Ewing's Sarcoma
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The value of local treatment in patients with primary, disseminated, multifocal Ewing sarcoma (PDMES)
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was investigated.We analyzed 120 patients registered into the European Ewing Tumor Working Initiative of National Groups (EURO-E.W.I.N.G. 99) trial at the trial center of Muenster from 1998 to 2006. Median age was 16.2 years. Local treatment of the primary tumor was surgery in 26 of 120 patients, surgery and radiotherapy in 21 patients, and definitive radiotherapy in 40 patients. For treatment of metastases, 6 of 120 patients received surgery; 9 patients, surgery and radiotherapy; and 33 patients, definitive radiotherapy. Forty-seven (39%) patients had local treatment of both the primary tumor and metastases, 41 (34%) pat...
Source: Cancer - November 18, 2009 Category: Cancer & Oncology Authors: Julia Haeusler, Andreas Ranft, Tobias Boelling, Georg Gosheger, Gabriele Braun-Munzinger, Volker Vieth, Stefan Burdach, Henk van den Berg, Heribert Juergens, Uta Dirksen Source Type: journals
Long-Term Evaluation of Ifosfamide-Related Nephrotoxicity in Children [Pediatric Oncology]
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Conclusion
Renal toxicity is moderate with a moderate dose of ifosfamide. However, since it can be permanent and can get worse with time, repeated long-term evaluations are important, and this risk should be balanced against efficacy. (Source: Journal of Clinical Oncology)
Source: Journal of Clinical Oncology - November 6, 2009 Category: Cancer & Oncology Authors: Oberlin, Fawaz, Rey, Niaudet, Ridola, Orbach, Bergeron, Defachelles, Gentet, Schmitt, Rubie, Munzer, Plantaz, Deville, Minard, Corradini, Leverger, de Vathaire Tags: Long Term Survival & Late Effects, Osteosarcoma & Ewing sarcomas:, Chemotherapy, Rhabdo & Other Soft Tissue Sarcomas:, Chemotherapy Pediatric Oncology Source Type: journals
Isolated CNS vasculitis: Unusual presentation of relapsed Ewing sarcoma
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We describe a 12-year-old boy male who presented with an expressive dysphasia after completion of treatment for unifocal Ewing sarcoma. CNS vasculitis was diagnosed by MRI/MRA and cerebral angiography. Extensive rheumatologic work-up failed to identify an underlying primary process. Restaging studies showed no evidence of tumor. Complete neurologic recovery was achieved on prednisone. Four months later the patient developed overt, extensive metastases, confirmed by biopsy to represent recurrent Ewing sarcoma. Despite intensive therapy the patient succumbed 6 months later. This case demonstrates the unique finding of isolat...
Source: Pediatric Blood and Cancer - November 3, 2009 Category: Cancer & Oncology Authors: Polina Stepensky, Elisha Waldman, Natalia Simanovsky, Iris Fried, Shoshana Revel-Vilk, Igor B. Resnick, Michael Weintraub Source Type: journals
MR Imaging of Primary Bone Tumors and Tumor-like Conditions in Children
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This article provides a review of the MR imaging features of the major primary malignant and benign bone tumors and tumorlike conditions encountered in the pediatric population. Malignant tumors discussed include osteosarcoma, Ewing sarcoma, chondrosarcoma, lymphoma, and malignant fibrous histiocytoma. Benign lesions discussed include simple bone cysts, aneurysmal bone cysts, giant cell tumor, osteochondroma, enchondroma, chondroblastoma, osteoid osteoma, osteoblastoma, nonossifying fibroma, fibrous dysplasia, osteofibrous dysplasia, hemangioma, and histiocytosis. The use of MR imaging in the diagnosis of these lesions is ...
Source: Radiologic Clinics of North America - November 1, 2009 Category: Radiology Authors: Sandra L. Wootton-Gorges Source Type: journals
Primary bone lymphoma.
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Primary bone lymphomas are rare, even though secondary involvement of the bone marrow is a common event in systemic lymphomas. Most primary bone lymphomas are primary bone diffuse large B-cell lymphomas (PBDLBCLs) with a rare occurrence of follicular, marginal zone, anaplastic large cell, Hodgkin, and T-cell lymphomas. The PBDLBCL affects the middle-aged to elderly population, with a slight predominance in men. The patients present with bone pain, palpable mass, fractures, or neurologic symptoms. The metaphysis of bones is a common location of PBDLBCL. Morphologically, the lymphoma consists of a polymorphous mixture of...
Source: Archives of Pathology and Laboratory Medicine - November 1, 2009 Category: Laboratory Medicine Authors: Bhagavathi S, Fu K Tags: Arch Pathol Lab Med Source Type: journals
MR imaging of primary bone tumors and tumor-like conditions in children.
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This article provides a review of the MR imaging features of the major primary malignant and benign bone tumors and tumor-like conditions encountered in the pediatric population. Malignant tumors discussed include osteosarcoma, Ewing sarcoma, chondrosarcoma, lymphoma, and malignant fibrous histiocytoma. Benign lesions discussed include simple bone cysts, aneurysmal bone cysts, giant cell tumor, osteochondroma, enchondroma, chondroblastoma, osteoid osteoma, osteoblastoma, nonossifying fibroma, fibrous dysplasia, osteofibrous dysplasia, hemangioma, and histiocytosis. The use of MR imaging in the diagnosis of these lesions is...
Source: Radiologic Clinics of North America - November 1, 2009 Category: Radiology Authors: Wootton-Gorges SL Tags: Radiol Clin North Am Source Type: journals
Cell cycle inhibition and apoptosis induced by curcumin in Ewing sarcoma cell line SK-NEP-1
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Abstract Curcumin is a naturally occurring polyphenolic compound found in the turmeric, which is used as food additive in Indian cooking
and as a therapeutic agent in traditional Indian medicine. Curcumin is currently under investigation as a chemotherapeutic
and chemopreventive agent in adult cancer models at both pre-clinical and clinical levels. In this preliminary study, we show
that curcumin is effective in causing cell cycle arrest, inducing apoptosis, and suppressing colony formation in the Ewing
sarcoma cell line SK-NEP-1. Curcumin causes upregulation of cleaved caspase 3 and downregulation of phosph...
Source: Medical Oncology - October 27, 2009 Category: Cancer & Oncology Tags: Medical Oncology Source Type: journals
Importance and mechanism of 'switch' function of SAP family adapters
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Summary: The signaling lymphocytic activation molecule (SLAM)-associated protein (SAP) family of adapters includes SAP, Ewing's sarcoma-associated transcript-2 (EAT-2), and EAT-2-related transducer (ERT). These Src homology-2 (SH2) domain-only molecules play critical roles in immune regulation. The prototype of the SAP family, SAP, is mutated in X-linked lymphoproliferative disease in humans. Moreover, genetically engineered mice lacking one or more SAP family members have defects in multiple immune cell types including T cells, natural killer (NK) cells, NKT cells, and B cells. Accumulating data show that SAP family adapt...
Source: Immunological Reviews - October 23, 2009 Category: Allergy & Immunology Authors: André Veillette, Zhongjun Dong, Luis-Alberto Pérez-Quintero, Ming-Chao Zhong, Mario-Ernesto Cruz-Munoz Tags: Review Articles Source Type: journals
Ewing sarcoma of the neck
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No Abstract. (Source: Pediatric Blood and Cancer)
Source: Pediatric Blood and Cancer - October 22, 2009 Category: Cancer & Oncology Authors: Sandrin Schmidt, Herwig Lackner, Christian Urban Source Type: journals
Primary <i>Ewing's sarcoma</i> of the frontal bone with intracranial extension
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We report a case of an 11-year-old male child with primary Ewing's sarcoma of the frontal bone with intracranial extension. We also discuss the imaging and intraoperative findings and describe our use of a split calvarial graft in the present case. (Source: Journal of Cancer Research and Therapeutics)
Source: Journal of Cancer Research and Therapeutics - October 16, 2009 Category: Cancer & Oncology Authors: Agrawal Amit, Dulani Rajesh, Mahadevan Anitha, Vagaha S J, Vagha Jayant, Shankar S K Source Type: journals
Critical signaling pathways in bone sarcoma: Candidates for therapeutic interventions
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Abstract Bone sarcomas cause disproportionate morbidity and mortality and desperately need new therapies as there has been little improvement
in outcomes in 20 years. Identification of critical signaling pathways, including type 1 insulin-like growth factor receptor
(IGF-1R) for Ewing sarcoma and possibly osteosarcoma, and the ERBB and the Wnt signaling pathways for osteosarcoma, have emerged
as receptors mediating vital signals for bone sarcoma. Akt, mammalian target of rapamycin (mTOR), phosphoinositide 3-kinases,
mitogen-activated protein kinase kinase, extracellular signal-regulated kinases, and Ras path...
Source: Current Oncology Reports - October 13, 2009 Category: Cancer & Oncology Tags: Current Oncology Reports Source Type: journals
The Insulin-like Growth Factor-1 Receptor-Targeting Antibody, CP-751,871, Suppresses Tumor-Derived VEGF and Synergizes with Rapamycin in Models of Childhood Sarcoma
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We examined the antitumor activity of CP-751,871, a human antibody that blocks IGF-1R ligand binding, alone and in combination with rapamycin against sarcoma cell lines in vitro and xenograft models in vivo. In Ewing sarcoma (EWS) cell lines, CP751,871 inhibited growth poorly (<50%), but prevented rapamycin-induced hyperphosphorylation of AKT(Ser473) and induced greater than additive apoptosis. Rapamycin treatment also increased secretion of IGF-1 resulting in phosphorylation of IGF-1R (Tyr1131) that was blocked by CP751,871. In vivo CP-751,871, rapamycin, or the combination were evaluated against EWS, osteosarcoma, and...
Source: Cancer Research - October 1, 2009 Category: Cancer & Oncology Authors: Kurmasheva, R. T., Dudkin, L., Billups, C., Debelenko, L. V., Morton, C. L., Houghton, P. J. Tags: Experimental Therapeutics, Molecular Targets, and Chemical Biology Source Type: journals
Molecular pathology of sarcomas: concepts and clinical implications
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Abstract The molecular genetic changes that have been described in sarcomas over the past era have aided our understanding of their
pathogenesis. The majority of sarcomas carry nonspecific genetic changes within a background of a complex karyotype. These
constitute the challenges in sarcoma research for unraveling a putative multistep genetic model, such as for chondrosarcoma,
and finding targets for therapeutic strategies. Approximately 15–20% of mesenchymal tumors carry a specific translocation
within a relatively simple karyotype. The resulting fusion products act either as transcription factors upregul...
Source: Virchows Archiv - September 29, 2009 Category: Pathology Tags: Virchows Archiv Source Type: journals
Molecular characterization of commonly used cell lines for bone tumor research: A trans-European EuroBoNet effort
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Usage of cancer cell lines has repeatedly generated conflicting results provoked by differences among subclones or contamination with mycoplasm or other immortal mammalian cells. To overcome these limitations, we decided within the EuroBoNeT consortium to characterize a common set of cell lines including osteosarcomas (OS), Ewing sarcomas (ES), and chondrosarcomas (CS). DNA fingerprinting was used to guarantee the identity of all of the cell lines and to distinguish subclones of osteosarcoma cell line HOS. Screening for homozygous loss of 38 tumor suppressor genes by MLPA revealed deletion of CDKN2A as the most common even...
Source: Genes, Chromosomes and Cancer - September 28, 2009 Category: Cancer & Oncology Authors: Laura Ottaviano, Karl-Ludwig Schaefer, Melanie Gajewski, Wolfgang Huckenbeck, Stefan Baldus, Uwe Rogel, Carlos Mackintosh, Enrique de Alava, Ola Myklebost, Stine H. Kresse, Leonardo A. Meza-Zepeda, Massimo Serra, Anne-Marie Cleton-Jansen, Pancras C. W. Ho Source Type: journals
Primary cutaneous Ewing sarcoma/primitive neuroectodermal tumour: a clinicopathological analysis of seven cases highlighting diagnostic pitfalls and the role of FISH testing in diagnosis [Original articles]
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Conclusions:
Although rare, primary cutaneous ES/PNET should be considered in the differential diagnosis of cutaneous "small blue cell tumours". Immunostaining for FLI-1 and molecular testing for evidence of an EWS rearrangement are useful ancillary investigations to confirm the diagnosis. The prognosis of primary cutaneous ES/PNET appears to be more favourable than extracutaneous ES/PNET. (Source: Journal of Clinical Pathology)
Source: Journal of Clinical Pathology - September 25, 2009 Category: Pathology Authors: Shingde, M V, Buckland, M, Busam, K J, McCarthy, S W, Wilmott, J, Thompson, J F, Scolyer, R A Tags: Immunology (including allergy), Skin cancer, Dermatology, Clinical diagnostic tests Original articles Source Type: journals
Peripheral blood stem cell mobilization with pegfilgrastim compared to filgrastim in children and young adults with malignancies
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Pegfilgrastim, the long acting agent of rh-GCSF, has been shown to be as effective as Filgrastim in children undergoing cytotoxic chemotherapy by reducing the duration of neutropenia. Recent studies in adults have also shown that Pegfilgrastim is effective to mobilize CD34+ stem cells, resulting in earlier peripheral stem cell collections (PSCC). The aim of the study was to compare the efficacy of Pegfilgrastim with Filgrastim for CD34+ stem cell mobilization in children.Three groups of patients were compared: Group 1: six patients with Ewing Sarcoma stimulated with Filgrastim; Group 2: five patients with Ewing Sarcoma, Ep...
Source: Pediatric Blood and Cancer - September 24, 2009 Category: Cancer & Oncology Authors: Peter Fritsch, Wolfgang Schwinger, Gerold Schwantzer, Herwig Lackner, Petra Sovinz, Gerald Wendelin, Martin Benesch, Sabine Sipurzynski, Christian Urban Source Type: journals
[Original articles] Primary cutaneous Ewing sarcoma/primitive neuroectodermal tumour: a clinicopathological analysis of seven cases highlighting diagnostic pitfalls and the role of FISH testing in diagnosis
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Conclusions:
Although rare, primary cutaneous ES/PNET should be considered in the differential diagnosis of cutaneous "small blue cell tumours". Immunostaining for FLI-1 and molecular testing for evidence of an EWS rearrangement are useful ancillary investigations to confirm the diagnosis. The prognosis of primary cutaneous ES/PNET appears to be more favourable than extracutaneous ES/PNET. (Source: Journal of Clinical Pathology)
Source: Journal of Clinical Pathology - September 24, 2009 Category: Pathology Authors: Shingde, M V, Buckland, M, Busam, K J, McCarthy, S W, Wilmott, J, Thompson, J F, Scolyer, R A Tags: Immunology (including allergy), Skin cancer, Dermatology, Clinical diagnostic tests Original articles Source Type: journals
Ewing's Sarcoma of the Ilium MimickiEng Inflammatory Arthritis of the Hip: A Case Report
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Classical clinical and radiological presentation of Ewing's sarcoma of ilium may not be the rule. One should be highly suspicious of the disease even if there is no direct pointer to the disease. Cases Journal (Source: Medscape Orthopaedics Headlines)
Source: Medscape Orthopaedics Headlines - September 22, 2009 Category: Orthopaedics Tags: Internal Medicine Source Type: info
Tyrosine Kinase Inhibitor Enhances the Bioavailability of Oral Irinotecan in Pediatric Patients With Refractory Solid Tumors [Pediatric Oncology]
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Conclusion
IV irinotecan given with 12 days of oral gefitinib is well tolerated in children. We observed one partial response. Gefitinib significantly enhances the bioavailability of oral irinotecan. This combination warrants further investigation, particularly with orally administered irinotecan. (Source: Journal of Clinical Oncology)
Source: Journal of Clinical Oncology - September 20, 2009 Category: Cancer & Oncology Authors: Furman, Navid, Daw, McCarville, McGregor, Spunt, Rodriguez-Galindo, Panetta, Crews, Wu, Gajjar, Houghton, Santana, Stewart Tags: Developmental Therapeutics & Pharmacology Pediatric Oncology Source Type: journals
Treatment results of the Ewing sarcoma of bone and prognostic factors
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The purpose of this study is to assess the clinical outcome of patients with ESFT of the bone treated with the European Intergroup Cooperative Ewings Sarcoma Study (EICESS)-92 treatment protocol at a single center, and to identify prognostic factors.Ninety-eight patients younger than 18 years of age, diagnosed with ESFT of the bone between 1992 and 2005, were analyzed retrospectively.Eighty-seven patients were treated with the EICESS-92 protocol. The median follow-up was 105 ± 38 months. The 5-year event-free survival (EFS) survival rate was 40%, the 5-year overall survival (OS) rate was 47%. For non-metastatic disease, t...
Source: Pediatric Blood and Cancer - September 15, 2009 Category: Cancer & Oncology Authors: Neriman Sar[inodot], Giray To[gbreve]ral, M. Faik Çetinda[gbreve], B. [Scedil]afak Güngör, [Idot]nci Ergürhan [Idot]lhan Source Type: journals
Use of FDG PET in Staging, Restaging, and Assessment of Therapy Response in Ewing Sarcoma [RSNA Education Exhibits]
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The article reviews the imaging evaluation of Ewing sarcoma; discusses and illustrates the role of FDG PET in staging, restaging, assessment of therapy response, and detection of tumor recurrence in Ewing sarcoma; and describes the limitations of FDG PET in this setting. (Source: Radiographics recent issues)
Source: Radiographics recent issues - September 14, 2009 Category: Radiology Authors: Bestic, J. M., Peterson, J. J., Bancroft, L. W. Tags: Musculoskeletal Radiology, Nuclear Medicine, Pediatric Radiology, Oncologic Imaging RSNA Education Exhibits Source Type: journals
Advances in Ewing's Sarcoma Research: Where Are We Now and What Lies Ahead?
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Ewing's sarcoma family tumors (EFT) are characterized by specific chromosomal translocations, which lead to EWS/ETS transcription factors. Elucidation of EWS/ETS target gene networks within the context of other signaling pathways, together with the identification of the initiating cell, and the development of genetically engineered mice will hopefully lead to biology-based therapeutic strategies for these tumors. [Cancer Res 2009;69(18):7140–50] (Source: Cancer Research)
Source: Cancer Research - September 13, 2009 Category: Cancer & Oncology Authors: Ordonez, J. L., Osuna, D., Herrero, D., de Alava, E., Madoz-Gurpide, J. Tags: Reviews Source Type: journals
Primitive Neuroectodermal Tumor of the Pelvis in an Elderly Patient.
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Conclusion: Our case is uncommon because of the age at diagnosis, the fortuitous way of revelation, and the choice of doseintense chemotherapy adapted from the Memphis protocol (cyclophosphamide- and doxorubicin-based) for children, which was efficient and safe. It supports the fact that an adult, and even an old patient, with good physical status, may be treated safely and radically even with dose-adapted aggressive chemotherapy.
PMID: 19745594 [PubMed - as supplied by publisher] (Source: Onkologie)
Source: Onkologie - September 13, 2009 Category: Cancer & Oncology Authors: Saada E, Thariat J, Follana P, Birtwisle-Peyrottes I, Haudebourg J, Trojani C, Bacque P, Thyss A Tags: Onkologie Source Type: journals
Antitumor Vectorized Oligonucleotides in a Model of Ewing Sarcoma: Unexpected Role of Nanoparticles
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Oligonucleotides , Vol. 0, No. 0. (Source: Oligonucleotides)
Source: Oligonucleotides - September 4, 2009 Category: Genetics & Stem Cells Tags: article Source Type: journals
Cytotoxic Effect of the Pentacyclic Oxindole Alkaloid Mitraphylline Isolated from Uncaria tomentosa Bark on Human Ewing's Sarcoma and Breast Cancer Cell Lines
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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 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,...
Source: Planta Medica - September 2, 2009 Category: Drugs & Pharmacology Tags: Original Papers Source Type: journals
Analysis of Ewing Sarcoma (EWS)-Binding Proteins: Interaction with hnRNP M, U, and RNA-Helicases p68/72 within Protein−RNA Complexes
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Journal of Proteome Research, Volume 0, Issue 0, Articles ASAP (As Soon As Publishable). (Source: Journal of Proteome Research)
Source: Journal of Proteome Research - September 1, 2009 Category: Biochemistry Tags: article Source Type: journals
New Hope For Deadly Childhood Bone Cancer
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Researchers at Huntsman Cancer Institute (HCI) at the University of Utah have shed new light on Ewing's sarcoma, an often deadly bone cancer that typically afflicts children and young adults. Their research shows that patients with poor outcomes have tumors with high levels of a protein known as GSTM4, which may suppress the effects of chemotherapy. The research is published online today in the journal Oncogene. (Source: Pediatrics News From Medical News Today)
Source: Pediatrics News From Medical News Today - September 1, 2009 Category: Pediatrics Tags: Cancer / Oncology Source Type: news
Bone sarcomas arising in patients with neurofibromatosis type 1.
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This study suggests a possible relationship between neurofibromatosis type 1 and the development of a bone sarcoma, the increased risk being estimated at eight times that of the normal population. We recommend that further research into this possible link should be considered.
PMID: 19721051 [PubMed - in process] (Source: The Journal of Bone and Joint Surgery. British volume)
Source: The Journal of Bone and Joint Surgery. British volume - August 31, 2009 Category: Orthopaedics Authors: Chowdhry M, Hughes C, Grimer RJ, Sumathi V, Wilson S, Jeys L Tags: J Bone Joint Surg Br Source Type: journals
Rare nerve lesions of non-nerve sheath origin: a 17-year retrospective series.
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CONCLUSIONS: Diverse benign and malignant conditions can affect peripheral nerve.
PMID: 19722745 [PubMed - in process] (Source: Archives of Pathology and Laboratory Medicine)
Source: Archives of Pathology and Laboratory Medicine - August 31, 2009 Category: Laboratory Medicine Authors: Strom T, Kleinschmidt-Demasters BK, Donson A, Foreman NK, Lillehei KO Tags: Arch Pathol Lab Med Source Type: journals
Extended posterior circumferential approach to thoracic and thoracolumbar spine.
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OBJECTIVE: Posterior spinal surgical approach to achieve a retropleural/ retroperitoneal corpectomy with circumferential spinal cord decompression following subtotal vertebrectomy, posterior instrumentation and interbody spacer placement under compression as well as kyphosis correction with spinal column shortening. INDICATIONS: Infective, traumatic or neoplastic lesions of the vertebral body that lead to vertebral body destruction, instability and neurologic deficit. Need for immediate postoperative loading stability to permit ambulation and rehabilitation. CONTRAINDICATIONS: Multiple contiguous vertebral disease. Ins...
Source: Operative Orthopadie und Traumatologie - August 31, 2009 Category: Orthopaedics Authors: Sundararaj GD, Venkatesh K, Babu PN, Amritanand R Tags: Oper Orthop Traumatol Source Type: journals
New hope for deadly childhood bone cancer
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(University of Utah Health Sciences) Researchers at Huntsman Cancer Institute at the University of Utah have shed new light on Ewing's sarcoma, an often deadly bone cancer that typically afflicts children and young adults. Their research shows that patients with poor outcomes have tumors with high levels of a protein known as GSTM4, which may suppress the effects of chemotherapy. The research is published online Aug. 31 in the journal Oncogene. (Source: EurekAlert! - Medicine and Health)
Source: EurekAlert! - Medicine and Health - August 31, 2009 Category: Global & Universal Source Type: news
c-Jun NH2-Terminal Kinase Activation Is Essential for DRAM-Dependent Induction of Autophagy and Apoptosis in 2-Methoxyestradiol-Treated Ewing Sarcoma Cells
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Ewing sarcoma and osteosarcoma are two aggressive cancers that affect bones and soft tissues in children and adolescents. Despite multimodal therapy, patients with metastatic sarcoma have a poor prognosis, emphasizing a need for more effective treatment. We have shown previously that 2-methoxyestradiol (2-ME), an antitumoral compound, induces apoptosis in Ewing sarcoma cells through c-Jun NH2-terminal kinase (JNK) activation. In the present study, we provide evidence that 2-ME elicits macroautophagy, a process that participates in apoptotic responses, in a JNK-dependent manner, in Ewing sarcoma and osteosarcoma cells. We a...
Source: Cancer Research - August 30, 2009 Category: Cancer & Oncology Authors: Lorin, S., Borges, A., Ribeiro Dos Santos, L., Souquere, S., Pierron, G., Ryan, K. M., Codogno, P., Djavaheri-Mergny, M. Tags: Experimental Therapeutics, Molecular Targets, and Chemical Biology Source Type: journals
Flow cytometric detection of Ewing sarcoma cells in peripheral blood and bone marrow
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A new method for detecting circulating Ewing sarcoma cells using flow cytometry is described. This strategy exploits the nearly universal expression of CD99 and the lack of expression of CD45 by Ewing sarcoma cells.Ewing sarcoma cell line A673, peripheral blood mononuclear cells (PBMCs), and bone marrow mononuclear cells (BMMCs) were stained for CD99 and CD45 in order to detect CD99+CD45- cells by flow cytometry. Known quantities of A673 Ewing sarcoma cells were spiked into control PBMCs to test the accuracy of this method. Control PBMCs were evaluated to assess the level of background staining.Flow cytometry was accurate ...
Source: Pediatric Blood and Cancer - August 25, 2009 Category: Cancer & Oncology Authors: Steven G. DuBois, C. Lorrie Epling, Juli Teague, Katherine K. Matthay, Elizabeth Sinclair Source Type: journals
Ewing's Sarcoma of the Inferior Vena Cava
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We describe the first reported case of Ewing’s sarcoma of the inferior vena cava. (Source: Vascular and Endovascular Surgery)
Source: Vascular and Endovascular Surgery - August 24, 2009 Category: Surgery Authors: Mahmood, A., Cleasby, M., Hubscher, S. G., Khaira, H. S. Tags: Article Source Type: journals
c-Jun NH2-Terminal Kinase Activation Is Essential for DRAM-Dependent Induction of Autophagy and Apoptosis in 2-Methoxyestradiol-Treated Ewing Sarcoma Cells.
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Ewing sarcoma and osteosarcoma are two aggressive cancers that affect bones and soft tissues in children and adolescents. Despite multimodal therapy, patients with metastatic sarcoma have a poor prognosis, emphasizing a need for more effective treatment. We have shown previously that 2-methoxyestradiol (2-ME), an antitumoral compound, induces apoptosis in Ewing sarcoma cells through c-Jun NH2-terminal kinase (JNK) activation. In the present study, we provide evidence that 2-ME elicits macroautophagy, a process that participates in apoptotic responses, in a JNK-dependent manner, in Ewing sarcoma and osteosarcoma cells. ...
Source: Cell Research - August 24, 2009 Category: Cytology Authors: Lorin S, Borges A, Dos Santos LR, Souquère S, Pierron G, Ryan KM, Codogno P, Djavaheri-Mergny M Tags: Cancer Res Source Type: journals
Prognostic relevance of CCN3 in Ewing sarcoma
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In this study, we assessed the prognostic value of CCN3 (Nov), a matricellular protein that play crucial roles in bone formation. Polyclonal antibodies directed against each of the different CCN3 modules were used to identify variant CCN3 proteins in tumors and to draw potential relationships between the expression of these variants and the outcome of patients with Ewing sarcoma. Our results confirmed that expression of the full-length CCN3 in Ewing sarcoma is associated to a worse prognostic. Furthermore, we report a possible relationship between the expression of a CCN3 protein lacking an internal module (von Willebrand ...
Source: Human Pathology - August 19, 2009 Category: Pathology Authors: Bernard Perbal, Noureddine Lazar, Diana Zambelli, Jose Antonio Lopez-Guerrero, Antonio Llombart-Bosch, Katia Scotlandi, Piero Picci Tags: Original Contributions Source Type: journals
Extraosseous Ewing sarcoma of the thyroid gland
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We report a case of extraosseous Ewing sarcoma
in the thyroid gland in a 9-year-old girl.
Content Type Journal ArticleCategory Case ReportDOI 10.1007/s00247-009-1388-1Authors
Pavani Adapa, Texas Children’s Hospital and Baylor College of Medicine E.B. Singleton Department of Diagnostic Imaging Houston TX USATae-Woong Chung, Texas Children’s Hospital and Baylor College of Medicine E.B. Singleton Department of Diagnostic Imaging Houston TX USAEdwina J. Popek, Texas Children’s Hospital and Baylor College of Medicine Department of Pathology Houston TX USAJill V. Hunter, Texas Children’s Hospital and Baylor College...
Source: Pediatric Radiology - August 19, 2009 Category: Radiology Tags: Pediatric Radiology Source Type: journals
[Askin tumor: A case report with literature review.]
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We report a case of Askin tumor in a 5-year-old child with reviewing the different data from the literature.
PMID: 19692278 [PubMed - as supplied by publisher] (Source: Cancer Radiotherapie)
Source: Cancer Radiotherapie - August 16, 2009 Category: Cancer & Oncology Authors: Tazi I, Zafad S, Madani A, Harif M, Quessar A, Benchekroun S Tags: Cancer Radiother Source Type: journals
Benefits of molecular pathology in the diagnosis of musculoskeletal disease
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Abstract The second part of this review, on the benefits of molecular pathology in the diagnosis disease, focuses on the genetics of
bone tumors and metabolic disease. Unlike soft tissue tumors, the number of currently exploitable molecular abnormalities
for diagnosing bone neoplasms is small, although the same gene rearrangements are found in primitive neuroectodermal tumor/Ewing
sarcoma in both skeletal and extraskeletal sites. Compared with soft tissue tumors, genetic abnormalities, which are valuable
to diagnosticians in skeletal disease, are often germline and post-zygotic aberrations rather than somati...
Source: Skeletal Radiology - August 10, 2009 Category: Radiology Tags: Skeletal Radiology Source Type: journals
Compressive Osseointegration of Tibial Implants in Primary Cancer Reconstruction.
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Compressive osseointegration technology, which provides immediate, mechanically compliant endoprosthetic fixation, has been adapted for massive proximal tibial reconstructions in an attempt to avoid aseptic failure encountered with conventional stems. A retrospective review of 16 patients with resected tumors was undertaken to determine whether compressive osseointegration can provide durable anchorage of tibial implants. Medical records, radiographs, and clinical examinations were reviewed to assess surgical, local disease control, and prosthetic outcomes. The average age was 18 years (range, 12-42 years). Diagnoses i...
Source: Clinical Orthopaedics and Related Research - August 3, 2009 Category: Orthopaedics Authors: O'Donnell RJ Tags: Clin Orthop Relat Res Source Type: journals
Primary vulvar Ewing's sarcoma/primitive neuroectodermal tumor in a post-menopausal woman: A case report.
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We describe the case of a 52-year-old woman who was admitted to our hospital for the local excision of a 4cm vulvar mass, originally thought to be a Bartholin's gland cyst. It was subsequently found to consist of small round cells positive for anti-CD99 antibody, thus suggesting a diagnosis of ES/pPNET. The demonstration of EWSR1 gene translocations by means of fluorescent in situ hybridization excluded small-cell carcinoma, squamous cell carcinoma of the small type, Merkel cell carcinoma, and lymphoblastic lymphoma. After surgery, the patient received six cycles of polychemotherapy and radiotherapy; she is still alive and...
Source: Pathology, Research and Practice - August 2, 2009 Category: Pathology Authors: Boldorini R, Riboni F, Cristina S, Allegrini S, Valentini S, Muscarà M, Ruspa G Tags: Pathol Res Pract Source Type: journals
[Pulmonary Ewing Sarcoma/Primitive Neuroectodermal Tumor: A Case Report and a Review of the Literature.]
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We present the case of a 22-year-old woman with Ewing sarcoma/PNET diagnosed following histologic, immunohistochemical, and in situ hybridization studies of a bronchial biopsy specimen. Radiography, ventilation-perfusion scintigraphy, and a bone marrow biopsy confirmed that the tumor was not metastatic. The patient was started on a chemotherapy regimen of vincristine, actinomycin, cyclophosphamide, doxorubicin, ifosfamide, and etoposide and responded well. She is now being seen regularly at our outpatient clinic.
PMID: 19656607 [PubMed - as supplied by publisher] (Source: Archivos de Bronconeumologia)
Source: Archivos de Bronconeumologia - August 2, 2009 Category: Respiratory Medicine Authors: Suárez Antelo J, Rodríguez García C, Montero Martínez C, Verea Hernando H Tags: Arch Bronconeumol Source Type: journals
Undifferentiated sarcoma: does it exist? A clinicopathologic study of 7 pediatric cases and review of literature
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Summary: Undifferentiated sarcomas are primitive mesenchymal tumors that cannot be classified among standardized histopathologic entities. Whether they represent a homogeneous group with common histogenesis and clinical behavior or comprise a variety of tumors able to differentiate along specific maturative lineages is still debated. To identify prognostic and histogenetic markers, we analyzed 7 undifferentiated sarcomas (4 in the trunk and 3 in the extremities). Mean patient age was 7.5 years, and mean follow-up was 18 months. Two clinicopathologic subtypes emerged from this study. Four primitive mesenchymal undifferentia...
Source: Human Pathology - August 2, 2009 Category: Pathology Authors: Rita Alaggio, Gianni Bisogno, Antonio Rosato, Vito Ninfo, Cheryl M. Coffin Tags: Original Contributions Source Type: journals
Sarcomas With Spindle Cell Morphology
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In the days before the term “high-grade undifferentiated pleomorphic sarcoma” came into use, one of the most common sarcoma diagnoses was “malignant fibrous histiocytoma,” and before that, in an era before immunohistochemistry, “fibrosarcoma” was used to describe most sarcomas. “Spindle cell” is a descriptive phrase that denotes the cellular shape of many of the sarcomas encountered in the adult population. As a result, they are usually treated differently from small round cell sarcomas, and have different biological characteristics than those tumors and sarcomas with epithelioid morphology. As a very broad...
Source: Seminars in Oncology - July 31, 2009 Category: Cancer & Oncology Authors: Paola Collini, Poul H.B. Sorensen, Shreyaskumar Patel, Jean-Yves Blay, Rolf D. Issels, Robert G. Maki, Mikael Eriksson, Xavier Garcia del Muro Source Type: journals
GSTM4 is a microsatellite-containing EWS/FLI target involved in Ewing's sarcoma oncogenesis and therapeutic resistance
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GSTM4 is a microsatellite-containing EWS/FLI target involved in Ewing's sarcoma oncogenesis and therapeutic resistance
Oncogene advance online publication, August 31, 2009. doi:10.1038/onc.2009.262
Authors: W Luo, K Gangwal, S Sankar, K M Boucher, D Thomas
& S L Lessnick (Source: Oncogene)
Source: Oncogene - July 30, 2009 Category: Cancer & Oncology Authors: W LuoK GangwalS SankarK M BoucherD ThomasS L Lessnick Source Type: journals
Aberrant methylation and reduced expression of RASSF1A in Ewing sarcoma
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The objective of this study was to analyze RASSF1A methylation and assess its clinical significance in ES.The methylation of RASSF1A was determined 31 ES tumor samples and 4 ES cell lines. ES cell lines were also treated with demethylating agent 5-aza-2[prime]-deoxycytidine to ascertain its effect on methylation. RASSF1A expression was studied in 12 ES tumors. The association between RASSF1A methylation, clinical parameters and outcome was also analyzed.Methylation of RASSF1A was observed in 21/31 (68%) tumors and in 3/4 ES cell lines. A significant correlation of methylation to reduced expression of RASSF1A was observed i...
Source: Pediatric Blood and Cancer - July 26, 2009 Category: Cancer & Oncology Authors: Smadar Avigad, Shruti Shukla, Inna Naumov, Ian J. Cohen, Shifra Ash, Isaac Meller, Yehuda Kollender, Josephine Issakov, Isaac Yaniv Source Type: journals
Irinotecan and temozolomide for Ewing sarcoma: The Memorial Sloan-Kettering experience
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The prognosis for recurrent/progressive Ewing sarcoma (ES) remains poor. Pre-clinical, adult phase I and II trials have demonstrated the combination of irinotecan and temozolomide to have schedule-dependent synergy and significant antitumor activity. A pediatric phase I trial has shown this regimen to be safe and active in advanced ES.We conducted a retrospective chart review to identify patients with recurrent/progressive ES treated with irinotecan [20 mg/m2/day × 5(×2)] and temozolomide (100 mg/m2/day × 5) in our institution. The best response achieved, time to progression (TTP), and associated toxicities were recorde...
Source: Pediatric Blood and Cancer - July 26, 2009 Category: Cancer & Oncology Authors: Denise A. Casey, Leonard H. Wexler, Melinda S. Merchant, Alexander J. Chou, Pamela R. Merola, Anita P. Price, Paul A. Meyers Source Type: journals
Epidemiology of bone tumours in children and young adults
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Although the epidemiology of malignant bone tumours in children and young adults has been explored, no definitive causation of any specific tumour has yet been identified. We performed a literature review (1970-2008) to find all papers covering possible aetiological factors involved in the development of bone tumours in children and young adults. Several associations have been reported with some consistency: the presence of hernias and Ewing sarcoma; high fluoride exposure and osteosarcoma; and parental farming and residence on a farm, younger age at puberty and family history of cancer for all bone tumours, especially ost...
Source: Pediatric Blood and Cancer - July 16, 2009 Category: Cancer & Oncology Authors: Rachel Eyre, Richard G. Feltbower, Emmanuel Mubwandarikwa, Tim O.B. Eden, Richard J.Q. McNally Source Type: journals
Caveolin-1 promotes resistance to chemotherapy-induced apoptosis in Ewing's sarcoma cells by modulating PKC[alpha] phosphorylation
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This report shows that CAV1 expression determines the sensitivity of ESFT cells to clinically relevant chemotherapeutic agents. Analyses of endogenous CAV1 levels in several ESFT cells and ectopic CAV1 expression into ESFT cells expressing low endogenous CAV1 showed that the higher the CAV1 levels, the greater their resistance to drug treatment. Moreover, results from antisense- and shRNA-mediated gene expression knockdown and protein re-expression experiments demonstrated that CAV1 increases the resistance of ESFT cells to doxorubicin (Dox)- and cisplatin (Cp)-induced apoptosis by a mechanism involving the activating phos...
Source: International Journal of Cancer - July 15, 2009 Category: Cancer & Oncology Authors: Oscar M. Tirado, Caitlin M. MacCarthy, Naheed Fatima, Joaquín Villar, Silvia Mateo-Lozano, Vicente Notario Source Type: journals
