),100% (32/32) versus 66% (25/38, P < 0.01 ), 22% (7/32) versus 58% (22/38, P = 0.002) and 28% (9/32) versus 63% (24/38, P = 0.003 ), respectively.No significant difference were observed between undetermined GTN group and relapsed GTN group in clinical features, previous and current treatment or treatment outcome ( all P >0.05 ).Conclusions In order to evaluate accurately the treatment outcome of refractory GTN, it seems more appropriate for the patients who had reached the normal value of

  10. CNS Metastases from Bone and Soft Tissue Sarcomas in Children, Adolescents, and Young Adults: Are They Really So Rare?

    Science.gov (United States)

    Duczkowska, Agnieszka; Duczkowski, Marek; Bragoszewska, Hanna; Romaniuk-Doroszewska, Anna; Iwanowska, Beata; Szkudlinska-Pawlak, Sylwia; Madzik, Jaroslaw; Bilska, Katarzyna; Raciborska, Anna

    2017-01-01

    Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas' metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas' CNS metastases. Methods. Patients with first diagnosis in 1999–2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI)/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded. Results. 550 patients were included. MRI revealed CNS metastases in 19 patients (incidence 3.45%), 14 boys, aged 5–22 years. There were 12/250 osteosarcoma cases, 2/200 Ewing's sarcoma, 1/50 chondrosarcoma, 3/49 rhabdomyosarcoma (RMS), and 1/1 malignant mesenchymoma. There were 10 single metastases and 7 cases of multiple ones; in 2 RMS cases only leptomeningeal spread in brain and spinal cord was found. Calcified metastases were found in 3 patients and hemorrhagic in 4. In one RMS patient there were numerous solid, cystic, hemorrhagic lesions and leptomeningeal spread. Conclusions. CNS metastases are rare and late in children with bone/soft tissue sarcomas, although in our material more frequent (3.45%) than in other reports (0.7%). Hematogenous spread to brain and hemorrhagic and calcified lesions dominated in osteosarcoma. Ewing sarcoma tended to metastasize to skull bones. Soft tissue sarcomas presented various morphological forms.

  11. 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.

  12. The Danish Sarcoma Database

    Directory of Open Access Journals (Sweden)

    Jorgensen PH

    2016-10-01

    Full Text Available Peter Holmberg Jørgensen,1 Gunnar Schwarz Lausten,2 Alma B Pedersen3 1Tumor Section, Department of Orthopedic Surgery, Aarhus University Hospital, Aarhus, 2Tumor Section, Department of Orthopedic Surgery, Rigshospitalet, Copenhagen, 3Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark Aim: The aim of the database is to gather information about sarcomas treated in Denmark in order to continuously monitor and improve the quality of sarcoma treatment in a local, a national, and an international perspective. Study population: Patients in Denmark diagnosed with a sarcoma, both skeletal and ekstraskeletal, are to be registered since 2009. Main variables: The database contains information about appearance of symptoms; date of receiving referral to a sarcoma center; date of first visit; whether surgery has been performed elsewhere before referral, diagnosis, and treatment; tumor characteristics such as location, size, malignancy grade, and growth pattern; details on treatment (kind of surgery, amount of radiation therapy, type and duration of chemotherapy; complications of treatment; local recurrence and metastases; and comorbidity. In addition, several quality indicators are registered in order to measure the quality of care provided by the hospitals and make comparisons between hospitals and with international standards. Descriptive data: Demographic patient-specific data such as age, sex, region of living, comorbidity, World Health Organization's International Classification of Diseases – tenth edition codes and TNM Classification of Malignant Tumours, and date of death (after yearly coupling to the Danish Civil Registration System. Data quality and completeness are currently secured. Conclusion: The Danish Sarcoma Database is population based and includes sarcomas occurring in Denmark since 2009. It is a valuable tool for monitoring sarcoma incidence and quality of treatment and its improvement, postoperative

  13. Soft Tissue Sarcoma

    Science.gov (United States)

    ... other body structures. The soft tissues include muscle, fat, blood vessels, nerves, tendons and the lining of your joints. Many types of soft tissue sarcoma exist. Some types are more likely to ...

  14. The Danish Sarcoma Database

    DEFF Research Database (Denmark)

    Jørgensen, Peter Holmberg; Lausten, Gunnar Schwarz; Pedersen, Alma B

    2016-01-01

    AIM: The aim of the database is to gather information about sarcomas treated in Denmark in order to continuously monitor and improve the quality of sarcoma treatment in a local, a national, and an international perspective. STUDY POPULATION: Patients in Denmark diagnosed with a sarcoma, both...... skeletal and ekstraskeletal, are to be registered since 2009. MAIN VARIABLES: The database contains information about appearance of symptoms; date of receiving referral to a sarcoma center; date of first visit; whether surgery has been performed elsewhere before referral, diagnosis, and treatment; tumor...... in order to measure the quality of care provided by the hospitals and make comparisons between hospitals and with international standards. DESCRIPTIVE DATA: Demographic patient-specific data such as age, sex, region of living, comorbidity, World Health Organization's International Classification...

  15. 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.

  16. What Are the Key Statistics about Kaposi Sarcoma?

    Science.gov (United States)

    ... Kaposi Sarcoma What Are the Key Statistics About Kaposi Sarcoma? Before the AIDS epidemic, Kaposi sarcoma (KS) was ... in Kaposi Sarcoma Research and Treatment? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...

  17. What's New in Kaposi Sarcoma Research and Treatment?

    Science.gov (United States)

    ... Kaposi Sarcoma About Kaposi Sarcoma What’s New in Kaposi Sarcoma Research and Treatment? A great deal of research ... in Kaposi Sarcoma Research and Treatment? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...

  18. Wastage of ova in young Merino ewes.

    Science.gov (United States)

    de Blockey, M A; Parr, R A; Restall, B J

    1975-06-01

    Eight sub-flocks each of 48 to 51 Merino ewes were mated for 18 days with 1 ram to each subflock. Four of the sub-flocks were of non-parous 1.5-year-old ewes and the other 4 were of parous 2.5-year-old ewes. Fertilisation rates for the subflocks of 1.5-year-old ewes varied from 83 to 94%, and the sub-flocks of 2.5-year-old ewes ranged from 81 to 94%. By day 29 post coitum 42% and 56% of 1.5-year-old ewes in sub-flocks 1 and 2 respectively were no longer pregnant. Embryonic mortality was low in other sub-flocks of 1.5- and 2.5-year-old ewes. Virtually all embryonic death occurred after day 12 post coitum. The sporadic occurrence of high ova wastage in maiden 1.5-year-old ewes in this experiment and in others is discussed in relation to the short duration of oestrus in young ewes and the great variation in service activity of rams.

  19. Vascularized fibula grafts for reconstruction of bone defects after resection of bone sarcomas

    DEFF Research Database (Denmark)

    Petersen, Michael Mørk; Hovgaard, Dorrit; Elberg, Jens Jørgen;

    2010-01-01

    We evaluated the results of limb-sparing surgery and reconstruction of bone defects with vascularized fibula grafts in 8 consecutive patients (mean age at operation 13.6 years (range 4.1-24.2 years), female/male = 6/2) with bone sarcomas (BS) (osteosarcoma/Ewing's sarcoma/chondrosarcoma= 4......'s sarcoma had an early hip disarticulation, developed multiple metastases, and died 9 months after the operation. The remaining patients (n = 7) are all alive 50 months (range 26-75 months) after surgery. During the follow-up the following major complications were seen: 1-2 fractures (n = 4), pseudarthrosis...... (n = 2), and hip dislocation (n = 1). Limb-sparing surgery with reconstruction of bone defects using vascularized fibular grafts in BS cases is feasible with acceptable clinical results, but fractures should be expected in many patients....

  20. Disseminated Intracranial Ewing’s Sarcoma in an Adult:A Rare and Difficult Diagnosis

    Directory of Open Access Journals (Sweden)

    Emil Lou

    2012-06-01

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

  1. Soft tissue reconstruction after lower extremity limb-sparing pediatric sarcoma resection

    Institute of Scientific and Technical Information of China (English)

    Kevin Shultz; Nicholas Webster; Miguel A Soto-Miranda; Anas Eid; Jon P Ver Halen

    2016-01-01

    Aim:Limb salvage is the treatment of choice for lower extremity bone sarcomas in children. To date, peers have not described algorithms for soft tissue reconstruction of these defects. This paper is to report a large single center series of lower extremity salvage after sarcoma treatment, with algorithm. Methods:The authors performed a retrospective review of patients undergoing resection of lower extremity bone sarcomas at a single center over 12 years. Results:In total, 65 children (29 girls, 36 boys) with a mean age of 13 years (range 2.9-23.3 years) underwent resection of a lower leg sarcoma with limb-salvage. Tumors types included 50 osteosarcomas, and 15 Ewing sarcoma family of tumors. The types of reconstruction utilized included:34 primary closures, 22 gastrocnemius and soleus lfaps, 3 bipedicled lfaps, 2 sural artery lfaps, 1 pedicled anterolateral thigh lfap, 3 pedicled posterior thigh lfaps for subsequent above-knee amputations. No free lfap based reconstructions were performed. An algorithm for reconstruction of leg defects in the setting of limb-salvage surgery is presented. Successful limb salvage rate was 95.4%. Limb salvage failed in 3 patients and they required amputation. Finally, 56 patients were able to ambulate without assistance at last follow-up. Conclusion:The authors present an algorithm for the reconstruction soft tissue after resection of lower extremity bone sarcomas. The use of these techniques helps to decrease complications and maximize function in children with these tumors.

  2. Treatment Option Overview (Kaposi Sarcoma)

    Science.gov (United States)

    ... Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  3. Cinnamaldehyde Derivative (CB-PIC Sensitizes Chemo-Resistant Cancer Cells to Drug-Induced Apoptosis via Suppression of MDR1 and its Upstream STAT3 and AKT Signalling

    Directory of Open Access Journals (Sweden)

    Jianzhong Xi

    2015-03-01

    Full Text Available Background/Aims: Our group reported that cinnamaldehyde derivative, (E-4-((2-(3-oxopop-1-enylphenoxymethylpyridinium malonic acid (CB-PIC induced apoptosis in hypoxic SW620 colorectal cancer cells via activation of AMP-activated protein kinase (AMPK and extracellular signal regulated kinase (ERK. Herein, sensitizing effect of CB-PIC was investigated in resistant cancer cells such as paclitaxel (PT resistant lung cancer cells (H460/PT, and Adriamycin (Adr resistant breast cancer (MCF7/Adr and colon cancer (HCT15/cos cells. Methods: Various drug resistant cell lines were treated with CB-PIC, and the signalling pathway and functional assay were explored by Western blot, Rhodamine assay, FACS, RT-PCR and MTT assay. Results: We found that CB-PIC effectively exerted cytotoxicity, increased sub G1 population and the cleaved form of poly (ADP-ribose polymerase (PARP and caspase 9 in drug resistant cancer cells. Furthermore, CB-PIC sensitized resistant cancer cells to adriamycin via downregulation of survival proteins such as survivin, Bcl-xL and Bcl-2, along with MDR1 suppression leading to accumulation of drug in the intracellular region. Of note, CB-PIC transcriptionally decreased MDR1 expression via suppression of STAT3 and AKT signalling in three resistant cancer cells with highly expressed P-glycoprotein. Nonetheless, CB-PIC did not affect transport activity of P-glycoprotein in a short time efflux assay, while epigallocatechin gallate (EGCG accumulated Rhodamine 123 into intracellular region of cell by direct inhibition of MDR1 transport activity. Conclusions: These data demonstrate that CB-PIC suppresses the P-glycoprotein expression through inhibition of STAT3 and AKT signalling to overcome drug resistance in chemo-resistant cancer cells as a potent chemotherapeutic sensitizer.

  4. Studies of pituitary function in lactating ewes.

    Science.gov (United States)

    Restall, B J; Kearins, R D; Starr, B G

    1977-03-01

    The release of LH from the pituitary of lactating ewes was studied. In Exp. 1, ewes were injected with 50 microng oestradiol benzoate (OB), 2-0 mg testosterone propionate (TP) or oil only (control) on days 5, 10, or 20 after lambing. LH was measured in peripheral plasma samples obtained 20-38 h after treatment, and the ovulations were recorded. The number of ewes in which an LH release was detected, and the amount released, declined between Day 5 and 20 after OB treatment but increased after TP treatment. The releases of LH were not always accompanied by ovulation and the incidence of ovulation was higher in ewes treated with TP. In Exp. 2, lactating ewes were injected with 1 or 5 (at 2-h intervals) doses of 50 microng Gn-RH, on Days 12 or 25 after lambing. LH was measured in peripheral plasma samples collected every 2 h for 10 h and every 3 h for a further 70 h. Release of LH occurred in all ewes, the amount being greater in ewes receiving multiple injections and in ewes treated on Day 25. The incidence of ovulation was higher after treatment on Day 25. Multiple injections of Gn-RH appeared to reduce the incidence of abnormal corpora lutea.

  5. [Moritz Kaposi and his sarcoma].

    Science.gov (United States)

    van Kessel, Anne; Quint, Koen D

    2011-01-01

    Nowadays, Kaposi sarcoma is a multidisciplinary condition, not only observed by dermatologists. Since the HIV epidemic in the 80s and 90s of the last century, more insight into the aetiology of Kaposi sarcoma has been acquired. However, this sarcoma had already been described in 1872 by a Hungarian dermatologist named Moritz Kaposi (1832-1902). Kaposi described the entity as 'idiopathic multiple pigmented sarcoma of the skin'. This entity was an extraordinary diagnosis at that time, mostly observed in Jewish or Mediterranean men. In 1912, 10 years after the death of Moritz Kaposi, the entity name was changed to Kaposi sarcoma.

  6. GLI1 is a central mediator of EWS/FLI1 signaling in Ewing tumors.

    Directory of Open Access Journals (Sweden)

    Jay Joo

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

  7. Microenvironmental targets in sarcoma

    Directory of Open Access Journals (Sweden)

    Monika eEhnman

    2015-11-01

    Full Text Available Sarcomas are rare malignant tumors affecting all age groups. They are typically classified according to their resemblance to corresponding normal tissue. Their heterogeneous features, for example in terms of disease-driving genetic aberrations and body location, complicate both disease classification and development of novel treatment regimens. Many years of failure of improved patient outcome in clinical trials has lead to the conclusion that novel targeted therapies are likely needed in combination with current multimodality regimens. Sarcomas have not, in contrast to the common carcinomas, been the subject for larger systematic studies on how tumor behavior relates to characteristics of the tumor microenvironment. There is consequently an urgent need for identifying suitable molecular targets, not only in tumor cells, but also in the tumor microenvironment. This review discusses preclinical and clinical data about potential molecular targets in sarcomas. Studies on targeted therapies involving the tumor microenvironment are prioritized. A greater understanding of the biological context is expected to facilitate more successful design of future clinical trials in sarcoma.

  8. 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....

  9. Leukosis/Sarcoma Group

    Science.gov (United States)

    The leukosis/sarcoma (L/S) group of diseases designates a variety of transmissible benign and malignant neoplasms of chickens caused by members that belong to the family Retroviridae. Because the expansion of the literature on this disease, it is no longer feasible to cite all relevant publications ...

  10. Epidemic Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  11. Classic Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  12. Extremity perfusion for sarcoma

    NARCIS (Netherlands)

    Hoekstra, Harald Joan

    2008-01-01

    For more than 50 years, the technique of extremity perfusion has been explored in the limb salvage treatment of local, recurrent, and multifocal sarcomas. The "discovery" of tumor necrosis factor-or. in combination with melphalan was a real breakthrough in the treatment of primarily irresectable ext

  13. Sarcoma Foundation of America

    Science.gov (United States)

    ... Google+ Twitter LinkedIn YouTube © 2017 Sarcoma Foundation of America | All Rights Reserved. | Terms of Use | Privacy Policy Website Design & Hosting by 270net Technologies, Inc. X - Enter Your Location - - or - Get your current location Home About Us History People Public Filings News & Media SFA in the ...

  14. Chemo Resistance of Breast Cancer Stem Cells

    Science.gov (United States)

    2006-05-01

    cell self-renewal pathways generates tumors driven by cells that maintain stem cell character- istics. Materials and Methods Dissociation of mammary...of America Q12) was placed s.c. on the back of the neck of the mouse by using a trocar , and 400 mammospheres were mixed with 2.5 105 normal human

  15. Chemo Resistance of Breast Cancer Stem Cells

    Science.gov (United States)

    2007-05-01

    Moreover, Pece et al, showed that inhibition of the Notch pathway in breast tumors with increased Notch activity can reduce the tumor growth (152... Pece S, Serresi M, Santolini E, Capra M, Hulleman E, Galimberti V, et al. Loss of negative regulation by Numb over Notch is relevant to human breast

  16. 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)

    2016-08-22

    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

  17. Radiation Therapy for Soft Tissue Sarcomas

    Science.gov (United States)

    ... Stage Soft Tissue Sarcoma Treating Soft Tissue Sarcomas Radiation Therapy for Soft Tissue Sarcomas Radiation therapy uses ... spread. This is called palliative treatment . Types of radiation therapy External beam radiation therapy: For this treatment, ...

  18. Vascularized Fibula Grafts for Reconstruction of Bone Defects after Resection of Bone Sarcomas

    Science.gov (United States)

    Petersen, Michael Mørk; Hovgaard, Dorrit; Elberg, Jens Jørgen; Rechnitzer, Catherine; Daugaard, Søren; Muhic, Aida

    2010-01-01

    We evaluated the results of limb-sparing surgery and reconstruction of bone defects with vascularized fibula grafts in 8 consecutive patients (mean age at operation 13.6 years (range 4.1–24.2 years), female/male = 6/2) with bone sarcomas (BS) (osteosarcoma/Ewing's sarcoma/chondrosarcoma= 4/3/1) operated on form 2000 to 2006. The bone defects reconstructed were proximal femoral diaphysis and epiphysis (n = 2), humeral diaphysis (n = 2), humeral proximal diaphysis and epiphysis (n = 1), femoral diaphysis (n = 1), ulnar diaphysis (n = 1), and tibial diaphysis (n = 1). One patient with Ewing's sarcoma had an early hip disarticulation, developed multiple metastases, and died 9 months after the operation. The remaining patients (n = 7) are all alive 50 months (range 26–75 months) after surgery. During the follow-up the following major complications were seen: 1-2 fractures (n = 4), pseudarthrosis (n = 2), and hip dislocation (n = 1). Limb-sparing surgery with reconstruction of bone defects using vascularized fibular grafts in BS cases is feasible with acceptable clinical results, but fractures should be expected in many patients. PMID:20490263

  19. Primary hepatic sarcomas: CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Yu, Ri-Sheng; Chen, Ying; Jiang, Biao; Wang, Liu-Hong [Zhejiang University School of Medicine, Department of Radiology, Hangzhou (China); Xu, Xiu-Fang [Zhejiang Medical College, Teaching and Research Group of Radiology, Hangzhou (China)

    2008-10-15

    Primary hepatic sarcomas are rare tumors that are difficult to diagnose clinically. Different primary hepatic sarcomas may have different clinical, morphologic, and radiological features. In this pictorial review, we summarized computed tomography (CT) findings of some relatively common types of hepatic sarcomas, including angiosarcoma, epithelioid hemangioendothelioma (EHE), liposarcoma, undifferentiated embryonal sarcoma (UES), leiomyosarcoma, malignant fibrous histiocytoma (MFH), and carcinosarcoma (including cystadenocarcinosarcoma). To our knowledge, hepatic cystadenocarcinosarcoma has not been described in the English literature. The CT findings in our case are similar to that of cystadenocarcinoma, a huge, multilocular cystic mass with a large mural nodule and solid portion. The advent of CT has allowed earlier detection of primary hepatic sarcomas as well as more accurate diagnosis and characterization. In addition, we briefly discuss the MRI findings and diagnostic value of primary hepatic sarcomas. (orig.)

  20. What Happens After Treatment for Ewing Tumors?

    Science.gov (United States)

    ... Ewing tumors can cause leukemia in a small fraction of children later on. If this occurs, it ... Cancer Atlas Press Room Cancer Statistics Center Volunteer Learning Center Follow Us Twitter Facebook Instagram Cancer Information, ...

  1. ewes

    Directory of Open Access Journals (Sweden)

    M. R. Dally

    2004-01-01

    Full Text Available Con el fin de determinar la tasa de ovulación, parición, tamaño de la camada y supervivencia embrionaria, 225 borregas de siete genotipos se examinaron mediante endoscopio 7-8 días después de su cruza. Como resultado se encontró que un mayor número de ovulaciones unilaterales sencillas y dobles procedieron del ovario derecho (P0.05. Por otra parte, no se encontró diferencia en la supervivencia embrionaria (P>0.01 al comparar entre ovulaciones sencillas, dobles y triples (69.8%, 76.0% y 63.0%, respectivamente ni entre las ovulaciones unilaterales y bilaterales. La línea cuatera Targhee seleccionada (TW produjo las camadas más numerosas (media = 2.0±1.4 mientras que el rebaño Targhee comercial produjo las menos numerosas. La línea ½ Targhee, ¼ Barbados, ¼ Dorset mostró la tasa de parición mayor (94%, seguida de la Polypay (87%, sin encontrarse diferencia (P>0.05 entre estas dos líneas. Se concluye que: el ovario derecho es más activo que el izquierdo en cuanto a la producción de óvulos, de entre los genotipos estudiados; las ovulaciones dobles fueron más frecuentes que las sencillas y triples, y la línea cuatera Targhee seleccionada produjo las camadas más numerosas en comparación con el hato comercial Targhee o sus cruzas.

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

    Science.gov (United States)

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

    1998-05-01

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

  3. Comparison of energy rationing systems for late gestation ewes: Impacts on ewe and lamb performance.

    Science.gov (United States)

    Campion, F P; McGovern, F M; Lott, S; Fahey, A G; Creighton, P; Boland, T M

    2016-08-01

    The objectives of this study were 1) to compare the effects of a ME () or a NE () system for rationing ewes during late gestation on ewe and progeny performance and 2) to investigate incremental increases in NE allocation above 100% of recommendation during late gestation on ewe and progeny performance. Fifty-two twin-bearing ewes ( = 13 per treatment) were rationed to either 100% of recommended ME requirements (100% ME) or 100, 110, or 120% of recommended NE requirements (100% NE, 110% NE, and 120% NE) from d 112 of gestation to parturition. Mean energy intake, measured as ME and NE, from Day 112 of gestation to parturition was higher in all NE treatments compared with 100% ME ewes ( = 0.01). Ewes offered the 3 NE treatments had a higher live weight at parturition compared with 100% ME ewes ( = 0.02), with 100% NE and 120% NE ewes still being heavier than 100% ME ewes at 35 d postpartum ( = 0.02). Increasing NE allowance resulted in a linear decrease in the level of BCS loss prepartum ( = 0.01) and a linear increase in the level of BCS loss postpartum ( = 0.01). There was no difference observed between any of the treatments in total colostrum produced to 18 h postpartum ( = 0.29) or in total colostrum intake to 18 h postpartum ( = 0.27). Increasing maternal NE allowance led to a linear increase in lamb serum IgG concentration at 24 h postpartum ( = 0.03). The estimated milk production of 120% NE ewes tended to be higher than all other treatments at wk 6 of lactation ( = 0.08). Colostral SFA levels from 100% ME ewes was lower than that of all 3 NE treatments ( = 0.01), and unsaturated fatty acid (UFA) levels in colostrum of the 100% ME ewes were lower than those of the 100% NE ewes ( = 0.01). Cumulative levels of milk SFA, UFA, and MUFA did not differ between treatments ( = 0.19). Lamb growth rates during the first 5 wk postpartum were unaffected by treatment ( = 0.18) as were days to slaughter ( = 0.34). It can be concluded that both ME and NE systems used in this

  4. Sex of littermate twin affects lifetime ewe productivity

    Science.gov (United States)

    Ewe productivity is synonymous with annual litter-weight weaned (LWW) per ewe exposed to rams for breeding, and LWW is largely a function of number of lambs born (NLB) and weaned (NLW). Selecting for LWW should increase litter size and numbers of ewe-ram co-twins. Thus, we used historical records to...

  5. [Synovial sarcoma. Case report].

    Science.gov (United States)

    Deme, Dániel; Abdulfatah, Bishr; Telekes, András

    2016-02-07

    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

  6. 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.

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

    Science.gov (United States)

    ... What Should You Ask Your Doctor About Kaposi Sarcoma? Kaposi Sarcoma Early Detection, Diagnosis, and Staging What Should You Ask Your Doctor About Kaposi Sarcoma? As you cope with Kaposi sarcoma (KS) and ...

  8. 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.

  9. Hypocalcemia in ewes after a drought

    Energy Technology Data Exchange (ETDEWEB)

    Larsen, J.W.A.; Constable, P.D.; Napthine, D.V.

    1986-01-01

    A marked increase in the incidence of hypocalcemia of ewes 2 to 6 weeks before lambing was observed in the Western District of Victoria, following the break of the severe 1982/3 drought. A similar observation was made after the 1968 drought. In western Victoria, hypocalcemia is usually seen annually as sporadic cases or in sporadic outbreaks associated with some predisposing stress. After the drought broke in 1983, many farms reported cases of hypocalcemia in ewes. The incidence of hypocalcemia on the 9 farms the authors studied varied from 1 to 8% of all ewes, with some mobs having an incidence of over 10%. Detailed investigation of 9 farms that affected sheep were grazing pasture of unusually low calcium (Ca) content. Near record rains fell after the drought broke in late March 1983 resulting in luxuriant pasture growth with subterranean clover Trifolium subterraneum and capeweed Arctotheca calendula the dominant species. Cases of hypocalcemia commenced in May 1983 reaching a peak in June-July corresponding with flocks' lambing times, and continued into August. Most occurred spontaneously in mature ewes. A few farms experienced many cases during prelambing crutching.

  10. Myeloid Sarcoma in the Orbit.

    Science.gov (United States)

    Qian, Xiaoxiao; Gigantelli, James W; Abromowitch, Minnie; Morgan, Linda A; Suh, Donny W

    2016-12-08

    The authors describe a case of myeloid sarcoma of the orbit in a pediatric patient. An 8-month-old male infant presented to the ophthalmology clinic with a left orbital mass, which had been increasing in size over the previous 2 months. The mass was initially diagnosed at another clinic as an infantile hemangioma, and had been treated with a topical formulation of timolol. In the ophthalmology clinic, orbital magnetic resonance imaging showed a solid enhancing mass. A biopsy was performed, and histopathology revealed myeloid sarcoma. The disease responded well to a standard chemotherapy regimen. Myeloid sarcoma is a rare, extra-medullary presentation that can occur as an isolated tumor, concurrently with or at relapse of acute myeloid leukemia. Because few cases of myeloid sarcoma in the orbit have been reported, this case report aids in the management of myeloid sarcoma in pediatric patients. The report describes an 8-month-old male infant, the youngest patient to develop myeloid sarcoma without preexisting acute myeloid leukemia. [J Pediatr Ophthalmol Strabismus. 2016;53:e64-e68.].

  11. 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

  12. 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 ...

  13. Low pasture allowance until late gestation in ewes: behavioural and physiological changes in ewes and lambs from lambing to weaning.

    Science.gov (United States)

    Freitas-de-Melo, A; Ungerfeld, R; Hötzel, M J; Orihuela, A; Pérez-Clariget, R

    2017-02-01

    Low pasture allowance during gestation affects ewes' BW at parturition, the bond with their lamb, lamb development, and thus also may affect their responses to weaning. The objectives were to determine if native pasture allowance from before conception until late pregnancy affects ewe-lamb behaviours at lambing, ewes' milk yield, lambs' BW, and the behavioural and physiological changes of ewes and lambs at weaning. From 23 days before conception until 122 days of pregnancy, 24 ewes grazed on two different native pasture allowances: high (10 to 12 kg of dry matter (DM)/100 kg of BW per day; HPA treatment; n=12) or low (5 to 8 kg of DM/100 kg of BW per day; LPA treatment; n=12). Thereafter, all ewes grazed on Festuca arundinacea and received rice bran and crude glycerine. Ewes' body condition score (BCS) and BW were recorded during pregnancy and postpartum periods. Milk yield was determined on days 32, 41 and 54 after lambing. Lambs' BW was recorded from birth until 72 days after lambing. Latency from parturition until the ewe licked her lamb, maternal behaviour score (a test that evaluates maternal attachment to the lamb) and latency for lamb to stand up and suckle were determined. The behaviour of the lambs and ewes was recorded before and after weaning (at 65 days). The ewes' serum total protein, albumin and globulin concentrations were measured before and after weaning. The HPA ewes presented greater BW (Plate pregnancy, this did not affect the behaviours that lead to the establishment of the mother-young bond, nor the ewes' behavioural responses at weaning. Lambs reared by ewes that grazed on low pasture allowance during pregnancy presented fewer behavioural changes and a lower decrease of albumin concentration after weaning. Lambs' BW was not affected by the feeding received by their mothers.

  14. A RARE CASE OF ATYPICAL PRIMARY EWING’S SARCOMA OF OCCIPITAL BONE

    Directory of Open Access Journals (Sweden)

    K. Srihari

    2016-07-01

    Full Text Available BACKGROUND Ewing’s Sarcoma is an aggressive malignant neoplasm most frequently manifesting in the second decade of life and accounting for 4% of childhood and adolescent malignancies. These tumours were first described by James Ewing in 1921 as tumours that arise from bone. These osseous lesions have since become infamous for their highly aggressive course with 20% to 30% of patients having evidence of metastasis at the time of diagnosis and an estimated 10-year survival rate of 50%. Metastases to the CNS have most recently been estimated to occur in less than 5% of cases and are usually due to direct extension of an osseous lesion into the extradural space or more rarely through haematogenous spread. CASE REPORT In this article, we report a case of 17-year-old boy who presented to the radiology department with complaints of recurrent episodes of headache and vomiting for the past 3 days. On radiological investigation, there was a large well-defined, lobulated, extra-axial mass lesion measuring 3.6 X 5.7 X 5.9 cm noted in the supratentorial left occipital region which was fairly enhancing after contrast administration. The mass was causing permeative type of destruction of the left occipital bone and extending into extracranial soft tissue. Final diagnosis was done by biopsy and histopathology which showed “Atypical Ewing’s Sarcoma” of the left occipital bone. Considering its unusual site and soft tissue extension, we report this case of Primary Atypical Ewing’s sarcoma of occipital bone. CONCLUSION Primary cranial Ewing's sarcoma is to be considered in the differential diagnosis in children with a tumour involving the skull with destruction of the bone and presence of extra-axial soft tissue swelling. CT is the excellent modality for demonstration of bone destruction while MRI depicts soft tissue extension and metastasis if any.

  15. A case of primary mediastinal Ewing′s sarcoma /primitive neuroectodermal tumor presenting with initial compression of superior vena cava

    Directory of Open Access Journals (Sweden)

    Alessia Reali

    2013-01-01

    Full Text Available Ewing′s sarcomas and peripheral primitive neuroectodermal tumors (ES/PNETs are high grade malignant neoplasms. These malignancies are characterized by a chromosome 22 rearrangement, arise from bone or soft tissue, predominantly affect children and young adults, and are grouped in the Ewing family of tumors. Multimodality treatment programs are the treatment of choice. Primary localization of ES/PNET in the mediastinum is extremely rare. We describe a case of ES/PNET presenting as a mediastinal mass with tracheal compression and initial signs of superior vena cava in a 66-year-old woman.

  16. Energy and protein nutrition of ewes in late pregnancy

    OpenAIRE

    Hallfríður Ósk Ólafsdóttir 1978

    2012-01-01

    Feeding of the pregnant ewe affects its weight and condition and subsequently the ability to supply the lambs with adequate nutrition. Supplementing is commonly used to secure adequate birth weight and growth rate but for efficient use the condition of the ewes as well as total composition of the diet has to be considered thoroughly. In the experiment described here the effect of feeding different concentrates during the last month prepartum on ewe and lambs health and performa...

  17. Skin Ultrasound in Kaposi Sarcoma.

    Science.gov (United States)

    Carrascosa, R; Alfageme, F; Roustán, G; Suarez, M D

    2016-05-01

    The use of ultrasound imaging has recently been increasing in numerous dermatologic diseases. This noninvasive technique provides additional details on the structure and vascularization of skin lesions. Kaposi sarcoma is a vascular tumor that typically arises in the skin and mucosas. It can spread to lymph nodes and internal organs. We performed B-mode and color Doppler ultrasound studies in 3 patients with a clinical diagnosis of Kaposi sarcoma confirmed by histological examination. We found differences in the ultrasound pattern between nodular and plaque lesions, in both B-mode and color Doppler. We believe that skin ultrasound imaging could be a useful technique for studying cutaneous Kaposi sarcoma, providing additional information on the structural and vascular characteristics of the lesion.

  18. Clinical Benefit of Second-Line Palliative Chemotherapy in Advanced Soft-Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Anna Minchom

    2010-01-01

    Full Text Available Background. This paper aimed to assess the utility of second-line chemotherapy in patients with advanced soft-tissue sarcoma. Materials and Methods. A retrospective search of a prospectively maintained database identified patients treated between 1991 and 2005. Patients with gastrointestinal stromal tumours, small round cell tumours, and Ewing's sarcoma were excluded. Response was assessed using WHO and RECIST. Patients who achieved stable disease for 6 months or more were classified as having disease control. Results. Three hundred and seventy-nine patients received second-line chemotherapy. Eighty-six (22.7% achieved disease control. Median duration of response was 11 months (95% CI: 9–13. On multivariate analysis, pathological subtype, absence of lung metastases, and the use of combination chemotherapy were independent predictors of disease control. Twenty-eight (16.1% patients who failed to respond to first-line therapy achieved disease control. Eight (2.1% patients had sufficient downstaging to enable complete surgical resection. Progression-free survival was 23% at 6 months. Median overall survival was 8 months (95% CI: 7–10 months. On multivariate analysis, synovial histology and absence of lung metastases were associated with improved survival. Conclusion. Second-line chemotherapy can provide clinical benefit in over 20% of soft-tissue sarcoma patients.

  19. Mast cell sarcoma: clinical management.

    Science.gov (United States)

    Weiler, Catherine R; Butterfield, Joseph

    2014-05-01

    Mast cell sarcoma is a disorder that results in abnormal mast cells as identified by morphology, special stains, and in some publications, c-kit mutation analysis. It affects animal species such as canines more commonly than humans. In humans it is a very rare condition, with variable clinical presentation. There is no standard therapy for the disorder. It can affect any age group. It is occasionally associated with systemic mastocytosis and/or urticaria pigmentosa. The prognosis of mast cell sarcoma in published literature is very poor in humans.

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

    DEFF Research Database (Denmark)

    Mörse, Helena; Elfving, Maria; Turkiewicz, Aleksandra;

    2016-01-01

    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...

  1. 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.

  2. CNS Metastases from Bone and Soft Tissue Sarcomas in Children, Adolescents, and Young Adults: Are They Really So Rare?

    Directory of Open Access Journals (Sweden)

    Monika Bekiesinska-Figatowska

    2017-01-01

    Full Text Available Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas’ metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas’ CNS metastases. Methods. Patients with first diagnosis in 1999–2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded. Results. 550 patients were included. MRI revealed CNS metastases in 19 patients (incidence 3.45%, 14 boys, aged 5–22 years. There were 12/250 osteosarcoma cases, 2/200 Ewing’s sarcoma, 1/50 chondrosarcoma, 3/49 rhabdomyosarcoma (RMS, and 1/1 malignant mesenchymoma. There were 10 single metastases and 7 cases of multiple ones; in 2 RMS cases only leptomeningeal spread in brain and spinal cord was found. Calcified metastases were found in 3 patients and hemorrhagic in 4. In one RMS patient there were numerous solid, cystic, hemorrhagic lesions and leptomeningeal spread. Conclusions. CNS metastases are rare and late in children with bone/soft tissue sarcomas, although in our material more frequent (3.45% than in other reports (0.7%. Hematogenous spread to brain and hemorrhagic and calcified lesions dominated in osteosarcoma. Ewing sarcoma tended to metastasize to skull bones. Soft tissue sarcomas presented various morphological forms.

  3. 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.)

  4. Kaposi sarcoma associated with lipoedema.

    Science.gov (United States)

    Ekmekci, T R; Ayabakan, O; Sakiz, D; Koslu, A

    2005-05-01

    Lipoedema is a form of lipodistrophy, which consists of abnormal accumulation of fat in subcutaneous tissue of the lower limbs. It does not cause any disease and it has not been reported association with malignity. We describe a 63-year-old woman occurring of Kaposi sarcoma on the lipoedema base.

  5. Treatment Options for Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  6. General Information about Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  7. Potential Therapeutic Targets in Uterine Sarcomas

    OpenAIRE

    2015-01-01

    Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undi...

  8. Kaposi sarcoma: review and medical management update.

    Science.gov (United States)

    Fatahzadeh, Mahnaz

    2012-01-01

    Despite recent advances in our understanding of pathogenic mechanisms involved, the true nature of Kaposi sarcoma remains an enigma. Four clinical variants have been described for the disease, differing in natural history, site of predilection, and prognosis. All forms of Kaposi sarcoma may manifest in the oral cavity and Kaposi sarcoma-associated virus appears essential to development of all clinical variants. The spectrum of therapeutic strategies is broad and selection of appropriate intervention mandates a thorough understanding of disease spread and the patient's symptomatology, as well as risks and benefits of therapy. This article provides an overview of epidemiology, subtypes, clinical course, pathogenesis, and management strategies for Kaposi sarcoma.

  9. 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.

  10. 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.

  11. Primary Establishment of EWE Model in Caohai Nature Reserve

    Institute of Scientific and Technical Information of China (English)

    2011-01-01

    According to environmental data,Ecopath with Ecosim (EWE) model can quantitatively describe the energy flow in the production and consumption of function components of system by using trophodynamics,and accurately assess the biomass and stable state of aquatic ecosystem.In the paper,the basic principle and parameters of EWE model were introduced firstly,and the relationship between Q/B (the important parameter of EWE model) and basic life indices of fish was discussed,then the current study and typical r...

  12. The management of clear cell sarcoma

    NARCIS (Netherlands)

    Kuiper, DR; Hoekstra, HJ; Veth, RPH; Wobbes, T

    2003-01-01

    Clear cell sarcoma is a rare soft tissue tumour, constituting approximately 1% of all soft tissue sarcomas. Prognosis is reported to be poor due to the great propensity to metastasise regionally and distantly. In this paper, we report the surgical experience of two university hospitals. Both disease

  13. 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.

  14. Gastrointestinal Kaposi sarcoma with appendiceal involvement.

    Science.gov (United States)

    Egwuonwu, Steve; Gatto-Weis, Cara; Miranda, Roberto; Casas, Luis De Las

    2011-04-01

    Kaposi sarcoma is a vascular tumor manifesting as nodular lesions on skin, mucous membranes, or internal organs. This is a case of a 42-year-old human immunodeficiency virus- (HIV) positive bisexual male, not on highly active antiretroviral therapy (HAART) since diagnosis four years ago. He presented with a three-day history of abdominal pains, fever, vomiting, and a one-week history of melena stools. Endoscopy revealed Kaposi sarcoma in the stomach and duodenum. Postendoscopy, he developed acute abdomen. Exploratory laparotomy revealed extensive Kaposi sarcoma of the gastrointestinal tract with appendiceal involvement. The patient underwent appendectomy and had an uneventful recovery. A review of the literature discusses appendiceal Kaposi sarcoma with appendicitis, a rare but critical manifestation of gastrointestinal Kaposi sarcoma.

  15. Multiple trait genetic evaluation of ewe traits in Icelandic sheep.

    Science.gov (United States)

    Arnason, T; Jónmundsson, J V

    2008-12-01

    The prolificacy of the ewes was measured as the number of lambs born per ewe mated (NLB) when the ewes were 1-4 years of age. The ewe productivity related to the same age interval was measured by special ewe production indices (EPI). The genetic parameters for these traits were estimated by a series of bivariate REML analyses using animal models. The material used for the genetic analysis contained records on 193,213 ewes. The heritability estimates for NLB were h(2) = 0.17, 0.13, 0.11, 0.10 for the four respective age classes. Corresponding estimates for EPI were h(2) = 0.16, 0.17, 0.17, 0.15. The genetic correlations among NLB at different ages ranged from 0.63 to 0.98 and among EPI from 0.82 to 0.99. The genetic correlations between NLB and EPI were generally low. The material used for estimating the breeding values by the MT-BLUP Animal Model consisted of 1.5 million individuals in the pedigree file. In total 815,782 ewes had records for the NLB and 763,491 ewes had production index (at least 1 year). The records were registered in the years 1990-2006. All possible missing patterns were present in the data. In the iteration process expected values for missing traits were generated and solutions were obtained on canonical transformed scale. The genetic evaluations were run independently for NLB and EPI for computational convenience given the correlations between these traits were negligible.

  16. Undernutrition affects embryo quality of superovulated ewes.

    Science.gov (United States)

    Abecia, J A; Forcada, F; Palacín, I; Sánchez-Prieto, L; Sosa, C; Fernández-Foren, A; Meikle, A

    2015-02-01

    To determine the effect of undernutrition on embryo production and quality in superovulated sheep, 45 ewes were allocated into two groups to be fed diets that provided 1.5 (control, C; n = 20) or 0.5 (low nutrition, L; n = 25) times daily requirements for maintenance, from oestrous synchronization with intravaginal sponges to embryo collection. Embryos were collected 7 days after the onset of oestrus (day 0). Low nutrition resulted in lower live weight and body condition at embryo collection (P < 0.05). Diet (P < 0.01) and day of sampling (P < 0.001) significantly affected plasma non-esterified fatty acid (NEFA) and insulin concentrations. Plasma leptin concentrations decreased on day 7 only in L ewes. A significant effect of dietary treatment (P < 0.05) and day (P < 0.0001) was observed on plasma insulin-like growth factor (IGF)-I concentrations. The number of recovered oocytes and embryos did not differ between the groups (L: 15.4 ± 0.4; C: 12.4 ± 0.4). Recovery rate was lower (P < 0.05) in the L (60%) than in the C group (73%). The total number of embryos and number of viable-transferable embryos (5.0 ± 0.3 and 3.4 ± 0.3 embryos, respectively) of the L group were lower (P < 0.1) when compared with controls (8.4 ± 0.4 and 6.2 ± 0.4 embryos, respectively). Undernutrition during the period of superovulation and early embryonic development reduced total and viable number of embryos. These effects might be mediated by disruption of endocrine homeostasis, oviduct environment and/or oocyte quality.

  17. 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.

  18. Lipid requirements in the nutrition of dairy ewes

    Directory of Open Access Journals (Sweden)

    Andrea Serra

    2010-01-01

    Full Text Available The aim of this review was to contribute to the knowledge of lipid requirements in dairy ewes, by reviewing experi- mental papers about lipid supplementation in dairy ewe feeding. The number of trials in ewe feeding is lower than that in dairy cow feeding and, leaving calcium soap of palm oil out of consideration, there is a lack of knowledge regarding the effects of protected and unprotected lipid sources on milk yield and quality from dairy ewes. On the basis of data reported in the literature, the optimal dose of calcium soap of palm oil resulted to be 100-120 g/d. Also, milk fatty acid composition may be improved by adding calcium soap of fatty acids to ewe diets. The general effect of calcium salt sup- plementation is an increase in milk unsaturated fatty acids and a decrease in saturated ones. However, more research is needed in order to explain the effect of different fat sources (protected and unprotected on milk yield and quality from dairy ewes.

  19. ESF-EMBO Symposium Molecular Biology and Innovative Therapies in Sarcomas of Childhood and AdolescenceSept 29 – Oct 4, Polonia Castle Pultusk, Poland

    Directory of Open Access Journals (Sweden)

    Beat W Schäfer

    2013-06-01

    Full Text Available Rhabdomyosarcoma (RMS and Ewing sarcoma (ES are among the most common pediatric sarcomas (Arndt et al., 2012. Despite sarcomas representing a highly heterogeneous group of tumors, ES and alveolar RMS (ARMS typically share one common genetic characteristic, namely a specific chromosomal translocation (Helman and Meltzer, 2003; Lessnick and Ladanyi, 2012. These translocations generate fusion proteins, which are composed of two transcription factors (TF. Typically, one TF is a developmentally regulated factor that is essential for proper specification of a given lineage and provides the DNA-binding domain, while the partner TF contributes a transactivation domain that drives aberrant expression of target genes. Based on these common genetic characteristics, the first ESF-EMBO research conference entitled "Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence" with special focus on RMS and ES was held at the Polonia Castle in Pultusk, Poland. The conference gathered 70 participants from more than 15 countries and several continents representing most research groups that are active in this field.

  20. ESF-EMBO Symposium “Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence” Sept 29–Oct 4, Polonia Castle Pultusk, Poland

    Science.gov (United States)

    Schäfer, Beat W.; Koscielniak, Ewa; Kovar, Heinrich; Fulda, Simone

    2013-01-01

    Rhabdomyosarcoma (RMS) and Ewing sarcoma (ES) are among the most common pediatric sarcomas (Arndt et al., 2012). Despite sarcomas representing a highly heterogeneous group of tumors, ES and alveolar RMS (ARMS) typically share one common genetic characteristic, namely a specific chromosomal translocation (Helman and Meltzer, 2003; Lessnick and Ladanyi, 2012). These translocations generate fusion proteins, which are composed of two transcription factors (TF). Typically, one TF is a developmentally regulated factor that is essential for proper specification of a given lineage and provides the DNA-binding domain, while the partner TF contributes a transactivation domain that drives aberrant expression of target genes. Based on these common genetic characteristics, the first ESF-EMBO research conference entitled “Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence” with special focus on RMS and ES was held at the Polonia Castle in Pultusk, Poland. The conference gathered 70 participants from more than 15 countries and several continents representing most research groups that are active in this field. PMID:23761860

  1. ESF-EMBO Symposium "Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence" Sept 29-Oct 4, Polonia Castle Pultusk, Poland.

    Science.gov (United States)

    Schäfer, Beat W; Koscielniak, Ewa; Kovar, Heinrich; Fulda, Simone

    2013-01-01

    Rhabdomyosarcoma (RMS) and Ewing sarcoma (ES) are among the most common pediatric sarcomas (Arndt et al., 2012). Despite sarcomas representing a highly heterogeneous group of tumors, ES and alveolar RMS (ARMS) typically share one common genetic characteristic, namely a specific chromosomal translocation (Helman and Meltzer, 2003; Lessnick and Ladanyi, 2012). These translocations generate fusion proteins, which are composed of two transcription factors (TF). Typically, one TF is a developmentally regulated factor that is essential for proper specification of a given lineage and provides the DNA-binding domain, while the partner TF contributes a transactivation domain that drives aberrant expression of target genes. Based on these common genetic characteristics, the first ESF-EMBO research conference entitled "Molecular Biology and Innovative Therapies in Sarcomas of Childhood and Adolescence" with special focus on RMS and ES was held at the Polonia Castle in Pultusk, Poland. The conference gathered 70 participants from more than 15 countries and several continents representing most research groups that are active in this field.

  2. Effect of ewe prolificacy potential and stocking rate on ewe and lamb performance in a grass-based lamb production system.

    Science.gov (United States)

    Earle, E; McHugh, N; Boland, T M; Creighton, P

    2017-01-01

    The objective of the present study was to investigate the effect of ewe prolificacy potential (PP; predicted number of lambs born per ewe per year) as dictated by sire breed type, stocking rate (SR; ewes per ha), and their interaction on ewe and lamb performance in a temperate grass-based lamb production system. The study was a 2 × 3 factorial design, consisting of 2 differing ewe PP and 3 SR which included 180 medium prolificacy potential (MP- Suffolk-sired crossbred ewes) and 180 high prolificacy potential ewes (HP- Belclare-sired crossbred ewes) allocated to 1 of 3 ( = 60 ewes) SR: low (LSR; 10 ewes per ha), medium (MSR; 12 ewes per ha) or high (HSR: 14 ewes per ha). Each treatment was managed in a 5-paddock rotational grazing system for the duration of the study. Medium prolificacy ewes were consistently heavier ( < 0.001) compared to HP ewes, with HP ewes having a higher BCS at lambing and 6 wk post-lambing (PL; < 0.05). Low SR ewes had a higher BW ( < 0.05) and BCS ( < 0.05) at mating, 6 wk PL, and weaning relative to MSR and HSR ewes which did not differ from each other. Lambs born to MP ewes were heavier at birth and weaning ( < 0.001) and achieved a higher ADG from birth to weaning ( < 0.05). Ewe PP had no effect on lifetime ADG or d to slaughter (DTS) with HP lambs yielding a higher carcass weight ( < 0.001). Low SR and MSR lambs achieved higher ADG from birth to weaning ( < 0.001) and weaning weight ( < 0.001) relative to HSR lambs and did not differ from each other, while post-weaning and lifetime lamb ADG was highest at the LSR, intermediate at the MSR, and lowest at the HSR ( < 0.001). A ewe PP by SR interaction existed for DTS, with MP lambs at the LSR reaching slaughter weight earlier ( < 0.01) relative to HP lambs, while at the MSR and HSR, MP and HP lambs did not differ from each other. High PP ewes produced a higher average born ( < 0.001) and weaned litter size per ewe ( < 0.01), with live weight weaned per ha ( < 0.001) increasing as ewe PP

  3. Primary osteogenic sarcoma of the breast

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    Akang Effiong E

    2006-12-01

    Full Text Available Abstract Background Primary extra-osseous osteogenic sarcomas have been reported in many tissues of the body but their occurrence in the breast is extremely rare. It can arise as a result of osseous metaplasia in a pre-existing benign or malignant neoplasm of the breast or as non-phylloides sarcoma from the soft tissue of a previously normal breast. Case presentation A 40 year-old Nigerian woman was clinically diagnosed to have carcinoma of the left breast. The histology report of core-needle biopsy of the mass showed a malignant neoplasm comprising islands of chondroblastic and osteoblastic stromal cells. This report changed the diagnosis from carcinoma to osteogenic sarcoma of the breast. She had a left modified radical mastectomy, however there was significant post surgery skin deficit. A latissimus dorsi musculocutaneous flap was used to cover the anterior chest wall defect. Sections from the mastectomy specimen confirmed the diagnosis of osteogenic sarcoma. She died six months after mastectomy. Conclusion A diagnosis of osteogenic sarcoma of the breast was made based on histology report and after excluding an osteogenic sarcoma arising from underlying ribs and sternum. This is the second documented case of primary osteogenic sarcoma of the breast coming from Nigeria

  4. Diagnostic dilemma in Kaposi′s sarcoma

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    Rao Satish

    2006-01-01

    Full Text Available Kaposi′s sarcoma is described as cutaneous and extracutaneous neoplasm predominantly affecting older individuals. Though earlier uncommon and endemic to certain African areas, its incidence is on a rise due to infections with human immunodeficiency virus and also due to transplant-associated immunosuppression. Further, certain benign conditions like Pseudo Kaposi′s sarcoma, certain infective conditions like bacillary angiomatosis of acquired immunodeficiency syndrome can mimic Kaposi′s sarcoma both clinically and histologically leading to a diagnostic dilemma. We report such a case here.

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

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    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

  6. Stress-induced changes in immune response of dairy ewes

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    Agostino Sevi

    2010-01-01

    Full Text Available two groups of 16 Comisana ewes were selected from a group of 30, and divided, according to their cortisol secretion after isolation in a novel environment, into HC ewes, having a cortisol secretion >90 ng/mL, and LC ewes having a cortisol secretion <80 ng/mL. Blood samples were collected immediately before and immediately after isolation, and 60, 120, 300min and then 24h and 48h after isolation, to evaluate the percentage of lymphocyte T-helper (CD4+ and lymphocyte T-cytotoxic (CD8+ by flow cytometry analysis, and IL-1β and IL-6 concentrations by ELISA. The day before the isolation test, the ewes were milked in the afternoon at 1500h (-19h from isolation, and then milked for the three days starting from the day of the isolation test (at 0, 5, 24, 29, 48 and 53h from isolation to evaluate milk cortisol secretion, and IL-1β and IL-6 concentrations. Results suggest that the levels of cortisol secretions can influence the immune competence of dairy ewes and cytokines concentrations. Milk cytokine concentrations, and not milk cortisol concentrations, can be considered indicators of the magnitude of the hypothalamic-pituitary-adrenal axis activation.

  7. Yeasts associated with Sardinian ewe's dairy products.

    Science.gov (United States)

    Cosentino, S; Fadda, M E; Deplano, M; Mulargia, A F; Palmas, F

    2001-09-19

    In the present work, the occurrence of yeasts in different types of typical Sardinian ewe's cheeses (32 samples of pecorino, 32 of caciotta, 40 of feta, 56 of ricotta) was determined. For the strains isolated the following properties were studied: proteolytic and lipolytic activities, the ability to grow at different temperatures, different concentrations of salt, and to assimilate and/or ferment compounds like lactate, citrate, lactose, glucose, galactose, lactic acid. Of 160 samples analysed, 76.2% yielded growth of yeasts. Yeast counts showed a certain variability among the samples. The highest levels were observed in caciotta and feta cheeses. A total of 281 strains belonging to 16 genera and 25 species were identified. In general, Debaryomyces hansenii was the dominant species, representing 28.8% of the total isolates. Other frequently appearing species were Geotrichum candidum, Kluyveromyces lactis and K. marxianus. Other genera encountered were Pichia, Candida, Dekkera, Yarrowia and Rhodotorula. With regard to the biochemical and technological properties of the yeasts, only K. lactis, K. marxianus and Dek. anomala assimilated and fermented lactose, whereas the majority of the species assimilated lactic acid. The assimilation of citrate was a characteristic of D. hansenii, R. rubra and Y. lipolytica. On the whole, the yeasts were weakly proteolytic while lipolytic activity was present in several species. A high percentage of strains showed a certain tolerance to low temperatures while only some strains of D. hansenii and K. lactis were able to grow at a 10% NaCl concentration.

  8. Safety and efficacy of low-dose, subacute exposure of mature ewes to sodium chlorate

    Science.gov (United States)

    The objective was to determine the safety and efficacy of low-dose, subacute exposure of mature ewes to NaClO3 in the drinking water. Twenty-five ewes (BW = 62.5 ± 7.3 kg) were placed indoors in individual pens with ad libitum access to water and feed. After 7 d of adaptation, ewes were assigned ran...

  9. Do ewes born with a male co-twin have greater longevity with lambing over time?

    Science.gov (United States)

    Based on a recent analysis of historical records, ewes born co-twin to a ram had greater lifetime reproductive performance than ewes born co-twin to a ewe. We are interested in determining what component(s) of lifetime reproductive performance may be associated with a ewe’s co-twin sex. As an initi...

  10. Effects of lipopolysaccharide (LPS) induced inflammatory response on early embryo survival in ewes

    Science.gov (United States)

    Early pregnant ewes were used to determine the effects of endogenous (through LPS activation) and exogenous TNF-alpha tumor necrosis factor-alpha (TNF-alpha) on embryonic loss. Thirty-eight Dorset x Texel ewes were synchronized for estrus and bred to fertile rams (d0). On d5/6, ewes were assigned t...

  11. Myeloid sarcoma of submandibular salivary gland

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    Federico Dagna

    2016-01-01

    Full Text Available Objective: To report a rare case of a myeloid sarcoma of submandibular salivary gland. Methods: A 65-year-old woman with a history of successfully treated myelodysplastic syndrome, presenting with periodic painful swelling of her right submandibular area. Results: Physical evaluation, ultrasound and CT scan revealed the presence of a 3-cm mass contiguous to the submandibular salivary gland. A core needle biopsy confirmed the diagnosis of myeloid sarcoma. Bone marrow biopsy was still showing complete remission and the submandibular gland was the only extramedullary site involved. The patient was submitted to chemotherapy. Conclusion: Myeloid sarcoma is a rare extramedullary neoplasm. It can virtually involve any anatomic site, but it usually involves lymph nodes, paranasal sinuses, skin, soft tissue and periostium. Myeloid sarcomas of salivary glands are very rare and ENTs should be aware of this disease in order to include it in the differential diagnosis of a solitary neck mass.

  12. CLINICAL ANALYSIS AND SURGICAL RESULTS IN SARCOMA

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    Basavaraju

    2016-02-01

    Full Text Available INTRODUCTION Sarcomas are quite rare with only 15,000 new cases per year in the United States. Sarcomas therefore represent about one percent of the 1.5 million new cancer diagnoses in that country each year. Sarcoma can be defined as cancer whose cells originate from the cells of mesenchymal origin. The bones, cartilages, muscles are a few examples to be mentioned. This is in contrast to a malignant tumour originating from epithelial cells, which are termed carcinoma. AIMS AND OBJECTIVES 1. To clinically analyze the sarcomas. 2. To analyze the surgical outcome of this disease. The survival of the patient depends on the extent of metastasis and the primary identification. The study forms a base for further studies. So atleast it could be diagnosed earlier and treated to the full extent.

  13. Pulmonary Artery Intimal Sarcoma: A Case Report

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    Joseph P. Kriz

    2016-04-01

    Full Text Available Pulmonary artery intimal sarcomas are rare and lethal malignant tumors that typically affect larger vessels: the aorta, inferior vena cava, and pulmonary arteries. Since symptoms and imaging of pulmonary arterial intimal sarcomas mimic pulmonary thromboembolism, the differential diagnosis of a patient presenting with chest pain, dyspnea, and filling defect within the pulmonary arteries should include intimal sarcoma. Often right ventricular failure is observed due to pulmonary hypertension caused by the obstructive effect of the tumor and concomitant chronic thromboembolism. We report the case of a 72-year-old African-American male with arterial intimal sarcoma of the left and right pulmonary artery with extension through the right artery into the bronchus and right lung.

  14. Kaposi`s sarcoma; Sarcome de Kaposi

    Energy Technology Data Exchange (ETDEWEB)

    Kirova, Y.M.; Belembaogo, E.; Frikha, H.; Yu, S.J.; Le Bourgeois, J.P. [Hopital Henri-Mondor, 94 - Creteil (France)

    1997-09-01

    Moriz Kaposi was the first who, in 1872, described five patients presenting with `sarcoma idiopathicum multiple hemorrhagicum`. In 1912 Sternberg termed this disease Kaposi`s sarcoma. Since then various forms of this rare disease have been observed. In 1914 Hallenberg described the first cases of African or endemic Kaposi`s sarcoma. In the 1960`s the first reports discussing Kaposi`s sarcoma following organ transplantation and immunosuppressive therapy were published. After 1981, the epidemic form associated with the acquired immunodeficiency syndrome (AIDS) was described. All these forms, their history, treatment methods and the role of radiation therapy in the management of this rare malignancy are discussed, and the literature is reviewed. (authors)

  15. Treatment Option Overview (Adult Soft Tissue Sarcoma)

    Science.gov (United States)

    ... Vascular Tumors Treatment Research Adult Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft ... dye reacts to the light. Certain factors affect treatment options and prognosis (chance of recovery). The treatment ...

  16. Treatment Options for Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... Vascular Tumors Treatment Research Adult Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft ... dye reacts to the light. Certain factors affect treatment options and prognosis (chance of recovery). The treatment ...

  17. Drugs Approved for Soft Tissue Sarcoma

    Science.gov (United States)

    ... 2015 2014 2013 2012 Media Resources Media Contacts Multicultural Media ... This page lists cancer drugs approved by the Food and Drug Administration (FDA) for soft tissue sarcoma. The list includes ...

  18. Primary granulocytic sarcoma of the ovary.

    Science.gov (United States)

    Sreejith, G; Gangadharan, V P; Elizabath, K A; Preetha, S; Chithrathara, K

    2000-06-01

    Granulocytic sarcomas are rare extramedullary tumors of malignant myeloid precursor cells. Exceedingly rare in childhood, it commonly involves skin, lymph nodes, bone, and the spine. Ovarian involvement is rare. It can arise de novo, precede the development of acute nonlymphocytic leukemia, or be the sole manifestation of relapse. We describe a 26-year-old woman with granulocytic sarcoma of the ovary without any hematologic disorder.

  19. Acroangiodermatitis (Pseudo-Kaposi sarcoma

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    Satyendra Kumar Singh

    2014-01-01

    Full Text Available Acroangiodermatitis or Pseudo-Kaposi sarcoma is a rare angioproliferative entity, related to chronic venous insufficiency or certain other vascular anomalies. It is often associated with chronic venous insufficiency, arteriovenous malformation of the legs, chronic renal failure treated with dialysis, paralyzed legs and amputation stumps. We hereby describe a case of 45 year old female presenting with pitting pedal edema, multiple ulcers over bilateral lower limbs with irregular margins with erythema and hyperpigmentation of the surrounding skin. Color Doppler study of bilateral lower limbs was normal. Histopathological examination from one of the lesions showed hyperplastic epidermis, proliferation of capillaries in dermis, hemosiderin deposits and lymphocytic infiltrate. These features thus confirmed the diagnosis of Acroangiodermatitis.

  20. Undifferentiated Pleomorphic Sarcoma in Mandible.

    Science.gov (United States)

    Kim, Chul-Hwan; Jang, Jong-Won; Kim, Moon-Young; Kim, Yong-Hwan; Kim, Hang-Gul; Kim, Joo-Hwan

    2014-11-01

    Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, occurs commonly in the soft tissues in adult, but is rare in the maxillofacial region. It consists of undifferentiated mesenchymal tumor cells resembling histiocytes and fibroblasts. The purpose of this article is to report a case of UPS in the mandible. A 44-year-old patient presented with a painful growing mass in the mandible of two months' duration. Computed tomography and positron emission tomography-computed tomography revealed an ill-defined heterogenous, hypermetabolic mass about 4 cm in size in the left mandible invading adjacent soft tissues. A left mandiblulectomy and reconstruction with a fibular free flap were performed. Immunohistochemical study gave a diagnosis of UPS. The patient was referred for adjuvant chemotherapy after surgical removal of the tumor.

  1. Primary synovial sarcoma of lung

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    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.

  2. Potential Therapeutic Targets in Uterine Sarcomas

    Science.gov (United States)

    Cuppens, Tine; Tuyaerts, Sandra; Amant, Frédéric

    2015-01-01

    Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach. PMID:26576131

  3. Potential Therapeutic Targets in Uterine Sarcomas

    Directory of Open Access Journals (Sweden)

    Tine Cuppens

    2015-01-01

    Full Text Available Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach.

  4. Genetic Parameters of Some Economic Traits in Arabi Ewes

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    S F. Al-Dabbagh

    2013-12-01

    Full Text Available 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. Heritability estimates were low- moderate ranged (0.09-0.45. Repeatability estimates were low-high ranged (0.10-0.56. Genetic and phenotypic values of correlation between the traits were all highly significant (p≤ 0.01 except for the genetic and phenotypic correlation between birth weight and both daily milk yield, fertility, phenotypic correlation between greasy fleece weight and both fertility, litter size were non significant.

  5. Effects of experimental Trypanosoma evansi infection on pregnancy in Yankasa ewes.

    Science.gov (United States)

    Adeyeye, A A; Ate, I U; Lawal, A I; Adamu, S

    2016-03-15

    Twenty pregnant Yankasa ewes were assigned to three groups to determine the effect of Trypanosoma evansi infection on pregnancy. Groups A and B comprising seven ewes each were infected with approximately 1.0 × 10(6) cells of T evansi per ewe through venepuncture at the second and third trimesters of pregnancy, respectively. Group C comprising six ewes served as uninfected control. There was slight pyrexia in the infected groups (groups A and B) but was absent in group C. The mean body weight, glucose concentration, and packed cell volume of ewes in group A were not significantly different from those in group C throughout the study. There was also no significant difference in mean glucose concentration between groups B and C. However, in group B, mean body weight was significantly (P gestation length between ewes in the infected groups (groups A and B) compared with those in group C. However, there were significant (P < 0.05) decreases in lamb birth weights of ewes in group B compared with ewes in groups A and C. Mice inoculation with blood from infected ewes postpartum was parasitemic 18 to 25 days pi, for ewes in group B, whereas none of the mice in groups A and C were parasitemic. Lambs born from the infected groups (groups A and B) were also aparasitemic for 40 days postpartum. It was therefore concluded that the T evansi isolate used caused mild trypanosomosis when infected at third trimester, whereas ewes infected at second trimester were resistant.

  6. The spectrum of EWSR1-rearranged neoplasms at a tertiary sarcoma centre; assessing 772 tumour specimens and the value of current ancillary molecular diagnostic modalities

    Science.gov (United States)

    Noujaim, Jonathan; Jones, Robin L; Swansbury, John; Gonzalez, David; Benson, Charlotte; Judson, Ian; Fisher, Cyril; Thway, Khin

    2017-01-01

    Background: EWSR1 rearrangements were first identified in Ewing sarcoma, but the spectrum of EWSR1-rearranged neoplasms now includes many soft tissue tumour subtypes including desmoplastic small round cell tumour (DSRCT), myxoid liposarcoma (MLPS), extraskeletal myxoid chondrosarcoma (EMC), angiomatoid fibrous histiocytoma (AFH), clear cell sarcoma (CCS) and myoepithelial neoplasms. We analysed the spectrum of EWSR1-rearranged soft tissue neoplasms at our tertiary sarcoma centre, by assessing ancillary molecular diagnostic modalities identifying EWSR1-rearranged tumours and reviewing the results in light of our current knowledge of these and other Ewing sarcoma-like neoplasms. Methods: We retrospectively analysed all specimens tested for EWSR1 rearrangements by fluorescence in situ hybridisation (FISH) and/or reverse transcription–PCR (RT–PCR) over a 7-year period. Results: There was a total of 772 specimens. FISH was performed more often than RT–PCR (n=753, 97.5% vs n=445, 57.6%). In total, 210 (27.9%) specimens were FISH-positive for EWSR1 rearrangement compared to 111 (14.4%) that showed EWSR1 fusion transcripts with RT–PCR. Failure rates for FISH and RT–PCR were 2.5% and 18.0%. Of 109 round cell tumours with pathology consistent with Ewing sarcoma, 15 (13.8 %) cases were FISH-positive without an identifiable EWSR1 fusion transcript, 4 (3.7%) were FISH-negative but RT–PCR positive and 4 (3.7%) were negative for both. FISH positivity for DSRCT, MLPS, EMC, AFH and CCS was 86.3%, 4.3%, 58.5%, 60.0% and 87.9%, respectively. A positive FISH result led to diagnostic change in 40 (19.0%) EWSR1-rearranged cases. 13 FISH-positive cases remained unclassifiable. Conclusions: FISH is more sensitive for identifying EWSR1 rearrangements than RT–PCR. However, there can be significant morphologic and immunohistochemical overlap between groups of EWSR1-rearranged neoplasms, with important prognostic and therapeutic implications. FISH and RT–PCR should be used as

  7. What Are the Risk Factors for Soft Tissue Sarcoma?

    Science.gov (United States)

    ... not been proven to cause soft tissue sarcomas. Arsenic has also been linked to a type of ... Tissue Sarcoma Causes, Risk Factors, and Prevention Early Detection, Diagnosis, and Staging Treatment After Treatment Back To ...

  8. 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

  9. Immunotherapy for Bone and Soft Tissue Sarcomas

    Directory of Open Access Journals (Sweden)

    Takenori Uehara

    2015-01-01

    Full Text Available Although multimodal therapies including surgery, chemotherapy, and radiotherapy have improved clinical outcomes of patients with bone and soft tissue sarcomas, the prognosis of patients has plateaued over these 20 years. Immunotherapies have shown the effectiveness for several types of advanced tumors. Immunotherapies, such as cytokine therapies, vaccinations, and adoptive cell transfers, have also been investigated for bone and soft tissue sarcomas. Cytokine therapies with interleukin-2 or interferons have limited efficacy because of their cytotoxicities. Liposomal muramyl tripeptide phosphatidylethanolamine (L-MTP-PE, an activator of the innate immune system, has been approved as adjuvant therapeutics in combination with conventional chemotherapy in Europe, which has improved the 5-year overall survival of patients. Vaccinations and transfer of T cells transduced to express chimeric antigen receptors have shown some efficacy for sarcomas. Ipilimumab and nivolumab are monoclonal antibodies designed to inhibit immune checkpoint mechanisms. These antibodies have recently been shown to be effective for patients with melanoma and also investigated for patients with sarcomas. In this review, we provide an overview of various trials of immunotherapies for bone and soft tissue sarcomas, and discuss their potential as adjuvant therapies in combination with conventional therapies.

  10. Estimation of udder cistern size in dairy ewes by ultrasonography

    Directory of Open Access Journals (Sweden)

    Pavol Makovick

    2015-07-01

    Full Text Available We studied the size of mammary cistern in ewes of 9 genotypes (purebred Improved Valachian (IV, purebred Tsigai (T, purebred Lacaune (LC and their crosses with genetic proportion of specialized dairy breeds Lacaune and East Friesian (EF - (25 %, 50 % and 75 % were evaluated. Dana were evaluated using REML methodology and MIXED procedure (SAS/STAT. The effect of genotype showed the highest influence (P<0.001 on the length and area of the left and right udder cisterns measurements. In purebred IV ewes, the average areas of the left and right udder cisterns sizes were obtained by using the side method were (1519.39±77.212 mm2 and 1558.45±74.480 mm2. In purebred T ewes, the average areas of the left and right udder cisterns were (1438.70±70.43 mm2 and 1418.68±67.952 mm2. These were significantly smaller than in purebred LC (2694.44±71.95 mm2 and 2693.48±69.340 mm2. The udder cistern areas were significantly higher in crosses with 25 %, 50 % and 75 % genetic proportion of specialized dairy breeds LC and EF, than in purebred IV and T ewes. The analyses showed that crossbreeding of IV with LC and EF and T with LC and EF considerably increases ewe‘s cistern size.

  11. Kaposi sarcoma following postmastectomy lymphedema.

    Science.gov (United States)

    Montero Pérez, Iria; Rodríguez-Pazos, Laura; Álvarez-Pérez, Adriana; Ferreirós, M Mercedes Pereiro; Aliste, Carlos; Suarez-Peñaranda, Jose Manuel; Toribio, Jaime

    2015-11-01

    Classical Kaposi sarcoma (KS) usually appears on lower extremities accompanied or preceded by local lymphedema. However, the development in areas of chronic lymphedema of the arms following mastectomy, mimicking a Stewart-Treves syndrome, has rarely been described. We report an 81-year-old woman who developed multiple, erythematous to purple tumors, located on areas of post mastectomy lymphedema. Histopathological examination evidenced several dermal nodules formed by spindle-shaped cells that delimitated slit-like vascular spaces with some red cell extravasation. Immunohistochemically, the human herpesvirus type 8 (HHV-8) latent nuclear antigen-1 was detected in the nuclei of most tumoral cells confirming the diagnosis of KS. Lymphedema could promote the development of certain tumors by altering immunocompetence. Although angiosarcoma (AS) is the most frequent neoplasia arising in the setting of chronic lymphedema, other tumors such as benign lymphangiomatous papules (BLAP) or KS can also develop in lymphedematous limbs. It is important to establish the difference between AS and KS because their prognosis and treatment are very different. Identification by immunohistochemistry of HHV-8 is useful for the distinction between KS and AS or BLAP.

  12. 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.

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

    Science.gov (United States)

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

    2013-08-01

    Abrupt weaning, a usual management in sheep productive systems, may provoke behavioural and physiological responses indicative of stress in ewes and lambs. Progesterone (P4) has anxiolytic and sedative effects through the union of its metabolites that contain 3α-hydroxyl group to the γ-aminobutyricacidA receptor. Our first aim was to determine whether P4 administration reduces the behavioural and physiological responses of ewes to abrupt weaning of lambs. A complementary aim was to determine whether P4 treatment affects the milk yield and composition of ewes, and the BW of their lambs. In experiment 1, seven ewes received P4 treatment for 32 days (group E1-P4), and eight ewes remained as an untreated control group (group E1-C). BW of the lambs was recorded during P4 treatment. Lambs were weaned at 59 days (Day 0 = weaning). The main behaviours of the ewes before and after weaning were recorded using 10 min scan sampling. The ewes' serum total protein, albumin and globulin concentrations were measured before and after weaning of the lambs. In experiment 2, milk yield and composition were determined in two different groups of six ewes treated with P4 (group E2-P4) for 16 days and in five untreated controls (group E2-C). The BW of lambs increased with time (P = 0.001) in both groups and did not differ. The percentage of observations in which the ewes were seen pacing on Day 0 was greater in the E1-C group than in the E1-P4 group (P = 0.0007). Similarly, the percentage of observations in which the ewes were recorded vocalizing on Day 0 was greater in the E1-C group than in the E1-P4 group (P = 0.04). The percentage of observations in which E1-C ewes were recorded lying did not change from Days 0 to 1; however, it increased in E1-P4 ewes. Total serum protein concentration did not change in E1-P4 ewes from Days 0 to 3, although a decrease was seen in E1-C ewes (P = 0.04). Serum globulin concentration was greater in E1-P4 ewes on Day 3 than in E1-C ewes (P = 0.0008). In

  14. 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.

  15. 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.)

  16. Primary Breast Sarcoma. A Case Report

    Directory of Open Access Journals (Sweden)

    Lidia Torres Ajá

    2013-06-01

    Full Text Available Primary breast sarcoma is the least frequent non-epithelial malignant tumour, representing less than 1 % of all breast cancers. It has a dismal prognosis with the presence of early metastases, mainly in the lungs and bones. Survival is very poor at 5 years. A case of a 79 year-old female patient with a large ulcerated sarcoma in the left breast is presented. The patient was examined in an interdisciplinary consultation, and the presence of a stromal sarcoma of the breast without apparent visceral or bone metastases was confirmed by an aspiration biopsy with thick needle, excisional biopsy by paraffin and by immunohistochemical studies. The publication of this report has clinical interest for health professionals due to the rarity of the disease.

  17. Sarcoma derived from cultured mesenchymal stem cells.

    Science.gov (United States)

    Tolar, Jakub; Nauta, Alma J; Osborn, Mark J; Panoskaltsis Mortari, Angela; McElmurry, Ron T; Bell, Scott; Xia, Lily; Zhou, Ning; Riddle, Megan; Schroeder, Tania M; Westendorf, Jennifer J; McIvor, R Scott; Hogendoorn, Pancras C W; Szuhai, Karoly; Oseth, Leann; Hirsch, Betsy; Yant, Stephen R; Kay, Mark A; Peister, Alexandra; Prockop, Darwin J; Fibbe, Willem E; Blazar, Bruce R

    2007-02-01

    To study the biodistribution of MSCs, we labeled adult murine C57BL/6 MSCs with firefly luciferase and DsRed2 fluorescent protein using nonviral Sleeping Beauty transposons and coinfused labeled MSCs with bone marrow into irradiated allogeneic recipients. Using in vivo whole-body imaging, luciferase signals were shown to be increased between weeks 3 and 12. Unexpectedly, some mice with the highest luciferase signals died and all surviving mice developed foci of sarcoma in their lungs. Two mice also developed sarcomas in their extremities. Common cytogenetic abnormalities were identified in tumor cells isolated from different animals. Original MSC cultures not labeled with transposons, as well as independently isolated cultured MSCs, were found to be cytogenetically abnormal. Moreover, primary MSCs derived from the bone marrow of both BALB/c and C57BL/6 mice showed cytogenetic aberrations after several passages in vitro, showing that transformation was not a strain-specific nor rare event. Clonal evolution was observed in vivo, suggesting that the critical transformation event(s) occurred before infusion. Mapping of the transposition insertion sites did not identify an obvious transposon-related genetic abnormality, and p53 was not overexpressed. Infusion of MSC-derived sarcoma cells resulted in malignant lesions in secondary recipients. This new sarcoma cell line, S1, is unique in having a cytogenetic profile similar to human sarcoma and contains bioluminescent and fluorescent genes, making it useful for investigations of cellular biodistribution and tumor response to therapy in vivo. More importantly, our study indicates that sarcoma can evolve from MSC cultures.

  18. Efficacy of repeated doses of levamisole, morantel, fenbendazole, and ivermectin against gastrointestinal nematodes in ewes.

    Science.gov (United States)

    Hembry, F G; Miller, J E; Sims, D; Rodriguez, S; Stagg, L C

    1986-08-01

    Levamisole, morantel, fenbendazole, or ivermectin was administered at 2-week intervals from May 1 through Sept 14, 1985, to breeding ewes (20 ewes/drug) infected with various gastrointestinal nematodes. All ewes had fewer gastrointestinal nematode eggs per gram of feces (epg) after 2 treatments, compared with pretreatment epg counts. Ewes administered ivermectin continued to have a low mean epg (0 to 3) throughout the study. The mean epg counts of ewes treated with levamisole increased from 3 to 483 during the study. This increase was similar to that of ewes treated with morantel (7 to 485 epg). The mean epg count of fenbendazole-treated ewes increased from 4 to 192 during the study. By the end of the study, the mean epg counts when expressed as a percentage of the pretreatment epg counts were 4% (ivermectin), 249% (fenbendazole), 627% (levamisole), and 630% (morantel). With the exception of the ivermectin-treated ewes, the epg count increased almost linearly in the ewes after the 2nd anthelmintic treatment. These data indicate that the gastrointestinal nematodes (including Haemonchus contortus) may have developed more resistance to levamisole and morantel than to fenbendazole. On the basis of the epg counts, resistance to ivermectin did not develop during the 4.5-month treatment period. The percentage of ewes shedding eggs after 2, 4, and 6 anthelmintic treatments was lowest for ewes treated with ivermectin (20%) and was similar (40%) for ewes treated with 1 of the other 3 anthelmintics. At the conclusion of the study, most of the ewes (90%) were shedding at least a small number of eggs, regardless of the anthelmintic treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

  19. Primary mediastinal giant synovial sarcoma: A rare case report

    Directory of Open Access Journals (Sweden)

    Gaetano Rea

    2015-03-01

    Full Text Available Synovial sarcoma has been defined by the World Health Organization (WHO in 2002 as a type of mesenchymal tissue cell tumor that exhibits epithelial differentiation and represents the third most common soft-tissue sarcoma in adults, accounting for approximately 10% of soft-tissue sarcomas. To date, only few reports have focused on mediastinal synovial sarcoma imaging findings. Herein, we report a case of a 13 cm primary mediastinal giant synovial sarcoma, diagnosed in a 56-year-old patient admitted in our Department of Radiology with a six-month history of dyspnea and back pain.

  20. Kaposi's sarcoma following immune suppressive therapy for Wegener's granulomatosis.

    Science.gov (United States)

    Deschênes, Isabelle; Dion, Louise; Beauchesne, Claude; de Brum-Fernandes, Artur

    2003-03-01

    The association between Kaposi's sarcoma and infection with human herpesvirus 8 is now well recognized. Immunologic impairment is associated with 2 forms of Kaposi's sarcoma, epidemic [associated with human immunodeficiency virus (HIV) infection] and iatrogenic (associated with immunosuppressive treatment); both forms have become more common during the last decade. We describe an HIV negative 54-year-old man who developed Kaposi's sarcoma 2 months after the beginning of immuno-suppressive therapy for Wegener's granulomatosis (WG). With tapering of medication, complete remission of Kaposi's sarcoma was achieved in one year. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with WG.

  1. 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.

  2. Amputation for histiocytic sarcoma in a cat.

    Science.gov (United States)

    Teshima, Takahiro; Hata, Takashi; Nezu, Yoko; Michishita, Masaki; Matsumoto, Hirotaka; Mizutani, Hisashi; Takahashi, Kimimasa; Koyama, Hidekazu

    2012-02-01

    A 9-year-old spayed female domestic shorthair cat presented with a skin lesion of the left tarsus. The lesion was biopsied and, based on the microscopic appearance and immunohistochemical characteristics, histiocytic sarcoma was diagnosed. Amputation was performed with improved demeanor seen postoperatively. However, between 44 and 60 days following the surgery, relapse of skin lesions appeared in multiple locations, including at the previous amputation site, and euthanasia was elected. This is the first report of a histiocytic sarcoma treated with amputation in a cat.

  3. 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.

  4. Molecular piracy of Kaposi's sarcoma associated herpesvirus.

    Science.gov (United States)

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

    2001-01-01

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

  5. Limb salvage with microvascular free fibula following primary bone sarcoma resection

    Directory of Open Access Journals (Sweden)

    Sahasrabudhe Parag

    2016-01-01

    Full Text Available Background: Extremity sarcomas are challenging to manage. Total eradication of tumour has to be balanced with restoration of limb function to prevent mortality and morbidity. Disease-free survival with maximum limb function is the ultimate goal in these patients. Materials and Methods: We present a series of ten cases of extremity malignancies, where limb salvage was attempted with microvascular free fibula for limb reconstruction from the period of 2008 to 2015. Results: Of the ten cases in the study, there were two females and eight males. There were nine patients with lower limb malignancies and one patient with upper limb malignancy. There were four patients with Ewing's sarcoma of femur, five patients with osteosarcoma of femur and one patient with chondrosarcoma of the humerus. The follow-up period ranged from 1.2 to 6.2 years with mean follow-up of 3.1 years. There were two deaths during follow-up, both were due to distant metastasis. The assessment of the function was done on the basis of Musculoskeletal Tumour Society functional score. Maximum score was 30 and minimum score was 24, the average score being 26. Of the eight surviving patients, three patients had full weightbearing, four patients had partial weightbearing at end of 2 years and one patient of upper limb reconstruction had complete upper limb function. None of the patients had to undergo limb amputation. Conclusion: Limb salvage with vascularised fibula graft offers good functional outcome along with good disease-free survival rates.

  6. Bilateral Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Breast: A Very Rare Entity and Review of the Literature

    Directory of Open Access Journals (Sweden)

    N. Majid

    2013-01-01

    Full Text Available Peripheral primitive neuroectodermal tumors (PNET are rare malignant tumors, affecting mostly children and adolescents and have been described in breast in eight case reports only. In this paper, we present a case of bilateral mammary ES/PNET where distinction between primary and metastatic diseases was discussed through a literature review. The aim of this work is to demonstrate that although rare, the possibility of PNET should be kept in mind while evaluating a palpable breast abnormality in a young female.

  7. Treatment Option Overview (Childhood Soft Tissue Sarcoma)

    Science.gov (United States)

    ... first formed. This summary is about the following types of soft tissue sarcoma: Fat tissue tumors Liposarcoma . This is a rare cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In ... types of liposarcoma. Myxoid liposarcoma is usually low grade ...

  8. General Information about Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... first formed. This summary is about the following types of soft tissue sarcoma: Fat tissue tumors Liposarcoma . This is a rare cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In ... types of liposarcoma. Myxoid liposarcoma is usually low grade ...

  9. Treatment Options for Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... first formed. This summary is about the following types of soft tissue sarcoma: Fat tissue tumors Liposarcoma . This is a rare cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In ... types of liposarcoma. Myxoid liposarcoma is usually low grade ...

  10. Stages of Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... first formed. This summary is about the following types of soft tissue sarcoma: Fat tissue tumors Liposarcoma . This is a rare cancer of the fat cells. Liposarcoma usually forms in the fat layer just under the skin. In ... types of liposarcoma. Myxoid liposarcoma is usually low grade ...

  11. 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

  12. The Value of Surgery for Retroperitoneal Sarcoma

    Science.gov (United States)

    Gholami, Sepideh; Jacobs, Charlotte D.; Kapp, Daniel S.; Parast, Layla M.; Norton, Jeffrey A.

    2009-01-01

    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 (P = .006). Complete surgical resection improved overall survival for high-grade tumors (P = .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. PMID:19826633

  13. 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.

  14. 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.

  15. Treatment of classical Kaposi's sarcoma with gemcitabine

    NARCIS (Netherlands)

    Brambilla, L; Labianca, R; Ferrucci, SM; Taglioni, M; Boneschi, [No Value

    2001-01-01

    Background: Several drugs are active in aggressive classical Kaposi's sarcoma (CKS); chemotherapeutic agents with fewer side-effects, more rapid response and able to overcome resistance to previous treatment are advisable when treating patients in a second line. Gemcitabine, an analogue of deoxycyti

  16. Combination Therapy for Advanced Kaposi Sarcoma

    Science.gov (United States)

    In this clinical trial, adult patients with any form of advanced Kaposi sarcoma will be treated with liposomal doxorubicin and bevacizumab every 3 weeks for a maximum of six treatments.  Patients who respond to this therapy or have stable disease will rec

  17. 3 cases of radiation-induced sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Shiba, K.; Fukuma, H.; Beppu, Y.; Hirota, T. (National Cancer Center, Tokyo (Japan). Hospital); Shinohara, N.

    1982-03-01

    Criteria for the diagnosis of radiation-induced sarcoma have been previously described. All cases must have a history of irradiation and the second neoplasm must have arisen in the area of the radiation field. A latent period of several years must have elapsed after irradiation before clinical evidence of a second malignant neoplasm. Most important thing is that, all suspected cases must have been proved histologically. We have experienced 3 cases of radiation-induced sarcoma, they were 42-years-old man who developed an osteosarcoma of the lumbar spine at the field of postoperative irradiation for seminoma 7 years previously, 69-years-old woman who developed a malignant fibrous histiocytoma of the buttock at the field of radical radiation for uterine carcinoma 7 years previously and 59-years-old woman who developed an extraskeletal osteosarcoma of the abdominal wall at the field of postoperative irradiation for uterine sarcoma 7 years previously. The last case is very rare and only 8 cases of radiation-induced extraskeletal osteosarcoma have been reported. Since there has been a definite trend in the treatment of cancer toward employing radiation for more favorable cases, in addition to technical improvements in the administration of radiotherapy and more modern equipment, survival data may have been altered considerably in many malignant tumors. Accordingly, more radiation-induced tumors may be encountered in the future. The clinical presentation and histopathology of these radiation-induced sarcomas are presented with a review of the literature.

  18. Immunosuppressive Therapy-Related Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  19. 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.

  20. Feline postvaccinal sarcoma: 20 years later.

    Science.gov (United States)

    Wilcock, Brian; Wilcock, Anne; Bottoms, Katherine

    2012-04-01

    Comparison of the annual prevalence of feline postvaccinal sarcomas among 11 609 feline skin mass submissions from 1992 to 2010 revealed no decrease in disease prevalence or increase in the age of affected cats in response to changes in vaccine formulation or recommended changes in feline vaccination protocols.

  1. Feline postvaccinal sarcoma: 20 years later

    OpenAIRE

    Wilcock, Brian; Wilcock, Anne; Bottoms, Katherine

    2012-01-01

    Comparison of the annual prevalence of feline postvaccinal sarcomas among 11 609 feline skin mass submissions from 1992 to 2010 revealed no decrease in disease prevalence or increase in the age of affected cats in response to changes in vaccine formulation or recommended changes in feline vaccination protocols.

  2. Therapeutic strategies for epidemic Kaposi's sarcoma

    NARCIS (Netherlands)

    Aldenhoven, M.; Barlo, N. P.; Sanders, C. J. G.

    2006-01-01

    Kaposi's sarcoma (KS) remains the most commonly diagnosed malignancy in HIV-infected patients, and is one of the AIDS-defining diagnoses. Several different therapeutic options are available, but the optimal therapy is still unclear. The incidence of KS has sharply declined since highly active antire

  3. Effects of exogenous progesterone on gestation length, foetal survival and colostrum yield in ewes.

    Science.gov (United States)

    Crosby, T F; O'Donnell, A; O'Doherty, J V; Quinn, P J; Evans, A C O

    2005-09-15

    Twin bearing mature ewes (n=40) were treated with exogenous progesterone (100mg daily in oil) or vehicle (oil control) from Day 143 of gestation until lambing to investigate the effects on gestation length, foetal survival and colostrum yield and composition. Compared to control ewes, progesterone treated ewes had increased (Pgestation length (150.4+/-0.6 days versus 147.8+/-0.6 days, Pgestation length and reduced lamb survival but did not lower colostrum yield.

  4. 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.

  5. 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

  6. Phenotypic characterization of the population of creole wool ewes in the highlands of Puebla State, Mexico.

    Science.gov (United States)

    Vargas-López, Samuel; Guerrero-Rodríguez, Juan de Dios; Rojas-Álvarez, Joel; Bustamante-González, Angel

    2012-12-01

    This study characterized the population of wool ewes in the highlands of the State of Puebla, Mexico, considering traits such as fleece color, weight, and body measurements. In this region, dominated by a temperate climate, sheep are a traditional animal species for farming systems. To carry out the work, 2,082 ewes were randomly selected from 14 communities and 124 flocks belonging to the six municipalities that have the largest inventory of sheep in the state. For each ewe, live weight, breed, fleece color pattern, and 18 other body measurements were recorded. Descriptive statistics were estimated for weight and body traits and the morphotype was classified by multivariate analysis. Factor analysis identified the bulk, size, and breed standard as the attributes that best describe the population of ewes. These elements varied in importance among the groups (p < 0.05). Cluster analysis helped to classify the population into small black-faced ewes (28.5 %), small white ewes (11.9 %), black-faced medium-sized ewes (24.1 %), large ewes (12.3 %), and white medium-sized ewes (23.2 %). The groups identified were similar to creole sheep present in rural communities in other environments, but have lower morphostructural values than specialized breeds.

  7. GLUT1 Expression in Synovial Sarcomas

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    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.

  8. Prions in milk from ewes incubating natural scrapie.

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    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.

  9. Prions in milk from ewes incubating natural scrapie.

    Science.gov (United States)

    Lacroux, Caroline; Simon, Stéphanie; Benestad, Sylvie L; Maillet, Séverine; Mathey, Jacinthe; Lugan, Séverine; Corbière, Fabien; Cassard, Hervé; Costes, Pierrette; Bergonier, Dominique; Weisbecker, Jean-Louis; Moldal, Torffin; Simmons, Hugh; Lantier, Frederic; Feraudet-Tarisse, Cécile; Morel, Nathalie; Schelcher, François; Grassi, Jacques; Andréoletti, Olivier

    2008-12-01

    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.

  10. The Challenges of Detecting Circulating Tumor Cells in Sarcoma

    Science.gov (United States)

    Tellez-Gabriel, Marta; Brown, Hannah K.; Young, Robin; Heymann, Marie-Françoise; Heymann, Dominique

    2016-01-01

    Sarcomas are a heterogeneous group of malignant neoplasms of mesenchymal origin, many of which have a propensity to develop distant metastases. Cancer cells that have escaped from the primary tumor are able to invade into surrounding tissues, to intravasate into the bloodstream to become circulating tumor cells (CTCs), and are responsible for the generation of distant metastases. Due to the rarity of these tumors and the absence of specific markers expressed by sarcoma tumor cells, the characterization of sarcoma CTCs has to date been relatively limited. Current techniques for isolating sarcoma CTCs are based on size criteria, the identification of circulating cells that express either common mesenchymal markers, sarcoma-specific markers, such as CD99, CD81, or PAX3, and chromosomal translocations found in certain sarcoma subtypes, such as EWS-FLI1 in Ewing’s sarcoma, detection of osteoblast-related genes, or measurement of the activity of specific metabolic enzymes. Further studies are needed to improve the isolation and characterization of sarcoma CTCs, to demonstrate their clinical significance as predictive and/or prognostic biomarkers, and to utilize CTCs as a tool for investigating the metastatic process in sarcoma and to identify novel therapeutic targets. The present review provides a short overview of the most recent literature on CTCs in sarcoma. PMID:27656422

  11. Resumption of cyclic ovarian activity in postpartum ewes: a review

    Directory of Open Access Journals (Sweden)

    Ivan Júnior Ascari

    2016-04-01

    Full Text Available The reproduction rate is considered a crucial factor that affects the ability of producers to meet the growing demand for ovines meat, and optimizing this factor will maximize the production rate and increase the efficiency of livestock. A reduction in the length of the postpartum anestrus period results in a shorter interval between lambing and conception and enables more lambs to be produced throughout the life of a ewe. This review discusses the current knowledge of several factors associated with the resumption of cyclic ovarian function in postpartum ewes and how the suckling management of ewes may affect this process. Factors that influence the resumption of cyclic ovarian activity include uterine involution, follicular development, occurrence of silent ovulation and short estrous cycles, breed, reproductive seasonality, nutrition and lactation. Controlled suckling and early weaning are management practices that may be used to obtain three lambings in a 24-month period. In the majority of the studies the interval between lambing and first estrus was reduced when a controlled suckling management strategy was used, therefore the controlled suckling appears to be the best alternative because it has produced good results and is easy to implement. Currently, however, this practice is still not widely used.

  12. Early decrements in bone density after completion of neoadjuvant chemotherapy in pediatric bone sarcoma patients

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    Hardes Jendrik

    2010-12-01

    Full Text Available Abstract Background Bone mineral density (BMD accrual during childhood and adolescence is important for attaining peak bone mass. BMD decrements have been reported in survivors of childhood bone sarcomas. However, little is known about the onset and development of bone loss during cancer treatment. The objective of this cross-sectional study was to evaluate BMD in newly diagnosed Ewing's and osteosarcoma patients by means of dual-energy x-ray absorptiometry (DXA after completion of neoadjuvant chemotherapy. Methods DXA measurements of the lumbar spine (L2-4, both femora and calcanei were performed perioperatively in 46 children and adolescents (mean age: 14.3 years, range: 8.6-21.5 years. Mean Z-scores, areal BMD (g/cm2, calculated volumetric BMD (g/cm3 and bone mineral content (BMC, g were determined. Results Lumbar spine mean Z-score was -0.14 (95% CI: -0.46 to 0.18, areal BMD was 1.016 g/cm2 (95% CI: 0.950 to 1.082 and volumetric BMD was 0.330 g/cm3 (95% CI: 0.314 to 0.347 which is comparable to healthy peers. For patients with a lower extremity tumor (n = 36, the difference between the affected and non-affected femoral neck was 12.1% (95% CI: -16.3 to -7.9 in areal BMD. The reduction of BMD was more pronounced in the calcaneus with a difference between the affected and contralateral side of 21.7% (95% CI: -29.3 to -14.0 for areal BMD. Furthermore, significant correlations for femoral and calcaneal DXA measurements were found with Spearman-rho coefficients ranging from ρ = 0.55 to ρ = 0.80. Conclusions The tumor disease located in the lower extremity in combination with offloading recommendations induced diminished BMD values, indicating local osteopenia conditions. However, the results revealed no significant decrements of lumbar spine BMD in pediatric sarcoma patients after completion of neoadjuvant chemotherapy. Nevertheless, it has to be taken into account that bone tumor patients may experience BMD decrements or secondary osteoporosis

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

    Science.gov (United States)

    2016-09-23

    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

  14. The relationship between trace mineral concentrations of amniotic fluid with placenta traits in the pregnancy toxemia Ghezel ewes

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    Ali Olfati

    2016-07-01

    Conclusions: Overall, determinations of these trace minerals in the AF ewes could have been used to obtain information on nutritional and reproductive status for the diagnosis of pregnancy toxemia in Ghezel ewes.

  15. Effect of protein degradability on milk production of dairy ewes.

    Science.gov (United States)

    Mikolayunas-Sandrock, C; Armentano, L E; Thomas, D L; Berger, Y M

    2009-09-01

    The objective of this experiment was to determine the effect of protein degradability of dairy sheep diets on milk yield and protein utilization across 2 levels of milk production. Three diets were formulated to provide similar energy concentrations and varying concentrations of rumen-degradable protein (RDP) and rumen-undegradable protein (RUP): 12% RDP and 4% RUP (12-4) included basal levels of RDP and RUP, 12% RDP and 6% RUP (12-6) included additional RUP, and 14% RDP and 4% RUP (14-4) included additional RDP. Diets were composed of alfalfa-timothy cubes, whole and ground corn, whole oats, dehulled soybean meal, and expeller soybean meal (SoyPlus, West Central, Ralston, IA). Estimates of RDP and RUP were based on the Small Ruminant Nutrition System model (2008) and feed and orts were analyzed for Cornell N fractions. Eighteen multiparous dairy ewes in midlactation were divided by milk yield (low and high) into 2 blocks of 9 ewes each and were randomly assigned within block (low and high) to 3 pens of 3 ewes each. Dietary treatments were arranged in a 3 x 3 Latin square within each block and applied to pens for 14-d periods. We hypothesized that pens consuming high-RUP diets (12-6) would produce more milk and milk protein than the basal diet (12-4) and pens consuming high-RDP diets (14-4) would not produce more milk than the basal diet (12-4). Ewes in the high-milk-yield square consumed more dry matter and produced more milk, milk fat, and milk protein than ewes in the low-milk-yield square. There was no effect of dietary treatment on dry matter intake. Across both levels of milk production, the 12-6 diet increased milk yield by 14%, increased milk fat yield by 14%, and increased milk protein yield by 13% compared with the 14-4 and 12-4 diets. Gross N efficiency (milk protein N/intake protein N) was 11 and 15% greater in the 12-6 and 12-4 diets, respectively, compared with the 14-4 diet. Milk urea N concentration was greater in the 12-6 diet and tended to be

  16. 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 ( < 0.05) in nutrient-restricted ewes compared with adequately fed ewes. Maternal RP-Arg or NCG supplementation did not alter ( = 0.26) maternal BW in nutrient-restricted ewes. Weights of most fetal organs were increased ( < 0.05) in RP-Arg-treated and NCG-treated underfed ewes compared with 50% NRC-fed ewes. Supplementation of RP-Arg or NCG reduced ( < 0.05) concentrations of β-hydroxybutyrate, triglycerides, and ammonia in serum of underfed ewes but had no effect on concentrations of lactate and GH. Maternal RP-Arg or NCG supplementation markedly improved ( < 0.05) concentrations of AA (particularly arginine-family AA and branched-chain AA) and polyamines in maternal and fetal plasma and in fetal allantoic and 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.

  17. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience.

    OpenAIRE

    Llombart-Cussac, A.; Pivot, X; Contesso, G; Rhor-Alvarado, A.; Delord, J P; Spielmann, M.; Türsz, T.; Le Cesne, A.

    1998-01-01

    The effect of additional treatments after surgery in patients with primary cardiac sarcoma (PCS) remains unknown. The present study aims to evaluate the benefit of chemotherapy in patients with non-metastatic cardiac sarcomas after optimal resection. Between October 1979 and December 1995, 15 patients with a median age of 45 (range 16-66) and a resected primary cardiac sarcoma [angiosarcoma (six), malignant fibrous histiocytoma (three), leiomyosarcoma (two), rhabdomyosarcoma (two), liposarcom...

  18. Radiation-induced prostatic sarcoma: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Scully, J.M.; Uno, J.M.; McIntyre, M.; Mosely, S. (Bay Area Hospital, Coos Bay, OR (USA))

    1990-09-01

    A 64-year-old man had a prostatic sarcoma 8 years after transurethral prostatectomy and radical bilateral pelvic lymph node dissection with insertion of 125-iodine implants for stage B1N carcinoma of the prostate. Therapy for the sarcoma consisted of isolated pelvic perfusion and then pelvic exenteration with creation of an ileal conduit and colostomy. The pathology report showed well encapsulated grade 2 spindle cell sarcoma of the prostate. Multiple small metallic particles were embedded in the tumor specimen.

  19. Osseous Kaposi sarcoma in an HIV-positive patient

    Energy Technology Data Exchange (ETDEWEB)

    Thanos, Loukas; Mylona, Sofia; Kalioras, Vasilios; Pomoni, Maria; Batakis, Nikolaos [Radiology Department, ' ' Korgialeneio-Benakeio' ' , Red Cross Hospital of Athens, 1 Athanasaki Street, 11526, Athens (Greece)

    2004-04-01

    A case of osseous Kaposi sarcoma in a 35-year-old man is described. The patient (HIV-positive for 8 years) suffered from cutaneous Kaposi sarcoma and presented with right-sided chest pain. He underwent a chest CT scan that revealed three osteolytic lesions involving rib and vertebra with large soft tissue masses, without cutaneous lesions at these sites. CT-guided core needle biopsy led to a histological diagnosis of Kaposi sarcoma. (orig.)

  20. Subcentimeter Pulmonary Nodules Detected in Patients with Sarcoma

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    Michelle S. Ginsberg

    2000-01-01

    Full Text Available Background. Subcentimeter pulmonary nodules are being detected with increasing frequency in patients with sarcoma due to the greater use of chest CT, the advent of helical (spiral CT scanning and multidetector scanners, and the attendant decrease in image section thickness.Assessing the clinical significance of these pulmonary nodules is of particular importance in sarcoma patients, due to the frequent occurrence of pulmonary metastasis from sarcomas.

  1. Endometrial stromal sarcoma: a rare tumour

    Directory of Open Access Journals (Sweden)

    Amrit Pal Kaur

    2014-02-01

    Full Text Available Endometrial stromal sarcomas (ESS are rare endometrial tumours arising from stroma of endometrium i.e. connective tissue of endometrium rather than glands. Usually a pre-operative diagnosis is difficult. Total abdominal hysterectomy with bilateral salpingo-oophorectomy is main line of treatment. Adjuvant hormone therapy in the form of progesterones, GnRH analogues, aromatase inhibitors are effective for prevention of recurrences as these tumours are invariably positive for oestrogen & progesterone receptors. Surgical excision, radiotherapy, hormone therapy are recommended for recurrences. We report a 52 yrs widow with undifferentiated endometrial stromal sarcoma weighing 3.75 kg with a short history of 3 months diagnosed only after histopathology. [Int J Reprod Contracept Obstet Gynecol 2014; 3(1.000: 276-278

  2. 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

  3. 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...

  4. Ewing Marion Kauffman School Evaluation Impact Report Year 4. Final Report

    Science.gov (United States)

    Johnson, Matthew; Demers, Alicia; Johnson, Cleo Jacobs; Gentile, Claudia

    2016-01-01

    As part of its ongoing efforts to raise the academic achievement of children from low-income families in Kansas City, Missouri, the Ewing Marion Kauffman Foundation founded the Ewing Marion Kauffman School in fall 2011. The Kauffman School's mission is "to prepare students to excel academically, graduate from college, and apply their unique…

  5. Sonographic Findings of Uterine Endometrial Stromal Sarcoma

    OpenAIRE

    Kim, Jeong-Ah; Lee, Myung Sook; Choi, Jong-Sun

    2006-01-01

    Objective The study was performed to present the sonographic findings of uterine endometrial stromal sarcoma (ESS). Materials and Methods We conducted a retrospective review of sonographic findings of 10 cases that were diagnosed as uterine ESS. The patients' ages ranged from 25 to 51 years (mean age: 36.1 years). The reviews focused on the location, margin, size, number and echotexture of the lesions. Hysterectomy (n = 9) and myomectomy (n = 1) were performed and a pathologic diagnosis was o...

  6. 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.)

  7. 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

  8. Primary Thyroid Sarcoma: A Systematic Review.

    Science.gov (United States)

    Surov, Alexey; Gottschling, Sebastian; Wienke, Andreas; Meyer, Hans Jonas; Spielmann, Rolf Peter; Dralle, Henning

    2015-10-01

    Different types of malignant tumors can occur within the thyroid. Primary cancer is the most common type of thyroid malignancy. Non-epithelial malignancies can also arise within the thyroid. The aim of the present study was to analyze clinical and radiological characteristics of reported primary thyroid sarcomas (PTS), based on a large sample of cases. The PubMed database was screened for articles from between 1990 and 2014. Overall, 86 articles with 142 patients were identified. Ultrasound evaluation was reported for 36 patients. Data regarding computed tomography of the neck were available for 29 cases. Magnetic resonance imaging was performed for eight patients. The following data were retrieved for the identified sarcomas: localization, size, homogeneity, internal texture, and margin characteristics. In most cases, PTS occurred in patients over 40 years of age, with a peak incidence for the group aged 60-79 years. Angiosarcoma was diagnosed in 29 cases (20.4%), followed by malignant hemangioendothelioma (n=23, 16.3%), malignant fibrous histiocytoma (n=20, 14.1%), leiomyosarcoma (n=16, 11.3%), and fibrosarcoma (n=13, 9.2%). In most patients (n=113, 79.6%), PTS manifested clinically as a painless goiter. On ultrasound, PTS were predominantly mixed hypo-to-hyperechoic in comparison to the normal thyroid tissue. On non-contrast computed tomography, most sarcomas were inhomogeneous hypo-to-hyperdense. On post-contrast magnetic resonance images, most sarcomas showed marked non-homogenous enhancement. In 26.8%, infiltration of the adjacent organs was seen. The trachea or esophagus was affected more frequently in patients with malignant histiocytoma and liposarcoma. Different strategies were used in the treatment of PTS. Our analysis provides clinical and radiological characteristics of PTS. The described features should be taken into consideration in the differential diagnosis of thyroid tumors.

  9. 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.

  10. Sarcoma Immunotherapy: Past Approaches and Future Directions

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    S. P. D'Angelo

    2014-01-01

    Full Text Available Sarcomas are heterogeneous malignant tumors of mesenchymal origin characterized by more than 100 distinct subtypes. Unfortunately, 25–50% of patients treated with initial curative intent will develop metastatic disease. In the metastatic setting, chemotherapy rarely leads to complete and durable responses; therefore, there is a dire need for more effective therapies. Exploring immunotherapeutic strategies may be warranted. In the past, agents that stimulate the immune system such as interferon and interleukin-2 have been explored and there has been evidence of some clinical activity in selected patients. In addition, many cancer vaccines have been explored with suggestion of benefit in some patients. Building on the advancements made in other solid tumors as well as a better understanding of cancer immunology provides hope for the development of new and exciting therapies in the treatment of sarcoma. There remains promise with immunologic checkpoint blockade antibodies. Further, building on the success of autologous cell transfer in hematologic malignancies, designing chimeric antigen receptors that target antigens that are over-expressed in sarcoma provides a great deal of optimism. Exploring these avenues has the potential to make immunotherapy a real therapeutic option in this orphan disease.

  11. Lymphangioma-like Kaposi sarcoma: case report.

    Science.gov (United States)

    Posada García, Celia; García-Cruz, Aranzazu; García-Doval, Ignacio; De La Torre, Carlos; Cruces, Manuel José

    2009-09-15

    Kaposi sarcoma (KS) is a multifocal vascular disease with uncertain histogenesis. It is characterized by clinical and histologic polymorphism. The "lymphangioma-like" variant is very uncommon, accounting for less than 5% of all cases. We report the case of a 76-year-old woman, HIV negative, with a 4-year history of classic Kaposi sarcoma treated with cryotherapy who developed new bullous lesions on her lower extremities. Biopsy revealed histologic findings of lymphangioma-like KS (LLKS), together with areas of classic KS; HHV-8 staining was positive. Diagnosis of LLKS was made and the patient was proposed for radiotherapy. The lymphangioma-like Kaposi sarcoma is a rare morphologic expression of KS characterized by dilated and bizarrely shaped vascular channels lined by flattened endothelium permeating the dermis. "Bulla-like" lesions have been considered as a clinical hallmark of this variant. Its histologic appearance suggests a lymphatic origin of KS and it may resemble other vascular tumors. Findings of areas of typical KS and positive staining for HHV-8 may help to make a definitive diagnosis.

  12. Estimation of milk production in hair ewes by two methods of measurement

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    Irina Peniche G

    2015-05-01

    Full Text Available ABSTRACT Objective. The objective of the current study was to compare two methods to estimate daily milk production in crossbred hair ewes. Materials and methods. Eight multiparous, crossbred (Pelibuey x Katahdin lactating hair ewes were used in a completely randomized design with repeated measurements, for 56 days. Ewes were fed ad libitum with a diet based on concentrate. Milk production was estimated twice a week for each ewe by both methods, suckled-hand (SH and weigh-suckle-weigh (WSW method. Results. Milk production (p>0.05 means were 031.9±95.6 and 1119.0±95.6 g/day/ewe for SH and WSW method, respectively. Conclusions. No significant differences (p>0.05 were found between methods for milk yield.

  13. Optimizing the interval between G-CSF therapy and F-18 FDG PET imaging in children and young adults receiving chemotherapy for sarcoma

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    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.)

  14. Implantação intracerebral experimental de sarcomas Experimental intracerebral implantation of sarcomas

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    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

  15. The role of Kaposi sarcoma-associated herpesvirus in the pathogenesis of Kaposi sarcoma.

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    Gramolelli, Silvia; Schulz, Thomas F

    2015-01-01

    Kaposi sarcoma (KS) is an unusual vascular tumour caused by an oncogenic-herpesvirus, Kaposi sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV 8). KS lesions are characterized by an abundant inflammatory infiltrate, the presence of KSHV-infected endothelial cells that show signs of aberrant differentiation, as well as faulty angiogenesis/ vascularization. Here we discuss the molecular mechanisms that lead to the development of these histological features of KS, with an emphasis on the viral proteins that are responsible for their development.

  16. 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

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    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

  17. Avaliação funcional dos pacientes portadores de sarcomas ósseos submetidos à tratamento cirúrgico utilizando a endoprótese total ou parcial, na substituição da extremidade distal do fêmur Functional assessment of patients with bone sarcomas submitted to surgical treatment using total or partial prosthesis in replacement of the distal femoral end

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    Sandra Maria Holanda de Mendonça

    2008-01-01

    Full Text Available OBJETIVO: O Osteosarcoma e o Sarcoma de Ewing são as principais neoplasias malignas primárias ósseas, que acometem indivíduos menores de 15 anos. O objetivo deste estudo é comparar, retrospectivamente, os resultados funcionais dos pacientes submetidos à ressecção da extremidade distal do fêmur e à reconstrução com endoprótese não convencional, total ou parcial, do joelho. MÉTODOS: Foram analisados 26 pacientes portadores de sarcomas ósseos da extremidade distal do fêmur, acompanhados no Centro Infantil Boldrini, no período de 1990 a 2003. Vinte e quatro eram portadores de Osteossarcoma e 2 de Sarcoma de Ewing. O sistema de avaliação foi o proposto por Enneking (1987, preconizado pela Musculoskeletal Tumor Society. Para a comparação das médias entre cada critério e também entre os escores finais, utilizou-se o teste de Wilcoxon, com erro alfa de 5%. RESULTADOS: A idade variou de 5 a 17 anos, média=11,9 anos. A predominância foi no sexo feminino (61,5%. Na avaliação funcional, a comparação entre as médias de cada critério, foi encontrada diferença estatisticamente significativa somente relacionada ao item estabilidade (p=0,0037. Nos demais critérios, não foi observado diferença estatisticamente significativa: movimento (p=0,7546, dor (p=0,4848, deformidade (p=0,8695, força (p=1,0000, atividade funcional (p=0,9127 e resultado funcional (p=0,5866. CONCLUSÕES: O escore final global da avaliação funcional não apresentou diferença estatisticamente significativa (p=0,6027. O tipo de endoprótese utilizado para reconstrução do fêmur não interferiu nos resultados funcionais dos pacientes.OBJECTIVES: Osteosarcoma and Ewing's sarcoma are the most common malignant primary bone tumors in individuals under the age of 15 years. The purpose of the study is to retrospectively compare functional outcomes of patients submitted to resection of the distal femoral end and to reconstruction with total or partial non

  18. Adamantinoma-like Ewing family tumor of soft tissue associated with the vagus nerve: a case report and review of the literature.

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    Kikuchi, Yoshinao; Kishimoto, Takashi; Ota, Satoshi; Kambe, Michiyo; Yonemori, Yoko; Chazono, Hideaki; Yamasaki, Kazuki; Ochiai, Hidemasa; Hiroshima, Kenzo; Tanaka, Mio; Tanaka, Yukichi; Horie, Hiroshi; Nakatani, Yukio

    2013-05-01

    Adamantinoma-like Ewing family tumor (EFT) is a rare subset of EFTs showing mixed features of Ewing sarcoma and adamantinoma of the long bones. All currently reported cases of the adamantinoma-like type have been associated with bone. Recently, a unique type of EFT was reported showing complex epithelial differentiation associated with the vagus nerve. Here we describe another unique type of EFT arising in the soft tissue of the neck associated with the vagus nerve. An 11-year-old girl presented to our hospital with a neck tumor on her right side. Surgical resection was performed, and histopathologic examination demonstrated a high-grade malignant neoplasm. The tumor was composed of sheets of small round proliferating cells, basaloid tumor nests with marked squamous differentiation, biphasic growth pattern with epithelioid tumor nests, and spindle cell proliferation. Immunohistochemically, the tumor cells showed diffuse expression of CD99 and FLI-1. In addition, small round cells and basaloid/squamoid components were immunoreactive for AE1/AE3, CAM5.2, cytokeratin 5/6, high-molecular weight keratin, p63, and p40 (ΔNp63). Reverse transcription polymerase chain reaction and direct sequencing analysis revealed that the tumor harbored a t(11;22) translocation, involving EWSR1 and FLI-1, which are characteristic of EFTs. According to these findings, our case has characteristics of both a subset of adamantinoma-like EFT and EFT with complex epithelial differentiation. We suggest that EFT with complex epithelial differentiation is in a common spectrum with the adamantinoma-like type and that adamantinoma-like EFTs can arise in soft tissue, leading to difficulty in differential diagnosis with malignant epithelial tumors.

  19. Analysis of the Lambing Process in Suffolk Ewes

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    ŠULCOVÁ, Veronika

    2015-01-01

    The Suffolk is the most well-known of the English lowland sheep breeds. A black-faced sheep with short-stapled wool, the breed is used primarily for meat production. The Suffolk is classified as one of the breeds with the best ability to fatten and its meat yield is approximately 60%. The breed is characterised by its good adaptability to different climate and breeding conditions and overall good health. The ewes are known for their high fertility rate, shorter oestrus cycle, excellent matern...

  20. Synchronization of ovulation and fixed time intrauterine insemination in ewes.

    Science.gov (United States)

    Deligiannis, C; Valasi, I; Rekkas, C A; Goulas, P; Theodosiadou, E; Lainas, T; Amiridis, G S

    2005-02-01

    A novel method for oestrus-ovulation synchronization in sheep followed by fixed time insemination is presented herewith. Mature dry ewes (n = 28) of Karagouniko breed being at an unknown stage of the oestrous cycle, were used during the middle of breeding season. The treatment protocol consisted of an initial administration of a GnRH analogue followed 5 days later by a prostaglandin F2alpha injection. Thirty-six hours later a second GnRH injection was administered to synchronize ovulation, and laparoscopic intrauterine insemination was performed 12-14 h later. Three days after insemination, fertile rams were introduced into the flock twice daily and oestrus-mating detection was carried out. For progesterone (P(4)) determination, blood samples were collected on alternate days, starting 2 days before the first GnRH injection and continuing for 17 days after insemination. An additional sample was taken on the day of insemination. Pregnancy diagnosis was carried out by trans-abdominal ultrasonography. Fourteen ewes (50%) conceived at insemination and maintained pregnancy; from the remainder 14 ewes 10 became pregnant at natural service, while four, although they mated at least two to three times, failed to conceive. In response to the first GnRH, P(4) concentration increased at higher levels in ewes that conceived at AI compared with those that failed to conceive (47.54 and 22.44%, respectively; p < 0.05). Significant differences (p < 0.05) in mean P(4) concentration between pregnant and non-pregnant animals were detected 1 day before AI (0.17 +/- 0.06 and 0.26 +/- 0.14 ng/ml, respectively) on the day of AI (0.15 +/- 0.04 and 0.24 +/- 0.08 ng/ml, respectively) as well as 9 and 11 days thereafter (0.48 +/- 0.12 and 0.38 +/- 0.12 ng/ml; 0.68 +/- 0.14 and 0.50 +/- 0.18 ng/ml, respectively). These results indicate that using the proposed protocol, an acceptable conception rate can be achieved which could be further improved by modifying the time intervals between

  1. A Case of Enzootic Nasal Adenocarcinoma in a Ewe

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    Devorah Marks Stowe

    2012-01-01

    Full Text Available An approximately 2-year-old open Suffolk ewe presented to the North Carolina State University College of Veterinary Medicine Veterinary Health Complex for evaluation of a left nasal mass. An ultrasound-guided aspirate and core biopsies were performed. An epithelial neoplasia with mild mixed inflammation (neutrophils and plasma cells was diagnosed on cytology and confirmed on histopathology. Immunohistochemistry (IHC, reverse transcriptase polymerase chain reaction (RT-PCR, and transmission electron microscopy were also performed. IHC and RT-PCR identified the presence of enzootic nasal tumor virus and confirmed the final diagnosis of enzootic nasal adenocarcinoma.

  2. Estrus synchronization and artificial insemination of hair sheep ewes in the tropics.

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    Godfrey, R W; Collins, J R; Hensley, E L; Wheaton, J E

    1999-04-01

    Hair sheep ewes (St. Croix White and Barbados Blackbelly) were used to evaluate 3 methods of estrus synchronization for use with transcervical artificial insemination (TAI). To synchronize estrus, ewes (n = 18) were treated with PGF2alpha (15 mg, im) 10 d apart, with controlled internal drug release (CIDR) devices containing 300 mg progesterone for 12 d (n = 18), or with intravaginal sponges containing 500 mg progesterone for 12 d (n = 18). On the day of the second PGF2alpha injection or at CIDR or sponge removal, sterile rams were placed with the ewes. Jugular blood samples were collected from the ewes at 6-h intervals until the time of ovulation, and daily for 16 d after estrus (Day 0). Plasma was harvested and stored at -20 degrees C until LH, and progesterone concentrations were determined by RIA. There was no difference (P>0.10) in time to estrus among the CIDR-, PGF2alpha- or sponge-treated ewes. All of the ewes in the CIDR group and 94.4% of the sponge treated ewes exhibited estrus by 36 h after ram introduction, while only 72.2% of PGF2alpha-treated ewes showed signs of estrus by this time (P0.10) among the CIDR-, PGF2alpha- or sponge-treated ewes. The time to the preovulatory LH surge was similar (P>0.10) among CIDR, PGF2alpha and sponge treated ewes. Progesterone levels through Day 16 after the synchronized estrus were not different (P>0.10) among treatment groups. Hair sheep ewes (n = 23) were synchronized using PGF2alpha and bred by TAI using frozen-thawed semen 48 h after the second injection. The conception rate to TAI was 2/23 (8.7%) and produced 3 ram lambs. In a subsequent trial, 17 ewes were synchronized with CIDR devices and bred by TAI using frozen-thawed semen 48 h after CIDR removal, resulting in a conception rate of 52.9% (9/17). It is possible to synchronize estrus in hair sheep using either CIDRs, sponges or PGF2alpha. Even though there were no significant differences in the timing of ovulation or the LH surge among the treatment groups, a

  3. Synovial sarcoma presenting as iliotibial band friction syndrome.

    Science.gov (United States)

    Mesiha, Mena; Bauer, Thomas; Andrish, Jack

    2009-10-01

    Iliotibial band friction syndrome is a common entity that is often quickly diagnosed in orthopedic clinics. However, synovial sarcoma is an elusive clinical entity that appears around many joints with variable presentations. This case report is an example of a patient with a classic presentation of iliotibial band friction syndrome that was diagnosed as a synovial sarcoma on further investigation.

  4. Intimal sarcoma of the pulmonary artery--diagnostic challenge.

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    Fukuda, Wakako; Morohashi, Satoko; Fukuda, Ikuo

    2011-08-01

    Pulmonary artery intimal sarcoma is a rare tumour and the diagnosis is often delayed. We report the case of a woman with a primary pulmonary artery intimal sarcoma who presented with massive pulmonary embolism. The definitive diagnosis was elucidated after the patient's death by autopsy specimen. We discuss the diagnosis and lessons learned from this case.

  5. The effect of dietary restriction, pregnancy, and fetal type in different ewe types on fetal weight, maternal body weight, and visceral organ mass in ewes.

    Science.gov (United States)

    Scheaffer, A N; Caton, J S; Redmer, D A; Reynolds, L P

    2004-06-01

    Our objectives were to evaluate maternal body changes in response to dietary restriction or the increased nutrient requirement of fetal growth. In Exp. 1, 28 mature crossbred ewes (61.6 +/- 1.8 kg initial BW) were fed a pelleted forage-based diet to evaluate effects of pregnancy and nutrient restriction on visceral organ mass. Treatments were arranged in 2 x 3 factorially, with dietary restriction (60% restriction vs. 100% maintenance) and reproductive status (nonpregnant [NP], d 90 or d 130 of gestation) as main effects. Dietary treatments were begun at d 50 of gestation, and restricted ewes remained at 60% of maintenance throughout the experiment. Nonpregnant and d-90 ewes were fed dietary treatments for 40 d and slaughtered. The d-130 ewes were fed dietary treatments for 80 d and then slaughtered. In Exp. 2, four Romanov ewes were naturally mated (Romanov fetus and Romanov dam; R/ R), and two Romanov embryos were transferred to each of four Columbia recipients (Romanov embryos and Columbia recipient; R/C). Three Columbia ewes were naturally mated (Columbia fetus and Columbia recipient; C/C). In both experiments, maternal organ weights were reported as fresh weight (grams), scaled to empty body weight (EBW; grams per kilogram) and maternal body weight (MBW; grams per kilogram). In Exp. 1, ewe EBW and fetal mass were decreased (P < 0.02) with restriction compared with maintenance. Dietary restriction decreased liver mass (16.7 vs. 14.5 g/kg EBW or 18.8 vs. 16.4 g/kg MBW; P < 0.01), but dietary restriction did not affect total digestive tract mass. In Exp. 2, ewe BW was less for the R/R compared with R/C and C/C (44.8 vs. 110.4 and 98.1 +/- 7.9 kg, respectively; P < 0.01). Fetal weight at d 130 was less for the R/R than for R/C and C/C (2.2 vs. 3.3 and 4.7 +/- 0.3 kg, respectively; P < 0.01) when measured as individual fetuses; however, when measured as total fetal mass carried in each ewe, there was no effect of ewe type. These data suggest that the

  6. The roles and implications of exosomes in sarcoma.

    Science.gov (United States)

    Min, Li; Shen, Jacson; Tu, Chongqi; Hornicek, Francis; Duan, Zhenfeng

    2016-09-01

    Better diagnostic biomarkers and therapeutic options are still necessary for patients with sarcomas due to the current limitations of diagnosis and treatment. Exosomes are small extracellular membrane vesicles that are released by various cells and are found in most body fluids. Tumor-derived exosomes have been proven to mediate tumorigenesis, intercellular communication, microenvironment modulation, and metastasis in different cancers, including in sarcomas. Recently, exosomes have been considered as potential biomarkers for sarcoma diagnosis and prognosis, and as possible targets for sarcoma therapy. Moreover, due to their specific cell tropism and bioavailability, exosomes can also be engineered as vehicles for drug delivery. In this review, we discuss recent advances in the roles of tumor-derived exosomes in sarcoma and their potential clinical applications.

  7. Granulocytic Sarcoma of the Stomach Presenting as Dysphagia during Pregnancy

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    Anuradha Sekaran

    2011-01-01

    Full Text Available Granulocytic sarcoma also known as extramedullary myeloid sarcoma or chloroma is an uncommon manifestation of leukemia and presents as a deposit of leukemic cells outside the bone marrow. We report a case of a twenty-five-year-old pregnant woman who presented with progressive dysphagia and recurrent postprandial vomiting. Upper GI endoscopy had shown large flat laterally spread nodular lesions in the cardia and proximal body of stomach. Biopsies from the gastric lesion showed granulocytic sarcoma of the stomach. Concurrent peripheral and bone marrow picture was suggestive of acute myeloid leukemia (AML–M4. There is limited reported literature on granulocytic sarcoma of the stomach. Concurrent gastric granulocytic sarcoma involving cardia and AML in pregnancy has not been reported till date.

  8. Two Cases of Ectopic Hamartomatous Thymoma Masquerading as Sarcoma

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    Sato, Yukiko; Tanaka, Hiroko; Sasaki, Toru; Kawabata, Kazuyoshi; Mitani, Hiroki; Yonekawa, Hiroyuki; Fukushima, Hirofumi; Shimbashi, Wataru

    2017-01-01

    Ectopic hamartomatous thymoma (EHT) is an extremely rare benign tumor. EHTs are difficult to differentiate from sarcomas, especially synovial sarcomas. We encountered two cases of EHT that were referred from other hospitals because sarcoma was suspected. In these cases, fusion gene detection via polymerase chain reaction or fluorescence in situ hybridization was useful for differentiating EHT from synovial sarcoma. EHT requires accurate diagnosis before surgery to avoid excessive treatment. Both tumor location and the presence of fat inside the tumor are important imaging findings for EHT, and confirmation of spindle cells, epithelial cells, and mature adipose cells in the tumor is an important pathological finding. It is important to exclude synovial sarcoma from the differential diagnosis via fusion gene analysis. PMID:28168073

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

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    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.

  10. Ovarian responses to undernutrition in pregnant ewes, USA

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    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.

  11. Ewe welfare and ovine milk and cheese quality

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    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.

  12. The effect of maternal nutritional status during mid-gestation on placental characteristics in ewes.

    Science.gov (United States)

    Sen, U; Sirin, E; Kuran, M

    2013-02-01

    The aim of this study was to determine the effects of maternal nutritional status during mid-gestation on placental characteristics in ewes. Time of estrus of 3-5 years old Karayaka breed ewes was synchronized and mating was monitored to determine the day 0 of gestation. The ewes had similar body weights (47.8±0.7kg) and loin eye muscle values (thickness; 20.9±1.0mm and fat thickness; 4.7±0.5mm) at mating. The ewes were allocated into two treatment groups at day 30 of gestation; under-fed (UF; n=12) and well-fed (WF; n=13) groups. The ewes in UF group were fed with a diet to provide 50% of their daily requirement from day 30 to day 80 of gestation and 100% of their daily requirement during the rest of the gestation period. The ewes in WF group were fed at least 100% of their daily requirement throughout gestation. The singleton bearing ewes in the UF group had a lesser (Ppregnancies.

  13. Retroperitoneal Sarcoma Target Volume and Organ at Risk Contour Delineation Agreement Among NRG Sarcoma Radiation Oncologists

    Energy Technology Data Exchange (ETDEWEB)

    Baldini, Elizabeth H., E-mail: ebaldini@partners.org [Department of Radiation Oncology, Dana-Farber Cancer Institute, Brigham and Women' s Hospital, Boston, Massachusetts (United States); Abrams, Ross A. [Department of Radiation Oncology, Rush University Medical Center, Chicago, Illinois (United States); Bosch, Walter [Department of Radiation Oncology, Washington University, St. Louis, Missouri (United States); Roberge, David [Department of Radiation Oncology, Centre Hospitalier de l' Universite de Montreal, Montreal, Quebec (Canada); Haas, Rick L.M. [Department of Radiotherapy, Netherlands Cancer Institute, Amsterdam (Netherlands); Catton, Charles N. [Department of Radiation Oncology, Princess Margaret Cancer Centre, Toronto, Ontario (Canada); Indelicato, Daniel J. [Department of Radiation Oncology, University of Florida Medical Center, Jacksonville, Florida (United States); Olsen, Jeffrey R. [Department of Radiation Oncology, Washington University, St. Louis, Missouri (United States); Deville, Curtiland [Department of Radiation Oncology, University of Pennsylvania, Philadelphia, Pennsylvania (United States); Chen, Yen-Lin [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Finkelstein, Steven E. [Translational Research Consortium, 21st Century Oncology, Scottsdale, Arizona (United States); DeLaney, Thomas F. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Wang, Dian [Department of Radiation Oncology, Rush University Medical Center, Chicago, Illinois (United States)

    2015-08-01

    Purpose: The purpose of this study was to evaluate the variability in target volume and organ at risk (OAR) contour delineation for retroperitoneal sarcoma (RPS) among 12 sarcoma radiation oncologists. Methods and Materials: Radiation planning computed tomography (CT) scans for 2 cases of RPS were distributed among 12 sarcoma radiation oncologists with instructions for contouring gross tumor volume (GTV), clinical target volume (CTV), high-risk CTV (HR CTV: area judged to be at high risk of resulting in positive margins after resection), and OARs: bowel bag, small bowel, colon, stomach, and duodenum. Analysis of contour agreement was performed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: Ten radiation oncologists contoured both RPS cases, 1 contoured only RPS1, and 1 contoured only RPS2 such that each case was contoured by 11 radiation oncologists. The first case (RPS 1) was a patient with a de-differentiated (DD) liposarcoma (LPS) with a predominant well-differentiated (WD) component, and the second case (RPS 2) was a patient with DD LPS made up almost entirely of a DD component. Contouring agreement for GTV and CTV contours was high. However, the agreement for HR CTVs was only moderate. For OARs, agreement for stomach, bowel bag, small bowel, and colon was high, but agreement for duodenum (distorted by tumor in one of these cases) was fair to moderate. Conclusions: For preoperative treatment of RPS, sarcoma radiation oncologists contoured GTV, CTV, and most OARs with a high level of agreement. HR CTV contours were more variable. Further clarification of this volume with the help of sarcoma surgical oncologists is necessary to reach consensus. More attention to delineation of the duodenum is also needed.

  14. Acroangiodermatite (pseudo-sarcoma de Kaposi

    Directory of Open Access Journals (Sweden)

    Azulay Rubem David

    2004-01-01

    Full Text Available Acroangiodermatite é enfermidade rara, caracterizada por lesões eritêmato-violáceas bem delimitadas que acometem pernas e pés com aspecto semelhante ao do sarcoma de Kaposi. É relatado o caso de paciente do sexo feminino, de 57 anos, com início súbito de lesões eritêmato-violáceas nas pernas sem outras alterações. O caso acrescenta aprendizado por sua dificuldade diagnóstica e reafirma a importância da imuno-histoquímica. Trata-se da publicação do primeiro caso brasileiro.

  15. Histological variants of cutaneous Kaposi sarcoma

    Directory of Open Access Journals (Sweden)

    Pantanowitz Liron

    2008-07-01

    Full Text Available Abstract This review provides a comprehensive overview of the broad clinicopathologic spectrum of cutaneous Kaposi sarcoma (KS lesions. Variants discussed include: usual KS lesions associated with disease progression (i.e. patch, plaque and nodular stage; morphologic subtypes alluded to in the older literature such as anaplastic and telangiectatic KS, as well as several lymphedematous variants; and numerous recently described variants including hyperkeratotic, keloidal, micronodular, pyogenic granuloma-like, ecchymotic, and intravascular KS. Involuting lesions as a result of treatment related regression are also presented.

  16. 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.

  17. Effect of Map-vaccination in ewes on body condition score, weight and Map-shedding.

    Science.gov (United States)

    Hüttner, Klim; Krämer, Ulla; Kleist, Petra

    2012-01-01

    Vaccination against Mycobacterium avium subspecies paratuberculosis (Map) in sheep receives growing attention worldwide, particularly in countries with national Map control strategies. A field study was conducted, investigating the effect of GUDAIR on body condition, weight and Map-shedding in a professionally managed but largely Map-affected suffolk flock prior and after vaccination. For this, 80 ewes out of 1000 animals were randomly sampled. In the univariate analysis body condition scores of ewes twelve months after vaccination improved significantly compared to those sampled prior to vaccination. At the same time the rate of ewes shedding Map was reduced by 37%.

  18. Effects of progesterone withdrawal on uterine secretion of prostaglandin F2 alpha in response to oxytocin in ewes.

    Science.gov (United States)

    Kaminski, M A; Hayes, S H; Silvia, W J

    1997-01-01

    Two experiments were conducted to determine if withdrawal of progesterone during the luteal phase of the oestrous cycle affected the ability of the ovine uterus to secrete prostaglandin F2 alpha (PGF2 alpha) in response to oxytocin. In Experiment 1, 18 ewes were ovariectomized on Day 9 and Day 12 after oestrus. Ewes were subdivided into three treatment groups (n = 6 per group): Group-1 ewes underwent sham surgery; Group-2 ewes received oestradiol (OVX + O); and Group-3 ewes received oestradiol + progesterone (OVX + O,P). Oxytocin was administered to each ewe on Days 10, 13 and 15 after oestrus. Concentrations of 13, 14-dihydro-15-keto-PGF2 alpha (PGFM) were determined in samples of jugular venous blood for 2 h after oxytocin challenge. The magnitude of the PGFM response 24 h after ovariectomy was greater (P Day 11 and assigned to 1 of 4 treatment groups (n = 6 per group): Group 1, no steroid replacement (OVX); Group 2, oestradiol replacement (OVX + O); Group 3, progesterone replacement (OVX + P); or Group 4, progesterone + oestradiol replacement (OVX + O,P). Ewes received oxytocin on Day 12 and Day 15. On Day 12, uterine secretory responsiveness to oxytocin was greatest in ewes in the OVX + O group (P < 0.1). Responsiveness was low in ewes in the OVX group, as it was in ewes in both groups that received progesterone replacement. Therefore, the increase in uterine secretory responsiveness to oxytocin following progesterone withdrawal is dependent on oestradiol replacement.

  19. Kaposi sarcoma incidence in Mozambique: national and regional estimates.

    Science.gov (United States)

    Meireles, Paula; Albuquerque, Gabriela; Vieira, Mariana; Foia, Severiano; Ferro, Josefo; Carrilho, Carla; Lunet, Nuno

    2015-11-01

    Kaposi sarcoma is expressed in four clinical variants, all associated with human herpes virus type 8 infection, namely, classic, endemic, immunosuppression-related and AIDS-related. The latter currently accounts for most of the burden of Kaposi sarcoma in sub-Saharan Africa, reflecting the frequency of HIV infection and its management. We aimed to estimate the incidence of Kaposi sarcoma in Mozambique and in its provinces. We estimated the number of incident cases of Kaposi sarcoma by adding up the expected number of endemic and AIDS-related cases. The former were estimated from the rates observed in Kyandondo, Uganda (1960-1971). The latter were computed from the number of AIDS-related deaths in each region, assuming that the ratio between the AIDS-related Kaposi sarcoma incident cases and the number of AIDS-related deaths observed in the city of Beira applies to all regions. A total of 3862 Kaposi sarcoma cases were estimated to have occurred in Mozambique in 2007, mostly AIDS-related, in the age group 25-49 years, and in provinces from South/Centre. The age-standardized incidence rates were 36.1/100 000 in men and 11.5/100 000 in women, with a more than three-fold variation across provinces. We estimated a high incidence of Kaposi sarcoma in Mozambique, along with large regional differences. These results can be used to improve disease management and to sustain political decisions on health policies.

  20. Sonographic Findings of Uterine Endometrial Stromal Sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Jeong Ah; Lee, Myung Sook; Choi, Jong Sun [Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2006-12-15

    The study was performed to present the sonographic findings of uterine endometrial stromal sarcoma (ESS). We conducted a retrospective review of sonographic findings of 10 cases that were diagnosed as uterine ESS. The patients ages ranged from 25 to 51 years (mean age: 36.1 years). The reviews focused on the location, margin, size, number and echotexture of the lesions. Hysterectomy (n = 9) and myomectomy (n = 1) were performed and a pathologic diagnosis was obtained in all cases. The masses were located in the uterine wall (n = 6), or they presented as a polypoid mass protruding into the endometrial cavity from the myometrium (n = 3) or as a central cavity mass (n = 1). The lesion margins were smooth (n = 5), ill defined (n = 2), or smooth with partially nodular extensions (n = 3). The maximal mass length was 38 mm to 160 mm with a mean mass length of 83.5 mm. There were single lesions in eight cases and multiple lesions in two cases. The lesion echotextures were hypoechoic solid (n = 3), heterogeneously intermediate echoic (n = 5), diffuse myometrial thickening with heterogeneous echogenicity (n = 1) and septated cystic (n = 1). Endometrial stromal sarcoma presents with four patterns of its sonographic appearance; a polypoid mass with nodular myometrial extension, an intramural mass with an ill defined margin and heterogeneous echogenicity, an ill defined large central cavity mass or, diffuse myometrial thickening.

  1. Primary Synovial Sarcoma of the Kidney

    Directory of Open Access Journals (Sweden)

    Takashi Kawahara

    2009-10-01

    Full Text Available The case was a 40-year-old female. She visited a local doctor with a chief complaint of right side abdominal pain. A right kidney tumor measuring 10 cm in diameter was observed in an abdominal Computed Tomography (CT scan. Based on the CT image, the possibility of angiomiolipoma (AML could not be ruled out, but a high maximum standardized uptake value (SUVmax of 7.8 was observed in a Positron Emission Tomography CT (PET-CT scan and there was a possibility of malignancy. We therefore performed a transperitoneal right radial nephrectomy. Although adhesion of the tumor to the duodenum and the inferior vena cava was observed, it was possible to perform an excision. The tumor accounted for a large proportion of the excised kidney; the surrounding areas had taken on a cyst-like structure, and the interior comprised grayish brittle tissue exhibiting solid growth. Histologically, gland-like and cyst-like structures composed of cylindrical cuboidal cells and mainly characterized by the solid growth of short fusiform-shaped and oval-shaped basophilic cells were observed, and we believed it was a synovial sarcoma. There were no malignant findings in the adrenal gland. There have been approximately 30 reported cases around the world of synovial sarcoma that developed in the kidney, and we herein report this case with bibliographic considerations.

  2. Pulmonary Kaposi sarcoma in six children

    Energy Technology Data Exchange (ETDEWEB)

    Theron, Salomine [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); University of Stellenbosch, Department of Radiology, Tygerberg Hospital, Cape Town (South Africa); Andronikou, Savvas; Plessis, Jaco du; George, Reena; Mapukata, Ayanda; Grobbelaar, Marie; Hayes, Murray [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); Goussard, Pierre [University of Stellenbosch, Department of Child Health, Tygerberg Academic Hospital, Cape Town (South Africa); Wieselthaler, Nicky [University of Cape Town, Department of Radiology, Red Cross Children' s Hospital, Cape Town (South Africa); Davidson, Alan [University of Cape Town, Department of Oncology, Red Cross Children' s Hospital, Cape Town (South Africa)

    2007-12-15

    Pulmonary involvement in Kaposi sarcoma is rare in children and can be difficult to distinguish from other pathology. To describe the radiological findings in paediatric pulmonary Kaposi sarcoma. Sequential chest radiographs of six children and CT scans of four of these children were evaluated retrospectively. Their ages ranged from 18 months to 10 years; four were male and two were female. All six children were HIV-positive. The observers were two radiologists. Chest radiographs revealed air-space (100%) and reticular (83%) opacification in the mid- and lower lung zones; pleural effusions were present in 83% of the children. All the children showed progressive air-space opacification on follow-up radiography. CT demonstrated bilateral air-space opacification in a perihilar distribution in all the children; reticular opacification was seen in 75%. All the children had mediastinal and axillary lymphadenopathy; 75% had bilateral hilar lymphadenopathy. In both adults and children, chest radiography demonstrates perihilar and lower zone involvement. Pleural effusions are more common on radiographs in children. Air-space disease and lymphadenopathy are much more common on CT in children than adults. (orig.)

  3. Metastatic endometrial stromal sarcoma: a case report

    Directory of Open Access Journals (Sweden)

    Shobha S. Pillai

    2014-06-01

    Full Text Available Endometrial Stromal Sarcoma (ESS is a rare slow growing tumour of mesodermal origin arising from the stroma of the endometrium and accounting for less than 1% of all uterine cancers. It is characterized by late recurrences and distant metastases. This report presents a case of ESS in a 40 year old nulliparous woman who had a myomectomy for a clinically suspected Leiomyoma uterus in a local hospital. The histopathological examination of the specimen revealed ESS and the patient was referred to our tertiary institute. Here after investigations including a CT scan which also revealed pulmonary metastases, patient underwent Modified Radical Hysterectomy with Bilateral Salpingo-oophorectomy with pelvic lymph node sampling. Histopathological Examination of the uterine specimen confirmed the diagnosis. The patient was given the option of referral to a thoracic surgeon for resection of the isolated lung metastasis, but she refused this and opted instead for hormone therapy which she is presently undergoing. ESS is a very rare tumour often presenting with clinical and examination findings suggestive of leiomyoma of the uterus and hence misdiagnosed. In cases of rapidly growing tumours and suspicious radiological features, suspect sarcoma and initiate timely diagnosis and proper treatment. Recommended long-term follow up in view of late recurrences. [Int J Reprod Contracept Obstet Gynecol 2014; 3(3.000: 812-815

  4. Multidisciplinary Management of Soft Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Lukas M. Nystrom

    2013-01-01

    Full Text Available Soft tissue sarcoma is a rare malignancy, with approximately 11,000 cases per year encountered in the United States. It is primarily encountered in adults but can affect patients of any age. There are many histologic subtypes and the malignancy can be low or high grade. Appropriate staging work up includes a physical exam, advanced imaging, and a carefully planned biopsy. This information is then used to guide the discussion of definitive treatment of the tumor which typically involves surgical resection with a negative margin in addition to neoadjuvant or adjuvant external beam radiation. Advances in imaging and radiation therapy have made limb salvage surgery the standard of care, with local control rates greater than 90% in most modern series. Currently, the role of chemotherapy is not well defined and this treatment is typically reserved for patients with metastatic or recurrent disease and for certain histologic subtypes. The goal of this paper is to review the current state of the art in multidisciplinary management of soft tissue sarcoma.

  5. Primary intravascular synovial sarcoma: case report.

    Science.gov (United States)

    Tuncer, Osman Nuri; Erbasan, Ozan; Golbasi, Ilhan

    2012-10-01

    Synovial sarcoma (SS), a mesenchymal spindle cell tumor, displays variable epithelial differentiation, including glandular formation, and features a specific chromosomal translocation, t(X;18)(p11;q11). SS accounts for 5% to 10% of soft-tissue sarcomas. These tumors occur mostly in the joints, especially near the knee, but they also occur in other locations. Primary intravascular SS (IVSS) are extremely rare; only 6 well-documented cases have been reported in the English literature. We describe a new case of primary IVSS of the superior vena cava (SVC) in a 16-year-old boy. A transthoracic echocardiogram confirmed a large (4.8 × 4.6 cm) circumscribed mass filling the right atrium, as well as a moderate pericardial effusion. The mass extended from the SVC to the tricuspid valve but did not prevent valve coaptation. Surgery via a transatrial approach revealed a huge mass (8 to 12 cm) attached to the SVC via a 5-mm pedicle. The tumor was excised, and the patient experienced an uneventful postoperative course. Fluorescence in situ hybridization analysis revealed the presence of the SS-specific translocation.

  6. Histiocytic sarcoma that mimics benign histiocytosis.

    Science.gov (United States)

    Boisseau-Garsaud, A M; Vergier, B; Beylot-Barry, M; Nastasel-Menini, F; Dubus, P; de Mascarel, A; Eghbali, H; Beylot, C

    1996-06-01

    A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge.

  7. Primary fibro sarcoma of the heart.

    Science.gov (United States)

    Kabashi, Serbeze; Hoxha, Naim; Gashi, Shkelzen; Ahmegjekaj, Ilir; Bejta, Ilir; Sadiku, Muharrem; Ymeri, Halit; Kabashi, Antigona; Bicaj, Xhavit; Mucaj, Sefedin

    2013-01-01

    Primary malignant heart tumors represent rare entities where fibro sarcoma represents about 3% of all. Introducing the patient: A 15 years old patient with cardiac insufficiency (heart failure) symptoms, such as weakness, cyanosis, palpitations and breathing difficulties; enlargement of upper mediastinum and pleural effusion. Through echocardiography a pericardial effusion and intracavitary thrombus in atrium was diagnosed. With computed tomography is diagnosed a tumoral mass in right atrium which is also spread in the right ventricle of the heart. Tumor is completely removed; pat histology result showed primary fibro sarcoma of the heart. At that time no metastasis was found. Conclusion. Primary malignant heart tumors may manifest like cardiac insufficiency or like systemic diseases. Fibrosarcomas are rare and have bad prognosis. On average patients can live around six months after initial symptoms appeared and diagnosis of the tumor was done. In the case of cardiac insufficiency with differential diagnosis we should also think of heart tumors, which could certainly be proved for or eliminated by echocardiography.

  8. Assessment of histological response of paediatric bone sarcomas using FDG PET in comparison to morphological volume measurement and standardized MRI parameters

    Energy Technology Data Exchange (ETDEWEB)

    Denecke, Timm; Misch, Daniel; Steffen, Ingo G.; Plotkin, Michail; Stoever, Brigitte [Charite - Universitaetsmedizin Berlin, Klinik fuer Strahlenheilkunde, Campus Virchow-Klinikum, Berlin (Germany); Hundsdoerfer, Patrick; Henze, Guenter [Charite - Universitaetsmedizin Berlin, Klinik fuer Paediatrie m.S. Onkologie und Haematologie, Otto-Heubner-Zentrum, Campus Virchow-Klinikum, Berlin (Germany); Schoenberger, Stefan [Universitaetsklinikum der Heinrich-Heine-Universitaet Duesseldorf, Klinik fuer Kinder-Onkologie, -Haematologie und -Immunologie, Duesseldorf (Germany); Furth, Christian; Ruf, Juri [Otto-von-Guericke-Universitaet Magdeburg, Klinik fuer Radiologie und Nuklearmedizin, Universitaetsklinikum Magdeburg A.oe.R., Magdeburg (Germany); Hautzel, Hubertus [Universitaetsklinikum der Heinrich Heine Universitaet Duesseldorf, Nuklearmedizinische Klinik, Duesseldorf (Germany); Kluge, Regine [Universitaetsklinikum Leipzig A.oe.R., Klinik und Poliklinik fuer Nuklearmedizin, Leipzig (Germany); Bierbach, Uta [Universitaetsklinikum Leipzig A.oe.R., Abteilung fuer Kinder-Haematologie, -Onkologie und -Haemostaseologie, Leipzig (Germany); Otto, Sylke [Universitaetsklinikum Greifswald, Institut fuer Diagnostische Radiologie und Neuroradiologie, Greifswald (Germany); Beck, James F. [Universitaetsklinikum Greifswald, Abteilung fuer Paediatrische Haematologie und Onkologie, Greifswald (Germany); Franzius, Christiane [MR- und PET/CT-Zentrum, Bremen-Mitte (Germany); Universitaetsklinikum Muenster, Klinik und Poliklinik fuer Nuklearmedizin, Muenster (Germany); Amthauer, Holger [Charite - Universitaetsmedizin Berlin, Klinik fuer Strahlenheilkunde, Campus Virchow-Klinikum, Berlin (Germany); Otto-von-Guericke-Universitaet Magdeburg, Klinik fuer Radiologie und Nuklearmedizin, Universitaetsklinikum Magdeburg A.oe.R., Magdeburg (Germany)

    2010-10-15

    The objective of this study was to evaluate positron emission tomography (PET) using {sup 18}F-fluoro-2-deoxy-D-glucose (FDG) in comparison to volumetry and standardized magnetic resonance imaging (MRI) parameters for the assessment of histological response in paediatric bone sarcoma patients. FDG PET and local MRI were performed in 27 paediatric sarcoma patients [Ewing sarcoma family of tumours (EWS), n = 16; osteosarcoma (OS), n = 11] prior to and after neoadjuvant chemotherapy before local tumour resection. Several parameters for assessment of response of the primary tumour to therapy by FDG PET and MRI were evaluated and compared with histopathological regression of the resected tumour as defined by Salzer-Kuntschik. FDG PET significantly discriminated responders from non-responders using the standardized uptake value (SUV) reduction and the absolute post-therapeutic SUV (SUV2) in the entire patient population ({nabla}SUV, p = 0.005; SUV2, p = 0.011) as well as in the subgroup of OS patients ({nabla}SUV, p = 0.009; SUV2, p = 0.028), but not in the EWS subgroup. The volume reduction measured by MRI/CT did not significantly discriminate responders from non-responders either in the entire population (p = 0.170) or in both subgroups (EWS, p = 0.950; OS, p = 1.000). The other MRI parameters alone or in combination were unreliable and did not improve the results. Comparing diagnostic parameters of FDG PET and local MRI, metabolic imaging showed high superiority in the subgroup of OS patients, while similar results were observed in the population of EWS. FDG PET appears to be a useful tool for non-invasive response assessment in the group of OS patients and is superior to MRI. In EWS patients, however, neither FDG PET nor volumetry or standardized MRI criteria enabled a reliable response assessment to be made after neoadjuvant treatment. (orig.)

  9. AIDS-related primary Kaposi sarcoma of the nasopharynx.

    Science.gov (United States)

    Çelenk, Fatih; Yilmaz, Metin; Asal, Korhan; Ekinci, Özgür; Tokgöz, Nil

    2011-06-01

    Primary nasopharyngeal Kaposi sarcoma is extremely rare, as only 1 case has been previously reported in the literature. We report a new case, which occurred in a 37-year-old man with a known history of acquired immune deficiency syndrome (AIDS). The patient presented with complaints of recurrent epistaxis and postnasal hemorrhage. Endoscopic examination detected a bluish, smooth, firm, nonpulsatile mass in the nasopharyngeal wall. Histopathologic findings on biopsy were consistent with Kaposi sarcoma. The tumor was successfully treated with radiotherapy. Kaposi sarcoma should be considered in the differential diagnosis of any AIDS patient who presents with recurrent unilateral nasal bleeding.

  10. Pseudo-Kaposi sarcoma (acroangiodermatitis): occurring after bullous erysipelas.

    Science.gov (United States)

    Kutlubay, Zekayi; Yardimci, Gürkan; Engin, Burhan; Demirkesen, Cuyan; Aydin, Övgü; Khatib, Rashid; Tuzun, Yalçın

    2015-05-18

    Pseudo-Kaposi sarcoma is a benign reactive vascular proliferative disorder, which can be seen at any age. It occurs when the chronic venous pressure changes result in vascular proliferation in the upper and mid dermis. This disease is divided into two subtypes: the most frequent subtype is the Mali type and seen in early ages. The Mali type is seen in chronic venous insufficiency and in those patients with arteriovenous shunts. The rare subtype is the Stewart-Bluefarb type. This disease must be distinguished from Kaposi sarcoma because of their clinical resemblance. Herein, we present a patient with pseudo-Kaposi sarcoma, which developed after bullous erysipelas.

  11. Myeloid Sarcoma Presenting as Multiple Lymphadenopathy: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Hong, Sun Hwa; Suh, Sang Il; Seol, Hae Young; Cho, Jea Gu; Shin, Bong Kyung [Guro Hospital, Korea University College of Medicine, Seoul (Korea, Republic of)

    2010-10-15

    Myeloid sarcoma manifesting as multiple lymphadenopathy is quite rare. We present here a case of myeloid sarcoma that first presented with palpable bilateral neck masses. A 53-year-old woman complained about repetitive swelling in the right infraauricular and submental areas for 3 years. The results of computed tomography showed multiple lymphadenopathy in both areas of the neck as well as other parts of the body. So, the presumptive diagnosis was lymphoma, but the result of the excisional biopsy of the neck mass confirmed it to be a myeloid sarcoma.

  12. Extremely underwound chromosomal DNA in nucleoids of mouse sarcoma cells.

    Science.gov (United States)

    Hartwig, M; Matthes, E; Arnold, W

    1981-07-01

    The superhelical properties of chromosomal DNA from cells of a mouse sarcoma were investigated in neutral sucrose gradients containing ethidium bromide. Removal of negative supercoiling from the DNA of the sarcoma cells required a substantially higher dye concentration than was necessary in the case of DNA from cultured mouse fibroblasts. The calculated value of the mean superhelical density in malignant cells (sigma = -0.14) appears abnormally high compared with the value (sigma = -0.09) obtained for DNA of mouse fibroblasts. Chromosomal DNA from mouse sarcoma cells is therefore concluded to be highly deficient in helical turns.

  13. 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

  14. The importance of PET/CT in the evaluation of patients with Ewing tumors

    Directory of Open Access Journals (Sweden)

    Júlio Brandão Guimarães

    2015-06-01

    Full Text Available Abstract 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.

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

    DEFF Research Database (Denmark)

    Nielsen, Mette Vestergaard; Nadeau, E.; Markussen, Bo

    2015-01-01

    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...... × ME02 × CIcor, where MEI is the daily metabolizable energy intake, ME0 is considered the theoretical maximum intake capacity of the animal in a theoretical situation with no physical constraint on intake, and parameter k represents the decline in MEI with the increasing CIcor of the ration. The model...

  16. Influence of growth hormone on growth and onset of puberty of Rahmani ewe lamb

    Directory of Open Access Journals (Sweden)

    K.H. El-Shahat

    2014-09-01

    Conclusion: Somatotropin administration enhanced puberty in Rahmani ewe lambs. This is due to increased provision of trophic signals (represented by increased Serum IGF-1 secretions and/or blood-borne metabolites (glucose, cholesterol and lipid.

  17. 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)

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

    Directory of Open Access Journals (Sweden)

    Eiríksson Tryggvi

    2008-06-01

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

  19. Granulocytic sarcoma masquerading as Ewing′s sarcoma: A diagnostic dilemma

    Directory of Open Access Journals (Sweden)

    Haresh Kunhi

    2008-01-01

    Full Text Available An eleven-year-old boy presented with a swelling in his left elbow. Radiologically the features were that of an Ewing′s sarcoma involving the ulna. Histopathology showed small round cell tumor strongly positive for Monoclonal Imperial Cancer research fund 2 (MIC2 antigen. Similar cells in the bone marrow were involved with MIC2 positivity. The patient developed skin lesions, which on biopsy were found to be chloromas. The initial biopsies were reevaluated with special stains revealing granulocytic sarcomas in acute myeloid leukemia masquerading as Ewing′s due to its MIC2 positivity. The possibility of myeloid neoplasms should be considered routinely with known MIC2 positive round cell tumors.

  20. Parturition induction in ewes by a progesterone receptor blocker, aglepristone, and subsequent neonatal survival: Preliminary results.

    Science.gov (United States)

    Özalp, R G; Yavuz, A; Orman, A; Seker, I; Udum Küçükşen, D; Rişvanlı, A; Demiral, Ö O; Wehrend, A

    2017-01-01

    The clinical effects of aglepristone treatment to induce parturition in ewes and their newborns were reported. Three experimental groups were defined: group AG5 (n = 5), group AG10 (n = 5), and group CG (n = 5) in which ewes were injected twice with 5, 10 mg/kg of aglepristone, and saline solution of ewes, respectively. Different parameters associated with parturition in ewes and their newborns were investigated. Serum progesterone, oxytocin, and free and conjugated total estrogens were measured after treatments until parturition. No statistical difference was found from first aglepristone administration to onset of lambing between AG5 and AG10 (23.90 ± 6.20, 40.00 ± 6.71 hours). Parturition induction in two groups shortened the gestational length significantly compared with the control group (P = 0.003). Dystocia was observed in two ewes in group AG10. The placental weight showed statistically significant difference only between the AG10 and CG (P = 0.039), but no difference was observed in the placental expulsion period between the groups. Decrease in food consumption 24 to 36 hours after parturition in all ewes and skin necrosis in an ewe in group AG5 were observed. Progesterone concentration was significantly lower in AG5 than that in ewes in group AG10 and CG (P birth weight (4.29 ± 0.28 kg), which was significantly different from the induced groups. No significant difference of blood pH and blood gases values between groups was identified both at birth and 12 hours after parturition for lambs. Significant differences could clearly be observed in total protein and blood urea nitrogen and total protein findings 12 hours after parturition (P control lambing time without any side effects in either mothers or lambs.

  1. Effect of ewe and lamb genotype on gestation length, lambing ease and neonatal behaviour of lambs.

    Science.gov (United States)

    Dwyer, C M; Lawrence, A B; Brown, H E; Simm, G

    1996-01-01

    To distinguish between ewe and lamb breed effects on prenatal growth, ease of parturition and early lamb behaviour, an embryo-transfer study was carried out using a hill breed (Scottish Blackface; liveweight: 54.25 +/- 1.03 kg, mean +/- s.e.m.) and a lowland breed (Suffolk; 80.33 +/- 1.52 kg) to obtain the four possible combinations of ewe and lamb. Data were collected from 38 Blackface ewes (18 with Blackface lambs and 20 with Suffolk lambs) and 41 Suffolk ewes (20 with Blackface lambs and 21 with Suffolk lambs); all ewes were given single embryos. Suffolk lambs had a significantly longer gestation than Blackface lambs (1.5 days, P < 0.01), regardless of ewe breed. Suffolk lambs also had a longer labour (20 min, P < 0.05) and were significantly more likely to require birth assistance (17/21, 81% of all assisted deliveries; P < 0.001), as were male lambs (19/21, 90%; P < 0.01). These variables were independent of ewe breed. Blackface lambs were significantly more active than Suffolk lambs in the first 2 h after birth; ewe breed had little effect on lamb behaviour. Blackface lambs stood twice as quickly as Suffolk lambs after birth (13 min v. 24 min; P < 0.001), and were significantly more likely to suckle within the first 2 h after birth (92% v. 66%; P < 0.05). The behavioural retardation of Suffolk lambs may be a consequence of their birth difficulty which increases their likelihood of suffering birth trauma and hypoxia at parturition. Together, these factors may increase the probability of neonatal death in these lambs.

  2. Effect of undernutrition in foetal life on energy expenditure during gestation in ewes

    DEFF Research Database (Denmark)

    Kiani, Alishir; Chwalibog, André; Tygesen, Malin P;

    2008-01-01

    The long-term effect of early life undernutrition on late gestation energy expenditure (EEgest) was investigated in sheep. Ewes were fed either adequate (100%) or restricted (60%) energy and protein during late foetal life as well as during last trimester of gestation later in life, resulting in ...... nutrient restriction in early life impairs the ability of ewes to respond to nutritional restriction in terms of energy expenditure of gestation....

  3. Radioimmunoimaging of osteogenic sarcoma xenografts in nude mice using monoclonal antibodies to osteogenic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Sakahara, H.; Endo, K.; Nakashima, T.; Koizumi, M.; Ohta, H.; Kunimatsu, M.; Torizuka, K.; Nakamura, T.; Tanaka, H.; Kotoura, Y.

    1985-05-01

    The authors have developed several monoclonal antibodies against human osteogenic sarcoma, one of which; OST7 (IgGl) selectively localized in osteogenic sarcoma xenografts in nude mice. In the present study, F(ab')/sub 2/ fragment was compared with whole IgG and those labeled with In-111 as well as I-131 were used as a radiotracer for the scintigraphic imaging of tumors. IgC and F(ab')/sub 2/ were labeled with I-131 using chloramine-T method and injected into nude mice bearing human osteogenic sarcoma. Scintigrams at day 2 clearly delineated the site of tumors with almost no radioactivity in other organs with F(ab')/sub 2/, which yielded much better images than whole IgG. Tumor-to-blood ratio of 6.09-27.87 was obtained at day 2 using F(ab')/sub 2/, whereas it was 0.76-1.12 at day 2 and 2.05-3.27 at day 7 with IgG. I-131 labeled nonspecific F(ab')/sub 2/ or IgG resulted in no or very low tumor uptake with tumor-to-blood ratio of 0.94-1.18 at day 2 for F(ab')/sub 2/ and 0.67-0.76 at day 7 for IgG, respectively. In-111 labeled F(ab')/sub 2/ fragment of OST7, which was prepared using DTPA as a bifunctional chelate, also showed a high tumor accumulation with tumor-to-blood ratio of 11.67-17.54 at day 2, but higher background activity in the liver and kidney was observed than I-131 labeled one. These results indicate that F(ab')/sub 2/ fragment of OST7 labeled with either I-131 or In-111, has a great potential for the radioimmunoimaging of osteogenic sarcoma.

  4. Management of fetal dystocia caused by carpal flexion in ewe: A case report

    Directory of Open Access Journals (Sweden)

    Faez Firdaus Jesse Abdullah

    2015-06-01

    Full Text Available Dystocia or difficulty in parturition in a ewe may need veterinary assistance for the successful parturition. An adult ewe weighing 30 kg was presented to the Hospital of Veterinary Medicine, Universiti Putra Malaysia with the history of difficulty in giving birth. Physical examination of the ewe revealed that the animal was weak and in recumbent position. Head of a dead fetus was observed as protruding out from the vulva region. Through physical evaluation of the ewe by per vaginal examination, the condition was diagnosed as fetal dystocia. Treatment and management plans given to the ewe were episiotomy and manipulative delivery of the dead fetus via mutation and traction method. Post-operative treatment was given with Flunixin meglumine (dosed at 2.2 mg/kg bwt for 3 days, and Norodine (dosed at 1 mL/16-kg bwt once intramuscularly. The case was completely cured after 2 weeks. The risk of losing the lamb as well as the ewe increases with delay in treatment of dystocia.

  5. A preliminary study of the effects of organic farming on oocyte quality in ewe lambs.

    Science.gov (United States)

    Casao, A; María, G A; Abecia, J A

    2017-02-01

    This study tested whether feeding Rasa Aragonesa ewes certified organic feed, from 15 days before mating until lamb weaning, improved oocyte quality and in vitro maturation (IVM) and fertilization (IVF) performances of the offspring. In a second experiment, ovaries from ewe lambs that were bred on an organic farm and were of the same breed were compared with those from conventionally bred animals. The number (± standard error of the mean) of healthy oocytes per ewe lamb did not differ significantly between organic (12.2 ± 3.3) and conventionally (13.6 ± 4.0) fed ewes. Ovaries from ewe lambs born on an organic farm had significantly (P farm (25.0 ± 4.2), and higher IVM (76.5% vs. 53.1%, P organic procedures on the sheep oocyte quality indicates that the total integration in the complete organic system improved the oocyte quality of ewe lambs, although organic feeding alone was insufficient to improve quality.

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

    Science.gov (United States)

    Viñoles, C; Meikle, A; Martin, G B

    2009-07-01

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

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

    Science.gov (United States)

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

    1990-11-01

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

  8. Myeloid sarcoma of the rib: An atypical isolated chest finding

    Directory of Open Access Journals (Sweden)

    Antonio Raucci

    2015-03-01

    Systemic treatment was administered and currently neither systemic nor local relapse has been identified. Our experience suggests surgical resection could be a valid treatment in isolated myeloid sarcoma patients.

  9. Fine needle cytology of Kaposi's sarcoma in heterosexual male

    Directory of Open Access Journals (Sweden)

    Anjali R. Dhote

    2014-04-01

    Full Text Available Kaposi's sarcomas the most common malignancy associated with Human Herpesvirus-8 (HHV8 infection. Though name is sarcoma but it is low grade vascular neoplasm. It is the tumour which arises from endothelial lining of vessels as well as lymphatic channels. So it involved all sites such as skin, Gastro intestine, lungs along with lymph nodes. We are presenting one such case of 65 year immunocompromised Indian male presented with multiple non blanching reddish bluish nodules on all extremities, chest, back with submandibular and cervical lymphadenopathy. Fine needle aspiration cytology (FNAC was performed and diagnosis was given low grade spindle cell neoplasm consistent with Kaposi's sarcoma which was confirmed on histopathology as Kaposi's sarcoma. [Int J Res Med Sci 2014; 2(2.000: 789-791

  10. Dasatinib and Doxorubicin Treatment of Sarcoma Initiating Cells

    DEFF Research Database (Denmark)

    Aggerholm-Pedersen, Ninna; Demuth, Christina; Safwat, Akmal;

    2016-01-01

    Background. One of the major challenges affecting sarcoma treatment outcome, particularly that of metastatic disease, is resistance to chemotherapy. Cancer-initiating cells are considered a major contributor to this resistance. Methods. An immortalised nontransformed human stromal (mesenchymal) s...

  11. Sarcoma of the breast: an update on a rare entity.

    Science.gov (United States)

    Lim, Sue Zann; Ong, Kong Wee; Tan, Benita Kiat Tee; Selvarajan, Sathiyamoorthy; Tan, Puay Hoon

    2016-05-01

    Breast sarcoma is a rare condition. It consists of a heterogeneous group of non-epithelial tumours arising from the mesenchymal tissue of the breast. It has a distinctly different natural history, treatment response and prognosis as compared with carcinoma of the breast. A different diagnostic approach and treatment strategy have to be defined for this group of tumours. Due to its rarity, the current understanding on breast sarcoma is limited and is mostly based on small retrospective case series or case reports. Hence, the management generally follows the algorithms derived from randomised control trials of soft tissue sarcomas in the extremities and chest wall. Through this review, we discuss the results of major retrospective studies on breast sarcomas including data on epidemiology, aetiology, diagnostic approach, treatment strategies and outcomes of this challenging and potentially aggressive condition.

  12. Radiation-induced spindle cell sarcoma: A rare case report

    Directory of Open Access Journals (Sweden)

    Khan Mubeen

    2009-01-01

    Full Text Available Ionizing radiation has been known to induce malignant transformation in human beings. Radiation-induced sarcomas are a late sequel of radiation therapy. Most sarcomas have been reported to occur after exposure to a radiation dose of 55 Gray (Gy and above, with a dose ranging from 16 to 112 Gys. Spindle cell sarcomas, arising after radiotherapy given to treat the carcinoma of head and neck region is a very uncommon sequel. This is a rare case report of spindle cell sarcoma of left maxilla, in a 24-year-old male, occurring as a late complication of radiotherapy with Cobalt-60 given for the treatment of retinoblastoma of the left eye 21 years back.

  13. Pulmonary Kaposi's sarcoma after heart transplantation: a case report

    Directory of Open Access Journals (Sweden)

    Kristiansen Glen

    2010-07-01

    Full Text Available Abstract Introduction Kaposi's sarcomas have been associated with different conditions of immunosuppression and are also known to be a typical complication of solid organ transplantations. Case presentation We report the case of a 65-year-old Turkish man with a history of heart transplantation 10 months ago who presented for clarification of his dyspnea. The patient had a known history of chronic obstructive pulmonary disease and a smoking history of 40 pack years. Radiologically, three progressively growing intra-pulmonary nodules were detected. The histology was diagnostic for a Kaposi's sarcoma. Visceral and especially primary intra-pulmonary Kaposi's sarcomas are very rare and have been described to have a rather unfavorable prognosis. Conclusions Even with a history suggestive for conventional lung cancer, Kaposi's sarcomas should be considered in patients after transplantation of solid organs. It should be noted that in a minority of cases this tumor exists in the absence of the typical cutaneous lesions.

  14. Breast sarcoma. A case report and review of literature

    Directory of Open Access Journals (Sweden)

    Nuria Li

    2016-01-01

    Conclusion: Surgery represents the only potentially curative therapy for breast sarcoma. Tumor size and adequate resection margin are the most important prognostic factors. Approximately 80% of recurrences appear in the first two years.

  15. Activity of alkaline and acidic phosphatase in glandular cells of uterine endometrium of puerperal ewes after exposure to polychlorinated biphenyls

    OpenAIRE

    Valocky I.; Krajničakova Maria; Legath J.; Lenhardt L.; Ostro A.; Danko J.; Tkačikova L`udmila; Mojžišova Jana; Fialkovičova Maria; Mardzinova Silvia

    2005-01-01

    The study is focused on the observation of alkaline and acidic phosphatase activity in the glandular cells of uterine endometrium in puerperal ewes after exposure to polychlorinated biphenyls. Ewes of Slovak merino breed (n=25) divided into 2 groups were included in the experiment. The animals in the experimental group (n=14) and control group (n=11) were euthanised on day 17, 25 and 34 postpartum. The ewes in the experimental group were given per os capsules of the chemical preparation Delor...

  16. Effects of calcium salts of fatty acids (Megalac) on reproductive performance and blood parameters of Kalkohi ewes

    OpenAIRE

    2012-01-01

    To determine the effects of Megalac addition on Iranian ewes' reproductive parameters, a complete randomized design was used. Multiparous Kalkohi ewes (n = 32; 3 years old) were randomly assigned to 2 groups which consumed isoenergetic and isonitrogenous control (C) or Megalac (M; 5% Megalac in diet DM) diet. All ewes were synchronized by prostaglandin injection. Blood samples were collected from d 8, 10, and 12 of estrus cycle. Diets offered 4 wk prior to mating and up to 4 wk after mating a...

  17. Study of Blood Metabolites Changes of Purebred Ghezel and Crossbred Arkhar Merinos × Ghezel Ewes during Late Pregnancy

    OpenAIRE

    2016-01-01

    Introduction As pregnancy progresses, nutrient requirements of fetus and thereby ewe increases and some changes in the levels of blood metabolites including glucose, cholesterol and total protein may occur. During gestation, maternal tissues contribute to supplying energy that required for fetus resulting in changes of ewe blood metabolites; however other factors such as breed, age, feeding type of ewes during gestation and season may influence them. The study of blood metabolic changes in di...

  18. Sarcoma cutáneo mixto radioinducido Radiation-induced mixed cutaneous sarcoma

    Directory of Open Access Journals (Sweden)

    Isidoro Rubio-Correa

    2012-06-01

    Full Text Available Introducción: Los sarcomas son tumores malignos poco frecuentes, siendo raros en cabeza y cuello. En su etiología se involucran factores como agentes químicos, radiación, inmunosupresión y síndromes y anomalías genéticas. Caso clínico: Varón de 64 años, que presenta lesión en piel de mejilla derecha de un año de evolución, localización en la que presentó hace veinte años un carcinoma basocelular tratado con radioterapia. Tras descartar existencia de metástasis, se realizó exéresis de la lesión con márgenes de seguridad y reconstrucción con colgajo de Mustardé. Se complementó el tratamiento con radioterapia. Discusión: El diagnóstico es anatomopatológico, siendo fundamental descartar afectación metastásica. Para mejorar la supervivencia y disminuir su elevada tasa de recidiva, deberían tratarse de forma multidisciplinar (cirugía, radioterapia y/o quimioterapia. Conclusión: A pesar de su baja frecuencia, los sarcomas deben estar presentes en el diagnóstico diferencial de toda lesión que aparezca en zonas radiadas previamente, especialmente en la piel facial.Introduction: Sarcomas are malignant tumors that are infrequent, being rare in the head and neck. Factors such as chemical agents, radiation, immunosuppression, and genetic syndromes and abnormalities are involved in their etiology. Case report: A 64-year-old man developed a skin lesion on the right cheek one year earlier at the site where he had presented a basal cell carcinoma 20 years earlier that was treated with radiation therapy. After ruling out the existence of metastasis, the lesion was treated by surgical resection with safety margins and reconstruction with the Mustardé flap. Treatment was supplemented with radiation therapy. Discussion: The diagnosis of sarcomas is histopathologic and it is essential to rule out metastasis. To improve survival and reduce the high rate of recurrence, a multidisciplinary approach to treatment should be used (surgery

  19. Recent Progress in the Management of Retroperitoneal Sarcoma

    Directory of Open Access Journals (Sweden)

    Rona Cheifetz

    2001-01-01

    Full Text Available Retroperitoneal sarcomas (RPS are rare tumours that typically present late and carry a poor prognosis even following grossly complete resection. In an attempt to improve the outlook for patients with RPS, sarcoma specialists have employed various adjuvant therapies, including extermal beam radiation, intraoperative radiation, brachyradiation and systemic chemotherapy. This article reviews the presentation and prognosis of RPS, and focuses on the results of new treatment strategies compared with conventional management.

  20. Hepatic involvement of histiocytic sarcoma: CT and MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Kubo, Takatosh; Ohtomo, Kuni [Graduate School of Medicine, University of Tokyo, Tokyo (Japan); Kiryu, Shigeru; Akai, Hiroyuki; Ora, Yasunori; Tojo, Arinobu; Yoshida, Hideo; Kato, Naoya; Nakano, Yoshiyasu [Institute of Medical Science, University of Tokyo, Tokyo (Japan)

    2016-09-15

    Histiocytic sarcoma in the liver is an extremely rare hematological malignancy. Herein, we reported the case of a 68-year-old woman who presented with characteristic wedge-shaped abnormality bounded by hepatic veins on computed tomography and magnetic resonance imaging of the liver. In the wedge-shaped area, decreased portal flow and the deposition of iron were observed. These imaging findings are consistent with intrasinusoidal tumor cell infiltration. A liver biopsy was performed, and histiocytic sarcoma was confirmed histopathologically.

  1. Hepatic Involvement of Histiocytic Sarcoma: CT and MRI Findings

    Energy Technology Data Exchange (ETDEWEB)

    Kubo, Takatoshi [Department of Radiology, Graduate School of Medicine, University of Tokyo, Tokyo 113-8654 (Japan); Kiryu, Shigeru; Akai, Hiroyuki [Department of Radiology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Ota, Yasunori [Department of Pathology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Tojo, Arinobu [Department of Hematology and Oncology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Yoshida, Hideo [Department of Gastroenterology, Japanese Red Cross Medical Center, Tokyo 150-8935 (Japan); Kato, Naoya [Advanced Medical Science, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Nakano, Yoshiyasu [Department of Radiology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Ohtomo, Kuni [Department of Radiology, Graduate School of Medicine, University of Tokyo, Tokyo 113-8654 (Japan)

    2016-11-01

    Histiocytic sarcoma in the liver is an extremely rare hematological malignancy. Herein, we reported the case of a 68-year-old woman who presented with characteristic wedge-shaped abnormality bounded by hepatic veins on computed tomography and magnetic resonance imaging of the liver. In the wedge-shaped area, decreased portal flow and the deposition of iron were observed. These imaging findings are consistent with intrasinusoidal tumor cell infiltration. A liver biopsy was performed, and histiocytic sarcoma was confirmed histopathologically.

  2. Myxoinflammatory fibroblastic sarcoma: An uncommon tumour at an unusual site

    Directory of Open Access Journals (Sweden)

    Varuna Mallya

    2014-01-01

    Full Text Available Myxoinflammatory fibroblastic sarcoma is a low grade sarcoma that is composed of a mixed inflammatory infiltrate along with spindled, epithelioid and bizarre appearing cells in a background of hyaline and myxoid zones. Seen affecting the distal extremities commonly, with an equal sex predilection, these tumors are rare and require an extensive immunohistochemical work up for proper diagnosis. They have a tendency to recur.

  3. Osteogenic Sarcoma: A 21st Century Review.

    Science.gov (United States)

    Osasan, Stephen; Zhang, Mingyong; Shen, Fan; Paul, Paulose J; Persad, Sujata; Sergi, Consolato

    2016-09-01

    Compared to other bone tumors, bone osteogenic sarcoma (BOS) continues to confer a much grimmer prognosis as the survival benefit of traditional chemotherapy treatment regimens is still unsatisfactory. Chemotherapy was demonstrated to be effective in eradicating both primary tumor and pulmonary metastases in the last century, with effective agents used in various combination regimens having changed the survival rate from less than 10% to 75%. The most common primary bone cancer, BOS is conventionally a primary intramedullary high-grade malignant tumor characterized by malignant cells forming immature bone or osteoid. BOS is a disease with diverse morphological presentations. The treatment of all morphological variants seem to have been the same for over 30 years. The introduction of antiproliferative agents such as insulin growth factor-binding protein 3 hold promise of a potentially veritable therapeutic target. In this review, we highlight recent data on osteosarcoma to consolidate a platform able to connect bench and bedside.

  4. Primary Kaposi sarcoma of the subcutaneous tissue

    Directory of Open Access Journals (Sweden)

    Dezube Bruce J

    2008-09-01

    Full Text Available Abstract Background Involvement of the subcutis by Kaposi sarcoma (KS occurs primarily when cutaneous KS lesions evolve into deep penetrating nodular tumors. Primary KS of the subcutaneous tissue is an exceptional manifestation of this low-grade vascular neoplasm. Case presentation We present a unique case of acquired immune deficiency syndrome (AIDS-associated KS manifesting primarily in the subcutaneous tissue of the anterior thigh in a 43-year-old male, which occurred without overlying visible skin changes or concomitant KS disease elsewhere. Radiological imaging and tissue biopsy confirmed the diagnosis of KS. Conclusion This is the first documented case of primary subcutaneous KS occurring in the setting of AIDS. The differential diagnosis of an isolated subcutaneous lesion in an human immunodeficiency virus (HIV-infected individual is broad, and requires both imaging and a histopathological diagnosis to guide appropriate therapy.

  5. Cytogenetics Findings in a Histiocytic Sarcoma Case

    Science.gov (United States)

    Alonso-Dominguez, J. M.; Calbacho, M.; Talavera, M.; Villalon, C.; Abalo, L.; Garcia-Gutierrez, J. V.; Lozano, S.; Tenorio, M.; Villarrubia, J.; Lopez-Jimenez, J.; Ferro, M. T.

    2012-01-01

    Histiocytic sarcoma (HS) is a neoplasm derived from histiocytes. Its diagnosis was not clear until its immunohistochemistry profile was correctly established. Not much is known about its genetic properties. We report a case of a 48-year-old male patient whose bone marrow was almost completely occupied by monomorphic medium size neoplastic cellularity. Its immunohistochemical profile was CD68+, CD4+, CD45+ with negativity of other dendritic cells, and other lineage markers. Cytogenetic study showed 4 related clones: one with trisomy 8 and extra material on the short arms of chromosome 4; a second line with tetrasomy of chromosome 8, add(4)(p16); the third clone had the same alterations as the previous and deletion of chromosome 3 at q11; the fourth line had tetrasomy 8 and translocation t(3;5)(q25;q35). To our knowledge this is the first HS case showing chromosome 8 trisomy and tetrasomy and the other described alterations. PMID:22937328

  6. Cytogenetics Findings in a Histiocytic Sarcoma Case

    Directory of Open Access Journals (Sweden)

    J. M. Alonso-Dominguez

    2012-01-01

    Full Text Available Histiocytic sarcoma (HS is a neoplasm derived from histiocytes. Its diagnosis was not clear until its immunohistochemistry profile was correctly established. Not much is known about its genetic properties. We report a case of a 48-year-old male patient whose bone marrow was almost completely occupied by monomorphic medium size neoplastic cellularity. Its immunohistochemical profile was CD68+, CD4+, CD45+ with negativity of other dendritic cells, and other lineage markers. Cytogenetic study showed 4 related clones: one with trisomy 8 and extra material on the short arms of chromosome 4; a second line with tetrasomy of chromosome 8, add(4(p16; the third clone had the same alterations as the previous and deletion of chromosome 3 at q11; the fourth line had tetrasomy 8 and translocation t(3;5(q25;q35. To our knowledge this is the first HS case showing chromosome 8 trisomy and tetrasomy and the other described alterations.

  7. Imaging features of alveolar soft part sarcoma

    Institute of Scientific and Technical Information of China (English)

    Teng Jin; Ping Zhang Co-first author; Xiaoming Li

    2015-01-01

    Objective The aim of this study was to analyze the imaging features of alveolar soft part sarcoma (ASPS). Methods The imaging features of 11 cases with ASPS were retrospectively analyzed. Results ASPS mainly exhibited an isointense or slightly high signal intensity on T1-weighted imaging (T1WI), and a mixed high signal on T2-weighted imaging (T2WI). ASPS was partial, with rich tortuous flow voids, or “line-like” low signal septa. The essence of the mass was heterogeneous enhancement. The 1H-MRS showed a slight choline peak at 3.2 ppm. Conclusion The wel-circumscribed mass and blood voids, combined with “line-like” low signals play a significant role in diagnosis. The choline peak and the other signs may be auxiliary diagnoses.

  8. Myxoinflammatory fibroblastic sarcoma of the face.

    Science.gov (United States)

    Gómez Martín, Cristina; Ortega, María I; Aramburu, José A; Fernández-Cañamaque, José L

    2012-08-01

    Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade tumor of modified fibroblasts, with tendency to local recurrence. This unusual entity typically presents as a slow-growing painless mass in the distal extremities of middle-aged subjects. A 48-year-old woman presented to our clinic with a painless subcutaneous mass in the right temporal region. Excisional biopsy made the rare diagnosis of MIFS. Histologic examination showed the unique features that characterize this lesion: a myxoid component with a superimposed inflammatory infiltrate and the presence of distinctive, large, and bizarre Reed-Stemberg-like cells. A second wide tumor bed resection was performed, achieving clear margins. No adjuvant therapy was administered, and the patient is free of disease at 18 months postoperatively. To the best of our knowledge, this is the first reported case of MIFS presenting in the face. This adds another possibility for differential diagnoses of soft tissue tumors of the face.

  9. Epithelioid sarcoma with unusual radiological findings

    Energy Technology Data Exchange (ETDEWEB)

    Yamato, M.; Nishimura, G. [Department of Radiology, Dokkyo University School of Medicine, Tochigi (Japan); Yamaguchi, Takehiko [Department of Pathology, Dokkyo University School of Medicine, Tochigi (Japan); Tamai, Kazuya; Saotome, Koichi [Department of Orthopaedic Surgery, Dokkyo University School of Medicine, Tochigi (Japan)

    1997-10-01

    The case of a patient with epithelioid sarcoma in the right arm is reported. The diagnosis was delayed because of misinterpretation arising from complexity in the MR findings, including a honeycomb pattern in the subcutaneous fat simulating lymphedema, and an intramuscular diffuse high signal intensity on T2-weighted images without a discrete mass lesion. The histological findings revealed that the diffuse muscular abnormality mainly resulted from denervation of the muscles due to perineural invasion by the tumor, and subcutaneous edema from lymphedema secondary to lymphatic tumor spread concurrent with lymphatic fibrosis. Multiple foci of cortical erosions in the humerus, a rare manifestation of this tumor, were detected 6 months later. (orig.) 11 refs.

  10. Contemporary Management of Retroperitoneal Soft Tissue Sarcomas.

    Science.gov (United States)

    Olimpiadi, Yuliya; Song, Suisui; Hu, James S; Matcuk, George R; Chopra, Shefali; Eisenberg, Burton L; Sener, Stephen F; Tseng, William W

    2015-08-01

    Management of retroperitoneal soft tissue sarcomas (RP STS) can be very challenging. In contrast to the more common extremity STS, the two predominant histologic subtypes encountered in the retroperitoneum are well-differentiated/dedifferentiated liposarcoma and leiomyosarcoma. Surgery remains the mainstay of treatment for RP STS. Preoperative planning and anticipation of the need for resection of adjacent organs/structures are critical. The extent of surgery, including the role of compartmental resection, is still controversial. Radiation therapy may be an important adjunct to surgery to provide locoregional disease control; this is currently being evaluated in the preoperative setting in the EORTC STRASS trial. Systemic therapy, tailored to the specific histologic subtype, may also be of benefit for the management of RP STS. Further investigation of novel therapies (e.g., targeted therapies, immunotherapy) is needed. Overall, multi-institutional collaboration is important moving forward, to continue to better understand and optimize management of this disease.

  11. Kaposi’s Sarcoma Herpesvirus Genome Persistence

    Directory of Open Access Journals (Sweden)

    Franceline Juillard

    2016-08-01

    Full Text Available Kaposi’s sarcoma-associated herpesvirus (KSHV has an etiologic role in Kaposi’s sarcoma, primary effusion lymphoma and multicentric Castleman’s disease. These diseases are most common in immunocompromised individuals, especially those with AIDS. Similar to all herpesviruses, KSHV infection is lifelong. KSHV infection in tumor cells is primarily latent, with only a small subset of cells undergoing lytic infection. During latency, the KSHV genome persists as a multiple copy, extrachromosomal episome in the nucleus. In order to persist in proliferating tumor cells, the viral genome replicates once per cell cycle and then segregates to daughter cell nuclei. KSHV only expresses several genes during latent infection. Prominent among these genes, is the latency-associated nuclear antigen (LANA. LANA is responsible for KSHV genome persistence and also exerts transcriptional regulatory effects. LANA mediates KSHV DNA replication and in addition, is responsible for segregation of replicated genomes to daughter nuclei. LANA serves as a molecular tether, bridging the viral genome to mitotic chromosomes to ensure that KSHV DNA reaches progeny nuclei. N-terminal LANA attaches to mitotic chromosomes by binding histones H2A/H2B at the surface of the nucleosome. C-terminal LANA binds specific KSHV DNA sequence and also has a role in chromosome attachment. In addition to the essential roles of N- and C-terminal LANA in genome persistence, internal LANA sequence is also critical for efficient episome maintenance. LANA’s role as an essential mediator of virus persistence makes it an attractive target for inhibition in order to prevent or treat KSHV infection and disease.

  12. The effect of superovulation prior to mating on fetal growth in Iambs from Javanese thin-tail ewes

    Directory of Open Access Journals (Sweden)

    W Manalu

    1999-12-01

    Full Text Available Twenty-nine fetuses (11 fetuses from 9 non-superovulated ewes and 18 fetuses from 8 superovulated ewes were used to study the effect of superovulation of ewes prior to mating on fetal weight, fetal length, the length of the body and limbs, chest circumference, weights of the body, head, neck, limb, and viscera. Superovulated ewes, though with a higher litter size, had a greater fetal growth as was indicated by the greater fetal weight and length, the length and weight of the body and limb on day 49 of pregnancy. On day 105 of pregnancy, superovulated ewes with multiple fetuses (≥3 had similar fetal growth than nonsuperovulated ewes with single and twin fetuses. However, superovulated ewes with a single fetus had greater fetal growth as was shown by the greater fetal weight and length, the length of the body and limbs, chest circumference, and weight of the body, limb, and viscera when compared to those non-superovulated ewes with a single or twin fetuses. The results of the experiment suggested that superovulation of ewes prior to mating could be used to improve fetal prenatal growth during pregnancy

  13. Myofibroblastic sarcomas: a clinicopathological study of 20 cases

    Institute of Scientific and Technical Information of China (English)

    MENG Guo-zhao; ZHANG Hong-ying; BU Hong; ZHANG Xian-liang; PANG Zong-guo; KE Qi; LIU Xi; YANG Guo

    2007-01-01

    Background Myofibroblastic sarcoma was used to be a controversial neoplasm. This study investigated the clinicopathological features of 20 cases of myofibroblastic sarcoma arising in different locations.Methods The paraffin-embedded tissue samples from 20 cases of patients with myofibroblastic sarcoma were stained immunohistochemically, and 5 cases examined by electron microscopy. Student's t test was used to analyze the difference of Ki-67 labeling index between grade 1 and grade 2 myofibroblastic sarcomas.Results Histologically, the tumors were composed of slender spindle cells with eosinophilic cytoplasm, and fusiform,tapering, wavy, or plump ovoid; vesicular nuclei and a small central eosinophilic nucleoli. Immunohistochemically, the tumor cells expressed smooth muscle actin (18/20), muscle specific actin (16/20), fibronectin (20/20) and desmin (2/20).Ultrastructurally, the tumor cells revealed abundant rough endoplasmic reticulum and longitudinally arranged fine filaments with focal densities in the cytoplasm. A clinical follow-up of 19 patients showed that 2 cases experienced local recurrence and distant metastasis 6 months to 4 years after the initial operation. Nine cases recurred locally 17 to 46 months after the initial excision, and 9 cases were alive with no evidence of disease.Conclusions Myofibroblastic sarcomas, which exhibit diverse histological appearance, can easily be misdiagnosed as benign tumors. Myofibroblastic sarcomas are local destructive lesions with frequent recurrence, and may metastase distantly.

  14. Assessing the usefulness of prostaglandin E2 (Cervidil) for transcervical artificial insemination in ewes.

    Science.gov (United States)

    Bartlewski, Pawel M; Candappa, Ivanka B R

    2015-12-01

    The underlying theme of this study involved the evaluation of the dilatory effects of prostaglandin E2 on the ovine cervix and thus the assessment of its potential applicability to transcervical artificial insemination (TCAI) in ewes. A novel method of prostaglandin E2 administration (controlled slow-release vaginal inserts) was examined, and the practical implications of this approach including cervical penetrability and posttreatment pregnancy rates were evaluated. The Guelph method of TCAI was performed during the seasonal anestrus (n = 40) and the breeding season (n = 40) on multiparous Rideau Arcott × Polled Dorset ewes, with or without the pretreatment with Cervidil (for a duration of 12 hours or 24 hours before TCAI). Cervical penetration rates averaged 82.5% (66 of 80), and they varied neither (P > 0.05) between the two seasons nor between Cervidil-treated ewes and their respective controls. Cervidil priming significantly reduced the total time required for TCAI during the breeding season in comparison with controls (54 vs. 98 seconds), especially after the 24-hour exposure (38 vs. 108 seconds). The time taken to traverse the uterine cervix was negatively correlated (P < 0.05) with the breed (percentage of Rideau Arcott genotype) and lifetime lamb production in seasonally anestrous ewes. Four out of 36 (11%) successfully penetrated ewes in the breeding season (three ewes allocated to the 12-hour control group and one ewe that had received Cervidil for 12 hours) became pregnant and carried the lambs to term. Vaginal mucus impedance at TCAI was significantly and positively correlated with the total time required to complete the procedure in cyclic ewes, and the negative correlation between vaginal mucus impedance and total time values at the time of controlled intravaginal drug release device removal approached to significance in anestrous ewes. The present results indicate a moderate benefit of using Cervidil for inducing cervical dilation before

  15. [Soft tissue sarcoma in children and adolescents: experiences of the cooperative Soft Tissue Sarcoma Group Studies (CWS-81 - 96)].

    Science.gov (United States)

    Brecht, I B; Treuner, J

    2004-10-01

    The very heterogeneous group of paediatric soft tissue sarcomas account for approximately 7 % of all malignant childhood tumours. More than one half of all cases are rhabdomyosarcomas, some of the over 20 entities are very rare. The prognosis and biology of soft tissue sarcomas in children and adolescents vary greatly depending on histological subtype, the age of the patient, the primary site, the tumour size, tumour invasiveness and the extent of disease at diagnosis. Since 1981, 2918 children and adolescents with soft tissue sarcomas were treated prospectively according to the common treatment protocols of the Cooperative Soft Tissue Sarcoma Study Group (CWS-81 - 96). The known prognostic factors were used to develop a more and more detailed risk stratification. The multimodal treatment includes the use of surgery, chemotherapy and radiotherapy and should be planned by a multidisciplinary team. That way, an overall survival of nearly 70 % over all risk groups could be achieved.

  16. Hormonal treatments in metastatic endometrial stromal sarcomas: the 10-year experience of the sarcoma unit of Royal Marsden Hospital

    OpenAIRE

    2015-01-01

    Background Hormonal manipulation is sometimes recommended in the treatment of metastatic endometrial stromal sarcoma, but there are few data assessing the efficacy of endocrine therapies in this subtype of uterine sarcomas. Methods We performed a retrospective electronic medical record review of patients with metastatic ESS treated with a hormonal agent at Royal Marsden Hospital between 1999 and 2011. We assessed progression-free survival (PFS), objective response and toxicity profile among p...

  17. 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.

  18. 论英语复合词ewe lamb的合成原理%How is the compound word ewe lamb coined?

    Institute of Scientific and Technical Information of China (English)

    吴小晶

    2006-01-01

    本文从语义复合的角度分析了ewe lamb一词的合成原理,以及ewe和lamb当选构件的条件,提出了相关的语义原则(或规则).语义复制和语义渗透在ewelamb的合成中发挥了重要作用.语义复制扩大了元素的分配范围,提高了构件选择的语义标准.这就解释了为什么ewe能入选,而female、bitch、hen、girl等词则被淘汰.语义渗透原则维护了lamb的义位在其下位词ewe lamb中的状态,使得ewe 和lamb之间的矛盾彻底消解.这也解释了为什么ewe没有因为包涵[ADULT]而落选.在整个复合过程中没有发现任意性的环节,ewe lamb的逻辑性丝毫不亚于定中词组femalelamb.

  19. Mesenchymal Stromal Cell Dependent Regression of Pulmonary Metastasis from Ewing's

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    Andrea Anita Hayes-Jordan

    2014-05-01

    Full Text Available Introduction: Ewing’s sarcoma (ES is the second most common bone tumor in children. Survival has not improved over the last decade and once pulmonary metastatic disease is present, survival is dismal. Mesenchymal stromal cell (MSC therapy has shown potential benefit for Kaposi's sarcoma; however, the role of progenitor cell therapies for cancer remains controversial. MSC treatment of ES or pulmonary metastatic disease has not been demonstrated. We have developed an orthotopic xenograft model of ES in which animals develop spontaneous pulmonary metastases. Within this model, we demonstrate the use of MSCs to target ES lung metastasis. Materials and MethodsHuman ES cells were transfected with luciferase and injected into the rib of nude mice. Development of pulmonary metastases was confirmed by imaging. After flow cytometry based characterization, MSC’s were injected into the tail vein of nude mice with established local ES tumor or pulmonary metastasis. Mice were treated with intravenous MSCs weekly followed by bioluminescent imaging.ResultsThe intravenous injection of MSCs in an ES model decreases the volume of pulmonary metastatic lesions; however, no effect on primary chest wall tumor size is observed. Thus verifying the MSC preferential homing to the lung. MSCs are found to ‘home to’ the pulmonary parenchyma and remain engrafted up to 5 days after delivery. DiscussionMSC treatment of ES slows growth of pulmonary metastasis. MSC’s have more affinity for pulmonary metastasis and can effect a greater decrease in tumor growth in the lungs compared to the primary tumor site

  20. Effects of Using Melatonin Implants and Syncro-Part Pessaries + PMSG on Reproduction Performance in Transylvanian Merino Breed Ewes

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    Ioan Padeanu

    2013-10-01

    Full Text Available Investigations were carried out in a commercial farm from Sinnicolau Mare, western Romania, on a number of 70 yearling ewes of 17-18 months of age from the native Transylvanian Merino breed. Ewes included into study lambed for the first time as maiden-ewes at the age of 14-15 months, with their lambs being weaned at the age of two months. First group (V1, numbering 25 ewes received during out of season breeding (30.05.2011 a dose of 18 mg subcutaneous melatonin implants (Melovin®, while the second group (V2 consisting out of 25 ewes were treated with subcutaneous melatonin implants + 30 mg FGA (Syncro-Part pessaries + 500 IU PMSG. Control group was represented by 20 intact females. Ewes were put to ram in July 7th 2011. Researches shown that in ewes treated with melatonin, 30 mg FGA + 500 IU PMSG (V2 the conception rate was of 80%, with a prolificacy of 140%, with a weaning rate of 1 lamb/ewe. In V1 group, the weaning rate was of 0.84 lambs/ treated ewe, with significantly lower production costs. Ewes from the control group produced a weaning rate of 0.55 lambs/ewe, significantly less (p≤0.05 comparing to V1 and V2 experimental groups. It was concluded that in Transylvanian Merino breed, although the V2 group performed best (producing 28 lambs compared to V1 group (23 lambs, the difference of 5 lambs does not cover additional production costs, therefore the use of melatonin implants alone should be recommended.

  1. Genomic signatures predict poor outcome in undifferentiated pleomorphic sarcomas and leiomyosarcomas

    DEFF Research Database (Denmark)

    Silveira, Sara Martoreli; Villacis, Rolando Andre Rios; Marchi, Fabio Albuquerque;

    2013-01-01

    Undifferentiated high-grade pleomorphic sarcomas (UPSs) display aggressive clinical behavior and frequently develop local recurrence and distant metastasis. Because these sarcomas often share similar morphological patterns with other tumors, particularly leiomyosarcomas (LMSs), classification...

  2. 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

  3. Feline injection-site sarcoma / Sarcoma de aplicação felino

    Directory of Open Access Journals (Sweden)

    Julia Maria Matera

    2008-08-01

    Full Text Available The feline injection-site sarcoma (FIS is a challenge for the veterinarian and the affected cat’s owner. The injectable applications (vaccines, medications seems to be the reason for that neoplasia, more specifically, the inflammation caused by injury of given drugs or antigens to the health tissue. Generally the FIS presents a more aggressive behavior when compared to sarcoma not associated to application. The most effective treatment has not been established yet, but it is believed that a multimodality of therapies, surgery, radiotherapy, and chemotherapy would be the most indicated option. The knowledge of the illness in all of its aspects will supply to professionals colleges subsidies in relation to the best way to approach its diagnosis and treatment.O sarcoma de aplicação felino (SAF é atualmente um grande desafio para o médico veterinário e também para o proprietário do felino acometido. Aplicações injetáveis por via subcutânea ou intramuscular, como vacinas e medicações, aparecem como iniciadoras do processo de neogênese dessa neoplasia, mais precisamente a inflamação persistente, causada pela lesão ao tecido sadio decorrente do fármaco ou antígeno administrado. Geralmente o SAF apresenta comportamento mais agressivo quando comparado ao sarcoma não associado à aplicação. O tratamento mais eficaz ainda não está estabelecido, mas acredita-se que a multimodalidade de terapias, cirurgia, radioterapia e quimioterapia seja a opção mais indicada. O conhecimento da afecção em todos os seus aspectos irá fornecer aos colegas profissionais subsídios em relação a melhor maneira de abordá-la em termos de diagnóstico, tratamento e prevenção.

  4. Epidemiology and effects of gastrointestinal nematode infection on milk productions of dairy ewes

    Directory of Open Access Journals (Sweden)

    Suarez V.H.

    2009-06-01

    Full Text Available 66 Pampinta breed ewes were studied during milking to evaluate the infection and the effect of gastrointestinal nematode on milk production sheep system. Naturally infected ewes on pasture were randomly allocated to two groups: TG, suppressively treated group every four weeks with levamisole and UG, untreated group. Faecal nematode egg counts and larval differentiation were conducted monthly. Successive groups of worm free tracer lambs were grazed with ewes and then slaughtered for worm counts. Test-day milk yield of individual ewes was recorded and ewe machine-milking period length (MPL were estimated. Faecal egg counts and tracer nematode numbers increased towards midsummer and declined sharply toward the end of the study. TG (188.0 ± 60 liters produced more (p < 0.066 milk liters than UG (171.9 ± 52.2 and TG had significantly more extended (p < 0.041 MPL than those of UG. The present study showed that dairy sheep were negatively affected by worms, even when exposed to short periods of high acute nematode (mainly Haemonchus contortus infection.

  5. Reproductive characteristics of Awassi ewes under Cornell alternate month accelerated lambing system

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    Sabri Gül

    2010-04-01

    Full Text Available We investigated the reproductive responses of Awassi ewes under Cornell alternate month accelerated lambing (CAMAL system. Ewes were randomly allocated to two experimental groups. The first group (control group consists of 20 ewes exposed to rams in September under conventional management system while the second group (CAMAL were divided into four sub-flocks contain 20 head of ewes each one were exposed to rams to obtain three lambing in two years with different breeding and lambing months. In CAMAL ewes, oestrus was synchronized using intra-vaginal sponges with progesterone and PMSG administration. Results revealed that within CAMAL group, the percentage of animals in heat, onset of oestrus, litter size, birth weight and weaning weight were affected by mating months. September and November were the most appropriate months for oestrus ratio (97.5 % and litter size (1.18 and 0.98, respectively. Lambs of control group were heavier at birth and weaning than those of CAMAL group. On the other hand lamb yield was not affected statistically by the CAMAL administration.

  6. The Relationship between Selenium and T3 in Selenium Supplemented and Nonsupplemented Ewes and Their Lambs

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    Abd Elghany Hefnawy

    2014-01-01

    Full Text Available Twenty pregnant ewes were selected and classified into two groups. The first group received subcutaneous selenium supplementation (0.1 mg of sodium selenite/kg BW at the 8th and 5th weeks before birth and 1st week after birth while the other was control group without selenium injection. Maternal plasma and serum samples were collected weekly from the 8th week before birth until the 8th week after birth and milk samples were taken from ewes weekly, while plasma and serum samples were collected at 48 hours, 1st, 2nd, 3rd, 5th, and 8th weeks after birth from the newborn lambs. Results demonstrated significant positive relationship between maternal plasma selenium and serum T3 in supplemented and control ewes (r=0.69 to 0.72, P<0.05. There was significant (P<0.001 increase in T3 in supplemented ewes and their lambs until the 8th week after birth. There was positive relationship between milk, selenium concentration, and serum T3 in the newborn lambs of the supplemented group (r=0.84, P<0.01, while the relationship was negative in the control one (r=-0.89, P<0.01. Muscular and thyroid pathological changes were independent of selenium supplementation. Selenium supplementation was important for maintaining T3 in ewes and newborn lambs until the 8th week after birth.

  7. 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.

  8. Ewe maternal behavior score to estimate lamb survival and performance during lactation

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    Andreia Barros de Moraes

    2016-08-01

    Full Text Available Mortality of perinatal lambs and low weight at weaning cause huge liabilities to farmers. Current study describes maternal-filial behavior and evaluates the use of maternal behavior score (MBS to estimate the behavior of ewes and lambs soon after birth, and correlate it with lamb mortality and performance during lactation. Thirty-seven Corriedale ewes were used in a completely randomized design. MBS was assessed up to 24 hours after birth, taking into consideration the distance of the ewe from the lamb at the approach of a person. Maternal behavior, placental weight, weight gain of the lambs until weaning and their survival rate were also evaluated until two hours after lambing. More than 90% of the ewes had adequate maternal behavior, with parental care, even though ewes were very sensitive to the presence of people. There was no significant correlation between MBS and maternal behavior, lamb mortality rate and live weight gain. Under these conditions, MBS was not a useful tool to estimate maternal behavior and performance of lambs.

  9. Induction of ovulation in anestrus ewes using a dopamine receptor antagonist.

    Science.gov (United States)

    Saxena, Vijay Kumar; De, Kalyan; Kumar, Davendra; Naqvi, Syed Mohammad Khursheed; Krishnaswamy, Narayanan; Tiwari, Ashok Kumar

    2015-11-01

    Estradiol decreases the pulse frequency of LH during the nonbreeding season through dopaminergic neurons that results in anestrus in the ewe. Long-term administration of sulpiride, a dopamine antagonist, induced ovulation in seasonally anestrus mares. Accordingly, we tested whether sulpiride would induce ovulatory estrus in seasonally anestrus Malpura ewes. A total of 12 Malpura ewes were divided into sulpiride (at 0.6 mg/kg b.i.d.) or control groups. Anestrus was defined on the basis of the absence of heat signs for 2 months through twice-a-day heat detection during the nonbreeding season (October-November) and progesterone level of less than 1 ng/mL. Rates of estrus induction, ovulation, multiple ovulations, and lambing in the sulpiride-treated ewes were 83.3%, 100%, 16.6%, and 66.7%, respectively. The mean interval from treatment to estrus was 5.25 ± 1.49 days. Progesterone levels were elevated after ovulation significantly on Days 5 and 7 after estrus as compared to Day 0 after sulpiride treatment (P sulpiride treatment and estrus induction rate as well as ovulation rate (P < 0.05). It is concluded that the result provides proof of concept that the dopamine antagonist can induce ovulation in seasonally anestrus ewes.

  10. Vaginal mucus from ewes treated with progestogen sponges affects quality of ram spermatozoa.

    Science.gov (United States)

    Manes, Jorgelina; Ríos, Glenda; Fiorentino, María Andrea; Ungerfeld, Rodolfo

    2016-03-15

    The use of intravaginal sponges (IS) to synchronize estrous onset in ewes provokes vaginitis, an increase in the vaginal bacterial load, and growth of bacterial species that are not present during spontaneous estrous behavior. The objective of the study was to compare the functional sperm parameters after incubating it with mucus collected from the vagina of ewes during spontaneous estrus or estrous synchronized with IS. Pooled spermatozoa were co-incubated with: (1) vaginal mucus collected from ewes in spontaneous estrus; (2) vaginal mucus collected from ewes in estrus pretreated with progestogen-impregnated IS; (3) synthetic mucus; and (4) medium without mucus as a control group. Sperm samples were evaluated after incubating it for 30 and 90 minutes. The number of colony-forming units (CFUs/mL), pH, and osmolality were greater in the mucus collected from ewes treated with IS than from those untreated (P = 0.046; P sheep in spontaneous estrus for 30 and 90 minutes (P synchronization treatments. This may partially explain the decrease in conception rate obtained with treatments with IS.

  11. 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.

  12. 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.

  13. Contribution of macrophages to plasmin activity in ewe bulk milk

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    M. Albenzio

    2010-04-01

    Full Text Available A total of 225 bulk sheep milk samples were collected throughout lactation to assess the contribution of macrophages to the regulation of the plasmin/plasminogen system. Samples were analyzed for composition, milk renneting parameters, and for activities of plasmin (PL, plasminogen (PG and plasminogen activators (PA. Isolation of macrophages from milk was performed using a magnetic positive separation; separated cells were lysed and activity of urokinase-PA was determined. PL activity in milk decreased during lactation (P < 0.001. The reduction in plasmin activity recorded in the mid and late lactation milk matched with the increase in PG/PL ratio (P < 0.001. The activity of PA increased throughout lactation (P < 0.001, the highest value being recorded in the late lactation milk.The amount of isolated and concentrated macrophages was higher in early and mid lactation milk than in late lactation milk (P < 0.01. Stage of lactation did not influence the activity of u-PA detected in isolated macrophages. The activity of u-PA associated with macrophages was lower than total PA activity detected in milk. Our results lend support to the hypothesis that in ewe bulk milk from healthy flocks macrophages only slightly contributed to the activation of plasmin/plasminogen system.

  14. Soft tissue sarcoma with metastasis to the stomach: A case report

    Institute of Scientific and Technical Information of China (English)

    Lemuel; Leon; Dent; Cesar; Yamil; Cardona; Michael; Clause; Buchholz; Roosevelt; Peebles; Julie; Denise; Scott; Derrick; Jerome; Beech; Billy; Ray; Ballard

    2010-01-01

    Soft tissue sarcomas are unusual malignancies comprising 1% of cancer diagnoses in the United States. Undifferentiated pleomorphic sarcoma accounts for approximately 5% of sarcomas occurring in adults. The most common site of metastasis is the lung, with other sites being bone, the brain, and the liver. Metastasis to the gastrointestinal tract has rarely been documented. We present an unusual case of high-grade pleomorphic sarcoma with metastasis to the stomach, complicated by upper gastrointestinal bleedin...

  15. Synovial Sarcoma of the Larynx: Report of a Case and Review of Literature

    Science.gov (United States)

    Baby, Anto; Somanathan, Thara; Konoth, Sreedevi

    2017-01-01

    Sarcomas account for less than 1% of malignant neoplasms arising in the head and neck in adults. Laryngeal synovial sarcoma is an extremely rare form of laryngeal malignancy with less than 20 cases reported in the literature. We report the case of a 48-year-old man with synovial sarcoma of the larynx. He underwent excision of the tumor followed by radiation. He is alive in remission at 36 months. The literature on synovial sarcoma of the larynx is reviewed. PMID:28280643

  16. Pulmonary involvement in Kaposi sarcoma: correlation between imaging and pathology.

    Science.gov (United States)

    Gasparetto, Taisa Davaus; Marchiori, Edson; Lourenço, Sílvia; Zanetti, Gláucia; Vianna, Alberto Domingues; Santos, Alair A S M D; Nobre, Luiz Felipe

    2009-07-14

    Kaposi sarcoma is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four variants of this disease, each presenting a different clinical manifestation: classic or sporadic, African or endemic, organ transplant-related or iatrogenic, and AIDS-related or epidemic. Kaposi sarcoma is the most common tumor among patients with HIV infection, occurring predominantly in homosexual or bisexual men. The pulmonary involvement in Kaposi sarcoma occurs commonly in critically immunosupressed patients who commonly have had preceding mucocutaneous or digestive involvement.The etiology of Kaposi sarcoma is not precisely established; genetic, hormonal, and immune factors, as well as infectious agents, have all been implicated. There is evidence from epidemiologic, serologic, and molecular studies that Kaposi sarcoma is associated with human herpes virus type 8 infection. The disease starts as a reactive polyclonal angioproliferative response towards this virus, in which polyclonal cells change to form oligoclonal cell populations that expand and undergo malignant transformation.The diagnosis of pulmonary involvement in Kaposi sarcoma usually can be made by a combination of clinical, radiographic, and laboratory findings, together with the results of bronchoscopy and transbronchial biopsy. Chest high-resolution computed tomography scans commonly reveal peribronchovascular and interlobular septal thickening, bilateral and symmetric ill-defined nodules in a peribronchovascular distribution, fissural nodularity, mediastinal adenopathies, and pleural effusions. Correlation between the high-resolution computed tomography findings and the pathology revealed by histopathological analysis demonstrate that the areas of central peribronchovascular infiltration represent tumor growth involving the bronchovascular bundles, with nodules corresponding to proliferations of neoplastic cells into the pulmonary parenchyma. The interlobular septal thickening may represent

  17. Primary synovial sarcoma of the abdominal wall: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Alsaif H Saif

    2008-01-01

    Full Text Available Synovial sarcoma is a malignant mesenchymal neoplasm which commonly occurs in the extremities of adults, in close association with joint capsules, tendon sheaths, bursae and fascial structures. Only a few cases of synovial sarcoma occurring in the abdominal wall have been reported. A case of a primary synovial sarcoma arising from the anterior abdominal wall fascial aponeurosis is presented.

  18. Uterine sarcomas-Recent progress and future challenges

    Energy Technology Data Exchange (ETDEWEB)

    Seddon, Beatrice M., E-mail: beatrice.seddon@uclh.nhs.uk [London Sarcoma Service, Department of Oncology, University College Hospital, 1st Floor Central, 250 Euston Road, London, NW1 2PG (United Kingdom); Davda, Reena [London Sarcoma Service, Department of Oncology, University College Hospital, 1st Floor Central, 250 Euston Road, London, NW1 2PG (United Kingdom)

    2011-04-15

    Uterine sarcomas are a group of rare tumours that provide considerable challenges in their treatment. Radiological diagnosis prior to hysterectomy is difficult, with the diagnosis frequently made post-operatively. Current staging systems have been unsatisfactory, although a new FIGO staging system specifically for uterine sarcomas has now been introduced, and may allow better grouping of patients according to expected prognosis. While the mainstay of treatment of early disease is a total abdominal hysterectomy, it is less clear whether routine oophorectomy or lymphadenectomy is necessary. Adjuvant pelvic radiotherapy may improve local tumour control in high risk patients, but is not associated with an overall survival benefit. Similarly there is no good evidence for the routine use of adjuvant chemotherapy. For advanced leiomyosarcoma, newer chemotherapy agents including gemcitabine and docetaxel, and trabectedin, offer some promise, while hormonal therapies appear to be more useful in endometrial stromal sarcoma. Novel targeted agents are now being introduced for sarcomas, and uterine sarcomas, and show some indications of activity. Non-pharmacological treatments, including surgical metastatectomy, radiofrequency ablation, and CyberKnife radiotherapy, are important additions to systemic therapy for advanced metastatic disease.

  19. Epithelioid sarcoma with muscle metastasis detected by positron emission tomography

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    Oya Masafumi

    2008-08-01

    Full Text Available Abstract Background Epithelioid sarcoma is an uncommon high-grade sarcoma, mostly involving the extremities. Case presentation A 33-year-old man was referred to our institute with a diagnosis of Volkmann's contracture with the symptom of flexion contracture of the fingers associated with swelling in his left forearm. Magnetic resonance imaging (MRI showed abnormal signal intensity, comprising iso-signal intensity on T1- and high-signal intensity on T2-weighted images surrounding the flexor tendons in the forearm. Diagnosis of epithelioid sarcoma was made by open biopsy, and amputation at the upper arm was then undertaken. [18F]-2-fluoro-2-deoxy-D-glucose-positron emission tomography (FDG-PET detected multiple lesions with an increased uptake in the right neck, the bilateral upper arms and the right thigh, as well as in the left axillary lymph nodes, with maximum standardized uptake value (SUVmax ranging from 2.0 to 5.5 g/ml. Magnetic resonance imaging confirmed that there was a lesion within the right thigh muscle which was suggestive of metastasis, even though the lesion was occult clinically. Conclusion Increased uptake on FDG-PET might be representative of epithelioid sarcoma, and for this reason FDG-PET may be useful for detecting metastasis. Muscle metastasis is not well documented in epithelioid sarcoma. Accordingly, the frequency of muscle metastasis, including occult metastasis, needs to be further analyzed.

  20. Cystic synovial sarcomas: imaging features with clinical and histopathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Nakanishi, Hirofumi; Araki, Nobuhito [Department of Orthopedic Surgery, Osaka Medical Center for Cancer and Cardiovascular Diseases, 1-3-3, Nakamichi, Higashinari-Ku, 537-8511, Osaka (Japan); Sawai, Yuka [Department of Radiology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Kudawara, Ikuo [Department of Orthopedic Surgery, Osaka National Hospital, Osaka (Japan); Mano, Masayuki; Ishiguro, Shingo [Department of Pathology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Ueda, Takafumi; Yoshikawa, Hideki [Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Suita, Osaka (Japan)

    2003-12-01

    To characterize the radiological and clinicopathologic features of cystic synovial sarcoma. Seven patients with primary cystic synovial sarcoma were evaluated. Computed tomography (CT) and magnetic resonance (MR) imaging were undertaken at the first presentation. The diagnosis of synovial sarcoma was made on the basis of histological examinations followed by molecular analysis. Radiological and clinicopathologic findings were reviewed. CT showed well-defined soft tissue mass without cortical bone erosion and invasion. Calcification was seen at the periphery of the mass in three cases. T2-weighted MR images showed multilocular inhomogeneous intensity mass in all cases, five of which showed fluid-fluid levels. On gross appearance, old and/or fresh hematomas were detected in six cases. In the one remaining case, microscopic hemorrhage in the cystic lumen was proven. Four cases had poorly differentiated areas. In five cases prominent hemangiopericytomatous vasculature was observed. Histologic grade was intermediate in one tumor and high in six. One case had a history of misdiagnosis for tarsal tunnel syndrome, one for lymphadenopathy, two for sciatica and two for hematoma. All cystic synovial sarcomas demonstrated multilocularity with well-circumscribed walls and internal septae. Synovial sarcoma should be taken into consideration in patients with deeply situated multicystic mass with triple signal intensity on T2-weighted MR imaging. (orig.)

  1. Sarcomas arising after radiotherapy for peptic ulcer disease

    Energy Technology Data Exchange (ETDEWEB)

    Lieber, M.R.; Winans, C.S.; Griem, M.L.; Moossa, R.; Elner, V.M.; Franklin, W.A.

    1985-06-01

    Therapeutic gastric irradiation has been used to reduce peptic juice secretion in patients with peptic ulcer disease. Between 1937 and 1968 a total of 2049 patients received such therapy at the University of Chicago. Three of these patients are known to have developed sarcomas in the field of radiation. Two gastric leiomyosarcomas of the stomach were diagnosed 26 and 14 years after treatment and a malignant fibrous histiocytoma of the anterior chest wall was removed six years after gastric irradiation. Of 743 peptic ulcer patients treated without irradiation and constituted as a control group for the study of therapeutic gastric radiation, none is known to have developed sarcoma. As the incidence of sarcoma in these patient groups is known only from the tumor registry of the University of Chicago, other cases of sarcoma may exist in the groups. While an increased incidence of sarcoma has not been proven to occur in patients who received therapeutic gastric irradiation for peptic ulcer disease, the possibility of such a risk should be borne in mind by physicians caring for such patients.

  2. RADIOGRAPHIC IMAGING OF BIPHASIC SYNOVIAL SARCOMA ON LEFT ELBOW

    Directory of Open Access Journals (Sweden)

    Sitanggang Firman P

    2013-04-01

    Full Text Available Synovial sarcoma is the fourth most common type of sarcoma. It represents between 5% -10% of all soft tissue sarcomas and most prevalent in aged between 15 - 40 years. Synovial sarcoma is the most commonly misdiagnosed soft tissue malignancy, initially as an inflammation process,often because it may be slow-growing, have a benign appearance on imaging studies, may vary in size, and may have pain similar to that associated with trauma.    A rare case is presented of 17 years old women with a synovial sarcoma biphasic. The primary tumor originated in the left elbow since 2004. Since then, the patient has had repeated passive or active left elbow pain and tenderness. No history of trauma. She has not developed metastases of the lung. The patient refused to have surgery and others medical procedures that already planned and explained to her. The conclusion of this case report point that radiology is important to diagnosis and planning for further management

  3. Imaging of the most frequent superficial soft-tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Morel, Melanie; Taieb, Sophie; Ceugnart, Luc [Centre Oscar Lambret, Department of Radiology, Lille (France); Penel, Nicolas [Centre Oscar Lambret, Department of Oncology, Lille (France); Mortier, Laurent [Centre Hospitalier Universitaire de Lille, Department of Dermatology, Hopital Claude Huriez, Lille (France); Vanseymortier, Luc [Centre Oscar Lambret, Department of Surgery, Lille (France); Robin, Y.M. [Centre Oscar Lambret, Departement of Pathology, Lille (France); Gosset, Pierre [Groupement Hospitalier de l' Institut Catholique-Faculte Libre de Medecine de Lille, Department of Pathology, Hopital Saint-Philibert, Lomme (France); Cotten, Anne [Centre Hospitalier Universitaire de Lille, Department of Musculoskeletal Radiology, Centre Hopital Roger Salengro, Lille (France)

    2011-03-15

    Superficial soft-tissue sarcomas are malignant mesenchymal tumors located within the cutaneous and/or subcutaneous layers. Most superficial soft-tissue sarcomas are low-grade tumors; yet, the risk of local recurrence is high, and initial wide surgery is the main prognostic factor. Some of these superficial sarcomas may grow, following an infiltrative pattern, and their real extent may be underestimated clinically. Imaging techniques are useful to determine precisely the real margins of the tumor, especially in cases of clinically doubtful or recurrent or large superficial lesions. Imaging tools enable one to determine the relationship with the superficial fascia separating the subcutaneous layer from the underlying muscle. In our institution ultrasonographic examination is followed by magnetic resonance (MR) imaging when the size of the lesion exceeds 3-5 cm. Imaging assessment is performed prior to biopsy, enabling optimal surgical management. Imaging features of the main superficial sarcomas are detailed in the following article, according to their major locations: those arising in the epidermis and/or dermis, which are most often diagnosed by dermatologists, and the subcutaneous sarcomas. (orig.)

  4. Immediate versus Delayed Sarcoma Reconstruction: Impact on Outcomes

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    Kyle J. Sanniec

    2016-01-01

    Full Text Available Background. Sarcoma is a rare malignancy, and more recent management algorithms emphasize a multidisciplinary approach and limb salvage, which has resulted in an increase in overall survival and limb preservation. However, limb salvage has resulted in a higher rate of wound complications. Objective. To compare the complications between immediate and delayed (>three weeks reconstruction in the multidisciplinary limb salvage sarcoma patient population. Methods. A ten-year retrospective review of patients who underwent sarcoma resection was performed. The outcome of interest was wound complication in the postoperative period based on timing of reconstruction. We defined infection as any infection requiring intravenous antibiotics, partial flap failure as any flap requiring a debridement or revision, hematoma/seroma as any hematoma/seroma requiring drainage, and wound dehiscence as a wound that was not completely intact by three weeks postoperatively. Results. 70 (17 delayed, 53 immediate patients who underwent sarcoma resection and reconstruction met the inclusion criteria. Delayed reconstruction significantly increased the incidence of postoperative wound infection and wound dehiscence. There was no difference in partial or total flap loss, hematoma, or seroma between the two groups. Discussion and Conclusion. Immediate reconstruction results in decreased wound complications may reduce the morbidity associated with multidisciplinary treatment in the limb salvage sarcoma patient.

  5. Spinal intradural extraosseous Ewing’s sarcoma

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    Daniel Lachance

    2011-03-01

    Full Text Available Extraosseous Ewing’s sarcoma (EES involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuroectodermal tumors (PNET by identification of the chromosomal translocation (11;22(q24;q12. We report EES arising from the spinal intradural extramedullary space, based on imaging, histopathological, and molecular data in two men, ages 50 and 60 years old and a review of the literature using PubMed (1970-2009. Reverse transcriptase polymerase chain reaction (RT-PCR identified the fusion product FL1-EWS. Multimodal therapy, including radiation and alternating chemotherapy including vincristine, cyclophosphamide, doxorubicin and ifosfamide and etoposide led to local tumor control and an initial, favorable therapeutic response. No systemic involvement was seen from the time of diagnosis to the time of last follow-up (26 months or death (4 years. This report confirms that EES is not confined to the earliest decades of life, and like its rare occurrence as an extra-axial meningeal based mass intracranially, can occasionally present as an intradural mass in the spinal canal without evidence of systemic tumor. Gross total resection followed by multimodal therapy may provide for extended progression free and overall survival.

  6. Myeloid Sarcoma: The Clinician's Point of View

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    M. Malagola

    2011-01-01

    Full Text Available Myeloid Sarcoma may occur in patients with an acute or chronic myeloproliferative disorder as well as de novo, with no apparent sign or symptom of concomitant haematological disease. The patients are preferentially young male and the site of disease localization may vary from central nervous system to pleura and thorax, with a common involvement of the reticuloendothelial system. The disease often shows chromosomal rearrangements, involving chromosomes 7, 8 and 3 and sometimes a complex karyotype (more than 3 abnormalities is detected at diagnosis. The prognosis of this disease is dismal and only high-dose chemotherapy with autologous or allogeneic stem cells transplantation (auto or allo-SCT may be potentially curative. In the absence of definitive elements that can define the prognosis of extra-medullary localization of “standard risk” AML, Clinicians should pursue the collection of data from different Centres and design of homogeneous treatment strategies, that could integrate standard chemotherapy with specific approaches, such as radiotherapy, transplant procedures or, in selected cases (such as those displaying molecular abnormalities involving protein tyrosine-kinases, molecularly targeted therapies.

  7. Sarcoma de Kaposi en paciente con SIDA

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    Jesús Ramón León Polanco

    2015-01-01

    Full Text Available Se presenta el caso de un paciente masculino de 33 años de edad, con antecedentes de VIH-SIDA desde hace 10 años, que se mantiene en tratamiento con antirretrovirales. Durante todo este tiempo ha presentado varios episodios de infecciones respiratorias, incluyendo tuberculosis pulmonar 5 años atrás. Acude a consulta refiriendo edemas en miembros inferiores acompañado de lesiones en piel de color violáceo de un año de evolución, previamente interpretado como linfangitis rebelde al tratamiento y que se extendió a la cara interna de los muslos y a los miembros inferiores. Con pérdida de peso, no prurito en las lesiones, fiebre, lesiones en la mucosa oral. Se determinó hemoglobina 89 g/L, leucocitos 4,5 x 109 /L, se estudiaron las funciones hepática y renales resultando normales. Radiografías de tórax y ultrasonido abdominal normales. Se realizó estudio anatomopatológico de piel que informó Sarcoma de Kaposi. Se impuso tratamiento con quimioterapia

  8. [Retroperitoneal sarcoma: report of 6 cases].

    Science.gov (United States)

    Joual, A; Faik, H; Rabii, R; Hafiani, M; Bennani, S; el Mrini, M; Benjelloun, S

    2000-06-01

    Retroperitoneal soft tissue sarcomas (RPS) are uncommon tumors. The diagnosis is frequently made later in the evolution of the disease due to the absence of specific symptomatology. Surgery with total resection of the tumor is the treatment of choice, but is only possible in 38 to 75% of cases. Six cases of RPS have been retrospectively reviewed; the mean time to diagnosis was 5 months; diagnosis was established via CT scan, which determined the retroperitoneal tumor location and its relationship to the neighboring organs. The most common symptoms were the presence of an abdominal mass and accompanying abdominal pain; signs of urinary and vascular compression were not found until later. Surgery with total resection was performed in 4 cases, and with partial resection in 2 cases. The histological findings were as follows: 3 liposarcomas, 2 rhabdomyosarcomas, and 1 fibrosarcoma. Tumor recurrence developed in 2 cases (liposarcomas), necessitating further surgery and complete resection. Two patients were lost to follow-up. RPS are characterized by locoregional relapse and metastases. Disease outcome depends on the histological type, tumor grade, and on the possibility of carrying out complete resection.

  9. EFFECTS OF FEEDING AND MANAGEMENT SYSTEMS ON BODY WEIGHT AND REPRODUCTIVE PERFORMANCE OF BALOCHI EWES

    Directory of Open Access Journals (Sweden)

    M. MUNIR, A. W. JASRA1 AND M. A. MIRZA

    2007-07-01

    Full Text Available This study was conducted to assess the performance of Balochi ewes under three management systems i.e. T1-Extensive (grazing only, T2-Semi-extensive (grazing plus daily feed supplementation for nine months @ 0.625 kg/ewe/day and T3-Intensive (experimental ration only @ 1.5625 kg/ewe/day. Animals under T1 lost as high as 7.9 kg body weight during winter. T2 flock maintained their body weights and T3 achieved significantly higher body weight (i.e. 15.1 kg. Highest conception and lambing percentages (93.8% were attained under T3 group, followed by T2 (87.5% and T1 (75%. These results strongly recommend appropriate feed supplementation to small ruminants on Balochistan ranges for sustainable pastoral livelihood.

  10. 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

    , 47 and 44 g kg-1 h-1 and DM intake was 2.5, 2.1 and 1.5 kg d-1 for ECS, MCS and LCS silage, respectively. Faeces samples were collected during four days, washed in nylon bags, freeze dried and sieved into six sieving fractions; bottom bowl (B), 0.106 (C), 0.212 (D), 0.5 (S), 1.0 (M) and 2.36 (O) mm......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 particle size in faeces from ewes fed grass silages....

  11. The influence of season of lambing and lactation on reproductive activity and plasma LH concentrations in Merino ewes.

    Science.gov (United States)

    Restall, B J; Starr, B G

    1977-03-01

    Groups of Merino ewes which were lactating for 40 days (Group I) or had had their lambs removed at birth (Group II) after lambing in the winter (June) or spring (November) were fed on a high plane of nutrition. Ovarian inspections were carried out at 15 and 30 days after lambing and plasma LH levels were measured at 6-h intervals for 20-30 days. First ovulation was earlier in ewes lambing in the winter (16-6 days, range 11-26) than in the spring (24-7 days, range 15-30) but there was no difference in the number of ewes ovulating. LH levels were higher in winter-lambing ewes (2-79 +/- 3-4 ng/ml) than in those lambing in the spring (1-78 +/- 0-23 ng/ml). LH peaks were usually associated with an ovulation in spring lambing ewes but were not consistently so in the others. More ewes ovulated in Group II (72%) than in Group I (40%) but the mean time of first ovulation was similar. In the winter-lambing ewes the mean daily LH concentration was 2-40 +/- 0-32 ng/ml in Group I and 3-18 +/- 0-31 ng/ml in Group II but there were no differences between the spring-lambing ewes, (I, 1-75 +/- 0-20 ng/ml; II, 1-80 +/- 0-26 ng/ml). There were more elevations in LH levels in Group II ewes (64%) than in Group I ewes (43-8%). After lambing the LH levels increased slowly, indicating a gradual recovery of pituitary function.

  12. Effect of available space and previous contact in the social integration of Saint Croix and Suffolk ewes.

    Science.gov (United States)

    Orihuela, A; Averós, X; Solano, J; Clemente, N; Estevez, I

    2016-03-01

    Reproduction in tropical sheep is not affected by season, whereas the reproductive cycle of temperate-climate breeds such as Suffolk depends on the photoperiod. Close contact with tropical ewes during the anestrous period might induce Suffolk ewes to cycle, making the use of artificial light or hormonal treatments unnecessary. However, the integration of both breeds within the social group would be necessary to trigger this effect, and so the aim of the experiment was to determine the speed of integration of 2 groups of Saint Croix and Suffolk ewes into a single flock, according to space allowance and previous experience. For this, 6 groups of 10 ewes (half from each breed) from both breeds, housed at 2 or 4 m/ewe (3 groups/treatment) and with or without previous contact with the other breed, were monitored for 3 d. Each observation day, the behavior, movement, and use of space of ewes were collected during 10 min at 1-h intervals between 0900 and 1400 h. Generalized linear mixed models were used to test the effects of breed, space allowance, and previous experience on behavior, movement, and use of space. Net distances, interbreed farthest neighbor distance, mean interbreed distance, and walking frequencies were greater at 4 m/ewe ( movement activity ( < 0.05). Experienced ewes walked longer total distances during Day 1 and 2 ( < 0.05). Lying down frequency was greater for Day 3 than Day 1 ( < 0.05), and Suffolk ewes kept longer interindividual distances during Day 1 ( < 0.05). After 3 d of cohabitation, Suffolk and Saint Croix ewes did not fully integrate into a cohesive flock, with each breed displaying specific behavioral patterns. Decreasing space allowance and previous experience resulted in limited benefits for the successful group cohesion. Longer cohabitation periods might result in complete integration, although practical implementation might be difficult.

  13. EFFECT OF SHEARING DURING PREGNANCY ON PRODUCTIVE PERFORMANCE IN THE POST-PARTUM PERIOD OF EWES ON EXTENSIVE HUSBANDRY

    Directory of Open Access Journals (Sweden)

    Viviane Marques Guyoti

    2015-04-01

    Full Text Available The use of shearing during pregnancy has been described as a tool for improving productivity in sheep and for minimizing perinatal mortality in lambs through the increase of fetal development. This study assessed the effect of shearing around 74 days of gestation on the productive performance of ewes and lambs during the first month of life. Forty Corriedale ewes were inseminated in autumn in Southern Brazil. All ewes were kept together at the same pasture under extensive husbandry conditions. The ewes were randomly separated into two treatment groups: twenty animals were completely sheared at 74 ± 6 days of pregnancy, and twenty were kept without sheared during pregnancy, composing the control group. Ewes and their lambs were evaluated at three different times during the experiment: at birth, between 15 and 21 days post-partum and between 22 and 45 days post-partum. Ewes had their body condition score, body weight, placental weight, milk production and serum concentrations of beta-hydroxybutyrate measured, while lambs had hematocrit, hemoglobin, and plasma lactate and glucose, as well as body weight at birth and until wean determined. Values of hematocrit and hemoglobin were lower and body weight at birth and at wean was higher in the group of lambs born from sheared ewes. Placenta weight was higher in sheared ewes. Body condition score and beta-hydroxybutyrate showed no differences between groups. Milk production of sheared ewes (1.26 L/day was higher than in control group (0.93 L/day. Shearing ewes at 74 days of pregnancy was efficient for the better development of lambs at post-birth, reducing perinatal mortality rates.

  14. Effect of weaning age on hair sheep lamb and ewe production traits in an accelerated lambing system in the tropics.

    Science.gov (United States)

    Godfrey, R W; Weis, A J

    2016-03-01

    This study was designed to evaluate the impact of weaning age on lamb and ewe productivity in an accelerated lambing system. St. Croix White (STX) and Dorper × St. Croix White (DRPX) lambs were assigned at birth based on breed, gender, and litter size to be weaned at 63 (Early-1; 106 lambs and 68 ewes) or 90 d of age (Late-1; 99 lambs and 60 ewes) in Exp.1 or at 63 (Early-2; 77 lambs and 57 ewes) or 120 d of age (Late-2; 75 lambs and 56 ewes) in Exp. 2. After weaning, lambs were weighed weekly and fed a concentrate ration (2% BW·lamb·d) while grazing guinea grass pastures. In Exp. 1, weaning weight was greater ( 0.06) between Early-2 and Late-2 lambs (5.1 ± 0.2 vs. 5.6 ± 0.3 kg, respectively). In Exp. 1 and 2, ewe BW at breeding and lambing and weaning and lambing rate were not different among weaning ages of lambs ( > 0.17). The Early-1 ewes exhibited estrus earlier than Late-1 ewes (10.9 ± 0.9 vs. 13.9 ± 1.0 d, respectively) but there was no difference ( > 0.63) between Early-2 and Late-2 ewes. Weaning hair lambs at 90 or 120 d of age can be done in an accelerated lambing system with no detrimental effect on lamb or ewe productivity. Late weaning resulted in a decreased number of days that lambs received high-cost, imported feed without a reduction in growth, resulting in savings of US$6 to $15 per lamb.

  15. Reproductive responses and productive characteristics in ewes supplemented with detoxified castor meal for a long period

    Directory of Open Access Journals (Sweden)

    Liliane Moreira Silva

    2014-08-01

    Full Text Available The objective of the present study was to evaluate the effects of supplementation with detoxified castor meal (DCM in the diet of ewes during pregnancy, partum, and post-partum on the weight development of their offspring and at slaughter. The study included 56 ewes with synchronized estrus that were naturally mated. At the beginning of pregnancy and in post-partum, hepatic and renal function-related parameters and progesterone levels were measured. At slaughter, the proximate composition and fatty acid profile were determined in the loin of ewes. There was no effect of diet on reproductive response after estrus synchronization. At the beginning of pregnancy, albumin and creatinine levels were lower in the DCM group. Supplementation with DCM did not alter the weight or body condition of ewes at partum. However, at weaning, the DCM group showed a higher loin-eye area (LEA in relation to the group fed diets without detoxified castor meal (WDCM. At partum, as well as at weaning, the offspring of the ewes supplemented with DCM had a larger LEA than the WDCM group. In post-partum, levels of glucose, urea, protein, and cholesterol were lower in the DCM group. The return to cyclicity was similar in both groups, with an average of 47 days after partum. At slaughter, neither anatomical and carcass components nor the results of the proximate analysis were affected by the type of diet, except for an increase in heptadecanoic acid in the DCM group. Supplementation with detoxified castor meal in the diet of ewes does not affect lambing, pregnancy, prolificacy, return to cyclicity, milk production, blood biochemical parameters, or carcass characteristics.

  16. [Immune response and reproductive consequences in experimentally infected ewes with Brucella ovis during late pregnancy].

    Science.gov (United States)

    Paolicchi, Fernando A; Nuñez, Marta; Fiorentino, María A; Malena, Rosana C; Trangoni, Marcos; Cravero, Silvio; Estein, Silvia M

    2013-01-01

    Ovine brucellosis by Brucella ovis is a highly prevalent disease in Argentina. This study aimed to evaluate the pathogenicity of B. ovis and the serological response in ewes during late pregnancy and in their offspring. Six adult ewes were distributed in two groupsG1 (pregnant females, n = 4) and G2 (nonpregnant females, n = 2). Three pregnant ewes at 15 days prepartum and one nonpregnant eve were inoculated with B. ovis. Sera of sheep and their offspring were analyzed by different serological tests. Samples of cervicovaginal mucus, placenta and milk were studied by bacteriology. A Brucella genus-specific PCR assay was carried out in placenta and milk samples. Placenta samples were hystopathologically processed. g1 females gave birth to live lambs, but one died hours postpartum. Serological techniques employed detected antibodies in serum of inoculated pregnant animal 5 days postchallenge. sera of female controls G1 and G2 remained negative throughout the study. Cervicovaginal mucus of infected ewes in G1 and G2 yielded negative results to bacteriology, but B. ovis was isolated from milk. The PCR assay was positive for the placenta and milk from inoculated pregnant ewes. Histopathology revealed necrotic suppurative placentitis in one placenta. However, although results demonstrated that B. ovis can invade the placenta and mammary gland, this bacterium did not cause abortion when it was inoculated intravenously at 15 days prepartum. B. ovis infection induced an early humoral response in pregnant ewes, but their lambs remained seronegative, indicating that there was no transfer of antibodies in infancy. Placenta colonization and milk excretion of B. ovis involves a potential source of infection for lambs, which could play a role as latent carriers of infection.

  17. Salmonella Indiana as a cause of abortion in ewes: Genetic diversity and resistance patterns.

    Science.gov (United States)

    Luque, I; Echeita, A; León, J; Herrera-León, S; Tarradas, C; González-Sanz, R; Huerta, B; Astorga, R J

    2009-03-02

    Salmonella enterica subspecies enterica Indiana, a food-borne serovar uncommon in most countries, was responsible for an outbreak of abortion in a flock of Lacaune dairy ewes in southern Spain. Drinking water and feedstuff samples were analysed in an attempt to determine the source of the infection. Pigeons (Columba livia) and turtledoves (Streptopelia turtur) in close contact with the ewes were captured and examined for the bacterium. Seventeen S. Indiana strains were isolated from the ewes and wild birds and the genetic similarity among them analysed by Pulsed Field Gel Electrophoresis (PFGE) after the digestion of their genomic DNA with the restriction enzyme XbaI. The results suggest the wild birds might be responsible for the outbreak in the ewes. The strains recovered were fully susceptible to 15 out of the 16 antimicrobial agents tested: ampicillin, amoxycillin clavulanate, cephalothin, ceftriaxone, gentamicin, neomycin, streptomycin, tetracycline, ciprofloxacin, enrofloxacin, sulphonamides, trimethoprim-sulphamethoxazole, apramycin, colistin and chloramphenicol. Differences in the resistance pattern to nalidixic acid were observed; 11 strains (64.7%) were nalidixic acid resistant (R-Nx) and 6 (35.3%) sensitive (S-Nx). Among the R-Nx strains, a substitution of Gly to Cys at position 81 (Gly81àCys) of the gyrA gene in 10 strains isolated from wild birds and ovine foetuses, and of Asp to Tyr at position 87 (Asp87àTyr) in one strain isolated from ewe faeces, were revealed by sequencing the gene. To control the outbreak, enrofloxacin treatment was administered for 5 days. The same therapy was used to prevent infection during following gestation cycles, administering the antimicrobial agent at presentation and over 4 weeks before birth. Anti-bird meshes and closed drinking and feeding troughs were also installed to prevent further contact of the ewes with wild birds.

  18. The comparison of ewe udder morphology traits of Improved Valachian, Tsigai, Lacaune breeds and their crosses

    Directory of Open Access Journals (Sweden)

    Pavol Makovický

    2014-05-01

    Full Text Available Morphological udder traits are very important for dairy animals. During this experiment, mor¬phological udder traits were subjectively assessed with the use of linear scores from selected ewes (381 ewes belonging to nine genotypes based on the basis of Improved Valachian, Tsigai and Lacaune breeds (7 udder traits; using a scale of 9 points; 1275 statements for each trait during the milking period from 2002 to 2008. Linear udder traits were: udder depth, cistern depth, teat position, teat size, udder cleft, udder attachment and udder shape. Collected data were processed with restricted methodology and maximal probability using a MIXED procedure from the SAS statistical package. During the research, all traits were influenced by the ewe genotype (P<0.001, furthermore, parity and stage of lactation had its effect as well. In most cases, crosses with 25 to 75 % share of dairy breeds, such as Lacaune and East Friesian, had larger udder cisterns than purebred Tsigai ewes and Improved Valachian breed. In¬convenient teat placement was found in the purebred Lacaune ewes, which, also, had the largest udders and cisterns among studied genotypes. Tsigai crossbreds and Improved Valachian breed, together with specialized dairy breeds had larger udders than purebred ewes (Tsigai and Improved Valachian breeds, but with negative placement of teat for milking. Compliances were statistically significant (P<0.001 due to influences on parameters for determining the size of udder (udder depth, cistern depth, teat size and udder attachment. In conclusion, udder depth, teat position and teat size may be used as suitable characteristics for marker selection, to improve milking ability of listed breeds.

  19. Uterine involution and progesterone level during the postpartum period in Barbary ewes in north Libya

    Directory of Open Access Journals (Sweden)

    M.S. Medan

    2015-02-01

    Full Text Available The objectives of the present study were to determine the time of uterine involution and ovarian activity using ultrasound examination and progesterone assay. Weekly progesterone levels were measured starting one week postpartum until two weeks after the 1st postpartum estrus in Barbary ewes lambed during winter in AL-Bayda city, north of Libya. A total of 15 Barbary ewes were used in the present study distributed in three groups according to the month of lambing as group 1 (lambed in January, group 2 (lambed in February and group 3 (lambed in March. Ewes were examined weekly by trans-rectal ultrasound to check involution of the uterus starting one week after lambing until complete uterine involution. Blood samples were collected from the jugular vein, and serum was separated and stored at ˗20 ºC until measuring progesterone using ELISA. Results showed that uterine involution completed at day 35 postpartum in groups 1 and 2, while it occurred at day 28 in group 3. The mean progesterone level was basal (less than 1 ng/ml for a long period and started to increase at days 119, 99 and 77 postpartum in group 1, 2 and 3, respectively. One ewe did not show estrus at all during the period of study in group 2 and there were no growing follicles on their ovaries. The obtained results indicate that, uterine involution as determined by ultrasound completed earlier in ewes lambed in March than those lambed in February or January. Also, progesterone level and ultrasound examination showed that there was no ovarian activity for a longtime after parturition indicating that reproduction in Barbary ewes tends to be seasonal in AL-Bayda city, north Libya.

  20. SYNOVIAL SARCOMA IN CHILDHOOD: CLINICAL AND RADIOLOGICAL FINDINGS

    Institute of Scientific and Technical Information of China (English)

    Xu Deyong; Zhan Alai; Luan Hongmei; Feng Weihua; Sun Xihe; Yang Zuwen

    1998-01-01

    Objective: To study the clinical characteristics and radiological features of synovial sarcoma in childhood and its relation to the diagnosis and treatment. Methods:The clinical radiological features of 15 children with synovial sarcoma proved surgically and pathologically were analyzed. Results: In children, the tumor boundaries are poorly defined due to paucity of fat, and metastasis usually occurs early. Eight patients in this series had bone involvement, including: direct erosion by tumor causing cortical destruction, indirect pressure defect with sharp margin and reactive bone sclerosis and bone destruction of the primary intraosseous synovial sarcoma.Conclusion: The tumor is often misdiagnosed, the final confirmed diagnosis must be made by histological examination with imaging findings. It is emphasized that the patients should be treated with radiotherapy and chemotherapy preoperatively and postoperatively.

  1. Delayed phlegmon with gallstone fragments masquerading as soft tissue sarcoma

    Science.gov (United States)

    Goodman, Laura F.; Bateni, Cyrus P.; Bishop, John W.; Canter, Robert J.

    2016-01-01

    Complications from lost gallstones after cholecystectomy are rare but varied from simple perihepatic abscess to empyema and expectoration of gallstones. Gallstone complications have been reported in nearly every organ system, although reports of malignant masquerade of retained gallstones are few. We present the case of an 87-year-old woman with a flank soft tissue tumor 4 years after laparoscopic cholecystectomy. The initial clinical, radiographic and biopsy findings were consistent with soft tissue sarcoma (STS), but careful review of her case in multidisciplinary conference raised the suspicion for retained gallstones rather than STS. The patient was treated with incisional biopsy/drainage of the mass, and gallstones were retrieved. The patient recovered completely without an extensive resectional procedure, emphasizing the importance of multidisciplinary sarcoma care to optimize outcomes for potential sarcoma patients. PMID:27333918

  2. Primary pulmonary synovial sarcoma: a rare case report

    Directory of Open Access Journals (Sweden)

    Sumalatha Kasturi

    2014-08-01

    Full Text Available Primary lung sarcoma is an extremely rare tumor, accounting for less than 0.5% of all lung tumors. Histological subtypes are differentiated on the basis of immunohistochemical markers, such as vimentin, desmin, actin, CD99, and epithelial membrane antigen. A 50-year-old male presented with progressively increasing shortness of breath with cough for 2 months. On Contrast Enhanced Computed Tomography (CECT of thorax a large heterogeneous mass with multiple areas of necrosis, occupying almost whole of left hemithorax was seen. CT-guided Fine Needle Aspiration Cytology (FNAC revealed spindle cell neoplasm. Histopathological examination revealed a spindle cell sarcoma. On immunohistochemistry the tumor cells expressed both epithelial membrane antigen and vimentin. Hence, final impression from immunohistochemistry was primary monophasic synovial sarcoma of lung. [Int J Res Med Sci 2014; 2(4.000: 1729-1731

  3. 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...... the variables and with mitotic score were investigated. Proliferative activity was assessed by use of monoclonal antibody MIB-1 and staining for iododeoxyuridine (IdUrd), and evaluated in multiple, random, systematically sampled fields of vision. MIB-1 indices were higher than those of IdUrd but for each case...... 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....

  4. Epithelioid Sarcoma: Opportunities for Biology-driven Targeted Therapy

    Directory of Open Access Journals (Sweden)

    Jonathan eNoujaim

    2015-08-01

    Full Text Available Epithelioid sarcoma is a soft tissue sarcoma of children and young adults for which the preferred treatment for localised disease is wide surgical resection. Medical management is to a great extent undefined, and therefore for patients with regional and distal metastases, the development of targeted therapies is greatly desired. In this review we will summarize clinically-relevant biomarkers (e.g., SMARCB1, CA125, dysadherin and others with respect to targeted therapeutic opportunities. We will also examine the role of EGFR, mTOR and polykinase inhibitors (e.g., sunitinib in the management of local and disseminated disease. Towards building a consortium of pharmaceutical, academic and non-profit collaborators, we will discuss the state of resources for investigating epithelioid sarcoma with respect to cell line resources, tissue banks, and registries so that a roadmap can be developed towards effective biology-driven therapies.

  5. Kaposi´s sarcoma, epidemic type. Case presentation

    Directory of Open Access Journals (Sweden)

    Gilberto Serrano Ocaña

    2009-05-01

    Full Text Available Before the AIDS epidemic, Kaposi's sarcoma was found mainly in elderly men of Mediterranean coast, eastern European background and Jewish ancestry (rarely in older women and is a slow growing skin tumor. In AIDS patients, the KS tends to develop more rapidly compromising the skin, lungs, gastrointestinal tract and other organs. In people with AIDS, Kaposi's sarcoma is caused by an interaction between HIV, a weakened immune system and human herpes virus 8. It affects approximately 20% of people with HIV that don’t take antiretroviral drugs. It is more common in homosexual’s patients, but may appear in any HIV positive individual, in Africa where heterosexual HIV transmission route is the most important can also be found in children and women. We are presenting a case of Kaposi sarcoma in a young female admitted at the Internal Medicine Department of Dora Nginza Hospital.

  6. The frequency of p53, Ki67, CD99 and Fli-1 protein expression in paraffin-embedded tissue blocks in Ewing’s sarcoma

    Directory of Open Access Journals (Sweden)

    Bagheri Hossein-Abadi Z

    2011-06-01

    Full Text Available "n Normal 0 false false false EN-US X-NONE AR-SA MicrosoftInternetExplorer4 /* Style Definitions */ table.MsoNormalTable {mso-style-name:"Table Normal"; mso-tstyle-rowband-size:0; mso-tstyle-colband-size:0; mso-style-noshow:yes; mso-style-priority:99; mso-style-qformat:yes; mso-style-parent:""; mso-padding-alt:0in 5.4pt 0in 5.4pt; mso-para-margin:0in; mso-para-margin-bottom:.0001pt; mso-pagination:widow-orphan; font-size:11.0pt; font-family:"Calibri","sans-serif"; mso-ascii-font-family:Calibri; mso-ascii-theme-font:minor-latin; mso-fareast-font-family:"Times New Roman"; mso-fareast-theme-font:minor-fareast; mso-hansi-font-family:Calibri; mso-hansi-theme-font:minor-latin; mso-bidi-font-family:Arial; mso-bidi-theme-font:minor-bidi;} Background: Ewing sarcoma family tumors (ESFTs are among the most malignant tumors in children and young adults. ESFTs include Ewing sarcoma (ES and peripheral primitive neuroectodermal tumors (pPNETs. As there seemed to be few studies on the molecular biology of ESFTs, we investigated the frequency of CD99, Ki67, p53 and Fli-1 protein expression in 15 Iranian patients with ESFTs. In addition, the correlation between expression rate of these proteins and various clinical factors, including age, sex and survival was computed."n"nMethods: The expression of the aforesaid proteins was studied by immunohisto-chemistry in formalin-fixed and paraffin-embedded blocks of 15 ESFTs specimens. Stained sections were classified according to the percentage of stained tumor cells."n"nResults: The results showed the membrane expression of CD99 protein in all of the specimens. The nuclear expression of Fli-1 protein was observed in 86.7% and the over-expression of p53 nuclear protein was seen in 53.3% of the specimens. The expression rate of Ki67 protein was 60%. Although a significant correlation was not shown between the expression levels of Ki67, p53 or Fli-1 proteins with age, sex or survival of the patients, there was a significant

  7. Some dairy traits of Istrian ewes kept in semi-intensive farming conditions

    Directory of Open Access Journals (Sweden)

    Marina Pliško

    2016-02-01

    Full Text Available Istrian sheep has the highest milk yield among indigenous Croatian sheep breeds though originally belongs to a group of sheep of combined production traits. Since milk of Istrian sheep is traditionally processed into cheese and that processing possibilities of milk, among other things, are defined by its chemical composition and hygienic quality, the aim of this study was to determine the influence of some environmental factors (year, parity, stage of lactation, season (month of lambing on daily and lactation milk yield, lactation length, milk chemical composition and the somatic cell count (SCC in the milk of Istrian ewes. A total of 83 purebred, dairy Istrian sheep, during three consecutive lactations (from 2012 to 2014, were involved in this research. Due to conditions of feeding, care and housing, all ewes were kept in identical (semi-intensive farming conditions throughout the whole study period. During milking period of lactation regular milking controls were carried out (AT method and, on these occasions, individual milk samples for chemical composition analysis and determination of somatic cell count were taken. During average lactation length of 206 days Istrian ewes produced on average 190.77 kg of milk, or 1.1 kg of milk per day. Milk of Istrian ewes on average contained 6.81% fat, 5.90% protein, 4.32% lactose, 18.08% total solids and 11.31% non-fat solids. The geometric mean of SCC was 316*103*mL-1 of milk (log 5.50±0.02. A significant (P < 0.001 effect of the year is determined on the milk yield and the lactation length, as well as the chemical composition of milk (with the exception of protein and SCC. Ewes in the fourth lactation achieved the highest average daily (P < 0.001 and lactation milk yield (P < 0.05, while the first-lambing ewes produced milk with the highest content of total solids, milk fat and proteins. Stage of lactation significantly (P < 0.001 affected the daily milk yield, milk chemical composition, as well as

  8. Evaluation of udder cisterns and effects on milk yield of dairy ewes.

    Science.gov (United States)

    Rovai, M; Caja, G; Such, X

    2008-12-01

    Nine Manchega (0.94 L/d) and 10 Lacaune (2.07 L/d) ewes at the same stage of lactation (90 d in milk) were used to study the interbreed differences in milk yield, mammary morphological traits, and machine-milking ability. Udder traits were measured after 6 h of udder filling before the start of the experiment. Cisternal area (by ultrasonography), cisternal milk (by teat cannula drainage), and alveolar milk (by machine milking after an intravenous oxytocin injection) were randomly measured 8 h after milking for 2 wk consecutively either with an intravenous injection of an oxytocin receptor blocking agent (atosiban, AT) or without (control, C) to avoid the occurrence of milk letdown before milking. Lacaune ewes had greater udder depth (22.5 +/- 0.9 vs. 19.6 +/- 0.9 cm) and cistern height (27.1 +/- 3.8 vs. 15.6 +/- 3.5 mm), whereas Manchega ewes had longer (42.7 +/- 1.5 vs. 32.7 +/- 1.5 mm) and wider teats (17.4 +/- 0.5 vs. 13.9 +/- 0.5 mm). Values per half udder for Manchega and Lacaune ewes differed in cisternal area (12.8 +/- 0.7 and 23.7 +/- 0.6 cm(2)) and cisternal milk (120 +/- 0.6 and 269 +/- 0.5 mL), but not in alveolar milk (95 +/- 0.5 and 102 +/- 0.4 mL), respectively. Cisternal area and cisternal milk were positively correlated (r = 0.79). Ratios between cisternal and alveolar milk were 56:44 and 73:27 for Manchega and Lacaune ewes, respectively. Cisternal milk volumes obtained with the AT or C treatment were similar in Manchega (111 +/- 10 vs. 122 +/- 8 mL) but differed in Lacaune ewes (239 +/- 8 vs. 299 +/- 8 mL), respectively. Consequently, alveolar milk with AT vs. C was similar in Manchega (104 +/- 8 vs. 86 +/- 7 mL) but different in Lacaune ewes (115 +/- 7 vs. 89 +/- 7 mL). Results of this experiment confirm the need for the use of an oxytocin-blocking agent for accurate evaluation of milk contained in the udder of dairy ewes. Moreover, despite the differences in daily milk yield, alveolar milk did not vary between breeds, emphasizing the role of the

  9. Anti-Leptospira sp. agglutinins in ewes in the Federal District, Brazil.

    Science.gov (United States)

    Seixas, Luiza de S; de Melo, Cristiano Barros; Leite, Rômulo C; Moreira, Elvio C; McManus, Concepta M; de Castro, Márcio B

    2011-01-01

    To define the prevalence of anti-Leptospira sp. agglutinins in ewes in the Federal District, Brazil, serum samples from 157 ewes were tested for antibodies against serovars of Leptospira sp. by the microscopic agglutination test. Antibodies were detected in three flocks in a prevalence of 3% (95% CI = 0.4%-5.7%). Considering that sheep and cattle were raised together, the lack of sanitary control could represent a risk to cattle production, which is the most important activity in the Centre-West region of Brazil.

  10. Hidden Pictures of Kaposi's Sarcoma in Psoriatic Lesions: A Diagnostic Challenge

    Science.gov (United States)

    Yoo, Jisook; Jo, Mingyul; Kim, Min-Soo; Choi, Kwang-Hyun; Park, Hyang-Joon

    2016-01-01

    Kaposi's sarcoma is a multifocal proliferative vascular tumor involving the skin and other organ and psoriasis is a chronic cutaneous disease with papules and plaques with white scale. Development of Kaposi's sarcoma in psoriasis patients has been reported rarely. A 71-year-old man presented with multiple brownish to violaceous plaques on both feet and arms which were found 4 months ago. The biopsy confirmed Kaposi's sarcoma. The patient was diagnosed with psoriasis vulgaris 10 years ago and Kaposi's sarcoma lesions developed between psoriatic plaques. We herein report a rare case of simultaneous occurrence of Kaposi's sarcoma and psoriasis vulgaris which need quite different treatment. PMID:27904275

  11. Primary renal undifferentiated sarcoma as an infiltrative mass in a 12 year old boy

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yong Hee; Kim, Myung Joon; Lee, Mi Jung [Dept. of Radiology and Research Institute of Radiological Science, Severance Children' s Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, Se Hwa [Dept. of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2015-09-15

    Undifferentiated sarcomas are rare tumors not classified into any sarcoma subtype. Due to their rarity, imaging findings of undifferentiated sarcomas are poorly characterized. The purpose of this report was to present imaging findings of a pathologically confirmed undifferentiated sarcoma originated from the left kidney of a 12-year-old boy. The mass was infiltrative involving the renal pelvis. It mimicked massive hilar lymphadenopathy with a preserved renal contour visible by both ultrasonography and CT. Renal vein thrombosis was also observed. Although undifferentiated sarcomas are rare, they should be considered in differential diagnosis of infiltrative renal masses with renal pelvis invasion in children.

  12. Follicular Dendritic Cell Sarcoma of the Abdomen: the Imaging Findings

    Energy Technology Data Exchange (ETDEWEB)

    Kang, Tae Wook; Lee, Soon Jin; Song, Hye Jong [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)

    2010-04-15

    Follicular dendritic cell sarcoma is a rare neoplasm that originates from follicular dendritic cells in lymphoid follicles. This disease usually involves the lymph nodes, and especially the head and neck area. Rarely, extranodal sites may be affected, including tonsil, the oral cavity, liver, spleen and the gastrointestinal tract. We report here on the imaging findings of follicular dendritic cell sarcoma of the abdomen that involved the retroperitoneal lymph nodes and colon. It shows as a well-defined, enhancing homogenous mass with internal necrosis and regional lymphadenopathy.

  13. Clear cell sarcoma: A case mimicking primary cutaneous malignant melanoma

    Directory of Open Access Journals (Sweden)

    Rodriguez-Martin M

    2009-01-01

    Full Text Available Clear cell sarcoma (CCS is a recently described variant of sarcoma characterized by prominent clear cells showing features similar to clear cell melanoma. This neoplasm was first described by Dr. Franz M. Erzinger. Primary CCS usually arises in deeper soft tissues, in association with fascia, tendons, or aponeuroses. Characteristic translocation t(12;22 (q13;q12 has been considered pathognomonic for CCS. Prognosis is related to tumor size. An early recognition and initial radical surgery is the key to a favourable outcome. We present a patient with an unusual neoplasm that resembled malignant melanoma.

  14. SARC: Development and Support of a Sarcoma Research Consortium Infrastructure

    Energy Technology Data Exchange (ETDEWEB)

    Arkison, Jim

    2007-10-29

    SARC is a non-for-profit organization whose mission and vision is to advocate for the collaboration on the design of clinical trials on sarcoma, to further the knowledge regarding the diagnosis and treatment of sarcoma and provide accurate and up to date information to physicians, patients and families. The objectives are to assist in the development of the infrastructure for the continued growth and spectrum of clinical research, to facilitate biannual meeting of investigators, and to develop a preclinical research base that would design and conduct research that would improve the process of drug treatments selected for clinical research trials.

  15. [Fast neutrons in the treatment of soft tissue sarcomas].

    Science.gov (United States)

    Chernichenko, V A; Tolstopiatov, B A; Monich, A Iu; Konovalenko, V F; Galakhin, K A; Palivets, A Iu; Vorona, A M

    1990-01-01

    Results of treatment of 101 cases of soft tissue sarcoma are presented in the paper. Preoperative irradiation technique and radical program of treatment are described. Combined radiation and surgical treatment was given to 45 patients whereas conservative--to 56. Sixty-three cases received adjuvant combination chemotherapy. Response and three-year survival rates were compared to those in control group treated by photons. The results observed in patients of combined and conservative treatment groups who had been irradiated with fast neutrons proved significantly better than in controls. These data suggest vistas in application of fast neutron irradiation for the treatment of soft tissue sarcomas.

  16. Primary Pulmonary Synovial Sarcoma: A Very Rare Presentation

    Directory of Open Access Journals (Sweden)

    Ekrem Cengiz Seyhan

    2014-01-01

    Full Text Available Synovial sarcoma (SS is a rare tumor originating from mesenchymal tissue and accounting for approximately 5–10% of all soft tissue sarcomas. A rare case of primary pulmonary SS in an asymptomatic 18-year-old man admitted to our hospital for investigation of a 6 × 6.5 cm, oval-shaped, well-delineated pleural based peripheral mass in the left lower lobe in his thorax CT is presented. Left lower lobectomy was done. Immunohistochemically, tumor cells were positive for cytokeratin, epithelial membrane antigen (EMA, and vimentin so that the histopathological diagnosis was compatible with biphasic spindle cell type SS in the lung.

  17. Imaging diagnosis--spinal cord histiocytic sarcoma in a dog.

    Science.gov (United States)

    Taylor, Amanda; Eichelberger, Bunita; Hodo, Carolyn; Cooper, Jocelyn; Porter, Brian

    2015-01-01

    A 12-year-old mixed breed dog was presented for evaluation of progressive paraparesis and ataxia. Magnetic resonance (MR) imaging was performed and identified multifocal intradural spinal cord mass lesions. The lesions were hyperintense in T2-weighted sequences, isointense to mildly hyperintense in T1-weighted sequences with strong contrast enhancement of the intradural lesions and spinal cord meninges. Spinal cord neoplasia was suspected. A diagnosis of intramedullary spinal cord histiocytic sarcoma, confined to the central nervous system, was confirmed histopathologically. Spinal cord histiocytic sarcoma is a rare neoplasm, but should be included in the differential diagnosis for dogs with clinical signs of myelopathy.

  18. Simultaneous lymph node involvement by Castleman disease and Kaposi sarcoma

    Directory of Open Access Journals (Sweden)

    Luciana Wernersbach Pinto

    2011-02-01

    Full Text Available Both multicentric Castleman disease and Kaposi sarcoma are more frequently observed in HIV infected patients. The coexistence of these Human herpesvirus 8 related lesions, in the same tissue, has been observed, but literature reports are scant. On the other hand, the expression of HHV-8-LANA-1 is easily demonstrable by immunohistochemistry. This has been shown to be a powerful tool for the diagnosis of these entities. The aim of this report is to communicate our experience with a case of multicentric Castleman disease occurring in the setting of HIV infection, which demonstrated microscopic Kaposi sarcoma in the same lymph node during the pathological work-up

  19. Pathogenesis and Associated Diseases of Kaposi's Sarcoma-associated Herpesvirus

    Institute of Scientific and Technical Information of China (English)

    Lin-ding WANG

    2007-01-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) is the primary etiological agent of Kaposi's sarcoma, primary effusion lymphoma and muticentric Castleman's disease. In common with the other herpesviruses, KSHV exhibits both latent and lytic life cycles, both of which are characterized by distinct gene expression profiles and programs. KSHV encodes proteins which play essential roles in the inhibition of host adaptive and innate immunity, the inhibition of apoptosis, and the regulation of the cell cycle. KSHV also encodes several proteins which have transforming and intrcellular signalling activity.

  20. INFLUENCE OF DIETARY SELENIUM SUPPLEMENTATION OF EWES ON PRODUCTION TRAITS, ANTIOXIDANT STATUS AND METABOLIC PROFILE OF LAMBS

    Directory of Open Access Journals (Sweden)

    Josip Novoselec

    2013-12-01

    Full Text Available The aim of this study was to determine the effect of dietary selenium supplementation (organic, inorganic of high pregnant ewes on the production traits of lambs, the concentration of selenium in the blood of ewes and their lambs, indicators of antioxidant status in the blood of ewes and their lambs, the metabolic profile of ewes and their lambs and concentrations of thyroid hormones. Ewes were in the last third of pregnancy, the average age of four years, healthy and in good condition, divided into three groups of 10 animals. The research lasted 4 months respectively, 2 months with ewes during high pregnancy, 2 months with ewes during lactation and on their lambs during suckling period. Ewes ration from control group one was composed from 300 g/day/animal feed mixture without addition of selenium and 150 g/day/animal barley and alfalfa hay that they had ad libitum. Feed mixture from second group of ewes was supplemented with 0.3 mg/kg organic form of selenium (Sel-Plex®, and feed mixture from third group with the same amount inorganic form of selenium (sodium selenite. Selenium supplementation of ewes feed mixture did not significantly influence on the production traits of their lambs postpartum. Selenium supplementation of ewes and their lambs had influence on a significant (P<0.01; P<0.05 increase in the concentration of selenium, GSH-Px and SOD in whole blood compared to control group of ewes. Organic selenium supplement had a more significant impact on the increase in concentration of selenium and GSH-Px in the blood. In the ewes and lambs blood was determined decrease of MDA with increasing concentrations of selenium in the blood. Generally, the selenium supplementation led to an increase (P<0.05 in the number of WBC and lymphocytes in the blood of ewes and lambs. Also, the increase in the number of RBC, HGB content and MCV in lambs and MCH as well as MCHC in ewes that had a selenium supplement in feed mixture were determined